A 58-year-old man, married with two children aged 17 and 23, has been diagnosed with motor neurone disease. Initially he suffered with weakness of his legs and over a period of 6 months needed to use a wheelchair. He has started to have problems with swallowing and speech and he tends to choke when he is eating. He has not been sleeping well and tends to wake several times at night.
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1. What are the most important issues?
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Correct answer:
The main issues are swallowing – he may need to look at altering his meals and consider a gastrostomy;
breathing – this may be related to respiratory muscle weakness and needs to be assessed; and psychosocial
issues – he and his family may need support to cope with these changes.
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2. What ways are there to help with his swallowing problems?
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Correct answer:
There is a need for careful assessment of his swallowing, including a speech and language therapy. He may benefit from an alteration in the consistency of his diet, to a soft custard consistency and thickening of fluids. A gastrostomy may need to be considered, in conjunction with the respiratory assessment as mortality and morbidity increase with compromised respiratory muscle function.
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3. How can the breathing issues be managed?
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Correct answer:
There is the need to assess his respiratory muscle function – including measurement of his oxygen levels, using an oximeter, and taking a careful history and examination – if there is orthopnoea (breathlessness on lying down), poor sleep with dreams and nightmares, multiple rousing and morning headache, further investigation should be undertaken – overnight pulse oximetry, respiratory testing and blood gas measurement.
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4. If non-invasive ventilation is starting, are there any issues to discuss?
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Correct answer:
Non-invasive ventilation may extend life, and although the respiratory symptoms may ease, the general deterioration of the disease may continue. Careful discussion of future plans and expectations is necessary, including consideration of end-of-life wishes and coping with future deterioration, and even possible withdrawal of treatment. Many patients may wish to state their wishes as an advance decision to refuse treatment (ADRT).
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5.How can professionals best support the patient and family?
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Correct answer:
Careful listening to the concerns and fears of the patient and their family is essential. They may wish to talk of their plans and wishes for the future, including advance care planning. For this man, the support of his sons, allowing them to talk about their concerns and, if possible, to talk more openly with their parents is important.
