Discussion Forums > Thalassemia Major
Little Ari
Zaini:
Charishma,
Little Ari is in our prayers :hugfriend .
Zaini.
Sharmin:
Dear Carisma,
I understand your husband's concern about chelation, but the good news is that your son will most likely be started on exjade rather than desferal. At the conference the doctors agreed that for young children exjade is the best option for many reasons, such as the effects on bones and growth that desferal can have and the quality of life (it is best not to poke very young children if you don't have to).
In thalassemia, it is very important to remember the balance between hemoglobin and chelation. For optimal growth it is best for a child's hg to be kept above 10 and for the iron levels to remain as low as possible. With exjade, these days it is possible to keep iron levels near normal.
I hope that the doctor decides to transfuse little Ari soon. Please keep us posted and give our love to the little guy :hugfriend :hugfriend
Ari is always in our prayers,
Sharmin
Andy Battaglia:
Hi Carisma,
The Physiological Nadir is a phenomenon that happens in all newborns and occurs in the 8-12 week period. This is a low point in hemoglobin levels as the transition from fetal to adult hemoglobin takes place. In normal babies, this will be a temporary drop, as the Hb is lower but the percentage of HbA is increasing. With beta zero, the percentage of hemoglobin that is HbA will remain zero, so the only hemoglobin will be the fetal hemoglobin. Again, I see no reason to wait, as there will not be any HbA and the child will develop at a more normal rate if the Hb can be increased. I don't think transfusion would be put off at any of the Comprehensive Centers, once the Hb has dropped this low. A big advantage of having the treatment program designed at a Comprehensive Center, is that they will always be at the forefront of advances in understanding of how best to treat thalassemia, and won't be relying on outdated practices and information. The current thinking is to never allow a child's Hb to drop, as it slows growth and development, and with modern chelation, putting off transfusion because it delays when chelation is needed, has become somewhat outdated. We have heard about current trials using Exjade at meal time and also using it twice per day in half doses, and I think this will quickly lead to new recommendations that will make the oral chelator even easier to administer. Avoiding chelation as long as possible is no longer part of the strategy in thalassemia treatment.
And as I should advise all parents in the US, make sure you are registered with the Cooley's Anemia Foundation.
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
Cari:
Ari is at 6 but they do not want to transfuse. They want me to come back on the 30th!
Andy Battaglia:
Hi Carisma,
For now, watch Ari and look for any signs that he's becoming listless or lethargic, has poor appetite or begins to look noticeably paler, with pale lips being a sign that blood is needed. Meanwhile make sure you have read about the transfusion process, so you know what to expect. All medical procedures are a bit less scary when you understand what will be done. Also, at this age, the Hb is lower than normal so 6 is not as low as it would be once the nadir period has passed, so the actual physical condition is a big factor in deciding when to begin transfusions. Your own observations about his condition can be very important. Try to be as objective as possible and if you feel there has been a significant change in his behavior, call the doctor.
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