David,
The masses that were irradiated, the extramedullary hematopoiesis, are a direct result of the low Hb level. When your bone marrow can't make enough hemoglobin to adequately serve your body's needs, attempts will be made to grow red blood cells outside the marrow, usually within the body cavity. This can result in pressure on the spine and organs and can cause serious permanent damage if left to grow. This can even occur at the cellular level where it looks very much like lymphoma in scans. It is not and any thal diagnosed with lymphoma needs to be aware that it may not actually be what it appeared to be in a scan. The drug Hydroxyurea is often used to help shrink these masses. Hydroxyurea is also a hemoglobin inducing drug, and should be considered as a possible therapy in thal intermedia. Have you ever tried it? Raising the Hb level is a key component of the treatment for extramedullary hematopoiesis, so I am upset to see that your Hb has been kept so low. This is an outdated style of treatment for intermedia and is now rejected by most experts, because iron chelation drugs are available to counter the iron load, and patients do much better when the Hb is kept above 9 at all times.
Where are you being treated? Are you open to the idea of being seen once per year at a Thal Center of Excellence in the US? I think this is the key to living a fairly normal life as a thal, especially as you age. A good treatment program can keep patients healthy by doing an annual assessment and fine tuning the treatment program. I would also suggest that you register with CAF. They are very good about finding the best centers in an area and also providing information about the various Centers of Excellence.
Cooley's Anemia Foundation
330 Seventh Avenue, #900
New York, NY 10001
(800)522-7222
Fax: 212-279-5999
Patient Services Manager
Eileen Scott
eileen.s@cooleysanemia.org
I am a great believer in the comprehensive approach to care that is found at this type of center around the world. I do not believe you are getting the comprehensive care that you need, especially being that you have the responsibility of a family.
I wanted to ask something about what you were told about the odds of having thal children. There is absolutely no difference in the odds from one pregnancy to the next. It is purely chance. What I do want to know is your wife a carrier? If not, there is no chance of you having an intermedia thal. Minor or non-carrier only if your wife is not also a carrier.