Sickle Cell Disease in Social Security Disability Evaluations: Pain and Treatment Settings (2025)

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Sickle Cell Disease in Social Security Disability Evaluations Pain and Treatment Settings Paul A. Volberding, Jennifer I. Koop, and Carol Mason Spicer, Editors Committee on Sickle Cell Disease in Social Security Disability Evaluations Board on Health Care Services Health and Medicine Division Consensus Study Report PREPUBLICATION COPY—Uncorrected Proofs 

NATIONAL ACADEMIES PRESS 500 Fifth Street, NW Washington, DC 20001 This activity was supported by a contract between the National Academy of Sciences and the Social Security Administration. Any opinions, findings, conclusions, or recommendations expressed in this publication do not necessarily reflect the views of any organization or agency that provided support for the project. International Standard Book Number-13: 978-0-309-XXXXX-X Digital Object Identifier: https://doi.org/10.17226/29137 This publication is available from the National Academies Press, 500 Fifth Street, NW, Keck 360, Washington, DC 20001; (800) 624-6242; http://www.nap.edu. The manufacturer’s authorized representative in the European Union for product safety is Authorised Rep Compliance Ltd., Ground Floor, 71 Lower Baggot Street, Dublin D02 P593 Ireland; www.arccompliance.com. Copyright 2025 by the National Academy of Sciences. National Academies of Sciences, Engineering, and Medicine and National Academies Press and the graphical logos for each are all trademarks of the National Academy of Sciences. All rights reserved. Printed in the United States of America. Suggested citation: National Academies of Sciences, Engineering, and Medicine. 2025. Sickle cell disease in Social Security disability evaluations: Pain and treatment settings. Washington, DC: National Academies Press. https://doi. org/10.17226/29137. PREPUBLICATION COPY—Uncorrected Proofs 

The National Academy of Sciences was established in 1863 by an Act of Congress, signed by President Lincoln, as a private, nongovernmental institution to advise the nation on issues related to science and technology. Members are elected by their peers for outstanding contributions to research. Dr. Marcia McNutt is president. The National Academy of Engineering was established in 1964 under the charter of the National Academy of Sciences to bring the practices of engineering to advising the nation. Members are elected by their peers for extraordinary contributions to engineering. Dr. John L. Anderson is president. The National Academy of Medicine (formerly the Institute of Medicine) was established in 1970 under the charter of the National Academy of Sciences to advise the nation on medical and health issues. Members are elected by their peers for distinguished contributions to medicine and health. Dr. Victor J. Dzau is president. The three Academies work together as the National Academies of Sciences, Engineering, and Medicine to provide independent, objective analysis and advice to the nation and conduct other activities to solve complex problems and inform public policy decisions. The National Academies also encourage education and research, recognize outstanding contributions to knowledge, and increase public understanding in matters of science, engineering, and medicine. Learn more about the National Academies of Sciences, Engineering, and Medicine at www.nationalacademies.org. PREPUBLICATION COPY—Uncorrected Proofs 

Consensus Study Reports published by the National Academies of Sciences, Engineering, and Medicine document the evidence-based consensus on the study’s statement of task by an authoring committee of experts. Reports typically include findings, conclusions, and recommendations based on information gathered by the committee and the committee’s deliberations. Each report has been subjected to a rigorous and independent peer-review process and it represents the position of the National Academies on the statement of task. Proceedings published by the National Academies of Sciences, Engineering, and Medicine chronicle the presentations and discussions at a workshop, symposium, or other event convened by the National Academies. The statements and opinions contained in proceedings are those of the participants and are not endorsed by other participants, the planning committee, or the National Academies. Rapid Expert Consultations published by the National Academies of Sciences, Engineering, and Medicine are authored by subject-matter experts on narrowly focused topics that can be supported by a body of evidence. The discussions contained in rapid expert consultations are considered those of the authors and do not contain policy recommendations. Rapid expert consultations are reviewed by the institution before release. For information about other products and activities of the National Academies, please visit www.nationalacademies.org/about/whatwedo. PREPUBLICATION COPY—Uncorrected Proofs 

COMMITTEE ON SICKLE CELL DISEASE IN SOCIAL SECURITY DISABILITY EVALUATIONS PAUL A. VOLBERDING (Chair), University of California, San Francisco (emeritus) JENNIFER I. KOOP (Vice Chair), Medical College of Wisconsin and Children’s Wisconsin MARILYN S. BAFFOE-BONNIE, University of Pennsylvania CECELIA L. CALHOUN, Yale University School of Medicine and Smilow Cancer Hospital JEFFREY A. GLASSBERG, Mount Sinai Center for Sickle Cell Disease, Icahn School of Medicine at Mount Sinai CORETTA M. JENERETTE, University of California, San Francisco School of Nursing ASHLEY M. JENKINS, University of Rochester School of Medicine and Dentistry SHIRLEY A. JOHNSON, Virginia Commonwealth University PATRICIA L. KAVANAGH, Boston University Chobanian & Avedisian School of Medicine MARTHA O. KENNEY (through April 2025), Duke University School of Medicine JERLYM PORTER, St. Jude Children’s Research Hospital ALEXANDRA POWER-HAYS, Cincinnati Children’s Hospital Medical Center and University of Cincinnati TED WUN, University of California (UC) Davis Clinical and Translational Science Center and UC Davis Comprehensive Cancer Center Study Staff CAROL MASON SPICER, Study Director ELIZABETH FERRÉ, Research Associate ELIANA PIEROTTI, Senior Program Assistant JOSEPH GOODMAN, Senior Program Assistant SHARYL NASS, Senior Board Director, Board on Health Care Services Consultants JOE ALPER, Science Writer v PREPUBLICATION COPY—Uncorrected Proofs 

