A patient with worsening cardiomyopathy and decreased ejection fraction has been experiencing increased muscle weakness, shortness of breath, and weight loss. Which nursing intervention is the most appropriate to address his respiratory complications?

B) Advise the patient to use an incentive spirometer regularly to enhance pulmonary function.

A) Encourage the patient to perform high-intensity aerobic exercises to improve lung capacity.

B) Advise the patient to use an incentive spirometer regularly to enhance pulmonary function.

C) Restrict fluid intake to prevent further fluid overload and respiratory distress.

D) Instruct the patient to avoid lying flat to prevent orthopnea and improve breathing.

A patient with worsening cardiomyopathy and declining respiratory function presents with increased muscle weakness, fatigue, and shortness of breath. Which nursing intervention is the highest priority to address the patient's immediate health concerns?

B) Initiate oxygen therapy to address the patient's respiratory distress

A) Educate the patient on low-sodium dietary options to manage blood pressure

B) Initiate oxygen therapy to address the patient's respiratory distress

C) Schedule a follow-up appointment with a dietitian to address weight loss

D) Arrange for a physical therapy consult to improve muscle strength

A patient with muscular dystrophy and cardiac involvement is experiencing increased nighttime awakenings with gasping for air and elevated BNP levels. Which nursing intervention should be prioritized to address the patient's current symptoms?

B) Elevate the head of the bed to reduce orthopnea and improve breathing.

A) Administer supplemental oxygen to assist with respiratory function.

B) Elevate the head of the bed to reduce orthopnea and improve breathing.

C) Encourage the patient to increase fluid intake to maintain hydration.

D) Initiate a consult with a respiratory therapist for pulmonary rehabilitation.

A patient with muscular dystrophy and persistent pulmonary hypertension is experiencing mild respiratory acidosis and orthopnea. Which nursing intervention is most appropriate to address the patient's respiratory symptoms and support ventilation?

C) Arrange for non-invasive ventilation support during nighttime.

A) Increase the patient's diuretic dosage to reduce fluid overload.

B) Encourage the patient to perform incentive spirometry exercises hourly.

C) Arrange for non-invasive ventilation support during nighttime.

D) Position the patient in high Fowler's position to alleviate breathlessness.

A patient with muscular dystrophy and cardiac complications has an ejection fraction of 42% and is experiencing mild respiratory acidosis with a pH of 7.32 and pCO2 of 48 mmHg. Which nursing action should be prioritized to address the patient's respiratory status?

C) Initiate non-invasive ventilation to improve nocturnal breathing

A) Administer the prescribed low-dose furosemide to reduce fluid overload

B) Educate the patient on energy conservation techniques to manage fatigue

C) Initiate non-invasive ventilation to improve nocturnal breathing

D) Monitor dietary intake to ensure adequate nutritional support

A patient with muscular dystrophy is experiencing moderate tricuspid regurgitation and mild respiratory acidosis. The medical team initiated bilevel positive airway pressure (BiPAP) therapy at night. Considering the patient's clinical status and recent lab findings, which nursing intervention should take priority?

C) Assess the patient's renal function regularly due to the rising serum creatinine levels.

A) Monitor the patient's blood pressure closely to assess for hypotension related to diuretic therapy.

B) Educate the patient about the importance of daily weight monitoring to track fluid status.

C) Assess the patient's renal function regularly due to the rising serum creatinine levels.

D) Encourage the patient to perform incentive spirometry every hour to improve lung expansion.

A patient with muscular dystrophy has been experiencing nocturnal dyspnea and mild respiratory acidosis. The healthcare team has initiated BiPAP therapy at night. Which nursing intervention would be most appropriate to monitor the effectiveness of this treatment?

C) Monitor the patient's arterial blood gas (ABG) results to evaluate changes in pH and pCO2 levels.

A) Measure the patient's blood pressure every four hours to monitor for hypertension.

B) Check the patient's serum potassium levels daily to assess for electrolyte imbalance.

C) Monitor the patient's arterial blood gas (ABG) results to evaluate changes in pH and pCO2 levels.

D) Assess the patient's pedal pulses every shift to check for peripheral circulation issues.

A patient with a history of muscular dystrophy is exhibiting signs of hemodynamic instability, renal impairment, and anemia. Which nursing intervention is most appropriate to address the patient's current condition?

C) Monitor the patient's renal function closely and adjust medications as needed

A) Increase the patient's diuretic dosage to reduce fluid overload

B) Administer intravenous fluids to improve blood pressure and urine output

C) Monitor the patient's renal function closely and adjust medications as needed

D) Encourage the patient to reduce protein intake to decrease renal workload

A patient with muscular dystrophy presents with an increased heart rate, decreased blood pressure, lethargy, decreased urine output, and elevated serum creatinine. Which nursing intervention is most appropriate to address the potential hemodynamic instability and renal impairment?

