Kai is on a lot of different meds. Most we can get from our local pharmacy. A lovely little family pharmacy that delivers (I can’t tell you how much I love that they deliver. They are amazing and I adore them and our hectic life is made a kajillion times easier by this simple service).
However, there are two vital medications that Kai is on that we can’t get from our local pharmacy.
Sodium Benzoate and Dextromethorphan.
Sodium Benzoate is typically used as food preservative. Dextromethorphan is usually in American cough syrup. Buuuut we go rogue with off-label use. (Side note: rogue under the direction of our metabolic consultant… medical rogue).
Sodium Benzoate pulls glycine (the bane of NKH) out of Kais blood, transforming it into hipperate so he can pee it out. This normalises the glycine level in his blood, and reduces slightly the levels in his brain/spinal fluid. This is the medication we rely on most to manage NKH.
However, because it doesn’t normalise levels in his brain, we use Dextromethorphan (DXM). By the time it gets to the brain, DXM is able to occupy the glutamate binding site of an NMDA receptor. The other binding site is glycine, which essentially means the NMDA receptor can’t fire and the neurone is given a break.
It definitely does a bit more for Kai than suppressing coughs, that’s for sure.
Because they’re off license, they have to be prescribed and ordered through the hospital. We are the only regulars at our hospital that have NKH. We are the only ones they order Sodium Benzoate and DXM for.
DXM takes 14 days to ship over from the states, where it is an everyday over the counter medication. We get ours in the form of Delsym. A grape flavoured cough syrup. It’s violently purple.
Sodium Benzoate takes 10 days from a company in Oxfordshire. So for both it’s not a small ask – they require forethought and planning to get hold of.
Kai takes these medications four times a day. When he doesn’t have Sodium Benzoate, his glycine levels sky rocket. High glycine levels means excessive sleepiness, seizures, and a coma. When he doesn’t have DXM, the NMDA receptors in his brain are constantly overfiring. Which means more seizures, neurone death, and more brain damage.
So not having them is not an option for us. Not while his brain is so young.
Long story short, there was an monumental miscommunication between the pharmacy, our community care nurse and me. I asked for the prescription to be chased within the two week window well before we needed it, it was chased outside the two week window, and the pharmacy got the prescription two days before we needed a new supply.
I only found out when our new CCN called to say it was available. Which was awesome, so down I toddled to the hospital to pick up the meds. I congratulated myself on going on a Sunday morning, when the pharmacy was empty and I wasn’t going to have to wait hours and hours. I feel like fate was quietly lol-ing at me and my pride. Seriously… look at that smug face in the empty pharmacy:
Panic. Extreme extreme panic on my part.
In the end in a flurry of calls to our CCN, and a dire ‘without these meds Kai will go into a coma and have an inordinate amount of seizures’ to the pharmacy staff – I was asked very politely to leave it with the pharmacy staff and they would call me. In the end, despite my nervousness, they did. They sourced a few bottles to tide us over from another hospital. Thank goodness for that.
In the meantime I’d gone to our amazing NKH support network with an ‘OMG SOS’ help request, and so many wonderful generous people came back and said they’d ship us a bottle if we needed.
I’m grateful we didn’t, but I can’t tell you how reassuring it was to have people who could help if we got desperate. (Our NKH family is the best).
So, crisis averted.
The day I need to ask for the prescription to be organised has been marked in my calendar regularly for months and months. Now there are several chase up the prescription reminders, just in case.
I never thought my life would be so dependant on others, that the care of my son would very literally come down to other people doing their jobs and doing their jobs well. It irks that so much of his care is out of my control. That I have to trust people who do this for money, instead of love.
Le sigh. Fingers crossed it doesn’t happen again.