Sickle Cell Disease

Abstract
Background
Sickle cell disease (SCD) is an inherited blood disorder associated with acute pain crisis and other complications that lead to frequent emergency department (ED) visits. To improve outcomes, the National Heart, Lung and Blood Institute (NHLBI) published recommendations for management of acute pain crisis. NHLBI also funded eight centers across the United States to participate in the Sickle Cell Disease Implementation Consortium. This six-year effort consists of two phases. Phase one involved conducting needs assessments of barriers and facilitators to SCD care. The aim of this study was to describe challenges and facilitators to caring for SCD from the perspective of ED providers in central North Carolina (NC).

Methods and findings
We conducted a needs assessment survey with ED providers throughout NC. We also conducted focus groups and an interview with ED providers from three healthcare facilities in central NC. One hundred and eleven surveys (60.6% physicians, 26% registered nurses, 13.5% physician assistants) were completed and 13 providers participated in 3 focus groups and 1 interview. Slightly more than half (50. 4%) utilized individualized dosing protocols to treat sickle cell pain. Only 32.4% of the providers were aware of the NHLBI SCD recommendations. Barriers to care from the survey included: patient behavior (67.57%), the opioid epidemic (67.57%), overcrowding (64.86%), and concern about addiction (49.55%). Perceived barriers to care identified in the focus groups and interview included: high patient volumes, lack of SCD care protocols, poor communication among providers and stigma. Facilitators to care included: individualized pain plans, comfort prescribing opioids and electronic medical records.

Conclusion
SCD care is influenced by many factors. Our results illuminate the need for increased use of the NHLBI SCD recommendations, individualized pain protocols, and use of electronic medical records and other care-interventions, specifically geared towards improving provider knowledge and mitigating provider bias.

Abstract
Study objective
Individuals living with sickle cell disease (SCD) often require urgent care; however, some patients hesitate to present to the emergency department (ED), which may increase the risk of serious clinical complications. Our study aims to examine psychosocial, clinical, and demographic factors associated with delaying ED care.

Methods
This was a cross-sectional study of 267 adults with SCD from the national INSIGHTS Study. The binary outcome variable asked whether, in the past 12 months, participants had delayed going to an ED when they thought they needed care. Logistic regression was performed with clinical, demographic, and psychosocial measures.

Results
Approximately 67% of the participants reported delaying ED care. Individuals who delayed care were more likely to have reported higher stigma experiences (odds ratio [OR]=1.09; 95% confidence interval [CI] 1.03 to 1.16), more frequent pain episodes (OR=1.15; 95% CI 1.01 to 1.32), lower health care satisfaction (OR= 0.74; 95% CI 0.59 to 0.94), and more frequent ED visits (OR=6.07; 95% CI 1.18 to 31.19). Disease severity and demographics, including sex, age, and health insurance status, were not significantly associated with delay in care.

Conclusion
Psychosocial factors, including disease stigma and previous negative health care experiences, are associated with delay of ED care in this SCD cohort. There is a need to further investigate the influence of psychosocial factors on the health care–seeking behaviors of SCD patients, as well as the downstream consequences of these behaviors on morbidity and mortality. The resulting knowledge can contribute to efforts to improve health care experiences and patient-provider relationships in the SCD community.

Abstract
Objective: To assess disparities in the quality of healthcare provider communication experienced by African-American adults with and without sickle cell disease (SCD) in the U.S.

Methods: Poor provider communication was assessed by the Provider Communication subscale of the Consumer Assessment of Healthcare Plans and Systems survey. The SCD sample was obtained from participants in a multicenter observational study of healthcare experiences. The national African-American sample data was obtained from published national estimates.

Results: The SCD sample was more likely than the national sample to report poor communication in 3 out of 4 communication domains: listening (22.3% vs. 11.5%, p<0.0001); showing respect (26.1% vs. 9.5%, p<0.0001); and spending enough time (38.3% vs. 16.2%, p<0.0001). Differences were consistent in young, but not old, patients and showed some variation by self-reported health status and education.

Conclusions: The communication difficulties experienced by persons with SCD do not appear reducible to their predominantly African-American race, but may result from more disease-specific factors.

Practice implications: Healthcare providers should take particular care in recognizing and demonstrating recommended communication skills with SCD patients as these patients may be particularly vulnerable to, and cognizant of, poor quality interactions.

Abstract
Background: Sickle cell disease (SCD) is a complex chronic disease requiring multidisciplinary care that involves primary care physicians (PCPs) working with a hematologist or SCD specialists. However, PCPs often lack access to SCD specialists and are unaware of SCD guidelines or efficacious treatment.

Methods: We partnered with Community Care of North Carolina (CCNC) to identify assigned PCPs for SCD patients with Medicaid across North Carolina. CCNC network administrators distributed a web-based questionnaire for completion. The questionnaire involved 12 self-reported items on a yes-no or a 1 to 5 Likert-type scale that assessed PCP attitudes toward SCD care, awareness of recent guidelines, and comanaging hydroxyurea.

Results: Of the 53 PCPs who completed the electronic survey, 73% felt they were comfortable with the number of SCD patients in their practice. Most PCPs reported having infrequent communications with an SCD specialist (67%) and most were also not aware of the 2014 SCD guidelines (66%). Many reported that they would frequently use the new SCD guidelines if provided to them (76%). Furthermore, 51% of PCPs expressed comfort with using mobile apps to access SCD guidelines and provided email contact to receive further information. The majority also reported being comfortable comanaging hydroxyurea with an SCD specialist (65%).

Conclusion: Few PCPs in North Carolina were aware of the new SCD guidelines or had regular communication with an SCD specialist. The majority of PCPs, however, demonstrated a favorable attitude toward receiving the SCD guidelines and comanaging hydroxyurea with a specialist. In response to this gap in care, we have developed a mobile-based SCD toolbox specifically for PCPs to provide guidelines, algorithms, and a method to communicate with local SCD specialists. With the interest in receiving these guidelines, we are confident the toolbox will provide an easy to use platform to assist PCPs to utilize the SCD guidelines.