Updates

On Cheddar Gorge in 2022

Time for a few brief updates. Thanks to those who have read the CLIPPERS book and those who left a rating on Amazon; I hope you enjoyed it or found it useful, or both.  You may have read that Amazon are raising their global printing costs in June 2023, but the CLIPPERS book will remain at it's original pricing for now.

I had a telephone review with my consultant recently and, following a brain-scan last year which reported essentially normal (or at least no change), had nothing much to update him with. It's nice to have retained the same consultant through my CLIPPERS journey, which makes catching up easier (for both of us!). We had our now traditional conversation about future treatment strategy. I've been lucky to remain stable on Azathioprine since being weaned off steroids about 9 months after diagnosis. Taking Azathioprine comes with a small hypothetical risk but trying to compare that against the risk of not taking it for CLIPPERS is very hard. My view has always been that CLIPPERS can cause serious and potentially long-lasting problems, which in my case meant a month in hospital and easily six months in recovery; but at least I did recover. The risk of relapse is real but unfortunately unpredictable. Judging by the number of CLIPPERS case reports still appearing, relapse is still common so as before,  I said I thought that relapse was a bigger gamble and my consultant was happy to support that decision.

The fact that I still can't gauge a risk of relapse made me think. I don't have an inside track to the latest knowledge about CLIPPERS, but reading some of the recent papers  makes me feel that in some ways things haven't changed very much. Here's an example from 2023:

"The diagnosis of CLIPPERS is difficult and requires extensive differential diagnosis. A specific biomarker in serum or cerebrospinal fluid (CSF) for this disorder is currently unknown. The pathogenesis of CLIPPERS remains poorly understood and its nosological* position has not yet been established. Whether CLIPPERS represents an independent, genuine new disorder or a syndrome in the course of diseases with heterogeneous aetiology and/or their precursor stages remains debatable and incompletely clarified."

(*nosological = disease classification including an understanding of mechanism)

I accept everything said in this extract, but it could have been written for virtually any CLIPPERS paper over the last ten years. I hope in another ten years a similar extract will read differently.

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

Has it really been that long?

Dartmoor 2022 Some rare sun.

You know how it is; there's something at the back of your mind that you feel you really should do but you don't quite get round to doing it. And so when I looked back at this blog page it was a bit of a shock that no new posts have appeared for well over a year. (I'm pretty sure I did a bit of tidying up behind the scenes since then, but it's a poor excuse). I don't have an enormous amount to say, which is why there has been such an update drought, but I did manage to miss my own anniversary of a decade since being diagnosed with (probable) CLIPPERS in 2011. I've partly been prompted to write here after a nice email from a fellow CLIPPERS patient who hoped in passing that the blog silence didn't mean anything bad had happened.

Since the last post I've managed not to catch COVID helped by working almost exclusively from home and by taking every vaccine offered. The booster program is a little random here but they seem to be erring on the side of caution as I've had two initial jabs (Astra-Zeneca) and three Pfizer "boosters". I felt pretty mouldy for a day or so after the second Pfizer one but took more care with hydration and ibuprofen after the third which seemed to help. CLIPPERS symptoms have remained absent and I've now been taking Azathioprine for ten years which is not ideal but probably better than the alternative.

I very recently had my annual neurology appointment in London. It was strange travelling in to the centre on the train for only the third or fourth time in the last couple of years when I used to do the journey most weekdays. My status was reviewed by the same neurologist I saw when I was first diagnosed and I was judged to be stable. There's always the question about whether I want to stop the medication and I ask "what are the risks of relapse and what would be the consequences if I came off it" and no-one really knows; so I stay on the medication as it's kept me well so far.  I'll get another MRI in due course to provide an up-to-date reference in case there are any changes further down the line.

Hopefully it won't be so long before the next post. I hope everyone out there is staying as well as possible.

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

CLIPPERS diagnosis criteria revisited

Autumn mushrooms

The Mayo team have been busy as another interesting paper has appeared which really emphasises the continuing problems of diagnosis in CLIPPERS.

