Desire for parenthood and reproductive health knowledge in adolescents and young adults with sickle cell disease and their caregivers

Background/objective Sickle cell disease (SCD) and hydroxyurea have implications for fertility and reproductive health. The goal of this study was to examine desire for parenthood and reproductive health knowledge among a cohort of adolescent and young adult (AYA) with SCD receiving hydroxyurea and their caregivers at a large pediatric academic center. Methods Patients with SCD were approached from September 2016 to July 2017 if they were: (1) 12–20 years old, (2) prescribed hydroxyurea for at least 6 months, (3) proficient in English, and (4) accompanied by a caregiver who was proficient in English and willing to participate.  Participants self-reported sociodemographic characteristics and completed surveys to assess their/their child's desire for parenthood and other life goals, and reproductive health knowledge. Results Eighteen patient–caregiver dyads completed the study (78.3% of those eligible); 61.1% indicated that they wanted to have future biological children.  Few participants reported receiving information about fertility (16.7% of AYA and 27.8% of caregivers) or birth control (11.1% of AYA and 22.2% of caregivers) from their/their child's health care provider, and the majority had received no information on these topics. Less than half of participants reported that SCD (22.2% of AYA and 50.0% of caregivers) or hydroxyurea (11.1% of AYA and 27.8% of parents) could potentially impair fertility. Conclusions Biological parenthood was important to this cohort yet fertility and reproductive health knowledge was low, suggesting that clinicians should prioritize conversations about infertility risk and birth control options with AYA with SCD on hydroxyurea and their caregivers.  More research is needed to identify optimal approaches to these discussions.