Pediatric Neuromuscular/Muscular Dystrophy NCLEX

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.

Traumatic Fracture

Bone was broken from activity, force, trauma, etc

Fracture Complications from FES

1. Osteomyelitis (Prevent it - Vanc) 2. Compartment Syndrome (Prevent it-fasciotomy) 3. Fat embolus (nothing to prevent it except immobilize the area & keep pt calm so they don't move) NO ANTICOAGs

Acute Compartment Syndrome

A serious condition in which increased pressure within one or more compartments reduces circulation to the area.

Fascia

An inelastic tissue that surrounds a group of muscles, blood vessels, & nerves in the body

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 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

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 are common presenting signs. TEST-TAKING HINT: The test taker must know the presenting signs and symptoms of systemic lupus erythematosus.

Which of the following 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. TEST-TAKING HINT: The dynamics of the cerebrospinal fluid change after closure of the sac.

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. Meditation is a good strategy to learn to decrease stress TEST-TAKING HINT: The test taker must know the physiology of the illness

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.

The nurse is caring for a newborn with a myelomeningocele who will have a surgical repair tomorrow. The nurse should do which of the following? 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. Priority care for an infant with a myelomeningocele is to protect the sac. A wet dressing keeps it moist with less chance of tearing. TEST-TAKING HINT: Realizing the defect is on the back eliminates answer 2. Knowing newborns are sleepy and do not eat on a schedule eliminates answer 1.

Non-traumatic

bone broke on its own because it was diseased (osteoporosis, osteomyelitis, diabetes, etc)

First s/s of ACS

numbness, tingling, paresthesia The extremity then becomes pale and cool as a result of decreased arterial perfusion

Purpose of Skeletal Traction

Aids in bone realignment. These allow the use of longer traction time & heavier weights (15-30lbs)

For patients with an external fixtator, in the first 48-72 hours what is an expected finding?

CLEAR fluid draining or weeping. Pay particular attention to pin sites for s/s of infection/inflammation

Purpose of Skin Traction

Decrease painful muscle spasms that accompany hip fractures. The weight is a pulling force & is limited to 5-10lbs to prevent injury to skin

When you have a fracture, what is the first things that should be done and why?

IMMOBILIZE the area to have less pain, less inflammation, less chance of lacerations of nerves, arteries, & vessels -Promote comfort, analgesia -RICE -Basic immobilization: casts,, splints, paster of paris, fiberglass casts, traction

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. 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.

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.

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 nurse is admitting a child with a spinal cord injury. A plan of care should be based on the fact that a patient suffering from complete spinal cord injury will experience which of the following symptoms? 1. Loss of motor and sensory function below the level of the injury. 2. Loss of interest in normal activities. 3. Have extreme pain below the level of the injury. 4. Loss of some function, with sparing of function below the level of the injury.

1. Patients with complete spinal cord injury lose motor and sensory function below the level of the injury as a result of interruption of nerve pathways TEST-TAKING HINT: A spinal cord injury causes loss of motor and sensory function below the level of the injury.

A school-aged child is admitted to the unit preoperatively for bladder reconstruction. The child is latex-sensitive. Which of the following interventions 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. 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. TEST-TAKING HINT: The test taker must know which supplies have latex and about contact allergies.

Which of the following 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. "Young men stop producing testosterone and sperm after their injury." 4. "Young women lack estrogen and no longer ovulate after their injury.

