JOURNEY TO US

Quick Updates

2012-02-06T18:25:00.001-06:00

It's been a long day.
Eli's new nutritionist called. She has been working with Eli's Geneticist/Mito doc, as well a specialized GI. They have been discussing all of Eli's blood sugar and feeding issues. They are concerned that he's impacted and are ordering a KUB. Then they are wanting to work out a plan for dealing with bowel issues. Next they are wanting to increase his fluids by another 80ml's a day because of the increased calories from the cornstarch. Right now Eli's free water is at 273ml's a day, and that's on top of his 975ml's of elecare a day. So if we add another 80 ml's that's 353ml's of water for a total of 1328 ml's a day of fluid. That's 44.26 oz a day. Mind you I would need to do the 353 ml's of free water during 4 hrs off the pump. She's taking away his 1/2 hr extra off the pump and putting it back to calories. That's the end of the line. The 4 hrs he's off the pump (2 in the morning and 2 in the evening) are required to get medication done that can't be during a feed. After his surgery they have also decided that they want to change his formula. They want him on a more complete protein formula. She wants to switch him over to peptamen jr w/fiber. Apparently this is normally only used for G tube feedings but they think by adding in the fiber it will be a good fit for Eli's J tube. This change will be made very slowly over time. We will still need to add in the extra salts, iron, vitamins, and cornstarch. We are overwhelmed with all of the changes and concerned on how his system will handle this much drastic change. Working with a J tube, and a system of sensitive as Eli's changes this large can be catastrophic. In the next few weeks they will be running alot of lab work and preparing a full detailed plan. In the mean time we will again work to increase his fluids, but we seem to be pretty close to our peak.
Eli's been pretty good. He's been busy during the day practicing alot with his walker. He's also been working alot on his signing. He's learned how to sign "help" and now uses it for everything! He's been pretty exhausted making for a decent bit of O2 sat drops during nights and naps so the O2 has been very helpful during those times.

Emily is preparing for her all district 5th grade honor choir concert at the end of the week. She's super excited! She's been going thru a little bit of a struggle, working to figure out who SHE is and what SHE thinks and feels. We are confident she will figure it all out!

Jace has been busy as always outdoors. He spends as much time as possible outside with his friends!

Benjamin has been a disaster. He stole an Ipod from one of his classmates, and then his teacher caught him with some stolen items in his desk today. He's back to swiping everything and nothing anyone is saying/doing is making any difference. He's currently grounded from all electronics and that's a rough one for him. We are hoping to get our point across!

Ariana is preparing for her 5th bday! She's having her birthday party this weekend and is planning what colored sprinkles she wants for her cupcakes. She's still loving school. She's currently dancing around the living room to pandora!
Ari had her appt with the renal clinic last week. They did some testing and asked her alot of questions. They have determined that she has an overactive bladder and that it is causing alot of her issues. They are going to try her on a medication to help lessen some of the impulses and urges that her bladder is constantly sending. They will recheck in 3 months and if no change they will begin deeper testing!

As a family we have spent the last 2 sundays checking out a new church in our area, Shawnee Mission UU church. We have LOVED it! We all have really enjoyed the services and classes and have met some really neat people. We all enjoy the openness and accepting atmosphere and are excited to go back next week!

I will update more in the next few days as we get more information and test dates and results!

Quick General Update

2012-02-02T12:33:00.000-06:00

It's been a quiet few days on the doctor front. We have kept in close contact with Eli's medically complex doc and have spoken with the geneticist's dietitian multiple times but other than that not much. The dietitian is working hard to come up with the perfect feeding and fluid schedule for Eli. That's tough to do taking into account all of the issues that are going on with him. She's not rushing into any changes or decisions so we are patiently waiting for her final recommendations. Eli is up to 2 teaspoons of cornstarch a day mixed into feeds, and today we will trial out 3 teaspoons. So far his GI system seems to be handling it a bit better and this morning was one of his highest blood sugars in quite some time. He has an issue going on with a lymph node in his groin that we are keeping a close eye on. He also seems to be currently having a flare up of his skin conditions and we are working to keep that under control.
We are laying low this month and allowing his body to rest as much as possible. As of right now he is rescheduled for surgery on March 6th. We are hoping to keep life as calm as possible for him between now and then. Also hoping to get the feeds and fluids and sugars nice and stable by that point as well.
We have just finished up training a nurse and we are currently working to train another nurse. We are going to end up having to have 3 nurses to get our weeks schedule covered. They are working to find a 3rd nurse and we will see what we think tomorrow when we possibly meet an option.

The older 4 are doing pretty good. Emily is staying busy. She's been hanging out after school with a friend next door and has really enjoyed getting to know her. She's getting prepared for her honor choir concert next week and she is also making her final selections for her song for the talent show.
Jace has been busy after school with a friend from next door who is also in his class. They spend almost every afternoon together.
Benjamin has been struggling. He's apparently been staying up half the night playing his kindle fire and nintendo and as a result of this he's incredibly grouchy and having trouble staying awake at school. He's gotten into alot of trouble in class, and ended up going to the nurses office yesterday to sleep. We keep taking his things away and then he just ends up stealing one of the other kids items. It's a challenge.
Ariana is doing good. She's getting ready for her 5th birthday! She's super excited to be turning 5 and is happily planning what special treat that she wants to take to school with her. She's also making her birthday present lists and WOW. She wants a real live pink puppy, a unicorn with wings, and the perfect dress to marry her king. :)
Other than that not much new. I will get some more updates up soon.

Doc called

2012-01-25T19:51:00.000-06:00

I know I know 2 posts in one day. Super fancy. BUT that being said this post is important and so I wanted to give it it's own post.
Eli's geneticist/Mito doc just called. We chatted for about 20 minutes. Eli's lab work showed ok on hydration and kidney function BUT his CoQ10 levels were definitely low. He wants Eli on a supplement TID (which is 3 times a day). We also discussed Eli's weight loss and frequent rounds of dehydration. We also discussed the blood sugars and the problems we are having with the cornstarch. He's having us adjust Eli's cornstarch to be continuous mixed in with his feeds. He's hoping this will help some of the GI discomfort as well as help keep his sugars a bit more stable. If this doesn't work we'll move to the emergency shot.
Lastly he discussed the possibility of getting permanent access again to allow for TPN and D10. This will be our next option. Between the sugars, lack of growth, hydration issues, and the sensitivity of his GI system we may end up with no choice. A line for Eli brings up ALOT of risks. Eli is more prone to infections because of his weakened immune system. He also has a blood clotting disorder so a line obviously puts him at a much higher risk of a clot. He would have to be on blood thinners the entire time he had the line (which it would be permanent) and he would require frequent ECHO's to keep an eye on his heart.
We are going to try the changes with cornstarch for the next few days, as well as chat with the dietitian to see if there is anything differently we can do with the feeds. His doc is afraid we are close to max on calorie concentration and feed rate for Eli's GI system but it's worth trying some basic changes.
He and I will check in here in a few days to see how it's going and determine what's next.
These aren't decisions we are taking lightly but like always we are low on choices.

Super fast Update but mostly Pics...FINALLY :)

2012-01-25T18:53:00.000-06:00

Eli's heart rate and resps have been running high today. He had a fever this morning and was pretty tired but by this evening the fever was gone but ended up needing back on O2 to help control his sats. Hoping for a quiet evening but holding my breath.

Here are some pics!

Super Quick Update

2012-01-24T19:55:00.000-06:00

It has been a fairly slow few days.
The weekend was a bit rough, as they boys were in a mood. It makes for a hard few days.
Monday was NICE. My friend picked me up and we spent 4 or 5 hrs going to tons of thrift stores digging and searching for treasures! I found some cool things and had a great time getting out and about!
Today has been a bit off. Eli had a weird night and the day has continued that way. His heart rate has been high, and his GI system is still very thrown off. His sugars have been decent today but he's flushed and exhausted. He napped over 4 hrs and has still been tired this evening. I have a call into his Geneticist/Mito doc and hoping to get some more info from him. Eli also lost weight again, which simply doesn't make sense. I will update as soon as I hear back from the doc!

Emily found out at the end of last week that she was chosen to be in the 5th Grade All District Honors Choir! She will be performing at a concert at and all district assembly the beginning of February. She's super excited and we are all excited for her!

Not much is going on with everyone else. It's not been a very info heavy few days!
We are still working on training a nurse, although she is struggling a bit with the stairs and the floor. We still don't have other prospects on nurses. It leaves us pretty close to home right now!

In closing...Comments on my blog are all moderated. I have a few readers who seem to have nothing better to do with their time but harass me. Because of this all comments have to be approved before they are posted. If you sign your name to your comment, it has a higher chance of being published.
Lastly...If you think you can do it better, or if you disagree with how we are doing something, or if you think that we are making things up, feel free to shoot me an email or give me a call and I would be more than happy to set up a weekend for you to come stay with us and follow us around participating in EVERY aspect of our lives. We will then see how easy you believe it all to be.
We 120% appreciate all of the amazing people in our lives and all of the support that we receive from you guys! You all are amazing!

Complicated. It's all so complicated.

2012-01-18T18:58:00.001-06:00

It's been a rough week. We were training a brand new nurse and after 45 hrs she quit because Eli was to complicated. Then this week Eli's other nurse quit because she's moving to florida to get away from a bad relationship. So we ended up with no nurses.
Then to top it off Eli had a rough week. He started with alot of respiratory issues and needing alot of extra breathing treatments and CPT to get thru it all. Then the fevers started. Then for a day or two he almost seemed like he was getting better.
Then this morning his sugars started dumping fast and we kept doing his emergency meds and they just kept dropping. They ended up dropping over 30points in 40 minutes. We spent a whole lot of time on the phone with his doctor getting orders and constantly adjusting the care plan. Eli has been on continuous feeds and has ended up needing 6 rounds of emergency treatments and he's still barely above his lowest limit for sugars this evening. His GI system is also very upset by it all and he's having diapers multiple times an hr which is making it even harder to keep up with his sugars and hydration. His doctor has been amazing today at calling me frequently to keep up with everything going on.
This afternoon Eli had an appt with his neurologist. We discussed the grand mal seizure and Eli's neurological state in general. He believes we will see even more seizure activity with each illness and that there aren't medication options to keep that from occurring. He also discussed Eli's therapies and where he was with his growth and advancement. He informed me that he thinks with Eli's "severe neurological and cognitive delays and impairments that we will need to make a choice at some point if we continue aggressive therapy." He doesn't believe that Eli will learn these skills and he says by the age of 3 or 4 we will definitely need to be ready to stop intensive therapy and pushing. I was very offended by all of this and will be requesting a new neurologist. I don't think there is ever a point to stop providing therapy and a chance for him to learn. It's not a medication with tons of horrible side effects. It's simply therapy. I don't see what it can do to harm him, only help him.
Lastly Eli saw his optometrist. Eli's eyes have had a slight improvement with consistent use of his glasses. We discussed the irritation Eli is having with the patch so he has decided for now to hold off on using the patch and to continue with using his glasses during all the waking hrs.
Right now we are doing everything we can to try to keep Eli as stable as possible. We are working round the clock to keep his respiration and heart rate down, his O2 up, and his sugars up. He has lost 1 1/2 lbs in the last 2 weeks and is constantly bordering on dehydration. The doctors have no idea why this is all happening or what to do about it all.

In other kids news.
They all had doc appts this week.
Emily ~ She's up to 5ft 3 in. The doc ran some lab work to check on some issues she had been having and the tests all came back OK on that. They are going to watch for 2 months and check back in. They also did some lung function tests. The first go around showed some issues, but after the breathing treatment she did much better on the 2nd go around. They are changing around some of her meds and hoping to get her at a better point with her asthma. She also showed some struggles with the vision test so she meets with the optometrist on Friday. We also talked about Emily's sleep walking and they are referring her to sleep clinic for a full evaluation.

Jace ~ He's up to 4ft 6in. The doc said he's looking pretty good. She's happy where he is with all of his stomach meds and is leaving all that to be followed by Jace's GI doc. She didn't currently have any concerns with Jace!

Benjamin ~ He's up to 4ft. The doc looked him over and all looked good till she got to his ears. All she could see in his left ear canal was green. After much work it was determined that Ben had a large green eraser stuffed all the way into his ear canal. She was finally able to remove it and it doesn't appear like any permanent damage has occurred. Other than that Ben looked pretty good as well. He's still definitely under weight but has gained some so we will continue with what we are currently doing.

Ariana ~ She's up to 3ft 8in. She was the roughest appt. First we went over her hand issues and she has been referred to the OT dept. She is also being referred to the speech dept for her trouble with making certain sounds. Then we discussed Ari's urinary issues. The doctor agreed that the cluster peeing and not being able to fully empty her bladder was a concern. She ran some tests (blood and urine). It looks like Ari might have a UTI and that she also has an abnormally high PH level in her urine. The UTI doesn't explain away the other issues as they have been going on for a long time but it is obviously something that needs fixed. She is referring Ari to the renal clinic for a full work up. Ari also failed her vision test and will be seeing the optometrist on Friday with Emily.

Bob and I like always are exhausted. Earlier in the week I cried, really cried for the first time in a long time. Everything just hit me and the emotions were overwhelming. At this point I feel numb. I keep hearing doctors say there is nothing more we can do, it's the mito, etc.... Eli's medical care is at the point of simply waiting for his next crisis. There is no more trying to fix it, very minimal prevention, it's simply waiting and planning for the next crisis. Nobody worries about baseline anymore, we don't shoot for baseline. We just take slightly stable and run with it. Every morning I'm scared to find out what that day will medically bring. I can never quite relax, already ready to jump up when the next beep happens. Every time my phone rings I'm wondering which doctor it is and already getting all the information straight in my mind, knowing exactly what they want to know. I feel like I'm always planning for the next crisis. I can't really blame the doctors because we do it here at home as well. It's always right around the corner. I don't know how much longer my nerves can handle it. We just float thru the middle point planning and organizing and waiting. The waiting is the worst.
Every special time and moment has been taken from us. It's simply going thru the motions for the family. I can't remember the last time we were able to celebrate a holiday. Eli has been sick, and by sick I mean very sick and unstable for each and everyone. This last monday was Bob and I's first anniversary. It was a horrible day. Our nurse called in, Eli was very sick, and the day was a mess. All I could think about is how I felt as though every special moment had been stolen from us. It's very depressing and hard to stay positive.
Most days are simply going thru the motions of it all. Numb is where I try to remain, but that being said real emotions sneak in there sometimes no matter how hard I try to hold them off.
I apologize to all those I'm ignoring right now. I'm not purposefully doing it, well mostly not. I'm depressed and tired and exhausted and honestly grumpy. I feel horrible putting that burden on my friends. I know most all of you are going thru so many of the same things, and I'm assuming many of the same emotions. I don't want to drag my medical friends deeper down into their own struggles and battles....and for my non medical friends...I know you don't understand and that's ok. You ask how Eli is, not really wanting to know the answer. Eli scares you and I get that. Eli scares me sometimes too. I want to talk to you about non-medical stuff, I want to know about your life, I want to talk about the latest gossip, but for some reason everyone seems scared to talk about those things with me. I NEED those conversations. I need life outside of this medical vortex.
I know I'm being a horrible friend to all of you out there and I want to change it I really do, but I'm so exhausted and blah...It's just not easy. It's never easy anymore.
I guess enough rambling for today. Congrats if you made it all the way thru all of that! :)
I guess what I was trying to get at is be a pain, reach out to me, bug the heck out of me, show up at my house unannounced to chat or drag me out to a movie. Call me and complain about your boss, or you kids, or your childs doctor. Don't be afraid to reach out, it's ok.
love to all.

Geneticist / Mito Doc

2012-01-12T19:12:00.000-06:00

We met with Eli's geneticist and Mito doc today. We spent a little over 2 hrs there.
1. Blood Sugars. He agrees that this is a huge issue that needs to be immediately gotten under control. He agrees that we need to try one more option before our final 2 options. Right now we are going to try water and cornstarch. He's hoping that it will be enough to help increase his sugars enough to protect from these big hypoglycemic drops. If this doesn't work we are left with 2 options. If this doesn't work we will discuss Glucagon injections, and if that doesn't work we will be discussing (AGAIN) permanent access to allow for Iv fluids to help keep everything up.

2. Next we discussed Eli's growth and weight. He knows that Eli is in on the technical highest dose of HGH BUT we CAN go up if we need to. Currently Eli is back on a downward spiral on his weight and his still struggling to stay hydrated. He's not sure why this is happening as he is technically getting way more than what he needs in calories, fluids, and everything else. He ran some lab tests today and is going to go over everything with a fine tooth comb and see if he can come up with a better feed/fluid schedule. Again this could end up with permanent access that could allow for some IV fluids and TPN as needed.

3. We then discussed Eli's secretions and his sleep apnea. He is concerned about the size of Eli's nasal passages...this is something that has been a concern since birth. It was always assumed that they would grow and adjust. That's not happened. We can barely get a 5Fr down one side and a 6Fr down the other. He has put in a referral for plastic surgery and ENT to see about dilating the nasal passages.

4. We discussed Eli's seizures. He's not surprised that we are seeing an increase and a change in those. He warned us that the brain is a weaker point in Mito kiddos like Eli and that Eli is very susceptible to seizures and such, especially when he's body is under any kind of stress. He is in favor of Eli's seizure care being switched over to the epileptologist instead of just the neurologist, and he really likes the one that Eli has already seen.

5. There was also some discussions on a brand new clinical lab that will be opening here in KC in the next 2 or 3 months. They are almost finished with the research labs. They are testing Mitochondria fully for mutations and deletions and such. He has had 1 patient go thru the research piece and they were able to get the last piece of the puzzle and he strongly encourages us to finish this testing with Eli as well. We will go ahead with it, although we don't know how many more diagnosis' we can handle.

6. Lastly we discussed Eli's critical care plans. I voiced my concerns over doctors giving up because of the Mito diagnosis. He discussed end of life, terminal care, and agrees that we aren't at that point and at this point it simply isn't fair to deny Eli ANY medical helps that can possibly help him medically and quality of life wise. He did forewarn that there will come a point where we will need to start questioning EVERY single intervention and picking what is truly "worth" doing, but he strongly believes that we aren't there yet. This was an awkward conversation to have. We know the in's and out's of Mito. We know the statistics. We know what can/will/could happen. These are obviously things that Bob and I have discussed quietly...privately. That being said when a doctor openly discusses these things it's scary and comforting all at the same time. He is going to put together a FULL critical care plan so there shouldn't be as much question as to what we need to do in situations where Eli is struggling.
He mentioned to me that it is hard to expect 1 doctor to know everything about a child as complex as Eli. I told him I understand that, and that Eli has 36 doctors, and I looked him right in the eyes and said..."In the same turn it is hard to expect 2 parents with NO medical training to know everything about what to do." He looked right at me, and quietly responded "We can't be putting this on you, we will get it done." I sure hope he's right. Bob and I can't continue to guess and struggle thru each and every day, it's not fair to Eli. On a slightly funny side note, his geneticist/Mito doctor informed me with a small laugh, that he can see why some are afraid of Eli's complexities, he said that honestly he is too! :)

He has decided to follow Eli every 3 months. He says he normally follows his patients yearly when they are stable, but believes that every 3 months is best for Eli. We tend to agree.

Eli had a rough day with the temps and being out and about. He couldn't handle sitting up in his wheelchair that long and got very tired and droopy. The afternoon brought a lot of napping, and this evening a lot of laying around. Tomorrow is a slow day at home, which will help him catch up a touch.
Monday brings his new cough assist and all of the training involved with that. Next week also brings a recheck with his eye doctor and his neurologist.
I will get another update up in the next few days. Maybe I can even get organized and get some pictures up too! :)

Wow I'm behind

2012-01-11T18:38:00.000-06:00

Holy cow I didn't realize it had been so long since I had updated. It's been just one huge blur, which seems to be the story of my life lately.
Eli has been home from the hospital for a week now. We pushed and he was able to come home because we have such a medical setup here and he has nursing. He did decent after getting home, although he struggled alot with his blood sugar.
We met with Eli's new medically complex doctor 48hrs after he was discharged. They ended up needin to change all of his feeds and his fluids. His blood sugars are dropping quickly while he's off feeds, and he is still running dehydrated. So he is now on more calories and carbs, and his water has been increased. He also is not getting as much time off the pump. He gets 2 hrs in the morning and he's done decent with maintaining his blood sugar. He also gets 2 1/2 hrs at night and he is doing horrible keeping his sugars up. He has yet to actually get that whole time off the pump. We have had to put his emergency sugars plan into effect every night. We are also still seeing many signs of being a bit "dry" and are working to combat that.
He has had some wheezing issues again as well and had to go back to his complex doc. He made a bit better management plan and has also decided that we are going to ultimately need to provide Eli more support to help him conserve energy and properly handle clearing secretions so they don't settle into his lungs. He has decided to increase Eli's rescue nebulizer treatments, as well as his CPT and he has also placed an order for Eli to have a Cough Assist machine. Hopefully if we can help Eli cough and clear everything a bit better on his own, we can provide a bit of extra protection for his lungs.
Eli is still struggling with not being on O2. We had a day or 2 where he did well, and then right back to it. We are seeing his O2 sats drop and his heartrate and resps shoot right back up the moment he takes his O2 off.
We have also learned this week that Eli's endocrinologist is no longer able to manage Eli's care when it comes to his hypoglycemia. He believes it is related to the Mito and is unsure what needs to be done to help stabilize it all. He is referring Eli back to his geneticist/Mito doctor to deal with all of it.
Eli also had another nurse quit this week. Eli was yet again to complicated. We are unsure of how to prevent this from occurring so frequently. We are 110% upfront and honest with the nurses when they interview. We go over every drop of his medical care. They are again looking for a new nurse to help cover 40hrs a week. We are changing around some of the requirements, and hoping that helps weed out some of the problems a bit earlier on in the process.
Eli meets with his geneticist tomorrow. They were able to get him a rush appt. He will meet with his eye doctor and his neurologist next week as well. We are working to get back to a new "baseline" and to figure out where we go from here.
With each illness we are learning new things about Eli, and learning of new problems. We have definitely learned that Eli's lungs are by far his weakest point, and for Eli they seem to be the hardest thing to heal. Each illness is placing a stronger and stronger hold on his lungs and we are struggling to get back to stable, let alone baseline.
We have already informed Eli's new complex doctor that in a few weeks we would like to have a very serious sit down talk and discuss where we are, where we go from here, what we start, what we stop, what we change. We feel so many times that Eli's doctors all tip-toe around alot of subjects that we all know are right there needing discussed. We are hoping that his new primary doctor will be able to openly honestly handle these types of discussions.

Where the rest of us are...
Bob and I are exhausted. Currently Eli only has 36 if his 84 hrs a week covered. This means that Bob and I are 100% in charge of his care 132 hrs a week. It's exhausting and Eli is continuing to be more and more work each day. We are constantly reassessing and making changes. It's a games of numbers and we are always re-figuring and just trying to keep up.

Emily ~ She's busy as always with school. She is involved in her 2nd book club of the year, still in choir and strings and student counsel as well. To top it all off she has decided to audition for the school talent show and is working on picking the perfect song! She's hitting the age where she is becoming boy crazed and that's always fun for Bob and I to hear all about!

Jace ~ He's doing pretty well with school. He made some advancements on his last report card as well on his IEP speech goals. Some things are still proving to be quite the challenge. Assessments are coming up and this will be the first time that his IEP accommodation will be in place. We are really hoping that will help Jace out and allow him to feel more comfortable witht he testing. He's still enjoying school and making new friends. He won an award this month with this school and we were all super excited about that!

