Treatment of Acute Pain in Adults With Sickle Cell Disease in an Infusion Center Versus the Emergency Department : A Multicenter Prospective Cohort Study.

BACKGROUND: Patients with sickle cell disease (SCD) have vaso-occlusive crises (VOCs). Infusion centers (ICs) are alternatives to emergency department (ED) care and may improve patient outcomes. OBJECTIVE: To assess whether care in ICs or EDs leads to better outcomes for the treatment of uncomplicated VOCs. DESIGN: Prospective cohort. (ClinicalTrials.gov: NCT02411396). SETTING: 4 U.S. sites, with recruitment between April 2015 and December 2016. PARTICIPANTS: Adults with SCD living within 60 miles of a study site. MEASUREMENTS: Participants were followed for 18 months after enrollment. Outcomes of interest were time to first dose of parenteral pain medication, whether pain reassessment was completed within 30 minutes after the first dose, and patient disposition on discharge from the acute care visit. Treatment effects for ICs versus EDs were estimated using a time-varying propensity score adjustment. RESULTS: Researchers enrolled 483 participants; the 269 who had acute care visits on weekdays are included in this report. With inverse probability of treatment-weighted adjustment, the mean time to first dose was 62 minutes in ICs and 132 minutes in EDs; the difference was 70 minutes (95% CI, 54 to 98 minutes; E-value, 2.8). The probability of pain reassessment within 30 minutes of the first dose of parenteral pain medication was 3.8 times greater (CI, 2.63 to 5.64 times greater; E-value, 4.7) in the IC than the ED. The probability that a participant's visit would end in admission to the hospital was smaller by a factor of 4 (0.25 [CI, 0.18 to 0.33]) with treatment in an IC versus an ED. LIMITATION: The study was restricted to participants with uncomplicated VOCs. CONCLUSION: In adults with SCD having a VOC, treatment in an IC is associated with substantially better outcomes than treatment in an ED. PRIMARY FUNDING SOURCE: Patient-Centered Outcomes Research Institute.

Mortality and Access to Kidney Transplantation in Patients with Sickle Cell Disease-Associated Kidney Failure.

BACKGROUND AND OBJECTIVES: Patients with sickle cell disease-associated kidney failure have high mortality, which might be lowered by kidney transplantation. However, because they show higher post-transplant mortality compared with patients with other kidney failure etiologies, kidney transplantation remains controversial in this population, potentially limiting their chance of receiving transplantation. We aimed to quantify the decrease in mortality associated with transplantation in this population and determine the chance of receiving transplantation with sickle cell disease as the cause of kidney failure as compared with other etiologies of kidney failure. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Using a national registry, we studied all adults with kidney failure who began maintenance dialysis or were added to the kidney transplant waiting list in 1998-2017. To quantify the decrease in mortality associated with transplantation, we measured the absolute risk difference and hazard ratio for mortality in matched pairs of transplant recipients versus waitlisted candidates in the sickle cell and control groups. To compare the chance of receiving transplantation, we estimated hazard ratios for receiving transplantation in the sickle cell and control groups, treating death as a competing risk. RESULTS: Compared with their matched waitlisted candidates, 189 transplant recipients with sickle cell disease and 220,251 control recipients showed significantly lower mortality. The absolute risk difference at 10 years post-transplant was 20.3 (98.75% confidence interval, 0.9 to 39.8) and 19.8 (98.75% confidence interval, 19.2 to 20.4) percentage points in the sickle cell and control groups, respectively. The hazard ratio was also similar in the sickle cell (0.57; 95% confidence interval, 0.36 to 0.91) and control (0.54; 95% confidence interval, 0.53 to 0.55) groups (interaction P=0.8). Nonetheless, the sickle cell group was less likely to receive transplantation than the controls (subdistribution hazard ratio, 0.73; 95% confidence interval, 0.61 to 0.87). Similar disparities were found among waitlisted candidates (subdistribution hazard ratio, 0.62; 95% confidence interval, 0.53 to 0.72). CONCLUSIONS: Patients with sickle cell disease-associated kidney failure exhibited similar decreases in mortality associated with kidney transplantation as compared with those with other kidney failure etiologies. Nonetheless, the sickle cell population was less likely to receive transplantation, even after waitlist registration.

