scholarly journals Level of utilization and provider-related barriers to the use of hydroxyurea in the treatment of sickle cell disease patients in Jos, North-Central Nigeria

2021 ◽  
Vol 21 (2) ◽  
pp. 765-774
Author(s):  
Akinyemi OD Ofakunrin ◽  
Edache S Okpe ◽  
Tolulope O Afolaranmi ◽  
Rasaq R Olaosebikan ◽  
Patience U Kanhu ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Health Care Providers ◽  
Clinical Guidelines ◽  
Cross Sectional Study ◽  
Medical Doctors ◽  
Care Providers ◽  
Cell Disease ◽  
Cross Sectional ◽  
North Central

Background: Hydroxyurea is underutilized by sickle cell health-care providers in Nigeria despite available evidence of its effectiveness in reducing the manifestations and complications of sickle cell disease (SCD). Objectives: To assess the level of utilization and provider-related barriers to the use of hydroxyurea in SCD therapy in Jos, Nigeria. Methods: A cross-sectional study conducted among 132 medical doctors providing care for SCD patients. Data on so- cio-demographics, utilization and barriers to hydroxyurea use were obtained. The barriers were fed cumulatively into the logistic regression model as predictors of utilization. Results: Of the 132 care providers, 88 (67%) had been in medical practice for ≥6years. The level of utilization of hy- droxyurea was 24.2%. The significant barriers that predicted the non-utilization of hydroxyurea included lack of expertise (OR=5.1; 95% CI=2.65–9.05), lack of clinical guidelines (OR=3.84; 95% CI=2.37-14.33), fear of side-effects (OR=0.50; 95% CI=0.22–0.68) and doubt about its effectiveness (OR=0.30; 95% CI=0.20–0.90). Conclusion: The level of utilization of hydroxyurea in the treatment of SCD among the care providers is sub-optimal with the lack of expertise in its use identified as the most prominent barrier. There is an urgent need for the training of sickle cell care-providers and the development of clinical guidelines on hydroxyurea use. Keywords: Hydroxyurea utilization; barriers to hydroxyurea; sickle cell disease; Nigeria.

scholarly journals Level of Utilization and Provider-Related Barriers to Hydroxyurea Use in the Treatment of Sickle Cell Disease in Jos, Nigeria

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 1029-1029 ◽  
Author(s):  
Akinyemi Olugbenga David Ofakunrin ◽  
Kehinde Adekola ◽  
Edache Sylvanus Okpe ◽  
Stephen Oguche ◽  
Tolulope Afolaranmi ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Health Care Providers ◽  
Conflicts Of Interest ◽  
Sampling Technique ◽  
P Value ◽  
Care Providers ◽  
Cell Disease ◽  
Cross Sectional ◽  
Inadequate Knowledge

Background: Hydroxyurea is one of the currently approved medications capable of modifying the pathogenesis of sickle cell disease (SCD), and its use has transformed the management of this disease worldwide.However, available evidences suggest that hydroxyurea is underutilized by sickle cell health-care providers in Nigeria despite the huge burden of the disease. Objectives: This study assessed the level of utilization and provider-related barriers to the use of hydroxyurea in the treatment of SCD patients in Jos, Nigeria. Methods: A cross-sectional study conducted among 132 medical doctors providing care for SCD patients in four tertiary hospitals in Jos using a multistage sampling technique. In this setting, SCD patients are cared for by the Hematologists, Pediatricians, Family Physicians and General Practitioners. Data on socio-demographics of the respondents, knowledge, utilization and barriers to the utilization of hydroxyurea were obtained using an interviewer-administered questionnaire. The data were processed and analysed using SPSS version 23. Hydroxyurea was adjudged utilized if a provider has prescribed hydroxyurea to any SCD patient within the last 12 months. Chi square test was used to test the association between the demographic, provider-related barrier variables and the level of utilization of hydroxyurea. The barriers were fed cumulatively into logistic regression model as predictors of utilization of hydroxyurea. Adjusted odds ratio and 95% confidence interval were used as point and interval estimates respectively. A P-value of <0.05 was considered statistically significant. Results: Of the 132 respondents, 88 (67%) had been in medical practice for upward of six years while 80 (60.6%) of them affirmed that they have attended to more than 10 SCD patients in the last 6 months. Sixty-seven (50.8%) of the participants had inadequate knowledge of hydroxyurea use in SCD management while the level of utilization of hydroxyurea in SCD treatment was 24.2%. The odds of non-utilization of hydroxyurea was 5.1 times higher in providers with no expertise in its use (OR =5.1; 95% CI =2.65-9.05; P<0.0001). Other barriers that predicted its non-utilization included inadequate knowledge (OR =0.17; 95% CI =0.29-0.71; P=0.017), fear of side-effects (OR =0.50; 95% CI =0.22-0.68; P=0.019) and doubt about the effectiveness of the medication (OR =0.30; 95% CI =0.20-0.90; P=0.002). Conclusion: The level of utilization of hydroxyurea in the treatment of SCD among the sickle cell care-providers is sub-optimal with the lack of expertise in its use identified as the most prominent barrier. Therefore, training of Nigeria sickle cell care-providers to attain and maintain competence in the use of hydroxyurea for the treatment of SCD is required. Keywords: Sickle cell disease, hydroxyurea, utilization, barriers, Jos, Nigeria Disclosures No relevant conflicts of interest to declare.