1. The reproductive system continues to function properly after a spinal cord injury. Much sexual activity and response occurs in the brain as well TEST-TAKING HINT: Spinal cord injuries have little effect on reproduction

Children with high-level spinal cord injuries may be afflicted with many complications, a serious one being autonomic dysreflexia due to unregulated sympathetic hyperactivity. Some of the causes of autonomic dysreflexia that the nurse should be aware of 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. 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 TEST-TAKING HINT: Autonomic dysreflexia most often occurs due to an irritating stimulus within the body below the level of spinal cord injury

A nurse working in the neuro-intensive care unit has a patient with a spinal cord injury at T4. The patient suddenly becomes dangerously hypertensive and bradycardic. Which of the following interventions is appropriate in this situation? 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 bladder is not distended, which would trigger autonomic dysreflexia. TEST-TAKING HINT: The test taker must know which symptoms are suggestive of 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 of the following 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 is a good source of protein, and broccoli is a good choice for naturally occurring vitamins. TEST-TAKING HINT: The test taker must know good-quality foods that should be offered to children.

The parent of a child diagnosed with Werdnig-Hoffmann disease notes times of not being able to hear the child breathing. The nurse should first do which of the following? 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. 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. TEST-TAKING HINT: The test taker would first count respirations to determine if the rate is normal for a child that age. Auscultation comes next, then pulse oximetry if needed.

A 2-month-old has had a myelomeningocele repair and has been brought in by a parent for the well-child checkup and shots. Over the last week, the baby has had a high-pitched cry and has been irritable. Height, weight, and head circumference have been at the 50th percentile. Today height 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. The increase in head size is one of the first signs of increased intracranial pressure; other signs include highpitched cry and irritability TEST-TAKING HINT: The test taker should know how fast an infant's head size changes

After spinal cord surgery, a patient suddenly complains of a severe headache. What 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. TEST-TAKING HINT: Autonomic dysreflexia is usually caused by a full bladder or bowel

The nurse is caring for an infant with myelomeningocele who is going to surgery later today for closure of the sac. Which of the following 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. 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. TEST-TAKING HINT: The preoperative priority is risk of infection, especially when effort is necessary to keep a sterile saline dressing on the sac.

A parent brings the 2-week-old to the clinic for a checkup. The infant has a brachial plexus injury. Which of the following should the nurse expect? Select all that apply. 1. A history of a normal vaginal delivery. 2. A small infant. 3. An absent Moro reflex on one side. 4. No sensory loss. 5. An associated clavicle fracture

3. Damage at T1-T4 manifests at or just below the nipple line. Every area below would be affected. TEST-TAKING HINT: Deficits occur at and below the level of injury

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.

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.

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. TEST-TAKING HINT: The test taker must know what a brachial

Following surgical repair and closure of a myelomeningocele shortly after birth, which of the following is true of an infant? 1. The infant will not need any long-term management and should be considered cured. 2. The infant will no longer be at risk of urinary tract infections or movement problems. 3. The infant will have continual drainage of cerebrospinal fluid, needing frequent dressing changes. 4. The infant will need lifelong management of urinary, orthopedic, and neurological problems

4. Although immediate surgical repair decreases infection, morbidity, and mortality rates, these children will require lifelong management of neurological, orthopedic, and elimination problems. TEST-TAKING HINT: The test taker can eliminate answer 1 due to the complexity of myelomeningocele.

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 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

Complete Fracture

The break is across the entire width of the bone that it is divided into 2 bones

Genetic Considerations of DMD & BMD

-Both are liked recessive disorders -Women are CARRIERS (able to pass on the gene without having the disorder) -Have a 50% change of passing on the MD gene to their daughters, who are then carriers, and to their sons who will have the disease. -These MD only affect males -DMD patients die young and do not have children -BMD live longer & may have children

Mechanical Traction can be either:

-Continuous, as in fracture treatment -Intermittent, for relief of muscle spasms or other MM/Neuro trauma

Potential complications r/t severe impairment in mobility with Spica Cast

-Skin breakdown -Respiratory dysfunction (pneumonia, atelectasis) -Constipation -Joint Contractures

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.

A nurse is receiving an infant with myelomeningocele from an outside hospital. Which of the following priority items should be placed at the newborn's bedside? 1. A bottle of normal saline. 2. A rectal thermometer. 3. Extra blankets. 4. A blood pressure cuff.

