Friday, August 3, 2012

Tools to Help

This morning we had Dean's appointment with the neurologist/sleep doc who read his sleep study the other week. When I called for a follow-up appointment, I was told that the next one was in November. NOVEMBER. Sorry, you can't give me limited information about the extent to which my child is able to breathe at night and then say, 'see you in November.'

So I did what I learned in PWS Mama Bear School and politely called, and emailed, and called again. It follows some pattern of acknowledging their extreme patient load, asking them to understand the position that *you* are in, and then asking how we can work together to get things done sooner. Offer to do some of the work yourself even though you shouldn't have to. Be polite, but firm. If the doctor rocks (which this guy - Dr. David Leszczyszyn at VCU - does), they'll figure it out.

They called me Wednesday and told us to come in this morning. Sweet! The doctor doesn't even see patients on Friday, he's going on vacation tomorrow, and he squeezed us in this morning. Like I said, he rocks. :) My mom came down to help since Bob was home and going to Emmett's 18-month appointment at the same time.

There was actually a lot of good news in Dean's sleep report. This was good to hear.

-Dean spends a lot of time in the deep sleep phases. He said that you or I would kill for the amount of deep, restorative sleep that Dean is getting.

-Dean does not have central apnea.

-Dean's O2 levels are for the most part great during sleep. He has some small desaturations, but these are not nearly as significant as they used to be.

-His CO2 levels during sleep (which can be elevated in PWS) are fine.

The "bad" news is that Dean is having obstructive events (something physical is blocking his airway while he is sleeping), about 10 in an hour. This is considered moderate, so it is significant enough to treat, but not severe enough that it is likely to pose any immediate or even shorter-long-term threat to his heart and lungs. If you missed what happened in that space, it was me being able to take a deep breath just knowing that.

I had a question of why Dean's last sleep study in January showed insignificant apnea if he suddenly now had it again. If you remember, we found out the next day (from Dean's crappy results that night) that Dean actually had pneumonia during that study. Dr. L's theory is that, since Dean was so sick that night, he was probably spending very little time in deep sleep, which is the time that Dean is/was having almost all of his apnea events. Sort of a clinical didn't-see-it, didn't-happen scenario. Except it did. And it likely means that his tonsil & adenoid surgery didn't do much. :/ Oh well, we tried.

Sleep apnea is very, very common in PWS, mostly because of low tone, but for other reasons. Dean, like many others with PWS, will need to sleep each night with a BiPAP machine. The machine will deliver enough pressure to keep Dean's airway open so as not to have an obstruction, and it will deliver different pressures depending on whether he is inhaling or exhaling. If you've heard of a CPAP machine, it's similar except that the CPAP delivers continuous (hence the "C" in the name) pressure to keep the airway open.

Image courtesy of

We won't get the BiPAP until Dean's next study at the beginning of September, where a tech assigned just to him will help him tolerate the mask while titrating the pressure at night to make sure it's just right for him. We'll follow up with the doctor from there.

I've spent a lot of time these past three years shaking my fists at the sky and just asking, 'WHY?' I'm guessing this question is among the most unanswered in the world. And I'm learning that while PWS is kinder to some, it's not horribly kind to Deano. We can only control so much (and for the record, we're working our butts off to help whatever actually *can* be helped).

So I can get mad at the why (and I probably always will a little - what mom is *ok* with their children having to deal with this stuff?), or I can be grateful for any tools that can help my child get through this. We are grateful for whoever developed the BiPAP so that Dean and others can sleep safely at night. Obviously I'll keep you posted as it all goes down. Thanks for all the prayers and support.


Giulia said...

I am by no mean a doctor. But would it be possible to ask to redo the sleep study when Dean is not sick ?
Because if the study shows apnea during Dean's sleep when he is sick, it worth the effort to know if it happens during a non-sick moment.
A sleep study, like a blood lab, or any test you want, is a photo as a time T. The results are never written in the stone. They can evolve during the course of time.

I say that because my IQ test gave a gross result of 120 when I was 13 years old (seems unrelated, but I will go ahead).
The psychologist said that my test gave a misleading gross result, and suspected a sensorial or anxiety disorder (difference between verbal and performance IQ, verbal was much higher than performance). The giftedness was found on all the other findings, but not on the test. Does it mean that giftedness is a no no 6 She said that no, it does not mean that it is a no no, it means only that independent factors ended to a misleading gross result.
Well, I have been diagnosed with a Single Sided Deafness (difficulties with laterality and spatiality, since you have no stereo at all) and ADHD.
I took again the test a few days ago, and the psychologist told me that my lack of self confidence may impact the final gross result.
What I really mean is that a test needs to be often repeated sometimes to see what is the root problem.
A test is only one element to lead to a diagnosis.
Don't over rely on tests, for yourself and for Dean. They can be very misleading due to external factors.
A diagnosis cannot rely only on a test, as accurate as it is. It has to rely on many elements.

I also know that mom snors a lot when she is sick, whereas she does not when she is not sick.
I know that PWS is a very specific condition.
But don't think that Dean will need to wear his BIPAP all his life. It may be, it may be not.
It is not written in the stone.

So, please, take it one day at time.
Because BiPAP for persons with PWS on a lifelong basis is not written in the stone.
It happens, yes. But it does not mean that it will happen for Dean : it may happen, it may not happen.

Since you are very confident with Dean's pulmologist, you can talk about it with him next time.
I am sure you will end to the best solution for Dean's needs.

Ali Foley Shenk said...

Hi Giulia,
Dean was not sick for this sleep study. He was sick for the previous one, which is why we did it again. I am confident in the results, as I have seen the moments where Dean has an obstructive event while sleeping. It was nice to get results that we knew we could trust.
Ali :)

Giulia said...

Hello Ali,
I understand better what you mean.

But it does not change what I say about the fact that BiPAP for all Dean's life is not written in the stone. It may be, it may be not.

If it helps, good. And you'll see over the time if he continues to need it.
Only time can tell.

Take care

Ali Foley Shenk said...

Yes, absolutely! We will take it one sleep study at a time :)