It was the Best of Times, It was the Worst of Times...

Where do I even begin for this month? I'll start with the best stuff first...

MASON IS ONE!!!!!!!!!!!! 

 Of course, he had a Mickey Mouse party (who else?!) ..

 It's a pity he didn't get any presents.

Or that nobody had any fun at his party.

 He wasn't too convinced about the whole cake idea.

He got a rocking horse for tummy time (and his own lazy boy) for his birthday. He LOVES the fuzzy buzzy whinnying horse, especially it's flappy tail!

Naturally, he had his first pony ride...

 

He's had improvements in exploring objects with his hands this month...dry macaroni, wet rags, toys - all things he's been unwilling to touch much in the past!

And he's grown very tall this month. He was 31 1/4 at his well-baby checkup! As you can see, the walker came and went in about a week because he just is way too tall. But you can tell by the expression on his face how much he enjoys having a little control :D We can't wait to get him into something more his size that he can get around in....

 He has had so much progress in so little time!

The hard part has been that this month has been one of the hardest yet on him physically.

So many things happened at the end of July/beginning of August that sent things downhill....

1. he had an extra Prevnar shot to boost his immune response to strep/pneumoccocal (his leg where he got has been red and bumpy like this since he got it, over a month ago...). He has not had his 12-mo shots until we get some feedback on what is/isn't safe for him. He also got put on an antibiotic, Cefdinir, to be on "forever." Or until his immune function improves.

.

2. He had an MRI and the intubation caused stridor. The rest of that week he seemed unwell. He had a possible UTI but the docs are pretty sure the sample was just contaminated, so we didn't treat it.

3. As he got over the stridor, both boys got sick with a bad cold. Maybe 5th Disease due to the rash shown here. But maybe not. The rash might be something like hives from an allergy (no welts though) or a flushing disorder of some sort (he gets it worst when he's active/upset). Regardless, Mason ended up in an ambulance with respiratory distress the likes of which I haven't seen in him ever. He had secretions coming out everywhere. And blood. Nuff said. Scared is not even the word for how I was feeling that morning :( He was in the hospital Aug 5-7 trying to dry him out and get his oxygen saturations where they needed to be.

notice the bright "slapped cheek" look that was suspicious for 5th disease, along with the flat rash on limbs...

4. During all this he started having episodes of "spacing out". We called them "staring episodes" or "the frownies," because he frowns like this when he does them and clenches his fists/feet...

This is what happens during an episode (just very repetitious mouth motions):

 We were nervous these "frownies" were seizure related, and/or that he had an undiagnosed infection. We told neurology that we weren't sure what he was doing, but he was doing it a LOT. They ordered an EEG. And bumped up his Keppra in case he needed an adjustment for weight (hadn't had one in over 6 months, and he's grown quite a bit).

4. The day after the Keppra bump, he had a new kind of seizure. He lost all muscle tone and turned gray for 10 seconds or so. Very scary. He also had 2-3 of his classic seizures over the next week or two - the ones he hadn't had since April. His rash continued. The worst was the screaming that started. And didn't stop, for long stretches on end. He had an episode of screaming during the EEG. And throughout the next week, to the point of severe exhaustion for all. He had "the frownies" in the EEg too. It was an ordeal, to put it mildly.
The picture says it all ... I think we all looked about this way :(

We ended up back in the ER. He was not himself and he was screaming WAY too much. And having seizures. This only added to his misery but the docs assured us all his labs were "great" (the ones they were able to get back that night, including xray, ultrasound, CBC and urinalysis).

Two days later the ER called to say he had a UTI and we needed to put him on 3 mLs of nitrosomethingerother antiobiotic every 6 hours for 10 days (that's 12 mLs a DAY). Then urology and pediatrics called to tell us NOT to give him meds, because it was again, a "contaminated" sample most likely and not to listen to ER doc. Sigh.  And drs wonder why we have a hard time trusting them sometimes?

The EEG showed he was having seizure activity. No surprise there, we had seen it. BUT the good news and the surprise, was that "the frownies" were NOT seizure related! Brian and I asked to go back to his original dose of Keppra because the fact was that he had not had any NEW seizures until we'd agreed to the Keppra increase for the frownies (which weren't seizures!). And all the screaming had started after we increased it as well, not before. Just google "Kepprage." Both his pediatrician and ER docs agreed this medication is the #1 best guess as to his trouble :( They want us to "get all the junk out of his system and let's see what he can do!"

