Chronic Neuromusculoskeletal Disorders - PEDS

Define Cerebral Palsy (3)

• Nonprogressive impairment of motor function, especially that of muscle control, coordination, & posture

• Causes abnormal perception & sensation & manifests differently in each child

• Congenital or acquired

Risk Factors - Cerebral Palsy

• Prenatal - Malnutrition, drug use, genetic/chromosomal abnormalities, infections, placental insufficiency, bleeding, RH incompatability

• Perinatal - Chorioamnionitis, Infection, preeclampsia, LBW, PROM, long labor, premature, meconium aspiration, asphyxia, seizures

• Postnatal - brain anomalies, anoxia to brain, multiple births, stroke, brain injury, cerebral infections, head trauma

Expected Findings Motor & Reflex - Cerebral Palsy (8)

1. Gagging or choking with feeding

2. Poor sucking

3. Consistent tongue thrusting

4. Asymmetric crawl

5. Early hand preference

6. Toe walking

7. Persistent primitive reflexes (Moro or tonic neck)

8. Hyperreflexia

Expected Findings Posturing & Muscle Tone - Cerebral Palsy (3)

Rigid posture & extremities

Arching back & stiff posture

Difficulty diapering

Associated Problems & Conditions - Cerebral Palsy (3)

• Vision, speech, or hearing impairments

• Seizures

• Cognitive/intellect impairment 

Define Spastic (Pyramidal) Cerebral Palsy (7)

• 80% of cases

• Hypertonicity (muscle tightness/spasticity)

• Increased DTRs

• Clonus & poor control of motion, balance & posture

• May present in all 4 extremities (tetraplegia), lower more than upper (diplegia), 3 limbs (triplegia), 1 limb (monoplegia), or one side of body (hemiplegia)

• Gait - crouched with scissoring leg motion & feet plantar flexed

• Babinski reflex

Define Dyskinetic (Nonspastic Extrapyramidal) Cerebral Palsy (4)

• 15% of cases

• Involuntary jerking movements that appear slow, writing & wormlike

• Dystonic - Slow, twisting movements affect trunk or extremities with abnormal posturing from muscle contractions

• Drooling & speech impairments

Define Ataxic (Nonspastic Extrapyramidal) Cerebral Palsy (3)

• Wide-Based Gait

• Lack of coordination with purposeful movement

• Poor ability to do repetitive movement

Diagnostics - Cerebral Palsy (3)

• Metabolic & genetic testing

• MRI

• Neuro assessment

Nursing Care - Cerebral Palsy (4)

• Promote independence as much as possible

• Maintain an open airway by elevating the HOB

• Ensure suction is available & suction secretions as needed

• Position upright after feeds

Medications - Cerebral Palsy (4) BAD B

• Baclofen

• Antiepileptics - Valproic acid, carbamazepine, inhibits seizures

• Diazepam

• Botulinum Toxin A

Baclofen - Cerebral Palsy (2)

• Prescribed intrathecal & administered via a surgically implanted pump

• Used as a centrally acting skeletal muscle relaxant that decreases muscle spasm & severe spasticity.

Diazepam - Cerebral Palsy (2)

• Skeletal muscle relaxant used to decrease muscle spasms & severe spasticity

• For older children & adolescents

Botulinum Toxin A - Cerebral Palsy (3)

• IM, reduces spasticity in specific muscle groups (quads)

• Primarily for clients with spasticity in lower extremities

• Decreases muscle movement by inhibiting release of acetlycholine

Complications - Cerebral Palsy

Aspiration

Potential for Injury

Aspiration - Nursing Actions Cerebral Palsy (3)

• Keep child’s head elevated.

• Keep suction available if copious oral secretions are present or the child has difficulty swallowing foods or fluids

• Teach parents CPR & feeding techniques

Potential Injury - Nursing Actions Cerebral Palsy (4)

• Make sure bed rails are raised to prevent falls from the bed.