Benjamin ~ He's doing decent in school. Like always still struggling on many things. He has moved into the class clown role as he's learned that he can make his classmates laugh and he enjoys that interaction. That combined with his constant moving and spinning and pacing has gotten him in trouble multiple times lately. He's still getting all of his IEP accommodation and is making VERY slow progress towards reaching his goals. At home he still stays super busy on his kindle fire or the nintendo or Wii.

Ariana ~ She's turning into such a stubborn little lady. She's girly as could be but beyond rough and tough. Just last week she used Emily's razor to "practice" shaving like Emily and Bob. She shaved all of the skin right off her chin. Then 2 days later she got a huge splinter in the palm of her hand. She makes it thru all of these events with virtually no tears and her hair perfectly in pig tails or braids. It's interesting to watch. She is loving school and doing well. She has learned almost all of her ABC's and now knows her numbers up to 13! She knows all of her shapes and colors!

I think that sums us all up for now. I will get on tomorrow after the geneticist and update. By that point I should hopefully have some more detailed information on where we go for here.

We are frequently asked for specific things that people can send thoughts or prayers for...
1. For Eli. He is my little fighter and I'm constantly amazed by his strength. That being said his lungs are really slowing him down. He's struggling with keeping up with what he wants to do and it's really interfering with his desire to play. He ends up needing to take frequent breaks and have others help him do some of the things he would normally on his own. He so badly wants to be a normal 2 yr old and play and his lungs are making that impossible.

2. For Eli's doctors and nurses. We are losing so many people to the complexities of our little man. We need a strong team that is as strong a fighter as our little man. We need them to be able to make good decisions that balance Eli body AND Eli's quality of life. We also need them to communicate with us openly and honestly and clearly.

3. For us. Bob and I struggle each day to just get thru it. We are lonely, so lonely. We feel isolated and honestly very depressed quite often. It is such a struggle caring for a little one like Eli. It is literally taking EVERYTHING that we have. It is also tough for the oldest 4. They understand only pieces of it, and it can be scary for them. They do such an amazing job staying strong for our little man and they shower him with so much love it's overwhelming to watch. Their connection with him is intense.

4. Lastly and VERY importantly we ask for thoughts and prayers for all of the other families out there. I know that you are here reading our journey BUT please never forget that there are families on similar journeys. There are other little warriors out there fighting thru each and every day. There are other exhausted parents out there fighting for what is best for their kiddos, fighting to make it thru each day. Please please please never forget that there is a whole community of us, a whole community fighting thru each and every day, leaning on each other, sticking together as one big unconventional family held together by this horrible disease of Mito.

New Years Day Updates

2012-01-01T09:32:00.000-06:00

Sorry we are so behind on updates here. Alot has happened over these last few days and Bob and I have had to process it all.
When we got to the ER monday, Eli was started choking at the triage desk. I asked for suction and they couldn't find it. So they decided to have a CNA rush us back to a room. 3 steps down the hall is when the bleeding started. She took us to the room and stood there frozen. I kept yelling at her to get me suction and a doctor. At this point Eli was choking and changing colors and blood was pouring out of his nose, mouth, and stomach. Bob and I had 1 blanket and a bulb syringe. A RN heard me yelling for help and came running in, while the CNA still stood frozen. They grabbed everything up and ran us down to the Trauma rooms. So this was issue Number 1.
Then I kept telling them they had to get D10 running because Eli wouldn't maintain his sugars for very long on his own, especially when he's sick. Doc put in orders. We were transferred from the ER upstairs to his room. I kept pushing for D10 and sugar checks. Eli started getting really shaky. I again demanded D10 and a sugar check. The care assistant said he'd never done a sugar check and stuck Eli with the big heel stick razors. Eli's sugar was 56. Finally the nurse brought D10 but it was to late. 2 minutes later Eli had a grand mal seizure. Bob yelled into the hall for help and a nurse walking by stood in the hall watching and said holy cow he's lifting off the bed. She didn't come help and ran to get a doc. The floor nurses kept saying we aren't a diabetic floor we don't know what to do. Eli was given no meds to raise his sugar. He laid there after the seizure shaking uncontrollably. Issue Number 2.
So then we had to get all of his orders fixed. It was a mess. His meds were all wrong, his feeds and fluids were jacked. The nurses had no idea what they were doing because it wasn't a neuro floor or a diabetic floor. (dumb asses it's a children's hospital you should know what you are doing.)
The residents kept screwing up his orders and his labs. When we had the CT the resident originally told me that he had some pneumonia in the right lung. Then when we got transferred to PICU we were shown the CT and talked to about it and both of Eli's lungs were full of pneumonia and there was collapse in both lungs, and the right lung had significant collapsing.
Also orders were placed for Eli to be getting his cough assist Q4, IPPB Q6, and his hypertonic saline Q8. Apparently these weren't all getting done. The RT was coming in during the night and if he was sleeping she was chosing not to do it. We ended up in the PICU because of this. He was getting so sick and so they weren't sure what was going on and decided he needed PICU. We got down there and they wanted to simply follow the orders for a few and see how he did. He immediately started improving. So he simply needed the floor orders followed, and didn't need any additional supports.
When it was time to go back out to the floor I refused the same floor we had been on. So we were sent to a new floor. As soon we got here the charge nurse called the floor supervisor complaining that they couldn't deal with this many complex kids. I was like WOW really. So then get settled and basically closed ourselves in our room.
Then we lost Eli's midline. The line they swore would last 2 weeks didn't even make it 2 full days. Not only did we lose it but Eli had streaking all the way up his leg and a large red hard spot on his groin. They were unsure if it was a clot or infection. Finally got that all fixed.
Then they couldn't draw lovenox levels. They had trouble drawing so the nurse just gave up. So we were on lovenox for days with no levels drawn. We were struggling to get any blood out of him at all and couldn't even get finger sticks. So they DC'd the lovenox finally.
Then Eli kept swelling and having weird pee issues and nobody could figure out why. Yesterday the nurse and I were trying to trouble shoot it. Come to find out they had screwed up his formula orders. They were mixing all of his formula with 28oz of pedialyte instead of water. Nobody knows why this was happening. But pedialyte and Eli don't mix. Mind you I had told them exactly what he needs for formula as well as written it all down for them 16 times by this point. Then as we were going thru the computer and every single order we found another MAJOR issue. They had been giving Eli the wrong medication. He is supposed to have 1/8 tsp of salt mixed into his days worth of formula. The resident was confused an ordered sodium chloride in a 20 ml syringe. They were giving him the whole thing. So he was getting like 10 times the sodium his little body needs. That kicked everyone into high gear. He had to have multiple sticks and alot of labs all run stat. His sodium levels weren't to the danger high but well on there was and WAY higher than when we got to the hospital.
By this point I was ready to explode. The attending were here, the hospital supervisor, charge nurses, everyone. There was a long talk over all of the mistakes that have happened since we arrived here. They admitted that they had messed up ALOT and that they had in fact made Eli sicker with some of the mistakes. I told them I didn't trust a single person here and that I was so scared that they were going to kill my child.
We spent hrs going thru every single med, formula, treatment, etc. Now the nurses are required to let me look at every med label, every formula bottle, every order in the computer, all of his MARS, etc. The residents are no longer able to change any orders and everything has to go thru me and the attending.
It's very scary. At the time we didn't know all these things were happening. It was alot of new meds and treatments and we didn't understand all of it and didn't realize that it wasn't all being done.

So currently Eli is improving, slowly. I'm spending every waking second monitoring him and all of the nurses and RT's and such. They docs are being incredibly cautious. I have been apologized to so many times I'm just going to scream if 1 more person says I'm sorry. I don't want an I'm sorry I want them to fix it.

Eli is awake more. He's still not able to sit up on his own but has been able to prop up in a tumbleform chair a few times. They have brought him a table to put in bed with him so that he can watch his Ipad and some of his Signing Times movies. He's still requiring O2 and we know immediately if he pulls his nasal canulas because he immediately drops his sats to the high 70's and low 80's. He's now getting IPPB and hypertonic saline every 6 hrs and these really seem to help him. We are on J feeds and he's tolerating them really well so far. The antibiotics are really starting to cause some issues but we are hoping to be able to keep up with that without having to get another line placed. They are holding all salts while his sodium levels try and resituate by themselves.

Bob and I are exhausted. All of the things that we have found out over the last 24 to 48 hrs have really caused us alot of concern and we needed time to absorb it all and sort it all out and to figure out where to go from there. Tomorrow we will be meeting with a special patient advocate team to file formal complaints and investigations into all of it. The doctors have already filed all of that on their end, but now we have to on our end as well.

I promise to keep up better with udpates at this point! Thank you!!

Thursday Evening Updates

2011-12-29T19:14:00.000-06:00

This morning Eli and I got to cuddle for about 30 minutes. He was fidgetty and upset so the nurse thought it would be a good opportunity to give it a try. He did pretty decent. His resps were higher and his O2 was a bit lower, but all in all he did pretty well.

This morning Eli was also a bit wheezy with more crackle in his lungs. By early afternoon his sats were hanging out in the low 90's. So they did his IPPB and decided to leave him on BiPAP to try and help inflate his lungs some more. At this point we were informed that the CT showed full on pneumonia in BOTH lungs as well as lung collapse. They decided with his increased need for O2, the fact that he was still retracting, and the sounds of his lungs that it was going to be best to move him back into a full PICU setting. So here we sit. Since being back in PICU he has done great on high flow O2 mask and hasn't required BiPAP. The intensivist came in and spent some time going over his history and all of this current illness. She understand him being a super complex kiddo and does agree that Eli can do things quickly and dramatically. That being said at the moment he's doing really well considering everything that is going on. The secretions are still super thick and super far down so that's still a major hurdle we have to get over. They are unsure of if he's at the worst of it or if it's going to continue to get worse before it gets better. So right now it's a wait and see game. It's really up to his little body.
So currently PICU is maintianing where he is at and waiting to see what he does this evening. Bob and I are beyond exhausted, especially in light of all of the last minute dramatic changes today.
We will have alot better idea of where we are in the next 24-48 hrs.
oh and on an extra side note...Eli woke for a few while here in PICU and smiled!!! :) It was the most beautiful smile ever!!!
More updates tomorrow!
In closing here is a pic of little man with his brand new sock monkey with a mito ribbon!!!

Final Cultures and Labs Bearing Answers

2011-12-29T08:05:00.000-06:00

Well we just got Eli's final cultures and labs back. Not only is Eli's right lung full of pneumonia but he also has Metapneumovirus (closely related to RSV). So not only are his lungs blocked and full of fluid but so are all of the tiny tubes that carry O2 back and forth. She said that Eli is very ill and that this isn't something he will move past quickly. She expects to see him hospital for a few more weeks. The meds we are on and all of the treatments are the best that they can do right now. They aren't at the point of leveling off yet, so we still have to reach the plateau of it all and then finally start the weaning off process. They are hoping that they can keep his lungs expanded as much as possible to help keep secretions from settling. He will continue to receive high O2 supports as well. They are hoping that the increase in feeds won't put to much pressure on his lungs and his breathing so this is something that will be watched closely.
Neuro never came yesterday so she is pushing for them to come today. She is hopeful that he will fully recover and go back to the same neurological state we were at before but that it is to early to tell. She said he is unfortunately dealt with being hypoxic and that is what is causing him pain and panic. She said that she isn't surprised that he isn't comfortable with allowing us to move him as when he's moved it's even harder for him to breath causing more hypoxia and she said he can feel that when it is occuring.
So again we are trying to absorb all of this information and hoping that we are as strong of fighters as our little man because the doctor made it very clear that we have a long road ahead.

Wednesday ( I think)

2011-12-28T15:13:00.000-06:00

days are running as a blur at this point. Here are a list of facebook posts for the day to atleast keep those people updated that aren't on facebook but do read my blog!

Morning resident rounds have finished up. Eli's right lung is sounding worse and he's wheezing and secretions are sooo sooo thick. Neurologically something is still very deeply off. Pain is controlled until he's touched or moved. Fevers still. They are calling in Eli's pulmonologist and neurologist for consults today. We appear to still be missing something, but unsure what. We will update today as we know more. All of the thoughts, prayers, texts, posts, and emails are greatly appreciated!!!! Thank you!

docs all rounded. pulm was called and they came by. Waiting on neuro. The did critical labs to check his blood gas. pulm thinks we need to back down greatly on robanul, adding a new med, and is thinking bipap full time is needed. He's concerned on how thick the secretions are. Also breathing is wearing eli out so bad. he's still dehydrated although he's getting almost double his fluid needs. we are waiting on neuro as everyone agrees he isn't neurologically normal. Also we are losing the IV so they want to put in a midline, which is like a picc line. So they are calling hemoc to discuss the risk of clots and what we need to do to prevent. Eli has opened his eyes once or twice briefly, still having alot of pain response to movements and touch, and hasn't peed since 6am.

Vascular access is getting ready to run a midline, which is like a picc line but doesnt go all the way into the chest. Lasts 1-2 weeks but can be rethreaded to make into a picc. Hemoc wants lovenox restarted. :( With the line and eli refusing repositioning because of pain we will now go on lovenox injections BID. They are starting IPPB as well and hoping that will help him some with his breathing. They have also started J feeds at 10ml/hr to see how his body will handle. Still waiting on Neuro.

Intermittent Positive Pressure Breathing (IPPB). how it was explained to us is that it is like a bipap concept where it will help push special meds and breathing treatments down into his lungs which opens things up deeper helping to get more meds in there better and can also help push out and break up some of the pneumonia. He gets his first one here in a few so I will have a way better answer after actually seeing it in action. :

Resting earlier this morning

Resting

his brand new midline in his right foot. They got it on the 1st stick!!!!

And lastly in news the oldest 4 kiddos have headed for a mini vacation in Wichita with my parents. My parents have been up here over Christmas but have taken the kids back there for a few days. The kids will enjoy a mini break away!
More updates later this evening!

Evening update

2011-12-27T20:37:00.002-06:00

Docs have made evening rounds. Some changes will happen tonight. Starting him on some new pain meds and increasing some as well. Giving him some benadryl to try and help comfort. Fever is back up (103.3), and he's retracting and wheezing some again. sugars have been stable. Still only using IV. Will trial J tube tomorrow. Also attempting to lower his O2 a bit to try and figure out how much he's requiring and how much is just being given as extra. The biggest priorities for the night is to help handle the pain. His mental status is off and he's very aggitated and uncomfortable. He is still refusing to open his eyes or have any interaction with us. Hoping to see some changes tomorrow. Oh and the ECHO came back showing that his damn blood clot is still there, small but there. Damn clot.

Early Evening Update

2011-12-27T17:45:00.000-06:00

Eli had his CT scan. They ended up having to go an LMA instead of a full intubation. He did decent. Immediately following they did his ECHO. We got back to his room and he was still having alot of trouble with breathing and secretions and such.
well the good news is he doesn't have a blood clot in his lungs. The bad news is his entire right lung has a very bad pneumonia. They are changing around his antibiotics and putting him on 2 high dose ones. They are also upping his pain meds as he's still in alot of pain, to the point we still can't hold him or touch him. He's going on 48 hrs with only 1 wet diaper. The goal for tonight is to get all the new meds going and try to get his pain a bit more under control. We are still waiting on the results of his echo.
As always we will update as soon as we know more!

Early Afternoon Update **Updated

2011-12-27T12:11:00.001-06:00

What an emotionally draining morning.
The residents did first rounds this morning and she was concerned about the changes in Eli's breathing. he is requiring more O2 and is retracting, wheezing, choking alot, and in general working very hard to breath. The team all met and spent a ton of time going over Eli.
They have decided to run every single virus panel that is available to them. They also have started a high dose IV antibiotic as they are concerned about pneumonia.
He still isn't awake or alert and we are at 24hrs of this. His fever is currently down, but unsure how long that will remain stable.
The doctors all voiced a deep concern over how drastically sick he is and how they are unsure why. they are also concerned about his heart and the pressure it is currently under. they have called his cardiologist and ordered echos and a full heart workup.
They have also spoken with his pulmonologist and are starting him on cough assist every 4 hrs as well as increasing all of his rescue breathing treatments.
They are going to be doing alot of things in a short amount of time. The doctors were very serious and very concerned with the downward decline of his respiratory status and the lack of waking and such.
He did go down to get his GJ checked and the placement is good, his intestines are just not sure if they want to properly function yet. Currently he's only on Iv fluids and meds but we will start some trials with the J tube at some point to see if we can get something pushed thru.
We are exhausted and so is Eli. We are nervous about what is happening and nervous about the doctors uncertainty and concerns.
Like always we will update more as we know more.
We appreciate all of the kind thoughts, prayers, emails, texts, and phone calls! It is so nice to know that we aren't alone and that so many people are joining together to support our little man!

Updated 12:35pm

how things change so quickly in the hospital. 103 fever. doc came in. apparently eli is the talk of the hospital. They are taking him to a CT scan to make sure he doesn't have a blood clot in his lungs. With a blood clotting disorder and a history of blood clots they think it's a good idea to check. they are hoping to make it thru the CT without any anesthesia or intubation as it will be sooo hard to wean him off afterwards with him being sick.

Eli updates

2011-12-27T04:21:00.000-06:00

Eli woke sick Christmas eve. By mid afternoon his heartrate and resps were very high and his temp was around 101.5. Called the doc and they had us take him in to the ER. They did Xrays and ran alot of labs. His lungs looked pretty good, and his stomach xrays showed alot of gas but didn't appear to be an issue either. We were sent home, thinking it was a virus and to just see how he did.
Christmas morning Eli had a 103.4 fever and was very lethargic. Still very high heart rate and resps. Doc called. He had us give a higher dose of tylenol in hopes of getting everything back under control. Eli pretty much slept the day away.
Eli woke up the day after really struggling. his temp was still pretty high and his heart rate and resps as well. But to add to it all he was retracting a bit and grunting with his breathing. By this point he could barely hold himself up. Called the docs again and they had us take him into the main campus trauma center. We walked in the front door of the ER and Eli started choking. Next thing everyone knew there was blood coming out of his nose, mouth and stomach. We are obviously rushed back to a trauma room and people came from every direction. They immediately type and crossed him and had the blood bank hold blood. Ran tons of labs and xrays. Eli had to be put on 6liters/min O2 by mask and was put on tons of monitors. By this point his heart rate was 180's and his resps were around 77. His J tube and intestines stopped working and so did his urinary system. Eli was quickly admitted and transferred to the surgical / PICU floor so he would be close to all units. He became even more unstable once in his room. His temp shot up to 104.4, heart rate and resps even higher. His blood sugar dropped fast and he had a full body grand mal seizure, followed by 2 hrs of shaking. It took a while for his blood sugar to finally stabilize. He has yet to wake and has been in quite a bit of pain. If he's touched or moved he winces and cries out. His secretions are unmanageable for him. He's not got the energy to cough and with his stomach and intestines not moving there is no where for it all to go. The docs are concerned to suction to much with the major bleeding from earlier. Eli will start choking and you can hear it all in there, and he starts panicking which makes it even worse. We are doing everything we can to help him, and trying not to move him. Within the last hr his blood pressure has dropped as well, now resting around 70/32. His O2 has also dropped some, but not wanting to turn his O2 up much more, because we don't want to dry him out to much, again after all the bleeding earlier.
The plan is to simply get thru the night so we can start investigating in the morning. They have a ton of tests ordered and need to get his main docs on board. Right now he's getting vitals every hr, a TON of tylenol, he's on IV fluids and meds, and has cold rags being constantly rotated out. He's only peed once in 24hrs, so that is being watched closely as well. There are emergency meds and emergency plans in place to deal with the blood sugars and seizures and all of that. His doc and nurse tonight are really good and have been communicating well and really staying on top of things.
bob and I are resting in shifts, with no actual sleep involved. We are exhausted and really scared. Eli has never been this sick and the docs have never been this unsure of what is happening. Please keep my little guy in your thoughts and prayers as he really needs it!
Here are 2 pics from last night.

A Normal Moment

2011-12-20T17:49:00.000-06:00

Monday evening I was working on getting Eli ready for bed. This unto itself is a LOOONG process. He was playing with some toys on the floor while I got ready to start meds. He comes over and starts playing with his fridge door. Slamming it and trying to pull it open. He grabbed it and tried to pull and I held the door so he couldn't slam it into himself. He tipped over and landed on the carpet, busting up laughing. I of course laughed back. He got up crawled over and grabbed the cup full of syringe plungers and threw it on the floor. He immediately started laughing. I laughed, picked them up, put them back, and he immediately did it again. This went on 3 or 4 times with him laughing as hard as he could each time he threw the syringes over. I of course laughed in turn. It was one of the most precious moments ever. I scooped him up and hugged him so darned hard. For Eli and I this WAS communication. This was his game. For Eli this was a total normal kid moment. It was so amazing. It was a game that Eli and I were able to simply play interacting back and forth. For us this was HUGE.
I can't wait for more experiences like this. I hope we get more, and I hope we get them soon.

Care Conference

2011-12-15T19:46:00.000-06:00

Today was Eli's care conference. We were there for a few hrs. It was probably the most exhausting meeting we have ever been to.
I honestly don't have the energy to go into all the details so I will give highlights!
1. We are going to slowly begin backing down on the robanul.
2. We are going to add a new medication that's given by nebulizer. It helps lower secretions and supposedly has less side effects.
3. No trach right now.
4. O2 can be used from 1/2 to 1 1/2 liters without needing to call the doc on prior auth. It can be used day and night.
5. Hold on the BiPAP right now as he chokes and gags so much worse because it's pushing more secretions down.
6. Lastly and most importantly. Eli's heartrate is very high and so are his respirations still. He is also consistently running a low grade temp as well as having alot of bright red spells. His docs believe he's experiencing some respiratory distress issues. They also believe the robanul is contributing some. They also believe that Eli's heart is in general struggling to keep up. It's having a hard time keeping up with pumping enough blood to his body, keeping things regulated, dealing with the respiratory distress, and all those other things. They aren't sure why this is happening, beyond the fact that Eli has this horrible fucking disease. Damn Mito. His cardiologist is concerned about Eli's body using up so many heart beats so fast, and he's concerned the long term stress this is putting on his body. He will be watching closely for signs of Cardiac Myopathy as these issues increase the risk.

Basically we are going to try a few things, change some others, and simply deal with the fact that Eli's needs are again changing. We have settled in decently to the use of full time O2, and we do definitely see improvements with it.

We are most concerned about the heart issues. There are a few organs that are obviously the scariest for us as parents and this is definitely one of them. We are concerned about the risks, and so are his doctors. We are at another brick wall, where the doctors can't fix it and they have nothing more they can test, nothing more they can try, nothing more they can do, but wait and see. These are the most frightening situations for us. As parents we want it fixed, NOW.

There was alot of emotion at the meeting, by all of us. After we left we stood in the parking garage with Eli's primary speech therapist talking and crying. She has gotten to be a close friend of our families and we value her so very much.

We came home from the meeting and immediately following I got another phone call from Emily's school. Her asthma was flaring up worse than yesterday and she had required 2 breathing treatments in less than 3 hrs. We called her doc and they said ER. We got there and her chest was hurting and she was having a decent bit of trouble breathing. They gave her steroids and changed around her entire asthma action plan. She is now on a more concentrated meds for her prevention meds and for her rescue meds. Instead of using the nebulizer she is now using inhalers with spacers to get the full dose of meds. She will also continue steroids for atleast the next 5 days. She's doing a bit better this evening but still pretty sore and wheezing a bit. Hopefully after her new meds tonight she can get some decent sleep.

