Just adopted 2 year old possible intermedia

Hi everyone,
I have recently returned from China with my 2 year old who was diagnosed in China as Thal major but is now suspected to be thal intermedia.  We are awaiting gene study, but have found some interesting lab results.  Her HbF is 26.2%.  Reportedly, the gene test in China showed B+/Bo (it has been repeated here, but still do not have the results).  The orphanage in China began transfusions, assuming she was major.  The lowest hemoglobin she had in China was 75 (7.5 here in the US).  We are now trying to figure out the best treatment plan for her.  I do have another daughter who is 5 with thal major, so am used to the transfusion routine.  However, I am not certain if that is the best route for my newest daughter if she is in fact thal intermedia. 
I am reading alot here about hydroxyurea as a treatment to raise HbF levels.  Has anyone used it for a child with similar HbF as Hannah with 26% and had success? 
How low should her hemoglobin go before transfusion?  Is 7's to low (she is currently at 9.1)?
Thanks so much,
Cindy
Mom to Mia (beta thal major)
Mom to Hannah (? thal intermedia)

I should also add her HbA is 71.3
                             HbA2 is 2.5
Thanks!
Cindy

Hi Cindy,

I would advise full genetic testing to determine the exact mutations and to also check for the XmnI polymorphism. If that is present, it makes the child a better candidate for hydroxyuea. Keep in mind that many doctors in the US will not prescribe hydroxyurea for thals. so you may find your doctor is not supportive of the idea. Her high HbF does suggest she is a good candidate for hydroxyurea, but unless she also carries the polymorphism, you amy not get much support for using the drug.

Thanks Andy!  If she does not have the polymorphism, would Hydroxyurea still be worth trying, or does it really not seem to help.
Cindy

There is very little support for using hydroxyurea among doctors in the US, so I would say no.