A nationwide survey of hospital-based thalassemia patients and standards of care and a preliminary assessment of the national prevention program in Sri Lanka

• Published in: PloS one 2019-08, Vol.14 (8), p.e0220852-e0220852
• Main Authors: Premawardhana, Anuja P, Mudiyanse, Rasnayaka, De Silva, Shamila T, Jiffry, Nilam, Nelumdeniya, Udaya, de Silva, Udaya, Lamabadusuriya, Sanath P, Pushpakumara, K, Dissanayaka, Randima, Jansz, M, Rifaya, I, Navarathne, Upul, Thirukumaran, V, Arambepola, Mahinda, Dayanada Bandara, Wijesundara, Vaidyanatha, U, Mendis, Devan, Weerasekara, K, De Silva, Nalika, Shantha Kumara, D K, Amarasena, Sujeewa D, Hemantha, K K, Refai, M A C M, Silva, Ishari, Hameed, Nizri, Rajiyah, F, Mettananda, Sachith, Allen, Angela, Weatherall, David J, Oliveri, Nancy F
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; beta-Thalassemia - epidemiology; beta-Thalassemia - prevention & control; beta-Thalassemia - therapy; Biology and Life Sciences; Blood diseases; Cancer; Care and treatment; Child; Child, Preschool; College graduates; Cross-Sectional Studies; Deferasirox; Female; Ferritin; Glycosylated hemoglobin; Graduates; Health surveys; Hematology; Hemoglobin; Hemoglobins; Humans; Infant; Male; Medical care; Medical care quality; Medical personnel training; Medical records; Medicine; Medicine and Health Sciences; Middle Aged; Mortality; Patients; Pediatrics; People and places; Polls & surveys; Prevention; Quality management; Quality of Health Care; Sickle cell disease; Sri Lanka; Sri Lanka - epidemiology; Teaching hospitals; Thalassemia; Thalassemia - epidemiology; Thalassemia - prevention & control; Thalassemia - therapy
• ISSN: 1932-6203; 1932-6203
• DOI: 10.1371/journal.pone.0220852

Our aim was to describe the numbers and distribution of patients with different types of thalassemia and to assess the standards of care in all thalassemia treatment centers throughout Sri Lanka and the success of the ongoing prevention programme. This cross-sectional island-wide survey was conducted by two trained medical graduates, who visited each thalassemia center to collect data from every patient, using a standardized form. Data was collected through review of patient registers and clinical records. We collected data on 1774 patients from 23 centers. 1219 patients (68.7%) had homozygous β-thalassemia, 360 patients (20.3%) had hemoglobin E β-thalassemia, and 50 patients (2%) had sickle β-thalassemia. There were unacceptably high serum ferritin levels in almost all centers. The annual number of births of patients with β-thalassaemia varied between 45-55, with little evidence of reduction over 19 years. Central coordination of the treatment and ultimately prevention of thalassemia is urgently needed in Sri Lanka. Development of expert centers with designated staff with sufficient resources will improve the quality of care and is preferred to managing patients in multiple small units.