Neuromuscular Disorders in Children

a. Temperature instability b. Irritability c. Lethargy

The nurse is caring for an infant with myelomeningocele who is scheduled for surgical repair in the morning. Which early signs of infection should the nurse monitor on this infant? (Select all that apply.) a. Temperature instability b. Irritability c. Lethargy d. Bradycardia e. Hypertension

Frequent rest periods are needed

Nursing Care for Cerebral Palsy: § Education of prognosis: children who don't achieve ambulation by 2-7 years old likely will not develop ambulation and independence. Many children have cognitive impairments, but those with Spastic Tetraplegia often have normal intelligence. Many children remain in the home in adulthood due to disability § *Use of mobile infant walkers and door frame jumping seats should not be used. They pose injury risk to normal children and are very hazardous for children with CP. Instead, use stationary musical activity jumper § Assess developmental milestones, developmental level, family coping/support. Structure interventions around the client's developmental level § Keep HOB elevated to open airway, especially if the child has excess secretions. Ensure suction equipment is on hand to use PRN § _______________________________________________________ because more energy is spent doing ADLs § Nutrition is important (GER, feeding/swallow difficulties, constipation, anorexia). Diet tailored to activity and metabolic needs and may include gastronomy feeding · Assess aspiration risk, position upright after feedings · Provide foods that are similar to those eaten at home and high in fiber to prevent constipation · Skin-level GT is preferred · Feeding assistance with manual jaw control: middle finger of nonfeeding hand is placed posterior to the body portion of the chin, the thumb is placed below the bottom lip, and the index finger is placed parallel to the child's mandible. This helps with head control, correction of neck/trunk hyperextension, and jaw stabilization. · Practice proper dental care § Skin Care: assess skin under splints/braces, turning, keep skin clean/dry § Have children wear protective helmet if at risk for fall. Bring protective helmetor other equipment from home to hospital appointments. § Raise bed rails to prevent the child from falling out of bed. Pad side rails or wheelchairs (spasms, seizures are common with CP) § Immunizations to prevent illness (flu, pneumonia) § Children should ride in rear-facing position in car as long as possible due to poor head/neck and trunk control. § Mild-moderate CP children can usually participate in regular classes. For those unable to benefit from formal education, vocational training programs are arranged. § Recreation and after school programs: athletic, artistic programs § Encourage independence with self-care and hygiene. Educate on diaper use or self-catheterization when needed due to possible incontinence § Emotional support and coping for family. Supportive counseling, education on diagnosis, expectations for child § Care coordination for at home care with home-health nursing § Assist parents with problem-solving and help them address concerns/frustrations. Appreciate their skills and approaches in helping child. When the child is hospitalized, include family in care and ask them about routines Parental support groupsfor sharing experiences, accomplishments, problems, and concerns.Respite care for parents

a. Monitoring and maintaining systemic blood pressure. b. Administering corticosteroids. e. Monitoring for respiratory complications.

A 14 year old is in the intensive care unit after a spinal cord injury 2 days ago. Which nursing care interventions are needed for this child? (Select all that apply.) a. Monitoring and maintaining systemic blood pressure. b. Administering corticosteroids. c. Minimizing environmental stimuli. d. Discussing long-term care issues with the family. e. Monitoring for respiratory complications.

c. Folic acid for all women of childbearing age b. 0.4 mg b. prone, turn head to side, and nipple feed.

A current recommendation to prevent neural tube defects is the administration of what supplement? a. Vitamin A throughout pregnancy b. Multivitamin preparations as soon as pregnancy is suspected c. Folic acid for all women of childbearing age d. Folic acid during the first and second trimesters of pregnancy How much folic acid is recommended for women of childbearing age? a. 0.1 mg b. 0.4 mg c. 1.5 mg d. 2 mg The nurse is caring for a neonate born with a myelomeningocele. Surgery to repair the defect is scheduled the next day. The most appropriate way to position and feed this neonate is to place him: a. prone and tube feed. b. prone, turn head to side, and nipple feed. c. supine in infant carrier and nipple feed. d. supine, with defect supported with rolled blankets, and nipple feed.

A. Baclofen (Lioresal) B. Diazepam (Valium)

A nurse is caring for a child who has cerebral palsy. Which of the following medications should the nurse expect to administer to treat painful muscle spasms? (SATA) A. Baclofen (Lioresal) B. Diazepam (Valium) C. Oxybutynin chloride (Ditropan) D. Methotrexate (Rheumatrex) E. Prednisone (Deltasone)

C. Muscular weakness in lower extremities D. Unsteady, wide‑based or waddling gait

A nurse is caring for a child who has muscular dystrophy. For which of the following findings should the nurse assess? (Select all that apply.) A. Purposeless, involuntary, abnormal movements B. Spinal defect and saclike protrusion C. Muscular weakness in lower extremities D. Unsteady, wide‑based or waddling gait E. Upward slant to the eyes

B. Evaluate the toddler's need for an evaluation of hearing ability. D. Apply a sterile, moist dressing on the sac. a. Hypertonicity and poor control of posture, balance, and coordinated motion

A nurse is developing a plan of care for a toddler who has cerebral palsy. Which of the following actions should the nurse include? A. Structure interventions according to the toddler's chronological age. B. Evaluate the toddler's need for an evaluation of hearing ability. C. Monitor the toddler's pain level routinely using a numeric rating scale. D. Provide total care for daily hygiene activities. A nurse is caring for an infant who has a myelomeningocele. Which of the following actions should the nurse include in the preoperative plan of care? A. Assist the mother with cuddling the infant. B. Assess the infant's temperature rectally. C. Place the infant in a supine position. D. Apply a sterile, moist dressing on the sac. Spastic cerebral palsy is characterized by what presentation? a. Hypertonicity and poor control of posture, balance, and coordinated motion b. Athetosis and dystonic movements c. Wide-based gait and poor performance of rapid, repetitive movements d. Tremors and lack of active movement

a. Recommending genetic counseling d. "There may be a variety of different causes." d. Visible defect with an external sac-like protrusion containing meninges, spinal fluid, and nerves.

A young boy has just been diagnosed with pseudohypertrophic muscular dystrophy. The management plan should include which intervention? a. Recommending genetic counseling b. Explaining that the disease is easily treated c. Suggesting ways to limit the use of muscles d. Assisting the family in finding a nursing facility to provide his care What is the most appropriate nursing response to the father of a newborn infant with myelomeningocele who asks about the cause of this condition? a. "One of the parents carries a defective gene that causes myelomeningocele." b. "A deficiency in folic acid in the father is the most likely cause." c. "Offspring of parents who have a spinal abnormality are at greater risk for myelomeningocele." d. "There may be a variety of different causes." Which statement best describes a myelomeningocele? a. Fissure in the spinal column that leaves the meninges and the spinal cord exposed. b. Herniation of the brain and meninges through a defect in the skull. c. Hernial protrusion of a sac-like cyst of meninges with spinal fluid but no neural elements. d. Visible defect with an external sac-like protrusion containing meninges, spinal fluid, and nerves.

Dental hygiene, dental visits, brushing, fluoride, and flossing routinely

Complications & Special Needs of Cerebral Palsy: § __________________________________________________________ to decrease tartar build-up if limited PO intake and especially if taking phenytoin for ataxia due to gingival hyperplasia § Constipation can be caused by neuro deficit and lack of exercise § Poor bladder control and urinary retention § PT vest for chest physiotherapy to mobilize secretions and minimize chronic respiratory infections § Aspiration (& aspiration pneumonia) may result from GER, abnormal muscle tone, immobility, and altered positioning § skin care due to pressure areas, malalignment, poor bracing, nutrition, and immobility § Potential for injury is increased due to muscle impairment, so safe environments need to be implemented § Osteopenia can occur due to immobility and decreased exercise § SLP, Computerized toys, games, and "speaking" devices for hand/eye coordination, and improvement of speech difficulties § Sensors activated by using head stick or tongue or other voluntary movement. Voice-activated computers § Neuromuscular electrical stimulation devices (NMES) and splinting for increased muscle strength, range, and function being investigated § ST/OT/PT § Behavioral and learning therapy if ADHD or learning difficulties § Transitional care decisions when older

