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.

Feeding therapy

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

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

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

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

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

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

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

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!!!

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

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

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

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.