Sickle Cell Disease in Social Security Disability Evaluations: Pain and Treatment Settings (2025)
Chapter: Summary
Summary1
Sickle cell disease (SCD) is a group of inherited blood disorders that affects approximately 100,000 people in the United States. A gene mutation that causes the body to produce abnormal hemoglobin, the protein that transports oxygen in the human body, characterizes all forms of the disease. The abnormal hemoglobin causes red blood cells, which are normally disc-shaped and flexible, to become crescent- or “sickle”-shaped. SCD primarily affects people of African, Mediterranean, Middle Eastern, and South Asian descent. In the United States, more than 90 percent of individuals with SCD are non-Hispanic Black or African American, and an estimated 3 to 9 percent are Hispanic or Latino. The estimated life expectancy at birth for individuals with SCD in the United States is 52.6 years, more than 20 years shorter than the expected average, and quality-adjusted life expectancy, which factors in likely quality of life as people with SCD age, is more than 30 years shorter.
CONTEXT FOR THIS STUDY
SCD is a chronic, life-long condition that affects every organ system in the body. Symptoms and SCD-associated complications, which can vary from mild to severe, include anemia; acute and chronic pain; infections; pneumonia and acute chest syndrome; stroke; and kidney, liver, and heart disease. For example, an individual may experience an ischemic stroke at
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1 This summary does not include references. Citations to support the text and conclusions herein are provided in the main text.
age 3 years and require chronic transfusion therapy; another may have frequent acute pain episodes requiring emergency department visits (three or more times per year) before age 10 years, leading to chronic pain by adolescence; while up to 30 percent may only require an acute care visit once every 3 to 4 years in adulthood. This variability among individuals living with SCD adds to the complexity of the disease and its manifestations, progression, and response to treatment. The life of an individual with SCD is often complicated by frequent bouts of extreme pain and hospitalizations, fatigue, organ damage, and mental health concerns. The cumulative burden of all the SCD-related health effects experienced by a person living with SCD can significantly affect their quality of life, including the ability of many individuals with SCD to attend and participate fully in school and work on a regular basis.
It is in this context that the Social Security Administration (SSA) determines whether applicants with SCD qualify for benefits under one of SSA’s disability programs. As part of the disability determination process, SSA considers whether an applicant would qualify based on criteria specified in SSA’s Listing of Impairments, including those in the Hematological Disorders listings for children and adults.
STUDY CHARGE AND SCOPE
In September 2024, SSA requested the Health and Medicine Division of the National Academies of Sciences, Engineering, and Medicine to convene an ad hoc committee of relevant experts2 to review the latest published research and science and produce two reports addressing best practices and community experiences in the management and treatment of SCD. Per the Statement of Task (Box S-1), this report presents the committee’s findings and conclusions pertaining to objectives 3a (hospitalizations) and 3b (use of opioid3 pain medications in SCD). The current report also addresses objectives 2 (acute SCD [pain] crises) and 3c (interventions for SCD pain crises that are equivalent to parenteral opioid medication in terms of indicating the medical severity of the underlying acute crisis) in the Statement of Task.
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2 The committee included experts in pediatrics and internal medicine, including hematology, emergency medicine, pain management, psychology and neuropsychology, nursing, social work, health care case management, health disparities, and SSA disability policy.
3 Opioid (or narcotic) pain medication refers to a class of drugs that block pain signals from the brain by activating receptors on cells located in many areas of the brain, spinal cord, and other organs in the body. This report preferentially uses the term “opioid” rather than “narcotic,” which is used in the Statement of Task, to refer to this class of drugs because the term narcotic is less informative and is generally used in the context of law enforcement with negative connotations.
BOX S-1
Statement of Task
An ad hoc committee of the National Academies of Sciences, Engineering, and Medicine will conduct a study to review the latest published research and science and produce a report addressing best practices and community experiences in the management and treatment of sickle cell disease (SCD). The committee’s report will
- Describe the current health care landscape for the management and treatment of SCD and its symptoms for adults and children, including traditional and alternative therapies and home remedies, their prevalence, efficacy, and side-effects, and recent changes or challenges in the provision of such care;
- Describe the typical course of an acute sickle cell crisis for adults and children, including phases of the crisis and the typical symptoms, signs, complications, functional impact, and treatment throughout the duration of the crisis, and the expected range of time to return to baseline; and
- Answer the following questions based on published evidence (to the extent possible) and professional judgment (where published evidence is lacking):
- When and how often is hospitalization used in the treatment of SCD, what factors impact the decision for a patient to present to a hospital for treatment, what factors lead to hospital staff’s decision on whether to admit a patient, what factors determine the length of hospitalization, what factors contribute to hospital readmissions within 30 days of discharge, are any of the discussed factors unique to SCD or used in a different way when making decisions related to hospitalization for individuals with SCD compared to individuals with other impairments, and how have these considerations changed over the past 10 years?
