Chapter 30: The Child with Neuromuscular or Muscular Dysfunction
A 4-year-old child has just been diagnosed with pseudohypertrophic (Duchenne) muscular dystrophy. The management plan should include which action? a. Recommend genetic counseling. b. Explain that the disease is easily treated. c. Suggest ways to limit use of muscles. d. Assist family in finding a nursing facility to provide his care.
ANS: A Pseudohypertrophic (Duchenne) muscular dystrophy is inherited as an X-linked recessive gene. Genetic counseling is recommended for parents, female siblings, maternal aunts, and their female offspring. No effective treatment exists at this time for childhood muscular dystrophy. Maintaining optimal function of all muscles for as long as possible is the primary goal. It has been found that children who remain as active as possible are able to avoid wheelchair confinement for a longer time. Assisting the family in finding a nursing facility to provide his care is inappropriate at the time of diagnosis. When the child becomes increasingly incapacitated, the family may consider home-based care, a skilled nursing facility, or respite care to provide the necessary care.
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 which position? a. Prone and tube-fed b. Prone, head turned to side, and nipple-fed c. Supine in an infant carrier and nipple-fed d. Supine, with defect supported with rolled blankets, and nipple-fed
ANS: B In the prone position, feeding is a problem. The infant's head is turned to one side for feeding. If the child is able to nipple-feed, tube feeding is not needed. Before surgery, the infant is kept in the prone position to minimize tension on the sac and risk of trauma.
Latex allergy is suspected in a child with spina bifida. Appropriate nursing interventions include which action? a. Avoid using any latex product. b. Use only nonallergenic latex products. c. Administer medication for long-term desensitization. d. Teach family about long-term management of asthma.
ANS: A Care must be taken that individuals who are at high risk for latex allergies do not come in direct or secondary contact with products or equipment containing latex at any time during medical treatment. There are no nonallergenic latex products. At this time, desensitization is not an option. The child does not have asthma. The parents must be taught about allergy and the risk of anaphylaxis.
The nurse is conducting a staff in-service on common problems associated with myelomeningocele. Which common problem is associated with this defect? a. Hydrocephalus b. Craniosynostosis c. Biliary atresia d. Esophageal atresia
ANS: A Hydrocephalus is a frequently associated anomaly in 80% to 90% of children. Craniosynostosis is the premature closing of the cranial sutures and is not associated with myelomeningocele. Biliary and esophageal atresia is not associated with myelomeningocele.
The nurse is planning a staff in-service on childhood spastic cerebral palsy. Spastic cerebral palsy is characterized by: 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.
ANS: A Hypertonicity and poor control of posture, balance, and coordinated motion are part of the classification of spastic cerebral palsy. Athetosis and dystonic movements are part of the classification of dyskinetic (athetoid) cerebral palsy. Wide-based gait and poor performance of rapid, repetitive movements are part of the classification of ataxic cerebral palsy. Tremors and lack of active movement may indicate other neurologic disorders.
The nurse is teaching a group of nursing students about newborns born with the congenital defect of myelomeningocele. Which common problem is associated with this defect? a. Neurogenic bladder b. Mental retardation c. Respiratory compromise d. Cranioschisis
ANS: A Myelomeningocele is one of the most common causes of neuropathic (neurogenic) bladder dysfunction among children. Risk of mental retardation is minimized through early intervention and management of hydrocephalus. Respiratory compromise is not a common problem in myelomeningocele. Cranioschisis is a skull defect through which various tissues protrude. It is not associated with myelomeningocele.
The nurse in the neonatal intensive care unit is caring for an infant with myelomeningocele 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
ANS: A, B, C The nurse should observe an infant with unrepaired myelomeningocele for early signs of infection, such as temperature instability (axillary), irritability, and lethargy. Bradycardia and hypertension are not early signs of infection in infants.
The nurse is conducting discharge teaching to parents of a preschool child with myelomeningocele, repaired at birth, 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 (Ditropan) as prescribed. c. Reduce fluid intake in the afternoon and evening hours. d. Monitor for signs of a recurrent urinary tract infection. e. Administer furosemide (Lasix) as prescribed.
ANS: A, B, D Discharge teaching to prevent renal complications in a child with myelomeningocele include: (1) regular urologic care with prompt and vigorous treatment of infections; (2) a method of regular emptying of the bladder, such as clean intermittent catheterization (CIC) taught to and performed by parents and self-catheterization taught to children; (3) medications to improve bladder storage and continence, such as oxybutynin chloride (Ditropan) and tolterodine (Detrol). Fluids should not be limited and Lasix is not used to improve renal function for children with myelomeningocele.
Which assessment findings should the nurse note in a school-age child with Duchenne muscular dystrophy (DMD)? (Select all that apply.) a. Lordosis b. Gower sign c. Kyphosis d. Scoliosis e. Waddling gait
ANS: A, B, E Difficulties in running, riding a bicycle, and climbing stairs are usually the first symptoms noted in Duchenne muscular dystrophy. Typically, affected boys have a waddling gait and lordosis, fall frequently, and develop a characteristic manner of rising from a squatting or sitting position on the floor (Gower sign). Lordosis occurs as a result of weakened pelvic muscles, and the waddling gait is a result of weakness in the gluteus medius and maximus muscles. Kyphosis and scoliosis are not assessment findings with DMD.