Comparison of US Federal and Foundation Funding of Research for Sickle Cell Disease and Cystic Fibrosis and Factors Associated With Research Productivity.

Importance: Sickle cell disease (SCD) and cystic fibrosis (CF) are severe autosomal recessive disorders associated with intermittent disease exacerbations that require hospitalizations, progressive chronic organ injury, and substantial premature mortality. Research funding is a limited resource and may contribute to health care disparities, especially for rare diseases that disproportionally affect economically disadvantaged groups. Objective: To compare disease-specific funding between SCD and CF and the association between funding and research productivity. Design, Setting, and Participants: This cross-sectional study examined federal and foundation funding, publications indexed in PubMed, clinical trials registered in ClinicalTrials.gov, and new drug approvals from January 1, 2008, to December 31, 2018, in an estimated US population of approximately 90 000 individuals with SCD and approximately 30 000 individuals with CF. Main Outcomes and Measures: Federal and foundation funding, publications indexed in PubMed, clinical trial registrations, and new drug approvals. Results: From 2008 through 2018, federal funding was greater per person with CF compared with SCD (mean [SD], $2807 [$175] vs $812 [$147]; P < .001). Foundation expenditures were greater for CF than for SCD (mean [SD], $7690 [$3974] vs $102 [$13.7]; P < .001). Significantly more research articles (mean [SD], 1594 [225] vs 926 [157]; P < .001) and US Food and Drug Administration drug approvals (4 vs 1) were found for CF compared with SCD, but the total number of clinical trials was similar (mean [SD], 27.3 [6.9] vs 23.8 [6.3]; P = .22). Conclusions and Relevance: The findings show that disparities in funding between SCD and CF may be associated with decreased research productivity and novel drug development for SCD. Increased federal and foundation funding is needed for SCD and other diseases that disproportionately affect economically disadvantaged groups to address health care disparities.

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.

Correction to: Do Words Matter? Stigmatizing Language and the Transmission of Bias in the Medical Record.

In the HTML version of this paper, Ms. Goddu was incorrectly listed. The correct citation should be AP Goddu. Exposure to the stigmatizing language note was associated with more negative attitudes towards the patient (20.3 stigmatizing vs. 25.1 neutral, p < 0.001). Reading the stigmatizing language note was associated with less aggressive management of the patient's pain (4.7 stigmatizing vs. 5.3 neutral, p < 0.001).

Clinical Outcomes Associated With Sickle Cell Trait: A Systematic Review.

Background: Although sickle cell trait (SCT) is largely a benign carrier state, it may increase risk for certain clinical outcomes. Purpose: To evaluate associations between SCT and clinical outcomes in children and adults. Data Sources: English-language searches of PubMed, CINAHL, the Cochrane Library, Current Contents Connect, Scopus, and Embase (1 January 1970 to 30 June 2018) and bibliographies of review articles. Study Selection: Observational controlled studies (published in English) in children or adults that examined an association between SCT and any of 24 clinical outcomes specified a priori in the following 6 categories: exertion-related injury; renal, vascular, pediatric, and surgery- or trauma-related outcomes; and overall mortality. Data Extraction: A single reviewer extracted study data, which was checked by another; 2 reviewers independently assessed study quality; and strength of evidence was assessed by consensus. Data Synthesis: Of 7083 screened studies, 41 met inclusion criteria. High-strength evidence supported a positive association between SCT and risk for pulmonary embolism, proteinuria, and chronic kidney disease. Moderate-strength evidence supported a positive association between SCT and exertional rhabdomyolysis and a null association between SCT and deep venous thrombosis, heart failure or cardiomyopathy, stroke, and pediatric height or weight. Absolute risks for thromboembolism and rhabdomyolysis were small. For the remaining 15 clinical outcomes, data were insufficient or strength of evidence was low. Limitation: Publication bias was possible, and high-quality evidence was scant. Conclusion: Sickle cell trait is a risk factor for a few adverse health outcomes, such as pulmonary embolism, kidney disease, and exertional rhabdomyolysis, but does not seem to be associated with such complications as heart failure and stroke. Insufficient data or low-strength evidence exists for most speculated complications of SCT. Primary Funding Source: National Human Genome Research Institute.

