Sickle cell disease: The psychological toll

In 2024, 12-year-old Kendric Cromeropens in new window made medical history as the first sickle cell patient to receive a revolutionary gene therapy that effectively cured what would have been a lifelong condition. Cromer’s therapy targeted the root cause of sickle cell disease (SCD): a genetic mutation that causes atypical hemoglobin and distorts red blood cells into a crescent or “sickle” shape. These rigid cells struggle to flow through small blood vessels, often clumping together and causing painful blockages called vaso-occlusive crises (see Sickle cell disease 101).

The treatment Cromer received, one of two approved by the Food and Drug Administration (Lyfgenia and CASGEVY), consisted of modifying his stem cells to produce normal red blood cells. The impact of this gene therapy could extend far beyond Cromer. For the estimated 100,000 Americans living with SCD, most of whom are of African descent, it promises liberation from a painful and often exhausting disorder that shortens life expectancy by an average of 20 yearsopens in new window.

“I didn’t think I would ever see the day,” said Sharee Turpin, a patient advocate for the pediatric hematology and oncology unit at the University of Rochester’s Medical Center, who lives with sickle cell. “It’s finally giving a glimpse of what life could be, figuring out who we are without this illness and the hurdles that it continuously puts in front of us.” Currently, gene therapy has a hefty price tag of more than $2.2 millionopens in new window, which is out of reach for most SCD patients.

Living with sickle cell disease creates significant challenges for a person’s mental health and quality of life. Psychologists are crucial for helping those living with SCD manage numerous neurocognitive, emotional, and psychosocial issues. These include depression, anxiety, poor sleep quality, and social isolation as they grapple with their condition (Essien, E. A., et al., Medicine, Vol. 102, No. 47, 2023opens in new window). Frequent vaso-occlusive crises disrupt daily life and can lead to missed work, school, and activities. Acute and chronic pain, which is common to SCD patients, takes a significant toll on their mental health.

People with SCD face a unique set of challenges at the intersection of race, poverty, and disability, placing them at heightened risk of inadequate mental and physical health care. Recent research shows that SCD coupled with socioeconomic hardship may accelerate cognitive aging. In a study of 200 young Black adults from St. Louis and the surrounding region, researchers found that participants with the disease had brains that appeared, on average, 14 years older than their actual age (Ford, A. L., et al., JAMA Network Open, Vol. 8, No. 1, 2025opens in new window).

Two of the most common aspects of the disease—chronic anemia and decreased oxygen supply—can increase the probability of strokes, which directly affect cognition. As a result, researchers have found that some who have SCD have “spatial and constructional deficits, attention and memory weaknesses, working memory issues, lower academic functioning, poor social skills, and abnormal executive functioning” (Braniecki, S., et al., Frontiers in Neurology, Vol. 15, 2024opens in new window). For children and teenagers, these symptoms can cause difficulties in the classroom and with building social skills, while adults may find it difficult to maintain steady employment or healthy relationships.

Sickle cell disease is an invisible disability, leading many youth to hesitate in disclosing their condition. Jeffrey Karst, PhD, a pediatric psychologist at the Midwest Athletes Against Childhood Cancer (MACC) Fund Center for Cancer and Blood Disorders at Children’s Wisconsin and the Medical College of Wisconsin, notes that for many children and teenagers, admitting to having a disease can be uncomfortable. Younger children may fear that their condition is contagious or worry about being ridiculed. Furthermore, because of the nature of their illness, they often have lower energy levels, which can prevent them from participating fully in activities. Many teenagers may even choose not to inform friends about hospital admissions: “They often are very worried about admitting or acknowledging that. So both the disease and its impact have stigma,” Karst explained.

Recognizing the complexity of SCD, psychologists are providing comprehensive support for children and youth with the disease. In pediatric settings, psychologists often work as part of multidisciplinary teams to deliver care. They help parents of young children understand SCD and managing their child’s care, including developmental screenings to identify early therapeutic needs.

As children living with SCD grow to school age, psychologists can support parents in requesting a meeting with the school principal, school psychologist, teachers, and special education coordinator to make sure these individuals are informed about the physical aspects of SCD and the child’s specific cognitive disabilities, if any, and to set up school-based testing and accommodations. Because testing and accommodations in schools require significant resources in terms of time and funds, many school systems—especially those unfamiliar with SCD—may rely on the expert recommendations of psychologists, when available, on how to proceed. Psychologists also provide emotional support as children form friendships and figure out how to discuss their illness in social interactions.

