K A Anie1, P Massaglia. 1. Brent Sickle Cell and Thalassaemia Centre, Central Middlesex Hospital, Acton Lane, London, UK, NW10 7NS. kofi@sickle-psychology.com
Abstract
BACKGROUND: Thalassaemia is a group of genetic blood disorders characterised by the absence or reduction in the production of haemoglobin. Severity is variable from less severe anaemia, through thalassaemia intermedia, to profound severe anaemia (thalassaemia major). In thalassaemia major other complications include growth retardation, bone deformation, and enlarged spleen. Blood transfusion is required to treat severe forms of thalassaemia, but this results in excessive accumulation of iron in the body (iron overload), removed mostly by a drug called desferrioxamine through 'chelation therapy'. Non-routine treatments are bone marrow transplantation (which is age restricted), and possibly hydroxyurea, designed to raise foetal haemoglobin level, thus reducing anaemia. In addition, psychological therapies seem appropriate to improving outcome and adherence to medical treatment. OBJECTIVES: To examine the evidence that in patients with thalassaemia, psychological treatments improve the ability to cope with the condition, and improve both medical and psychosocial outcome. SEARCH STRATEGY: The Cochrane Cystic Fibrosis and Genetic Disorders Group specialist trials register which comprises references from comprehensive electronic database searches, handsearching relevant journals and handsearching abstract books of conference proceedings. Also, searches on the Internet were performed. SELECTION CRITERIA: All randomised or quasi-randomised controlled trials comparing the use of psychological intervention to no (psychological) intervention in patients with thalassaemia. DATA COLLECTION AND ANALYSIS: No trials of psychological therapies were found in the literature for inclusion at the present time. MAIN RESULTS: There are no results to be reported at present. REVIEWER'S CONCLUSIONS: As a chronic disease with a considerable role for self-management, psychological support seems appropriate for managing thalassaemia. However, no conclusions can be made about the use of specific psychological therapies in thalassaemia from the information currently available. This systematic review has clearly identified the need for well designed, adequately-powered, multicentre, randomised controlled trials assessing the effectiveness of specific psychological interventions for thalassaemia.
BACKGROUND: Thalassaemia is a group of genetic blood disorders characterised by the absence or reduction in the production of haemoglobin. Severity is variable from less severe anaemia, through thalassaemia intermedia, to profound severe anaemia (thalassaemia major). In thalassaemia major other complications include growth retardation, bone deformation, and enlarged spleen. Blood transfusion is required to treat severe forms of thalassaemia, but this results in excessive accumulation of iron in the body (iron overload), removed mostly by a drug called desferrioxamine through 'chelation therapy'. Non-routine treatments are bone marrow transplantation (which is age restricted), and possibly hydroxyurea, designed to raise foetal haemoglobin level, thus reducing anaemia. In addition, psychological therapies seem appropriate to improving outcome and adherence to medical treatment. OBJECTIVES: To examine the evidence that in patients with thalassaemia, psychological treatments improve the ability to cope with the condition, and improve both medical and psychosocial outcome. SEARCH STRATEGY: The Cochrane Cystic Fibrosis and Genetic Disorders Group specialist trials register which comprises references from comprehensive electronic database searches, handsearching relevant journals and handsearching abstract books of conference proceedings. Also, searches on the Internet were performed. SELECTION CRITERIA: All randomised or quasi-randomised controlled trials comparing the use of psychological intervention to no (psychological) intervention in patients with thalassaemia. DATA COLLECTION AND ANALYSIS: No trials of psychological therapies were found in the literature for inclusion at the present time. MAIN RESULTS: There are no results to be reported at present. REVIEWER'S CONCLUSIONS: As a chronic disease with a considerable role for self-management, psychological support seems appropriate for managing thalassaemia. However, no conclusions can be made about the use of specific psychological therapies in thalassaemia from the information currently available. This systematic review has clearly identified the need for well designed, adequately-powered, multicentre, randomised controlled trials assessing the effectiveness of specific psychological interventions for thalassaemia.
Authors: Dimitrios Farmakis; John Porter; Ali Taher; Maria Domenica Cappellini; Michael Angastiniotis; Androulla Eleftheriou Journal: Hemasphere Date: 2022-07-29