OUTCOMES

There have been deaths reported to the DBAR.

 

Some of the deaths are treatment-related, mostly due to:

  • Infections

  • Complications from vascular access device

  • Iron overload – This is due to the amount of iron a patient receives everytime he or she has a blood transfusion. The amount accumulates and can cause death from iron overload in the heart or liver.

  • SCT complications - It is important to note that the majority of patients who died from complications of SCT had been transfusion-dependent prior to SCT. In most cases the reason they were having a transplant was because of the transfusion dependence. Thus many of these patients were iron-overloaded. SCT survival may be improved if patients can have good iron balance prior to SCT.

  • mortality

 

Other deaths have been due to the DBA itself:

 

  • Malignancy (Cancer)

  • Hydrops fetalis – This occurs when a baby is born severely anemic.

  • Aplastic anemia – This has occurred in some DBA patients. It presents with other cell lines being low, such that the patient also develops neutropenia (low neutrophil count) and/or thrombocytopenia (low platelet count).

 

For some patients the cause of death is unknown or has not been reported to the DBAR.