Psychologists can help patients manage acute and chronic pain, improve medication adherence, as well as support emotional well-being and foster self-advocacy skills to deal with ongoing health and social challenges. Effective interventions include cognitive behavioral therapy, acceptance and commitment therapy, distraction methods, deep breathing exercises, and mindfulness practices.

Psychologists are also at the forefront of developing interventions for children with SCD-related cognitive difficulties. A randomized controlled trial of an adaptive cognitive training program for children with SCD showed promising results. Those who completed the intervention showed significant improvement in visual working memory compared with a waiting list group. Treatment effects were especially notable for patients who completed 10 sessions. They demonstrated improved visual working memory, verbal short-term memory, and math fluency (Hardy, S. J., et al., Journal of Pediatric Psychology, Vol. 46, No. 8, 2021opens in new window).

Research has revealed a troubling pattern of discriminatory treatment toward SCD patients in medical settings (Power-Hays, A., & McGann, P., The New England Journal of Medicine, Vol. 383, No. 20, 2020opens in new window). Pain treatment is a key concern for patients with SCD. Studies on racial biases in health care have shown a common erroneous belief among medical trainees that Black patients have a higher tolerance for pain than Whites, leading to racial disparities in treatment of pain (Hoffman, K. M., et al., Proceedings of the National Academies of Science, Vol. 113, No. 16, 2016opens in new window). Similarly, a recent meta-analysis showed that both Black and Hispanic patients were less likely than non-Hispanic White patients to receive opioid medication (Hirani, S., et al., Journal of Pain Research, Vol. 17, 2024opens in new window). Additionally, research evidence shows how race-based discrimination can exacerbate depressive symptoms and pain among those living with SCD (McGill, L. S., et al., Stigma and Health, advance online publication, 2024).

In health care settings, patients with SCD often experience microaggressions and even racial trauma, especially if they are in crisis. These experiences can lead to misunderstandings and mistreatment, particularly when it comes to pain management. For example, patients might face skepticism and disbelief when seeking emergency medical attention during a crisis. Katlyn Rice, PhD, a pediatric psychologist at the University of Rochester Medical Center, noted that some physicians and nurses do not always fully understand the intense pain and other symptoms of the disease, which may require pain medications to treat, especially if they are not connected to a hematology center. “Patients with sickle cell sometimes worry they will be discriminated against in the emergency room and subsequently receive inappropriate or harmful care because they may be seen as ‘drug seekers.’ It’s a pervasive problem without proper education about the disease and its effects. It’s rooted in systemic racism.”

The challenges are even more pronounced for young Black males. Justin Williams, PhD, a pediatric psychologist at Children’s Healthcare of Atlanta, addresses this directly with her patients: “I often say, ‘You are a young Black male, and you have a chronic health condition that requires you to take opioids. Put that together, and there can be increased challenges when engaging with the health care system.” She advises her patients on effective self-advocacy strategies to navigate these systemic biases when they are in moments of discomfort or crisis.

Socioeconomic barriers further complicate care for people with SCD. About 50% to 60%opens in new window of SCD patients are enrolled in Medicaid, but high treatment costs and limited access to specialized care exacerbate existing disparities. The average cost of care for a Medicaid enrollee with SCD is more than double that of the general Medicaid population (Speller, J., et al., Health Affairs Scholar, Vol. 2, No. 4, 2024opens in new window).

Funding challenges present significant obstacles in delivering comprehensive care. Rice points out that Medicaid’s limited diagnostic coding and low reimbursement rates for mental health services make it difficult for providers to sustainably offer these crucial services. Moreover, medical institutions often view the sickle cell population as “expensive” rather than prioritizing their specific needs, which includes multidisciplinary and systemic care.

The disparity in research funding is also stark. For instance, despite being 3 times more prevalent than cystic fibrosis in the United States, sickle cell receives significantly less funding. Researchers found that federal funding per person for cystic fibrosis was 3.4 times greater than for SCD ($2,807 versus $812) and foundation funding was 75 times greater ($7,690 versus $102) (Farooq, F., et al., JAMA Network Open, Vol. 3, No. 3, 2020opens in new window). Rice noted that this disparity is likely tied to the demographics of those affected and systemic racism: “There are many layers to that, but sickle cell primarily affects people of color.”

