PWS Awareness Month- Day 8

Hi Everyone,

Part three in our series will look at pulmonology, ENT and cranio-facial plastic surgery.

  • Dr. Kang is our pulmonologist and sleep study specialist.  He has lots of experience with PWS kids, so he understands us, which is so important.  We probably see Dr. Kang the most because of Kemett’s asthma, for sick visits and PWS.  We absolutely LOVE Dr. Kang and his staff, who all spend as much time with us as possible, and answer any questions we have for him.  His nurse always answers her phone, and gets us an answer right away.  Last time we visited Denver, I called because I was nervous about the altitude, and Dr. Kang took the time to go over what might happen and where the closest children’s hospitals were located.
  • Dr. Zapata is our ENT and is also great.  We see her every 6 months to look at Kemett’s tonsils and adenoids.
  • Dr. Kelley is our cranio facial plastic surgeon.  We see him yearly now (actually have an appointment next Monday!).
  • Dr. Jacobs is our dental surgeon, part of Dr. Kelley’s team.  We won’t discuss this in the blogs, but here is a link to read about dental issues in PWS.

Our Story

Before you can get on growth hormones you must have a sleep study done to see if you have sleep apnea and what may be causing it.  You can have sleep apnea and not even know you have it.  The more you stop breathing in your sleep, the less time Kemett would have to develop and grow.  It’s also scary thinking that your child is not breathing consistently through the night.  Here is an article discussing respiratory issues and PWS.

Dr. Kang did a consult with Kemett. When he looked at Kemett, he noticed that he had micrognathia (small jaw).  He was concerned that this would be causing sleep apnea and decided that we also needed to see a cranio facial plastic surgeon, who would determine if Kemett needed jaw distraction surgery.  Kemett had a sleep study and the results showed that Kemett had severe obstructive sleep apnea.  Between low tone and a small jaw, his tongue was more than likely falling back and blocking his airway.  Dr. Kang decided we would wait on starting growth hormones until we saw Dr. Kelley.

We saw Dr. Kelley, who agreed that Kemett did have micrognathia and he might need the jaw distraction, so he scheduled an airway evaluation with Dr. Zapata.  He did say that if on growth hormones, Kemett’s jaw could grow.  And Dr. Miller actually did not think Kemett needed a jaw distraction surgery (we saw her during this time period).

We had been approved for growth hormones but were told we could not start them until the airway evaluation.  If he did have something serious going on, it would be dangerous to start growth hormones.

Dr. Zapata did not find anything wrong with Kemett’s bronchial tubes or airway!  And while under Dr. Kelley manipulated Kemett’s tongue by putting a piece of thread at the end and pulling it different ways.  He decided that Kemett was not a candidate for a jaw distraction!  They also both said they had no problems with Kemett starting Growth Hormones.

Dr. Kang and Dr. Miller soon followed with their OK’s, but only if Kemett was monitored by a Pulse oximeter.  Kemett had to be on this when he was asleep.  It monitors heart rate and oxygen saturation levels.  This was a love hate relationship.  I loved that Kemett was being monitored in case levels dropped (this only happened when he was sick).  I hated that it would beep for no reason because it would disconnect from Kemett’s foot.  I was up most nights just to turn the machine on and off.

We saw Dr. Kelley three months later and he was so impressed by how well Kemett could hold his head up, and by how much his face shape had changed.  He said he did not think Kemett would ever need surgery.  Now we just see him yearly.  We also see his dental surgeon for Kemett’s cleanings because of his small jaw.

Like I said above we see Dr. Zapata every 6 months.  Tonsil and adenoids can grow quickly when kids are on growth hormones, which can cause severe sleep apnea.  They  must be removed immediately if this happens.  So far we are in the clear.

Pulmonology and Sleep Studies Update

Kemett had his third sleep study in March.  It was a process to get here.  In August Kemett had his second sleep study that showed his severe obstructive sleep apnea worsened.  He also got very sick last August, and had trouble breathing and had retractions.  We ended up in the ER where they told us he might have pneumonia.  We followed up with Dr. Kang who said he really thought it was asthma.  We immediately got on Qvar.  Dr. Kang wanted to be aggressive in our approach to prevent Kemett from getting seriously sick in the winter.  We also got on singulair to help with inflammation in the nasal cavities.  Dr. Zapata put Kemett on nasonex because he had fluid in his ears.  And reflux can effect sleep studies.  Dr. Scheimann increased Kemett’s dose after his second scope in December so this helped as well. All of this, plus having the sleep study in March outside of winter, was setting us up for good results.

We made it through winter without getting seriously ill, and had our sleep study.  The results were MILD obstructive sleep apnea!  All of Dr. Kangs hard work and expertise helped us!  At the moment we are only on the pulse ox as needed if he gets sick.  We are off of the Qvar for the summer, and we see Dr. Kang in June to check in.

We will have yearly sleep studies, as well as regular check ups with all of the doctors listed above to make sure Kemett is safe while on growth hormones.

See you tomorrow!

Love,

The Demands

 

 

 

Sleep Study Number 1!

Sleep Study Number 3!

 

Breathing Treatments

Breathing Treatments

 

Kemett at our first visit with Dr. Kelley- pre growth hormones.

Kemett at our first visit with Dr. Kelley- pre growth hormones.

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4 thoughts on “PWS Awareness Month- Day 8

  1. SO many nuances to Kemett’s condition and care. Do you feel like a living, breathing, evolving virtual search engine when you wok through the world and when you speak with people? I think it’s awesome that his evaluations have gone so well in this area. ❤

  2. Pingback: PWS Awareness Month- Day 3 | Love, the Demands

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