First Transfusin related question

My son turned 4 yrs recently, he has E-Beta Thal (in between thal major and inter),  his HGB is in early 7, for almost last 2 years. He is a cute and naughty boy, normal like any 4 yr olds. He goes to Montessori school. He has been maintaining ok in his height/weight percentiles. He goes to Children's Medical in Dallas for quaterly follow up - check ups, his doctor has noticed some bony changes in his forehead so she is planning to start him on monthly BT, from October end.

We had also taken him to Oakland once 2 years back and I had recently sent his details to Dr. Vichinsky he adviced me, that we can start him with BT for 6 months and see how he is doing or we can also wait for another 6 months and see how he is doing and based on that make a decision.

We are going ahead and thinking of starting him on a regular BT for next 6 months and evaluating , I would like to check with all of you what are the test/things i should ask his doctor to make sure they do before we start on the BTs? I asked his doctor, several times to match all the antigens and have the red Phenotype done, she said that that they only match 5 antigens her in CHildren;s Dallas and will not be matching the others, i think they have the red Phenotype done.

Dr Vichinsky had told me to do the echo but his doctor here in Dallas said that is not reqd. to be done for his age before we start the BT.
So i am very confused so I would really appreciate if you can pls. guide me the things I should make sure that are taken care of before the BT's begin, any advise or any points that you could thnk of for us to take care of before/during/after his transfusion.

Thanks so much.

Hello,

Children's Hospital Oakland site has very good information on Blood Transfusion and what needs to be done prior to transfusion. You can find the link at www.thalassemia.com and select Treatment/Blood Transfusion from the menu options.

I am copying some of it here for reference.

Baseline laboratory tests prior to regular transfusions
An extended red cell phenotype must be obtained to reduce the future probability of developing alloantibodies. If a child has already started transfusions, the red cell antigen genotype can be determined by DNA testing, and at the minimum, should include the C, E, and Kell alleles.

Although the hemoglobin level can define a patient’s disease type, seldom does it alone determine the need for transfusion. Antibodies to hepatitis B, hepatitis C, and HIV should also be determined. Patients should demonstrate immunity to hepatitis B. The bilirubin, transaminase, and serum ferritin levels should be checked.

You cannot find a better thal doctor than Dr Vichinsky. If he says do it, tell your doctor you want it done. Baseline tests before transfusion are very important and should be done.

Thanks Narendra and Andy, for your suggestions.

The issue is that his doctor, here, will not agree, for the ECHO, I have requested her and her staff severl times for the tests.

I wanted to also check with you that in the Phenotype testing, will they not know the type of the blood etc, I asked the Nurse for BT, here, the question on last Friday, and she said that they do not know his blood type yet   

I am surprised, he has been going to this clinic for the past 4 years, they have done DNA/Phenotyping and numerous other blood work on him.
So not sure how they do not know his blood type.

The nurse said that they will do the cross matching etc on the morning of his blood transfusion, so I was trying to find out from parents, whose children have BT, in other diff, centers, all over US/Canada, what are the blood tests that are done before the first transfusion and then before every consecutive transfusions, as the cneter here, is not helping me much to give me the information, I keep asking them questions  always and it seems like that I am th eonly parent who is asking them the questions.

I will be looking forward to hear back from you all and thanks in advance for your time.

Hi MomofABabyThalMajor,

If your insurance covers it, you can get half yearly check-up done in Oakland children's hospital and get echo or all other tests done at Oakland. My daughter's echo was done in phoenix but when we visited Oakland recently, they did it again as it was not done (or read) the way it is supposed to be for a Thal patient. So, i will suggest not to waste your time fighting for getting an echo done in a center that does not specialize treating Thals.

If you can visit Oakland before your child's first transfusion, you may be able to make sure you are ready for all the things that you need if you are having hard time getting it done at your current hospital. Raquel Manzo is the coordinator for scheduling visit to Oakland and she is extremely helpful. Her contact info is 510-428-3347. WE have relatives so we have never used their help with accomodation but I have heard that they can arrange special rates in hotels in oakland. Good luck!

Don't allow doctors or nurses to push you around. Your baby's health depends on proper treatment and the best treatment is found only at the Centers of Excellence. Talk to Oakland and ask their advice on how to proceed. Bostonian has given you contact info that will help a lot for making arrangements.

I am going to be brutally honest. Far too many thals in the US are still dying young because their local center will not follow the Standards of Care Guidelines for Thalassemia. It breaks my heart to see patients in their 20's and 30's succumbing simply because their treatment was not the comprehensive program required for MANAGING thalassemia. The founder of this group falls into that category, even though she was treated at the hospital where the term thalassemia was first used. There are very specific guidelines for keeping thals healthy and if not followed. the patients suffer. There is not a single medical professional on earth whose ego is more important than any patient. Parents need to take charge and demand when it is necessary. Oakland can help counsel you on how to approach this, as they deal with it constantly.

Thanks you both for your replies.

I contacted Dr. Vichinsky directly, last afternoon, directly and spoke to him in details, he has told me that he will personally talk to my son's doctor, and discuss the situation.
He told that he will be more than happy to check my son there for a second time, in Oakland as he does not feel that BT is reqd. as an emergency (it can wait for 2-3 months).

Yes you all are right, we need to be very careful.....we are planning to take him to Oakland for his 2nd check up and get the tests done before, properly, we start him on a monthly BT program.

Thanks so much for all of your support, I will keep you guys posted.