The transition from pediatric to adult care marks a critical juncture for young people with SCD. As they step into adolescence and young adulthood, they must quickly learn to navigate a complex health care system that often lacks the nurturing support they experienced in pediatric settings. This shift is not just a matter of changing doctors; it’s a movement into a new world in which they must become their own primary advocates. The stakes are high—mortality rates more than double for patients ages 20 to 24 compared with those ages 15 to 19 (Kayle, M., et al., Pediatric Blood & Cancer, Vol. 66, No. 1, 2019opens in new window).

Recognizing these challenges, psychologists are stepping up to bridge the gap. Christopher McCormick, PhD, a pediatric psychologist in the sickle cell program at Seattle Children’s Odessa Brown Children’s Clinic, employs a proactive approach, gradually shifting responsibilities to young patients starting as early as age 12. This method aims to build a foundation of knowledge and skills crucial for navigating adult health care systems.

They begin by ensuring young patients know basic information, from their clinic’s contact information, medication details, and disease subtype to more practical skills for navigating adult settings. “We try to find a balance between supporting autonomy, skill development, and mastery with the understanding that if they’re not successful, their families and the care team will step in to support them because the consequences of not doing so are devastating,” he said.

Caryn R. R. Rodgers, PhD, a pediatric psychologist at the Children’s Hospital at Montefiore at the Albert Einstein College of Medicine in Bronx, New York, focuses on empowering her college-age patients through support groups. Given the systems at play that often put her patients at a disadvantage, Rodgers emphasizes building self-efficacy and advocacy skills to help them navigate adult health care.

Many of her patients have identified the burden of navigating health care with SCD. Rodgers works with her patients by validating their struggle and helping them build skills to navigate adult health care systems. Rodgers encourages her patients to ask questions, identify appropriate resources, and persist when they encounter barriers or insensitivity from providers.

“When you have an invisible disease, your voice needs to be bigger to ensure you get your needs met,” Rodgers said. “I tell my patients, ‘Figure out what you want to let people know. Figure out the person to ask about follow-up appointments. If someone ignores your requests for help, who is the next person you need to go to?’”

Amy Williams, PhD, a pediatric psychologist at Riley Hospital for Children at the Indiana University School of Medicine, is examining how perceptions of injustice in medical care impact young SCD patients’ mental and physical health. Her 2-year study of 50 youth ages 11 to 18 aims to develop targeted interventions that address the unique challenges faced by this population, including the emotional toll of chronic pain and social burdens. “We’re interested in understanding what it is like to live with sickle cell disease and the experiences they have with injustice so we can support them with the most effective intervention,” said Williams.

Williams and her team have begun analyzing baseline data while continuing to collect information on longitudinal health outcomes. “Some teens report a lot of experiences with stigma and injustice, while others don’t report as much. That helps us to identify the patients who might be at highest need and be more effective in delivering interventions because of those types of experiences,” she said. “Some are health care–related experiences of feeling pain-related stigma, of ‘people don’t believe my pain’ or ‘I’ve been treated poorly because of my sickle cell disease.’ Some of it is racial bias more broadly that they’ve experienced outside of a health care setting as well. There is a lot of variability.”

Innovative tools are also emerging to support this transition. The iManage for SCD app—codeveloped by Lori Crosby, PsyD, director of the Crosby Research Lab at Cincinnati Children’s Hospital, and her team—offers a holistic approach to managing their health. The app allows users to monitor and track their pain, symptoms, and overall mood. It also allows them to create and complete goals for how they manage their disease. Data gathered from users show favorable results, with frequent app use linked to improved mood, reduced pain, and better self-management skills (Hood, A. M., et al., Translational Behavioral Medicine, Vol. 11, No. 10, 202opens in new window1).

A number of new medicationsopens in new window are in various stages of development. In December 2024, the Centers for Medicare and Medicaid Services (CMS) reached agreement on a payment modelopens in new window with two gene therapy manufacturers. CMS invited statesopens in new window to join the payment program between January 2025 and January 2026. This initiative could significantly improve access to gene therapy for those who need it most, potentially covering not just the treatment itself but associated behavioral health services as well.

For SCD patients who are already adults, living with the disease may not get easier anytime soon. But the promise of widespread use of the revolutionary gene therapy that liberated Kendric Cromer—and emotional, cognitive, and psychosocial support from psychologists—puts hope on the horizon.