- When and how often are parenteral narcotic pain medications used to manage SCD pain crises, what are indicators or contraindicators for their use, how have best practices regarding narcotic pain medication changed over the past 10 years, and what circumstances or factors might lead providers not to prescribe narcotic pain medication even when indicated by current best practices? What other pain medications might be administered in the context of a severe pain crisis and what, if anything, can the choice of alternative medications tell us about a patient’s symptom severity?
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- What treatments or interventions used for acute sickle cell crises are equivalent to parenteral narcotic medication in terms of medical severity of the underlying acute crisis (e.g., acute transfusion)?
- How and why are regular red blood cell (RBC) transfusions used differently in the treatment of SCD and beta thalassemia? What indications or thresholds, if any, would show that SCD requiring chronic RBC transfusions had an equivalent level of medical severity to beta thalassemia requiring chronic RBC transfusions?
- To what extent does end-organ damage in an individual with SCD contribute to the disease burden and overall severity of SCD, and how, if at all, does this vary by type of organ or organ-specific severity or diagnostic threshold? Might similar objective measurements of end-organ damage denote higher levels of severity or functional impact in an individual with SCD than in an individual without SCD?
The committee will prepare an interim report and a final report, both containing findings and conclusions but not recommendations. The interim report will present the committee’s findings and conclusions pertaining to objectives 3.a (hospitalizations) and 3.b (use of narcotic pain medications in SCD). The final report will present the committee’s findings and conclusions addressing all the objectives in the Statement of Task.
The second report will present the committee’s findings and conclusions addressing all the objectives in the Statement of Task, including end-organ damage, other chronic complications of SCD, and the overall burden of the disease. At SSA’s request, the two reports contain conclusions but no recommendations. Box S-1 contains the committee’s full Statement of Task.
STUDY APPROACH
The committee met three times to discuss the questions posed in the Statement of Task and held three public webinars to hear from invited speakers about topics related to the Statement of Task: Hospitalization and SCD, Pain Management and SCD, and Functional Outcomes in SCD. Each webinar comprised two panels: one panel of subject matter experts, including one living with SCD, and one of other individuals with lived experience
with SCD, including individuals living with the disease and caregivers. The committee also issued a Call for Perspectives to solicit information from individuals with SCD and caregivers on the following topics:
- Their experiences living with or caring for someone with SCD.
- Their experiences with acute pain/SCD crises and the typical course of a crisis for them.
- The ways in which they manage pain and other symptoms associated with SCD at home, including traditional and alternative therapies and home remedies, and how it has changed over time.
- If applicable, the factors that contribute to their decision to seek care in a hospital or other medical facility for treatment of SCD, including any barriers they might face in accessing care.
- Any current and past effects of SCD on their day-to-day physical and mental health functioning. For example, does living with SCD affect their ability to engage in school or full-time work?
- The overall burden of living with SCD and how it may have changed over time.
- If relevant, their experience applying for disability benefits for SCD through SSA.
- Any additional information or lived experience relevant to the Statement of Task that they wanted the committee to consider.
The committee discussed objectives 2, 3a, 3b, and 3c in the Statement of Task and reached consensus on conclusions based on published evidence, information gathered during the webinars and Call for Perspectives, and their own professional judgment where published evidence was lacking.
THE COMMITTEE’S OVERARCHING CONCLUSIONS
The committee formulated four overarching conclusions that pertain to SCD pain crises, pain management, and treatment settings and are relevant to SSA disability determinations.
Sickle Cell Disease and Disability Determination
SSA uses a multi-step process (five steps for adults, three steps for children) to determine whether an applicant meets the statutory definition of disability for adults or children. After SSA determines an applicant’s administrative eligibility and the presence of a medical impairment of sufficient duration and severity in steps 1 and 2 of this process, it assesses in step 3 whether the applicant’s impairment meets or medically equals the criteria listed for a condition in SSA’s Listing of Impairments, which is organized
by major body system and describes impairments that SSA considers to be sufficiently severe to qualify a person to receive disability benefits.4 If an adult is not found to qualify for disability benefits at this step, they are not denied; rather, the assessment moves on to step 4 and, if necessary, step 5. Step 4 assesses whether the applicant’s physical or mental residual functional capacity allows the person to perform past relevant work, and step 5 assesses whether the individual can perform other work in the national economy, taking into account vocational factors such as age, education, and work experience, including transferable skills.
