WHO global guideline for the treatment of sickle cell disease in pregnancy

WHO global guideline for the treatment of sickle cell disease in pregnancy

The World Health Organization (WHO) today released its first global guidelines for the management of sickle cell disease (SCD) during pregnancy, addressing a critical and growing health challenge that can have life-threatening consequences for both women and babies.

SCD is a group of inherited blood disorders characterized by abnormally shaped red blood cells that resemble half-cases or sickles. These cells can block blood flow, leading to severe anemia, episodes of severe pain, recurrent infections, and medical emergencies such as strokes, sepsis, or organ failure.

Health risks associated with SCD are exacerbated during pregnancy due to increased demands on the body's oxygen and nutrient supply. Women with SCD are 4 to 11 times more likely to experience maternal death than those without. They are more likely to experience birth complications such as preeclampsia, while their babies have a higher risk of stillbirth or being born early or small.

With quality health, women with inherited blood disorders such as sickle cell disease can have safe and healthy pregnancies and births. This new policy aims to improve pregnancy outcomes for those affected. With sickle cell on the rise, further investment is urgently needed to expand access to evidence-based treatments during pregnancy, as well as diagnosis and information about this neglected disease. “

Dr. Pascale Altey, Director of Sexual and Reproductive Health and Research at WHO and the United Nations Special Program on Human Reproduction (HRP)

Around 7.7 million people worldwide live with SCD - a number that has increased by over 40% since 2000. SCD is estimated to cause over 375,000 deaths each year. The disease is most common in malaria-endemic regions, particularly sub-Saharan Africa - accounting for around 8 in 10 cases - as well as parts of the Middle East, the Caribbean and South Asia. With population movements and improvements in life expectancy, the sickle cell gene is also becoming more widespread worldwide, meaning more maternity providers need to know how to treat the disease.

To date, clinical guidelines for the treatment of SCD in pregnancy have largely relied on protocols from high-income countries. The new WHO guideline aims to provide evidence-based recommendations that are also relevant to low- and middle-income settings, where most cases and deaths from the disease occur. Accordingly, the directive contains over 20 recommendations that exceed:

• Folsäure- und Eisenergänzung, einschließlich Anpassungen für Malaria-Endemien-Gebiete;
• Management von Sichelzellenkrisen und Schmerzlinderung;
• Prävention von Infektionen und Blutgerinnseln;
• Verwendung prophylaktischer Bluttransfusionen; Und
• Zusätzliche Überwachung der Frau und der Gesundheit des Babys während der gesamten Schwangerschaft.

Critically, the guideline highlights the need for respectful, individualized care that is tailored to women's specific needs, medical histories and preferences. It also addresses the importance of combating stigma and discrimination within the healthcare system, which can be a major challenge for people with SCD in several countries around the world.

"It is important that women with sickle cell disease discuss their care options early in pregnancy - or ideally before with knowledgeable providers," said Dr. Doris Chou, medical officer and lead author of the guideline. “This supports informed decisions about treatment options to continue or adopt and to agree on ways to manage potential complications to optimize outcomes for the woman, her pregnancy and her baby.”

Given the complexity of these disorders, the guideline notes the importance of including qualified and knowledgeable staff on your care team. These include specialists such as hematologists, as well as midwives, pediatricians and obstetricians-gynecologists who provide reproductive and newborn health services.

SCD is a neglected health condition that remains significantly underfunded and understudied despite its growing prevalence worldwide. As treatment options for the general population improve, the guidance highlights the urgent need for more research into the safety and effectiveness of SCD treatments for pregnant and breastfeeding women - populations that have historically been excluded from clinical trials.

This publication is the first in a new WHO series on the management of non-communicable diseases in pregnancy. Future guidelines will address cardiovascular disease, diabetes, respiratory disease, mental illness and substance use. Chronic diseases are increasingly recognized as major contributors to maternal and newborn death and illness.

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