Increased acute care utilization in a prospective cohort of adults with sickle cell disease.

The ESCAPED (Examining Sickle Cell Acute Pain in the Emergency vs Day Hospital) trial is an ongoing prospective study comparing outcomes of people with sickle cell disease (SCD) seeking care for acute pain management in either an emergency department or specialty infusion clinic. The objective of this paper is to describe the baseline characteristics and health care utilization of patients in the trial. This is a multicenter study across 4 US cities that enrolled all adults with SCD living within 60 miles (96.6 km) of a study site who were expected to have acute care utilization over the study period. Twenty-one percent of participants had no acute care visits in the first 12 months of follow-up. Using negative binomial regression, we describe subject characteristics that predict acute care utilization. Three hundred ninety-one subjects have completed 12 months of follow-up with a mean age of 34.5 years (standard deviation, 11.4), 60% are female. Fifty-four percent of subjects with hemoglobin SS disease and 46% with hemoglobin SC disease had 3 or more acute visits over the study period. The prevalence of chronic pain in this cohort was 68%. Predictors of higher rates of acute care utilization included being unemployed, having chronic pain, being on chronic transfusion therapy, having a history of stroke, and being on disability or on Medicaid. This is the first prospective cohort in the modern era, and it demonstrates much higher rates of acute care utilization than reported in the Cooperative Study of Sickle Cell Disease.

Do Words Matter? Stigmatizing Language and the Transmission of Bias in the Medical Record.

BACKGROUND: Clinician bias contributes to healthcare disparities, and the language used to describe a patient may reflect that bias. Although medical records are an integral method of communicating about patients, no studies have evaluated patient records as a means of transmitting bias from one clinician to another. OBJECTIVE: To assess whether stigmatizing language written in a patient medical record is associated with a subsequent physician-in-training's attitudes towards the patient and clinical decision-making. DESIGN: Randomized vignette study of two chart notes employing stigmatizing versus neutral language to describe the same hypothetical patient, a 28-year-old man with sickle cell disease. PARTICIPANTS: A total of 413 physicians-in-training: medical students and residents in internal and emergency medicine programs at an urban academic medical center (54% response rate). MAIN MEASURES: Attitudes towards the hypothetical patient using the previously validated Positive Attitudes towards Sickle Cell Patients Scale (range 7-35) and pain management decisions (residents only) using two multiple-choice questions (composite range 2-7 representing intensity of pain treatment). KEY RESULTS: Exposure to the stigmatizing language note was associated with more negative attitudes towards the patient (20.6 stigmatizing vs. 25.6 neutral, p < 0.001). Furthermore, reading the stigmatizing language note was associated with less aggressive management of the patient's pain (5.56 stigmatizing vs. 6.22 neutral, p = 0.003). CONCLUSIONS: Stigmatizing language used in medical records to describe patients can influence subsequent physicians-in-training in terms of their attitudes towards the patient and their medication prescribing behavior. This is an important and overlooked pathway by which bias can be propagated from one clinician to another. Attention to the language used in medical records may help to promote patient-centered care and to reduce healthcare disparities for stigmatized populations.

Symptomatic Avascular Necrosis: An Understudied Risk Factor for Acute Care Utilization by Patients with SCD.

