Table 2.

Synthesis of Articles

Author/year	Purpose	Approach	Relevant Findings
Adeyemo et al. (2015)	To assess health-related quality of life and perceived stigma in adolescents with SCD.	Location: Nigeria Design: Cross-sectional with quantitative components Sample: 80 adolescents ages 15–18 with SCD and a control group of 80 unaffected adolescents	Adolescents with SCD (70%) reported moderate to very high stigma and stigma was negatively associated with all domains of health-related QoL. Adolescents with SCD had worse health-related QoL than their unaffected peers.
Bediako et al. (2014)	To test the Measure of Sickle Cell Stigma Scale (MoSCS).	Location: US Design: Cross-sectional with quantitative components Sample: 262 adolescents and adults ages 15–70	The 11 items loaded on four factors: social exclusion, internalized stigma, disclosure concerns, and expected discrimination. Cronbach’s alpha for the entire scale was. 86.
Blake et al. (2017))	To understand stigma and illness uncertainty experiences in adults with SCD.	Location: Jamaica Design: Cross-sectional with quantitative components Sample: 101 adults	Stigma and illness uncertainty had a small but significant correlation.
Cobo Vde et al. (2013)	To describe and assess the development of sexuality in individuals with SCD.	Location: Brazil Design: Cross-sectional with quantitative and quantitative components Sample: 20 adults ages 19–47	In this mostly (75%) female sample, 65% said that they had impaired sexuality due to SCD. Participants reported their impairment being due to pain and discrimination and negative feelings experienced in intimate relationships.
Cole (2007)	To describe the chronic illness experience from the perspective of Black women with SCD, including disease related stress and mental health.	Location: US Design: Cross-sectional with qualitative components Setting: 10 women	Themes that emerged from the interviews included stressful experiences during hospital care, stigmatizing experiences as result of their disease status, stressful events that precede pain episodes, and disease related stress impacting perceptions about mental health.
Derlega et al. (2014)	To describe the disclosure experiences of individuals with SCD.	Location: US Design: Cross-sectional with quantitative components Setting: 73 adults	Participants disclosed their thoughts and feelings concerning pain episodes to God and to their primary medical providers than to both friends and family. Taking with God and parents about pain was associated with better psychological adjustment, including stigma consciousness. Participants who talked about pain episodes to parents were less self-conscious about being stigmatized or stereotyped because of their disease status.
Dyson et al. (2010)	To describe the disclosure patterns of children, adolescents, and young adults with SCD in school.	Location: UK Design: Cross-sectional using mixed method methodologies Sample: 569 questionnaires and 40 interviews; ages 4–25	Over 75% of the sample reported at least one negative experience at school (i.e. called lazy, prevented from drinking water). Participants that reported that more adults and peers knew about their disease status reported more negative experiences. Participants described a cost benefit analysis of whether or not they should disclose their disease status in school.
Ezenwa et al. (2015)	To examine the associations between perceived injustice, stress, and pain in adults with SCD.	Location: US Design: Cross-sectional correlational pilot study Sample: 52 adults	Perceived injustice from doctors and nurses was a significant predictor of perceived stress and pain.
Ezenwa et al. (2016)	To examine the pain coping strategies of individuals with SCD that experience healthcare injustice when seeking care.	Location: US Design: Cross-sectional comparative study Sample: 52 adults	Participants who reported healthcare injustice from physicians and nurses were more likely to use pain coping strategies associated with negative pain outcomes (catastrophizing, isolation, fear self-statements), while those reporting healthcare justice were more likely to use strategies associated with positive pain outcomes (hoping/praying, massage, calming self-statements).
Haywood et al. (2014)	To examine perceived discrimination in healthcare and pain burden among individuals with SCD.	Location: US Design: Cross-sectional with quantitative components Sample: 291 participants ages 15 and older	Discrimination was associated with greater emergency room utilization, having difficulty persuading providers about pain, and experiencing daily chronic pain. Disease and not raced-based discrimination was associated with greater self-reported pain.
Haywood et al. (2014))	To evaluate the association between perceived discrimination from healthcare providers and nonadherence to physician recommendations among individuals with SCD.	Location: US Design: Cross-sectional with quantitative components Sample: 273 participants ages 15 and older	58% of the non-adherent group, compared to 43% of the adherent group, reported at least one experience of discrimination. The non-adherent group also reported significantly lower levels of trust of healthcare providers than did the adherent group” Individuals that had experienced previous discrimination were 53% likely to report nonadherence
Haywood et al. (2013)	To determine whether SCD patients have longer wait times to see physicians in the ED compared to a Long Bone Fracture (LBF) patient group and the General Patient Sample group.	Location: US Design: Cross-sectional with quantitative components Sample: 171,789 records used from a national database	SCD patients waited 8% longer compared to general patient sample and 32% longer compared to LBF patients, after controlling for differences. Mean wait times were 50% longer for Black LBF patients.
Holloway et al. (2016)	To investigate internalized stigma amongst individuals with SCD.	Location: US Design: Cross-sectional with quantitative components Sample: 69 adults	Participants reported low depressive symptoms and internalized stigma. Depressive symptoms and internalized stigma were positively related.
Jenerette et al. (2012)	To test the Sickle Cell Disease Health-Related Stigma Scale (SCD-HRSS) in young adults with SCD.	Location: US Design: Cross-sectional with quantitative components Sample: 77 adults ages 18–35	Preliminary analysis of the SCD-HRSS revealed Cronbach’s alpha reliabilities for the entire scale (0.84) as well as for each of the subscales (Doctors = 0.68, Family = 0.82, Public = 0.73).
