Thalassemia Patients and Friends
Discussion Forums => Living with Thalassemia => Topic started by: Canadian_Family on November 10, 2007, 01:00:37 AM
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Hello Friends
This is an urgent message, we recieved a call from the hospital today regarding the results of hearing test. Somehow my daughter failed the high point test (whatever that means). We are advised to stop Desferal for one month and redo the test after month. In accurate words her high point came low than previuosly recorded.
Doctors are not 100% sure if this is Desferal related issue or its because she had cold or she is naturally like that.
Has anyone experienced this situation before. Comments are welcome.
PS. We will not be able to access the internet starting November 11 to December 7.
Thank You.
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Hearing loss is a known long term side effect of desferal. If it is found to be related to desferal use, it does not necessarily mean that she needs to discontinue desferal, but 4 weeks off desferal is the standard treatment and diagnosis tool. The doctor is referring to her ability to hear high frequency sounds as being below normal.
From http://www.jpho-online.com/pt/re/jpho/abstract.00043426-200512000-00004.htm;jsessionid=H1Fh814XfQRVf7hDgL2Ldb0DGwn2pGnjTtYDb3vLs4LHDTvw4vmb!1821113646!181195629!8091!-1
Auditory and Visual Toxicity During Deferoxamine Therapy in Transfusion-Dependent Patients.
Original Article
Journal of Pediatric Hematology/Oncology. 27(12):651-653, December 2005.
Chen, Shu-Huey MD *; Liang, Der-Cherng MD *; Lin, Hung-Ching MD, MSc +; Cheng, Shu-Yun BSc +; Chen, Lee-Jen MD ++; Liu, Hsi-Che MD *
Abstract:
Summary: Deferoxamine is a chelating agent that has extended the life expectancy of patients with thalassemia. In the 1980s, many investigators reported otologic and visual toxicity caused by deferoxamine. In July 1999 and 2 years later, the authors performed audiologic and ophthalmologic assessments in 30 transfusion-dependent patients receiving deferoxamine therapy (40-50 mg/kg per dose, subcutaneously for 8-10 hours, 4-7 days per week). In 1999, six patients (20%) had deferoxamine-related hearing impairment (>25 dB), all at high frequencies. Because the authors believed the benefits of chelation therapy outweighed the risk of ototoxicity, the dose of deferoxamine was not reduced. Two years later, the hearing impairment had not progressed in any of the patients. There was no association between ototoxicity and ferritin level. No patients had abnormalities of visual acuity or funduscopy in either 1999 or 2001. Based on this experience, deferoxamine at doses lower than 50 mg/kg/d was safe for the eyes and slightly toxic to the ears. The ototoxicity probably relates to individual susceptibility. Regular monitoring of auditory function and close follow-up of abnormal findings are recommended. According to this limited experience, reducing the dose or withdrawing deferoxamine might not be necessary if the hearing loss is stable in the face of ferritin levels above 2,000 ng/mL. Because of the relatively small patient numbers, more data are needed to confirm these conclusions.
(C) 2005 Lippincott Williams & Wilkins, Inc.
After one month, the doctor will have a better idea of whether this is desferal related and may decide to temporarily reduce the dosage. It is possibly related to sinus problems. It has been a terrible year for allergies and sinus illnesses, so if she's been bothered by sinus, her hearing may be slightly affected. We've all had that plugged up feeling with colds where our hearing is slightly muffled, so let's hope that is all this is.
Don't worry too much on your holiday. This is a relatively common occurrence with desferal users and most of the time it is readily reversed. I hope all goes well on your trip. :smiley
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Hi C.F,
I really hope that it is cold related as I too am unable to enjoy the high notes in music when I have a severe cold and it sounds like everyone is talking through a telephone :biggrin
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Hi Canadian Family,
I am sorry to hear about your daughter's hearing test and i hope everything will turn well,Good luck.
ZAINI.
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Thank you everyone for the prompt reply.
Andy,
The article was very helpful, as I understand the hearing loss is really an impairment at high frequency and not a total loss of hearing. The impairment can be reversed or not progressed. On that note, is there any seriousness attached to it and what is possible treatment if there is any.
Thank You.
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Hope everything goes well and turns to be nothing serious
Good luck
manal
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??? actually i hear a bout desferal cause some problem in the hearing :huh but i used to give my son high dose of desferal to reduce the lever fibroses i remember i gave him desferal everyday for 20 houres just relax for 4 houres that all and he still his hearing better than the rabit ... i hope it's kind of comon cold and it will go AMEEN ..
khalifa
state of kuwait
one for all and all for one
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Thank you all for the kind words.
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Usually any hearing loss is readily reversible by stopping desferal for awhile. When there is fear of long term damage, it may result in the use of a different chelator. This should be considered by all thals over time. The consensus among the top doctors is that thal patients will do better if they use more than one chelator in their lifetimes, as this will help minimize the long term side effects of these drugs.
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Hello All,
We are back from the vacations and my daughter is off the Desferal for four weeks now. I am going for a hearing test today. Will update soon.
