Chapter 34: The Child with Neuromuscular or Muscular Dysfunction

A 12-year-old child with Guillain-Barré syndrome (GBS) is admitted to the pediatric intensive care unit. She tells you that yesterday her legs were weak and that this morning she was unable to walk. After the nurse determines the current level of paralysis, which should the next priority assessment be? a. Swallowing ability b. Parental involvement c. Level of consciousness d. Antecedent viral infections

ANS: A Assessment of swallowing is essential. Both pharyngeal involvement and respiratory function are usually involved at the same time. The child may require ventilatory support. The inability to swallow also contributes to aspiration pneumonia. Parental involvement is important after the physiologic assessment is complete. The child is answering questions and describing the onset of the illness, which demonstrates she is alert and oriented. Information regarding antecedent viral infections can be obtained after the child is assessed and stabilized.

The clinic nurse is assessing infant reflexes. What assessment indicates a persistence of primitive reflexes? a. Tonic neck reflex at 8 months of age b. Palmar grasp at 4 months of age c. Plantar grasp at 9 months of age d. Rooting reflex at 3 months of age

ANS: A Persistence of primitive reflexes is one of the earliest clues to CP (e.g., obligatory tonic neck reflex at any age or nonobligatory persistence beyond 6 months of age and the persistence or even hyperactivity of the Moro, plantar, and palmar grasp reflexes). The palmar grasp disappears by 6 months, the plantar grasp disappears by 12 months, and the rooting reflex disappears at 4 months, so these are normal findings.

The nurse is teaching the family of an infant with cerebral palsy how to administer a diazepam (Valium) pill by gastrostomy tube. What should the nurse include in the teaching session? a. The pill should be crushed and mixed with a small amount of water. b. The pill should be crushed and mixed with the infant's formula. c. After administering the medication, flush the tube with air. d. Before administering the medication, check the placement of the tube.

ANS: A Pills may be crushed and mixed with small amounts of water but not other liquids, such as formula or elixir medications, because these may act together to form a sludge that can interfere with gastrostomy tube function. When crushed pills or tablets are administered, flush the feeding tube with more water after instilling the dissolved pill in water. The tube should not be flushed with air, and placement does not need to be checked because it is directly into the stomach.

An adolescent whose leg was crushed when she fell off a horse is admitted to the emergency department. She has completed the tetanus immunization series, receiving the last tetanus toxoid booster 8 years ago. What care is necessary for therapeutic management of this adolescent to prevent tetanus? a. Tetanus toxoid booster is needed because of the type of injury. b. Human tetanus immunoglobulin is indicated for immediate prophylaxis. c. Concurrent administration of both tetanus immunoglobulin and tetanus antitoxin is needed. d. No additional tetanus prophylaxis is indicated. The tetanus toxoid booster is protective for 10 years.

ANS: A Protective levels of antibody are maintained for at least 10 years. Children with serious "tetanus-prone" wounds, including contaminated, crush, puncture, or burn wounds, should receive a tetanus toxoid booster prophylactically as soon as possible. This adolescent has circulating antibodies. The immunoglobulin is not indicated.

The nurse is caring for a neonate born with a myelomeningocele. Surgery to repair the defect is scheduled the next day. What is the most appropriate way to position and feed this neonate? a. Prone with the head turned to the side b. On the side c. Supine in an infant carrier d. Supine, with defect supported with rolled blankets

ANS: A The prone position with the head turned to the side for feeding is the optimum position for the infant. It protects the spinal sac and allows the infant to be fed without trauma. The side-lying position is avoided preoperatively. It can place tension on the sac and affect hip dysplasia if present. The infant should not be placed in a supine position.

The nurse is preparing to admit a 2-year-old child with spina bifida occulta. What clinical manifestations of spina bifida occulta should the nurse expect to observe? (Select all that apply.) a. Dark tufts of hair b. Skin depression or dimple c. Port-wine angiomatous nevi d. Soft, subcutaneous lipomas e. Bladder and sphincter paralysis

ANS: A, B, C, D Clinical manifestations of spina bifida occulta include dark tufts of hair; skin depression or dimple; port-wine angiomatous nevi; and soft, subcutaneous lipomas. Bladder and sphincter paralysis are present with spina bifida cystica but not occulta.

