A patient with chronic hemolytic anemia post-splenectomy is experiencing notable dyspnea, fatigue, and elevated bilirubin and ferritin levels. The nursing team is adjusting her chelation therapy regimen. Which nursing intervention is most critical to prioritize to prevent further complications related to iron overload?

B) Educate the patient on the importance of adhering to the increased dose of Deferasirox.

A) Monitor the patient's hemoglobin levels weekly to assess anemia severity.

B) Educate the patient on the importance of adhering to the increased dose of Deferasirox.

C) Schedule regular echocardiograms to detect early signs of iron-induced cardiomyopathy.

D) Perform daily abdominal assessments to monitor for signs of hepatomegaly.

A patient with chronic hemolytic anemia and recent laboratory findings of high ferritin levels despite ongoing chelation therapy is being evaluated. Which nursing intervention is most appropriate to prevent complications associated with iron overload?

B) Educate the patient about the importance of adhering to the adjusted chelation therapy regimen.

A) Encourage increased dietary iron intake to compensate for anemia.

B) Educate the patient about the importance of adhering to the adjusted chelation therapy regimen.

C) Recommend the patient reduce physical activity to minimize fatigue.

D) Advise the patient to take vitamin C supplements to enhance iron absorption.

A patient with Beta thalassemia major is undergoing chelation therapy and presents with intermittent dizziness, fatigue, and mild hypotension. Her laboratory results show elevated ferritin levels, decreased hemoglobin, and elevated liver enzymes. What is the priority nursing intervention to address her symptoms and lab findings?

A) Encourage the patient to increase fluid intake and monitor her blood pressure regularly.

B) Advise the patient to increase dietary iron intake to compensate for anemia.

C) Suggest the patient rest more frequently to manage fatigue symptoms.

D) Educate the patient about reducing physical activity to prevent dizziness.

A patient with Beta thalassemia major is on an adjusted regimen of Deferasirox for iron overload. Following the adjustment, she reports dizziness and increased fatigue, and her lab results show mild hypotension, tachycardia, and elevated bilirubin levels. What nursing intervention should the nurse prioritize to address the patient's current symptoms and lab findings?

A) Increase the patient's oral fluid intake to manage the mild hypotension and support renal function.

B) Administer a transfusion of packed red blood cells to address the decreased hemoglobin level.

C) Educate the patient on dietary modifications to reduce iron absorption and support liver function.

D) Recommend rest periods throughout the day to manage fatigue and prevent hypotension episodes.

A patient with Beta thalassemia major is experiencing symptoms of orthostatic hypotension and has a hemoglobin level of 7.0 g/dL. Which nursing intervention is most appropriate to address these concerns while considering her current treatment plan?

A) Encourage the patient to increase fluid intake and rise slowly from a sitting or lying position.

B) Administer an additional dose of chelation therapy to prevent further iron overload.

C) Suggest the patient take over-the-counter iron supplements to improve hemoglobin levels.

D) Instruct the patient to avoid any physical activity to prevent dizziness and fatigue.

A patient with Beta thalassemia major presents with dizziness upon standing, low hemoglobin levels, and elevated liver enzymes. Which of the following nursing interventions is most appropriate to address the symptoms of orthostatic hypotension?

A) Encourage the patient to increase fluid intake and perform slow position changes.

B) Advise the patient to avoid all physical activities to prevent falls.

C) Instruct the patient to consume a high-sodium diet to increase blood pressure.

D) Recommend the patient to lie flat for at least 8 hours daily.

A patient with Beta thalassemia major is being managed for orthostatic hypotension and iron overload. The care team switches the chelation therapy due to concerns about renal function. Which nursing intervention is most important in monitoring the patient's response to this change in therapy?

C) Assess the patient's renal function through creatinine and urine output measurements.

A) Encourage the patient to increase fluid intake to prevent dehydration.

B) Monitor the patient’s blood pressure and heart rate regularly for signs of orthostatic changes.