Other than that the older kiddos are busy with finishing up school for the semester. Tomorrow is the oldest 3's last day and Monday is Ari's. So alot of Christmas parties coming up! The kids are super excited!

More updates soon after we have time to absorb all this information.

Santa

2011-12-07T20:08:00.000-06:00

Eli's therapy group hosted a night with Santa and we were able to go this evening. Eli cried the whole time he was on Santa's lap but had fun the rest of the evening . The older 4 enjoyed themselves!
Here's a photo of all of them!

Eli's again struggling

2011-12-06T12:01:00.000-06:00

Eli is still struggling. We went from the aspiration and requiring O2 to worse.
Eli met with his complex doc on Friday and we spent 3 1/2 hrs discussing everything. He gave us permission to go ahead and trial out full time O2 to see if we can keep his heart rate and respirations down. This is in hopes of avoiding a trach. Eli's normal O2 use is 1/2 liter while sleeping. 1/2 didn't work so we went up to 1. Then he started coming down with something or his aspiration getting worse. He's now on full time around 1 1/2 liters, has needed tons of suction, emergency breathing treatments, CPT, and is being kept upright at all times. His temp is up and so is his heart rate and respirations. He's barely coughing and it's pretty weak. His lungs are now sounding junky. I've already talked to his pulmonologist 3 times this morning. He's now having him on BiPAP with O2 and his breathing treatments run thru there. We have already had to cut back on his rescue treatments as they are making his heart rate even higher. CPT still. Increasing fluids. He again discussed the need to stop the robanol. He's not only concerned that it's raising his heart rate and increasing his respirations but now he's concerned that it's making his secretions too thick, and for a kiddo who doesn't have enough energy and muscle strength to cough, it's making it near impossible for him to cough it up.
We all will be meeting on Dec 15th, first thing to discuss all of this. We were confident that we were making progress on avoiding the trach, but after talking to his pulmonologist, we aren't so sure that we have alot of arguments left. He's worried and when Eli's doctors get that worried it really scares me.
The goal is still currently to keep Eli away from the hospital so our doc is calling here multiple times a day to keep checking in and changing nursing orders and sending med orders straight to the pharmacy to deliver here at home. Fingers crossed we can ride this whole thing out here. I am sooo thankful to have nursing in home 12 hrs a day 6 days a week. I have no idea how we would be getting thru this round without them.
More updates soon.

Happy 2nd Birthday!!!

2011-12-01T08:37:00.002-06:00

I don't have time to get a full blog post up but I did want to post the below pictures!

Updates W/ Pictures

2011-11-30T15:31:00.000-06:00

What a week. We went to Wichita for thanksgiving and had a nice time. We spent time with family, did some basic christmas shopping, family photos along with Eli's 2 year pictures, eli's 2nd birthday party, and Bob and I even snuck in a date night.
Here are a few pictures from our weekend.

Then we got back to KC and Eli decided to remind us in a BIG way that we were back to the real world. Sunday evening his blood sugar dropped and he was super fussy. He was being put to bed and his feeding tube got ripped out. I was able to get a G tube in and off to the hospital Eli and I went. They immediately got an IV going with fluids and sugar. The ER doc begged IR to come in so that Eli wouldn't have to be admitted during winter sick season. He came in and got the tube replaced. He ran the contrast dye in to check placement and accidentally ran it into the G tube side ( Eli's stomach.) Eli choked and gagged. The next morning Eli woke up with a fever, high heartrate, super fast respirations and a yucky sound in his lungs. We had an appt with GI so he ordered chest xrays. He also had us meet with the nutritionist as Eli is again losing weight. Right now they are more worried about his fluid needs than his calories. He's already getting twice the protein needs and 30% more calories. He's getting a ton of fluid but still showing "dry" on all of his labs, even though he is constantly swollen and puffy. The GI is also concerned about Eli's fundo and is ordering an EGD to be done in january. Then we went for chest xrays. They showed ok, although his pulmnologist says it sometimes takes time for aspiration to show up on film. By the afternoon when his pulm called Eli's heartrate was 170 plus, and his respirations were between 45 and 60. Doc said to put him on O2 and attempt to ride it out at home. Eli couldn't handle a lower level O2 so is on double his norm. Everytime we've tried to wean him off his HR and resps shoot back up. He's super tired and has been resting alot.
We are holding well at home currently but concerned about the fact that we can't seem to get back off the O2.
Eli also met with the dermatologist over a rash on his back and neck and a different type of rash on his head and ear. The doc talked about what they were and what to do about them. So now Eli has 2 new meds to help with all of that.
Here are a few pics of Eli these last few days.

The rest of us are ok. Exhausted. The kids are back into the full swing of school and life. Bob is keeping up well with them and the house. I'm staying busy keeping up with Eli and all of these things going on.

We are still awaiting Eli's care conference. They've decided to invite a larger group of his docs and this takes a longer period of time. We get more nervous by the day, especially watching Eli struggle.

Tomorrow is a HUGE day for our family. Eli will be 2. We are so blessed to be at this point and we cherish each and every day.
Here is a pic of my little man taken this weekend by an amazing photographer!

I promise to get more updates up soon. The kids have all just walked in the door from school and I need to get some more done around here.

Words

2011-11-22T19:50:00.000-06:00

Eli's rough stretch is still going strong. He had a rough night last night and beeped alot. This morning after being off his feeds for 1 1/2 hrs his blood sugar was 171. Which for Eli is ridiculously high. Endocrine called. We chatted. A one time sugar like that isn't to concerning but if it continues they want to begin further testing and his HGH (human growth hormone) will need to be re-evaluated.

His new bed also came today. It got here early so he was able to take his 1st morning nap on it. He did great! Stayed perfectly in the right position and slept well with only ONE O2 drop!!! We are looking forward to seeing how he does tonight.

His primary medically complex doc called me to chat. Never a good sign. He was concerned about the fact that he got a refill request for the robinul. He thought it had been stopped. I was told it wasn't going to stop until we had the care conference and had a clear plan in place. He said that they were working to get the care conference set up and that there was more to the trach than because of Eli's need for the robinul to be stopped. They want it for secretions, for his central mixed sleep apnea, and apparently because they are feeling the need to get a clear cut long term care plan in place for Eli as they still strongly believe that the underlying issue here is progression. From the sounds of his doctor they have all already made their choices and are just hoping to get Bob and I on board.

This worries me. It worries me that they are planning for and thinking ahead. I know that "ahead" is there but I try not to think about it, I sure don't plan for it....Okay that is maybe a lie...I sure don't plan for it out loud. Please don't crucify me for saying that, but it's true. As a mother of a kiddo like Eli I know what the future can hold, what it is predicted (guessed) to hold, and what the possibilities are. There aren't scenarios rushing thru my mind of Eli walking across the stage with his diploma in hand, walking down the aisle with his bride on his arm, or being present at my funeral.

In the top of my closet there is a box, the weight of it is immense. It is full of books, books given to us by the hospital, by social worker and doctors and nurses. Bob and I know they are there. They are for that moment, that time where life is uncertain, where there is no turning back, no fixing it. They are written for children, they are to help understand life and death and all that is involved. The thought of them terrifies me on a daily basis. As they and EVERYTHING else are always in the back of my mind.

What terrifies me even more is that the doctors are so willing to talk about and plan for the future. They say these things outloud. They say them to me and Bob, they write them on Eli's charts, they discuss them together with all of his care team. They try to keep us grounded, keep us knowing, understanding. They try to push us to think like them, to talk like them.

I can't do that. Each conversation takes another piece of me, it takes another piece of my little man. Words spoken can never be taken back. I don't want these talks, I don't want to be grounded. I want to wrap my arms around my little man and my family and float off into the clouds, flying freely all around, immune to "care plan meetings".

This is what I want. This is not what I will get. I will prepare myself. I will prepare the only way I know how. I will go to the meeting and listen, I will talk, I will argue and push, I will negotiate and try to compromise, I will beg and plead, I will present evidence and emotion, tears will fall freely...Ultimately I will not win, my little man will not win, in all honesty the doctors will not win either, Mito will win...After the meeting I will return home and do the only thing I know to do, crumple into my husbands arms, cuddling my little man until his head is soaked in my tears....I will talk to those families closest to us, those who understand what it's like to never speak these horrible thoughts outloud....

....and finally then I will make formula, pull meds, clean syringes, run spo2 monitors, apnea monitors, bipaps, O2, walkers, wheelchairs, OT, ST, PT, sign language, AFO's, SMO's, glasses, hearing aids, and books....Eli's favorite....books. I will read them, touching the pages, signing the pictures he sees, laughing with him, hiding behind them, and reading them over and over until they are so worn out that we have to re-buy them. After so many books the words from the meeting begin to fade and the lines begin to fade on if they were actually spoken or if they solely existed inside of my head....This will begin to help the pain again fade back to manageable...until that next dreadful meeting.

The cycle is torturous....for bob and i, for our older 4 children, for our families and friends, for Eli's doctors and nurses and therapists....But we push forward, just as Eli does in his day to day life. Eli is truly my hero. He is the strongest person I have ever encountered. My children are strong and amazing and supportive and so madly in love with their little brother. Our community is amazing and without them we wouldn't be able to make it thru each day. I hope one day I can be as strong for all of you out there as you all are for me, for my family, for my little man. Thank you.

Individual updates

2011-11-21T18:28:00.000-06:00

I apologize for how far behind I am. So much has happened and it has been hard for me to actually sit down and put it all together.
Things won't really be in chronological order as it's to much to keep track of that way.

Eli ~ We have met with the pulmonologist, cardiologist, and the ENT. They are all agreeing that the trach is the way to go. The ENT also is considering tubes in his ears. His left ear drum isn't moving and it is a different color and the doc believes it has fluid trapped behind it. He will most likely do the tubes during Eli's surgery in January. He will also either do a full scope or the trach, depending on the final decision. We are still awaiting the care conference. Eli also had a rough week last week. He stopped peeing and pooping. He also had a seizure. The first day he lost 17 oz, then gained 20, then lost 18, then gained and lost some more. He ended up in the ER on friday after no pee and the seizure. Lots of labs and IV fluids. He finally started peeing but it still isn't back to normal. He also finally had a movement after 4 1/2 days but again still not back to normal. He's been incredibly tired and has had alot of temperature maintenance issues. We aren't sure what is going on with him and we are hoping that it regulates itself quickly, because the doctors also have no idea what is going on.
In other big Eli news he is getting his Comfy Lift Bed!!! Some amazing Mito moms got together and held a fundraiser for my little man. They had the entire bed, plus shipping, plus a spare cover paid for in right over 24 hrs!! We are so blessed and so damn lucky to have such an amazing support system in our lives! I can't imagine this journey without them.

Emily ~ Life is fun with a preteen. I don't know how many eye rolls, hair flips, sighs, and her ignoring us that I can deal with. She can be the sweetest most helpful amazing girl and the next minute she can be a holy terror. It's fun times all around. She's trying to stretch her independence and we allow her as much space as possible. She now has her ears double pierced, wears strange feathers in her hair, wears sleeveless gloves, is addicted to black and neon green, and listens to music I have never even heard off! It's interesting to say the least. We give her this freedom as long as she keeps up with her responsibilities. She's still maintaining all A's and is keeping up her extras. She is in all the advanced programs, as well as now helping out every Friday in a kindergarten classroom with some special needs kiddos. She's very active in student counsel still and enjoying that. She's loving choir, and has even been selected for a solo in the Christmas concert. She's loving violin, and is preparing for her first strings concert as well. In other words she stays super busy with school, activities, and driving her parents insane!

Jace ~ He's doing decent. His meds seem to go in waves on if they are working well or not. We aren't sure why that is. We will be meeting with both of his primary doctors next week to discuss how the meds are going. His new IEP is in place and we are hoping to start seeing some changes. He's struggling a bit with Speech as he gets older. His stubbornness is starting to shine thru and his ST has had some issues getting him to participate and work hard. He will start his state testing in December and with the new plans in place we are hoping to see some improvement. He's still enjoying school and we are so thankful for that. He is finding some new activities to enjoy around the house as well, one of those being some craft projects and music! He also loves his playtime with Eli. They play brotherly rough and tough, which normally involves flying thru the house on Eli's car (with his helmet on of course). Eli loves this time and so does Jace!

Benjamin ~ He's doing decent. This last week he had a weight loss BUT all in all he has still gained 4 1/2 lbs. Which for Ben is HUGE. He's still horribly underweight but a gain is a gain! We are still trying to find ways to get more water into him, that has definitely been a slower process. He's doing ok in school, but still struggling to stay focused sometimes. He's doing decent with his spelling words, but not so great getting his homework done. He has made a friend at school it seems and has come home a few times very excited about playing with him. He's spending alot of time playing the Wii still and is loving it! He has also figured out some of the games on our cell phones and enjoys that as well. He's been working on some basic self help goals, mainly showering and getting dressed in the morning. He's not there yet but doing well learning!

Ariana ~ She's absolutely loving school! She has made many friends and enjoys the activities and the interactions. She's learning some new things and is very happy with it all! When she's home she enjoys playing with her dolls and has been enjoying some of the Wii games. Her favorite game is JumpStart and she's learning so much with it. She loves her mornings with the big kids gone so she can rule the big TV and do her things. She's still girly as ever and that's always interesting for the rest of us. Her hair is now halfway down her back and she loves it! Always fun for the rest of us to get to maintain it though!

Bob and I ~ It's always so hard to say how we are. Bob has had some major GI flair ups and has even ended up in the ER. Changed around some of his meds and it's doing much better. I've had multiple migraines lately. We've been busy with the kids and the house. I've been working my way thru 5 quilts, so far I have 3 1/2 done. I'm also working on a crochetted blanket for Eli. I even read a book this month!

This weekend we are going to go to Wichita for thanksgiving! It will be nice to get away for a few days and visit with family and friends. We will also celebrate Eli's birthday as he will be 2 on December 1st. My how time flies! More updates this weekend!

Cardiologist

2011-11-08T18:00:00.000-06:00

Last night was rough. Eli's apnea monitor alarmed 28 times for high heart rate. Today he had his appt with the cardiologist. They did his ECHO and then we met with the cardiologist. The ECHO looked good. The clot is definitely gone and the calcified leason is mostly gone as well. Now it's simply scar tissue and a thickening of the heart wall there. The heartrate was again high.
The cardiologist agress that the robanol is causing heart issues. He wants him off of it. He also believes that Eli's trouble with secretions is causing a small amount of respiratory distress and causing an increase in heart rate as well. He says meds to lower the heart rate are going to have to many side effects for a kiddo like Eli. He's also very concerned about Eli's risk of developing cardiac myopathy. He says that basically each heart is born with so many beats. Eli is plowing thru his heartbeats at a ridiculously fast rate. Long term this is obviously a major issue and by long term he means we will begin seeing effects from it fairly soon. He agrees that a trach is the best option. He will obviously continue to follow Eli during the whole next stage of the process and continue to closely monitor his heart thru all of these changes.
So it seems as though we are basically out of choices. It's rough.
We are working thru all of our thoughts and feeling and fears.
Tonight is Emily's choir conference so we are going to try to go and simply enjoy that and try not to think about it all.
More updates tomorrow.

Ortho and quick updates

2011-11-07T20:03:00.000-06:00

We had a very quiet weekend. Saturday evening Bob and I went out for dinner, shopping and a movie. It was sooo nice to get 6 hrs out alone. Very needed after these last few weeks.
Sunday we all laid incredibly low and didn't do a darned thing.
Today we have a new nurse shadowing. This morning was slow letting her get used to Eli. Eli had therapy with his speech path plus his sign language teacher. His IPAD was approved!!! Plus all of the extras that were requested. We are super excited!

Then this afternoon he had his appt with his new orthopedic surgeon. She says that when his muscles were growing in utero and as a baby they weren't able to grow properly which made his bones distorted. She says that ultimately he needs surgery. What she will do is cut both femurs in half and then rotate them to the proper place. Then they will be anchored down with metal plates and rods. Then he will have leg casts while they heal. She said that right now she wants to wait until he's a bit stronger. She's concerned that doing it now will cause way to far of a setback muscle wise. We are waiting to hear back on his care meeting for the trach. hoping to hear something tomorrow.

This evening Ari fell and got hurt. She was going over the baby gate and slipped and fell backwards hitting the back of her head on the tile floor. She has a massive knot and cried for a good 45 minutes. Alot of ice. No signs of concussion or anything else. We will wake her multiple times during the night, but I have no doubt that she will still have quite the headache come morning.

Tomorrow we meet with Eli's cardiologist. I'm anxiously awaiting this appt. This is the first of many times the trach will be seriously discussed, and his answers tomorrow will almost make our decision for us. I'm so damn nervous.
I will update tomorrow as soon as I know more!

Phone call with pulmonology

2011-11-04T17:29:00.000-05:00

Just when I think I'm as emotionally drained as is imaginably possible I'm hit again.

We have been working closely with Eli's docs to figure out the secretion issues and the medication issues. The robanol is definitely helping with secretions but it's also causing issues with Eli's heart. Ultimately Eli needs to be taken off the robanol. Eli's pulmonologist called me today and chatted with me for almost 20 minutes. He believes that with Eli's diagnosis of Mito(and the progression involved), plus his esophageal motility, plus the hypotonia and the respiratory issues that we are looking at the need for a long term permanent fix. He told me he feels a very strong connection to Eli and our family and that he really wanted to talk to me in person but he believes we are hitting the point where we need to have a care team meeting and discuss a trach. He believes that this is going to be a more permanent solution for Eli that helps protect his heart. I began crying immediately and the compassion in his voice was overwhelming. He really really cares about what is best for Eli and for our family. He is calling together eli's primary docs as well as his geneticist and cardiologist and wants to sit down with us and go over what's going to be best.
I'm terrified. This is something we have been warned could be discussed some day but we didn't expect it this soon. We are going to obviously be questioning everything and push on what other less invasive options are available. In the mean time his pulmonologist is calling all of the docs and is going to check in with me on monday to get this all set up.
I will update more as we know more.

Burden of Life

2011-11-03T18:39:00.000-05:00

Wow. I don't even know where to start. It's been a rough week for me.
The weight of responsibility of raising a kiddo like eli has hit me hard this week. I'm exhausted. Every moment of every day I have to be ready to give every drop of his medical history in order with ALL of the correct info, all of his surgeries and procedures, every name and phone number of every doctor(35)/nurse(2 in home and over 40 in office)and/therapist(8), every medication with concentration, dosage and time, and every single drop of info about blood sugar, swelling, seizures, sleep, vital and EVERYTHING else. If I miss ONE single fact it can be my childs life. To some this may sound like overkill but it's not. This is life with a complex kiddo like Eli. I have to maintain PERFECTION. So far I've not missed a single detail. I've always remembered every single thing at the exact moment that I needed it. I've always been able to have it all right there.
That being said though I'm TIRED. Beyond tired. Exhausted. My brain and body are nearing the end of what they can handle. I have maintained perfection for 23 months and I'm soooo scared I can't continue to do it much longer.
I have lost every drop of me and I have become an extension of Eli. I am Eli. Every doctors appt, every therapy appt, every progress or setback...the doctors and nurses praise me for how good I'm doing, they remind me that I'm the reason that Eli is still alive. I wholeheartedly believe that BUT it's such a huge burden to carry. It's lonely and exhausting and painful. oh so fucking painful.
For some of you I know this post won't make much sense, for some of you I'm sure you'll be nodding your head with tears in your eyes. I totally get that. I would have never understood until I'm living this life.
I sit here and look at Eli and tears automatically come to my eyes, my breath catches, my heart skips a beat. This happens virtually every time I look at him. The bond I have with him is amazing and I'm sooo very thankful for that. The problem is at the same time I almost find it hard to breath because the weight of it all comes crashing down upon me in waves. Sometimes my heart hurts so bad I can hardly stand to look at him and I briefly have to look away.

No family should have to live like this. It's not just rough on me. It's hard on Bob and the oldest 4 as well, just in a different way. Eli is my primary responsibility, leaving Bob primary caregiver for the oldest 4. That's not to say that I'm not involved in their care as well, because I am. But primary doesn't fall on me. It can't.

The top of my blog says "We've got what it takes, but it will take everything we've got." This is so true. I know deep down in my heart that we will make it thru this, but I'm so scared to know what will be left of me. I don't know who I am anymore outside of Eli. I don't know what to do with real "non-medical" life anymore. Life IS medical, beeps, medications, abbreviations, diagnosis, schedules, vitals, and dme's.

Life is keeping my baby alive.

Feeding therapy

2011-10-26T14:12:00.000-05:00

It was a fairly slow end of the week last week. Ben had a field trip to the pumpkin patch and loved it.

On Sunday all 7 of us went to the pumpkin patch. It was a HUGE patch with over 50 activities. The kids had so much fun. Eli got to go on a barrel train ride, a horse tire swing, and played in the corn pit. He also got to pick out his very own pumpkin! It got warmer than expected and the day really wore him out alot. He spent the rest of the evening sleeping.

Monday was a very slow quiet day!
Tuesday Jace had his appt with the developmental/behavioral psychologist. It was just alot of basic information and discussions. She is getting to know Jace and then will work to formulate a plan that will be the best. Currently she will be focusing on his physical aggression and his eating issues.
Last night Eli had what appeared to be a 3 to 5 min seizure with O2 sat drop. Makes for a fun start to the evening. His neuro has been called and deciding if they want to make any changes.
Benjamin also had his appt with the feeding team today. He did pretty well. They said he is definitely malnourished and are wanting to start him on vitamins as well as a few other things. The OT also wants to get a full sensory diet started for Ben. They will be working in conjunction with his doctor and psychologist. We are hoping to find a good solution for Ben when it comes to nutrition. He is so darned tiny.

Tonight is our ASL class. It's fall parties tonight for us and for the kids. They are super excited to get to wear their costumes. We have made cheesecake bites for our class. I can't wait to try them.

Tomorrow is Emily's 11th birthday. Since there is no school tomorrow she took her treats today. This weekend my parents will be in town to celebrate her birthday!

Today is Ben's Parent/Teacher conference, and tomorrow is Emily, Jace, and Ari! So nobody has school for the rest of the week. Hoping to get some housework and pumpkin carving done! Pictures and updates soon.

Wants

2011-10-20T15:16:00.001-05:00

Tuesday afternoon Eli's respiratory therapist came and downloaded his apnea monitor and O2 sat monitor information. She was going to fax it to the pulmnologist /sleep medicine doctor, Dr B, before we saw him Wednesday morning. She called me saying that it was over 100 pages and that she was going to drop it off for us to hand carry to the doc. I looked over it and was shocked by what all I saw.
Wednesday we had Dr B first thing. He went over the report and initially wondered if some of the high heart rate issues were because of a malfunction, some of the HR's all the way up to 370. There were also some low heart rates and quite a few apnea episodes. He got to going over the entire report as well as Eli's meds and was concerned. Eli is way maxed out on his robanol and that can cause high heart rate. With Eli's heart problems they are taking it seriously. He is calling his cardiologist and they will be doing an EKG and Echo. I received a call today from Eli's respiratory therapist saying that she met with the pulmonologist and they went thru all of the printouts AGAIN and that they do NOT believe it's a problem with the machine. I've heard them say that multiple times now but I keep hoping that it's a malfunction. But after hrs of pouring over it they are sure it's not. He also is concerned about the amount of issues he's having with his mask. He sent orders to the RT to work on refitting that. He has also ordered a special bed that will keep Eli elevated and on his back. We also discussed the ferratin / iron levels and he has decided with as much problems as Eli is having with his legs and arms that it would be a good idea to start iron. We are again hitting another crossroads. Lungs are a primary function obviously and we are stuck. The medication that is helping to protect his lungs (and is working pretty well mind you) is now causing another primary organ (the heart) to suffer. I have no idea what is going to happen and I'm left simply having to trust the doctors that they know what the right choice is.

