Tuesday, October 4, 2011

Monday, October 3rd, 2011

A note from Mommy:

After four years of talking to surgeons, pediatricians, ENT's and pulmonologists you would think I would be an expert on Peyton's airway (or lack thereof).  When Peyton had her bronchoscopy a few months back, Dr Muntz was clear that her airway was still very small but didn't get into much detail and I was anxious to get back to recovery to see her.  Today when we saw Dr Muntz I felt I had a little more time to ask some questions.  The original reason for the appointment was to discuss the abutement surgery for Peyton's BAHA's.  When he indicated that he doesn't do these any longer and to see one of the two others in the practice that specialize in it - it presented an opportunity to get in a general discussion about Peyton's airway.

Dr Muntz presented a check list to decannulation:

1. Seeing the larynx during bronchoscopy
2. Daytime capping trials
3. Nightime capping trials

When Dr Muntz did Peyton's bronchoscopy this summer, not only was her airway small, he could not even visualize her larynx and it was almost impossible to get the tube in.  So when he is talking small he means very very small.  For kids with micrognathia there are usually two ways to "grow" the airway, one being the jaw distraction and one being general growth of the child.  Since Peyton is not even a year out from her jaw distraction, what did that mean?  Dr Muntz has treated a handful of patients while in St Louis (13 years) and since being at Primary Children's - maybe 15 in total.  And one thing he has seen is that Nager patients don't respond the same way to distraction as other children with micrognathia - the results just aren't the same.  Nager patients are unique to themselves (something I seem to keep hearing).    So what about general growth of the child - what should we expect with that?  Peyton is really small for her age and each child of course is different but kids who grow into a bigger airway through general growth of their bodies he felt happened around 8 or so. 

Dr Muntz said with the airway we want to watch it every year because one year he may not be able to see the larynx and then all of a sudden the next year it is there.  He also wanted to clarify that there have been children decannulated that there larynx was not visable.  We'll get to that in a minute....

The second requirement is daytime capping and the ability for Peyton to cough up her secretions so that she doesn't develop pneumonia.  Peyton can already cap for long stretches during the day so we know she can tolerate capping.  Another good test would be to have her capped during a cold to see if she can get everything up and out.

The last requirement is nightime capping.  Since we are not anywhere close to completing surgeries, Dr Muntz is in no rush to complete one.  But if surgeries were done, her daytime capping continues as it is and she failed her nightime sleep study it doesn't mean she could not be decannulated.  His recommendation for most kids is that if the only issue with the airway is sleeping, he would trade a trach for decannulation and CPAP.  When I brought up the low compliance rate for adults with CPAP he was quick to agree - he said somewhere around 75% of adults using CPAP are not compliant.  However, he felt that children were actually the opposite - he estimated 75% of kids were compliant espesically if they were old enough to understand the trade off - trach or cpap. 

So what we are left with is that the airway may grow enough on it's own or it may not.  It may require additional jaw distractions or it may not.  If we get to a point where most of Peyton's surgeries are completed, she is completely stable and capping trials during the day are still going well but she is failing a sleep study there is a possibility of removing the trach. But it would be risky if an emergent need for an airway arose.

Knowing what I now know about Nager kids and Peyton in general, I can pretty much bet we are going to be stuck making that very difficult decision in the future.  There is no crystal ball to tell us if there would be an emergent need for an airway in the future.  We also can't rely on outcomes for surgeries such as jaw distractions based on kids that do not have Nager Syndrome - these kids are just not comparable.  We are blazing our own trail with not much more than educated guesses from doctor's to help direct us along the way. 

On the way home I wasn't crying or angry - but I still have a lot of emotional investment in getting her trach out and I wasn't sure why.  And then it hit me.  For some reason I believe that decannulation is my personal responsibility and if it doesn't happen it is ultimately my personal failure.  As a result of today's conversation with Dr Muntz I finally have a sense that none of this is really in my control.  Ok I know that sounds funny - it never really has been in my control - but I don't think I have really believed that before now.  In the back of my mind if I got her to the right doctors at the right time for the right surgeries I could make it happen.  Today driving home it occurred to me that despite getting her to the right doctors at the right time for the right surgeries her body may just not ever allow for a decannulation and there is not a damn thing I or anyone else can do about it.  You may think that was a devestating concept but just the opposite - although it was a very sobering thought - I also felt some weight lifting off my shoulders.  So there is a new mantra running through my head now - It is not my fault she has a trach and it won't be my fault if she can't get it out.  And someday it may be her mantra as well - It is not my fault I have a trach and it won't be my fault if I can't get it out. 

1 comment:

  1. I REALLY understand what you are saying. It is a relief to take decannulation off the table. When you can accept that the trach may be for life, it frees you to live your life and stop waiting for D-day. Then if it happens it's just a bonus.