Thursday, July 07, 2011

LOTS to Update

Whew...after a WONDERFUL vacation to Orlando for a week (just Biscuit and I - Daddy and Grandma stayed home with Bear) that included lots of family time and celebrating my Grandpa's 80th birthday, a trip to Clearwater Beach, Rock Springs, Downtown Disney, an uber-fun splash park, and lots of other adventures, we hit the ground running back at home with a string of drs and lots of news. It's shaping up to be one busy fall! Here's what we know right now:
The good news...

1. Mason had his first swallow study and passed! No consistencies of food (fluid or thickened fluid or purees) caused him to aspirate food into his lungs. This is AWESOME news - especially considering he was screaming his head off. However, he did reflux some of the milk into his nose and was diagnosed with "severe dysphagia (difficulty swallowing) secondary to severe oral aversions." We have the green light to let him try solids in small amounts, twice a day, at least from their department ... the OT said to avoid the fluids for now since he had them in his nose and that's no fun and will make him even more aversive to drinking until he can learn to not reflux...we're trying to make it as pleasant as possible so we can save the liquids for later.

2. Mason had allergy skin prick testing (this itself wasn't any fun for him - he again screamed the whole time because not only did he itch but he had to lay on his tummy for 15 solid minutes and was not a fan to say the least) ... but the good news is, he wasn't IgE allergic to ANY of the five biggest food allergens or dust or mold - not even peanuts, so this was wonderful considering Micah's history. The question mark is how eating by mouth will affect his GI tract (right now he doesn't tolerate volumes so he can handle tastes but once he starts getting regular amounts by gravity not pump it may have to be slow going on that.... they are optimistic that he will tolerate puree volume better than fluids.)

3. Mason had x rays taken of his adenoids which showed that they were not too big and didn't need to be removed - they weren't contributing to his constant night-time nasal congestion! :)

The not - so - good news...

1. Mason had to have immunology testing. We've had this coming a long time and it needed to be done, but I dreaded it and for good reason. The amount of blood needed for this workup was astounding. The limit for blood from a baby his size was 20 mL , the max in a month like 45 or 50. They needed 35 mLs. The sheet of lab stickers had to be lifted over my head and fell to the floor to view them all, it was like SIX FEET LONG, not kidding!! So they were planning to split it into two blood draws. This in itself is a disaster with Mason because every blood draw is such an ordeal - not only are his veins microscopic and wobbly, they are now buried under several layers of baby chub. So the tech basically took one look at him, remembered how hard of a stick he is, and called the transport nurses. They spent about thirty minutes with a tourniquet, systematically going around all his veins. Just the forceful rubbing and tightly-pinched band bruised him right up and got him majorly stressed out. Of course they couldn't find anything either - they made ONE attempt (a long, painful attempt that brought me to tears) in his foot using an IV catheter since they were afraid the vein wouldn't hold out for that much blood - and even with the catheter, it blew out before they had 2 mLs. At this point they said they would call the "resource team" to bring someone with an ultrasound machine to try to find it. I told them they could try once with the ultrasound and if they didn't get it that was it. The lady running the machine said "We're gonna get it, Lord willing!" The lady helping said, "We really should get a nun or a sister to pray for us." The first answered, "Oh no...we don't need anyone to do it for us. We've got it covered..." I was thankful for the direct route too and was praying right there with them, and He answered bigtime, because not only did they find a big juicy vein on the first try - they were able to get ALL 35 mLs and even enough for some thyroid labs for endocrinology that were also due. So no return trips for labs anytime soon, praise God!! Mason still had a lot of trauma from the ultrasound stick because they strapped him down and used an arm board to get him super still so he was very freaked out and majorly stressed out and sweating ... then we had to go out to the 95 degree oven of a car...I think he actually had a small seizure in the car from the stress plus the heat, but by the time I got pulled over with the oxygen he was back to himself. All in all it was a very bad memory that we hope to all put behind us but VERY glad it is over but especially that so many things about it worked out for good - especially being able to get all of it in one day (the doc had called to tell them that all 35 mLs would be safe at once...cutting it close, but safe.)

