PEDs Chapt 22 Nursing Care of the Child with a Neuromuscular Disorder

The nurse is caring for a newborn with facial nerve palsy from birth trauma. The mother is very upset and concerned about the child's prognosis. Which response by the nurse would be most appropriate? a) "In most cases treatment is not necessary, only observation." b) "Was this from pressure resulting from forceps?" c) "Have you seen any signs of improvement?" d) "This is the most common facial nerve palsy."

"In most cases treatment is not necessary, only observation." Correct Explanation: The nurse should reassure the mother by reminding her that in most cases treatment is not necessary, only observation. Asking about signs of improvement might alarm the mother because in some cases it can take many months for the palsy to resolve. Asking whether this was a result of pressure from forceps does not address the mother's concerns about the child's prognosis. The mother may not understand or know why the condition occurred. Telling the mother that this is the most common facial nerve palsy does not address the mother's concerns about the child's prognosis.

How would the nurse best describe Gowers' sign to the parents of a child with muscular dystrophy? a) A transfer technique b) A waddling-type gait c) Muscle twitching present during a quick stretch d) The pelvis position during gait

A transfer technique Correct Explanation: Gowers' sign is a description of a transfer technique present during some phases of muscular dystrophy. The child turns on the side or abdomen, extends the knees, and pushes on the torso to an upright position by walking his hands up the legs. The child's gait is unrelated to the presence of Gowers' sign. Muscle twitching present after a quick stretch is described as clonus.

The nurse is teaching a health and wellness course to young women of childbearing age. Which vitamin will the nurse encourage all to take daily? a) Ascorbic acid b) Calcium c) Niacin d) Folic acid

Folic acid Correct Explanation: It is recommended that all women of childbearing age ingest 0.4 mg of folic acid daily. Ascorbic acid is vitamin C, niacin is a B vitamin, and calcium is a mineral, not a vitamin.

The mother of a 3-year-old with a myelomeningocele is thinking about having another baby. The nurse should inform the woman that she should increase her intake of which acid? a) Folic acid to 0.4 mg/day b) Ascorbic acid to 4 mg/day c) Folic acid above 0.4 mg/day d) Ascorbic acid to 0.4 mg/day

Folic acid above 0.4 mg/day Correct Explanation: The American Academy of Pediatrics recommends that a woman who has had a child with a neural tube defect increase her intake of folic acid to above 0.4 mg per day 1 month before becoming pregnant and continue this regimen through the first trimester. A woman who has no family history of neural tube defects should take 0.4 mg/day. All women of childbearing age should be encouraged to take a folic acid supplement because the majority of pregnancies in the United States are unplanned. Ascorbic acid hasn't been shown to have any effect on preventing neural tube defects.

Which finding will cause the nurse to refer a 6-month-old child for further neuromuscular testing? a) Equal withdrawal of lower extremities from pain b) Head lag when pulled from supine to sitting c) Bilaterally open rather than closed hands d) Supporting own weight when placed in standing position

Head lag when pulled from supine to sitting Explanation: Head lag in the child requires referral. By 4 to 5 months, the infant should be able to maintain the head in a neutral position. The other assessment findings are normal for age, indicating no need for referral.

An infant has been born and diagnosed with a meningocele. Which action will the nurse incorporate into each contact with this infant? a) Auscultation for bowel sounds b) Listening for a shrill cry c) Inspection of the cystic sac on the child's back for leakage d) Careful supine positioning

Inspection of the cystic sac on the child's back for leakage Correct Explanation: Leakage from the cystic area indicates loss of cerebrospinal fluid (CSF) and risk of infection of the central nervous system. Prompt intervention is needed, probably surgical. Listening for bowel sounds confirms intestinal peristalsis but is not necessary with each infant contact. A shrill cry may indicate increased intracranial pressure (ICP). This is important to note yet is not as pressing as being aware of leakage. The baby would be positioned prone, not supine, to protect the sac.

Which of the following strategies would be the first choice in attempting to maximize function in a child with muscular dystrophy? a) Manual wheelchair b) Motorized wheelchair c) Walker d) Long leg braces

Long leg braces Correct Explanation: Long leg braces are functional assistive devices that provide increased independence and increased use of upper and lower body strength. Wheelchairs, both motorized and manual, provide less independence and less use of upper and lower body strength. Walkers are functional assistive devices that provide less independence than braces.

The nurse caring for a client with suspected muscular dystrophy would prepare her client for which diagnostic test? a) Muscle biopsy b) EEG c) Assessment of ambulation d) X-ray

Muscle biopsy Correct Explanation: A muscle biopsy shows the degeneration of muscle fibers and infiltration of fatty tissue. It's used for diagnostic confirmation of muscular dystrophy. X-ray is best for identifying an osseous deformity. Ambulation assessment alone wouldn't confirm diagnosis of this client's disorder. EEG wouldn't be appropriate in this case.