All this to say, as soon as his surgery is over in October, we want to go WAY DOWN on Keppra (and maybe off it altogether!!) He is on a very high dose (has been since he came home from NICU) and we cannot help but wonder how much of Mason's "syndrome" is actually being caused by this crazy med :( On its list of side effects are WEAKNESS (he has "severe hypotonia"), INCOORDINATION (hmm, like with swallowing/eating?? he ate his entire bottle right before he went on Keppra, after they forgot phenobarbital), irritability, immune deficiency ....ARRRRRRRRRRRGGG!! Are we detecting a theme here?? Needless to say I am heartsick about what this med may have done to him all this time (the simple fact is we have only seen Mason without seizure meds in his system for like 2 DAYS of his life. There's no proof he would even have seizures except under extreme stress off the meds. (And as I have mentioned, I have had a seizure too under severe stress but don't let ANYONE put me on seizure meds ever please, now that I see what they are capable of!!) Please, please please pray for us in the "going off" process starting next month, as withdrawing itself can cause more seizures, and his neurology may be very reluctant to help us :( But we truly want to see if he improves off this (and all) seizure medication.

Just since we have gone back to the old dose, his seizures have gone away (rash is still there) and I found him the other day having SCOOTED on his tummy completely off his blanket!!

Thank You Lord for revealing to us such a potentially HUGE piece of info and help us to be able to get our little guy back to himself very soon!! He has come so far... WHAT A YEAR!! :D

"The living, the living—they praise you, as I am doing today; parents tell their children about your faithfulness." Isaiah 38:19

Wordless Wednesday

What a Week of Milestones :)

Monday was Micah's first day of preschool! He had a very good day!!

First Day Success Smiles :)

And Mason rolled over for the first time (back to tummy)!

Today Mason ATE this much food by mouth!! (well, this is what is left!)

He decided he doesn't like thin foods like stage 1 and 2 baby food. He skipped to chicken and stars (chunky stage 3) and didn't protest or retch AT ALL even with the spoon in his mouth (he usually won't tolerate ANYTHING in his mouth)!! :D What a BIG BOY!! :D I cannot even begin to explain how excited I am about this!!

Tomorrow we are meeting with OT to go back to once a week, and add speech therapy. His PT is going well too...look at how he can hold up his head now!

 And he is pushing up so much better during tummy time! Next stop, crawling :)


Friday is his FIRST BIRTHDAY!! Has it really been a year?? Oh my. Anyway, anyone who would like to stop by about 7 pm Friday night (19th), we will sing to him and see if he wants some cake :)

All we can say is GLORY TO GOD!! :D
______________________________________________________________________

HOSPITAL UPDATE: Mason still has his rash from Fifth Disease/parvob19 virus (he was in the hospital from the 5th to the 7th after a trip by ambulance due to respiratory distress from uncontrollable secretions). But his wheezing and congestion are immensely better!! He seems to be having some new types of seizure-like episodes though, that don't affect his oxygen saturations/heart rate ... the neuro has upped his seizure med to see if this improves the situation. Pray it works like a charm so he can continue his good progress (hard to eat if you're staring off into space unresponsive, in an absence seizure). Pray for Micah too; doc says he also has hypotonia to a lesser degree and could use PT/OT for some of his issues, especially low muscle tone/weakness around his joints/ankles/shoulders and for sensory issues/fine motor help (his hands get tired easily and affects the length of time he wants to do crafts/writing/coloring/drawing, etc). So we will be getting him evaluated for that in next couple weeks. They are both also getting bloodwork Thursday to check to see if their re-vaccination helped them build more antibodies to pneumococcal strains.

“I am the LORD, the God of all mankind. Is anything too hard for me? Jeremiah 32:27

This Is Where the Healing Begins...

That's the song by Tenth Ave North that was playing on the radio at 5:15 am as Mason and I headed to the hospital for his repeat MRI, (mainly to determine the status of his tethered spinal cord so the neurosurgeons could schedule his surgery sometime in August). It felt like God was whispering those words straight to my heart, and I prayed that it could be true for our guy ... I can just imagine the sparks flying as His grace comes crashing in :)

Mason was cheerful all the way to the OR by 7:30 am, giggling his cute little way down the hall with the nurses. The rest of the day was long and hard...the best part of it all is that they were able to sedate him with gas before they tried to get an IV going, and intubation. They put in a catheter too to do a test when he woke up to see how his nerve function/spinal cord was performing. When he came back (3 hours later) to recovery, at first glance he was doing well...

