desferal for a baby of 5 months?

Hi,

A baby with pyruvate kinase deficiency has a ferritin of 1007. I have no other iinformation than this at the moment. I asked a lot of things, so I hope my questions will get an answer soon.

Is it possible that a baby of only 5 months has a ferritin of 1000? Can you give those small children desferal? I never heard of this, and I coudln't find any info. Maybe I am just a bad searcher.

Thank you in advance, Dore

Dear Dore,

Has the baby been transfused?  At five months the baby probably has not had more than a couple of transfusions.  The ferritin level may not be a reflection of the baby's iron levels - rather it may be a response to infection, cold or flu.   I don't think that the baby would need desferal at this time - commonly desferal is introduced closer to 18months - 2yrs of age.   The baby's ferritin should be measured again at a later time, my guess is that it should be much lower at that time, unless if there is another underlying condition causing the iron levels to be higher (such as hyperchromotosis). 

Ferritin is a value measured over time to get a general idea of iron levels, so no action should be taken based on a single ferritin vallue. 

Best of luck for the little one,

Sharmin

Sharmin,

That was one of the question for the mother of the child, because it seems so strange for me.

HI

As I know, he/she must be begin with desferal after 15 blood transportation process, as Doctor say and as wrote in desferal sheet, of course after making Ferritin analysis.

I agree that the high ferritin is probably not caused by iron overload, but more likely is the result of infection. In cases of pyruvate kinase deficiency, serum ferritin is often elevated even in non-transfused patients, but at 5 months this would be very unlikely, even if the child also carries hemochromatosis. More information about the child's case would be helpful.

New fact:

Adam had have 7 blood transfusions so far.

His doctor was Beutler, but sadly he passed away in October. Maybe you don't  recognize this name, but he was the king of my disease. I am thankful for what he did for us.

There's planned a consult by dr. Cohen from philadelphia.

It's stilll not enough to say something about it. I hope the doc can figure out what's going on.

I went through pubmed this weekend and I found some other cases like that. I found one article about sickle cell and pkd. And there was on a thai family and all members had thal, except one daugther.

And the first bmt with pk def. was done in 2003 in Bangkok. The little boy was 5 year old at that time and he was doing fine in the beginning of this year.  My friend N. is going to be the second one - at the age of 36 (?).

A BMT at the age of 36, usually those kinds of operations are done at a very young age. Dore please update us with this case.

manal