PEDIATRIC SUCCESS NEUROMUSCULAR OR MUSCULAR DISORDERS CHAPTER 11

4. The nurse is teaching the parents of a child with Duchenne (pseudohypertrophic) muscular dystrophy. The nurse should tell them that some of the progressive complications include which of the following? 1. Dry skin and hair, hirsutism, protruding tongue, and mental retardation. 2. Anorexia, gingival hyperplasia, and dry skin and hair. 3. Contractures, obesity, and pulmonary infections. 4. Trembling, frequent loss of consciousness, and slurred speech.

3. The major complications of muscular dystrophy include contractures, disuse atrophy, infections, obesity, respiratory complications, and cardiopulmonary problems.

42. The nurse is doing a follow-up assessment of a 9-month-old. The infant rolls both ways, sits with some support, pushes food out of the mouth, and pushes away when held. The parent asks about the infant's development. The nurse responds by saying which of the following? 1. "Your child is developing normally." 2. "Your child needs to see the primary care provider." 3. "You need to help your child learn to sit unassisted." 4. "Push the food back when your child pushes food out."

2. A 9-month-old should be able to sit alone, crawl, pull up, not push food out of the mouth (tongue thrust), and push away when held when wanting to get down. This child is not developing normally and must see the primary care provider.

16. The nurse receives a call from the local Emergency Medical Services stating that an ambulance is arriving with an 8-month-old with a decreased level of consciousness. When assessing the neurological status of an 8-month-old, the nurse should check for which of the following? 1. Clarity of speech. 2. Interaction with staff. 3. Developmental delay. 4. Ability to follow instructions.

2. Assessment for alteration in developmentally expected behaviors, such as stranger anxiety, is helpful. Interaction with staff is not to be expected due to stranger anxiety.

10. The nurse is teaching family members of a child newly diagnosed with muscular dystrophy about early signs. The nurse knows that teaching was successful when a parent states that which of the following signs may indicate the condition early? 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.

44. A 3-year-old child with CP is admitted for dehydration following an episode of diar rhea. The nurse's assessment follows: awake, pale, thin child lying in bed, multiple contractures, drooling, coughing spells noted when parent feeds. T 97.8°F (36.5°C), P 75, R 25, weight 7.2 kg, no diarrheal stool for 48 hours. Which of the following nursing diagnoses is most important? 1. Potential for skin breakdown: lying in one position. 2. Alteration in nutrition: less than body requirements. 3. Potential for impaired social support: mother sole caretaker. 4. Alteration in elimination: diarrhea.

2. This is the priority nursing diagnosis for this severely underweight child. Weight is average for a 4-month-old. The coughing episodes while feeding may indicate aspiration. The parent needs help to learn how to feed so less coughing occurs.

3. The parents of a preschooler diagnosed with muscular dystrophy are asking ques tions about the course of their child's disease. The nurse should tell them which of the following? 1. Muscular dystrophies are disorders associated with progressive degeneration of muscles, resulting in relentless and increasing weakness. 2. The weakness that the child is currently experiencing will probably not increase. 3. The child will be able to function normally and require no special accommodations. 4. The extent of degeneration depends on performing daily physical therapy.

3. 1. Muscular dystrophies are progressive degenerative disorders. The most com mon is Duchenne muscular dystrophy, which is an X-linked recessive disorder.

41. A child with spastic CP had an intrathecal dose of baclofen in the early afternoon. What is the expected result 31/2 hours post dose that suggests the child would benefit from a baclofen pump? 1. The ability to self-feed. 2. The ability to walk with little assistance. 3. If the spasticity were decreased. 4. If the spasticity were increased.

3. If baclofen were going to work for this patient, one could tell because spastic ity would be decreased.

45. The parent of an infant with CP asks the nurse if the infant will be mentally retarded. Which of the following is the nurse's best response? 1. "Children with CP have some amount of mental retardation." 2. "Approximately 20% of children with CP have normal intelligence." 3. "Many children with CP have normal intelligence." 4. "Mental retardation is expected if motor and sensory deficits are severe."

3. Many children with CP have normal intelligence.

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.

40. The parent of a young child with CP brings the child to the clinic for a checkup. Which of the parent's following statements indicates an understanding of the child's long-term needs? 1. "My child will need all my attention for the next 10 years." 2. "Once in school, my child will catch up and be like the other children." 3. "My child will grow up and need to learn to do things independently." 4. "I'm the one who knows the most about my child and can do the most for my child."

3. This statement indicates that the par ent understands the long-term needs of the child.

37. The parent of a toddler newly diagnosed with CP asks the nurse what caused it. The nurse should answer which of the following? 1. Most cases are caused by unknown prenatal factors. 2. It is commonly caused by perinatal factors. 3. The exact cause is not known. 4. The exact cause is known in every instance.

