7 research outputs found
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Newborn Screening for Sickle Cell Disease Using Point-of-Care Testing in Low-Income Setting
Newborn screening provides early diagnosis for children with sickle cell disease (SCD), reducing disease-related mortality. We hypothesized that rapid point-of-care (POC) Sickle SCAN would be reliable in Haiti and would assist newborn screening.
Dried blood specimens were obtained from infant heel sticks and analyzed by isoelectric focusing (IEF) at a public hospital in Cap-Ha茂tien during a 1-year period. A total of 360 Guthrie cards were also analyzed for quality assurance by high-performance liquid chromatography at the Florida Newborn Screening Laboratory. In addition, two-thirds of the infants were also screened by the POC to assess differences with the IEF. The hemoglobinopathy incidence and the specificity and sensitivity of the POC scan were assessed.
Overall, 1.48% of the children screened positive for SCD. The specificity and the sensitivity of POC Sickle SCAN were 0.97 (confidence interval 0.95-0.99) and 0.90 (confidence interval 0.55-1.00), respectively, relative to high-performance liquid chromatography gold standard. The confirmatory testing rate was 75% before POC and improved to 87% after POC was added for dual screening. Confirmatory testing revealed that 0.83% of children screened had SCD. Children who screened positive for SCD by POC started penicillin earlier, had their first pediatric follow-up a median of 38 days earlier, and received antipneumococcal vaccination on time when compared with those who screened positive for SCD by IEF alone.
The observational study revealed a high incidence of SCD among Haitian newborns. Sickle SCAN had excellent specificity and sensitivity to detect SCD during newborn screening and shortened health care access for children positive for SCD
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Role of Point-of-Care Device in Improving Sickle Cell Newborn Screening Feasibility in Haiti
Abstract Introduction: Until 2017, hemoglobinopathy newborn screening (NBS) was not offered in Haiti, a country with about 243,000 births per year. Sickle SCAN is a rapid point-of-care (POC) with lateral flow immunoassay technology, but its role in NBS is unknown. Recently, over 100 dried blood samples from newborns were tested [Nguyen-Khoa T, Ann Biol Clin (Paris), 2018] in France with accurate results. Objective: This work had the objective to be proof-of-concept that a hospital-based NBS program is feasible in Haiti and to examine the role of Sickle SCAN in NBS in a larger sample size. Methods: The Ethics Committee at the Universitaire Justinien Hospital (HUJ) and the Institutional Review Board at the University of Miami approved this study. We formed a Haitian team composed of a pediatrician on staff (RSF), two dedicated NBS nurses, a program coordinator (MV), two community health workers, a laboratory technician (RR), and a data manager (UL). We acquired isoelectric focusing equipment (IEF) and performed NBS from dried blood samples only for the first three months while the technician gained proficiency. Thereafter, we have performed dual screening method with the POC device Sickle SCAN and IEF. Confirmatory testing was obtained with both methods. Data were entered into RED Cap. Results: Beginning in August 2017 until present (June 2018), we have screened 1,800 newborns, of which 10.5% have sickle cell trait, 3.3% have hemoglobin C trait, 0.78% have hemoglobin SS, 0.27% have hemoglobin SC and one child has been confirmed to have sickle beta thalassemia plus. Currently, there are 15 children followed at HUJ, for a SCD incidence of 0.83% (8 SS, 6 SC and 1 S-beta thalassemia+). Before the POC screening was implemented two infants have already died when the mothers were contacted (one who screened positive for FS and another for FC). The POC allows for immediate referral and penicillin prophylaxis for at-risk children until the cases are confirmed. Systemic barriers encountered are the electrical outages and intense heat which impacted IEF performance, material procurement (laboratory materials, NBS materials, oral penicillin) which are dependent of shipments from outside of Haiti. Conclusions: A hospital-based NBS Program is feasible. SCD is highly prevalent with an incidence of 0.83% among newborns in the population studied. Based on this incidence, we estimate that every year around 2,000 children will be born with sickle cell disease in Haiti. The point-of-care device enhanced the screening program by obtaining immediate screening results and maximizing family notification for confirmatory testing. Disclosures No relevant conflicts of interest to declare
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Capacity building and networking to make newborn screening for sickle cell disease a reality in Haiti
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Implementation of Hospital-Based Sickle Cell Newborn Screening and Follow-Up Programs in Haiti