relieve pain from muscle spasms

Medications for Cerebral Palsy: § Dantrolene, baclofen and diazepam (older children and adolescents) to _______________________________________________ · Baclofen side effects: weakness, fatigue, sweating, HA, nausea, constipation, hepatotoxicity · Diazepam side effects: drowsiness, fatigue, hepatotoxicity · Side effects: hepatotoxicity (dantrolene), drowsiness, fatigue, muscle weakness, CNS depression, hypotension, sweating, constipation § Botulinum toxin A (Botox) to reduce spasticity in targeted muscles, especially in children with spasticity confined to lower extremities · Injection Side effects: pain at injection site, temporary weakness · Oral Side effects: drowsiness, confusion § Implantable intrathecal baclofen pumps for children with severe spasticity that interferes with ADLs. Patients must first be screened with a "test dose" via lumbar puncture (monitor for hypotonia, somnolence, seizures, n/v, HA) · Benefits: fewer side effects, dosage titration for max effects, reversibility of therapy with removal of pump if desired · Outpatients visits to refill pump every 3-6 months · Abrupt withdrawal of intrathecal baclofen results in rebound spasticity, itching, hyperthermia, rhabdomyolysis, DIC, multiorgan failure, and death § Antileptics such as carbamazepine, valproic acid, divalproex, levetiracetam, oxcarbazepine, and lamotrigine for seizures § Levodopa/carbidopa or trihexyphenidyl for dystonia § Reserpine for hyperkinetic and movement disorders, chorea, athetosis § gabapentin for decreasing spasticity pain

Dystrophin

Muscular Dystrophy (Duchenne) · Muscular Dystrophy: these disorders have a genetic origin in which there is gradual degeneration of muscle fibers characterized by progressive weakness and wasting of symmetric groups of skeletal muscles, with increasing disability and deformity. · Duchenne Muscular Dystrophy (Pseudohypertrophic): Most severe muscular dystrophy characterized by an X-linked recessive gene defect usually with family history, males almost exclusively affected, and female carriers § ________________________ (protein product in muscles) is absent from the muscles

Myelomeningocele

Neural Tube Defects · Rachischisis or Spina Bifida: midline defect with failure of osseous body (spine) to close (most common) (fissure in the spinal column that leaves the meninges and spinal cord exposed) § Spina Bifida Cystica: visible defect with saclike protrusion of meninges, spinal fluid, and nerves with varying degrees of neuromuscular, limb, and sensory deficits § Meningocele: hernial protrusion of a saclike cyst of meninges filled with spinal fluid. It is not associated with neurologic deficit § _________________________________________________: hernia protrusion of a saclike cyst containing meninges, spinal fluid, and a portion of the spinal cord with its NERVES. It is associated with varying degrees of neurologic deficit

Spina Bifida Cystica

Neural Tube Defects · Rachischisis or Spina Bifida: midline defect with failure of osseous body (spine) to close (most common) (fissure in the spinal column that leaves the meninges and spinal cord exposed) § ___________________________: visible defect with saclike protrusion of meninges, spinal fluid, and nerves with varying degrees of neuromuscular, limb, and sensory deficits

Spina Bifida Occulta

Neural Tube Defects · Rachischisis or Spina Bifida: midline defect with failure of osseous body (spine) to close (most common) (fissure in the spinal column that leaves the meninges and spinal cord exposed) § ________________________________________________: a defect that is not visible externally; it usually occurs at L4 and L5 vertebrae and may be asymptomatic. It is often noticed when there are associated cutaneous manifestations or neuromuscular disturbances · S/S: skin depression or dimple, port-wine angiomatous nevi, dark tufts of hair, soft/cutaneous lipomas, progressive gait disturbance and foot weakness, bowel/bladder sphincter disturbances

independence with self-care and hygiene. Educate on diaper use or self-catheterization when needed due to possible incontinence

Nursing Care for Cerebral Palsy: § Education of prognosis: children who don't achieve ambulation by 2-7 years old likely will not develop ambulation and independence. Many children have cognitive impairments, but those with Spastic Tetraplegia often have normal intelligence. Many children remain in the home in adulthood due to disability § *Use of mobile infant walkers and door frame jumping seats should not be used. They pose injury risk to normal children and are very hazardous for children with CP. Instead, use stationary musical activity jumper § Assess developmental milestones, developmental level, family coping/support. Structure interventions around the client's developmental level § Keep HOB elevated to open airway, especially if the child has excess secretions. Ensure suction equipment is on hand to use PRN § Frequent rest periods are needed because more energy is spent doing ADLs § Nutrition is important (GER, feeding/swallow difficulties, constipation, anorexia). Diet tailored to activity and metabolic needs and may include gastronomy feeding · Assess aspiration risk, position upright after feedings · Provide foods that are similar to those eaten at home and high in fiber to prevent constipation · Skin-level GT is preferred · Feeding assistance with manual jaw control: middle finger of nonfeeding hand is placed posterior to the body portion of the chin, the thumb is placed below the bottom lip, and the index finger is placed parallel to the child's mandible. This helps with head control, correction of neck/trunk hyperextension, and jaw stabilization. · Practice proper dental care § Skin Care: assess skin under splints/braces, turning, keep skin clean/dry § Have children wear protective helmet if at risk for fall. Bring protective helmetor other equipment from home to hospital appointments. § Raise bed rails to prevent the child from falling out of bed. Pad side rails or wheelchairs (spasms, seizures are common with CP) § Immunizations to prevent illness (flu, pneumonia) § Children should ride in rear-facing position in car as long as possible due to poor head/neck and trunk control. § Mild-moderate CP children can usually participate in regular classes. For those unable to benefit from formal education, vocational training programs are arranged. § Recreation and after school programs: athletic, artistic programs § Encourage _______________________________________________________________________________________ § Emotional support and coping for family. Supportive counseling, education on diagnosis, expectations for child § Care coordination for at home care with home-health nursing § Assist parents with problem-solving and help them address concerns/frustrations. Appreciate their skills and approaches in helping child. When the child is hospitalized, include family in care and ask them about routines Parental support groupsfor sharing experiences, accomplishments, problems, and concerns.Respite care for parents

banana, avocado, kiwi, chestnut, milk

Nursing Care of Children with Neural Tube Defect: -Support the family and Educate about Home Care for Spina Bifida: § Support for home, education, vocational assistance, and transitional care § Teach positioning, skin care, feeding, ROM, CIC, and signs of complications (urinary, neuro, ortho, latex allergies) § Monitor for bladder dysfunction, infection, blood in urine. Administer antispasmodics or perform intermittent catheterization PRN. Stoma care PRN § Monitor for constipation, administer laxatives or fiber supplements. Give antegrade continence enemas (ACE) every 1-2 days PRN for older children to empty bowels § For orthopedic issues (scoliosis, clubfoot), give pain meds, prepare for surgery if needed, provide cast care, check for neuro deficits, and perform ROM exercises, positioning, and stretching exercises. § Often, the life expectancy for children can be long into adulthood, so educate on long-term goals, vocational training, education, living independently, having a mate, sexual relationships, and bearing children § Help children problem-solve and self-help for locomotion, urinary/bowel incontinence, and preparing for school § Address body image concerns and support the family/patient § Help family with obtaining medial equipment/resources needed at home -Monitor for signs of latex allergy and institute latex-free environment: § Children with SB are at high risk for latex allergy due to repeated exposure during surgery in early life § Children should not be exposed to latex from birth onward § S/S: urticaria, wheezing, watery eyes, rashes, anaphylactic shock § Monitor for cross reactions to foods: __________________________________________________________ § Always ask all patients, not just those with SB, about latex allergy § Carry ID bracelet Common items to avoid that may contain latex:water toys, pacifiers, plastic bags, floral bouquets, balloons, adhesive tapes (IVs), gloves

STERILE, MOIST, NONADHERENT DRESSING (NS). Dressings are changed q2-4h with inspection of the sac for leaks, abrasions, irritation, or infection. If contaminated by urine/stool, the sac must be cleaned

Nursing Care of Children with Neural Tube Defect: · At birth, examine for a membranous cyst and carefully prevent trauma to the area during assessment/transport. Assessment of neurologic involvement (neurologic status), movement of limbs, skin response, and anal reflex to determine motor/sensory impairment · Monitor for urine and stool output (if remaining dry and distended, it could indicate retention) · Monitor head circumference daily and assess fontanels for bulging/tension · Care of the Myelomeningocele Sac: § Place infant in incubator or warmer to maintain temperature (they can't wear clothing or covers yet because it could irritate the sac). Dressings over the defect need frequent moistening due to dehydrating effects of warmer § The sac is prevented from drying using a _________________________________________________________________________________________ § Signs of infection: temperature instability, irritability, lethargy, § Avoid rectal temperatures with spina bifida because the bowel sphincter is often affected and the thermometer can cause irritation or prolapse § Observe for early signs of infection or ↑ICP § Prone positioning before and after surgical repair. This minimizes tension on the sac, risk of trauma, and helps with hip dysplasia. Legs are maintained in abduction with pads between knees to counteract hip subluxation. A roll is kept under the ankles for neutral foot position.

