ALBERT

Description: INTRODUCTION Hemoglobinopathies, such as sickle cell disease (SCD) and thalassemia, are chronic blood disorders with a profound impact on a patient's life expectancy and quality. Patients often come from marginalized populations with variable health literacy. Prior studies have demonstrated improved outcomes in chronic illness as a result of empowering patients through various education strategies. This systematic review investigates the current state of patient education for SCD and thalassemia with the goal of elucidating which strategies have been effective in improving patient knowledge and/or ability to cope with illness. METHODS Literature was searched up to August 2018 in Medline, PsycINFO, Scopus, CINAHL, Cochrane Central, Cochrane Database of Systematic Reviews, EMBASE, Emcare, and the International Clinical Trials Registry Platform (ICTRP). Randomized controlled trials (RCTs) examining the effects of patient education on patients aged 18 years and older or transitioning into adulthood were included. Psychological interventions were excluded. Preliminary analysis focused on three outcomes: disease knowledge, self-efficacy, and coping ability. Meta-analysis was performed using RevMan 5.3. A random effects model with the inverse variance method was chosen. Standardized mean difference (SMD) was calculated for continuous outcomes due to the different scales used by each study. Changes from baseline with missing standard deviations were imputed from known standard deviations from other studies (Higgins 2011). Heterogeneity was evaluated using the I2 statistic. Domain-based approach was used to assess risk of bias (Higgins 2011). RESULTS The search yielded 12173 citations, 3575 were duplicates. Two reviewers screened the title and abstracts for full text retrieval. Of the 28 full-text citations reviewed, 8 met the inclusion criteria, representing 6 unique RCTs. Of the 6 RCTs, 2 were at high risk of attrition bias. The other domains generally exhibited an unclear risk of bias. Improvement in patient knowledge was a measured outcome in 3 RCTs totalling 351 participants with SCD, mostly of African-American descent. Standard deviations (SD) were lacking in the baseline and 6 month knowledge questionnaire results from Krishnamurti 2018. Attempts to impute the SDs were unsuccessful. Meta-analysis was conducted in the remaining 2 RCTs (n=274). Statistically significant SMD of 0.39 (95% CI 0.05 to 0.72, p=0.02, Figure 1A) in favour of the intervention group with low heterogeneity (I2=24%). These interventions were delivered as either a web-based multimedia education program or in a classroom setting. An increase in self-efficacy was reported in 2 RCTs (n=67) with a statistically significant SMD of 0.72 (95% CI 0.22 to 1.22, p=0.005, Figure 1B) in favour of patient education with no observed statistical heterogeneity (I2=0). Coping ability was reported in 2 RCTs (n=107) but one study was excluded from analysis due to lack of sufficient randomization data. For the remaining study (n=40), the SMD at 0.16 was not statistically significant (95% CI -0.46 to 0.78, p=0.60, Figure 1C). CONCLUSIONS This review summarizes the current state of patient education for adult hemoglobinopathy patients in which some efforts have been made for SCD but no comparable interventions exist for thalassemia. Interventions demonstrated a significant effect on improvement in knowledge and self-efficacy. However, this effect is of uncertain clinical significance due to lack of reporting on clinically relevant illness and healthcare utilization-related outcomes. Only one RCT adequately reported on improvement in coping ability with no statistical significance. Given these results, there is currently insufficient evidence to determine what intervention strategies might benefit adult patients with hemoglobinopathies. This review is limited by the paucity of high-quality RCTs addressing the impact of patient education on self-management. Notably, a considerable volume of studies that employ a non-RCT design were identified and also warrant appropriate analysis. Rigorously designed trials are needed to draw clinically-relevant conclusions regarding type and effectiveness of patient education for adult hemoglobinopathy patients. REFERENCE: Higgins JPT, Green S (eds.). Cochrane Handbook for Systematic Reviews of Interventions Version 5.1.0. The Cochrane Collaboration, 2011. Disclosures Kuo: Agios: Consultancy; Alexion: Consultancy, Honoraria; Apellis: Consultancy; Bioverativ: Other: Data Safety Monitoring Board; Bluebird Bio: Consultancy; Celgene: Consultancy; Novartis: Consultancy, Honoraria; Pfizer: Consultancy.

Description: Background: Sickle Cell Disease (SCD) patients are amongst the most frequent utilizers of the emergency department (ED) for acute vaso-occlusive pain episodes ("crisis"). Unfortunately, ED SCD care often fails to meet guideline recommendations. This results in preventable morbidity and mortality as well as substantial resource utilization. Current care interventions across multiple domains have not led to sustained improvements, likely due in part to negative attitudes and beliefs among health care providers (HCP). Novel mechanisms of physician education are required to improve outcomes. An emerging method of education in SCD is the use of patients as "just in time" teachers. We refer to this as Patient Directed Physician Education (PDPE) and define it as a broad range of activities in which patients relay actionable information to HCPs in the clinical setting. While there is a growing body of research supporting patients as teachers, no group has specifically examined point of care PDPE in a SCD population. Objective: This study seeks to understand the current use and feasibility of SCD PDPE in the ED. Methods: This is a multi-centered, prospective descriptive qualitative design with semi-structured, audio taped individual interviews. SCD patients and ED physicians across Ontario, Canada were recruited to participate in the study through purposeful sampling. Qualitative analysis was performed using phenomenological inquiry, which encompasses qualitative approaches to inductively and holistically understand a human phenomenon (i.e. teaching and learning in sickle cell disease) in a context specific setting. Results: In total, 11 patients and 8 physicians participated in the study. Patient ages ranged from 21-67 and physician years in practice ranged from 3-21. The patient group predominately consisted of female Black Canadians (81%) while the physician group was mainly Caucasian males (63%) but did include representation from other ethnicities. There was significant variability in both self-described disease severity (patients) and frequency of SCD exposure (physicians). Both groups assessed themselves as having good to excellent SCD general knowledge or clinical acumen respectively. Within the qualitative analysis both groups responded favourably to PDPE and expressed confidence in its feasibility and ability to improve ED care. Barriers to the implementation of PDPE were expressed across three major domains; 1) patient factors, 2) health care worker factors, and 3) health care system factors. The ability of patients to present reliable health information was highlighted as a key factor in the success of PDPE by both groups. However, physicians and patients had strikingly different perspectives towards the inherent challenges. Patients identified triage delays and difficulty speaking due to intractable pain as the major barriers to shared decision making. Conversely, physicians largely did not appreciate these elements of the vaso-occlusive pain episode experience and instead expressed concerns regarding patient credibility, particularly in the context of high dose opiates. Overt racism or the perception of patient as "drug seeker" were not identified as major themes from either group. There was consensus towards written patient care plans, corroborating electronic health records, and collaboration from SCD experts as mechanisms to bridge the SCD patient/ED physician divide and facilitate PDPE. In addition, despite not being directly involved in PDPE, nurses experienced in SCD management were consistently noted to be instrumental in supporting the patient-physician interaction. Conclusions: Patient directed physician education is a feasible and novel SCD care innovation with support from both patients and ED physicians. Successful implementation will require credible educational adjuncts (e.g. patient pocket cards, care plans) and support from ED nurses and SCD experts. Our findings will serve as part of a continuum of research to engage patients as partners in improving SCD knowledge and care outcomes. Disclosures No relevant conflicts of interest to declare.