Women with sickle cell disease often have unique instances and timelines in terms of reproductive development. At the 48th Annual Convention hosted by the Sickle Cell Disease Association of America (SCDAA), Lydia H. Pecker, MD, an assistant professor in the Division of Hematology at Johns Hopkins, gave a presentation on the unique reproductive challenges women with sickle cell disease (SCD) face and how they should go about navigating those challenges.
According to Dr. Pecker, a hematologist, women with SCD are at risk for infertility and their egg supply may decline sooner and steeper than other women. Menarche is expected to be delayed in girls with SCD but hydroxyurea and blood transfusions may reduce chances of delayed onset. Dr. Pecker notes that the delay in the onset of menarche is constitutional, not endocrine. While one-third of women with SCD may experience dysmenorrhea associated with SCD pain, NSAIDS may be helpful for painful periods. Moreover, menstrual suppression could also be an option for those with painful periods and Dr. Pecker recommends progesterone-only therapy as a first line of defense. Women with SCD are expected to have otherwise regular cycles, but they may be at risk for early menopause.
When it comes to contraception, progesterone is the preferred option for women with SCD. This includes pills, IUDs, the birth control implant implanon, and the depo-provera shot. According to Dr. Pecker, contraception containing estrogen should only be considered if the risks of pregnancy outweigh the risks of contraception. She explains that stroke, blood clot, obesity, or tobacco make estrogen completely contraindicated.
While reproductive life planning is not currently a part of standard care, Dr. Pecker advises that physicians properly counsel their female patients with SCD about issues like ideal age for parenthood, plans for contraception, sickle cell trait testing in partners, approaches to SCD treatment during pregnancy, and education around approaches to high risk pregnancies. Dr. Pecker also speaks of the need for preconception counseling and for it to include factors like
- Hip assessment
- Partner status and interpretation of test results
- Partner testing for sickle cell trait must be the hemoglobin electrophoresis test; sickledex is not the right test
- Treatment plan
- May include discontinuing hydroxyurea and starting chronic transfusions
- Assessing opioid and marijuana use and fetal risk
- Additional modifications may include ceasing the use of NSAIDS, chelators, and aspirin
- Artificial Reproductive Technologies
- Dr. Pecker does mention that the risk of SCD is stable with each pregnancy
- Postpartum care
- May include depression screening
- Reassessing vitamin deficiencies
It is important for physicians to initiate these important conversations about reproductive health with their patients to help clarify their choices. More research and comprehensive systems are needed so that women with SCD can better navigate the challenges of reproductive health and be better informed about treatment choices.