C) Assess the patient's renal function through creatinine and urine output measurements.

D) Educate the patient about signs of anemia and encourage rest during periods of fatigue.

A patient with Beta thalassemia major is experiencing orthostatic hypotension and has recently started a different chelation therapy due to increased creatinine levels. Which intervention should the nurse prioritize to ensure patient safety considering the current clinical situation?

B) Advise the patient to rise slowly from seated to standing positions to prevent dizziness.

A) Encourage the patient to consume a high-protein diet to support erythropoiesis.

B) Advise the patient to rise slowly from seated to standing positions to prevent dizziness.

C) Increase the patient's fluid intake to 4 liters per day to manage hypotension.

D) Schedule frequent blood transfusions to manage low hemoglobin levels.

A patient with Beta thalassemia major is on chelation therapy and erythropoiesis-stimulating agents. During follow-up, the patient reports improvement in dizziness and has stable liver function tests, but a slight increase in creatinine levels is observed. Which nursing intervention is most appropriate to prioritize at this time?

B) Educate the patient about the signs and symptoms of worsening renal function.

A) Encourage the patient to continue hydration and slow positional changes.

B) Educate the patient about the signs and symptoms of worsening renal function.

C) Adjust the erythropoiesis-stimulating agent dosage to further improve hemoglobin levels.

D) Schedule an additional liver function test to monitor for potential hepatotoxicity.

A patient with Beta thalassemia major has shown improvement in hemoglobin levels with the use of erythropoiesis-stimulating agents but presents with an increased creatinine level. Which nursing intervention should be prioritized to address this new development?

D) Schedule a follow-up appointment with a nephrologist

A) Encourage increased fluid intake to support renal function

B) Educate the patient on signs of worsening renal function

C) Monitor and document blood pressure and heart rate regularly

D) Schedule a follow-up appointment with a nephrologist

Beta thalassemia major - Nursing Case Study

Pathophysiology

• Primary mechanism: Beta thalassemia major is caused by mutations in the HBB gene leading to reduced or absent production of beta-globin chains. This imbalance results in excess alpha-globin chains, which precipitate in erythroid precursors causing ineffective erythropoiesis and anemia.

• Secondary mechanism: The accumulation of unpaired alpha-globin chains leads to oxidative damage and apoptosis of red blood cell precursors. This further exacerbates anemia and triggers compensatory extramedullary hematopoiesis, often leading to bone deformities and splenomegaly.

• Key complication: Chronic anemia and ineffective erythropoiesis result in increased intestinal iron absorption, leading to iron overload. This excess iron deposits in organs such as the liver, heart, and endocrine glands, potentially causing organ dysfunction and increasing morbidity.

Patient Profile

Demographics:

16-year-old female, high school student

History:

• Key past medical history: Diagnosed with Beta thalassemia major at age 2, regular blood transfusions since diagnosis, splenectomy at age 10

• Current medications: Deferasirox, folic acid supplements

• Allergies: Penicillin

Current Presentation:

• Chief complaint: Fatigue and shortness of breath

• Key symptoms: Pallor, jaundice, mild abdominal pain, and joint pain

• Vital signs: Blood pressure 105/65 mmHg, heart rate 110 bpm, respiratory rate 22 breaths per minute, temperature 37.4°C (99.3°F)

Section 1

During the initial assessment, the nursing team noted that the patient appeared visibly fatigued and was experiencing notable dyspnea with mild exertion, such as walking short distances. Her skin and sclera exhibited signs of jaundice, indicative of hyperbilirubinemia, a common symptom in patients with chronic hemolytic anemia. Her pallor was significant, suggesting a low hemoglobin level. The patient's abdominal examination revealed mild tenderness in the right upper quadrant, raising concerns about hepatomegaly, a potential consequence of chronic extramedullary hematopoiesis and iron overload, especially post-splenectomy.