In the paper they perform a detailed comparison of 35 patients who had symptoms suggestive of CLIPPERS. (This blog gets a credit in the text as a patient advocacy site which helped connect some of the subjects with the Mayo). After re-assessment including detailed consideration of imaging and response to steroids, CLIPPERS was confirmed in 23 of the patients. One of the most striking results is that when the differences between the CLIPPERS and non-CLIPPERS groups were analysed, there were no significant differences in terms of symptoms commonly associated with CLIPPERS (e.g. gait ataxia, diplopia, dysarthria etc) or in terms of pre-existing cancers or smoking status. 

There is a lot of detail on the cases here which leads the authors to suggest a distinction between CLIPPERS cases: "probable" CLIPPERS for patients who fit all criteria but didn't have brain tissue biopsy and "definite" CLIPPERS for patients who fit all criteria but also had brain tissue biopsy with supportive findings. Unlike the paper from Dr Taieb's group I talked about in June, there is no focus here on the relapsing nature of the disease.

A revised set of diagnostic criteria is presented which includes some statements on neuropathology (i.e. tissue analysis). Interestingly both the clinical presentation and neuropathological criteria include the requirement "no better explanation" which emphasises that CLIPPERS is still regarded as somewhat of a "last resort" diagnosis.

On a slightly more optimistic note, I am now over 6 years since being discharged from hospital with a bag full of drugs, double vision and problems walking straight. The diagnosis was "probable" CLIPPERS and the outlook was distinctly uncertain. The outlook is still not exactly clear but everything else is pretty good.

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

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.

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

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.

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

2016

The Round Table (allegedly) in Winchester

Just a quick note to wish everyone who follows this site - or even just visits occasionally - a happy and prosperous 2016. I will endeavour to keep you all updated with any news about CLIPPERS I hear about. As always, if anyone has anything they want to share then please get in touch. Please note that I am changing my preferred email address for the blog to wrcrum@hotmail.com but entries to the old one should still get through.

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A Brazillian CLIPPERS case

The Peak District in September

To date, the majority of reported CLIPPERS cases have been from the United States, Europe and Australia. As awareness of the condition spreads, cases are being reported from other parts of the world. Recently the first Brazillian case report has been published where a 49 year-old first presented with CLIPPERS-like symptoms in 2001; of course CLIPPERS wasn't first published in a scientific journal until 2010. 

Interestingly, straight away this patient received pulse-steroid treatment which is now fairly standard when CLIPPERS is suspected; his symptoms improved. Unfortunately, Multiple Sclerosis was suspected and so other drug treatments which were not effective were subsequently used. In 2014, after significant deterioration, treatment for CLIPPERS was begun (Prednisolone and Azathioprine). There was then marked improvement of the lesion appearance in MRI but much less clinical (i.e. patient) improvement.

Although this is only a single case, it adds to the evidence that early diagnosis and treatment is important in CLIPPERS, as there may be progressive damage over time which cannot be completely reversed if treatment is delayed. I began steroid  treatment 3 months after I had my first symptoms which I think was quite fast. In my case my first symptom was diplopia and I was lucky to be spotted by someone in an eye clinic (once I'd been back a couple of times) who was worried about my worsening symptoms and referred me quickly to a neuro-opthalmology clinic. From there, I was lucky to be seen by a neurologist who was also sufficiently worried - especially when I failed all the balance tests - to get me admitted quickly to a neurology ward for in-patient investigation. It still took a month in hospital (in 2011) before they realised CLIPPERS was the likely culprit and began treatment. I think given that CLIPPERS was not widely recognised in 2011, this was still very fast and I am profoundly grateful to the people who pushed me rapidly through the healthcare system.

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

More blood.

Filled tubes, 9 of them.

So, following on from last time, I finally managed to get my blood sample collected for the Mayo CLIPPERS study. In fact, I had no problem filling all 9 tubes - I have good veins apparently. Hopefully it survived the trip over the Atlantic with FedEx and will contribute in some small way to helping understand more about CLIPPERS. 

Two warts merged into one.

As of the beginning of June, I am over four years since first symptoms and still taking Azathioprine with few side-effects. Apart from occasional blips in liver scores, I have a few warts (7ish) which is apparently quite common on Azathioprine. They are confined to fingers with a few periungual ones for good luck. Not very pleasant, but could be worse - I had them blasted with cryotherapy recently which will hopefully help. That's enough about warts.