A nurse is conducting a physical examination of a 5-year-old boy with spinal muscular atrophy (SMA) type 2. What assessment findings would the nurse expect to find? a) Pectus excavatum b) Loss of strength in ankle dorsiflexion c) Pseudohypertrophy of the calves d) Loss of strength in hip extension

Pectus excavatum Explanation: Pectus excavatum develops in children with SMA type 1 and type 2 who exhibit paradoxical breathing. The chest becomes funnel shaped and the xiphoid process is retracted. Pseudohypertrophy of the calves is associated with Duchenne muscular dystrophy. Loss of strength in hip extension is associated with Duchenne muscular dystrophy. Loss of strength in ankle dorsiflexion is associated with Duchenne muscular dystrophy.

The nurse is caring for a 10-year-old girl with myasthenia gravis. The nurse suspects myasthenic crisis based on which of the following? a) Increased salivation b) Sweating c) Tachycardia d) Bradycardia

Tachycardia Correct Explanation: Tachycardia is a sign of myasthenic crisis. Bradycardia is a sign of cholinergic crisis. Sweating is a sign of cholinergic crisis. Increased salivation is a sign of cholinergic crisis.

The nurse will prepare the 17-year-old with myasthenia gravis for which surgical procedure to treat the disorder? a) Splenectomy b) Thymectomy c) Cholecystectomy d) Thyroidectomy

Thymectomy Correct Explanation: Myasthenia gravis is an autoimmune disease that can be treated in several ways. Thymectomy can be used for children who have reached puberty. Cholecystectomy is removal of the gallbladder. Thyroidectomy removes the thyroid gland, and splenectomy removes the spleen.

Parents of a preschooler with cerebral palsy ask the nurse what the surgeon plans to implant in their child's body to control spasticity. What is the nurse's answer? a) Botulinum toxin b) Baclofen pump c) Vagal nerve stimulator d) Central venous catheter

Baclofen pump Correct Explanation: A baclofen pump can be placed surgically to deliver continuous medication intrathecally. Baclofen can also be taken orally. Botulinum toxin is injected by a practioner into specified muscle groups to reduce spasticity. A central venous catheter places medication directly into rapidly moving blood and would not be used. A vagal nerve stimulator is used to control seizures.

What reflex response will the nurse consider abnormal when assessing the 8-month-old infant? a) Babinski b) Brisk deep tendon c) Parachute d) Plantar grasp

Brisk deep tendon Correct Explanation: By 8 months, the infant should have +2 or average deep tendon reflexes. The protective parachute reflex would have developed between 6 and 7 months. The plantar grasp will remain until about 9 months and the Babinski until 12 months.

The nurse caring for an infant with myelomeningocele before surgical intervention will prioritize care in what way? a) Keep the mass uncovered and dry b) Prevent cold stress using an Isolette and blankets c) Change position from side to side hourly d) Cover the sac with a saline-moistened dressing

Cover the sac with a saline-moistened dressing Correct Explanation: Protection of exposed neural tissue is of high priority. Keeping the cystic mass moist prevents damage to neural elements from drying. Blankets may cause trauma to the sac. An Isolette can be used for warmth, but much attention will have to be directed toward keeping the sac moist. Side-to-side hourly position changes increase the risk of damage to protruding nervous tissue. Unnecessary handling should be avoided.

Given knowledge of muscular dystrophy, the nurse would expect to see which form of this condition most commonly in children? a) Becker's b) Myotonic c) Duchenne's d) Limb-girdle

Duchenne's Correct Explanation: Duchenne's accounts for 50% of all cases of muscular dystrophy.

The nurse receives a report on a child admitted with severe muscular dystrophy. The nurse suspects the child has been diagnosed with the most severe form of the disease, known as: a) myotonia. b) limb-girdle. c) Duchenne's. d) facioscapulohumeral.

Duchenne's. Correct Explanation: Studies have shown that Duchenne's is the most severe form of muscular dystrophy. Myotonia isn't a form of the disease; it's a symptom.

Muscular dystrophy is a result of which cause? a) Unknown nongenetic origin b) Gene mutation c) Genetic and environmental factors d) Chromosomal aberration

Gene mutation Correct Explanation: Muscular dystrophy is a result of a gene mutation. It isn't from a chromosome aberration or environmental factors. It's genetic and there's a known origin of the disease.

Which nursing diagnosis is most relevant in the first 12 hours of life for a neonate born with a myelomeningocele? a) Risk for infection b) Delayed growth and development c) Impaired physical mobility d) Constipation

Risk for infection Correct Explanation: All of these diagnoses are important for a child with a myelomeningocele. However, during the first 12 hours of life, the most life-threatening event would be an infection. The other diagnoses will be addressed as the child develops.

After teaching a group of students about medications commonly used for neuromuscular disorders, the nursing instructor determines that the teaching was successful when the students identify which agent as a centrally acting skeletal muscle relaxant? a) Botulin toxin b) Prednisone c) Lorazepam d) Baclofen

Baclofen Correct Explanation: Baclofen is a centrally acting skeletal muscle relaxant used to treat painful spasms and decrease spasticity in children with motor neuron lesions. Prednisone is a corticosteroid that is used to help slow the progression of Duchenne muscular dystrophy. Lorazepam is a benzodiazepine used for adjunctive relief of skeletal muscle spasm associated with cerebral palsy. Botulin toxin is a neurotoxin used to relieve spasticity in cerebral palsy.

