New From California

Hi, I'm a 27 year old mom and upon birth of our son Tyler, we were told that he has Thalassemia. This came as a suprise to us but after notifying our family members it turns out that my husband and I both had it in our family. My mother, grand mother and sister law all have Thalassemia minor. Surprisingly enough, they completely minimized the condition stating that it was completely common and normal. I am of Filipino ancestry while my husband is Portuguese and apparantly it is very common for Pacific Islanders while it is very uncommon for Portuguese to have it. None the less, our infant had further testing done at Stanford Lucille Packard and they confirmed that he has Alpha Thalassemia Hemoglobin H. I'm trying to get a much information as possible on the condition but I feel like I am getting so lost in all of the information that is out there. Not only that but I am getting mixed responses from our doctors. My OB was notified of by the hospital that my son has Barts disease (is that what he really has?), his pediatrician and hematologist stated that he will have a normal lifestyle the only thing we need to worry about is blood transfusions when he gets ill. Is this the truth or are they just telling me what I want to hear?

I just feel so lost and don't know where to start. I don't know what kind of questions I should be asking our hematologist during his appointments. Any suggestions? Is there anyone else out there have an infant with Hemoglobin H whose disease has not affected their growth or lifestyle? I just want to know what I should expect and prepare for it.

Thanks so much!

Hi LeanneMay,

Hemoglobin H disease is a 3 gene affected alpha thalassemia. It is NOT Bart's, as this is the 4 gene deletion variety and the affected babies do not normally survive pregnancy, as no useful hemoglobin is produced. There wil be some hemoglobin Bart's detected at birth but this does not mean the child has Bart's. With HbH, if the hemoglobin level is sufficient, transfusions are not required but there will most likely be a life long anemia, and as the doctors have told you, transfusions can be necessary during some illnesses, as the hemoglobin level will drop when ill. Folic acid is the main therapy and the child should take it daily throughout his life. Please also review the post at http://www.thalassemiapatientsandfriends.com/index.php?topic=3410.msg34830#msg34830  This will tell you which foods, drugs and chemicals should be avoided with alpha thal, so as to avoid hemolytic crises.

I want to recommend something. Register with the Cooley's Anemia Foundation and talk to Eileen about getting a referral to see Dr Vichinsky at Oakland Children's Hospital. Dr Vichinsky is probably the foremost authority on alpha thalassemia in the US and is a fantastic doctor. A full evaluation would be recommended, along with any necessary program. Dr Vichinsky will be able to answer all your questions and give your doctors any guidance they may need.


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

Thanks Andy, I actually think that the hematology program at Lucille Packard is a division of the one at Oakland Children's Hospital. But I will definitely reach out to Eileen and register. Quick question and please pardon ignorance on the matter, is hemoglobin H the same as Cooleys anemia?

Also, what is a sufficient hemoglobin level? Are these questions I should be asking the hematologist?

There are two basic forms of thalassemia, depending on which of the hemoglobin genes are affected (hemoglobin is made from both alpha and beta globin). These are alpha thalassemia and beta thalassemia. Technically, Cooley's anemia refers to beta thal, but the focus of CAF is on all thalassemias.

Sufficient hemoglobin levels are somewhat subjective and have to be partly determined by the health of the patient. With HbH, you may see normal Hb levels in the 8-11 range, and as long as this is maintained, transfusions are generally not required. And yes, you should write down and ask every single question on your mind when you talk to the hematologist.

Thanks Andy!

Welcome to the site LeanneMay.

Hi LeaneMay,
  .... keep in-touch and keep posting .....

Best Regards
Take Care
Umair

Welcome to the site!!!

Do not hesitate in asking everything you need to know.

Yours,

Laura.