Nursing Guide to Huntington's Disease: Nursing Diagnosis, Interventions, & Care Plans

Huntington's Disease Etiology and Epidemiology 

Huntington’s disease or Huntington's chorea is a genetic disease caused by a mutation in the Huntington gene. Huntington’s disease causes the breakdown of nerve cells in the brain. Over time this breakdown causes damage leading to deterioration in the individual’s physical, mental, and emotional abilities. 

Huntington’s disease symptoms usually appear between the ages of 30 and 50 but can occur earlier (National Institute of Neurological Disorders and Stroke, 2019). If symptoms occur before the age of 20, the diagnosis is juvenile Huntington's disease. Children that have a parent with Huntington's disease have a 50 percent chance of developing the disease at some point in their life. 

Huntington's Disease Diagnosis 

A definitive diagnosis of Huntington's disease is made by genetic testing. Individuals who have the gene will develop the disease. MRI and CT scans may be performed to show images of the brain. These tests may show areas of the brain affected and rule out other causes of symptoms. 

A preliminary diagnosis of Huntington's disease is made by review of symptoms, family history, and physical, neurological, and psychiatric exams. The symptoms the individual experiences and the order in which symptoms appear can vary from person to person. 

Symptoms may include: 

• Unsteady gait 
• Involuntary movements (chorea) 
• Increasingly slow and rigid movements 
• Forgetfulness 
• Impaired judgment 
• Difficulty concentrating 
• Obsessive-compulsive symptoms 
• Personality changes 
• Slurred speech 
• Mood swings and depression 
• Difficulty swallowing 
• Weight loss 

Huntington's Disease Management 

There is currently no treatment to slow or stop the progression of Huntington's disease (Huntington’s Disease Society of America, 2021). Current treatment focuses on easing the symptoms associated with Huntington's disease. Treatment requires an interdisciplinary approach and may consist of a: 

• Neurologist 
• Psychiatrist 
• Psychologist 
• Speech therapist 
• Occupational therapist 
• Physical therapist 

Individuals with Huntington’s disease usually die 10-30 years following the onset of symptoms (National Institute of Neurological Disorders and Stroke, 2019). 

Medications used to treat symptoms incorporate the following classifications: 

• Anticonvulsants 
• Antidepressants 
• Antipsychotics 
• Benzodiazepines 
• Mood stabilizers 

Tetrabenazine (Xenazine®) and deutetrabenazine (Austedo®) are used to suppress involuntary movements but may worsen symptoms of depression. Antipsychotic medication has also been shown to assist in the management of involuntary movements as well as managing psychiatric symptoms. 

Huntington's Disease Nursing Care Plan

Nursing Considerations 

Use the nursing process to develop a plan of care for individuals. The nursing assessment (with common findings listed), diagnoses, interventions, expected outcomes, and education for Huntington’s disease are listed below. 

Assessment 

A history and physical assessment for individuals with Huntington’s disease may show: 

• Abnormal eye movements 
• Confusion 
• Emotional and mental changes 
• Family history of Huntington’s disease 
• Fatigue 
• Flailing of extremities 
• Gait disturbances 
• Grimacing 
• Intellectual decline 
• Irritability 
• Loss of bladder and bowel control 
• Loss of interest in activities 
• Loss of musculoskeletal control 
• Memory problems 
• Problems chewing and swallowing 
• Seizures 
• Speech disturbance 
• Tics 
• Tongue smacking 
• Untidiness 

Nursing Diagnosis/Risk For 

• At risk for falls with injury related to gait disturbance, loss of musculoskeletal control as evidence by gait unsteady 
• Altered nutrition less than body requirement related to constant movement, difficulty chewing and swallowing as evidence by weight loss 
• At risk for aspiration related to dysphagia as evidence by difficulty swallowing 
• Impaired communication related to difficult to understand speech 
• Anxiety related to diagnosis as evidence by difficulty making wants and needs known, and disease process 
• Disturbed thought process related to disease process as evidence by depression, memory problems, confusion, emotional changes, and insomnia 
• At risk for skin breakdown related to constant movement, chorea, and incontinence 
• Self-care deficit related to depression, memory problems, chorea as evidence by requires assistance with activities of daily living 

Interventions 

• Keep skin clean and dry 
• Assist with ambulation and transfers 
• Assist the individual in sitting up during meals 
• Monitor for choking and coughing during meals 
• Actively listen to feelings and concerns 
• Assess for potential suicide risk and suicidal ideation 
• Speech, physical, and occupational therapy referrals 
• Attempt to anticipate needs 
• Ask yes or no questions when appropriate 
• Encourage daily exercise 
• Follow speech therapist diet recommendations 
• Administer medications as ordered 
• Modify environment to support safety and independence 
• Provide nutritional support as needed 
• Refer to community agencies for support 
• Provide respiratory support such as suctioning, oxygenation as needed 
• Assist with activities of daily living including feeding, bed positioning, transferring, toileting, dressing, and bathing as needed 
• Have suction available at the bedside 
• Provide communication aids 

Expected Outcomes 

• Utilizes techniques to reduce the amount and frequency of depressive episodes or insomnia 
• Makes needs known utilizing verbal or nonverbal methods 
• Maintains functional mobility as long as possible within disease limitations 
• Experiences no complications related to immobility 
• Shows no signs or symptoms of aspiration 
• Maintains adequate amounts of nutrients 
• Remains free from falls with injuries 
• Demonstrates effective coping strategies for anxiety and disturbed thought process 
• Remains free from open areas on the skin 
• Remains clean and well-groomed 

Individual/Caregiver Education 

• Condition, treatment, and expected outcomes 
• Action and potential side effects of medication 
• Diet recommendations including consistency and signs of aspiration 
• Genetic counseling and testing with family members 
• Recommended follow-up with healthcare providers 
• Notify healthcare provider or seek medical assistance for: 
• Fall with serious injury 
  • Choking with meals or new cough with meals 
  • Signs of pneumonia such as elevated temperature, shortness of air, coughing 

Content Release Date

6/11/2024

Content Expiration

12/31/2025

Course Contributor

The content for this course was created by Kim Matthews, RN.

Ms. Matthews obtained a nursing degree from Western Kentucky University in 1998. Ms. Matthews possesses over 20 years of nursing experience with over 17 of those in the Skilled Nursing industry. Ms. Matthews has extensive experience in MDS, restorative nursing programs, and nursing management. Ms. Matthews is currently a Post-Acute Care Content writer and subject matter expert for MDS.

References

• Huntington’s Disease Society of America. (2021). Overview of Huntington's disease. https://hdsa.org/what-is-hd/overview-of-huntingtons-disease/
• National Institute of Neurological Disorders and Stroke. (2019). Huntington’s disease information page. https://www.ninds.nih.gov/Disorders/All-Disorders/Huntingtons-Disease-Information-Page