oral breastfeeding and early feeding after surgery

Nursing Care of Children with Neural Tube Defect: · Postoperative Care for Spina Bifida Repair: § Vitals, I/O, nourishment, monitor for infection, managing pain § Care of operative site is done under supervision of surgeon. Monitor for CSF leakage, keep incision clean and do not allow feces/urine to contaminate it. Use paper tape § Prone position is maintained. Some physicians allow side-lying if no hip dysplasia. § If permitted, the child can be held upright against the body with care to avoid pressure on incision § Resume _________________________________________________________________________ § Monitor for increased ICP (high-pitched cry, lethargy, vomiting, bulging fontanels, wide cranial suture lines, increasing head circumference; children show HA, leathery, n/v, double vision, decreasing school performance, decreased LOC, seizures) · Use gentle motions when performing ROM exercises, decrease environmental stimuli (noise, visitors), manage pain

caressing, stroking, touching, and other comfort measures are used for tactile stimulation and developmental care.

Nursing Care of Children with Neural Tube Defect: · Pre-op Care Spina Bifida Repair: Prevent Complications § Prepare the family for surgery (within 24-72 hr after birth). Protect the sac from injury. Measure head circumference for baseline measurement § Apply a sterile, moist, non-adherent dressing with NS on the sac and change it q2h. If it becomes dry, do not remove it. Apply more NS § Prone position makes it hard to keep baby clean, prevent pressure ulcers, and feed. The infants head is turned to the side for feedings § Diapering may be contraindicated until defect is repaired and healing is advanced. Padding beneath the diaper area is changed PRN to keep skin clean and dry. If urinary retention is present, clean intermittent catheterization (CIC) is used. § To prevent stool contamination of SB defect, obtain a surgical drape, cut the portion of the drape to fit the infant's sacrum, and secure the drape using non-latex tape. Place the rest of the drape loosely over the dressing, covered the defect and preventing exposure to stool § Use special mattresses to prevent skin breakdown on knees/ankles. Cleaning, lotion, and gentle massage aid circulation. § Assess for s/s of infection: irritability, fever, lethargy. Antibiotics as ordered § Gentle ROM and stretching to prevent contractions. Consult PT § Cuddling the infant pre-op is contraindicated, so _________________________________________________________________________________

higher level lesion without use of any of the 4 extremities; can involve phrenic nerve → diaphragmatic paralysis

Spinal Cord Injuries (SCIs) · The injury sustained can affect any of the spinal nerves. The higher the injury, the more extensive the damage. § Paraplegia: complete or partial paralysis of lower extremities § Tetraplegia: ______________________________________________

a. Continue to perform the clean intermittent catheterizations (CIC) at home. b. Administer the oxybutynin chloride as prescribed. d. Monitor for signs of a recurrent UTI.

The nurse is conducting discharge teaching with parents of a preschool child with a myelomeningocele, repaired at birth, who is being discharged from the hospital after a urinary tract infection (UTI). Which should the nurse include in the discharge instructions related to management of the child's genitourinary function? (Select all that apply.) a. Continue to perform the clean intermittent catheterizations (CIC) at home. b. Administer the oxybutynin chloride as prescribed. c. Reduce fluid intake in the afternoon and evening hours. d. Monitor for signs of a recurrent UTI. e. Administer furosemide as prescribed.

d. "My child should not eat bananas or kiwis." a. Avoiding using any latex product b. Positive Babinski reflex

The nurse is talking to a parent with a child who has a latex allergy. Which statement by the parent would indicate a correct understanding of the teaching? a. "My child will have an allergic reaction if he comes in contact with yeast products." b. "My child may have an upset stomach if he eats a food made with wheat or barley." c. "My child will probably develop an allergy to peanuts." d. "My child should not eat bananas or kiwis." Latex allergy is suspected in a child with spina bifida. What intervention should be included in the child's plan of care? a. Avoiding using any latex product b. Using only nonallergenic latex products c. Administering medication for long-term desensitization d. Teaching the family about long-term management of asthma Which finding should cause the nurse to suspect a diagnosis of spastic cerebral palsy? a. Tremulous movements at rest and with activity b. Positive Babinski reflex c. Writhing, uncontrolled, involuntary movements d. Clumsy, uncoordinated movements

d. Implantation of a pump to deliver medication into the intrathecal space to decrease spasticity has recently become available. a. Neurogenic bladder a. Hydrocephalus

The parents of a child diagnosed with cerebral palsy ask the nurse if any drugs can decrease their child's spasticity. The nurse's response should be based on what knowledge? a. Anticonvulsant medications are sometimes useful for controlling spasticity. b. Medications that would be useful in reducing spasticity are too toxic for use with children. c. Many different medications can be highly effective in controlling spasticity. d. Implantation of a pump to deliver medication into the intrathecal space to decrease spasticity has recently become available. What is the most common problem for children born with a myelomeningocele? a. Neurogenic bladder b. Intellectual impairment c. Respiratory compromise d. Cranioschisis Which problem is most often associated with myelomeningocele? a. Hydrocephalus b. Craniosynostosis c. Biliary atresia d. Esophageal atresia

neurogenic bladder, urostomy or vesicostomy, and self-catheterization for prevention of UTI

Therapeutic Management of Neural Tube Defects: § When identified before birth, C-section is preferred way of birth § Surgical closure within 24 hours if sac ruptured/leaking CSF or within 24-72 if intact to prevent infection and trauma. The goal of surgery is adequate skin coverage of the lesion and meticulous closure § Surgical placement of VP shunt if hydrocephalus is present. § Surgical correction of Chiari II Malformation if present and infant symptomatic (high-pitched cry, stridor, respiratory distress, apnea, oral-motor difficulties, upper extremity spasticity) § Antibiotics for ventriculitis, meningitis, UTI, pneumonia § Interventions during Infancy: · Prevent infection, broad spectrum antibiotics · Careful neuro assessment to identify deficits/anomalies early § Orthopedic management to prevent joint contractures, correct deformities, prevent/minimize effects of motor/sensory deficits, prevention of skin breakdown, and obtaining the best possible function of affected lower extremities · Monitor for deformities of the hips, knees, feet, spine (kyphosis and scoliosis), insensate skin (SKIN CARE) · Braces, "walking" deices, wheelchairs for mobility · Early and regular PT with passive ROM exercises for positioning and stretching beginning in infancy to prevent contractures § Management of urologic problems: _________________________________________________________ · Ongoing urologic monitoring for chronic UTI, nephrosis, reflux, or renal insufficiency · Prompt treatment of UTI, regularly emptying the bladder (catheterization) · Medication to improve bladder storage and continence such as oxybutynin (Ditropan) and tolterodine (Detrol) § Managing Bowel Control: High fiber diet, laxatives, enemas, suppositories, bowel habit training, or surgical procedures to achieve bowel continence and prevent constipation/impaction/incontinence · Dietary fiber supplements: 10g/day Surgically placed antegrade continence enema that is flushed every 1-2days

High-dose steroids (methylpredisone) IV

Therapeutic Management of Spinal Cord Injuries (SCIs) § Immobilize cervical, thoracic, and lumbar spine (C-collar, back board with strapping) § ABC'S: AIRWAY OPENED WITH JAW-THRUST MANEUVER. § Advanced airway support if ineffective or absent respiratory effort § Monitor cardiac output. With MVAs, organ damage and internal bleeding may cause ABD distention, so interventions are taken to prevent systemic shock (BP support) § Assess for other internal or external injuries § After child is at trauma center, spinal is stabilized, neuro assessment is performed, CT scans, MRIs § ________________________________________________ in first 12 hours to decrease inflammation/edema and minimize further injury and long-term effects § Consult and planning for early rehabilitation, orthoses, and ambulation aids · Functional electrical stimulation (FES): electrical stimulator is placed under skin in ABD and electrode leads to paralyzed leg muscles, enabling the child to sit, stand, and walk with the aid of crutches, walker, or orthoses. Often requires surgical tendon transfers, which also helps restore hand or arm function (ADLs, self-catheterization) § SCI rehabilitation: exercise § Clonidine may be used to improve ambulation in patients with partial SCIs Surgical cord decompression or cervical/thoracic fusion

a. Lordosis b. Gower's sign e. Waddling gait

Which assessment findings should the nurse note in a school-age child diagnosed with Duchenne's muscular dystrophy (DMD)? (Select all that apply.) a. Lordosis b. Gower's sign c. Kyphosis d. Scoliosis e. Waddling gait

c. Progressive weakness and wasting of skeletal muscle b. Covered with a sterile, moist, nonadherent dressing b. Assessing respiratory efforts d. Administer gentamicin sulfate 10 mg per intravenous piggyback every 12 hours.

