Vaso-occlusive pain episodes in sickle cell disease and menstruation: Is there a common connection?

I. Advancements in the Field of Sickle Cell Disease

The field of sickle cell disease has undergone many medical advancements over the past 40 years, including early detection of the disease with newborn screening, improved management of vaso-occlusive events, such as acute pain episodes and acute chest syndrome, early identification of neurological complications, and widespread vaccination against life-threatening infections such as Streptococcus pneumoniae. Because of these breakthroughs, this previously designated “disease of childhood” has now become a chronic “disease of adulthood.”

II. Vaso-Occlusive Pain Associated with Periods in Adolescents and Women with Sickle Cell Disease

Strong clinical evidence indicates that adolescents and women with sickle cell disease have vaso-occlusive pain episodes immediately prior to or during their periods. As more children with sickle cell disease become adults, health care providers have shifted their focus to improve the care of the most common complication of sickle cell disease: vaso-occlusive pain. The largest natural history study on sickle cell disease demonstrated that women have more vaso-occlusive pain than men throughout their lifetime, which may be explained by differences in hormones over the course of a woman’s menstrual cycle. Additional studies have indicated that women who use contraception have lower rates of vaso-occlusive pain, possibly as a result of menstrual cycle regulation. However, the biological basis between vaso-occlusive pain and menstruation in sickle cell disease is unknown.

III. Hormones in the Menstrual Cycle and Nitric Oxide

Certain hormones are involved in different phases of the menstrual cycle. Estrogen is higher in the beginning of the menstrual cycle (called the follicular phase). Progesterone, a hormone that is involved in maintaining the uterine lining for implantation, is higher later in the menstrual cycle (the luteal phase). If no implantation takes place, progesterone levels decrease, causing the uterine lining to shed in the process menstruation.

IV. What We’ve Learned Thus Far

Vaso-occlusive pain associated with menstruation (VOPAM) demands more attention in the research field, as further knowledge about the frequency and biological basis for this association may lead to improved management of vaso-occlusive pain for women during their menstrual cycles. Preliminary evidence from our pediatric sickle cell clinic suggests that as many as 50% of adolescents experience VOPAM. We will continue to collect information about VOPAM in both adolescents and adults and plan to investigate its biological basis, with the ultimate goal of improving therapeutic modalities for VOPAM.

V. How Can You Help Us Understand Pain Associated with Periods

If you or someone you know has experienced vaso-occlusive pain around your period, then we invite you to participate in our online survey about VOPAM, which can be found at: Questionnaires about Sickle Cell Pain during your Menstrual Period.