The nurse is caring for an 8-month-old boy presenting with poor feeding, listlessness, and a weak cry. What assessment finding would lead the nurse to suspect a diagnosis of botulism? a) Inadequate sucking b) Drooping eyelids c) Diminished gag reflex d) Floppy extremities

Diminished gag reflex Correct Explanation: A diminished gag reflex is indicative of botulism and not typically associated with other conditions. The other symptoms could be indicative of a number of neuromuscular diseases. Botulism is a rare disease and is difficult to diagnose since its symptoms are similar to those of other neuromuscular diseases.

A client with muscular dystrophy has lost complete control of his lower extremities. He has some strength bilaterally in the upper extremities, but poor trunk control. Which mechanism would be the most important to have on the wheelchair? a) Antitip device b) Extended breaks c) Wheelchair belt d) Headrest support

Wheelchair belt Correct Explanation: This client has poor trunk control; a belt will prevent him from falling out of the wheelchair. Antitip devices, head rest supports, and extended breaks are all important options but aren't the most important options in this situation.

A child is to undergo testing for suspected muscular dystrophy and is scheduled for the following tests. Which test would the nurse identify as most important to be completed first? a) Electromyogram b) Creatine kinase c) Muscle biopsy d) Nerve conduction velocity

Creatine kinase Correct Explanation: The sample for creatine kinase must be obtained before the electromyogram or muscle biopsy because those tests may lead to a release of creatine kinase and provide false results. Nerve conduction velocity tests could be done at anytime.

The nurse is planning to teach the parents of a child with newly diagnosed muscular dystrophy about the disease. Which definition should she use to best describe this condition? a) Lesions of the brain cortex b) A demyelinating disease c) Degeneration of muscle fibers d) Upper motor neuron lesions

Degeneration of muscle fibers Correct Explanation: Degeneration of muscle fibers with progressive weakness and wasting best describes muscular dystrophy. Demyelination of myelin sheaths is a description of multiple sclerosis. Lesions within the brain cortex and the upper motor neurons suggest a neurologic, not a muscular, disease.

A child with cerebral palsy is referred for physical therapy. When describing the rationale for this therapy, the nurse would emphasize which of the following as the primary goal? a) Development of gross motor movement b) Enhance feeding capabilities c) Promote optimal self-care ability d) Development of fine motor skills

Development of gross motor movement Correct Explanation: Physical therapy focuses on assisting in the development of gross motor movements such as walking and positioning and helps the child develop independent movement. Occupational therapy assists in the development of fine motor skills and fashioning orthotics and splints. Occupational therapy assists the child in performing optimal self-care ability by working on skills such as activities of daily living. Speech therapy assists with feeding techniques for the child who has swallowing problems.

The nurse caring for a toddler immediately after a fall from a grocery cart will avoid moving which body area as the child is examined? a) Head and neck b) Lower extremities c) Clavicle d) Torso

Head and neck Correct Explanation: The head and neck should remain immobilized until cervical spine injury is ruled out. Motion in this area could damage the spinal cord. The rest of the child's body should be examined carefully so as not to aggravate an unsuspected injury. The clavicle is the bone most frequently fractured during childhood.

Through which mechanism is Duchenne's muscular dystrophy acquired? a) Heredity b) Virus c) Autoimmune factors d) Environmental toxins

Heredity Correct Explanation: Muscular dystrophy is hereditary and acquired through a recessive sex-linked trait. Therefore, it isn't caused by viral, autoimmune, or environmental factors.

Which nursing diagnosis will the nurse prepare for the infant who is placed prone to protect the myelomeningocele repair site? a) Peripheral neurovascular dysfunction b) Disorganized infant behavior c) Risk for activity intolerance d) Risk for impaired skin integrity

Risk for impaired skin integrity Explanation: The skin of the infant's knees and elbows is exposed to both pressure and friction. Leakage of urine and stool makes skin cleanliness a challenge. Should voluntary movement of the legs be affected, they become more vulnerable to skin integrity problems. The neuromuscular dysfunction the infant experiences is neither peripheral nor vascular. Disorganized infant behavior does not reflect the reality of the situation, and risk for activity intolerance is not appropriate because little activity occurs.

Which type of spinal neural tube defect does the nurse recognize as common and usually benign? a) Myelomeningocele b) Meningocele c) Spina bifida d) Spina bifida occulta

Spina bifida occulta Correct Explanation: Spina bifida occulta usually is benign and is estimated to affect 20% of the population. It is a defect in the vertebral body without protrusion of the spinal cord or its coverings. Spina bifida is a general term that is often used to refer to all neural tube disorders of the spinal cord. Meningocele and myelomenigocele do involve protrusion of elements of the spinal portion of the central nervous system and require treatment.

