How to manage a sickle cell crisis in a pregnant patient?

Management of Sickle Cell Crisis in Pregnant Patients

Pregnant patients with sickle cell disease experiencing a crisis require immediate multidisciplinary management at a specialized center, with aggressive pain control using opioids (including epidural analgesia when appropriate), careful hydration with 5% dextrose solutions, oxygen therapy to maintain SpO2 ≥96%, and prompt treatment of any fever with broad-spectrum antibiotics while monitoring closely for life-threatening complications like acute chest syndrome. 1, 2, 3

Immediate Assessment and Stabilization

Pain Management

• Initiate parenteral opioids (morphine) immediately for severe pain without delay, as inadequate pain control worsens the crisis and leads to complications 2, 3
• Consider epidural analgesia as a primary intervention for severe pain crisis, as it provides excellent pain relief and improves peripheral blood flow through sympathetic blockade 2
• Continue baseline long-acting opioid medications if the patient is already taking them for chronic pain management 3
• Use patient-controlled analgesia with scheduled around-the-clock dosing rather than as-needed dosing for moderate to severe pain 3
• Implement multimodal analgesia techniques including appropriate hydration and oxygenation 2

Hydration Protocol

• Administer 5% dextrose solution or 5% dextrose in 25% normal saline rather than normal saline alone, as patients with sickle cell disease have impaired urinary concentrating ability and cannot excrete sodium loads effectively 4, 3
• Oral hydration is preferred when possible, but IV fluids should be started if oral intake is inadequate 4, 3
• Monitor fluid balance carefully to prevent overhydration, which can lead to pulmonary edema 3

Oxygen and Temperature Management

• Maintain SpO2 above baseline or 96% (whichever is higher) with continuous oxygen monitoring 2, 4, 3
• Keep the patient normothermic, as hypothermia leads to shivering, peripheral stasis, hypoxia, and increased sickling 2, 3
• Document baseline oxygen saturation and continue monitoring until saturation is maintained at baseline in room air 3

Infection Surveillance and Management

Fever Protocol

• If temperature reaches ≥38.0°C, obtain blood cultures and start broad-spectrum antibiotics immediately without waiting for culture results, as functional hyposplenism makes these patients vulnerable to overwhelming sepsis from encapsulated organisms like Streptococcus pneumoniae within hours 4, 3
• Obtain a chest radiograph to evaluate for pneumonia or acute chest syndrome 4
• Never delay antibiotics while waiting for culture results, as sepsis can progress to death within hours in functionally asplenic patients 4

Monitoring for Pregnancy-Specific Complications

High-Risk Pregnancy Context

• Pregnancy precipitates sickle complications through increased metabolic demand, susceptibility to infection, pro-thrombotic state, and aortocaval compression 1, 2
• Pregnant patients with sickle cell disease have a 57% incidence of painful crises, 38% cesarean delivery rate, 23% ICU admission rate, and 5-6% premature delivery rate 1, 2
• Maternal mortality is estimated at 1-3%, though recent data from specialist centers report no maternal deaths 1

Acute Chest Syndrome Surveillance

• Acute chest syndrome is a life-threatening complication characterized by new segmental infiltrate on chest radiograph, lower respiratory tract symptoms, chest pain, and/or hypoxemia 4, 3
• Implement incentive spirometry every 2 hours for prevention, especially in patients with thoracoabdominal pain 3
• Early recognition and aggressive treatment with oxygen, incentive spirometry, analgesics, and antibiotics are essential 3
• Simple or exchange transfusions may be necessary in severe cases 3

Thromboprophylaxis

• Administer thromboprophylaxis, as patients with sickle cell disease have increased risk of deep vein thrombosis, especially during pregnancy 2
• Pay attention to the timing of low molecular weight heparin when planning central neuraxial blockade 1
• Post-partum low molecular weight heparin should be prescribed with advice from the haematologist, based on post-delivery weight and usually continued for 6 weeks after cesarean section 1

Transfusion Considerations

Indications for Transfusion

• Prophylactic transfusion is not routinely offered except for high-risk patients or multiple pregnancies 1
• Consider transfusion therapy based on hemoglobin levels and clinical status during crisis 2
• For severe acute chest syndrome or acute anemia, transfusion may be indicated 3, 5
• Automated red blood cell exchange is preferred over other transfusion methods for patients with iron overload or severe acute chest syndrome 5

Transfusion Requirements

• Donor red cells should be HbS negative and must be compatible for ABO, Rh and Kell antigens and for additional known allo-antibodies 1
• Blood should ideally be <10 days old for simple transfusion and <8 days old for exchange transfusion 1
• If the patient has been transfused within 28 days, there should be a minimum of 72 hours between the group and save specimen and blood cross match 1

Multidisciplinary Team Involvement

Essential Team Members

• Immediately notify the obstetric anaesthetist when a patient is admitted with a sickle cell crisis during pregnancy 1
• Inform the haematology team when a patient with sickle cell disease is admitted and requires management 1
• Involve Maternal-Fetal Medicine specialists for high-risk obstetrical management 2
• Engage transfusion medicine specialists for complex transfusion requirements 1

Level of Care Decisions

• There should be a low threshold for admitting patients to high dependency or intensive care units, as emergency presentations convey higher risk and patients are more likely to have a sickle crisis either as the precipitating cause or as a complication 4, 3
• High-dependency or ICU care should be considered, especially following general anaesthesia 1
• Labour and the early puerperium are high-risk periods requiring intensive monitoring 1

Analgesia for Labour and Delivery

Regional Anesthesia Preference

• Epidural analgesia is ideal for labour, particularly if there is opioid tolerance or sickle-related pain in the lower body 1
• Regional anaesthesia is preferred for caesarean section as general anaesthesia carries additional risks compared with the non-sickle cell disease population 1
• If general anaesthesia is required, give particular attention to effective oxygenation during intubation using passive nasal oxygenation with nasal cannulae or high-flow nasal oxygenation 1
• Consider 'ramping' using an Oxford Head Elevating Laryngoscopy Pillow or similar, especially for the obese parturient, to optimize the position for intubation 1

Postoperative Management

• Optimize postoperative analgesia after caesarean section 1
• Continue multimodal pain management strategies 2

Critical Pitfalls to Avoid

• Never delay adequate pain control due to concerns about medication effects; inadequate pain management worsens the crisis and leads to complications 2
• Never use normal saline alone for hydration; use 5% dextrose or 5% dextrose in 25% normal saline instead 4
• Never delay antibiotics while waiting for culture results if fever ≥38.0°C is present 4
• Avoid hypoxia, as it precipitates sickling 3
• Avoid hypothermia, which leads to shivering and peripheral stasis 2, 3
• Do not fail to involve a multidisciplinary team including hematology, obstetrics, and anesthesiology 2
• Routine surgery should be avoided if the patient is febrile or having a painful crisis 1, 4