Then come afternoon time we met with Eli's new optometrist. His eyes have again gotten worse. The optic nerve and retina still look good, but the eye sight has changed. What the doc was concerned could happen, has. Eli's right eye is barely functioning and his left eye is trying to do all of the work to make up for it. He has ordered new glasses and has also decided to begin patching. Eli will start with patching 2 hrs a day and we can work up from there. They will continue to monitor his eyes closely for any new changes.

After that we ran by and picked up his new SMO's. These are braces that cover his feet and right above his ankles. They are hoping that we can work up to the point where he wears them all the time he's awake, except for when he's upright working on PT he will continue to wear his AFO's. The AFO's are the ones that cover his foot, ankle, and leg to right under his knee. Hopefully we will begin to see some changes with these.
He's worked a bit more with his walker and is slowly getting the hang of how it works. He just doesn't have the strength or endurance to keep up with it.
He's been incredibly exhausted today and is already back down for another nap.

We are here. Scared and exhausted. This isn't our first time to have unwanted side effects from a medication BUT it is our 1st time to have 2 primary systems effected by the choices that we have to make on this one. I trust Eli's doctors, mostly, but when it comes to something like this it's hard to trust anyone, hard to make a choice if we compromise the lungs or the heart. I don't want to compromise anything. I want a magic fix. I want it now.

I WANT A CURE.

Therapy changes

2011-10-17T18:50:00.000-05:00

Eli had a fairly quite morning. Bob and I got some grocery shopping done and a few errands. This afternoon Eli had therapy. It was PT, ST, and ASL. We had a long talk. Alot of discussion about his legs and walking abilities. Nobody is sure what the best course is. His in home leads will be changing. His ASL therapist will become the lead. Then his ST will come maybe twice a month to work on his IPAD for communication. Then he will have OT and PT in home. We have been going to 2 different therapy centers to get all of his therapies as well as working with in home. It has gotten to be to much. Eli struggles with that much and so do we. We have decided to stop 1 therapy center where he was getting all 3 therapies and switch to simply in home. He will still receive intensive PT and water therapy at 1 outpatient facility. His in home PT agrees that some of the physical issues are getting worse BUT she believes if we weren't doing the intense therapies and interventions that it would be considerably worse than it already is. That's rough to hear. She worked with him for a bit today in his hip helpers and in his AFO's and he really struggled with exhaustion. They wear him out so quickly. He did do a bit of work on the stairs and did well with that.

It has been a bit of a struggle to decided to lessen Eli's therapies. There is a slight guilt over not giving him every single thing out there. On the other hand he is exhausted and it puts an added strain on his body. We are trying to find the balance of giving Eli what he needs, but also allowing him to be a kid and have a large amount of down time. Eli is already sleeping so much, and already needing so much more energy. He sleeps 12 hrs a night and around 4 hrs a day. For a total of 16hrs a day. Mind you there are only 24 hrs in a day, meaning he is only awake for about 8hrs max. So it's hard to find time to fit in all of the other activities. We are hoping that we are making the best choices. We have chatted with so many of his docs and therapists and they all agree. It's not giving up, but it's allowing Eli a break. It's realizing that no matter how many therapy opportunities we provide he simply won't catch up. He won't reach all of the milestones that we have been pushing him so hard to reach. I'm sure to some that this sounds like giving up, but I promise you it's not. It's about loving our little man unconditionally and allowing him to be simply Eli. We are hoping this is the right choice, because we don't see many other options and we truly believe this is for the best.

Emily came home today with big news today! They had a school spirit assembly and she won an award. She made principal's honor roll. She was super excited and so are we!

Tomorrow we have Eli's ASL tutor who comes once a week to teach us ASL. We enjoy this time and learn a lot.

Something's up with Tiny

2011-10-16T18:38:00.000-05:00

I'm constantly reminded of the pain and devastation and heartache and instability of Mito. My little man reminds me, as well as the other little Mito warriors in our group. It's so hard.
My tiny man has had a rougher few days. He's had his cough, and has had some blood sugar instability. Today he ran a fever again (highest we've seen for a few weeks) as well as had some blood sugar drops. He has a bright red rash on his cheeks and has been very sleepy. Yesterday he had a lot of drainage, but today that is gone. Some upper respiratory crackles but nothing down in his lungs. Not sure which way it's going to go but it's definitely been getting progressively worse over the last few weeks. This week we meet with his pulmonologist /sleep study doctor and I'm anxious for that. I'm hoping we can find some relief for the restless leg syndrome and for the breathing issues. His Mito doc AND his endocrinologist don't believe that the growth hormone is causing the increase in issues, BUT his pulmonologist and pediatrician believe that it is. His Mito doc doesn't sugar coat anything and he simply feels it the progression of the disease. I hear docs say that but I struggle so very much to believe it...to grasp it. I'm tired of hearing it. I can't give up and I wish they wouldn't give up. Any way you look at it, this is a major appt and we are ready to get some solutions for the sleeping issues. It's so hard for a kiddo who already has energy issues to struggle so deeply with sleep, it makes the energy ten-folds worse.
We also will be making some phone calls this week to change around a few meds as well as to rework his feeds. He's still swelling quite a bit, and waking up many a morning with a very swollen face. Hoping to get some answers on that as well.

Emily is super busy this week. She's got to be at school early every day to help run the united way fundraiser. It's also spirit week at school and since she's part of student counsel she is actively involved in that as well.
Jace is going to hopefully be meeting with his doc. He again lost a lot of weight this week and we can't go like this much longer.
Ben has his 1st field trip this week to the pumpkin patch. He's excited to get to go.
Ari enjoys every week of school!

Tomorrow is Bob's birthday! We are hoping to have a nice quite family evening at home. We went to the farmers market this last weekend so we have a lot of fresh yummy veggies to make up this week!

Please keep some of our Mito kiddos in your prayers. There are a few in the hospital right now who are really struggling. Please pray for them, their families, and their doctors!!!

Garage Sale and updates

2011-10-14T19:15:00.000-05:00

Again. I feel like I keep falling so far behind. Last saturday we had a garage sale. The kids set up a table and sold "Muffins for Mito". They did well and enjoyed spreading awareness about Mito and their little brother.
The week started with a rough Monday. My little man had his renal ultrasounds and his appt with the surgeon. His kidneys look the same, a touch larger, but not scary bad. His surgeon is still not comfortable operating and is also not comfortable stopping the antibiotics. Eli has been on them since 3 days old and will probably be on them for a long time to come. They are helping prevent infection, although they do cause some risks as well. The surgeon was concerned though about his testicle surgery. The left one has already tried to go back up and has shrunk considerably. The right still can't be palpated although we know it's there from pictures at the last surgery. He is afraid to try and re-operate on the left as he's afraid touching it will cause it to break off. He does however want to operate on the right. He wants to go in and attempt to pull it down. He isn't confident however that it is salvageable at this point after being inside Eli's body that always runs hot because of his autonomic issues. He will either remove it or lower it. He's hoping it's still partially good as Eli needs those hormones since his pituitary gland isn't functioning. We shall see. He has scheduled the surgery for January 3rd. He wants all the other docs contacted so they can join in and combine under his anesthesia. Eli can only be under so long so we will back to prioritizing surgeries. Something we have had to do many times before. He will as always reserve a bed, and we will have a full sit down with anesthesia beforehand.

On Tuesday Eli had the orthopedic surgeon. &%^#$*&%$#*....that is how I felt about it. She says his hips are good BUT his bones are improperly shaped at the hip AND knee, that his hypotonia is causing major issues AND that his achilles tendon is way to tight, BUT walking isn't important for a kid like him and we can just worry about it when he's older or he can just learn to compensate for it. I fired her and DEMANDED a new doc. I told her that just because he has mito that he deserves EVERY opportunity to do basic life skills like walking. I have NEVER been so angry at a doctor. It was clearly not a good experience.

Eli had PT on Wednesday. This was his 1st time going into the heated therapy pool. The first and last time that we took Eli swimming he had alot of issues maintaining his body temp so we never took him back. He did great in the therapy pool and after a bit really seemed to enjoy the water. He will try again in a few weeks.

Today we again had the garage sale. The kids didn't have school so they got to help out. They again did their Muffins for Mito. They did amazing! Towards the end of the day they had 1 muffin left. We saw a man chatting with them. Next thing we know he is walking up to us, muffin in hand. He talks to us about how Emily told him about Mito and Eli. He said how strong we must be, and how he was thinking about us and would be praying for us. He told us to stay strong. He said he couldn't donate much but he gave the kids some money. When we walked up to Emily she was crying. The man had given her $100 for 1 muffin. Bob walked across the street and thanked the man even more than we had originally. The man cried and chatted with Bob for a bit. We are forever amazed by the people we have met along this road. In 2 days our kiddos sold 10 dozen muffins and earned $190 for the UMDF and Eli's medical care! We are super proud of them!!

Alot of changes have been going on around our household this week. We are prioritizing and reorganizing. Some things are having to come to an end and some things are being added. The stress level has hit the critical point. We are beyond exhausted and so is Eli. We are having to end some things that we didn't want to end. It's been a long road coming and we've had to really talk and decide what is best for all of us.

Eli has struggled a bit today, his allergies are again flaring up. He's been coughing quite a bit which has resulted in alot of gagging and dropping of his sats. This next week he meets with his Sleep Medicine Doc/Pulmonologist. We will hopefully be working out a better plan for Eli's BiPAP and sleeping issues, as well as the restless leg syndrome, and a full plan for his upcoming surgery. We are also hoping to get this last round of genetic testing back. He has an appt with his endocrine doc coming up, alot of phone calls with docs for his upcoming surgery, and some very long talks with his hematologist to discuss how we want to handle this surgery and the risk of clots with Eli.

Emily ~ She's staying busy. She has turned into quite the advocate, event coordinator and fundraiser. She is working hard with her Mito awareness, has been actively involved in student council and has been selected to help run the United Way campaign at their school this week, as well as all of the fundraising for her trip later this school year. She's also still enjoying choir, strings and everything else!

Jace ~ He's finishing up his 1st book for book club. They meet next week. He's been involved with the good news club at school and is enjoying that. He's enjoying his ASL class and is learning alot!

Ben ~ He's been a mess. We just finally got his meds back up to a full dose so we are hoping that helps. He's enjoying school, mostly. He's working hard in speech and some extra social skills help. He's also going to ASL class and seems to be learning some.

Ari ~ She's loving school. She has made some friends and is so excited when she gets there each day to sit with them and chat. We've been struggling some still with getting her to eat, but constantly trying new things to see if we can work thru that.

Bob and I are same as always. Like I said above we are really adjusting some things in our life. We have been working out a minimum of 3 times a week, which normally ends up around 6 times a week. I've lost 16lbs at this point, and am enjoying getting back into a workout routine.

More updates soon. I will also try to get a few pictures up soon. Tomorrow Bob and I are going on a date morning. We are heading to the farmers market and to a health and wellness fair. In the afternoon we are hoping to take the oldest 4 out to the park. We will see how the day goes!

Quick Update

2011-10-07T17:47:00.000-05:00

It's been a slow week, which was very much needed.
Eli's allergies have been causing him some issues, which has caused the choking & gagging to come back. Finally got orders from his doctor today and we are going to try and double his allergy meds as well as continuing with the spray. Next step will be to either find a new allergy med, add another one, or to up his meds that help keep his secretions low. We shall see. He had his ASL playgroup again today and fell and hit his head fairly hard. He's doing ok so far, so that's good. He also is apparently allergic to something at playgroup and has a rash all over his hands and face. Not sure what it is.

The others are doing well. Tonight we are going to the oldest 3's school for a Family Bingo night. The kids are excited about that.

More updates later this weekend.

Quick Check In

2011-10-04T20:04:00.000-05:00

Eli has had a rough day. His allergies are getting worse and worse. He's choking and gagging more. The side of his face is now swollen and he's pulling on his ear. Low grade fever and slightly elevated heart rate. I spent the entire day talking with docs. Endocrine and pulmonology both want to see him ASAP. Pulm got his labs back. His iron was high and his feratin (protein in iron) was low. They aren't sure what's up with that or what to do about it at this point. He has been incredibly clingy all day. With him being sick the rest of his body is attempting to keep up. His blood sugars have been low today so that's taking some adjusting.

Emily is doing well. She had student counsel today, and has continued to stay busy with choir and strings. She has all A's and is doing good with her studies.

Jace is doing super. His new meds are AMAZING. We are shocked by how he's been. He's calm and quiet, and respectful and has been super focused on his work and everything. We are curious for parent teacher conferences to see how he's doing in school.

Ben is doing a bit better. We are going to be raising his meds back up to a full dose, and hopefully that takes this last bit of issue away. He's eating decent, although still struggling with his weight. School is still reporting some attention issues, but working to come up with a better plan. His new IEP is in place and we are super happy with it. He has even more than we had hoped for!

Ari is ok. She's enjoying school, and making some friends there. She's enjoying having her little "homework" backpack, which is a book to bring home and share with the family. She's still struggling with eating at home, and trying to find her place with her siblings. Alot of defiance. Hoping as she's in school that she settles in more.

We started all of our ASL classes last week. The kids enjoyed them. Ari isn't able to go, and we aren't sure if Ben is going to be able to handle going, but Emily and Jace will definitely continue. Bob and I enjoyed the adult class as well and feel like we will learn alot.

More updates over the next few days as I get more information on what's going on / what we will change/do with little man.

Geneticist and Neurologist

2011-09-29T19:06:00.001-05:00

Today was a day from hell. The morning at home started out rough with a lot of issues. We got out of the door 20 minutes late. Eli ended up missing all of OT. I dropped Eli and his nurse off at therapy and I ran to CMH to pick up some medical records. Eli had PT and ST. I got all of his papers and showed back up for the end of PT. We had to immediately leave following for his appt. with the geneticist /neurologist who specializes with Mitochondrial Disease.
We met with the genetic counselor for over 1 hr. She went thru a lot of history. Then the doctor came in and spent over an hr going thru all of his end. He talked alot about Eli's significant clinical history. He praised how good of a job Bob and I have done. He was amazed at how well Eli looks compared to what he expected from Eli's clinical history, tests, and diagnosis! We discussed alot about Mito and his thoughts on Eli. He believes there is still a significant piece of the puzzle left to find. He has a feeling he MAY know what that is but the road to testing won't be fast or easy. He does believe that Eli has a neurodegenerative genetic disorder, as well as a chromosomal issue. He ran some tests today(alot of chromosomal tests) and is working on getting the final test done. He says no matter what the FINAL diagnosis/outcome is there is NO cure and that we are currently doing everything he believes that we should be doing treatment wise. Once he has the final piece of the puzzle he will be able to give a better idea to what we should be expecting and may possibly see and a possible prognosis. Not conversations we are looking forward to having.
He also discussed Eli's growth hormone and the increase in apnea issues. He does not believe they are linked. He believes the increase in apnea is simply because Eli's disease is progressing which is resulting in more problems with his central mixed sleep apnea. So the good news is he believes that the growth hormone injections are very important and that we should continue, the bad news is again this horrible disease is progressing.
There was brief discussion of how well Eli did on TPN, and how he wasn't surprised int he least. This is unfortunately/fortunately an issue that is currently no open for discussion since Eli has a blood clotting disorder and has already had active clots, and still has the calcified leason in his heart.
After this Eli had all of his lab work and we headed to his main neurologist. He said Eli's keppra levels looked much better and that since we haven't been seeing any seizure activity that he wants to stay at this same dose for now. He was also encouraged that his legs have improved some. There isn't quite as much tightness on one side, with looseness on the other. His hips are definitely still struggling as are his knees and ankles but improvement on the mixed tone. He is happy with the progress he is seeing, and so are we!
Eli didn't get much in the way of sleep at all today. We left the house at 8am and didn't get home until after 3. Considering his day he did well, but boy is he exhausted. His temp is up, his blood sugar is down, his little cheeks are bright red, and his extremities are moddled. Fun times!
I will update more in the next few days.
Thank you to everyone for the prayers and kind thoughts today!!!

2011-09-27T07:41:00.001-05:00

Last week ended fairly quietly. Eli had therapy and just laid low at home resting. We got all of his new equipment started, which has been way more of an adjustment than expected.
This weekend we spent time together as a family relaxing. We went to the park on saturday. We spent over 2 hrs there. It was great! The park is huge with walking paths and lakes and tons of areas to explore, as well as the playgrounds. Eli loved being outdoors.
We watched some movies, I worked on some quilting and we just hung out. The kids played alot of Wii. Ben is currently addicted to it.
Monday brought a few appts, as well as Eli's therapy. He worked a bit with the ipad, and his in home physical therapist brought him a gait trainer to use here at home until his comes in.
These next few days bring multiple appts. The most important being the Mito doc here in KC, on Thursday. This is the doctor that we came to see in January. This is when Eli crashed at the Ronald McDonald house and ended up being rushed by ambulance to CMH. We are finally going to be seeing him this Thursday. I'm nervous and excited. Eli really needs someone local on his team who truely gets mito.

Emily ~ She is doing well. Enjoying choir and violin. She was voted by her class to be in student counsel and has her 1st mtg today. She's enjoying school and is making some friends. A little girl from her class called yesterday and asked if Emily wants to get together later this week. She's also received her first book for her book club and is excited for their first mtg in Oct.

Jace ~ Jace is ok. He's on another medication adjustment right now and hopefully it will see more benefit. He's still on a food strike and has lost a substantial amount of weight. He and I meet with his doctor today to find out what next. His GI system is doing much better with the change of diet..now if only we could get him to eat enough. He also has his 1st book for book club and will have his 1st mtg in Oct as well. School has gotten his IEP in place and he is working with speech twice a week, and enjoying working with his new speech therapist.

Ben ~ Ben is a mess. When he's on his new med he's a mess at school and decent at home. Now that his doc is weaning him off of it, because of the school's complaints, he's a mess at home. Unsure yet how he is doing at school. We will be calling the doc to find out what next. We also have his IEP mtg tomorrow so we will get more info as well. Ben's current addiction is the Wii, specifically the game lumberjacks. He wants to play from sun up to sun down. It's been a challenge. He's eating decently and is at least maintaining his weight right now.

Ari ~ She's loving school. She's such a social girl and is really enjoying making friends and being around so many kids her age. She's hit or miss with the food, and is doing a baseline job of maintaining her weight.

Bob and I ~ same, same. Just keeping up with the house, and the 4 olders, and Mr Eli. This week begins our family sign language classes at the Kansas School for the Deaf. Everyone but Ari and Eli gets to go an participate. We are excited to get to learn more and meet more families.

I will get pictures up later today from the weekend. Currently blogger photo upload is a mess.

Yet Another

2011-09-23T08:48:00.000-05:00

It is with a heavy heart that I have to send my thoughts and prayers to yet ANOTHER family this week. May you be flying with the angels and chasing butterflies Mason! Again, THIS is why we are trying SO HARD to raise awareness about this disease. that's 3 children(Robert, Eva, Mason) lost in 4 DAYS...

Song and Quote

2011-09-22T12:14:00.002-05:00

What a day so far. Eli had therapy this morning, OT and PT. He did decent at OT, exploring some new textures and working with some grasping skills. Also alot of hand/eye coordination work. Then he headed over to PT. She stood him up behind a walker and he walked halfway across the room!!! By the time he got to the other side he was exhausted and his little legs were shaking and he just toppled over.

Afterwards I needed to run to Green Acres for Eli's probiotics. Eli was in his wheelchair and had his tray with his toys attached. A little girl was drawn to him and spent some time playing with him. We got to chatting with her mom. She asked us about Eli and his diagnosis. We chatted openly about Mitochondrial disease and how it affects our little man. We chatted with her about it being awareness week and how there is so little awareness about Mito. We gave her one of Eli's cards. Come to find out she is a TV producer and has done some work with Discovery Health and such. I was shocked. I hope that meeting our little man will plant that thought in her mind to go out and learn about Mito and to help spread awareness as well. Heavens knows that a disease like this needs awareness!! As we got into the van my phone dinged. It was an email that a dear friend of our had posted a video with us tagged. I sat in the parking lot, listening...crying.

I was again reminded how blessed we are. My little man is such a warrior, if only we could be as strong as him.

Upon getting home I saw a quote a friend had posted. It made my heart flutter.

I will share the quote and song here, as maybe it will do the same for others.

"Some people never meet their heros, I gave birth to mine."

Nothing

2011-09-21T18:26:00.000-05:00

This morning Eli woke up really croupy and raspy/wheezy. Called his doc and they had us bring him in. After a warm bath and all of that he was breathing much better. Doc did a full check over and wanted to chat. Never a great sign. He had seen the sleep study results. He was concerned about Eli beginning to gag more again. He decided to increase his robanol (the med that helps lower Eli's secretions). He discussed his concerns about the dosing we are already on. He's concerned that much more and it will thicken his secretions so much that he won't be able to cough them up (Eli is a weak cougher. Takes alot of work). The problem with not increasing it though is he's having more secretions again that he is choking on. Hard to make a full choice. He also informed us that Eli has restless leg syndrome and that the severity of it made them think that he is struggling with anemia again and low feretin levels. He is also concerned that the growth hormone is causing eli's sleep apnea to get considerably worse, something that they knew could possibly happen. He suggested we speak with the pulmonologist.
We left and within a few the pulmonologist/sleep study doc was calling me. We spoke for 20ish minutes. Apparently the sleep study came back with alot of issues. Eli had alot of central mixed sleep apnea, worse than on previous sleep studies. He also had alot of leg movement, 38 an hr on average, leading to a restless leg syndrome diagnosis. He believes it's an anemia/feretin issue and has placed blood draw orders and will most likely be ordering iron supplements. He is placing Eli on BiPAP and O2 bled into the line. He is also placing a high end rescue level on the BiPAP to help push Eli when he isn't able to sufficiently breath. He's placing Eli on a special apnea monitor that also does heartrate and O2 levels. He is adding flonase spray to help open the nasal passages before starting the BiPAP each night. He agrees with the robanol increase and is concerned we may have to push farther on it. He's concerned if Eli isn't dry enough that the BiPAP will push fluids down into his lungs. He warned that we are fighting an uphill battle.
Eli's seizure meds cause a depressed respiratory status. His seizures do as well. So do the growth hormones. And so can mito. Not to mention his central mixed sleep apnea, reactive airway disorder, and in general permanent lung damage from all the other shit his poor little lungs have had to endure. He's simply hoping to get Eli out of the critical point where he is now of dropping his sats 2 to 4 dozen times a night, to where Eli can get a bit more sleep...us too.
It has come up now multiple times that we may need to discontinue some of Eli's treatments and interventions because they are making other things so much worse. There are many dangers to stopping the other treatments BUT we have to protect the primary life function of his lungs. None of Eli's doctors seem to be sure what the right choice is here.