scholarly journals The Practices of Anaesthesiologists in the Management of Patients with Sickle Cell Disease: Empirical Evidence from Cameroon

Healthcare ◽  
2021 ◽  
Vol 9 (12) ◽  
pp. 1617
Author(s):  
Dominique Djomo Tamchom ◽  
Aristide Kuitchet ◽  
Raymond Ndikontar ◽  
Serge Nga Nomo ◽  
Hermine Fouda ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Perioperative Management ◽  
Treatment Guideline ◽  
Binary Logistic Regression ◽  
Cross Sectional Study ◽  
Cell Disease ◽  
Binary Logistic Regression Model ◽  
Cross Sectional ◽  
Elective Surgical Procedures

Patients with sickle cell disease are more likely to undergo surgery during their lifetime, especially given the numerous complications they may develop. There is a paucity of data concerning the management of patients with sickle cell disease by anaesthesiologists, especially in Africa. This study aimed to describe the practices of anaesthesiologists in Cameroon concerning the perioperative management of patients with sickle cell disease. A cross-sectional study was carried out over four months and involved 35 out 47 anaesthesiologists working in hospitals across the country, who were invited to fill a data collection form after giving their informed consent. The data were analysed using descriptive statistics and a binary logistic regression model. Among the 35 anaesthesiologists included in the study, most (29 (82.9%)) had managed patients with sickle cell disease for both emergency and elective surgical procedures. Most of them had never asked for a haematology consultation before surgery. Most participants (26 (74.3%)) admitted to having carried out simple blood transfusions, while 4 (11.4%) carried out exchange transfusions. The haemoglobin thresholds for transfusion varied from one practitioner to another, between < 6 g/dl and < 9 g/dl. Only 6 (17.1%) anaesthesiologists had a treatment guideline for the management of patients with sickle cell disease in the hospitals where they practiced. Only 9 (25.7%) prescribed a search for irregular agglutinins. The percentage of haemoglobin S before surgery was always available for 5 (14.3%) of the participants. The coefficient (0.06) of the occurrence of a haematology consultation before surgery had a significant influence on the probability of management of post-operative complications (coefficient 0.06, 10% level of probability). This study highlights the fact that practices in the perioperative management of patients with sickle cell disease in Cameroon vary greatly from one anaesthesiologist to another. We disclosed major differences in the current recommendations, which support the fact that even in Sub-Saharan countries, guidelines applicable to the local settings should be published.

scholarly journals Sexual Maturity in Adolescents Suffering from Sickle Cell Disease: A Cross-sectional Study

2021 ◽  
Author(s):  
Vijay Shah ◽  
Akash Patel ◽  
Praful Bambharoliya ◽  
Jigisha Patadia
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sexual Maturity ◽  
Tanner Stage ◽  
Cross Sectional Study ◽  
Reproductive Capacity ◽  
Sectional Study ◽  
Cell Disease ◽  
Cross Sectional ◽  
The Mean

