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

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

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.

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

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

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

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

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

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)

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

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

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

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

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

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

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

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

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!!!

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

Updates W/ Pictures

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

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

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

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

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

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

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.