Emby,
Would it be possible to take the boys to Whittington for an annual visit. I'm not sure if it works the same in the UK, but in the US many patients travel to one of the Centers of Excellence once a year for an evaluation, which can then be followed by any adjustments that need to be made to the treatment program. It is quite valuable to the patients and the patients who are seen by these centers tend to have fewer problems, as their care is so well managed. One main benefit of these centers is you get to deal with doctors and nurses who work as a team to design treatment programs for each patient and these professionals are thal specialists with great experience working with thals. If going to London once per year is feasible, you should contact Emma Prescott at Whittington and ask her if this would be possible to set up. I think this would be a very positive step, especially as they move into adulthood.
Lena,
I really have exposed myself to a vast range of information about all facets of thalassemia from the medical to the psychological and emotional, and feel that in some ways I have a broader view of thalassemia than most, but then I meet someone like Dr Vichinsky, who has such an incredible grasp of thalassemia in every possible way, that I feel like I hardly know anything, so your words, coming from a very experienced and knowledgeable person such as yourself, mean a lot to me. As far as extramedullary hematopoiesis, I feel this is one of the most under-diagnosed conditions in thalassemia. I believe that many thals have back pain that is attributed to arthritis, when in fact it is these growths pressing on the spine that are causing the debilitating pain. And they may remain under-diagnosed because it takes a trained tech who knows what to look for in a scan to see them, and they have to be looking for them in the first place, which just usually is not done. However, I think if logic was followed, they would be suspected far more often. Basically, when your body does not have enough oxygen, which is caused by an insufficient hemoglobin level, the body will try to compensate by growing red blood cells outside the bone marrow, often within the organs, the body cavity and even within individual cells throughout the body. In those with thalassemia, this only leads to production of the same bad red blood cells that do not carry much if any hemoglobin, rendering the body's effort to boost the hemoglobin level, futile. There has been a general assumption that these are not very common in majors but I would dispute this, as I personally know of many majors, many of whom are well transfused, who do have these extramedullary growths. This tells me that the hemoglobin does not have to be severely below normal for these growths to begin. Majors tend to maintain their hemoglobin in the same range that is seen in the more symptomatic thal minors, yet many of these majors do get these growths. So, is it far fetched to think that low Hb minors may also get them? Courtenay's is large enough to affect her breathing. I really think that if logic was followed, there would have to be an admission that minors can suffer many of the same things found in majors, but most likely, to a lesser degree. They have one bad gene that causes defective red blood cells to be manufactured. My question to those who claim minors have no symptoms is "why wouldn't they, since half of their hemoglobin production is affected by the thal gene?"