MYASTHENIA GRAVIS

Is an autoimmune disorder affecting the myoneural junction, is characterized by varying degrees of weakness of the voluntary muscles. Women tend to develop the disease at an earlier age (20 to 40 years of age) compared to men (60 to 70 years of age), and women are affected more frequently.

PATHOPHYSIOLOGY
autoantibodies directed at the acetylcholine receptor sites impair transmission of impulses across the myoneural junction. fewer receptors are available for stimulation, resulting in voluntary muscle weakness that escalates with continued activity These antibodies are found in 80% to 90% of the people with myasthenia gravis 80% have either thymic hyperplasia or a thymic tumor thymus gland is believed to be the site of antibody production In patients who are antibody negative, it is believed that the offending antibody is directed at a portion of the receptor site rather than the whole complex

(A) Normal ACh receptor site. (B) ACh receptor site in myasthenia gravis.

CLINICAL MANIFESTATIONS
MG is purely a motor disorder, with no effect on sensation or coordination Early symptoms: Diplopia and ptosis

Sleepy, masklike expression because facial muscles are affected (bulbar symptoms) Dysphonia (voice impairment), with nasal sound or difficulty in articulation Weakness of arm and hand muscles, less commonly leg muscles problems with chewing and swallowing, which can present danger of choking and aspiration progressive weakness of diaphragm and intercostals muscles, which may produce respiratory distress (an acute emergency)

ASSESSMENT & DIAGNOSTIC FINDINGS

anticholinesterase test Anticholinesterase agents stop the breakdown of acetylcholine, thereby increasing acetylcholine availability. Injection of edrophonium chloride (Tensilon) IV 2 mg at a time to a total of 10 mg Improvement in muscle strength after 5 to 30 minutes represents a positive test and usually confirms the diagnosis have atropine 0.4 mg available for side effects like bradycardia, sweating, and cramping MRI may demonstrate an enlarged thymus gland. Electromyography (EMG) to measure electrical potential of muscle cells

COMPLICATIONS
Myasthenic Crisis Sudden onset of muscle weakness is usually the result of undermedication or no cholinergic medication at all May result from progression of the disease, emotional upset, systemic infections, medications, surgery, or trauma Manifested by acute respiratory distress and inability to swallow or speak Cholinergic crisis Caused by overmedication with cholinergic or anticholinesterase drugs Produces muscle weakness and respiratory depression

MEDICAL MANAGEMENT
directed at improving function and reducing and removing circulating antibodies. PHARMACOLOGIC THERAPY 1. Anticholinesterase medications Pyridostigmine bromide (Mestinon) neostigmine bromide (Prostigmin) to increase the response of the nerve impulses and improve strength effect can be expected within 1 hour after administration Adverse effects: abdominal pain, diarrhea, nausea, and increased oropharyngeal secretions. therapy is not complete or long-lasting

MEDICAL MANAGEMENT
2. Immunosuppressive therapy Corticosteroids (Prednisone) suppress the patients immune response, thus decreasing the amount of antibody production. As the corticosteroid dosage is gradually increased, the anticholinesterase dosage is lowered azathioprine (Imuran), cyclophosphamide (Cytoxan), and cyclosporine reduce the circulating antiacetylcholine receptor antibody titers Procaine (Novocain) should be avoided, and the patients dentist is so advised.

MEDICAL MANAGEMENT
3. Plasma exchange (plasmapheresis) A technique used to treat exacerbations produces a temporary reduction in the titer of circulating antibodies patients plasma and plasma components are removed (large-bore double-lumen catheter) blood cells and antibody-containing plasma are separated cells and a plasma substitute are reinfused

MEDICAL MANAGEMENT
4. IV immune globulin (IVIG) has recently been shown to be nearly as effective as plasmapheresis in controlling symptom exacerbation 5. Thymectomy (surgical removal of the thymus gland) can produce antigen-specific immunosuppression and result in clinical improvement. It can decrease or eliminate the need for medication. After removal, it may take up to 1 year to benefit from the procedure due to the long life of circulating T cells

NURSING INTERVENTION
Improving Respiratory Function Assess respiratory status frequently (respiratory rate, depth, and breath sounds) and monitor the results of pulmonary function tests to detect pulmonary problems before changes in ABG levels appear. Provide chest physical therapy, including postural drainage, to mobilize secretions, and suction to remove secretions. Postural drainage should not be performed for 30 minutes after tube feeding. Administer oxygen as needed If impending respiratory failure is noted, initiate appropriate action. Avoid sedatives and tranquilizers (can aggravate hypoxia and hypercapnia and can cause respiratory and cardiac depression)

NURSING INTERVENTION
Increasing Physical Mobility Teach patient about anticholinesterase drugs: action, timing, dosage, symptoms of overdose, and toxic effects. Emphasize importance of taking medications on time to improve strength and endurance Encourage patient to keep a diary of symptoms Teach patient to avoid factors that may increase weakness and precipitate myasthenic crisis: emotional upset, infections (respiratory), vigorous physical activity, exposure to heat and cold. Advise patient to wear an identification bracelet

NURSING INTERVENTION
Improving Communication Teach patients with weakened speech muscles techniques for improving communication (e.g. blink eyes, wiggle fingers or toes) Providing Eye care Help patient cope with impaired vision (e.g. taping eyes open for short intervals, instilling artificial tears to prevent corneal damage) Suggest patient use a patch over one eye for double vision and wear sunglasses to diminish the effects of bright light that increase eye problems

NURSING INTERVENTION
Preventing Aspiration Assess for drooling, regurgitation through the nose, and choking while attempting to swallow. Provide standby suction. Encourage rest before meals; place patient in an upright position to facilitate swallowing. Provide soft foods that are easily swallowed. Schedule meals to coincide with the peak effects of anticholinesterase.

NURSING INTERVENTION
Monitoring and Managing Potential Complications: Myasthenic and Cholinergic Crisis Provide immediate ventilatory assistance. Suction as needed. Monitor ABGs, serum electrolytes, I & O, and daily weights. Assist with endotracheal intubation and mechanical ventilation; place patient in ICU for constant monitoring. Assist with administration of IV edrophonium to differentiate type of crisis; this great agent improves mysasthenic crisis but temporarily worsens cholinergic crisis. Neostigmine methylsulfate is given with myasthenic crisis. Assist with nasogastric tube feedings if patient is unable to swallow.

NURSING INTERVENTION
Promoting Home and Community-Based care Instruct patient to consult with physician before taking any new medications.(may aggravate MG) Teach patient the importance of taking medications as prescribed. Inform patient and family about crisis interventions, ways to deal with daily needs, and ways to cope with the disease. Teach family emergency measures that may be needed; provide opportunity to practice these.

MANALOTO, ROMINA T. JANUARY 27, 2009.