Musings of a Marfan Mom

It’s been a whirlwind week and I’ll update on conference soon, but first I wanted to write about yesterday. As you know, Baby J had a sleep study 3 weeks ago. It was like pulling teeth trying to get the results…the hospital was terrible about returning calls from either the pediatrician or me and when they would call back, they’d have a vague reason for the lack of information. I finally got them to fax the results at the 11th hour…Thursday evening at 10 minutes to 5 so that I could take them to our clinic cardiology consult the next morning.

I expected J might have some mild form of obstructive sleep apnea, but I was surprised to learn it’s actually severe. Both the pediatrician and the cardiologist we saw at the conference clinic advised us to get started with treatment immediately.

When I called the hospital today, I was told the earliest appointment was early September. Long story short, I was polite but quite firm that that wouldn’t work, and was eventually given a cancelled slot for that afternoon.

The pulmonology fellow was very nice and understood why we weren’t willing to wait 2 months without a treatment plan. Her attending convinced the ENT to see Baby J that afternoon for a scope to look for any potential surgical cause. The scope showed that Baby J’s adenoids are slightly enlarged, but most likely not enough to be problematic, and that he has a mild case of Laryngomalacia, but that wouldn’t be enough alone to cause his severity of apnea. He recommended J have some further testing under general anesthesia to totally rule out any surgical causes of the obstruction. He wanted to take out the adenoids at the same time “just in case,” but I said no. I can’t bring myself to do surgery on a baby that isn’t 100% necessary, especially a Marf baby where we don’t always heal like a non-Marf person does.

Given the results of the scope, the pulmonologist recommended oxygen for Baby J whenever he sleeps. She said that although his apnea is bad, it’s not life-threatening at current levels so we could opt to not use anything in the short term, but I know how rough apnea can be on the aorta so I agreed to the oxygen. In late August or early September Baby J will have his bronchoscopy, etc. and we’ll make a final determination about treatment at that time: either surgery or a cpap. The oxygen should relieve some of the pressure on his aorta in the meanwhile.

So, it was 5:00 when I left the hospital. The oxygen tanks had been delivered to our apartment by 8:00. We’ve got 4 little tanks for trips out of the house where J will nap on the go (I’ve named them all “Ollie the Oxygen Tank”), along with a backpack, and a great big tank for the bedroom (“Calvin”). [You’ll remember I like to name medical equipment.]

Being totally honest, I’m feeling a little scared/overwhelmed. I was already unnerved learning that J stops breathing about 12 times an hour…now I have visions of him choking to death on his canula or the whole house exploding from an accident with Calvin. Yes, I know I’m entirely irrational.

This is just one of the first in a long line of medical decisions we’ll have to make for J that don’t have a clear right or wrong answer. I hope he takes to sleeping with the tubing easily. If you have any advice to share about what worked with you/your child on oxygen, please let me know!