Bibliographic information
GuidelineWHO recommendations on the management of sickle cell disease during pregnancy, childbirth and the interpregnancy period
Year of Publication2025
Issuing InstitutionWorld Health Organization
Recommendation
New
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
Recommended
Notes and Remarks
Conceptual approach
- The GDG noted that the interpregnancy phase cannot be defined prospectively. Beginning from the time of birth / termination of pregnancy, it extends beyond the initial six weeks after birth. To support health throughout the reproductive continuum and in the interests of inclusivity, all women of reproductive age who have been pregnant may be considered to be in the ‘interpregnancy’ period, unless they have undergone menopause or taken steps to permanently avoid pregnancy.
- After the initial postnatal period, women need to be transitioned back to specialized care for SCD. When the woman is cared for by a multidisciplinary team during pregnancy, this transition will likely be straightforward and is a fundamental rationale for “integrated” care in and outside of pregnancy. The GDG acknowledged that these care systems may not exist, may be inadequately organized, or remain aspirational in many settings. At a minimum, referral systems and mechanisms need to be in place to communicate a woman’s pregnancy and postnatal course to her general care providers, including those managing her SCD.
- Although most women with SCD will have had previous contact with health-care facilities to manage the condition, the postnatal period (immediate and beyond) may provide additional opportunities for “catch up care”, such as ensuring up-to-date immunization. Risks in the early postnatal period
- The immediate postnatal period (i.e. in the first week postnatally) is often when complications occur.
- Women with SCD have an increased risk of severe maternal morbidity compared to women without SCD (178). In addition, women in active vaso-occlusive crisis at the delivery admission have an approximately nine-fold higher risk of severe maternal morbidity up to 42 days’ post-discharge compared with women with SCD not in crisis at the delivery admission (178).
- Women with SCD have an increased risk of VTE compared to the general population (see ‘Thromboprophylaxis in pregnant women with uncomplicated SCD’; page 26). In the immediate postnatal period, this risk may be further magnified especially in the context of decreased mobility, following a caesarean birth, or if the woman has a history of VTE. Contraception
- The WHO medical eligibility criteria wheel for contraceptive use (179) suggests that for women with SCD, progestogen-only pills, progestogen-only injectables, implants, and levongestrel-releasing intrauterine devices can all be used in any circumstance; and that combined hormonal contraceptives and copper intrauterine devices can be suggested on a case-by-case basis. The availability of each method, however, may vary depending on context, and local guidelines may also provide specific guidance. The GDG also noted that copper intrauterine devices may increase bleeding in the first three months of use. Decisions on contraception need to be tailored to the individual women, taking into account the woman’s preferences and noting that these may change over time. Care for the newborn
- Guidance from the WHO African Region supports systematic newborn screening for SCD if available (23). Integration of newborn screening into existing primary health-care immunizations has been shown to be feasible in settings with limited resources (180). Point-of-care screening tests have been shown to provide an affordable, reliable, and easy-to-use method to screen for SCD, ensuring the earliest diagnosis possible, the highest level of follow-up of participants, access to treatments locally (including penicillin prophylaxis, pneumococcal vaccinations, and hydroxycarbamide) and effective prevention procedures regionally (e.g. transcranial doppler for risk of stroke) (180). Medications while breastfeeding
- Pain medications: Paracetamol and NSAIDs are considered safe during breastfeeding.
- Hydroxycarbamide: Breastfeeding has typically been avoided for women with SCD receiving hydroxycarbamide therapy, despite sparse pharmacokinetics data. In a recent study (n=16) hydroxycarbamide transferred into breastmilk with a relative infant dosage of 3.4%, which is below the recommended 5–10% safety threshold (181).
- Counselling for women on the safety of breastfeeding for the baby needs to include what is known about the transfer of hydroxycarbamide to the baby and the strength of the evidence. Such counselling aims to enable the woman to be a part of a shared decision-making process involving the woman and her health care providers. In addition to the woman herself, members of that decision-making group may include obstetricians, midwives, paediatricians and specialist SCD care providers).