Anaesthesia in patients with sickle cell disease

Overview

Patients with sickle cell disease are at increased risk of perioperative complications, including vaso-occlusive crises and acute chest syndrome. Careful perioperative planning aims to prevent sickling, optimize pain control, and minimize complications.

Perioperative considerations

Avoid factors that precipitate sickle cell crisis

Prevent the following throughout the perioperative period:

• Hypoxia

• Vascular stasis

• Hypothermia

• Hypovolaemia or hypotension

• Acidosis

Pain management

• Optimize perioperative pain control.

• Provide adequate hydration and maintain normothermia.

Monitor for complications

Closely monitor for:

• Vaso-occlusive crisis

• Acute chest syndrome

• Aplastic crisis

• Splenic sequestration syndrome

• Right upper quadrant syndrome

Preoperative optimization

Preoperative optimization should be performed in consultation with a haematologist whenever possible.

Assess risk factors for acute pain crises

Evaluate for:

• Increasing age

• Frequent hospital admissions for pain crises

• Previous blood transfusions

• Evidence of organ damage, including:

  • Low baseline oxygen saturation

  • Elevated serum creatinine

  • Cardiac dysfunction

• Previous central nervous system events

• Concurrent infection

Assess surgical risk

Low-risk procedures

• Minor surgery (e.g., inguinal hernia repair and extremity surgery)

Intermediate-risk procedures

• Intra-abdominal surgery (e.g., cholecystectomy)

High-risk procedures

• Intracranial surgery

• Intrathoracic surgery

• Hip surgery

Haematology consultation

Optimize treatment before surgery by considering:

• Hydroxyurea to increase fetal haemoglobin production.

• Postponing non-emergency surgery if the patient is experiencing a sickle cell crisis.

• Intravenous fluids during the fasting period to prevent dehydration while the patient is nil by mouth (NPO).

Preoperative transfusion

The role of routine preoperative transfusion remains controversial because supporting evidence is limited.

The objectives of transfusion are to:

• Correct pre-existing anaemia.

• Reduce haemoglobin S concentration.

• Increase adult haemoglobin concentration.

Recommendations:

• Consider a target haemoglobin of 6–10 g/dL before surgery.

• Ensure compatible blood is available for any surgical procedure.

• Routine exchange transfusion is not recommended.

Pain management

Treatment should include:

• Rest

• Warmth

• Reassurance

• Adequate analgesia

• Fluid replacement

Analgesic options include:

• Oral analgesics for minor pain episodes.

• Opioids administered by oral, intravenous, intramuscular, or subcutaneous routes.

• Patient-controlled analgesia (PCA) with background infusion or fentanyl patch where appropriate.

• Paracetamol.

• Non-steroidal anti-inflammatory drugs (NSAIDs), particularly for bone pain.

• Ketamine as an adjunct analgesic.

• Regional anaesthesia or nerve blocks when appropriate, including epidural analgesia where indicated.