The progressive nature of Huntington’s disease (HD) has a significant impact on the person living with this disease, but also on their families and caregivers.¹ In this panel discussion video series, psychiatrists, social workers, and physical therapists explore the challenges involved in managing the care of people with HD, touching on their experiences with real patients. 

Dealing with the emotions of a HD diagnosis

HD is an autosomal-dominant disease, caused by a cytosine, adenine, and guanine (CAG) trinucleotide repeat expansion in the huntingtin gene on chromosome 4.² The inheritance pattern of HD means children of a person with HD have a 50% chance of developing the condition themselves.³ In this video, social workers discuss the challenges faced by people living with or who are at risk of developing HD. These include dealing with the financial aspect of the disease and navigating the care of a close family member while also dealing with anxiety surrounding their own future. The social workers highlight the difficulty of supporting a person with HD and their family through the burden of a diagnosis, especially if the person with HD feels like a burden themselves. They highlight the importance of providing an individual with HD and their families or caregivers with access to healthcare providers, and the tools to understand and navigate these emotions to help them adjust to a new normal. 

Learn more about supporting people with HD and their families or caregivers through the emotional burden of a HD diagnosis in this video. 

The challenge of a decline in cognitive function

Chorea is the most common motor manifestation of HD, which results in involuntary movements that become more severe and can result in significant functional limitations.⁴ HD also causes other motor impairments that can be managed through physical therapy.² Physical therapists are part of the multidisciplinary team involved in caring for a person with HD, and can help with motor symptoms including abnormal gait and poor balance.² The physical therapists in this video discuss different types of walking aids available to assist people with HD, and the optimal time to introduce these. They highlight that these physical tools should be introduced when the individual is receptive to using a walking aid or tool, but still has the cognitive function to learn a new skill. They note the importance of people with HD using a walking aid, when necessary, as people are likely to be more understanding of their needs.

Watch this video to explore the challenges physical therapists are presented with when treating people with HD.

The challenge of psychiatric symptoms affecting communication 

The degenerative nature of HD can impact the individual’s ability to communicate, and can lead to speech disturbances.⁵ As the disease progresses, these difficulties are coupled with a decline in cognitive processing which can further exacerbate communication challenges.⁵ The cognitive deterioration of a person living with HD is often the most devastating symptom for both the person and their family.⁶ In this video four psychiatrists discuss the importance of allowing people with HD ample time to explain their experiences and disease symptoms, as rephrasing or rushing to the next question can promote irritability in the individual . Promoting these open conversations can enable family, friends, healthcare professionals (HCPs) or carers to understand a problem from the perspective of the person with HD, which may differ to the opinions of others. They highlight that in cases where patients are unaware of their symptoms, listening to the perspectives of the family or caregivers is key.

Discover more about the communication challenges faced by people with HD in this short video. 

Empowering caregivers of people with HD

HD has a large impact on the family of an individual with HD, and the prolonged disease course means that the nature of the caregiver role may change many times as the disease progresses, thus, taxing the ability of the family to provide support over an extended period of time.^7,8 In this video, four social workers highlight the importance of designing a plan for people with HD which ensures caregivers are also supported and empowered to face the challenges of HD. The social workers note the specific challenges faced by those who take on the caregiving role, focusing not just on the practical and financial difficulties, but also on how they experience a different kind of hardship outside of HD i.e., grief, loss, dealing with the changes a person with HD can undergo. They agree that the biggest part of empowering caregivers is to encourage them ‘to be the squeaky wheel’ so that they can be heard and receive the support that they need. 

Watch the video to find out more about supporting caregivers to honor and advocate for patients. 

References

1. Yu M, Tan K, Koloms K, and Bega D. Assessment of caregiver burden in Huntington’s disease. J Huntingtons Dis 2019;8:111–4.
2. McColgan P and S J Tabrizi. Huntington's disease: a clinical review. Eur J Neurol 2018;25:24–34.
3. Myers RH. Huntington’s disease genetics. NeuroRx 2004;1:255–62.
4. Gibson JS and Claassen DO. State-of-the-art pharmacological approaches to reduce chorea in Huntington’s disease. Expert Opin Pharmacother 2021;22:1015–24. 
5. Hamilton A, Ferm U, Heemskerk AW, et al. Management of speech, language and communication difficulties in Huntington’s disease. Neurodegener Dis Manag 2013;2:67–77.
6. Testa CM and Jankovic J. Huntington disease: A quarter century of progress since the gene discovery. J Neurol Sci 2019;396:52–68.
7. Røthing M, Malterud K, Frich JC. Caregiver roles in families affected by Huntington's disease: a qualitative interview study. Scand J Caring Sci 2014;28:700–5.
8. Williams JK, Skirton H, Barnette JJ, and Paulsen JS. Family carer personal concerns in Huntington disease. J Adv Nurs 2012;68:137–46.

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