Consensus Study Report

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This activity was supported by Contract No. 28321323D00060012 between the National Academy of Sciences and the U.S. 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.

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Suggested citation: National Academies of Sciences, Engineering, and Medicine. 2026. Sickle cell disease in Social Security disability evaluations. Washington, DC: The National Academies Press. https://doi.org/10.17226/29319.

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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 and Alnylam Pharmaceuticals

MARTHA O. KENNEY (through April 2025), Duke University School of Medicine

JERLYM S. 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 Division of Hematology and Oncology, UC Davis School of Medicine

Study Staff

CAROL MASON SPICER, Study Director

ELIZABETH FERRÉ, Research Associate

LYLE CARRERA (since May 2025), Research Associate

ELIANA PIEROTTI, Senior Program Assistant

JOSEPH GOODMAN, Senior Program Assistant

SHARYL NASS, Senior Board Director, Board on Health Care Services

Consultant

JOE ALPER, Science Writer

Reviewers

This Consensus Study Report was reviewed in draft form by individuals chosen for their diverse perspectives and technical expertise. The purpose of this independent review is to provide candid and critical comments 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:

Although the reviewers listed above provided many constructive comments and suggestions, they were not asked to endorse the conclusions 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.

Preface

Sickle cell disease (SCD) is a complex medical condition that affects the individual with SCD from birth through adulthood. SCD is primarily a hematological disease in which atypical sickle-shaped red blood cells carry less oxygen than normal to organ systems throughout the body. Although SCD is inherently a chronic condition, the course of the disease is marked by unpredictable acute exacerbations or crises because of the “stickiness” of sickle-shaped red blood cells and the resultant clotting. These chronic and acute aspects of the disease can affect any or all body systems at some point in the disease course.

This unpredictable course affects not only the treatment of the disease but also the treatment experiences and needs of each patient. There are also unique relevant social determinants with SCD, as it is a disease that primarily affects Black Americans or those of African descent. As a result, it is always important to consider barriers to access to health care and disparities in health care, as well as the impact of various social drivers of health. The unpredictable course and system dynamics further confound the effect of SCD on individuals’ mental, behavioral, and cognitive status in addition to physical status, with varying functional implications.

The understanding of the full impact of and standard of care for SCD has changed significantly over the course of the last decade. Given this changing treatment landscape and the increased recognition of the individual disease course with fluctuating functional effect, the Social Security Administration (SSA) contracted the National Academies of Sciences, Engineering, and Medicine to convene an ad hoc committee to examine the recent literature on and clinical knowledge of SCD to inform the SSA disability evaluation criteria specific to SCD.

The committee’s information gathering included an extensive review of published literature as well as three public webinars. It also included a Call for Perspectives during which additional experts shared their knowledge and caregivers and individuals with SCD shared their lived experiences.

The committee extends its sincere thanks to the many individuals, particularly those living with SCD and their caregivers, who shared their time, experience, and expertise to support its work and inform its deliberations. We thank representatives from SSA for their guidance and support; in particular, we thank Michael Goldstein, Vincent Nibali, Andrea Bento, and Megan Butson. The committee also acknowledges representatives at SSA for verifying relevant technical content pertaining to the disability determination process for accuracy. The committee benefited greatly from discussions with individuals, including those with lived experience, 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, and 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), Lyle Carrera (research associate), Eliana Pierotti (senior program assistant), and Joe Goodman (senior program assistant). The committee extends great thanks and appreciation to Sharyl Nass, senior director, Board on Health Care Services, who oversaw the project. Christopher Lao-Scott (senior librarian) performed literature searches for the committee; Greysi Patton (finance business partner), Wahidullah Nazari (finance business partner), Julie Wiltshire (senior finance business partner), and Ron Brown (deputy director, Health and Medicine Division 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 provided superb writing and editorial assistance in preparing the report. Thanks also go to Mark Goodin and Robert Pool for copyediting the report.

Jennifer I. Koop, Vice Chair
Paul A. Volberding, Chair
Committee on Sickle Cell Disease in Social Security Disability Evaluations
July 2025

