Pedi neuromuscular

20. An adolescent with a T4 spinal cord injury suddenly becomes dangerously hypertensive and bradycardic. Which intervention is appropriate? 1. Call the neurosurgeon immediately, as this sounds like sudden intracranial hypertension. 2. Check to be certain that the client's bladder is not distended. 3. Administer diazoxide (Hyperstat) to treat the blood pressure. 4. Administer atropine for bradycardia.

2. The first assessment is of the bladder and bowel to check for fullness. The presence of either could trigger autonomic dysreflexia.

12. The nurse knows that teaching has been successful when the parent of a child with muscle weakness states that the diagnostic test for muscular dystrophy is which of the following? 1. Electromyelogram. 2. Nerve conduction velocity. 3. Muscle biopsy. 4. Creatine kinase level.

3. Muscle biopsy confirms the type of myopathy that the patient has.

14. Which should a nurse in the ED be prepared for in a child with a possible spinal cord injury? Select all that apply. 1. Severe pain. 2. Elevated temperature. 3. Respiratory depression. 4. Increased intracranial pressure. 5. Multiple sites of injury.

3. Spinal cord injury without radiographic abnormality results from the spinal cord sliding between the vertebrae and then sliding back into place without injury to the bony spine. It is thought to be the result of an immature spinal column that allows for reduction after momentary subluxation. 5. The nurse should expect that the child may have multiple injury sites as many spinal cord injuries are the result of trauma from falls from heights, violence, or sporting injuries. A complete head- to-toe assessment of the child should be performed. Wrong answers: 1. Severe pain is unlikely, but the child may have pain at the injury site. 2. An elevated temperature is not common in a spinal cord injury. In fact, most trauma patients are hypothermic in spite of high ambient temperatures. 4. Spinal cord injury with an open or closed head injury does not cause an increase in intracranial pressure.

52. A child with GBS has had lots of oral fluids but has not urinated for 8 hours. Which is the nurse's first action? 1. Check the child's serum blood urea nitrogen level. 2. Check the child's complete blood count. 3. Catheterize the child in and out. 4. Run water in the bathroom to stimulate urination.

3. The child must be in-and-out catheterized to avoid the possibility of developing a urinary tract infection from urine left in the bladder for too long.

11. The nurse is caring for a school-age child with Duchenne muscular dystrophy in the elementary school. Which would be an appropriate nursing diagnosis? 1. Anticipatory grieving. 2. Anxiety reduction. 3. Increased pain. 4. Activity intolerance.

4. The child would not be able to keep up with peers because of weakness, progressive loss of muscle fibers, and loss of muscle strength.

17. Which symptoms will a child suffering from complete spinal cord injury experience? Select all that apply. 1. Loss of motor and sensory function below the level of the injury. 2. Loss of interest in normal activities. 3. Extreme pain below the level of the injury. 4. Loss of some function, with sparing of function below the level of the injury. 5. Loss of bowel and bladder control.

1. Children with complete spinal cord injury lose motor and sensory function below the level of the injury as a result of interruption of nerve pathways. 5. The nerves to the perineal are affected when injuries occur to the lumbar region. The child develops a neurogenic bladder and bowel.

25. Which intervention should be included in the plan of care for a newborn with a newly repaired myelomeningocele? 1. Offer formula/breast milk every 3 hours. 2. Turn the infant back to front every 2 hours. 3. Place a wet dressing on the sac. 4. Provide pain medication every 4 hours.

1. Following surgery, a newborn may want formula/breast milk every 2 to 4 hours. Be sure to monitor intake and output. Wrong answers: 2. Following surgery, the infant should not be positioned on the back. Monitor respirations when the infant is on the abdomen. 3. Before surgery, priority care for an infant with a myelomeningocele is to protect the sac. 4. Infants with myelomeningocele do not have pain because of lack of nerve innervations below the level of the defect.

