Maternal mortality among women with sickle-cell disease in France, 1996-2009

Eur J Obstet Gynecol Reprod Biol. 2015 Nov:194:183-8. doi: 10.1016/j.ejogrb.2015.09.016. Epub 2015 Sep 25.


Objective: To describe maternal mortality among women with sickle-cell disease in France.

Study design: Data from the national confidential enquiry into maternal deaths and from reference centres for sickle-cell disease were examined to identify women with this disease who died in France during 1996-2009. The maternal mortality ratio among women with sickle-cell disease was estimated and compared with the ratio in the general population. Characteristics of these women and their pregnancies and circumstances of their deaths were examined in detail.

Results: Fifteen maternal deaths occurred among an estimated 3300 live births to women with sickle-cell disease, for a maternal mortality ratio of 454 per 100000 live births (95% CI [254; 750]), versus 9.4/100000 in the general population. Ten women were homozygous (SS) for sickle-cell disease, and five were composite heterozygotes. The episode leading to death appeared in the antepartum period for seven women (47%). Two women died of septic shock during pregnancy, one at 6 weeks, the other at 24 weeks. The other 13 women (87%) died postpartum. Thirteen deaths were directly attributable to sickle-cell disease. The other two maternal deaths, both considered direct obstetric causes, were due to amniotic fluid embolism and septic shock after post-amniocentesis chorioamnionitis. The expert committee on maternal mortality judged seven of these 15 deaths (47%) to be avoidable.

Conclusion: Sickle-cell disease is responsible for a major excess risk of maternal death in France, due mainly to direct complications of the disease.

Keywords: Confidential enquiry into maternal deaths; Maternal mortality; Sickle-cell disease.

MeSH terms

  • Adolescent
  • Adult
  • Anemia, Sickle Cell / genetics
  • Anemia, Sickle Cell / mortality*
  • Cause of Death
  • Female
  • France / epidemiology
  • Heterozygote
  • Homozygote
  • Humans
  • Infant, Newborn
  • Live Birth / epidemiology
  • Maternal Mortality*
  • Medical Errors / mortality*
  • Postpartum Period
  • Pregnancy
  • Pregnancy Complications / mortality*
  • Prenatal Care
  • Stillbirth / epidemiology
  • Young Adult