Contents

ACRONYMS AND ABBREVIATIONS

SUMMARY

Context for This Study

Study Charge and Scope

Study Approach

The Committee’s Overarching Conclusions

1 INTRODUCTION

Study Charge and Scope

Terminology and Definitions

Background and Basics of Sickle Cell Disease

The Treatment Landscape of Sickle Cell Disease

Social Security Administration Disability Evaluation Process

Social Security Administration’s Current Listings for Sickle Cell Disease

Study Approach

Organization of the Report

References

2 PAIN IN SICKLE CELL DISEASE

Terminology

Acute Pain Pathophysiology

Chronic Pain Pathophysiology

Experience of Pain

Timing and “Phases” of Vaso-Occlusive Crises

Quantifying the Burden of Pain

Triggers

Diagnosis

Functional Impact

Summary and Conclusions

References

3 PAIN MANAGEMENT IN SICKLE CELL DISEASE

The Overall State of Pain Management in Sickle Cell Disease

Pain Mitigation in Sickle Cell Disease

Pain Management in Sickle Cell Disease

Summary and Conclusions

References

4 HEALTH CARE DELIVERY IN SICKLE CELL DISEASE: PATIENT CHOICES, CARE SETTINGS, AND HEALTH CARE PROVIDERS

Care Settings and Health Care Providers Involved in Care

Deciding to Escalate Care

Health Care Provider Decisions to Admit to the Hospital

Factors Unique to Sickle Cell Disease in Making Decisions Related to Hospitalization

Factors Affecting Length of Hospitalization

Navigating Care Transitions and the Discharge Process

Factors Contributing to Hospital Readmission

Summary and Conclusions

References

5 EFFECTS OF SICKLE CELL DISEASE ON THE CENTRAL NERVOUS SYSTEM

Stroke and Silent Cerebral Infarct

Neurocognitive Disorders

Depression, Anxiety, and Trauma

Summary and Conclusions

References

6 EFFECTS OF SICKLE CELL DISEASE ON BODY SYSTEMS OTHER THAN THE CENTRAL NERVOUS SYSTEM

Special Senses

Cardiovascular Complications

Respiratory Complications

Genitourinary Complications

Musculoskeletal Complications

Digestive and Other Abdominal Complications

Skin Disorders

Growth Faltering

Chronic Transfusion in Sickle Cell Disease

Summary and Conclusions

References

7 CUMULATIVE BURDEN OF LIVING WITH SICKLE CELL DISEASE

Quality of Life and Cumulative Burden of Living with Sickle Cell Disease

Challenges in Access to Care for Sickle Cell Disease

Challenges of Caregiving in Sickle Cell Disease

Effects on School and Work

Summary and Conclusions

References

8 SICKLE CELL DISEASE AND DISABILITY EVALUATIONS

Sickle Cell Disease and Disability

Lived Experience of Applying for SSA Disability

Social Security Listings Specific to Sickle Cell Disease

Considerations Pertaining to the Cumulative Burden of Sickle Cell Disease

Summary and Conclusions

References

9 OVERARCHING CONCLUSIONS

Sickle Cell Disease and Disability Determination

Sickle Cell Disease

Proxies for the Severity of Sickle Cell Disease

Access to Coordinated Care for Sickle Cell Disease

Sickle Cell Disease and Disability Evaluation

APPENDIXES

A Public Meeting Agendas

B Draft Medical Report Forms for Children and Adults with Sickle Cell Disease

C Biographical Sketches of Committee Members

Boxes, Figures, and Tables

BOXES

S-1 Statement of Task

1-1 Statement of Task

1-2 Definitions of Selected Terms

4-1 Decision Tree: Managing Sickle Cell Pain at Home to Hospitalization

8-1 Selected SSA Listings Specific to SCD

FIGURES

1-1 The shape of normal and sickled red blood cells

1-2 Models of care for adults with SCD

1-3 Roadmap illustrating the SSA disability evaluation process for adults

1-4 Roadmap illustrating the SSA disability evaluation process for children

1-5 Major body systems and examples of childhood listings applying to common complications of SCD

1-6 Major body systems and examples of adult listings applying to common complications of SCD

2-1 Overview of initiation and progression of vaso-occlusive crisis in SCD

2-2 Two sequential painful crises with residual pain of variable severity during the time between them

4-1 Average number of emergency department visits that resulted in release after treatment by age for people living with SCD in two states

4-2 Variation in emergency department visits for one person living with SCD

4-3 Hospital admissions that began in the emergency department by age for people living with SCD in two states

4-4 Average number of hospital admissions by age for people living with SCD in two states

4-5 Average length of hospitalization by age for people living with SCD in two states

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

TABLES

4-1 Acute SCD Care Settings

7-1 Selected Patient-Reported Outcome Measures and Functional Status Questionnaires in SCD

ANNEX TABLES

5-1 Selected Central Nervous System Conditions Associated with SCD

5-2 SSA’s Functional Equivalence Domains for Children

5-3 Physical Activities; Vision, Hearing, and Speaking Activities; and Mental Activities

6-1 Selected Special Senses and Speech Conditions Associated with SCD

6-2 Selected Cardiovascular Conditions Associated with SCD

6-3 Selected Respiratory Conditions Associated with SCD

6-4 Selected Genitourinary Conditions Associated with SCD

6-5 Selected Musculoskeletal Conditions Associated with SCD

6-6 Selected Digestive and Other Abdominal Conditions Associated with SCD

6-7 Selected Skin Condition Associated with SCD

Acronyms and Abbreviations