I call him lungs

I also call him Slappy because he gets slap happy. Sometimes, I call him bootchie. I have no idea where that one came from.

Well, it's been nearly 12 days since he had his lung biopsy. Alls I can say is at least it's done. I'm not sure how revealing it will in the end. Ah poor li'l isaac to have gone through it. It really was a traumatic ordeal. But the boy's resiliant and is quickly getting back to good health.

Dr. Nichols says he has inflammation, extra cells, in the interstitia, the part of the lung that isn't the alveoli. And these extra cells "sluff off" (or collect) into the alveoli causing the crackling sound. Why there is inflammation, I have a feeling no one will ever know. We are waiting to hear back from Dr Claire Langston, a pathologist at Baylor College of Medicine in Texas. This lab specializes in examining for rare pediatric pulmonary disorders. Hopefully, by the end of next week they'll tell us what they know. Only to tell us, they don't know either...

I find myself thinking about the past four or five months. It's just been crazy, i tell you. crazy. on so many levels. dr. ochs says when considering isaac, it's a team effort. all the doctors must collaborate to decide what's best for him. i mean i know where he's coming from. but, at the end of the day, it's not. it's up to me and robert to decide what's best for isaac. we're his parents. i'm not sure what my point is. i'm still trying to figure out the best articulation... it's just that the doctors really don't know that much more than you or i.

ok, the baby wakes, now. adieu for now.

Optimistic Antibodies

This time, when we sent blood up to Dr. Ochs' lab, they received it. Yes. Dr. Ochs left a message today saying the tests they ran looked completely normal for a baby his age. He is able to generate an appropriate antibody response to a foreign pathogen. Yay!

Since we didn't speak to him, we're a little unclear where this leaves us with continuing IVIG and prophylactic antibiotic treatment. I think he will still want us to get the booster -- where they re-introduce the virus into his bloodstream -- to see if his antibodies remember. I guess the idea is if they remember, they generate an attack to the foreign body much more quickly.

Well, so it's looking like it's just a lung thing. It's really good it's not both a lung and an immune system thing. Of course, the lung thing is no minor issue.

Isaac has his lung biopsy early monday morning. Fingers crossed, ladies and gentlemen! It will be nice to be past that point, let me tell you.

Meanwhile, Isaac's a scream. Literally. He has a bloodcurdling earshattering scream that leaves people a bit stunned. I _LOVE_ his vitality. :-) He's crawling all over the place. He loves bright colors. He loves his daddy. And his mama, too. Let's not forget Zoe the super dog.

Immunologically speaking

ok, some updates here. two weeks ago we all drove up to seattle very very early one morning (5 am!) and had some blood drawn from isaac and infected him with a virus that is harmless to humans. they performed two tests on the drawn blood. the first test was to count isaac's white blood cells. they counted the various types of t cells. they counted the various proteins found on his t cells, like cd3, cd4, cd8, etc... i have no idea what all they are for or what they do. i know hiv folks pay attention to their cd4 levels, but that's all i know. obviuosly all these proteins are quite essential. they counted his hematocrit level which was a little high. he was slightly neutropenic but not such that it seemed to raise a lot of flags. all his other counts were very normal.

the rest of the blood drawn was used for what they call a functional test. in a test tube or some such device they would introduce pathogens to the blood and see how the b and t cells would respond. and, i guess, it all was normal. so that's very encouraging. this seems to rule out SCIDs (Severe Combined Immunodeficiency Disorder), a very terrible immune disorder. it is what the original bubble boy had.

and as far as the infection of the virus goes, well, we've drawn blood last week and this week. we are going to be sending up the blood to dr ochs this week to see how isaac is responding. as a background -- we've infected him a bacteriophage, a virus that attacks a variant of E.Coli. it was administered into him by IV. there should be no e.coli in his blood anyway. so the virus will just float around and get absorbed into isaac's system at worst. at best, the antibodies in isaac will recognize the foreign body and attack it. the weekly blood draws will indicate an immune response, i.e. his body will generate the necessary antibodies to kill it. at least this is my highlevel understanding.

meanwhile, pulmonogocially, we are meeting with a surgeon tomorrow to find out what all's entailed with a lung biopsy.

i've got a link to a document revised last year about the various chronic interstitial lung diseases of infancy that you should check out written by a very prominant peds pulmonologist named Leland Fan.

Second Opinion

I wrote Edeana this week in an email (instead of rewriting this all over again):

we got an opinion from Dr Michael Wall of Doernbecher
children's hospital. he seems to think isaac's a "classic" (as
classic as you can get with an extremely rare condition) case of
chronic interstitial pneumonitis of infancy. he thinks isaac was born
with this, and that they'd completely misdiagnosed him at emanuel. he
suspects isaac's immune system is just fine. he says we need a lung
biopsy to be sure, however (about the pneumonitis, i mean). he says
there are only a handful of cases in oregon that appear a year (like 4 or 5). and
we need to treat him now, as opposed to later, to avoid lung scarring.
once the lung scars, there's nothing to be done. except perhaps a
lung transplant. so to fix him now, once the biopsy is complete and
labwork comes back confirming, we treat isaac with a huge dose of
steroids over several weeks, maybe months depending on how he
responds. yeah, most likely there'll be some side effects from the
steroids such as extra hair growth. perhaps some overall growth
slowing down -- but i think i'd prefer this to him having bad lungs
when he's older. i'd rather be short and able to hike a mountain than
tall and forced to sit all the time. i think the prognosis is good...
i'm a little unclear. i sure hope it is! and why does isaac have
this? well, that, i'm afraid, is a question we'll probably never know
the answer to.

this diagnosis feels a little more correct to me. mostly because
isaac's been breathing so fast his whole life. it says to me that
there was something wrong with this lungs from the start. also, isaac
really started to breathe nicely there towards the end of our first
stay in the hospital. more so than i have ever seen him. dr. wall
thought that instead of isaac responding to the antibiotic which was
given to him to treat the pcp, he was responding to the steroids (which had been given to him in conjunction with the antibiotic). and
i remember once he was off of them steroids, his breathing really took
a turn for the worse and he was still on the antibiotic...

it's interesting, isn't it? we have an appointment with a surgeon who
would be performing the biopsy next week. i think if this is really
what's wrong with isaac i'm very interested in treating him very very
soon.

in the meanwhile he's holding steady. and breathing as fast as can
be. he's crawling now. he's an energetic little feller... someone as
energetic as he needs those lungs!!!