Sickle Cell Disease in Social Security Disability Evaluations (2025)
Chapter: 9 Overarching Conclusions
9
Overarching Conclusions1
This chapter presents seven overarching conclusions derived by the committee from evidence presented throughout the report and from its expert assessment. Each section provides a narrative summary of evidence supporting these overarching conclusions. The narratives incorporate the chapter-specific conclusions at the end of Chapters 2 through 8.
SICKLE CELL DISEASE AND DISABILITY DETERMINATION
As described in Chapter 1, the Social Security Administration (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.2 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.
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1 This chapter does not include references. Citations to support the text and conclusions herein are provided in previous chapters of the report.
2 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.
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 sickle cell disease (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
As described in Chapter 2, 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 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.
As Chapter 4 discussed, 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 copays 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.
Similarly, SCD treatment in emergency department settings and the number of times a person is hospitalized are too restrictive as measures of disease severity. 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.
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 (SCD) 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. Growing use of alternative models of care has enabled similar levels of care in outpatient or home settings.
- In other words, people living with SCD 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.
ACCESS TO COORDINATED CARE FOR SICKLE CELL DISEASE
In a coordinated care model, a person’s health care is organized across a range of different health care providers, but the health care paradigm for SCD, which parallels the larger United States model of care, has historically been fragmented, including limitations in care integration among providers (primary care and specialists) and care settings (outpatient and inpatient). Access to coordinated comprehensive SCD care across the lifespan can lead to better health outcomes, but most individuals living with SCD, especially adults, do not have access to such coordinated care. In addition, shortages of health care providers who specialize in SCD add to the challenges individuals living with SCD face in accessing high-quality care. Given the multisystem health effects of SCD, individuals often require specialized care from a variety of health care providers across a broad range of disciplines. Challenges in accessing appropriate SCD care may increase the burden of the disease process. For example, constrained access to specialists limits the diagnosing of central nervous system complications of SCD, including silent stroke, neurocognitive disorders, and mental or behavioral health disorders.
The transition from childhood to adulthood is particularly complex for individuals with SCD because they often must navigate the transfer of multiple services and supports for their condition in addition to dealing with the typical changes faced by anyone transitioning from childhood to adulthood. The transition from pediatric to adult health care for individuals with SCD is a complex process with high stakes for long-term health outcomes. Adolescents and young adults who are better supported during the transition process may have better outcomes, which may have longitudinal ramifications for the risk of functional impairments over time. Nevertheless, access to care for adults with SCD remains lower than that for other diseases. Despite efforts to introduce new ways of treating adults living with SCD, children with SCD generally receive a higher quality of care because of increased access to comprehensive sickle cell care compared to adults living with SCD. Unaddressed needs, including during transitions, can exacerbate and accelerate complications, leading to disability.
The transition period has implications for SSA disability as well. Children receiving SSA disability benefits may need to be re-evaluated at age 18 based on adult criteria. People with SCD as well as providers have challenges navigating the differences between child-based and adult-based SSA criteria.
Based on its review of the literature and its expert assessment, the committee reached the following conclusions:
Overarching Conclusion 5: Access to coordinated comprehensive sickle cell disease (SCD) care across the life course can be expected to improve outcomes for people with the disorder and provide documented evidence of the multisystem effects of the disease.
- Optimal care requires coordinated, comprehensive, multispecialty care across the lifespan, but most individuals with SCD, especially adults, do not have access to such coordinated care.
- Lack of access to coordinated care makes it more difficult for patients to obtain accurate diagnosis and treatment of their SCD-related disorders as well as documentation of those disorders in their medical records.
Overarching Conclusion 6: The transition from adolescence to adulthood is particularly challenging for adolescents and young adults living with sickle cell disease (SCD).
- The change in the Social Security Administration’s disability determination process during the transition from adolescence to adulthood introduces challenges for determining disability and sustaining benefits across the 18-year-old threshold.
- Complexities in the transition from pediatric to adult SCD care and follow-up may lead to disengagement from care, which can result in more severe adverse outcomes.
- Attrition at the point of transfer from pediatric to adult care cannot be explained solely by issues of access. Special support is necessary to keep children with SCD engaged in the health care system into and through adulthood.
SICKLE CELL DISEASE AND DISABILITY EVALUATION
SCD is a complex and progressive disorder that potentially affects multiple organ systems over the lifespan. Although some people with SCD do not experience complications, most do and may be expected to have them in combination, including associated neurocognitive and mental–behavioral
disorders. These complications may manifest as disabling functional impairments, often affecting a person’s ability to participate in school or work.
