Pediatric Success Neuromuscular or Muscular Disorders

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. "Physical and occupational therapy will be helpful to stimulate the senses and improve cognitive skills." Children with decreased activity due to illness or trauma are helped by physical and occupational therapy. The varied activities stimulate the senses.

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. Risk of infection A normal saline dressing is placed over the sac to prevent tearing, which would allow the cerebrospinal fluid to escape and microorganisms to enter and cause an infection.

The mother of a newborn relates that this is her first child, the baby seems to sleep a lot, and does not cry much. Which 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. "How many ounces of formula does your baby take at each feeding?" 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.

Which priority item should be placed at the bedside of a newborn with myelomeningocele? 1. A bottle of normal saline. 2. A rectal thermometer. 3. Extra blankets. 4. A blood pressure cuff.

1. A bottle of normal saline. 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.

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. Children can suffer momentary severe subluxation and trauma to the spinal cord. 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.

Which developmental milestone 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. Crawl. 1. Most infants are able to crawl unassisted by 8 months. 2. Infants learn to cruise (walk around holding onto furniture) at about 9 to 10 months. 3. Walking occurs on average at about 12 months. 4. Pincer grasp (thumb and forefinger) occurs at about 9 to 10 months.

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

1. Head control. A 3-month-old has good head control.

Which symptoms will a child suffering from complete spinal cord injury experience? 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.

1. Loss of motor and sensory function below the level of the injury. 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.

The parent of a toddler newly diagnosed with cerebral palsy (CP) asks the nurse what caused it. The nurse should answer with 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. Most cases are caused by unknown prenatal factors. At least 80% of cases of CP result from unknown prenatal factors.

Which should a nurse in the ED be prepared for in a child with a possible spinal cord injury? 1. Severe pain. 2. Elevated temperature. 3. Respiratory depression. 4. Increased intracranial pressure.

3. Respiratory depression. A spinal cord injury can occur at any level. The higher the level of the injury, the more likely the child is to have respiratory insufficiency or failure. The nurse should be prepared to support the child's respiratory system.

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

After surviving a motor vehicle accident but enduring a spinal cord injury, an adolescent is unable to walk but can use his arms, has no bowel or bladder control, and has no sensation below the nipple line. Referring to the following figure, identify the vertebral/spinal cord area most likely injured. PICTURE QUESTION- COULDN'T COPY PICTURE 1. Cervical, C1-C5. 2. Cervical, C5-C7. 3. Thoracic, T1-T4. 4. Thoracic, T5-T12. 5. Lumbar, L2-L5. 6. Sacral, S1-S5.

3. Thoracic, T1-T4. 1. C1-C5 is too high, as the patient has the use of the arms and sensation to the nipple line. 2. C5-C7 is too high, as the patient still has the use of the arms. 3. Damage at T1-T4 manifests at or just below the nipple line. Every area below would be affected. 4. Sensation ends at the nipple line, so T5-T12 is too low. 5. L2-L5 is too low, as the patient has sensation only to the nipple line. 6. S1-S5 is too low.

The nurse judges teaching as successful when the parent of a child with myasthenia gravis states which of the following? 1. "My child should play on the school's basketball team." 2. "My child should meditate every day." 3. "My child should be allowed to do what other kids do." 4. "My child should be watched carefully for signs of illness."

2. "My child should meditate every day." Meditation is a good strategy to learn to decrease stress.

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. Hypothermia.

2. Abdominal distention. 3. Bladder distention. 5. Tight clothing. 2. Autonomic dysreflexia may be caused by abdominal pressure from a fecal impaction. 3. An overdistended 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.

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 nursing diagnosis 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. Alteration in nutrition: less than body requirements. 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.

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. Check for a full bladder. 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.

The parent of a child diagnosed with Werdnig-Hoffmann disease notes times of not being able to hear the child breathing. Which should the nurse do first? 1. Check pulse oximetry on the child. 2. Count the child's respirations. 3. Listen to the child's lung sounds. 4. Ask the parent if the child coughs at night.

2. Count the child's respirations. The first intervention is to check the respiratory rate of the child to see if it is abnormal, then listen to the lung sounds, and then check pulse oximetry.

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

The nurse is performing an admission assessment on a 9-year-old who has just been diagnosed with systemic lupus erythematosus. Which assessment findings should the nurse expect? 1. Headaches and nausea. 2. Fever, malaise, and weight loss. 3. A papular rash covering the trunk and face. 4. Abdominal pain and dysuria.

