Myasthenia Gravis Grave Muscle Weakness 11282020 dr yekefallahphd

Myasthenia Gravis “ Grave Muscle Weakness”

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Myasthenia Gravis Autoimmune disease affecting the neuromuscular junction Not a brain disorder – brain functions normally Characterized by fluctuating muscle weakness and fatigability Disease may be generalized or ocular specific 11/28/2020 dr yekefallah---phd of nursing 4

Pathophysiology Antibodies attack Acetylcholine (ACH) receptors at the motor end plate Decreased number of (ACH) receptor sites at neuromuscular junction Prevents neurotransmitter (ACH) from attaching and stimulating muscle contraction Resulting in loss of muscle strength 11/28/2020 dr yekefallah---phd of nursing 5

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Causes No single cause has been identified Abnormal thymus tissue found in most patients with MG Thymic tumors found in 15% of patients Virus infections have been found in some cases and are a suspected cause 11/28/2020 dr yekefallah---phd of nursing 8

Incidence Occurs in all races MG affects 14 per 100, 000 people in the United States Can affect any age group Women – peak incidence 20's to 30's Men – peak incidence 50's to 60's Three times more common in women than men Juvenile onset or over the age of 70 onset is extremely rare 11/28/2020 dr yekefallah---phd of nursing 9

Genetic Factors Myasthenia Gravis is not a genetically inherited disease Some families appear to carry a gene that increases the risk for developing the disease No specific gene has been identified and there are no tests for genetic screening 11/28/2020 dr yekefallah---phd of nursing 10

Diagnosis Edrophonium test (Tensilon) Blood analysis Repetitive nerve stimulation Single-fiber electromyography (EMG) Imaging scans 11/28/2020 dr yekefallah---phd of nursing 11

Tensilon Test Injection may result in a sudden, although temporary, improvement in muscle strength — an indication of myasthenia gravis. Acts to block an enzyme that breaks down acetylcholine, the chemical that transmits signals from nerve endings to muscle receptor sites. 11/28/2020 dr yekefallah---phd of nursing 12

Signs and Symptoms Affects any of the muscles that you control voluntarily, certain muscle groups are more commonly affected than others n Eye, face, throat, neck, limb muscles Difficulty speaking (dysarthria) Difficulty swallowing (dysphagia), Drooping eyelids (ptosis) Double vision (diplopia) Nasal-sounding speech and weak neck muscles that give the head a tendency to fall forward or backward. 11/28/2020 dr yekefallah---phd of nursing 13

Signs and Symptoms tend to progress over time, usually reaching their worst within a few years after the onset of the disease Worsening muscle weakness with repeat activity 11/28/2020 dr yekefallah---phd of nursing 14

Exacerbation Trigger Factors Infection Stress Fatigue Cathartics (laxatives) Heat (sauna, hot tubs, sunbathing) 11/28/2020 dr yekefallah---phd of nursing 15

Diet/Nutrition Eat small meals and snacks five to six times a day Avoid using low fat or diet products when possible Avoid eating lemons or tonic water Eat warm rather than hot food Runny or puree diet when swallowing is difficult Alternate sips of liquid to avoid food from sticking AVOID eating chewy or dry crumbly foods 11/28/2020 dr yekefallah---phd of nursing 16

Medications/Treatment Immunosuppressive Therapy Prednisone n Azathioprine n Acetylcholinesterase Inhibitors First line of therapy n Neostigmine bromide (Pyridostigmine) n Edrophonium chloride (Tensilon) n 11/28/2020 dr yekefallah---phd of nursing 17

Medications/Treatment Plasmapheresis Immunoglobulin Therapy Thymectomy 11/28/2020 dr yekefallah---phd of nursing 18

Myasthenic Crisis VS. Cholinergic Crisis Myasthenic Crisis Cholinergic Crisis Under medication Overmedication Decreased BP Increased HR/BP/RR Abd cramps Bowel and bladder N/V, Diarrhea incontinence Blurred vision Decreased urine output Pallor Absent cough and Facial muscle twitching swallow reflex Constriction of pupils May need mechanical Tensilon has no effect ventilation Symptoms improve with Temporary improvement administration of of symptoms with anticholinergics (Atropine) administration of 11/28/2020 dr yekefallah---phd of nursing Tensilon 19

Nursing Interventions 11/28/2020 dr yekefallah---phd of nursing 20

Nursing Interventions Maintain patent airway Assess swallowing to prevent aspiration Keep appropriate equipment available at the bedside: Oxygen, suction, Ambu bag, endotracheal intubation Promote energy conservation measures Consult Physical Therapy for medical equipment needs (i. e. walkers, wheelchairs) 11/28/2020 dr yekefallah---phd of nursing 21

Nursing Interventions Consult OT for assistive devices to facilitate ADLs Consult with speech and language therapist if weakening facial muscles impact communication Monitor I/O, serum albumin levels, and daily weights Know the signs and symptoms of both Myasthenic Crisis and Cholinergic Crisis Administer Medications Immunosuppressants: Prednisone, Imuran 11/28/2020 dr yekefallah---phd of nursing n Cholinesterase Inhibitors: Pyridostigmine n 22

Patient Teaching 11/28/2020 dr yekefallah---phd of nursing 23

Patient Teaching Teach patient/family disease process, complications, and treatments Teach patient about their medications uses dosage etc Teach medications to use with caution d/t muscle exacerbation n Beta blockers, calcium channel blockers, quinine, quinidine, procainamide, some antibiotics, neuromuscular blocking agents Avoid certain medications n D-penicillinamine, A-interferon, botulinum toxin 11/28/2020 dr yekefallah---phd of nursing 24

Patient Teaching Teach s/sx of both Myasthenic Crisis and Cholinergic Crisis Help patient plan daily activity to coincide with energy peaks Stress need for rest periods Explain that exacerbations, remissions, and daily fluctuations are common Avoid strenuous exercise, stress, infection, exposure to hot or cold temperatures Teach patient to wear medic-alert bracelet Provide Info on Support groups 11/28/2020 dr yekefallah---phd of nursing 25

Prognosis Chronic disease with periods of exacerbation and sometimes remissions Disease course is highly variable Symptoms respond well to treatment and in most cases the patient can live a normal or nearly normal life Ocular Myasthenia has the best prognosis 11/28/2020 dr yekefallah---phd of nursing 26

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