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 In addition, the authors report that when this corticosteroid was withdrawn a new relapse followed.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.

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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.

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Friday, 17 June 2016


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.

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Wednesday, 25 May 2016

Up and down, like the FTSE 100.

Wormwood Scrubs Common (London)
Well not quite as bad as the FTSE 100 but that's another story (not helped by lots of people on strike and those that aren't on strike going into meltdown over potential Brexit). Of course I'm talking about my meandering white blood cell count which has been wandering a bit further than it should recently.

As my CLIPPERS medication of choice, Azathioprine, willfully interferes with the immune system (which is presumably why it helps keep CLIPPERS at bay), it is expected for my white blood cells to be more thinly populated compared with the norm. The expected range is somewhere between 4 and 7 (I am not a doctor) and mine tends to hover between 3 and 4. About 7 weeks ago it dipped to a historic low of 2.6 and there was much muttering amongst the medics behind closed doors which resulted in me being allowed to stay on Azathioprine for now, but in exchange for having further tests every 3 weeks or so.

On the next test the count had gone back up to 3.something - hurrah! However, on the one I had last week it had dipped back down to 2.9; this is still better than my previous historic low of 2.8. So the tests will continue and I'll let you know if anything strange happens. 

There are no specific symptoms associated with low white blood cells but I was already being as careful as possible to avoid sources of infection - lots of hand-washing and keeping hands away from my mouth, nose, eyes etc. Public transport in the mornings makes me uneasy - too many germs! My doctor emphasised that if I get fevers or unexplained illness I need to see him immediately - presumably under these circumstances I will be able to bypass the 3 week wait for an appointment that is the current situation here.

Read other articles in this series at Living With CLIPPERS.

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Living With CLIPPERS by Bill Crum is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.