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

Premawardhana, Anuja P; Mudiyanse, RM; Silva, Shamila De; Jiffry, Nilam; Nelumdeniya, Udaya; Silva, Udaya de; Lamabadusuriya, Sanath P; Pushpakumara, Kalavitigoda; Dissanayaka, R; Jansz, Michael; Rifaya, I; Navarathne, Upul; Thirukumaran, V; Arambepola, M.; Bandara, W.D.M.S.; Vaidyanatha, U; Mendis, Devan; Weerasekara, Kumudu; Silva, Nalika De; Kumara, D; Amarasena, Sujeewa; Hemantha, K K; Refai, Majed; Silva, Ishari; Hameed, Nizri; Rajiyah, F; Mettananda, Sachith; Allen, Angela; Weatherall, D. J.; Oliveri, N.F.

doi:10.1371/journal.pone.0220852

Cited by 28 publications

(26 citation statements)

References 17 publications

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“…One important limitation of our study is that we are unable to generalise the finding of this study to all centres caring for patients with thalassaemia in Sri Lanka. We previously showed that the care provided by larger specialised thalassaemia centres is better than the care provided by smaller centres in the country [9]. Therefore, it is likely that the psychological morbidity of children taking treatment from smaller thalassaemia centres is worse than what is described here.…”

Section: Clinical Characteristicsmentioning

confidence: 49%

“…In this paper, we examined the psychological health among children with TDBT and their mothers. We studied almost half of the TDBT patient population in Sri Lanka in the specified age group attending the three main thalassaemia centres of Sri Lanka which are situated in three different provinces of the country [9]. We found that children with TDBT in Sri Lanka have significantly higher prevalence of psychological symptoms in emotional, conduct and hyperactivity domains and abnormal peer relationships and social skills.…”

Section: Discussionmentioning

confidence: 97%

“…β-thalassaemia gene frequency in Sri Lanka is reported as 2.8% whilst 1800 patients with transfusion dependent β-thalassaemia (TDBT) are being treated in twenty-six thalassaemia centres across the country [8]. However, over sixty percent of these patients are managed in the three largest thalassaemia referral centres in Kurunegala, Anuradhapura and Ragama [9].…”

Section: Introductionmentioning

confidence: 99%

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Psychological morbidity among children with transfusion dependent β-thalassaemia and their parents in Sri Lanka

et al. 2020

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Background Thalassaemia is a chronic disease which requires lifelong treatment in a majority. Despite recent advances in the medical care, minimal attempts are made to improve psychological health in these patients. In this study, we aim to describe the psychological morbidity in patients with transfusion dependent β-thalassaemia and their mothers in Sri Lanka. Methods This case control study was conducted in the three largest thalassaemia centres of Sri Lanka. All patients with transfusion dependent β-thalassaemia aged 4-18 years were recruited as cases whilst a randomly selected group of children without chronic diseases were recruited as controls. Psychological morbidity of children was assessed using the Strengths and Difficulties Questionnaire and depressive symptoms of mothers was assessed by the Centre for Epidemiological Studies Depression Scale. Results 288 transfusion dependent β-thalassaemia patients and equal number of controls were recruited. Abnormal emotional, conduct, hyperactivity and peer relationship symptom scores were reported by 18%, 17%, 9% and 14% of patients with thalassaemia respectively. Prevalences of abnormal psychological symptom scores in all domains were significantly higher among patients compared to controls. Abnormal conduct symptoms were significantly more prevalent among patients with HbE β-thalassaemia and those with suboptimal pretransfusion haemoglobin levels, lower transfusion volumes, hypothyroidism and undernutrition. Short stature was associated with abnormal emotional and hyperactivity scores. Depressive symptoms were significantly higher among mothers of patients with

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Section: Clinical Characteristicsmentioning

confidence: 49%

Section: Discussionmentioning

confidence: 97%

Section: Introductionmentioning

confidence: 99%

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Psychological morbidity among children with transfusion dependent β-thalassaemia and their parents in Sri Lanka

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“…The same survey identified significant inconsistencies in care of SCD patients between centres. [11]. Genetic information including haplotype analysis for SCD in Sri Lanka is scarce, and has been reported for a single patient with SBT [12] only.…”

Section: Introductionmentioning

confidence: 99%

Sickle cell disease in Sri Lanka: Clinical and molecular basis and the unanswered questions about disease severeity.

