scholarly journals Grapes: Trivia Game Increases Sickle Cell Disease Knowledge in Patients and Providers and Mitigates Health Biases

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 917-917
Author(s):  
Amy Ouyang ◽  
Manasa Gadiraju ◽  
Veda Gadiraju ◽  
Landon Power ◽  
Vinitha Gadiraju ◽  
...  

Abstract Introduction Pediatric patients with sickle cell disease (SCD) face racial bias and healthcare stigma compounded by limited age-appropriate health education. Due in part to a lack of dedicated SCD education, providers can hold misconceptions about SCD and biases against patients. Gamification improves disease education and mitigates stigma in various clinical settings. In this study, we created the GRAPES tool (Game to Raise Awareness for Patient/Provider/Public Education of SCD, www.tinyurl.com/GRAPESgame), a free, online, trivia-based educational game. We hypothesized that utilization of the GRAPES tool will improve patient and provider SCD knowledge and mitigate SCD stigma among providers. Methods The GRAPES tool is a single-player, interactive game developed using Figma, a collaborative user-interface design software. Multiple-choice questions are structured into five educational modules (science, treatment, epidemiology, myths, and symptoms) with customizable red blood cell characters and hand-drawn screens to promote user-friendliness. Hyperlinked resources and an answer booklet provide further education. To promote health literacy for users of all ages, especially children, content is written at a fifth-grade Lexile level. The GRAPES tool was tested in two cohorts at St. Louis Children's Hospital: 1) pediatric patients 10 years of age or older with any genotype of SCD and 2) registered nurses at any career stage who care for patients with SCD. Both cohorts completed a 20-question, multiple-choice knowledge questionnaire pre- and post-gameplay to assess knowledge gains. Provider healthcare experiences were assessed pre- and post-gameplay through the 17-item short-form General Perceptions About Sickle Cell Patients Scale (GPASPS, Haywood et al., 2010). Post-gameplay, both cohorts completed a satisfaction scale and provided written feedback for game improvement. Non-parametric testing was used to compare paired-sample pre- and post-assessments. Results Of the 29 pediatric hematology-oncology nurses approached, 25 nurses (18 inpatient, 7 outpatient) were recruited. 49 patients with SCD were approached, and 25 patients (20 HbSS, 2 HbSβ 0, 1 HbSC, 1 HbSβ +, 1 HPHF) consented (Table 1). Four of the patients with SCD had prior curative hematopoietic stem cell transplants. The providers worked in the nursing field for a median of 6.0 [IQR 3.0-9.0] years. Recruited participants completed all study activities, including pre- and post-gameplay assessments. Patients with SCD completed the game with a median time of 17.0 [IQR 11.5-20.5] minutes. With data from 12 providers, the median gameplay time was 12.5 [IQR 10.0-16.5] minutes. All participants (p < 0.001), including within the provider (p < 0.001) and patient (p = 0.019) cohorts, showed increase in knowledge after gameplay (Fig. 1). Both providers and patients indicated the game was of high quality and relevance (Table 2). Patients wrote: "It was helpful to know about my sickness. The treatment part was helpful," and, "The terms were helpful because I didn't know them before." One provider wrote, "I liked how there were helpful links that I could click on for more info." Some suggestions for improvement included an introductory slide about SCD and a back button. Provider negative attitudes (Q1-6, Fig. 2) were reduced (p = 0.0072) post-gameplay, but positive attitudes did not significantly change (Q7-10, Fig. 2). Providers also showed a significant decrease (p=0.0014) in the belief that patients changing their behavior around providers indicates inappropriate drug-seeking behavior (Fig. 3). Conclusion Patients and providers demonstrated significant improvement in SCD knowledge after playing GRAPES. Providers also held reduced negative attitudes towards patients with SCD post-gameplay. In the context of positive feedback from both cohorts, this study demonstrates the feasibility and acceptability of the GRAPES tool as a potential digital, behavioral intervention to decrease stigma against SCD patients and provide engaging educational materials for patients and their providers in different clinical settings. Our GRAPES tool won first prize in the 2021 NHLBI Hope for SCD Challenge (www.tinyurl.com/NIHgrapes). We are excited to continue evaluating the benefits of the GRAPES tool in a larger multi-institutional study and collaborate with the NHLBI for future dissemination among patients, families, and providers. Figure 1 Figure 1. Disclosures Badawy: Vertex Pharmaceuticals Inc: Consultancy; Bluebird Bio Inc: Consultancy; Sanofi Genzyme: Consultancy. Fields: Global Blood Therapeutics: Consultancy; Proclara Biosciences: Current equity holder in publicly-traded company.

