More lessons in trust

It's been a crazy few weeks around here...kinda one thing then the next but I'm gonna post a "happy pic" or two of Mason enjoying a warm day by the pool!

The short story is first Mason got pneumonia, presumably from a virus since Micah also had croup. We managed to get through that by God's grace at home and even avoided steroids (which we try hard to do now that we know how those meds affect his glaucoma--not to mention wiping out the benefit of his IgG infusions). Instead we used NAC (N acetyl cysteine) starting 2 days after the diagnosis - it's an antioxidant that's supposed to help in some cases of viral pneumonia and wheezing. It worked well and he was doing MUCH better by Sunday.

Crisis averted but then Monday he started getting fussy again and by Tuesday he was a screaming disaster :( It didn't have to do with his breathing though - now it was related to feedings, at least at first; eventually it got to the point where he was inconsolable off and on all through the day with "stress rash" as bad as after a surgery. We took him to the dr and he wanted him admitted for some tests or at least a break from feeds for IV fluids, etc. He thought since Mason was drawing up his legs and very tender in abdominal area that he needed to rule out things like stones (gallbladder/kidney), pancreatitis, etc. He already had his appendix out so that we weren't worried about. But he'd had 3 seizures that week too, and stopped an antibiotic so there was the possibility of infection as well.  The docs were able to draw some labs but couldn't get an IV going even under ultrasound guidance so they tried slow drips for feeds (just as miserable) and took an xray, ultrasound and got neuro consult in case all the GI stuff was seizure related (that can happen).

Blood work they proclaimed "normal" (but in reality it showed elevated sed rate, a marker for inflammation--we hope this was just from the previous pneumonia, and elevated AST/SGOT liver enzymes (which can be elevated from gallbladder disease, liver disease, heart damage, other muscle damage, infectious abscesses, etc.-we hope this was just due to seizures earlier in the week which can affect his muscles). No one could deny he appeared to be in awful misery and pain but everyone was at a loss as to how to help. He had another seizure Thursday morning. The imaging was so obscured by gas/stool that they could not see the other organs well enough for ruling out the issues they were concerned about. Basically all they could tell us was to give him a laxative and increase gas meds and other "comfort measures" because the amount of gas he has is "impressive" and must be excruciatingly painful (he cannot burp due to his fundo, and fights us when we try to vent his tummy. If he is producing gas that's trapped in the intestines it doesn't really do much good to open his tube. We also were told to increase his Keppra for the seizures (which we are doing veerrry slow as opposed to the all in one day increase they had us do last time which resulted in new and worse seizures). If he still doesn't get better they offered a gallbladder med to keep bile out of his GI tract in case he has bile reflux. Once he gets his seizures under control they might want to do a scope test to rule out ulcers, etc. But if gas is the main problem we are not eager to pump him full of more gas to sedate him :(

We came home Friday night and worked our way up to full feeds again which is where we are now. He is tolerating them somewhat better but now is back to where we were before the pneumonia....green slime coming out of his eyes, a cough and more junky throat - we are guessing another sinus infection which seem to hit with the Keppra increases :( The immunologist is increasing dose of IgG but is baffled (as are we) how he keeps getting all these infections despite having a much higher level of IgG in his system (before infusions, he was 150 or less - now he is over 1000 and still sick one after another). Something may be wrong not just with the number of antibodies (which tag germs for destruction) but actually mobilizing his T cells to kill the germs.

So...we are worn out but trying to lean hard on Him to carry us through and give us wisdom/direction/guidance to keep our guy feeling good and making progress.

In other news, his genetics team sent  his medical file today to the NIH's "Undiagnosed Diseases Program" in Bethesda, MD so please pray that God's will would be done in whether or not he is selected to participate, whether they can find some answers on what is going on and that can be acted on for treatment, etc.

The title of this post was regarding a great challenge Sunday by our pastor in his sermon after a hard week ... he wanted to know how many of us who say we trust God can actually be trusted with His name - in other words if I claim to be His and that I'm trusting in Him, do I also live in a way that brings Him glory or am I an embarrassment to Him when I fail to really act on the trust I say I have in Him, or when I live in a way that dishonors Him (believe me this happens a lot when I let myself get too down or frustrated/exhausted). As Pastor Steve said, the problem with the church is not it's Head...so please pray for me in particular that I would by His strength be able to live out this verse:

"...that you may live a life worthy of the Lord and may please him in every way: bearing fruit in every good work, growing in the knowledge of God..." Col. 1:10

Sedated eye exam today

Mason is a happy boy to be home, even if it's infusion night!

