Sickle cell disease (SCD) is associated with disproportionate emergency department (ED) use. This study described the social determinants of health associated with ED visits and hospital admission from the ED among children with SCD using a nationally representative dataset. We analyzed data from 126 children 0 to 17 years of age with SCD included in the 2011 to 2017 rounds of the National Health Interview Survey (mean age, 8 y; 50% female individuals; 74% African American). Study variables were summarized using weighted means and proportions and compared according to ED use and admission by Wald tests. Fifty-two identified children had visited the ED within the last 12 months and 21 were admitted to the hospital after their most recent ED visit. Children living in a single-mother household were more likely to visit the ED (P=0.040), as were younger children (mean age, 6 vs. 9 y; P=0.034), with no evaluated social determinants of health significantly impacting hospital admission from the ED. The lack of association between ED use and either poverty or insurance type may be related to the overall high level of social disadvantage among children with SCD. Our findings demonstrate the need to better characterize specific social factors impacting acute care use among children with SCD. Abstract source

Objective: These evidence-based guidelines developed by the American Society of Hematology (ASH) are intended to support patients, clinicians, and other health care professionals in pain management decisions for children and adults with SCD. Methods: ASH formed a multidisciplinary panel, including 2 patient representatives, that was thoroughly vetted to minimize bias from conflicts of interest. The Mayo Evidence-Based Practice Research Program supported the guideline development process, including updating or performing systematic reviews. Clinical questions and outcomes were prioritized according to importance for clinicians and patients. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used, including GRADE evidence-to-decision frameworks, to assess evidence and make recommendations, which were subject to public comment. Results: The panel reached consensus on 18 recommendations specific to acute and chronic pain. The recommendations reflect a broad pain management approach, encompassing pharmacological and nonpharmacological interventions and analgesic delivery. Conclusions: Because of low-certainty evidence and closely balanced benefits and harms, most recommendations are conditional. Patient preferences should drive clinical decisions. Policymaking, including that by payers, will require substantial debate and input from stakeholders. Randomized controlled trials and comparative-effectiveness studies are needed for chronic opioid therapy, nonopioid therapies, and nonpharmacological interventions. Abstract source

Objective: To evaluate the association between timely ED opioid administration and hospitalization rates in children with SCD. Methods: Retrospective cohort of children presenting to a children's hospital ED with SCD pain between January 1, 2014, and April 30, 2018. Visits were extracted using ICD codes, chief complaints, and receipt of at least one opioid, and then reviewed to confirm the visit was an uncomplicated pain crisis. The primary outcome was hospitalization, yes or no. Generalized estimating equations were used to determine adjusted odds of hospitalization for the timely administration of initial and second doses of opioids. Results: Of the 902 eligible visits, 368 (40.8%) resulted in hospitalization. The mean (SD) age was 11.9 (± 5.2) years. The first opioid was administered within 60 minutes of arrival in 601 (66.6%) visits. The second opioid was administered within 30 minutes of the first in 84 (12.3%) visits. Receipt of the first opioid within 60 minutes of arrival was not associated with decreased hospitalization (1.30 [0.96‐1.76]). However, receipt of the second dose within 30 minutes of the first was associated with decreased hospitalization (0.56 [0.33‐0.94]). Conclusion: This study suggests an association between children with SCD receiving a second dose within 30 minutes of the first opioid dose and decreased hospitalizations. Abstract source

Objective: In the United States, sickle cell disease (SCD) is the most prevalent genetic disorder affecting African American and Hispanic youth. Disparities in care have been reported for this population with adolescents and young adults (AYA) with SCD at increased risk for poor health outcomes. It is essential that AYA with SCD receive high-quality care and have access to disease-modifying therapies, yet engaging them in their care and clinical research studies to discover new treatments remains a challenge. Method: Our team developed an innovative approach to engage AYA with SCD in clinical care and research: the SCD Teen Symposium, which uses motivational interviewing strategies to educate AYA with SCD, their family members, and friends while providing concurrent opportunities for research participation. Results: Over a 6-year period, 81 AYA with SCD (47% male; Mage = 14.58, SD = 2.9) and 162 family and friends (48% male; Mage = 13.37, SD = 2.1) participated. Participants rated the symposium as acceptable (Mrating = 1.74, SD = 0.85) on a scale from 1 (excellent) to 5 (poor), and 72% reported they would recommend it to others. Research was rated as very important (Mrating = 1.21, SD = 0.46) by 81% of attendees on a scale from 1 (very important) to 4 (not important), and 82% reported increased trust in research. AYA suggested improvements including separate programming for younger and older teens. Conclusions: Results support the feasibility of the Teen Symposium as a model for increasing trust and potentially increasing participation in research. Future studies will determine whether it can be disseminated by other SCD clinics. Abstract source

Objective: Even with optimized medical care for the management of pain, many pediatric patients with sickle cell disease (SCD) report persistently high pain and have corresponding functional impairments. The Comfort Ability Program (CAP), a day-long pediatric pain management intervention, was adapted via a 4-phase knowledge translation process into a video-based intervention to specifically and flexibly address sickle cell pain (SCP). CAP for SCP introduces psychological and biobehavioral pain management techniques to adolescents with SCD and their parents or caregivers. A primary goal of our structured development process and feasibility testing was to ensure ease of access and delivery by addressing identified barriers to care that have historically limited widespread dissemination of evidence-based programs for patients with SCD. Method: The development of CAP for SCP was completed in 4 phases: (1) convening an expert panel to review the literature, discuss logistical and cultural barriers to care, and adapt the curriculum of a 1-day pain management workshop to a video-based format that could specifically meet the needs of a SCD population; (2) conducting a pilot testing of content with patients and parents followed by semistructured focus group discussion; (3) generating and producing the interactive video, audio materials, and corresponding workbook for intervention delivery; and (4) testing feasibility in both inpatient and outpatient clinics. Results: Results suggest that this intervention is highly acceptable to adolescents and parents and can be administered during an inpatient hospitalization or in conjunction with a routine outpatient appointment. Conclusion: CAP for SCP was created using an integrative approach to translational science and adhering to patient centered research practices. The result is a supportive, psychoeducational intervention offering an introduction to CBT skills and strategies for SCP management. Further research on CAP for SCP is needed. Abstract source

