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MOTOR NEURONE DISEASE

Dr Anthony M Smith, Medical Director of Pilgrims Hospices in East Kent

Motor Neurone Disease is an uncommon but insidious1y progressive neurological disease (reference 1) leading to muscle wasting and increasing weakness of the affected area. This weakness gradually affects the whole body. There are three varieties, one affecting initially and mainly the limbs, the second affecting the swallowing and speech centres initially and the third having a mixed picture from an early stage.

The average length of a patient’s life from onset of the disease is about two years. In some cases, of course, the life span is shorter while other people have a longer {and even very much longer) life span. 

Increasing weakness and weight loss, loss of co-ordination and balance; changed body image and (in some cases) speech and swallowing difficulties can lead to severe isolation and loneliness. Such people need good support and value the help of local multi-professional teams headed up by local neurologists. The help of the Motor Neurone Disease Association is also much valued (reference 2).  In many cases devoted and dedicated carers provide invaluable support. Hospice care and the support of Macmillan and other Community Support Nurses at home is also invaluable {reference 3).

However people are inevitably very anxious about the prob1ems of the late stage of the disease and issues surrounding dying from Motor Neurone Disease There are a number of common questions asked by patients on which we will comment.

“Shall I choke?” - No; In a long series of two hundred motor neurone disease patients cared for at St Christopher's Hospice none died of choking.

“Shall I have pain?" - Pain is due to weakened muscles and stiff joints in over half the patients suffering from Motor Neurone Disease.  It is not usually severe and is effectively relieved with appropriate pain killers. Quite a number of these patients will need regular low doses of Morphine or other strong pain killer but only small doses are needed in most cases.

“Will I feel breathless?" - Breathlessness is part of the illness for a number of patients with Motor Neurone Disease and is helped by relaxation, appropriate exercises, a cool draft (perhaps by a fan) and appropriate medications (including a small regular dose of Morphine in some cases) including antibiotics. These greatly relieve this symptom.

“What if I can't swallow pills or medicines?” - Medication can be given through a stomach tube if this has been introduced for feeding.  Alternatively, a small syringe pump can give you your medicines through a “butterfly needle” placed under the skin and this will maintain pain and symptom relief.

“Will drooling be a problem?” – Yes, it can be, but there are medications that will reduce saliva flow and head supports will reduce the head droop that encourages drooling.

"What if speech becomes very difficult?” - One of the professional team members who are particularly helpful for Motor Neurone Disease patients is a speech therapist. She will enable speech to be sustained as long as possible and will advise on various mechanical aids that are available while people are strong enough to use them. Even for people whose speech has gone, use of a special small typewriter or other communication aid makes communication possible. The understanding care of personal carers, of community nurses, of hospital or nursing home staff or - particularly - hospice staff is invaluable in supporting people if speech has failed.

So how do Motor Neurone Disease patients die?

Most MND patients at some stage develop a chest infection which rapidly and peacefully leads to loss of consciousness and a rapid and peaceful death when the time comes. So much can now be done with modern medications, routes of administration and, above all, trained and understanding carers, that the end can be made peaceful and without distress even if the months before death have been challenging.

Reference 1

MND affects between four and six people per hundred thousand at any one time.

Reference 2

For the Motor Neurone Disease Association contact:

MNDA, PO Box 246, Northampton NN1 2PR Tel: 01604 250505.

Email: enquiries@mndassociation.org

Reference 3

For information about local hospices, Macmillan nurses and Community Support Teams, contact:

Hospice Information Service, Hospice House, 34-44 Britannia Street, London WC1X 9JG or at St Christopher’s Hospice, 51-59 Lawrie Park Road, London SE26 6DZ Tel: 0870 903 3 903.