Written evidence submitted by Huntington's Disease Association


About the Huntington’s Disease Association


The Huntington’s Disease Association (HDA) was founded in 1971 with the objective of providing support, advice and care for people living with HD in England and Wales.


The charity champions the needs of the Huntington’s community and through a multifaceted approach helps to educate families, friends, and healthcare professionals about HD. These efforts are accompanied by seeking to influence decision makers and keeping the wider Huntington’s community well informed about key developments. The HDA provides advice, support,information and specialist services, including:


         Specialist Huntington’s Disease Advisory Service: Experienced and compassionate advisors support anyone at any stage of the HD journey. They can help those living with the disease, those at risk of inheriting it, family members/carers, children and young people. They also advise friends, neighbours, employers and a patient’s medical team.

         Youth Engagement Service (HDYES): This confidential service is for anyone aged 8-25 living in a family affected by the disease (including extended family).

         Peer support: There’s a range of peer support for those affected by HD, including: local branches and support groups, carer meetings, virtual groups, courses and webinars, young adults’ events, a JHD family weekend and family conferences.

         Training programme for health and social care professionals: The HDA runs bespoke specialist training and essential professional development courses for any health social care practitioner working with Huntington’s patients. Topic areas include: genetics, neuropsychology, speech therapy, and palliative care.


Reason for submitting evidence


As Huntington’s disease (HD) is a rare genetic neurodegenerative disorder affecting 1 in 10,000 people (around 8,000 people in the UK and up to 32,000 people live at risk of developing it) we are in regular contact with clinicians to support our members with the condition. This complex disease is not fully understood so there is currently no cure and only limited treatment options are available to help manage symptoms.


Our Specialist Huntington’s Disease Advisors across the country support members of the HD community at different stages of the disease; from those considering undertaking predictive testing, to those who are symptomatic and are at early stages of the disease, to those who are at the latter stages of the disease and require residence in specialist care homes. Our support includes engaging with clinicians of all kinds, including advocating for those living with HD to ensure they get the best possible support.


We are keen to submit evidence because we have long called for increased integration of health and social care and a clear, coordinated and consistent care pathway for those with the disease. As Huntington’s targets nerve cells within the brain causing a triad of symptoms (motor, cognitive and psychiatric), which get progressively worse, the management of symptoms needs to be overseen by a variety of specialists, including in neurology, psychiatry, genetics and psychology.


In addition, by bringing health and social care closer together, this will help provide more coordinated services, including ensuring that local authority social care teams are working in tandem with medical services to help those with HD secure the highest quality, most independent lives possible.


The HDA’s views on introducing ICSs


Our experience of the introduction of ICSs has unfortunately been largely negative. We have found that there is a lack of transparency of key staff members and responsibility and accountability for service delivery, making it difficult to ensure those living with HD get the support they need.


We have also found that there are differences in the length of time taken to set up ICSs, with some well established and engaging with a wide range of stakeholders, to others still forming membership. This is creating a disparity in the quality of service delivery and the ability of voluntary organisations to engage with the structures and decision-making processes.


What is more, the differing operational plans from ICSs has the potential to exacerbate the postcode lottery of care and support that already exists across the country. HD patients are already faced with managing a challenging and complex disease, which requires a complex care pathway - if operational plans differ from place to place, HD patients might not get the correct support they need at the right stages of the development of symptoms, worsening quality of life for the patient, their carers and families.


There is a general concern that rarer diseases such as Huntington's disease will get lost when the focus is on population health as the number of people affected by HD are so small in comparison to other conditions. We also have concerns about the lack of knowledge about this complex rare disease, which may lead to poor decision making in ICSs’ approach.


At present, people with rare diseases are currently seen in specialist centres, wherever they are located. We are worried that those with HD attending such centres outside of their new ICS will no longer be referred there or be able to access them (as these are not nationally commissioned services).

Overall, we are keen to see how ICSs will help to bring health and social care services together - we hope this is the case to provide more coordinated support to people with HD. However, we hope that the disparity in ICS establishment will soon be addressed, so that we can continue to advocate for the support and services we know the HD community desperately needs.


October 2022