Daily Loneliness Affects Quality of Life in Sickle Cell Disease.

BACKGROUND: Loneliness is related to psychosomatic challenges in chronic illnesses; however, very little research focuses on loneliness in sickle cell disease (SCD), the most common genetic blood disorder. This study used a daily diary method to illustrate how loneliness and quality of life co-occur in the day-to-day lives of people living with SCD. METHOD: Seventy-nine adults living with SCD (63 women; mean age = 31.76 years) completed daily electronic surveys comprised of a brief loneliness scale and a single-item measure of quality of life. Participants completed each survey once per day for up to 42 consecutive days. We evaluated the effects of daily changes in loneliness on next-day quality of life through multilevel regression models. RESULTS: Central findings indicated that there were significant between-person (b = - .993, p < .001, 95% CI = - 1.26, - .725) and within-person (b = - .202, p < .005, 95% CI = .327, - .089) effects. Specifically, participants who reported higher mean levels of loneliness also reported lower quality of life. Further, days on which participants reported higher loneliness were followed by days on which they reported lower quality of life. CONCLUSIONS: These results may be the first to suggest a connection between loneliness and psychological outcomes in adults living with SCD. Daily fluctuations in loneliness appear to be associated with decrements in next-day quality of life. Future studies should elucidate the clinical relevance and broader health-related implications of these findings.

Illness Intrusiveness in Adults with Sickle Cell Disease: The Role of Fatigue.

Chronic illness experiences often interfere with daily functioning (a concept known as illness intrusiveness) and health-related quality of life (HRQoL). However, less is known about the role of specific symptoms in predicting illness intrusiveness in sickle cell disease (SCD). This exploratory study examined associations between common SCD-related symptoms (i.e., pain, fatigue, depression, and anxiety), illness intrusiveness, and HRQoL among adults with SCD (n = 60). Illness intrusiveness significantly correlated with fatigue severity (r = .39, p = .002), depression severity (r = .45, p < .001), anxiety severity (r = .41, p = .001), physical HRQoL (r = - .53, p < .001), and mental HRQoL (r = - .44, p < .001). Multiple regression revealed a significant overall model, (R2 = .28, F(4, 55) = 5.21, p = .001), with fatigue, but not pain, depression, or anxiety, significantly predicting illness intrusiveness (ß = .29, p = .036). Results suggest that fatigue may be a primary factor contributing to illness intrusiveness-a determinant of HRQoL-in individuals with SCD. Given the limited sample size, larger confirmatory studies are warranted.

Psychosocial and Clinical Risk Factors Associated with Substance Use in Observational Cohort of Patients with Sickle Cell Disease.

BACKGROUND: Patients with sickle cell disease (SCD) experience high rates of chronic pain, and have a high burden of mental health comorbidities shown to negatively influence health. There is limited research on substance use among individuals with SCD. Objective: The aim of this study is to measure the prevalence of substance use in patients with SCD and determine whether psychosocial or clinical risk factors are associated with substance use. Methods: This study was conducted as part of an observational study of patients with SCD at two academic medical centers. We asked participants (ages 15 and older) about the lifetime use of heroin, cocaine, amphetamines, and marijuana/cannabis. We measured stigma, depression, urban life stress, pain catastrophizing, and asked about a brief pain inventory. Results: Of 258 participants, 24.9% (n = 71) reported substance use. Marijuana was the most common substance used (22.5%; n = 65). The mean depressive score met criteria for positive screen amongst individuals who reported a history of substance use (mean 10.7(5.76)). Adjusting for age, sex, yearly family income, and education level, odds of substance use increased with higher levels of internalized stigma (aOR: 1.38; 95% CI: 1.07, 1.77; p = 0.012); higher urban life stress scores (aOR 1.06; 95% CI: 1.01, 1.12; p = 0.017) and higher pain catastrophizing scores (aOR: 1.03; 95% CI: 1.01, 1.06; p = 0.008). Conclusions: Among individuals with SCD who endorse substance use, there was markedly more stress and distress with higher rates of depression and poorer quality of life. Interventions focusing on improving distress tolerance and coping to not only pain, but also social stressors, might reduce substance use.

