Monday, April 2, 2012

Piecing the Puzzle Together, and Coming Out of the Closet

PWS is a mystery. Many more common conditions, like autism, are largely un-figured-out (that's a technical term), so all the more so with something rare like PWS.

One thing we've heard more times than I care to hear is "I don't know," from doctors who know a lot or a little about PWS and what's going on with Dean. This is really no one's fault; more, it speaks to the unknown aspects of PWS.

No one is exactly sure what landed Dean in the hospital last weekend. Some combination of pneumonia, dehydration, and asthma. No one is quite sure why Dean is still requiring oxygen at night over a week later (this is not typical since his surgery in October). We had a few nights where he didn't need it, and then back to this. Could be asthma. Could be an unresolved sinus infection related to the possible pneumonia junk (he's on another antibiotic). Could be something called "borderline respiratory reserve" (I have no clue what this is - waiting to hear more from Dr. Schmidt, the pulmonologist). Treatments for any of these include nebulizer around the clock, antibiotics, stimulants, or long-acting steroids, depending on which etiology you subscribe to.

Which leads me to another topic: stimulants. I have some each morning in the form of diet Coke. Ok, kidding. Well, I do, but not for medicinal purposes in the way that Dean might need it. Dr. Schmidt suggested caffeine (they can prescribe a low dose for children) as a respiratory stimulant to get Dean to ventilate more efficiently on his own, and therefore better his oxygen saturations. Thing is, Dean is already on something called Provigil.

Provigil is a central stimulant that is used in people with PWS to combat excessive daytime sleepiness (EDS, not to be confused with Ehlers-Danlos Syndrome) and narcolepsy. See this link for information about the research behind this: http://fpwr.org/blog/research/11/06/modafinil-improves-daytime-sleepiness-pws.

Several of Dean's doctors think he might indeed have narcolepsy (we're waiting a neurology appointment in June) and he does deal with some degree of EDS, affecting therapy and school. He tires very quickly in these activities and then acts out. Yes, he's 2, but the acting out is from this fatigue that goes beyond "normal" fatigue. We were very wary about starting such a young child on a stimulant, and if you'd asked me even a year ago if I would have considered something like that, I'd have shaken my head at you and told you absolutely not. We somewhat secretly asked other parents whose kids were on Provigil how they came to make the leap and what effects they'd seen. The reports were literally all positive except for one (too many side effects).

But Dr. Miller has assured us that Provigil is safe for Dean and we trust her. And we knew how much the EDS was interfering with Dean's ability to do work and to stay awake while eating. His energy levels are otherwise pretty good.

So, that's where we are. We're going to continue on with the new antibiotic for a little bit before trying anything else because otherwise it's just too many moving variables. It's weird and sad to play scientist with your kid. I saw a sign the other day that said, "It's your kid, not a gerbil." Yeah, well, to whoever wrote that sign, I'm sure your kid didn't have a rare disease. Unfortunately, sometimes we just have to pray and consider, and then go a direction we never thought we'd go.

So, we're out of the closet. We put our almost-3-year-old on a stimulant. Judge away. As with other situations that I've judged from the outside, I never really knew what I'd do until I was in that situation. When even Bob was on board, I knew there was something to this (he's the more rational one of the two of us, in case you hadn't noticed ;)).

6 comments:

Kevin said...

Breathing is important, not quite something that should be debated.

Katie said...

Ali, I commend you and Bob on the decisions you have made with regard to Dean. He is your child and you love him dearly and want the best for him. Trust that God is giving you the wisdom to make the right decisions and don't try to carry the burden all by yourselves. I hate to think how I would decide such major issues if I were faced with the problems you folks have all the time.Love you all and pray each day for you. Grandma

Maegan0514 said...

Ali, Dr. Miller gave us a Cataplexy diagnosis..I knew it all along. I know we have discussed this before so thought I would let you know. We will be starting Provigil as well when Cade is old enough.

Ali Foley Shenk said...

Maegan,
Dr. Miller believes that Dean has it, too, even though his episodes look different from Cade's. Provigil has really eliminated a lot of the things that we saw, as well as the other benefits we've noticed. I think it will be a good thing for Cade! How old did she say she wanted him to be before going on it?

Mary said...

Ali, I think its great you have Dean on Provigil. Oscar's had EDS forever, and while it is definitely getting better it has certainly interfered with his development. While other kids had play dates after school Oscar always went home to nap. He fell asleep in school, has needed tons of sensory activities during the day. We are hoping to get him on it this year -- but we have a long overdue sleep study to complete first. Congrats to you for helping Dean fight this frustrating and debilitating aspect of PWS!

Ali Foley Shenk said...

Thanks, Mary!! he's been able to have much more productive therapy sessions and school days since starting. Seemingly, there are no side effects for him. I think it was the right decision.