Introduction: Sickle Cell Disease (SCD) is an inherited chronic haemolytic anaemia. The diseased person suffers from various complications such as anaemia, frequent infection, fever, hand-foot syndrome, stroke, etc. Puberty changes includes the appearance of the secondary sexual characteristics, increase in height, change in body composition and development of reproductive capacity. Aim: To study the sexual maturity and effect of multiple blood transfusions in adolescents suffering from SCD. Materials and Methods: It was a cross-sectional study conducted on 35 adolescents of age group 11 to 15 years, suffering from SCD. Study was conducted over a period of six months from March 2018 to September 2018 at Department of Paediatrics. SCD was diagnosed by Haemoglobin (Hb) electrophoresis. Weight and height were measured of all the participants. For assessing the sexual maturity, Tanners staging was used. Unpaired t-test was done for data analysis. Results: The mean age of the patients was 13.03±1.7 years. There were 25 males and 10 females. The mean age of male patients between Tanner stage 2(14.63±0.52 years) and Tanner stage 3 (14.75±0.5 years) was significantly higher than the Indian data for males (11.3 and 12.8 years, respectively). The mean age of female patients between Tanner stages 2 (13.5±2.12 years) and Tanner stage 3 (14.33±1.16 years) was higher than the Indian reference data for girls (10.2 and 11.6 years respectively). Conclusion: This study concluded that adolescents with SCD were significantly shorter in height and weight than the standard reference population. Sexual maturity is delayed in adolescents with sickle cell anaemia.

scholarly journals Asymmetric Dimethyl Arginine Level in Children with Sickle Cell Disease: A Cross Sectional Study

2021 ◽  
pp. 93-98
Author(s):  
Seham Fathy Khedr ◽  
Mohamed Hosny El Bradaey ◽  
Hala Mohamed Nagy ◽  
Mohamed Ramadan El-Shanshory ◽  
Eslam Elhawary
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Cross Sectional Study ◽  
Sectional Study ◽  
Cell Disease ◽  
Adma Level ◽  
Cross Sectional ◽  
Asymmetric Dimethyl Arginine ◽  
Dimethyl Arginine

Background: Sickle cell disease (SCD) consists of a group of hemoglobinopathies in which individuals inherit hemoglobin variants derived from single point mutations. Asymmetric dimethylarginine (ADMA) contributes to limiting Nitric Oxide (NO)  bioavailability in SCD. The aim of the present study was to assess the level of the Asymmetric Dimethyl Arginine in children with sickle cell. Methods: This cohort cross-sectional study was carried out on 60 children which were divided in to 3 equal groups. Group I: SCD children with sickle retinopathy. Group II: SCD children without retinopathy. Group III: healthy control children who were selected from the outpatient clinic. Results: There was a significant increase in ADMA level among participants withSCD. There was a positive significant correlation between ADMA  level and family history as well as the  incidence of hepatomegaly. There was no significant correlation between ADMA level and demographic and laboratory parameters except LDH. Conclusions: The level of ADMA is elevated in children with sickle cell anemia. High plasma ADMA level is a risk for hepatomegaly in children with sickle cell anemia.

scholarly journals A cross sectional study of growth of children with sickle cell disease, aged 2 to 5 years in Yaoundé, Cameroon

2019 ◽  
Vol 34 ◽  
Author(s):  
Suzanne Sap Ngo Um ◽  
Judith Seungue ◽  
Anastasie Yanda Alima ◽  
Ritha Mbono ◽  
Hubert Mbassi ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cross Sectional Study ◽  
Sectional Study ◽  
Cell Disease ◽  
Cross Sectional

scholarly journals Placenta growth factor in sickle cell disease: association with hemolysis and inflammation

Blood ◽  
2010 ◽  
Vol 115 (10) ◽  
pp. 2014-2020 ◽  
Author(s):  
Julia E. Brittain ◽  
Ben Hulkower ◽  
Susan K. Jones ◽  
Dell Strayhorn ◽  
Laura De Castro ◽  
...  
Keyword(s):  
Growth Factor ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Predictive Accuracy ◽  
Cross Sectional Study ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Placenta Growth Factor ◽  
Cross Sectional

Abstract Placenta growth factor (PlGF) is released by immature erythrocytes and is elevated in sickle cell disease (SCD). Previous data generated in vitro suggest that PlGF may play a role in the pathophysiology of SCD-associated pulmonary hypertension (PHT) by inducing the release of the vasoconstrictor, endothelin-1. In this cross-sectional study of 74 patients with SCD, we confirm that PlGF is significantly elevated in SCD compared with healthy control subjects. We found significantly higher levels of PlGF in SCD patients with PHT but observed no association of PlGF with the frequency of acute pain episodes or history of acute chest syndrome. The observed correlation between PlGF and various measures of red cell destruction suggests that hemolysis, and the resultant erythropoietic response, results in the up-regulation of PlGF. Although relatively specific, PlGF, as well as N-terminal pro-brain natriuretic peptide and soluble vascular cell adhesion molecule, has low predictive accuracy for the presence of PHT. Prospective studies are required to conclusively define the contribution of PlGF to the pathogenesis of PHT and other hemolytic complications in SCD.

