No second transfusion ? Please advise Andy

Hi Zahra,
All I can say if that God is very good to us and I rely on my faith to help keep me going. Some days are better than others but that is life. Olivia was around 8 months when the drs suspected a problem. She had a virus and became dehydrated and needed to be in the hospital. It wasn't until she was 2 that a DNA test showed she was a Thal Intermedia. We tried Hydroxyurea at around 2 1/2 when her hb was around a 6.4 or so. Before that she was always around a 7. With the hydroxyurea she went up to a 9.3 for one month and then she lived around an 8. Then after 2 yrs the meds seemed to not be working. the drs told us we can go up in the dose but then side effects would be more of an issue. I will have to try to figure out how to download pictures so you can see the difference. I have to go for now Kathleen

Zahra, if no type of scan is available, then liver biopsy is the method used to determine liver iron concentrations. Hopefully, as the availability of MRI machines increases, we will see scans available everywhere.

You are welcomed Zahra. For L-carnitine, i know that it will be safe for your child to take it and it is taken as 50mg/kilo 5days per week. You can find it in the form of syrup and has a lot of benefitss to thal as mentioned above. By the way, it tastes good. As for vitamin E, i found that it can be taken starting the age of 1 year as mentioned in this link
http://lpi.oregonstate.edu/infocenter/vitamins/vitaminE/

What is important is that since you will be taking it permentantly,it should be from a natural source (alpha-tocopherol). But the good thing is that L-carnitne works as an antioxidant too, so you can start it now. You child should also be on a good multivitamin and mineral togather with calcium, magnesium and zinc.

Personally i use, Osteocare for (calcium,magnesium,zinc and vit D3) it is found in form of a syrup and suitable for infants. It is available in Dubai.
I use Kiddie pharmaton with no iron or vitamin C OR i use the yummi bears multivitamin
As for the Vitamin E,i used to buy a local one but now i will order the Vitamin E of Puritan Pride cause it is from a natural source

As for Osteoproseis, contious monitering of child growth in general and detecting bone deformity should be a regular routine for you. DEXA is the name of the scan used to measure the bone denisty and it is the only thing that indicate Osteoproseis. If everything goes well, it will not be required to do it before he age of 10 years

Please feel free to ask anything and take care
manal

Hi Manal,
Just wanted to know how your son was doing? What are his Hb levels? Is he taking any medication?  Thanks Kathleen

my daughter is a beta thal intermedia and our pedi hematologist recommended transfusing her every 6 to 8 weeks to keep her growth the way it has been going.  she is a beautiful little girl at 17 months weighing in at 23 pounds now.  i can't remember her height but i know she's in the 80th precentile.  also, our doctor transfused her more frequently during the winter season to help her body rest up so she could fight off whatever cold or bug came her way.  i think we followed the right course and she is thriving because of it.  hope this helps

Dear Mom of Izzie,
I am glad to hear that you r duaghter is doing so well. My son is also now one year old and doing well. He weighs a little over 10kg and looks happy and healthy. I do worry about what it will be like once they stop transfusing when he is two to try the hydroxy urea. They say a Hb of b/w 8 and 9 is enough to grow normally but thathe will look pale. What are the plans for your daughter in the future. Wishing you all the best
Zahra

Dear Manal ,
I have been giving my son osteocare for sometime now. I dont know if its related but his teeth have turned bright white froma translucent grey colour before. I asked my doc about giving him mixavit along with it and she also gave kiddie pharmatron instead but the dose isnt quite clear tome. For now I am giving him 2.5 ml of Osteocare and 2.5 ml of Kiddie pharmatron but that makes his Vit D to the higher side as the doctor said for his age it should be less than 400.Kiddie pharmatron has Vit E in it . Is i t necessary to add more? Thanks again for all your help
Zahra

Dear Zahara,

Thank you for your kind words.  Plans for my daughter, as of now, our hematologist wants to keep her hgb between 9 and 10.  My husband and I are also looking to maybe have a port placed so that Izzie will not have to go through so many needle sticks.  As you well know, it can be very tramatizing and hearing her screams makes me heart ache.  Our hematologist is hoping that as she grows older she will grow out of the need for transfusions.  We're keeping our fingers crossed!

Dear mommyofizzie

First of all welcome to the site

When a baby is classified of being intermedia, this means that he/she can maintain a Hb of 6 or 7 without transfusion. The decesion to transfuse is really very hard to decide in intermedia,it is hard to decide even by doctors but there are  important things that you should consider if you choose not to transfuse
1- Normal growth for the child
2- No bone deformity
3- Child activity

If you can achieve the above three criterias, usually transfuion is avoided until needed.  But at the same time many hematologist follow the school of always keeping the child above the Hb of 9 to avoid any complications.

So as you can see transfuisng intermedias is a controversial issue but it is always the growth that weighs  which side you will go.

Also it is proven that  fetal HB inducers are of a very important usage for intermedias and are usually tried before decidng to transfuse. This ranges from natural ones (wheat grass, resveratrol, carao,aloe vera....) to drugs like hydroxurea. Many intermedias respond to these inducers and transfusion can be eliminated.

As for the port-a cath-, many of our uembers had expierence with it and i advice you to read about these post by writing the word ''port cath'' in the search of the site so you get to know previous expierence, but the important thing is to know what are the precautions to avoid infections caused by the port cath.

I hope that you can read as much as you can especially in the section of intermedias and please feel free to ask what everyou want.

One more thing i want to comment on,

Our hematologist is hoping that as she grows older she will grow out of the need for transfusions

Usually this does not happen because the body becomes dependent on transfusions and also the more you grow the more you need to have a higher HB

manal

Zahra,

To tell you the truth i have no idea if this is related to Osteocare or not but actualy i didn't observe this on my son.  As for Kiddie Pharmaton, i used to give it to my son too,my only concern about it is that it has no minerals in it, that is why i shifted toYummi birds.

Zahra,I really don't get your other question, are you asking about the dose??

manal

Zahra,

I don't know how much Vitamin E does your multivitamin contain? 100 IU would be enough i think for your son,important thing is that it should be from natural source and not a synthetic one,natural would say d-alpha tocopherol on it and synthetic would say dl-alpha tocopherol on it.

Hope this helps.

Zaini.

How is Izie doing now mam?