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Management of Older Adults with Sickle Cell Disease: Considerations for Current and Emerging Therapies

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Abstract

People with sickle cell disease (SCD) are living longer than ever before, with the median survival increasing from age 14 years in 1973, beyond age 40 years in the 1990s, and as high as 61 years in recent cohorts from academic centers. Improvements in survival have been attributed to initiatives, such as newborn screening, penicillin prophylaxis, vaccination against encapsulated organisms, better detection and treatment of splenic sequestration, and improved transfusion support. There are an estimated 100,000 people living with SCD in the United States and millions of people with SCD globally. Given that the number of older adults with SCD will likely continue to increase as survival improves, better evidence on how to manage this population is needed. When managing older adults with SCD (defined herein as age ≥ 40 years), healthcare providers should consider the potential pitfalls of extrapolating evidence from existing studies on current and emerging therapies that have typically been conducted with participants at mean ages far below 40 years. Older adults with SCD have historically had little to no representation in clinical trials; therefore, more guidance is needed on how to use current and emerging therapies in this population. This article summarizes the available evidence for managing older adults with SCD and discusses potential challenges to using approved and emerging drugs in this population.

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Acknowledgements

The authors would like to acknowledge the contribution of people with SCD who participated in the clinical studies included in this review article and the investigators who led these studies.

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Authors and Affiliations

  1. Division of Hematology, Department of Medicine, Duke University School of Medicine, Durham, NC, USA

    Charity I. Oyedeji, Kimberly L. Hodulik, Marilyn J. Telen & John J. Strouse

  2. Duke Claude D. Pepper Older Americans Independence Center, Durham, NC, USA

    Charity I. Oyedeji & John J. Strouse

  3. Department of Medicine, and Duke Comprehensive Sickle Cell Center, Duke University School of Medicine, 315 Trent Dr., Suite 266, DUMC Box 3939, Durham, NC, 27710, USA

    Charity I. Oyedeji & John J. Strouse

  4. Department of Pharmacy, Duke University Hospital, Durham, NC, USA

    Kimberly L. Hodulik

  5. Division of Pediatric Hematology-Oncology, Duke University, Durham, NC, USA

    John J. Strouse

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  1. Charity I. Oyedeji
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Correspondence to Charity I. Oyedeji.

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Funding

This study was funded by the Duke Center for Research to Advance Healthcare Equity (REACH Equity), which is supported by the National Institute on Minority Health and Health Disparities under award number U54MD012530 and the National Institute on Aging (NIA) Grants for Early Medical/Surgical Specialists' Transition to Aging Research (GEMSSTAR) award number 1R03AG074054-01 to CIO in the preparation of this manuscript. JJS received support from the Health Resources and Services Administration (HRSA) for the Education and Mentoring to Bring Access to Comprehensive Care (EMBRACE) Network (U1EMC42461-01) for the preparation of this manuscript.

Conflict of interest

Charity I. Oyedeji has received research funds from the NIA (National Institutes of Health [NIH]). Kimberly L. Hodulik declares that she has no conflicts of interest. Marilyn J. Telen has received research funds from the Doris Duke Charitable Foundation, National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) and the NHLBI (NIH), FDA, Forma Therapeutics, and CSL Behring, and has also served on an Advisory Board for Pfizer, Inc. and a Data Monitoring Committee for Novartis. John J. Strouse receives research funds from the NHLBI, Centers for Disease Control and Prevention (CDC), Takeda, and Agios, and has served on a scientific advisory board on gene therapy for sickle cell disease for Aruvant.

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Oyedeji, C.I., Hodulik, K.L., Telen, M.J. et al. Management of Older Adults with Sickle Cell Disease: Considerations for Current and Emerging Therapies. Drugs Aging 40, 317–334 (2023). https://doi.org/10.1007/s40266-023-01014-8

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