2. Fever, malaise, and weight loss. Fever, malaise, and weight loss are common presenting signs.

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. Vision test. 4. Romberg test.

2. Interaction with staff. 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.

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. Measure head circumference. 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.

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. Potential for infection related to the physical defect. Because this infant has not had a repair, the sac is exposed. It could rupture, allowing organisms to enter the cerebrospinal fluid, so this is the priority.

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. Bulging fontanel and downwardly rotated eyes. 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.

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. Decreased spasticity. 4. Increased spasticity.

3. Decreased spasticity. If baclofen were going to work for this child, one could tell because spasticity would be decreased.

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. Impaired skin integrity related to infectious disease process. The goal is to prevent complications related to immobility. Efforts include maintaining skin integrity, maintaining respiratory function, and preventing contractures.

The mother of an infant diagnosed with Werdnig-Hoffmann disease asks the nurse what she could have done during her pregnancy to prevent this. The nurse explains that the cause of Werdnig-Hoffmann is which of the following? 1. Unknown. 2. Restricted movement in utero. 3. Inherited as an autosomal-recessive trait. 4. Inherited as an autosomal-dominant trait.

3. Inherited as an autosomal-recessive trait. Werdnig-Hoffmann disease is inherited as an autosomal-recessive trait.

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

Which should be included in the plan of care for a newborn with a myelomeningocele who will have a surgical repair tomorrow? 1. Offer formula 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.

3. Place a wet dressing on the sac. Priority care for an infant with a myelomeningocele is to protect the sac. A wet dressing keeps it moist with less chance of tearing.

The parent of an infant asks the nurse what to watch for to determine if the infant has CP. Which is the nurse's best response? 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. "If the infant has clenched fists after 3 months." 1. Children with CP frequently have developmental delays, including not being able to sit alone by 8 months. Sitting alone usually occurs by 6 months, so 8 months would be the outer limit of normal development and cause for concern. 2. Tongue thrust is common in infants younger than 6 months, but if it goes on after 6 months it is of concern. 3. Good head control is normally attained by 3 months. 4. Clenched fists after 3 months of age may be a sign of CP.

The nurse is caring for a school-aged 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. Activity intolerance. The child would not be able to keep up with peers because of weakness, progressive loss of muscle fibers, and loss of muscle strength.

The nurse evaluates teaching of parents of a child newly diagnosed with cerebral palsy (CP) as successful when the parents state that CP is which of the following? 1. Inability to speak and uncontrolled drooling. 2. Involuntary movements of lower extremities only. 3. Involuntary movements of upper extremities only. 4. An increase in muscle tone and deep tendon reflexes.

4. An increase in muscle tone and deep tendon reflexes. 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.

Which should the nurse expect as an intervention in a child in the recovery phase of GBS? 1. Assess for respiratory compromise. 2. Assess for swallowing difficulties. 3. Evaluate neuropsychological functioning. 4. Begin an active physical therapy program.

4. Begin an active physical therapy program. Beginning active physical therapy is important for helping muscle recovery and preventing contractures.

The nurse is developing a plan of care for a child recently diagnosed with cerebral palsy (CP). Which 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. Ensure that the child reaches full potential. The priority for all children is to develop to their full potential.

Which foods would be best for a child with Duchenne muscular dystrophy? 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. Low-calorie foods to prevent weight gain. 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.

Which should the nurse prepare the parents of an infant for following surgical repair and closure of a myelomeningocele shortly after birth? The infant will: 1. Not need any long-term management and should be considered cured. 2. Not be at risk for urinary tract infections or movement problems. 3. Have continual drainage of cerebrospinal fluid, needing frequent dressing changes. 4. Need lifelong management of urinary, orthopedic, and neurological problems.

4. Need lifelong management of urinary, orthopedic, and neurological problems. Although immediate surgical repair decreases infection, morbidity, and mortality rates, these children will require lifelong management of neurological, orthopedic, and elimination problems.

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 nursing diagnosis takes priority? 1. Potential for infection. 2. Alteration in mobility. 3. Alteration in elimination. 4. Potential body image disturbance.

4. Potential body image disturbance. 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.

An adolescent presents with sudden-onset unilateral facial weakness with drooping of one side of the mouth. The teen is unable to close the eye on the affected side, but has no other symptoms and otherwise feels well. The nurse could summarize the condition by which of the following? 1. The prognosis is poor. 2. This may be a stroke. 3. It is a fifth CN palsy. 4. This is paralysis of the facial nerve.