A nurse is developing a teaching plan for the parents of a child with myasthenia gravis. Which of the following would the nurse include? a) Establishment of plans for rest periods b) How to administer anticholinergic drugs c) Signs and symptoms of infection d) Ways to increase the temperature of the child's environment e) Stress management techniques

• How to administer anticholinergic drugs • Establishment of plans for rest periods • Signs and symptoms of infection • Stress management techniques Explanation: The teaching plan for a child with myasthenia gravis should include instructions about administering anticholinergic agents, usually 30 to 45 minutes before meals, on time and exactly as ordered; measures to allow for rest periods for energy conservation; signs and symptoms of infection and the need to notify the physician because infection can precipitate a myasthenic crisis; stress management techniques because stress can precipitate a myasthenic crisis; and ways to maintain the child's environmental temperature because exposure to extreme temperatures can precipitate a myasthenic crisis.

What methods can a nurse use to evaluate extremity function in an 18-month-old? Select all that apply. a) Ask the child to squeeze the nurse's fingers simultaneously. b) Have the child push against resistance with both feet. c) Look for symmetric motion in the arms and legs. d) Elicit from the parent a description of fine and gross motor activities. e) Observe the child in developmentally appropriate play.

• Look for symmetric motion in the arms and legs. • Elicit from the parent a description of fine and gross motor activities. • Observe the child in developmentally appropriate play. Correct Explanation: Observing play, eliciting parental descriptions, and looking for symmetry in motion are all developmentally appropriate and effective methods of assessing extremity function in this toddler. Expecting the child to cooperate in squeezing fingers or pushing feet against resistance is not realistic and is likely to cause reluctance to participate in later assessments.

A multidisciplinary team meeting is being called by the nurse to identify methods to reduce spasticity in a school-age child with cerebral palsy. Input from which discipline will not be needed at this gathering? a) Dietary b) Pharmacy c) Orthotics d) Surgery

Dietary Explanation: No dietary interventions are known to reduce spasticity in the child with cerebral palsy. However, dietitians are essential in helping to meet the nutritional needs of children with cerebral palsy, who may have chewing and swallowing disorders. All the other disciplines have interventions that may be helpful to the child in reducing spasticity, thereby increasing function and/or mobility.

Which statement about cerebral palsy would be accurate? a) "Cerebral palsy is a condition that doesn't get worse." b) "Cerebral palsy is a condition that runs in families." c) "Cerebral palsy occurs because of too much oxygen to the brain." d) "Cerebral palsy means there will be many disabilities."

"Cerebral palsy is a condition that doesn't get worse." Correct Explanation: By definition, cerebral palsy is a nonprogressive neuromuscular disorder. It can be mild or quite severe and is believed to be the result of a hypoxic event during pregnancy or the birth process and doesn't run in families.

The nurse will teach parents of children with myelomeningocele to maintain an environment free of what element? a) Latex b) Cat dander c) Alcohol gel d) Peanuts

Latex Correct Explanation: A latex-free environment is important because research shows that up to 73% of children with repeated surgeries for spina bifida are sensitive to latex. Those with known sensitivity must be managed in a latex-free environment in the health care setting and in the home, in the school, and beyond. Children at risk for latex sensitivity should wear medical alert identification. The other options may present risks to individual children but are not a threat to those with spina bifida as a group.

In caring for the child with Guillain-Barré syndrome, the nurse will provide much supportive care while watching carefully for signs of deterioration in which body system? a) Urinary b) Integumentary c) Cardiovascular d) Respiratory

Respiratory Correct Explanation: Guillain-Barré is a life-threatening disease; the greatest risk occurs during the acute stage, when respiratory failure may occur. The child with this syndrome will be ill and will have limited mobility for an extended time. All body systems will be stressed, requiring supportive care.

The nurse is assessing a child with spastic cerebral palsy. Which of the following would the nurse expect to assess? Select all that apply. a) Poor control of balance b) Dysarthria c) Exaggerated deep tendon reflexes d) Hypertonicity e) Hemiplegia f) Drooling

• Poor control of balance • Exaggerated deep tendon reflexes • Hypertonicity • Hemiplegia Correct Explanation: Spastic cerebral palsy is associated with exaggerated deep tendon reflexes; poor control of posture, balance, and movement; hypertonicity of the affected extremities; and hemiplegia, quadriplegia, or diplegia, based on the limbs affected. Drooling and dysarthria are associated with athetoid cerebral palsy.

What will be the nurse's next action after noting dimpling and a tuft of hair located in the lumbosacral area of the preschool child during examination? a) Record and refer the finding for follow-up to the pediatrician b) Move on to other assessments without calling attention to the difference c) Snip the tuft of hair off close to the skin for hygienic reasons d) Inspect for precocious hair growth in the genital and underarm areas

Record and refer the finding for follow-up to the pediatrician Correct Explanation: Dimpling and hair growth may signal spina bifida occulta, which usually is benign. However, some complications can be associated, and further investigation is warranted to prevent possible damage to the spinal cord. Magnetic resonance imaging (MRI) is often the diagnostic tool used. No hygienic concerns need prevail. These findings do not suggest development of precocious puberty or any other hormonal problem. The dimpling and hair tuft must be clearly explained to the parents.

