WATCH: 'How much are our lives worth': Longfordian battling deadly disease makes HSE plea
Kiara Lynch (42) was diagnosed with Friedreich's ataxia (FA) at the age of just 13
A North Longford woman with a rare, neuromuscular disease, which causes loss of mobility, balance, and coordination, who is campaigning for access to life-changing treatment for her and 200 patients, has asked how much their lives are worth to the HSE?
Kiara Lynch was diagnosed with Friedreich's ataxia (FA) at the age of 13 and for more than 28 years she has been battling what she describes as 'the relentless progression' affecting all of her abilities.
The effects of FA are having a significant adverse impact on her body and Kiara said she has 'cardiac issues, lung issues, bladder issues, pressure sores, and constant pain in my neck and back'.
Kiara said there was a very welcome and much-needed glimmer of hope with this new drug treatment Skyclarys, the first treatment in the world for FA was approved.
Skyclarys or omaveloxolone was first given the green light for use in the US in 2023 and Europe followed less than 12-months later.
However, the 'game-changer' treatment is at the centre of protracted price negotiations between the HSE and the marketing authorisation holder, Biogen (Idec) Ireland Limited.
The cost of the treatment is said to be approximately €280,000 per person and Kiara and the other 200 FA patients said it is unaffordable to them and they have appealed to the Government to fund it.
Kiara, who features in a powerful video featuring seven female FA sufferers and one man, asked, "How much are our lives worth to the HSE?
The HSE has the authority to approve Skyclarys for treatment under the public system, but it has not yet done so, and the application remains under what has been described as 'active consideration'.
There was a major setback when the National Centre for Pharmacoeconomics (NCPE), which provides independent assessments for the HSE in relation to whether new drugs are cost-effective and should be provided to the public, issued a recommendation and referred to the “cost-effectiveness” of Skyclarys.
However, there are still hopes a deal can be reached and it will eventually be approved.
The first woman in the video says 'Friedreich's ataxia or FA is a rare progressive neuromuscular condition with effects found in 200 people in Ireland'.
A second female sufferer says 'FA causes nerve damage, muscle weakness and mobility issues' and it also results in 'cardiac issues, scoliosis, diabetes and early death'.
A third woman says "Skyclarys is the only approved treatment and It was shown in clinical trials to slow progression.
She adds: "We all know this drug is not a cure but this drug has the power to change our lives and preserve what we still have."
A fourth female patients says there is a 'problem' and they are still waiting for the HSE to cover the cost of Skyclarys in Ireland before adding, "We need the HSE to reimburse Skyclarys now."
A fifth female FA sufferer says "without reimbursements Skyclarys will cost patients €280,000 a year and "this sum is unaffordable for the average person but not for the government".
A sixth woman says "FA does not wait and neither can we", while another patients states, "We need to slow progression and preserve the ability we still have."
The FA patients and campaigners said they need public support. "Please share this video to show your solidarity with Ireland's FA community. Thank you."