I want to hate them. I want to scream at them. I want to demand 3rd and 4th and 5th opinions. I want to tell them how stupid they are and how they don't know anything and how I want a doctor that knows more than they do. I want to berate them for not coming up with a solution. For not treating Eli until he's all better. I was complaining in the car....so angry that all of the doctors are giving up. I want to push them until they figure it out, until they fix the problem.
Halfway home it hit me. There is no fixing it. These aren't stupid doctors. Eli's pulmonologist/sleep study doc has been practicing since 1972, he is the head of the pulmonology AND sleep studies dept at our childrens hospital. He is brilliant. He truely cares about his patients and is trying everything he knows to try. Most of Eli's doctors are the same. Eli sees mostly dept heads. These are amazing docs. It's not their fault. It's nobody's fault. For the first time it truely hit me that it's not that we are seeing bad doctors, or that they are refusing to help my son, or that they don't want to spend the time trying to help him, it's that they can't. They can't fix my little boy. He has a horrible horrible disease. A disease that is robbing my little man of his entire being. It's stealing everything from him, from us. This is killing me as a mother. It's horrible. Words can't describe the crushing feeling I felt inside when it finally came to me that it's not for lack of a better doctor. It's not fair. It's not how life should be. My little mans disease is progressing and there is nothing that I can do, nothing that bob can do, nothing that my kids can do, nothing that the doctors can do. Nothing.

Alot of Updates with Pictures!!

2011-09-20T19:20:00.001-05:00

Last week was a busy emotional blur. We spent most of the week rushing around getting ready for the walk. We made 40 something shirts, we made 40 something crocheted flower pins, we cleaned house, planned menus, grocery shopped, and kept up with all of the appts and meetings and such.
Eli had a decent week. He was tired alot and had alot of O2 issues. We were up alot messing with his CPAP and O2. He ran low grade fevers alot, and had alot of temperature control issues with the goofy weather changes.
Friday evening our family and friends got into town. We had a full house.
Saturday morning started early and we were at the walk by 8am. It was amazing. It was so awesome seeing other families and people effected by Mito. We met alot of great people and exchanged contact info! It was just so surreal for me.
Our family and friends left on Sunday and we spent the rest of the day relaxing.
This week has been busy as well.
Ari started preschool yesterday. She goes 5 afternoons a week for 3 hrs each day. She had alot of fun and has already made some new friends.
Ben has been a mess. His teacher is struggling with him in class, and we are having many of the same issues at home. He and I met with the nutritionist and she's out of ideas. he's being referred over to a feeding therapy team. He also seems to be struggling with the medication change and we are all unsure what to do. We are keeping in close contact with his doctors.
Jace is doing ok. His meds are being slowly increased and he's done well with that but we still don't seem to be at the perfect mix. He's enjoying school and has made some friends. When he's not at school he's super busy at home with friends in the neighborhood.
Emily has been beyond busy. She's in choir twice a week, strings (violin) twice a week, math club once a week, AND she was voted into Student Counsel by her class. Top all of that with homework and violin practice and the book club she's participating in, she's always on the go! She is enjoying it though and we are beyond happy about that.

Food. We are doing well. It's been such a huge change for our family. We have been vegetarian for 3 months, and Vegan and Gluten free for almost 7 weeks. We have found so many great recipes, and have been able to replace almost all of our favorite foods! It's been quite the learning process for all of us, and we have all really had to step out of our comfort zone! As most of you know this isn't the only household change we have made. We are still going strong on cleaning supplies, no paper products, no disposable containers, etc.. We are super happy with the changes that we have made!

Bob and I are doing ok. Life is hectic. Like always it takes everything we have to keep going thru each day. We struggle finding the balance of time, and rarely succeed with it.

Eli.. Eli is Eli. It's always something. He's still struggling with his O2 at night. He alarmed over 2 dozen times last night. He's still been running his low grades. He's had a bit of a struggle with his change in feeds. His Keppra (seizure meds) levels were barely within normal limits so his doc has again upped his meds. I got a call today from our DME saying that they needed to bring out Eli's new BiPAP and get all of that set up. I knew nothing about this. Apparently his sleep study came back. They thought that Eli was on O2 during the day as well. I said no. She said I needed to call his pulmonologist/sleep study doc immediately tomorrow morning. I have no idea what is going on. Eli has alot of appts coming up. We meet with his neurologist, pulmonologist, opthamologist, his new AFO's come in, nutritionist, his main doc to discuss his anticoagulation precautions, and he meets with his wheelchair DME and PT to get his stander and gait trainer ordered. It's alot going on and it's a struggle to keep up with it all. It's so hard to see the progressive disease, progressing. It happens so fast something and it's so hard to deal with. They tell you it can happen, but when it does...it's just almost to much to bear.

this week is mitochondrial disease awareness week. Our oldest 4 have been amazing for mito week. They wore their team Eli tshirts on monday with their pins. They also had a pocket full of Eli info cards to pass out. All their own ideas. We have all worn our Eli pins each day, and will continue to for the rest of the week! I would like to share some things that have meant alot to me!

Mitochondrial Disease: Cunning, Baffling, and Insidious. Like any disease without a cure, it robs, it steals, and it ruins lives. It takes our infants from us, before we can even hold them. It puts too many angels in the grave, before they can even grow up. It robs a grandparent from being able to hold their grandchild. Mito robs a mother or father from being able to raise their children. Please make awareness, so that our world, realizes that we are not invisible any longer. ♥ WE NEED A CURE

Mitochondrial diseases result when there is a defect that reduces the ability of the mitochondria to produce energy.
As the mitochondria fails to produce enough energy, the cell will not function properly and if this continues, cell death
will eventually follow. Organ systems will begin to fail and the life of the individual is compromised, changed or ended.

The real cost of mitochondrial disease is not in money, or time spent in the dr offices. The cost is in the little ones that don't live to see their next birthday. The adult that has a stroke & can never hold their child again. The teen that has dreams to be married w/ a family someday & watches those dreams slip away through a hospital window. Mito doesn't care if you are male/female, race, age, or wealth. It will take you life. Please help us search for a cure!

In closing I will post a few pics from the last few!

Our team tshirts all finished!

my little man during a bad day of temp regulation

same as above. just face closeup because i'm addicted to his little face! :)

a team picture. sorry for the glare. it was that time of day.

my little man at the mito walk looking beyond adorable!

eli with his poster at the mito walk

our front door decorated for mito awareness week. and yes our porch light is green too!

Ari on her first day of preschool

one of our dinner ingredients (minus carrots, pecans and raisins) turned out super yummy and colorful!

Eli with his brand new tubie baby. He's laughing in this picture! i love his laugh face.

Precautions and Discrimination

2011-09-12T10:42:00.000-05:00

It's been a busy week. It takes everything I have in me to stay going and to slightly keep up. Blogging has fallen to the wayside a bit because of life.

Last week Eli had PT at his new therapy center. He again worked with the gait trainer, then tried out the treadmill. She also showed us a stander that she is wanting to try out with him as well. His PT called insurance and his wheelchair DME and they will be meeting us at therapy in 2 wks to get a gait trainer and stander ordered for him.
On Wednesday I also played phone tag with Eli's main doctor. Insurance again denied 2 of Eli's meds, so we were originally going to do them as shots if this occured. So I called the doc to get that done. He finally calls me back with unexpected information. They have decided in light of Eli's history, and his blood clots, and the mito, and the factor V leiden, that they are placing Eli on anticoagulation precautions. The doctor informed me that every single time we give Eli a shot we are increasing his risk of developing a blood clot. He's not comfortable giving any more shots. This changes so many of Eli's protocols. He is meeting with Eli's hematologist to work out the best and safest plan. We weren't told that they were concerned about this and that it was something that would just be monitored. They apparently have come across more info in all of his testing and labs that tells them otherwise.

On Thursday Eli had OT/PT/ST. It was a mess. They had wanted Eli to go to their group therapy sessions . They wanted him to go 5 days a week for 2 1/2 hrs a day. It's like a preschool type program. We were leary. They told us on Thursday that they didn't want his nurse in the room with him and just wanted her to hang somewhere in the building for if he had problems. They also didn't want him on his feeding pump because it is to much of a liability for them they claim. They are afraid one of the other kids will pull it out. They said during class they want him to be a normal kid and therefore don't want all of his equipment or medical interventions or nurses. This really bothered me. It to me was really discriminatory. Secondly it really hurt me. I want my little man to be like a "normal" boy too, BUT he isn't. If you take all the medical interventions and equipment away from my little man you no longer have my little man. They are apart of Eli and these interventions have helped keep my little man alive, and have allowed him to live the life that he is living. This was our first time that it has truely hit me in the face that my little man won't be able to live a normal life, interacting with normal kids in a normal environment. It was rough. (as a side note we turned them down and will NOT be sending Eli there...obviously!)

On Friday we went to Eli's first ASL playgroup. He did great. Ari enjoyed herself as well. It was definitely overwhelming to try and keep up with all of the signing and trying to remember everything I have learned so far. But we did well. Can't wait until our next one!

The weekend we stayed around the house as a family. We worked on Eli's shirts and buttons for the walk. So far we are up to 28 people on our team and more are going to be joining us on Thursday. We are super excited and looking forward to this weekend.

This week we have quite a bit going on. Eli is going to get refitted for glasses, get fitted for his new AFO's, have 6 therapy sessions, 2 nurses, 3 doctors appts, and 1 lab draw. Emily has violin twice, and choir twice. Jace has book club and boy scouts. Ben also has boy scouts. Ari has a quiet week. Bob and I just run in circles trying to keep it all going!

I will take lots of pictures this weekend at the walk and get it all updated soon!

playing catch up

2011-09-06T19:34:00.000-05:00

Behind like always. We went out of town for the holiday weekend. We were gone thursday afternoon until monday afternoon. It was nice to get away for a few days. I got to go to my knitting group and hang out with everyone. We got to do some shopping and spent time with the kiddos. Bob and I got to go on a date night and really enjoyed the time alone. We got to visit with some friends and really enjoyed reconnecting with everyone.
We took it easy monday evening and hit the ground running this morning. Little man woke up and his nurse was disconnecting him from his feed, when she called me over. Eli's GJ tube had broken. I immediately called IR and we had to head up to the hospital. They replaced it and said that they had multiple tubes break in the same place lately. I immediately went from the hospital to Ben's IEP meeting. We agreed to a month long IEP while they get his re-evaluation finished up. We are also going to work towards getting a full health plan in place. I was able to come home for a bit then it was back out again. Ari had her in school evaluation. We met with the main child development worker, speech pathologist and occupational therapist. She will have her in home meeting with all of them on thursday and then we will have the full assessment meeting at the end of the month. Ari is really hoping to be able to start PreK soon. Then it was back home and a touch of down time before picking up the kiddos from school. Then Eli's ASL tutor came. She normally comes on Tuesday morning but since we had to go to the hospital she was able to change to this afternoon. Today we learned some more basic communication signs, as well as our days of the week, and how to sign the ABC's song! Eli is really enjoy the ABC's song.
On top of all of this I have a sick husband. He seems to have caught my cold and is coughing so much he is light headed and simply not feeling well. Fun times.

Today on facebook it gave me the whole 1 year ago today post. Wow. I year ago today Eli was in the PICU with his 1st round of pertussis. We had learned to suction and use O2 at high levels, we had learned advanced life support and how to keep him always positioned upright, keep his airway open, and how to use an ambu bag. 1 year ago today we went and picked up Ambu bags for his bedroom and the play area in our home. 1 year later Eli still has an ambu bag hanging next to an O2 tank in each room he is in primarily. It's such a strange thought to think about this. It's something you never expect to see in your home. Heck it's something that most see in a hospital and don't even realize that they see it. Yet here it sits right next to his push toy and bookshelf. I have those moments where it just hits me that this really is my life, and today was again one of those moments.
More in the next day or two. Promise.

Quick Week to Date Overview

2011-09-01T05:45:00.000-05:00

It's been a fairly busy week.
Monday ~ Little man had his in home speech and signing. He did decent, but was exhausted so didn't learn a lot of new things. We however learned all of our color signs. Ari had her hearing tests in the afternoon. She passed all of them! We were super relieved about that. Emily had her first day in strings. She was super excited. They didn't do much on monday, apparently most of the kids didn't have their instruments yet. Emily can't wait to get started with the violin. After school we met with Ben's new case managers and went over alot of his information and paperwork, preparing for his IEP mtg. We are really impressed with his managers here!

Tuesday ~ Eli had his in home ASL (signing) tutor. We learned alot. We learned all about basic beginning and ending conversation when we meet new people. Then we had Ben's IEP mtg. Alot of good information and a decent base plan in place. There are definitely still some concerns and everyone is working on a good plan to put in place. One of the biggest concerns is Ben's eating and drinking. He has lost over 2 lbs since starting school, as he is basically refusing to eat. He's been drinking maybe 5 or 6 sips of water a day. Everyone is working hard to get this all resolved. Later in the afternoon Eli had his 1st appt with the muscle and nerve clinic. We were super impressed with the doctor. He believes that it's mitochondrial myopathy. He believes that the seizures are causing a large part of the issue and that it's simply neurological degeneration ( his word not mine, because I don't like words like that). He's like, "obviously as you know with mitochondrial disease the brain alot of times will begin a degeneration process, and we are obviously seeing that with eli." Like always I just wanted to scream at him to stop saying those things. Those are the kind of statements that bother me the most. Back to point...He doesn't believe that botox, or casting, or surgeries are the best choice for Eli right now (neither do we), because he believes at this point none of them will help him to walk. He ordered a soft side helmet to help protect his head. When Eli gets tired he falls over frequently. The goal is to protect his head the best we can. We will continue to follow with him in clinic.

Wednesday ~ Eli had his PT. He did super awesome. She put him in a gate trainer and he loved it. This was his 1st time in one and we weren't sure what he would think. He took 2 tiny steps by himself! I was beyond excited! She's going to try him in it a few more times during therapy and if he continues to do well she will get one ordered for at home! Wed night began Eli's sleep study. We got here and they got him all hooked up. He fell asleep pretty quickly.

Which brings me to Thursday morning. This is where we still are. He was badly positioned so the sleep tech shifted him at about 3:45am. He struggled to get re situated and back to sleep. He's finally sleeping and i'm now wide awake. We leave for Wichita today and I'm ready to get away from it all for a few day!

Here are a few pictures.

Eli's new soft side helmet

Trying out the Gait Trainer at PT

Ready to go to sleep for his sleep study

2 Years

2011-08-28T07:31:00.000-05:00

Wow. It's been 2 years. 2 years ago today I was laying on an ultrasound table, excited to see our little baby and find out if we were having a girl or a boy. I knew during an ultrasound they checked to make sure the baby was well and healthy and growing well, but after 4 previous pregnancies and countless ultrasounds I had grown so used to everything being great, I had learned to enjoy the time of getting to see my little one. No other thoughts had every really crossed my mind.
Not so on August 28th, 2009. We did find out that we were having a boy. We also found out that something was wrong. He wasn't growing properly, and something was definitely going horribly wrong with his kidney. They weren't sure the extent of it all, they weren't sure what was causing it to happen, and they weren't sure if it would get any worse. But we knew that it wasn't right. From this point on our world has never been the same.
From this point on life began to slip. Slip into the medical realm. We didn't know the road it would ultimately take us down, but looking back this is where the road began. We were so scared then. The uncertainty. Looking back it seems like it was so easy then, that's knowing what we know NOW. Then we still had a glimmer of hope, a glimmer that it could be some simply growth issue that he would ultimately catch up from. Glimmers of hope that the kidney could be saved, or at worst he would need surgery to remove the one that wasn't working well. But to parents of healthy children, that sounded oh so scary.
We wouldn't have chosen to do anything different, if we had known then what we know now. Every choice we have made in these last 2 years has been well thought out and well researched. We are just as in love with our little man now as we were then, probably more so.
I simply cant believe it's been 2 years today. The amount that has happened since that day. The changes in Eli, in our family, in me. wow. I'm terrified to think ahead 2 years, but in some ways it's just as terrifying to think back over these last 2 years.
As you can probably tell by the above slightly disorganized post, I simply can't find the right words to describe how I'm feeling.
Wow. 2 years.

LOOOONG Day

2011-08-25T18:15:00.000-05:00

Little man had a long night last night making for a long day today. His feeding pump malfunctioned and he went 12 1/2 hrs without food last night. By this morning when we figured it out at 6am, he was dehydrated and very much lacking calories. Of course this would happen on a Thursday, when he has PT, OT and ST first thing in the morning. We got to therapy and he struggled and was super fussy. By the time he got to PT he was so grumpy and she really needed to work with him today. She did alot of testing with his hips and legs and she's still very concerned about it all. She tried him in some special shorts that keep his legs together and he struggled with these. It causes him alot of pain when we are trying to work to bring his hips together and to work with the right foot that is super tight. We are hoping to make progress, but it's going to be very slow.
Tomorrow morning is his MRI. We check into the hospital at 645 in the morning. They will immediately get an IV going before stopping the pedialyte running thru his J. His MRI is scheduled for 9am. I will update tomorrow as i know more!

Quick Catch Up

2011-08-23T19:38:00.000-05:00

Monday ~ Eli had his in home speech and sign language. He did really well watching signing and trying to play with his fingers and check it all out. He got to play with his speech pathologists IPad as well, and like always he loved that. Then in the afternoon the wheelchair technician came to our house. He reclined the back seat, changed around the hip/thigh piece and adjusted some straps. Ultimately he won't be able to make Eli's wheelchair do what is needed, he thinks. They are going to try and see if we can add upper body supports and such for when he's tired. Ultimately we may need to switch over to a tilt in space chair. We are waiting it out until his appt with the muscle and nerve clinic at the end of the month.

Tuesday(today) ~ We met with The Whole Person project. It's an organization that does lots of things, but the piece of it we are doing is in home ASL (sign language) tutoring. We finished all the paperwork and signed on with them for 2 years to start. We even got to meet our tutor today. She will come into our home and teach us ASL weekly. We begin next week and are super excited to get started. I spent the rest of the day on the phone. 15 something phone calls. We got most of his scripts refilled, worked on a new med schedule (AGAIN), and got all of his doctors orders EMAILED to us for his CPAP and O2 and such. It was a super busy day! Bob and I got a TON of baking and cooking done. We made up 3 snacks, 2 breakfasts, 2 loaves of dinner bread, and 2 dinners!
This evening has been pretty stressful and I'm definitely ready for it to end.
I will update more tomorrow.

Critical Neuro Appt Results

2011-08-20T14:01:00.000-05:00

Today was Eli's appt with his new epilepsy doctor/neurologist. The frontal lobes of his brain had sharp spikes and showed seizure activity. His brain overall showed decrease in activity and such. Basically his brain isn't functioning at the proper capacity/level that it should be anymore. He is also concerned on the increase in hypertonia in his left side and has ordered an MRI to figure out what is happening. He says if this can't be fixed with his AFO's and such then Eli will need to have Botox injections. He is also ordering a soft side helmet, to help protect his head. He put Eli on Keppra (a seizure medication) as well. This first week he's on a lower dose and then it's doubled next week. They will monitor from there what they need to change next. He chose the Keppra as it's safest with all of the other medications that he's on. Hoping it works.
We are going to spend the rest of the weekend attempting to relax and let all of this new information soak in, something we seem to do all the dang time lately.

Renal Doc

2011-08-18T21:35:00.000-05:00

Today was Eli's Renal appt. The doc asked tons of questions and we went thru alot of history. Then he did his physical exam. He decided that he needed to run blood and urine tests and also a renal ultrasound. Eli had ZERO interest in peeing for the test. Vascular access was able to get blood work, and the ultrasound was long, but Eli fell asleep!
Now we wait for results. Hopefully we will get them in the next day or two. Another exhausting appt where we just wait for answer.

Pulmonologist Yesterday

2011-08-18T08:16:00.000-05:00

Yesterday was a LOOOONG day. Eli had his EEG first thing. He was exhausted. He couldn't physically keep his eyes open and stay awake and she was rushing to get everything hooked up before he was completely out. Such a cruel thing to make a Mito kid go without sleep, it's definitely asking alot! He did well for the EEG and slept thru the whole thing, until the end. She had to wake him and do the strobe lights 10 times. He hates light and was soooo unhappy with it. When we left he fell right to sleep.
When we got home he took a 5 hr nap. He kept dropping his O2 and was having very strange things occur while he was sleeping. He was having strange eye movements, he was retracting and his nostrils were clamping CLOSED. His nurse took a 5 minute video of it all.
Then in the afternoon he had his new pulmonologist appt, the doc is also the head of the sleep studies team. He went over all Eli's info and past tests, as well as asked us alot of questions and watched the video from the earlier nap. He immediately was concerned. He said that it appears like seizures that are causing diaphragmatic spasms. The seizure is basically causing his diaphragm to clamp shut and it's causing so much air to suck in that it's literally causing his nostrils to suck in and the major retractions. Then if he goes without O2 to long that can obviously cause another seizure. He is concerned with the number of sleep occurances he is having. He is concerned as well that the reason Eli is such a mouth breather is because of undervelopment in the external nose and mouth area. Right in the room he picked up the phone and called the main hospitals sleep study unit. He told them he wanted him fitted for a full face CPAP mask and wanted his pressures increased and O2 added onto the CPAP. He wants Eli using it ANY time he's sleeping, be it naps or overnights.
He has also scheduled an emergency 2 Tier sleep study. They have that scheduled for the 31st unless they get a cancel. So we headed over and they got him fit with a mask. At first he was trying to rip it off, so they left it at the lowest setting of 4. As he got used to it they were able to ramp the setting up. He did great and seemed to be very calm and playful while it was on. They are getting his ordered and it will be here soon.
Here are a few pictures from his day.

Eli right before officially starting his EEG


Eli checking out his new CPAP mask


Eli sound asleep after dinner

Last night I took Emily to finally get her hair cut. She has been wanting to cut it for some time and we just have never been able to get it all set up. We are pretty open about letting our kids decide what to do with their hair and such, so this was all her choice!

Emily before her hair cut


Emily after her hair cut

the back of her hair after

Today Eli has his new appt with the renal doc. I will try and get a quick update up this afternoon after we know more from that appt. We won't be getting the EEG results until Monday.

AFO's Redone

2011-08-16T19:40:00.000-05:00

Today Eli had his appt to get his AFO's refitted. He agrees that they aren't working at all and that Eli's muscles have definitely changed. He was unsure what to do and consulted multiple other people to try and get some ideas. Eli's left leg/ankle/foot now show hypertonia(over high muscle tone) and his right leg/ankle/foot now show hypotonia(low muscle tone). He made some adjustments to the AFO's to see if that will help his feet stay in them a bit more. He says that Eli is a puzzle and he's not really sure what to do next if this doesn't work. He also doesn't have any suggestions on the legs turning out and the massive frogging weakness in the hips. He agrees that it all sounds neurological.
Eli has again been exhausted today. Which isn't good because tomorrow is his EEG. He has to stay up tonight until 10pm and then he has to get up tomorrow morning at 4am. Not going to be fun for a kiddo like Eli who already has major issues with low energy.
After his EEG he has an appt with his new pulmonolgist. I will update more once I know more.

More Neurological Changes

2011-08-15T19:18:00.000-05:00

We decided that we needed a very quiet weekend. We bought all the food we needed to cook food for 2 days, rented 6 movies, had no nurses, wore pj's, ate food, watched movies, played games, and never left our house. It was beyond nice. We ate some super yummy food and had a really nice time. I even got some knitting time!!!
Sunday evening I was cooking dinner and Eli was laying on the kitchen floor. I sat down next to him and his entire head and upper bed started shaking. I grabbed him off the floor and his body was shaking. It lasted about 1 min. It happened again 45 minutes later, this time lasting about 30 sec. Afterwards he was very glazed over and exhausted. We called his neurologist today and the nurse agrees that it sounds like partial seizures. They have him scheduled for an EEG on Wednesday. Which means virtually no sleep on Tuesday for any of us.