6. A 5-year-old has been diagnosed with pseudohypertrophic muscular dystrophy. Which nursing intervention(s) would be appropriate? Select all that apply. 1. Discuss with the parents the potential need for respiratory support. 2. Explain that this disease is easily treated with medication. 3. Suggest exercises that will limit the use of muscles and prevent fatigue. 4. Assist the parents in finding a nursing facility for future care. 5. Encourage the parents to contact the school to develop an IEP.

1. Muscles become weaker, including those needed for respiration, and a decision will need to be made about whether respiratory support will be provided. 3. Physical therapy will be part of the treatment plan, but respiratory support is a priority. 5. Parents should be encouraged to allow the child to go to school and participate in activities as tolerated. Wrong answers: 2. This is a progressive disease, which medications do not treat. 4. The parents need to decide eventually if they will keep the child home or cared for in a nursing facility, but that is not an immediate concern.

3. The parents of a preschooler diagnosed with muscular dystrophy are asking questions about the course of their child's disease. Which should the nurse tell them? Select all that apply. 1. "Muscular dystrophies usually result in progressive weakness." 2. "The weakness that your child is having will probably not increase." 3. "Your child will be able to function normally and not need any special accommodations." 4. "The extent of weakness depends on doing daily physical therapy." 5. "Your child may have pain in his legs with muscle weakness."

1. Muscular dystrophies are progressive degenerative disorders. The most common is Duchenne muscular dystrophy, which is an X-linked recessive disorder. 5. The child may have pain due to loss of strength and muscle wasting. Wrong answers: 2. The weakness is progressive. 3. The child will require assistance, and the need for it will increase with time and age. 4. Daily therapy may be helpful in decreasing contractures, although it will not deter the disease progression.

13. Why does spinal cord injury without radiographic abnormality sometimes occur in children? 1. Children can suffer momentary severe subluxation and trauma to the spinal cord. 2. The immature spinal column in children does not allow for quality films. 3. The hemorrhaging that occurs with injury obscures radiographic abnormalities. 4. Radiographic abnormalities are not evident because of incomplete ossification of the vertebrae.

1. Spinal cord injury without radiographic abnormality results from the spinal cord sliding between the vertebrae and then sliding back into place without injury to the bony spine. It is thought to be the result of an immature spinal column that allows for reduction after momentary subluxation.

18. The nurse is planning care for a child with a T12 spinal cord injury. Which lifelong complication should the child and family know about? Select all that apply. 1. Skin integrity. 2. Incontinence. 3. Loss of large and small motor activity. 4. Loss of voice. 5. Spasticity.

1. Spinal cord-injury clients experience many issues because of the loss of innervation below the level of the injury. Skin integrity and incontinence are issues because of immobility and loss of pain receptors below the level of the injury. 2. Skin integrity and incontinence are issues because of immobility and loss of pain receptors below the level of the injury. 3. Loss of motor activity is also a result of loss of innervation below the level of the injury. 5. With incomplete severing of the spinal cord, flaccid spasticity occurs initially as a result of the injury and shock, but oftentimes converts to muscle spasticity during the rehabilitation stage.

8. Which will help a school-age child with muscular dystrophy stay active longer? Select all that apply. 1. Normal activities, such as swimming. 2. Using a treadmill every day. 3. Several periods of rest every day. 4. Using a wheelchair upon getting tired. 5. Sleeping as late as needed.

1. Swimming is an excellent exercise that uses many muscles and helps build strength. Children who are active are usually able to postpone use of a wheelchair. It is important to keep using muscles for as long as possible, and aerobic activity is good for a child. 3. Any child with a chronic disease should be kept as active as possible for as long as possible; short rest periods built into the day are helpful in maintaining stamina. 4. Children with neuromuscular diseases oftentimes will use a wheelchair to conserve energy and increase mobility. The wheelchair acts as the child's means of getting to where they want to go as independently as possible. Wrong answers: 2. Use of a treadmill is not fun for children or adults, so keeping the child using the treadmill might be an issue. 5. The child should be on a regular daily schedule including the same bedtime and getting up time. Rest times should be provided during the day.