Laboratory results confirmed the expected anemia with a hemoglobin level of 7.5 g/dL, considerably below the normal range, correlating with her symptoms of fatigue and pallor. Liver function tests revealed elevated bilirubin levels, and ferritin levels were notably high at 2,500 ng/mL, indicating iron overload, despite ongoing chelation therapy with Deferasirox. This suggests that the current chelation regimen may need adjustment to prevent further complications related to iron deposition in vital organs. The echocardiogram was performed to assess cardiac function, given her tachycardia and the risk of iron-induced cardiomyopathy. The results showed mild left ventricular hypertrophy, but no significant cardiac dysfunction at this stage.

The multidisciplinary team, including hematology and cardiology, convened to discuss the findings and plan for the patient's ongoing care. It was decided to adjust her chelation therapy regimen, increasing the dose of Deferasirox while closely monitoring her renal and hepatic function. Additionally, the team emphasized the importance of regular cardiac evaluations to detect early signs of iron-induced cardiomyopathy. The patient and her family were educated about the importance of adherence to the new treatment plan and the need for consistent follow-up appointments to monitor her condition and prevent further complications.

Section 2

Two weeks after the adjustment of the chelation therapy regimen, the patient returned for a follow-up appointment, during which she reported experiencing intermittent episodes of dizziness and increased fatigue. On examination, her vital signs revealed mild hypotension with a blood pressure of 95/60 mmHg and a heart rate of 105 beats per minute. Despite the increase in Deferasirox dosage, her laboratory results showed a slight reduction in ferritin levels to 2,300 ng/mL, which, while an improvement, remained significantly elevated. Her hemoglobin level had also decreased slightly to 7.2 g/dL, raising concerns about the adequacy of her red blood cell production or possible increased hemolysis.

Further diagnostic workup included a repeat liver function test, which showed persistently elevated bilirubin levels and a mild increase in liver enzymes, suggesting potential hepatic stress or injury. A renal panel indicated stable kidney function, but with a creatinine level at the higher end of normal, highlighting the need for continued vigilance given her intensified chelation therapy. An abdominal ultrasound was performed to assess for hepatomegaly and any potential liver complications. The imaging confirmed mild hepatomegaly without evidence of cirrhosis or significant liver damage, but did reveal increased echogenicity, consistent with fatty infiltration, likely secondary to chronic iron overload.

The multidisciplinary team reviewed these new findings and determined that while the patient was responding to the increased chelation therapy, the response was not as robust as expected, indicating the need for further optimization. The team decided to introduce adjunctive measures, including dietary adjustments and consideration of combination therapy with another chelator to enhance iron clearance. Patient education was reinforced, particularly emphasizing the importance of hydration and dietary modifications to support liver function. The patient’s care plan was revised to include more frequent monitoring of liver and cardiac function, aiming to preemptively address any emerging complications and adjust treatment as necessary. This approach underscored the necessity of a dynamic treatment strategy tailored to the evolving clinical picture in managing Beta thalassemia major.

Section 3

Two weeks after the implementation of adjunctive measures, the patient returned for another follow-up appointment. During the visit, she reported mild improvement in fatigue but continued to experience episodes of dizziness, especially upon standing, which suggested possible orthostatic hypotension. Her blood pressure was measured at 92/58 mmHg when supine and dropped to 85/50 mmHg upon standing, with an accompanying increase in heart rate to 110 beats per minute. These findings prompted the team to reconsider her fluid and electrolyte management, emphasizing the need for adequate hydration and possibly adjusting her current medications.

Laboratory results revealed a further decrease in ferritin levels to 2,000 ng/mL, indicating a gradual but ongoing reduction in iron overload. However, her hemoglobin level remained low at 7.0 g/dL, raising concerns about her bone marrow's ability to compensate for ongoing hemolysis or the potential for inadequate erythropoiesis. The liver function test showed a slight improvement in bilirubin levels, yet liver enzymes remained elevated, suggesting persistent hepatic stress. The renal panel showed stable kidney function, but creatinine levels had risen slightly to the upper limit of normal, necessitating close surveillance of renal function given her chelation therapy.

