Improving Quality of Life for Patients with Sickle Cell Disease

Sarah-Jo Stimpson, M.D., and Michael R. DeBaun, M.D., MPH

Vaso-Occlusive Pain Associated with Menstruation in Sickle Cell Disease

Over the last 40 years, a major change has occurred in the care for individuals with sickle cell disease (SCD) as the life expectancy has increased from the mid-40s to as high as 60 years old. The focus has shifted from decreasing early mortality in childhood and adolescence to improving quality of life for individuals with SCD in the United States.

Vaso-occlusive pain remains the hallmark clinical manifestation and leading cause for hospitalization for individuals affected by SCD. Women were found to have higher pain rates when compared to men, particularly during their reproductive years (ages 19-39 years old), in the Cooperative Study on Sickle Cell Disease. To date, this study was the largest, multicenter, longitudinal, natural history study on individuals with SCD. Based on this observation, we have elected to determine if there are strong associations between the onset of pain, either prior to or during their menstrual period. If true, this association could explain why women have higher rates of vaso-occlusive pain when compared to men.

The estimated prevalence and biological correlates for vaso-occlusive pain associated with menstruation (VOPAM) have not been elucidated in adolescent girls or adult women with SCD. Furthermore, single center studies suggesting that VOPAM is a clinically relevant and potentially modifiable occurrence were conducted prior to the widespread use of hydroxyurea and chronic blood transfusion therapy. We postulate that the VOPAM affects as high as one-third of all adolescent girls and adult women with SCD. However, the risk factors, biological basis, and optimal treatment for this poorly characterized syndrome are not well-defined. We and collaborating institutions have recently decided to seek a better understanding for the implications of VOPAM in adolescents and women. Our initial approach is to simply ask the question of whether pain is temporally associated with the onset of menses, and if so, how frequently? If we find preliminary evidence to support our hypothesis, then a more in depth study, with biological correlates will be pursued. 

As research continues to focus on improving the quality of life for individuals with SCD, areas of research that were previously ignored are now becoming higher priorities, such as pain associated with menstruation in adolescent girls and adult women with SCD. 

For more information about sickle cell disease, click here to view Research!America’s fact sheet. 

Sarah-Jo Stimpson, M.D., is a clinical fellow in pediatric hematology/oncology at Monroe Carell Jr. Children's Hospital at Vanderbilt University. Michael R. DeBaun, M.D., MPH, is a professor of Pediatrics and Medicine and Director of the Vanderbilt-Meharry Center for Excellence in Sickle Cell Disease at Vanderbilt University.

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If concerted, long-term investments in research are not made, America will lose an entire generation of young scientists.
Brenda Canine, PhD; McLaughlin Research Institute, Montana