Information
What is Huntington’s disease (HD)?
HD is a rare disorder most often diagnosed in middle age. It progresses over time and is uniformly fatal. People with HD are typically progressively affected by varying combinations of cognitive problems, psychiatric problems (commonly depression sometimes accompanied by self-harm, more rarely psychosis, aggression or other features) and movement disorder over many years. Currently HD is diagnosed if there is a positive genetic test along with a compatible movement disorder. A few people supported by the Huntingtons Disease management team may not have a definite diagnosis of HD, for instance those with a positive genetic test who have not yet developed a movement disorder.
Treatment
There is no treatment currently for HD itself. There is intensive research going on in this area. There are various symptomatic therapies available, often with limited evidence. Typically medications used overlap strongly with those used in other conditions. Depression is particularly common and the risk of suicide should be borne in mind. See one useful treatment algorithm https://bestpractice.bmj.com/topics/en-gb/513 (BMJ Best Practice).
Scottish Huntington’s Lothian team
Our small team comprises:
Two HD specialists (health care/social sciences background offering outreach support)
– Trevor Law (senior HD specialist)
– Katherine Spence (HD specialist)
A lead consultant
– Dr Peter Foley (Consultant Neurologist)
We also have a senior financial wellbeing officer, specialist youth adviser, and an admin/resource worker. We provide proactive support for people and their families affected by HD throughout the Lothians. There are also research projects recruiting in Lothian.
People with HD are generally seen in the community by an HD Specialist and often also in the HD management clinic which is neurology Consultant-led.
Multidisciplinary team
We work closely with a variety of other teams including psychiatry and neurorehabilitation. Close psychiatry input may be particularly important for a few people with HD.
Detailed guidance on available multidisciplinary support in Lothian is available on the Lothian Care Framework for HD.
Contacts;
Lothian Huntington’s Disease Management service: We are happy to discuss cases. Please email huntingtons@nhslothian.nhs.scot.uk and one of the HD Specialists will call you back (checked in office hours, weekdays).
Clinical Genetics: queries regarding predictive testing or other aspects of genetic testing may be best addressed by clinical genetics.
We are happy to discuss cases where the best approach or route of referral is uncertain
Who to refer:
- Adults with known Huntington’s Disease wishing any ongoing input from Huntington’s Disease management service
- Adult with known positive predictive test, who wishes to be seen by the Huntington’s Disease management service (whether or not definitely affected by HD)
- Children (<18 years old) in families affected by HD may be eligible for support from SHA specialist youth worker
Who not to refer:
(Please discuss with the team directly at above contacts if required, or any uncertainty)
- Person with suspected new diagnosis HD. For instance suspected chorea in presence of family history, or other high index of suspicion.
- Usually refer to movement disorder clinic (neurology) or general neurology clinic for initial assessment, and testing if indicated.
- If a test for Huntington’s Disease is carried out and is positive, they will then usually be referred to Huntington’s service for ongoing care.
- Person interested in predictive testing – in other words testing for risk of developing HD in the future, but not currently suspecting that they are affected. Commonly a family member of an affected patient, who is currently “well”.
- Please refer to genetics service (counselling and testing available for age 18 or older).
- Genetics service also appropriate for issues including pregnancy planning in affected families, where specialised techniques may be available
- Significant psychiatric issues developing in known Huntington’s Disease. Most often, patients in this position will be known to the Huntington’s service and psychiatry team already involved.
- If psychiatry services are already involved, direct discussion may be most appropriate
- If this is not the case psychiatry input may be most appropriate
- However please discuss if required.
How to refer: (e.g. SCI & which option etc)
Sci-gateway> Royal Infirmary> Neurology
Primary Care Management
(Information to help GP’s treat patient without hospital referral (where appropriate)
- Patients with Huntington’s disease should be monitored for signs of depression and can respond well to typical anti-depressants
- There is a higher risk of suicide in this group and so care should be taken with prescribing to reflect this
- Please see BMJ best practice for more detailed advice on specific symptoms and management
- Detailed multidisciplinary input (HD team, psychiatry, neurorehabilitation, SLT, PT, OT, others) may be appropriate particularly in complex or more advanced cases – see link to Lothian Care Framework above for multidisciplinary team contacts, and please discuss if required.
BMJ Best Practice-Huntington’s disease BMJ paper.pdf
Scottish Huntingtons Association- Lothian Care Framework Lothian Care Framework for HD.
