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A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
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Thalassemia in Sri Lanka.
Anuja P Premawardhena1, H D Prabath Madushanka1
1Department of Medicine, Faculty of Medicine, University of Kelaniya, Ragama, Sri Lanka.
Hemoglobin
|August 11, 2022
Summary
Sri Lanka has a significant burden of thalassemia, particularly beta-thalassemia major. Despite free healthcare, patient survival rates lag behind Mediterranean countries, indicating challenges in the national prevention program.
Area of Science:
- Hematology
- Genetics
- Public Health
Background:
- Sri Lanka, with 22 million people, has an estimated 2000 severe thalassemia patients.
- The majority suffer from beta-thalassemia major (β-TM), with Hb E/β-thal accounting for most remaining cases.
- Carrier rates for alpha+ thalassemia trait (9.9%) and beta-thalassemia trait (2.5%) are similar across major ethnic groups.
Purpose of the Study:
- To analyze the distribution and impact of thalassemia in Sri Lanka.
- To evaluate the effectiveness of the national thalassemia prevention program.
- To identify challenges in patient survival and healthcare outcomes.
Main Methods:
- Epidemiological analysis of thalassemia prevalence and types.
- Review of national healthcare provisions for thalassemia patients.
- Assessment of the national thalassemia prevention program initiated in 2007.
Main Results:
- Thalassemia type distribution shows significant variation across the island, potentially linked to historical malaria patterns.
- Despite free state-provided healthcare, including transfusions and chelation, β-TM patient survival is suboptimal compared to Mediterranean regions.
- The national prevention program, primarily focused on marriage dissuasion, has shown limited success.
Conclusions:
- Thalassemia poses a considerable public health challenge in Sri Lanka.
- Current prevention strategies require re-evaluation for improved effectiveness.
- Enhancing patient care and survival outcomes necessitates addressing systemic healthcare and prevention program deficiencies.
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