A patient with beta thalassemia minor presents with moderate anemia, microcytic red blood cells, and elevated serum ferritin levels. Which nursing intervention is most appropriate to manage the patient's condition and prevent complications?

C) Monitor liver enzymes and collaborate with the healthcare team to initiate iron chelation therapy

A) Administer oral iron supplements to correct anemia

B) Encourage a diet high in iron to increase hemoglobin levels

C) Monitor liver enzymes and collaborate with the healthcare team to initiate iron chelation therapy

D) Advise increased physical activity to improve cardiac function

A patient with beta thalassemia minor presents with moderate anemia, elevated serum ferritin, and mild jaundice. Based on the diagnostic results, which nursing intervention is most appropriate to prevent further complications related to iron overload?

C) Recommend the patient avoid high-iron foods and cease iron supplements.

A) Encourage the patient to increase dietary iron intake.

B) Educate the patient on the importance of regular blood transfusions.

C) Recommend the patient avoid high-iron foods and cease iron supplements.

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

A 32-year-old female with beta thalassemia minor presents with increased fatigue, mild shortness of breath on exertion, occasional dizziness, and tachycardia. Her hemoglobin has decreased to 8.2 g/dL, and her serum ferritin level is elevated at 480 ng/mL. Which of the following nursing interventions is most appropriate to address the patient's current symptoms and prevent further complications?

C) Discuss the potential benefits of iron chelation therapy with the patient and her healthcare team.

A) Encourage increased dietary iron intake to address anemia.

B) Educate the patient about the importance of hydration to prevent vaso-occlusive episodes.

C) Discuss the potential benefits of iron chelation therapy with the patient and her healthcare team.

D) Advise the patient to reduce physical activity to minimize symptoms of fatigue and dizziness.

A 32-year-old female with beta thalassemia minor presents with increased fatigue, mild shortness of breath on exertion, and occasional dizziness. Her laboratory results indicate a drop in hemoglobin to 8.2 g/dL, increased ferritin level at 480 ng/mL, and elevated LDH at 350 U/L. Considering the patient's clinical presentation and laboratory findings, which nursing intervention is most appropriate to address the risk of iron overload in this patient?

B) Suggest the initiation of iron chelation therapy to manage iron overload.

A) Educate the patient about dietary changes to include more iron-rich foods.

B) Suggest the initiation of iron chelation therapy to manage iron overload.

C) Encourage increased physical activity to improve hemoglobin levels.

D) Suggest the patient limit fluid intake to manage tachycardia.

A patient with anemia and suspected iron overload is being considered for iron chelation therapy. The nurse knows that which of the following is the most important factor to assess before initiating this therapy?

A) The patient's current liver function tests

B) The patient's history of hypertension

C) The patient's dietary intake of iron-rich foods

D) The patient's current resting heart rate

A patient with anemia secondary to iron overload is being considered for iron chelation therapy. Which nursing intervention is most important to implement while monitoring the effectiveness of this treatment?

C) Evaluate liver function tests regularly to detect any hepatic impairment.

A) Monitor the patient's blood pressure and heart rate for signs of cardiovascular instability.

B) Assess the patient's skin and sclera for changes in pallor and icterus.

C) Evaluate liver function tests regularly to detect any hepatic impairment.

D) Educate the patient on increasing dietary iron intake to support hemoglobin levels.

A patient with beta thalassemia presents with increased abdominal discomfort, hepatomegaly, and worsening scleral icterus. Laboratory findings indicate iron overload, elevated liver enzymes, and thrombocytopenia. The care team plans to initiate iron chelation therapy. What is the primary nursing consideration in the management of this patient?

C) Ensuring compliance with chelation therapy to prevent further organ damage

A) Monitoring for signs of infection due to immunosuppression

B) Assessing for changes in neurological status due to potential cerebral edema

C) Ensuring compliance with chelation therapy to prevent further organ damage

D) Educating the patient on dietary modifications to reduce iron intake

A patient with beta thalassemia presents with increased abdominal discomfort and fullness after meals, mild hepatomegaly, and scleral icterus. Laboratory findings include elevated serum ferritin, increased liver enzymes, and thrombocytopenia. What is the most appropriate nursing intervention to address the patient's current condition?

C) Educate the patient about the importance of adhering to iron chelation therapy to prevent further organ damage.

A) Encourage the patient to increase dietary fiber intake to alleviate abdominal discomfort.

B) Monitor the patient’s vital signs and oxygen saturation closely to detect early signs of respiratory distress.

C) Educate the patient about the importance of adhering to iron chelation therapy to prevent further organ damage.

D) Suggest the patient perform regular abdominal exercises to reduce sensations of fullness and discomfort.

A patient with beta thalassemia is undergoing iron chelation therapy with deferasirox but presents with increased fatigue, abdominal discomfort, and elevated serum ferritin levels. Which nursing intervention is most appropriate to address the patient's current condition and support her treatment plan?

B) Discuss with the healthcare provider the possibility of adjusting the chelation therapy to a more effective regimen.

A) Educate the patient about increasing dietary intake of iron-rich foods to boost hemoglobin levels.