Finally, I'm not sure how long this has been live, but CLIPPERS now has a short entry on Radiopaedia (think of Wikipedia, but for doctors)

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

CLIPPERS 2015

A "Happy" meal I had over Christmas

A belated Happy New Year to everyone out there. January 2015 marks the 3-year "anniversary" of when my CLIPPERS symptoms retreated far enough for me to function more or less normally. At the time I was still on tapering Prednisolone and attempting to ramp up Azathioprine while dealing with a few wobbles in various blood tests along the way. I remember book-keeping the changing doses of various drugs needed a certain amount of organisation. There were worries about long-term prospects in 2012 (there still are!) but I have been lucky to be stable on Azathioprine for some time now. 

Although Azathioprine is a common CLIPPERS treatment in conjunction with steroids, I don't recall any cases being reported who have remained stable for this long after the complete removal of steroids. It would be interesting to know if anyone out there is being treated similarly. Am I simply lucky, an anomaly or living on borrowed time? I still occasionally try walking "heel-to-toe", standing on one leg with/without eyes shut etc to try and pick up any early signs of recurring problems. It will be interesting if, as part of their CLIPPERS study, the Mayo team have any comments on my case compared with others.

Finally, a reminder that for those interested in events which raise the profile of rare conditions, the annual Rare Disease Day is coming in February (28th to be precise).

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

News Update

About 3 weeks ago, I had my first brain-scan of 2014, this time at the Institute of Neurology in London. I did ask my neurologist whether they were going to try anything different, as the IoN scanners are more research-oriented, but he told me they were just spreading the load of clinical imaging cases. It's funny, that having scans less often is in some ways more stressful because you wonder what might have been going on in the interim. Anyway, I have now had the unofficial feedback on the scans which was that:

"Everything is good. There was a tiny bit of signal change in the pons, as before and no enhancement. No change from last year."

So this is good news, especially as I have now been off Prednisolone for two years. Sounds like a bit of residual CLIPPERS-related damage which I will have to put up with, but I can't associate that with anything specific in day-to-day life.

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

CLIPPERS Christmas 2013

To finish off 2013, two CLIPPERS cases are reported in a single paper which focuses on movement disorders. In the first case, the problem was limb tremor and mild dystonia. In the second case, the problem was hand and facial jerks, which manifested two years after CLIPPERS was diagnosed. The authors suggest that movement disorders, although not "classic" CLIPPERS symptoms, may be present because of the lesion location in particular cases. They point out that, in such cases, specialised treatment for the specific movment disorder may be warranted. (Incidentally, I'm not sure if this paper is freely available, but if anyone is interested and has problems accessing it, they can click through to the author information on the journal web-site and try emailing them for a "pre-print".)

Interestingly, I developed some intermittent stiffness and jerking movements in my legs after I began steroid treatment in 2011, but these gradually went away. They were definitely coincident with the start of treatment, but I've no idea why they should have appeared. My (no doubt completely wrong) theory was that as the brain inflammation improved slowly in response to steroids there was a period where some affected bits of brain were only partly functional?

Anyway, many thanks as ever to friends old and new who follow this site, and I wish you a happy and healthy 2014.

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NEWS FLASH: CLIPPER climbs Snowdon

Half-way up, before the cloud came down.

Two years ago this week I was discharged from hospital with unsteady (but improving) gait, coordination problems, double vision and a bag full of Prednisolone. I also had some new symptoms of occasional jerking and rigid limbs, and stalling of speech. Today I walked up Snowdon, the highest mountain in Wales, in what turned out to be fairly atrocious conditions. It still feels like a birthday though.

Everything could still turn 180 degrees in the future, but things are stable for now. I want to thank everyone who reads the blog for making it worthwhile and in particular those who have contacted me personally over the last couple of years. I know it's been a bit quiet recently, but unfortunately there just hasn't been much CLIPPERS news to report. If you know differently, let me know, or feel free to post something in the forum. 

Best Wishes,

 -Bill

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