Today we went to Children's Hospital (first time there for a sedation) to have his EUA (exam under anesthesia). He was also getting some labs drawn while he was out.

The reason for the exam was that at his regular eye checkup a couple weeks ago, his intra-ocular pressures were very high (30+) which is very rare for him, even with his history of glaucoma (usually they're in the high teens. low 20s). He wasn't upset at all for the non sedated test so this was a very surprising finding--usually he's screaming his head off and they're still not too high so that he was calm and still high that was odd. He doesn't use drops though so we had to rule out a pressure problem by doing it again sedated.

I was praying that the reason for the elevation was not a deterioration in the glaucoma situation per se, but rather a temporary effect of his ear drops (ciprodex, an antibiotic/steroid combo) that he has been off a few weeks now. (Last year when I was on a similar steroid ear drop I had an eye exam and MY pressures were high, which they've never been before! And steroids are known to be able to increase eye pressure-but docs don't usually remember to say anything about it.)

Anyway, overall today went very smoothly.

First answer to prayer - the pressures were great in both eyes!! Although we had to sign a waver saying he could get a laser treatment to relieve pressure if needed, he doesn't even need to go on drops! Yay! :D Maybe it really was just the steroid ear drops! Here are a few other praise reports...

- No need for intubation! (they just used gas)
- They found a vein for IV backup after he was sedated (took a few tries but thankful they got one)
- They were able to draw the labs (one was drawn incorrectly but at least they got the others and saved him some awake stress).
-Some unexpected good news: his lens is no longer adhered to his cornea as it was in the past in his left eye! Now it is just a cataract, which could be fixed with a lens implant, depending on how well his optic nerve in that eye can see even with a new lens (the visual evoked potential testing, like an EEG of his eyes, will be in mid April and will guide us further on the potential for repairing his cataract).
-He did okay in recovery (not happy but not big distress either - and he was 100% himself the minute he hit the stroller and knew he was headed home!)

So thanks for your prayers, we are happy it all went so well and maybe some potential for vision improvement to pray for direction on in coming weeks!

You can continue to pray for him as he has some unresolved GI distress issues for about a month now (an upper GI xrays series yesterday didn't give any clues). We are going to be asking about something called "bile reflux" because of how we have seen neon green on his feeding tube gauze in the past couple weeks and bile shouldn't be sitting in his stomach. If that's the issue maybe they can give him something to help move out the bile. Just not sure how they can "know" without another test sedated which we are not eager to do anytime soon.

Splish, Splash!

This week I found the coolest little gadget on Amazon - a neck float called "My Baby Can Swim!" It's great because systems like this designed for "special needs" are over $100 (and some are pretty clunky looking). But this one was $12.99 because it's for "any" baby! It's so perfect for Mason (I may even get one for Micah I love it so much--but apparently I got the last one, because now it says unavailable...sheesh, I hope we don't pop it anytime soon!)...anyway, he got to be submerged in his bath for the first time ever!! I have never seen the boy light up with so many smiles at once!! He was SO content and happy and best of all, relaxed!! His little fists and feet were open, not balled up like usual! He just loved it!! Next stop...the pool! :D Stay tuned...!

Do-it-yourself deep pressure vest

At physical therapy a while back, Mason tried out something called a Bear Hug Vest. It was great because he has low body awareness (he can't easily sense where the parts of his body are in space, or what muscles to activate for different movements) - so the compression vest gives him that input he needs for his upper trunk to make it easier for him to sit (which he fights aggressively and tries to squirm down in that position), bear weight on his legs (for brief periods against a chair or couch) and play upright.

The actual bear hug vests range from 75-250 online so I adapted an idea I found online to make him one myself. The idea suggested using a $5 neoprene waist slimmer belt from Walmart but when I got there to buy it I found something that is even better  for a small guy like Mason -
a two pack of thigh slimmers (also neoprene) by Gold's gym.

