Common gynecological challenges in adolescents with sickle cell disease

ABSTRACT

Sickle cell anemia is one of the most common genetic blood disorders worldwide. Individuals with sickle cell disease (SCD) experience clinical manifestations such as chronic anemia, developmental delay, vaso-occlusive pain, acute chest syndrome, and neurological complications. Adolescent girls with SCD face unique gynecological challenges including delayed puberty marked by a later onset in menarche, vaso-occlusive pain associated with their menstrual cycle, and underdiagnosed abnormal uterine bleeding. This review focuses on these challenges with particular emphasis on delayed menarche and vaso-occlusive pain episodes associated with menstruation, in addition to the evaluation and initial management of heavy menstrual bleeding for adolescents with SCD. We highlight research opportunities in this neglected area to help enhance the comprehensive care model for this population.

KEYWORDS:

• Contraception
• menstrual cycle
• progesterone
• sickle cell anemia
• sickle cell disease
• vaso-occlusive pain
• menorrhagia
• iron deficiency anemia

Acknowledgments

The authors thank the members of the Vanderbilt-Meharry Center of Excellence in Sickle Cell Disease for their thoughtful and helpful comments in reviewing the manuscript.