FERROPROX

HELLO ALL
Can anybody guide me about the tablet ''Ferriprox'' as our physician had suggested me to give this tablet to my daughter Maira........but a guy told me that Ferriprox can make complications in my daughter.Is it right???Who will guide me about ''Ferriprox'' please???
Irfee

Hello Irfan,

Welcome back to the website. Did you get the HLA match up done for your daughter?

Anyway, Ferriprox(also known as L1 or Kelfer) is a drug which is widely used in Asia for Iron Chelation.

Adverse Effect of L1
The most serious unwanted effect of L1 is a reduction in the number of white blood cells, particularly neutrophils, which play an important role in protecting the body against infection.

A moderate reduction in the level of neutrophils (500-1500/mm3) is known as neutropenia, while a severe
reduction (<500/mm3) is known as agranulocytosis.

According to a number of studies assessing the use of L1, neutropenia is more common, while agranulocytosis occurs in a significantly smaller number of cases.

However, both can be reversed if patients stop using deferiprone. It is therefore essential that any patient
using L1 is assessed for total white cell count and the number and percentage of different types of white cells (differential white cell count) at least every 2-3 weeks. Patients should be checked more frequently if they show any sign of infection. At the first sign of fever, sore throat or any other indication of infection, the patient should immediately stop using deferiprone, a full blood check should be carried out and any course of antibiotics prescribed completed. In most cases, the white cell count returns to normal and deferiprone therapy can be started again. However, it is not advisable to restart treatment with L1 until the total white cell count is equal to or greater than 3,000/mm3, the total number of neutrophils
equal to or greater than 1,000/mm3 and the platelet number equal to or greater than 100,000/mm3.


Other side effects of deferiprone
A number of patients using L1 experience pain and swelling of the joints - usually in the knees, ankles,
elbows, hips and lower back, as well as stiffness and difficulties in movement.

Gastrointestinal problems such as nausea, dizziness, vomiting, diarrhoea and pain in the abdomen occur
in some patients using L1. Symptoms are usually mild and may not require treatment. Drugs (antacids,
antiemetics) will generally reduce symptoms. In addition, taking L1 with food may help reduce nausea

I think your doctor should be able to explain all this in details before you start the medicine. Also, what is her Ferritin level?

Hi Irfee,

Ferriprox is used by many people around the world and is a good chelator, performing better than desferal at removing iron from the heart. 5-8% of patients who use ferriprox will develop neutropenia or a loss of white cells. Often this is temporary and may be related to infections in the body also. About 1 % of patients will get the more serious agranulocytosis and will have to stop using it permanently.

The complete text of the updated warning information for ferrirpox is posted at

http://www.thalassemiapatientsandfriends.com/index.php?topic=644.0

This information now recommends weekly testing for white blood cells. It should never be used except under a doctor's supervision, as is the case with all chelating drugs.

If you decide to try ferriprox with your daughter, start on a low dose until you see how well her stomach tolerates the drug. Slowly build up to the normal dose and many of the reactions to it can be avoided, just as it appears to happen with exjade.

HI IRFAN,,

my daughter zainab,six years old,is taking ferriprox,since last 1 year,and it's more easier then desferal ofcourse,as about the side affects,Narendra told u everything,in simple words,just watch her for stomach disorder,and try to have a test for whitecells count every 15 days or so.i hope it won't create any problem .wich u the best.

Zaini.

Hello All
I am so glad to see the replies and comments on ''Ferriprox'',specially i m thankful to Narendra that she noticed my absence and welcomed me again to this site and proviide me useful information about ''Ferriprox''.Andy thanx for your literature too........and my country fellow Zaini...who has the same disorder like my daughter Maira.Currently the ferritine level of Maira is about 1700 and we do not find any physical reaction of this tablet except she has very offesnive smell in her stool. Narendra,I successfuly got my daughter's HLA typing and my other daugther, 7 years old, is a perfect match but after the test i m silent now as to take the risk to go to India or Italia is really hard for me.I m just trusting on transfusion and desferal and Ferriprox.Let's see what happens in future.Thanx.
Irfee

Hello Irfan,

You are right. BMT is tough decision. There are lot of things to consider before going for a BMT, but as of date, that is the best option for Cure. What is Maria's age? If she is young, there are less chances of complications, but again a transplant is definately risky and it brings fears in our minds, so I know what you are saying

Respected Narendra,thanx for your concern about my case.At the moment my daughter is four years old and a week back i got a chance to visit an Italian doctor (I forgot his name) here in our city karachi.He scrutinised my daughter and suggested me for BMT here in Karachi but i really can not take this decision for BMT,just trusting on trnasfusion......thanx

Irfee

The decision of BMT is indeed a difficult one. It's a risky procedure but you would have to consider it against the life long transfusion and the painful iron chelation experience that would reduce the quality of life. It's almost a year after my daughter's BMT and although we're currently encounter GVHD, we did not regret of the BMT.
Best Regards
omega

OH MY GOD,

I ma so happy u found a match,my son was born 2 years before,we were relying heavily on his HLA typing to be matched with my daughter, but unfortunately it was not,i can't tell u disappointed i was, cryin for hours and hours, :'( it was like a blow.though i hv over come it now, ,,and yes it's true that BMT has it's own risks, but i've seen people who selected the option  and there kids are doing fine by now,i can give you the numbers of these people if u wish to, and my personal opinion is that India is a good place for BMT,Dr Mamman Chandi at CMC Vellore is the best.I wish u luck with any decision u make.


