Hello!  This is my first post.  I have been reading and learning and am grateful that this forum exists.  I have a few questions that I'm hoping someone can address.  Don't worry if you can't, because we have found that even our doctors are uncertain which direction to lead us.

Jonah is 9 and has Down syndrome.  He had T&A at 15 months, due to OSA.  3 weeks after surgery he was trached, due to ???  Constant infection and swelling was leading to chronic hypoxia, both day and night time.  Once trached he only deteriorated.  He began aspirating everything, including his own secretions.  He was hospitalized for almost 9 weeks, until we decided to decannulate him.  Docs weren't sure what would happen, but he thrived.  

Fast forward to today.  Jonah has had 2 open heart surgeries, and currently has moderate mitral regurgitation, left ventricular outflow tract obstruction, pulmonary hypertension and severe OSA.

My main questions relate to understanding his sleep study.  We are scheduled to be evaluated at the end of October in Cincinnati, OH, where they are actively working to understand OSA in children with DS.  Here is the synopsis of his sleep study.  Any input would be welcome!

Sleep efficiency was fair (74%) with a normal latency of 23 mins.  11 awakenings 60 arousals (AI=9.9) All stages of sleep achieved with prolonged REM latency 321 mins w/48 mins of REM (13%) of TST.  No abnormal limb movements.

Desat to 73% awake (1%) and 87% asleep during REM (7%) with mean sat of 94.6.  EtCO2 max was 69 torr with mean of 44 torr, resp rate of 14-24.  Heart rate 60-93 bpm.  13 obstructive apneas max 27 secs (mean 15.8s) 57 partial obstructions max 32 secs (mean 15.3s) and 3 mixed apneas max 23 secs (mean 21.9)  AHI=12.2

We haven't been able to determine if the pulmonary hypertension is being caused by cardiac problems, or OSA.  He is experiencing severe growth failure.  At 9 1/2 he weighs 48 lbs and is 42" tall.  Also extreme daytime fatigue (still naps almost daily) and just wants to sleep and/or lay around all the time.  Very rarely we see a behaviour problem, but he is such a laid back kid that that really hasn't been a problem.

He will not tolerate CPAP at all.  Period.  It's not going to happen for him.  Not sure where we go from here...

Thanks for any insight!

Anji

We have been told that we don't have to worry about pulmonary hypertension in children.  They would only have problems with this in adulthood if they were not treated as a child.  Not sure if other's have heard the same thing.  We have also been told that with children the behavior and school performance is what becomes an issue when they have OSA.

What will they be evaluating when you go to OH?  Keep us updated on what happens.

Michelle :  )
Mommy to Alex (cleft lip/palate, asthma, OSA, TTD, mild hearing loss) and Ryan - 4/30/95
                Christopher (glaucoma, apraxia) - 11/22/01
CarePage: www.carepages.com   (CuttlersJourney)

doublethefun+1 wrote:

We have been told that we don't have to worry about pulmonary hypertension in children.  They would only have problems with this in adulthood if they were not treated as a child.  Not sure if other's have heard the same thing.  We have also been told that with children the behavior and school performance is what becomes an issue when they have OSA.

What will they be evaluating when you go to OH?  Keep us updated on what happens.

Michelle :  )
Mommy to Alex (cleft lip/palate, asthma, OSA, TTD, mild hearing loss) and Ryan - 4/30/95
                Christopher (glaucoma, apraxia) - 11/22/01
CarePage: www.carepages.com   (CuttlersJourney)

Michelle,
I'm not a doctor so take this with a grain of salt.  Typically, children with Down syndrome and certain heart/lung defects can develop pulmonary hypertension at a much earlier age than children without DS.  That is one of the reasons that heart surgery is performed so early in these children.  My son's surgery was performed at 2 1/2 months of age, due to SEVERE pulmonary hypertension.  If you have been told EXPLICITLY that your child will never develop PH, simply because he is a child....I would question that.  Certainly children with "syndromes" or a conglomeration of congenital issues would be at a statistically greater risk, but that does not mean that children without syndromes will never develop PH.  One of the things I've learned from Jonah's medical issues is to NEVER SAY NEVER!!  

In Cincinnati we will be looking at several things.  They (doctors in Cincy) have been finding that children with DS, with prior T&A who still struggle with OSA have several factors leading to continued obstruction.  One of those things are the lingual tonsils (tonsils located on the underside of the tongue), size of tongue (some kids with DS have tongues that seem to be "too large" for their mouths), they also sometimes can pull the tongue forward without resecting it, they are also using radio frequency ablation to the back of the tongue to make it "stiffer" and less likely to fall back and occlude the airway, hyoid advancement and finally mandible advancement for those children whose midface regions are severely underdeveloped.

Behavior is an indicator of OSA, but with my son's delayed development is not as reliable as in typical children.  The BIGGEST indicator, for us, is daytime sleepiness.  He simply cannot get through the day without a nap, goes to be very early and sleeps 12 hours per night.  

The best thing about testing in Cincinnati is that it MAKES SENSE.  One of the biggest tests we'll be having done there is a sleep CINE MRI.  What a novel idea!  Why not look at our kids airways, while sleeping, in a format that will SHOW US WHERE THE OBSTRUCTION IS?!?!?    Sorry, I'm just really excited about this opportunity.  And, at least in Cincinnati, it has applications outside of the population with Down syndrome.  They are using this technique with children with different types of complex airway issues.  

I will be glad to post an update once the testing is completed and we have the results.  

Alex is a sweetie, by the way!

Anji