The nurse is preparing to admit a 7-year-old child with ataxic cerebral palsy. What clinical manifestations of ataxic cerebral palsy should the nurse expect to observe? (Select all that apply.) a. Wide-based gait b. Rapid, repetitive movements performed poorly c. Slow, twisting movements of the trunk or extremities d. Hypertonicity with poor control of posture, balance, and coordinated motion e. Disintegration of movements of the upper extremities when the child reaches for objects

ANS: A, B, E Clinical manifestations of ataxic cerebral palsy include a wide-based gait; rapid, repetitive movements performed poorly; and disintegration of movements of the upper extremities when the child reaches for objects. Slow, twisting movements of the trunk are seen with dyskinetic cerebral palsy, and hypertonicity with poor control of posture, balance, and coordinated motion are seen with spastic cerebral palsy.

The nurse is preparing to admit a 5-year-old child with a lower motor neuron syndrome. What clinical manifestations of a lower motor neuron syndrome should the nurse expect to observe? (Select all that apply.) a. Loss of hair b. Babinski reflex present c. Skin and tissue changes d. Marked atrophy of atonic muscle e. Hyperreflexia with tendon reflexes exaggerated

ANS: A, C, D Clinical manifestations of a lower motor neuron syndrome include loss of hair, skin and tissue changes, and marked atrophy of atonic muscle. Babinski reflex present and hyperreflexia with tendon reflexes exaggerated are manifestations of an upper motor neuron syndrome.

A goal for children with spina bifida is to reduce the chance of allergy development. What is a priority nursing intervention? a. Recommend allergy testing. b. Provide a latex-free environment. c. Use only powder-free latex gloves. d. Limit use of latex products as much as possible.

ANS: B A latex-free environment is the goal. This includes eliminating the use of latex gloves and other medical devices containing latex. Allergy testing would provide information about whether the allergy has developed. It will not reduce the chances of developing the allergy. Although powder-free latex gloves are less allergenic, latex should not be used. Limiting the use of latex products is one component of providing a latex-free environment, but latex products should not be used.

The nurse is preparing a staff education in-service session for a group of new graduate nurses who will be working in a long-term care facility for children; many of the children have cerebral palsy (CP). What statement should the nurse include in the training? a. Children with dyskinetic CP have a wide-based gait and repetitive movements. b. Children with spastic pyramidal CP have a positive Babinski sign and ankle clonus. c. Children with hemiplegia CP have mouth muscles and one lower limb affected. d. Children with ataxic CP have involvement of pharyngeal and oral muscles with dysarthria.

ANS: B CP has a variety of clinical classifications. Spastic pyramidal CP includes manifestations such as a positive Babinski sign and ankle clonus; ataxic CP has a wide-based gait and repetitive movements; hemiplegia CP is characterized by motor dysfunction on one side of the body with upper extremity more affected than lower limbs; and dyskinetic CP involves the pharyngeal and oral muscles, causing drooling and dysarthria.

The nurse should suspect a child has cerebral palsy (CP) if the parent says what? a. "My 6-month-old baby is rolling from back to prone now." b. "My 4-month-old doesn't lift his head when on his tummy." c. "My 8-month-old can sit without support." d. "My 10-month-old is not walking."

ANS: B Delayed gross motor development is a universal manifestation of CP. The child shows a delay in all motor accomplishments, and the discrepancy between motor ability and expected achievement tends to increase with successive developmental milestones as growth advances. The infant who does not lift his head when on the tummy is showing a gross motor delay, as that is seen at 0 to 3 months. The other statements are within normal growth and development expectations.

A child, age 3 years, has cerebral palsy (CP) and is hospitalized for orthopedic surgery. His mother says he has difficulty swallowing and cannot hold a utensil to feed himself. He is slightly underweight for his height. What is the most appropriate nursing action related to feeding this child? a. Bottle or tube feed him a specialized formula until he gains sufficient weight. b. Stabilize his jaw with caregiver's hand (either from a front or side position) to facilitate swallowing. c. Place him in a well-supported, semi-reclining position. d. Place him in a sitting position with his neck hyperextended to make use of gravity flow.

ANS: B Jaw control is compromised in many children with CP. More normal control is achieved if the feeder stabilizes the oral mechanisms from the front or side of the face. Bottle or tube feeding will not improve feeding without jaw support. The semi-reclining position and hyperextended neck position increase the chances of aspiration.

The most important nursing intervention when caring for an infant with myelomeningocele in the preoperative stage is which? a. Take vital signs every hour. b. Place the infant in the prone position to minimize tension on the sac. c. Watch for signs that might indicate developing hydrocephalus. d. Apply a heat lamp to facilitate drying and toughening of the sac.