61. Causes of autonomic dysreflexia include which of the following? Select all that apply. 1. Decrease in blood pressure. 2. Abdominal distention. 3. Bladder distention. 4. Diarrhea. 5. Tight clothing. 6. Hyperthermia.

2. Autonomic dysreflexia may be caused by abdominal pressure from a fecal impaction. 3. An distended bladder is usually the precipitating factor causing an increase in abdominal pressure. 5. Tight clothing can increase pressure to the central core of the body. Wrong answers: 1. A decrease in blood pressure does not contribute to autonomic dysreflexia. Increased blood pressure usually occurs with autonomic dysreflexia. 4. Fecal impaction and constipation, not diarrhea, can be causes of autonomic dysreflexia 6. Hyperthermia does not cause autonomic dysreflexia.

15. The nurse evaluates the teaching as successful when a parent states that which of the following can cause autonomic dysreflexia? 1. Exposure to cold temperatures. 2. Distended bowel or bladder. 3. Bradycardia. 4. Headache.

2. Autonomic dysreflexia results from an uncontrolled, paroxysmal, continuous lower motor neuron reflex arc due to stimulation of the sympathetic nervous system. It is a response that typically results from stimulation of sensory receptors such as a full bladder or bowel.

27. Which should be the priority nursing diagnosis for a 12-hour-old newborn with a myelomeningocele at L2? 1. Altered bowel elimination related to neurological deficits. 2. Potential for infection related to the physical defect. 3. Altered nutrition related to neurological deficit. 4. Disturbance in self-concept related to physical disability.

2. Because this infant has not had a repair, the sac is exposed. It could rupture, allowing organisms to enter the CSF, so this is the priority.

10. The nurse knows that teaching was successful when a parent states which of the following are early signs of muscular dystrophy? 1. Increased muscle strength. 2. Difficulty climbing stairs. 3. High fevers and tiredness. 4. Respiratory infections and obesity.

2. Difficulty climbing stairs, running, and riding a bicycle are frequently the first symptoms of Duchenne muscular dystrophy.

26. Which should the nurse do first when caring for an infant who just had a repair of a myelomeningocele? 1. Weigh diapers for 24-hour urine output. 2. Measure head circumference. 3. Offer clear fluids. 4. Assess for infection.

2. Hydrocephalus occurs in about 90%of infants with myelomeningocele, so measuring the head circumference daily and watching for an increase are important. Accumulation of cerebrospinal fluid can occur after closure of the sac.

19. After spinal cord surgery, an adolescent suddenly complains of a severe headache. Which should be the nurse's first action? 1. Check the blood pressure. 2. Check for a full bladder. 3. Ask if pain is present somewhere else. 4. Ask if other symptoms are present.

2. The sympathetic nervous system responds to a full bladder or bowel resulting from an uncontrolled, paroxysmal, continuous lower motor neuron reflex arc. This response is usually from stimulation of sensory receptors (e.g., distended bladder or bowel). Because the efferent pulse cannot pass through the spinal cord, the vagus nerve is not "turned off," and profound symptomatic bradycardia may occur.

34. A newborn with a repaired myelomeningocele is assessed for hydrocephalus. Which would the nurse expect in an infant with hydrocephalus? 1. Low-pitched cry and depressed fontanel. 2. Low-pitched cry and bulging fontanel. 3. Bulging fontanel and downwardly rotated eyes. 4. Depressed fontanel and upwardly rotated eyes.

3. An alteration in the circulation of the cerebrospinal fluid causes hydrocephalus. The anterior fontanel bulges because of an increase in cerebrospinal fluid, and an increase in intracranial pressure causes a high-pitched cry in infants and downward deviation of the eyes, also called sunset eyes. With sunset eyes, the sclera can be seen above the iris.