Background: The study investigated the economic burden of vaso-occlusive crisis (VOC) among sickle cell disease (SCD) patients, through assessment of overall utilization and costs and costs per VOC episode (regarding the number of VOC episodes and health care setting, respectively). Methods: Using the Medicaid Analytic Extracts database, the first SCD-related diagnosis claim (index claim) between June 1, 2009–December 31, 2012 was identified among eligible adults. Patients were required to have continuous medical and pharmacy benefits for 6 months pre- and 12 months postindex. Discrete VOC claims identified within a 3-day gap were combined as a single VOC episode. Annual all-cause and SCD-related medical resources and costs were identified and stratified by number of VOC episodes during the 1-year follow-up period. Health care costs per VOC episode were also examined, stratified by care setting. Results: Enrollees included 8521 eligible patients with a mean age of 32.88 years (SD=12.21). Of these, 66.5% had a Charlson Comorbidity index (CCI) score of 0 (no comorbidities) and 67.3% were female. The average total medical costs were US$34136 (median=US$12691) annually, and SCD accounted for 60% of the total costs (mean=US$20 206, median=US$1204). Patients with >3 episodes had the highest annual SCD-related costs (mean=US$58950) across all settings. Health care resource utilization (HCRU) and costs increased substantially as the number of VOC episodes increased. This study was limited to observation of associations rather than causal inference, and by possible coding and identification discrepancies and the restricted generalizability of the population. Conclusions: VOC has a severe impact on medical resource use and costs among the adult SCD population. Further research among broader study populations is needed to facilitate the reduction of VOC episodes and thereby improve clinical and economic outcomes for SCD patients. Abstract source

Older adults and people with underlying medical conditions remain at increased risk for severe illness, but now CDC has further defined age- and condition-related risks. As more information becomes available, it is clear that a substantial number of Americans are at increased risk of severe illness – highlighting the importance of continuing to follow preventive measures. “Understanding who is most at risk for severe illness helps people make the best decisions for themselves, their families, and their communities,” said CDC Director Robert Redfield MD. “While we are all at risk for COVID-19, we need to be aware of who is susceptible to severe complications so that we take appropriate measures to protect their health and well-being.” Abstract source

This spiritual intervention addresses the often-unspoken concerns of African American, 18-28 years, with Sickle Cell Disease (SCD). Understanding spirituality, while managing the many facets of SCD, is vital for holistic health. These participants find themselves at critical junctures in their spiritual development - seeking, exploring, even questioning - how spirituality may play a role in their overall well-being. To that end, participants met 1:1 with a chaplain over six encounters to share resources, skills and tools for increasing everyday coping and self-efficacy. This pilot came to realize that to truly be effective, additional core encounters are needed for true impact. Abstract source

Introduction: Knowledge on the characteristics of neuropathic pain in people with sickle cell disease (SCD) may help to provide more effective treatment procedures. Objective: To describe the characteristics of neuropathic pain in patients with sickle cell disease and identify the impact on their quality of life. Method: A cross-sectional study (CAAE 57274516.8.0000.5544) was conducted at a reference center in Salvador, Bahia, Brazil. The instruments used were the Brief Pain Inventory (BPI), the Douleur Neuropatique Questionnaire (DN-4), the Anxiety and Depression Hospital scale (ADH) and the abbreviated version of the World Health Organization of Quality of Life questionnaire (WHOQOL-brief). The Mann-Whitney test was used to evaluate the association between the scores (5% alpha). Results: A total of 100 adults with SCD participated in the study, 69.7% of whom had neuropathic pain. Anxiety was present in 99% of the sample and depression, in 100%. Patients with neuropathic pain had worse scores in all domains of quality of life (p < 0.05), but no association was found with pain intensity. Conclusion: Neuropathic pain was more frequent than nociceptive pain in adults with SCD and generated worse scores in all domains of quality of life. Anxiety and depression were present in patients with both types of pain. Abstract source

Objective: To test the hypothesis that caregivers' or adult participants' low ratings of provider communication are associated with more hospital admissions among adults and children with sickle cell disease (SCD), respectively. Secondarily, we determined whether there was an association between the caregivers' or participants' health literacy and rating of providers' communication. Methods: Primary data were collected from participants through surveys between 2014 and 2016, across six sickle cell centers throughout the U.S. In this cross-sectional cohort study, 211 adults with SCD and 331 caregivers of children with SCD completed surveys evaluating provider communication using the Consumer Assessment of Healthcare Providers and Systems (CAHPS), healthcare utilization, health literacy, and other sociodemographic and behavioral variables. Analyses included descriptive statistics, bivariate analyses, and logistic regression. Results: Participants with better ratings of provider communication were less likely to be hospitalized (odds ratio (OR) = 0.54, 95% confidence interval (CI) = [0.35, 0.83]). Positive ratings of provider communication were associated with fewer readmissions for children (OR = 0.23, 95% CI = [0.09, 0.57]). Participants with better ratings of provider communication were less likely to rate their health literacy as lower (regression coefficient (B) = -0.28, 95% CI = [-0.46, -0.10]). Conclusions: Low ratings of provider communication were associated with more hospitalizations and readmissions in SCD, suggesting the need for interventions targeted at improving patient-provider communication which could decrease hospitalizations for this population. Abstract source