Huntington’s Disease

Huntington’s Disease (HD) is a hereditary neurodegenerative disorder, distinguished by cognitive, psychiatric, and motor manifestations that progressively intensify over time. In post-acute and long-term care (PALTC) environments, managing HD presents intricate challenges due to the multifaceted nature of its symptoms and the necessity for holistic care.

Clinical Manifestations and Progression

• Motor Disturbances: The hallmark choreiform movements – irregular, uncontrollable, brief jerky motions – often become evident as the initial symptom. With disease progression, patients might develop dystonia, bradykinesia, and, in advanced stages, rigidity and akinesia.
• Cognitive Impairment: Initially, patients might exhibit attention deficits, executive dysfunction, and difficulties in planning. Over time, these cognitive disruptions can amplify, leading to severe dementia.
• Psychiatric Symptoms: HD patients frequently experience depression, anxiety, irritability, and in some cases, psychosis. The psychiatric manifestations, often distressing for patients and caregivers alike, necessitate comprehensive assessment and intervention.

Management Strategies in PALTC Settings

• Pharmacologic Interventions: While there is no cure for HD, symptomatic management remains pivotal. Tetrabenazine and deutetrabenazine can help manage chorea. Antipsychotics might be used judiciously for movement control and psychiatric symptoms. Antidepressants can help with depression and anxiety, while mood stabilizers can address mood fluctuations.
• Rehabilitative Therapies: Physical therapy aids in addressing motor impairments, enhancing mobility and decreasing fall risk. Occupational therapy offers strategies for daily activities, while speech therapy can manage dysarthria and dysphagia.
• Psychosocial Support: Given the chronic and progressive nature of HD, both patients and families require robust emotional and psychological support.  
• Advanced Care Planning: Conversations about prognosis, disease progression, and end-of-life decisions should commence early. A well-documented advanced directive, in alignment with the patient's wishes, ensures that care remains person-centric even in advanced stages.