This has been
written after reading through the fact sheets provided by the MDC . Please read the Muscular Dystrophy
Campaign Factsheets for more precise information. Link to their Factsheets
Written by Prof. KMD Bushby,
Professor of Neuromuscular Genetics, University of Newcastle upon Tyne, for the
Muscular Dystrophy Campaign.
Please note the fact sheets may have been moved
and so the link may not work if so search for “Becker” on their site and you
should find it .
Note
It is best to
compare my notes in this item with the Fact sheets it is not a replacement for these sheets it is my observations from my own
life.
Becker muscular dystrophy is a genetic disorder, which cause muscle
weakness first recognized in 1956 and is now known to be a much milder variant
of the better known Duchene type of muscular dystrophy. In this item I wish to
refer to my childhood memories and experiences which mirror a lot of what the
fact sheets say but not all as each case is different.
Symptoms
With BMD the average
age of diagnosis is
about 11 years but sometimes this may be well into adult life as the range may
vary a great deal
I was not actually diagnosed until about 1995 at the age of
around 36 !
The symptom usually begin early in childhood my only symptoms
were a tendency to fall over quite often and cramps. I learnt to walk as
quickly as any child would but in some cases a child may be late in learning to
walk
In my case my parents
noticed I walked on my toes a lot and this led to me having my Achilles tendon
lengthened on my left leg at the age of 7. I often got cramps and at around the
age of 9 I can remember being carried back to school from the sports field
because of severe cramp. A similar thing happened after going on a long slide
called the “Astra Glide” at a small amusement park in
At the age of 11 I
fell over during a cross country run and again severe cramp made me unable to straighten my legs –this was my
first ever cross country and had to be picked up by the games teacher and
driven back to school. I was never any good at Exercise, Games and running at
school but didn’t think much about it.
I was 21 before I
started to notice problems with stairs and a boss at work pushed me into seeing
a doctor. By this time I saw a big change in my muscle bulk I seemed to loose
95% of my outer thigh muscle on both legs
-I never noticed it till I saw I had no muscle there. This is in keeping
with the fact sheet which says that “some muscles can become weak and wasted, especially
certain muscles of the shoulders, upper arms and thighs” but in my case
this didn’t seem to be over a period of years but within a year , There is some wastage on my Shoulders and
upper arms I am a little skinny there but not enough to cause me any problems .Other muscles particularly the calf
muscles are often enlarged which is true in my case.
Typically with Becker
md you may become unable to walk in your 40’s or 50
and in some more rapidly progressive variants this may occur in your 20’s or
30’s.Many of our members are in their forties they manage to live normal
lives but mobility is much harder for
us. I am now almost 47 I can walk on the flat easily but I need a stick for
longer distances – I can walk over 2 ½ miles and be okay so maybe I can walk
4miles at most with a stick. This is
even walking uphill but it take me 5- 10 times longer to get uphill than for a
normal fit adult . I gradually built up the distance I
can walk over a period of a few years –my mother bought a dog and I gradually
built up the distance I walked. The dog sadly died about 1O years ago but I
kept up the walking I just rely more on the stick now and it takes me longer.
I do have difficulty
getting up from a chair it takes 3 steps,
1. Straighten and lock my
Knees-Usually by pressing my hands on one knee
2.
Straighten
and lock my Back ( Outside this means walking my hands up a wall or tree to do
this )
3.
Push
myself to 90°
and get my balance
I climb steps with
difficulty, narrow steps like in my mothers house are not too bad I push my
left leg into the corner between the steps and left wall .
I then pull my self up with the rail pushing my right foot into the right
corner of the step and drag my left foot up. I repeat this for each leg until I
get to the top of the stairs –going downstairs I go backwards reversing what I
did to get up. I keep a tight hold on the rail as I am scared of my leg giving in .
I am fortunate in
that weight was never a problem –I weigh 8- 8 ½ Stone ,
weigh can cause problems you are prone to overbalance more easily and do
yourself some damage.
I usually fall on average once every 3 months but
fall twice again that week and maybe the week after ( last week I fell over 4 times in 3days which
included being knocked over for the second time by a door at the local B&Q
DIY store, I fell over at work once and the other two were due to bending down too far
–Tim 27Th April 2006). I
try and be careful around furniture with square edges and if
possible buy furniture with rounded edges. A couple of years ago I nearly broke
my jaw twice and my nose once and gashed the corner of my mouth this made me
think again about my furniture and be very cautious. I am trying to get
everything sorted while I can, I own a bungalow so only a step to get in, furniture is bought
to suite my problems. I built a desk for
myself that was higher up than normal so I could use it stood up if needed, I
also bought a computer chair and raise it up as high as it would go - that
helped a lot.
Exercise
I try to keep as fit
and active as possible is important active exercise strengthens my normal
muscle fibres. . Regular daily exercise is better
than occasional sudden bouts of exertion (and the great majority are normal in
the early years of
Becker MD .
Some of our members (usually parents of children with Becker )
have told me they use ‘night splints’ (plastic splints to maintain a gentle
stretch of the calf muscles overnight) . At the Neuromuscular Centre many
patients some with Becker are treated with sessions of compression with
air-filled boots which seems to help with cramps.
I intend to keep
walking as long as possible –we have some members well into their 60’s and one
in his 70’s who only need the use of a stick and not all the time. A wheelchair
is likely to be needed in the later stages at least for getting about
independently over longer distances. There is also a great deal of other
equipment that may be useful to individuals at home and also to help at work
–See the access to work scheme .By careful choice of furniture and Bathroom
equipment etc certain tasks can be made.
Advice and help with
these matters is increasingly available and the Muscular Dystrophy Campaign
will be able to put you in touch with the best sources of advice.
School
“Most young men with
Becker MD leave school without having had any major muscle problems except that
they are usually slow at running in their teens and not very successful at PE
or games”
This is true in my
case the best position in cross country running was a magnificent last place -I
found at the age of 15 that I was able to walk round.
“In a few cases boys
with Becker md also have learning problems, usually
of a mild degree but sufficient sometimes to limit their academic success at
school. It is important to realize that this is not true of most affected boys”
I was certainly no
good at writing at school my writing is still not too good but I was good at
mathematics –I use Trigonometry often at work. I studied at college to get a
HND (Higher National Diploma) in computers, I didn’t bother continuing to go on
for a degree it was becoming too much of a hassle with all the extra work. I
have written a dozen or so programs at work which we use daily I developed them
over a few years. We are a PVCu window and door manufacture and one program
lines up a door panel with a midrail another calculates sizes for Bow windows.
We use some commercial software to process orders costing £10k -£40K with links
to saws but my programs work out things in seconds.
Employment
As I have said above I work fulltime in an office, this is a clearly
suitable job for me and I have done this work for 20 years not all at the same
firm.
Having Becker does
not prevent us working for a living It is true that our physical capabilities
are unlikely to improve and will eventually gradually decline so we need to
plan ahead. What is important though is as the sheet says “to work for the best
possible educational qualifications at school, to make good use of any
opportunities for further education and then either to plan a career that will
depend as little as possible on physical strength and mobility”.
In the
I hope to be a good
example of a disabled person who is a good worker; I am reliable and have had
no days off sick in the last 5 maybe 8 years. With the right job having Becker
does not impair you and you can be as good as the next person.
Please note in this article I can only speak for myself and
similar who are still walking as it is written from experience.
I hope this will be of help to everyone please also read our
members stories of “Living with Becker”.