15.4 Motor Neurone Disease
15.4.2 Care Needs and Mobility Considerations
15.4.1 Introduction
(i) This is the name given to a group of related diseases affecting the motor nerve cells (which control movement) in the brain and spinal cord. In these conditions the motor nerve cells degenerate and the muscles which they supply become wasted and lose their ability to contract fully. The cause of the disease is unknown. The onset occurs most commonly between 40 and 60 years of age, although it can affect younger people.
(ii) The way in which the condition first presents depends on which groups of motor nerve cells are affected. Weakness of the limbs is often the initial presentation with loss of fine movements of the hands and difficulty in walking. In addition to gradual paralysis of the limbs, difficulties with speaking, swallowing and breathing develop (bulbar palsy).
(iii) The disease is progressive and its relentless nature means that disabilities may develop rapidly over a relatively short time scale. Overall the length of the illness can be between three to five years. Diagnosis may however be delayed and the average life expectancy after diagnosis is fourteen months. For many sufferers it is a rapidly progressive disease with a fatal outcome. Those cases presenting early with bulbar palsy may deteriorate quickly and have an especially poor prognosis as a result of respiratory difficulties.
(iv) About 10 to 15 percent of people may have survival times lasting up to five years. These tend to be those who develop the condition at a younger age, where the limbs are predominantly affected initially and who have some of the rarer types of the group of motor neurone related diseases.
15.4.2 Care Needs and Mobility Considerations.
(i) The need for attention with personal care and bodily functions will depend on the muscles involved and the extent of the weakness. Involvement of the upper and lower limbs will lead to the need for assistance in feeding, dressing, washing, rising from a chair and with the toilet. Difficulty may be experienced in turning at night in bed. The debilitating effects of fatigue, difficulty in swallowing saliva, weight loss, anxiety and depression may exacerbate the other physical difficulties increasing the need for care as the disease progresses.
(ii)(ii) Unsteadiness and a propensity to fall will affect mobility. As weakness of the lower limbs worsens the ability to walk is severely restricted and use of a wheelchair becomes necessary.
(iii) Ten percent of sufferers may develop a mild form of dementia. It may be necessary to consider the need for some supervision, although it is likely that a need for attention with bodily functions will be more predominant.
(iv) When difficulties with swallowing, speech and breathing develop (bulbar palsy) a small feeding tube inserted directly into the stomach through the abdominal wall (percutaneous endoscopic gastrostomy) will enable liquid feeds to be given to maintain nutrition. Help in communicating may be needed as the ability to speak deteriorates. In an increasing number of people assisted ventilation is used to aid breathing.
15.4.3 Further Evidence
(i) A hospital factual report from a neurologist or specialist nurse attached to the hospital clinic will usually confirm the diagnosis including the type of disease and indicate the extent of disability at that time. Occupational therapists may also have made assessments of disability with recommendations for aids and equipment. Physiotherapists and speech therapists advise sufferers on means of alleviating disability as the disease progresses.
(ii) In the latter stages of the disease people may be cared for predominantly by general practitioners, palliative care nurses and hospice doctors. These can be a valuable source of medical evidence that the disease is in an advanced stage and that life expectancy is reasonably expected to be less than six months. Referring factual medical reports to Medical Services both at this stage, and earlier in the process, can help in making an accurate assessment of mobility/care needs. This is of importance in determining the prognosis of a condition in which the severity of the disabling effects may evolve quickly.
(iii) An EMP report would not be the first choice of medical evidence in most cases. It is likely to be of greatest use in the small number of cases who have a longer life expectancy, and when disability is less severe earlier in the disease.