Intermedia

[babylegs]

Hello everyone....my name is Joanne

What about thal intermedia?
I dont have major I had only one transfusion in Nov 2004 when I had my son.. Im 37 but i do have a very large spleen 20 cm...low platelet count 99 and low hemoglobin 97
anyone know what it means to be in the middle?

I live in Toronto Canada and I am greek

[marientina]

Dear Joanne,

Welcome to ThalForum. I am greek with thal minor and I live in Los Angeles. A few questions first, have you been officially diagnosed with thal intermedia? With childbirth, thalassemia minor can result in moderate anemia. Make sure your hematologist makes an accurate diagnosis. If both your parents have the trait then you may have thalassemia intermedia but I don't think you can get thal intermedia otherwise.

Diagnosis are sometimes based on symptoms rather than genes and that can make treatment inappropriate. So you can be a thal minor genetically but if your anemia becomes severe you may be labeled as thal intermedia even though you are not genetically thal intermedia...Has your son been tested?

Some info for you: http://www.thalassemia.com/beta_thal_3.html

[babylegs]

Hi Marientina
I am seeing a specialist at the hospital here in the thalassemia clinic.
My whole life I thought I had trait but my spleen is very very big...measuring 20cm
I am dead tired all the time and my hemoglobin dropped to 60 during pregnancy...it is never higher than about 96
I also have problems with my platelets...they sayall this is due to my enlarged spleen
I have not tested my son yet but his father does not have THAL at all.

[marientina]

Your spleen is indeed large and so is your hemoglobin. Mine after whole year of serious effort is at 10.8. I will test again in two weeks because I am also dead tired again and I know it has dropped yet again. The fatigue is horrible. You are quite brave to have your son. I hope that he is healthy. You should test him for thal minor since he may have inherited that from you.

We have several folks here who have had splenectomies and I am sure they will reply soon. I don't know at which point that decision is to be made. You're not quite low enough for another transfusion and if indeed you have thal intermedia more transfusions mean having to start worrying about iron overload. What is your ferritin level lately?

My spleen is slightly enlarged and I have just thal trait. It was huge when I was a child. I always had a big tummy...

[marientina]

Here's some more detailed information on thal intermedia and thalassemia in general...This is one of the more concrete explanations I have found.

The fundamental abnormality in cases of thalassemia is impaired production of either the alpha or beta hemoglobin chain. Thalassemia is a difficult subject to explain, since the condition is not a single disorder but a group of defects with similar clinical effects. More confusion arises from the fact that the clinical descriptions of thalassemia were in use before the molecular basis of the thalassemias were uncovered. A review of thalassemia is best approached by examining it from a genetic basis and from it’s clinical expression.

...

(b) 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 and 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 Hemoglobin of much below 7 or 8gm/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 9-10gm/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 an 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.

A patient can change status, and the spleen is enlarged in these patients. The spleen plays a role in clearing damaged red cells from the blood stream. Since all of the red cells in patients with severe thalassemia have some degree of damage, clearance by the spleen accelerates the rate of cell loss. Therefore the bone marrow has to work harder to replace these cells. In some patients, removal of the spleen slows the rate of red cell destruction just enough, that they can manage without transfusion, and still not have the unacceptable side-effects. In this case, the patient converts clinically from thalassemia major to thalassemia intermedia.

http://www.diagnose-me.com/cond/C311039.html

[babylegs]

I never knew my son could get Thal minor...now i feel awful andanxious about it!
I thought Thal minor had no symptoms...
I have never had transfusions except when I had my Csection.
What is considered low for hemoglobin?

[marientina]

I have written about this classification problem before (symptom vs. genetic classifications). Thal minors often ask if they can have thal intermedia or major. I suppose the answer is that symptom-wise yes, but genes don't miraculously mutate or degenerate on one person in thalassemia as far as we know in this century...Maybe gene therapy will one day change this notion.

[babylegs]

I dont know how to read the hemoglobin for instance if mine is 96 how would that be read in the way i see it written example:7 or 8gm/dl
Would it be 9gm?
im confused

[marientina]

You should test absolutely your son. In greece, you would have to be screened for thalassemia if you were married there. My parents screened themselves for that and Rh and they decided to have me and my brother anyway. We both have thal minor, inherited from my father and we have been quite sickly since birth.

Many carriers are asymptomatic but if you look into the thal minor forum, you will discover that for many others many problems arise from thal minor and they can complicate life and other illness if it occurs concurrently.

[marientina]

Yours reads 9.6 to me. Pretty low from normal. If I feel like crap at 10.8, I know you must feel awful. I have the numbers posted in the thal minor forum but I think 12-14 is ideal for women.

[marientina]

I am in the chat right now if you want to talk.

[ariespar]

Hi everyone, my hemoglobin level is at 7.9 on 3/15/06. I was at a 9.6 for the longest time but now it has dropped 2 points! My spleen is also very enlarged, over 20 c.m. I believe. I was supposed to take it out but they said I needed a blood transfusion prior so I did that and had a very bad allergic reaction. That was over three weeks ago and we are going to try it again. My Onc/Hema said that taking out the spleen will make my hemobglobin higher and I will probably not have to use desferral anymore too. Right now I feel pretty healthy but I'm not sure because I have lived like this for so long.....I do have shortness of breath sometimes. BTW I have Thalasemia Intermedia and I am 35 Yrs old.

[babylegs]

Hi...my spleen is also 20 cm...

[Vik]

7.9 is pretty low.

Being thal major i get I transfused often. I had my spleen removed long time back as my HB dropped to low too soon after transfusions and my spleen was huge too(dont remember how big it was). Shortness of breath could be due to low hB as the oxygen is not reaching the tissues in the quantity required BUT nonetheless you should have it checked out by a cardiologist. Since you mentioned u are on desferal, which means you have an iron overload - so its a good idea to have it checked out.

[ariespar]

anyone ever get treatments of Procrit before?? It stimulates your bone marrow to produce more RBC.

Joanne, has any of your Drs. recommend a splenectomy ( removing the pleen)?