Reproductive Health Assessment and Reports of Fertility Counseling in Pediatric and Adolescent Patients with Sickle Cell Disease After Hematopoietic Cell Transplantation

Sobenna A. George, Department of Pediatrics, Division of Endocrinology, Emory+ Children's Pediatric Institute, Atlanta, Georgia. Electronic address: sgeorg4@emory.edu.
Anirudh Veludhandi, Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Atlanta, Georgia.
Yijin Xiang, Department of Pediatrics, Pediatrics Biostatistics Core, Children's Healthcare of Atlanta, Emory University, Atlanta, Georgia.
Katie Liu, Department of Pediatrics, Pediatrics Biostatistics Core, Children's Healthcare of Atlanta, Emory University, Atlanta, Georgia.
Elizabeth Stenger, Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Atlanta, Georgia.
Staci D. Arnold, Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Atlanta, Georgia.
Akanksha Mehta, Department of Urology, Emory University School of Medicine, Atlanta, Georgia.
David A. Schirmer, Department of Gynecology and Obstetrics, Division of Reproductive Endocrinology & Infertility, Emory University School of Medicine, Atlanta, Georgia.
Jessica B. Spencer, Department of Gynecology and Obstetrics, Division of Reproductive Endocrinology & Infertility, Emory University School of Medicine, Atlanta, Georgia.
Gregory M. Guilcher, Department of Oncology and Pediatrics, Section of Oncology/Cellular Therapy, Alberta Children's Hospital, University of Calgary, Calgary, Alberta, Canada.
Monica Bhatia, Pediatric Stem Cell Transplant, Irving Medical Center, Columbia University, New York, New York.
Allistair Abraham, Center for Cancer and Immunology Research, Children's National Hospital, George Washington University School of Medicine and Health Sciences, Washington, District of Columbia.
Veronica Gomez-Lobo, Pediatric and Adolescent Gynecology, NICHD, Bethesda, Maryland.
Lakshmanan Krishnamurti, Section of Pediatric Hematology/Oncology/BMT, Yale School of Medicine, New Haven, Connecticut.
Lillian R. Meacham, Department of Pediatrics, Division of Endocrinology, Emory+ Children's Pediatric Institute, Atlanta, Georgia; Department of Pediatrics, Division of Hematology Oncology and BMT, Emory + Children's Pediatric Institute, Atlanta, Georgia.

Document Type

Journal Article

Publication Date

7-5-2024

Journal

Transplantation and cellular therapy

DOI

10.1016/j.jtct.2024.06.029

Keywords

Fertility counseling; Infertility risk; Ovarian damage; Sickle cell disease; Testicular damage; Transplant

Abstract

Conditioning regimens for hematopoietic cell transplant (HCT) in patients with sickle cell disease (SCD) place patients at risk for reproductive health issues. The purpose of this study was to assess reproductive health and reports of fertility counseling in patients with SCD who received a transplant. This was a secondary analysis of gonadal hormone production, future infertility risk assessment, and parent-proxy/patient reports of fertility counseling in SCD transplant recipients who are currently pubertal and were enrolled in the Atlanta sites of the Sickle Cell Transplant Evaluation of Long-term and Late Effects Registry (STELLAR) between May 2017 and October 2023. Clinical information was abstracted from medical records and reproductive health survey data from the STELLAR database. Descriptive statistics were reported as median (interquartile range [IQR]) or percentages. There were 20 females and 12 males in the study population. Females were median (IQR) 19.6 (9.4) years old and males 20.8 (11.4) years old at the time of the study. Transplants most commonly occurred in the decade 2010 to 2019 at 10.7 (4.8) years old for females and 11.1 (4.1) years old for males. Most participants received bone marrow stem cells (95.0% females, 100.0% males) from matched sibling donors (90.0% females, 100.0% males). Participants received one of seven HCT conditioning regimens with cyclophosphamide equivalent doses ranging from 3388 to 9706 mg/m. The majority of females (90.0%) had diminished ovarian reserve with low anti-Mullerian hormone levels, and 61.1% had premature ovarian insufficiency with two follicle-stimulating hormone levels (FSH) ≥40 mIU/mL post-HCT. All males had normal testosterone levels, but 63.6% had elevated FSH levels suggestive of impaired spermatogenesis post-HCT. Parent proxies (for patients <18 years old) and patients ≥18 years old completed surveys 9.0 years (5.2) and 7.9 years (9.3) since HCT in females and males respectively. Twenty-five percent of parent proxies and 45% of patients reported that they had not been informed by a healthcare provider of the risk of infertility post-transplant. There are high rates of gonadal dysfunction post-HCT, but many parent proxies and patients do not recall being told of the risk for future infertility. More effective methods of education are warranted to ensure SCD patients and their families clearly understand the risk for reproductive health issues post-HCT.

Department

Pediatrics

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