One in 120 children are born with sickle cell disease (SCD) in Haiti. However, healthcare challenges include isolated newborn screening (NBS) activities and lack of transcranial Doppler ultrasound (TCD) to assess stroke risk. The implementation activities of the "Comparative Study of Children in Haiti and Miami with Sickle Cell Disease (CSHSCD) involved both NBS and TCD implementations in four Haitian clinical sites. We hypothesized that hospital-based SCD NBS and follow-up programs would be feasible in Haiti. Traditional NBS laboratory method with dried blood samples was performed at two Port-au-Prince sites, and the traditional method plus point-of-care (POC) testing was utilized at the two north sites. The rate of clinical follow-up for newborns with SCD as outcome for the NBS intervention was compared to the NBS method. The NBS programs identified SCD in 0.77% of 8,224 newborns over a 24-month period. In the rural hospital assigned to the combination screening, 56% of newborns identified with POC returned for follow-up compared to 0% when POC was not available (p=0.044). Positive newborns and children <6 years of age with SCD at the clinical sites were eligible for study follow-up. Accrual was successful: 165 participants (mean age 42 months, 53% males, 93% SS) were recruited and received oral penicillin. TCD screening was hampered by poor internet and trained staff leaving Haiti, with only one active site conducting screening. Despite challenges, the implementation of NBS and sickle cell programs in Haiti is feasible. We are in the process of understanding how to mitigate implementation limitations
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First Year Comparison of Sickle Cell Pediatric Cohorts from Haiti and Miami (CSHSCD Multicenter Study): Baseline Data
AbstractBackground: The NIH-sponsored observational study "Comparative Study of Haiti and Miami Cohorts of Sickle Cell Disease CSHSCD" (R01HL149121) coordinates the follow up of children with sickle cell disease (SCD) in Haiti and compares it to a Miami cohort of children of either Haitian or African American ethnicity for the purpose of assessing barriers through questionnaires and examining differences in the care received in their respective environments.Methods: Children less than 6 years of age with SCD are eligible for enrollment in five participating sites: University of Miami (UM, Miami, Florida), H么pital Saint Damien (HSD, Tabarre, Haiti), H么pital de l' Universit茅 d'Etat d'Ha茂ti (HUEH, Port-au-Prince, Haiti), H么pital Universitaire Justinien (HUJ, Cap Haitien, Haiti), and H么pital Sacr茅 Coeur (HSC, Milot, Haiti).Medians and interquartile ranges or percentages were compared at baseline regarding demographics, clinical and growth parameters, laboratory tests, and the children's hydroxyurea (HU) utilization during the first year of enrollment (May 25, 2020-May 24,2021). A Likert-scale barrier questionnaire was distributed at baseline to assess differences in healthcare access. A P value <0.05 was considered statistically significant to establish differences.Results: 130 children were enrolled during the reported period. Significant differences were observed in age, weight percentiles, hemoglobin levels, pain rates, HU treatment, and pneumococcal vaccination.Penicillin prophylaxis was always given by oral route in Miami, but only 39.8% times in Haiti, with 58% of children receiving prophylaxis by intramuscular injection every month and 2.2% (N=2 children) with either unknown or not receiving prophylaxis. Previous medication outsourcing accounted for the oral tablet form in Haiti. Parents in Haiti had more barriers regarding not able to afford treatment (21.5% compared to 8.1% in Miami) and had similar responses regarding not able to afford coming to clinic (21.5% vs. 18.9%). Parents in Miami expressed living far away from clinic (70.2% compared to 25.8% in Haiti), but had more help from other family members (78.4% vs. 33.3%). Interestingly, parents in Miami did not know sometimes what to do when the child was sick (40% respondents vs. 11% in Haiti). There were no major differences between the responses from the African Americans and Haitians living in Miami, except for not knowing sometimes what to do when child is sick (African-Americans having less doubts than Haitians; 25% vs. 52.6%). In short-term follow up, no enrolled children died, although two eligible children in Haiti died before enrollment. One child developed COVID-19 in Miami with only mild symptoms, which resolved.Conclusion: At entry children in Haiti are older, weigh less, are more anemic, have more pain episodes, and fewer receive hydroxyurea treatment. Under-vaccination with pneumococcal 13-valent conjugate (Prevnar-13) is notable in Haiti. There were significant differences detected on the barrier questionnaire among respondents in both countries.Acknowledgment: We acknowledge NHLBI for supporting this work.Figure 1 Figure 1.DisclosuresAlvarez:鈥圙BT: Membership on an entity's Board of Directors or advisory committees; Forma Therapeutics: Membership on an entity's Board of Directors or advisory committees
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Comparative Study of Haiti and Miami Cohorts of Sickle Cell Disease (CSHSCD): Methods, Accomplishments, and Implementation
Abstract
Background: There are significant limitations in Haiti for the diagnosis and management of sickle cell disease (SCD), including the non-availability of universal newborn screening (NBS) and transcranial Doppler (TCD) ultrasound screening, and the lack of diagnostic laboratory resources, oral penicillin and hydroxyurea (HU).