4. The nurse should tell the parents of a child with Duchenne (pseudohypertrophic) muscular dystrophy that some of the progressive complications include: Select all that apply. 1. Dry skin and hair, hirsutism, protruding tongue, and mental retardation. 2. Anorexia, gingival hyperplasia, dry skin and hair. 3. Contractures, obesity, and pulmonary infections. 4. Trembling, frequent loss of consciousness, and slurred speech. 5. Increasing difficulty swallowing and shallow breathing.

3. The major complications of muscular dystrophy include contractures, disuse atrophy, infections, obesity, respiratory complications, and cardiopulmonary problems. 5. The muscles of a child with MD tend to show increasing weakness and atrophy over time. The children are at risk for swallowing, aspiration, and pneumonia. Wrong answers: 1. These symptoms are common with Down syndrome. 2. Duchenne muscular dystrophy does not produce these symptoms. 4. These symptoms are evidence of a possible head injury.

The nurse is caring for an infant with myelomeningocele who is going to surgery later today for closure of the sac. Which would be a priority nursing diagnosis before surgery? 1. Alteration in parent-infant bonding. 2. Altered growth and development. 3. Risk of infection. 4. Risk for weight loss.

3. The unrepaired myelomeningocele is oftentimes a thin membrane that covers the neural contents of the spine. A normal saline dressing is placed over the sac to prevent tearing. The tearing would allow the CSF to escape and microorganisms to enter. The infant is at high risk for spinal cord infections. The priority nursing diagnosis is risk of infection.

54. Which should the nurse expect as an intervention in a child in the recovery phase of GBS? Select all that apply. 1. Assist with self-feeding skills. 2. Assist with grooming and dressing. 3. Arrange for in-home schooling. 4. Begin an active physical therapy program. 5. Begin active PT.

3. Working with the child's teacher and the school staff to keep the child at the same pace as his classmates is crucial for the healthy recovery of the child. 5. Beginning active physical therapy is important for helping muscle recovery and preventing contractures. Wrong answers: 1. Referral to OT would have been done depending on the degree of fine motor involvement. 2. Because grooming and dressing are life skills the child may need to relearn, referral to PT would be appropriate and done earlier in the course of treatment. 4. GBS does not affect cognitive functioning.

7. Which foods would be best for a child with Duchenne muscular dystrophy? Select all that apply. 1. High-carbohydrate, high-protein foods. 2. No special food combinations. 3. Extra protein to help strengthen muscles. 4. Low-calorie foods to prevent weight gain. 5. Thickened liquids and smaller portions that are cut up.

4. As the child becomes less ambulatory, moving the child will become more of a problem. It is not good for the child to become overweight for several health reasons in addition to decreased ambulation. 5. As the child loses muscle control, the need for thickened liquids and small, well-cut-up solids becomes essential. Wrong answers: 1. As the child with muscular dystrophy becomes less active, diet becomes more important. Attention should be paid to quality and quantity of food, so the child does not gain too much weight. 2. Good-quality foods are important as the child continues to grow. 3. Extra protein will not help the child recover from this disease.

Which priority item should be placed at the bedside of a newborn with myelomeningocele? Select all that apply. 1. A bottle of normal saline. 2. A rectal thermometer. 3. Extra blankets. 4. A blood pressure cuff. 5. Latex-free gloves.

1. Before the surgical closure of the sac, the infant is at risk for infection. A sterile dressing is placed over the sac to keep it moist and help prevent it from tearing. Because the dressings dry out at least every hour, it is important to assess them frequently and apply saline as needed. Good hand washing is also important. 5. Latex-free clean gloves would be used for all care of this infant. A box should be kept at the infant's bedside. Children with spina bifida are at risk for latex allergy and should not be exposed to latex. Wrong answers: 2. The infant's temperature will be taken, but prevention of infection is the priority. 3. Infants with myelomeningocele intact are often naked and placed in an incubator for warmth. Temperature does need to be maintained and the infant protected from sick people. 4. Blood pressure is difficult to monitor in the newborn period, especially if the baby is lying on the abdomen. Prevention of infection is the priority.