Individuals with SCD may qualify for SSA disability benefits under SSA listing criteria not specific to SCD, often in the same way that other people without SCD would qualify. However, there are unique considerations in applying the listing criteria for some conditions, including cardiac and renal, to people with SCD. Individuals with such conditions may experience the same functional limitation as someone without SCD but not meet some part of the criteria because of a consideration specific to SCD. For example, ventricular hypertrophy may not be present in individuals with SCD who experience heart failure with preserved ejection fraction as it is in individuals without SCD. Applicants with SCD and cardiomyopathy therefore may not meet all the criteria for cardiomyopathy under SSA Listing 4.02, which states that patients must have evidence of ventricular hypertrophy, despite their otherwise experiencing the same limitations as someone without SCD who does meet all the criteria. In addition, a person with SCD may have a related health condition that fails to meet all the listing-level criteria for the specific condition, but the additive effect of the condition plus SCD or the cumulative effect of multiple conditions may severely affect that person’s overall functioning. Because of SCD’s chronic and multisystem nature, focusing on a single set of symptoms or on the effects on a single body system is unlikely to capture the full burden or severity of disease experienced by individuals living with SCD, including effects on their day-to-day functioning.
Considering the cumulative burden of SCD across all body systems is critical, especially when none of the effects on individual body systems is sufficient to meet or medically equal any of SSA’s current listing criteria for those conditions. In fact, one of the adult listings for SCD, Listing 7.18, recognizes “repeated complications of [SCD] causing significant documented symptoms or signs (for example, pain, severe fatigue, malaise, fever, night sweats, headaches, joint or muscle swelling, or shortness of breath), AND marked limitations in one area of functioning (activities of daily living, maintaining social functioning, or completing tasks in a timely manner because of deficiencies in concentration, persistence, or pace).”3
Indeed, the cumulative medical and social burden of the multiple conditions associated with SCD often results in functional impairment that is comparable in severity to the criteria specified in Listing 7.18.
Notably, Listing 7.18 is unique to adults, with no corresponding listing for children. This appears to be because children are evaluated using functional equivalence criteria at step 3 of the disability determination
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3 Social Security Administration. 2024a. Sickle cell disease and the Social Security disability evaluation process for adults. https://www.ssa.gov/pubs/EN-60-003.pdf (accessed April 8, 2025).
process for children (see footnote 2). However, specifying a childhood listing equivalent to Listing 7.18 for adults could yield benefits. Such a listing would clarify that similar criteria for disability are considered for children with SCD under the concept of functional equivalence. As a result, applicants, health care providers, educators, and others who provide evidence for disability determinations but are unfamiliar with the concept of “functional equivalence” might better understand the type of information that is useful to SSA in evaluating the cumulative burden of SCD on a child applicant. In addition, the transition from pediatric to adult status in individuals with SCD is a particularly vulnerable and complex time representing the intersection of many factors and resulting in increased morbidity and mortality and potentially affecting an individual’s disability status. A childhood listing equivalent to Listing 7.18 also could help facilitate the redetermination process at age 18 and potential qualification for adult disability. The same type of information required to qualify under Listing 7.18 would already be familiar and potentially available in the applicant’s file from childhood.
A challenge associated with a listing like 7.18 is obtaining the necessary information from medical and other sources to document the full range of SCD-related health conditions and symptoms experienced by an individual living with the disease and their effects on the person’s functioning. Individuals who receive care at a comprehensive SCD care center likely will have relatively complete information in their medical record, but many other people living with SCD will not have such a complete and integrated medical record. The use of evaluation templates or forms specific to SCD for collecting necessary information from health care providers could help mitigate this challenge and facilitate collecting comprehensive information about an individual’s SCD-related health conditions and symptoms.
Based on its review of the literature and its expert assessment, the committee reached the following conclusion:
Overarching Conclusion 7: Individuals with sickle cell disease (SCD) can have other conditions for which Social Security Administration listings currently exist, although they may not meet the listing criteria for any single condition.
- The cumulative medical and social burden of the multiple conditions associated with SCD often results in functional impairment that is comparable in severity to the criteria specified in Listing 7.18.
- A childhood listing equivalent to Listing 7.18 for adults would clarify that similar criteria for disability are considered for children with SCD under the concept of functional equivalence and could facilitate the transition from child to adult disability.
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