Which signs and symptoms are associated with Werdnig-Hoffmann disease? a. Spinal muscular atrophy b. Neural atrophy of muscles c. Progressive weakness and wasting of skeletal muscle d. Pseudohypertrophy of certain muscle groups Which interventions should the nurse include in the plan of care for the infant awaiting surgical closure of a myelomeningocele sac? a. Open to air b. Covered with a sterile, moist, nonadherent dressing c. Reinforcement of the original dressing if drainage noted d. A diaper secured over the dressing The nurse is admitting a school-age child with suspected Guillain-Barré syndrome (GBS). Which nursing intervention is a priority in the care for this child? a. Monitoring intake and output b. Assessing respiratory efforts c. Placing on a telemetry monitor d. Obtaining laboratory studies The nurse is caring for an intubated infant with botulism. Which health care provider prescriptions should the nurse clarify with the health care provider before implementing? a. Administer 250 mg botulism immune globulin intravenously (BIG-IV) one time. b. Provide total parenteral nutrition (TPN) at 25 mL/hr intravenously. c. Titrate oxygen to keep pulse oximetry saturations greater than 92. d. Administer gentamicin sulfate 10 mg per intravenous piggyback every 12 hours.

prolonged shortening of the muscle or other soft tissue around a joint, preventing movement of the joint

Words You Need to Know · Hypertonia/Rigidity: abnormal increase in smooth or skeletal muscle tone · Hyperreflexia: abnormal response to a stimulus applied to the sensory components of the nervous system and may take the form of increased, decreased, or absent reflexes · Autonomic Dysreflexia: syndrome associated with damage to the spinal cord above the mid thoracic level characterized by a marked increase in the sympathetic response to minor stimuli such as bladder or rectal distention · Atrophy: derangement in size and number of muscle fibers occurring with aging, reduction in blood supply, or following immobilization, prolonged weightlessness, malnutrition, and particularly in denervation · Clonus: involuntary shock-like contractions, irregular in rhythm and amplitude, followed by relaxation, of a muscle or a group of muscles · Ataxic: impairment of the ability to perform smoothly coordinated voluntary movements, which may affect the limbs, trunk, eyes, pharynx, larynx, and other structures · Spastic: involuntary contraction of a muscle or group of skeletal or smooth muscles · Dyskinetic: abnormal involuntary movements which primarily affect the extremities, trunk, or jaw that occur as a manifestation of an underlying disease process · Contracture: __________________________________________________________

polymerase chair reaction (PCR)

o Clinical Presentation of Duchenne Muscular Dystrophy (Pseudohypertrophic): § Usually normal G&D but can have subtle delays § Evidence of progressive muscle weakness, wasting, and contractures by age 3-7 § Difficulty riding bicycles, running, or climbing stairs are s/s often first noticed § Enlarged muscles in calves, thighs, and upper arms (pseudohypertrophy) due to fatty infiltrates (this often feels firm or woody on palpation) § Waddling gait, lordosis, falling frequently, Grower sign (a characteristic manner of rising from squatting or sitting on the floor) § Profound muscle atrophy, contractures, and deformities, often with eventual inability to ambulate by age 12 § Immobility increases complications: osteoporosis, fractures, constipation, skin breakdown, psychosocial/behavioral problems § Slowly progressive, generalized weakness during teenage years § Atrophy of facial, oropharyngeal and respiratory muscles in late stages → death § Obesity is a common complication that leads to premature loss of ambulation. Children with restricted opportunities get bored and eat more. Proper dietary intake and diversified recreational program helps reduce obesity Mild to moderate cognitive impairment.Deficient dystrophin in the brain causes impairment, so they should be involved in early learning programs and regular classrooms as much as possible · Diagnosis of Duchenne Muscular Dystrophy (Pseudohypertrophic): § Serum blood _____________________________________________ positive for dystrophin gene mutation § Prenatal diagnosis via genetic testing by 12 weeks § Muscle biopsy and EMG § Serum CK levels high in first two years before signs of weakness

refill pump every 3-6 months

o Medications for Cerebral Palsy: § Dantrolene, baclofen and diazepam (older children and adolescents) to relieve pain from muscle spasms · Baclofen side effects: weakness, fatigue, sweating, HA, nausea, constipation, hepatotoxicity · Diazepam side effects: drowsiness, fatigue, hepatotoxicity · Side effects: hepatotoxicity (dantrolene), drowsiness, fatigue, muscle weakness, CNS depression, hypotension, sweating, constipation § Botulinum toxin A (Botox) to reduce spasticity in targeted muscles, especially in children with spasticity confined to lower extremities · Injection Side effects: pain at injection site, temporary weakness · Oral Side effects: drowsiness, confusion § Implantable intrathecal baclofen pumps for children with severe spasticity that interferes with ADLs. Patients must first be screened with a "test dose" via lumbar puncture (monitor for hypotonia, somnolence, seizures, n/v, HA) · Benefits: fewer side effects, dosage titration for max effects, reversibility of therapy with removal of pump if desired · Outpatients visits to ______________________________________________________ · Abrupt withdrawal of intrathecal baclofen results in rebound spasticity, itching, hyperthermia, rhabdomyolysis, DIC, multiorgan failure, and death § Antileptics such as carbamazepine, valproic acid, divalproex, levetiracetam, oxcarbazepine, and lamotrigine for seizures § Levodopa/carbidopa or trihexyphenidyl for dystonia § Reserpine for hyperkinetic and movement disorders, chorea, athetosis § gabapentin for decreasing spasticity pain

if present and infant symptomatic (high-pitched cry, stridor, respiratory distress, apnea, oral-motor difficulties, upper extremity spasticity)

o Therapeutic Management of Neural Tube Defects: § When identified before birth, C-section is preferred way of birth § Surgical closure within 24 hours if sac ruptured/leaking CSF or within 24-72 if intact to prevent infection and trauma. The goal of surgery is adequate skin coverage of the lesion and meticulous closure § Surgical placement of VP shunt if hydrocephalus is present. § Surgical correction of Chiari II Malformation _________________________________________________________ § Antibiotics for ventriculitis, meningitis, UTI, pneumonia § Interventions during Infancy: · Prevent infection, broad spectrum antibiotics · Careful neuro assessment to identify deficits/anomalies early § Orthopedic management to prevent joint contractures, correct deformities, prevent/minimize effects of motor/sensory deficits, prevention of skin breakdown, and obtaining the best possible function of affected lower extremities · Monitor for deformities of the hips, knees, feet, spine (kyphosis and scoliosis), insensate skin (SKIN CARE) · Braces, "walking" deices, wheelchairs for mobility · Early and regular PT with passive ROM exercises for positioning and stretching beginning in infancy to prevent contractures § Management of urologic problems: neurogenic bladder, urostomy or vesicostomy, and self-catheterization for prevention of UTI · Ongoing urologic monitoring for chronic UTI, nephrosis, reflux, or renal insufficiency · Prompt treatment of UTI, regularly emptying the bladder (catheterization) · Medication to improve bladder storage and continence such as oxybutynin (Ditropan) and tolterodine (Detrol) § Managing Bowel Control: High fiber diet, laxatives, enemas, suppositories, bowel habit training, or surgical procedures to achieve bowel continence and prevent constipation/impaction/incontinence · Dietary fiber supplements: 10g/day Surgically placed antegrade continence enema that is flushed every 1-2days

changes in white matter or other anomalies

§ Assessment of Early Signs of Cerebral Palsy: · Diagnosis cannot be confirmed until age two because motor tone abnormalities can be related to other disorders · Failure to meet developmental milestones, like rolling over, raising head, crawling · Persistence of primitive reflexes (Babinski reflex) · Poor head control (head lag) and clenched fists after 3 months of age · Stiff/rigid arms or legs; scissoring legs · Pushing away or arching back, stiff posture · Floppy or limp body posture, especially while sleeping · Inability to sit up without support by 8 months of age · Using only one side of the body or arms to crawl · Feeding difficulties or persistent gagging/choking while feeding · After 6 months, tongue pushing soft food out of mouth · Extreme irritability or crying · Failure to smile by 3 months · Lack of interest in surroundings § Diagnostics for Cerebral Palsy: · Neurologic exams and history of neuromotor dysfunction · CT/MRI, EEG, skull X-ray to identify ________________________________________ · Metabolic and genetic testing Examination of persistent reflexes

