Knowledge of professional healthcare providers about sickle cell disease: Impact of a distance education course hematol transfus cell ther. 2 0 1 9;4 1(1):62–68 w w w . r b h h . o r g Hematology, Transfusion and Cell Therapy Original article Knowledge of professional healthcare providers about sickle cell disease: Impact of a distance education course Katy Karoline Santos Diniz ∗, Adriana Silvina Pagano, Ana Paula Pinheiro Chagas Fernandes, Ilka Afonso Reis, Leonardo Gonçalves Pinheiro Júnior, Heloísa de Carvalho Torres Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG, Brazil a r t i c l e i n f o Article history: Received 9 March 2018 Accepted 20 June 2018 Available online 20 July 2018 Keywords: Sickle cell disease Healthcare provider Knowledge Health education a b s t r a c t Objective: To assess the impact of the distance education course “Sickle Cell Disease: Primary Health Care Line” on knowledge acquisition of professional healthcare providers. Methods: A cross-sectional study was conducted with a quantitative approach at the Edu- cational and Support Center for Hemoglobinopathies (Cehmob-MG), state of Minas Gerais, Brazil, in 2016. One hundred and fifty-three out of 300 professional healthcare providers were invited to participate in the proposed distance course. Of the participating professional healthcare providers, 72 (47%) successfully concluded the course (Group A), whereas 81 (53%) did not complete their course assignments and did not meet the minimum requirements for regular attendance (Group B). Knowledge acquisition was assessed with the Knowl- edge of Sickle Cell Disease Instrument, DFConhecimento, applied using the web tool eSurv. Univariate analysis by Poisson regression was employed to assess the influence of sociode- mographic variables on the DFConhecimento score and to select variables to compose the initial multivariate regression model (p-value < 0.20). The analysis was performed in the statistical programming environment R. Results: The average score was 9.76 for Group A and 6.54 for Group B. The two groups were considered statistically different (p-value < 0.05) for all items with the proportion of correct items being greater in Group A. Professional healthcare providers who concluded the course had a significantly higher DFConhecimento score (45%) when compared to those who did not successfully conclude the course. Conclusion: Participation in a distance education course on sickle cell disease had a posi- tive impact on the acquisition of knowledge about the disease by professional healthcare providers. o Bra © 2018 Associaçã by Elsevier Editora Lt ∗ Corresponding author. E-mail address: katykarolinesd@gmail.com (K.K. Diniz). https://doi.org/10.1016/j.htct.2018.06.004 2531-1379/© 2018 Associação Brasileira de Hematologia, Hemoterapia open access article under the CC BY-NC-ND license (http://creativecom sileira de Hematologia, Hemoterapia e Terapia Celular. Published da. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). e Terapia Celular. Published by Elsevier Editora Ltda. This is an mons.org/licenses/by-nc-nd/4.0/). https://doi.org/10.1016/j.htct.2018.06.004 http://www.rbhh.org http://crossmark.crossref.org/dialog/?doi=10.1016/j.htct.2018.06.004&domain=pdf http://creativecommons.org/licenses/by-nc-nd/4.0/ http://creativecommons.org/licenses/by-nc-nd/4.0/ http://creativecommons.org/licenses/by-nc-nd/4.0/ http://creativecommons.org/licenses/by-nc-nd/4.0/ http://creativecommons.org/licenses/by-nc-nd/4.0/ http://creativecommons.org/licenses/by-nc-nd/4.0/ http://creativecommons.org/licenses/by-nc-nd/4.0/ http://creativecommons.org/licenses/by-nc-nd/4.0/ http://creativecommons.org/licenses/by-nc-nd/4.0/ http://creativecommons.org/licenses/by-nc-nd/4.0/ mailto:katykarolinesd@gmail.com https://doi.org/10.1016/j.htct.2018.06.004 http://creativecommons.org/licenses/by-nc-nd/4.0/ http://creativecommons.org/licenses/by-nc-nd/4.0/ http://creativecommons.org/licenses/by-nc-nd/4.0/ http://creativecommons.org/licenses/by-nc-nd/4.0/ http://creativecommons.org/licenses/by-nc-nd/4.0/ http://creativecommons.org/licenses/by-nc-nd/4.0/ http://creativecommons.org/licenses/by-nc-nd/4.0/ http://creativecommons.org/licenses/by-nc-nd/4.0/ http://creativecommons.org/licenses/by-nc-nd/4.0/ http://creativecommons.org/licenses/by-nc-nd/4.0/ ther I C h I c c a d s o h l o u d l t t e c k c C t ( p a c t e a e p s b c n i c e L p M T c p o H t T hematol transfus cell ntroduction onsidered a public health problem, sickle cell disease is a ereditary condition of great importance globally and in Brazil. t predominantly affects the Black population and presents linical manifestations within the first years of life with reper- ussions on morbidity and mortality.1,2 Despite technological nd scientific advances to improve the prognosis of sickle cell isease, its management persists as a challenge for profes- ional healthcare providers, since it involves complex aspects f diagnosis, treatment and prevention of complications.3–5 Studies point to insufficient knowledge of professional ealthcare providers about important aspects of the quality of ife of people with sickle cell disease, such as growth and devel- pmental consultations, immunization, school performance, se of folic acid, prophylactic antibiotic therapy, complications ue to vaso-occlusion, transfusions, prevalence of iron over- oad, strokes in children and screening of stroke risk using ranscranial Doppler ultrasonography.2,6–9 In order to improve he quality of care provided to people with sickle cell dis- ase and to reduce morbidity and mortality rates, there is a lear need to develop educational interventions to increase nowledge of professional healthcare providers about sickle ell disease.3,10–12 To that end, a distance education course entitled “Sickle ell Disease: Primary Health Care Line” has been offered by he Educational and Support Center for Hemoglobinopathies CEHMOB-MG) of the state of Minas Gerais since 2010. The urpose of the course is to build competence of profession- ls and to improve the quality of care for people with sickle ell disease.2,3 Distance education is considered an important tool in he dissemination of up-to-date knowledge and information, specially in emerging countries. Its educational resources llow interaction, shared experience, improvement of knowl- dge in different health areas and can affect a large number of rofessionals in different regions of the country. In addition, tudies have demonstrated the effectiveness of this approach y educational interventions targeting professional health- are providers.13–16 Despite the relevance of distance courses, o studies were found in the literature that investigate the mpact of distance education on the management of sickle ell disease. This study aims to evaluate the impact of the distance ducation course “Sickle Cell Disease: Primary Health Care ine” on knowledge acquisition by professional healthcare roviders. ethods his is a cross-sectional study with a quantitative approach arried out from April to June 2016 at the Educational and Sup- ort Center for Hemoglobinopathies (Cehmob-MG) in the state f Minas Gerais. The distance education course “Sickle Cell Disease: Primary ealth Care Line” aims at promoting an educational strategy o improve knowledge within a virtual learning environment. he course workload is 95 h over three months with follow-up . 2 0 1 9;4 1(1):62–68 63 sessions managed by tutors with expertise in sickle cell dis- ease. The course content is divided into modules dealing with topics related to health surveillance actions targeting children, adolescents and adults, management of acute events, sickle cell behavior and other hemoglobinopathies (Table 1). Con- tent is developed through student engagement in case study analysis, discussion forums and group activities with debate and reflection on the provision of professional care, leading to knowledge construction and proposals to improve the quality of care for people with sickle cell disease. To recruit participants, local and regional authorities are contacted and alerted as to the relevance of build- ing professional competence to deal with the disease. As a result, authorities delegate healthcare providers working with sickle cell disease (physicians, nurses, dentists, social assis- tants, psychologists, physical therapists, physical educators, dieticians