B) Monitor the patient closely for signs of fluid overload and adjust diuretic therapy as needed.

A) Increase the patient's fluid intake to improve blood pressure and urine output.

B) Monitor the patient closely for signs of fluid overload and adjust diuretic therapy as needed.

C) Administer a blood transfusion to address the slight decline in hemoglobin levels.

D) Suggest immediate dialysis to correct renal impairment and stabilize the patient's condition.

muscular dystrophy - Nursing Case Study

Pathophysiology

• Primary mechanism: Genetic mutations in the dystrophin gene lead to the absence or dysfunction of the dystrophin protein, a crucial component of the muscle cell membrane, causing structural instability and increased susceptibility to damage during muscle contractions.

• Secondary mechanism: The repeated muscle damage triggers cycles of inflammation and repair, which are ineffective due to the lack of functional dystrophin, leading to progressive muscle fiber degeneration and replacement with fibrotic and fatty tissue.

• Key complication: This progressive muscle weakening and loss of functional muscle mass result in impaired mobility, respiratory difficulties, and in severe cases, cardiac complications, significantly impacting the patient's quality of life and longevity.

Patient Profile

Demographics:

28-year-old male, software engineer

History:

• Key past medical history: Diagnosed with Becker muscular dystrophy at age 15, history of cardiomyopathy

• Current medications: Lisinopril 10 mg daily, Albuterol inhaler as needed, Vitamin D supplements

• Allergies: Penicillin

Current Presentation:

• Chief complaint: Increasing difficulty in climbing stairs and lifting objects

• Key symptoms: Progressive muscle weakness in the legs and arms, occasional shortness of breath, mild chest pain

• Vital signs: Blood pressure 135/88 mmHg, Heart rate 102 bpm, Respiratory rate 20 breaths per minute, Temperature 98.6°F

Section 1

Change in Patient Status:

During a follow-up visit two weeks later, the patient reports worsening symptoms, specifically increased muscle weakness and fatigue that now interferes with daily activities. He mentions experiencing more frequent episodes of shortness of breath and a persistent, dull chest pain that intensifies with exertion. His vital signs reveal a blood pressure of 142/92 mmHg, a heart rate of 110 bpm, and a respiratory rate of 24 breaths per minute, indicating a potential escalation of his cardiomyopathy. In addition, he has lost 4 pounds since his last visit, which raises concerns about his nutritional status and potential muscle wasting.

The clinical team decides to perform a comprehensive assessment, including an echocardiogram, which shows a decline in ejection fraction from 45% to 38%, suggesting worsening heart failure. Pulmonary function tests reveal a decrease in forced vital capacity, indicative of compromised respiratory function. These findings underscore the need for immediate intervention to address the progression of his cardiac and respiratory complications and prevent further deterioration of his condition.

The care team discusses potential adjustments to his management plan, including modifying his medication regimen to better control his heart rate and blood pressure, possibly introducing a beta-blocker, and considering a referral to a cardiologist for further evaluation. Additionally, the team highlights the importance of respiratory therapy to improve lung function and suggests dietary consultations to address his weight loss and ensure adequate caloric intake to support muscle maintenance. These steps aim to stabilize his condition and improve his overall quality of life, setting the stage for ongoing monitoring and tailored interventions.

Section 2

The patient's response to the interventions was closely monitored over the following weeks. Initially, the introduction of a beta-blocker seemed to stabilize his heart rate, which decreased to a more manageable 88 beats per minute. However, his blood pressure remained elevated at 138/90 mmHg, warranting further adjustment of his antihypertensive regimen. Despite these changes, his symptoms of fatigue and shortness of breath persisted, albeit with slight improvement in exertional tolerance. A follow-up echocardiogram showed a slight improvement in ejection fraction to 40%, suggesting a positive response to the cardiac management plan. However, his respiratory function did not show significant improvement; repeat pulmonary function tests continued to demonstrate reduced forced vital capacity, indicating ongoing respiratory muscle weakness.

Dietary consultations revealed inadequate caloric intake, likely contributing to his weight loss and muscle wasting. A tailored nutritional plan was implemented, emphasizing high-calorie, high-protein meals to support muscle maintenance and overall energy levels. After a few weeks, the patient reported a slight increase in weight and felt marginally more energetic, though muscle weakness remained a significant concern.

Despite these efforts, new complications arose. The patient began experiencing increased frequency of nighttime awakenings with gasping for air, suggestive of orthopnea and potential fluid overload. Further laboratory tests showed elevated brain natriuretic peptide (BNP) levels, a marker of heart failure exacerbation. This development prompted the clinical team to consider more aggressive heart failure management, including the possible introduction of diuretics to manage fluid retention. The situation underscored the need for continuous, multidisciplinary monitoring and adjustment of his treatment plan, highlighting the complexity of managing muscular dystrophy with cardiac involvement.

