Firstly, I feel ashamed at my lack of initiative in getting in touch with the local thal society-Johor State Thalassemia Society. In fact, they have not sent any representative for the conference. Radlan and I were the only ones from Johor and we were self-sponsored. The Malaysian parents & patients had gone there in throngs, mainly from Kuala Lumpur, Penang, Perak, Sarawak & Sabah. Hey, friends from Malaysia, it was indeed a pleasure meeting and getting to know all of you there! There's much passion from Malaysians even on the international level. I hope we all keep that spirit going, we can do much more when we are united with our cause. Right now, I should get myself registered as a member of the Johor State Thalassemia Society. How? Well, someone please send me a link?
Secondly and most importantly, we are working towards the best care and management posssible for Adel Rais until he gets his bone marrow transplant. I've discussed this with Radlan and we are going to enlist the world's best haemotologist to work with team Singapore. Singapore may have one of the best hospital in terms of facilities, but we have to be realistic that the numbers of thalassemic birth is close to zero, hence their experience for thalassemia is lacking. We have indentified whom we wish to care for Adel. I'm going to figure out how, it may turn out that, we may have to go overseas every now and then.
In order to get him the best care and management, it is probably apt for us to start from the "drawing board". It's been my nagging suspicion that the rare mutation that Adel carry from Radlan which is from Mediteranean decent, may manifest in a way which is different, perhaps more severe than the South Asian mutation which I carry or others from this region. The question we had, which went unanswered, like why was his iron absorption so quick causing hepatosplenomegaly at such a young age? It got my attention when we heard that Thal Intermedia, which once thought as a milder form of Thalassemia can manifest in a huge spleen very quickly if it's not treated with consistent transfusion and chelation. We are going to start by sending our blood and Adel's to a research lab in Bangkok and get them to look into the genotype and phenotype of his mutations. Our current suspicion, is Radlan's mutation could be of Thal-Intermedia type.
Next thing to do is, to get MRI Ferriscan or a T2* done on his liver/heart. By doing so, we can properly assess the amount of iron in his liver/heart. I have often suspect the serum ferritin test on blood is an inaccurate assessment for iron levels in Adel. This is another gained knowledge from the conference attended because, Thal Intermedias have low serrum ferritin, however, they can get so sickly beyond comprehension totally inconsistent with the measure of iron by serrum ferritin levels. I will have to discuss this with the doctors on our options, and meanwhile "educate" lil boy about Ferriscan/T2* so we can avoid g.a for this. He's a good boy, I hope he will trust us and allow us to do it without a g.a. Doing an MRI on 6 monthly basis, will let us know if our current program is working for him or if needed tailor a chelation program that is optimum for Adel.
Lastly, we are hopeful that the preparative regime to bone marrow transplant is getting less toxic and better by the years. Once this little boy is stronger, we will work towards a bone marrow transplant.
It was indeed God sent, that we have met the most amazing doctors in this conference, made contacts with people in the manner which I had least expected. All seem able to contribute when they have heard my story. I kind of believe that there are "secret hands at work" in the universe. I will not say more until there are more concrete plans ahead. For now, let the journey begin towards a cure for Adel.