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Reviewers This Consensus Study Report was reviewed in draft form by indi- viduals chosen for their diverse perspectives and technical expertise. The purpose of this independent review is to provide candid and critical com- ments that will assist the National Academies of Sciences, Engineering, and Medicine in making each published report as sound as possible and to ensure that it meets the institutional standards for quality, objectivity, evidence, and responsiveness to the study charge. The review comments and draft manuscript remain confidential to protect the integrity of the deliberative process. We thank the following individuals for their review of this report: LAKIEA J. BAILEY, Sickle Cell Consortium GILDA A. BARABINO, Olin College of Engineering C. PATRICK CARROLL, Johns Hopkins University ROBERT M. CRONIN, Ohio State University PAYAL C. DESAI, Levine Cancer Institute, Atrium Health, and Wake Forest University MONICA L. HULBERT, Boston Children’s Hospital/Harvard Medical School JEAN L. RAPHAEL, Baylor College of Medicine CAROLYN ROWLEY, Cayenne Wellness Center and Children’s Foundation WALLY R. SMITH, Virginia Commonwealth University SHAREE TURPIN, Golisano Children’s Hospital DEBORA L. WAGNER, Cornell University vii PREPUBLICATION COPY—Uncorrected Proofs 

viii REVIEWERS R. GENTRY WILKERSON, University of Maryland DIANA J. WILKIE, University of Florida LARA ZADOR, Henry Ford Health Although the reviewers listed above provided many constructive com- ments and suggestions, they were not asked to endorse the conclusions or recommendations of this report nor did they see the final draft before its release. The review of this report was overseen by JUDITH GREEN MCKENZIE, University of Pennsylvania and DAN G. BLAZER, Duke University Medical Center. They were responsible for making certain that an independent examination of this report was carried out in accordance with the standards of the National Academies and that all review comments were carefully considered. Responsibility for the final content rests entirely with the authoring committee and the National Academies. PREPUBLICATION COPY—Uncorrected Proofs 

Acknowledgments The committee extends its sincere thanks to the many individuals who shared their time and expertise to support its work and inform its delibera- tions. The Social Security Administration (SSA) funded the study, and we thank Michael Goldstein, Vincent Nibali, Andrea Bento, and Megan Butson for their guidance and support. The committee also acknowledges repre- sentatives at SSA for verifying relevant technical content pertaining to the disability determination process for accuracy. The committee also benefited greatly from discussions with individuals, including those with lived experi- ence, who participated in the committee’s webinars: Amanda Brandow, Pat Carroll, Payal Desai, Ruchi Doshi, Omini Ewah, Titilope Fasipe, Caroline E. Freiermuth, Kelly Harris, Teanika Hoffman, Golda Houndoh, Mary Hulihan, Vesha Jamison, Eboni Lance, Tristan Lee, Lakeya McGill, Jean Leclerc Raphael, Carolyn Rowley, Christelle Salomon, Wally Smith, Daniel Sop, Sharee Turpin, Nikia K. Vaughan, Teonna Woolford, Lara Zador. The committee also thanks all the individuals who submitted written responses to the committee’s Call for Perspectives. Our appreciation goes to the reviewers for their invaluable feedback on an earlier draft of the report and to the monitor and coordinator who oversaw the report review. The committee acknowledges the many staff within the Health and Medicine Division who provided support in various ways to this project, including Carol Mason Spicer (study director), Elizabeth Ferré (research associate), Eliana Pierotti (senior program assistant), and Joseph Good- man (senior program assistant). The committee extends great thanks and appreciation to Sharyl Nass, senior director, Board on Health Care Services, ix PREPUBLICATION COPY—Uncorrected Proofs 

x ACKNOWLEDGMENTS who oversaw the project. Christopher Lao-Scott (senior librarian) per- formed literature searches for the committee; Greysi Patton (finance business partner), Julie Wiltshire (senior finance business partner), and Ron Brown (deputy director, HMD program finance) oversaw finances for the project, and the report review, production, and communications staff all provided valuable guidance to ensure the success of the final product. Joe Alper pro- vided superb writing and editorial assistance in preparing the report. PREPUBLICATION COPY—Uncorrected Proofs 