and others) to take part in the course. Other profes- sionals wishing to participate in the course are also welcome. During the period from 2010 to 2016, 2446 professionals were selected to participate in the Cehmob distance education course. Of these, 1171 (48%) successfully concluded the course obtaining a minimum score of 70% for course work and atten- dance. The remaining 1275 (52%) did not obtain the minimum score or dropped out of the course. The present study used a database of all professional healthcare providers for the selection of prospective partici- pants. In April 2016, an invitation to participate in this study with a brief explanation of the research, and an informed consent form was sent by email to 300 randomly selected pro- fessionals among those enrolled in the course. As the study aimed to assess participants who successfully concluded the course as well as those who did not, 150 professionals were randomly selected from each group. Of the invitees, 153 (51%) accepted to participate in this study; 72 (47%) had successfully concluded the course (hence- forth referred to as Group A) and 81 (53%) had not met the minimum requirements for course completion (Group B). Participants were requested to answer a questionnaire applied using the web tool e-Surv. This questionnaire comprised three sections: (1) sociodemographic data: sex, education, city of residence, and professional category; (2) details of professional performance: type of health service in which the participant worked and the number of years working in healthcare services; and (3) the DFConhecimento instrument (Attachment 1). Thirteen multiple-choice ques- tions were included about the disease covering: 1. Newborn screening program; 2. Sickle cell disease; 3. Sickle cell anemia genotype; 4. Sickle cell trait; 5. Clinical manifestations; 6. Acute events; 7. Conditions favoring red blood cell hemolysis; 8. Signs of sickle cell disease; 9. Medications; 10. Prophylaxis; 11. Ado- lescence; 12. Pregnancy; and 13. Prevention of leg ulcers. The DFConhecimento instrument was constructed and val- idated by the study authors from May 2015 to April 2016 at the Universidade Federal de Minas Gerais (UFMG). Instrument content validation was carried out by 11 experts in instrument validation and in sickle cell disease that made up a panel of experts. The instrument presented an average content validity index of 0.88. Regarding the reliability analysis, the instrument presented satisfactory indexes. Cronbach’s alpha presented a value of 0.818, indicating a high internal consistency. The 64 hematol transfus cell ther. 2 0 1 9;4 1(1):62–68 Table 1 – Modules, units and aims of the distance course “Sickle Cell Disease: Primary Health Care Line”. Module Unit Aim Getting familiar Course introduction To get familiar with the virtual learning environment and tools 1. Monitoring children with sickle cell disease Active search, prophylaxis, immunization Understanding risks of sickle cell disease and the need to identify the disease in primary healthcare for planning monitoring by all professional healthcare providers 2. Managing acute events Acute splenic sequestration, infection, pain crisis, stroke, cholecystitis, acute chest syndrome Learning about acute events and establishing protocols for dealing with them at basic healthcare units and referral 3. Monitoring adolescents and adults with sickle cell disease Pregnancy, puberty Understanding special needs of adolescents and adults with sickle cell disease. Identifying risks in pregnancy 4. Caring for patients with sickle cell disease and other Hb AS—sickle cell trait; Hb CC—hemoglobin C Acquiring advanced knowledge about sickle cell disease and other hemoglobinopathies to plan guidelines for instruction and follow up hemoglobinopathies 5. Discussing healthcare promotion strategies End of course assignment intraclass correlation coefficient between the test and retest presented a value of 0.67 [95% confidence interval (95% CI): 0.55–0.76], indicating that the temporal reproducibility is acceptable. The instrument presents dimensions composed of items compatible with themes indicated as priorities for knowledge on sickle