The mother of two teenagers with Friedreich's Ataxia has said it is “absolutely brilliant news” that the HSE has decided to reimburse a drug that slows the disease’s progression. 

Friedreich's Ataxia is a rare condition that slowly damages muscles, nerves and causes patients to lose their voice. 

It is estimated that there are around 200 people in Ireland with the disease. 

Skyclarys reduced the progression of Friedreich's Ataxia, but at an annual cost of €280,000 per patient, the HSE initially concluded it did not represent value for money for taxpayers. 

However, officials have since changed their assessment after a “substantially improved financial offer” from the company, BioGen, following intense negotiations.  

On Lunchtime Live, Teresa Kane, whose two children have Friedreich's Ataxia, described it as “absolutely brilliant news” for her family. 

“It's going to mean a lot to Ciarán and Emily, anyone else with Friedrich's Ataxia that they now have a chance to slow down the progression and have a better quality of life,” she said.

This morning ahead of the announcement, Ms Kane said she “wasn’t getting her hopes up” and planned to wait until the news was released.

“I wasn't thinking either way,” she said. 

“I was hopeful and hoping they would say yes, but I just wasn't letting it in on me in case it was going to be a no.” 

Friedreich's Ataxia protest outside the Dáil. Picture by: Leah Farrell/RollingNews.ie.

Ms Kane’s son Ciarán was diagnosed with Friedreich's Ataxia in 2019 at the age of nine, while Emily was diagnosed the following year when she was eight-years-old. 

Although they are siblings, Ms Kane said that Ciarán is experiencing a much faster progression of the disease. 

“So, he's affected an awful lot worse than her,” she said. 

“He has scoliosis, he's fully fused from the top of his spine the whole way down to his pelvis.

“His voice has deteriorated; so, I already have banked his voice and his heart is affected.

“He's on a heart tablet the last three years; Emily's heart is affected as well - she's on the heart tablet the same for the last three years.

“The two of them are in the power chairs, but she's still fit to put a bit of pressure on her legs.” 

Ms Kane hopes that once Ciarán is prescribed Skyclarys it will slow the deterioration of his voice and he will retain the ability to speak. 

Main image: Ciarán and Emily. Picture by: Teresa Kane.