Children with sickle cell disease migrating to the United States from sub-Saharan Africa

2018 ◽  
Vol 65 (6) ◽  
pp. e27000 ◽  
Author(s):  
Courtney D. Thornburg ◽  
Russell E. Ware
Keyword(s):  
United States ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
The United States ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Sub Saharan

Living with sickle cell disease: voices from sub-Saharan Africa

2021 ◽  
Author(s):  
Arafa Said Salim ◽  
Emmy Mwita ◽  
Joseph Sarfo Antwi ◽  
Olamide Agunkejoye ◽  
Paul Mdliva
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Sub Saharan

Abstract 65: Anemia-related Heart Failure in Sub-saharan African Sickle Cell Disease Patients

2017 ◽  
Vol 121 (suppl_1) ◽  
Author(s):  
Adebayo C Atanda ◽  
Yahya Aliyu ◽  
Oluwafunmilayo Atanda ◽  
Aliyu Babadoko ◽  
Aisha Suleiman ◽  
...  
Keyword(s):  
Heart Failure ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Systolic Pressure ◽  
Left Ventricular ◽  
Hemoglobin Level ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Jet Velocity ◽  
Sub Saharan

Introduction: Anemia has been implicated in heart failure. Existing literatures, involving predominantly African-Americans, suggests that Sickle Cell Disease (SCD) maybe linked to various cardiovascular complications including pulmonary hypertension and left venticular dysfunction. Peculiarly, our study involves exclusively Sub-Saharan population. Method: We conducted a cross sectional observational study of 208 hydroxyurea-naive consecutive SCD patients aged 10-52 years at steady state and 94 healthy non-matched controls who were studied in an out patient clinic in Sub-Saharan Africa. SCD patients were required to have electrophoretic or liquid chromatography documentation of major sickling phenotypes. Control group was required to have non-sickling phenotypes. Cardiac measurements were performed with TransThoracic Echo according to American Society of Echocardiography guidelines. Hemoglobin level was also obtained. Results: Hemoglobin level in SCD group (8.5+/- 1.5) was significant (P<0.001) compared to control (13.8+/- 1.7). Although SCD group had significantly higher values of left ventricular (LV) size, there was no qualitative evidence of LV dysfunction. SCD group had higher values of Ejection Fraction but not statistically significant. There was no evidence of LV wall stiffening to impair proper filling in SCD group, with the ratio of early to late ventricular filling velocities, E/A ratio elevated (1.7+/-0.4 compared to 1.6+/- 0.4; P=0.010). Right ventricular systolic pressure was determined using the formula of 4x Tricuspid Reugurgitant jet (TRV) square as an indirect measurement of Pulmonary arterial systolic pressure. SCD patients had significantly higher mean±SD values for tricuspid regurgitant jet velocity than did the controls (2.1±0.6 vs. 1.8±0.5; p= 0.001). Within the SCD group, there was no clear pattern of worsening diastolic function with increased TRV. Furthermore, E/A had a significant positive relationship with jet velocity in bivariate analysis (R=0.20; P=0.013). Conclusions: We were unable to demonstrate existence of anemia-associated left ventricular dysfunction in Sub-Saharan African with SCD. Further studies is required to highlight the reason behind this finding.

scholarly journals Advancing Healthcare Outcomes for Sickle Cell Disease in Nigeria Using Mobile Health Tools

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 2173-2173
Author(s):  
Arwa Fraiwan ◽  
Muhammad Noman Hasan ◽  
Ran An ◽  
Amy J. Rezac ◽  
Nicholas J. Kocmich ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Mobile Health ◽  
Sub Saharan Africa ◽  
Diagnosis And Treatment ◽  
Test Results ◽  
Cell Disease ◽  
The World ◽  
Equity Ownership ◽  
Sub Saharan

