First Birthday!

Happy Birthday To Us

Please excuse the slightly length entry - I think I can afford to be a little more verbose than usual to mark a year of blogging.

How It All Began

A year ago this week I was at home, very slowly improving, after nearly 5 weeks in hospital which culminated with a CLIPPERS diagnosis. A combination of high-dose steroids and cabin-fever from being at hospital and home meant I could talk about very little else than CLIPPERS (and boy could I talk). There were also a fair few people who wanted to know what was happening and despite my babbling I was getting bored telling them the same story and answering the same questions. So in an effort to save my marriage (by reducing the babbling burden on my lovely wife) and my sanity (by putting down a definitive account of my illness) LIVINGWITHCLIPPERS was born.

At the time I had no idea if I would persevere with it, not least as the diagnostic uncertainty meant there was a small but finite chance I could be dead by Christmas 2011. However I did gradually get better and the emphasis changed from just focussing on my own problems to providing a digest of what CLIPPERS news I could glean from the web.

Beginnings of a Community

Number of readers of livingwithclippers on a monthly basis

The readership has naturally ebbed and flowed over time and was initially quite high which I attribute to friends and family finding out what the hell had been going on! (When I say "high" I mean up to 20 visits per day - Rupert Murdoch has little competition from me although he has his own problems to deal with.) After Christmas 2011 the visits declined a little but in recent months there has been much more activity. I think there has been a shift towards people who don't know me personally but have some other interest in CLIPPERS for personal (or maybe even professional) reasons. I have also started to receive emails from fellow sufferers (what are we - CLIPPERS?, CLIPPYS?) from Europe, America and Australia.

Whatever their reasons for visiting, the readers of livingwithclippers are a global community as you can see from the map. (No recorded visits from Greenland yet, but I'm working on that.) And let's be quite clear that you have to want to come here. If you just search for CLIPPERS via Google you are highly likely to find other CLIPPERS before you get here: e.g. a popular basketball team, haircare products, boats, tea-bags etc etc Whatever your reasons for visiting, it's seeing the visits rack up week on week which keeps the blog going - there's nothing worse, at least in blog-land, than feeling you're posting straight to a black hole.

The Future

If you're living with CLIPPERS the future is uncertain but there have been some hints in the last year that although the disease is chronic it can often be managed and suppressed, at least in the short-to-medium term. I plan to keep the blog going while there is demand and I have something (hopefully) interesting to say. Maybe a more official support forum will appear at some point but for now you're stuck with my ramblings! But it doesn't all have to come from me. If you have some CLIPPERS news, or a CLIPPERS experience you want to share then send me an email and I'm more than happy to put some guest posts up here. Anyway, thanks for reading and hope to see you next year.

Brecon Beacons, South Wales, Summer 2012

 

 

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.

September Sun

A CLIPPER on Holiday

Wales

I've been enjoying the last of the Summer sun in South Wales. The day after we arrived we climbed Corn Du and Pen Y Fan in the Brecon Beacons National Park. I looked at my watch and noticed it was exactly one year to the day since I was discharged from the NHNN with a bag full of pills and a diagnosis of presumptive CLIPPERS. My balance had improved after 5 days of IV steroids but was still not great. I also still had double vision and some strange intermittent jerkiness when walking or talking. A fuller account of those times is here. Now I have a smaller bag of pills and the various symptoms are pretty much resolved. From what I can gather I've had a good result from treatment but this is largely down to the luck of being in the right place at the right time and getting a diagnosis relatively quickly. CLIPPERS is tricky to identify and other things need to be excluded, but equally, it doesn't seem to get better by itself and needs treating. I was definitely in slow decline before and during my time in hospital but the pills are working for now.

CLIPPERS News

The debate about possible mechanisms for CLIPPERS continues. A short response to the paper by Dr Ortega (describing CLIPPERS as a complication of Multiple Sclerosis) has recently appeared. I don't pretend to understand the detail of the argument but more discussion about possible causes can only be a good thing and may eventually lead to firmer diagnostic criterion.

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.

Blood and Bone

My regular blood-testing arm.
I stopped counting how many needles have been stuck in this bad boy a long time ago.

Blood

So the saga of the blood-tests continues. People like me who are on long-term Azathioprine have to have regular blood tests to make sure the "cure" isn't worse than the disease. I've had the occasional blip in a liver enzyme which has settled down for now fortunately. I was hoping to move back to three-monthly tests but a couple of other things to keep an eye on so another panel of tests in a month. Better safe than sorry though. Still taking 100mg twice a day which I think is about as high as it gets.

