Final exam material Flashcards
Exam 4
MUSCULAR DYSTROPHIES
Epidemiology: What are primary symptoms?
Primary symptoms are progressive muscle weakening and wasting of skeletal or voluntary muscle groups
MUSCULAR DYSTROPHIES
Pathophysiology: What are the two types?
Duchenne and Becker muscular dystrophies (MDs)
MUSCULAR DYSTROPHIES
Pathophysiology: Duchenne and Becker muscular dystrophies (MDs) are caused by what?
Duchenne and Becker muscular dystrophies (MDs) are caused by a mutation to the dystrophin gene
Muscular Dystrophy:
What is Dystrophin?
Dystrophin is a protein responsible for muscle repair.
What does Muscular dystrophy (MD) have? What is it caused by?
Muscular dystrophy (MD) is a disease with many different subtypes that affect certain muscle groups, are age specific in relation to presenting symptomatology, have variations in severity, and are caused by a defect in different genes.
Muscular dystrophy (MD)
What age does Muscular dystrophy occur?
Muscular dystrophy can appear at any age from infancy until middle age and beyond.
Muscular Dystrophy
What is the most well known for of muscular dystrophy and is most common in children? When is age of onset?
Duchenne muscular dystrophy (DMD) is the most well-known form and is the most common in children. Onset is 2–4 years of age
Becker muscular dystrophy (BMD): Who is it primarily present in? When does it typically occur?
Becker muscular dystrophy (BMD) is also primarily prevalent in males and typically occurs in childhood. Delayed onset, slower progression affects 5–25 years of age
Muscular Dystrophy;
What are clinical manifestations?
Progressive muscle weakness
Chronic pain, most notably in lower back and legs
Muscular Dystrophy;
How is diagnosis usually begin?
Diagnosis of MD usually begins with a preliminary review of serum muscle enzymes such as Creatine Kinase (CK), which is usually an early sign of the disease and requires frequent and close monitoring.
Muscular Dystrophy;
What is an early sign of the disease?
Creatine Kinase (CK), which is usually an early sign of the disease and requires frequent and close monitoring.
Muscular Dystrophy;
What are other diagnostic measures? What are they for exactly?
Other diagnostic studies such as:
chest x-ray,
electrocardiogram,
computed tomography (CT), and electromyography
are frequently warranted to determine the extent of muscle wasting or the presence of cardiopulmonary compromise associated with the disease.
Muscular Dystrophy;
What does medical management of MD focus on?
The medical management of MD focuses on the prevention of progressive deterioration of the disease, supportive measures to assist maximization of functional capacity, and pain management.
Muscular Dystrophy;
What is the cure or pharmacological intervention?
There is no specific cure or pharmacological intervention.
Muscular Dystrophy;
What medication can help? What do they do?
glucocorticoids such as prednisone have been shown to decrease the rate of muscle deterioration and increase restorative functional abilities.
Muscular Dystrophy;
What are severe complications of this disease?
Spinal deformity and neuromuscular deterioration causing weakness of the trunk and lower extremities are severe complications of MD.
Muscular Dystrophy;
Severe complications: What can develop with scoliosis and overall thoracic-lumbar changes?
With the development of scoliosis (curvature of the spine) and overall thoracic-lumbar changes, respiratory and cardiac compromise can develop.
Muscular Dystrophy;
Nursing interventions: What must be assessed?
Assess vital signs.
Muscular Dystrophy;
Nursing interventions: When assessing vs, what may indicate deterioration of cardiovascular and respiratory status?
Increased heart rate,
decreased blood pressure, and
decreased SpO2 may indicate deterioration of cardiovascular and respiratory status.
Muscular Dystrophy;
Nursing interventions: What must be administered?
Administer corticosteroid therapy as ordered
Muscular Dystrophy;
Nursing interventions: What must be managed? How?
Pain management—administer aspirin, ibuprofen, Tylenol as ordered for pain control.
Muscular Dystrophy;
Nursing interventions: What is the importance of managing pain?
Managing pain is essential to maintaining mobility as much as possible
Muscular Dystrophy;
Nursing interventions: What must the nurse encourage? What kind of precautions should they have?
Range-of-motion exercises.
Maximize function and decrease potential contractures
Put patient on fall precautions
What is the most common bone disease in humans and creates a major public health concern for the future?
Osteoporosis