Marijuana use and health behaviors in a US clinic sample of patients with sickle cell disease.

INTRODUCTION: As marijuana use becomes more common, it is essential clinicians understand the relationship between marijuana use and health behaviors. METHODS: Using a retrospective cohort of adolescents and adults with sickle cell disease (SCD) stratified into a young (<25 years) and older cohort (> = 25 years), we conducted multiple linear regression examining relationship of marijuana use (independent variable) on each dependent variable (SCD self-management score and pain management). RESULTS: Among young cohort, 16.9% used marijuana compared to 21.8% of older cohort. The younger cohort reporting marijuana use had lower mean self-care scores (ß = -2.74;p = 0.009) and were more likely to have admissions to the hospital for pain (ß = 0.87;p = 0.047) compared to non-users. In contrast, the older cohort reporting marijuana use had more days treating pain at home (ß = 0.44;p = 0.035). CONCLUSIONS: Only a minority of patients with SCD reported lifetime marijuana use. Among those reporting marijuana use, there were different associations with self-care and health-related behaviors by age. The older cohort who endorsed marijuana use reported more days of treating pain at home, although this did not translate into increased acute care visits for pain crisis. Among youth, endorsing marijuana use was associated with worse SCD self-care.

Sickle Cell Disease, More Than Just Pain: The Mediating Role of Psychological Symptoms.

OBJECTIVES: Perceived stress is associated with sickle cell disease (SCD) pain; however, little is known about psychological mechanisms that may clarify this link among adult patients. This study explored whether anxiety and depression symptoms explained the relation between perceived stress and SCD pain episode frequency among 70 African-American adults (51.4% women, mean age 35.6 years). METHODS: Participants completed measures of perceived stress, pain, and psychological symptoms in an outpatient clinical setting. RESULTS: A serial multiple mediation model showed that psychological symptoms collectively reduced the association between perceived stress and SCD pain frequency (b = 0.116, P = 0.141). However, only the indirect effect of stress on pain frequency through anxiety symptoms was significant (b = 0.089). CONCLUSIONS: Anxiety but not depression symptoms best explain the association between stress and SCD pain. Further research is needed to identify the specific components of negative affect that drive the experience of SCD pain.

Metabolic syndrome among adults living with sickle cell disease.

Metabolic syndrome (MetS) is a key risk factor for cardiovascular disease (CVD) incidence and all-cause mortality. MetS prevalence among adults with sickle cell disease (SCD) is not well known. We report initial findings from a cross-sectional study that examined MetS risk factors within a cohort of adults living with SCD. 50 adult SCD participants (ages 21-66 years; 72% female) completed demographic and health behavior surveys, health-related family and personal histories, and anthropometric and laboratory measurements. Descriptive and inferential statistics were used to summarize and compare CVD risk factors, stratified in separate analyses by SCD genotype and sex. Triglyceride, blood pressure, and fasting glucose levels were within normal limits. 78% of the cohort reported moderate to high physical activity. However, 46% of this cohort was overweight and dietary saturated fat intake exceeded both the national average (11%) and US Dietary Guidelines (<10%). 14.3% of the cohort fulfilled criteria for MetS with large waist circumference and reduced HDL levels prominently accounting for this status. We evaluated the prevalence of MetS in a cohort of adults living with SCD. Our findings suggest that increased attention to eating habits and physical activity may generate new approaches for decreasing cardiovascular morbidity in SCD.

Communalism Moderates the Association Between Racial Centrality and Emergency Department Use for Sickle Cell Disease Pain.

Sickle cell disease (SCD) is a genetic blood disorder that predominantly affects people of African descent. However, there is limited information on how social and cultural contexts affect SCD-related health care use. We explored whether communalism moderated the relation between racial centrality and emergency department use for SCD pain in a sample of 62 adults who were seen at a comprehensive clinic. Bivariate analyses showed a significant correlation between racial centrality and emergency department use (r = -.30, p = .02). Pain-adjusted regression analyses indicated a moderating effect of communalism (b = .77, p < .01) such that an inverse association between racial centrality and emergency department use was observed only at mean and low levels of communalism. Additional studies are needed to replicate these findings with larger samples. There is also a need for further studies that elucidate the role of culturally centered coping strategies on health care use in this patient group.

