Maintaining HB at 9

So the current guidelines for thalassemia recommends to maintain a 9 HB. I go for transfusions every three weeks and by the time I am due I usually fall right under 8. It doesn't really affect me, and I can still workout that last week. Perhaps I am used to it by now. However, I am 19 and I don't know if I am fully grown. I was wondering if by allowing such a low hb, am I hurting my growth. Both height and muscle wise? What are some complications of having such a low hb.
I am also hesitant to switch the frequency because I don't want anymore unnecessary iron.

Thanks in advance

Hi,

How long have you been following the same schedule of transfusion,if it hasn't hindered your growth yet it shouldn't do so now,being said that,i don't think that keeping that low hb is a very good idea,because chronic low hb does damage your body in the long run,i know you are worried about unnecessary iron but when your hb is that low,your body absorbs more iron through the gut,low hb means low oxygen levels in blood and since you work out too,it made me think what if those last week work outs force your heart to work harder with low oxygen,BUT I am no doctor, its just my personal opinion so i think a hematologist might be able to help you more.

Zaini.

P.S whats your weight and how much blood do you take every three weeks?

I receive two full bags of blood. It varies but it's usually 600-650, not sure what the unit of measure is. They do  something like 15xkg to determine max.

I'm 57-58 kg. I understand what you are saying about the extra heart strain, somehow my heart rate never gets above 120 even with my heavy workouts. So that's what I go by to make sure I'm not overdoing it.

Also, can bone marrow deformities happen to someone who is no longer a child?
Wouldn't taking hydroxyurea prevent this, and actually benefit from the increased bone marrow function?

Btw, I think it's stunning growth because  grown but my bones, feet, and hands are pretty small. Also I haven't had a real growth spurt. I'm not too short so I'm not too worried, I just think that the reason my hb goes low so fast is that I am using it for growth, the doctor mentioned this when I was younger.

Thanks for the response btw.

Apollo,

You are not getting enough blood,according to your weight it should be around 850-870,meaning more then 2 bags,coz two bags are usually around 600 some of which remains in the tubing and all,so i think if you increase the quantity you might be able to give your hb a boost.

I don't know if bone deformities can occur in adults,but i'll assume that extra pressure on bone marrow might weaken the bones,and yes we have members who take hydroxyurea along with their transfusions,and that has helped them to increase intervals between their transfusions,but keep in mind,that hydroxyurea works differently for everyone.

Zaini.

Apollo,

You should try to get your Hb up higher and keep it above 9. It won't really result in a significant added iron load, because once you are at the higher level, it shouldn't take any more blood than normal per transfusion to maintain that level. Low Hb can definitely affect muscle growth, as adequate oxygen to the muscles is necessary for growth. You've mentioned pale stool while using Exjade. This is a common side effect of Exjade. Are your liver values being checked at least once per month? One thing you might consider is splitting the Exjade dose into two and taking half in morning and half in evening. This may help reduce the side effects, while actually giving you a true 24 hour chelation period.

Great idea Andy, I've read that the half life of exjade is around 14 hours. So I may do one dose in the morning and another at night.
However, as far as stools go, I'm not really sure it's the exjade. I've read up on the issue a little more, and increased bilirubin can cause pale stool.

In terms of the HB, I'm not sure why you meant by saying it will require the same amount of blood. Do you mean I should get one large transfusion every three weeks (ie I'm not getting a large enough amount now) or I should receive smaller ones every two weeks.

Currently my hb is around 7.9 on the third week when I get my transfusion.

Also I was under the impression that muscles needed only A surplus of nutrients to grow, and the oxygen only came into play in terms of endurance  while working out. If what you said is true then I hope the increased hb will help. I eat like a maniac lol.

Thanks to both of you for the responses thus far.

Also Andy, Since I have you here, won't taking zinc in such large quanties as you recommended 30-50 mg) interfere with iron chelation. I have a ZMA supplement but my doc told me not to take it for this reason. I'm not deficient btw.

Apollo,

When sometimes hb gets low because of an infection or something,or like in your case when hb is kept low,we hypertransfuse,you'll have to take a couple or three big transfusions in a row,or may be shorten the interval between transfusions for a couple or more transfusions,then once you reach a higher level of hb,you can go back to your normal schedule.Pre transfusion hb of 7.9 is very very low,it should be some where in between 9.5 or 10.

Zaini.

Just went to the doctor and explained the situation. She told me that she wouldn't transfuse unless I was below 9. I told her that a hypertranafusion or two transfusions closer together would raise my hb and after that I would require the same amount of blood at the same intervals as now. She told me that the higher a hb is the faster the rate of hemolysis is. She said she has patients who transfuse more but end up with the same hb.

Any ideas as to how this is going to work? She said she could do it every two weeks instead of three but my iron intake would be higher.

Edit:What do you guys suggest I do to get this level of hb up to 9? I'm starting to have more faith in you guys than my doc!

Let me quote from the Standards of Care Guidelines for Thalassemia. You can find these attached to the post at
http://www.thalassemiapatientsandfriends.com/index.php/topic,1762.msg14639.html#msg14639

4.3.3 Target hemoglobin and frequency of transfusions
The target is to maintain the pre-transfusion hemoglobin
level between 9 and 10 g/dL. Attempts to maintain pretransfusion
hemoglobin at above 10 g/dL increase transfusion
requirements and the rate of iron loading....
4.5 Splenectomy
The use of splenectomy in thalassemia has declined in recent years. This is partly due to a decreased prevalence
of hypersplenism in adequately transfused patients...
Often, hypersplenism develops because of a low pre-transfusion hemoglobin. Increasing the pre-transfusion hemoglobin
to between 9.5 and 10 may reverse hypersplenism.

The last sentence explains it all and is the opposite of what you have been told. To reduce excess destruction of red blood cells by the spleen, keep the Hb above 9. Please make sure your doctor has access to these guidelines. This goes for everyone, please. Make sure your doctors have access to these guidelines, so they can also benefit from the work of the experts that have brought a new day of hope to thalassemics.