Latest Update On AQEEL

Hi,

 

Just to update on Aqeel. He is now in standard 4 (coming to ten years old). I initially sent him to a chinese school from standrd 1 till 3. But middle of last year i transfered him to a non chinese school but i still send him to his chinese tuition classes. Seems like he cannot cope much on the homeworks as it was all given in chinese and i decided that Aqeel enjoy his life as a kid. Dont want to much stress him out on it. ;)

He recently was awarded the iron Hero from the hospital as his iron was the most reduced among all the kids. his ferritin is now 1+++.

Aqeel was warded for 13 days few weeks back as hes having mycoplasma and denggue that effected his lungs. He is now still coughing though. Yesterday was his monthly BT, and he was given antibiotics for another 5 days.

BTW, My wife is expecting our third child. Please pray for us that the baby is just a minor like my second child. My daughter is a 6 year old active kid having G6PD. So far she have not encountered anything difficulties of breathing etc.

Well thats about it really. Will update again later. (one of my new year resolutions is to be active back in this group) huhu.

 

Thank you for the update.
If I am correct you can do a test during the pregnancy to see if the child has thalassemia  or not.
All the best to Aqeel and your family!

AL,

Thank you for the update.  Glad to know that Aqeel is doing well. Sending best wishes for your wife and hoping that your third child will be thal minor or have no thal at all.

Best,

Sharmin

Hello
May I know after how many transfusions , spleen increases.  If one starts transfusing for a year or so , will the bone marrow stop producing hb and get transfusion dependent?

Hello
May I know after how many transfusions , spleen increases.  If one starts transfusing for a year or so , will the bone marrow stop producing hb and get transfusion dependent?

No, the bone marrow will not stop producing red blood cells. But the bone marrow does  not hsve to work crazy hard to try to get hb up. Transfusions bring a little bit of peace.

Thank you for your reply! So basically if my baby keep hb level of at least 6.5 without transfusion and we start regular transfusion for a yer or so and then stop, the bone marrow should at least keep the hb leve of 6.5 not less , right?

No, I do not think it would like that. A hb is not precize stable. You should see and watch, I think. Do you talk about 6,5 gl/dl? Why so low? Where do you live?

Hopefully Andy will see this post too.

I live in USA.  We have seen hb fluctuate between as high as 8.6 and as low as 6.5. We are monitoring.  And in bothe the scenarios since last 4 months we have transfused her as soon as it drops below 7.  She is Thal intermedia and we came to know about this during annual check up.  I went to chop philly and they also said the same to constantly monitor the hb level and decide on next steps.  We transfused when she was 13 months old and then again at 16 months old.  It's been a week now and I will check the number after two weeks. 
My question was related to be on a regular transfusion.  She has some adult hemoglobin like 34 % and she beta + +.  I was thinking if we regularly transfuse her, will the bonemarrow get used to the outside blood and stop producing the current hb even if it's low. 

I am not sure if Thal intermedia major or minor makes any difference.  There should be just two caregoroies. One need regular blood transfusions the other not. 
Praying some day the medicine will be available for kids after clinical trial so that we can avoid the transfusions.  Very difficult to find Iv for a one year old.

If the Hb is kept below 9, bone marrow activity will continue. The higher the Hb is kept through transfusion, the less your bone marrow will work. The the goal in transfusing thal majors is exactly that, as their bone marrow works and produces nothing of value. If you are trying to minimize transfusions in an intermedia, you want to keep the Hb below 9. In this case, hydroxyurea and wheatgrass may be of significant value in raising Hb without transfusing.

Thanks Andy.  That was precisely my question. I want to transfuse so that it stays above 8 and at the same time not transfuse chronically so that bone marrow does not produce anything.  Let's see. Will draw next week which is after 3 weeks of transfusion

hydroxyurea and wheatgrass may be of significant value in raising Hb without transfusing.

What the dose or how hydroxyurea and wheatgrass should be take for raising Hb without transfusing.My baby is now 3 yrs old and take transfusion after every 5 weeks...

Please advice Andy..

The dose of hydroxyurea is determined by the doctor. It can only be taken under a doctor's supervision. The typical dose used in thal is 10-20 mg/kg. The dose of wheatgrass is determined by what form the wehatgrass is in. If it's a packaged product, it will have dosing information on the label.

Hi Everyone,  

Aqeel is now 11 years old this year, and the eldest of 3. Yesterday was his BT day. Dr said that his spleen size is 4cm. liver 1cm. Pre Transfusion HB 8.3.

Later, I will try to give him MADULINA as a lab test has been done by one of our members here and were found to be safe (comprises of Spirulina, honey etc) as i was told that it may have an effect in increasing his HB. Though, to some others it may not. As for me, i'm willing to try it for Aqeel & Aamara sake.

Btw, my youngest daughter Aamara is also as same as Aqeel (Thalasemic) she is now a one year old girl. Did ask the Doctor to do do the electrophoresis test. Waiting for the result. Planning soon to find out if the cord blood from her sister matches should we need to opt for BMT. If possible i dont want my daughter to go through what Aqeel went trough all these while. Cant imagine a little girl has to go be poke every now and then.

FYI, Here in Malaysia, the government Hospitals does not do Unrelated Donor for BMT, wonder which hospitals does it? Anyone here in Malaysia knows?. The fees surely gonna be expensive but he will try to work on it.

  Gene therapy
  Luspatercept  

Later ya

love u all