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neuromuscular dysfun

neuromuscular dysfunctions in peds

QuestionAnswer
Cerebral palsy a group of permanent disorders of the development of movement causing activity limitations that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. 15-60% have epilepsy
types of CP: spastic, Dyskinetic and Ataxia 80% of all cases, primitive reflexes, positive Babinski, exaggerated stretch reflexes, development of contractures./ slow wormlike writhing movements & slow, twisting movements of trunk./ wide-based gait, rapid repetitive movements poorly performed.
Diagnosis of CP neurologic exam and history, neuroimaging, metabolic and genetic testing, poor head control after 3 mo, arching back, floppy tone, unable to sit w/o support after 8 mo, clenched fists after 3 mo, no smiling by 3mo, feeding difficulty.
Goals of therapy with CP establish locomotion, communication and self-help skills. Gain optimal appearance and integration of motor functions. Provide educational opportunities adapted to child’s capability (30-50% cognitively impaired). Promote socialization experiences
Therapeutic management of CP ankle foot braces, orthopedic surgery, Valium for pain r/t spasms & seizures. Botulinum A injections to reduce spasticity in targeted muscles. Dental hygiene to prevent gym hyperplasia. the more severe the functional disability, the worse the prognosis.
Nursing care with CP frequent rest periods, weight gain is a measure of good oral feeling.helmets if child subject to falls. home & play environment adapt to child’s needs. Immunizations to prevent illness and protect against URI. physical, speech & occupational therapy.
Neural tube defect girls > boys. Failure of the neural tube to close during embryo’s early development (3-5) weeks. Detected by elevated alpha-fetoprotein in amniotic fluid. Uterine ultrasound. Prevention is supplementation of 0.4mg of folic acid. prevent 50-70% of cases
Spina bifida (myelomeningocele) **both words interchangeable neural tube fails to close may be anywhere along the spinal column but lumbar and lumbosacral areas most common. Sac contains meninges, spinal fluid and nerves. Location and magnitude of defect determine nature and extent of impairment
Myelomeningocele sac may be fine membrane, prone to leakage of CSF and easily ruptured. May be covered with Dura, meninges or skin, rapid epithelialization. Initial care involves prevention infection, before surgery use a moist nonadherent dressing and inspect sac q2-4h
Myelomeningocele sac surgery early closure after birth (24-72hrs) favorable outcomes. A shunt procedure provides relief from imminent or progressive hydrocephalus. Meningitis, UTI, hydrocephalus triculitis treated w/ vigorous antibiotic therapy. mortality rate from surgery is 4%.
Spina bifida with laxet allergies High risk for allergy from repeated exposure (80%). uticarial, wheezing, rash-anaphylaxis. Cross-reactions with foods: bananas, avocado, kiwi, chestnust. Create a latex-safe environment and reduce expose to latex
Management of Spina bifida fecal continence achieved w/ diet & regular toilet habits. Treat renal problems with urologic care, prompt & vigorous treatment of UTI. Teach parents S/S of complications, positioning, feeding, skin care, ROM, encourage breast feeding. Plan long-term goal
Muscular dystrophies All have genetic origin with gradual degeneration of muscle fibers, progressive weakness and wasting of skeletal muscles. All have increasing disability and deformity with loss of strength. X-linked progressive in males
Duchenne muscular dystrophy (DMD) most severe and most common. X-linked inheritance; 1/3 are mutations. Onset 3-7 y, progressive muscle weakness, wasting and contractures. Calf muscle hypertrophy, death from respiratory or cardiac failure.Ambulation normally stops 9-11 years.
clinical manifestations of DMD waddling gait, lordosis, frequent falls. Enlarged muscles; feel unusually firm or woody on palpation. Later stages have profound muscular atrophy. Cognitive impairment (20 points lower), contracture deformities of hips, knees, & ankles. Disuse atrophy
Therapeutic management of DMD no effective treatment. Primary goal to maintain function in unaffected muscles as long as possible. Keep child as active as possible. ROM, bracing, ADL, surgical release of contractures prn, genetic counseling for family
Nursing care of DMD help child and family cope, design program to foster independence and activity as long as possible. Teach child self-help skills. Appropriate health care assistance as child’s needs intensify. child should be involved in the decisions regarding care
Spinal cord injuries indirect trauma- MVA w/o child restraints. Vertebral compression: blows to the head or butt (diving, surfing, horses). Stabilization & transport to pediatric trauma center. Maintain maximum function of extremities. rehab for evaluation & support.
types of spinal cord injuries Higher injury: most extensive damage. Paraplegia is complete or partial paralysis of lower extremities. Tetraplegia: lacking functional use of all four extremities. High cervical cord injury affects phrenic nerve, paralyzes diaphragm (vent dependent)
Treatment with functional electrical stimulation implantable electrical stimulator with leads attached to the paralyzed muscle or nerves, allows children with certain SCI greater mobility & functional use of paralyzed muscles to sit; stand and walk with air of crutches, a walker or other orthoses.
Nursing care during acute phase of injury airway and establish baseline for neurologic functioning. Child should be calmed, reassured and told not to move. Do not move unless you can stabilize the whole spine. If child is in car seat, remove the whole seat, do not remove child from the seat.
Nursing care after acute phase of injury airway & ventilation. special attention to clearance of secretions. Temperature is poorly regulated so monitor closely. Skin care practices needed use Braden Q scale. Focus on bowel and bladder function; may need CIC to prevent UTI
Created by: smarti13