Can any body explain????

Hi

Can any body explain this to me? When Ahmad HB is 6.6 , his Ret. count is 11% and when the HB decreased to 6.2 , the Ret. count became 6%. To my understanding the relation between them should be inversely propotional. So why is that???

Manal

PS: Last month Ahmad started tennis and Karate classes and he is doing very good

Hi

Can any body explain this to me? When Ahmad HB is 6.6 , his Ret. count is 11% and when the HB decreased to 6.2 , the Ret. count became 6%. To my understanding the relation between them should be inversely propotional. So why is that???

Manal

PS: Last month Ahmad started tennis and Karate classes and he is doing very good

Maybe that's a miracle all by itself! 

Hi, Manal.

Reticulocytes are immature red blood cells. The reticulocyte count is a reflection of recent bone marrow activity.  So, if the bone marrow is responding appropriately to the demand for increased numbers of RBCs, then the bone marrow will allow for the early release of more reticulocytes, which will then increase the reticulocyte count.

If a patient has chronic blood loss, in cases like Thalassemia Major/Intermedia, then the number of reticulocytes will stay at an increased level as the marrow tries to keep up with the demand for new RBCs. If it's unable to keep up, or is not functioning normally, then the number of reticulocytes may be normal, or only slightly elevated despite demand.  However, they will eventually decrease due to insufficient production. The normal range for a healthy individual does not usually exceed 2%, but if your son's reticulocyte count is rising, that means that his bone marrow is still capable of making some new cells, which is good.  If the number of reticulocytes is not elevated in an anemic patient, then it is likely that there is some measure of bone marrow dysfunction or a deficiency of erythropoietin, which is usually the case with Thalassemia Major/Intermedia patients.

I think because he is still young, his bone marrow is still capable of making its own cells, and will continue to do so, unless he eventually becomes hypertransfused as some Intermedas that I know have done.  If he is only transfused every once in a while, that will not happen, though.

If you have any questions, don't hesitate to ask.  I started to confuse myself just now, so hopefully you didn't get confused by my explanation.   

That's a good explanation Danielle and I'm learning!     I was checking through my blood test files and there was one time I experienced the same thing.  Now there's an explanation for it! Thanks! My hugs for you! 

Thanks Lei and Danielle

I think because he is still young, his bone marrow is still capable of making its own cells, and will continue to do so, unless he eventually becomes hypertransfused as some Intermedas that I know have done.  If he is only transfused every once in a while, that will not happen, though.

Danielle so does this mean that he can continue like this? and if his ret. count stops elevating, this is what they call bone marrow shut down?
I understood that this is something good for him, but is it also an indication that he is vulnrable to bone deformation?

Guess that my questions are the confusing ones

Thanks alot
Manal

Hi Manal,

Ahmad's hemoglobin is quite low, and if he stays at that level, there will be at least some kind of bone deformation as he gets older.  As long as he does not get frequent blood transfusions, then his bone marrow will continue to work at some level on making red cells, but not enough to keep him at a high enough level.  If the reticulocyte stops elevating, then it just means that the bone marrow has given up to the fact that there is a dysfunction going on.  Bone marrow shut down is not going to happen, because it will continue to work, even though it's making defective RBCs.  The only way the bone marrow will shut its RBC production off is if he is regularly transfused.

Honestly, this is usually where the difficult decision takes place for a parent of a child with Intermedia, which my parents had to do with me when I was stabilizing at a very low hemoglobin as a baby.  My bones started to change, and that's when my parents decided to hypertransfuse me.  I was probably a Major anyway, but I could've probably held off being transfused for a little while longer if my bones hadn't started changing.  However, my bones eventually went back to normal after the transfusions.

You may decide that you want Ahmad to get a transfusion here and there, so that his bone marrow doesn't have to work as hard, and cause bone deformities.  On the other hand, you may decide that he is doing well without them, and take it one step at a time.  Intermedias seem to adjust to the very low hemoglobin that they stabilize at, but the bone marrow will continue to try and make cells, even though they are dysfunctional cells.  He may not even need any transfusions, or may need them on occasion as an adult.

He will be ok either way, but there are always pros and cons of transfusing with an Intermedia, as there is for anything.  That is something that you need to work out with his doctors.  If you are not comfortable with the decisions that his doctor is making, then I am a complete advocate for getting different, and numerous, opinions.

If you need anything, or have any other questions, please do not hesitate to ask.  I will be happy to give you any of my advice, and I'm sure Andy and others will give you their opinions as well.  Sometimes other parents of Intermedias, or patients themselves, can give better advice than any professional can.  Experience in the matter is very important.   

Thanks Danielle alot for your explaination

Actually, i am taking it step by step as you said. For the time being i checked his skull ( since it is the first to be deformed) and the X-ray showed that it still normal in all the dimensions. But at the same time i do believe that this is a matter of time. So before this time comes i am trying to work on increasing his HB. First trying to use any natural source (resveratrol, aloe,.....) and if they didn't work, hydroxy urea may be an option. I hope i am doing the right thing for him

Manal

You are his mom, and you care about him more than anything in this world, so I know you will do what's best for him. 

That's exactly what happened to me when I was a baby.  My skull started to separate, and that's why they decided to transfuse me, and then it eventually came back together.

But anyway, you will know what to do for your child, and taking it step by step is a good idea.  Hydroxyurea may end up working for him, so give it a shot.  If Ahmad does eventually need a transfusion, it will be ok.  Try not to be scared of it.