Pediatric Neuromuscular/Muscular Dystrophy NCLEX

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.

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

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)

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.

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

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

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

3. The major complications of muscular dystrophy include contractures, disuse atrophy, infections, obesity, respiratory complications, and cardiopulmonary problems. TEST-TAKING HINT: The test taker should be able to identify signs and symptoms attributable to the loss of muscle function.

The nurse is discussing nutrition with the parents of a child with Duchenne muscular dystrophy. The nurse tells the parents that which of the following foods would be best for their child? 1. High-carbohydrate, high-protein foods. 2. No special food combinations. 3. Extra protein to help strengthen muscles. 4. Low-calorie foods to prevent weight gain.

4. As the child becomes less ambulatory, moving the child will become more of a problem. It is not good for the child to become overweight for several health reasons in addition to decreased ambulation. TEST-TAKING HINT: Knowing that nutrition is important for every child as is awareness that as the child becomes less ambulatory, weight concerns arise.

The Gower sign for assessing Duchenne muscular dystrophy can be elicited by having a patient do which of the following? 1. Close the eyes and touch the nose with alternating index fingers. 2. Hop on one foot and then the other. 3. Bend from the waist to touch the toes. 4. Walk like a duck and rise from a squatting position.

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

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

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

Acute Compartment Syndrome

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

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

Purpose of Skeletal Traction

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

Fascia

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

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

Traumatic Fracture

Bone was broken from activity, force, trauma, etc

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

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.

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

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

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.

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

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

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

Non-traumatic

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

Disadvantages of external fixation

increased risk of pin site infection which can lead to osteomyelitis

First s/s of ACS

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

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