MAIRA

Hello Everyone
I m Irfan from karachi Pakistan.I m quite new to this site and i don't know much how to use this site but i m trying to learn.Well i have two baby girls Marium six years old and Maira,three years old.Maira is a Thalassaemia Major patient.When she was nine months old she was diagonesd by this disorder.It was really shocking for us and we started her blood transfusion in Hussaini Blood Bank Karachi,Pakistan.The Haematologist and docotors are really co operative of Hussaini Blood Bank.Every month we go there and get Maira's blood transfusion.Maira has started using the infusion pump four times a week.We use Desferal for iron chelation but its really painful.I have heard that a medicine ''Exjade Deferasirox''has been introduced by FDA and Novartis is the manufacturer of this medicine.I need to ask that where can i find this medicine as here in our country its not available.I really need it.Secondly i intend BMT of Maira and she has a real sister Marium as a donor,so shall i go for BMT??What do u friends suggest??
Irfan

Asalam-o-Alaikum,

There are many posts on this site regarding Exjade. Sadly it isn't available in Pakistan. I heard that Novartis were giving Exjade in Rawaklpindi when it was in the trials(you get the medicine from USA and you had to send them the results regularly), but now the Novartis branch of Rawalpindi/Islamabad has ended the trials. You can contact the Head office of Novartis in Karachi and ask them to introduce it to Pakistan (and also make sure they do it in Rawalpindi/Islamabad too and not just in Karachi ) as the Karachi branch is the Head Quarters for the whole country.

Desferal is really a painfull experience and I hope Exjade arrives to Pakistan soon.

Secondly i intend BMT of Maira and she has a real sister Marium as a donor,so shall i go for BMT??What do u friends suggest??

Hello Irfan,

Welcome to the site. You can find a lot of information here and can ask any questions here. There are lot of patients/friends who can share their experience.

Bone Marrow Transplant is an Cure option only if the donor and recipient's HLA(Human Leukocyte Antigen) match. There have been successful bone marrow transplants done and there are more chances of success, if the patient is younger. First, you should get your daughter's HLA type checked and see if they match. Maximum chance of HLA match is with the patients siblings(in your case - Marium - your younger daughter).

Also, you can read the stories posted here Successful BMT story

Also, there have been successful BMT (HLA matched with parents) - so in case you want to explore that option, you can get your and your wife's HLA match up done.

We recently got my niece's HLA type checked but her's did NOT match with her brother or either of the parents - in that case BMT with related donor is NOT possible. There have been some success stories of BMT with un-related donor (but it is difficult to find a match with unrelated donors)

Also, note BMT is a risky (Transplant needs the WBC count low so the procedure destroys the patients immune system) option and you should get all information before you go for BMT. Patients/Friends here might be able to share experience with stories they have heard about BMT.

-Narendra

Asalam-o-Alaikum,

There are many posts on this site regarding Exjade. Sadly it isn't available in Pakistan. I heard that Novartis were giving Exjade in Rawaklpindi when it was in the trials(you get the medicine from USA and you had to send them the results regularly), but now the Novartis branch of Rawalpindi/Islamabad has ended the trials. You can contact the Head office of Novartis in Karachi and ask them to introduce it to Pakistan (and also make sure they do it in Rawalpindi/Islamabad too and not just in Karachi ) as the Karachi branch is the Head Quarters for the whole country.

Desferal is really a painfull experience and I hope Exjade arrives to Pakistan soon.

Assalam o Alaikum  Mr Sajid Rauf
How are u sir,i hope u are fine and having a nice time.First of all thank you very much for taking interest in my case and thanx for your informative reply.May God bless u.I hope u send your experiences to me time to time.I have one more question and i hope u reply it.Well one of the upper teeths of my baby Maira is going to gray or black.At the moment its looking gray and i think it would turn black.Why its happeneing?Hope u reply it,thanx.

Hello Irfan,

Welcome to the site. You can find a lot of information here and can ask any questions here. There are lot of patients/friends who can share their experience.

Bone Marrow Transplant is an Cure option only if the donor and recipient's HLA(Human Leukocyte Antigen) match. There have been successful bone marrow transplants done and there are more chances of success, if the patient is younger. First, you should get your daughter's HLA type checked and see if they match. Maximum chance of HLA match is with the patients siblings(in your case - Marium - your younger daughter).