Depressive symptoms and sickle cell pain: The moderating role of internalized stigma.

Recent studies describe the clinical implications of sickle cell disease (SCD) stigma. However, little is known about its link to depressive symptoms or its relative influence on the association between depressive symptoms and SCD pain. We examined whether internalized stigma about SCD moderated the relation between depressive symptoms and pain among 69 adults attending a SCD clinic who reported pain episodes and healthcare use over the past three months. Unadjusted bivariate analyses showed a marginal association between depressive symptoms and SCD pain frequency (r = .21, p < .10). Further analyses adjusting for health care use indicated a moderating effect of internalized stigma (b = -.10, p < .05): a significant association between depressive symptoms and pain was observed among participants low in internalized stigma, but this relationship was attenuated at moderate and high stigma levels. These results denote counterintuitive consequences of internalized attitudes about SCD and suggest a need for further study of the psychosocial and clinical implications of SCD stigma.

Multiple Levels of Suffering: Discrimination in Health-Care Settings is Associated With Enhanced Laboratory Pain Sensitivity in Sickle Cell Disease.

OBJECTIVE: People living with sickle cell disease (SCD) experience severe episodic and chronic pain and frequently report poor interpersonal treatment within health-care settings. In this particularly relevant context, we examined the relationship between perceived discrimination and both clinical and laboratory pain. METHODS: Seventy-one individuals with SCD provided self-reports of experiences with discrimination in health-care settings and clinical pain severity, and completed a psychophysical pain testing battery in the laboratory. RESULTS: Discrimination in health-care settings was correlated with greater clinical pain severity and enhanced sensitivity to multiple laboratory-induced pain measures, as well as stress, depression, and sleep. After controlling for relevant covariates, discrimination remained a significant predictor of mechanical temporal summation (a marker of central pain facilitation), but not clinical pain severity or suprathreshold heat pain response. Furthermore, a significant interaction between experience with discrimination and clinical pain severity was associated with mechanical temporal summation; increased experience with discrimination was associated with an increased correlation between clinical pain severity and temporal summation of pain. DISCUSSION: Perceived discrimination within health-care settings was associated with pain facilitation. These findings suggest that discrimination may be related to increased central sensitization among SCD patients, and more broadly that health-care social environments may interact with pain pathophysiology.

The Measure of Sickle Cell Stigma: Initial findings from the Improving Patient Outcomes through Respect and Trust study.

Research about the influence of stigma on health outcomes in sickle cell disease is limited. We administered the recently developed Measure of Sickle Cell Stigma to 262 patients in the United States. The Measure of Sickle Cell Stigma yielded very good internal consistency and four interpretable factors. Significant associations among stigma, pain-related healthcare utilization, and perceived disease severity were observed for three of the four stigma factors (F range = 2.78-5.44). The Measure of Sickle Cell Stigma appears to be a useful tool for measuring disease-specific stigma among adults living with sickle cell disease, and further assessment of its clinical utility is warranted.

Race and social attitudes about sickle cell disease.

OBJECTIVE: Sickle cell disease is perhaps the most racialized condition in the history of modern medicine, yet very little research has focused on how racial perceptions influence social attitudes about the disease. Subsequently, the implications of these perceptions for public health prevention efforts and the provision of clinical care are not well known. DESIGN: In this brief commentary, we posit that social cognitive and media framing theories provide useful approaches for assessing relations between race and social attitudes about sickle cell disease. CONCLUSION: Such inquiries might lead to more rigorous study of mechanisms that shape perceptions about sickle cell risk, interpersonal empathy toward patients, and public support for sickle cell-related policies.

Religious coping and hospital admissions among adults with sickle cell disease.