But then it became clear that he was getting another roaring case of stridor from the intubation, so he was wheezing and honking like an awful case of croup. He really started crying in distress after about 15 minutes in recovery. (We told them in advance - several days actually - that he was a tough intubation and they promised to use a special device to make it less traumatic but clearly it was still pretty rough for his lungs.) They tried 2 albuterol treatments with no help and he was so miserable with sore throat and lungs that he was mostly inconsolable the rest of the day. Finally a dose of Decadron (sp?) steroids and Tylenol brought him to a more comfortable level and he was able to get a little sleep before his test.

The IV on his arm was really bugging him too...the one that took no less than 15 (should I spell that..FIFTEEN holes all over his body to get in him???) His ankles and arms look like he got into hornets :( I had also asked the anesthesiologist to use the ultrasound guide to find a spot faster like last time...clearly he ignored that bit and chose instead to use Mason as a pincushion. Sigh. So note to self....(a note that has already been added to his Plan of Care at the hospital since it apparently carries more weight than mom's pleading) ...ALWAYS use ultrasound guidance for Mason getting IVs and labs...and ALWAYS use preventive Decadron/and or Tylenol when he's still in the OR to try to head off the stridor (takes a good five days to resolve once it hits). Anyway, the stridor so bad they wouldn't let us leave and kept having the doc come in to re-check him before he was allowed to be discharged for his test. That took another very distressing hour and we had to go back to the surgery area to be checked again. We were double rooming with a poor little guy getting his tonsils out and he was in bad shape too...couldn't stop throwing up :( Finally around 4 pm we got the all-clear and finally were able to get a proper meal in him around 5:30 that night.  The next day Mason started feeling even worse, but his stridor was a little improved...seemed like another UTI with soaked diapers/bed, not sleeping, fever, diarrhea, etc. The catheter probably helped it along :( The test at the ped's office came back positive but they weren't convinced it was "real" vs. contamination so we are waiting for the full culture results before having to add another antibiotic to the regime. That is a current prayer request...antibiotics just make the UTI trouble worse in the future, and his eating/tummy issues worse while he's on them. We're hoping he gets better without all that!

In the meantime we didn't have to wait long for the MRI results...which were a HUGE and WONDERFUL suprise!!

The next day we found out his tethered spinal cord is... NOT TETHERED ANYMORE!! Meaning, where the cord was originally adhered to his spinal column as a newborn and they were worried it was stretching/pulling as he grew and would need to be freed surgically to avoid nerve damage - it wasn't just not tethered, they said it now appears NORMAL!!! :D (previously it was also too long and had an abnormal fatty area at the bottom. SO NO SURGERY!! And no laying on his tummy 96 hrs sedated with a ventilator!!! No repeat cath test after the surgery because there will be NO surgery!! WHOOO HOOO!! Hmm... I wonder Who could've had a hand in all that? There's that music again... "This is where the healing starts..."  !!

It gets better :) As for his brain, he does NOT have a Chiari Malformation. :) So no decompression surgery either!! DOUBLE YAY! He does still have Dandy-Walker Variant. No action to take there. He does have new lateral ventricle swelling in his brain but the neuro assured us, it's NOT fluid :D So no shunt surgery needed!! TRIPLE YAY!!! And his brain growth is slowed which we kind of expected. And the missing/undeveloped area of his brain is the part controlling, in part, his trunk and that's the reason for his profound hypotonia especially upper body. She thinks with good PT he'll be able to overcome it, in his own time :D QUADRUPLE YAY!!

The only thing we're still unclear on is if he has "Molar Tooth Sign" (a symptom of one group of syndromes, COACH/Joubert-related syndromes) - we sent a question to the radiologist about it through the anesthesiologist since genetics had wanted us to ask them to rule that out, but I guess he didn't pass that on.


In other good news, Mason is getting glasses! He won't get a huge benefit from them visually but it should help some nearsightedness/astigmatism and anything is better than nothing when it comes to sight :) It should also help protect his good eye since it's the only one he has really any vision in. And he gets some transition lenses with them too since he is so photosensitive outside... I can hardly stand how cute he is in them!

It will take about a week for them to be ready to pick up...then there will be more pics of course :D Anyway all we can say is PRAISE GOD...HE IS GOOD, ALL THE TIME :D

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 results...at 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)