How Was Your Intermedia Diagnosed?

Hi my name is Kathleen...
MY daughter Olivia was just diagnosed with Thal Intermedia on 7/27/06. Olivia is 2 yrs 5 mo. we have had our concerns since she was 8 mo old when she got the rotavirus and her hemoglobin was 8.6. Since then it has dropped slowly and now as of yesterday it was 7.1. Unlike my husband I knew I had the trait since i was a child. He was tested when we were 1st married but his dr. said he didn't have the trait and we went on to have children. (we also have a 6 yr old girl & 4 yr old by who have the trait). I always knew something was wrong with her health. She dosen't look like my other children. She does have minor deformities in her forehead area and now we were told the dr. can feel her spleen. Although her hemoglobin is at 7.1 the drs. do not want to tranfuse her because eventhough she is growing slow she is still growing. I guess they feel she is stable right now.  We feel so overwhelmed  and scared, so any comments or experiences would be helpful to us.

Thank you    Kathleen

Hi Kathleen,

Where is Olivia being treated?

I'm troubled by the fact that she is not growing at a normal pace and also exhibits some skeletal deformity at such a young age. The Hb level is also very marginal. I would suggest a second opinion. Beliefs about how to treat intermedia vary greatly, but this seems to be a serious case of intermedia to be exhibiting such symptoms at an early age. The spleen is enlarged because her body is overproducing deficient red blood cells in a mostly futile attempt to raise her Hb. These cells are destroyed by the spleen, thereby enlarging the spleen.

Keep Olivia on a low iron diet, as non-transfused thals intermedias often suffer from dietary iron overload due to over absorption in the gut...the rate may be up to 100 times what a non thal would absorb. This can create levels high enough to require chelation to remove the excess iron.

Kathleen,

Do not feel scared and you have come to the right place. There are quite a few patients who have had different experiences with Thal(Intermedia). What type of Thalassemia(Minor) do you have? I think sometimes the diagonosis for trait is NOT done properly. I have heard atleast one family whose trait testing did NOT show both parents having trait and then later on after DNA testing it was confirmed they both had some form of Thal.

Thal(Intermedia) is better than Thal(Major). Also, you might want to check if using Wheatgrass is helpful in Olivia's case.

-Narendra

Thank you for your reply....

Olivia is being treated here in Phx Az. The office is Arizonia ped. hem/onc... Just yesterday I asked her Dr. how many pts. they see at their office with thal intermedia or major and he said very few b/c of the pop here..he said in India he saw 5 transfuse pt a day. What  also worries me is that before her dx they said she had an iron def. and had her on iron therapy....I asked the dr. adout this and he said it was for a short time that he dosen't think that it would affect her but the are checking her iron level now (we don not have the results yet) and he is starting her on folic acid.....Its funny that you suggested a 2nd opinion because I have this feeling it more than what they say.....they did x-rays and felt that the bone deformities are mild and to tranfuse now would be for cosmetic reasons only?? As for her growth she was in the 5-10% up until Feb 06 now she is in the 10-25% and she is growing in height and he was pleased with this......I have been looking for a new dr. here I have to keep searching....Thank you again!

I don't know if you have been there but Phoenix Children's Hospital does treat thalassemia. If you find you are not happy there, you might consider contacting the Oakland Children's Hospital and ask if your child's records be reviewed to see if they might suggest a different course of treatment. In Intermedia, the individual doctor plays a major role in determining whether or not to transfuse.
You can also contact Eva Chin at the Cooleys' Anemia Foundation and ask for referrals in your area.
      eva.chin@cooleysanemia.org

from  http://sickle.bwh.harvard.edu/thalover.html

Thalassemia intermedia. Thalassemia intermedia is a confusing concept. The most important fact to remember is that thalassemia intermedia is a description, and not a pathological or genetic diagnosis. Patients with thalassemia intermedia have significant anemia, but are able to survive without blood transfusions. The factors that go into the diagnosis are:

    * The degree to which the patient tolerates the anemia.
    * The threshold of the physician to transfuse patients with thalassemia.

With regard to the tolerance of the anemia, most patients with thalassemia have substantial symptoms with a Hb of much below 7 or 8 gm/dl. With hemoglobins of this level, excess energy consumption due to the profound hemolysis can produce small stature, poor weight gain, poor energy levels, and susceptibility to infection. Further, the extreme activity of the bone marrow produces bone deformities of the face and other areas, along with enlargement of the spleen. The long bones of the arms and legs are weak and fracture easily. Patients with this clinical condition usually do better with regular transfusions. The need for regular transfusions would then place them under the heading of thalassemia major (see below).On the other hand, some patients with marked thalassemia can maintain a hemoglobin of about 9 to 10 gm/dl. The exercise tolerance of these patients is significantly better. The question then becomes whether the accelerated bone marrow activity needed to maintain this level of hemoglobin causes unacceptable side-effects such as bone abnormalities or enlarged spleen. This is a judgment decision. A given patient at the critical borderline would be transfused by some physicians to prevent these problems, even if they are slight. The patient then would be clinically classified as having thalassemia major. Another physician might choose to avoid the complications of chronic transfusion. The same patient then would be clinically classified as thalassemia intermedia. The patient has thalassemia that is more severe than thalassemia trait, but not so severe as to require chronic transfusion as do the patients with thalassemia major.