A nurse is preparing a plan of care for an infant who has undergone surgery to repair a myelomeningocele. The nurse would include placing the infant in which position postoperatively? Select all that apply. a) Right side lying b) Left side lying c) Semi-Fowler d) Supine e) Prone

• Right side lying • Left side lying • Prone Correct Explanation: Postoperatively, the nurse would position the infant in the prone or side-lying position to allow the incision to heal.

A nurse is caring for an infant with spinal muscle atrophy (SMA) type 1. What will the nurse note when assessing the child? a) Enlarged head with low-set ears b) Lusty cry with voracious appetite c) Narrow chest and protuberant abdomen d) Spastic upper and lower extremities

Narrow chest and protuberant abdomen Correct Explanation: SMA type 1 is also known as Werdnig-Hoffman disease and infantile SMA. It is the most severe of the three types. This disease is autosomal recessive and affects the ability of spinal nerves to communicate with muscle, eventually leading to atrophy. The infantile form progresses rapidly to early childhood death, usually from respiratory complications. The narrow chest and large abdomen are characteristic. Over time, the chest develops pectus excavatum, which restricts respiration further when combined with muscle weakness. Extremities would not be spastic but hypotonic. Head size and ear placement are normal in the infant with SMA type 1. Difficulties in sucking and swallowing are common, and a lusty cry is not found.

The pediatric nurse practioner (PNP) records "positive Gowers' sign" after finishing the assessment of a young boy. How will the student nurse reading the PNP's note interpret this? a) Severe lordosis is evident in the lumbar spine. b) The boy rises from the floor by walking his hands up his legs. c) The head is held tilted with limited side-to-side motion. d) The boy has a large tan skin lesion on his torso.

The boy rises from the floor by walking his hands up his legs. Correct Explanation: Gowers' sign is a hallmark finding of Duchenne muscular dystrophy as muscles weaken. The boy cannot rise from the floor in the usual way and needs to turn to hands and knees, move feet under the body, and "walk" hands up his legs to stand. The other options do not describe Gowers' sign, although lordosis is often a manifestation of Duchenne muscular dystrophy.

The child with a surgically repaired myelomeningocele has a neurogenic bladder. How will the nurse best explain this problem to the parents? a) "Your child cannot properly control holding urine or emptying the bladder. " b) "Your child dribbles urine because the bladder either is overactive, pushing urine out, or is not active enough, becoming overfilled and causing urine to leak." c) "While your child is young, urine leaking from the bladder will not be a problem because diapering is expected." d) "Old urine remains in the bladder because of poor emptying or overfilling, putting your child at risk for urinary tract infection."

"Old urine remains in the bladder because of poor emptying or overfilling, putting your child at risk for urinary tract infection." Correct Explanation: Parents need to understand that lack of urinary control is not the greatest problem. The larger threat is of urinary tract infection, which can result in kidney damage. Only one of the responses by the nurse carries the infection message. Continence is important. This along with the infection risk can be managed by clean intermittent catheterization (CIC) or other procedures.

A young child with Duchenne muscular dystrophy is placed on both prednisone and calcium. Parents view these two medications as rather "common" and question their importance for the child. What explanation by the nurse will be most helpful to the parents? a) "Prednisone will help protect his vulnerable respiratory tract from developing reactive airway disease. Calcium is needed to guard against muscle cramping." b) "Prednisone will stimulate weight gain and appetite. Calcium is needed to ensure adequate supplies for the development of permanent teeth." c) "Prednisone will protect against nerve inflammation in his hips and legs. Calcium is necessary should dietary intake be insufficient to meet growth needs." d) "Prednisone helps to keep muscle fibers strong and delays progression of the disease. Calcium protects against osteoporosis caused by both prednisone and lack of weight bearing."

"Prednisone helps to keep muscle fibers strong and delays progression of the disease. Calcium protects against osteoporosis caused by both prednisone and lack of weight bearing." Explanation: Studies have shown that boys treated with prednisone have improved muscle strength and function. This is thought to be due to the protection that prednisone provides to muscle fibers. Calcium is needed to prevent osteoporosis, which is a side effect of prednisone that also occurs when weight bearing is limited. Respiratory infection is a risk in that those muscles weaken with progression of the disease, but reactive airway disease is not a particular risk. No peripheral nerve involvement is observed in Duchenne muscular dystrophy. Side effects of prednisone include weight gain and appetite stimulation, but these are not the reasons for the prednisone therapy. Calcium does augment dietary intake of the mineral and is important for tooth development, and it may play a role in prevention of muscle cramps, but these are not the main reasons for taking the calcium supplement.