Although a well-established literature implicates religiosity as a central element of the African American experience, little is known about how individuals from this group utilize religion to cope with specific health-related stressors. The present study examined the relation between religious coping and hospital admissions among a cohort of 95 adults with sickle cell disease-a genetic blood disorder that, in the United States, primarily affects people of African ancestry. Multiple regression analyses indicated that positive religious coping uniquely accounted for variance in hospital admissions after adjusting for other demographic and diagnostic variables. Specifically, greater endorsement of positive religious coping was associated with significantly fewer hospital admissions (ß = -.29, P < .05). These results indicate a need for further investigation of the roles that religion and spirituality play in adjustment to sickle cell disease and their influence on health care utilization patterns and health outcomes.

Predictors of employment status among African Americans with sickle cell disease.

Adults living with sickle cell disease (SCD) have extremely high rates of unemployment; however, very little is known about factors that contribute to their vocational outcomes. This study examined demographic, illness perception, and psychological variables as predictors of employment status among 115 adult respondents who completed a cross-sectional survey as part of the Cooperative Study of Sickle Cell Disease. Logistic regression analysis indicated that gender, assertiveness, and perceived impact of SCD were unique predictors of employment status. Women were 2.88 times more likely to be employed than men, and the odds of being employed increased by a factor of 2.47 for each one unit decrease in assertiveness. More favorable perceptions of SCD were also associated with a two-fold increase in employment. The results suggest that demographic and psychosocial factors may play a more important role in predicting employment outcomes in adults with SCD than previously recognized.

Problematic hospital experiences among adult patients with sickle cell disease.

BACKGROUND: Adults with sickle cell disease (SCD) have often reported difficulties obtaining care during vaso-occlusive crisis (VOC) in qualitative studies. METHODS: We measured the experiences of 45 SCD patients who received in-hospital care for VOC using the Picker Patient Experience Questionnaire (PPE-15), and used the one sample binomial test to compare with national norms. RESULTS: Most SCD patients reported that they were insufficiently involved in decisions (86%), staff gave conflicting information (64%), it wasn't easy to find someone to discuss concerns (61%), doctors' answers to questions were not clear (58%), nurses' answers to questions were not clear (56%), doctors did not always discuss fears and anxieties (53%), and nurses did not always discuss fears and anxieties (52%). A greater percentage of SCD patients than the U.S. sample in 9 of 12 areas reported problems. CONCLUSIONS: Further research is needed to determine the consequences of and potential interventions to improve these poor experiences.

Hospital self-discharge among adults with sickle-cell disease (SCD): associations with trust and interpersonal experiences with care.

BACKGROUND: Patient self-discharge from hospitals has been associated with a number of negative clinical outcomes. Research suggests that low patient trust and poor quality interpersonal experiences with care may be associated with hospital self-discharge. Although adults with sickle-cell disease (SCD) often report poorer quality healthcare experiences, research examining hospital self-discharge and its associations with both patient trust and quality of healthcare experiences is lacking for this patient population. OBJECTIVE: To examine the association of interpersonal experiences with care and trust in the medical profession with hospital self-discharge history among patients with SCD. DESIGN: Cross-sectional study. SETTING: A large, urban academic medical center. PATIENTS: Adults (age 18+ years) with SCD seeking outpatient or inpatient care. MEASUREMENTS: We compared patient characteristics, patient perceptions of the quality of interpersonal experiences with care, and levels of trust between patients with and without a history of hospital self-discharge. RESULTS: Adjusted analyses indicated that having a history of hospital self-discharge was associated with more negative interpersonal experiences and lower levels of trust. CONCLUSIONS: Hospital self-discharge may be an important indicator of the quality of care received by adults with SCD. Further research is needed to better understand this phenomenon so that effective interventions can be designed to prevent its occurrence.

The association of provider communication with trust among adults with sickle cell disease.

BACKGROUND: Adults with sickle cell disease often report poor interpersonal healthcare experiences, including poor communication with providers. However, the effect of these experiences on patient trust is unknown. OBJECTIVE: To determine the association between patient ratings of the previous quality of provider communication and current trust in the medical profession among adults with sickle cell disease. RESEARCH DESIGN: Cross-sectional survey. PARTICIPANTS: A total of 95 adults with sickle cell disease. MEASUREMENTS: The four-item Provider Communication Subscale from the Consumer Assessment of Healthcare Plans and Systems Survey; The five-item Wake Forest Trust in the Medical Profession Scale. MAIN RESULTS: Better ratings of previous provider communication were significantly associated with higher levels of trust toward the medical profession. A 10% increase in provider communication rating was associated with a 3.76% increase in trust scores (p < 0.001, 95% CI [1.76%, 5.76%]), adjusting for patient-level demographic, clinical, and attitudinal characteristics. CONCLUSIONS: Poorer patient ratings of provider communication are associated with lower trust toward the medical profession among adults with sickle cell disease. Future research should examine the impact of low trust in the medical profession on clinical outcomes in this population of patients.