Section 3

As the clinical team delved deeper into the patient's evolving condition, new diagnostic results provided further insights into his complex health status. A comprehensive cardiopulmonary evaluation revealed that his ejection fraction had slightly improved to 42%, but his pulmonary pressures remained elevated, suggesting persistent pulmonary hypertension. In parallel, arterial blood gas analysis indicated mild respiratory acidosis, with a pH of 7.32 and an elevated pCO2 of 48 mmHg, pointing toward hypoventilation due to respiratory muscle weakness.

Meanwhile, a repeat chest X-ray showed subtle bilateral pleural effusions and mild interstitial edema, corroborating the findings of fluid overload. These results prompted the medical team to fine-tune the patient's diuretic therapy, opting to introduce furosemide at a low dose to mitigate fluid retention while monitoring renal function closely. Concurrently, the team discussed the potential benefits of non-invasive ventilation to support nocturnal breathing and alleviate the symptoms of orthopnea.

The patient’s nutritional status also underwent reassessment, revealing a slight but notable improvement in serum albumin levels, now at 3.6 g/dL, reflecting better nutritional intake. Despite these encouraging signs, the persistence of symptoms such as fatigue and breathlessness necessitated ongoing adjustments in his comprehensive care plan. This case underscores the intricate balancing act required in managing muscular dystrophy, especially when compounded by cardiac and respiratory complications, and highlights the importance of a dynamic, multidisciplinary approach to optimize patient outcomes.

Section 4

As the clinical team continued to monitor the patient’s progress, a new set of diagnostic results emerged, providing a deeper understanding of his current health challenges. The echocardiogram, while confirming the slight improvement in ejection fraction to 42%, revealed a new complication: moderate tricuspid regurgitation. This finding suggested a potential exacerbation of his pulmonary hypertension, further complicating his cardiovascular status. Additionally, a follow-up arterial blood gas analysis showed persistent mild respiratory acidosis with a pH of 7.34 and pCO2 remaining elevated at 46 mmHg, reinforcing the need for enhanced respiratory support due to ongoing hypoventilation.

In light of these developments, the medical team decided to escalate the patient's respiratory support. They implemented a trial of bilevel positive airway pressure (BiPAP) during nighttime to assist with his breathing, aiming to reduce the burden on his weakened respiratory muscles and improve gas exchange. Clinically, the patient reported a slight reduction in nocturnal dyspnea and an improvement in morning fatigue, suggesting a positive initial response to the intervention.

However, new laboratory results indicated a rising serum creatinine level, now at 1.6 mg/dL, raising concerns about potential renal impairment secondary to the adjusted diuretic therapy. This prompted a careful reassessment of his fluid management strategy, balancing the need to address fluid overload without compromising renal function. The team initiated a multidisciplinary conference to explore alternative therapeutic strategies, ensuring a holistic approach to the patient's complex and evolving condition. This careful navigation of the patient's care plan highlighted the importance of continuous reassessment and adaptation in managing the multifaceted complications of muscular dystrophy.

Section 5

In the subsequent days, the patient's condition revealed a change in status that required immediate clinical attention. The nursing team, during routine vital sign checks, noted a mild but significant increase in heart rate to 102 beats per minute and a subtle decrease in blood pressure, now at 100/60 mmHg. The patient also appeared more lethargic, with decreased urine output compared to previous days. These changes indicated a potential shift towards hemodynamic instability, necessitating further examination and diagnostic testing to explore the underlying causes.

The medical team promptly ordered additional laboratory tests, which revealed a further increase in serum creatinine to 1.8 mg/dL, underscoring the progression of renal impairment. Concurrently, a complete blood count showed a slight decline in hemoglobin levels to 11 g/dL, raising concerns about potential anemia, which could exacerbate the patient's overall fatigue and contribute to the observed lethargy. The team hypothesized that the patient's fluid status and renal function might be influencing his cardiovascular and hematologic parameters, prompting a focused reassessment of his diuretic and fluid management plan.

In response to these findings, the multidisciplinary team decided to adjust the patient's diuretic regimen, opting for a more renal-friendly approach to mitigate further renal compromise while still addressing fluid overload. The patient was also scheduled for a comprehensive renal ultrasound to evaluate structural changes and rule out obstructive causes. Additionally, dietary modifications were discussed, emphasizing adequate protein intake to support hematologic health and considering the introduction of erythropoiesis-stimulating agents if anemia persisted. This strategic pivot in the patient's care plan highlighted the necessity of vigilance and adaptability in the face of emerging complications, ensuring a personalized approach to managing the complexities of muscular dystrophy.