Monday, 2 February 2015


Growing up, I lived on a housing estate called Walton Court in Aylesbury, Bucks. Walton Court was born in the 1970s as part of a major housing expansion. The land had formerly been farmland, and for some time the privately owned housing area was known as Walton Court Farm, after the estate.

With the new expansion of what some might call a red brick and concrete jungle came neighbours, young families from all over the United Kingdom, all on the social housing ladder. The Hywoods (my family) lived next door to the Potts family and we all got on extremely well, just like one big family actually. Caroline was the middle child in the Potts family and despite growing up and growing apart, location wise, we are all still very good friends. Caroline has always been an advocate of my ability to raise awareness about disability and is very vocal to support myself and others. However, Caroline also thinks that I do not tell people about myself and my condition enough and all the hardship that comes with it. In fact I told Caroline I was writing this, a blog about living with Limb Girdle Muscular Dystrophy and my determination and she said “Tell everyone what you go through, for once! No holds barred… Don’t play this down and please be completely honest and I know I’ll love it and I think people will too... Tell them, tell them everything."
I took all this into account and the fact that I still have very good friends asking "What is Limb girdle muscular dystrophy?"

So, here goes, I’ll start with the facts…

Limb-girdle muscular dystrophy (LGMD) is a genetically and clinically heterogeneous group of rare muscular dystrophies. It is characterised by progressive muscle wasting which affects predominantly hip and shoulder muscles.

LGMD has an autosomal pattern of inheritance and currently has no known cure.

The term limb-girdle is used to describe these disorders because the muscles most severely affected are generally those of the hips and shoulders—the limb girdle muscles.

Symptoms of limb-girdle muscular dystrophy vary widely, but most commonly are muscle weakness and atrophy, myoglobinuria, myotonia, elevated serum CK, and, in ~20% of cases, cardiomyopathy.

The disease inevitably gets worse over time, although progression is more rapid in some patients than others. The disease commonly leads to dependence on a wheelchair within twenty to thirty years of symptom onset, but there is high inter-patient variability, with some patients maintaining mobility.

The muscle weakness is generally symmetric, proximal, and slowly progressive.

LGMD can begin in childhood, adolescence, young adulthood or even later. The age of onset is usually between 10 and 30. Both genders are affected equally. When limb-girdle muscular dystrophy begins in childhood the progression appears to be faster and the disease more disabling. When the disorder begins in adolescence or adulthood the disease is generally not as severe and progresses more slowly.
In most cases, pain is not present with LGMD, and mental function is not affected.

Symptoms – a person with LGMD generally has difficulty walking, going both up and down stairs and raising from a chair or a squatting position. Difficulty bending over and falling on a regular basis are also common. Difficulty lifting certain objects is also a common presentation of LGMD as well as difficulty holding your arms out or above your head. Eventually the ability to walk/run deteriorates. It is advised that someone with the disorder not put themselves in situations of potential peril. For example, walking on floors that have even the slightest incline can cause the individual to lose balance and fall. It is not recommended for someone with the disease to walk on terrain which is not stable such as ice or other slippery surfaces to avoid a severe injury. This is mainly due to the fact that LGMD weakens the leg muscles so an individual with LGMD would be more inclined to fall than someone who does not suffer from the disorder. At times, heart palpitation can occur.

So that’s LGMD from a factual perspective and that of wikipedia, what about the patient, those affected including partners? Some might say the harsh reality.

Despite the facts saying that a sufferer of LGMD would hardly ever feel pain from the condition, I’ll beg to differ. I push myself a lot to keep on top of this and to try to stay as mobile as I can for as long as I can. With that in mind imagine going to your gym and working your thigh muscles (Quadriceps Femoris) to the point of a severe burning sensation, well that’s exactly what LGMD feels like constantly due to the lack of hamstring (a group of three muscles called biceps femoris, semitendinosus, semimembranosus) that has deteriorated and is now no longer effectively working, therefore being compensated by the thighs. The same can be said for the neck muscles (trapezius muscles); it’s a constant pain that feels like a burning or needling sensation.