The SSA listings for SCD include criteria specifying the number of times (at least six) within a 12-month period, with at least 30 days between crises, that a person with SCD must experience documented painful (vaso-occlusive) crises, each requiring parenteral (intravenous or intramuscular) opioid medication, or complications that require at least three hospitalizations lasting at least 48 hours each within a 12-month period and occurring at least 30 days apart.
Based on its review of the literature and its expert assessment, the committee reached the following conclusion:
Overarching Conclusion 1: There is an opportunity to improve accuracy in the determination of disability by considering the broad variability in sickle cell disease complications and approaches to both acute and chronic pain management in a variety of settings by different types of health care providers.
Sickle Cell Disease
Acute and chronic pain are hallmarks of SCD, contributing to morbidity, reduced quality of life, and disability among individuals living with the disease. There is growing recognition that most individuals with SCD suffer daily or persistent pain, as well as acute pain crises. In keeping with the published literature on SCD and diagnostic codes used in medical records available to SSA, the committee has chosen to use the phrases “pain crisis” to refer to acute or acute-on-chronic episodes of pain above baseline and “chronic pain” to refer to persistent pain that is experienced daily or on most days. Given that pain, in general, is an underlying source of impaired function and disability regardless of whether it is acute or chronic, the committee may also frequently just refer to “pain.” Pain is a subjective experience, the severity of which can only be measured through self-report. Everyone experiences and responds differently to both chronic pain and
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4 For children, SSA also assesses in step 3 whether the impairment functionally equals (i.e., the impairment results in functional limitations equivalent in severity to) the listings.
acute pain crises, and there is increased recognition of the individual variation in the timing, intensity, symptoms, quality, duration, location, and subsequent complications of pain in those with SCD. Indeed, the frequency and severity of pain crises can vary widely among individuals, influenced by their unique genetic, environmental, and psychosocial factors.
The pathogenesis of pain in individuals with SCD is complex and multifactorial. Acute and chronic pain have distinct pathophysiologies, which is important for determining appropriate pain management. People with chronic pain can experience exacerbations of their chronic pain as well as identify distinctly separate acute crises, which may prompt different approaches to pain management. Although the distinctions between acute, acute-on-chronic, and chronic pain help inform decisions about pain management, they are less relevant to the individuals experiencing pain. Ultimately, it is the individual’s experience of pain, regardless of type, and its effect on their ability to function that are important.
People living with SCD experience significant functional effects from pain. Typically, individuals with SCD try to manage their pain at home whenever possible, which means the frequency of pain individuals with SCD experience may be underreported in the literature. Acute pain crises have an effect on an individual’s family and social life, and chronic pain can have major effects on daily functioning, health care utilization, and behavioral health, with children often experiencing moderate to severe levels of functional disability, recurrent school absences, depression, fear, and poor quality of life from chronic pain. Many of these effects continue into adulthood and may worsen.
In some areas of the country, outpatient care settings, such as infusion centers or day hospitals, may provide an alternative to hospital emergency departments when individuals are no longer able to manage their pain at home. Many people with SCD seek care in emergency departments only as a last resort because of prior negative treatment experiences. Despite affecting people of all racial and ethnic backgrounds, in the United States SCD disproportionately affects populations of African ancestry. Black individuals in the United States, in general, experience prolonged wait times in the emergency department and are often undertreated for pain of any etiology.
People living with SCD may face greater barriers because of the frequency of their pain crises or acute-on-chronic pain episodes and because they possess knowledge of specific medications and dosages that have previously proven effective for them. Emergency department personnel are often unfamiliar with SCD and erroneously believe that acetaminophen, for example, is sufficient for treating SCD pain. While acetaminophen can be used to treat mild to moderate pain, it is insufficient for treatment of severe pain. They may also misinterpret individuals’ coping strategies, such as distraction techniques or sleep, as indications that their pain is less severe than reported
and individuals’ knowledge of and request for specific drugs and dosages, especially requests for a particular opioid and dose to be administered intravenously, as indicators of red-flag drug-seeking behavior. Despite the evidence, clinicians may expect an SCD crisis to involve some change in vital signs or laboratory test values and do not accept an individual’s report of pain as valid when they do not see objective measures.
