Description

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Description

1. Myasthenia Gravis is a neuromuscular disease characterized by


considerable weakness and abnormal fatigue of the voluntary muscles.

2. A defect in the transmission of nerve impulses at the myoneural junction


occurs.
3. Causes include insufficient secretion of acetylcholine, excessive secretion
of cholinesterase, and unresponsiveness of the muscle fibers to
acetylcholine.
Causes
MG, thought to be an autoimmune disorder, is caused by a loss of
acetylcholine (ACh) receptors in the postsynaptic neurons at the
neuromuscular junction. About 80% of all MG patients have elevated titers for
ACh receptor antibodies, which can prevent the ACh molecule from binding to
these receptor sites or can cause damage to them. MG is often associated
with thymic tumors.

Assessment
1. Weakness and fatigue
2. Difficulty chewing
3. Dysphagia
4. Ptosis
5. Diplopia
6. Weak, hoarse voice
7. Difficulty breathing
8. Diminished breath sounds
9. Respiratory paralysis and failure
Complications
Myasthenic Crisis
 This sudden onset of muscle weakness is usually the result of
undermedication or no cholinergic medication at all. Myasthenic crisis
may result from progression of the disease, emotional upset, systemic
infections, medications, surgery, or trauma. The crisis is manifested by
sudden onset of acute respiratory distress and inability to swallow or
speak.
Cholinergic Crisis
 Caused by overmedication with cholinergic or anticholinesterase drugs,
cholinergic crisis produces muscle weakness and the respiratory
depression of myasthenic crisisas well as gastrointestinal symptoms
(nausea, vomiting, diarrhea), sweating, increased salivation, and
bradycardia.
Primary Nursing Diagnosis
 Ineffective airway clearance related to difficulty in swallowing and
aspiration
Diagnostic Evaluation
 Injection of edrophonium (Tensilon) is used to confirm the diagnosis
(have atropine available for side effects). Improvement in muscle strength
represents a positive test and usually confirms the diagnosis.
 MRI may demonstrate an enlarged thymus gland.
 Test includes serum analysis for acetylcholine receptor and
electromyography (EMG) to measure electrical potential of muscle cells.

Holding your arms above your shoulders until they drop is one exercise that may be performed during the
Tensilon test. In this test, the drug Tensilon is administered, and the response in the muscles are evaluated to
help diagnose myasthenia gravis or to help differentiate between myasthenia gravis and other conditions.

Medical Management
Management is directed at improving function through the administration of
anticholinesterase medications and by reducing and removing circulating
antibodies. Patients with MG are usually managed on an outpatient basis
unless hospitalization is required for managing symptoms or complications.

Pharmacologic Highlights
 Anticholinesterase drugs such as Pyridostigmine bromide (Mestinon),
neostigmine bromide (Prostigmine); neostigmine methylsulfate can be
given as a continuous infusion if the patient cannot take oral medication.
Anticholinesterase drugs blocks the action of the enzyme
anticholinesterase, thereby producing symptomatic improvement;
atropine must be readily available to treat cholinergic side effects and
medications must be administered on time, or the patient may be too
weak or unable to swallow the drug.
 Prednisone is used to suppresses the autoimmune activity of MG
 Nonsteroidal immunosuppressants such as Azathioprine (Imuran),
cyclophosphamide (Cytoxin) is used to suppress autoimmune activity
when patients do not respond to prednisone; can produce extreme
immunosuppression and toxic side effects

Nursing Interventions
1. Monitor respiratory status and ability to cough and deep breathe
adequately.
2. Monitor for respiratory failure.
3. Maintain suctioning and emergency equipment at the bedside.
4. Monitor vital signs.
5. Monitor speech and swallowing abilities to prevent aspiration.
6. Encourage the client to sit up when eating.
7. Assess muscle status.
8. Instruct the client to conserve strength.
9. Plan short activities that coincide with times of maximal muscle strength.
10. Monitor for myasthenic and cholinergic crises.
11. Administer anticholinesterase medications as prescribed.
12. Instruct the client to avoid stress, infection, fatigue, and over-the counter
medications.
13. Instruct the client to wear a Medic-Alert bracelet.
14. Inform the client about services from the Myasthenia Gravis
Foundation.
Documentation Guidelines
 Respiratory status: Rate, quality, depth, ease, breath sounds, arterial
hemoglobin saturation with oxygen
 Ability to chew, swallow, and speak (swallowing can be subjectively rated
by the patient in anticipating ability to swallow food [0 =unable to swallow
liquids to 5 = able to swallow regular diet]), food intake, daily weights
 Muscle weakness and strength, speed and degree of fatigue, ability to
perform activities of daily living, response to rest, and plans for
modification of activity
 Ptosis (can be rated by the nurse [0 = unable to open lid to 5 =uppermost
edge of iris visible])

Discharge and Home Healthcare Guidelines


 Instruct the patient and family on the importance of rest and avoiding
fatigue. Be alert to factors that can cause exacerbations, such as infection
(an annual flu shot is suggested), surgery, pregnancy, exposure to extreme
temperatures, and tonic and alcoholic drinks. Instruct the patient and
family about drug actions and side effects, the indications for dosage
alteration, and the selective use of atropine for any overdose.
 Stress the importance of taking the medication in a timely manner. It is
advisable to time the dose 1 hour before meals for best chewing and
swallowing. Explain the potential drug interactions (especially
aminoglycosides and neuromuscular blocking agents, which include many
pesticides). Encourage the patient to inform the dentist, ophthalmologist,
and pharmacist of the myasthenic condition.
 Instruct patients about the symptoms that require emergency treatment,
and encourage them to locate a neurologist familiar with MG
management for any follow-up needs. Suggest that they collect a packet
of literature to take to the emergency department in case the available
physician is unfamiliar with this disease. (The Physician’s Handbook is
available on request from the MG Foundation.)
 Instruct patients to wear MG identification jewelry. Suggest having an
“emergency code” to alert family if they are too weak to speak (such as
ringing the phone twice and hanging up).
 Instruct the family about cardiopulmonary resuscitation techniques, how
to perform the Heimlich maneuver, how to contact the rescue squad, and
how to explain the route to the hospital.
 Make a referral to a vocational rehabilitation center if guidance for
modifying the home or work environment, such as a raised seat and
handrail for the toilet, would be beneficial.
 

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