Failures of care for Huntington’s Disease show little sign of abating

Despite the recent publication of a HSE plan, access to the care pathway for people with Huntington’s disease is likely to become a ‘postcode lottery’

Earlier this year, a case brought by a family affected by Huntington’s disease (HD) was settled after mediation as a result of failures in care. The HSE and the relevant hospital apologised. The judge was scathing about the quality of services for those with HD and called on the HSE to deliver on an integrated care plan. However, to date, despite the publication of a new plan, nothing substantial has happened.

HD is a relatively rare adult-onset genetic condition. Carriers of the Huntington’s gene have a 100 per cent probability of developing the condition. Parents carrying the gene have a 50 per cent chance of transmitting the gene to their offspring. There are probably over 1,000 people in Ireland with the condition, but the number of gene carriers is much higher.

We do not have exact numbers because there is no centralised care system or register. Because HD affects behaviour, mood, and impulse control, there are acknowledged higher rates of addiction, homelessness, unplanned pregnancies, and suicide among those affected, in addition to cognitive changes and chorea. Due to the combination of psychological, psychiatric, and neurological features of HD, affected families are often deeply impacted. Patients are at very high risk of falling through the large cracks in our heavily siloed healthcare system.

Current services

Although HD is not currently curable, it does not mean that it is not treatable. Best practice requires access to a centralised and highly specialised multidisciplinary team (MDT). Ideally, such an MDT would understand the disease, recognise the early symptoms, understand the impact of the condition on all family members, and link all services in a person-centred holistic care system. Fragmentation of care leads to mistakes and misdiagnoses.

The failures leading to litigation now risk becoming embedded within the new regional health structure. At present, those with HD have access to only one Dublin-based advanced nurse practitioner (ANP), with a second post recently appointed in Galway. These ANPs link with the national voluntary organisation – the Huntington’s Disease Association of Ireland.

However, there is no nationally funded dedicated specialist MDT. The two appointed ANPs work hard. They identify those with most urgent needs across the entire country, and advocate on their behalf across a labyrinthine healthcare bureaucracy. However, they need much more support.

MND

Contrast this with motor neurone disease (MND). Since 2022, and through a sensible and evidence-based HSE-funded modernised care programme, those with MND now have access to a fully funded and highly centralised specialist MDT of healthcare professionals based at Beaumont Hospital, Dublin. This has links to centres in Galway and Cork and with the Irish Motor Neurone Disease Association (IMNDA). We know exactly how many people with MND are in the country as a population-based register has been in operation for 32 years.

The MDT, along with six IMNDA-funded specialist nurses, provides home visits and links with hospital, palliative care, and community-based services in a patient-centred manner.

And because the Irish MND service has an international reputation for excellence, Irish people with MND can participate in clinical trials of potential new treatments, including new gene-based therapies.

Care pathway

Why is there such a stark difference in the provision of care for two rare neurodegenerative conditions, and why are those with HD now at higher risk of fragmentation of care?

This is a direct consequence of the recent regionalisation of the HSE.

In 2022, as part of the modernised care pathway – the same programme that has funded and centralised MND care – a working group, comprising of all the stakeholders involved in the care of those with HD, was convened by the HSE.

The task was to design a similarly structured pathway for HD, drawing on learnings from the MND experience.

The HSE provided a management consultant from EY (formerly Ernst & Young) to facilitate the intensive consultation process and to calculate the budget.

Given its complexity, the objective was to generate a five-year plan for integrated care, from presymptomatic/’at-risk’ individuals through to the later stages of disease. It would include multidisciplinary support across primary care, neurology, clinical genetics, neuropsychiatry, addiction services, social care, and palliative care.

The final detailed plan was submitted to the HSE as part of the modernised care pathway. It was costly, but there were clear health-economic benefits. However, following the establishment of the health regions, the modernised care programme within the HSE was quietly disbanded.

All the detailed submissions, which had been painstakingly developed, were shelved.

Instead, in late 2025, a second HSE working group for HD was convened. A second, revised document for HD was provided to the HSE Chief Clinical Officer. The new pathway has been recently published.

However, this much simplified version does not have a budget attached due to the budgetary discretion of the health regions.

This effectively means that those with HD will be subject to a ‘postcode lottery’ for their care. There does not appear to be any mechanism for joined-up thinking across the different regions and there is no scope for the clinical programmes to recommend funding for supra-regional care pathways.

Accordingly, families affected by HD will be at the mercy of the priorities set by their local health region. And because there is no current structure to provide for supra-regional or national centres, those with HD will not only risk being disenfranchised with respect to high-quality specialist multidisciplinary care, they will be locked out of clinical trials of potentially beneficial drugs.

Accordingly, families affected by HD will be at the mercy of the priorities set by their local health region

It is not appropriate to expect that health regions should prioritise rare conditions without an overall national plan and without the necessary funding.

False economy

Not to put appropriate resources in place is a false economy. The wider health economic benefits of proper centralised care have been fully established. Moreover, there are already two centres where there is advanced nursing expertise.

Supra-regional centres could easily be nationally recognised and appropriately funded from a central budget. But this necessitates sensible joined-up thinking, and the political and administrative resolve to implement, over a five-year period, what was outlined in the original (EY-supported) submission.

Failure to act on this obvious solution will not only lead to unnecessary hardship for this cohort of patients. It will also be costly.

Care will continue to be fragmented, expensive, and reactive rather than proactive and will, unfortunately, invite additional costly litigation by distraught and disenfranchised families.