The Department of Health and Human Services (DHHS) Sickle Cell Disease (SCD) Initiative: Increasing Access and Improving Care

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4834-4834
Author(s):  
Susan B. Shurin ◽  
Hani Atrash ◽  
Coleen Boyle ◽  
R. Lorraine Brown ◽  
Janet L. Collins ◽  
...  
Keyword(s):  
Health Care ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Health Care Providers ◽  
The United States ◽  
Evidence Based ◽  
Care Providers ◽  
Cell Disease ◽  
Evidence Based Guidelines ◽  
Support Research

Abstract Abstract 4834 Over the past half century, the course of sickle cell disease has been transformed in the United States through the conduct of rigorous biomedical research and broad application of the results. Universal newborn screening with comprehensive medical care has dramatically reduced death and disability in childhood, and increased the numbers of patients surviving into adulthood. However, access to health care has not kept up with the changing demographics of those affected by sickle cell disease. Health care often becomes fragmented when patients transition from pediatric to adult health care providers. Access to comprehensive care has impeded both conduct of clinical and implementation of research results. To address these needs in this changing environment, HHS Secretary Kathleen Sebelius has charged six agencies of HHS – NIH, CDC, HRSA, FDA, AHRQ and CMS – and the Offices of Minority Health and Planning and Evaluation, to improve the health of people with SCD. The agencies are coordinating their programs and collaborating with the Office of the Secretary, to achieve the following goals:create a comprehensive database of individuals with SCD to facilitate the monitoring of health outcomes and clinical research;improve the care of adults and children through development and dissemination of evidence-based guidelines, which are anticipated in Spring, 2012, with broad implementation plans;identify measures of quality of care for individuals with SCD and incorporate them into quality improvement programs at HHS;increase the availability of medical homes to improve patient access to quality primary and specialty care;provide State Medicaid officials, health care providers, patients, families and advocacy groups with information about resources related to SCD care and treatment;work with the pharmaceutical industry and academic investigators to increase the development of effective treatments for patients with SCD;support research to improve health care for people with SCD;support research to understand the clinical implications of SC trait;engage national and community-based SCD advocacy organizations and experts in ongoing discussions to ensure that issues of importance to persons affected are addressed. Organizational and strategic actions are being taken at each agency to enhance implementation of research advances; provide evidence-based guidelines to families, health care providers, and payers; facilitate new drug development; and provide public health data to impact both the health care delivery and research agendas. The enthusiastic support of the American Society of Hematology and its members is essential for long-term success of this endeavor. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Relationship between painful manifestations and the use of hydroxyurea in children and adolescents with sickle cell disease

2020 ◽  
Vol 9 (10) ◽  
pp. e6949109046
Author(s):  
Suzana Silva Lira ◽  
Caroline Maria Igrejas Lopes ◽  
Gabriella Aguiar Rodrigues Veras ◽  
Valdenice Aparecida de Menezes
Keyword(s):  
Sickle Cell Disease ◽  
Children And Adolescents ◽  
Sickle Cell ◽  
Short Form ◽  
Cross Sectional Study ◽  
High Rate ◽  
Physical Factors ◽  
Cell Disease ◽  
Cross Sectional ◽  
Painful Events

To analyze the profile of pain, its triggering factors, and the relationship with the use of hydroxyurea in children and adolescents with sickle cell disease. This was an analytical cross-sectional study of 80 patients with sickle cell disease, both male and female, aged 6 to 18 years, seen at the Center for Hematology and Hemotherapy of Pernambuco, Brazil. To assess the pain profile, forms with the adapted visual scale from the “Fear of Dental Pain Questionnaire – Short Form” were used. Fisher's exact test was used to evaluate the association between pain manifestations and the use of hydroxyurea. Pain was reported by 68.7% of the patients and 52.7% of them reported severe pain, which eventually affected their daily routines. Physical triggers presented the highest rate (78.2%). The most frequent sites of pain were the trunk (80%) and lower extremities (54.5%), and constricting (40%) and deep (40%) pain were the most frequent types. Thirty percent of the patients reported being treated with hydroxyurea for prevention of painful events. It is concluded that the patients with sickle cell disease showed a high rate of painful events and physical factors, such as cold temperature, trauma and physical effort, were considered to be the most prevalent. There was no association between the use of hydroxyurea and pain improvement.

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