4. This is paralysis of the facial nerve. This patient has Bell's palsy, which is an idiopathic mononeuritis of CN VII (the facial nerve) that innervates the face and muscles of expression.

Which can elicit the Gower sign? Have the patient: 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. Walk like a duck and rise from a squatting position. 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.

The parent of an infant with CP asks the nurse if the infant will be mentally retarded. Which 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." 1. Children with CP have a range of intellectual abilities, from being profoundly retarded to having a high intelligence quotient. Many have normal intelligence. If a child has severe speech problems, some may assume that the child's intelligence is severely affected when that may not be true. 2. Children with CP have a range of intellectual abilities, from being profoundly retarded to having a high intelligence quotient. Many have normal intelligence. If a child has severe speech problems, some may assume that the child's intelligence is severely affected when that may not be true. 3. Many children with CP have normal intelligence. 4. Children with CP have a range of intellectual abilities, from being profoundly retarded to having a high intelligence quotient. Many have normal intelligence. If a child has severe speech problems, some may assume that the child's intelligence is severely affected when that may not be true.

Which should the nurse expect in a 2-week-old with a brachial plexus injury? Select all that apply. 1. History of a normal vaginal delivery. 2. Small infant. 3. Absent Moro reflex on one side. 4. No sensory loss. 5. Associated clavicle fracture.

3. Absent Moro reflex on one side. 4. No sensory loss. 5. Associated clavicle fracture. 1. A brachial plexus injury in an infant (resulting from tearing or stretching of a nerve) usually occurs with large babies and breech delivery. 2. A brachial plexus injury in an infant (resulting from tearing or stretching of a nerve) usually occurs with large babies and breech delivery. 3. The infant will have an absent Moro reflex on one side and no sensory loss. 4. The infant will have an absent Moro reflex on one side and no sensory loss. 5. The injury may be associated with a fractured clavicle.

A child is admitted to the pediatric unit with spastic CP. Which would the nurse expect a child with spastic CP 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. Increased deep tendon reflexes. 3. Scoliosis. 4. Contractures. 5. Scissoring. 1. Children with spastic CP have increased deep tendon reflexes. 2. Children with spastic CP have increased muscle tone. 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. 6. Children with spastic CP have poor control of posture. 7. Children with spastic CP have poor fine motor skills.

Which 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 upon getting tired.

1. Normal activities, such as swimming. 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.

Which is the best advice to offer the parent of a 6-month-old with Werdnig-Hoffman disease on how to treat the infant's constipation? 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. Offer extra water every day. Constipation means hard stools and infrequent passage. Adding extra water to the diet helps make the stool softer in this age child.

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. "Your child needs to see the primary care provider." 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.

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 patient's bladder is not distended. 3. Administer Hyperstat to treat the blood pressure. 4. Administer atropine for bradycardia.

2. Check to be certain that the patient's bladder is not distended. Check to be certain that the bladder is not distended, which would trigger autonomic dysreflexia.

The parents of a toddler diagnosed with Werdnig-Hoffmann disease ask the nurse what they can feed their child that would be quality food. Which would be good choices for the nurse to recommend? 1. A hot dog and chips. 2. Chicken and broccoli. 3. A banana and almonds. 4. A milkshake and a hamburger.

2. Chicken and broccoli. 1. Hot dogs and chips are too high in sodium and fat. 2. Chicken is a good source of protein, and broccoli is a good choice for naturally occurring vitamins. 3. A banana is a food toddlers usually like. A child under 5 years should not eat nuts, because they are a choking hazard for a child who does not chew food well. 4. A milkshake has a high amount of fat, as does a hamburger.

Over the last week, an infant with a repaired myelomeningocele has had a highpitched cry and been irritable. Length, weight, and head circumference have been at the 50th percentile. Today length is at the 50th percentile, weight is at the 70th percentile, and head circumference is at the 90th percentile. The nurse should do which of the following? 1. Tell the parent this is normal for an infant with a repaired myelomeningocele. 2. Tell the parent this might mean the baby has increased intracranial pressure. 3. Suspect the baby's intracranial pressure is low because of a leak. 4. Refer the baby to the neurologist for follow-up care.

2. Tell the parent this might mean the baby has increased intracranial pressure. The increase in head size is one of the first signs of increased intracranial pressure; other signs include high-pitched cry and irritability.

The parent of a young child with CP brings the child to the clinic for a checkup. Which parent's statement 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. "My child will grow up and need to learn to do things independently." This statement indicates that the parent understands the long-term needs of the child.

