One of primary purposes of this blog is to document the changes in my condition. l. have Facioscapulohumeral muscular dystrophy (FSH), the 3rd most common form of the disease. The name describes the usual distribution of weakened muscles: facio=facial; scapulo=shoulder blade; humeral= upper arm It is estimated that between 1,200 to 3,000 have the condition in the UK. Now hang on for the sciencey, medical bit and you will know more about FSH than 9.999% of said population and more than most doctors. I quote from the Muscular Dystrophy Campaign website.
FSH is a muscle wasting condition that
affects both men and women. The
condition is caused by a deletion (loss) of
a piece of genetic material (DNA)
towards the end of chromosome 4. This area
contains a number of repeated units
of DNA. Normal individuals have between
10 and 100 of these repeats whereas
those with FSH have less that nine. In
general, the fewer the number of repeats,
the more severe the condition.
I have just 2 repeats. As it was explained to me – I'm a sucker for a metaphor – imagine a train. You, dear reader, are like one of those trains you see in movies set in the Mid-West of America, a seemingly endless string of carriages, wagons and boxcars trundling through the prairie. I on the other hand am the equivalent of Thomas the tank engine pulling Annie and Clarabell along his branch line. In short, I've been short changed by the Fat Controller.
The degree of muscle weakness varies
greatly from person to person - 10 to
20% of people eventually require a
wheelchair while up to 30% can remain unaware
of symptoms into old age. The
majority of people affected by FSH though will
present symptoms somewhere in
between these two points.
So on a scale of severity (where 1 is the most severe and 10 is where you are still skipping in your 90s) 1 come in at about 0.5. I tell you this not because I crave sympathy, I'm far too brave and uncomplaining for that, but to set myself in some kind of context for you. My experience of the condition is that it deteriorates in steps. You go along for a period of time with nothing much changing and then you have a brief period of fairly rapid deterioration before things level out again. At the moment I'm going through one of those stages of deterioration which is one of the reasons for this blog, to help me keep track of what's going on.
This week has seen a particularly significant marker for me. For the first time l needed to be hoisted off the loo. I got on the blooming thing but it took so long and so much effort that I simply couldn't get off it. Faced with being stuck 'from Monday to Saturday` I had to consent to being hoisted. So, humming 'He Flies Through the Air with the Greatest of Ease` I was lifted high and swung around towards a new stage in my life.
See you next time.