Darshana

Bandara²,

Nawarathne³

et al. 2020

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Background Though case reports and limited case series of Sickle cell disease in Sri Lanka have been reported previously, no attempt has been made hitherto to undertake a comprehensive genotypic-phenotypic analysis of this “rare” group of patients.Results All accessible Sickle cell disease patients, totaling 60, including, 51 Sickle β-thalassaemia and 9 homozygous sickle patients were enrolled from seven thalassaemia treatment centres between December 2016 - March 2019. The majority of patients were of Sinhalese ethnicity (n=52, 86.67%). Geographically, two prominent clusters were identified and the distribution of Sickle haemoglobin in the island contrasted markedly with the other haemoglobinopathies. 3/ 9 homozygous sickle patients and 3/ 51 Sickle β-thalassaemia patients were receiving regular transfusion. Joint pain was the commonest clinical presentation among all sickle cell disease patients (n=39, 65.0%). Dactylitis was significantly more common in homozygous sickle patients compared with the Sickle β-thalassaemia group ( p 0.034). Two genetic backgrounds sickle mutation were identified namely, Arab Indian and Benin. Among the regulators of Foetal hemoglobin in Sickle patients of the present study rs1427407 G>T seemed to be the most prominent modifier, with a significant association with Foetal haemoglobin levels ( p 0.04).Conclusions Overall, the clinical course of the Asian version of Sickle cell disease in Sri Lanka appears to be milder than that described in India.

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“…The same survey identi ed signi cant inconsistencies in care of SCD patients between centres. (11). Genetic information including haplotype analysis for SCD in Sri Lanka is scarce, and has been reported for a single patient with SBT (12) only.…”

Section: Introductionmentioning

confidence: 99%

Sickle cell disease in Sri Lanka: Clinical and molecular basis and the unanswered questions about disease severeity.

Darshana

Bandara²,

Nawarathne³

et al. 2020

Preprint

Self Cite

View full text Add to dashboard Cite

Background Though case reports and limited case series of Sickle cell disease in Sri Lanka have been reported previously, no attempt has been made hitherto to undertake a comprehensive genotypic-phenotypic analysis of this “rare” group of patients. Results All accessible Sickle cell disease patients, totaling 60, including, 51 Sickle β-thalassaemia and 9 homozygous sickle patients were enrolled from seven thalassaemia treatment centres between December 2016 - March 2019. The majority of patients were of Sinhalese ethnicity (n=52, 86.67%). Geographically, two prominent clusters were identified and the distribution of Sickle haemoglobin in the island contrasted markedly with the other haemoglobinopathies. 3/ 9 homozygous sickle patients and 3/ 51 Sickle β-thalassaemia patients were receiving regular transfusion. Joint pain was the commonest clinical presentation among all sickle cell disease patients (n=39, 65.0%). Dactylitis was significantly more common in homozygous sickle patients compared with the Sickle β-thalassaemia groups (p 0.027). Two genetic backgrounds sickle mutation were identified namely, Arab Indian and Benin. Among the regulators of Foetal hemoglobin in Sickle patients of the present study rs1427407 G>T seemed to be the most prominent modifier, with a significant association with Foetal haemoglobin levels (p 0.04). Conclusions Overall, the clinical course of the Asian version of Sickle cell disease in Sri Lanka appears to be milder than that described in India.

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

Cited by 28 publications

References 17 publications

Psychological morbidity among children with transfusion dependent β-thalassaemia and their parents in Sri Lanka

Psychological morbidity among children with transfusion dependent β-thalassaemia and their parents in Sri Lanka

Sickle cell disease in Sri Lanka: Clinical and molecular basis and the unanswered questions about disease severeity.

Sickle cell disease in Sri Lanka: Clinical and molecular basis and the unanswered questions about disease severeity.

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