What to do immediately if a patient with sickle cell disease (SCD) develops fever and chills during blood transfusion?

Immediate Management: Stop the Transfusion

Stop the transfusion immediately when a sickle cell patient develops fever and chills during blood transfusion, as this represents a potential acute transfusion reaction that requires urgent evaluation and can affect patient outcomes 1.

Critical First Steps

The transfusion must be stopped immediately and the blood bank notified, as symptoms of fever and chills during transfusion are nonspecific and overlapping signs that could represent serious complications including acute hemolytic transfusion reaction, febrile non-hemolytic reaction, or bacterial contamination 1.

Immediate Actions After Stopping Transfusion

• Maintain IV access with normal saline to keep the vein open, but disconnect the blood product 1
• Obtain blood cultures immediately if temperature reaches ≥38.0°C, as sickle cell patients are functionally asplenic and vulnerable to overwhelming sepsis from encapsulated organisms within hours 2, 3
• Start broad-spectrum antibiotics without delay if fever ≥38.0°C is confirmed, as delaying antibiotics while awaiting cultures is a critical error in sickle cell patients 2, 3
• Send the blood bag and tubing back to the blood bank for investigation of potential hemolytic reaction or bacterial contamination 1

Why Not the Other Options?

Hydroxyurea (Option A) - Incorrect

• Hydroxyurea is a chronic preventive therapy, not an acute intervention for transfusion reactions 4, 5
• It has no role in managing acute transfusion complications and would not address the immediate fever and chills 4

Hydration Alone (Option C) - Insufficient

• While aggressive hydration is essential in sickle cell crisis management using 5% dextrose solution or 5% dextrose in 25% normal saline (not normal saline alone due to impaired sodium excretion) 2, 3, 6, hydration alone does not address the transfusion reaction
• Hydration should be implemented after stopping the transfusion, not instead of stopping it 2, 3

Complete Management Algorithm After Stopping Transfusion

Immediate Assessment (0-15 minutes)

• Check vital signs including temperature, blood pressure, heart rate, respiratory rate, and oxygen saturation 2
• Maintain oxygen therapy to keep SpO2 above baseline or ≥96% (whichever is higher) 2, 7, 3
• Assess for signs of hemolytic reaction: back pain, chest pain, dyspnea, hemoglobinuria 1, 8

Laboratory Evaluation

• Blood cultures if temperature ≥38.0°C 2, 3
• Complete blood count to assess hemoglobin and detect hemolysis 7
• Direct antiglobulin test (DAT) to evaluate for hemolytic transfusion reaction 1, 8
• Chest radiograph to evaluate for acute chest syndrome or pneumonia 2, 7

Supportive Care

• Aggressive hydration with 5% dextrose or 5% dextrose in 25% normal saline (not normal saline alone) 2, 3, 6
• Active temperature management to maintain normothermia, as hypothermia causes shivering and peripheral stasis that increases sickling 7, 3
• Continue pain management if the patient was experiencing acute chest syndrome or vaso-occlusive crisis 2, 7

Special Considerations for Sickle Cell Patients

Sickle cell patients have unique transfusion risks including:

• High alloimmunization rates (7-30% of patients), making future transfusions more difficult 4, 8
• Risk of delayed hemolytic transfusion reactions with hyperhemolysis, which can be life-threatening 4
• Transfusions can paradoxically trigger sickle cell events including pain crises and acute chest syndrome due to increased blood viscosity 8

When to Resume or Modify Transfusion

• If simple febrile non-hemolytic reaction is confirmed (no hemolysis, cultures negative), transfusion may be resumed with premedication (acetaminophen, antihistamines) after discussion with hematology and blood bank 1
• If acute chest syndrome is severe (bilateral infiltrates), exchange transfusion rather than simple transfusion should be considered once the acute reaction is managed 4, 7
• If hemolytic reaction is confirmed, do not resume transfusion and consult hematology urgently for management of potential hyperhemolysis 4

Critical Pitfall to Avoid

Never continue the transfusion while investigating fever and chills, as bacterial contamination can progress to fatal sepsis within minutes, and acute hemolytic reactions can cause rapid clinical deterioration 1, 8. The risk of stopping a transfusion temporarily is far lower than the risk of continuing a potentially contaminated or incompatible transfusion 1.