• Pad side rails & wheelchair arms

• Secure child in mobility devices (wheelchairs)

• Use helmets, seat belts

Define Spina Bifida

NTD present at birth characterized by failure of osseous spine to close with CNS effects.

Types of Spina Bifida (4)

• Spina Bifida Occulta - Mostly affects lumbosacral area, NOT visible externally; Surface of vertebral bone is missing; no spinal cord involvement.

• Spina Bifida Cystica - Protrusion of sac is visible

• Meningocele - Sac contains spinal fluid & meninges; Increased risk for infection if ruptures. No neurologic deficits.

• Myelomeningocele (most common) - Sac contains spinal fluid, meninges, & nerves; causes decreased motor & sensory function.

Risk Factors - Spina Bifida (4)

• Medications/substances taken during pregnancy

• Maternal malnutrition & insufficient folic acid during pregnancy

• Exposure to radiation or chemicals during pregnancy

• Genetic predisposition

Expected Findings - Occulta Spina Bifida (4)

• Dimpling in lumbosacral area (occulta)

• Port wine angioma nevi (flat area of pigmentation)

• Dark hair tufts

• Subcutaneous lipoma

Expected Findings - Cystica Spina Bifida (5)

• Flaccid muscles flaccid paralysis, absent DTRs 

• Lack of bowel control

• Constant dribbling of urine or urine overflow

• Foot contractures

• Scloliosis

Labs - Spina Bifida (2)

• Maternal Blood Tests - Blood alpha-fetoprotein during the 2nd trimester of gestation indicate possible NTD.

• Infant Blood Tests - Determine causative pathogen if appropriate.

Prenatal Diagnostics - Spina Bifida (3)

• Ultrasound - Visual defects

• Amniocentesis - Confirmation, done after elevated alpha-fetoprotein to detect anencephaly or myelomeningocele

• CVS

Nursing Care - Spina Bifida (5)

• Assess the sac (cystica).

• Perform a routine newborn assessment.

• Obtain accurate output measurements.

• Assess head circumference and fontanels.

• Prevent infection

Therapeutic Procedure - Spina Bifida (2)

• Closure of a myelomeningocele sac is done ASAP to prevent complications of injury and infection.

• Risk for the development of hydrocephalus.

Pre Op Nursing Actions - Spina Bifida (9)

1. Prep for surgery within first 24 to 72 hr after birth

2. Protect sac from injury

3. Place in incubator or warmer without clothing

4. Apply a sterile, moist,non-adhering dressing with sterile NS on sac & change q2h

5. Add more sterile solution if dressing dries, do not remove

6. Inspect sac for leaks, irritation, abrasions, infection signs

7. Place in prone position & give antibiotics as prescribed

8. Avoid rectal temps & putting pressure on sac

9. Measure head circumference to establish baseline (ICP)

Post OP Nursing Actions - Spina Bifida (5)

• Vitals, I&O, weight

• Provide Incision Care - Keep clean, no urine or feces contamination

• Assess for CSF leakage & S/S of increased ICP

• Maintain prone position unless otherwise prescribed

• Provide ROM to extremities

Latex Allergy - Spina Bifida (4)

• Child is at an increased risk for a latex allergy

• Avoid exposing the child to latex.

• Be aware of household items that can contain latex (water toys, pacifiers, plastic storage bags).

• Linked to foods - Bananas, kiwi, avocado, chestnuts

Increased ICP - Spina Bifida (2)

• Caused by shunt malfunction or hydrocephalus.

• Prepare for surgery for shunt or shunt revision.

Infant ICP Findings - Spina Bifida (5)

1. High-pitched cry

2. Lethargy

3. Vomiting

4. Bulging fontanels &/or widening cranial suture lines

5. Increased head circumference

Define Juvenile Idiopathic Arthritis (2)

• Chronic autoimmune inflammatory disease affecting joints & other tissues.

• Chronic inflammation of synovium of joints leads to wearing down & damage to articular cartilage.

