- Huntington’s disease (also called Huntington’s chorea) is a rare abnormal hereditary disorder of the CNS. It is characterized by chronic progressive chorea (involuntary purposeless, rapid movements) and mental deterioration that results in dementia.
- Chorea, the Greek word meaning “dance”.
- Hereditary (autosomal dominant gene transmission)
- There is destruction of cells in the caudate nucleus and putamen areas of the basal ganglia and extrapyramidal motor system. The neurotransmitters, gamma-aminobutyric acid (GABA) and Ach are decreased. Dopamine is not affected, but the decrease of Ach cause relative increase of dopamine in the basal ganglia. The excess dopamine causes uncontrolled movement in Huntington’s chorea.
Assessment/Clinical Manifestations/Signs and Symptoms
- Intellectual decline
- Abnormal movements
- Restlessness, forgetfulness, clumsiness, frequent falls
- Problems with speech, coordination and balance
- Depression, memory loss, emotional lability and impulsiveness
- Facial grimaces, protrusion of the tongue, jerky movements of the arms and legs
- Gait disturbances, patient at risk for falls
- Although no treatments halts or reverse the underlying processes, several medications prescribed.
- Disease management includes rehabilitative therapy, teaching, counseling and professional legal, financial and estate planning advice.
- Pharmacological methods of treatment are carefully considered when treating Huntington’s disease. Many medications can be used and it is important to assess the combination of medications that are used.
- Medications administered to manage Huntington’s disease:
- Rilutek (riluzole) is currently being studied to decrease cognitive manifestations
- Skeletal muscle relaxants to modify the choreiform movements
- Antipsychotics to block the dopamine receptors in the brain
- Antidepressants to help control the chorea, behavioral changes and depression
- Weight loss
- Congestive heart failure
- Bed sore
- Risk for injury from falls and possible skin breakdown (pressure ulcers, abrasions), resulting from constant movement
- Imbalanced nutrition: less than body requirements due to inadequate intake and dehydration resulting from swallowing or chewing disorders
- Risk for aspiration related to swallowing difficulty
- Anxiety and impaired communication from excessive grimacing and unintelligible speech
- Disturbed thought processes and impaired social interaction
- Prevent injury and possible skin breakdown
- Pad the sides and head of the bed
- Keep the skin meticulously clean
- Encourage ambulation with assistance to maintain muscle tone
- Secure the patient in bed or chair with padded protective devices making sure they are loosened frequently
- Keep patient as close to upright as possible while feeding. Stabilize patient’s head gently with one hand while feeding
- The nurse needs to educate and support the patient and family as they adjust to the lifestyle changes that are required.
- The actions and potential side effects of medication regimen need to be taught, monitored and adjusted to the desired patient response.
- Regular moderate exercise can reduce stiffness and tremors.
- As the disease progresses, the patient and family will require more assistance with activities of daily living, emotional support, and potential financial concerns.