Muscular Dystrophy, CP: Pediatric Success

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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. TEST-TAKING HINT: Muscle biopsy is the definitive test for myopathies.

A 3-month-old with spina bifida is admitted to the nurse's unit. Which of the following gross motor skills should the nurse assess at this age? 1. Head control. 2. Pincer grasp. 3. Sitting alone. 4. Rolling over.

1. A 3-month-old has good head control. TEST-TAKING HINT: The test taker must know normal developmental milestones.

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.

1. A child not fully immunized and who has a tetanus-prone wound should receive tetanus immunoglobulin to prevent tetanus. Tetanus-prone wounds include puncture wounds and those contaminated with dirt, feces, or soil. TEST-TAKING HINT: The test taker must know about wound care and which wounds are considered contaminated

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.

1. At least 80% of cases of CP result from unknown prenatal factors TEST-TAKING HINT: The test taker must know the latest information to answer this question correctly.

The mother of a newborn brings her infant in for a 2-week checkup. The mother relates that this is her first child, that the baby seems to sleep very often, and that the baby does not cry much. What question would the nurse ask the mother? 1. "How many ounces of formula does your baby take at each feeding?" 2. "How many bowel movements does your baby have in a day?" 3. "How much sleep do you get every night?" 4. "How long does the baby stay awake at each feeding?"

1. Babies can lose up to 10% of birth weight but should regain it by 2 weeks of age. Knowing how much the baby eats can help the nurse determine if the infant is receiving adequate nutrition. TEST-TAKING HINT: The "red flags" in this question are that the baby sleeps a lot and does not cry much, both unusual behaviors. Follow-up questions need to be asked to determine if the infant is gaining weight as expected.

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.

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. TEST-TAKING HINT: Appropriate interventions for different kinds of chronically ill children can be similar, so think about what would be best for this child.

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

1. Children with spastic CP have increased deep tendon reflexes. 3. Children with spastic CP have scoliosis. 4. Children with spastic CP have contractures of the Achilles tendons, knees, and adductor muscles. 5. Children with spastic CP have scissoring when walking. TEST-TAKING HINT: The test taker must know the typical signs of CP.

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

1. Constipation means hard stools and infrequent passage. adding extra water to diet helps make stool softer in this age child.

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.

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 TEST-TAKING HINT: Knowing that Duchenne muscular dystrophy is inherited as a X-linked trait excludes brother, uncle, and male cousins as carriers.

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

1. Most infants are able to crawl unassisted by 8 months. TEST-TAKING HINT: The test taker must know developmental milestones

A 5-year-old has been diagnosed with pseudohypertrophic 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

1. Muscles become weaker, including those needed for respiration, and a decision will need to be made about whether respiratory support will be provided. TEST-TAKING HINT: Pseudohypertrophic muscular dystrophy is a progressive neuromuscular disease with no cure.

The parents of a preschooler diagnosed with muscular dystrophy are asking questions 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

1. Muscular dystrophies are progressive degenerative disorders. The most common is Duchenne muscular dystrophy, which is an X-linked recessive disorder. TEST-TAKING HINT: The test taker should know that muscular dystrophy is a progressive degenerative disorder

The parent of a 6-year-old with a repaired myelomeningocele is in the clinic for her child's regular examination. The child has frequent constipation and has been crying at night because of pain in the legs. After an MRI, the diagnosis of a tethered cord is made. Which of the following should the nurse tell the parent? 1. Tethered cord is a postsurgical complication. 2. Tethered cord occurs during times of slow growth. 3. Release of the tethered cord will be necessary only once. 4. Offering laxatives and acetaminophen daily will help control these problems.

1. Tethered cord is caused by scar tissue formation from the surgical repair of the myelomeningocele and may affect bowel, bladder, or lower extremity functioning TEST-TAKING HINT: Tethering is caused by scar tissue from any surgical intervention and may recur as the child grows

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 TEST-TAKING HINT: The test taker must know normal developmental milestones. Rolling occurs about 4 months, sitting alone occurs at 6 months, and pushing food out of the mouth decreases by 4 months when the tongue thrust reflex wanes.

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. TEST-TAKING HINT: The test taker must know about infant development

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 TEST-TAKING HINT: Early symptoms have to do with decreased ability to perform normal developmental tasks involving muscle strength

A 3-year-old child with CP is admitted for dehydration following an episode of diarrhea. 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. TEST-TAKING HINT: The test taker should convert the weight in kilograms to pounds

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 spasticity would be decreased. TEST-TAKING HINT: The test taker must know the purpose of baclofen

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. TEST-TAKING HINT: Children with CP have a wide range of intellectual abilities.

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. TEST-TAKING HINT: The test taker should be able to identify signs and symptoms attributable to the loss of muscle function.

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 parent understands the long-term needs of the child. TEST-TAKING HINT: The test taker must understand the goals for children with chronic illnesses or disorders. One goal is to ensure that the child be diagnosed as early as possible so that interventions can be started. Another is to help the child realize as much potential as possible.

A 15-year-old with spina bifida is seen in the clinic for a well-child checkup. The teen uses leg braces and crutches to ambulate. Which of the following nursing diagnoses takes priority? 1. Potential for infection. 2. Alteration in mobility. 3. Alteration in elimination. 4. Potential body image disturbance

4. As an adolescent on crutches and wearing braces, the teen would have the issue of body image disturbance, which must be addressed. This is a priority. TEST-TAKING HINT: The test taker must know normal development

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.

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. TEST-TAKING HINT: Knowing that nutrition is important for every child as is awareness that as the child becomes less ambulatory, weight concerns arise.

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 display the Gower sign, which is great difficulty rising and standing from a squatting position due to the lack of muscle strength. TEST-TAKING HINT: By eliminating cerebral activities, the test taker would know that the Gower sign assists in measuring leg strength

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. TEST-TAKING HINT: The test taker must know normal developmental milestones to identify those that are abnormal.

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, progressive loss of muscle fibers, and loss of muscle strength. TEST-TAKING HINT: Knowing that the child has decreased strength helps to answer the question

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 intelligence. Spastic CP is the most common 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. TEST-TAKING HINT: The test taker must know the definition of CP.

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. TEST-TAKING HINT: All of these are important goals, but determining the priority goal for a special-needs child is the key


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