CHOP: Day 2 (Grow, Baby, Grow!)

The mission for today was doing Will's pulmonary function test (PFT).  We were a bit nervous as to whether this would actually happen today because, as luck would have it, Will caught a cold.  Yep, that's right.  He caught a cold.  We have been running around here like crazy people trying to keep ourselves cleaned and sanitized, but it didn't work.  He isn't super sick, just a little nasal congestion, but it was enough to have us stressed.

We went in bright and early this morning to see Dr. Mayer, the Director of the Pulmonary Function Lab and attending pulmonologist.  After meeting with him for about 30 minutes and discussing a few questions and reviewing Will's history, he took a quick peek at him and said he thought he was healthy enough to proceed with the PFT.  Thankfully, we had a bit of a heads up from other families about what was going to happen during the PFT.

During the test, they put a mask over Will's nose and mouth.  They used this to monitor all of the air flow.  The funny part is that they use medical grade silly putty to seal around the mask.  That was the tiny bit of humor we could find in this procedure...  It was pretty tough seeing Will sedated though.  They let me administer the medicine to him and then I was able to hold him until he fell asleep and then I also placed him into the 'bed'.  It was nice to be able to be so involved.  It made it all a little less scary. There was also a vest around Will they used to press air back out of his lungs after they had been fully inflated.  They called this giving him a hug and it was one of the more nervous parts of the test for me because his body seemed to kind of jolt when they did it.

Will taking a good nap during the test thanks
to some chloral hydrate.

There's also a sliding box (technically called a body plethysmograph) they use to completely enclose the child during one portion of the evaluation.  

Will in the final stages of the evaluation.

As the test goes along, a computer charts everything and once it's all done Dr. Mayer just sat in the room and reviewed all the data while we woke Will up.  It was pretty amazing to see how real time everything was.     

Dr. Mayer reviewing the results

So, here are the very basic results.  Will technically has restrictive pulmonary disease.  This isn't really a surprise since he has Jeune Syndrome.  This basically means that his lung expansion is restricted because his chest wall is rigid.  Unlike a normal rib cage that expands adequately during respiration, Will's chest doesn't move very much.  This means, he can't take a super deep breath, thus resulting in the need for him to take more breaths.  

The good news right now is that Will's total lung capacity is at 77%.  This is on the low end of normal. If you think about it on a bell curve, 100% would be perfectly average.  Some people, like crazy mountain climbers or divers, may have lung capacity greater than 100%.  Other people, like smokers or people with other diseases, may have capacities lower than 100%.  Anyway, the short version of all these test results is that Dr. Mayer doesn't think Will needs the VEPTR surgery immediately.  Today's PFT has given us an outstanding baseline.  We now have the data we'll need to use in the future to determine whether Will's lung capacities are improving, decreasing or staying the same.  

In the case of people with Jeune Syndrome, it is unlikely that their lung capacities will improve without surgical intervention.  HOWEVER, Dr. Mayer made it a point to note that Will has a mosaic form of Jeune.  As far as any of us know, there has been no other documented case of a mosaic form of Jeune.  SOOO, Will is a bit of an unknown.  Our plan with Dr. Mayer is to come back within some agreed timeframe, probably within 6-9 months, to do the PFT again.  If those numbers have stayed the same, we will probably keep watching Will.  If the numbers have gone down, he will probably heavily recommend doing the VEPTR right away.  

Soooooo.... we are kind of waiting.  We still have the MRI and CT Scans to do tomorrow.  Those will give us even more information about Will's lungs.  Then, we'll meet with Dr. Campbell again Friday morning to discuss all of the tests this week and probably get an initial recommendation from him on what he thinks we should do.  We're pretty confident right now that we'll come home, give it a few more months, then reevaluate.

If someone made me guess what was going to happen, I would guess that we'll end up doing the VEPTR surgery sometime within the next twelve months (Hubby's guess is that we won't).  Here's the thing, the surgery still scares me.  I am going to be praying, praying, praying that The Great Physician touches Will and helps his chest grow enough to continue supporting the increasing needs of his growing body.  The hard part is that Will is just kind of uncharted territory.  Nothing with this kid is ever by the book..he's full of surprises!  I pray this continues and that his chest grows, grows, grows!  If there's one prayer we covet right now, it's that.  We are praying for his chest to grow enough to support his body.

For now, Will sleeps!  He was thoroughly unimpressed this morning and doesn't have a clue why we're being so paranoid.  

Will sleeping after his PFT.

And to end on a ridiculous note, here's what I did while waiting on the test results.  Can you tell that I get a little wacky when stressed?  Who knew about medical grade silly putty?!?  I may have even slipped it in my bag on the way out.  Emme will be so thrilled.  :)

Silly (putty) mommy!