Prat's check up

Puja, children shouldn't take aspirin. It is recommended that children under age 19 don't take aspirin unless prescribed by a doctor for a specific condition where it is considered the benefit outweighs the risk. In addition to vitamin E, you can include foods like ginger, garlic and fish oil to provide anticoagulant properties. However, if any blood thinning drugs are taken, the dose may have to be modified because of the dietary sources of blood thinners. Once he is older, he may try a daily aspirin if it seems necessary. I do think diet can play a very positive role. Prat's blood counts are really good. I'm thrilled with his HB. The platelet count is moderated by the note that they are adequate and normal in size. This report could very easily be from a minor rather than an intermedia.

Manal, antioxidants are safe to take and a healthy diet will provide many different antioxidants on a daily basis. These supplements are all food products. A fruit salad and a whole grain muffin would provide an even wider variety of antioxidants, but the supplements are more concentrated and we always need to remember that these are supplements and not replacements for a good diet. Thals do have to supplement no matter how good their diet is because of the incredible demand for vitamins and minerals that the stresses of thalassemia cause.

I am very interested in what is observed using FPP.

By the way Manal, you have stated the seeming paradox with hydroxyurea. It works as a chemo drug by suppressing bone marrow activity, but when used in smaller doses with thals it does have some suppressing effect but at the same time, it increases the amount of fetal hemoglobin being produced. You might say it's pulling in both directions at once. Because in a way hydroxy partly negates itself, there has been much research into other drugs that only induce hemoglobin and don't suppress the bone marrow. If the Hb can be raised enough, this in itself will regulate bone marrow activity.

And hematocrit is used more in the US while hemoglobin level is used more in the rest of the world. Hematocrit will be roughly triple the Hb level. A complete blood count will often include both values, but they can be used equally to determine if the patient should transfuse. Hematocrit tells the percentage of red blood cells in the blood.

Hi Andy

You dont have any idea, how thrilled I am to read your reply. I cannot thank you enough for your continuous support and advice. 

On a lighter note, I guess Andy if you sit for the medical exam you will straight away pass as a Doctor or a Super specialist.

Thank you.

Andy  you have mentioned that kids should not take Aspirin. Just for curiousity I want to know if kids fall down or hurt themselves  badly,  what kind of pain killer should be given to them which is not too harsh on their system. I have  heard contradicting things abt ibruofen.

Manal what do you give Ahmad. Sharni/Zaini any advice.

Andy

If the Hb can be raised enough, this in itself will regulate bone marrow activity.

1- So do we expect Prat retic count to decrease when HB stablises for a while at 10??

much research into other drugs that only induce hemoglobin and don't suppress the bone marrow

2- Isn't suppressing the bone marrow activity a target in itself in thals to avoid bone deformity

3- Do the long term use of hydrea  cause depression in bone marrow activity or this is not the case since it is used in small quantities with thal ''hydroxy partly negates itself''

4- Why we sometimes we have intermedia patients on hydrea with same Hb range and with big difference in retic ?? Does the mutation play a role here??

5- With many children taking hydrea and with the confirmation of Oakland that it is safe drug why do they still write in the leaflet of the drug ..''Safety & effectivness in pediatric patients have not been established''


manal

Puja

I wasn't faced by a situation where i wanted to give a pain killer but when Ahmad has a fever the ped prescribe Brufen (antipyretic- analgesic- anti inflammatory) alternating with voltaren and both are gernerally pain killers

manal

Puja,

Doctors here usually prescribe Paracetamol for kids,for fever and pain.But it's not a blood thinner ofcourse.I think you should ask your doctor first if Prat needs blood thinner.I know he has had splenectomy.But did dr Marwaha or any other doctor prescribed him Aspirin or any other blood thinner?

Zaini.

