Wednesday, June 12, 2013

We Have An Answer....

And it's quite good!!

First, if you didn't see the comment in the previous post, please go and read it and continually weep like I have over 65 Roses.  And then we'll share that our cystic fibrosis test came back negative!!  We never thought we'd have traditional CF, because it's not presenting this way.  But there is such a thing as atypical CF, which is much milder and often less life threatening but still "CF."  So we were happy with our negative skin test for (mostly) ruling that out, too.

So what is it?  A form of ASTHMA that presents with unusual symptoms.  Both our new pediatric pulmnologist and the hospital pulmonologist came to this conclusion separately and both follow "several" cases of this in their offices.  It's rare enough that any one pediatrician's office is unlikely to see it, but common enough that they have a couple of patients with it.

As an aside: atypical CF is so common that every pediatrician likely has a patient with it whether they know it or not.  So Bridget's form of asthma really is not very common.  ((An aside: however, it's not as rare as plastic bronchitis, which scared the bejeesus out of me when the doctor described it.  I can't find a simple link to it, but basically the lung makes secretions so thick, it looks like the patient inhaled gum.))

So weird asthma!  WOOHOO!!  Objectively, I know that's not a great diagnosis but subjectively we think it's good enough that Dave and I did a happy jig.  (Well, I did. Dave is an effusive midwesterner. I think he blinked twice in happiness.  So we both did a happy jig in our own way) What is different about this:  Bridget doesn't wheeze like a typical asthmatic.  Her lungs always sound clear at the docs, even in the hospital.  When she has an asthmatic reaction, mucus plugs her  tiniest lung parts so her lungs cannot exchange oxygen with her blood.  Indeed, the first time they have an incident, it is often diagnosed as pneumonia. Dave and I both sighed with relief at that news:  this asthma may be weird, but it's absolutely the textbook case.  The doctor also said an underlying bacterial infection or autoimmune issue is unlikely because generally Bridget is a very strong, healthy little girl who all of a sudden gets very, very sick.

Also, I felt like a proud Mama when one of the first things the pulmonologist said to me was how glad he was that I had gotten that pulse ox. I am lame enough to like it when an authority figure compliments me.

So the treatment protocol is going to be different than for Christopher.  Another characteristic of this is that Bridget's asthma does not respond to albuterol.  She'll be doing daily medicine in an inhaler for the duration and then we she gets a cough, we'll also treat her with a hypotonic saline solution--essentially a neti pot for her lungs.

IT'S ALL GOOD NEWS, PEOPLE!!!  We are thrilled.  We may be here a few more days as she gets this stuff out of her lungs and they heal.  But we've got a diagnosis objectively agreed upon by two high end pulmonologists and it's treatable.


1 comment:

Rebecca said...

So glad to hear you guys have answers. And, it sounds more hopeful than originally thought. I hope they come up with a treatment plan that keeps your little girl out of the hospital.