Conference Papers

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Hepatic and renal status of paediatric patients with thalassaemia
(Sri Lanka Medical Association, 2023) Wijenayake, W.; Pathiraja, H.; Thennakoon, R.; Fernando, M.; Bandara, D.; Mettananda, S.
INTRODUCTION: Thalassaemia is a chronic disorder affecting many organ systems. Although cirrhosis is a well-recognised complication, sub-clinical hepatic and renal dysfunction in thalassaemia are poorly studied. OBJECTIVES: We aimed to describe the hepatic and renal status and dysfunction of paediatric patients with thalassaemia. METHODS: A cross-sectional descriptive study was conducted at Kurunegala and Ragama Thalassaemia Centres during February and March 2023. All patients aged less than 16 years attending the thalassaemia centres were recruited. Data were collected using a data collection form by interviewing parents and perusal of clinical records and analysed using SPSS 27.0. Ethical approval was obtained from the Sri Lanka College of Paediatricians. RESULTS: Sixty-five patients (mean age-7.7; males-46%) were recruited. Of them 48(73%) had homozygous beta-thalassaemia and 17(26%) had HbE thalassaemia; 52(80%) were transfusion-dependent and 13(20%) were non-transfusion-dependent. Hepatomegaly and splenomegaly were found in 45(69%) and 30(46%), respectively. Regarding hepatic status, 34(52%) had high (>40IU/L) alanine transaminases, of which 8(12%) had >3-fold elevation of alanine transaminases. A higher proportion of children with HbE thalassaemia (71%) had elevated alanine transaminases compared to homozygous beta-thalassaemia (46%, χ2=3.0, p=0.07). Also, a higher proportion of children on deferasirox (57%) had elevated alanine transaminases compared to those who were not on the drug (27%, χ2=3.3, p=0.06). Four (5%) had <2+ proteinuria in urinalysis however, the urine protein: creatine ratio was normal in all. CONCLUSION: High alanine transaminases were noted in over 50% of paediatric patients with thalassaemia. HbE thalassaemia type and use of deferasirox were associated with high alanine transaminase levels.
20 year follow up and survival analysis in a cohort of patients with Haemoglobin E beta Thalassaemia.
(Sri Lanka Medical Association., 2019) Olivieri, N.F.; Premawardhena, A.P.; Amir-Arsalan, S.; Ediriweera, D.; Mettananda, S.; Bandara, W.D.; Arambepola, M.; de Silva, S.; Refai, M.A.C.M.; Allen, A.
INTRODUCTION & OBJECTIVES: Haemoglobin E beta thalassaemia (EBT) is the commonest beta thalassaemia syndrome in the world and is extremely phenotypically variable. Unlike for transfusion dependent thalassaemia (TDT) there are no clear guidelines for the management of this disease. We have followed up a cohort of 109 patients with EBT for 20 years. Objective of the study was to study the 20-year survival and factors that affect survival. METHODS: Study was conducted at Kurunegala Thalassaemia centre. Transfusions were stopped in 1997 in all 109 patients. Since then they were assessed every three months by the clinical team for the next 20 years. Relevant haematological, biochemical, radiological assessments were done periodically. RESULTS: 32 (30%) of patients were dead at 20 years. Kaplan Meir survival curve identified the median survival to be 51 years. Splenectomy had been done in 73/109 (67%) patients. Splenectomy allowed 66% to be off transfusions even 9.7± 1.3 years post- surgery. However, 33% had to return to transfusions. The commonest cause of death in the cohort was infections (34.3%). Most (72%) infective deaths happened in those who were splenectomised. Transfusions needed to be restarted in 60%, of whom 33% went back to (>8 per year) regular transfusions at a mean 8.4 ±0.8 years after stopping transfusions. CONCLUSION: In this first ever long term follow up study of EBT, significantly shortened survival is observed. Though splenectomy allows prolonged transfusion free phases in many it increases risk of infective deaths. Overall the disease is far less benign than previously thought with a high prevalence of morbidity and mortality.
Quality of life and psychological morbidity among children with transfusion dependent thalassaemia and their parents
(Sri Lanka Medical Association, 2018) Mettananda, S.; Pathiraja, H.; Peiris, R.; Bandara, D.; de Silva, T.U.N.; Mettananda, K.C.D.; Premawardhena, A.
INTRODUCTION AND OBJECTIVES: Thalassaemia is a chronic illness which requires life-long supportive care. We aim to describe quality of life and psychological morbidity among children with transfusion-dependent thalassaemia and their parents.METHODS: This case-control study was conducted from September to December 2017. All patients with transfusion-dependent thalassaemia aged 2-18 years attending three largest thalassemia centers of Sri Lanka (Kurunegala, Anuradhapura and Ragama) were recruited as cases Children without chronic diseases admitting to same hospitals for acute non-life threatening illnesses were recruited as controls. Data were collected using an interviewer-administered questionnaire with validated questionnaires to measure quality of life (PedsQL 4.0 Generic core scales) and psychological morbidity (Strengths and difficulties questionnaire) of children and depressive symptoms of parents (Centre for epidemiological studies depression scale). Ethical approval was obtained from Ethics Committee of University of Kelaniya. RESULTS: 321 (male-46.4%; mean age-9 9±4 2years) cases and 194 (male-47.4% mean age- 8.9±3 7years) controls were recruited Mean quality of life score was significantly lower in cases (72.6±12.0%) compared to controls (88.6±11.6%), (p<0 001) Prevalence of psychological issues were significantly higher among patients with thalassaemia compared to controls in emotional (17 6% vs 0%; 2=32.8, p<0 001), conduct (18.6% vs 0 6%;2=35.4, p<0 001), hyperactive (9 5% vs 0%; 2=18 9 p<0.001) and peer relationship (14.4% vs 2 5%; 2=15 7 p<0 001) domains Significantly higher proportion of mothers of cases reported depressive symptoms compared to controls (40 1% vs 8.9%; 2=57 0 p<0.001).CONCLUSION: Patients with transfusion-dependent thalassaemia experience a poor quality of life and are victims of psychological and behavioural problems compared to their peers. Similarly, mothers of children with thalassaemia have higher prevalence of depression Psychological support should be an essential component in management of thalassaemia

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