Expected Findings - Juvenile Idiopathic Arthritis (5)

• Joint swelling & stiffness (worse in morning or after inactivity)

• Mobility limitations

• Fever & rash

• Gait with a limp

• Delayed growth

Nursing Care - Juvenile Idiopathic Arthritis (4)

• Goal - Control pain, minimize damage from inflammation, perserve joint function & promote normal growth & development

• Encourage participation in PT to increase mobility & prevent deformities

• Encourage daily physical activity & full ROM exercises as tolerated

• Apply heat or warm moist packs to affected joints

Medications - Juvenile Idiopathic Arthritis (4)

• NSAIDs - Ibuprofen, naproxen, diclofenac; Pain & inflammation

• Disease-Modifying Antirheumatic Drugs (DMARDs) - Methotrexate

• Biologic DMARDs - Etanercept

• Glucocorticoids

NSAIDs - Juvenile Idiopathic Arthritis (2)

• Take as directed & watch for overdose

• Often prescribed 4x/day for 6-8 weeks even if feeling better

DMARDs - Juvenile Idiopathic Arthritis (5)

1. Methotrexate

2. Slows joint degeneration & progression of rheumatoid arthritis when NSAIDs do not work alone.

3. Administered weekly in a low dose.

4. Avoid alcohol & take at bedtime to prevent nausea

5. Utilize effective birth control to avoid birth defects!!

Glucocorticoids - Juvenile Idiopathic Arthritis (6)

1. Prednisone

2. Provides relief of inflammation & pain

3. Reserved for life-threatening complication, severe arthritis, pericarditis, & uveitis

4. Short term therapy, lowest effective dose & discontinue by tapering

5. Weight gain is a common adverse effect

6. Monitor height & weight

Biologic DMARDs - Juvenile Idiopathic Arthritis (3)

• Etanercept is a tumor necrosis factor alpha-receptor inhibitor, used when methotrexate is not effective for immunosuppressive action by decreasing the inflammatory response.

• Infliximab can be given IV in outpatient setting.

• Once of twice a week by subq injection

Define Muscular Dystrophy (2)

• Group of inherited disorders with progressive degeneration of symmetric skeletal muscle groups causing progressive muscle weakness & wasting

• Leads to disability & deformity

Define Duchenne (Pseudohypertrophic) Muscular Dystrophy (DMD) (4)

• Most common form of MD

• Inherited as an X-linked recessive trait

• Onset between 3 & 5 years of age & rapid progression with life expectancy of 15-30 years.

• Fat tissue replace muscles in lower limbs (gastrocnemius).

Expected Findings - DMD (10)

1. Hypotonia & poor head control

2. Fatigue

3. Muscle weakness beggining in lower extremties

4. Unsteady gait with a waddle

5. Lordosis

6. Frequent falls

7. Gower’s Sign

8. Learning difficulties & mild cognitive delays

9. Progressive difficulty walking with loss of ability to walking occuring by age 12

10. Progressive muscle atrophy of chest, face, & neck (ANY Muscle in body)

Gower’s Sign - DMD (2)

• Difficulty getting out of bed, rising from a seated position, or climbing stairs

• Child walks hands up legs for support while going to a standing position

Labs - Muscular Dystrophy

Blood creatine kinase - Elevated VERY highly (20000) & can be elevated prior to manifestations

Nursing Care - Muscular Dystrophy (5)

1. Assess & monitor ability to perform ADLs, respiratory function (depth, rhythm, RR) during sleep & daytime hrs

2. Encourage independence as long as positive

3. Monitor SpO2 & maintain respiratory function (IS use, positioning, O2 use)

4. Encourage adequate fluid intake

5. Low calorie, high protein & fiber

Medications - Muscular Dystrophy (3)

• Corticosteroids - Prednisone increases muscle strength

• Okay to use due to low life expectancy

• Monitor for infection & avoid infectious agents

Interprofessional Care - Muscular Dystrophy (6)

• Neurologists

• Genetic conselor

• PT, OT, RT

• Dietician

• Case manager & social worker

• Psychiatrist

Complications - Muscular Dystrophy (2)

• Scoliosis

• Makes respiratory function worse may cause respiratory collapse