(mentions CMT): Muscular dystrophy care 'lottery'

[Guest guest]

Muscular dystrophy care 'lottery'

The diseases cause muscle weakness and wasting

http://news.bbc.co.uk/2/hi/health/7125437.stm

Some patients with neuromuscular diseases may die more than a decade

too soon because specialist services are too far away, a report

says. It found people with muscular dystrophy in north east England -

close to a centre of excellence - live an average of 30 years after

diagnosis.

However, in the south west the average was just 18 years.

The Muscular Dystrophy Campaign report found two-thirds of areas in

England do not offer the right kind of care.

The most common serious neuromuscular disease is Duchenne muscular

dystrophy - one in every 3,500 boys has it.

It is simply unacceptable that life expectancy for conditions such

as Duchenne muscular dystrophy can be halved, after diagnosis,

depending on your postcode

Other neuromuscular illnesses include Charcot-Marie-Tooth disease,

Becker muscular dystrophy and myasthenia gravis.

Neuromuscular diseases can lead to muscle weakness, wasting, and in

some cases eventually to serious breathing problems.

In recent years, techniques such as offering breathing support

machines at home have increased the average lifespan after diagnosis

for Duchenne muscular dystrophy from 19 years to 30 years.

But the report says this advance simply is not available in

a " systematic " way to patients in some parts of the country.

Patchy access

Muscular Dystrophy Campaign chief executive Philip Butcher

said: " People with rare illnesses are living longer thanks to

medical advances, and yet local NHS services are failing to meet

this demand.

It is simply unacceptable that life expectancy for conditions such

as Duchenne muscular dystrophy can be halved, after diagnosis,

depending on your postcode.

" It is essential that the Department of Health and the NHS recognise

the specialist nature of the care needed by patients with

neuromuscular diseases, and ensure that such services are available

to all patients, regardless of where they live. "

Report co-author and consultant neurologist Professor Mike Hanna

said that proper care was " essential " to keep patients with

neuromuscular diseases alive.

The report says that there are only four centres of excellence in

the country but that these are often dependent on just a few expert

members of staff, and reliant on charitable funding to keep running.

'A desert'

The report includes a survey of primary care trusts in England

conducted via Freedom of Information Act requests, which revealed

that two out of three did not support a " muscle clinic " at local

hospitals for patients.

One muscular dystrophy patient, Steve Ledbrook, described his local

region, the south west, as a " desert " for treatment.

" Expecting disabled people and their families to travel long

distances just to gain access to the clinical care they need is very

disappointing and simply unfair, " he said.

The report calls for the Department of Health to set guidelines for

the care of patients with neuromuscular diseases.

A Department of Health spokesperson said the National Service

Framework for Long-term Conditions, published in 2005, specifically

covered the needs of patients with neuromuscular disease.

" It is the responsibility of local health services to provide

services to meet the needs of people with Duchenne Muscular

Dystrophy and other musculo-skeletal conditions. "