Symptomatic treatment for HD has improved greatly in recent years. Behavioural changes including depression, anxiety, suicidal ideation, irritability, apathy and perseveration have a significant impact on the Functional Capacity and Quality of Life of HD sufferers, and many of these respond to appropriate symptomatic treatments. However, the progressive nature of the underlying disease process causes the clinical picture to change and evolve over time, so that treatment appropriate at one point in time may be inadequate or inappropriate two or three years later. In the United Kingdom, there is a national system called the Care Programme Approach (CPA) to ensure that every patient with a serious psychiatric disorder has a Care Coordinator and regular multidisciplinary reviews, but this does not extend to those with neurological disorders such as HD. There is a clear case for manifest HD patients to have regular clinical reviews so that treatment can be tailored to current needs, and emerging problems identified and treated before serious consequences can occur. The present effort to recruit as many patients as possible for the Enroll-HD study provides an opportunity to promote research and improve care simultaneously.
The needs of presymptomatic gene carriers are more complex. Many are keen to participate in Enroll-HD, and find engagement in research a helpful coping strategy. Some prefer to cope by denial (which regular clinical or research assessments may interfere with) while others complain that doctors are reluctant to diagnose HD when they are convinced they are developing symptoms. Symptoms such as irritability and depression often precede the onset of motor manifestations, and regular reviews in the HD clinic may enable treatment for these problems, while regular follow-up allows changes in motor or cognitive performance to be detected, facilitating earlier diagnosis. Lack of awareness (or denial) of symptoms is a well-recognised clinical feature of manifest HD, and there is evidence to suggest that many affected individuals do not participate in specialist HD services even if these are available; regular check-ups after presymptomatic testing may help to overcome this problem.
- Clinical care
- Symptomatic Treatment