“Grave Muscle Weakness” Myasthenia Gravis “Grave Muscle Weakness”

dr yekefallah---phd of nursing 12/1/2018 dr yekefallah---phd of nursing

dr yekefallah---phd of nursing 12/1/2018 dr yekefallah---phd of nursing

dr yekefallah---phd of nursing 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 12/1/2018 dr yekefallah---phd of nursing

dr yekefallah---phd of nursing 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 12/1/2018 dr yekefallah---phd of nursing

dr yekefallah---phd of nursing 12/1/2018 dr yekefallah---phd of nursing

dr yekefallah---phd of nursing 12/1/2018 dr yekefallah---phd of nursing

dr yekefallah---phd of nursing 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 12/1/2018 dr yekefallah---phd of nursing

dr yekefallah---phd of nursing 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 12/1/2018 dr yekefallah---phd of nursing

dr yekefallah---phd of nursing 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 12/1/2018 dr yekefallah---phd of nursing

dr yekefallah---phd of nursing Diagnosis Edrophonium test (Tensilon) Blood analysis Repetitive nerve stimulation Single-fiber electromyography (EMG) Imaging scans 12/1/2018 dr yekefallah---phd of nursing

dr yekefallah---phd of nursing 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. 12/1/2018 dr yekefallah---phd of nursing

dr yekefallah---phd of nursing Signs and Symptoms Affects any of the muscles that you control voluntarily, certain muscle groups are more commonly affected than others 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.  12/1/2018 dr yekefallah---phd of nursing

dr yekefallah---phd of nursing Signs and Symptoms 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 12/1/2018 dr yekefallah---phd of nursing

Exacerbation Trigger Factors Infection Stress Fatigue Cathartics (laxatives) Heat (sauna, hot tubs, sunbathing) 12/1/2018 dr yekefallah---phd of nursing

dr yekefallah---phd of nursing 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 12/1/2018 dr yekefallah---phd of nursing

Medications/Treatment Immunosuppressive Therapy Prednisone Azathioprine Acetylcholinesterase Inhibitors First line of therapy Neostigmine bromide (Pyridostigmine) Edrophonium chloride (Tensilon) 12/1/2018 dr yekefallah---phd of nursing

Medications/Treatment Plasmapheresis Immunoglobulin Therapy Thymectomy 12/1/2018 dr yekefallah---phd of nursing

Myasthenic Crisis VS. Cholinergic Crisis Under medication Increased HR/BP/RR Bowel and bladder incontinence Decreased urine output Absent cough and swallow reflex May need mechanical ventilation Temporary improvement of symptoms with administration of Tensilon Cholinergic Crisis Overmedication Decreased BP Abd cramps N/V, Diarrhea Blurred vision Pallor Facial muscle twitching Constriction of pupils Tensilon has no effect Symptoms improve with administration of anticholinergics (Atropine) 12/1/2018 dr yekefallah---phd of nursing

Nursing Interventions 12/1/2018 dr yekefallah---phd of nursing

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) 12/1/2018 dr yekefallah---phd of nursing

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 Cholinesterase Inhibitors: Pyridostigmine 12/1/2018 dr yekefallah---phd of nursing

dr yekefallah---phd of nursing Patient Teaching 12/1/2018 dr yekefallah---phd of nursing

dr yekefallah---phd of nursing 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 Beta blockers, calcium channel blockers, quinine, quinidine, procainamide, some antibiotics, neuromuscular blocking agents Avoid certain medications D-penicillinamine, A-interferon, botulinum toxin 12/1/2018 dr yekefallah---phd of nursing

dr yekefallah---phd of nursing 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 12/1/2018 dr yekefallah---phd of nursing

dr yekefallah---phd of nursing 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 12/1/2018 dr yekefallah---phd of nursing

dr yekefallah---phd of nursing 12/1/2018 dr yekefallah---phd of nursing

dr yekefallah---phd of nursing 12/1/2018 dr yekefallah---phd of nursing