No crystal stair: supporting fertility care and the pursuit of pregnancy in women with sickle cell disease

Abstract

Growing recognition that the ovary is an end organ in sickle cell disease (SCD), advances in SCD treatment and cure, and innovations in assisted reproductive technologies invite progressive challenges in fertility care for women with SCD. The reproductive life span of women with SCD may be reduced because ovarian reserve declines more rapidly in people with SCD compared to unaffected people. Some young women have diminished ovarian reserve, a risk factor for infertility. Referrals for fertility preservation may be offered and anticipatory guidance about when to seek infertility care provided. For a subset of people with SCD, this information is also applicable when pursuing in vitro fertilization with preimplantation genetic testing to avoid implantation of an embryo with SCD. Here we explore the dimensions of SCD-related fertility care illustrated by the case of a 28-year-old woman with hemoglobin SS disease who initially presented for a hematology consultation for preconception counseling. This case highlights the complexity of preconception SCD management and care and the need to partner with patients to help align pregnancy hopes with SCD treatment and the many associated uncertainties.

Family planning needs of young adults with sickle cell disease

“…People with testicles are at risk for hypogonadism, priapism, and infertility [2–4]. People with ovaries are at risk for menstruation‐associated sickle cell pain and dyspareunia, have thrombophilic risks exacerbated by hyperestrogenemic states that complicate pregnancy and contraceptive choices, and they may also have infertility risk factors [5–8]. Moreover, pregnancy is high risk [9, 10], and, in some healthcare systems, pregnant people in pediatric care are automatically transitioned to adult care [11].…”