Passive ROM and orthoses PRN to prevent contractures. Eventually as diseases stabilizes, active PT is implemented to facilitate recovery (active exercise, gait training, bracing)

· Care Management for Guillain-Barré Syndrome (Infectious Polyneuritis): § Ventilator support or temporary tracheostomy in acute phase because of respiratory and pharyngeal involvement § IVIG within first two weeks of symptoms § Possible course of steroids § Plasmapheresis (side effects: hypotension, fever, bleeding disorders, chills, urticaria, bradycardia) § immunosuppressive medications § Laxative or stool softeners to prevent constipation § Low-molecular weight heparin, SCDs, compression stockings, early mobilization/ambulation to prevent DVT § Gabapentin or carbamazepine for residual neuropathic pain; acetaminophen for other pain § H2 antagonists or PPIs for prevention of GI ulcers § PT/OT/ST rehabilitation · Nursing Care for Guillain-Barré Syndrome (Infectious Polyneuritis): § Recovery of muscle strength progresses in reverse order of onset of paralysis, with lower extremity strength being last to recovery. Most patients regain full muscle strength, but those with cranial nerve involvement, severe disability at diagnosis, and intubation. § Frequent neuro checks, vitals, and LOC § Frequent assessment of respiratory status, swallowing ability. If intubated, child requires close monitoring of O2, ABGs, patent airway, and postural changes to prevent pneumonia. § Keep O2, insufflation bag and mask, ET intubation and suctioning equipment, tracheostomy tray, and vasoconstrictor drugs available at all times § Suction (strict asepsis) to keep airway patent, HOB elevated of 30 degrees, frequent mouth care § NG or OG feedings for children with oral/pharyngeal involvement. Monitor for decreased gastric emptying, constipation, and feeding residuals (immobilization = less GI motility) § Temporary catheterization for urinary retention and monitoring output § Skin care due to sensory impairment/paralysis in lower limbs § _________________________________________________________________ § Communicate to the child regarding procedures and treatments, especially ventilation

IVIG

· Care Management for Guillain-Barré Syndrome (Infectious Polyneuritis): § Ventilator support or temporary tracheostomy in acute phase because of respiratory and pharyngeal involvement § ____________________________________ within first two weeks of symptoms § Possible course of steroids § Plasmapheresis (side effects: hypotension, fever, bleeding disorders, chills, urticaria, bradycardia) § immunosuppressive medications § Laxative or stool softeners to prevent constipation § Low-molecular weight heparin, SCDs, compression stockings, early mobilization/ambulation to prevent DVT § Gabapentin or carbamazepine for residual neuropathic pain; acetaminophen for other pain § H2 antagonists or PPIs for prevention of GI ulcers § PT/OT/ST rehabilitation · Nursing Care for Guillain-Barré Syndrome (Infectious Polyneuritis): § Recovery of muscle strength progresses in reverse order of onset of paralysis, with lower extremity strength being last to recovery. Most patients regain full muscle strength, but those with cranial nerve involvement, severe disability at diagnosis, and intubation. § Frequent neuro checks, vitals, and LOC § Frequent assessment of respiratory status, swallowing ability. If intubated, child requires close monitoring of O2, ABGs, patent airway, and postural changes to prevent pneumonia. § Keep O2, insufflation bag and mask, ET intubation and suctioning equipment, tracheostomy tray, and vasoconstrictor drugs available at all times § Suction (strict asepsis) to keep airway patent, HOB elevated of 30 degrees, frequent mouth care § NG or OG feedings for children with oral/pharyngeal involvement. Monitor for decreased gastric emptying, constipation, and feeding residuals (immobilization = less GI motility) § Temporary catheterization for urinary retention and monitoring output § Skin care due to sensory impairment/paralysis in lower limbs § Passive ROM and orthoses PRN to prevent contractures. Eventually as diseases stabilizes, active PT is implemented to facilitate recovery (active exercise, gait training, bracing) § Communicate to the child regarding procedures and treatments, especially ventilation

Dyskinetic (nonspastic, EPS)

· Classification of Cerebral Palsy: § _______________________________________ Cerebral Palsy: · Athetoid: chorea; slow, wormlike, writhing movements in extremities, trunk, neck, facial muscles, and tongue · Dystonic: slow, twisting movements of trunk/extremities, abnormal posture and muscle contractions · Involvement of pharyngeal, laryngeal, and oral muscles causing drooling and dysarthria (impaired speech) § Ataxic (nonspastic, EPS) Cerebral Palsy: · Wide-based gait and difficulty with coordination · Poorly performs rapid or repetitive movements · Disintegration of movements of the upper extremities when the child reaches for objects § Mixed Type Cerebral Palsy: Combination of spastic and dyskinetic CP

Spastic (pyramidal)

· Classification of Cerebral Palsy: § _____________________________________________ Cerebral Palsy: persistent primitive reflexes, positive Babinski reflex, ankle clonus, exaggerated stretch reflexes, eventual development of contractions · gait can appear crouched with a scissoring motion of the legs with feet plantar flexed · Diplegia: all extremities effected, more lower than upper · Tetraplegia: all 4 extremities involved: legs and trunk, mouth, pharynx, and tongue · Triplegia: 3 limbs involved · Monoplegia: one limb involved · Hemiplegia: motor dysfunction on one side of the body; upper extremity more than lower extremity · Hypertonicity with poor control of posture, balance, and coordinated motion; increased DTRs · Impairment of fine and gross motor skills

Unilateral cord lesion with a motor deficit on the opposite side of the body from the primary insult; absence of pain and temperature sensation on the opposite side of injury

· Classifications of Spinal Cord Injuries (SCIs) § American Spinal Injury Association Impairment Scale (ASIA) classifies injuries as "complete" or "incomplete" based on motor or sensory deficits below the injury (Box 49.13) · Complete: no motor or sensory function more than 3 segments below the neurologic level of injury. · Incomplete lesions have several characteristics § Complete or incomplete lesions classified as: · Central Cord Syndrome: central gray matter destruction and preservation of peripheral tracts; tetraplegia with sacral sparing common; some motor recovery gained · Anterior Cord Syndrome: complete motor and sensory loss with trunk and lower extremity proprioception and sensation of pressure · Posterior Cord Syndrome: loss of sensation, pain, and proprioception with normal cord function, including motor function; able to move extremities but have difficulty controlling movements · Brown-Séquard Syndrome: ________________________________________________________________________ · Spinal Cord Concussion: transient loss of neural function below the level of the acute spinal cord lesion, resulting in flaccid paralysis and loss of tendon, autonomic, and cutaneous reflex activity; may last hours to weeks · Spinal Cord Compression: mild but frightening form of cord trauma, characterized by a temporary neural dysfunction without visible damage to the cord

Cerebral Palsy

· Clinical Manifestations of _________________________________ (at time of Diagnosis) § Delayed gross motor development · Delay in all motor accomplishments, increases as growth advances · delays in milestones are more obvious as growth advances § Abnormal motor performance · Preferential unilateral hand preference · Abnormal/asymmetric crawl; poor head control · Standing or walking on toes · Uncoordinated/involuntary movements · Poor sucking, feeding difficulties (choking/gagging), persistent tongue thrust § Alterations in muscle tone · Increased or decreased resistance to passive movement · Opisthotonos posturing: arching of back · Feels stiff on handling or dressing, difficulty in diapering (stiff arms/legs) · Rigid and unbending at the hip/knee joints when pulled to a sitting position § Abnormal postures · Maintains hips higher than trunk in prone position with legs and arms flexed under body · Scissoring and extension of the legs with feet plantarflexed in supine position · Persistent infantile resting/sleeping position · Arm abducted at shoulders, elbows flexed, hands fisted § Reflex abnormalities · Persistent of primitive infantile reflexes (positive Babinski reflex) · Obligatory tonic neck reflex at any age · Nonpersistent beyond 6 months of age · Persistence or hyperactivity or Moro, tonic neck, plantar/palmar grasp reflex · Hyperreflexia, ankle clonus, and stretch reflex elicited in many muscle groups on fast, passive movements § Associated disabilities that may or may not be present · Altered learning and reasoning · Seizures · Impaired behavioral and interpersonal/social relationships · Sensory impairment (vision, hearing, speech); strabismus

