Periods may trigger pain for many who have sickle cell disease

Pain related to sickle cell disease (SCD) increases during menstruation, as do emergency department (ED) visits and hospitalizations, for many women and girls—according to a new, nationwide study led by researchers at UC San Francisco. The study, published in JAMA Network Open, included 211 female patients from 13 SCD centers across 11 states.

Sixty-four percent of the patients reported having disease-related pain during menstruation, suggesting periods may be an important, overlooked trigger for disease-related pain. Among these patients, the pain was so severe that 44% had one to three ED visits and 19.1% had four or more ED visits, as well as higher rates of hospitalizations, over six months.

“Not asking women and girls with sickle cell disease about their periods as standard of care not only ignores the pain of many female patients, but, now, the research,” said pediatrician Neha Bhasin, MD, director of the Pediatric Comprehensive Sickle Cell Center at UCSF Benioff Children’s Hospital Oakland and senior author of the study.

Sickle cell disease is a genetic blood disorder that causes red blood cells to become rigid and misshapen, leading to blocked blood flow, severe pain, organ damage, and shortened life expectancy. While pain is a well-known feature of SCD, female patients experience higher rates of pain and hospitalization than male patients.

Female patients with SCD also commonly experience delayed puberty, pregnancy complications, early menopause, and abnormal uterine bleeding.

Gaps in reproductive health care

Female reproductive health treatments may help reduce bleeding and pain, yet more than 80% of the study patients were not using birth control medication. Further, nearly one in four patients said they had never heard of reproductive health treatments related to SCD.

While periods can cause severe pain in many women and girls with or without SCD, menstrual and female reproductive health histories are not a standard part of SCD care, said Bhasin. However, medical schools tend to emphasize the impact of SCD on male patients, such as how they can develop a condition called priapism that can lead to erectile dysfunction and death, she said.

“We designed the study to understand women and girls’ experiences better, and also to open up a dialogue between patients, hematologists, and reproductive health specialists about the serious pain these patients are facing,” said Bhasin.

The researchers developed a publicly accessible conversation guide on reproductive health for SCD patients and clinicians. They recommend that a menstrual history screening become a routine part of annual sickle cell care and that patients connect with reproductive health specialists—such as gynecologists, maternal-fetal medicine specialists, and genetic counselors.

The study patients came from 11 states across the country—including California, North Carolina, Michigan, Indiana, Connecticut, Louisiana, Tennessee, Pennsylvania, Delaware, Arizona, and Illinois.