Sickle Cell Crisis in Pregnancy

Sickle cell crisis in pregnancy is an acute episode of vaso-occlusion with severe pain, caused by sickled red cells blocking small vessels. Triggers include dehydration, infection, cold, hypoxia and stress, and pregnancy makes crises more frequent and severe. It matters because acute chest syndrome, stroke and sepsis can follow, and fetal distress and preterm labour are common.

• Common in women with HbSS in pregnancy.
• Pain is the main symptom; fever means look for infection.
• Treat early with fluids, oxygen and analgesia.

Women have sudden severe pain in bones, back, chest or abdomen, often with fever, pallor and jaundice. Examine temperature, oxygen saturation, chest, abdomen, spleen and fetal heart.

• Chest pain with fever and hypoxia suggests acute chest syndrome.
• Painful joints and swelling suggest bone infarction or osteomyelitis.
• Differential diagnosis: abruption, preterm labour, appendicitis, pyelonephritis, cholecystitis, pulmonary embolism, preeclampsia and HELLP syndrome, malaria, osteomyelitis.
• Complications: acute chest syndrome, stroke, sepsis, severe anaemia, fat embolism, renal failure, venous thromboembolism, preterm labour, fetal distress, maternal death.

The approach is clinical with tests to find the trigger and complications.

• Full blood count, reticulocytes (compared with baseline), blood culture, urine culture, malaria test.
• Urea, creatinine, liver enzymes, lactate dehydrogenase, group and save.
• Chest X-ray with shielding for chest symptoms; ultrasound for abruption, fetal wellbeing and gallstones.
• Oxygen saturation continuously; cardiotocography.

The principle is rapid analgesia, hydration, oxygen and treatment of the trigger.

• Supportive: admit; warm room; oxygen to keep saturation at 95 percent or above; oral fluids 3 litres per day, plus IV sodium chloride 0.9 percent 1 litre over 6 hours if dehydrated (avoid overload); incentive spirometry every hour while awake; left lateral position; fetal monitoring.
• Pharmacological: paracetamol 1 g oral or IV 6-hourly (maximum 4 g in 24 hours); morphine 2.5 to 10 mg IV or 5 to 10 mg IM every 2 to 4 hours as needed for severe pain, then oral morphine 10 to 20 mg 4-hourly; avoid pethidine; avoid NSAIDs after 20 weeks; ceftriaxone 2 g IV daily for fever or chest signs, plus azithromycin 500 mg oral daily for 5 days for acute chest syndrome; artesunate 2.4 mg/kg IV at 0, 12 and 24 hours then daily for severe malaria; enoxaparin 40 mg subcutaneous daily; folic acid 5 mg oral daily.
• Procedures: simple transfusion if haemoglobin is below 6 g/dL or 2 g/dL below baseline; exchange transfusion for acute chest syndrome with hypoxia, stroke or multiorgan failure; delivery for obstetric reasons after stabilisation.
• Special groups: women with kidney or lung disease have fluids guided by saturation and urine output; give betamethasone 12 mg IM twice, 24 hours apart, before 34 weeks if delivery is likely.
• Red flags: chest pain, oxygen saturation below 94 percent, fever above 38 degrees Celsius, weakness or confusion, rapidly falling haemoglobin, reduced fetal movements.
• Follow-up: daily review in hospital; discharge when pain is controlled with oral drugs; growth scan 2 weeks later; review in 1 week; haemoglobin monthly.