Tuesday, 17 January 2017

Parallels in this case study.

Making bread while I wait for more CLIPPERS news.
Happy New Year - it's been a while. I'm afraid everything has  been a bit quiet recently.

Anyway, new year, new post, new case-study. When I finally got around to reading this paper I was pleasantly surprised for two reasons. First, if you have been hanging around here for a while and caught up with some of the jargon, it has a quite concise review of the state of CLIPPERS knowledge in 2016. Second, the treatment strategy is more like my own experience than most of the other reports out there.

In this study a 46 year-old woman first had problems with horizontal eye movement . She had dizziness and problems with gait which developed over 4 months. So this lady was eventually treated with high-dose Prednisolone (in fact in three phases of 500mg/day, 250mg/day and 120mg/day over 3 weeks in total) followed by tapering oral steroids which resulted in very good improvement of symptoms. The patient remained stable on Azathioprine (which was introduced while still on Prednisolone) for at least 6 months - i.e. the length of time reported in the paper. This is far too short a time-period in CLIPPERS to be very significant but encouraging nonetheless. The authors note that treatment with Azathioprine is rarely reported but they consider it important (and I agree) to investigate drugs which may work in the absence of corticosteroids, at least in some patients.

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.

Saturday, 19 November 2016

A neurologist calls ...

The greenest caterpillar I ever saw!
... or rather, I call on a neurologist. 

Last week was my first (and likely only) appointment this year with my neurologist who has been managing my care since I was diagnosed in 2011. Since I last saw him my drug regime changed slightly due to some worries about blood tests. My understanding was that my total white cell count was dropping too low which would make me susceptible to infection. 

My neurologist clarified this by explaining that there are several different types of cells in the total. The one which was causing concern in my case was the neutrophil count. The web tells me that neutrophils fight infection and if there are too few of them even bacteria normally found in the mouth, on the skin and in the stomach can cause serious problems. Now the drug (Azathioprine) I take for CLIPPERS aims to reduce the lymphocyte count - lymphocytes are the cells associated with CLIPPERS. But clearly for whatever reason, both cell counts were being driven down over the summer. He was reassured by my more recent test results and my Azathioprine dose is high enough (based on body weight) to still be having an effect on lymphocytes. We did the usual eye-tracking and finger-pointing tests and some checks on muscle-tone and reflexes and nothing cropped up there.

We also had a discussion about long term Azathioprine use. Unfortunately we don't know for sure if that is what is keeping me well. I said that if the risks of serious illness associated with taking Azathioprine long-term were low then I felt I had more to lose if I stopped taking it and suffered a CLIPPERS relapse. He said he agreed but it was hard to be definitive because of the lack of knowledge about how CLIPPERS works. So I said I would keep taking the tablets. 

It is also sobering when reminded that my status is "in remission from cerebellar disease" which sounds like I am stuck in some kind of waiting room.

Read other articles in this series at Living With CLIPPERS.

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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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