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Medical Surgical

Huntington’s Disease

Huntington;s disease is a chronic, hereditary disease of the nervous system that results in progressive involuntary choreiform (dance-like) movements and dementia.¬† Researchers believe that glutamine abnormally collects in certain brain cell nuclei, causing cell death.¬† Huntington’s disease affects men and women of all races. It is transmitted as an autosomal dominant genetic disorder. Each child of a parent with Huntington’s has a 50% risk of inheriting the illness. Onset usually occurs between 35 and 45 years of age.

CLINICAL MANIFESTATIONS

  • The most prominent clinical features are abnormal involuntary movements (chorea), intellectual decline, ans emotional disturbance.
  • Constant writhing, twisting and uncontrollable movements of the entire body as the disease progresses.
  • Facial movements produce tics and grimaces; speech becomes slurred, hesitant, often explosive, and then eventually unintelligible.
  • Chewing and swallowing are difficult, and aspiration and choking are dangers.
  • Gait becomes disorganized, and ambulation is eventually impossible; patient is eventually confined to a wheelchair.
  • Bowel and bladder control is lost.
  • Progressive intellectual impairment occurs with eventual dementia.
  • Uncontrollable emotional changes occur but become less acute as the disease progresses. Patient may be nervous, irritable, impatient. During the early stages of illness: uncontrollable fits of anger; profound , often suicidal depression; apathy; or euphoria.
  • Hallucinations, delusions, and paranoid thinking may precede appearance of disjointed movements.
  • Patient dies in 10 to 15 years from heart failure, pneumonia, or infection or as a result of a fall or choking.

ASSESSMENT AND DIAGNOSTIC FINDINGS

  • Diagnosis is made on the basis of clinical presentation, positive family history, and exclusion of other causes.
  • Imaging studies, such as computed tomography (CT), and magnetic resonance imaging (MRI), may show atrophy of striatum.
  • A genetic marker for Huntington;s disease has been located. It offers no hope pf cure or even specific determination of onset.

MEDICAL MANAGEMENT

No treatment stops or reverse the process; palliative care is given.

  • Medications such as phenothiazines (haloperidol), butyrophenones, and thioxanthenes, which block dopamine receptors, and reserpine¬† and tetrabenazine. Anti-parkinsonism therapy (L-dopa) may improve chorea and temporarily decrease rigidity in some patients.
  • Motor signs are continually assessed and evaluated. Akathisia (motor restlessness) in the overmedicated patient is dangerous and should be reported.
  • Psychotherapy aimed at allaying anxiety and reducung stress may be beneficial; antidepressants are given for depression or suicidal ideation.
  • Patients needs and abilities are the focus of treatment.

NURSING MANAGEMENT

  • Reinforcing understanding that Huntington;s disease takes emotions, physical, social and financial tolls on every member of the patients family.
  • Encourage genetic counseling, long-term psychological counseling, marriage counseling, and financial and legal support.
  • Teach patient and family about medications, including signs indicating need for change in dosage and medication.
  • Address strategies to manage symptoms (chorea, swallowing problems, ambulation problems, or altered bowel or bladder function).
  • Arrange for consultation with a speech therapist, if needed.
  • emphasize the need for regular follow-up.
  • Refer for home care nursing assistance, respite care, day care centers, and eventually skilled long-term care to assist patient and family to cope.
  • Provide information about the Huntington’s Disease Foundation of America, which gives information, referrals, education, and support for research.

 

Reference:

Handbook for brunner & Suddarth’s textbook in Medical-Surgical Nursing 11th edition by Joyce Young Johnson

Lippincott Williams & Wilkins pp.450-452

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