Bone

I also mentioned in passing that I had some pain in my joints ("Arthralgia" - you learn something every day!), most noticeably in my knees, so I promptly got booked in for some x-rays to check for early osteoarthritis. One or two people on the interweb mention joint/muscle pain in the same breath as Azathioprine, but as I've noted in this blog before, the problem with the interweb is you can usually find someone with any combination of symptoms and possible causes you can think of!

Other than that, pleased to be ticking along. Almost exactly a year ago I was  being wired up for my first 1000mg intravenous dose of Prednisolone after three weeks of investigation in the NHNN and hoping it would do some good. Fortunately it did.

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.

Being a smart aleck?

smart aleck = someone who is pretentious about their own cleverness

Just For the Record

A couple of months ago I posted an entry here moaning about a CLIPPERS case study which I felt confused cause and effect and made far too much of a supposed link between CLIPPERS and flu vaccination. Anyway I felt strongly enough to write a letter to the journal in question to point out the weaknesses in the paper. Now letters to journals are sent for external review before publication which took a while, but I thought it a good sign that they didn't immediately reject it. In the end they waited for a review to come back ... and then rejected it, which is of course their privilege. As I put several hours work into this letter and because I think I made some legitimate points I've put a copy below for posterity. In the interests of balance, I've also included the reviewer's comments so you can see why they rejected it and my subsequent email to the editor of the journal saying why I thought the reviewer was wrong too. If you work in academia you frequently think reviewers are wrong and sometimes they are, but often it's just  you being a smart aleck ... ;-)

Regarding ”Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids Following Influenza Vaccination” by Hillesheim et al

To the Editor. – The article titled ”Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids Following Influenza Vaccination” by Paul Hillesheim et al (1) reports a new case of CLIPPERS, a recently characterised inflammatory condition of the central nervous system (2). This is an interesting case which adds to the description of CLIPPERS-like disorders but raises questions about the definition and diagnosis of CLIPPERS. The authors also make an unjustified association between CLIPPERS and recent flu vaccination which is not supported by the available data and is potentially misleading.

This case is somewhat atypical; in 19 reported cases to date only 3 others did not suffer diplopia. In addition, the present case does not exhibit the distributed pattern of enhancing punctuate lesions seen previously but has a larger focal lesion within the central pons. The current case is also at the upper end of the reported age range – only one older case has been described, with 13 out of 19 cases aged between 45 and 75 (Figure 1). Clearly though, this case is CLIPPERS-like and could simply represent population variation in a condition which is currently poorly understood.

Figure 1

The connection with influenza vaccination warrants further investigation. Dr Hillesheim associates vaccination with CLIPPERS in the article title but I am of the strong belief that this is misleading and unwarranted. The authors make several statements which are technically true but not defendable in this context. For instance, “… this is the first reported case of CLIPPERS after vaccination” and “vaccination … raises the tantalizing possibility that this may be the triggering event”.  While it is strictly true that the patient was vaccinated shortly before symptom onset, no evidence for a linkage with CLIPPERS is presented and no mechanism is suggested. It is quite right that the vaccination is recorded, but its significance cannot be established from this single case. Finally, the authors list existing neurologic complications of vaccination and state “CLIPPERS is another complication that should be added to this list”. There is no evidence to support this assertion and indeed no other reported CLIPPERS cases have mentioned vaccination. Therefore it is premature to make this connection on the basis of a single case and to link CLIPPERS with vaccination in the article title.

I should declare an interest; I was diagnosed with CLIPPERS in September 2011 and am currently well on tapering Prednisolone and Azathioprine. I am not one of the literature cases. The first and only flu vaccination I received was in November 2011.

1. Hillesheim PB, Parker JR, Parker JC, Jr., Escott E, Berger JR. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids following influenza vaccination. Arch Pathol Lab Med. 2012;136(6):681-5. doi: 10.5858/arpa.2011-0428-CR.

2. Pittock SJ, Debruyne J, Krecke KN, Giannini C, van den Ameele J, De Herdt V, et al. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Brain. 2010;133:2626-34. doi: 10.1093/brain/awq164.

Comments for the Author

The author does make a valid point in that it cannot be assumed that influenza vaccination directly leads to CLIPPERS; however, I do not believe that Hillesheim et al are implying an absolute cause and effect. They are merely reporting a case of a patient who developed CLIPPERS after receiving the vaccination. With this being such a rare disease with no definitive undeniable cause/trigger or mechanism only associations can be made between the disease and its possible causes, which is what the article is doing, similar to the other reports of CLIPPERS in the literature, until enough cases are reported to find investigable commonalities.

My Response

I still believe Dr Hillesheim over-emphasises the vaccination aspect in his original paper but accept this is a matter of opinion. However as noted in my letter, his statement that CLIPPERS "is another complication ..." (of influenza vaccination) "... which should be added to this list" surely goes too far; however the reviewer disagrees and so  I must defer.

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.