OBJECTIVES: Sickle cell disease (SCD) is associated with high healthcare utilization rates and poor outcomes in a subset of patients, although the underlying factors that predict this phenotype are poorly understood. Prior studies suggest that comorbid avascular necrosis (AVN) contributes to high healthcare utilization. We sought to clarify whether AVN independently predicts acute care utilization in adults with SCD and to identify characteristics of those with AVN that predict higher utilization. METHODS: We reviewed the medical records of 87 patients with SCD with symptomatic AVN and compared acute care utilization and clinical characteristics with 87 sex- and age-matched patients with SCD without symptomatic AVN. Patients with ≥2 years of follow-up were included. Outcomes were compared using bivariate analysis and multivariate regression. RESULTS: Our study included 1381 follow-up years, with a median of 7 years per patient. The AVN cohort had greater median rates of urgent care visits (3.2/year vs 1.3/year; P = 0.0155), admissions (1.3/year vs 0.4/year; P = 0.0002), and admission days (5.1 days/year vs 1.8 days/year; P = 0.0007). History of high utilization (odds ratio [OR] 4.28; P = 0.001), acute chest syndrome (OR 3.12; P = 0.005), pneumonia (OR 3.20; P = 0.023), hydroxyurea therapy (OR 2.23; P = 0.0136), and long-term transfusion (OR 2.33; P = 0.014) were associated with AVN. In a median regression model, AVN, acute chest syndrome, and pneumonia were independently associated with greater urgent care visits and admissions. CONCLUSIONS: Symptomatic AVN was found to be an independent risk factor for acute care utilization in patients with SCD. Because this is a potentially modifiable factor, further studies are urgently needed to determine whether AVN prevention/early treatment interventions will alter utilization and improve outcomes for patients with SCD.

Utility of the Montreal Cognitive Assessment as a Screening Test for Neurocognitive Dysfunction in Adults with Sickle Cell Disease.

OBJECTIVES: Neurocognitive dysfunction is an important complication of sickle cell disease (SCD), but little is published on the utility of screening tests for cognitive impairment in people with the disease. The purpose of this study was to evaluate the Montreal Cognitive Assessment (MoCA) as a screening tool and identify predictors of MoCA performance in adults with sickle cell disease. METHODS: We conducted a retrospective, cross-sectional study of the first 100 adult patients with SCD who completed the MoCA as part of routine clinical care at the Johns Hopkins Sickle Cell Center for Adults. We abstracted demographic, laboratory, and clinical data from each participant's electronic medical record up to the date that the MoCA was administered. The factorial validity of each MoCA domain was analyzed using standard psychometric statistics. We evaluated the abstracted data for associations with the composite MoCA score and looked for independent predictors of performance using multivariable regressions. RESULTS: Components of the MoCA performed well in psychometric analyses and identified deficits in executive function that were described in other studies. Forty-six percent of participants fell below the cutoff for mild cognitive impairment. Increased education was an independent predictor of increased MoCA score (3.1, 95% confidence interval [CI] 1.5-4.7), whereas cerebrovascular accidents and chronic kidney disease were independent predictors of decreased score (-3.3, 95% CI -5.7 to -0.97 and -3.2, 95% CI -6.2 to -0.11, respectively). When analysis was restricted to patients with SCA, increased education (3.7, 95% CI 2.2-5.2) and a history of hydroxyurea therapy (2.0, 95% CI -0.022 to 4.0) were independent predictors of a higher score, whereas chronic kidney disease (-3.3, 95% CI -6.4 to -0.24) and increased aspartate transaminase (-0.045, 95% CI -0.089 to -0.0010) were independent predictors of a decreased score. CONCLUSIONS: The MoCA showed promise by identifying important cognitive deficits and associations with chronic complications and therapy.

Effect of Free Dental Services on Individuals with Sickle Cell Disease.