In other news the big 3 started school today. It was only a half day, but a nice chance for them to get back into the swing of school life. Here are 2 pics of them right before heading out the door. My silly kids! :)

More Bad News

2011-08-11T16:06:00.000-05:00

Today Eli had PT. It was disastrous. Eli was struggling to move his right leg at all. His left leg is so tight that he can't even get it in his AFO. He would stand with help and then just crumple down onto his right leg. The PT was concerned. She called his neurologist. The neurologist, hematologist, and medically complex doc all chatted.
The PT did some muscle testing and believes it's neurological. The docs all agree. They are concerned about a bleed or a blood clot in his brain, basically a mini stroke.
Eli has an emergency appt with his neurologist tomorrow to try to figure out what is going on how to stop it, or atleast slow it and what to do next.
We as you can imagine are beyond terrified of the thought that our tiny 20 month old man may have had a stroke. We will update as we know more.

Progression SUCKS

2011-08-09T19:14:00.000-05:00

Today Eli was simply supposed to have an appt with his new nutritionist. In true Eli fashion that isn't how the day went. We got there and waited for over 45 minutes. UGGGGH. Then the ARNP that works closely with Eli's complex kiddos doc came in. She had seen the massive amount of weight gain that he has had and was concerned. He is gaining over 46grams a day and he's supposed to gain 6grams a day. That a LOT. She is concerned about his kidneys no longer working properly to keep all of this flushed. Especially since he isn't peeing near as much and not nearly as often lately. We also voiced our concern over his legs and the tightness on the left side and the lack of movement on the right side. She did some range of motion and basic movements. She was as well concerned. The left side has a constricture (sp?) and is so tight you can hardly bring it up at all. The right side is dropping so bad that it's just floppy. His legs are rotating at the knee to the point you can almost completely turn it around. The hips are slightly stable at the front point and when she would continue the rotation they are becoming unstable and completely laying flat out. This is obviously not good and NOT what the body is supposed to do. This has all happened in the last week or so, well the advancement of it. He's always had the foot drop and some of those things but nothing like this. The NP was concerned that it's a neurological issue and called his neurologist immediately. She's also concerned that the bloating and swelling and fluid retention are being caused by the new HGH (human growth hormone) and immediately called the endocrinologist. The nutritionist is changing up his formula a bit, trying to lower the pedialyte as she is concerned that it's going to cause some issues with his electrolytes and sodium levels. His blood sugar levels have already been running a bit higher.
We ended up being there most of the day. We left with alot of information and alot of new emergency appts scheduled. Eli will be meeting with his neurologist, endocrinologist, a brand new nephrologist and getting ultrasounds of his kidneys.
I will update more as we know more. Right now we are AGAIN trying to sort out all of this information and process what's happening. We are so exhausted from taking so many steps backwards. Today is another one of those days that I HATE this fucking progressive disease that is taking over my little mans body.

Jace's 8th Birthday Weekend

2011-08-07T19:55:00.001-05:00

I can't believe that my oldest boy is 8 yrs old already. Time flies.
My parents came in town for his bday weekend.
Saturday we went to the KC Zoo. It was our 1st time there and everyone had alot of fun. We saw lots of animals, went on a tram ride, a boat ride, a sky safari ride, and a train ride. Their favorite was the sky safari!
Here are a few pictures from our zoo trip.

Afterwards we all went out to dinner at the Melting Pot. The kids loved the experience.
Jace opened presents after dinner.


an accessory kit for his new ipod


Jace got the Ipod he has been wanting so badly

Today was grocery shopping and just laying low. My parents headed home today as well.
Here are some random pics from this last week.


My super handsome always smiling husband


Jace playing angry birds on bob's cell phone


Tiny man hauling all the tupperware lids out. His favorite game.

Here are some pictures from Eli's therapy last week.

Eli working with glue during sensory time at OT

Eli working with signing at ST. He actually signed sheep!


Eli working at PT, trying to walk behind a push toy.

lastly here are 2 pictures from Emily's Macbeth performance. It was dark so I had trouble getting many good pictures.

Emily playing the part of Malcolm

tiny enjoying watching Emily's play

Enjoy the photos! I promise to get more up soon!

My boys

2011-08-04T11:20:00.000-05:00

It seems as though my boys are all falling apart!
Jace ~ He's got a cough going on and now some sinus stuff adding to it. Saturday is his 8 yr birthday and he's sick. Go figure. He usually bounces back quickly so I'm assuming he'll do the same here.

Benjamin ~ He saw the doctor here a few weeks ago. She was concerned about his size and nutrition and behavior and decided to run multiple tests. She called us late yesterday with the results...not what we were expecting. On allergy testing a 2 and above means you are allergic. Ben scored an 18!!! He's allergic to wheat, dairy, and eggs. She said that these items are literally poisoning his body. She is having him meet with a nutritionist and very possibly a feeding therapy team. As most of you know, atleast those who know Ben, he only eats a few foods, and the few foods he eats are ALL on the above list of severe allergies. Go figure. Benjamin doesn't have not 1 single drop of weight to lose. This is going to be a long hard road.

Eli ~ We are still waiting on his MRI results. I have another call into the doc but haven't received a call back. Also a problem was noted at PT today. Eli's only using his left leg. He's turning his right leg completely out and dragging it behind his body, refusing to use it at all. His PT isn't sure why this is happening or what to do about it. I'm going to call his doctor to find out who I need to contact to get this newest thing figured out.

More updates soon!

Quick MRI update

2011-08-02T19:28:00.000-05:00

Today was little mans MRI. They were late getting his IV and getting him switched over to fluids. So his J feeds ran pedialyte until 1 hr before the sedation. The anesthesiologist came and talked with me for quite a bit discussing how he wanted to run the procedure in light of the Mito. He decided to do a full general anesthesia with intubation. Eli did suprisingly well. He's been really sleepy the rest of the day and has had quite a few day time O2 dips but nothing compared to how he usually reacts to anesthesia.
We met so many people today who were shocked over the Mito diagnosis. I got many questions and wow we haven't seen someone with it, and i heard about it in school and all that. A bit bothersome when people are so fascinated by actually meeting someone with a disease that isn't common.
anyways i'm sleepy tonight so more soon.

Damn Mito

2011-08-01T14:11:00.003-05:00

I worked 4 hrs this morning packing up an unairconditioned upstairs loft that was full to the brim of collectibles. Fun times. Now I'm home cooling off and relaxing a bit.
My dear husband got the honor of taking a brand new nurse and ALL 5 children to Eli's doc appt. Mind you it's 104 degrees outside and this is Eli's complex kiddos doctors so these appts usually last about 2 or 3 hrs. The doc is again concerned about Eli. He has gained almost 2 lbs of weight in a week. It's all fluid retention. The doctor is concerned that his renal system isn't properly working. This much fluid retention can cause alot of pulmonary and cardiac issues, not to mention the pressure it in general puts on the renal system. The doc is running labs, looking back thru old labs trying to spot something that may not have been noticed in the past, and calling some docs in for help. If it's not one thing then it's another. I so wish we could just get to the bottom of it all and make it all better. But the problem is we have gotten to the bottom of it all and it's fucking mito. it's incurable. there isn't a damn thing we can do about it to make it better, to make it go away. i always hate mito, but some days i hate it even more. today is one of those days.

There is 1 other thing on my mind and heart. An incredibly dear friend of ours just received horrible news. She has bladder cancer. She has been there thru every step of this journey with Eli. Not only has she been his nurse from day 1, but she has been one of my very best friends and part of our family. I don't know how our family would have gotten thru this last 20 months if it hadn't been for her. Eli and I will be making a trip into Wichita soon to be there with her. If you can all please say a quick prayer for her and her family right now as she gets ready to undergo surgery, chemo, and everything else. thanks...oh and you guessed it...i HATE cancer AND mito!

Trying to Stay Afloat

2011-07-29T20:39:00.000-05:00

The title of my post pretty much sums it all up. I'm sure most of you reading that immediately think money. If you did, you would be partially right. Money is tight. As most of you know Bob was laid off right after we moved here to KC. So obviously money has been very tight. I am helping out a friend who works for a moving company and doing a bit of packing. It's nice to get out a bit and meet new people, and help earn a touch of money to help out the family. It's not an actual job. It's something I've done 2 days and will only do once in a while when she has a packing job and I'm able to sneak away from Eli's very demanding schedule.
HOWEVER...money isn't our big issue. It's life and health and all of those things in between. We are exhausted. We are exhausted for us, for our kids, for our little man, and for all of the families out there that we support and love oh so deeply. It's a constant battle to keep our little man going, let alone the rest of our household. We are exhausted, all 7 of us.
My heart breaks for us, for my tiny man, for all of the parents and fellow tubies and mito kiddos, for all of the "normal" siblings out there, and for everyone else involved in our lives. The pain that our community faces on a minute by minute basis is overwhelming.
At our care plan meeting while Eli was inpatient, our team discussed sooo many things with us. They discussed that nothing was going to be a miracle cure, that we had to always remember that Eli's underlying diagnosis trumps all others and that "typical" cures for all of his side diagnosis's won't work like they normally would because of the mito. They reminded us like always that we have to settle for less, less in everything from medical treatments to quality of life. They talked about how they know we didn't pick this road, how hard it is to have a kiddo like Eli and how much of a daily struggle it is to keep him going. They acknowledged how much work it was, and offered up expanded nursing and more round the clock care. They told us how good of a job we were doing and they said they can only imagine the amount of time, work, and energy it takes to keep afloat with a kiddo like Eli. Currently we have chosen to turn down the increased nursing hours. We feel like we already have sooo many hours a month (345 hrs a month) and we aren't even using all of them. The doctors don't understand how hard it is to find good nurses. It's simply not worth it. But none of it changes the fact that we are freaking exhausted. No I'm not saying we are going to give up, not like that's even a choice in my opinion. We are simply saying we are worn out. It takes everything we have in us to get up each morning and do it all over again.
We currently have many friends struggling right now...when don't we. One of them is my nearest and dearest "little sister" who I have known since her mother was pregnant with her, and the other is one my best friends, the person I call when I feel like Eli's world is crashing down upon me, the one who gets it with no words needed. I feel as though I'm letting them down. I haven't been available, I haven't been supportive, I haven't checked in like I should, I haven't physically been there by their sides. I've been so wrapped up in simply keeping myself and my family moving in a forward going direction. I feel as though I have nothing left in me to give.
Some days it's a minute by minute kind of day. Today is one of those days. I stayed afloat today...

As I know everyone wants kiddo updates... :)
Emily~ Tonight was her final Shakespeare performance. She did great and said all of her lines beautifully. She had a great time over these last 3 weeks and learned alot!
Jace~ He's been a super busy man. He went swimming today and helped a friend nail down some 2 by 4's. He loves working with tools. Jace's new meds seem to be slowly working. His doctor doubled the one a few weeks ago, and they added a new one last week. We are hoping to really be making progress within the next few weeks.
Ben~ He's been doing decent. He's been stimming alot more lately, but hasn't had tooo many outburst. He also went swimming today, and seems to really enjoy the water. He's also one 1 new med, same as Jace, and will be getting his other med tweeked in the next few weeks.
Ari~ She's busy as always. She went swimming as well and got to water flowers and help out in the garden at a friends house today. She enjoys being outdoors and loves checking everything out. She also fed the birds and thought that was beyond amazing!
Eli~ He's still struggling. He is bloated and swollen to the point of uncomfortable. He's back to bright red and splotchy and sweating constantly. He ran low grades all day yesterday and has had a bit of a higher heart rate. His sugars have been good so that part is a plus. He has been alarming more at night lately, but we are thinking that's mostly due to the heat. His sedation MRI is coming up on Tuesday so we are nervous about the sedation and the results. The anesthesia team called today to go over his NPO orders, and I'm not fond of what this anesthesiologist has put into place. I will be talking to his doctor on Monday to see if we can get the orders changed around a bit.
I'm exhausted so I'm going to close for now. Going to try and watch some TV for a few and then it's off to bed to do it all again tomorrow.
I need a vacation...heck we need a vacation. All 7 of us (and Eli's nurse) need to just disappear into the wild blue yonder for a full week. hmmmm....now to just figure out which tree will be growing the money for this dream! :)

Home

2011-07-26T16:27:00.000-05:00

Yesterday was supposed to be Eli's MRI. They got the IV going, stopped his feeds, and sent us down to MRI. We got there and they wanted to do a KUB to verify that the J feeds weren't going into his stomach. They read the KUB and claimed that his J tube was in his stomach not J. So we went back up to his room and they waited to go down to IR to get it changed out and replaced. The doctor immediately said his tube isn't in his stomach. So the idiot that read the KUB misread and we could have had the MRI after all. I was so frustrated.
They decided to simply do the MRI outpatient. The hospital was able to get Pfizer to donate 1 month worth of Eli's growth hormone($3000 worth of meds!). So we were able to come home!! We are so happy to be home.
We will be at the hospital ALOT over these next few weeks but atleast we will have some time home!
Tonight my dear hubby has planned to take us all out to dinner. The 7 of us rarely EVER go out so I'm excited to get to tonight!
More updates and some pictures tomorrow

Quiet Weekend / Start of Week

2011-07-25T07:29:00.000-05:00

Day 7
It was a fairly quiet weekend. Eli started his Growth Hormones and did pretty well with them so far. They have raised his BS a touch and kept his BP slightly low, but nothing to concerning. The docs have also decided to up his feeds a bit. We did a calorie increase yesterday going from 22kcals to 24kcals. Still mixing with the pedialyte which adds 3kcals more per ounce. He seems to be doing pretty good with it. They are concerned if we don't get his calories increased decently, that the growth hormone will kick in trying to push his body to grow, and if Eli doesn't have enough extra calories to support that his body will break down things it shouldn't for energy.
Eli has had alot of grouchy time these last 2 days. He's done with this entire hospital stay. Normally when we are here he's actually sick. But right now we are here solely to get this shot every day. That's causing for a bored grouchy man. I totally get it though! :)
Today is hopefully Eli's sedation MRI. Since he has the growth hormone issues and the front half of the pituitary gland not working he has to have the MRI. There is a slight chance that a tumor is what is causing all of these problems, although they don't believe that is the case. He will also be getting his GJ tube swapped out today.
I will update more as I know more!

Another Diagnosis Means Hospital Life Continues

2011-07-22T19:06:00.000-05:00

Yesterday was a long day. Eli had his endocrine/adrenal testing. It started with having to get an IV placed which is never an easy task with Eli. Then he was given the first phase of meds. They caused his blood sugars to go all over the place. The second phase brought another medicine. It messed up his blood sugar, dropped his blood pressure really low, made him sweaty, and put him to sleep. He had to be fasting for this test and someone screwed up and didn't get fluids ordered. By the time the test was done his body was bouncing back. He wasn't peeing and ended up needing bladder ultrasounds and IV boluses.
Today began with lots of docs and checkins and such. This afternoon was his team meeting. There were 10 plus doctors there. His endocrine tests came back showing a problem. He has a growth hormone deficiency. The entire front half of his pituitary gland isn't working. He will be started on growth hormone injections tonight. Normally they do 5 a week but Eli requires them 7 days a week. He will remain inpatient until the meds are started and going properly AND until they have all the approvals thru insurance and the meds ready to deliver. So we have to soooo hope that there are no denials or appeals as that would mean we could be here for months. His endocrinologist doesn't believe it is safe for him to be outside of the hospital without this medication.
They believe that this issue is causing alot of the sweating, dehydration, fluid retention issues, inability to control his body temp, and even some of the fatigue. The endocrinologist did caution us and all of the other doctors there to remember that Eli does have an underlying issue, Mitochondrial Disease, that is also contributing and may block the meds from being a "miracle cure". Right now we are just hoping for some relief.
We have a long road ahead and everyone admits that. There were alot of differing opinions and plans of care ideas at the meeting, but the difficult road ahead isn't one of them. There was discussion about us not choosing this road we are on, but having to follow it. How hard it is and how rough it is to see him suffer. How there are no other options and how we simply have to do what we can. I hate these kinds of conversations, and they are a real struggle to get thru.
We got moved to a more long term stay room with more space and a bathtub for little man tonight. We also got moved to a new team that has residents and such available at all hours of day and night, as well as an attending available during the day. We will see how it all goes. Bob brought up the 4 older kids and we did pizza for dinner and now everyone is watching a movie. Little man is exhausted but enjoying having time with his siblings.
We will update as we know more. Right now we are as always trying to learn how to cope with all of this new information the last few days and how to continue to be as much of a family as possible while having Eli and I live inpatient.

Lots of Info

2011-07-20T20:38:00.000-05:00

HOLY COW. It's been a heck of a day.
Eli's day started off with a meeting with his inpatient lead. She has some plans and is also coordinating a meeting with ALL of Eli's doctors.
Pulmonologist ~ Wants to do some basic sleep study type things. Wants more O2 monitoring and wants to figure out why on CPAP Eli seems to do worse, not better. Also wants to get the carseat issues figured out.
GI/Nutrition ~ Going to be working on changing his feeds. Trialing no pedialyte, as they are concerned it's causing alot of the swelling from all the extra sodium.
Endocrine ~ They have quite a few ideas as to what they think is going on but are going to be running over 16 tests tomorrow to try and figure it out for sure. They have also changed his sugars from every 6 hrs to every 2 hrs. The doctor is pretty aggressive and pretty thorough so we will see how it goes.
Eli met with Speech, OT, and speech/audiology. They got all of his therapies immediately regoing so that we don't lose any of the things we've been working towards.
Eli also got his AFO's today. They were able to get us in a few days early since we were already inpatient and they had a cancel today. He wore them for about 1 1/2 hrs. Hoping to get a bit more time with them on tomorrow.
I've left our biggest visit for last. Hematology came by. They have all of his lab results finally in. His genetic chromosomal testing came back abnormal. He has a condition called Factor V Leiden. This causing his body to struggle with controlling when it stops clotting. It increase his chances of having more blood clots, along with his risks of already having a clot and his other issues. We weren't expecting this at all. We all assumed that all of the testing would come back clear.
Eli is exhausted this evening and so are we. There were alot of meetings today and alot of information. Tomorrow we begin testing at 8am and it will apparently go most of the day. I will update when I'm able.

Rough Night

2011-07-20T06:08:00.000-05:00

Tiny mans blood sugars dropped around 4am. Doc paged. Wanted a ton of labs plus an IV to run some d10. 1 1/2 hrs later we still couldn't get labs or an IV. sugars at this point fly up the opposite direction. Doc says hold on IV and labs and give him a break. They will recheck his sugars at 7am. Nurses want to have a serious talk with the doctors about needing a PICC line at minimum. I'm not looking forward to the talk but my poor little man is miserable and it's a major risk.
Will update more this morning as I know more.

Admitted

2011-07-19T20:46:00.000-05:00

My little man was admitted this afternoon.
We met with his medically complex doc and talked for quite a bit. He's concerned about the swelling. He wants to figure out if it's caused by the adrenal system, his kidneys, or his heart. He also wants to get his feeds and fluids situated as Eli is struggling with these still.
We got here to the hospital and found out that the NP who had read his CDiff test was wrong. Eli does NOT have Cdiff. So now there is alot of confusion as to why he has pieces of his intestinal wall in his diapers. Something wacky is clearly going on with his GI system and they aren't sure what and why.
Eli is beyond exhausted at this point and it's just crying and crying and crying. He's swollen and grumpy and tired. Will update tomorrow when we know more.

Quick Saturday Updates

2011-07-16T19:04:00.000-05:00

Dr KT called late Friday afternoon. He doesn't want to admit on Monday as he is finishing up a nursery rotation and he wants to be in charge of a direct admit, not a voluntary admit. So he is having us come into the office on Tuesday right before lunch and he will do a direct admit from there. He is admitting Eli to the chronic care team at CMH. This team handles the long term resident patients. He feels that they will be best suited to help Eli with what is going on.
Right now we are just working to keep up with his hydration. He's had alot of diapers today and his nurse has really had to work to keep up with replacing the fluids, without losing calories. He's still been running his low grade fever. We are hoping the docs can get it all figured out.

In other news...Emily has finished her first week of Shakespeare and has enjoyed it. They are working to make costumes and she's learning her parts. All of the children have been in rare form lately. They aren't looking forward to this upcoming admit for Eli and are struggling to deal.

Bob and I went out last night. An incredibly dear friend of ours kept all 5 of our kiddos. Their little girl is just like Eli so she's super familiar! Bob and I went out with friends to dinner and dancing. We had a fabulous time. We didn't get to bed until 2am and got to sleep in until 8:30....which for us is incredibly late. We went out for breakfast together and then collected our children! :) It was our first time in almost 20 months to not have any children for an entire night. We are so blessed!

Results In

2011-07-13T15:58:00.000-05:00

The same doctor that thought we were all nuts and that Eli couldn't possibly have Cdiff...the same doctor that called me yesterday morning saying that since 1 result showed positive and 1 result showed negative and she was just certain that it wasn't positive, called me and said it's negative and he doesn't have CDiff. So this morning this same doc calls and says oooops. The 24 hr culture came back and sure enough he does have Cdiff. Humph. So Eli does in fact have Cdiff and we have wasted days that we could have already started the medicine.
Meds ordered and being delivered. We will update as we know more.

Admission Scheduled

2011-07-12T20:03:00.000-05:00

Today we met with Eli's GI doc. There was alot of conversation and we were there for over 3 hrs. He is concerned about Eli's bowel issues. Eli's test don't appear to show CDiff. Which is good. The bad news is he apparently is still passing pieces of his intestinal walls because his GI system is so stressed out. He's also having alot of diarrhea still and his bottom is so swollen and red. He has a yeast infection on his bottom from all of it. His urine is showing problems with clearing out his system. With the amount of fluids he's getting he should be passing more waste and he isn't.   Which means his body is holding waste. His kidney functions look ok though. He's still struggling with hydration and the more fluids we push the more his GI system freaks out. He's still sweating and his body temp is struggling even more to keep control. When we got to the doc he was as red as a lobster from head to toe, with heat rash all over, and over 100 degree temp. His docs talked while we were there and have decided that all signs and symptoms point to an adrenal crisis. They don't believe that his adrenal system is properly regulating hormones and levels and such, which is making it impossible for his body to figure out what to hold, what to dump, and when to do it all.
They have decided to begin a long term admission on Monday. They don't want to start one mid week as it's hard to get everyone organized.   So the rest of this week they organize and make plans and we admit monday. Tons of phone calls, tons of paperwork. His goals for discharge are HUGE.
He has to be able to gain steady real weight. They've given him his FTT back. (failure to thrive) as he isn't gaining anything but bloat weight that he loses the next day. He has to be able to maintain his hydration without upsetting his GI system.   We have to have the excessive sweating and adrenal issues under control.
To some these may sound like easy goals but for a kiddo like Eli who is already so fragile, these goals are massive.
Eli's not thrilled and isn't feeling well at all. His body is bucking everything right now and not handling much.
We will update as we know more.