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. TEST-TAKING HINT: Focus on the care and potential complications of an infant with spina bifida to answer the question correctly

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 nurse is planning care for a patient with a T12 spinal cord injury. Which lifelong complications should the patient 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. 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. TEST-TAKING HINT: The test taker must know the long-term effects of spinal cord injury

Spinal cord injuries are frequently misdiagnosed in children because of a phenomenon called spinal cord injury without radiographic abnormality. This occurs because of which of the following? 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. Children are more prone to spinal cord injuries because of their size. 4. Children are unable to quantify pain and do not report symptoms appropriately.

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 immature spines that allow for reduction after momentary subluxation. TEST-TAKING HINT: The test taker must understand the physiology of spinal cord injuries in children

A child has a provisional diagnosis of myasthenia gravis. Which of the following 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. 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. TEST-TAKING HINT: The test taker must know the correct symptoms of myasthenia gravis

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

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. The nurse can see some intercostal retractions, although the baby is otherwise in no distress. The parents say the baby eats very slowly and seems to fatigue rapidly. 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. 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 TEST-TAKING HINT: Consider the ABCs in this situation: airway, breathing, and cardiac status. These are priorities when caring for clients.

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

Concerning a child with post-traumatic spinal cord injury, the nurse knows teaching has been successful when the 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. TEST-TAKING HINT: The test taker must know what triggers autonomic dysreflexia and what the symptoms are

A newborn is diagnosed with a myelomeningocele at L2. Which of the following should be the priority nursing diagnosis for this infant at 12 hours of age? 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 cerebrospinal fluid, so this is the priority. TEST-TAKING HINT: Before surgery, the myelomeningocele is exposed, so risk of infection is much higher.

Which of the following 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. TEST-TAKING HINT: The test taker should know normal growth patterns

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

ORIF

Open Reduction Internal Fixation - Common method of reducing & immobilizing a fracture. Permits early mobilization.

6 P's of Acute Compartment Syndrome (ACS)

Pain, Pressure, Paralysis, Paresthesia, Pallor, Pulselessness (rare)

Skeletal Traction

Pins, wires, tongs (Crutchfield), or screws surgically inserted directly into the bone.

The nurse is preparing to receive a child in the emergency room. Radio report given by Emergency Medical Service indicates a possible spinal cord injury. The nurse should be prepared for a child with which of the following? 1. Severe pain. 2. Elevated temperature. 3. Respiratory depression. 4. Increased intracranial pressure.

3. A spinal cord injury can occur at any level. The higher the level of the injury, the more likely the patient is to have respiratory insufficiency or failure. The nurse should be prepared to support the child's respiratory system. TEST-TAKING HINT: The test taker must know the signs of a spinal cord injury.

A newborn with a repaired myelomeningocele is assessed for hydrocephalus. What would the nurse expect if the infant has 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. TEST-TAKING HINT: The test taker must know the difference in clinical signs of hydrocephalus in infants and older children. Infants' heads expand, whereas older children's skulls are fixed. The anterior fontanel closes between 12 and 18 months

A child with GBS is admitted to the pediatric unit. The child has had lots of oral fluids but has not urinated for 8 hours. The nurse's first action would be to do which of the following? 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 catheterized in and out to avoid the possibility of developing a urinary tract infection from urine in the bladder for too long. TEST-TAKING HINT: Urinary retention occurs with GBS and catheterization is necessary in a child who has had lots of fluids but not voided in 8 hours

Types of casts

Plaster of Paris (no longer commonly used b/c stockinette can be too tight & impair circulation or too loose & cause pressure ulcers) Fiberglass Casts-dry in 10-15 min and can bear weight 30 min after application Polyster-cotton knit casts-take 7 min to dry & can bear weight after 20min

The nurse is planning care for a child who was recently admitted with GBS. Which of the following 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, maintain respiratory function, and preventing contractures. TEST-TAKING HINT: The test taker must have a basic understanding of GBS and know that it affects the peripheral nervous system.

