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The Fine Art of Medically Managing Narcolepsy In Children

The very first medication prescribed to our then three year old daughter with Narcolepsy was  Modafinil. Here in the US it's known more commonly as Provigiland is used to promote wakefulnessI remember at the time back in 2010, thinking that it was sort of counter intuitive--to try and keep Mathilda alert in the day when she wasn't able to sustain restful sleep at night. Seriously, could someone explain to me how that might work? It was a question I threw at the the pediatric Neurologist heading up Bristol Children's Hospital. And that was a little cheeky really, because I knew they couldn't or wouldn't get hold of the night time drug (Sodium Oxybate) that might, just might manage the vast majority of Mathilda's symptoms. If that were possible, perhaps Provigil would have been more effective then.

It seemed pretty obvious to the Professor and me that addressing Mathilda's nighttime symptoms was paramount. It certainly came before forcing daytime wakefulness on a child who was beyond exhausted. Besides, it wasn't as if she was going to suddenly sleep through the odd night and be able to deal with the host of daytime symptoms too. Not as far as I could see anyway. The disruption to her sleep, the hypnogogic hallucinations and inability to achieve Non-REM were so troubling and undoubtedly gave rise to her inability to maintain periods of wakefulness in the day.

Within a few weeks of being on Provigil the first time, Mathilda was more alert for sure, but it came at a cost. At the time I sort of traded a little more day time wakefulness for aggression, moods wings and melt downs. All because we were desperate to offset excessive day time sleeping and see a girl who might have the energy to play or get up the stairs unaided.

This video was taken at home when Mathilda was three and only on Provigil. It's a good example of Excessive Daytime Sleepiness (EDS) and why CWN need effective management of their 24 hour symptoms. Stimulants alone didn't cut it for her. 

And as my pleas for Xyrem fell on deaf ears she was left with just the Provigil and another drug called Gabapentin for her leg pain which she was weaned off one we got to the USA. The dose was apparently way to high for a young child and side effects include, fatigue and weakness--just what you don't need when you already have Narcolepsy with Cataplexy.

In July 2011, we did then begin the fine art of titrating Xyrem for Mathilda, (prescribed by Stanford, delivered by the SDS pharmacy)-- a process that took over a year to figure out whilst tackling the side effects: mornings of nausea, vommitting and headaches.

In the time since Mathilda has tolerated the drug well and also began taking Strattera in the mornings to help with wakefulness and attention at school. Not a bad combo by any means.

So when last December Prof. Mignot at Stanford suggested Mathilda tries Provigil again, I was extremely skeptical. In fact I think I blurted out that I hated the drug and couldn't believe he was suggesting it. He had after all been the one to take her off it at our first appointment.

I countered his reasoning partly because of the memories and partly because I thought she was pretty optimal anyway. When I say optimal I mean taking Xyrem 3 hourly at night, having 2 long-ish naps a day (one in school) and having the energy to play at home, attend the odd birthday party, and complete the lesson she missed school. I'd long since given up on pushing extra-curricular. That's not been realistic.

Still, this has been a significant improvement on life before Xyrem. By a l-o-n-g way.

And frankly, because I can barely remember how bonny and bouncy she once was, I have sort of settled with the limitations it places on her and our family and genuinely delighted in all the things she can do.

 
 

I'm still trying to figure out how the US health system works here because it's so different to the NHS and because you'd think getting our hands on a prescription for commonly prescribed drug should be fairly straightforward, especially since we cough up our insurance money every month. But our particular medical insurance refused to pay for the new prescription. We appealed, twice, and finally, several months later Ralphs pharmacy delivered Provigil to our doorstep.

So 10 days ago, relieved that the appeal process had been successful I took a deep breath, popped another pill in Mathilda's mouth one morning (100mg Provigil) and crossed my fingers half thinking I would let Stanford know they were wrong. That a drug which didn't really help the first time, wouldn't now either.

I have to say the results have been really surprising.

Her naps are significantly shorter which means she doesn't miss out on so much at school, and perhaps more signifcantly her extreme grumpiness around 3-5 pm is a whole lot more manageable. In fact somedays, almost non-existent. I know I bang on about the behavioral changes that can come with Narcolepsy in children but that's because it is so utterly miserable, she's been so unlike the girl she once was and it's stressful on the whole family. Typically when Mathilda falls apart between the driveway and the doorstep, we all know we're in for a rough ride--Libby and Elliot withdraw to their rooms because there's not much we can do to help her and it's hard to deal with every single day. It's not exactly what you'd call happy hour.

But this recent new energy has meant coming home from school isn't the war zone it once was and Mathilda has found the capacity for extra-curricular things like Shakespeare club. We even got out one evening this week to an Ash Wednesday service.

 
 

It's almost too good to be true.

A not so tiny triumph of sorts.

It seems to me, getting the balance with the right cocktail of meds, and all that goes along with the complexities in accessing specialist care and treatment (wherever you live), is ultimately worth the enormous effort required in making what is a fine art a reality.

Because the difference is immeasurable, the benefits immense and because every CWN  and their family is worthy of it.

With Grateful thanks once again for the team at Stanford, to Janni at Ralphs Pharmacy and to my ever patient family. 

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