a different kind of perfect

Our CF Clinic Checkup (August 2011 - 16 months old)

Last week, we had our routine checkup.  Dean meets with his CF Specialist team every three months (barring any illnesses) to make sure that he's gaining weight and doing well.  I walked in with Dean knowing it was going to be a long day.  First we went for his yearly chest x-ray to determine if there was any mucus buildup.  After a registration/insurance snafu, we finally got to the radiation lab.  Dean cried but sat still for the four pictures as long as I stayed close.

Next we went for his routine appointment.  This is a team which includes about 10 people all dedicated to work with us to manage the CF.  Dean's weight and height and head circumference are measured (just as it is at a pediatrician visit) and his pulse ox level is taken (to measure the oxygen saturation of his blood). After routine questions, the doc then showed me Dean's x-ray from May 2010 compared to this one and expressed his concern over the mucus buildup in one of his lungs.  I was devasted.  Honestly, I walked in knowing we've attacked this CF thus far from a digestive point-of-view and he's looks so great, hasn't been sick/on antibiotics in over 3 months, is well above the 50%ile in head, weight and far above in height, and is developmentally where the other kids were at this age!  To me, all was good, I completely expected a "clean" bill of health.  I didn't forget that CF affects both the digestive and respiratory system, I just prayed that since we've triumphed over the digestive part, it was only right that the respiratory would be the same.  The doc explained that we needed to put Dean on Pulmozyme (an inhalation therapy drug) administered through a nebulizer daily which would break up the thick mucus in the airways allowing air to flow better and preventing bacteria build-up.  In older kids with CF, it has proven to reduce the number of lung infections and to improve lung function. The next part almost made me cry.  He said, as soon as he's ready for the Vest, we need to get him on that.   The Vest is just that, a life-jacket like vest that Dean wears that attaches to two hoses that are connected to a generator-like machine which thumps on Dean's lungs for a set amount of time at a set pressure and frequency. I took it as a sign that I failed in doing the manual thumpers.  I know it wasn't true, it's the natural progression of the disease but it's hard to take.  And, everything we've heard is that babies as early as 18 months go on the vest.  We didn't even think it was a possibility that we'd go on it, Dean's only 16 months!!  We've got this little man growing so his chest measured 21" (2 inches above the minimum for the Vest).  After that measurement, all the rest of the visit was a blur.  Instructions on how to get a nebulizer, how to clean it, how to get the Pulmozyme, how to store it, when to give it, the cost, process for ordering the Vest, when to use it, how the insurance deals with it, how to clean that, what type to order.  I was trying to absorb it all while trying not to cry.

More routine things went on but I'll save that for another post.  Finally, after leaving the appt 2.5 hours after arriving at the hospital, we still needed to have blood drawn for labwork.  12 ml lighter, and another 30 minutes later we were in the car and Dean was sleeping before we had driven a block. 

Our little man, so tough and awesome, continues to make us smile, now it's mom's turn to learn how to toughen up.  I feared life would change when the respiratory therapy had to start and I walked around in a daze for two days after the appt.- making and answering calls, filling out forms, figuring out how this is all going to work.  I think we're getting there though (and I know I think too much!).  It'll all be fine.  Jim's perspective is that we can't look at it as if we did anything wrong, just that we're blessed that we can get him started on this earlier than we thought- and we'll attack this mucus buildup just as we attacked his pancreatic issue.  Well said...