1. ¹Department of Haematology and Bone Marrow Transplantation, St George's Hospital, London, UK
2. ²Department of Haematology, City Hospital, Birmingham, UK

Correspondence: Dr S Chakrabarti, Department of Haematology and Bone Marrow Transplantation, St George's Hospital, London SW17 0QT, UK. E-mail: suparno@doctors.org.uk

Received 10 October 2006; Revised 8 January 2007; Accepted 22 January 2007; Published online 5 March 2007.

Abstract

The development of reduced-intensity conditioning (RIC) and the success of BMT for paediatric sickle cell disease (SCD) have raised the possibility of revisiting this prospect in adults as well. In a chronic debilitating disorder managed with supportive therapy, the patients' perception is critical in the advancement of any potential curative therapy. To explore this aspect, we undertook a questionnaire-based survey on 30 adults with SCD. Sixty two per cent of the patients were ready to accept a transplant-related mortality (TRM) >10%; 30% of them a TRM >30%. A risk of graft failure (GF) >10% was acceptable to 64%, with a risk >30% acceptable to 41%. Infertility was acceptable to only 50%. Chronic graft-versus-host disease (GVHD) was unacceptable to the majority (80%). Seventy six per cent% of patients had a full sibling and 60% were willing to participate in a clinical trial of RIC transplantation. This survey suggests that the majority of adults with SCD might be willing to consider a curative option such as RIC transplantation even with a high TRM or GF. The major concerns relate to chronic GVHD and infertility. There is an urgent need to explore RIC transplants in SCD patients within the framework of a clinical trial, considering patient perception regarding cure and complications.