Previous to Desferal, individuals with thalassemia could only expect to live into their late teens and early 20's, eventually succumbing to iron overload. Since the iron-removing Desferal was only introduced in the mid-70's, we are still watching the life span of adults with thalassemia. Our oldest patient is in her early 40's. The life expectancy of children who have used Desferal since a very young age remains to be seen, though researchers and physicians expect to see long, productive lives.

In affluent countries, a child who is born with thalassemia major (either beta thalassemia or alpha thalassemia) will face a lifetime of monthly blood transfusions and nightly infusions of desferoxamine. Over the past several decades with the progress of medical technology,this once universally fatal disease has been converted to a chronic illness. Now a fortunate few have the opportunity of cure through bone marrow transplantation.

Generally speaking, the younger the recipient, the more optimistic the outcome. The Lucarelli Staging System is based on the amount of liver damage sustained as a consequence of iron overload from chronic transfusion therapy and the adequacy of Desferal chelation therapy. The staging includes evaluations of liver size, the schedule and compliance of desferrioxamine therapy, and liver biopsy (in children over 3 years) to determine whether or not there has been microscopic liver damage. Only a precious few children who have this complex disease will be cured by a bone marrow transplant. The rest of these children need continued support and acceptance that they may live normal healthy lives in spite of their illness.

 

Alpha Thalassemia - Beta Thalassemia - Variant Hemoglobin