Contraception and Sickle Cell Disease

Sickle cell disease carries high maternal and perinatal risk in pregnancy: painful crises, acute chest syndrome, infection, pre-eclampsia, growth restriction and preterm birth. Hydroxyurea and some other medicines are teratogenic. Reliable contraception and planned pregnancy are therefore part of usual care, and contraceptive choice must weigh thrombotic and bone risks.

Assessment. Genotype, frequency of crises, previous stroke or acute chest syndrome, thrombosis, haemoglobin and iron status, kidney and liver function, hypertension, current medicines (hydroxyurea, folic acid, penicillin prophylaxis, analgesics, chelators), bone health and transfusion programme.

Reasonable certainty of non-pregnancy. The usual criteria apply.

Effects of the disease. Chronic haemolytic anaemia, risk of thrombosis during crises, delayed puberty and reduced bone density are common and affect selection.

Effectiveness. First-year pregnancies per 100 women with typical use: implants 0.05, intrauterine devices 0.2 to 0.8, injectables 3, combined pills 8.

Eligibility.

Method comparison. Progestin-only methods, especially DMPA, are favoured because they do not raise thrombotic risk and may reduce the frequency of painful crises. The levonorgestrel device reduces bleeding and anaemia. Copper devices can increase bleeding and are used with follow up and iron.

Bone density. DMPA lowers bone density, and sickle cell disease already does; use it with calcium and vitamin D, bone review in long-term users, and an implant or levonorgestrel device as the alternative.

Teratogenic and interacting medicines. Hydroxyurea is teratogenic; reliable contraception is required and patients stop it before conception. Enzyme-inducing medicines used for seizures reduce implant and pill effect.

Special groups. Adolescents benefit from long-acting methods. Women on regular transfusion have iron overload and need liver review. Women after stroke avoid combined methods.

Regimen lines.

• DMPA 150 mg intramuscularly every 13 weeks, up to 4 weeks late, or 104 mg subcutaneously every 13 weeks, up to 4 weeks late; backup 7 days if started after day 7 of bleeding.

• Etonogestrel 68 mg rod: 3 years; levonorgestrel two 75 mg rods: 5 years or 3 years by system; backup 7 days if inserted after day 7 of bleeding.

• Levonorgestrel 52 mg device: 5 years; copper T 380A: up to 12 years; no backup.

• Progestin-only pill: one tablet daily without a break; late window 3 hours for the traditional pill, 12 hours for desogestrel and 24 hours for drospirenone; backup 2 days after a late or missed pill.

• Combined pills: only when stable, non-smoking, with normal blood pressure and no thrombosis or stroke history.

Preconception. Plan pregnancy when disease is stable, stop hydroxyurea at least 3 months before conception, continue folic acid 5 mg daily, review immunisations and screen for organ damage.

Follow up. Review at routine sickle cell visits, including bleeding pattern, haemoglobin, bone health and blood pressure.

Crises on contraception. Most crises have other triggers; stop a combined method if crises worsen or vascular events occur.

Bleeding. Treat bothersome progestin-related bleeding with ibuprofen 800 mg three times daily for 5 days if kidney function is normal; avoid NSAIDs in renal impairment and use tranexamic acid dose-reduced if thrombosis risk is excluded. Correct iron and folate deficiency.

Infection. Pelvic infection with an intrauterine device in a patient with functional asplenia needs prompt antibiotics and low threshold for admission.

Red flags. Chest pain, fever, severe headache, focal deficit, severe pelvic pain.

Referral and escalation. Refer pregnancy, stroke, acute chest syndrome and suspected thrombosis urgently, and review contraception with the haematology team.