ANS: B The spinal sac is protected from damage until surgery is performed. Early surgical closure is recommended to prevent local trauma and infection. Monitoring vital signs and watching for signs that might indicate developing hydrocephalus are important interventions, but preventing trauma to the sac is a priority. The sac is kept moist until surgical intervention is done.

The nurse is preparing to admit a 7-year-old child with an upper motor neuron syndrome. What clinical manifestations of an upper motor neuron syndrome should the nurse expect to observe? (Select all that apply.) a. No flexor spasms b. Babinski reflex present c. No wasting of muscle mass d. Marked atrophy of atonic muscle e. Hyperreflexia with tendon reflexes exaggerated

ANS: B, C, E Clinical manifestations of an upper motor neuron syndrome include Babinski reflex present, no wasting of muscle mass, and hyperreflexia with tendon reflexes exaggerated. No flexor spasms and marked atrophy of atonic muscle are manifestations of a lower motor neuron syndrome.

The nurse is preparing to admit a 5-year-old with spina bifida cystica that was below the second lumbar vertebra. What clinical manifestations of spina bifida cystica below the second lumbar vertebra should the nurse expect to observe? (Select all that apply.) a. No motor impairment b. Lack of bowel control c. Soft, subcutaneous lipomas d. Flaccid, partial paralysis of lower extremities e. Overflow incontinence with constant dribbling of urine

ANS: B, D, E The clinical manifestations of spina bifida cystica below the second lumbar vertebra include lack of bowel control, flaccid, partial paralysis of lower extremities, and overflow incontinence with constant dribbling of urine. No motor impairment occurs with spina bifida cystica that was below the third lumbar vertebra, and soft, subcutaneous lipomas occur with spina bifida occulta.

The parents of an infant with cerebral palsy (CP) ask the nurse if their child will have cognitive impairment. The nurse's response should be based on which knowledge? a. Affected children have some degree of cognitive impairment. b. Around 20% of affected children have normal intelligence. c. About 30% to 50% of affected children have significant cognitive impairments. d. Cognitive impairment is expected if motor and sensory deficits are severe.

ANS: C Children with CP have a wide range of intelligence, and 30% to 50% have significant cognitive impairments. A large percentage of children with CP do not have mental impairment. Many individuals who have severely limiting physical impairment have the least amount of intellectual compromise.

A feeding technique the nurse can teach to parents of a child with cerebral palsy to improve use of the lips and the tongue to facilitate speech is which? a. Feeding pureed foods b. Placing food on the tongue c. Placing food at the side of the tongue d. Placing food directly into the mouth with a spoon

ANS: C Feeding techniques such as forcing the child to use the lips and tongue in eating facilitate speech. An example of this technique is placing food at the side of the tongue, first one side and then the other, and making the child use the lips to take food from a spoon rather than placing it directly on the tongue. Feeding pureed foods would not encourage use of the lips and tongue.

During a well-child visit, the mother tells the nurse that her 4-month-old infant is constipated, is less active than usual, and has a weak-sounding cry. The nurse suspects botulism and questions the mother about the child's diet. What factor should support this diagnosis? a. Breastfeeding b. Commercial formula c. Infant cereal with honey d. Improperly sterilized bottles

ANS: C Ingestion of honey is a risk factor for infant botulism in the United States. Honey should not be given to children younger than the age of 1 year. Botulism is not found with the use of commercial infant cereals. Although there is a slight increase in botulism in breastfed infants when compared with formula-fed infants, there is not sufficient evidence to support formula feeding as prevention. Thoroughly cleaning bottles used for formula feeding is sufficient for botulism prevention. Inadequate sterilization of home-canned foods can contribute to botulism.

A 14-year-old girl is in the intensive care unit after a spinal cord injury 2 days ago. What nursing intervention is a priority for this child? a. Minimizing environmental stimuli b. Administering immunoglobulin c. Monitoring and maintaining systemic blood pressure d. Discussing long-term care issues with the family

ANS: C Spinal cord injury patients are physiologically labile, and close monitoring is required. They may be unstable for the first few weeks after the injury. Increased blood pressure may be an indication of autonomic dysreflexia. It is not necessary to minimize environmental stimuli for this type of injury. Spinal cord injury is not an infectious process. Immunoglobulin is not indicated. 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.

