Abstract
BackgroundBeta thalassemia major is a severe inherited form of hemolytic anemia that results from ineffective erythropoiesis. Allogenic hematopoietic stem cell transplantation (HSCT) remains the only potentially curative therapy. Unfortunately, the subgroup of adult thalassemia patients with hepatomegaly, portal fibrosis and a history of irregular iron chelation have an elevated risk for transplantation-related mortality that is currently estimated to be about 29 percent.DiscussionThalassemia patients may be faced with a difficult choice: they can either continue conventional transfusion and iron chelation therapy or accept the high mortality risk of HSCT in the hope of obtaining complete recovery.Throughout the decision making process, every effort should be made to sustain and enhance autonomous choice. The concept of conscious consent becomes particularly important. The patient must be made fully aware of the favourable and adverse outcomes of HSCT. Although it is the physician's duty to illustrate the possibility of completely restoring health, considerable emphasis should be put on the adverse effects of the procedure. The physician also needs to decide whether the patient is eligible for HSCT according to the "rule of descending order". The patient must be given full details on self-care and fundamental lifestyle changes and be fully aware that he/she will be partly responsible for the outcome.SummaryOnly if all the aforesaid conditions are satisfied can it be considered reasonable to propose unrelated HSCT as a potential cure for high risk thalassemia patients.
Highlights
Beta thalassemia major is a severe inherited form of hemolytic anemia that results from ineffective erythropoiesis
The survival of patients with thalassemia major is continuously improving, but despite the advances made in iron chelation therapy [2,3], the prevalence of severe complications such as heart failure, arrhythmias, and diabetes remains high
The following steps are essential to conscious consent: 1 - patients must be made aware of their overall medical condition; 2 - physicians must communicate the reasons for which the treatment is recommended; 3 - the benefits of hematopoietic stem cell transplantation (HSCT) must be clearly explained, but at the same time, therapy-related risks need to be vigorously stressed
Summary
The clinical hematologist is faced with a multitude of risk factors (disease severity, early onset, the difficult management and treatment of complications of beta- thalassemia major) as well as the demanding task of identifying patients likely to incur a bad outcome [8]. When the negative consequences of a proposed treatment strategy are discussed with the patient there is generally a rise in the discrepancy between the information conveyed by the physician and the information perceived by the patient [28] This makes it important to emphasize the adverse and dramatic effects of HSCT, the development of severe acute and/or chronic GvHD and the possibility of death. The following steps are essential to conscious consent: 1 - patients must be made aware of their overall medical condition; 2 - physicians must communicate the reasons for which the treatment is recommended; 3 - the benefits of HSCT must be clearly explained, but at the same time, therapy-related risks need to be vigorously stressed. Competing interests The authors declare that they have no competing interests
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