Methods: Beginning in September 2019, CSHSCD (R01HL149121), a 5-year NIH-sponsored observational comparative study of children with SCD from Haitian ethnicity in Miami and in Haiti compared to children of African American ethnicity with SCD, was designed to increase access to care in Haiti. The study aims are 1) to compare the incidence of SCD among newborns from Haitian and African American ethnicity in Miami, 2) to establish NBS programs for hemoglobinopathies in Haiti, and 3) to compare cohorts of children in SCD at the study sites. The participating sites are the University of Miami (UM, Miami, Florida), H么pital Saint Damien (HSD, Tabarre, Haiti), H么pital de l'Universit茅 d'Etat d'Ha茂ti (HUEH, Port-au-Prince, Haiti), H么pital Universitaire Justinien (HUJ, Cap Haitien, Haiti), and H么pital Sacr茅 Coeur (HSC, Milot, Haiti). HUJ and HSC use two NBS screening methods (isoelectric focusing and Sickle SCAN rapid test) and HSD and HUEH use isoelectric focusing only. CSHSCD supplies penicillin and HU and trains TCD examiners to implement stroke risk screening. Data are collected in REDCap.
Results: During the first 2 years and despite the COVID-19 pandemic, we established NBS sites with a cohesive network of physicians and nurses trained in the care of children with SCD in Haiti. This capacity building will support sustainability of the program. We successfully identified at least 15 new cases of SCD via newborn screening, trained six TCD examiners, and enrolled 130 children with SCD in follow up, providing them with penicillin prophylaxis and hydroxyurea for severe cases according to local protocols . Implementation activities which have helped are close communications between the investigators, monthly Zoom meetings to coordinate efforts with enrollment updates every month, the availability of rapid tests (Sickle SCAN and Gazelle miniature cellulose acetate electrophoresis) for the diagnosis of SCD, especially when there is no laboratory equipment on site. Implementation challenges we have faced are mostly two. The first is the timely completion of DUNS and SAM registration for the two public hospitals, with one site achieving this after 9 months and the other site taking 18 months to complete. The reasons for the delay are the inability for the UM site to direct these efforts, following strict rules, and the Haitian hospital officers' lack of familiarity with website requirements. We were able to achieve these registrations with the assistance of one Haitian study staff who is very acquainted with internet navigation and became familiarized with requirements. Outsourcing materials to Haiti is another major challenge, with either gaps in the delivery of supplies because of multiple steps involved in ordering and shipping or with delays in releasing equipment once it is at the Port-au-Prince customs, resulting in gaps in NBS in one of the sites for 8 weeks. We have minimized these issues by opening a one-year ticket to order materials from the different companies involved. Also, Haiti's lack of infrastructure, available materials and medications, and political instability limit health care delivery.
Conclusion: Since its inception, we have achieved major milestones, including capacity building and implementation of NBS, TCD training, and enrollment of children with SCD into the prospective cohorts despite the current COVID-19 pandemic. Material outsourcing challenges have been the major implementation problem we have faced due to systemic factors. We anticipate that these factors will be corrected or minimized as we have learned how to handle them. These problems were expected as part of conducting an international study in a low-resource setting.
Acknowledgment: We acknowledge NHLBI for supporting this work.
Disclosures
Alvarez:鈥團orma Therapeutics: Membership on an entity's Board of Directors or advisory committees; GBT: Membership on an entity's Board of Directors or advisory committees. Romano:鈥圙enentech: Research Funding; Vycor: Current holder of individual stocks in a privately-held company; NovaVision: Consultancy