Also, you can read the stories posted here Successful BMT story

Also, there have been successful BMT (HLA matched with parents) - so in case you want to explore that option, you can get your and your wife's HLA match up done.

We recently got my niece's HLA type checked but her's did NOT match with her brother or either of the parents - in that case BMT with related donor is NOT possible. There have been some success stories of BMT with un-related donor (but it is difficult to find a match with unrelated donors)

Also, note BMT is a risky (Transplant needs the WBC count low so the procedure destroys the patients immune system) option and you should get all information before you go for BMT. Patients/Friends here might be able to share experience with stories they have heard about BMT.

-Narendra

Hello Narendra
How are u?I hope u are fine.I m quite glad to see your reply and your interest towards my daughter's case and i hope u inform me more time to time as i possess less information about Thalassaemia Major.Well Narendra i m keen to get my daughter's (Maira)HLA TYPING soon but just tell me that in which country i should go for BMT as here in our country Pakistan the results are not most successful then India or Italy or America or other countries.I have heard that India and Italy have quite good an dsuccessful results of BMT.Can u please suggest me more about it.Secondly what would be the expenses as per your calculations?I really intend for BMT but a bit hesitant too as its really hard task.Please update me.Thanx.
Irfan

Well one of the upper teeths of my baby Maira is going to gray or black.At the moment its looking gray and i think it would turn black.Why its happeneing?Hope u reply it,thanx.

A-o-A,
         Well, I am really not sure that the rottening of theeth is related to Thal. I too had my milk theeth(whatever the first batch of theeth are called) rotten, but I am sure that way because I loved sweets(I still do even now!). So, I had them removed as they were going to fall off eventually.

I hope that the people of the forum will add more to this topic!

i m keen to get my daughter's (Maira)HLA TYPING soon but just tell me that in which country i should go for BMT as here in our country Pakistan the results are not most successful then India or Italy or America or other countries.I have heard that India and Italy have quite good an dsuccessful results of BMT.Can u please suggest me more about it

I understand your concern regarding the success ratio. In India, there is quite a few number of cases which have been successful. I am NOT sure about Italy or America. Also, in India I have heard success of BMT from CMC(Christian Medical College) Hosptial in Vellore, Tamilnadu. They I think have a success ratio of around 73%. But, that again depends on the patients age. According to what i have learnt and readers do correct me if I am wrong - The younger the patient the better the chances are of success. For Maria - the chances of success might be more. The cases in Pakistan where you have heard about failures, did you check if they were older patients or younger patients? BMT is more risker in older patients.

Secondly what would be the expenses as per your calculations?

The approx. cost they sent me was around Rs. 10,00,000

These figures are approximate and depend on :

The disease
The age and weight of the patient
The post transplant complications
An uncomplicated transplant in a 12kg child can cost Rs.5 lakhs while serious complications (infections or graft versus host disease) after transplant can increase the case to cost as much as Rs.20 lakhs, mainly because of costs related to prolonged hospitalization, immunosuppressive drugs (ATG/ALG), antibiotics, transfusions and parenteral nutrition

As you can see above, the cost varies on the transplant and can range from Rs. 5 lakh to Rs. 20 lakh. and depends on how well the transplant goes.

But, the first thing you might want to think of is get Maira and Marium's HLA match up. This is the first step for the BMT option. If that does NOT match, you can also get your wife and your HLA type and see if it matches with Maria's HLA type. Get the HLA match done in Pakistan and then think of where you might want to get the BMT done. Friends from Pakistan can lead you to where the HLA match up can be done or ask the center where Maria gets Blood transfusions and they will be able to guide you.

Just an FYI, the HLA typing test costs around Rs. 7350

-Narendra

Hi Narendara.
Again thanx for your reply.Did u mention the cost of BMT 10,00,000 in India?The charges of test of HLA here in Karachi is about twenty three thousand and the donors tests costs 2500.So i would pay about thirty thousand rupees for HLA test of Maira and Marium and we both husband and wife as donors.The tests will be done in Pakistan's most famous and renowned Aga Khan Laboratory,Karachi.I will soon inform u about the test reports.Thanx.
Irfan

Hi Irfee!  Welcome to the group!  It's good to have you here.