2. Mason has immunology least from some of his labs. And although we have to wait a few more weeks for more definite explanations, what they know is that Mason DOES have a serious immune deficiency :( In laymen's terms, no it isn't AIDS or Bubble Boy syndrome, but the same basic problem - his body will be subject to recurrent infections due to the inability to effectively fight off infections and we will have to be very careful what he is exposed to :( That is why he has already had so many infections, so many times - ear, eye, UTI, gtube, sinus/cold, etc. We don't know how severe his is yet, or if it's expected to get better before he's ten or be lifelong - only that he must have SOME immune protection since his levels are all low, not absent, which means he at least is making SOME antibodies (praise God!) This I would hope rules out the most severe primary immunodeficiencies but they are pretty concerned about his lack of protection right now and want him on antibiotics all the time as a first step.  They will give us further recommendations once we get all the labs in, as to what PIDD ("primary immune deficiency disorder") he has (over 120 different ones?), prognosis, treatment, etc. Right now we know he is low almost across the board of immune globulins - IgG, IgA, and IgM and he has been put on Cefdinir. This itself presents problem because when he is on antibiotics he tends to get real congested to the point of it sounding like he's drowning at night, and bad bad cramps - we never know if he's allergic to the drugs, or just snotty because of an infection (he runs a fever almost all the time). I checked into getting donor breast milk for maternal antibodies to give him some extra protection (since he didn't grow an ounce in three weeks on mine, even fortified with formula, presumably due to the presence of too much thyroid hormone) but the doctor said it wouldn't help nearly enough to get him into the range of antibody protection he needs. :( And the small adenoids, as it turns out, may not be so good as I thought when he was saying how they were "quite small" and not obstructing breathing - small adenoids are a sign of the more long term immune problems. So we ask for your prayers that it is temporary and gets better on its own. This condition is also able to affect his GI system / food tolerances as immune deficiencies often cause inflammatory bowel disease , celiac sprue and other autoimmune issues . Please be in prayer for my cousin Jake who was just diagnosed with Ulcerative Colitis and his immune system might be part of the problem too :( And for Micah, who is chronically sick with every virus under the sun and ear infections one after the other again now that his tubes are out (just went to the doc for him for sinus infection today, after being up almost all night long with the loudest screaming boy in the city, guaranteed. Sorry to the neighbors! - they won't allow new tubes again until he's had 5-6 new infections this year!?? he's already had ruptured ear drum, doesn't that count for something?) Since these immune problems are genetic and Micah's allergist has tried to do this same immune workup on him several times already, but we haven't been able to get all that blood, we are very concerned that whatever is happening with Mason's immune is affecting Micah's too in a similar, but lesser way - he has constant mouth ulcers and terrible GI pain and related problems himself too and no real answers there either.

Ever stronger boy, ready to keep fighting no matter what his immune system says ....
3. Mason had his Mylomengicele Clinic to see Neurosurgery and Orthopedic. For the neuro part, he is due to have surgery for his tethered cord within the month. In order to do that he has to have a repeat MRI of his brain/spine to be sure his cord is still tethered. He also has to have a bladder function study before and after to see how the lower spine is functioning now, which they will attempt to get while he is sedated for the MRI. The orthos are concerned about Mason's lack of muscle development in his upper body (he can't breathe if we put him upright as if to stand - he has so much weakness in his shoulders he just hunches forward - they said he actually has more than low tone there - he doesn't even have muscles developing there at all :( And his arms and hands are very weak so they are looking for a specific malformation, a Chiari malformation, on his MRI , to see if the weakness may be caused by spinal compression (this is apparently pretty common in those with hereditary connective tissue problems like joint hypermobility, which we have, and we were actually told Mason had the Chiari in the NICU until someone said it was wrong interpretation, instead it was Dandy Walker, etc - if he indeed has the Chiari, that may (depending on type) be surgically treatable and could give him back some of his lost function in these areas. We pray whatever they find, is treatable easily and really frees him up to do all the baby things he is so eager to do but just physically doesn't have the strength for. They also want to get him a stander when he has his cord released to help get him weight bearing since we can't do it holding him up due to the breathing issue/too much pressure on his chest. The tethered cord surgery is supposed to be fairly easy but requires an ICU recovery the first night, on his tummy (ugg, looong night) and 2-3 total stay. All his surgeries are more risky for infection too due to the immune issue, so that is a HUGE prayer request, as when he recovers from the cord surgery, presumably in August, he is due to have another surgery in September. Speaking of hypermobility, these are all issues with Micah too and we have to address it at his 4 year checkup as he is so weak and loose in the upper body that he can't hang by his arms, like for the monkey bars, without them easily coming out of joint, and his ankles are real floppy so he twists them a lot. etc...

4. The MRI/urodynamics study and 2 surgeries will all require IV sedation/anesthesia, so please pray we can have it handled very smoothly for him - that the teams will be willing to make special arrangements to minimize his stress, like Versed before the MRI stick, and possibly gas in the OR before the IV is placed, and the use of an ultrasound both times to minimize the sticks (they did the gas before the IV during ear tubes and he never felt a thing, it was wonderful!!)

5. Mason outgrew his Mickey button so he's getting a bigger one tomorrow, Lord willing, after a search around the region to find someone who had the right size.

So . A lot to swallow (literally and not so much) in the coming weeks; not a fun time for our little bear, or biscuit for that matter, or any of us. But we are praying and hoping it is all for good and SO worth it! :D
Praise be to the Lord, to God our Savior,
   who daily bears our burdens.
 Our God is a God who saves; 
Summon your power, God...
   show us your strength, our God, as you have done before. 
(Ps. 68:19, 20, 28)

Mason's Mix

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