I used the first thigh slimmer (which fits up to 38") as the trunk wrap exactly as it came out of the box. I cut the other slimmer in half down the middle about halfway to make shoulder straps; I sewed a square of velcro to one end and reinforced the velcro square that came with it on the other end (since I had to cut it in half). Experts say to use nylon thread but mine worked out okay with all purpose ... I would recommend using thread in the color of the neoprene for a more finished look but i was just trying to make it functional.

It makes for a very custom fit and seems to work well! Just wanted to pass this idea along since it was so easy and inexpensive...much more accessible than the pro versions and does basically the same thing. Those of you who sew better (not me!!) could really make it cute too...

Look mom...no squirming....
but this sitting thing is gonna take
a lot of practice before i will agree to do it
 by myself...

News (Good or not? God knows.)

Mason had his CT scan yesterday morning to determine if he has craniosynostosis (early fusion of his sutures in his skull)...

GOOD NEWS...

The CT itself went great (Mason LOVED the laser light show during it!) and the results show that, to the neurosurgeon's surprise, he does NOT have craniosynostosis!! All of his sutures are still open :)

The "good" part of this news is that he is spared the brain surgery and does not have any evidence of elevated intracranial pressure, which we are VERY relieved to hear (he's had 8 seizures in the last 30 days or so, which is the first time in over 5 months he's gotten them so we were able to rule out high pressure being the problem - most likely it is a change in meds (going from generic Keppra to name brand and back since the end of Dec - in theory this shouldn't cause problems but in actuality and according to new studies just out last year, it does make a big difference - even generic to generic) so we are just going to stay the course and try not to change much on him in the future; it may also have a sinus infection component but we're treating that w/ Biaxin just in case); given his immune dysfunction and how easily he gets infections, the idea of cutting open his skull and putting his entire nervous system in jeopardy, not to mention subjecting him to more fentanyl/pain meds....made us wince to think about, so we are THRILLED he gets to avoid this surgery because of all the risk it carried.

NOT SO GOOD?
The other side of the coin in the situation is that, if he'd "only" had craniosynostosis, that was repairable and his head could continue to grow normally after the surgery to release the fused suture (his head circumference is so tiny as to not even be on the growth charts). Instead, he has a new diagnosis that is generally untreatable: microcephaly. We aren't sure if we'd rather have brain surgery or microcephaly based on what we have read. At Wikipedia the description says:

"Microcephaly is a neurodevelopmental disorder in which the circumference of the head is more than two standard deviations smaller than average for the person's age and sex. Microcephaly may be congenital or it may develop in the first few years of life. The disorder may stem from a wide variety of conditions that cause abnormal growth of the brain, or from syndromes associated with chromosomal abnormalities (Mason has a chromosomal deletion). Two copies of a loss-of-function mutation in one of the microcephalin genes causes primary microcephaly.


"In general, life expectancy for individuals with microcephaly is reduced and the prognosis for normal brain function is poor. The prognosis varies depending on the presence of associated abnormalities."


Mason has many "associated anomalies" but none of Mason's other "issues" appear to be life-threatening other than his low immune system which is being treated as aggressively as possible with antibody infusions weekly.  So ... we are going to hope and pray that he is one of the ones who is minimally affected ...

According to MedicineNet, "Depending on the severity of the accompanying syndrome, children with microcephaly may have:

• mental retardation,
• delayed motor functions and speech,
• facial distortions
• dwarfism or short stature,
• hyperactivity,
• seizures,
• difficulties with coordination and balance, and
• other brain or neurological abnormalities. 

BUT!!!...


Some children with microcephaly will have normal intelligence and a head that will grow bigger, but they will track below the normal growth curves for head circumference."

We'll be praying that even if he has a little head that he continues to grow and develop. No "diagnosis" can limit the power of God :D So far Mason has shown us that not only does he rarely ever conform to doctors' expectations, he is also a very happy, very smart little guy who knows how to problem solve very well and his congitive function continues to improve despite the small size of his head. We are going to see a craniofacial specialist next month to see if he may benefit still from a helmet for his positional plagiocephaly he got in the NICU from sleeping on his sides and back all the time.

"...the whole body, supported and held together by its ligaments and sinews, grows as God causes it to grow." Col. 2:19b