TAKE CARE OF MARIA AND UR FAMILY

ZAINI.

India is a good place for BMT,Dr Mamman Chandi at CMC Vellore is the best

I agree with this statement. I have personally met Dr. Chandy and even heard from one of the patients that he shared some money from his pocket and helped a patient for his BMT who was short on finance. Also, there are 2 posts here which show some success stories of BMT in Vellore by Dr. Chandy. You can find them here

http://www.thalassemiapatientsandfriends.com/index.php?topic=119.0 - Successful BMT from a matching Sibling

Dear Omega.Thanx for your comments and reply.Can u please tell me what is "GVHD"please?Where did u go for BMT?

Respected Narendra.....Thanx for your reply and links about BMT but to take the decision is really difficult for me as there are many problems here in life like my small business,my aged parents and furthermore arrangements of finacnce.Its a good sign that Dr Mamman Chandi give great concession for this treatment but at the end i become silent when BMT is mentioned.I will try to send some scanned reports of HLA Typing of Maira and her sister.Would u like to view the reports please?
Irfee.

Zaini......I hope u understand my feelings about BMT as its not my target now.......just pray for my Maira.Being my country felllow,i hope u understand the problems in life.Thanx.
Irfee

Dear Omega.Thanx for your comments and reply.Can u please tell me what is "GVHD"please?Where did u go for BMT?

GVHD is Graft Versus Host Disease. in some patients the donor's cells detect minor differences between the donor and patient and can attack the patient.  This manifests as skin rash, jaundice, diarrhoea and fever. This is controlled with cyclosporine and steroids.  If it is mild it does not cause significant problems but severe graft versus host disease can be fatal

Respected Narendra.....Thanx for your reply and links about BMT but to take the decision is really difficult for me as there are many problems here in life like my small business,my aged parents and furthermore arrangements of finacnce.Its a good sign that Dr Mamman Chandi give great concession for this treatment but at the end i become silent when BMT is mentioned.I will try to send some scanned reports of HLA Typing of Maira and her sister.Would u like to view the reports please?

Please do NOT give me respect. We are just like you. I am just trying to share information and trying to help you out. I know how you might be going through and in the sub continent how things are. It is really difficult. With BMT, the person has to take care of the patient for atleast few months due to the white blood cells count being down and the patient post transplant having high risk of catching viral infections. I assume the reports have a full HLA match up. HLA A, B, DR all matched

Dear Irfee

Our decision for the BMT was made within 2 weeks of notification that a marched Unrelated Donar was found. In fact we knew the risk but have no choice because the younger the patient the better the chance of success of the BMT. My daughter was then 6 years old, and time was running out for us. We have to stop working for about 9 months and we are glad it's finally done. We have done the BMT in a private hospital in Malaysia and the cost of the drugs used represented almost 90% of the total transplant cost. The costs of drugs are going up every now and then, and if our transplant is done today, it would easily cost us 20-30% more! We believe the drug costs would be increasing with time and it could be a major problem for transplant patients in Private Hospital. The alternate is to get the transplant done in a Government Funded hospital, but you might need to wait for a few years before your turn although it cost less in this case. We do not have time and furthermore, we were doing Unrelated BMT, which the Government hospital do not offer, so we have to get it done in a private hospital.

Your are very lucky to get a matched sibling donar and the GVHD should not be as severe as a Unrelated Donar BMT. You should consider the BMT because your child is still very young now and there is a higher chance of a successful BMT.

Thank you.

Omega.

Hi,

it's really bad,i know how conditins could be here in pakistan,but still don't loose hope,maria is only four years right now,u have a lot of time, gather your family talk about it, tell them u'll need their co-operation,i don know if u have bros or sis??if yes i am sure they'll be able to take care of your parents for some time,it's about six months.and about the finances,yes i know it's very tough,the last person i talked about BMT told me the cost was PK RS 1600000,anyways i'll pray for maria ,i wih i could do any more,as i always wanted to go for BMT for my daughter,but unfortunately we didn't had a match,and now it breaks my heart that u have a perfect match and u can't go for it,
i am sorry i am of no help, may ALLAH bless maria.

Zaini.

Irfee,

This is the best time to go for a BMT for your daughter because of her age and the 100% match. If you were to decide on a BMT, the sooner, the better.

However, only the parents can make this decision. Whether you are willing to take the risk, and yes, some patients do die from BMT's, whether or not you can raise the necessary funding, whether it is feasible for you to even do, since you're a small businessman, and as one myself, I fully understand how impossible it can be to take a lengthy absence from your business, whether you can give the adequate after-care necessary, and what I feel is a very important factor, is whether you feel you can see your child go through a BMT, because it is a very harsh process, with severe effects, are all things that must be considered and decided by you. This is a very difficult decision, and sometimes the realities of life make the decision for you. Your decision to continue with her current treatment of transfusion and chelation may be the only choice you can make right now. Please do continue to collect information about BMT so that you can continue to keep it under consideration at a later date.