An adolescent has just been brought to the emergency department with a spinal cord injury and paralysis from a diving accident. The parents keep asking the nurse, "How bad is it?" The nurse's response should be based on which knowledge? a. Families adjust better to life-threatening injuries when information is given over time. b. Immediate loss of function is indicative of the long-term consequences of the injury. c. Extent and severity of damage cannot be determined for several weeks or even months. d. Numerous diagnostic tests will be done immediately to determine extent and severity of damage.

ANS: C The extent and severity of damage cannot be determined initially. The immediate loss of function is caused by anatomic and impaired physiologic function, and improvement may not be evident for weeks or months. It is essential to provide information about the adolescent's status to the parents. Immediate treatment information should be provided. Long-term rehabilitation and prognosis can be addressed after the child is stabilized. During the immediate postinjury period, physiologic responses to the injury make an accurate assessment of damage difficult.

An 8-year-old girl with moderate cerebral palsy (CP) recently began joining a regular classroom for part of the day. Her mother asks the school nurse about joining the after-school Girl Scout troop. The nurse's response should be based on which knowledge? a. Most activities such as Girl Scouts cannot be adapted for children with CP. b. After-school activities usually result in extreme fatigue for children with CP. c. Trying to participate in activities such as Girl Scouts leads to lowered self-esteem in children with CP. d. Recreational activities often provide children with CP with opportunities for socialization and recreation.

ANS: D After-school and recreational activities serve to stimulate children's interest and curiosity. They help the children adjust to their disability, improve their functional ability, and build self-esteem. Increasing numbers of programs are adapted for children with physical limitations. Almost all activities can be adapted. The child should participate to her level of energy. Self-esteem increases as a result of the positive feedback the child receives from participation.

What functional ability should the nurse expect in a child with a spinal cord lesion at C7? a. Complete respiratory paralysis b. No voluntary function of upper extremities c. Inability to roll over or attain sitting position d. Almost complete independence within limitations of wheelchair

ANS: D Individuals who sustain injuries at the C7 level are able to achieve a significant level of independence. Some assistance is needed with transfers and lower extremity dressing. Patients are able to roll over in bed and to sit and eat independently. Patients with injuries at C3 or higher have complete respiratory paralysis. Those with injuries at C4 or higher do not have voluntary function of higher extremities. Injuries at C5 or higher prevent rolling over or sitting.

An 8-year-old child is hospitalized with infectious polyneuritis (Guillain-Barré syndrome [GBS]). When explaining this disease process to the parents, what should the nurse consider? a. Paralysis is progressive with little hope for recovery. b. Disease is inherited as an autosomal, sex-linked, recessive gene. c. Disease results from an apparently toxic reaction to certain medications. d. Muscle strength slowly returns, and most children recover.

ANS: D Recovery usually begins within 2 to 3 weeks, and most patients regain full muscle strength. The paralysis is progressive with proximal muscle weakness occurring before distal weakness. The recovery of muscle strength occurs in the reverse order of onset of paralysis. Most individuals regain full muscle strength. Better outcomes are associated with younger ages. GBS is an immune-mediated disease often associated with a number of viral or bacterial infections or the administration of vaccines.

When a child develops latex allergy, which food may also cause an allergic reaction? a. Yeast b. Wheat c. Peanuts d. Bananas

ANS: D There are cross-reactions between allergies to latex and to a number of foods such as bananas, avocados, kiwi, and chestnuts. Although yeast, wheat, and peanuts are potential allergens, currently they are not known to cross-react with latex allergy.

A woman who is 6 weeks pregnant tells the nurse that she is worried that, even though she is taking folic acid supplements, the baby might have spina bifida because of a family history. The nurse's response should be based on what? a. Prenatal detection is not possible yet. b. There is no genetic basis for the defect. c. Chromosome studies done on amniotic fluid can diagnose the defect prenatally. d. Open neural tube defects (NTDs) result in elevated concentrations of alpha-fetoprotein in amniotic fluid.

ANS: D Ultrasound scanning and measurement of alpha-fetoprotein may indicate the presence of anencephaly or myelomeningocele. The optimum time for performing this analyzing is between 16 and 18 weeks. Prenatal diagnosis is possible through amniocentesis. A multifactorial origin is suspected, including drugs, radiation, maternal malnutrition, chemicals, and possibly a genetic mutation. Chromosome abnormalities are not present in NTDs.