Nigeria leads the world in the number of cases of sickle cell disease (SCD). An estimated 150,000 babies are born annually in Nigeria with SCD, a heredity disorder, and 70-90% die before age 5. Only a small portion of affected infants and children in sub Saharan Africa (SSA) reach adolescence. Over 650 children die per day in sub-Saharan Africa from SCD. These dismal statistics are in sharp contrast to outcomes in high-income countries (HICs) where more than 90% of SCD patients reach adulthood. The World Health Organization (WHO) estimates that 70% of deaths could be prevented with a low cost diagnostic and treatment plan. Meaningful preventive care and treatment cannot be implemented without a structured plan for early diagnosis and patient tracking.Early diagnosis requires improved access to parents and guardians of children with SCD, and gaining this access remains a challenge in most of SSA. In 2015, Nigeria's Kano state government, with support from foreign partners, established a community-based program for newborn registration. This platform provides unique access to newborn babies in one of Nigeria's most populous cities, but still lacks a functioning patient testing, tracking, and monitoring system, which we plan to address in our ongoing study. This study will introduce mobile health in a low-income country with low literacy rate and hopefully accustom that segment of the population to more varied mobile health applications that will ultimately improve their health in the long run. Our current operational platform in Kano, Nigeria provides access to a large population with a high prevalence of SCD. We have previously completed pilot testing of 315 subjects for SCD using our microchip electrophoresis test. We are planning to test up to 4,500 additional subjects less than 5 years of age at Murtala Muhammed Specialist Hospital. The hospital staff includes 97 physicians and 415 nurses and outpatient clinics serve about 30,000 patients monthly. The maternity department has a 200-bed capacity and the antenatal clinic performs about 1,000 deliveries and serves an average of 3,000 mothers monthly. Enrollment is planned to start on September 15, 2019 and medical staff are currently being trained to run the tests. Our study is registered in the United States National Library of Medicine's ClinicalTrials.gov (Identifier: NCT03948516). Our technology is uniquely paired with an automatic reader and an Electronic Medical Record (EMR) and patient management solution to record POC test results, register new cases, and track patients for follow-up (Fig. 1). The reader enables automated interpretation of test results, local and remote test data storage, and includes geolocation (Global Positioning System) (Fig. 2). The system will generate reports for all cases of SCD, track hospital visits, appointments, lab tests, and will have mobile and dashboard applications for tracking patients and samples. The application will be installed on mobile devices provided to users. The proposed system will be compliant with the existing privacy standards to handle medical data (e.g., HIPAA in the US and GDPR in the EU). All communications between the parties will be secured via end-to-end encryption as a safeguard. We anticipate that our project will increase the rates of screening, diagnosis and timely treatment of SCD in Kano State of Nigeria. The project's broader impact will likely be the ability to track and monitor screening, disease detection, diagnosis and treatment, which can be scaled up to the whole nation of Nigeria, then to sub-Saharan Africa. The data obtained and analyzed will be the first of their kind and will be used to inform the design of programs to improve access to, and availability of, effective care for this underserved populations. The importance of increased access to diagnosis and treatment should not be underestimated - it is crucial for realizing effective management of people with SCD. The impact can be enhanced by complementing diagnosis and patient tracking with education for the families so they can provide or seek the necessary preventative treatment. Identification of the location of the patients in need would help identify the areas where family, parent, caregiver education should be provided. Disclosures Fraiwan: Hemex Health, Inc.: Equity Ownership, Patents & Royalties. Hasan:Hemex Health, Inc.: Equity Ownership, Patents & Royalties. An:Hemex Health, Inc.: Patents & Royalties. Thota:Hemex Health, Inc.: Employment. Gurkan:Hemex Health, Inc.: Consultancy, Employment, Equity Ownership, Patents & Royalties, Research Funding.

Red cell storage age policy for patients with sickle cell disease: A survey of transfusion service directors in the United States

2016 ◽  
Vol 54 (1) ◽  
pp. 158-162 ◽  
Author(s):  
Matthew S. Karafin ◽  
Arun K. Singavi ◽  
Mehraboon S. Irani ◽  
Kathleen E. Puca ◽  
Lisa Baumann Kreuziger ◽  
...  
Keyword(s):  
United States ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
The United States ◽  
Red Cell ◽  
Cell Disease ◽  
Cell Storage

scholarly journals Antimicrobial Resistance and Sickle Cell Protein Analysis in Invasive Non-Typhoidal Salmonella Isolated From Outpatient and Hospitalized Children Below 16 Years in Informal Settlements in Nairobi Kenya

2020 ◽  
Author(s):  
Susan Kavai ◽  
Cecilia Mbae ◽  
Celestine Wanjiku ◽  
Ronald Ngetich ◽  
Zillah Wakio ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Salmonella Typhimurium ◽  
Sickle Cell ◽  
Informal Settlements ◽  
The United States ◽  
Sub Saharan Africa ◽  
Cell Protein ◽  
Cell Disease ◽  
Resistance Phenotype ◽  
Stool Samples