2019 ◽  
Vol 54 (5) ◽  
pp. 610-619 ◽  
Author(s):  
Azza A. Tantawy ◽  
Amira A. Adly ◽  
Fatma S. E. Ebeid ◽  
Eman A. Ismail ◽  
Mahitab M. Hussein ◽  
...  

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 2106-2106
Author(s):  
Madiha Iqbal ◽  
Tea Reljic ◽  
Ernesto Ayala ◽  
Hemant S. Murthy ◽  
Ambuj Kumar ◽  
...  

Background: Sickle cell disease (SCD) is an inherited hemoglobinopathy which affects over 300,000 children born each year worldwide. In spite of improvement in supportive care in recent years, there is still a lack of effective treatment options. SCD leads to debilitating and cyclic episodes of erythrocyte sickling with progressive organ injury, contributing to lifetime morbidity and shortened life expectancy. Allogeneic HCT (allo-HCT) is a potentially curative therapy for SCD because engraftment is associated with resolution of the clinical phenotype of the disease and abrogation of its complications. Medical literature on allo-HCT for SCD is largely limited to children. Recent studies have evaluated the efficacy of allo-HCT in the adult population. Here, we conduct a systematic review/meta-analysis to assess the totality of evidence pertaining to the efficacy (or lack thereof) of allo-HCT in children and adults. Materials and methods: We performed a comprehensive search of the medical literature using PubMed/Medline, EMBASE and Cochrane library on July 3rd, 2019. We extracted data on clinical outcomes related to benefits (overall [OS] and disease free/event free survival [EFS/DFS]) and harms (non-relapse mortality [NRM] and graft failure [GF]), independently by two authors. Our search strategy identified 1001 references but only 30 studies (n= 1995 patients) were included in this systematic review/meta-analysis. We also performed a sub analysis on clinical outcomes for studies that included only pediatric patients (defined as <18 years) and those in patients ≥18 years of age. Results: Median age for patients enrolled in all the studies was at 10 years. Recurrent veno-occlusive crises represented the most common indication for allo-HCT followed by acute chest syndrome and stroke; nevertheless, most patients had more than one indication. Matched related donors (MRD) were the most common donor source (93%). Bone marrow was the most common source of hematopoietic stem cells (77%). Majority of patients underwent conditioning with myeloablative regimens (77%). Pooled OS rates (n=29 studies, 1681 patients) after allogeneic HCT was 95% (95%CI=93-96%) with low heterogeneity (I2=6.4%) among included studies (Figure 1). Pooled EFS/DFS rates (n=29 studies, 1894 patients) post-allografting was 90% (95%CI=87-93%) with moderate heterogeneity (I2=54%). Pooled NRM rates from 30 studies (1995 patients) was 4% (95%CI=2-6%) with low heterogeneity (I2=29.4%). Pooled GF rates from 28 studies (1851 patients) was 4% (95%CI=2-6%) with moderate heterogeneity (I2=55%). A subset analysis specifically for pediatric patients (n= 11 studies, 1009 patients, median age at 9.7 years) showed a pooled OS rate of 96% (95%CI=94-97%) with low heterogeneity (I2=0%); and for adult patients (n=3 studies, 51 patients, median age at 33.4 years) the pooled OS was 94% (95%CI=80-100%) with moderate heterogeneity (I2=52%). Pooled EFS/DFS for pediatric patients (n= 11 studies, 1009 patients) was at 89 %( 95%CI=84-93%) with moderate heterogeneity (I2=55.1%); and for adult patients (n=2 studies, 30 patients) was at 95% (95%CI=83-100%) with high heterogeneity (I2=96.5%). Pooled NRM from 10 studies with pediatric patients (281 patients) was at 6 % (95%CI=3-10%) with low heterogeneity (I2=0%); and from 3 studies with adult patients (51 patients) was at 1% (95%CI=0-7%) with low heterogeneity (I2=15.1%). Pooled GF from 10 studies with pediatric patients (281 patients) was at 3 % (95%CI=1-7%) with moderate heterogeneity (I2=40%); and from 2 studies with adult patients (30 patients) was at 5% (95%CI=0-17%) with high heterogeneity (I2=95.4%). Conclusions: The results of our systematic review/meta-analysis show excellent OS, EFS/DFS in children and adults undergoing allo-HCT with pooled OS rates exceeding 90%. The main limitation to offering an allo-HCT in SCD remains the availability of a suitable donor as 85% of patients meeting criteria do not have a MRD. We anticipate that with emergence of haploidentical transplantation the number of allo-HCT will increase in the future. GF remains a significant concern in this population and future studies should focus on novel immune suppression strategies to help reduce GF. Disclosures Kharfan-Dabaja: Pharmacyclics: Consultancy; Daiichi Sankyo: Consultancy.