B) Discuss with the healthcare provider the possibility of adjusting the chelation therapy to a more effective regimen.

C) Encourage the patient to take over-the-counter iron supplements to manage anemia.

D) Recommend strict bed rest to reduce fatigue and abdominal discomfort.

A patient with beta thalassemia who has been on deferasirox therapy presents with fatigue, abdominal discomfort, and an elevated serum ferritin level. A liver ultrasound reveals early signs of fibrosis, and the patient's hemoglobin level is 9.0 g/dL. What is the most appropriate nursing intervention to address the patient's current condition?

B) Monitor the patient's liver function tests closely and inform the healthcare provider of any significant changes.

A) Encourage the patient to increase dietary intake of iron-rich foods to improve hemoglobin levels.

B) Monitor the patient's liver function tests closely and inform the healthcare provider of any significant changes.

C) Advise the patient to reduce physical activity to conserve energy and reduce fatigue.

D) Discuss with the healthcare team the potential need for a blood transfusion to manage anemia.

beta thalassemia anaemia - Nursing Case Study

Pathophysiology

• Primary mechanism: Beta thalassemia anemia is caused by mutations in the HBB gene, leading to reduced or absent synthesis of the beta-globin chains of hemoglobin. This imbalance results in excess alpha-globin chains, which form unstable aggregates that damage red blood cell membranes and lead to hemolysis.

• Secondary mechanism: Ineffective erythropoiesis occurs as the bone marrow produces red blood cells that are destroyed before maturing due to abnormal hemoglobin. This process further contributes to anemia and stimulates compensatory erythropoiesis, often resulting in bone marrow expansion and skeletal deformities.

• Key complication: Chronic anemia and hemolysis increase iron absorption and storage, leading to iron overload. This can cause damage to vital organs such as the heart, liver, and endocrine glands, necessitating regular monitoring and management in affected patients.

Patient Profile

Demographics:

28 years old, female, school teacher

History:

• Key past medical history: Diagnosed with beta thalassemia minor at age 10, history of mild anemia-related fatigue, no history of blood transfusions

• Current medications: Folic acid supplements, occasional use of over-the-counter iron supplements

• Allergies: Penicillin

Current Presentation:

• Chief complaint: Increased fatigue and shortness of breath over the past month

• Key symptoms: Pallor, palpitations, mild jaundice, occasional dizziness

• Vital signs: Blood pressure 105/65 mmHg, heart rate 105 bpm, respiratory rate 22 breaths per minute, temperature 98.6°F, oxygen saturation 94% on room air

Section 1

New Diagnostic Results:

Following the initial assessment, the physician ordered a series of diagnostic tests to evaluate the severity of the patient's anemia and to assess for potential complications related to her beta thalassemia minor. Complete blood count results revealed a hemoglobin level of 8.5 g/dL, indicating a moderate anemia that has likely worsened since previous evaluations. The mean corpuscular volume (MCV) was measured at 68 fL, consistent with microcytic anemia, which is typical in beta thalassemia minor. Serum ferritin levels were elevated at 450 ng/mL, suggesting iron overload, a common complication due to increased intestinal absorption of iron secondary to chronic anemia and ineffective erythropoiesis. Elevated levels of indirect bilirubin, at 2.1 mg/dL, confirmed the occurrence of hemolysis, contributing to the patient's mild jaundice.

Additionally, liver function tests showed an increase in alanine aminotransferase (ALT) and aspartate aminotransferase (AST), raising concern for potential liver damage due to iron deposition. An echocardiogram was performed due to the patient's palpitations and revealed mild left ventricular hypertrophy, which could be a response to chronic anemia and increased cardiac workload. The findings of iron overload and early organ involvement underscore the need for a comprehensive management plan to prevent further complications.

These diagnostic results highlight the complexity of managing beta thalassemia minor with evolving complications. They necessitate careful clinical reasoning to balance the need for managing anemia while preventing iron overload. The patient's care team must consider interventions such as the cessation of iron supplements, initiation of iron chelation therapy, and potential lifestyle adjustments to optimize her quality of life and prevent further organ damage. These findings pave the way for a multidisciplinary approach, involving hematology, cardiology, and nutrition specialists, to address both immediate symptoms and long-term health outcomes.

Section 2

Change in Patient Status:

Two weeks after the initial diagnostic results, the patient, a 32-year-old female with beta thalassemia minor, presented to the clinic with new symptoms, including increased fatigue, mild shortness of breath on exertion, and occasional dizziness. Her physical examination revealed pallor, slight scleral icterus, and a resting tachycardia with a heart rate of 102 beats per minute. Blood pressure was recorded at 115/78 mmHg, and respiratory rate was 18 breaths per minute. Her oxygen saturation was stable at 97% on room air. The patient's weight remained stable, and she reported no new dietary changes or significant lifestyle alterations.

Recent laboratory tests indicated a slight further drop in hemoglobin to 8.2 g/dL, while the mean corpuscular volume remained low at 67 fL. The serum ferritin level increased to 480 ng/mL, suggesting progressive iron accumulation. An elevated lactate dehydrogenase (LDH) level of 350 U/L pointed to ongoing hemolysis. The patient's echocardiogram was repeated, showing no significant change in left ventricular hypertrophy, but a slight increase in left atrial size was noted, indicating a potential strain on the heart due to chronic anemia. Liver function tests continued to show elevated ALT and AST, though they remained stable compared to previous results.

