Thalassaemia is a group of genetic blood disorders. Blood transfusion is needed to treat severe forms of the condition, but this results in a high level of iron in the body. This excess iron is usually removed by drugs through 'chelation therapy'. Other less usual treatments are bone marrow transplantation, which is restricted by age, and hydroxyurea, a drug designed to raise the level of foetal haemoglobin and reduce anaemia. Psychological support would seem suitable to manage thalassaemia which is a chronic disease with a considerable role for self-management. We were not able to find any trials to include in this review. There are some reports that teaching people about their condition improves adherence to chelation therapy. However, from the information currently available, we can not recommend the use of specific psychological therapies in thalassaemia. We believe there is a need for large trials to assess the value of psychological therapies for thalassaemia.
Vollständige Zusammenfassung lesen
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.
Zielsetzungen
To examine the evidence that in people with thalassaemia, psychological treatments improve the ability to cope with the condition, and improve both medical and psychosocial outcomes.
Suchstrategie
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register which comprises of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Searches on the Internet were also performed.
Date of the most recent search of the Group's Haemoglobinopathies Trials Register: 11 November 2013.
Auswahlkriterien
All randomised or quasi-randomised controlled trials comparing the use of psychological intervention to no (psychological) intervention in people with thalassaemia.
Datensammlung und ‐analyse
No trials of psychological therapies have been found in the literature for inclusion in this review.
Hauptergebnisse
There are currently no results to be reported.
Schlussfolgerungen der Autoren
As a chronic disease with a considerable role for self-management, psychological support seems appropriate for managing thalassaemia. However, from the information currently available, no conclusions can be made about the use of specific psychological therapies in thalassaemia. 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.
