MNIRES DISEASE

(think hearing, balance; Marine, like being on a boat)

A. Assessment
a. Nausea and vomiting
b. Incapacitating vertigo, tinnitus
c. Feeling of pressure/ fullness in the ear
d. Fluctuating, progressive decreased hearing on involved side
e. Nystagmus, headache
B. Diagnose
a. Dilation of the membrane of the labyrinth
b. Recurrent attacks of vertigo with sensorineural hearing loss
c. Attacks recur several times a week; periods of remission may last several
years
d. Diagnostic tests Weber and Rinne test, CT
e. Complication hearing loss
C. Plan/ Implementation
a. Drug therapy
i. Antihistamines in acute phase (epinephrine, Benadryl)
ii. Antiemetics (e.g., Compazine)
iii. Antivertigo medications (e.g., Antivert, Valium, Reglan, Tigan, Antivert)
iv. Diuretics (e.g., Dyazide)
b. Bedrest during acute phase
c. Provide protection when ambulatory
d. Low-sodium diet (2,000 mg/ day); avoid caffeine, nicotine, alcohol
e. Decompression of endolymphatic sac with Teflon shunt (method of choice)
f. Total labyrinthectomy last resort due to possible complication of Bells palsy

GUILLAIN-BARR SYNDROME
A. Assessment
a. Paresthesias, pain often occurring in glove-and-stocking distribution; pain
b. Motor losses symmetrical, usually beginning in lower extremities, then extend
upward to include trunk, upper extremities, cranial nerves, and vasomotor
function; deep tendon reflexes disappear; respiratory muscle compromise
c. Excessive or inadequate autonomic dysfunction
i. Hypotension, tachycardia
ii. Vasomotor flushing
iii. Paralytic ileus
iv. Profuse sweating
d. Progression period average 10 days, with duration of maximum symptoms
10 days
e. Recovery period several months to a year; 10% residual disability
B. Diagnose
a. Progressive inflammatory autoimmune response occurring in peripheral
nervous system, resulting in compression of nerve roots and peripheral
nerves; demyelination occurs and slows or alters nerve conduction
b. Possible causes
i. Infective, viral
ii. Autoimmune response
iii. May follow immunizations
c. Course
i. Acute, rapidly ascending sensory and motor deficit that may stop at
any level of the CNS
ii. Protracted, develops slowly, regresses slowly
iii. Prolonged course with phases of deterioration and partial remission
C. Plan/ Implementation
a. Intervention is symptomatic
b. Steroids in acute phase
c. Plasmapheresis, IV immunoglobulins, adrenocorticotropic hormone,
corticosteroids
d. Mechanical ventilation, elevate head of bed, suctioning
e. Prevent hazards of immobility
f. Maintain adequate nutrition, hydration
g. Physical therapy, range of motion
h. Pain-reducing measures
i. Eye care
j. Prevention of complications URI, aspiration, constipation, urinary retention
k. Psychosocial support to deal with fear, anxiety, and altered body image
D. Evaluation
a. Has client recovered without major complications?
b. Has client comfort been maintained?

HUNTINGTONS DISEASE
A. Assessment
1. Depression and temper outbursts
2. Choreiform movements
a. Slight to severe restlessness
b. Facial grimacing
c. Arm movements
d. Irregular leg movements
e. Twisting, turning, struggling
f. Tongue movements
g. Person is in constant motion by end of diseased progression
3. Personality changes
a. Irritabilty
b. Paranoia, demanding, memory loss, decreased intellectual function
c. Dementia
d. Psychosis seen at end stage
B. Diagnose
1. Rare, familial, progressive, degenerative disease that is passed from
generation to generation (dominant inheritance)
C. Plan/ Implementation
1. Drug therapy intended to reduce movement and subdue behavior changes
a. Librium
b. Haldol
c. Thorazine
2. Supportive, symptomatic
3. Genetic counseling for all family members D. Evaluation
1. Has family undergone genetic counseling?

HIRSCHSPRUNGS DISEASE
1. Assessment
a. Newborn failure to pass meconium, refusal to suck, abdominal distention
b. Child failure to gain weight, delayed growth, constipation alternating with
diarrhea, foul-smelling stools, abdominal distension, visible peristalsis
2. Diagnose
a. A ganglionic disease of the intestinal tract; inadequate motility causes
mechanical obstruction of intestine
b. Diagnostic tests radiographic contrast studies
3. Plan/ Implementation
a. Help parents adjust to congenital defect in child
b. Foster infant/ parent bonding
c. Prepare for surgery
i. Enemas
ii. Low-fiber, high-calorie, high-protein diet
iii. TPN if needed
iv. Oral antibiotics
v. Measure abdominal girth at level of umbilicus
d. Postoperative care
i. Monitor fluid and electrolytes
ii. Maintain nutrition
iii. Let parents know colostomy is usually temporary, closed when child is
17 22 lb
4. Evaluation
a. Have the child and parents adjusted to the change in body image?
b. Are the parents knowledgeable about stoma care?

REYES SYNDROME (RS)

A. Assessment
1. Fever
2. Increased ICP
3. Decreased level of consciousness, coma
4. Decreased hepatic function
5. Diagnosed by liver biopsy
B. Diagnose
1. Acute metabolic encephalopathy of childhood that causes hepatic dysfunction
2. Causes
a. Link between use of aspirin with viral illness
b. Associated with viruses, especially URI, gastroenteritis
D. Plan/ Implementation
1. Neurological checks
2. Maintain hydration and electrolytes with IV
3. Endotracheal tube and respirator may be needed
4. Monitor CVP or Swan-Ganz catheter
5. Promote family support
E. Evaluation
1. Has child recovered from Reyes syndrome with minimal or no
neuropsychological deficit?