37. 1. At least 80% of cases of CP result from unknown prenatal factors.

38. Which of the following developmental milestones should the nurse be concerned about if a 10-month-old could not do it? 1. Crawl. 2. Cruise. 3. Walk. 4. Have a pincer grasp.

38. 1. Most infants are able to crawl unassisted by 8 months.

5. The Gower sign for assessing Duchenne muscular dystrophy can be elicited by having a patient do which of the following? 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 dis play the Gower sign, which is great diffi culty rising and standing from a squat ting position due to the lack of muscle strength.

39. The parent of an infant asks the nurse what to watch for to determine if the infant has CP. The nurse should reply which of the following? 1. If the infant cannot sit up without support before 8 months. 2. If the infant demonstrates tongue thrust before 4 months. 3. If the infant has poor head control after 2 months. 4. If the infant has clenched fists after 3 months.

4. Clenched fists after 3 months of age may be a sign of CP.

11. The nurse is caring for a school-aged child with Duchenne muscular dystrophy in the elementary school. Which of the following 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, pro gressive loss of muscle fibers, and loss of muscle strength.

36. The nurse knows that teaching of parents of a child newly diagnosed with CP is successful when the parents state that CP is which of the following? 1. Inability to speak and drooling. 2. Poor dentition due to poor hygiene. 3. Involuntary movements of upper extremities only. 4. An increase in muscle tone and deep tendon reflexes.

4. The primary disorder is of muscle tone, but there may be other neurological disorders such as seizures, vision disturbances, and impaired intel ligence. Spastic CP is the most com mon type and is characterized by a generalized increase in muscle tone, increased deep tendon reflexes, and rigidity of the limbs on both flexion and extension.

35. The nurse is developing a plan of care for a child recently diagnosed with CP. Which of the following should be the nurse's priority goal? 1. Ensure the ingestion of sufficient calories for growth. 2. Decrease intracranial pressure. 3. Teach appropriate parenting strategies for a special-needs child. 4. Ensure that the child reaches full potential.

4. The priority for all children is to develop to their full potential.

43. A child is admitted to the pediatric unit with spastic CP. Which of the following would the nurse expect this child to demonstrate? Select all that apply. 1. Increased deep tendon reflexes. 2. Decreased muscle tone. 3. Scoliosis. 4. Contractures. 5. Scissoring. 6. Good control of posture. 7. Good fine motor skills.

43. 1. Children with spastic CP have increased deep tendon reflexes. 3. Children with spastic CP have scoliosis. 4. Children with spastic CP have contrac tures of the Achilles tendons, knees, and adductor muscles. 5. Children with spastic CP have scissoring when walking.

57. The parent of a 6-month-old calls the clinic for advice on how to treat the infant's constipation. The best advice the nurse can offer is which of the following? 1. Offer extra water every day. 2. Add corn syrup to two bottles a day. 3. Give the infant a glycerine suppository today. 4. Let the infant go 3 days without a stool before intervening.

57. 1. Constipation means hard stools and in frequent passage. Adding extra water to the diet helps make the stool softer.

58. A 6-year-old living in a rural area sustains a puncture wound and goes to the clinic. The child is missing shots for school. The nurse should do which of the following? 1. Administer tetanus immunoglobulin. 2. Start the child on an antibiotic. 3. Cleanse the wound with hydrogen peroxide. 4. Send the child to the emergency department.

58. 1. A child not fully immunized and who has a tetanus-prone wound should re ceive tetanus immunoglobulin to pre vent tetanus. Tetanus-prone wounds include puncture wounds and those contaminated with dirt, feces, or soil.

6. A 5-year-old has been diagnosed with muscular dystrophy. Which of the following nursing interventions would be appropriate? 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.

6. 1. Muscles become weaker, including those needed for respiration, and a decision will need to be made about whether res piratory support will be provided.

7. The nurse is discussing nutrition with the parents of a child with Duchenne muscular dystrophy. The nurse tells the parents that which of the following foods would be best for their child? 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.

7. 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. 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.

8. Which of the following will help a school-aged child with muscular dystrophy stay active longer? 1. Normal activities, such as swimming. 2. Using a treadmill every day. 3. Several periods of rest every day. 4. Using a wheelchair on getting tired.

8. 1. Children who are active are usually able to postpone use of the wheelchair longer. It is important to keep using muscles for as long as possible, and aerobic activity is good for a child.

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. The mother and father. 2. The sister. 3. The brother. 4. The aunts and all female cousins. 5. The uncles and all male cousins.

9. 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. Women carry the disease, and males are affected. All female relatives should be tested. 4. Women carry the disease, and males are affected. All female relatives should be tested.