Marfan’s Syndrome

Yes, we hadn’t heard of it either!  But it’s a killer!

Marfan’s syndrome is a genetic connective tissue disorder that can affect the eyes, heart, skeleton, and the body’s other soft tissues.  To imagine how dangerous it can be, one area it can affect is the aorta, the main artery straight out of your heart.  Marfan’s can weaken the walls of this artery with the result that it just tears open.  As you can imagine, the results are most often fatal.

It’s as common as cystic fibrosis, affecting an estimated 1 in 5000 people; yet few people know about it; including doctors who typically do not test or screen for Marfan’s.

Simon Taylor, who worked for many, many years at the beer festival suffered from Marfan’s Syndrome.  And he never even knew it.  Nor did his parents, his friends, or his family.  He died suddenly towards the end of 2010, and it was only the post mortem that revealed Marfan’s.

Could you have it?

Signs worth worrying about include tall, thin builds, slender limbs with long fingers and toes or thin, weak wrists.  Think Nicholas Lyndhurst or Peter Crouch and you’re on the right track.  Undue fatigue or breathlessness are signs, as are cold arms, hands and feet (due to reduced blood circulation).

Is it something you should worry about?

We’re not doctors, although some might refer to us as beer doctors, so we can’t give you an answer on that one.  However it’s definitely worth a few minutes of your time to read a little about it.  The Marfan Association have a wealth of info on their site, and if you want a more scientific or technical explanation Wikipedia have a page about Marfan’s.

In honour of Simon Taylor, who worked for many, many years at the beer festival, The Bent and Bongs Charitable Trust has selected the Marfan’s Assocation as one of the main beneficiaries of the 2012 festival.  We hope you’ll help by spreading the word about Marfan’s wherever possible, as knowing about Marfan’s is the first step towards beating it.  You could also sponsor the festival, or just turn up for a few pints of fantastic beer.

The photo below shows Claire (center) and John (Simon’s father) presenting a cheque from the Bent and Bongs Charitable Trust to Beryl (left) from the Marfan’s association.  We fully hope that in 2012 we’ll be making another cheque presentation to the Marfan’s Association; next time for a significantly larger amount.