Fertility testing knowledge and attitudes in male adolescents and young adults with SCD and their caregivers: a pilot study

Abstract

Early diagnosis and effective use of disease-modifying and curative therapies have resulted in $93% of youth with sickle cell disease (SCD) living into adulthood with less disease morbidity. 1 Consequently, reproductive health (including fertility, contraception, and preimplantation genetic testing) is an emerging area of importance in this population. [2][3][4][5] Specifically in males with SCD, fertility can be diminished by many factors, including priapism, anemia, hypogonadism, and testicular infarction from the disease and/ or antineoplastic treatments, such as hydroxyurea. 2 Long-standing and recent research shows that semen abnormalities occur in males with SCD across the life course, including studies that show impaired semen parameters at baseline in adult males, which further decline after starting hydroxyurea 6 ; abnormal semen parameters in adolescents and young adults (AYAs) irrespective of hydroxyurea treatment 7 ; and reduced spermatogonial cell counts in prepubertal boys who received hydroxyurea. 8 National organizations (eg, the American Academy of Pediatrics) are urging providers to counsel at-risk SCD patients to optimize reproductive outcomes. 9 Experts also recommend fertility testing (ie, semen analysis) in male AYAs with SCD, 10 yet specific guidelines have not been developed. There are limited data on male AYAs with SCD and their caregivers' perspectives on providing semen samples for testing. This information is critical, as AYAs with SCD may face multiple barriers to obtaining testing and to fertility-related decision-making. AYAs with SCD frequently have poor access to health care 11-13 that may limit their opportunities to learn about and obtain testing, compromise their understanding and use of fertility information, and prevent them from getting testing that is not covered under insurance laws in many states. 14 These factors may be particularly impactful as AYAs become increasingly independent, develop life goals, and transition to adult care where they will make their own treatment decisions. 15 In this context, a small pilot study approved by Nationwide Children's Hospital's Institutional Review Board was conducted to examine knowledge and perspectives on fertility testing among male AYAs (14-21 years) with SCD and caregivers of the participating males at a large, midwestern pediatric academic medical center. English-speaking patients were approached during clinic visits and excluded if they had a preexisting developmental or cognitive deficit that would preclude their ability to independently complete electronic surveys or if they had previously undergone hematopoietic stem cell transplantation. After informed consent (assent, if ,18 years of age) was obtained, participants completed the Fertility Knowledge and Attitudes Questionnaire (FKAQ), a survey developed by the research team. The FKAQ was based on constructs from the Health Belief Model (eg, perceived risk of infertility and benefits and barriers to fertility testing) and items from prior fertility-related studies among m...

Parental perspective on the risk of infertility and fertility preservation options for children and adolescents with sickle cell disease considering hematopoietic stem cell transplantation

“…Nahata et al. report in a survey‐based study that while adolescent males had limited knowledge about the risk of infertility from SCD and related treatments, they expressed an interest in having biological children in the future 15 . Taken together these findings highlight the need to include adolescent males in age‐appropriate conversations about infertility and fertility preservation.…”

Family planning needs of young adults with sickle cell disease

“…The overrepresentation of young men with priapism in this sample raises the possibility of selection bias, but also that priapism is a protective factor for successfully transitioning to adult care. Delivering sex-specific education and care to boys and men with SCD is critical, and these long-standing issues are now well over 50-year old [30]; a recent study identifies ongoing needs and challenges integrating sexual and reproductive healthcare into pediatric SCD care [31,32]. Other nuances in providing this care need consideration.…”

Desire for biological parenthood and patient counseling on the risk of infertility among adolescents and adults with hemoglobinopathies

“…However, an insufficient level of patient education on fertility has been shown among a small number of evaluated patients with SCD. 6,35 With regard to potential fertility issues in patients with hemoglobinopathies, limited data exist on standard practice of fertility counseling and supporting patients in fulfilling their desire for a biological child. 6,36…”

“…Patient counseling on aspects of reproductive health in patients with hemoglobinopathies should include the (individual) risk of infertility, the purpose of fertility testing, and information on FP options. However, an insufficient level of patient education on fertility has been shown among a small number of evaluated patients with SCD 6,35 . With regard to potential fertility issues in patients with hemoglobinopathies, limited data exist on standard practice of fertility counseling and supporting patients in fulfilling their desire for a biological child 6,36 …”