Other than providing direct care to children, what is the major role of nurses in the care of nearly all children with neuromuscular disorders? a) Helping with specialized equipment b) Consoling parents c) Teaching children self-care d) Coordinating care by specialists

Coordinating care by specialists Correct Explanation: Being part of a multidisciplinary team and coordinating the care the child usually needs from a variety of specialists is an essential and major role. The other nursing activities are important as well, but many children/families require individual interventions.

A nurse is teaching the parents of a child who has been diagnosed with spina bifida. Which statement by the nurse would be the most accurate description of spina bifida? a) "Its presence indicates that many areas of the central nervous system (CNS) may not develop or function adequately." b) "It's a simple neurologic defect that's completely corrected surgically within 1 to 2 days after birth." c) "It's a complex neurologic disability that involves a collaborative health team effort for the entire first year of life." d) "It has little influence on the intellectual and perceptual abilities of the child."

"Its presence indicates that many areas of the central nervous system (CNS) may not develop or function adequately." Correct Explanation: When a spinal cord lesion exists at birth, it commonly leads to altered development or function of other areas of the CNS. Spina bifida is a complex neurologic defect that heavily impacts the physical, cognitive, and psychosocial development of the child and involves collaborative, lifelong management due to the chronicity and multiplicity of the problems involved.

A teenager has been admitted to the hospital with respiratory complications related to Duchenne muscular dystrophy. How can the nurse best provide support for the parents, who are the caretakers of this adolescent? a) Provide accommodations for both parents to room-in with their teen b) Assume responsibility for the teen's daily care while accepting input from parents and the teen c) Teach the parents how to add chest physical therapy to the care they provide d) Encourage the parents to assist their child with his activities of daily living while hospitalized

Assume responsibility for the teen's daily care while accepting input from parents and the teen Explanation: The parents have probably been caring daily for their son for longer than 10 years while his condition has deteriorated and his care has become more complex. They may use this hospitalization as an opportunity to be relieved briefly of some of this responsibility (respite). Chest PT and rooming-in are important but do not address the need for respite.

Which diagnostic measure is most accurate in detecting neural tube defects? a) Flat plate of the lower abdomen after the 23rd week of gestation b) Significant level of alpha-fetoprotein present in amniotic fluid c) Presence of high maternal levels of albumin after 12th week of gestation d) Amniocentesis for lecithin-sphingomyelin (L/S) ratio

Significant level of alpha-fetoprotein present in amniotic fluid Correct Explanation: Screening for significant levels of alpha-fetoprotein is 90% effective in detecting neural tube defects. Prenatal screening includes a combination of maternal serum and amniotic fluid levels, amniocentesis, amniography, and ultrasonography and has been relatively successful in diagnosing the defect. Flat plate X-rays of the abdomen, L/S ratio, and maternal serum albumin levels aren't diagnostic for the defect.

The nurse is caring for a 2-year-old boy with cerebral palsy (CP). The medical record indicates "hypertonicity and permanent contractures affecting both extremities on one side." Based on these findings, the nurse identifies this type of CP as which of the following? a) Spastic hemiplegia b) Athetoid or dyskinetic c) Spastic diplegia d) Ataxic

Spastic hemiplegia Correct Explanation: Spastic hemiplegia involves hypertonicity and permanent contractures on both extremities on one side. Athetoid (dyskinetic) involves abnormal involuntary movements affecting all four extremities and sometimes the face, neck, and tongue. Ataxic affects balance and depth perception. Spastic diplegia affects the lower extremities.

A nurse is preparing a presentation for a health fair focusing on prevention of congenital neuromuscular disorders. Which of the following would the nurse emphasize as most important in preventing neural tube defects? a) Ultrasound screening at 16 weeks' gestation b) Folic acid supplementation c) Genetic testing for gene identification d) Maternal serum α-fetoprotein levels screening

Folic acid supplementation Correct Explanation: Strong evidence exists that folic acid supplementation by the mother before conception decreases the incidence of neural tube defects by 50%. Ultrasound screening at 16 to 18 weeks' gestation can help identify fetuses at risk, but this would not prevent neural tube defects. Screening of maternal serum α-fetoprotein levels can help identify fetuses at risk, but this would not prevent neural tube defects. Neural tube defects are not related to genetic dysfunction, so genetic testing would be of no value.

The nurse is caring for a 6-year-old boy with myelomeningocele. The nurse is teaching the mother how to promote appropriate bowel elimination and avoid constipation. Which response from the mother indicates a need for further teaching? a) "I can palpate his abdomen to assess for constipation" b) "I need to figure out his usual pattern for passing stool." c) "He must have an adequate amount of fluid." d) "My son's activity is too limited to stimulate his bowels."

"My son's activity is too limited to stimulate his bowels." Correct Explanation: The nurse needs to point out to the mother that even minimal activity increases peristalsis. Together they can come up with appropriate activities within the child's limits or restrictions to promote peristalsis. It is important to determine the usual pattern for passing stool so that the mother and nurse can determine the best program. Palpating the abdomen can reveal distention suggesting constipation. Adequate fluid is necessary to stimulate peristalsis.

