Objectives: Sickle cell disease (SCD) is the commonest genetic disorder in Jamaica and greatly affects the quality of life (QOL) of those who are afflicted. The Short Form 36 survey (SF-36) questionnaire is one of the most commonly utilized measures of QOL. Physicians cannot interpret QOL measures until the instruments being used to make assessment are adequately established in their population. The Jamaican cultural and educational systems expose its people to many stresses which likely impact on their QOL. It is thus postulated that the QOL construct may exhibit a different structure for the population with sickle cell disease.
Subjects And Methods: The SF-36 v.2 was interviewer administered to the Jamaican Sickle Cell Disease Cohort Study participants ('Cohort' sample) and a random sample of adult sickle cell unit patients ('Main'sample). Demographic data were also collected on both groups. Both of the samples did not meet the five rule criteria for compliance with the original SF-36 component structure. Hence, principal components analysis was used to determine the component structure of the SF-36 in both groups.
Results: Three dimensions may underlie the SF-36 for both groups and these could be labelled 'Physical Health', 'Mental Health' and 'Role Limitations'. This solution accounted for 45.8% of the variability underlying the SF-36 in the 'Cohort'sample and 54.6% of the variability in the 'Main' sample.
Conclusions: It concluded that within Jamaican samples of patients with sickle cell disease, the SF-36 has a component structure which is quite distinct from that initially proposed by its creators.
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BMJ Glob Health
January 2025
Sickle Cell Programme, Department of Haematology and Blood Transfusion, Muhimbili University of Health and Allied Sciences, Dar es Salaam, Tanzania.
Despite progress in healthcare services for individuals living with sickle cell disease (SCD) in Africa, substantial gaps remain in advanced treatments for SCD. To help address this burden, Tanzania has established one of the largest single-centre SCD programmes in the world and developed an advanced therapy programme for SCD focused on patient engagement and advocacy, clinical activities involving exchange blood transfusion (ExBT) and haematopoietic stem cell transplant (HSCT), gene therapy (GT) preparedness, and enabling partnerships. This report describes the programme's genesis, structure and progress achieved.
View Article and Find Full Text PDFComput Biol Med
January 2025
SCOPIA Research Group, University of the Balearic Islands, Dpt. of Mathematics and Computer Science, Crta. Valldemossa, Km 7.5, Palma, E-07122, Spain; Health Research Institute of the Balearic Islands (IdISBa), Palma, E-07122, Spain; Laboratory for Artificial Intelligence Applications at UIB (LAIA@UIB), Palma, E-07122, Spain; Artificial Intelligence Research Institute of the Balearic Islands (IAIB), Palma, E-07122, Spain. Electronic address:
Sickle cell disease causes erythrocytes to become sickle-shaped, affecting their movement in the bloodstream and reducing oxygen delivery. It has a high global prevalence and places a significant burden on healthcare systems, especially in resource-limited regions. Automated classification of sickle cells in blood images is crucial, allowing the specialist to reduce the effort required and avoid errors when quantifying the deformed cells and assessing the severity of a crisis.
View Article and Find Full Text PDFBlood
January 2025
New York Blood Center, New York, New York, United States.
Babesiosis in sickle cell disease (SCD) is marked by severe anemia but the underlying red blood cell (RBC) rheological parameters remain largely undefined. Here, we describe altered RBC deformability from both primary (host RBC sickle hemoglobin mediated) and secondary changes (Babesia parasite infection mediated) to the RBC membrane using wild type AA, sickle trait AS and sickle SS RBCs. Our ektacytometry (LORRCA) analysis demonstrates that the changes in the host RBC bio-mechanical properties, pre- and post- Babesia infection, reside on a spectrum of severity, with wild type infected AA cells, despite showing a significant reduction of deformability under both shear and osmolarity gradients, exhibiting only a mild phenotype; compared to infected AS RBCs which show median changes in deformability and infected SS RBCs which exhibit the most dramatic impact of infection on cellular rheology, including an increase in Point of Sickling values.
View Article and Find Full Text PDFPak J Med Sci
January 2025
Khalid Khalil Security Forces Hospital Makkah, Makkah, Saudi Arabia.
Objective: To observe the fetomaternal outcome of therapeutic versus prophylactic blood transfusions in patients with sickle cell disease (SCD) during pregnancy.
Method: This single-center retrospective observational study was conducted on consecutive pregnant women with SCD between January 2018 and December 2020. All the pregnant women with SCD were included in this study.
Non-myeloablative hematopoietic cell transplantation (HCT) is a curative option for individuals with sickle cell disease (SCD). Our traditional goal with this approach has been to achieve a state of mixed donor/recipient chimerism. Recently, we reported an increased risk of hematologic malignancies (HMs) in adults with SCD following graft failure or mixed chimerism.
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