Yellowboy,
I suspect that you have HbH disease, a 3 gene deletion. I say this because your Hb is very low and this is not normally found in 2 gene deletion alpha thal. Also, your Hb is low even for HbH, so it might even be HbH constant spring, in which transfusions often are necessary. There is one other exception that could cause similar problems to what you describe and that is when one of the alpha genes is triplicated (the triplicated alpha gene is known to exist in Asian populations). When one triplicated gene is combined with one more alpha deletion, a thal intermedia-like condition can occur, even though only 2 of the 4 genes are affected. It would be helpful to know exactly what your true classification is, and this can only be done through a DNA analysis that would look for both deletions and mutations on the alpha genes. Have you ever had a DNA test done?
How much folic acid do you take daily? Do you also take any other supplements? Have you ever had a liver MRI? In thal intermedia, iron load often cannot be accurately be determined by ferritin test. A scan is necessary to determine if chelation should be continued. An Hb of 7 is considered to be the line where transfusions should begin. Your spleen is overactive and obviously affecting your Hb level. I believe to accurately determine why, you will need the DNA test, as the possibility of a triplicated gene must be investigated. When that gene is present and one other alpha deletion is present, an unstable hemoglobin can be produced which breaks down very quickly. This would explain why your spleen is so large, as it filters the "bad" blood out. It would be extremely unusually for a 2 gene deleted alpha thal to have your symptoms. I feel you would benefit greatly by being seen at one of the US Centers of Excellence. You need to be seen by doctors familiar with alpha thalassemia so you can get a proper diagnosis and proper treatment, if any is needed. You need to find out if you can improve the quality of your life. This can only begin with getting a diagnosis that explains your symptoms. Even if you are treated locally, being seen at one of the Centers is highly recommended. For transfusing patients, annual visits are the norm.
You can contact Eileen at CAF to find which center is closest to you.
Cooley's Anemia Foundation
330 Seventh Avenue, #900
New York, NY 10001
(800)522-7222
Fax: 212-279-5999
Patient Services Manager
Eileen Scott
Let us know about the folic acid and supplements. There may be some things you can do to help yourself, but I will be honest. There are not many prescribed treatments for alpha thal. But I do feel that you can do a few things to improve things. One thing you should be aware of is that certain flat beans, like fava beans should be avoided because they can cause a sudden hemolytic crisis, which will cause a drop in HB. Certain chemicals and fumes, like moth balls can also cause this. There is a partial list at
http://www.thalassemiapatientsandfriends.com/index.php/topic,3410.msg34830.html#msg34830 I have also asked Bobby to post about his management of his enlarged spleen.