Hi Sharmin,
Welcome to our group. I think you'll enjoy the discussions.
The autoantibody problem definitely needs corrective actions and steroids are the normal treatment. The major concern is autoimmune hemolytic anemia (AIHA) and that is the reason why the transfusion frequency increases. The red blood cells (RBC) that are affected by the autoantibodies are rendered useless and then destroyed by the spleen. Contributing factors to this include problems getting close matches for transfusion and use of blood not filtered for leukocytes, but the causes are not fully understood. This shows a great need for better matching which often just isn't possible with the available blood supply. This is seen as a growing problem in the US with Asians comprising a large part of the affected population. Because most of the donors are white and so many of the recipients are Asians, it has increased the matching difficulty, leading to more cases of autoimmune problems.
Some methods that are being used to reduce this are discussed at
http://www.bloodjournal.org/cgi/content/full/96/10/3369For prevention of alloimmunization, our results demonstrate that transfusion of blood matched for Rh and K antigens resulted in a significant difference in the alloimmunization rate...However, the parallel change to all leukodepleted blood could have also contributed to the decrease in alloimmunization. Although RBC matching was shown to be effective in preventing alloimmunization in the present study and in other studies,13,15,16 the expense and the feasibility of antigen-matched blood may not permit such a transfusion approach in some medical centers. Providing matched units for the ABO-D and Kell systems only, particularly in the Asian patients in whom anti-K antibodies were the most frequently detected (30%), is another, possibly more economic, approach. Transfusing matched blood only for patients who first proved to be "antibody producers" may reduce expenses and increase the availability of matched blood; however, patients may still develop clinically significant alloantibodies.
I have talked to many Maldivian thals who experience excess hemolytic anemia and have to transfuse weekly and still can only maintain a hemoglobin of 8. A main cause there seems to be a lack of leukocyte filters which also results in fevers being the norm after a transfusion.
Quite frankly, the deck is stacked against thalassemics and this is why even with better treatments we still have far too many thals dying young. This discussion touches on one reason. Frequent transfusions means a need for disciplined chelation and with desferal, that isn't always easy. The main cause of thal deaths is still related to iron overload and hopefully with the advent of oral chelators, this can be greatly reduced. However, we see so many other cases where other factors came into play, such as the infection from dental work. Some of these can be prevented with better education and hopefully we can help do some of that ourselves. One thing patients need to do is educate their health care providers about thal because so many have only a basic knowledge at best. Don't be shy about asking a doctor to look at something you found online. There are many great online resources for explaining and understanding the multitude of thal related problems, from the very basic to the highly technical articles. Most of what I know about thal has come from online research. The information is out there and doctors may have to be led to it so they can better understand what is going on and how to treat it. We have to do as much as we can to spread the word about thal. It is a far more serious problem than most people are aware of and the problem has the potential to get much worse with current migration patterns that have many carriers moving to western countries where it isn't common and most doctors never have had to deal with it in the past.
What is being done to reduce thal deaths? Centers like the thal center at Oakland Children's Hospital have developed comprehensive care programs for thals that involve all the health care providers working together with each other and the patients and families. This leads to better coordinated care, based on the specific patient's needs. This concept needs to spread throughout the world to all centers offering thal care. Once again, referring a doctor to these concepts may hopefully result in a doctor at least taking a look at how thal care is handled elsewhere.
I've lost some friends and friends of friends who were thals this year and it does break my heart too. Better treatment is needed worldwide and beyond that, thals everywhere have to be presented a life worth living if we expect them to comply with treatment.