The reticulocyte count is tied more to the rate of hemolysis than to Hb levels. so a higher Hb won't necessarily lead to a drop in reticulocyte count. Hemolysis is fairly normal in thalassemia and the rate can be affected by other factors sch as recent illness. Also, the normal reticulocyte count for children can be higher than that for adults with a range up to 4, so 8 doesn't seem quite as bad as it sounds. Why hemolysis takes place is a big question and research has shown that much of this actually takes place in the bone marrow and may be related to the extra alpha globin chains that exist in beta thal. It may in fact have little to do with functions of the liver and spleen in this case. The thing to remember in this case is that the Hb has gone up, which means it is effective erythropoiesis and not the ineffective type which leads to more and more expansion of the bone marrow. I don't think these reported counts are anything of alarm and may well be temporary. And yes, the genes do play some role in this alpha/beta imbalance, but more research is needed and is taking place to explain why hemolysis takes place in the bone marrow, killing the young erythrocytes.

In Prat's case, the higher HB will somewhat regulate the bone marrow activity. Even if it is high, it is not being stimulated by low hemoglobin levels, but by hemolysis, which is seen with the higher bilirubin level. While suppressing the bone marrow through transfusions is the goal with thal major, it is not the goal with non-transfusing intermedias, who need this activity to maintain an Hb high enough to live without transfusion. Hydroxyurea will cause some mild suppression of bone marrow at the levels used with thal but this is countered by its induction of fetal hemoglobin. This is actually why there is no clear cut protocol for determining whether transfusions are necessary in intermedia. A judgment is made weighing the amount of expansion against the hazards of transfusion (mainly iron overload) and why it becomes so necessary to accurately measure the iron load in the organs of non-transfusing intermedias. If it is shown that there is significant iron overload even without transfusions, then the reason to not transfuse vanishes even without other factors being present. Intermedias should pay special attention to avoiding iron rich foods because of this.

I also want to mention this very enlightening page, which really modifies how liver function tests should be seen. Higher SGPT can be expected in non-whites, so the "normal" levels we are told may not really be accurate for people of non-western lineage.

http://www.aafp.org/afp/990415ap/2223.html

Special Considerations in Interpreting Liver Function Tests

Mildly elevated ALT level (less than 1.5 times normal)    ALT value could be normal for gender, ethnicity or body mass index.

When we see rates around or slightly higher than the high end of normal, it is no cause for concern as many factors play a role. Something else of real note in this fantastic article is that it is the gallbladder that is often responsible for high LFTs and misdiagnosis of hepatitis may result. So, considering what our dear Umair has been through lately, I would suggest a gallbladder exam to see if this may be the reason for the ongoing stomach problems.

This article has great info and explains why we see certain levels in liver function tests. I highly recommend reading and keeping this article.

Acetaminophen products are considered safe for children and our doctors have often prescribed ibuprofen also to combat fever. Because of the connection between aspirin and reyes syndrome, aspirin is strongly discouraged in children.

Thanks Andy, the relation between the HB, retic and bilirubin is finally clear now

manal

Andy

Just wanted to bring to your notice, as i missed out telling you earlier.

In July 2008 Prat's ferritin was 202, now in Feb 2009 it is 140.

I plan to do his liver scan done once he is in S'pore. For Gall bladder..what is the recommended test. A normal ultra sound, will that suffice.

Thanks

Puja

Puja,

I think an ultra sound can detect gall bladder stones,Please check this link.


http://www.umm.edu/patiented/articles/how_gallstones_gallbladder_disease_diagnosed_000010_6.htm

Zaini.

Puja,

I think that an abdominal ultrasound is all that you need to check the gall bladder.  I think that our little Prat is doing great - god bless!

I read earlier in the thread - about pain relief - I use Tylenol (acetaminophen) or children's Advil (ibuprofen) for both of my children when they are sick or hurt. 

Best of luck with Prat's remaining tests dear,

Sharmin

Thanks Sharni.


Will get the ultra sound done.. then will update.

How is Armaan and how was your trip.

Hi All

I am sorry I have been missing in action lately. kids are really keeping me on toes.

Anyways just to update you Prat's hb has gone down from 10 to 8.5. I had got a finger prick check. Overall he is looking ok and doing well but yes I could see the difference in his lip  and tounge colour, there are not as pink as before. As of now he doesnt have any infection, no cold cough.

I am planning on giving it a month to see. He continues to be on his normal doses of hydrea, vit e, fpp, l carnitine folic acid and calcium.

Please advise shall i give him another multivitamin.. which has the other vitamins as well besides the above.

He has been wheat grass for almost 8-9 months now.

Any suggestions will be highly appreciated.


Thanks

Puja