There are other inconveniences too that are not mentioned. A shortness of breath, constantly unsteady on my feet with no confidence out and about and around other people and just the feeling of wanting to stop or at least slowdown in so many respects.

Then there’s the personal stuff. I’ve never lifted my youngest daughter, Skye (4) up. In fact both our youngest daughters have always been passed to me. I’ve never carried my girls on my shoulders to walk down the promenade or the beach. I cannot run, hardly walk, and most playful physical interaction that most parents have is non-existent. It always has been and this is what hurts me most about this condition. Sometimes the best hug in the world can hurt but I never show it, why on earth would I show my three girls or indeed my wife that their love and affection actually, physically hurts me? All this and the fact my older brother has the LGMD too, I see how it advances, I know all too well what to expect.

Recently I fell down the stairs at home, from the top to the bottom. I bruised my ribs, twisted my right knee (common occurrence) and also twisted my ankle. I believe this was due to my LGMD. A little bit of bad luck. It wasn’t even one week later and I had my car stolen and a local newspaper ran a story which was entitled ‘Car theft was like stealing my legs’. This may very well sound melodramatic to someone who knows nothing of this condition but in actual fact this was very true.

As I have said, I am an ambulant disabled man, I consider myself severely disabled yet I am still able to walk (just!). I surprise my specialist neurologist every visit I have to the John Radcliffe hospital in Oxford and most days I surprise myself too.

So what can I do about this? Not much really, as mentioned there is no cure so what’s the point in trying or to keep going. Well now you’ve heard all that let me tell you…

I believe that research will soon come up with treatments for these conditions (Muscular Dystrophies), this is all my own personal feelings and I need to help fund that research if not for me then for the newly diagnosed. I am 41 years old and considering my LGMD I feel very lucky for all that I have and also all that I have done in my life. In some circumstances I consider myself lucky to have this diagnosis, it is a great platform for me to help others and that sensation is rewarding beyond words.

The awareness I raise as well as the funds raised in the events I take part in may well help a family 10 years down the line and to me, that’s more than imperative. I would never want anyone to fall down the stairs or anything worse due to LGMD.

As well as my wife and my girls affected by this condition I also have friends within the muscular dystrophy community that I like to raise awareness for too but last year some news absolutely stunned me and really knocked me sideways. I got a message from a friend and he told me how his son had been diagnosed with Spinal MuscularAtrophy (SMA) and within days I got another message from an ex-colleague whose son was diagnosed with DuchenneMuscular Dystrophy (DMD).

This was recently after I’d just finished my ‘Mile For Muscles’ and after minutes I tried to think of a way to help, a way to tell them that they was not alone and that they had a friend who had a little bit of an understanding of the conditions and what they might be going through.

There was only one thing for it… Only hours after my fundraising retirement I had to step back up to the plate. I want to do something bigger and inclusive so people could get the incredible feeling that I had. I still find it hard to put into words what finishing my charity event was like, as mentioned above, it is beyond words.

Ever since I was a child I wanted to take part in a marathon which is now so unrealistic that it doesn’t bear thinking about. So I decided to create my own marathon with my own rules and my mates are coming to help too! On the 30th August 2015 we will take part in a 26.2 mile relay. With a relay baton changing hands every mile and between two natural cheer points a group of family and friends will do everything in our power to help others and you can take part too. Then will be the very last mile and that will be mine. I promise that by any means possible I will cover the finishing mile and a life time’s ambition will be fulfilled all in the name of a great cause for great people. Get in touch on the facebook page HERE to say ‘Count Me In!’

I am very determined to keep using my stairs, albeit with more caution than ever and a message to those who recently stole my independence, I have more determination and courage than you’ll ever know or indeed probably ever have yourself.

As for Caroline ‘pottsy’ Cunliffe, I hope you’re happy and approve of this, my latest article.

I told them, I told them everything. x

Myself, Caroline, my wife,Michelle and Carolines brother, David


Follow me on twitter @HywoodMartin

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Mile For Muscles - Count Me In

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