A 14-year-old girl is in the intensive care unit after a spinal cord injury 2 days ago. Nursing care for this child includes which action(s)? (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
ANS: A, B, E Spinal cord injury patients are physiologically labile, and close monitoring is required. They may be unstable for the first few weeks after the injury. Corticosteroids are administered to minimize the inflammation present with the injury. It is not necessary to minimize environmental stimuli for this type of injury. Discussing long-term care issues with the family is inappropriate. The family is focusing on the recovery of their child. It will not be known until the rehabilitation period how much function the child may recover.
The nurse is caring for an infant with myelomeningocele scheduled for surgical closure in the morning. Which interventions should the nurse plan for the care of the 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
ANS: B Before surgical closure, the myelomeningocele is prevented from drying by the application of a sterile, moist, nonadherent dressing over the defect. The moistening solution is usually sterile normal saline. Dressings are changed frequently (every 2 to 4 hours), and the sac is closely inspected for leaks, abrasions, irritation, and any signs of infection. The sac must be carefully cleansed if it becomes soiled or contaminated. The original dressing would not be reinforced but changed as needed. A diaper is not placed over the dressing because stool contamination can occur.
How much folic acid is recommended for women of childbearing age? a. 1.0 mg b. 0.4 mg c. 1.5 mg d. 2.0 mg
ANS: B It has been estimated that a daily intake of 0.4 mg of folic acid in women of childbearing age will prevent 50% to 70% of cases of neural tube defects; 1.0 mg is too low a dose; 1.5 to 2.0 mg are not the recommended dosages of folic acid.
The nurse is admitting a school-age child with suspected Guillain-Barré syndrome (GBS). Which 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
ANS: B Treatment of GBS is primarily supportive. In the acute phase, patients are hospitalized because respiratory and pharyngeal involvement may require assisted ventilation, sometimes with a temporary tracheotomy. Treatment modalities include aggressive ventilatory support in the event of respiratory compromise, intravenous (IV) administration of immunoglobulin (IVIG), and sometimes steroids; plasmapheresis and immunosuppressive drugs may also be used. Intake and output, telemetry monitoring and obtaining laboratory studies may be part of the plan of care but are not the priority.
Which should the nurse expect to find in the cerebral spinal fluid (CSF) results of a child with Guillain-Barré syndrome (GBS)? (Select all that apply.) a. Decreased protein concentration b. Normal glucose c. Fewer than 10 white blood cells (WBCs/mm3) d. Elevated red blood cell (RBC) count
ANS: B, C Diagnosis of GBS is based on clinical manifestations, CSF analysis, and EMG findings. CSF analysis reveals an abnormally elevated protein concentration, normal glucose, and fewer than 10 WBCs/mm3. CSF fluid should not contain RBCs.
A home care nurse is caring for an adolescent with a T1 spinal cord injury. The adolescent suddenly becomes flushed, hypertensive, and diaphoretic. Which intervention should the nurse perform first? a. Place the adolescent in a flat right side-lying position. b. Place a cool washcloth on the adolescent's forehead and continue to monitor the blood pressure. c. Implement a standing prescription to empty the bladder with a sterile in and out Foley catheter. d. Take a full set of vital signs and notify the health care provider.
ANS: C The adolescent is experiencing an autonomic dysreflexia episode. The paralytic nature of autonomic function is replaced by autonomic dysreflexia, especially when the lesions are above the mid-thoracic level. This autonomic phenomenon is caused by visceral distention or irritation, particularly of the bowel or bladder. Sensory impulses are triggered and travel to the cord lesion, where they are blocked, which causes activation of sympathetic reflex action with disturbed central inhibitory control. Excessive sympathetic activity is manifested by a flushing face, sweating forehead, pupillary constriction, marked hypertension, headache, and bradycardia. The precipitating stimulus may be merely a full bladder or rectum or other internal or external sensory input. It can be a catastrophic event unless the irritation is relieved. Placing a cool washcloth on the adolescent's forehead, continuing to monitor blood pressure and vital signs, and notifying the healthcare provider would not reverse the sympathetic reflex situation.
Which clinical manifestations in an infant would be suggestive of spinal muscular atrophy (Werdnig-Hoffmann disease)? a. Hyperactive deep tendon reflexes b. Hypertonicity c. Lying in the frog position d. Motor deficits on one side of body
ANS: C The infant lies in the frog position with the legs externally rotated, abducted, and flexed at knees. The deep tendon reflexes are absent. The child has hypotonia and inactivity as the most prominent features. The motor deficits are bilateral.
The nurse is reviewing prenatal vitamin supplements with an expectant client. Which supplement should be included in the teaching? 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
ANS: C The widespread use of folic acid among women of childbearing age has decreased the incidence of spina bifida significantly. Vitamin A is not related to the prevention of spina bifida. Folic acid supplementation is recommended for the preconception period and during the pregnancy. Only 42% of women actually follow these guidelines.
