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Fertility challenges for women with sickle cell disease

Djamila L Ghafuri1, Sarah-Jo Stimpson1, Melissa E Day1

  • 1a Department of Pediatrics, Division of Hematology/Oncology, Vanderbilt-Meharry-Matthew Walker Center for Excellence in Sickle Cell Disease , Vanderbilt University Medical Center , Nashville , TN , USA.

Expert Review of Hematology
|September 12, 2017
PubMed

Insights

Women with sickle cell disease (SCD) face unique reproductive health challenges, including infertility and premature menopause. Early consultation with fertility specialists is crucial for family planning and fertility preservation before treatments like HSCT.

Area of Science:

  • Reproductive Medicine
  • Hematology
  • Genetics

Background:

  • Sickle cell disease (SCD) is a common monogenic blood disorder affecting over 300,000 newborns annually.
  • Reproductive challenges in women with SCD are significant, impacting quality of life.
  • Evidence-based strategies for managing infertility and enhancing fecundity in women with SCD are lacking.

Purpose of the Study:

  • To review risk factors for infertility, low fecundity, and premature menopause in women with SCD.
  • To highlight unique pathophysiological mechanisms contributing to reproductive dysfunction in SCD.
  • To provide expert commentary on management and fertility preservation strategies.

Main Methods:

  • Literature review summarizing known risk factors and pathophysiological mechanisms.
  • Expert commentary on clinical management and recommendations.

Main Results:

  • Women with SCD experience infertility due to chronic inflammation, oxidative stress, hemochromatosis, and ovarian sickling.
  • Hydroxyurea therapy requires contraception and discontinuation for family planning due to teratogenicity.
  • Hematopoietic stem cell transplantation (HSCT) conditioning regimens can cause infertility and premature ovarian failure.

Conclusions:

  • Women with SCD have specific risk factors impacting fertility.
  • Contraception and careful management of hydroxyurea are essential.
  • Fertility preservation and surrogacy options should be discussed before HSCT or gene therapy, with referral to a reproductive endocrinologist recommended.
Abstract

Keywords:
Sickle cell diseaseinfertilitypremature ovarian failurepremature ovarian insufficiencysickle cell anemia

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