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.

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, and muscle tenderness; and has absent deep tendon reflexes in the lower extremities. Which of the following is true 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. TEST-TAKING HINT: Having a prior upper respiratory infection usually means this condition is not caused by bacteria, which eliminates answers 1 and 2. That leaves the choice between answers 3 and 4.

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.

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. Werdnig-Hoffmann disease is inherited as an autosomal-recessive trait TEST-TAKING HINT: The test taker needs to know how infants get this progressive disease.

Reduction

Reallignment of the bones ends for proper healing, is accomplished by a closed method or an open (surgical) procedure. (ex: a broken bone put back into place) Two types: Open & Closed

Which of the following 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. Beginning active physical therapy is important for helping muscle recovery and preventing contractures. TEST-TAKING HINT: The test taker must know the normal progress of the disease. A hint is provided by the word "recovery" in 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.

An adolescent presents with sudden-onset unilateral facial weakness. The teen has drooping of one side of the mouth, is unable to close the eye on the affected side, 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 patient has Bell palsy, which is an idiopathic mononeuritis of CN VII (the facial nerve) that innervates the face and muscles of expression. TEST-TAKING HINT: The test taker must know CNs and their actions.

Cast Syndrome

AKA Superior Mesenteric Artery Syndrome -Partial of complete upper intestinal obstruction results in classic symptoms: abd distention, epigastric pain, n/v, normal bowel sounds

Traction

Application of pulling force to part of the body to provide reduction, alignment, & rest. Also used as a last resort to decrease muscle spasm or prevent or correct deformity & tissue damage

The most common forms of MD

Duchenne MD (DMD) Becker MD (BMD)

If the patient reports discomfort that is unrelieved by analgesics if the banged, spring, or cast is too tight...

Elevate the fractured extremity higher than the heart & apply ice for first 24-48h as needed to reduce edema

Spica Cast

Encases a portion of the trunk & one or two extremities.

Skin traction

Involved use of Velcro boot (Buck's Traction), belt, or halter, which is usually secured around the affected leg

Advantages of External fixation

Minimal blood loss compared with internal fixation The device allows early ambulation & exercise of affected body part while relieving pain The device maintains alignment in closed fractures that will not maintain position in a cast & stabilizes comminuted fractures that require bone grafting

Crush Syndrome

Occurs from an external crush injury that compresses one or more compartments in the leg, arm, pelvis. It's a potentially life-threatening, systemic complication that results from hemorrhage & edema after a severe fracture injury

Cast

Rigid device that immobilizes the affected body part while allowing other body parts to move. Allows early mobility & reduced pain

Fat Embolism Syndrome (FES)

Serious complication in which fat globules are released from yellow bone marrow into the bloodstream within 14-48 hours after an injury or other illness. (Same concept as warming up leftover gravy. All the fat sits at the top)

Nursing care for MD

This is a genetic disease. There is no cure, no surgery, no medications to cure it.The patient will be at risk for IMPAIRED PHYSICAL MOBILITY. The nursing care will be comfort & palliative

Fixation

Two Types: Internal & external Can be external-pins, rods, etc on the outside of the body. Can also be casts, slings, etc Internal-putting pins, rods, on the inside of the body

Primary nursing concern for closed reduction

assessment & prevention of neuromuscular dysfunction or compromise. Assess neuromuscular status every hour for first 24h, then even 1-4h after depending on injury

Disadvantages of external fixation

increased risk of pin site infection which can lead to osteomyelitis

Muscular Dystrophy

slowly progressive or rapidly progressive disease that causes progressive muscle weakness. The major cause of death is respiratory failure. Cardiac failure is 2/2 cause

Incomplete Fracture

the fracture does not divide. The break is only through part of the bone

After a fasciotomy...

the open wound is packed & dressed daily or more until secondary closure occurs (4-5 days) depending on the patients healing ability. Debridement of the wound by the surgeon & may apply grafts to promote healing