End of life care in Huntington’s disease and how the Huntington’s Disease Association can help

Categories: Care.

Huntington’s disease (HD) is a rare genetic disorder that affects about 5,700 people in the UK. (1)

It is a complex illness that presents with a range of symptoms, including cognitive, emotional and motor symptoms, which result in a devastating combination of mental health problems, thinking changes and profound physical disability. Prognosis is 15 to 20 years from onset of symptoms. (2)

Every child born to an affected parent has a 50% chance of inheriting the gene that causes the illness, and if they inherit the gene they will develop HD. (3) So for many of the family carers caring for someone with HD, they are watching a mirror image of what may happen to them. This presents professionals involved in the care of people with HD with a unique challenge – especially in ensuring good quality end of life care.

Physical symptoms

Motor symptoms in HD include impaired voluntary movements and additional voluntary movements.

Often in later stage HD the large movements, known as chorea, are replaced by bradykinesia, stiffness and rigidity. However, if chorea is still present late stage it can cause particular challenges with skin break down and moving and handling can also be problematic.

Assessing and reassessing the person for a suitable bed and wheelchair can make a real difference to the person’s quality of life. (4)

Stiffness and rigidity can cause pain and discomfort and these may also lead to painful contractures.

Because of the lack of verbal communication, and a reduced ability to convey non-verbally any pain experienced, pain may be difficult to diagnose. Changes in behaviour to sudden agitation, restlessness or screaming may indicate the presence of pain, so constant evaluation is needed.

Involvement of physiotherapist, occupational therapist and wheelchair services are essential, and the European Huntington’s Disease Network has produced a series of guidelines for occupational therapists and physiotherapists to give some guidance. (5)

Changes in behaviour

In late stage HD some of the earlier behavioural problems such as aggression, impulsivity and lack of insight may lessen. These can be replaced by resistant behaviours and agitation.

It is important to point out that sudden changes in behaviour are not common so if these are experienced further investigation should take place. Ruling out head injury and underlying infection can be a good starting point. (4)

Environmental factors which may contribute to these behaviours should be considered and keeping a record of what is happening before an episode occurs may give some clue as to why the person is behaving in this way. It is important to recognise that frustration may play a part.

Psychiatric symptoms may still be present late stage but can be difficult to assess. They can, however, have a profound effect on someone’s quality of life and so it is important that changes in mood are investigated.

Late stages of the disease

In advanced HD people often become incontinent. Standard continence pads may be unsuitable because of the chorea. Urinary tract infections can occur regularly and may lead to an acute exacerbation of HD symptoms.

Ensuring people have a good fluid intake can be a challenge because of the eating and swallowing difficulties associated with HD. Constipation is common due to reduced mobility, poor nutrition and hydration and side effects of medication. (4)

Communication and swallowing both become increasingly affected during the course of the illness. Time and patience is essential. Allowing the person time to process and formulate a response is essential, as well as asking closed questions and limiting responses.

Involving a speech and language therapist and dietitian in advising and assessing the patient is essential and changes to diet will become necessary.

Some people will have a PEG tube fitted and this is one of the many reasons why it is essential to talk to people with HD about advanced care planning very early in the illness, before cognitive ability has deteriorated.

Support for those caring for someone with HD

In 2015, due to feedback from patients and their families about the lack of understanding of late and end stage HD, the HDA used their awareness week to focus on producing literature and developing a teaching package for palliative care services and hospices.

The HDA employs a team of specialist HD advisers (SHDAs) who work with professionals and families to improve the quality of life for people affected by HD.

Over 60 training sessions were given to relevant services and over 500 copies of ‘Care in advanced Huntington’s disease’ were distributed.

Here is an example of the feedback we received: “My overall understanding of the disease has been overly improved. I believe that the care I provide in future will be provided with the knowledge that I am working in the best interest of the service user I now understand their own thought process more fully.”

The overall aim of the Huntington’s Disease Association is to improve the quality of life of those impacted in the widest sense by this illness. Working to create better understanding of Huntington’s disease and a greater knowledge of the needs of patients and their families will help to achieve this aim which is especially important as an individual comes to the end of their life.

Empowering family and professional carers to better care for people affected by HD will hopefully lead to an improved journey for the patient to the end of their life.


  1. Evans SJW, Douglas I, Rawlins MD, et al. Prevalence of adult Huntington’s disease in the UK based on diagnoses recorded in general practice records. Journal of Neurology, Neurosurgery & Psychiatry. 2013; 84(10):1156-1160. Available from: doi:10.1136/jnnp-2012-304636
  2. Huntington’s Disease Association. A guide to Huntington’s disease for general practitioners and the primary health care team. Huntington’s Disease Association England & Wales; 2014. Available from: http://hda.org.uk/hda/factsheets
  3. Quarrel O. Huntington’s disease, the facts (2nd edition). Oxford: Oxford University Press; 2008.
  4. Stanley CA, Crowder W, Abuzaid R, Heavey A, Mardle-Aylett K, Taylor R, Salanio D. Care in advanced Huntington’s disease. Huntington’s Disease Association England & Wales; 2015. Available from: http://hda.org.uk/hda/factsheets
  5. European Huntington’s Disease Network Network Working Groups. Available from: http://www.euro-hd.net/html/network/groups [Accessed 5 February 2016]

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