In light of these findings, the multidisciplinary team decided to adjust the patient's care plan. This included exploring the potential benefit of adding erythropoiesis-stimulating agents to support red blood cell production and considering an alternative chelation therapy to mitigate potential renal strain. The team also emphasized patient education on recognizing signs of orthostatic hypotension and the importance of regular follow-ups to monitor her condition closely. The evolving clinical picture highlighted the complex interplay of managing Beta thalassemia major, necessitating a personalized and adaptive approach to her treatment regimen.

Section 4

As the weeks progressed, the patient returned to the clinic for a scheduled evaluation. This time, she reported a slight reduction in dizziness, although she still experienced episodes when rising quickly. Her compliance with increased hydration and lifestyle modifications seemed to have a positive effect, though not completely resolving the issue. Upon examination, her vital signs indicated a supine blood pressure of 94/60 mmHg, which decreased to 88/54 mmHg when standing, with a heart rate increase to 105 beats per minute. While these figures suggested some improvement in her orthostatic hypotension, the persistence of symptoms warranted further investigation into her fluid balance and autonomic function.

The most recent laboratory results showed a hemoglobin level of 7.5 g/dL, a modest improvement suggesting an initial positive response to the erythropoiesis-stimulating agents. However, the team noted a concerning trend in her renal function: the creatinine level had increased slightly above the normal range, now at 1.4 mg/dL. This raised a red flag about the potential nephrotoxicity of her current chelation therapy. Ferritin levels continued to decline, now at 1,800 ng/mL, indicating ongoing but controlled reduction of iron overload. The liver function tests showed stable enzyme levels, which was reassuring given the hepatic strain.

In response to these developments, the care team decided to switch to a different chelation agent with a more favorable renal profile, aiming to alleviate the renal strain. They also reinforced the importance of monitoring her orthostatic symptoms, advising her to rise slowly from seated positions and to maintain adequate fluid intake. Additionally, the team planned to closely monitor her renal function and hemoglobin levels over the coming weeks to assess the impact of these changes. This adaptive strategy highlighted the dynamic nature of managing Beta thalassemia major, necessitating ongoing vigilance and flexibility in treatment planning.

Section 5

Two weeks later, the patient returned for a follow-up appointment, reporting that her dizziness had lessened considerably, though she still occasionally felt lightheaded upon standing. She noted an improvement in her general well-being, attributing some of this to the recent switch in her chelation therapy. Her compliance with lifestyle adjustments, such as adequate hydration and slow positional changes, remained consistent. During the examination, her vital signs showed a supine blood pressure of 96/62 mmHg, with a standing blood pressure that now only marginally decreased to 92/60 mmHg; her heart rate increased to 98 beats per minute upon standing. These findings indicated a modest improvement in her orthostatic tolerance, suggesting that the interventions were beginning to stabilize her hemodynamic status.

New laboratory results, however, presented a mixed picture. Her hemoglobin level had further improved to 8.0 g/dL, a positive sign that the erythropoiesis-stimulating agent was effectively managing her anemia. Nonetheless, there was a slight uptick in her creatinine level to 1.5 mg/dL, indicating ongoing renal stress despite the change in chelation therapy. Ferritin levels continued to decrease, now at 1,500 ng/mL, reflecting effective iron load management without the previous chelation-associated nephrotoxicity. Liver function tests remained stable, providing some reassurance regarding her hepatic status.

In light of these findings, the care team decided to maintain the current chelation regimen but emphasized the necessity of regular renal monitoring to prevent further deterioration. Additionally, they recommended a nephrology consultation to explore potential renal-protective strategies. The team also considered adjusting the dosage of her erythropoiesis-stimulating agents to optimize her hemoglobin levels further without exacerbating her renal condition. This plan illustrated the complexity of balancing multiple treatment goals in Beta thalassemia major, emphasizing the importance of continuous assessment and adaptation in her care strategy.