myasthenia gravis

• autoimmune
• thymus gland continues to produce antibodies
• thymus tumor 15%

• nueuromuscular transmission of impulses are affected for voluntary muscles of the body
• upper body

• impairs transmission of impulses due to lack of acetycholine
• chronic
• progressive-slow
• NO ATROPHY OR LOSS OF SENSATION
• remissions and exacerbations

• profound muscle weakness produced by repeated movements and will disappear following rest
• sleepy/mask face
• diplopia and ptosis
• impaired speech and dysphagia-paralysis of pharyngeal muscles
• advanced-resp. distress to failure
• bladder/bowel incontinence
• INTELLECT OK
• CONSCIOUSNESS OK
• NO LOSS OF SENSATION OR MUSCLE ATROPHY

• history
• rest improve?
• Tensilon Test

• Mestinon
• prostigmin
• steriods
• plasmapharesis
• eye care
• rest and reduce stress-nsg care around rest
• conserve energy

• resp failure
• aspiration
• drug crisis
• myasthenia crisis

• will strengthen muscles for a short time, 5-10 min if the pt has myasthenia gravis
• given in a 2mg dose then an 8mg dose b/c of s/e such as bradycardia and bronchospasms
• -have ER equipment ready

• inhibit the destruction of acetylcholine
• improves nerve impulses
• Mestinon (pyridostigmine bromide)=antimyasthemics
• -least toxic, most commonly used
• -30-60 mg dose Q3-4 hrs initially, onset 30-35 min, duration 3-6 hrs
• -maintenace dose is 600 mg/day
• -s/e: GI-cramps, N/V, diarrhea, excess salivation, anorexia. Bradycardia, bronchospasms, hypotension, profuse sweating, seizures-most likely drug overdose

• adverse reaction-cholinergic crisis
• -teaching: drug therapy is lifelong-take med b/f doing activities, take 30 min b/f meals so they have the strength to eat, take w/ food or milk to dec. GI s/e, use lomotil for cramping, Atropine antidote for drug induced bradycardia,take as ordered-too early=overdose>>cholinergic crisis, too late=MG crisis

• undermedication
• missed doses'infections

sxs- inc BP, P, R, sudden motor weakness-inability to swallow, speak, ptosis, diplopia, DANGER-resp. arrest, aspiration

tx-inc dose of Mestinon, resp. support-watch for aspiration

• overmedication
• remissions
• after thymectomy

• sxs: severe muscle weakness, GI-cramps, diarrhea, bronchial spasms, increased secretions, dec P (brady), vertigo
• tx: temporary D/C meds, resp. support, Atropine to dec salivation
• DG:weakness w/i 1 hr after taking drugs: Mestinon if too much

Tensilon no longer used to diagnose crisis b/c of inc risk of resp arrest during a crisis. When used if myasthenia crisis you have improved strength after giving Tensilon, and in cholinergic crisis you have weakness w/i 1 hr after taking Tensilon.

• suppress the immune response and dec amount of blocking antibodies
• 80-90% have remission when taking these
• ex:
• ACTH
• corticosteroids-Prednisone oral, Solu-Medrol IV
• -sx may worsen for 7-10 days after starting
• nonsteroidal-Imuran, Cytoxan
• -takes 4-8 months to see effects
• -causes fetal deformities

• plasma exchange, temporarily decreases the number of circulating antibodies
• -removes components causing the problem, replace with NS, LR, FFP, or albumin and re-infuse-similar to dialysis
• -3-4 times over 8=10 days, takes 2-3 hrs each time'-usually used for crisis and prior to and after thymectomy

• remove thymus gland located in the mediastinum
• biggest problem postop is pulmonary complications-PN, atelectasis
• because it produces antibodies
• may have tumor here causing the problem
• effects may take up to 3 yrs b/c T cells have a long life

these pts are ususally in the hospital b/c of resp tract infections or acute myasthenic crisis

ineffective breathing pattern

• cough and deep breathe while they can
• incentive spirometry to muscle are too weak to cough
• communication-let them know what's going on

with MG, be alert for changes in resp status, the mosst severe involvement may result in resp failure, bedrest often relieves sxs, bladder and resp infections are often a recurring problem-need for teaching, nursing focus should be pt and family education-meds being high priority

• cascade cough-deep breath followed by 3-4 coughs, sputummoves up with each cough
• suction at bedside
• incentive spirometry
• HOB elevated 3-45 degrees
• turn side to side q 2 hrs
• O2 may need to be humidified if dry
• check pressure areas

dietician can help

FOR MG, ALS, AND BG

• swallowing evaluation
• rest b/f meals-peak med action ex. Mestinon
• eat slow
• soft or semisolid food-giove what they can tolerate
• inc fiber and fluids for bowels
• assess for drooling

• end up with tube feeding
• -PEG for GB until improvement
• -PEG for MG as needed
• PEG for ALS as disease progresses
• -check for residual, distrended abd, raise HOB

• medicate b/f
• space activities
• handicapped stricker
• have things close by, frequent rest periods

• express feelings
• ask questions, provide reliable information and reinforce it
• prepare significant others for changes and supprt them

• assist pt while still moving
• cane, walker, tripod, utensils, lift chair, hospital bed
• support extremities if bedridden, turn q 2 hrs
• prevent bed sores and swelling-dependent extremities
• moon boots
• ROM at leadt BID-bathing is a good time
• prevent DVT-lovenox, compression devices, ambulation if they can
• pressure ulcers-pad boney prominenes, turn
• monitor labs-PRO, albumin

• allow time to talk
• -progression of disease
• -how to handle things
• explain all procedures
• give time to ask questions, prepare mentally for procedures
• let pt control what they can -positon, am care, TV, radio, cards

• picture boards
• bells
• pad/pencil
• eye blinking
• keyboarding
• support lower jaw with hands when speaking-MG

• allow to verbalize feelings
• same kind of things as in powerlessness
• guided imagery, book tapes, music tapes
• support groups
• teach family care if willing so they won't be fearful
• visitors

• high risk for ineffective mgmt of htherapeutic regimen r/t lack of knowledge of condition, treatments
• pain
• disturbed sensory perception
• -tape eyes closed at night, artificial tears, crutches-MG
• -double vision-tape one eye closed, alternate
• -eyeglasses can have crutches attached to lift lids
• -sunglasses