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[vampirebaby]

Hello all,

Hope you can help me. My 9 year old daughter has been on desferal since 2015. She is very compliant so we do the infusion 7 days a week with an occasional 1 day break every few months. She’s on a dose of 800mg & weighs 20kg.

Recently she’s had high frequency hearing loss so we’ve been advised to stop desferal until her next hearing test in 3 weeks.

I’ve read in your blogs that quite a few of you have experienced this yourself or with your children. The question is if we were to continue with desferal after 3 weeks, would it lead to further hearing loss and eventually complete deafness? Would it help to perhaps infuse her 5 days a week instead of 7? Her ferritin has been hovering around 1400 for the last 12 months & last ferri-scan showed iron-free organs.

Awaiting your replies.

Many thanks,
Sameet

[Andy Battaglia]

You would be best to take a long break from desferal. I have heard doctors say that patients are better off if they do not use the same chelation drugs for years, due to the cumulative side effects (hearing loss being one of them) that patients can experience over many years. It may be time to try one of the Exjade drugs. I would not rule out desferal for the future, but a good break is called for.

[vampirebaby]

Andi, Eva was on exjade for 2 years. It ended up giving her a duodenal ulcer which perforated. Her LFTs were completely out & excretion of calcium increased. She just didn’t agree with exjade. So it’s no longer an option for her.

The only other option is deferriprone.

Would it no longer help give her a months break from desferal and then resume on a lower dose?

Do you know what the maximum dose of desferal is for kids?

[Andy Battaglia]

The standard dose is 20–40 mg/kg/day for children. That should only be exceeded by a doctor's orders.

I would suggest trying a low dose of deferiprone to see if it can be tolerated.

[Andy Battaglia]

If the hearing test improves after one month, desferal can be tried again.

[vampirebaby]

Unfortunately the repeat test suggests permanent hearing loss. Her Ferriscan results came back very reassuring with no organ iron loading so we’re taking a break from desferal for 2 months & restart on half the dose (400mg instead of 800mg). Currently Eva’s on no chelation.

We’re also reconsidering the option of bmt (haploidentical transplant) given her history with chelators.