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.

Tuesday, 26 April 2016

Unwanted Dips

Spring in my parent's back garden
I recently got called back by my local docs as my total white blood cell count has dipped below my usual acceptably low level of 3-and-a-bit. Apparently in an ideal world it would be above 4 and in my world it usually hovers around 3.2 or a little higher or lower. In March it was 2.6 beating my previous record of 2.8 a couple of years ago. At this point there was apparently some head-scratching behind the scenes as usually, at least at my practice, a level this low would often trigger a change or cessation of the medication causing it to dip - in my case Azathioprine. I have now been taking 100mg Azathioprine twice a day for about 4 years and not had too many problems on it to date. 

My neurologist has advised I remain on my current level of medication and have full blood counts every 3 weeks. I think one issue is that no-one knows the correct levels of medications for CLIPPERS except in general terms and the only thing they know about me is that I have been essentially symptom-free while taking Azathioprine. (Of course this doesn't prove that Azathioprine is the *reason* I have been symptom-free). So I think all concerned are a bit loathe to mess with the meds in case it triggers something worse. Having said that I have been told to be very watchful of illnesses which feature high temperatures / fevers and get myself down to the surgery ASAP if one appears. I'm having some more blood taken tomorrow, so in a few days should know if this is a blip or a trend. It's worth mentioning that I had no reason to think there were any problems so although these blood tests are a bit tedious after five years  they are well worth keeping up with as things can and do change.

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.

Sunday, 20 March 2016

Diagnosing CLIPPERS Using MRI is Tricky


Spring is in the air, as evidenced by this recent visitor who has successfully found a way through the garden fence and is getting bigger.

Contrast-enhanced MRI of my brain in 2011.
The bright spots in the middle indicate CLIPPERS-related damage.
One of the hallmarks of CLIPPERS is the characteristic pattern of enhancing lesions many of you will have seen in MRI. It is tempting to regard the presence of a pattern like this as sufficient evidence to diagnose CLIPPERS, particularly when there is no clear alternative. Unfortunately, life is rarely that simple and there may be other reasons why patterns of lesions like this can develop.

Dr Taieb and colleagues have helpfully written a guide to diagnosis in these cases. Their paper, Punctate and curvilinear gadolinium enhancing lesions in the brain: a practical approach, examines 39 cases of their own where similar patterns of brain lesions occur, together with other reported cases, and details the many different problems which could be the cause. (Unfortunately, this paper isn't freely available to read from Springer - you can try asking Dr Taieb for a pre-print.)


This (above) is perhaps the most interesting part, a diagnostic flow-chart for when lesions are present. Interestingly Dr Taieb suggests brain-biopsy only as a last resort. Fitting CLIPPERS into a standard diagnostic process is clearly important. This paper may not be the last word on the subject but is a step in the right direction.

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.

Sunday, 21 February 2016

Rare Disease Day 2016


In just over a week it is Rare Disease Day 2016. "The main objective of Rare Disease Day is to raise awareness amongst the general public and decision-makers about rare diseases and their impact on patients’ lives." This can only be a good thing, although I must admit to being a bit out of my comfort zone with these mass movement things. However, they have an important role to play, especially for conditions as rare as CLIPPERS. 

Rare Diseases UK have information about the scale of the problem where I am located - but the numbers translate more or less to most other places. The most important relationship is that although there may be very few sufferers of any particular rare condition (even down to a handful), the number of identified rare conditions is so large (around 7000) that (in the UK) 7% of the population will be affected by a rare condition at some point in their lives.

CLIPPERS seems to be a rare condition amongst rare conditions at the moment. Four-fifths of rare diseases have a genetic component and there is no evidence of that (so far) in CLIPPERS. Three-quarters of rare diseases affect children and although there are a few reports of CLIPPERS in children, most reported cases seem to be in adults, with some hints that it is more prevalent when heading towards middle age. According to Rare Diseases UK, a rare condition may affect up to 30,000 people in the UK, but I doubt there are more than 20 diagnosed cases of CLIPPERS here at the moment. Please prove me wrong on this if you know different and I'll report back here.

So, like it or not, to be diagnosed with CLIPPERS is to be a member of a small and exclusive club, and unless CLIPPERS turns out to a be a "missing link" which unlocks the mysteries of other, better-known, conditions, then it is cross-community campaigns like Rare Disease Day which can do the most to push the agenda of CLIPPERS as a rare disease in the wider world.

P.S. I received a nice graphic - posted below - from Cameron Von St. James, a mesothelioma cancer caregiver and advocate. Cameron works with the Mesothelioma Cancer Alliance to spread awareness of another rare disease, Mesothelioma, caused by exposure to asbestos, as well as all rare diseases.

Image provided by www.mesothelioma.com.


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.