Contents ACRONYMS AND ABBREVIATIONS xv SUMMARY 1 Context for This Study, 1 Study Charge and Scope, 2 Study Approach, 4 The Committee’s Overarching Conclusions, 5 1 INTRODUCTION 11 Study Charge and Scope, 13 Terminology and Definitions, 15 Background and Basics of Sickle Cell Disease, 15 The Treatment Landscape of Sickle Cell Disease, 22 SSA Disability Evaluation Process, 26 SSA’s Current Listings for Sickle Cell Disease, 28 Study Approach, 31 Organization of the Report, 32 References, 32 2 PAIN IN SICKLE CELL DISEASE 37 Terminology, 37 Acute Pain Pathophysiology, 38 Chronic Pain Pathophysiology, 40 Experience of Pain, 41 Timing and “Phases” of Vaso-occlusive Crises, 42 xi PREPUBLICATION COPY—Uncorrected Proofs 

xii CONTENTS Quantifying the Burden of Pain, 44 Triggers, 45 Diagnosis, 46 Functional Impact, 48 Summary and Conclusions, 48 References, 51 3 PAIN MANAGEMENT IN SICKLE CELL DISEASE 55 The Overall State of Pain Management in Sickle Cell Disease, 56 Pain Mitigation in Sickle Cell Disease, 57 Pain Management in Sickle Cell Disease, 62 Summary and Conclusions, 71 References, 73 4 HEALTH CARE DELIVERY IN SICKLE CELL DISEASE: PATIENT CHOICES, CARE SETTINGS, AND HEALTH CARE PROVIDERS 83 Care Settings and Health Care Providers Involved in Care, 84 Deciding to Escalate Care, 94 Health Care Provider Decisions to Admit to the Hospital, 97 Factors Unique to Sickle Cell Disease in Making Decisions Related to Hospitalization, 97 Factors Affecting Length of Hospitalization, 98 Navigating Care Transitions and the Discharge Process, 99 Factors Contributing to Hospital Readmission, 100 Summary and Conclusions, 101 References, 103 5 OVERARCHING CONCLUSIONS 109 Sickle Cell Disease and Disability Determination, 109 Sickle Cell Disease, 110 Proxies for Severity of Sickle Cell Disease, 113 APPENDIXES A Public Meeting Agendas 115 B Biographical Sketches of Committee Members 121 PREPUBLICATION COPY—Uncorrected Proofs 

Boxes, Figures, and Table BOXES S-1 Statement of Task, 3 1-1 Statement of Task, 13 1-2 Definitions of Selected Terms, 15 4-1 Decision Tree: Managing Sickle Cell Pain at Home to Hospitalization, 95 FIGURES 1-1 The shape of normal and sickled red blood cells, 12 1-2 Models of care for adults with SCD, 25 1-3 Roadmap illustrating the SSA disability evaluation process for adults, 26 1-4 Roadmap illustrating the SSA disability evaluation process for children, 27 1-5 Major body systems and examples of childhood listings applying to common complications of sickle cell disease, 29 1-6 Major body systems and examples of adult listings applying to common complications of sickle cell disease, 30 xiii PREPUBLICATION COPY—Uncorrected Proofs 

xiv BOXES, FIGURES, AND TABLES 2-1 Overview of initiation and progression of vaso-occlusive crisis in sickle cell disease, 39 2-2 Two sequential painful crises with residual pain of variable severity during the time in between them, 44 4-1 Average number of emergency department visits that resulted in release after treatment by age for people living with SCD in two states, 87 4-2 Variation in emergency department visits for one person living with SCD, 87 4-3 Hospital admissions that began in the emergency department by age for people living with SCD in two states, 88 4-4 Average number of hospital admissions by age for people living with SCD in two states, 89 4-5 Average length of hospitalization by age for people living with SCD in two states, 90 4-6 Hospital admissions with a hospital readmission or emergency department visit within 30 days of discharge by age for people ­living with SCD in two states, 100 TABLE 4-1 Acute Sickle Cell Disease Care Settings, 91 PREPUBLICATION COPY—Uncorrected Proofs 

Acronyms and Abbreviations ADA Americans with Disabilities Act APP advanced practice provider ASH American Society of Hematology CBT cognitive-behavioral therapy CDC Centers for Disease Control and Prevention DDS Disability Determination Services FDA Food and Drug Administration HCT hematopoietic stem cell transplantation I-STRONG Integrative Strong Body and Mind Training NHLBI National Heart, Lung, and Blood Institute NSAID nonsteroidal anti-inflammatory drug OIH opioid-induced hyperalgesia PTSD post-traumatic stress disorder RBC red blood cell xv PREPUBLICATION COPY—Uncorrected Proofs 

xvi ACRONYMS AND ABBREVIATIONS SCD sickle cell disease SCDIC Sickle Cell Disease Implementation Consortium SCT sickle cell trait SDOH social determinants of health SGA substantial gainful activity SSA Social Security Administration SSDI Social Security Disability Insurance SSI Supplemental Security Income SWAY Sickle Cell World Assessment Survey TCD transcranial doppler imaging PREPUBLICATION COPY—Uncorrected Proofs