Thanks for that info. We have an appointment on Aug. 14th at the University Of AZ medical canter.....We did see a different dr today at our drs office and he suggested that we try Olivia on Hydroxyrurea. He said that he has had many Thal Intermedia on this drug with great results. Is there anything that you can tell me about this or is there anyone out there on this we would like to know if it is working.....

     Thanks
         kathleen.

Hi Kathleen,

                 Andy has discussed that Hydroxyurea is used for Thal. Inter. He also pointed to an article for it's use with Thal. Major. I think you should read that thread as it might have useful information of how it is better for Thal. Intermedia. The link to the thread is http://www.thalassemiapatientsandfriends.com/index.php?topic=253.msg1598#msg1598

Hydroxyurea can raise fetal hemoglobin levels in thal patients. It has mostly been used in intermedias but has been used in some trials with majors also. It often eliminates the need for tranfusions. Wheatgrass is also known to raise fetal Hb levels. I would highly recommend wheatgrass shots from wheatgrassactive. If hydroxyurea is offered as a treatment it is very much worth considering.

Once again thank you so much.....my husband and I are going to go ahead with the hydroxyurea !!!! I pray to God it works for her we wont give up!!!!
I also mentioned the wheatgrass to my dr. and he didn.t know much about it but he was going to reasearch it.
Thank you
kathleen

PS....I spoke with Eva Chin this morning and she is tring to put us in touch with a family here with a thal child...we are also in the process of scheduling an appointment with Phx Childern's Hosp.

Hi everyone!!!!

I wanted to let you know that Olivia is on HYdroxyurea for 20 days now. We had her CBC and her hemoglobin went from a 6.7 to a 7.6  her white count and plt count is well within normal range.
This gives us hope that this will work for her. Her  Dr. was hoping for a higher count but we are happy and feel like this is the answer to our prayers right now,we stilll pray and take it day by day and day and keep positive. We were seen by Dr. # 1 who has been treating her for Tahl intermedia all along and 3 weeks ago when her count was 6.7 he did not want to do anything but wait and watch. I could not do that so he reffered us to speak with Dr. # 2 who is another Dr. within the same practice who has had alot of experience with hydroxyurea when he practiced in Detroit. So  I did as much reasearch as i could and spoke with Eva Chin asking her if she could provide me with any info on this drug & she told me about Dr. Titi SInger who has spoken about hydroxyurea. So I e-mailed a Dr. Titi Singer and we received positive information and this along with our reasearch helped us to decide to give it a try. I am happy we did !! If this drug keeps Olivia tranfusion free for a year or any amount of time I will be thankful.

Kathleen Gulko

Hello Kathleen,

Hb of 6.7 to a 7.6 is pretty darn low! Since your child is small that may not seem to be an issue, but I think she needs a normal level of Hb to ensure proper growth and development. In any case, staying off the Transfusions is the best thing that can happen to a Thal. but make sure her growth and development is following the normal trend.

Take care.

Hi,
    In response to the last post re Olivia.....at her last visit Olivia gained 1/2 pound and is now 26 lbs and in height was 3months ago 34 inches and is now 36 inches. She is active and wants to eat all the time which she has never done before. She also continues to take folic acid and is on low iron diet. I do think she is growing at a slower pace and is smaller than my other children were at this age. We only continue to hope and pray that the medicine works so that she does not need transfusion for some time. We hope to see that her hemoglobin increase at her next visit in 2 1/2 weeks. Thank you for your input it does help us to hear this from people with expierence and who are going through some of the things we are.
Our best to all  you are in our prayers
Kathleen

Kathleen,

I'm glad to hear she has an appetite now. That is very good news. I hope the hydroxyurea raises her Hb some. Staying off transfusions as long as possible is definitely the best strategy when dealing with intermedia, especially in children.

Keep feeding her as much as she wants and make it nutritious. 

Hi Kathleen,
I'm a thal intermedia(Beta + HbE), 31 years old. I wanted to tell you that during my childhood, at times my Hb use to go lil low than average but then it use to pick up. My growth was slow as compared to other classmates till middle school. But then at High school I picked up.
Don't be disheartened. I wanted to tell you that life for me was/is a little tough(health wise) but definately not bad.
I understand how you feel at this time, but please be strong. Olivia will be OK. Hydroxyurea treatment is definately a better choice than transfusions and you should feel lucky if the treatment suits Olivia. There are ppl who do not respond well to even this treatment. Hence, you have one reason to be happy

Keep us posted and ask any questions if you have.

Hallu.

Hello Kathleen,
                    That is a good news that Olivia is active and gaining weight. You are doing great. Hopefully Hydroxyurea will improve her Hb and she won't require any Transfusions at all. Just like Mr. Andy said, give her a good nutritional diet. Keep her happy and everything will be just fine.

Take care!