9. The mother of a child with Duchenne muscular dystrophy asks the nurse who in the family should have genetic screening. Who should the nurse say must be tested? Select all that apply. 1. Mother. 2. Sister. 3. Brother. 4. Aunts and all female cousins. 5. Uncles and all male cousins.

1. Genetic counseling is important in all inherited diseases. Duchenne muscular dystrophy is inherited as an X-linked recessive trait, meaning the defect is on the X chromosome. Women carry the disease, and males are affected. All female relatives should be tested. 2. The X chromosome carries the disease, and males are affected. The sistershould have genetic testing to determine whether she carries the gene and identify her risks for having male offspring with the disease. 4. The X chromosome carries the disease and males are affected. All female relatives should be tested.

Which should the nurse include when teaching sexuality education to an adolescent with a spinal cord injury? Select all that apply. 1. "You can enjoy a healthy sex life and most likely conceive children." 2. "You will never be able to conceive if you have no genital sensation." 3. "Development of secondary sex characteristics is delayed." 4. "A few females have regular menstrual periods after injury." 5. "You can get the same sexually transmitted infections that those without spinal cord injuries get."

1. The reproductive system continues to function properly after a spinal cord injury. Much sexual activity and response occurs in the brain as well. 4. Females may have irregular periods after the injury, but most return to their normal cycles. 5. Those with spinal cord injuries can acquire sexually transmitted infections and need to use safe sex practices.

29. Which should the nurse tell the parent of an infant with spina bifida? 1. "Bone growth will be more than that of babies who are not sick because your baby will be less active." 2. "Physical and occupational therapy will be helpful to stimulate the senses and improve cognitive skills." 3. "Nutritional needs for your infant will be calculated based on activity level." 4. "Fine motor skills will be delayed because of the disability."

2. Children with decreased activity due to illness or trauma are helped by physical and occupational therapy. The varied activities stimulate the senses. wrong answers: 1. Bone growth is related to weight-bearing as well as to secretion of the growth hormone. Decreased activity usually results in less bone growth. 3. This is partially true. Nutritional needs in children are also calculated based on growth needs. 4. Many children with myelomeningocele have low-level defects, usually in the lumbar area, which do not affect upper body fine motor skills.

53. The nurse is planning care for a child who was recently admitted with GBS. Which is a priority nursing diagnosis? 1. Risk for constipation related to immobility. 2. Chronic sorrow related to presence of chronic disability. 3. Impaired skin integrity related to infectious disease process. 4. Activity intolerance related to ineffective cardiac muscle function.

3. The goal is to prevent complications related to immobility. Efforts include maintaining skin integrity, maintaining respiratory function, and preventing contractures. Wrong answers: 1. The goal is to prevent complications related to immobility. Efforts include maintaining skin integrity, maintaining respiratory function, and preventing contractures. Constipation is a concern, but not the primary concern. 2. Most children recover completely, so there is no chronic sorrow. 4. GBS is a disease affecting the peripheral nervous system, not the cardiac muscle.

51. A child presents with a history of having had an upper respiratory tract infection 2 weeks ago; complains of symmetrical lower extremity weakness, back pain, muscle tenderness; and has absent deep tendon reflexes in the lower extremities. Which is important regarding this condition? 1. The disease process is probably bacterial. 2. The recent upper respiratory infection is not important information. 3. This may be an acute inflammatory demyelinating neuropathy. 4. CN involvement is rare.

3. This child probably has GBS, which is an acute inflammatory demyelinating neuropathy.

5. Which can elicit the Gower sign? Have the child: 1. Close the eyes and touch the nose with alternating index fingers. 2. Hop on one foot and then the other. 3. Bend from the waist to touch the toes. 4. Walk like a duck and rise from a squatting position.

4. Children with muscular dystrophy display the Gower sign, which is great difficulty rising and standing from a squatting position because of the lack of muscle strength. Wrong answers: 1. This is the Romberg sign, which measures balance. 2. This test measures balance and coordination. 3. This test measures flexibility.