Muscular Dystrophy Practice Questions

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 1. This diagnosis would relate to the family and not to the child. 2. This diagnosis would relate to the family and not to the child. 3. The child does not have pain with the muscular dystrophy process. 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 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 1. Muscles become enlarged from fatty infiltration, so they are not stronger. 2. Difficulty climbing stairs, running, and riding a bicycle are frequently the first symptoms of Duchenne muscular dystrophy. 3. High fevers and tiredness are not early signs of muscular dystrophy but could be later signs as complications become more common. 4. Respiratory infections and obesity are major complications as the disease progresses. TEST-TAKING HINT: Early symptoms have to do with decreased ability to perform normal development tasks involving muscle strength.

The nurse observes as a child with Duchenne's muscular dystrophy attempts to rise from a sitting position on the floor. After attaining a kneeling position, the child "walks" his hands up his legs to stand. The nurse documents this as which of the following? 1.Galeazzi's sign. 2.Goodell's sign. 3.Goodenough's sign. 4.Gower's sign

4 With Gower's sign, the child walks the hands up the legs in an attempt to stand, a common approach used by children with Duchenne's muscular dystrophy when rising from a sitting to a standing position. Galeazzi's sign refers to the shortening of the affected limb in congenital hip dislocation. Goodell's sign refers to the softening of the cervix, considered a sign of probable pregnancy. Goodenough's sign refers to a test of mental age.

The mother of a child with Duchenne's muscular dystrophy asks about the chance that her next child will have the disease. The nurse responds based on the understanding of which of the following? 1.Sons have a 50% chance of being affected. 2.Daughters have a 1 in 4 chance of being carriers. 3.Each child has a 1 in 4 chance of developing the disease. 4.Each child has a 50% chance of being a carrier.

1 Duchenne's muscular dystrophy is an X-linked recessive disorder. The gene is transmitted through female carriers to affected sons 50% of the time. Daughters have a 50% chance of being carriers.

The nurse teaches the mother of a young child with Duchenne's muscular dystrophy about the disease and its management. Which of the following statements by the mother indicates successful teaching? 1."My son will probably be unable to walk independently by the time he is 9 to 11 years old." 2."Muscle relaxants are effective for some children; I hope they can help my son." 3."When my son is a little older, he can have surgery to improve his ability to walk." 4."I need to help my son be as active as possible to prevent progression of the disease.

1 Muscular dystrophy is a progressive disease. Children who are affected by this disease usually are unable to walk independently by age 9 to 11 years. There is no effective treatment for childhood muscular dystrophy. Although children who remain active are able to avoid wheelchair confinement for a longer period, activity does not prevent disease progression.

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, 2, 4. 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 sister should have genetic testing to determine whether she carries the gene and identify her risks for having male offspring with the disease. 3. Because the disease is carried on the X chromosome, only females need to be genetically tested. Women carry the disease, and males are affected. All female relatives should be tested unless they are symptomatic. 4. The X chromosome carries the disease and males are affected. All female relatives should be tested. 5. The X chromosome carries the disease and males are affected. All female relatives should be tested. TEST-TAKING HINT: Knowing that Duchenne muscular dystrophy is inherited as an X-linked trait excludes fathers, brothers, uncles, and male cousins as carriers

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, 3, 4. 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. 2. Use of a treadmill is not fun for children or adults, so keeping the child using the treadmill might be an issue. 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. 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. 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 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, 3, 5. 1. Muscles become weaker, including those needed for respiration, and a decision will need to be made about whether respiratory support will be provided. 2. This is a progressive disease, which medications do not treat. 3. Physical therapy will be part of the treatment plan, but respiratory support is a priority. 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. 5. Parents should be encouraged to allow the child to go to school and participate in activities as tolerated. TEST-TAKING HINT: Pseudohypertrophic muscular dystrophy is a progressive neuromuscular disease with no cure. Children can have a good quality of life if parents are guided in how to help their child.

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, 5. 1. Muscular dystrophies are progressive degenerative disorders. The most common is Duchenne muscular dystrophy, which is an X-linked recessive disorder. 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. 5. The child may have pain due to loss of strength and muscle wasting. TEST-TAKING HINT: The test taker should know that muscular dystrophy is a progressive degenerative disorder.

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 1. The electromyelogram is part of the diagnostic workup, but muscle biopsy results classify muscle disorders. 2. Nerve conduction velocity is part of the diagnostic workup, but muscle biopsy results classify muscle disorders. 3. Muscle biopsy confirms the type of myopathy that the patient has. 4. Creatine kinase is in muscle tissue and is found in large amounts in muscular diseases. TEST-TAKING HINT: Muscle biopsy is the definitive test for myopathies

When interacting with the mother of a child who has Duchenne's muscular dystrophy, the nurse observes behavior indicating that the mother may feel guilty about her child's condition. The nurse interprets this behavior as guilt stemming from which of the following? 1.The terminal nature of the disease. 2.The dependent behavior of the child. 3.The genetic mode of transmission. 4.The sudden onset of the disease.

3 The guilt that mothers of children with muscular dystrophy commonly experience usually results from the fact that the disease is genetic and the mother transmitted the defective gene. Although many children die from the disease, the disease is considered chronic and progressive. As the disease progresses, the child becomes more dependent. However, guilt typically stems from the knowledge that the mother transmitted the disease to her son rather than the dependency of the child. The disease onset is usually gradual, not sudden.

When developing the plan of care for a child with early Duchenne's muscular dystrophy, which of the following nursing goals is the priority? 1.Encouraging early wheelchair use. 2.Fostering social interactions. 3.Maintaining function of unaffected muscles. 4.Preventing circulatory impairment.

3 The primary nursing goal is to maintain function in unaffected muscles for as long as possible. There is no effective treatment for childhood muscular dystrophy. Children who remain active are able to forestall being confined in wheelchair. Remaining active also minimizes the risk for social isolation. Preventing rather than encouraging wheelchair use by maintaining function for as long as possible is an appropriate nursing goal. Children with muscular dystrophy become socially isolated as their condition deteriorates and they can no longer keep up with friends. Maintaining function helps prevent social isolation. Circulatory impairment is not associated with muscular dystrophy

A nurse is making an initial visit to a family with a 3-year-old child with early Duchenne's muscular dystrophy. Which of the following findings is expected when assessing this child? 1.Contractures of the large joints. 2.Enlarged calf muscles. 3.Difficulty riding a tricycle. 4.Small, weak muscles.

3 Usually the first clinical manifestations of Duchenne's muscular dystrophy include difficulty with typical age-appropriate physical activities such as running, riding a bicycle, and climbing stairs. Contractures of the large joints typically occur much later in the disease process. Occasionally enlarged calves may be noted, but they are not typical findings in a child with Duchenne's muscular dystrophy. Muscular atrophy and development of small, weak muscles are later signs.

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, 5. 1. These symptoms are common with Down syndrome. 2. Duchenne muscular dystrophy does not produce these symptoms. 3. The major complications of muscular dystrophy include contractures, disuse atrophy, infections, obesity, respiratory complications, and cardiopulmonary problems. 4. These symptoms are evidence of a possible head injury. 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. TEST-TAKING HINT: The test taker should be able to identify signs and symptoms attributable to the loss of muscle function.

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, 5. 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. 5. As the child loses muscle control, the need for thickened liquids and small, well-cut-up solids becomes essential. TEST-TAKING HINT: Nutrition is important for every child; as the child becomes less ambulatory, weight concerns arise.