The Muscular Dystrophy Campaign’s position is that non-invasive ventilation should be available for all those people with Duchenne muscular dystrophy who want and can benefit from it.
Written by the Research Department at the Muscular Dystrophy Campaign.
A paper by Michelle Eagle, Kate Bushby et al has shown that non-invasive nocturnal ventilation can have a significant effect on extending the life span of children with Duchenne muscular dystrophy.
The paper was published in November 2002. The paper audits 197 patients with Duchenne muscular dystrophy between 1967 and 2002 and charts whether the use of nocturnal ventilation had an impact on survival rates.
The paper shows that the mean age at death for people with DMD in the 1960s was 14.4 years but this has now increased to at least 25 years in the 1990s for those who have been ventilated. Overall, better-coordinated care has probably improved the chances of survival to 25 years from 0% in the 60s to 4% in the 70s and 12% in the 80s. Nocturnal ventilation has increased this possibility to 53% in the 1990s, and these figures are continuing to improve all the time.
The Muscular Dystrophy Campaign’s position is that non-invasive ventilation should be available for all those people with Duchenne muscular dystrophy who want and can benefit from it. (People who may be able to benefit are those who are having problems due to poor breathing overnight.)
There are however different approaches to treatment around the country and non-invasive ventilation may not be suitable for all people with Duchenne Muscular Dystrophy. You will need to discuss the potential of ventilation with your local consultant and do what is best for you.
Contents:
Why has the information on ventilation been published now (Nov 2002)?
Ventilation in neuromuscular diseases is nothing new, but it can take many years to see if any treatment or management has a significant effect. This publication has been possible because Newcastle has been collating data since the 1960s, and using home non-invasive ventilation in DMD since the late 1980s. This has enabled the team to track the results and to determine whether effects on longevity (as observed anecdotally in the UK and elsewhere for many years) were real .
Are the findings significant?
Yes. The results show that ventilation significantly improves the chances of a person with Duchenne Muscular dystrophy to live until their mid twenties. The results indicate that the mean life span has improved from 14.4 years in the 1960s to at least 25 years for those people who have been ventilated over the last decade.
Is ventilation the only reason for the life extension?
No, the general management of the condition has improved significantly, which has also helped. This includes things such as flu immunisation, increased physiotherapy and use of antibiotics for chest infections. However, without ventilation the mean survival was only 19.3 years.
Does this mean everyone with Duchenne muscular dystrophy will live to 25 years if ventilation is applied?
No, this is a mean figure. Some will live longer, some less. The presence of a significant cardiomyopathy is likely to reduce the likelihood of someone living to 25 as well.
Is it complicated to deliver?
Ventilation can be delivered in a number of different ways. The simplest is a facemask attached to a small machine. The mask is put on at night and helps the person breathe.
Why is it effective?
Muscular dystrophy weakens the muscles and as DMD progresses it weakens the lung muscles. This effect is especially marked at night, which is why using a ventilator overnight improves how people feel during the day. The muscles find it difficult to keep pumping overnight. Ventilation just gives the muscles involved in breathing a helping hand.
Are there any other effects?
The impact of ventilation is better oxygenation of the body, particularly the brain, which leads to a better nights sleep. This usually means the person has better energy levels, is in better humour and is more able to take part in everyday life with his family, school, college or job. There are also less chest infections, which otherwise can be a very real problem for people with Duchenne muscular dystrophy.
At what age should ventilation be undertaken?
This will vary but it is usually started in the mid to late teens, when tests show a dropping off in the respiratory function and when the boys begin to feel the effects of this such as a lack of energy and appetite, poor sleep or increasing numbers of chest infections. Symptoms of respiratory problems can be very subtle and insidious and need to be specifically asked for. At this stage a trial of ventilation can be recommended to see if symptoms improve.
9. Does ventilation have an impact on all people with Duchenne muscular dystrophy?
Probably, but the heart problems which may be associated with Duchenne muscular dystrophy are responsible for death in around 10% of people. The average lifespan of people with DMD and a severe cardiomyopathy is still only 16.9 years, though better assessments and treatments of this complication are likely to have an effect in the future here as well. However, even in the presence of a cardiomyopathy, ventilation is still the correct treatment for respiratory problems which may be present as well, and in fact the better oxygenation of the heart does help to improve wellbeing and quality of life.
Is ventilation of use to other forms of muscular dystrophy?
This paper is showing statistical data on Duchenne muscular dystrophy. However, many types of muscle disease have an effect on the breathing muscles and in these conditions ventilation may also be very successful.
If the muscles continue to waste, what happens to the quality of life?
Ventilation is about improving the quality of life of the person. With modern living there are many activities and interests that a person with advanced stages of Duchenne muscular dystrophy can take part in, for example; getting out and about using an electrical wheelchair and networking via computers.
Are there any side effects?
Ventilators need to be properly set and maintained, but provided this is looked after there are very few negative effects of ventilation. Initially a face mask can be daunting for the person having the treatment, or a mask may require adjustment to avoid pressure problems, but the range of equipment now on the market means that most difficulties can easily be solved.
Factsheets
See also
Making breathing easier and
Questions about ventilation to ask your consultant
Accessibility
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