What is the management for a sickle cell crisis?

Management of Sickle Cell Crisis

Prompt pain control with opioid analgesics, aggressive hydration, oxygen therapy as needed, and prevention of complications through careful monitoring and supportive care are the cornerstones of managing a sickle cell crisis. 1

Pain Management

• Patient-controlled analgesia (PCA) is highly effective for moderate to severe pain during vaso-occlusive crisis, with scheduled around-the-clock dosing preferred over as-needed dosing 1
• For severe pain, administer parenteral opioids such as morphine promptly 1
• Continue long-acting opioid medications if the patient is already taking them for chronic pain management 2, 1
• Use appropriate validated pain assessment scales and reassess pain regularly 2
• Encourage patients to report pain, particularly pain similar to their usual sickle pain 2
• Notify the pain team in advance if a patient with sickle cell disease is undergoing major surgery, especially if there is a history of opioid tolerance 2

Hydration Therapy

• Provide aggressive hydration as patients with sickle cell disease have impaired urinary concentrating ability and dehydrate easily 1, 3
• Prefer oral hydration when possible, but administer intravenous fluids if oral intake is inadequate 1
• Consider using 5% dextrose solution or 5% dextrose in 25% normal saline for intravenous hydration rather than normal saline due to reduced ability to excrete sodium load 3
• Monitor fluid balance carefully to prevent overhydration, with accurate measurement of intake and output 1
• Continue intravenous fluids postoperatively until the patient can tolerate adequate oral fluid intake 2

Oxygen Therapy

• Document baseline oxygen saturation and monitor continuously 2, 1
• Administer oxygen to maintain SpO2 above baseline or 96% (whichever is higher) 2, 1
• Continue oxygen monitoring until saturation is maintained at baseline in room air 1
• For patients undergoing surgery, administer oxygen continuously for 24 hours postoperatively or until the patient can mobilize freely 2

Prevention and Management of Complications

Acute Chest Syndrome

• Implement early recognition and aggressive treatment with oxygen, incentive spirometry, analgesics, and antibiotics 1
• Promote early mobilization, physiotherapy, and incentive spirometry every 2 hours after moderate or major surgery 2
• Consider continuous positive airway pressure, high-flow nasal oxygen therapy, or nasopharyngeal prong airway if needed 2
• Consider bronchodilator therapy for patients with a history of small airways obstruction, asthma, or acute chest syndrome 2
• Simple or exchange transfusions may be necessary in severe cases 1

Infection Management

• Obtain blood cultures if the patient becomes febrile 1
• Start antibiotics promptly if temperature reaches ≥38.0°C or if there are signs of sepsis 1
• Administer vigorous antibiotic therapy as infections are a leading cause of morbidity and mortality 3

Stroke

• Urgently evaluate any acute neurologic symptom other than transient mild headache 1
• Initial evaluation should include CBC, reticulocyte count, blood type and crossmatch, and neuroimaging 1
• Consider partial exchange transfusion or erythrocytapheresis to reduce HbS to <30% and raise hemoglobin to 10 g/dL 1

Priapism

• Treat as a painful event with hydration and analgesia 1
• Immediately notify the hematology team 4
• Regularly examine men receiving regional anesthesia as they may not notice priapism due to altered sensation 2, 4

Transfusion Therapy

• For elective surgery, consider the following transfusion strategy 2:
  • High-risk surgery: Exchange transfusion aiming for Hb of 100 g/L
  • Low/medium-risk surgery with baseline Hb <90 g/L: Top-up transfusion aiming for Hb of 100 g/L
  • Low/medium-risk surgery with baseline Hb ≥90 g/L: Partial exchange transfusion aiming for Hb of 100 g/L
• Ensure donor red cells are HbS negative and compatible for ABO, Rh, and Kell antigens 2
• Use blood that is <10 days old for simple transfusion and <8 days old for exchange transfusion when possible 2

Temperature Management

• Maintain normothermia during the perioperative period 2
• Avoid hypothermia as it can lead to shivering and peripheral stasis, increasing sickling 4

Multidisciplinary Care

• Involve hematology specialists for regular assessment of patients with moderate to severe crises 1
• Consider low threshold for admission to high dependency or intensive care units after surgery 2, 4
• Work in partnership with patients and their families, keeping them informed of clinical decisions 1

Common Pitfalls and Caveats

• Avoid overhydration which can lead to complications such as pulmonary edema 1
• Avoid hypoxia as it can precipitate sickling 1
• Do not assume opioid dependency in patients with sickle cell disease; opioid sensitivity is more common 2
• Avoid excessive starvation periods before procedures as this can precipitate crises 2
• Reserve blood transfusion only for when really indicated, not routinely 3