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. Catheterize the child in and out. 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.

The nurse should tell the parents of a child with Duchenne (pseudohypertrophic) muscular dystrophy that some of the progressive complications include: 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. Contractures, obesity, and pulmonary infections. The major complications of muscular dystrophy include contractures, disuse atrophy, infections, obesity, respiratory complications, and cardiopulmonary problems.

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? 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."

1. "Muscular dystrophies usually result in progressive weakness." Muscular dystrophies are progressive degenerative disorders. The most common is Duchenne muscular dystrophy, which is an X-linked recessive disorder.

Which should the nurse include when teaching sexuality education to an adolescent with a spinal cord injury? 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."

1. "You can enjoy a healthy sex life and most likely conceive children." The reproductive system continues to function properly after a spinal cord injury. Much sexual activity and response occurs in the brain as well.

Which should the nurse do for a 6-year-old living in a rural area who is missing school shots and who has sustained a puncture wound? 1. Administer DTaP vaccine 2. Start the child on an antibiotic. 3. Clean the wound with hydrogen peroxide. 4. Send the child to the emergency department.

1. Administer DTaP vaccine A child less than 7 years of age and not fully immunized who has a tetanusprone wound should receive DTaP vaccine to prevent tetanus. Tetanusprone wounds include puncture wounds and those contaminated with dirt, feces, or soil.

A 5-year-old has been diagnosed with pseudohypertrophic muscular dystrophy. Which nursing intervention 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. Discuss with the parents the potential need for respiratory support. Muscles become weaker, including those needed for respiration, and a decision will need to be made about whether respiratory support will be provided.

A child has a provisional diagnosis of myasthenia gravis. Which should the nurse expect in this child? Select all that apply. 1. Double vision. 2. Ptosis. 3. Fatigue. 4. Ascending paralysis. 5. Sensory disturbance.

1. Double vision. 2. Ptosis. 3. Fatigue. 1. Symptoms in a child with myasthenia gravis include fatigue, double vision, ptosis, and difficulty swallowing and chewing. This is an autoimmune disease triggered by a viral or bacterial infection. Antibodies attack acetylcholine receptors and block their functioning. 2. Symptoms in a child with myasthenia gravis include fatigue, double vision, ptosis, and difficulty swallowing and chewing. 3. Symptoms in a child with myasthenia gravis include fatigue, double vision, ptosis, and difficulty swallowing and chewing.

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. Mother 2. Sister. 4. Aunts and all female 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. All female relatives should be tested. 4. Women carry the disease, and males are affected. All female relatives should be tested. All female relatives should be tested.

A school-aged child is admitted to the unit pre-operatively for bladder reconstruction. The child is latex-sensitive. Which intervention should the nurse implement? 1. Post a sign on the door and chart that the child is latex-allergic. 2. Use powder-free latex gloves when giving care. 3. Keep personal items such as stuffed animals in a plastic bag to avoid latex contamination. 4. Use a disposable plastic-covered blood pressure cuff that will stay in the child's room.

1. Post a sign on the door and chart that the child is latex-allergic. Posting a sign on the door and charting that the child has a latex allergy is important so others will be aware of the allergy.

Parents bring their 2-month-old into the clinic with concerns that the baby seems "floppy." The parents say the baby seems to be working hard to breathe, eats very slowly, and seems to fatigue quickly. The nurse assesses intercostal retractions, although the baby is otherwise in no distress. They add there was a cousin whose baby had similar symptoms. The nurse would be most concerned with what possible complications? 1. Respiratory compromise. 2. Dehydration. 3. Need for emotional support for the family. 4. Feeding intolerance.

1. Respiratory compromise. This baby may have Werdnig-Hoffman disease, which is characterized by progressive generalized muscle weakness that eventually leads to respiratory failure. Respiratory compromise is the most important complication.

The nurse is planning care for a child with a T12 spinal cord injury. Which lifelong complications 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. Flaccid paralysis.

1. Skin integrity. 2. Incontinence. 1. Spinal cord-injury patients experience many issues due to 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.

A child with a repaired myelomeningocele is in the clinic for a 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 should the nurse tell the parent? 1. Tethered cord is a post-surgical 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 a post-surgical complication. Tethered cord is caused by scar tissue formation from the surgical repair of the myelomeningocele and may affect bowel, bladder, or lower extremity functioning.

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. Distended bowel or bladder. 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.