Wednesday 4 October 2023

A Book on CLIPPERS promotion


Just a quick update that the ebook edition of "A Book on CLPPERS" will be free worldwide on Amazon from tomorrow (Thursday 5th October 2023) for 5 days.

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.
A Book on CLIPPERS is now available.

Saturday 24 June 2023

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.

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 11 February 2023

Systematic review of CLIPPERS studies


Wind-battered trees on Brean Down

One of the difficulties in studying conditions like CLIPPERS is their rarity meaning that there are comparatively few cases to draw from. CLIPPERS, with it's variability of symptoms and outcomes and difficulties in concrete diagnosis has additional challenges. However, now that there have been over ten years of scientific publications on CLIPPERS there are opportunities to get a better overview by combining together earlier work. Dr Al-Chalabi and colleagues from the University of Toeldo published a paper in 2022  titled "Clinical characteristics, management, and outcomes of CLIPPERS: A comprehensive systematic review of 140 patients from 100 studies" to try and achieve this. A systematic review is where a collection of previous works are combined following a protocol which strives to ensure quality and minimize bias to hopefully come to stronger conclusions than might be possible looking at the individual studies. In the case of this review, the "clinical characteristics, treatment strategies and outcomes" of CLIPPERS were assessed.

My reading of the review is that the authors drew together some common themes from the individual studies but that the amount of variability and uncertainty still hindered their ability to draw firm conclusions, even in over 100 nominal CLIPPERS patients. They found 60% of their cohort were male and the mean age of onset was 46 years (which is very close to the age I first got symptoms). The most common (but not the only) symptoms, in order, were ataxia , diplopia, and dysarthria; my own experience was diplopia first, then quite quickly followed by ataxia, and latterly by some dysarthria just as I began treatment. They also found that around 15% of the patients studies had some form of malignancy which presumably means CLIPPERS wasn't their ultimate diagnosis (but I am not a doctor). In terms of long-term treatment, Azathioprine and Methotrexate, were the most common, but not the only, drugs. 

The authors also report that a shorter time on steroids was associated with an increased risk of CLIPPERS relapse which, to me, is the most interesting of their conclusions, possibly, and very unscientifically and with no evidence, because I have long had a gut feeling that longer steroid tapers might be better. The authors suggest that, going forward, steroid tapers should be very slow, although they don't state what "slow" means - presumably months?

These studies are important and as well as making the most of previous studies can hopefully direct future research.

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.

Friday 13 January 2023

A Book on CLIPPERS : The Paperback


The paperback version of the CLIPPERS book is now available* as per the links on the last post and in the A Book on CLIPPERS tab on the main Living With CLIPPERS website.

*for some reason the Amazon India marketplace does not offer printed books via Kindle Direct Publishing

Read other articles in this series at Living With CLIPPERS.

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