OBJECTIVES: Poor oral health can have a negative impact on overall health. This is especially concerning for individuals with sickle cell disease (SCD), an inherited blood disorder that affects hemoglobin and can lead to an increased risk of infection and hyperalgesia. Because the majority of individuals with SCD have Medicaid insurance and no dental coverage, we provided free basic dental care to individuals with SCD to determine whether it decreased overall healthcare utilization. METHODS: Through a contract with a private dental office, we provided free basic dental care (eg, cleanings, fillings, x-rays) to individuals with SCD. We reviewed medical records for the 12 months before and after their initial dental visit to determine whether there were any changes in acute care visits (defined as a visit to the emergency department, sickle cell infusion center, or visits to both in the same day), hospitalizations, and total days hospitalized. We conducted a negative binomial regression to determine any differences in the pre-post periods. RESULTS: In our multivariable analysis, there was a statistically significant decrease in hospital admissions. In addition, there was a significant decrease in total days hospitalized if dental work was completed, but an increase in days hospitalized in men. CONCLUSIONS: Providing dental care to individuals with SCD who did not have dental insurance did not greatly alter acute care visits. A larger sample size may be necessary to observe an effect.

Examination of the Patient and Hospitalization Characteristics of 30-Day SCD Readmissions.

OBJECTIVES: Sickle cell disease (SCD) is associated with a high level of emergency department and hospital utilization, as well as a high rate of hospital readmissions. At Johns Hopkins Hospital, as at other institutions, SCD accounts for a large proportion of readmissions. Our study examined patient and hospitalization factors involved in readmissions at Johns Hopkins Hospital. METHODS: Patients at the Johns Hopkins Sickle Cell Center for Adults with a readmission in fiscal year 2011 were compared with an age- and sex-matched sample of clinic patients for comorbidities, complications, and prior utilization. Hospitalizations that were followed by readmissions were compared with those that were not as to admitting service, length of stay, and average daily opioid dose. RESULTS: Patients with readmissions had more complications and comorbidities and much higher prior utilization than typical clinic patients, whereas hospitalizations that were followed by readmissions had a longer length of stay but similar opioid doses. CONCLUSIONS: For patients with SCD with a high volume of hospital use, readmissions may be a natural consequence of a high-admission frequency associated with greater disease severity and higher comorbidity.

Chronic Opioid Therapy and Central Sensitization in Sickle Cell Disease.

Chronic opioid therapy (COT) for chronic non-cancer pain is frequently debated, and its effectiveness is unproven in sickle cell disease (SCD). The authors conducted a descriptive study among 83 adult SCD patients and compared the severity of disease and pain symptoms among those who were prescribed COT (n=29) with those who were not using COT. All patients completed baseline laboratory pain assessment and questionnaires between January 2010 and June 2014. Thereafter, participants recorded daily pain, crises, function, and healthcare utilization for 90 days using electronic diaries. Analyses were conducted shortly after the final diary data collection period. Patients on COT did not differ on age, sex, or measures of disease severity. However, patients on COT exhibited greater levels of clinical pain (particularly non-crisis); central sensitization; and depression and increased diary measures of pain severity, function, and healthcare utilization on crisis and non-crisis diary days, as well as a greater proportion of days in crisis. Including depressive symptoms in multivariate models did not change the associations between COT and pain, interference, central sensitization, or utilization. Additionally, participants not on COT displayed the expected positive relationship between central sensitization and clinical pain, whereas those on COT demonstrated no such relationship, despite having both higher central sensitization and higher clinical pain. Overall, the results point out a high symptom burden in SCD patients on COT, including those on high-dose COT, and suggest that nociceptive processing in SCD patients on COT differs from those who are not.

Psychometric Validation of the Insomnia Severity Index in Adults with Sickle Cell Disease.

