Sunday, August 28, 2011

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.

Thursday, August 25, 2011


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!

Tuesday, August 23, 2011

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.

Saturday, August 20, 2011

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.

Thursday, August 18, 2011

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.

Tuesday, August 16, 2011

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.

Monday, August 15, 2011

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!  :)

Thursday, August 11, 2011

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.

Tuesday, August 9, 2011

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.

Sunday, August 7, 2011

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!

Thursday, August 4, 2011

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!

Tuesday, August 2, 2011

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.

Monday, August 1, 2011

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!