Abstract Background: Invasive Non-typhoidal Salmonella (iNTS) disease continues to be a major public health problem, especially in sub Saharan Africa where incidence rates are 227 cases [range 152-341] per 100,000 population. Populations at risk of iNTS include adults with HIV infection, malnourished children, those with recent malaria or sickle-cell anaemia (SCA). Individuals with SCA are at an increased risk of invasive bacterial infections with the proportion of deaths from infection reported to be as high as 38% in the United States and 29% in Jamaica. In Kenya, iNTS disease is particularly a major challenge in poor informal settlements with infants and young children less than 5 years of age being the most affected; mortality rates can be 20-25% unless prompt treatment is administered. Methods Our study was conducted in 3 outpatient sites and 1 inpatient site, the outpatient sites were all located within Mukuru informal settlement, a densely populated slum, 15km East of Nairobi City. Blood and stool samples from children with fever alone and with fever and diarrhea were collected for processing for presence of iNTS using basic microbiology procedures. Dry blood spots were also taken and processed for sickle cell protein markers using High performance liquid chromatography (HPLC). Results A total of 22,246 blood and stool samples were collected from children < 16 years of age with fever/with or without diarrhea, for a period of 6 years and subjected to microbiological culture and detection of bacterial pathogens. Out of these 741 (3.3%) tested positive for Salmonella species. A total of 338/741(41%) NTS were isolated across all the sites; these consisted of 158/741(21%) Salmonella Enteritidis and 180/741 (24%) Salmonella Typhimurium. The most common resistance phenotype was ampicillin, cotrimoxazole and chloramphenicol (35.03%). We had 12/338 (3.6%) isolates (11 of them being Salmonella Typhimurium) that were ESBL producers conferring resistance to 3rd generation cephalosporins (Amp C β-lactamases) while only 0.3% were resistant to ciprofloxacin. A total of 118 (35.03%) isolates were MDR. Out of 2684 dry blood samples subjected to HPLC for investigation of sickle cell disease traits, 1820/2684 (67%) had normal hemoglobin (Hb AA/ Hb AF); (162/2684 (6%) tested positive for Sickle Cell Traits (Hb AS/Hb AFS); while 4/2684 (0.2%) tested positive for Sickle cell disease (Hb FS). Conclusion The high MDR resistance phenotype in iNTS isolates and emerging resistance to third generation cephalosporins is of great concern in management of iNTS in our settings. Sickle cell disease was not a major factor among children with iNTS disease and no significant association with iNTS was observed.

scholarly journals Management of chronic respiratory complications in children and adolescents with sickle cell disease

2020 ◽  
Vol 29 (157) ◽  
pp. 200054
Author(s):  
Michele Arigliani ◽  
Atul Gupta
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Current Knowledge ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Respiratory Complications ◽  
Progressive Decline ◽  
Blood Disorder ◽  
Life Threatening ◽  
Sub Saharan

Sickle cell disease (SCD) is a life-threatening hereditary blood disorder that affects millions of people worldwide, especially in sub-Saharan Africa. This condition has a multi-organ involvement and highly vascularised organs, such as the lungs, are particularly affected. Chronic respiratory complications of SCD involve pulmonary vascular, parenchymal and airways alterations. A progressive decline of lung function often begins in childhood. Asthma, sleep-disordered breathing and chronic hypoxaemia are common and associated with increased morbidity. Pulmonary hypertension is a serious complication, more common in adults than in children. Although there is a growing attention towards respiratory care of patients with SCD, evidence regarding the prognostic meaning and optimal management of pulmonary issues in children with this condition is limited.This narrative review presents state-of-the-art evidence regarding the epidemiology, pathophysiology and therapeutic options for chronic respiratory complications commonly seen in paediatric patients with SCD. Furthermore, it highlights the gaps in the current knowledge and indicates future directions for studies that aim to improve our understanding of chronic respiratory complications in children with SCD.

scholarly journals The Economic Burden of Sickle Cell Disease in the United States

2018 ◽  
Vol 21 ◽  
pp. S108 ◽  
Author(s):  
J Huo ◽  
H Xiao ◽  
M Garg ◽  
C Shah ◽  
DJ Wilkie ◽  
...  
Keyword(s):  
United States ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Economic Burden ◽  
The United States ◽  
Cell Disease

Childhood Hearing Loss in Patients With Sickle Cell Disease in the United States

2019 ◽  
Vol 41 (2) ◽  
pp. 124-128 ◽  
Author(s):  
Heather K. Schopper ◽  
Christopher F. D’Esposito ◽  
John S. Muus ◽  
Julie Kanter ◽  
Ted A. Meyer
Keyword(s):  
United States ◽  
Hearing Loss ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
The United States ◽  
Cell Disease ◽  
Childhood Hearing Loss

scholarly journals Liver Transplantation in Patients with Sickle Cell Disease in the United States

2020 ◽  
Vol 255 ◽  
pp. 23-32 ◽  
Author(s):  
Rachel Hogen ◽  
Michelle Kim ◽  
Yelim Lee ◽  
Mary Lo ◽  
Navpreet Kaur ◽  
...  
Keyword(s):  
United States ◽  
Liver Transplantation ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
The United States ◽  
Cell Disease
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