HUNTINGTON'S DISEASE (CHOREA)
Huntington’s disease or Chorea is a condition characterized by involuntary, purposeless, rapid motions, as flexing and extending the fingers, raising and lowering the shoulders or grimacing. In some forms, the person is also irritable, emotionally unstable, weak, restless and fretful.
Huntington's disease is a chronic, progressive, hereditary disease of the nervous system that results in progressive involuntary choreiform (dancelike) movement and dementia.
Causes
The cause is unknown. It is transmitted as an autosomal dominant genetic disorder: (Each child of a parent with this disease has a 50% chance of inheriting it however, the child who doesn't inherit it can't pass it on to his own children).
Pathophysiology
There is premature death of cells in the basal ganglia (the region deep within the brain involved in the control of movement). There is also loss of cells in the cortex, which is associated with thinking, memory, perception and judgment.
Research suggests that the disease may be related to a lack of [important brain chemicals) (gamma-aminobutyric acid (GABA) and acetylcholine (Ach) that inhibit nerve action.
Clinical Manifestations
1. Abnormal involuntary movements (chorea), intellectual decline, emotional disturbance-most prominent features.
2. As the disease progresses, constant writhing, twisting, uncontrollable movement may involve the entire body. (These motions are devoid of purpose of rhythm, although patients may try to turn them into purposeful movement.
3. Facial movements produce tics and grimaces. Speech hesitant, slurred, often explosive and eventually unintelligible.
4. Difficulty in chewing and swallowing [→ constant danger of choking and aspiration].
5. Gait becomes disorganized and eventually ambulation is impossible (Patient is confined to bed).
6. Control of bladder and bowel is lost.
7. Progressive intellectual impairment.
8. Patient may be nervous, clumsy, irritable or impatient. (Particularly in early stages, patients are subject to 9. uncontrollable fits of anger; profound, often suicidal depression; apathy or euphoria.)
10. Judgment and memory are impaired.
11. Hallucinations, delusions and paranoid thinking (may even precede the appearance of disjointed movements.)
12. Late stages emotional symptoms decrease but eventually dementia occurs. The patient may appear emaciated and exhausted.
Medical Management
Treatment is supportive, protective and based on patient's symptoms, because there is no known cure. The phenothiazines, butyrophenones and thioxanthene’s which block dopamine receptors, improve the chorea in many patients. Patients with depression, may be helped, by antidepressant medication. Antipsychotic drugs are given for psychotic symptoms. Psychotherapy reduces anxiety and stress.
Nursing Management
1. Provide psychological support to the patient and his family.
2. Identify patient's self-care deficits and provide physical support by attending to his basic needs, such as hygiene, kin care, bowel and bladder care and nutrition.
3. Administer medications as ordered.
4. Encourage the patient to remain as independent as possible. Provide demonstrations, and given the patient ample time to perform the tasks that he is capable performing.
5. If the patient has difficulty speaking, provide him with communication aids, such as an alphabet board. Allow sufficient time to communicate.
6. Take suicide precautions control the patient's-environment to protect him from suicide, or other self-inflicted injury. (eg. Pad the side rails).
7. If the patient has difficulty walking, provide a walker to help him maintain his balance. If his choreic movements are violent enough to cause injury, pad the bed rails and be sure the patient is secure if he's sitting in a chair.
8. If the patient is confined to bed, turn him every 2 hours.
9. Monitor the patient's temperature and his WBC count so that infection can be detected and treated early.
10. Elevate the head of the bed whenever the patient eats to reduce the risk of infection.
11. Provide the incontinent patient with bladder elimination devices, eg. Indwelling catheter and bowel incontinence aids. Eg. Pad and pants or bed protector pads