Another threat of admission

2011-07-11T18:28:00.001-05:00

Hemoc~ We've gotten Eli's 1st repeat round of testing back. The test that shows if he has a 15% chance of a blood clot in the next year came back negative this time. His hematologist thinks that it was so high because of the trauma getting the blood. We were all excited that we didn't need blood thinners and his doc said don't get excited...we just don't need them yet. So we keep waiting for the rest of the tests but his doc isn't optimistic.
Eli met with his complex kids clinic today. He met with a nurse practioner. It was a mess. First she wanted to admit him. She is concerned about his bowels and hydration. We voiced our concern about admitting him simply to force his docs to talk and make a choice. We were just inpatient last week and they were going to get all the docs on the same page and that it didn't happen. We explained that we can't admit each week to try and make his docs communicate and get a good care plan. So she ultimately agreed. She has added 2 new meds and again wanting to change around some of his feeds/fluids. She scheduled a 2 plus hour with his main medical complex doctor next week, and is also working to arrainge a care team meeting where all of his main docs meet in 1 room at the exact same time. Also some discussions need to happen on the fact that there were 2 pages of orders from Dr Shoffner and nobody is following them or letting me know what they are. hmmm. So we will see how that goes.
In the mean time they are running lab work as she's wondering if he has CDiff. If that's the case he's a guaranteed admit. We are hoping to still get those results tonight. He's running a low grade again today and we are struggling to keep him cool and hydrated with this massive heat. Our AC just isn't keeping up.
We will update as we know more.

Hemoc Called

2011-07-07T11:33:00.001-05:00

Eli's hemoc called. The test that was to check his possibility of having another blood clot in 1 year came back. We were told if his was higher than 15% then he was back on blood thinners. Well his is so high that they are rushing him in for am emergency ECHO and ultrasound. They are concerned that his blood clot in his heart, that was supposedly turned into a calcified leason that was growing is possibly a blood clot, potentially more. The risk for him throwing clots right now, if not already is super high. His hematologist is concerned and has called the cardiologist. We are scared, and his hemoc PA said rightfully so. We go in at 1:30 for echos and ultrasounds and tons more lab work. We will update more as we know more. please keep our little man in your thoughts and prayers and please keep our family as well...we are exhausted and feel like we are nearing the end of what we can emotionally, mentally and physically handle.

Gi for Jace and Hemoc for Eli

2011-07-06T17:55:00.000-05:00

Today was a long day.
This morning Jace had his 1st establish visit with his new GI doc. We decided to have him see the same GI doc that Eli sees for ease of purpose and he's a damn good doctor! We discussed Jace's GERD and CVS, and he believes that Jace most definitely has IBS as well. Which really isn't a shocker at all. He wants to continue with the same meds as they seem to be working pretty well. He wants deeper monitor on the GERD episodes and this will help him determine when he wants the next scopes and biopsies. Will recheck in 1 month.
This afternoon my little man had his appt with Hemoc. They had set this up for approximately 1 month after ending blood thinners. It was supposed to be an open and shut case appt with basic lab work. Not so. They discussed multiple things, including the confirmed Mito. In light of how fragile Eli's body system is he is completely changing his plans. When we get our next line he wants 1/2 strength blood thinners. He's also running a special test to check the probability of having another blood clot in the next year. If this comes back above 15% we are back on blood thinners. Lastly if Eli has 1 single blood clot then he's on blood thinners guaranteed for life. He will continue to follow Eli closely and wants us to make sure that ALL docs talk with him when there is any discussion on a new line. We weren't expecting this at all. He ran a TON of lab work. They needed more blood than Eli's body will allow, so we hit his body max at 20ml. It took over an hr, 2 sticks, and 4 nurses to get all of it. He's beyond exhausted and pretty dehydrated from the sweating, shaking, crying and blood loss. Hoping for a quiet evening at home to let him rest a bit.
More coming this week, but for now that's all. i'm exhausted.

Home from the hospital

2011-07-05T20:57:00.000-05:00

We are home. Eli got discharged this afternoon. We really loved his inpatient doc this time and were very lucky to have gotten onto the silver team with him! Eli's blood sugars are low enough to earn him the title of hypoglycemic, but not danger level. His mito doc, Dr Shoffner, does however say that they are going to make him feel way crappier than a normal kiddo. Eli now owns his own glucometer and all the supplies that go with it. He will test 6 times a day, and any others PRN. There is a base plan in place for when his sugars drop, and a more detailed plan will be made over the next few days. Dr Shoffner also wants his fluids increased by a lot. He wants 120ml/ per kilo, and currently Eli is getting 96.8ml/per kilo. So we are way short. The likelihood that Eli's body can handle that kind of increase is pretty slim. There are going to be meetings and talks tomorrow to try and figure out what next. They are also pushing endocrine to get him in quickly.
Tomorrow Eli has his hematologist and we are looking forward to what they have to say about his clotting and anemia.
As of this morning Eli is running a super low grade, and is really sleepy. We are hoping he didn't pick up anything inpatient. Only time will tell.
We will update more as we know more over these next few days.

Quick Admit Update

2011-07-04T18:42:00.000-05:00

Docs rounded, and so did nutrition. They did blood sugars every 1 hr this morning and around 2pm switched over to every 2 hrs. Now that he's off his feed for his evening break they are doing every hour again. His sugars are dropping now.
Doc is simply monitoring all day. Tomorrow we will have long chats with his pediatrician, nutrition and endocrine to figure out what is going on and what we need to do next. He's still got a rash on his face and arms and a touch on his legs. He's still red and a bit sweaty, not as clammy. He's incredibly sleepy and has been asleep or sleepy fussing most all day.
We just got back from the playroom where he spent almost an hr playing. Now he's laying back on his dad in the rocking chair watching a baby einstein video. Hoping he is able to get some decent sleep tonight. As of right now bob and i are at 38 hrs awake with MAYBE 3 hrs of cat naps. So we are dragging.
A dear friend of ours is sending us dinner tonight and a few extra meals to help cover food for the next day or two! We are so thankful to have amazing friends in our lives!
We will update tomorrow as we know more.

Eli's admitted

2011-07-04T09:31:00.000-05:00

Eli's blood sugars have been running low since Friday evening. He has had alot of episodes of sweaty clammy to go along with it. We called the doc yesterday when his sugars had consistently stayed low. She had us bring him up to CMH. We packed him up and made the drive from Wichita into KC. We got to the ER around 12:30am and were admitted around 430am. Waiting on the doctors to round this morning to figure out what next. We will update more when we know more.

Quick Thursday updates

2011-06-30T08:34:00.000-05:00

still here. Just busy.
This week has been Emily's girl scout camp. She got hurt her 1st day there but has done well the rest of the week. She's enjoyed it and met friends. We are so glad that she's done well.
We have kept the others fairly busy. We've been swimming, to the Y, to dinner and a s'mores roast at a friends house, on walks, had special snacks, and watched movies! :) Today we are going to a friends for a special craft project!
Eli has struggled this week yet again with his weight and hydration. He also had constipation issues this week. His docs are all talking to try and figure out what next. My little man lost another 3 oz today. He's going to wheelchair clinic today to see about getting his straps fixed on his chair.
We are super nervous about these next few days with this horrible heat coming. We are travelling tomorrow and it's supposed to be 103 here and 105 PLUS in Wichita. Hoping my cars AC can keep up with that as Eli's body sure can't.
More updates soon.

Monday Morning Memos

2011-06-27T10:24:00.000-05:00

Yesterday was a nice day. We played games and hung out in the morning. We let little man get a nap while we all ate lunch. Then we headed out to our local rec center for swimming. They have the most amazing indoor pool. There are 2 separate pools. The one we spent the most time in never goes deeper than 3ft 3in. So the little ones were able to stand and be in the entire pool. It is a zero depth entry with a big water stand that flows along these big gutter lines. You can block the water and manipulate it. Then there is a huge tower that randomly dumps bucket, big spray areas and fountains, a lazy river that was beyond fun for the kiddos, and a huge slide. They also had noodles and swim paddles and such. Then the 2nd pool had a medium depth area for the older 2 with water basketball and such. As well as swim lanes. The oldest 3 went down the slide more times than I can count, and everyone enjoyed alot of rides around the lazy river. Eli wasn't sure what to think of the water, and was very nervous at first. He never specifically played in it, but did relax a bit and enjoy just being held in the water. About 45 minutes into it he started getting really fussy and his skin was cold and moddled. Bob and I took turns keeping him out of the water and cuddled up under a towel tucked against one of us. He was beyond exhausted and darned near crashed on us. He at this point got super hot and red and splotchy. Gotta love issues with controlling body temp. In the end we were at the pool right over 2 hrs. Everyone had a great time and we will definitely be going back soon!

Today Emily starts girl scout camp. She's excited to go and meet people and try out all of the activities. The boys aren't quite as happy about her leaving! :)

Yesterday I re-posted something that has been circulating around facebook. It was something that greatly touched me and brought tears to my eyes. It was an author unknown piece, not something I take credit for at all. It was beautifully written and an amazing tribute. I hope everyone else enjoys it as much as I do.

This coming weekend we head back to Wichita for the holiday. The kids are excited to see their grandparents and I'm excited to get together with my friends. Bob and I are even going to sneak out for an evening together! It will be nice to have a bit of time with everyone to just relax.

Today Bob is at a job interview. Fingers crossed that all goes well. Then this afternoon I need to get a menu finished up and head to the store. We are on vegetarian week 2. The kids are doing great. Last night we again tried a new recipes and everyone loved it. We've had to reconfigure our fridge to allow room for all the fruits and veggies. There are so many things that I never thought I would like, foods I have never been willing to try...my kids are the same. But we have been experimenting and trying so many different foods and so far haven't found many things we don't like. I'm so proud of our journey into a greener healthier lifestyle. The kids are doing amazing and we are hardly getting any complaints at all. Makes us more excited to take our next steps in the next few weeks!

Upcoming this week:
Emilys camp...Eli's AFO fittings, 4 different therapies, Eli's new glasses, new meds, new appts schedualed, feeding/weight issues figured out for tiny man, new vegetarian recipes, our trip to wichita. (I think that covers it but our week is constantly changing!)

More udpates and pics soon!

Some Mothers Get Babies with Something More....

2011-06-26T15:16:00.002-05:00

SOME MOTHERS GET BABIES WITH SOMETHING MORE… My friend is expecting her first child. People keep asking what she wants. She smiles demurely, shakes her head and gives the answer mothers have given throughout the ages of time. She says it doesn’t matter whether it’s a boy or a girl. She just wants it to have ten fingers and ten toes. Of course, that’s what she says. That’s what mothers have always said. Mothers lie. Truth be told, every mother wants a whole lot more.

Every mother wants a perfectly healthy baby with a round head, rosebud lips, button nose, beautiful eyes and satin skin. Every mother wants a baby so gorgeous that people will pity the Gerber baby for being flat-out ugly. Every mother wants a baby that will roll over, sit up and take those first steps right on schedule. Every mother wants a baby that can see, hear, run, jump and fire neurons by the billions. She wants a kid that can smack the ball out of the park and do toe points that are the envy of the entire ballet class. Call it greed if you want, but we mothers want what we want.

Some mothers get babies with something more. Some mothers get babies with conditions they can’t pronounce, a spine that didn’t fuse, a missing chromosome or a palette that didn’t close. Most of those mothers can remember the time, the place, the shoes they were wearing and the color of the walls in the small, suffocating room where the doctor uttered the words that took their breath away. It felt like recess in the fourth grade when you didn’t see the kick ball coming and it knocked the wind clean out of you. Some mothers leave the hospital with a healthy bundle, then, months, even years later, take him in for a routine visit, or schedule her for a well check, and crash head first into a brick wall as they bear the brunt of devastating news. It can’t be possible! That doesn’t run in our family. Can this really be happening in our lifetime?

I am a woman who watches the Olympics for the sheer thrill of seeing finely sculpted bodies. It’s not a lust thing; it’s a wondrous thing. The athletes appear as specimens without flaw – rippling muscles with nary an ounce of flab or fat, virtual powerhouses of strength with lungs and limbs working in perfect harmony. Then the athlete walks over to a tote bag, rustles through the contents and pulls out an inhaler. As I’ve told my own kids, be it on the way to physical therapy after a third knee surgery, or on a trip home from an echo cardiogram, there’s no such thing as a perfect body. Everybody will bear something at some time or another. Maybe the affliction will be apparent to curious eyes, or maybe it will be unseen, quietly treated with trips to the doctor, medication or surgery.

The health problems our children have experienced have been minimal and manageable, so I watch with keen interest and great admiration the mothers of children with serious disabilities, and wonder how they do it. Frankly, sometimes you mothers scare me. How you lift that child in and out of a wheelchair 20 times a day. How you monitor tests, track medications, regulate diet and serve as the gatekeeper to a hundred specialists yammering in your ear. I wonder how you endure the praise and the platitudes, well-intentioned souls explaining how God is at work when you’ve occasionally questioned if God is on strike.

I even wonder how you endure schmaltzy pieces like this one saluting you, painting you as hero and saint, when you know you’re ordinary. You snap, you bark, you bite. You didn’t volunteer for this. You didn’t jump up and down in the motherhood line yelling, “Choose me, God! Choose me! I’ve got what it takes.”

You’re a woman who doesn’t have time to step back and put things in perspective, so, please, let me do it for you. From where I sit, you’re way ahead of the pack. You’ve developed the strength of a draft horse while holding onto the delicacy of a daffodil. You have a heart that melts like chocolate in a glove box in July, carefully counter-balanced against the stubbornness of an Ozark mule. You can be warm and tender one minute, and when circumstances require intense and aggressive the next. You are the mother, advocate and protector of a child with a disability. You’re a neighbor, a friend, a stranger I pass at the mall. You’re the woman I sit next to at church, my cousin and my sister-in-law. You’re a woman who wanted ten fingers and ten toes, and got something more. You’re a wonder.

author unknown

Updates and Pictures

2011-06-25T18:02:00.001-05:00

Our kiddos had a great time at VBS. They met alot of new friends, learned new songs, made lots of cool crafts, and played lots of games. On their last day, Friday, we took tiny man and went for their assembly and stayed for the carnival. Here are a few pictures from VBS.

Our good friends kiddos and ours. youngest to oldest.

Eli and his best friend


our good friends kids and our kids!

Eli enjoyed getting to go out and see people. All in all he's done pretty well. He's been running a low grade fever of and on. He's also been super fussy. I have had to talk to his dietician multiple times lately as Eli is losing weight. We have upped his feeds 3 times now and he's still losing. We are on this current increase until monday and then we up once more. If that doesn't work it's in the doctors hands. Here are a few pictures of my tiny from these last few days.

Eli and his puppy (from his best friend pictured above with him)


My super silly little man

Today I went and got my hair done. I got it colored and cut and I love it! Here's a pic!

Tomorrow we are taking all of the kids swimming. They are really looking forward to it. We found an indoor pool at our local recreation center with the zero depth entry. It will be perfect for Eli to actually get to go with the family and enjoy water, without having to deal with the heat of outside.

This week wrapped up our 1st full vegetarian week. As many of you know we have been slowly switching our family over to vegetarian food for the last few months. We started with 1 meal a week and slowly increased as we found more recipes and experiment more with foods. This week we had only vegetarian. Lots of fresh fruits and veggies and lots of super yummy cooking. The kids did great with it and we are really loving it. We also made more household changes. We've done cloth diapers, we've used towels and such instead of paper products. This week we also made our own household all purpose cleaner, dish soap, toilet scrub, window cleaner, and laundry detergent. We've taken the process slowly and switched over 1 item at a time.
We'll see what's in store for next week! :)

Quick Updates

2011-06-22T15:40:00.000-05:00

Multiple calls from docs yesterday.
Eli's main pediatrician called to again discuss all of the test results and what the "plan" was at this point. He is getting all of the orders done for all the new meds, a well as for the AFO's, car seat testing, and endocrinologists. He agrees with the possible need for growth hormones although he definitely wants the endocrine doc to deal with all of that. Discussed the fluid needs and all of those things a bit more as well.
Also got a call from the urologist saying that he urine catch looked good. Everyone is assuming at this point it is in fact a hydration issue not a kidney issue. So that's good.
Eli has also been offered a spot in a preschool class starting this fall. It's thru his therapy center. He would go 5 days a week for 2 1/2 hrs each morning. He would get intense OT, ST, and feeding therapy. We aren't sure if this is something we are willing to do, as he will only be 20 months old when class begins. It is something we are thinking about, but no firm decisions.
Eli also met with his in home speech therapist this last week. She brought him a signing language DVD and he watched it for the first time yesterday. He enjoyed it. We are pushing more and more with signing and hoping to start seeing some glimmer of understanding soon. We are also working alot on mimicing and hoping to see some soon as well.

Eli has struggled a bit these last few nights with his O2 levels and has dropped pretty quickly after falling asleep. No other symptoms so far so we are hoping it's just one of his weird body phases that he goes thru sometimes.

The oldest 4 are busy this week with VBS. They are really enjoying it. It's been a great time for them to get out and meet new people. This weekend we are going to go swimming for the first time and the kids are super excited about that. It will be Eli's 1st time ever to go to a swimming pool. Hopefully he enjoys it as well.

Bob and I are about the same. Just making it thru each day. We are hoping to get back to the gym next week and really looking forward to having that time to exercise and de-stress a bit.

I will update more later this week as we have more info!

A Few Pictures

2011-06-21T06:51:00.000-05:00

Neurologist, Optometrist, and Speech

2011-06-20T19:07:00.000-05:00

Eli's neurologist called today. He and I spent almost 20 minutes on the phone. He simply discussed all of the test results (which by this point we already knew). He also discussed all of the amino acids and vitamin supplements that he wanted to start. He is upping his levocarnitine since he's been on that for months and his levels are still showing major deficiencies. He also discussed a sick plan that needs to be in place. Fast fluids, and using d10, also quick use of TPN as needed and all of those types of things. He agreed that these things can't wait on a mito kid like they can on a "normal" child. He is going to get a full care plan put together as well as get all of the meds situated. He also is sending out a referral to the endocrinologist as he's concerned about Eli's lack of growing and excessive sweating with sleep.
I'm so hoping we are finally moving in the right direction with all of these test results.

Eli also had an appt with his new optometrist today. He says there was just a slight shift, but not enough to change his script. We also discussed Eli's light sensitivities. He is going to change Eli's lenses over to the transitions lenses so he will have some protection from bright lights. He is going to see Eli every 3 months to keep a close eye on the health of the retina, cornea and optic nerve. He is also sending Eli to an opthamologist so they can keep a close eye on if surgery will be needed in the future, although it looks like glasses have been working well. They are getting glasses ordered so he will get his new ones in a few weeks!

Eli also had his in home speech today and did pretty well. She has brought him a signing DVD and wants to see how he responds to it. We've also started the process to get an IPAD. We like his new in home speech therapist and are excited to see how Eli progresses with her.

Tomorrow begins VBS for the older 4 and they are excited to go and meet new people!

Family Updates

2011-06-19T09:34:00.000-05:00

I haven't done a family update in a while so it seemed like a good time!

Emily ~ Alot has been going on with her. She has joined the girl scouts first of all. She had her first get together last week. They made shirts and SWAPS for camp. She will be going to camp with them the last week of June. They will spend 4 days and 1 night at camp. She's excited and a bit nervous. She has always struggled a bit with making friends so this will be a great experience for her! She will be going to VBS this next week and is also looking forward to that. She has met a few friends in the area and has spent some time playing outside with them as well.

Jace ~ He's doing sooo much better. He had an appt last week with his pediatrician who got him in immediately for an appt with a doctor who specializes with special kiddos and medications for them. She agreed that Jace's meds weren't right AND that his diagnosis wasn't quite right. She agreed with the ADHD but also says he has an impulsivity / reactivity disorder. She has added a new med and given some creative ideas to help deal with the anger and physical violence. She said that ultimately the med will probably need to be doubled and a 2nd med added but wants to take it slow. We have already seen a huge change in his behavior. It isn't perfect but sooo much better already. The ideas to deal with violence also seem to be helping some. He will meet with her frequently as things get adjusted. Jace will also be going to VBS next week. He has met multiple kids in the neighborhood as well. A little boy has spent alot of time over here and there is also a little girl Jace's age who says she loves Jace and has pinned him to the ground and kissed him! :)

Benjamin ~ Ben is doing ok. His meds aren't working well either. When we moved they were in the middle of getting him to the right dose. He will be meeting with the same doctor that Jace is meeting with. He has also met a few friends in the area and has played a bit outside with everyone. He is mostly enjoying having a large house with 4 floors to roam around. He enjoys having his own time and space to be in. He will also be going to VBS and is looking forward to it.

Ariana ~ She's the same little diva as always. She struggles to find her space in her siblings. She's younger and so girly. She doesn't share the same interests as everyone else. This can cause alot of issues sometimes. She will hopefully be going to PreK in the fall and we are really hoping that helps her settle in a bit more. She will be going to VBS as well and she's very excited to finally be old enough to attend!

Elias ~ He's doing ok this week. A bit of a struggle with the heat, so pushing more fluids. He's still losing weight and noboby is sure why. His feeds have been upped twice in the last 10 days but the weight is still coming off. He's been incredibly clingy and wants Bob or I to always be holding him. Makes for fun times trying to get things done!

Bob and I are ok. Just trying to get life resituated. He got all of his unemployment papers finished up online and now we wait. He is applying for all the jobs he can possibly find. We are hoping to find a job soon. Unemployment is lovely in theory, but a job is our preference.

Today we are going to spend the day out and about as a family. We are going to the Art Museum for the day and then home for one of our family favorite dinners and a dessert of Bob's picking! The kids are going to help me make the dessert for fathers day! They are super excited!

This afternoon or tomorrow I will get pictures up from our day out today!

Happy fathers day to my dad and my husband! You both are amazing and I can't imagine life without you !!!!

Answers

2011-06-15T15:57:00.000-05:00

I am sitting here with an envelope full of 69 pages of Eli's report. We got it this afternoon and have already read thru it like 5 times.
Stage 1 of our journey has finally ended.
We found out at 18 weeks of pregnancy on July 28th, 2009 that there was a problem with our little man. We found out today at 18 months of age what that problem is.

Eli has Mitochondrial Disease Complex I and Complex III and OXPHOS. He also has multiple deficiencies, immune system issues and multiple other things.

I am sooo relieved to finally have an answer, but heartbroken as well. Not having 100% definitive results gave that tiny glimmer that they could all be wrong, and that glimmer is completely gone now.

We are unsure what's next. Atlanta wants Bob and I to come for a long sit down talk with them. Eli's pediatrician here is getting rush appts with neurology and genetics.
I am slightly dazed as I can't believe we are here, that this is truely the road we are on.

Right now I don't have much to say as we as a family are still trying to absorb it all. I will update more in a few days. Right now I just need to snuggle my little man.

results are in KC

2011-06-13T09:43:00.000-05:00

We got the long awaited email this morning. Eli's results are in. They have been sent from Atlanta to his doc in KC. I have already talked to both offices this morning. We are waiting for the doc here to get them and read them over and then decide if he wants to meet in person or if he will give them over the phone. Once we have spoken with a doc about them we can have our own copy. I'm so anxious and nervous and scared and fidgety. For those new to Eli's journey these are his Mitochondrial results. They ran 27 tests in April and the answers are finally here. i will update today as we know more.

Back for real this time!!!!