2021 ◽  
Vol 2 (3) ◽  
pp. 9-17
Author(s):  
Dafni Eleftherou ◽  
Aristidis Arhakis ◽  
Sotiria Davidopoulou

Aim: This literature review aims to update the evidence for orofacial manifestations and current treatment recommendations for children and adolescents with sickle cell disease. Background: Sickle cell disease is a frequent hemoglobinopathy and a life-threatening genetic disorder. The lifelong condition is characterized by chronic hemolytic anemia and vaso-occlusive crisis that may occur in a variable range of clinical presentations in different regions of the body, including the oral cavity. Review results: This review explored the most common orofacial alterations of pediatric patients with SCD. Dental caries is a common finding in SCD pediatric patients, especially in those who are socio-economically vulnerable. Moreover, malocclusions occur in high prevalence in SCD pediatric patients. Other oral health complications seen in SCD patients include periodontal inflammation, bone changes, infections, mental nerve neuropathy, facial overgrowth, delayed tooth eruption, dental anomalies, pulp necrosis, soft tissue alterations and salivary changes. Dental infections may trigger a vaso-occlusive crisis leading the patient to a higher probability on arriving in hospital emergency departments and in need for further hospital admission to deal with the correlated complications. Thus, preventive dental care and non-invasive dental procedures are the principal focus in SCD patients in order to avoid possible subsequent complications. Conclusion: The review showed that in pediatric patients with SCD the risk for orofacial manifestations and complications depends not only on the presence of SCD but also on other confounding factors such as oral hygiene, diet habits and social conditions. Moreover, more well-designed epidemiological studies are necessary to assess the real link between SCD disease and its impact on stomatognathic health.


2018 ◽  
Vol 11 (2) ◽  
pp. 75-81 ◽  
Author(s):  
Rasha Abdel-Raouf Afifi ◽  
Dina Kamal ◽  
Riham El. Sayed ◽  
Sherif M.M. Ekladious ◽  
Gehan H. Shaheen ◽  
...  

2018 ◽  
Vol 35 (3) ◽  
pp. 199-209 ◽  
Author(s):  
Ginny L. Schulz ◽  
Rebecca H. Foster ◽  
Valerie Kennedy Lang ◽  
Alison Towerman ◽  
Shalini Shenoy ◽  
...  

Hematopoietic cell transplantation (HCT) is an elective, curative treatment option for patients with sickle cell disease (SCD). Transplant requires extensive self-management behaviors to be successful. The purpose of this study was to describe potential barriers and facilitators to self-management in a group of pediatric patients with SCD prior to HCT and their medical outcomes post-HCT. A multiple case study approach was used to describe 4 pairs of transplant recipients grouped by age, donor type, and donor source. Each pair included a case with minimal and increased post-HCT complications. Complications included readmissions, graft-versus-host disease, systemic infections, and survival in the first year post-HCT. Variables were retrospectively collected and content analyzed to identify barriers and facilitators within and across pairs using existing self-management frameworks. While higher risk transplants experienced more complications, 3 of the 4 cases with increased complications had a larger number of modifiable barriers identified compared with those experiencing minimal complications. At least one modifiable barrier and multiple facilitators were identified in all cases. A standardized psychosocial assessment process with an established plan to mitigate barriers and promote facilitators to self-management is essential to optimize outcomes in patients with SCD undergoing elective HCT.


2018 ◽  
Vol 64 (6) ◽  
pp. 302-305 ◽  
Author(s):  
Sahar Alrayyes ◽  
Anne-Ashley Compton ◽  
Nadia Kawar

Author(s):  
Ahmet Yalcinkaya ◽  
Afshin Samadi ◽  
Incilay Lay ◽  
Selma Unal ◽  
Suna Sabuncuoglu ◽  
...  

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