Tetanus (lockjaw)

· Manifestations of ___________________________________: § Initial: · Progressive stiffness and tenderness of muscles in neck/jaw · Difficulty opening the mouth (trismus) · Risus sardonicus (sardonic smile) caused by facial muscle spasm § Progressive involvement; · opisthotonic positioning · board like rigidity of ABD and limb muscles · difficulty swallowing · extreme sensitivity to external stimuli (slight noise, gentle touch, bright light). Triggers muscle contractions that last seconds/minutes, contractions occur with increased frequency until almost continuous (tetanic) · Laryngeal and tetany of respiratory muscles: accumulated secretions, respiratory arrest, atelectasis, pneumonia § Other S/S: mentation unaffected, pain, anxiety, distress, rapid pulse, sweating, anxious facial expression, fever absent/mild

Waddling gait, lordosis, falling frequently, Grower sign (a characteristic manner of rising from squatting or sitting on the floor),

· Clinical Presentation of Duchenne Muscular Dystrophy (Pseudohypertrophic): § Usually normal G&D but can have subtle delays § Evidence of progressive muscle weakness, wasting, and contractures by age 3-7 § Difficulty riding bicycles, running, or climbing stairs are s/s often first noticed § Enlarged muscles in calves, thighs, and upper arms (pseudohypertrophy) due to fatty infiltrates (this often feels firm or woody on palpation) § ___________________________________________________________________ § Profound muscle atrophy, contractures, and deformities, often with eventual inability to ambulate by age 12 § Immobility increases complications: osteoporosis, fractures, constipation, skin breakdown, psychosocial/behavioral problems § Slowly progressive, generalized weakness during teenage years § Atrophy of facial, oropharyngeal and respiratory muscles in late stages → death § Obesity is a common complication that leads to premature loss of ambulation. Children with restricted opportunities get bored and eat more. Proper dietary intake and diversified recreational program helps reduce obesity § Mild to moderate cognitive impairment. Deficient dystrophin in the brain causes impairment, so they should be involved in early learning programs and regular classrooms as much as possible

↑ protein, normal glucose, ↓WBCs

· Clinical manifestations of Guillain-Barré Syndrome (Infectious Polyneuritis): § Muscle tension, paresthesia and cramps § proximal symmetrical muscle weakness § ascending paralysis from lower extremities § frequently involves muscles of trunk and upper extremities and those supplied by cranial nerves (facial) § Flaccid paralysis with loss of reflexes § May involve facial, extraocular, labial, lingual, pharyngeal, and laryngeal muscles § Intercostal and phrenic involvement: breathlessness in vocalizations; shallow/irregular respirations § Others: DTR depressed/absent, varying sensory impairment, muscle tenderness, muscle sensitivity to slight pressure, urinary incontinence/retention, constipation · Labs/Diagnostics for Guillain-Barré Syndrome (Infectious Polyneuritis): § Based on clinical manifestations § CSF analysis (_____________________________________________________) § EMG to identify acute muscle denervation

Guillain-Barré Syndrome (Infectious Polyneuritis)

· Clinical manifestations of ____________________________________________: § Muscle tension, paresthesia and cramps § proximal symmetrical muscle weakness § ascending paralysis from lower extremities § frequently involves muscles of trunk and upper extremities and those supplied by cranial nerves (facial) § Flaccid paralysis with loss of reflexes § May involve facial, extraocular, labial, lingual, pharyngeal, and laryngeal muscles § Intercostal and phrenic involvement: breathlessness in vocalizations; shallow/irregular respirations § Others: DTR depressed/absent, varying sensory impairment, muscle tenderness, muscle sensitivity to slight pressure, urinary incontinence/retention, constipation

genetic analysis for presence of gene marker for SMN gene

· Diagnosis of Spinal Muscle Atrophy (SMA): § Gold standard: __________________________________ § Prenatal diagnosis by genetic analysis of fetal cells in maternal blood or amniotic fluid § Muscle electromyography (EMG): shows a denervation pattern § Muscle Biopsy

-certain vaccines (rabies, influenza, polio and meningococcal) -viral or bacterial infections (measles, mumps, gastroenteritis, cytomegalovirus, Lyme disease, EBV, H. pylori)

· Guillain-Barré Syndrome (Infectious Polyneuritis) is an uncommon acute demyelinative polyneuropathy with a progressive, ascending flaccid paralysis. The hallmark sign is peripheral motor weakness § GBS is immune-mediated and often precipitated by: _________________________________________________________ § Onset of symptoms usually occurs within 10 days of infection. · Hallmark Presentation of Guillain-Barré Syndrome (Infectious Polyneuritis): acute peripheral motor weakness usually in late adolescence and young adulthood in three phases: § Acute Phase: starts with onset of symptoms and continues to when symptoms stop or deterioration ceases (up to four weeks) § Plateau Phase: symptoms constant without further deterioration (days to weeks) § Recovery Phase: improvement and progress to optimal/full recovery (weeks to months)

IV botulism immune globulin (BIG-IV)

· Infant Botulism (A&B) can occur in breastfed infants introduced to non-milk foods (specifically honey). It is caused by ingestion of spores or generative cells of C. botulinum and release of toxin from organisms in the GI tract § Constipation is an early sign, followed by weakness and decrease in spontaneous movements § Sequential loss of neurologic function and respiratory failure follow § Can be mistaken for sepsis or SMA-1 § S/S of Infant Botulism (A&B): Constipation, generalized weakness, decrease in spontaneous movements, diminished/absent DTR, loss of head control, poor feeding, weak cry, reduced gag reflex, progressive respiratory paralysis · Diagnosis of Botulism: based on history, physical exam, and positive organism in stool (serum less common). EMG may aid in diagnosis · Nursing Care and Treatment for Botulism: § Treated with _____________________________________________________________ if suspected with or without confirming lab results § Advanced airway support for respiratory failure (intubation, mechanical ventilation) § Nutritional support is crucial because these infants aren't able to feed § Teach parents not to give infants younger than 12 any raw honey § Nurses should report any poor feeding, constipation, and muscle impairment in infants with botulism § Advise parents that normal bowel activity may not return for several weeks

flushing face, sweating forehead, pupillary constriction, HTN, headache, bradycardia. The precipitating stimulus may be a full bladder or rectum, and can be catastrophic unless relieved

· Initial Symptoms of Spinal Cord Injuries (SCIs) § Absence of reflexes below the lesion § Flaccid/limp muscles below level of damage (spinal shock syndrome) § Loss of sensation/motor function § Autonomic instability (↓ BP, ↓/↑ Temp) § Loss of bladder or bowel control § Autonomic dysreflexia: ____________________________________________________________________________________ § Numbness, tingling, burning, priapism, weakness, loss of bowels/bladder control § Neurogenic shock (↓ BP, ↓HR, peripheral vasodilation)

Duchenne Muscular Dystrophy (Pseudohypertrophic)

· Manifestations of ___________________________________________________: § Fatigue, muscle weakness starting in lower limbs, unsteady gait § Relentless progression of muscle weakness, possible death from respiratory/cardiac failure § Waddling gait, lordosis, frequent falls § Gower Sign: child turns onto side/ABD; flexes knees to assume a kneeling position and then with knees extended, gradually pushes torso to an upright position by "walking" the hands up the legs. They often have difficulty getting out of bed, rising from seated position, or climbing stairs § Psuedohypertrophied calves, thighs, upper arms that feel woody/firm on palpation § Eventual profound muscle atrophy of face, chest, and neck § Mental deficiency: IQ 20 below normal, learning deficiency, cognitive delays § Complications: contracture, deformities of hips/knees/ankles, disuse atrophy, cardiomyopathy, obesity, undernutrition, respiratory compromise, cardiac failure

severe congenital malformation where both cerebral hemisphere are absent and not compatible with life (either stillborn or die within hours to weeks). If the fetus survives birth, degeneration of the brain to a spongiform mass with no bony covering occurs resulting in incompatibility with life. § There is no treatment. Infants have a functional portion of the brainstem that maintains vital functions (temperature, cardiac, respiratory) for a few hours but eventually die from respiratory failure

