Mosby items and derived items © 2005, 2001 by Mosby Inc. The Child with Neuromuscular or Muscular...

Mosby items and derived items © 2005, 2001 by Mosby Inc.Mosby items and derived items © 2005, 2001 by Mosby Inc.

The Child with Neuromuscular or Muscular Dysfunction


• Is a group of non progressive disorders resulting from malfunctions of the motor centers and pathways of brain.

• Noncurable


Cerebral Palsy (CP)• Non-progressive neuromuscular disorder

• Results from damage to brain or related structures by anoxia

• Characterized by early onset and impaired movement and posture. May be accompanied by developmental delays, and intellectual impairment (about 50% have some degree)

• Incidence 1.5-3 per 1000 live births

• Most common permanent physical disability in childhood


Etiology of CP• Caused by trauma/hemorrhage or anoxia

to areas of brain either before birth, during birth, or postnatally

• Most incidences occur from prenatal brain abnormalities

• 12%-23% of CP occurs in term infants with intrapartum asphyxia

• Premature delivery is important risk factor


Types of CP• Spastic (most common)—hypertonic, poor

posture, balance, coordination, impaired fine and gross motor skills

• Athetoid/dyskinetic—involuntary, writhing and jerking movements, drooling, poor articulation

• Ataxic—wide-based gait, rapid, repetitive, poorly performed movements

• Mixed/dystonic—combination of spastic and athetoid


Early Motor Signs of CP• Poor head control after age 3 months• Stiff or rigid limbs• Arching back/pushing away• Unable to sit without support at age

8 months• Clenched fists after age 3 months• Persistent tonic neck and Moro reflexes


Early Behavioral Signs of CP• Excessive irritability

• No smiling by age 3 months

• Feeding difficulties

– Persistent tongue thrusting

– Frequent gagging or choking with feeds


Interventions for CP• Goal is early recognition and promotion of

optimum development

• Most children need wide variety of support services—PT, OT, speech, medical, dental

• Considerable help may be needed in ADLs and training for self-help.

• Ortho needs include orthotics, motor devices, adaptive devices, surgery to correct tendon deformities or control spasticity and contractures


Interventions cont’d• Medication:

– Muscle relaxers to decrease spasticity (Dantrium, Baclofen, Robaxin, Valium, Botox). A Baclofen pump infused intrathecally has been approved.

– Antiseizure meds for those who need them (Tegretol, Depakote)


Interventions cont’d• Technical devices to improve functioning

and promote independence:

– Voice activated computer technology

– Toys controlled with head device when head and trunk are in alignment

– Computerized toys and games to improve hand-eye coordination


Interventions cont’d• Educational and social needs:

– Mainstreaming is encouraged. Many children have normal intelligence but are unable to demonstrate it on standardized tests.

– Vocational training for those who are unable to do formal education

– Encourage recreational activities and appropriate sports activities such as Special Olympics

– Refer family to Cystic Fibrosis Foundation


Neural Tube Defects• Failed closure of neural tube

• May involve entire length of the neural tube or small portion

• Incidence

– More girls than boys

– Occurs three times more often in whites than African-Americans


Neural Tube Defects (cont’d)• Causes:

– 50% or more = folic acid deficiency. Incidence has decreased from 1.3 per 1000 to 0.3 per 1000 live births with folic acid added to food, education, and prenatal diagnosis with pregnancy termination.

– Other cases = multifactorial—drugs, chemicals, radiation, genetic mutation, maternal obesity


Neural Tube Defects (cont’d)• Treatment = prevention

– Supplementation—0.4 mg/ day

– If history of NTD—4 mg/day

– Begin at preconception


Antenatal Diagnosis• Elevated alpha-fetoprotein in amniotic

fluid—16-18 wks gestation

• Uterine ultrasound

• C-section may result in less motor dysfunction

• Why do we want to know?


Spina Bifida• Failure of osseous spine to close.

• Two types

– Spina bifida occulta• Not visible externally

– Spina bifida cystica• Visible defect• Sac-like protrusion


Spina Bifida Occulta• Usually lumbosacral, L5 S1

• Skin indicators (absent, singly or combos)

– Sacral dimple

– Sacral angioma

– Sacral lipoma


Spina Bifida Occulta

– Abnormal adhesion to bony or fixed structure

– Puts traction on the cord• Altered gait• Bowel/bladder problems• Foot deformities


Spina Bifida Cystica• Definition: visible defect with external sac-

like protrusion

• Two types

– Meningocele

– Myelomeningocele


Meningocele• Sac contains meninges and spinal fluid

but no neural elements

• No neurologic deficits


Myelomeningocele• Neural tube fails to close

• May be anywhere along the spinal column

– Lumbar and lumbosacral areas most common

• May be diagnosed prenatally or at birth


Myelomeningocele (cont’d)• Sac contains meninges, spinal fluid, and

nerves. Usually a fine membrane, easy to rupture and leak CSF.