Regards
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Hi,
Hope you had a great vacation,wishing you luck for the hearing test. :goodluck
ZAINI.
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Thank you Zaini.
Freinds,
As it turned out my daughter again failed the test and she is ordered to be off of desferal for another one month.
She is starting the exjade in January, as she is eligible due to her sensitivity attached with desferal. My daughter is four and a half year old and she is the youngest to start the exjade. Doctor did warned us about some kidney problems associated with exjade and they will be running the test in January and going forward to keep them updated.
Lets cross the fingers and see what unfolds next.
Regards
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Exjade is being used with children as young as 2 in the US, so it does have some record of success with that age group. She will need to be checked regularly to test creatinine levels, which would indicate the problems in the kidneys that sometimes occur using Exjade. However, experience has shown that most of the patients having kidney problems with Exjade had some pre-exisiting kidney problem. At her young age, it is unlikely that she has had any prior kidney problems. As long as she is regularly monitored, she should be fine on Exjade. I am sure she will enjoy being liberated from the pump.
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Hi C.F!
Keep hopes high! I hope she's better off with Exjade and avoid Desferal totally. This will make her hearing better if the culprit is Desferal. Plus she will have face no more needles.
All the best.
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Thank you.
Andy,
It is nice to know that children as young as 2 have used Exjade. Indeed it is comforting for her and us to be liberated from pump.
Sajid,
I took your point "if the culprit is desferal". So far all signs point towards desferal. In early March her desferal dosage was increased to match her age. The hearing results were normal in March, October she started complaining about her ear "not working". Since November she has successively failed two test.
Thanks
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Hi Canadian Family,
I'd like to direct you to the post I did about the most recent studies concerning Exjade use in children. You can read it at
http://www.thalassemiapatientsandfriends.com/index.php?topic=1373.msg11148#msg11148
I would also like to suggest a bit of advice. Many patients get short term side effects from using Exjade. Chief among these are a skin rash and stomach discomfort. To minimize these side effects, it is advisable to start Exjade with a low dose and slowly work up to the maximum dosage. Don't worry about short term increases in ferritin as this is normal as a patient is working up to the high dose. Speak to your doctor about this and see if the doctor is willing to allow your daughter to be slowly introduced to Exjade.
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Thanks for the advice Andy.
We will discuss the dosage with doctor on our next visit.
Regards
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Hi,
Keeping an eye on the hearing test, once again my daughter had failed the test and this time its even worse in the right ear, left seems a little improved.
We have not heard anything from the doctors, the thalassemia nurse was a little concerned. We are not sure what is next.
Regards
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The studies I have seen on desferal and hearing loss show that even after cessation of desferal, a small percentage of patients continue to experience reduced hearing levels. None of these studies reported what happened long term though. Normally, the hearing returns to normal. In cases where desferal was not stopped, the hearing loss remained but did not progress. Most doctors agree that the benefit of desferal outweighs the limited damage to the hearing. However, other possibilities should also be considered and further examination may be helpful. I ran across an interesting recent case that found the hearing loss had nothing to do with desferal but rather, with bone marrow expansion in the ear.
From http://journals.cambridge.org/action/displayAbstract;jsessionid=DB571C8A171C750429D540B618AB9C8F.tomcat1?fromPage=online&aid=1379332
Objective: The aim of this report was to highlight the fact that hearing loss in thalassaemia patients can be related to marrow expansion affecting the ossicles, resulting in a conductive loss.
Case report: A six-year-old boy with transfusion-dependent beta-thalassaemia developed a unilateral hearing loss shortly after commencing desferrioxamine therapy. Otoxicity was assumed, but the deficit was later found to be of a conductive nature, due to marrow proliferation within the ossicular chain as a consequence of the disease process – a phenomenon previously unreported in the literature.
Conclusion: It is important to elucidate the precise nature of new onset hearing loss in patients receiving iron chelation therapy, in order to avoid unnecessary cessation of much needed medication, on the assumption of ototoxicity.
(Accepted July 16 2007)
This is a very recent report and it raises the question of whether desferal should be automatically assumed to be the cause of hearing loss. The authors of this report believe that desferal may be unnecessarily withdrawn in some of these cases.
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Thanks for the article Andy. I will print and deliver it to the doctor on my next visit.
Regards
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Unfortunately, the fourth hearing test came even worst, something needs to be done I am trying to reach the hematologist to get an opinion to have some procedure done. Will update soon.
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Just finished talking to the doctor, she is trying to get an ENT specialist appointment to clear the fluid from my daughter's ear. The procedure includes clearing the fluid and get understanding of what is going on inside the ear. The appointment date is not finalized yet.
Thanks.
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Best of Luck Canadian_Family
:hugfriend
Dimple
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Canadian Family,
Our prayers are with you :pray
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We had the same issue with our son - after being off of desferal for a number of weeks (I don't remember how long now) - his hearing did return to normal. I'm sure the same will be true for you child. All the best with exjade!
Sharmin
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Hello canadian family,
I know 3 kids who had started exjade when they were two years old. My daughter is now 18 month old and will start exjade when she turns two. Goodluck to your daughter. Hope she passes her next hearing test.