2011-06-12T20:36:00.000-05:00

I apologize for not actually getting back on and updating. We got internet and our computer promptly crashed. It was unfixable.
It's been a LOOOOOONG week. Alot has happened and I'm not even sure where to start.
We have been interviewing, orientating and training nurses. This has been such a long exhausting process for all of us. We MAY be at the point where we have a good staff but unfortunately it's to early to tell. When finding nursing there are so many things to consider. Are they in general a good nurse? Are they a good pediatric nurse? Are they a good personality fit for Eli, us, and the kids? How are they with schedules and flexibility? It's just way harder than good nurse, done.
Eli has also been getting situated with therapy. He is going to physical, occupational and speech therapy at a center based therapy group. We are really impressed with them so far and are happy with their current goals for him. He will also have inhome based therapy, mostly focusing on communication. The goal is to get Eli an ipad going and to begin working on a PECS based communication as well as still presenting him with alot of signing opportunities. Eli tried a pair of shoes this last week and his feet immediately buckled them out. His PT wants him in a good high top shoe and his doctor has send the orders over for AFO's. His OT is working on alot of sensory things and trying to introduce him to a wide range of different textures.
He also had an appt with his pediatrician/medical complex doctor. We love him and think he's a great doc for Eli. We all met for almost 3 hours. We went thru each and every system and doc and discussed where we were with each and what we needed next from each. He is getting Eli into an eye doctor up here to get his glasses fixed and get his eyes re-checked to make sure his script is still good. He is also getting him into the car seat clinic to get him fitted into a special car seat to put him in a better position for breathing while in the car. He's getting in touch with the pulmonologist to get O2 added to his CPAP so we won't have to keep switching back and forth between the 2 at night when he drops. He also agrees with the neurologist about Eli being functionally deaf. He agrees with getting him into the Kansas school for the deaf and pushing forward with the ipad and signing. We went thru all of Eli's equipment and all of the supplies that we have and all that we still need. We also discussed Eli's struggle with the heat and hydration and with his decrease in urine. He has a call into the urologist and is considering getting Eli into an endocrinologist.
Eli also met with his cardiologist this week. His cardio is going to go back and look at each ECHO and try to determine if the leason is in fact growing or if it's simply measured from a different angle or what not. We will wait to hear back from him soon.
Eli started running a fever yesterday morning which of course brought on the high heart rate. He still has both today but no other symptoms. We are hoping that it doesn't turn into anything more. He got to go out for a bit yesterday afternoon and today. He enjoyed being outside and getting to watch people around him.

My parents and Eli's nurse from Wichita, Paula, came up to visit for the weekend. We celebrated Ben's 6th birthday. Saturday we went to Legends and had lunch at T-Rex. This is one of the coolest restaurants and the kids absolutely love it there! We had a great time visiting with everyone and Eli was beyond happy to have his paula paula for a few days!

The last piece of news is in regards to Bob's job. His employer had been working to team up with another company and launch their partnership. So bob and many other people were hired for this launch. It didn't happen, and didn't happen. So many delays. On Friday a group of them got laid off...bob's group was one that did in fact get laid off. We are confident that he will find another job quickly but it doesn't change the fact that we definitely weren't expecting this massive bump in the road.
Like always we will simply stick together as a family and get thru all of it.
Alot of things going on right now, some that I can't mention right yet, but we definitely could use any and all prayers and positive thoughts possible!

Here are a few pictures from this past weekend.

The 7th of us

The 7 of us plus my parents and paula!

Paula and Eli

My dad and the oldest 2 boys

My beautiful Emily

My incredibly goofy Jace

My ridiculously adorable birthday boy Ben

my tom boy princess ari

My super big smiling tiny man

Super quick check in

2011-06-06T20:40:00.000-05:00

I'm exhausted and Ben is screaming so this will be beyond short. I just wanted to pop on now that we finally have internet. We are all still here, just finishing up unpacking and getting the house organized. Just got internet hooked up and situated this evening.
I promise to get on tomorrow for a full update!

Tiny's got wheels!

2011-05-24T17:51:00.000-05:00

Today was Eli's wheelchair appt. We were there for 3 1/2 hrs. They got it all fitted and adjusted and got his tray made. He absolutely loves it and was happy as could be going down the hallway and reading a book on his tray. He's wiped out now and has been asleep for quite some time and probably will be for the rest of the night.
The pharmacy has had an issue getting one of his meds sent out and therefore he is completely out. It's of course his GERD medication and he's choking and gagging even worse than normal. Hopefully they get it compounded and out here quickly tomorrow. His upper lungs still sound a bit yucky, mostly on the right. We are keeping a close eye on it all.

tiny man checking out his new wheelchair

Exhausted after his appt

Sound asleep after his appt

Here is the picture of Bob and I meeting Josh Groban!

We are still here!!

2011-05-23T05:26:00.001-05:00

I apologize for being MIA these last 10 days.
We have been moving. I spent a week packing and started getting a bit sick. I thought it was allergies, finaly went to the doc as it was getting worse and worse. Come to find out I had pneumonia. I got so weak I could hardly move, and breathing was a struggle. Lots of meds and rest later I'm somewhat better. Bob spent a few days in town helping finish up all the packing. The movers came this last Thursday and all of our things are now in KC.

During the move Emily got sick as well. Her asthma went out of control. The school called saying her O2 was 86. Rushed to the doc for tons of breathing treatments and steroids. It wouldn't come above 95. The next day the same thing happened. They doubled the breathing treatments and steroids. Still stayed around 95 to 96 for days. She is finally able to breath without having to work so hard and her O2 is hanging out around 98. Much better. She's hoping to try school today as she was out at the end of last week.

My little man. He's "stable" at the moment. It's been a rough time for him. His O2 has been suprisingly pretty good, but the last 2 days his upper lobes have sounded junky. We are hoping with agressive suctioning that will keep it from settling farther down in his lungs. He's running a bit of a low grade, but not enough for major concern. His heartrate has been strangely low for him, which we will be calling the cardiologist on Monday. With his heart issues and this dang calcified leason in his heart, we report any changes and let his cardio team decide what next! He has struggled the last few days with dehydration. He can't handle any heat at all. The air conditioner has had to be turned down low to allow his body to be able to keep itself cool enough. We are pushing as much free fluids as possible and as of last night he was finally peeing again. His new meds to help lower his secretions don't seem to be working effectively enough. A call is in to his pulmonologist to find out if we can slightly up the dose. This Tuesday Eli's wheelchair comes in. He will spend a few hours at seating clinic getting all the final adjustments done! We are excited to give him this opportunity to be up and moving around even when he's to tired to crawl on his own. Eli has also been trialing a new car seat this week. He struggles to breath well in the car and will routinely drop his O2 levels to 71. His pulmonologist suggested he meet with a therapist trained in carseat positioning. She has loaned us a seat to see if we can get some better positioning in the car. He has done a bit better with the new seat but is still dropping into the low 80's. Luckily with the move coming up soon he won't be in the vehicle as much, but will still obviously be in there. We are hoping soon to find something that will make it easiest for him to breath!

This past Saturday was an AMAZING night. My mom got Bob and I tickets to see Josh Groban in concert. It was my birthday present from my parents! Wednesday evening the phone rang and it was one of the hosts from our local radio station. They have followed Eli's journey (they are the ones who arrainged for Santa to come to Eli, since Eli couldn't go to Santa) and wanted to do something for Bob and I. They had arrainged back stage passes for us to meet josh groban and get pictures and autographs! I was beyond excited! Saturday evening we did get to meet him, and even got to chat with him about Eli and give him one of Eli's cards. He was an amazing guy and it was such a great experience. We were enjoying the concert and listening to all of the amazing songs. Next thing we know Josh is talking before going into his next song. He was talking about meeting us backstage and our little mans journey and fight to stay strong. He dedicate a song in front of 6000+ people to our little man! I curled up into my husband and cried as I listened to him sing for my tiny man.
No recording was allowed during the concert, but I wanted to share a video of the song. Here is the link to view the video and hear the song.
http://youtu.be/XL71Za0Kpqg

I promise to keep up with blogging. The kids and I are at my parents for this week and we officially move to KC friday. Our life is changing, we have all vowed to go into this and to stay strong for our little man. Sometimes I can't believe this is all happening. We are leaving our home, our family, our friends, all to be close to the childrens hospital. Who moves for a children's hospital?! Parents of medically complex kiddos do. I know it's not going to be easy, but I know we are doing what is best for Eli. I wish I could be as strong as my little man. Watching him get the last bit of energy to push himself into someone's lap. Everything he goes thru, smiling as he goes. He wakes up hooked up to tubes and machines and looks up at me and smiles. He's amazing and is constantly teaching everyone he meets the true meaning of life. We are constantly told how much Eli and our family have touched people, and we feel we have been blessed to have met so many other amazing people thru Eli's journey!

Pictures soon! I have to find my camera cable!

Eli's struggle

2011-05-13T18:14:00.000-05:00

It's been a rough week. Eli started struggling alot more....AGAIN.

We finally got the call from the Hematologist. His hemoc doc, cardiologist, and ECHO radiologist all met. The spot in his heart is no longer a blood clot, it is a calcified leason in the left atrium. This is why it is growing. He is off of the blood thinners as they will no longer help. Hemoc is going to do a full work up in 32 days to find out if he has a long term blood clotting disorder or something underlying that has caused the blood clots or if it's simply from the port. Now cardio is in charge as this is specificially a heart related issue at this point. Nobody is sure quite yet what is going to happen. He will have another ECHO in 4 weeks. If it continues to grow that is going to be a HUGE issue as it will block the flow of blood in his heart....

The new allergy meds don't seem to be working. Last night was one of his worst nights in I don't know how long. He started dropping his sats at 1am. I would rub his back vigorously and he would stay at 77 for his O2. I ended up having to put him on O2. He still consistently dropped his sats into the low 80's for over 4 hrs. He was so mucusy he was having trouble breathing. He was coughing and choking. No position seemed to help him at all. His heartrate was running between 175 and 194. By this morning he was miserable. He has slept a huge chunk of the day, and his O2 has still run low for his normal. His HR has still been running a bit high. I kept suctioning but it's to far down to get it. I put a call into the doctor 1st thing this morning. Lots of discussions with docs and nurses. His main doctor says that his secretions are just out of control and he's not handling them well, especially at night. The more secretions he has the more it wears him out trying to keep them under control, which means he handles them even worse. If that makes sense. It's an endless cycle. His main doctor has ordered catheters to suction him and wants us to do that as often as needed. If we can't clear it with the cath suctioning at home we need to take him to the ER. His doc is already discussing the need to order overnight nursing to help take care of Eli at night since that is when he struggles the most. I'm hoping it doesn't come to that.

I'm really struggling to watch my little man struggle with things he once had no trouble doing. I remember a day where he could curl up and nurse. It seems like such a distant memory. It's gone and so many other things that once were, are gone as well. In the beginning they would tell us that each change was just temporary, that he would get past it and gain that skill back. It's not so. They know it and I know it. They have stopped promising those kind of things.

I'm scared. I'm so very scared. I don't know what to do.

Ridiculously fast update

2011-05-09T20:32:00.000-05:00

We are home. We got in early this morning. All the kids went to school and I got everything unpacked and some laundry done.
This afternoon Eli fought his nap so hard. Tonight he went to bed and promptly began screaming. His HR is of course thru the roof. He has been quiet for an all of 5 minutes and he's already dropped his O2 below alarm setting twice. It's going to be a loooong night.
I will get pictures put up tomorrow!

Quick Sunday morning update

2011-05-08T08:37:00.000-05:00

Eli's hemoc pa called. His echo showed that his blood clot went from 3 x 3 to 3.5 to 5.5 which is definitely not what it is supposed to do. This means that the lovenox isn't working any more. She will meet with eli's hemoc doc Monday and they will make a plan and call us back. I wish my little man could just catch a break.

Ben is doing a bit better again this morning. He has eaten more and more and is consistently drinking. He's still got a lot of sensory overload issues but getting better.

Happy mothers day to all!!!

In KC

2011-05-07T08:08:00.000-05:00

We are in KC. We got here yesterday. As of yesterday morning Ben was still struggling alot. He wasn't able to stand up still and was hardly drinking. We all loaded up and headed to KC. He drank some sips of pedialyte on the road. When we got here he actually had a bit of energy and ate some applesauce and drank some more pedialyte. He was moving slower, but atleast moving on his own. His sensory issues are in major overload. By last night he all of a sudden had a meltdown and freaked out for food and drink. He ate 2 mini cheeseburgers, drank an apple juice and a whole cup of water. Last night he didn't have any nightmares. This morning he's having trouble staying warm and ate a few bites for breakfast, but he did drink his juice. Hoping to see more improvement as the day goes on.

Eli had his pediatrician check up yesterday as well. His ears aren't still infected. His allergies are so out of control that there is a ton of pressure behind his ear drums which is causing them to hurt, as well as causing them to be flat on the tympanogram. The doc put him on allergy meds and is hoping that makes a difference. So that is 2 new meds in 2 days. Lovely.

We will spend the rest of the weekend in KC. I feel better being closer to the childrens hospital right now as we make sure Ben is solidly on the road to recover. The kids and I will probably head back to Wichita Monday morning assuming all is well.

More updates soon.

Very sick Benjamin

2011-05-05T21:25:00.000-05:00

What a day. Benjamin started getting sick yesterday. He ran 102 fever and wouldn't move and slept. He barely drank. Lots of pushing to get him to drink, same again today. He was so weak he hadn't moved from the couch at all. He was sleeping again and next thing you know he's running screaming and grabbing his stomach yelling about flashlights and monsters and pain and needing meds. His heartrate was high. He was shaking so bad that he couldn't hold a cup. Immediately called the doc. They were concerned about appendix so had me rush him in. He couldn't walk so I had to carry him. He couldn't even stand on the scale so he had to be laid out across the baby scale to get a weight. Within minutes they had an IV going. Doc was checking him over and looked at his throat. When she swabbed it, it was literally bleeding. Ben has strep. Apparently in young children it presents with high fever, severe stomach pain, shakes, sometimes delusions, and tons of other weird things. Ben got 1000 mls of IV fluids, plus a massive shot of penicillin. He was so cold and shivering we had to keep covering him in blankets. We were there for hours. His fever crept back as we were leaving the doc. His pediatrician literally carried him out to the car. We are home and he was carried back to his place on the couch. He is hardly drinking again and sweating alot with the fever. We haven't seen a drop of pee in over 30 hrs. It's been decided that if he isn't better by morning he will be taken to the childrens hospital in KC and admitted. He's super sick and it's really scary.
As they were getting his IV going he was screaming at them "pretty pretty pretty please don't poke me" " pretty please stop" "I wish this was a dream" "I wish I didn't have to have 2 pokes" "I wish there was no such thing as sick"
The one that broke my heart the most was after they got him hooked up to the IV and he looked at it and said "I don't want to be like Eli". He's scared that because he has to have a tube that he will be super sick like Eli. Benjamin understand how much Eli suffers and that the quality of his life is hard. His pediatrician, nurse and I spent constant time at his side to help him not be scared.
He still doesn't have enough energy to sit up yet, so it took 2 people to get his shirt off...1 to sit him and hold him up and 1 to get it off. I will update more as I can. It's going to be a long night pushing fluids and keeping track of all of his vitals.

Home from KC

2011-05-04T21:02:00.000-05:00

We are back in Wichita.
Tuesday:
Eli had feeding therapy. He was a mess. I discussed with her his gagging issues and she wasn't quite understanding. Next thing I knew Eli had decided to demonstrate exactly what he does. She was like holy cow, and it made her nervous as she was grabbing him out of the high chair. She said that it is definitely very concerning.
Then we moved onto seating clinic. One of the vendors considered changing Eli's wheelchair that has been ordered but I wasn't happy with the changes he wanted to make. So the chair stays the same! It has been ordered and will be here in about 2 weeks! We also met with a doctor in charge of the rehab dept. He was very informative and it gives us a good idea of what next in that dept.
Then we headed to dermatology. She thinks she knows what the issue with the weird rash is and wants us to come in as soon as it comes back. It only shows up when he's sick so luckily it isn't there now! :)
He then headed to the audiologist. Hearing aids are all working well. They did a tympanogram and both eardrums were completely flat. Most likely because of fluid. He will be checked by his pediatrician on Friday.
Tuesday evening was Eli spending more time choking and gagging and struggling to keep his sats up.

Wednesday:
Eli had his ECHO 1st thing this morning. He was choking and gagging so much in the waiting room that people were getting concerned.
Then we moved on to Neurology. This was our 1st neuro appt at CMH. We were very impressed. He met with me for about 1 1/2 hrs. He agrees with the Mito. He says that he believes that Eli is functionally deaf. He believes that the mitochondria have caused road blocks in the neural pathways so they aren't allowing sound to go thru and be properly processed in the brain. He also agrees with the hypotonia. At this point he says it's hard to tell exactly what Eli is capable off. He wants us to continue all therapies and see what Eli can gain. He did say the longer it takes to gain it the less likely he will ever be to gain it. So really it's a waiting game. Which no parent wants to do. He is anxiously awaiting all of the paperwork from Atlanta. He also had his social worker get the referral process started for Eli to get major therapy, and also to get the family going with the Deaf school in KC.
Then we moved onto Pulmonology. We discusssed the choking alot. She believes that they hypotonia, weak swallow, oral aversion, and tracheal laryengal malaysia are all working against Eli to make it so hard for him to swallow. Everyone agrees that the problem is with his own secretions. First she is trying him on some meds to help lower the amount of secretions. We also discussed his sats still dropping with CPAP, so we will most likely be adding O2. We also discussed his issues with being in his car seat. Then in true Eli form on the drive him he dropped his sats to 71!!!
So none the less we are home now. The older 4 are super excited to see us.
Emily has finished all of her state assessments and did great on them! She also scored 105% on a spelling test!
Jace also got 105% on a spelling test, the 1st test where he has gotten everything including the challenge words!
Benjamin had his school program today and then came home from school and promptly fell asleep. My dad checked his temp and it was 102. He is super tired this evening with a bit of a temp and super sleepy. He says his legs hurt. Hoping he feels better in the morning.
Ariana is the princess as always. She has been busy hanging out at home and playing. Talking up a storm!
I am ok. Exhausted. I seem to have some major allergy issues going on which has caused a cough. No fun. Hopefully my allergy meds will kick in fast!

More updates soon!

Monday in overland park

2011-05-02T20:24:00.000-05:00

It's been a long few days. Eli is struggling so much with his secretions. We are running out of ideas on what to do to help him. I'm really looking forward to these up coming appointments and hoping to get some answers. Last night he was trying to crawl up a stair and fell and his glasses broke and cut the side of his face next to his eye. He wasn't thrilled.
We started the morning with a doc appointment. Not much new info there. Then I went to the nursing agency and met with the team up here in overland park. Eli and I also got the dme transferred. Then we met bob for lunch at his work. Afterwards I picked up all the papers to get the kids enrolled for school next year. I'm so relieved to have a house up here and to be getting everything situated.
More info soon.

Quick Updates

2011-04-30T20:54:00.000-05:00

Eli and I are in KC. We got in Friday evening. Went to look at a house that works perfectly for us! We got back and put Eli to bed. We hung out with our friends visiting and came to bed around 11pm. Eli had been struggling with some coughing fits and gagging, but this isn't anything new unfortunately. Bob woke with Eli at 1:30am as his sat monitor was beeping. He went to pick Eli up and felt wet everywhere. Eli's GJ had come completely out. So our friends had a foley cath and we got that in. Then our friend, who is an RN was able to finally able to get a G tube in. We called CMH and they said to go to the ER. We were there for hours and they finally decided to not drop an NJ or do an IV and to just have us come back at 8am. So we drove back and forth. So we slept like 2 hrs. He did finally get his GJ replaced. Now this evening he's super fussy. His belly seems to be bothering him from all of the irritation and the barium.
Tonight and tomorrow we are hoping to rest. More updates hopefully tomorrow.
lots of doc appts this upcoming week at CMH. Hoping for some more answers.

Easter Weekend

2011-04-25T20:33:00.000-05:00

I'm exhausted so a full blog update isn't happening tonight BUT here are a few pictures from Easter. I will get a full blog post up tomorrow!
I'm still trying to learn my new camera so red eye is horrible. I will have to figure that out!

Behind on Updates

2011-04-21T21:27:00.000-05:00

Again I'm a bit behind. Eli was in KC earlier in the week. He had his PH probe, as well as his kidney ultrasounds and urology appt. We are still waiting on the results of the PH probe. His kidney was still very large BUT looked slightly better than his last scan. His doc wants to redo in a few months. We will begin discussing his next testicular surgery.

Since coming home he's made a huge advancement. He has learned how to hold onto something and go to standing. He's such a big boy! Once he even figured out how to get back down to his bottom once. It makes me nervous as crap. Everytime I just keep thinking about his blood thinners. I sooo can't wait until he's off of them. He's still as exhausted as ever though. He is napping every few minutes, and every few crawls. I feel so bad for him.
He also had his next speech appointment. Finally we are all on the same page. The hearing aids don't appear to be making a difference. He isn't responding to more sounds, he isn't making any more sounds, and he still isn't responding to sign language at all. It has been decided to start Eli on a switch with pictures. We will be trying the basic "real life" pictures instead of stick figure drawings. If that doesn't work well for him we will switch over to actually pictures from OUR home. Right now his switch will be super big with a very large picture, since his eyes are so horrible. She will start him with 1, and then advance up to 2 or 3. I'm excited to try but also so nervous. What if it doesn't work? He is almost 17 months and has ZERO communication.

Today was/is my birthday. It has probably been the worst birthday of my life. I'm so glad that it's the end of the day and can't wait for it to be over. I will say that some special people in my life rallied together to help save the end of the day. Emily baked and decorated a cake; Paula, Eli's nurse, brought flowers, card, and a cake; and Sasha, the kids babysitter, brought a pamper mommy gift basket and a card! Thank you guys for helping end the day on a better note!

Quick Evening Update

2011-04-13T20:43:00.000-05:00

We tried doing his meds over 31 minutes, which equals 1ml/each minute. He still choked and gagged. looks like tomorrow we will try over 45 minutes and see if that helps any. He's miserable and oh so tired.
Here he is getting his meds (syringe push) and his breathing treatment.

GI issues

2011-04-13T15:51:00.000-05:00

Eli's GI system has never been cooperative. But seriously this is just getting ridiculous. When Eli was 1st born we were able to give his meds/feeds by mouth. Then he stopped tolerating so we switched to stomach. Then he again stopped tolerating so we switched to intestines. He has now been struggling with his J meds. We had tried to up his feeds and his body wouldn't handle it. He can handle 44ml/he but not 45ml/hr. He was handling his meds and flushes. Then a few weeks ago he started choking and gagging and not tolerating them. So the doctors had us change things around and experiment with different ways. His choking just keeps getting worse. The GI today has decided that Eli can't handle ANY bolus, not even 3ml's at a time. So now we are supposed to push all meds slowly over 1 hr. So basically load them all up into a 60ml syringe and slowly hand push over 1 hr.
We are running out of options to get medications into his body. It's so frustrating. I hate watching him get worse. It's devestating.
He is also struggling to bounce back from his Atlanta trip. He is constantly tired. He is sleeping 75% of the day if left to his own ways. During the times he is awake and playing he's back to taking frequent breaks and not having as good of control over his body and movements. I keep hoping that he just needs 1 more good night of sleep between him and the Atlanta trip and that he will wake up nice and refreshed in the morning. But so far that's not happening.
He had PT this morning and we were chatting about his development and she is getting concerned. She isn't the concerned type. She has always been laid back to the point sometimes of driving me insane. So that just concerns me even more. So much more information that we need. So many unanswered questions.
I wish life was cut and dry. I wish things were easy.