Individuals living with SCD also face challenges accessing high-quality care because of geographical, structural, and provider-level barriers. Geographical barriers limit initial access to care through the limited number of comprehensive sickle cell centers, limited proximity to a specialist in rural areas, and high burdens of transportation costs. At the structural level, individuals report difficulty acquiring insurance coverage and identifying and finding covered providers and also report facing high out-of-pocket costs such as co-pays and for medications, all of which affect access to consistent high-quality care. Limited coverage of therapies, including transfusions and prescription drugs, often compounds these challenges. Even when individuals can overcome these barriers, some face clinicians who refuse to treat them for various reasons, including limited knowledge of the disease. At the provider level, individuals living with SCD face shortages in health care providers who specialize in SCD, with studies indicating that lack of provider knowledge is a significant barrier to care. Given the complex and disabling effects of SCD, it is necessary to have health care providers with comprehensive education on SCD for optimal patient outcomes.
Based on its review of the literature and its expert assessment, the committee reached the following conclusions:
Overarching Conclusion 2: There is growing recognition of the full spectrum of pain experienced daily by many individuals living with sickle cell disease, which can affect functioning and is most often managed at home or in a variety of outpatient care settings.
Overarching Conclusion 3: Unlike other disorders that may require emergency department visits or hospitalization, sickle cell disease (SCD) is stigmatized in ways that may affect an individual’s decision to seek care.
- SCD is an invisible disease (no outward signs) that is rare in the United States and for which there is a shortage of health care providers who understand the disease, are well trained in its management, and are willing to care for people who have it.
- Despite affecting people of all racial and ethnic backgrounds, in the United States SCD disproportionately affects populations of African ancestry, who are often undertreated for pain.
- Pain is a personal experience, the severity of which can only be measured through self-report.
- SCD is typically characterized by repeated pain episodes that require the use of opioids to manage, the pursuit of which could inaccurately be perceived as drug-seeking behavior.
Proxies for the Severity of Sickle Cell Disease
Acute pain crises have variable times of resolution, and the interval between discrete acute complications of SCD that may require emergency department visits or hospitalization is not predictable. In addition, multiple discrete events may occur within 30 days. For example, a new pain crisis, stroke, and acute chest syndrome are discrete events that could all occur within 30 days of one another, with each requiring separate emergency department visits or hospitalizations.
Because everyone experiences and responds differently to a pain crisis, treatment also varies among individuals; for example, the use of opioid pain management for similar levels of pain severity. Access to different types of medications, including non-opioid analgesics, and to nonpharmacologic interventions for managing pain varies among individuals, depending on distance from different types of facilities, insurance coverage, and availability of specialists in SCD. In addition, the choice to prescribe or receive opioids for SCD pain management varies by institution, provider, and the person with SCD. For these reasons, the types of medications or interventions used to treat an acute pain crisis in a person with SCD are too restrictive as proxies for the severity of the pain or the underlying disease process.5
Similarly, SCD treatment in emergency department settings and the number of times a person is hospitalized are too restrictive as measures of disease severity.6 Growing use of alternative models of care, including home, infusion centers, or day hospitals, has enabled similar levels of care outside of the hospital. However, access to different care settings varies among individuals, depending on distance from types of facilities, insurance coverage, and the availability of specialists in SCD across a broad range of disciplines. Thus, many factors other than the severity of an acute pain crisis affect an individual’s decision to seek care and the provider’s decision to recommend admission. In addition, whether a hospitalization lasts at least 48 hours and whether readmission occurs within 30 days are both determined by a variety of factors not specific to disease characteristics, including access to care, health care provider and individual preferences, and disease stigma.
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5 This sentence was changed after release of the report to clarify the limitations of current proxies.
6 This sentence was changed after release of the report to clarify the limitations of the current measures.
Based on its review of the literature and its expert assessment, the committee reached the following conclusion:
Overarching Conclusion 4: The frequency of sickle cell disease treatment encounters for acute complications, such as pain crises, in the emergency department and inpatient settings, including the type or amount of pain medication, the duration of treatment, and treatment location, and disposition, is too restrictive a measure of disease severity.7 Growing use of alternative models of care has enabled similar levels of care in outpatient or home settings.
- In other words, people living with sickle cell disease who have frequent emergency department visits, hospitalizations, and need for opioid pain management would continue to qualify for disability, as they currently do under the existing listings.
- However, many people who do not meet these criteria experience an equivalent level of disease severity and significant functional limitations that also would qualify them for disability.8
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7 This sentence was changed after release of the report to clarify the limitations of the current measure.
8 This bulleted text was added after release of the report to clarify the implications of the conclusion.