Sickle cell disease in a "postracial" America.

Sickle cell disease was first identified as a clinical syndrome in the United States nearly 100 years ago. Since that time, perhaps no other medical condition has been as strongly associated with the concept of "race." In this commentary, the authors integrate multidisciplinary perspectives to argue for more rigorous analyses of how perceiving sickle cell disease through racial lenses impacts its public image and, more importantly, its perception among health care providers. The authors assert that concerted efforts involving collaborations among media, community-based organizations, medical professionals, and health care policy makers are needed to challenge popular misconceptions about sickle cell disease heritability and risk.

Health care provider attitudes toward patients with acute vaso-occlusive crisis due to sickle cell disease: development of a scale.

OBJECTIVE: Patients with sickle cell disease (SCD) often perceive negative provider attitudes, which may affect the quality of patient-provider communication and care during vaso-occlusive crises (VOCs). This study investigated the validity and reliability of a scale to measure provider attitudes toward patients with acute VOC. METHODS: Using a cohort of adults with VOC (September 2006 to June 2007), we administered a 10-item provider questionnaire within 72 h of patient encounters. After factor analysis, we created a 7-item Positive Provider Attitudes toward Sickle Cell Patients Scale (PASS); higher scores indicate more positive attitudes. We assessed internal consistency and evidence of construct validity, exploring bivariate relationships between provider or patient characteristics and the PASS score using multilevel modeling. RESULTS: We collected 121 surveys from 84 health care providers for 47 patients. Patients averaged 30.3 years in age, and 60% were women. Among providers, 79% were nurses, and 70% worked in inpatient settings. PASS scores averaged 24.1 (S.D. 6.7), ranged 7-35, and had high internal consistency (Cronbach's alpha=0.91). As hypothesized, inpatient vs. emergency department providers (Delta=4.65, p<0.001) and nurses vs. other providers (Delta=0.95, p<0.001) had higher PASS scores. Higher patient educational attainment (Delta per year=2.74, p<0.001) and employment (Delta=5.62, p=0.001) were associated with higher PASS scores. More frequent hospitalizations (Delta per episode=-0.52, p<0.001) and prior disputes with staff (Delta=-7.53, p=0.002) were associated with lower PASS scores. CONCLUSION: Our findings provide preliminary evidence for the reliability and construct validity of the PASS score in measuring provider attitudes toward patients with VOC. PRACTICE IMPLICATIONS: Future studies should examine the validity of PASS in other cohorts of patients with SCD and their providers. With further evidence, PASS may prove useful for investigating the impact of provider attitudes on the quality of communication and care provided to these patients.

Reducing and eliminating health disparities: a targeted approach.

Health disparities have dominated recent discourse among public health and medical researchers. Ever since the United States began to compile health statistics, differences in health status have been noted between majority and non-majority populations. Myriad approaches have been undertaken in an attempt to reduce or eliminate racial and ethnic disparities in health. However, the disparities continue to persist. We are at a point in our history where innovative strategies must be explored that will be more effective in addressing racial and ethnic disparities in health. In large part, health disparities exist as a result of inequitable distribution of goods, resources, services and power in America. We have learned that improvements in health cannot come about solely through primary and secondary interventions but rather through an examination of the availability of resources that would allow individuals to improve their health. The goal of this paper is to provide an overview of the contextual factors that affect health disparities, to integrate theory to address disparities and to provide recommendations to encourage systematic changes to eliminate health disparities. It is hoped that this paper will bring about a national discussion relating to addressing the real issues we face in reducing and ultimately eliminating health disparities.