Friday, 2 September 2016

The Alien in the Room

No aliens here.
Wow. It is 5 years since I was diagnosed with (probable) CLIPPERS and pumped full of intravenous Prednisolone for a week. I was still in hospital and waiting for an MRI to show "radiological improvement" before they would let me out. At the time there were considerable question marks, not only about the long-term prognosis for CLIPPERS, but also about whether the diagnosis was correct or whether I might have something potentially worse. Since then, I have made steady progress and been remarkably stable health-wise. But I am careful not to become complacent.

I am a child of the video-game generation and have been playing games of various kinds virtually my whole life. I tend to like games which have a strategic, exploratory and/or puzzle component but am not averse to a bit of (video-game) ultra-violence, especially if combined with one of the above. Currently I am playing a game which I stayed away from for a while as I thought it would be too far outside my comfort-zone - Alien : Isolation. This is a game set in the Alien universe where, to cut a long story short, your character is stranded on a space-station being stalked by a predatory alien and with virtually no effective weapons. You can't kill this creature, you can only hide from it, distract it and occasionally make it retreat for a very short while. The creature wanders the corridors, travels through air-vents and is systematically searching for you. When it finds you, you come to a grisly end. Spooky.

It made me think about my experience with CLIPPERS. Like my character in the game, I know I am trapped in an environment with something nasty which I can't see and can't do much about. In the game, sometimes the alien disappears for a while, sometimes you can hear it rattling around nearby vents and sometimes it comes out of the vents and searches the rooms. In the game I am always listening and looking for signs the alien is near and in real life, although CLIPPERS doesn't seem very close at the moment, I am still looking for signs that it may be lurking nearby. Unlike in the game, if CLIPPERS reappears I can't hide from it, but at least I have a bit more idea what to expect. Also, in the game, I know I will ultimately have a chance to escape completely and I'm hoping the same will one day be true of CLIPPERS.

Update: With Azathioprine reduced to 150mg/day, my white blood cell count has stabilised at 3.4 which is acceptably abnormal. No signs to date that this dose reduction has had any unwanted CLIPPERS effects.

Read other articles in this series at Living With CLIPPERS.

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Sunday, 10 July 2016

An interesting treatment case

My local sun-bathing blackbird.
I saw a new case study recently which suggested a variant on treatment. In their paper,
IFN beta 1a as Glucocorticoids-Sparing Therapy in a Patient with CLIPPERS, Dr Rico and colleagues, discuss a CLIPPERS patient who suffered approximately one relapse a year when tapering steroid treatment was used. The initial high-dose treatment gave (incomplete) improvement but once the lower dose treatment ended, the symptoms returned. This patient was first seen in 1996, well before CLIPPERS had been defined as an entity in the medical literature. The doctors suspected an auto-immune disease but had ruled out Multiple Sclerosis (MS) so they decided to try an alternative imuuno-suppressant therapy, namely interferon beta 1a. I don't know anything about this treatment beyond the fact that it has been used to treat MS. The patient subsequently only suffered one relapse every 4 or 5 years and relapses did not permanently worsen his condition.
 
However, the patient was also maintained on a corticosteroid dose equivalent to 25mg Prednisolone every 2 days which makes the interpretation of his improvement less clear. In addition, the authors report that when this corticosteroid was withdrawn a new relapse followed. So it is not clear to me how these two parts of the treatment are working and whether both are required or whether it is the continual low-dose steroid which is important. The authors also repeat an assertion I have read elsewhere, namely that "... except for methotrexate and possibly rituximab, no drug has been able to have sustained control of the disease without combined oral glucocorticoids ...". Maybe I need to put my hand up, as I have been stable on Azathioprine for 4 years after steroids were tapered to zero. Of course, technically I am an IPPER not necessarily a CLIPPER (see previous post) so may be atypical, and there is no guarantee I will stay this way (especially with my currently meandering white blood cell count and treatment changes). Unfortunately, treatment response is just one more part of the CLIPPERS story where there is still a lot of variability and uncertainty.

Read other articles in this series at Living With CLIPPERS.

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Update

Reflection on a London tube-train - curved glass.
Regular readers may recall I was having problems maintaining a reasonable white blood cell count while on 200mg/day Azathioprine. Since then, my readings went up slightly (3.a-little-bit) and then dipped down again to 2.7 at which point the doctors decided action was required. Of course I feel fine day to day but if the white blood cell count gets too low I could be at risk from infections - and this is someone who travels on public transport to work so I'm concious of being exposed to bugs from other people most days. So I was told to reduce my dosage from 100mg AM + 100mg PM to 100mg AM + 50mg PM , a 25% reduction overall. 

I am in uncharted territory now - and a bit paranoid to be honest - as I have been on this dose for 4 years and have no idea what the critical level is or how tightly connected the white blood cell count is to the chance of CLIPPERS relapse. My thinking is that this dose change is simply returning the white blood-cell count to the intended effect of the therapy in the first place so everything should be OK. I have my first blood test since changing the dose this week and I will keep you posted.

Read other articles in this series at Living With CLIPPERS.

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Friday, 17 June 2016

CLIPPERS or IPPERS?

Not a family-friendly beach!
In a recent letter, Dr Taieb remarks that "most reported cases of CLIPPERS are in fact only ... PPERS due to the short follow-up and the absence of biopsy". So he is pointing out that in the absence of biopsy the "Chronic Lymphocytic" part of CLIPPERS is unproven. (He also takes out the "I" for Inflammation but I would argue that MRI findings in presumptive CLIPPERS patients suggest some form of inflammation.) I am happy to call myself an IPPER instead of a CLIPPER (as I am one of those unbiopsied cases). I'm not sure what the alternative is - pePPERS? 

This hides a serious debate about diagnosis of CLIPPERS - the CLIPPERS population is a mix of folk with different kinds of diagnostic tests and different levels of diagnostic confidence. We have all at least had most other conditions excluded. Dr Taieb also suggests that a relapsing-remitting pattern should form part of the diagnosis for CLIPPERS (like in Multiple Sclerosis). However, that would mean that I don't have CLIPPERS as I have, so far, been relapse-free on medication since my first onset.

Read other articles in this series at Living With CLIPPERS.

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