· Neural Tube Defects: abnormalities that derive from the embryonic neural tube. Normally the spinal cord and cauda equina are encased in a protective sheath of bone and meninges. Failure of this neural tube closure produces varying defects that may involve the entire length of the cord or only a small area. § Risk Factors: drugs, radiation, maternal malnutrition, chemicals, genetics, maternal diabetes, maternal obesity, vitamin B12 and folate deficiency, maternal hyperthermia § Prevention: adequate folate intake during pregnancy · Cranioschisis: a congenital skull defect though with various tissues protrude · Exencephaly: brain is totally exposed or extruded through an associated skull defect; the fetus is usually aborted · Anencephaly: ____________________________________________________________ -_________________________ · Encephalocele: herniation of the brain and meninges through a defect in the skull, producing a fluid-filled sac; can be frontal or posterior · Diagnosis of Neural Tube Defects: Based on clinical manifestations, MRI, ultrasonography, CT, neurologic exam (evaluates any degree of bowel/bladder function, paralysis, absent deep tendon reflexes) § Maternal/fetal tests: blood alpha-fetoprotein in 2nd semester, amniocentesis, CVS

modifications to homes, transportation, and mobility devices. Hydration, nutritional needs, and nutrition modification are discussed § Diet: low calorie, high protein and fiber

· Nursing Care for Duchenne Muscular Dystrophy (Pseudohypertrophic): § Assess and monitor: Ability to do ADLs, respiratory function, cardiac function, child/caregiver understanding of long-term effects, and child/caregiver support/coping § Possible use of steroids to improve muscle strength and pulmonary function § Muscle and strength training with pulmonary exercises, incentive spirometry § O2 therapy, BiPAP, tracheostomy, or long-term ventilation § Home suction and home pulse oximetry for respiratory support § Mechanical in-exsufflator (MIE) delivers positive inspiratory pressure followed by negative pressure coordinated to the patient's breathing to facilitate effective cough and clear mucus. The device can be connected to a tracheostomy or used with a face mask § Manual cough-assisting techniques: glossopharyngeal breathing or air stacking (frog breathing); ABD thrust; or manual hyperinflation with a self-inflating resuscitation bag § Monitor for development of cardiomyopathy § Genetic counseling, routine physical exams, and immunizations § Help families reduce disabilities associated with MD and deal with limitations the disease poses on daily lives. § Case Management! Assisting families with ____________________________________________________ -__________ § Children tend to become socially isolated as they deteriorate while their classmates grow. Children should be encouraged to have peer relationships § Nurses should discuss eventual need for home-based care, assisted living, or respite care when children become incapacitated. § End-of-life and palliative care at home vs. other facility. Often death usually occurs in the 20's

Baclofen for muscle spasticity and gabapentin for neuropathic pain

· Nursing Care for Spinal Cord Injuries (SCIs) § Maintain spinal immobilization until cleared by x-ray and provider. Ensure spinal column stabilization with a rigid cervical collar with supportive blocks on a rigid backboard. This can be frightening, especially if child was unconscious, so reassure/comfort them to prevent anxiety § Maintain patient airway with ventilation for lesions above C3. Tracheostomy may be implanted or a breathing pacemaker device (phrenic nerve stimulator) may be used. § Suction to manage secretions, postural drainage, percussion, cough-assist device § Serial neuro assessment with GCS and assessments for developing neurogenic shock + hemodynamic assessments. Monitor for increased ICP (fluid restriction) § Serial motor assessments § Closely monitor temperature for hypo/hyperthermia § Detailed skin assessment for breakdown and use of alternating-pressure mattress PRN § Assess for DVT and PE, autonomic dysreflexia, and sleep-disordered breathing § Skin Care, pressure mattresses, pressure relief devices, skin inspection daily § _______________________________________________________________________________ § Adaptive Devices, Wheelchair use and training and rehabilitation planning § Bowel and bladder management to prevent autonomic dysreflexia, which would require prompt action to prevent encephalopathy and shock · ↑ BP · Headache · ↓ HR · Profuse diaphoresis and flushing · Arrhythmias · Piloerection · Blurred vision or spots · Anxiety · Nasal congestion

seizures

· Nursing Care for Tetanus (lockjaw) § Advanced airway management for laryngospasm and respiratory muscle tetany. Monitor respiratory status, and keep emergency equipment at all times § Muscle relaxants, opioids, and sedatives: respiratory depression, CNS depression § OG/NG feedings, hydration, IV infusions for fluid admin § Suction airway PRN § Control or eliminate stimulation from sound, light, and touch. Handle the child as little as possible and avoid loud noises to prevent ____________________ § If vecuronium (strong muscle relaxant) is used, the total muscle paralysis makes communication impossible. The drug is not a sedative, and anxiety should be considered in children who are intubated § Wound care and debridement § Diazepam for seizures or muscle relaxation § Ventilation may be required for other muscle relaxants or paralytics § Assess for complications of immobility (constipation, DVT, anorexia, pneumonia, skin breakdown) § Parents should stay and child should not be left alone. The child is aware and are often extremely anxious. They need support, information, and reassurance from the nurse

Spinal Cord Injury Without Radiographic Abnormality (SCIWORA)

· Spinal Cord Injuries (SCIs) are commonly caused by: § MVCs - 56% § Bicycles § All-terrain Vehicles § Sport Injuries (trampolines, diving, gymnastics, football) § Birth Trauma: breech presentation or shaken babies § Nonaccidental Trauma § Transverse Myelitis: can be caused by illness or intraarterial injection of long-acting penicillin (PCN) into buttocks. Damage may result in paraplegia or lower limb amputation § _____________________________________________________________________: in MVAs, most spinal cord injures result from hyperflexion or hyperextension of the neck with/with rotational force, resulting in trauma to the spinal cord without vertebral fracture/dislocation. This commonly occurs with MVA when seatbelts aren't used. Unrestrained children are ejected or sustain damage in the car. Air bags can also cause injury § Falls from heights, such as diving/surfing, falling off horses, or sports activities · SCI without fracture is common in children, whose spines are weaker and more mobile than adults'.

6

· Therapeutic Management Cerebral Palsy: § Goals of Therapy: · Establish locomotion, communication, and self-help skills · Gain optimal appearance and integration of motor functions · Correct associated defects as early and effectively as possible · To provide educational opportunities adapted to the child's needs and capabilities · Promote socialization experiences with other affected and unaffected children § Individualized treatment plan § Physical/occupational therapy: braces, splints, scooters, wheelchairs, feeding utensils, aids for ADLs § Ankle-foot orthoses to prevent deformity or contractures, increase energy efficiency of gait, and control alignment § Wheelchairs, scooter boards, or custom strollers to provide balance and mobility § Orthopedic surgery (not before age __________) to improve function and can include: · Correcting contractures, spastic deformities, for unstable joints, to balance muscle power, correct hip dislocation, or spinal fusion for scoliosis § SLP/Communication: flash cards, picture boards, touch-screen computers

Immobilize cervical, thoracic, and lumbar spine (C-collar, back board with strapping)

· Therapeutic Management of Spinal Cord Injuries (SCIs) § ___________________________________________________________________ § ABC'S: AIRWAY OPENED WITH JAW-THRUST MANEUVER. § Advanced airway support if ineffective or absent respiratory effort § Monitor cardiac output. With MVAs, organ damage and internal bleeding may cause ABD distention, so interventions are taken to prevent systemic shock (BP support) § Assess for other internal or external injuries § After child is at trauma center, spinal is stabilized, neuro assessment is performed, CT scans, MRIs § High-dose steroids (methylpredisone) IV in first 12 hours to decrease inflammation/edema and minimize further injury and long-term effects § Consult and planning for early rehabilitation, orthoses, and ambulation aids · Functional electrical stimulation (FES): electrical stimulator is placed under skin in ABD and electrode leads to paralyzed leg muscles, enabling the child to sit, stand, and walk with the aid of crutches, walker, or orthoses. Often requires surgical tendon transfers, which also helps restore hand or arm function (ADLs, self-catheterization) § SCI rehabilitation: exercise § Clonidine may be used to improve ambulation in patients with partial SCIs § Surgical cord decompression or cervical/thoracic fusion

airway clearance therapy (CPT, cough-assist machine, manual cough assistance)

