Kira in Baltimore

I would like to start by saying Hello to all of you.  We found out our daughter Kira had Thalassemia Major at birth when they performed the PKU test which is done at birth and then again 2 weeks later.  Since then the information regarding treatment and her health upkeep have been sparse.  We had doctors tell us not to read the stuff online because it most likely would misinform us. For something that is most common in the Anemia area, there is little information and very little support.  I would just like to be able to talk with people who are going through this so that I can help my daughter adjust to changes that she will most definitely will deal with.  We just found out last week that she is suffering from hearing problems that have nothing to do with chelation therapy.  Only when this came to the surface that the doctor informed me that Thal patients often have hearing problems which requires the use of an hearing aid.  Kira has 4 other siblings.  None were a match for marrow transplant.    Out of the other 4 siblings,  2 have trait.  The other 2 are free.  Funny thing is that neither my husband or I had an idea that we had Thalassemia.  He's of Italian decent and I of Irish and American Indian decent.  I look forward to any suggestion you may have in helping keep Kira well.  We had almost lost her twice due to corinary failure.  I welcome you all as an extension of our family.

Hello Denise  and welcome to the site

How old is Kira and which chelator is she using???

In thalassemia, hearing problems are always related to long usage of desferal and not because of having thal itself, especially if the patient is on regular transfusion and mataining a HB of 10 and above.

manal

Denise

I have checked the biography and found out that she is 7 years old, I wonder why has she suffered twice from corinary failure

Did she make T2* to check iron overload in heart?? Or the heart problems are related to something else other than iron overload??

manal

Hi Denise,

I am glad you came here in spite of the beware online sources warnings. You will learn more about thalassemia here than many doctors know. In fact, many doctors from throughout the world access this site for information. Some have even joined, including one doctor from the top bone marrow transplant center on earth. We do our best to guide people here.

The first thing you should do is contact Eileen Scott at CAF. Getting registered with CAF is important as they can direct you in many ways. Eileen can help you find a thal familiar doctor in your area. She will also most likely direct you to one of the US Centers of Excellence for thalassemia care. Her contact info is

Cooley's Anemia Foundation
330 Seventh Avenue, #900
New York, NY 10001
(800)522-7222
Fax: 212-279-5999

Patient Services Manager
Eileen Scott
eileen.s@cooleysanemia.org

I cannot overstress how important it is for your child to be under a treatment program directed by one of these centers. Closest to you is Dr Cohen at Children’s Hospital of Philadelphia. A complete evaluation is necessary as soon as possible. Hearing loss is not common in thalassemia outside of those whose hearing loss is directly caused by chelators. It is well known that desferal can cause hearing loss, but less known is that Exjade has also been shown to cause hearing loss in a small minority of users, so the true cause should be investigated. The difference between the Centers of Excellence and hospitals with no connection to these centers is night and day. All patients in the US should be seen at one of these centers or one of their satellite centers, at least once per year for an evaluation of the patient's progress and revisions to their treatment program, if needed. They design treatment programs specifically for each patient based on the needs of that patient. I can give you mountains of information and advice but nothing I can tell you is as important as call Children’s Hospital of Philadelphia and set up an appointment with Dr Cohen.

Children’s Hospital of Philadelphia
Appointments, consultations and referrals Contact: Marie Martin, 215-590-2197
Philadelphia, Pennsylvania

Director: Alan Cohen, MD

http://www.chop.edu/consumer/jsp/division/meet_the_team.jsp?sid=26685

Physician Referral Service     1-800-TRY CHOP (1-800-879-2467)

The source of your thal gene may be a mystery but thal carriers are found all over the earth and not just in the typically considered areas. It could be that some ancestor long ago traveled as a merchant or sailor and brought back home a wife from the Mediterranean area. We do often hear from people of Northern European descent who are carriers, so it is not quite as unusual as many believe.

I would like to add that there are many parents in this group who have similar stories and experience as you, who will share what they have learned with you about the day to day reality of raising a child with thalassemia. There are things you just won't hear from doctors, because they don't live it. The parents and patients do, and there are many things that only experience teaches. Please feel at home here and ask anything at all. This is a truly unique online site with a wonderful group of people from all over the world. We are here to help.

Thank you for the information you provided Andy.  We have an appointment set up on the 29th of this month with a hearing specialist.  Kira's hearing problem is with the middle part of the ear where the little bones are.  The doctor feels that something else may be the problem since hearing loss due to medicines effect the inner ear.  We are waiting for John Hopkins to contact us on a date for Kira's Fera-scan for the liver.  She's had a liver biospy done and a scan 2 years ago but her levels are still high in her liver.  She has been taking and has only taken Exjade for her chelation therapy.  Since Kira is only 7 and is unable to stay still, doctors want to wait a couple more years before they do the heart scan.  Thank you Manal for your concerns.  Kira's corinary problems were a result of the doctors not knowing exactly how to deal with Kira's condition.  We found better doctors.

Hello Denise,

Welcome on the forum you have come to right place.

I totally agree with everything Andy said,hearing loss is not something directly related to thal,but is a possible side effect of desferal use,but since Kira is not using desferal,i wonder if exjade can do the same damage  only time will tell,as exjade is a fairly new chelator,I am really sorry to hear about coronary problems she faced,i hope ferriprox is approved soon in US,as this is a very protective chelator for heart.

Take care,

Zaini.

Hello Denise
Kira`s hearing problem might not be thal related at all. My daughter who is not a thal had 40% hearing loss due to collection of fluids in the middle ear. At the age of 8 her tonsils were removed and had gromitz inserted in both her ears. Her hearing returned to normal but after 6 months the same problem repeated. She was also prone to ear infections. At the age of 11 the gromitz was repeated and her adenoids were also removed. Today she is 12 and has had no further hearing issues.

maha

Yes Maha, what your daughter suffered is so common these days among children. I know several cases.
One of them was diagnoised when teacher in school pointed out to my friend that her son always scors bad grades in dictation and she believes that he does not hear well and therefore writes wrong spelling. Actually she was right and found that collection of fluids had also caused hearing loss which was not really recognised by the mother

manal

Hello Denise,

Welcome to the forum.  I hope that Kira is doing great:)  You have come to the right place to get current information, friendship and guidance. 

Is Kira on desferal?  Sometimes desferal can cause temporary high frequency hearing loss due to toxicity and this can easily be reversed if desferal is held for a while. 

I look forward to getting to know you and hearing more from you,

Sharmin 

Sharmin

She has been taking and has only taken Exjade for her chelation therapy.

Kira used exjade only for chelation

Take care

manal

Whoops,
Thanks Manal.  I think that exjade has similar toxicity side effects as desferal though.  I wonder if exjade toxicity may have an effect.

Sharmin

The Exjade prescribing instructions mention that less than 1% of patients in trials had experiences hearing loss, so it does happen but supposedly not often.