cell disease by the health professional (diagnosis, treatment and prevention of complications).17 The DFConhecimento instrument scores were calculated with 1 being assigned for correct answers and 0 for incorrect answers (range: 0–13). The qualitative variables—sex, educa- tion, training category, number of years working in healthcare services, type of health service and previous experience assist- ing patients with sickle cell disease—are summarized using absolute and relative frequencies. The scores obtained with the instrument are summarized as mean, median, standard deviation (SD) and interquartile range. The Fisher exact or chi-square tests were used to analyze the homogeneity of groups in relation to sex, education, professional category, professional performance and number of years working in healthcare services. A level of significance of 5% or more (p- value > 0.05) indicated evidence of homogeneity.18 The chi-square test with a level of significance of 5% was adopted to compare the proportions of correct answers between Group A and Group B. The qualitative variables are presented using absolute and relative frequencies.18 The Poisson regression model was used to study the influ- ence of factors—having completed the course, sex, academic training, training category, number of years working in health- care, type of healthcare service and previous experience assisting patients with sickle cell disease.19 Univariate analy- sis allowed the selection of factors that potentially influenced the knowledge score with a level of significance equal to 25% being considered at this selection stage. Using the selected variables, univariate analysis employing a generalized linear Poisson model was employed to verify the direct influence of distance education on the scores of the professionals and con- firmed whether other characteristic variables of the sample had a significant influence on their knowledge. Thus, mul- tivariate Poisson regression models were fit with backward elimination being used to select the variables of the final model. A significance level of 5% was adopted for backward Exercise on planning educational actions about sickle cell disease as a joint task by a team of family healthcare providers elimination.17 All analyses were performed using the statisti- cal software R (R core team). The project was approved by the Research Ethics Commit- tee Involving Human Beings of the Universidade Federal de Minas Gerais (decision No. 1.717.975). The agreement of the subjects to participate in the study was recorded by means of the informed consent form available in the initial menu of the electronic questionnaire on the website e-Surv. Results Of the participants of the study, 127 (83%) were female, 82 (54%) only studied to degree level, 88 (58%) were nurses, 52 (34%) had worked in healthcare services for more than nine years, 118 (77%) had previously provided care to sickle cell disease patients and 134 (90%) work in municipalities in the State of Minas Gerais (Table 2). The groups under study were considered homogeneous in all analyzed variables. Table 3 shows the descriptive statistics of the knowledge scores according to the situation of the professionals in rela- tion to concluding the course. It can be observed that Group A had higher mean and median scores than Group B. Scores ranged from a minimum of 0 to a maximum of 13 points. The mean score was 9.76 (SD: 2.62) for Group A and 6.54 (SD: 3.22) for Group B. Table 4 presents a comparison of proportions of correct answers between Group A and Group B. There was a significant difference (p-value < 0.05) for all the questions with the propor- tion of correct answers being significantly higher among those who completed the course. The mean percentage of correct answers of subjects in Group A (75.0 ± 2.6%) was higher than the results of Group B (50.6 ± 3.2%). In the univariate analysis (Table 5), the following variables were selected: Group (A and B), academic training, health service type and previous experience assisting sickle cell dis- ease patients; variables were considered possible significant influences when p-values were <0.25. A significant difference regarding