Jenerette et al. (2014).	To identify factors that influence care-seeking for pain in young adults with SCD.	Location: US Design: Cross-sectional mixed methods pilot study Sample: 69 young adults ages 18–35	Participants waited until their pain was an average of 8.7 out of 10 before seeking care. The second most common reason for not seeking care was a desire to avoid the emergency departments due to prior treatment.
Jenerette et al. (2014)	To assess the efficacy of a self-care management intervention to lower self-perceived health-related stigma for young adults with SCD.	Location: US Design: Longitudinal, randomized control trial with quantitative components Sample: 90 adults with SCD ages 18–35 randomized into a Care-Seeking intervention or attention control group	The care seeking intervention was associated with significant increase in perceived total stigma and stigma from doctors.
Kass et al. (2004)	To determine if genetic cause of disease influences experiences of privacy, disclosure, and consequences of disclosure experiences.	Location: US Design: Cross-sectional with quantitative and qualitative components Sample: 99 adults with SCD; comparison groups had cystic fibrosis, diabetes, HIV, and risks for breast or colon cancer	Participants with SCD were very likely to: 1) regret others found about their disease status; 2) have individuals that they hope do not find out; 3) regret others found out; 4) to report discrimination experiences with employment and receiving healthcare as a result of their disease status. The main reason they regretted others knowing was because they may be treated differently (pitied, discriminated against stigmatized, etc.) as a result of others knowing.
Labore et al. (2017)	To explore transition to self-management in SCD in young adults.	Location: US Design: Cross-sectional with qualitative components Sample: 12 participants ages 21–25	All participants mentioned experiences or anticipation of stigma in adult care settings as a part of their transition experience.
Labrousse (2007)	To explore how young Haitian American women cope with having SCD.	Location: US Design: Cross-sectional with qualitative components Sample: 12 participants ages 21–31) Participants described experiences of racial, socioeconomic, and medical unfairness in their life narratives.	Participants described fearing seeking emergency room care due to anticipated stigma.
Mathur et al. (2016))	To examine the relationship between perceived discrimination and both clinical and laboratory pain.	Location: US Design: Cross-sectional with quantitative components Sample: 71 adults	Participants that reported discrimination in healthcare settings (38%) had greater clinical pain severity and functional pain interference.
Mulchan et al. (2016)	To examine the applicability of the Social-ecological Model of Adolescent and Young Adult Readiness to Transition (SMART) model for adolescents and young adults with SCD.	Location: US Design: Cross-sectional with qualitative and quantitative components Sample: 14 adolescents and young adults ages 14–24; 10 clinical experts	During interviews, both individuals with SCD and providers identified stigma and lack of community awareness of SCD emerged as a theme within the SMART framework. Elements of stigma and racism also emerged in themes related to medical status and patient-provider communications.
Nelson & Hackman (2013)	To explore perceptions of race and racism among persons with SCD and their families and healthcare staff.	Location: US Design: Cross-sectional with quantitative components Sample: 112 patients/families, ages 12–50; 135 staff members	Patients and their family members were more likely to report that race affects the quality of healthcare for individuals with SCD (50%) in comparison to healthcare providers (31.6%). While healthcare staff were more likely to report unfair treatment of SCD patients (20.9% vs. 10.9%).
Ola et al. (2016)	To explore experiences with depression in people living with SCD in Lagos, Nigeria.	Location: Nigeria Design: Cross-sectional using mixed method methodologies Sample: 103 completed questionnaires, ages 16–50; 15 interviewed and 10 completed focus groups	74 participants had some level of depression ranging from mild to severe. In interviews, participants reported experiencing stigma in the community, workplace, and school and also in healthcare settings. Some participants reported risk-taking behaviors, suicidal thoughts and suicide attempts. During the focus groups, participants discussed challenges and ways to overcome living with SCD in a discriminatory society.
Royal et al. (2011)	To examine the relationship between race, identity, and experience of living with SCD.	Location: US Design: Cross-sectional with qualitative and quantitative components Sample: 46 adults ages 18–59	Participants agreed with statements stating that their disease status affects how they are treated and that their race influences their experience with their disease. During interviews, participants described being treated differently due to their race and disease status in public and healthcare realms. 36% reported that their race did not impact their disease experience and 27% reported that their disease status had no effect on how they are treated.
Sankar et al. (2006)	To explore the relationship between genetic cause and stigma.	Location: US Design: Cross-sectional with qualitative components Sample: 22 adults with SCD ages 18–53; groups with cystic fibrosis, hearing loss/deafness, and breast cancer	Overall genetic cause did not automatically lead to perceptions of stigma. Participants with SCD associated their condition with their racial identity and discrimination; whereas participants with cystic fibrosis rarely associated their disease with their race.
Stanton et al. (2010)	To explore the association between optimism, perceived discrimination, and healthcare utilization in SCD.	Location: US Design: Cross-sectional with quantitative components Sample: 49 adults	There was an increase in hospitalizations among those with high optimism and a decrease among those with low optimism as perceived discrimination increased. There was also an increase in days hospitalized as perceived discrimination increased amongst those with high optimism and a decrease in those with low optimism.
Wakefield et al. (2017)	To determine the frequency of perceived racial bias and health-related stigma and how they are related to psychological and physical wellbeing in adolescents and young adults with SCD.	Location: Design: Cross-sectional with quantitative components Sample: 28 adolescents and young adults ages 13–21	All participants except 1 reported at least one experience of racial bias and perceived health-related stigma (both 96.4%). Perceived health-related stigma was positively correlated with perceived racial bias. Participants that reported less racial bias had higher quality of life. Participants that reported greater stigma had lower quality of life. There was a moderate positive relationship between perceived racial bias and pain burden.