Ligne directrice

WHO recommendations on the management of sickle cell disease during pregnancy, childbirth and the interpregnancy period

Année de publication

2025

Institution émettrice

World Health Organization

Liens

Recommandations

21 recommandations incluses

Advise pregnant women with sickle-cell disease (SCD) living outside malaria endemic areas to continue daily supplementation with up to 5 mg folic acid or to initiate supplementation at this dose as soon as possible.

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Advise pregnant women with sickle-cell disease (SCD) taking intermittent preventive treatment with sulfadoxine-pyrimethamine that 400 µg folic acid supplementation daily is appropriate, as higher doses may counteract the efficacy of the antimalarial.

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Advise pregnant women with sickle-cell disease (SCD) that iron supplementation is not needed unless there is evidence of iron deficiency.

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For pregnant women with sickle-cell disease (SCD) who are iron deficient, advise iron supplementation as for the general pregnant population

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For pregnant women with sickle-cell disease (SCD) and a history of severe intractable crises (i.e. recurrent painful crises and/or events unresponsive to other treatment modalities) or with lived experience of previous benefit from prophylactic transfusion outside of pregnancy, consider prophylactic blood transfusion.

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For pregnant women with sickle-cell disease (SCD) previously controlled with hydroxycarbamide (hydroxyurea), consider continuation or recommencement (after the first trimester) of the medication in the context of shared decision making involving the woman and a multidisciplinary team that includes experts in SCD and pregnancy. Base risk–benefit analyses on the woman’s symptom severity, stage of pregnancy and her views and preferences.

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For pregnant women with sickle-cell disease (SCD) (not hospitalized), consider additional risk factors for thromboembolism (e.g. prior venous thromboembolism (VTE) following vaso-occlusive events) and follow local recommendations for initiation of thromboprophylaxis for pregnant women with elevated risk of thrombotic events (e.g. prior VTE, obesity, inherited thrombophilia).

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For pregnant women with sickle-cell disease (SCD) , advise against routine infection prophylaxis and implement frequent screening for infection (such as urinary tract infection),using a low diagnostic threshold for bacterial urinary tract infection.

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Non recommandé

For pregnant women with sickle-cell disease (SCD) who are experiencing acute sickle-related pain, offer timely and optimal pain relief.

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Pain management for pregnant women with sickle-cell disease (SCD). When advising use of analgesia, options include oral paracetamol, nonsteroidal anti-inflammatory drug (NSAIDs), or opioids at the lowest effective dose for the shortest period of time required to manage pain.

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Pain management for pregnant women with sickle-cell disease (SCD). When advising use of analgesia, consider the stage of pregnancy and contraindications for specific medications, the woman’s views, preferences and previous experience of the medication, risk of dependence, and availability.

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Collaborate with pregnant women with sickle-cell disease (SCD) to develop individualized pain-management plans as early in pregnancy as possible, basing the plan on severity and frequency of pain crises, and the woman’s views and preferences, and including a multidisciplinary team approach to care.

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For pregnant women with sickle-cell disease (SCD) hospitalized with vaso-occlusive crisis and requiring intravenous fluid hydration, implement frequent clinical monitoring (such as with lung auscultation, oxygenation/pulse oximetry, respiratory rate) for early identification of fluid overload and pulmonary oedema.

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For pregnant women with sickle-cell disease (SCD) requiring intravenous fluid hydration in the context of obstetric complications such as pre-eclampsia, implement more intensive monitoring for signs of fluid overload.

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Offer thromboprophylaxis to pregnant women hospitalized with sickle-cell disease (SCD) unless contraindications are present.

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For pregnant women with sickle-cell disease (SCD) without complications (due to SCD or to obstetric causes), offer growth/biometric scans to identify fetal growth restriction every four weeks from 24 until 32 weeks’ gestation, and then every three weeks until birth.

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For pregnant women with sickle-cell disease (SCD) with complications (due to SCD or obstetric causes), offer individualized intensive fetal monitoring to guide management, taking into consideration the woman’s views and preferences, and the availability of equipment and staff skilled in their use.

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When making decisions about the timing of birth (awaiting spontaneous labour or planned birth) for women with sickle-cell disease (SCD), take an individualized approach based on the anticipated balance of the benefits of continuing pregnancy to allow fetal maturation and the risk of maternal and neonatal morbidities associated with continuation of the pregnancy, and the woman’s views and preferences.

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Base decisions about mode of birth for women with sickle-cell disease (SCD) on the presence or absence of medical or obstetric indications for caesarean birth, availability of local resources (including those required by women to make informed decisions), as well as the woman’s views and preferences. In the absence of medical or obstetric indications for caesarean birth, vaginal birth is preferable.

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Offer women with sickle-cell disease (SCD) in the immediate postnatal period and up to six weeks after childbirth care in addition to that outlined in WHO recommendations on maternal and newborn care for a positive postnatal experience (28): – to manage and treat SCD and its complications, using evidence-based interventions including diseasemodifying agents such as hydroxycarbamide (hydroxyurea) and pain-management strategies; – to prevent morbidities such as thrombotic events by considering the need for, and initiating, thromboprophylaxis, as per local recommendations; – to guide choice of contraceptive methods; – to screen the newborn for SCD; and – to guide counselling on the safety of breastfeeding for the baby

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For women with sickle-cell disease (SCD), integrate care across the life course, including sexual and reproductive health care, with specialized disease care, which may include: supporting optimal health and well-being; providing prepregnancy counselling and guidance on pregnancy planning; and optimizing treatment across and beyond the reproductive continuum.

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