A nurse is caring for a child with spina bifida. The child's mother asks the nurse what she did to cause the birth defect. Which statement would be the nurse's best response? a) "It has been linked to maternal alcohol consumption during pregnancy." b) "It's a common complication of amniocentesis." c) "The cause is unknown and there are many environmental factors that may contribute to it." d) "Older age at conception is one of the major causes of the defect."

"The cause is unknown and there are many environmental factors that may contribute to it." Explanation: There is no one known cause of spina bifida, but scientists believe that it's linked to hereditary and environmental factors; neural tube defects, including spina bifida, have been strongly linked to low dietary intake of folic acid. Maternal age doesn't have an impact on spina bifida. An amniocentesis is performed to help diagnose spina bifida in utero but doesn't cause the disorder. Maternal alcohol intake during pregnancy has been linked to mental retardation, craniofacial defects, and cardiac abnormalities, but not spina bifida.

The nurse is teaching the parents of a female child with a myelomeningocele how to perform clean intermittent catheterization. The nurse determines that the teaching was effective when the parents return demonstrate the procedure and state which of the following? a) "Before inserting the catheter, we need to wipe her labia with normal saline from back to front." b) "We need to insert the catheter about 6 inches so that we make sure the catheter is in the bladder." c) "We need to apply some petroleum jelly to her labia and the catheter before we attempt to insert it." d) "When the urine stops flowing, we should press on the lower belly to ensure the bladder is empty."

"When the urine stops flowing, we should press on the lower belly to ensure the bladder is empty." Correct Explanation: When the urine stops flowing, the parents should press on the lower abdomen or have the child lean forward to tense the abdominals to ensure that no more urine is in the bladder. For a female, the catheter is inserted about 2 to 3 inches. For a male, the catheter is inserted about 4 to 6 inches. Before the catheter is inserted, the labia is cleaned with a washcloth or disposable wipe from front to back. A generous amount of water-soluble lubricant, not petroleum jelly, is applied to the catheter. There is no need to apply the lubricant to the labia.

Why will it be necessary for the nurse to be very supportive of parents' attempts to feed the infant with recently repaired myelomeningocele? a) The infant will have a poor sucking reflex. b) Pain will interfere with the feeding process. c) Assuming the usual feeding position will be difficult. d) Nausea and vomiting often follow repair of the cystic mass.

Assuming the usual feeding position will be difficult. Correct Explanation: Because the repaired area will need to be protected, having to use an alternate feeding position is likely. The infant may need to be fed prone with the head turned to the side and may not be able to be held. Being able to provide food for the infant is central to parenting the child. Difficulty nurturing a child can be very stressful. Little pain will be experienced and should easily be controlled owing to loss of sensation in the area. The sucking reflex should not be affected by the myelomeningocele or its repair. Nausea and vomiting are unlikely after recovery from the anesthetic.

The nurse caring for a client diagnosed with muscular dystrophy would expect which laboratory values to be most abnormal? a) Creatinine b) Serum potassium c) Sodium d) Bilirubin

Creatinine Correct Explanation: Creatinine is a by-product of muscle metabolism as the muscle hypertrophies. Bilirubin is a by-product of liver function. Potassium and sodium levels can change due to various factors and aren't indicators of muscular dystrophy.

The nurse is assessing a child with spina bifida occulta. During the assessment, the parents say, "It's going to be so difficult taking care of our child. He'll never be able to walk." The nurse identifies which nursing diagnosis as the priority? a) Deficient knowledge related to diagnosis and condition b) Ineffective coping related to diagnosis of chronic condition c) Impaired physical mobility related to spinal cord defect d) Risk for injury related to lack of muscle control

Deficient knowledge related to diagnosis and condition Correct Explanation: The parents' statement indicates a lack of understanding about the condition. Spina bifida is a term that is often used to refer to all neural tube disorders that affect the spinal cord. This can be confusing and a cause of concern for parents. There are well-defined degrees of spinal cord involvement, and it is important for healthcare professionals to use the correct terminology. Spina bifida occulta is a defect of the vertebral bodies without protrusion of the spinal cord or meninges. This defect is not visible externally and in most cases has no adverse affects. In most cases, spina bifida occulta is benign and asymptomatic and produces no neurologic signs; it may be considered a normal variant. Mobility typically is not impaired with spina bifida occulta. The child is at no greater risk for injury as any other child. The parents demonstrate a lack of knowledge, not problems with coping.

A nurse who is discussing Duchenne muscular dystrophy characterizes it correctly using which descriptors? a) Duchenne muscular dystrophy is diagnosed in boys who develop gait changes during the late school-age years. b) Duchenne muscular dystrophy is a progressive disease of muscles and nerves that affects males and females equally. c) Duchenne muscular dystrophy causes progressive muscular weakness that ends in death. d) Duchenne muscular dystrophy is a nonprogressive disorder that severely affects muscle function through spinal cord atrophy.