· Treatment and Nursing Care of Spinal Muscle Atrophy (SMA): § There is no cure for the disease. Treatment is symptomatic and preventative § Prevent joint contractures and treating orthopedic problems (scoliosis, hip subluxation/dislocation) § Powered wheelchairs, lifts, special pressure mattresses, and accessible environmental controls § Control nutritional growth failure with supplemental gastrostomy feedings to maintain nutrition and weight § Restrictive lung disease is a serious complication of SMA. URIs should be treated promptly to prevent death. Sometimes they can experience sleep-disordered breathing during sleep, which can progress to respiratory failure which requires noninvasive mechanical ventilation (BiPAP) · Use ____________________________________________________ due to decreased cough ability § Promote independence in ADLs, normal life, and becoming involved in mainstream school (when possible). § Teach parents to assess adequate O2, especially during sleep when shallow breathing and hypoxemia may develop. Home pulse oximetry can be used § These children are intellectually normal and need verbal, tactile, and auditory stimulation for development. Support them so that they can see activities around them and transport them in appropriate ways (wagons, power-wheelchair) for a change of environment and social contact

corticosteroids (prednisone and deflazacort) to increase muscle strength, performance, pulmonary function. · Prednisone: monitor for infection, avoid infectious agents, eat a healthy diet

· Treatment for Duchenne Muscular Dystrophy (Pseudohypertrophic): § No treatment exists. There has been research on ________________________________________________________________________________ § Primary Goal: maintain optimum function in muscles for as long as possible § Secondary Goal: prevention of contractures § Children who remain active as possible avoid wheelchair confinement for a longer time. This involves stretching, muscle strength training, breathing exercises, surgery to fix contractures/deformities, bracing, and performance of ADLs § Influenza and pneumococcal vaccines are encouraged. Avoid sick people and seek immediate treatment for illness § Polysomnography should be performed when daytime symptoms of sleep-disordered breathing occur. Child and parents should be involved in a discussion of long-term ventilation opioids (BiPAP temporary or full-time, mechanically assisted coughing, MIE, or tracheostomy with relief of airway obstruction through suctioning) § Survival is prolonged with noninvasive ventilation and MAC § Children need extensive cardiac monitoring because they do not show typical symptoms of cardiac dysfunction. Weight loss, nausea/vomiting, cough, fatigue on ADLs, and orthopnea should be evaluated to detect cardiomyopathy

IM in separate syringes at separate injection sites. NEVER IV!

· Treatment for Tetanus (lockjaw) § Prevention with immunizations and boosters. Once an injury has occurred, preventative measures taken based on immune status and nature of injury. · Tetanus toxoid not necessary for clean, minor wounds. But tetanus toxoid or antitoxin may be given for traumatic wounds · Nonimmune child who get tetanus-prone wound (contaminated with dirt, soil, saliva, puncture wounds, avulsions, missile wounds, crush wounds, burns, frostbite) get tetanus immunoglobulin (TIG). TIG provides protection for 10 years or longer with boosters · TIG and tetanus toxoid are always given_____________________________________________________________________ § Acutely ill child is treated in ICU. Maintain airway, fluids, electrolytes, manage pain, and ensure caloric intake § NG tube feedings may be required. Laryngospasm may necessitate PN or GT feeding § TIG therapy to neutralize toxins, local wound care via surgical debridement and cleaning. § Wound cleaning several times during first 48 hours § Diazepam for seizure control and muscle relaxation § Intrathecal baclofen, IV mag sulfate, dantrolene, and midazolam may be given to manage muscle spasms. Often requires ET intubation or tracheostomy and constant cardiopulmonary monitoring

Botulism

· ____________________: Acute flaccid paralysis caused by Clostridium botulinum. Most common source is improperly sterilized home-canned foods. The disease has a wide variation in severity from constipation to progressive loss of neurologic function and respiratory failure § CNS symptoms within 12-36 hours after food exposure § Treatment involves IV botulism antitoxin and general support (respiratory and nutrition) § S/S of _________________: Weakness, dizziness, HA, difficulty talking, diplopia, vomiting, progressive and life threatening respiratory paralysis

Tetanus (lockjaw)

· __________________________________, is an acute, preventable, but often fatal diseases caused by exotoxin produced by Clostridium tetani. It is characterized by painful muscle rigidity involving masseter and neck muscles § Highly correlated to unclean wounds related to dirt, feces, soil, and saliva § Primary prevention is immunization and tetanus immunoglobulin § 4 things must happen for development of tetanus: presence of tetanus spores of bacillus, injury to tissues, wound conditions that encourage multiplication of organism, and susceptible host § Clostridium tetani found in soil, dust, and intestinal tract of humans/animals (especially herbivorous animals). It often enters the body by puncture wound, burn, or crushed area, but may enter the wound through a minor, unnoticed break in skin (thorn or needle prick, bee sting, scratch)

Cerebral Palsy

· __________________________________: a group of permanent disorders of the development of movement and posture causing activity limitation, that are attributed to nonprogressive disturbancesthat occurred in the developing fetal or infant brain. § ATI Definition: nonprogressive impairment of motor function, especially that of muscle control, coordination, and posture. § Characterized by motor disorders, disturbances of sensation, perception, communication, cognition, and behavior; secondary to musculoskeletal problems; and epilepsy · Risk factors: maternal infections, preterm birth, ELBW and VLBW, multiple births, viral encephalitis, MVC, child abuse (SBS), birth injuries, maternal drug use · Pathophysiology: gross abnormalities of brain, vascular occlusion, atrophy, loss of neurons or laminar degeneration. Anoxia is most significant in causing brain damage

Infant Botulism (A&B)

· ____________________________________ can occur in breastfed infants introduced to non-milk foods (specifically honey). It is caused by ingestion of spores or generative cells of C. botulinum and release of toxin from organisms in the GI tract § Constipation is an early sign, followed by weakness and decrease in spontaneous movements § Sequential loss of neurologic function and respiratory failure follow § Can be mistaken for sepsis or SMA-1 § S/S of ____________________________________: Constipation, generalized weakness, decrease in spontaneous movements, diminished/absent DTR, loss of head control, poor feeding, weak cry, reduced gag reflex, progressive respiratory paralysis

Werdnig-Hoffman Disease (SMA- Type 1)

· ______________________________________________: this spinal muscle atrophy disease is characterized by progressive weakness and wasting of skeletal muscles caused be degeneration of anterior horn cells. It is the most common paralytic form of the floppy infant syndrome (congenital hypotonia) § S/S are present at birth or always by age 2: "FLOPPY INFANT SYNDROME" · Inactivity · Infant lying in frog-leg position with legs externally rotated, abducted, and flexed at knees · Generalized weakness, absent DTR, limited movement of shoulder/arm muscles · Active movement usually limited to fingers and toes · Diaphragmatic breathing with sternal retractions · Abnormal tongue movements, weak cry, poor suck reflex, tiring quickly during feedings, growth failure · Alert facies, normal sensation and intellect · Affected infants not able to sit alone, roll over, or walk · Early death from respiratory failure or infection § SMN marker at chromosome 5q13 (autosomal recessive) § No cure with symptomatic treatment and death usually within two years due to respiratory failure § Muscle and joint contractures § Frequent respiratory infections due to abnormal breathing patterns and inability to sit upright without support § May require tracheostomy § FTT due to poor feeding

SMA-2 (intermediate spinal muscular atrophy)

· ________________________________________________: § Lower extremities more affected § Few can ambulate § Can live 7+ years § S/S: onset before 18 months · Early: weakness confined to arms and legs · Later: becomes generalized · Legs usually involved more than arms · Prominent pectus excavatum · Movements absent during complete relaxation or sleep · Some infants can sit if placed in position, but few can ambulate · Life span from 7 months to 7 years, but may have normal life expectancy

Kugelberg-Welander Disease (SMA-3)

· _____________________________________________________________________________: this SMA is a result of anterior horn cell and motor nerve degeneration characterized by muscle weakness similar to type 1. Proximal muscle weakness (especially lower limbs) and muscle atrophy are predominant features. Some children lose walking ability by 8 years old but others can still walk after 30 years § May be able to achieve walking with abnormal gait § Eventually confined to wheelchair by second decade § S/S: Onset after 18 months · Normal head control and can sit unassisted by 6-8 months of age · Weak thigh/hip muscles · Scoliosis is common · Failure to walk · Those who can walk: waddling gait, Genu recurvatum, protuberant ABD, ambulation becomes increasingly difficult, confinement to wheel chair by second decade, deep tendon reflexes possibly present early but disappear