BACKGROUND: The Insomnia Severity Index (ISI) is an instrument to evaluate insomnia symptoms. The psychometric properties have not been established in adults (18 years of age or older) with sickle cell disease (SCD). OBJECTIVE: Evaluate the reliability and validity of the ISI among adults with SCD. METHODS: Analysis included psychometric evaluation with exploratory factor analysis. RESULTS: Our 263 participants had a mean age of 35.6 years and primarily were female (54.8%) with HbSS genotype (69.2%). Almost 41% were classified as clinical insomnia cases (ISI ≥14) using the traditional scoring approach. Two factors, Insomnia Symptoms and Insomnia Impact, emerged during factor analysis. Reliability of both factor-scales was good and each correlated with pain severity and depressive symptomatology (r = 0.38 to 0.66, p<.01). CONCLUSION: The ISI demonstrated construct validity and reliability for evaluating insomnia symptomatology among adults with SCD and can be used in research and clinical practice.

Racial differences in acute kidney injury of hospitalized adults with diabetes.

OBJECTIVE: To determine whether there is a racial difference in the risk of acute kidney injury between hospitalized black and white adults with diabetes mellitus in the United States RESEARCH DESIGN AND METHODS: We analyzed cross-sectional data from the 2000-2010 National Hospital Discharge Survey (NHDS) to compare the odds of AKI among hospitalized black and white adults with diabetes. After excluding records in which race status was missing, race was other than white or black, discharge status was not provided, or end-stage renal disease was a diagnosis, we identified 276,138 eligible records for analysis. Multivariable logistic regression was used to analyze the association between race, AKI, and in-hospital mortality. Multivariable linear regression was used to analyze the association between length of stay and race among discharge records with a diagnosis of AKI. RESULTS: In this nationally representative sample of hospitalized U.S. adults with diabetes, blacks had a 50% higher age- and sex-adjusted odds of AKI compared to whites (odds ratio: 1.51; 95% CI 1.37-1.66). The association between black race and increased risk of AKI persisted after additional adjustment for multiple AKI-related risk factors, including chronic kidney disease, sepsis, hypertension, hypotension, length of stay, myocardial infarction, congestive heart failure, angiography, computed tomography scan, cirrhosis, admission source, payor source, hospital region, and hospital bed size (OR 1.71; 95% CI, 1.31-2.25). Among cases of AKI, there was no racial difference in length of stay or in-hospital mortality. CONCLUSIONS: Among hospitalized adults in the U.S. with diabetes, black race is associated with a higher risk of AKI compared to white race.

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.

An Evaluation of Central Sensitization in Patients With Sickle Cell Disease.

UNLABELLED: Central sensitization (CS), nociceptive hyperexcitability known to amplify and maintain clinical pain, has been identified as a leading culprit responsible for maintaining pain in several chronic pain conditions. Recent evidence suggests that it may explain differences in the symptom experience of individuals with sickle cell disease (SCD). Quantitative sensory testing (QST) can be used to examine CS and identify individuals who may have a heightened CS profile. The present study categorized patients with SCD on the basis of QST responses into a high or low CS phenotype and compared these groups according to measures of clinical pain, vaso-occlusive crises, psychosocial factors, and sleep continuity. Eighty-three adult patients with SCD completed QST, questionnaires, and daily sleep and pain diaries over a 3-month period, weekly phone calls for 3 months, and monthly phone calls for 12 months. Patients were divided into CS groups (ie, no/low CS [n = 17] vs high CS [n = 21]), on the basis of thermal and mechanical temporal summation and aftersensations, which were norm-referenced to 47 healthy control subjects. High CS subjects reported more clinical pain, vaso-occlusive crises, catastrophizing, and negative mood, and poorer sleep continuity (Ps < .05) over the 18-month follow-up period. Future analyses should investigate whether psychosocial disturbances and sleep mediate the relationship between CS and pain outcomes. PERSPECTIVE: In general, SCD patients with greater CS had more clinical pain, more crises, worse sleep, and more psychosocial disturbances compared with the low CS group.

Improving Emergency Providers' Attitudes Toward Sickle Cell Patients in Pain.

