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Sickle cell disease and pregnancy.

Julie Carrara1, Anoosha Habibi2, Alexandra Benachi1

  • 1Service de Gynécologie-Obstétrique, Hôpital Antoine Béclère, AP-HP, Université Paris Saclay, 92140 Clamart, France.

Presse Medicale (Paris, France : 1983)
|November 8, 2023
PubMed
Summary

Pregnancy poses significant risks for sickle cell disease (SCD) patients, increasing complications for both mother and baby. Careful planning and multidisciplinary care are essential for managing SCD during pregnancy.

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Area of Science:

  • Hematology
  • Obstetrics
  • Genetics

Background:

  • Pregnancy presents a high-risk period for individuals with sickle cell disease (SCD), encompassing various hemoglobinopathies like Hb S/C and S/β-thalassemia.
  • Physiological adaptations during pregnancy exacerbate risks such as vaso-occlusive crises (VOC), acute chest syndrome, thromboembolic events, and infections.

Purpose of the Study:

  • To outline the heightened risks and necessary management strategies for pregnant patients with sickle cell disease.
  • To emphasize the importance of preconception counseling and multidisciplinary care for optimizing maternal and fetal outcomes.

Main Methods:

  • Review of existing literature and clinical guidelines concerning SCD management in pregnancy.
  • Discussion of preconception counseling, including genetic transmission risks and parental screening (hemoglobin electrophoresis).
  • Analysis of treatment modifications, including discontinuation of hydroxyurea (HU) and indications for prophylactic blood transfusions.

Main Results:

  • SCD significantly increases the likelihood of obstetric complications, including preeclampsia, intrauterine fetal death, preterm delivery, and intrauterine growth restriction.
  • Preventive strategies like blood transfusions are recommended based on specific criteria, not universally applied.
  • Systematic corticosteroid administration for fetal lung maturation is contraindicated due to maternal risks; delivery timing recommendations are provided.

Conclusions:

  • Pregnancy in SCD patients necessitates meticulous planning and close monitoring by a specialized multidisciplinary team.
  • Management involves preconception counseling, adjusting medications like hydroxyurea, and selective use of interventions like blood transfusions.
  • Postpartum surveillance is crucial due to persistent risks, particularly thromboembolism.