the knowledge score (p-value < 0.001) was observed between Group A (average score was 49% higher) and Group hematol transfus cell ther. 2 0 1 9;4 1(1):62–68 65 Table 2 – Sociodemographic characterization of the professionals who answered the DFConhecimento instrument stratified depending on course completion. Sociodemographic characteristic n (%) p Group A Group B Total Sex Female 59 (82) 68 (84) 127 (83) 0.909a Male 13 (18) 13 (16) 23 (17) Education Degree 13 (18) 29 (36) 42 (27) 0.141b Specialization 40 (56) 42 (52) 82 (54) Master’s degree 10 (14) 8 (10) 18 (12) Doctorate degree 6 (8) 2 (2) 8 (5) Professional category Nursing 40 (56) 48 (59) 88 (58) 0.765a Others* 32 (44) 33 (41) 65 (42) Number of years working in healthcare services Up to 4 years 14 (19) 30 (37) 44 (29) 0.110a From 5 to 8 20 (28) 19 (23) 39 (25) 9 years or more 29 (40) 23 (28) 52 (34) None 9 (13) 9 (11) 18 (12) Type of health service Primary healthcare 32 (44) 41 (53) 74 (48) 0.706a Others** 40 (56) 40 (57) 79 (52) Previous experience assisting patients with sickle cell disease No 13 (18) 22 (27) 35 (23) 0.252a Yes 59 (82) 59 (73) 118 (77) a Chi-square test. b Fisher’s exact test. ∗ Others: biomedicine (2); physical education (1); pharmacy (7); physiotherapy (2); health management (3); manager (1); medicine (12); nutrition (8); odontology (4); pedagogy (1); psychology (5); social work (18); occupational therapy (1). ∗∗ Others: Technical references and projects of the state and municipal health departments (27); hospital (15); educational institution (10); emergency care unit (7); blood center (7); laboratory (3); social work department (5); social control (1); does not work in the health area (4). Table 3 – Descriptive analysis of scores. Scores Mean SD Min. 1st quartile Median 3rd quartile Max. Total 8.1 3.1 0 6 8 11 13 Group A 9.8 2.6 0 8 10 12 13 Group B 6.5 3.2 0 4 6 9 13 SD, standard deviation; Min., minimum; Max., maximum. Table 4 – Proportions of correct answers of professionals who answered the questionnaire and completed the course (Group A) and those that did not (Group B). Questions Total (n = 153) Group A (n = 72) Group B (n = 81) pa n (%) n (%) n (%) Q1 118 (77.1) 62 (86.1) 56 (69.1) 0.013 Q2 131 (85.6) 67 (93.1) 64 (79) 0.013 Q3 123 (80.4) 65 (90.3) 58 (71.6) 0.004 Q4 115 (75.2) 65 (90.3) 50 (61.7) 0.000 Q5 59 (38.6) 38 (52.8) 21 (25.9) 0.001 Q6 105 (68.6) 64 (88.9) 41 (50.6) 0.000 Q7 73 (47.7) 47 (65.3) 26 (32.1) 0.000 Q8 61 (39.9) 35 (48.6) 26 (32.1) 0.037 Q9 59 (38.6) 37 (51.4) 22 (27.2) 0.002 Q10 82 (53.6) 46 (63.9) 36 (44.4) 0.016 Q11 104 (68) 63 (87.5) 41 (50.6) 0.000 Q12 92 (60.1) 54 (75) 38 (46.9) 0.000 Q13 111 (72.5) 60 (83.3) 51 (63) 0.005 Mean 95 (62.1) 54 (75) 41 (50.6) 0.000 a Chi-square test. 66 hematol transfus cell ther. 2 0 1 9;4 1(1):62–68 Table 5 – Univariate analysis using the Poisson regression model. Variable ̌ EP(ˇ) Exp(ˇ) 95% CI p Group A 0.40 0.06 1.49 1.32–1.69 <0.001 B – – – Sex Female – – – Male 0.10 0.09 0.91 0.76–1.09 0.291 Education Degree – – Specialization 0.16 0.08 1.17 1.00–1.37 0.047 Master’s degree 0.28 0.11 1.32 1.06–1.64 0.015 Doctorate degree 0.44 0.14 1.55 1.17–2.04 0.003 Training category Nursing – – Other 0.02 0.07 1.02 0.89–1.17 0.764 Number of years working in healthcare None – – Up to 4 years 0.07 0.12 1.07 0.84–1.36 0.593 From 5 to 8 years 0.12 0.12 1.13 0.89–1.44 0.321 9 years or more 0.25 0.12 1.29 1.02–1.62 0.033 Type of health service Primary healthcare – – Other 0.08 0.07 1.09 0.95–1.24 0.227 Previous experience assisting patients with sickle cell disease No – – Yes 0.15 0.08 1.16 0.99–1.37 0.075 95% CI: 95% confidence interval. B. There was a significant difference (p-value < 0.005) between training levels. On average, compared to participants with just a degree, those with a specialization presented 17% higher scores; with a master’s degree or taking a master’s degree had scores 32% higher; while those with a doctorate degree had scores 55% higher. There was a significant difference (p- value = 0.033) in the number of years working in healthcare, since the knowledge score for professionals who had worked nine years or more was on average 29% higher than the score of those who had never worked