PAM’S STORY

I am incredibly lucky. Although I’ve got a diagnosis of Schnitzler’s syndrome, a rare autoinflammatory disease, it’s an unusual one in that there’s a medication that controls my symptoms entirely and which the NHS is willing to provide: Anakinra, or Kineret. (It’s also unusual in being adult-onset, as most patients are over 50).

The drawback is that it comes as daily injections. The first time I was shown how to self-inject I struggled a bit. Sitting there with the needle pointing to my tummy felt all wrong but there was no option: I stuck it in as instructed. A lot of people apparently find that Anakinra stings viciously as it goes in, but I’m fortunate that I hardly feel the injection. To avoid using the same site again too soon, I’ve got a system: odd numbered days, right side, even numbered days, left side, 31st of the month (or 29 February), thigh. It works for me. I get a sharps box on prescription from my GP and take the full box down to the surgery for disposal, when going there anyway for a blood test. So the injections themselves don’t really bother me.

The problem is that the injections have to be stored at two to eight degrees. That’s cooler than insulin needs to be stored, so diabetics don’t have quite the same concern, as I understand it. It leads to two problems.

THE KITCHEN FRIDGE

Firstly, our kitchen fridge. I bought a digital thermometer and discovered that the temperature was not reliable. So we went to our local white goods shop and said ‘We need a new fridge, has to fit such and such a space in the kitchen, top priority is reliable temperature.’ He pointed us to his most expensive range, made by a German company better known for cranes but who have diversified. It’s good, it’s reliable. I had a worrying event when I left the door ajar after removing a bottle of wine on a warm summer evening for a socially distanced drink in the next door’s garden. I got home to a thermometer beeping to tell us it had reached 15 degrees, but I surrounded the meds with ice packs from the freezer, cranked the fridge power up to max for a few hours, and haven’t suffered any ill effects so apparently the medication survived with full efficacy. It was all a bit stressful. And there’s the sheer space in the fridge: I get a 24 week supply, which fills a whole shelf in the fridge as six stacks of four boxes. Fortunately, I was almost out of meds over Christmas, so there was more room for turkey and trifle before the January delivery.

TRAVEL

Secondly, travel. I have a splendid coolpack which takes two ice packs and has room for probably three weeks’ supply of injections, and reckons to keep the meds at the right temperature for 36 hours, but those ice packs need to be frozen overnight (I have two pairs of them). So every bed & breakfast or hotel has to be happy to freeze them for me each night. I feel I can’t go on any unplanned travel – never again will we get the bus from Bulgaria to Greece and wander around Thessalonika looking for a hotel, as we did on the way home from our nephew’s wedding. Even a pre-booked train trip through Switzerland staying in three different hotels, the super holiday we had just before I started on the injections, now seems daunting: I’d need to contact each hotel, and the London pre-Eurostar bed & breakfast, before booking the trip. I know people have managed to do wonderful trips with Anakinra, but I feel my travelling opportunities have been cut short.

THE FUTURE

But these are trivial problems; I feel well, and as far as I know I have a normal life expectancy. I’m very lucky. Thank you, Liliane Schnitzler, for identifying this weird lurgy, and thank you, National Amyloidosis Centre (NAC) and NHS, for my treatment.

But if a fairy offered me three magic wishes, one of them would be ‘Can I have my Anakinra as tablets, please?’

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