Given these changes, the care team is concerned about the potential progression towards more severe complications, such as heart failure or significant liver damage. The current focus is on reassessing the appropriateness of the patient's current management plan, including the need for iron chelation therapy and potential adjustments in lifestyle or dietary habits to mitigate iron overload. The team is considering increasing the frequency of monitoring and possibly introducing a low-dose beta-blocker to manage the patient's tachycardia and reduce cardiac workload. These developments call for ongoing collaboration among her healthcare providers to ensure a holistic approach to her care and prevent further deterioration.

Section 3

Two weeks following the adjustments in her management plan, the patient returned to the clinic for an evaluation of her response to the new interventions. Upon assessment, it was noted that her resting heart rate had decreased to 88 beats per minute following the initiation of a low-dose beta-blocker, indicating an effective reduction in cardiac workload. Despite this improvement, the patient continued to experience episodes of fatigue and shortness of breath, particularly during moderate exertion. Her physical examination revealed persistent pallor and scleral icterus, although her blood pressure remained stable at 112/76 mmHg with oxygen saturation consistently at 97% on room air.

A new set of laboratory tests showed a slight improvement in hemoglobin, now at 8.5 g/dL, suggesting a marginal stabilization of her anemia. However, serum ferritin levels continued to rise, now at 510 ng/mL, underscoring the ongoing concern of iron overload. Liver function tests revealed a slight increase in ALT and AST levels, raising concerns about progressive liver involvement. The care team suspects that iron deposition in the liver may be contributing to hepatic strain, and thus the initiation of iron chelation therapy is being strongly considered to address the iron accumulation.

The patient's care team is now deliberating on the most effective iron chelation protocol, considering her current liver function and potential side effects. They are also evaluating dietary modifications and the potential inclusion of a nutritionist to aid in managing iron intake. The persistent symptoms and laboratory trends highlight the need for ongoing multidisciplinary management to prevent further complications, such as cirrhosis or cardiac complications, and to optimize the patient's quality of life. This comprehensive approach aims to address both the hematologic and systemic implications of her condition.

Section 4

During the following clinic visit, the patient reported a noticeable increase in abdominal discomfort and a sense of fullness, particularly after meals. Upon physical examination, mild hepatomegaly was palpated, suggesting further progression of hepatic involvement. Her vital signs remained stable, with a heart rate of 90 beats per minute and blood pressure at 110/74 mmHg. Her oxygen saturation was unchanged at 97% on room air, but her scleral icterus appeared more pronounced, reflecting worsening bilirubin levels.

Laboratory tests revealed a further increase in serum ferritin to 620 ng/mL, indicating continued iron overload despite dietary modifications. Liver function tests showed a continued rise in ALT and AST, now at 65 U/L and 70 U/L respectively, confirming hepatic strain. Additionally, new findings included a mild thrombocytopenia with platelet counts dropping to 130,000/μL, raising concerns about potential hypersplenism or bone marrow suppression due to iron deposition.

Given these developments, the care team is prioritizing the initiation of iron chelation therapy to mitigate further organ damage. They are also considering a liver ultrasound to assess for fibrosis and potential cirrhosis. The patient's ongoing fatigue and shortness of breath, coupled with these new complications, underscore the complexity of managing beta thalassemia with iron overload. This situation highlights the importance of timely intervention and careful monitoring to prevent further deterioration and preserve organ function. The team is also contemplating a referral to a hepatologist to provide specialized care for her liver-related issues as part of a comprehensive management plan.

Section 5

In the subsequent weeks, the patient's condition showed a change in status, prompting a reevaluation of her management plan. Although iron chelation therapy had been initiated with deferasirox, the patient began experiencing increased fatigue and persistent abdominal discomfort. A follow-up liver ultrasound revealed early signs of fibrosis, but no definitive cirrhosis was observed at this stage. This finding aligned with the ongoing hepatic strain seen in her liver function tests. Additionally, her spleen appeared slightly enlarged, suggesting potential hypersplenism as a contributing factor to her thrombocytopenia.

The care team noted a mild increase in her heart rate to 92 beats per minute, indicating a potential compensatory response to her anemia and ongoing organ stress. Meanwhile, her hemoglobin level had decreased to 9.0 g/dL, reflecting the chronic hemolysis associated with beta thalassemia. Despite the introduction of chelation therapy, her serum ferritin levels remained elevated, now at 680 ng/mL, indicating the need for potentially more aggressive treatment or alternative chelation strategies.

In light of these developments, a referral to a hepatologist was expedited to further evaluate the extent of liver involvement and to consider additional interventions that could support her hepatic function. The team also discussed the possibility of adjusting her chelation regimen or adding erythropoiesis-stimulating agents to manage her anemia more effectively. These changes aim to address her multifaceted condition, emphasizing the need for a multidisciplinary approach to prevent further complications and optimize her quality of life.