Knowledge of fertility and perception of fertility treatment among adults with sickle cell disease (KNOW FERTILITY)

IntroductionThis study assessed fertility knowledge in adults with sickle cell disease using the Cardiff Fertility Knowledge Scale and Fertility Treatment Perception Survey and compared knowledge scores in respondents with sickle cell disease to previously reported unaffected cohorts.MethodsThis cross-sectional study surveyed adults over age 18 with sickle cell disease at an adult sickle cell disease center using a 35-question survey addressing infertility risk factor knowledge and perceptions of fertility treatment. Analyses included summary statistics for continuous and categorical variables, univariate linear regression, and Mann-Whitney U tests for group comparisons of Fertility Knowledge Scale scores. Fertility Treatment Perception Survey scores were measured by medians of the two positive statements and four negative statements to generate separate positive and negative treatment belief scores. Statistical significance was set at p < 0.05 for all analyses.ResultsNinety-two respondents (71 female, 21 male) with median age of 32 years (IQR: 25.0, 42.5) completed the survey between October 2020-May 2021. Sixty-five percent of respondents reported taking sickle cell disease treatment and 18% reported refusing at least one sickle cell disease treatment due to fertility concerns. The mean Fertility Knowledge Score was 49% (SD: 5.2), lower than reported in an international cohort (57% vs. 49%, p = 0.001), and higher than in a cohort of reproductive-aged Black women in the USA (49% vs. 38%, p = 0.001). Less than 50% of respondents correctly identified common infertility risk factors including sexually transmitted infections, advanced age, and obesity. Mean positive fertility perception score was 3 (IQR 3, 4), and negative fertility perception score was 3.5 (IQR 3, 4). Factors associated with agreement with negative fertility perception statements included: trying to conceive, refusing sickle cell disease treatment, and undergoing fertility treatment.DiscussionOpportunities exist to improve knowledge of infertility risk factors among adults with sickle cell disease. This study raises the possibility that nearly one in five adults with sickle cell disease refuse SCD treatment or cure due to infertility concerns. Education about common infertility risks factors needs to be addressed alongside disease- and treatment- associated fertility risks

Knowledge insufficient: the management of haemoglobin SC disease.

Although haemoglobin SC (HbSC) accounts for 30% of sickle cell disease (SCD) in the United States and United Kingdom, evidence-based guidelines for genotype specific management are lacking. The unique pathology of HbSC disease is complex, characterized by erythrocyte dehydration, intracellular sickling and increased blood viscosity. The evaluation and treatment of patients with HbSC is largely inferred from studies of SCD consisting mostly of haemoglobin SS (HbSS) patients. These studies are underpowered to allow definitive conclusions about HbSC. We review the pathophysiology of HbSC disease, including known and potential differences between HbSS and HbSC, and highlight knowledge gaps in HbSC disease management. Clinical and translational research is needed to develop targeted treatments and to validate management recommendations for efficacy, safety and impact on quality of life for people with HbSC

Developmental disorders in children born to women with sickle cell disease: A report from the Boston Birth Cohort

Abstract Children exposed to maternal sickle cell disease (SCD) have many theoretical risks for developmental disorders, but little is known about long‐term outcomes for these children. We used the Boston Birth Cohort to compare developmental outcomes between children exposed to maternal SCD and matched, unexposed controls. Children with exposure to maternal SCD had increased risk of attention deficit hyperactivity disorder (OR 5.12, 95% CI 1.36–19.19, p = 0.02) and obesity (OR 2.74, 95% CI 1.10–6.87, p = 0.03). In utero and/or environmental exposures may help explain these findings. Further studies of outcomes of children born to women with SCD are needed

Marijuana use and health behaviors in a US clinic sample of patients with sickle cell disease.

IntroductionAs marijuana use becomes more common, it is essential clinicians understand the relationship between marijuana use and health behaviors.MethodsUsing a retrospective cohort of adolescents and adults with sickle cell disease (SCD) stratified into a young ( = 25 years), we conducted multiple linear regression examining relationship of marijuana use (independent variable) on each dependent variable (SCD self-management score and pain management).ResultsAmong young cohort, 16.9% used marijuana compared to 21.8% of older cohort. The younger cohort reporting marijuana use had lower mean self-care scores (β = -2.74;p = 0.009) and were more likely to have admissions to the hospital for pain (β = 0.87;p = 0.047) compared to non-users. In contrast, the older cohort reporting marijuana use had more days treating pain at home (β = 0.44;p = 0.035).ConclusionsOnly a minority of patients with SCD reported lifetime marijuana use. Among those reporting marijuana use, there were different associations with self-care and health-related behaviors by age. The older cohort who endorsed marijuana use reported more days of treating pain at home, although this did not translate into increased acute care visits for pain crisis. Among youth, endorsing marijuana use was associated with worse SCD self-care

Fertility after Curative Therapy for Sickle Cell Disease: A Comprehensive Review to Guide Care

Curative therapy for sickle cell disease (SCD) currently requires gonadotoxic conditioning that can impair future fertility. Fertility outcomes after curative therapy are likely affected by pre-transplant ovarian reserve or semen analysis parameters that may already be abnormal from SCD-related damage or hydroxyurea treatment. Outcomes are also likely affected by the conditioning regimen. Conditioning with myeloablative busulfan and cyclophosphamide causes serious gonadotoxicity particularly among post-pubertal females. Reduced-intensity and non-myeloablative conditioning may be acutely less gonadotoxic, but more short and long-term fertility outcome data after these approaches is needed. Fertility preservation including oocyte/embryo, ovarian tissue, sperm, and experimental testicular tissue cryopreservation should be offered to patients with SCD pursing curative therapy. Regardless of HSCT outcome, longitudinal post-HSCT fertility care is required