How to Assess and Document Huntington’s Disease

Introduction

Huntington’s disease (Huntington’s Chorea, HD) is a genetic, progressive neurological disorder. There is no cure and no treatment to slow the progression of the disease.

Characteristics of Huntington’s Disease

• Movement disorders: Instead of normal progression of movements, people with HD may perform one pattern of movement for a few seconds and then suddenly another pattern. Huntington’s chorea progresses from flicking movements to more severe distortional movements of the body. Speaking, swallowing and gait are also affected.
• Psychiatric/psychological manifestations: Symptoms may include depression, anxiety, obsessive behavior and possible psychosis.
• Cognitive deficits: Cognitive symptoms may include severe dementia that develops concurrently with motor dysfunction. Some of the first symptoms associated with cognitive impairment include forgetfulness, poor judgment and decreased attention span.

Course of the Disease

Individuals with HD experience a slow decline in the ability to walk, think, talk and reason. Signs of advanced disease may include severe rigidity/chorea, cognitive impairment, significant weight loss (despite food intake), fevers, lethargy and respiratory distress. As the disease advances, the patient will become totally dependent upon others for care. Most people with HD die from complications such as choking, injuries as a result of a fall, infection or heart failure.

What is the Prognosis?

Adults diagnosed with HD live approximately 15 years after diagnosis, and children live approximately 8-10 years after diagnosis.

Palliative Care

Pharmacological and non-pharmacological interventions should focus on symptomatic relief, prevention of complications, maintaining maximum function and optimal quality of life.

Pharmacological management of the symptoms may include:

Chorea

• Lioresal (Baclofen)
• Haloperidol (Haldol)
• Diazepam (Valium )
• Clonazepam (Klonopin)

Rigidity

• Amantadine (Symmetrel)
• Carbidopa/Levodopa (Sinemet)

Mania

• Valproic Acid (Depakote )
• Carbamazepine (Tegretol)
• Gabapentin (Neurontin)

Psychosis

• Haloperidol (Haldol)
• Chlorpromazine (Thorazine)
• Risperidone (Risperdal)

Aggressive psychological and spiritual support is needed as patients with HD and their families often experience fear, anxiety and depression. Support through HD groups (including online chat groups) are an excellent resource for patients as well as their caregivers. To find a support group, contact your local Huntington Disease Society of America (HDSA).

Documentation

Document the following signs and symptoms:

Physical

• Stage 7C or greater on the Functional Assessment Staging Tool
• Weight loss
• Aspiration pneumonia
• UTI
• Fever
• Sepsis
• Pressure ulcers/wound
• Motor deficits including: ✓ fidgety behavior ✓ lack of coordination ✓ involuntary movements (chorea, dystonia) ✓ difficulty with speech, swallowing, balance and walking

Cognitive

• Short term memory loss
• Difficulty with organization
• Coping issues
• Difficulty concentrating

Behavioral/emotional

• Irritability
• Depression
• Anxiety
• Aggressive outbursts
• Mood swings
• Social withdrawal

Documentation Tips

Document all signs and symptoms including onset and change over time. Include information which describes impact on the patient and family’s overall quality of life and functionality. Collaborate with psychosocial and spiritual professionals to confirm that symptoms are managed with the most effective combination of non- pharmacological and pharmacological interventions.

References

www.hdsa.org

http://www.mayoclinic.com/health/huntingtons-disease/DS00401

http://www.nlm.nih.gov/medlineplus/huntingtonsdisease.html

http://www.wemove.org/hd/hd_tre_mm.html

Grauer, P., Shuster, J. & McCrate-Protus, B. (2008). Palliative care consultant: A reference guide for palliative care 3RDed. Kendall/Hunt Publishing Co.

Kinzbrunner, B. (1993). Non-malignant terminal diseases: criteria for hospice admission. HospUpdate 3: 3-6.

Kuebler, K.K., Davis, M.P. & Moore C.D. (2005). Palliative practices: an interdisciplinary approach. Missouri: Elsevier/Mosby.

Stuart, B., et al. (1996). Medical guidelines for determining prognosis in selected non-cancer diseases. Arlington, VA: National Hospice Organization.

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