BACKGROUND: Provider biases and negative attitudes are recognized barriers to optimal pain management in sickle cell disease, particularly in the emergency department (ED). MEASURES: This prospective cohort measures preintervention and postintervention providers' attitudes toward patients with sickle pain crises using a validated survey instrument. INTERVENTION: ED providers viewed an eight-minute online video that illustrated challenges in sickle cell pain management, perspectives of patients and providers, as well as misconceptions and stereotypes of which to be wary. OUTCOMES: Ninety-six ED providers were enrolled. Negative attitude scoring decreased, with a mean difference -11.5 from baseline, and positive attitudes improved, with a mean difference +10. Endorsement of red-flag behaviors similarly decreased (mean difference -12.8). Results were statistically significant and sustained on repeat testing three months after intervention. CONCLUSIONS/LESSONS LEARNED: Brief video-based educational interventions can improve emergency providers' attitudes toward patients with sickle pain crises, potentially curtailing pain crises early, improving health outcomes and patient satisfaction scores.

Acute Pain and Depressive Symptoms: Independent Predictors of Insomnia Symptoms among Adults with Sickle Cell Disease.

No studies to date have systematically investigated insomnia symptoms among adults with sickle cell disease (SCD). The purpose of this study was to (1) describe the prevalence of insomnia symptoms and (2) identify biopsychosocial predictors in community-dwelling adults with SCD. Cross-sectional analysis of baseline data from 263 African American adults with SCD (aged 18 years or older). Measures included the Insomnia Severity Index (ISI), Center for Epidemiologic Studies in Depression scale, Urban Life Stress Scale, Brief Pain Inventory, and a chronic pain item. SCD genotype was extracted from the medical record. A slight majority (55%) of the sample reported clinically significant insomnia symptomatology (ISI ≥ 10), which suggests that insomnia symptoms are prevalent among community-dwelling African American adults with SCD. While insomnia symptoms were associated with a number of biopsychosocial characteristics, depressive symptoms and acute pain were the only independent predictors. Given the high number of participants reporting clinically significant insomnia symptoms, nurses should screen for insomnia symptoms and explore interventions to promote better sleep among adults with SCD, with an emphasis on recommending treatment for pain and depression. In addition, current pain and depression interventions in this population could add insomnia measures and assess the effect of the intervention on insomnia symptomatology as a secondary outcome.

Quantitative sensory testing and pain-evoked cytokine reactivity: comparison of patients with sickle cell disease to healthy matched controls.

Sickle cell disease (SCD) is an inherited blood disorder associated with significant morbidity, which includes severe episodic pain, and, often, chronic pain. Compared to healthy individuals, patients with SCD report enhanced sensitivity to thermal detection and pain thresholds and have altered inflammatory profiles, yet no studies to date have examined biomarker reactivity after laboratory-induced pain. We sought to examine this relationship in patients with SCD compared to healthy control participants. We completed quantitative sensory testing in 83 patients with SCD and sequential blood sampling in 27 of them, whom we matched (sex, age, race, body mass index, and education) to 27 healthy controls. Surprisingly, few quantitative sensory testing differences emerged between groups. Heat pain tolerance, pressure pain threshold at the trapezius, thumb, and quadriceps, and thermal temporal summation at 45°C differed between groups in the expected direction, whereas conditioned pain modulation and pain ratings to hot water hand immersion were counterintuitive, possibly because of tailoring the water temperature to a perceptual level; patients with SCD received milder temperatures. In the matched subsample, group differences and group-by-time interactions were observed in biomarkers including tumor necrosis factor alpha, interleukin-1ß, interleukin-4, and neuropeptide Y. These findings highlight the utility of laboratory pain testing methods for understanding individual differences in inflammatory cytokines. Our findings suggest amplified pain-evoked proinflammatory cytokine reactivity among patients with SCD relative to carefully matched controls. Future research is warranted to evaluate the impact of enhanced pain-related cytokine response and whether it is predictive of clinical characteristics and the frequency/severity of pain crises in patients with SCD.