The nurse is admitting a child with Werdnig-Hoffmann disease (spinal muscular atrophy type 1). Which signs and symptoms are associated with this disease? a. Spinal muscular atrophy b. Neural atrophy of muscles c. Progressive weakness and wasting of skeletal muscle d. Pseudohypertrophy of certain muscle groups
ANS: C Werdnig-Hoffmann disease (spinal muscular atrophy type 1) is the most common paralytic form of floppy infant syndrome (congenital hypotonia). It is characterized by progressive weakness and wasting of skeletal muscle caused by degeneration of anterior horn cells. Kugelberg-Welander disease is a juvenile spinal muscular atrophy with a later onset. Charcot-Marie-Tooth disease is a form of progressive neural atrophy of muscles supplied by the peroneal nerves. Progressive weakness is found of the distal muscles of the arms and feet. Duchenne muscular dystrophy is characterized by muscles, especially in the calves, thighs, and upper arms, which become enlarged from fatty infiltration and feel unusually firm or woody on palpation. The term pseudohypertrophy is derived from this muscular enlargement.
The nurse is preparing to admit a newborn with myelomeningocele to the neonatal intensive care nursery. Which describes this newborn's defect? 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 saclike cyst of meninges with spinal fluid but no neural elements d. Visible defect with an external saclike protrusion containing meninges, spinal fluid, and nerves
ANS: D A myelomeningocele is a visible defect with an external saclike protrusion, containing meninges, spinal fluid, and nerves. Rachischisis is a fissure in the spinal column that leaves the meninges and the spinal cord exposed. Encephalocele is a herniation of brain and meninges through a defect in the skull, producing a fluid-filled sac. Meningocele is a hernial protrusion of a saclike cyst of meninges with spinal fluid, but no neural elements.
The parents of a child with cerebral palsy ask the nurse whether any drugs can decrease their child's spasticity. The nurse's response should be based on which statement? 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.
ANS: D Baclofen, given intrathecally, is best suited for children with severe spasticity that interferes with activities of daily living and ambulation. Anticonvulsant medications are used when seizures occur in children with cerebral palsy. The intrathecal route decreases the side effects of the drugs that reduce spasticity. Few medications are currently available for the control of spasticity.
The nurse is conducting reflex testing on infants at a well-child clinic. Which reflex finding should be reported as abnormal and considered as a possible sign of cerebral palsy? a. Tonic neck reflex at 5 months of age b. Absent Moro reflex at 8 months of age c. Moro reflex at 3 months of age d. Extensor reflex at 7 months of age
ANS: D Establishing a diagnosis of cerebral palsy (CP) may be confirmed with the persistence of primitive reflexes: (1) either the asymmetric tonic neck reflex or persistent Moro reflex (beyond 4 months of age) and (2) the crossed extensor reflex. The tonic neck reflex normally disappears between 4 and 6 months of age. The crossed extensor reflex, which normally disappears by 4 months, is elicited by applying a noxious stimulus to the sole of one foot with the knee extended. Normally, the contralateral foot responds with extensor, abduction, and then adduction movements. The possibility of CP is suggested if these reflexes occur after 4 months.
Therapeutic management of a child with tetanus includes the administration of: a. nonsteroidal anti-inflammatory drugs (NSAIDs) to reduce inflammation. b. muscle stimulants to counteract muscle weakness. c. bronchodilators to prevent respiratory complications. d. tetanus immunoglobulin therapy.
ANS: D Tetanus immunoglobulin therapy, to neutralize toxins, is the most specific therapy for tetanus. Tetanus toxin acts at the myoneural junction to produce muscular stiffness and lowers the threshold for reflex excitability. NSAIDs are not routinely used. Sedatives or muscle relaxants are used to help reduce titanic spasm and prevent seizures. Respiratory status is carefully evaluated for any signs of distress because muscle relaxants, opioids, and sedatives that may be prescribed may cause respiratory depression. Bronchodilators would not be used unless specifically indicated.
The nurse is caring for an intubated infant with botulism in the pediatric intensive care unit. 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 (Garamycin) 10 mg per intravenous piggyback every 12 hours.
ANS: D The nurse should clarify the administration of an aminoglycoside antibiotic. Antibiotic therapy is not part of the management of infant botulism because the botulinum toxin is an intracellular molecule, and antibiotics would not be effective; aminoglycosides in particular should not be administered because they may potentiate the blocking effects of the neurotoxin. Treatment consists of immediate administration of botulism immune globulin intravenously (BIG-IV) without delaying for laboratory diagnosis. Early administration of BIG-IV neutralizes the toxin and stops the progression of the disease. The human-derived botulism antitoxin (BIG-IV) has been evaluated and is now available nationwide for use only in infant botulism. Approximately 50% of affected infants require intubation and mechanical ventilation; therefore, respiratory support is crucial, as is nutritional support because these infants are unable to feed.
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."
ANS: D There are cross-reactions between latex allergies and a number of foods such as bananas, avocados, kiwi, and chestnuts. Children with a latex allergy will not develop allergies to other food products such as yeast, wheat, barley, or peanuts.
