Huntington’s Disease (HD) is a genetic neurodegenerative disease which is currently incurable. At present about 1600 Australians live with this disease. Those with HD have a build-up of the ‘Huntingtin’ protein in their brains. This damages nerve cells in the frontal lobe and basal ganglia, affecting neurological function.
Symptoms of HD usually occur between ages 33 and 55, although there are rare cases of Juvenile HD with symptoms starting in children, teenagers and young adults. Life expectancy is usually 10 to 25 years post onset of symptoms. The symptoms of HD are many and varied, they can be grouped into three classes: cognitive, emotional/psychological and physical.
However HD doesn’t affect a person’s orientation – they can still know and recognise people around them, know where they are and know what day it is.
Most people with HD have voracious appetites, most likely caused by high levels of ghrelin (hormone which stimulates the appetite) and low levels of leptin (which usually promotes satiety). They can be known to ‘cram’ food in to satisfy hunger and due to lack of self-awareness. This leads to problems with choking and spillage. Behavioural problems can often be attributed to severe hunger, made more difficult when speech and communication problems are present. Although hungry, those with HD can become frustrated with difficulties self-feeding, chewing, swallowing and reduce their oral intake to avoid having upsetting experiences.
The Dietitian and Speech Pathologist play an important role in the management of persons with Huntington's Disease to optimise nutrition and hydration. It is important to ensure care and nursing staff are aware of the importance of HEHP diet, extra meal times, bowel & weight monitoring, and monitoring for dysphagia for the HD resident.
Education for service providers is available through Huntington’s Qld - this has been designed for nursing facility staff and can be delivered at the facility for staff and family.