in healthcare. There was no significant difference between mean scores when compar- ing variables such as gender, professional category, type of healthcare service and whether they had dealt with sickle cell disease patients previously. Table 6 presents the results of the Poisson regression model adjusted using the variables: group, academic training, health service type and previous assistance to patients with sickle cell disease (initial multivariate model). The final multivariate model was reached after step-by-step elimination of non- significant variables (p-value > 0.05) from the initial model (Table 6). The analysis of the final model shows that Group A maintained a significant difference, presenting on average 45% (95% CI: 28–64%) higher scores than Group B, even when this comparison is adjusted by the level of training. There was also a significant difference (p-value = 0.029) in the means of the knowledge score of professionals with different levels of aca- demic training; those with a doctoral degree had, on average, 34% (95% CI: 3–73%) higher scores when compared to profes- sionals just with graduation. For the levels of specialization and master’s degree, no statistically significant differences were observed in the score in relation to the level of gradu- ation. Discussion The characteristics of the sample studied related to the vari- ables of gender, professional category and specialization were similar to those observed in other studies with professional healthcare providers. In those, most of the professionals were female, with a nursing background and some level of specialization.16,20 A favorable aspect of this study, observed in the characterization of the sample, is related to the graduate and higher education groups, which were considered homo- geneous in all the variables analyzed. In the individual evaluation of the items of the DFCon- hecimento instrument, the proportion of correct answers for the whole instrument were on average 75% higher among the participants in Group A. This average was higher than that observed in a study carried out in the United States (65%) that investigated knowledge about sickle cell disease among physi- cians and nurses of emergency services who participated in a workshop that included ten lectures given by specialists in the area. The participants answered an electronic question- naire of multiple-choice questions prepared by the authors of the study before the beginning of the lectures and after the event.7 The results of DFConhecimento, verified by the descrip- tive analysis of the scores and by the generalized linear Poisson model, showed that members of Group A had signif- icantly more knowledge about the disease after the course compared to Group B. A similar result was obtained in a randomized study carried out in the northern part of the State of Minas Gerais, aimed at assessing an educational program about sickle cell disease that targeted commu- nity healthcare agents and nursing assistants working in primary healthcare. Participants in the study showed knowl- edge acquisition by improvements in their performance during the assessment (p-value < 0.001).21 Studies verified a significant positive impact on knowledge of professional healthcare providers after being submitted to an interven- tion in other health domains using the method of distance 14–16 education. The positive impact of the distance education course about sickle cell disease on the professionals who completed the study suggests that this educational strategy can be hematol transfus cell ther. 2 0 1 9;4 1(1):62–68 67 Table 6 – Results of multivariate analysis (adjustment of the generalized linear Poisson model). Variables Initial model Final model ̌ EP(ˇ) Exp(ˇ) 95% CI p ̌ EP(ˇ) Exp(ˇ) 95% CI p Group A 0.37 0.06 1.44 1.27–1.64 <0.001 0.37 0.06 1.45 1.28–1.64 <0.001 B – – – – – – – – – – Education Degree – – – – – – – – – – Specialization 0.08 0.08 1.09 0.94–1.26 0.280 0.11 0.07 1.12 0.96–1.29 0.143 Master’s degree 0.15 0.11 1.16 0.94–1.44 0.179 0.20 0.10 1.22 1.00–1.50 0.056 Doctorate degree 0.20 0.14 1.22 0.92–1.61 0.161 0.29 0.13 1.34 1.03–1.74 0.029 Number of years working in healthcare None – – – – – – – – – – Up to 4 years 0.16 0.12 1.17 0.93–1.48 0.173 – – – – – From 5 to 8 years 0.13 0.12 1.14 0.90–1.45 0.280 – – – – – 9 years or more 0.21 0.12 1.24 0.99–1.56 0.068 – – – – – Type of health service Primary healthcare – – – – – – – – – – Other 0.08 0.07 1.08 0.94–1.24 0.259 – – – – – Previous experience assisting patients with sickle cell disease No – – – – – Yes 0.07 0.08 1.07 0.92–1.25 0.390 – – – – – i p i k d p o a a e t w p o t B t k r p i a p p g C D k s w a C T r 95% CI: 95% confidence interval. ntroduced in the context of all professional healthcare roviders working in the healthcare network. Thus, it is mportant that this strategy includes content related to nowledge deficits of professional healthcare providers as escribed in the literature: content that addresses patho- hysiological complications, transfusion, prevalence of iron verload, stroke in children, clinical manifestations as well s the management of sickle cell disease.2,7,9,10,12 The results obtained from the professionals in this study nd reported in the literature show that a deficit of knowl- dge about sickle cell disease exists and persists despite the echnical-scientific advances and the availability of guidelines ith recommendations for professional practice. However, the ositive impact on the knowledge about sickle cell disease f professionals after participating in educational interven- ions was confirmed in recent studies with North American, razilian and Mexican professionals with different educa- ional levels using qualitative and quantitative methods of nowledge assessment.2,10,22–24 This study fills a gap highlighted in a previous study, which ecommended including physicians and nurses in educational rograms on sickle cell disease.21 Because this educational ntervention seeks to improve the knowledge of profession- ls in respect to sickle cell disease using technology, its strong oint is the viability of reproducing this study with more articipants and with geographical expansion to represent a reater proportion of professionals.15,16,25 onclusion istance learning courses have a positive impact on the nowledge acquired by professional health providers about ickle cell disease. It is essential to include professionals ho assist sickle cell disease patients in similar educational ctions. onflicts of interest he authors declare no conflicts of interest. Appendix A. Supplementary data Supplementary data associated with this article can be found, in the online version, at doi:10.1016/j.htct.2018.06.004. e f e r e n c e s 1. Cordeiro RC, Ferreira SL. Experiences of illness among individuals with sickle cell anemia and self-care strategies. Acta Paul Enferm. 2014;27(6):499–504. 2. Gomes LM, Vieira MM, Reis TC, Andrade-Brabosa TL, de Caldeira AP. Understanding of technical education level professionals regarding sickle cell disease: a descriptive study. Online Braz J Nurs. 2013;12(2):482–90. 3. Gomes LM, Pereira IA, Torres HC, Caldeira AP, Viana MB. Access and care of individuals with sickle cell anemia in a primary care service. ACTA Paul Enferm. 2014;27(4):348–55. 4. da Fonseca SF, Amorim T, Purificação A, Gonçalves M, Boa-Sorte N. Hemoglobin A 2 values in sickle cell disease patients quantified by high performance liquid chromatography and the influence of alpha thalassemia. Rev Bras Hematol Hemoter. 2015;7(5):296–301. 5. Silva Filho IL, Ribeiro GS, Pimenta-Bueno LM, Serpa MJ. The frequency of �-globin gene haplotypes, �-thalassemia and genetic polymorphisms of methylenetetrahydrofolate reductase, factor V Leiden and prothrombin genes in children with sickle cell disease in Rio de Janeiro, Brazil. Rev Bras Hematol Hemoter. 2010;32(1):76–8. 6. Gomes LM, de Andrade Barbosa TL, Vieira ED, Vieira LJ, Castro KP, et al. Community healthcare workers’ perception of an educational intervention in the care of patients with sickle cell disease in Brazil. Mediterr J Hematol Infect Dis. 2015;7(1):e2015031. 7. Tanabe P, Stevenson A, DeCastro L, Drawhorn L, Lanzkron S, Molokie RE, et al. Evaluation of a train-the-trainer workshop on sickle cell disease for ED providers. J. Emerg. Nurs. 2013;39(6):539–46. 8. 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