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.

Alot of Updates with Pictures!!

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

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

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

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

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

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

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

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

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

Our team tshirts all finished!

my little man during a bad day of temp regulation

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

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

my little man at the mito walk looking beyond adorable!

eli with his poster at the mito walk

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

Ari on her first day of preschool

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

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

Precautions and Discrimination

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

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

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

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

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

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

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

playing catch up

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

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

Quick Week to Date Overview

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

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

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

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

Here are a few pictures.

Eli's new soft side helmet

Trying out the Gait Trainer at PT

Ready to go to sleep for his sleep study

2 Years

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

LOOOONG Day

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

Quick Catch Up

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

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

Critical Neuro Appt Results

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

Renal Doc

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

Pulmonologist Yesterday

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

Eli right before officially starting his EEG

Eli checking out his new CPAP mask

Eli sound asleep after dinner

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

Emily before her hair cut

Emily after her hair cut

the back of her hair after

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

AFO's Redone

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

More Neurological Changes

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

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

More Bad News

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

Progression SUCKS

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

Jace's 8th Birthday Weekend

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

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

an accessory kit for his new ipod

Jace got the Ipod he has been wanting so badly

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

My super handsome always smiling husband

Jace playing angry birds on bob's cell phone

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

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

Eli working with glue during sensory time at OT

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

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

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

Emily playing the part of Malcolm

tiny enjoying watching Emily's play

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

My boys

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

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

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

More updates soon!

Quick MRI update

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

Damn Mito

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

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

Trying to Stay Afloat

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

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

Home

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

Quiet Weekend / Start of Week

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

Another Diagnosis Means Hospital Life Continues

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

Lots of Info

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

Rough Night

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

Admitted

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