Discussion Forums > Thalassemia Major
In Need Of Your Advise Andy
Emby:
Dear Andy,
Hope you are keeping young, well, fit and bouncy ;D !!
Andy i need to bug you about something. My young son had his T2 scan which revealed heart iron 17.8 (mild iron) liver 4.8 (mild iron). He was taking 3 1/2 vials of desferal (500 mg) 5 nights a week. His Doc has told him to discontinue with desferal and to take ferriprox 3 tabs 3x daily. He weighs 59.2kg. Is this okay? or do you think he could do better on combination of the two? His ferritin is 517.
Also the Doc has told us that from now the boys will be having transfusion worked out by desired hb of 15 (Hb 15 - actual Hb x body weight x 3). Our older sons Hb has been below 8.9 pre transfusion so i feel it might be okay for him. But the little ones Hb has only dropped the last 2 times pre transfusion. Do you think this is a good formula for BOTH of them ? Do you think that it be okay to go with this formula short term because I'm worried that too much blood annually is not good regarding iron levels although theirs is low?
Also is it safe to be transfused regularly with 5 units of blood averaging 250mls?
One last thing...our younger one has a slightly enlarged spleen...what does this mean? Would it be a bad thing to have increased blood transfusion? Can the size of the spleen be reduced? How could the size of the spleen be maintained without it getting bigger? He has been well but I'm worried about it.
Please, please, please help. I know that I have asked too many questions but i would really, really appreciate your help and valuable advise.
Take care and Big Hug
Emby
Narendra:
Hello Emby,
Andy might not be able to answer right away as he is in NY for a conference on Thalassemia. Such a good thing for all.
--- Quote ---My young son had his T2 scan which revealed heart iron 17.8 (mild iron) liver 4.8 (mild iron). He was taking 3 1/2 vials of desferal (500 mg) 5 nights a week. His Doc has told him to discontinue with desferal and to take ferriprox 3 tabs 3x daily. He weighs 59.2kg. Is this okay? or do you think he could do better on combination of the two? His ferritin is 517
--- End quote ---
I think as Ferriprox is well known to remove Iron from the heart, it is a good option to start with it. For the dose, I think it is best to start with a lower dose for the body to get adjusted to the new medicine. The optimal dose is 75 mg/kg, so depending on the body weight the child should take the medicine, but I have heard to start with a lower dose and gradually increase it. As his ferritin is not high, i think it would be ok to discontinue desferal for now. That also gives him a break from dialy needles.
Emby:
Hello Narendra,
Andy has gone to drink from the 'Fountain Of Thalasseamic Knowledge' ? He is the best. Hope he brings back some fantastic news :biggrin!
Thank you for getting back so soon. Our younger son has been put on the lower dose and I'm praying he stays well with it. He will have to go for weekly blood checks which might be worse than having desferal all weekdays because his veins just never want to bleed back :(.
Anticipating Andys return.
Emby
Andy Battaglia:
Hi Emby,
It really is a marvelous conference and an incredible group of doctors and researchers who have been gathered here by the Cooley's Anemia Foundation (a big pat on the back to everyone from CAF).
Narendra has steered you right. Ferriprox is far more effective at removing heart iron so this is the correct move. You sons should have a T2* over 20 and with work, over 30, so there is some work to be done. I agree about starting on Ferriprox because gastrointestinal complaints are the most common side effect, and starting with a low dose and working up to the required dose can help to minimize this. If possible, see if the oral Ferriprox solution is available in the UK and get that because the incidence of gastro problems has been greatly reduced in the liquid version of Ferriprox. If I run into Gerry from Apo Pharma again tomorrow, I will find out if the UK is one of the places the liquid is on the market.
I want to include a comment from one of the top doctors in the world in regards to Ferriprox. At the end of his presentation which showed once again that Ferriprox is the superior chelator for removing heart iron, the doctor said that he just cannot understand why this drug is not being made available to patients in every country. We're getting closer to approval in the US but once again, the FDA is taking their sweet time with the process.
I'm not sure I understand the question about blood. Are you saying 5 bags of 250 ml at once? I do believe that a higher Hb may be of some help with the enlarged spleen, but don't worry too much because slightly enlarged is not a big concern.
Lena:
What about T2 iron liver? Isn't this (4.8) too low? In the liver iron must be above 19 (T2*) and in heart above 22 (T2*). So Ferriprox is o.k but together with desferal or exjade that remove iron from the liver, because ferriprox has little effect on liver.(If I understood correctly 4.8 is T2* for liver iron.)
Lena.
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