Folic acid supplementation Correct Explanation: Strong evidence exists that folic acid supplementation by the mother before conception decreases the incidence of neural tube defects by 50%. Ultrasound screening at 16 to 18 weeks' gestation can help identify fetuses at risk, but this would not prevent neural tube defects. Screening of maternal serum α-fetoprotein levels can help identify fetuses at risk, but this would not prevent neural tube defects. Neural tube defects are not related to genetic dysfunction, so genetic testing would be of no value.

Which condition would alert the nurse that a child may be suffering from muscular dystrophy? a) Increased lumbar lordosis b) Upper extremity spasticity c) Hyperactive lower extremity reflexes d) Hypertonia of extremities

Increased lumbar lordosis Correct Explanation: An increased lumbar lordosis would be seen in a child suffering from muscular dystrophy secondary to paralysis of lower lumbar postural muscles. Increased lower extremity support may also be seen. Hypertonia isn't seen in this disease. Upper extremity spasticity isn't seen because this disease isn't caused by upper motor neuron lesions. Hyperactive reflexes aren't indications of muscular dystrophy.

Which characteristic is true of cerebral palsy? a) It results in mental retardation. b) It's progressive. c) It's reversible. d) It appears at birth or during the first 2 years of life.

It appears at birth or during the first 2 years of life. Correct Explanation: Cerebral palsy is an irreversible, nonprogressive disorder that results from damage to the developing brain during the prenatal, perinatal, or postnatal period. Although some children with cerebral palsy are mentally retarded, many have normal intelligence.

When a child is suspected of having muscular dystrophy, a nurse should expect which muscles to be affected first? a) Muscles of respiration b) Muscles of the foot c) Muscles of the hip d) Muscles of the hand

Muscles of the hip Correct Explanation: Positional muscles of the hip and shoulder are affected first. Progression later advances to muscles of the foot and hand. Involuntary muscles, such as the muscles of respiration, are affected last.

The nurse is caring for an infant with a myelomeningocele who has paralysis of the lower extremities. Which action would be most appropriate to help reduce friction resulting from this paralysis? a) Place the child on a special care mattress. b) Place a pad beneath the diaper area and change frequently. c) Place a folded diaper in between the legs. d) Place synthetic sheepskin under the infant's chest.

Place a folded diaper in between the legs. Correct Explanation: To protect the myelomeningocele, the child must always be placed in the prone position. Special attention to the infant's legs needs to occur when positioning them. Using a folded diaper in between the legs can help reduce pressure and friction from the legs rubbing together. Placing a pad beneath the diaper area helps to keep the child clean. Using a special care mattress helps to reduce pressure. Using sheepskin under the infant's chest reduces friction on the chest area but not the legs.

The nurse is taking a health history of a 6-year-old girl with suspected dermatomyositis. During the physical examination, which of the following would help confirm the nurse's suspicions? a) Ptosis or altered eye movements b) Tenting of skin c) Red-purple rash on upper eyelids, knuckles, elbows, and knees d) Delayed capillary refill

Red-purple rash on upper eyelids, knuckles, elbows, and knees Correct Explanation: The nurse would expect to find a rash involving the upper eyelids and extensor surfaces of the knuckles, elbows, and knees. Tenting of the skin would suggest dehydration. Ptosis or altered eye movements are more commonly seen with other forms of paralysis, like myasthenia gravis. Delayed capillary refill is associated with dehydration or problems involving circulation.

Based on knowledge of the progression of muscular dystrophy, which activity would a nurse anticipate the client having difficulty with first? a) Breathing b) Sitting c) Standing d) Swallowing

Standing Explanation: Muscular dystrophy usually affects postural muscles of the hip and shoulder first. Swallowing and breathing are usually affected last. Sitting may be affected, but a client would have difficulty standing before having difficulty sitting.

The young child has been diagnosed with Guillain-Barré syndrome and it is progressing in a classic manner. Rank the following sequence of events in the order that they typically occur. The child is having difficulty producing facial expressions. The child reports numbness and tingling in his toes. The child states that it is difficult to move his arms. The child states that it is difficult to move his legs.

The child reports numbness and tingling in his toes. The child states that it is difficult to move his legs. The child states that it is difficult to move his arms. The child is having difficulty producing facial expressions. Correct Explanation: Guillain-Barré syndrome paresthesias and muscle weakness. Classically it initially affects the lower extremities and progresses in an ascending manner to upper extremities and then the facial muscles. Progression is usually complete in 2 to 4 weeks, followed by a stable period leading to the recovery phase

Why does the nurse suspect cerebral palsy in the 8-month-old just assessed? a) The baby drools almost constantly. b) The child sits independently with a straight back. c) The child does not crawl. d) When startled, a strong Moro reflex is noted.

When startled, a strong Moro reflex is noted. Explanation: The Moro reflex is a primitive one that should have disappeared around 4 months of age. Persistence of this reflex may occur in children with cerebral palsy. Older children with cerebral palsy often drool owing to the inability to control oral muscles. Drooling in an 8-month-old is developmentally normal, as is sitting independently with the back straight. It fits developmentally that the 8-month-old does not yet crawl.