Thank you for the positive feedback for the post. Hopefully we will continue to learn from each other's experiences. Thalpal continues to be the most important resource in my son's care.
In 2006, Lil A developed an autoantibody which would quickly break down transfused red blood cells. There was no way to screen the blood to prevent this hemolysis because the antibody indiscriminately destroyed all red blood cells in his body. He was at times requiring two transfusions a week. His doctor used prednisone, which is often the first line of treatment but soon that was not enough and iron levels began to build up. His doctor, going by old standards did not see a need for increased chelation because his 'ferritin' levels were below 2000.
Fortunately, at that time Andy recommended that we place Lil A on high doses of antioxidants and urged us to consult Dr. Vichinsky. Dr. Vichinsky immediately enrolled Lil A in a combination chelation trial (exjade and desferal). Within a year his iron levels decreased from 2800 to below 1000 - and eventually Lil A was maintained on exjade alone. His iron levels since that time remain at 400 or below. Two factors protected Lil A during that stressful time, firstly he had been well chelated since 18 months of age therefore the short duration of iron accumulation did not effect him. Secondly and most important, Dr. Vichinsky stressed that he was surprised at how little affect the iron had on him at all - this he said it was due to the high anti oxidant regimine that Lil A was on. Thank god for Andy advising us about antioxidants such as IP6.
Dr. Vichinsky also helped us deal with the antibodies. Lil A is now transfused every 3 weeks and his hemoglobin is kept at 100 (10.0). This is to optimize his growth and energy - to prevent strain on the heart, spleen and bones. I would recommend this for all patients, especially kids.
Another thing I want to stress is the need for constant chelation. Despite what some medical professionals are saying - chelation should never be stopped. It can be decreased to minute amounts spit into small doses throughout the day when iron levels are low but never stopped. Even when iron levels are very low (below 500) free iron can roam in the body and settle in other organs - and it can cause rustic damage.
In 2010 - little A's iron levels were very low so our local doctor took him off of exjade. It was reasonable to think that we would give his stomach a break because he often complained about stomach ache. A month later, an MRI showed that while there was little iron overload in the liver, no iron in the heart or pituitary there was mild accumulaton of iron in the pancreas and lungs. The average child does not have an mri of the entire body on a regular basis - we were lucky to find out immediately so we could rectify the situation. I stress that this was at a time when his iron levels were very low and we only held the chelation for just over a month. Since that time, we have NEVER stopped chelation.
When Lil A's iron levels are very low we decrease his exjade to minute levels (we always give him 1/2 the dose in the am and 1/2 pm) and if the levels seem to be increasing we increase the dose. During times of low iron load, the chelation serves not to remove iron overload - but to bind free iron which is corrosive no matter how much or little iron overload exists in the body. This must become knowledge for everyone. It is not just iron overload that kills - it is also the free iron which is present even when iron loads are low. Antioxidants and chelating agents bind free iron which protect organs from the free radical damage and settling of free iron into other organs. Dr. Vichinsky and the Oakland program endorse this method and we follow it completely. Lil A remains on antioxdants at all times and is chelated at all times. Dr. Vichinsky says that a transfusing thalassemia patient should not go for more than 24 hours with chelation. We ensure that Lil A gets a boost of chelation every 12 hours.
We are happy to let you know that recent endocrinology testing indicates that all of Lil A's hormones and endocrine system are completely normal - he is completely unaffected by the thalassemia. His pituitary volume, shape and function are completely normal. At one time this was unheard of in thals but today it is achievable for all thals. If this can happen for little A - even though he has had trouble with antibodies and at times has increased need for transfusion - other kids can definitely achieve this.
For these reasons I urge everyone to keep their focus on timely transfusion and constant, constant chelation. On top of this antioxidants are absolutely essential for the cardiovascular system, for iron damage prevention and overall well being. Antioxidants are not just a form of 'alternative health' they are part of the mainstream medical treatment of thalassemia.
Thalassemics tend to be deficient - and have a higher need for vitamins such as vitamin E, Vitamin C, vitamin D, zinc, magnesium - and would benefit greatly from supplements. This is stressed at all of the medical conferences regarding thalassemia and needs to be followed by patients. It is not extra protection, it is necessary for thals.
I hope that this is helpful, please talk to your own medical health care providers about what I say before following my advise. If your healthcare providers are not aware of these trends then perhaps you can contact one of the comprehensive care centers for advise. I recommend Oakland but there are many other centers as well. Regardless of what you do, individual patients need individual attention for their unique care and should be followed closely by their own doctors. It is possible for your local doctors to be in contact with larger centers to coordinate your care.
Best of luck to all,
Sharmin