• Varying and serious degrees of neurologic deficit. Location and magnitude of defect determine nature and extent of impairments

• Clinically “myelomeningocele” term is interchangeably with phrase “spina bifida”


Manifestations of Myelomeningocele (SB)• May have joint deformities such as clubfoot,

kyphosis, scoliosis, hip dislocation• If defect below 2nd lumbar vertebra:

– Flaccid paralysis of lower extremities– Sensory deficit– Incontinence of bladder and bowel

• If defect below 3rd sacral vertebra:– No motor deficits– Paralysis of bladder and anal sphincter


Preop Management of SB• Prevent infection—watch for temp,

behavior changes, nuccal rigidity, IICP• Monitor for IICP—HC, fontanels• Prone position, no clothing, incubator,

drape or pad for urine and stool• Keep sac moist with sterile, nonadherent

dressing and change q2-4h• Assessment of neuro and associated

anomalies


Postop Management of SB• Closure within 12-72h to prevent further damage

and infection

• Continue prone position

• VS, I&O, feedings

• Monitor for infection and pain

• Watch for leaking CSF from incision

• Dsg change usually by MD or under strict orders

• Reduce latex exposure---high incidence in SB


Family Education• Feedings• Positioning• Skin care• ROM• CIC (SB most common cause of neurogenic

bladder)• S/S complications• Teach about latex allergy• Dealing with repeated hospitalizations and family

dynamics of coping with chronically ill child


• Most common ACUTE neuropathy in children

• Most common cause of rapidly progressive weakness – any age– ascending bilateral paralysis (acute / hours-days)– reflexes reduced or absent– “pins and needles” (sensory symptoms) in hands and

feet– back and hip pain common in children– medical emergency if autonomic nerves affected (ex.

cardiac dysrhythmia) or if respiration affected– symptoms may worsen in 1st 4 weeks

Guillain-Barre Syndrome (GBS)


• 2/3 report antecedent infection 1-3 weeks prior

– Campylobacter jejuni (esp. China) – CMV– EBV– Hepatitis– Flu– mycoplasma– HSV


• Diagnosis of GBS:– *CSF (> 1 week) – elevated protein, normal cells– Nerve Conduction Velocities (NCVs) – slowing – MRI – may show enhancement of spinal roots– Send titers for suspected pathogens

• Management:– No treatment if symptoms minimal– OT/PT

• Prognosis:– Usually good (~75%), recovery may take weeks to months– Poor prognostic signs:

• rapidly progressive weakness < 7 days• assisted ventilation• Axonal involvement (not just demyelination) – seen on

NCVs as decreased amplitudes


Muscular Dystrophies (MDs) (1204)• Largest group of muscular diseases in

children

• 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


Duchenne Muscular Dystrophy (DMD)• AKA pseudohypertrophic muscular dystrophy

• Most severe and most common of muscular dystrophies in childhood

• X-linked recessive inheritance (Appendix B)

• Mutation of gene that encodes dystrophin, a protein found in skeletal muscle

• Incidence: 1 in 3500 male births


Characteristics of DMD• Onset between age 3-5 yrs

• Progressive muscle weakness, wasting and contractures

• Progressive generalized weakness in adolescence

• Death from respiratory or cardiac failure


Diagnostic Evaluation of DMD• Suspected based on clinical appearance

• Confirmation by EMG, muscle biopsy, and serum enzyme measurement

• Serum CPK and AST levels high in first 2 yrs of life, before onset of weakness; levels diminish as muscle deterioration continues


Clinical Manifestations of DMD• Waddling gait, frequent falls, “Gower

sign”

• Lordosis

• Enlarged muscles, especially thighs and upper arms, calves

• Profound muscular atrophy in later stages

• Mental deficiency common


Gower Sign


Therapeutic Management of DMD• No effective treatment established

• Primary goal: maintain function in unaffected muscles as long as possible

• Keep child as active as possible

• ROM, bracing, performance of ADLs, surgical release of contractures prn

• Genetic counseling for family


Nursing Considerations for DMD• Helping child and family cope with chronic,

progressive, debilitating disease

• Help design a program to foster independence and activity as long as possible

• Teach child self-help skills

• Appropriate healthcare assistance as child’s needs intensify (home health, skilled nursing facility, respite care for family, etc.)

• Refer to MDA of America

Mosby items and derived items © 2005, 2001 by Mosby Inc. The Child with Neuromuscular or Muscular...

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