Care and mobility considerations

Muscular weakness affecting the hands and forearms leads to initial difficulties in fine manipulations, griping, lifting and carrying. Help will be needed with dressing, preparing food and personal hygiene. As the condition progresses to affect all the muscle groups of arms and shoulders, help will be required with all aspects of self-care including feeding and drinking.

Weakness of the feet and lower legs causes unsteadiness in walking, a tendency to trip and a reduction in the distance that can be covered without undue fatigue. The ability to stand for prolonged periods, rise from a chair, to bend down and walk diminishes as the disease progresses. Help will be needed with cooking, with stairs, with the toilet and with moving around. It becomes difficult for the individual to turn in bed at night. As the condition affects both lower limbs walking become severely restricted, and people are likely to use a wheelchair outside and in the home. They will be unable to stand without support and be at risk of falls.

A person with bulbar symptoms is likely to have devices and aids to facilitate nutrition, speech and respiration. Initially they may be able use these themselves if upper and lower limb function is not severely restricted. However symptoms are often rapidly progressive and the ability to use the devices will be compromised by fatigue, shortness of breath, weight loss and general debility, even if limb function remains reasonable. Elderly people with predominantly bulbar symptoms may be unable to learn how to use these devices/aids and need help from the outset. As limb function in people with bulbar palsy deteriorates they need help with all aspects of self-care, and walking starts to be restricted.

Some people with bulbar symptoms may be able to walk until late in the course of the illness. However walking ability will be compromised to a degree in most people in this group by shortness of breath, fatigue, weight loss, recurrent chest infections and the general debilitating nature of the condition. Overall people presenting with bulbar symptoms are likely to have a shorter life span than others.

About 10% of people develop mild symptoms of dementia. However it is likely that their need for help with all aspects of care and mobility will be much greater than any requirement for supervision.

People in the terminal phases of the illness will be receiving a high degree of assistance from others on a 24-hour basis.

Some people with amyotrophic lateral sclerosis which has been diagnosed early, and in whom the initial progression is slow, may have few functional restrictions at first, especially if only one hand or foot is affected. They may be able to care for themselves and walk a reasonable distance for between 1 to 3 years before functional restrictions become generalised. People with the less common variants of motor neurone disease that are known to have a long prognosis may have few functional restrictions for many years.

Amended June 2008