Devoted grandmother passes away just days after she begged HSE 'please don't put a price on my life'
A devoted grandmother passed away just days after she urged the HSE to make a "miracle" drug available that she claimed would give her a chance at a healthier life.
Marion Kelly (53) suffered from Alpha-1, a genetic condition that causes attacks lung and liver problems.
She was one of 21 people here who were taking the drug, Respreeza, as part of a trial and just last week she spoke to Independent.ie about how she feared the HSE's decision not to make the drug available to her could rob her of the chance to watch her grandchildren grow up.
Marion, who was from Nenagh in Co Tipperary, tragically passed away in Limerick University Hospital on Friday, surrounded by her devastated family.
She is survived by her daughter, son-in-law, granddaughters, father and stepmother Lelia, sisters, brothers and extended family and her many friends.
Marion told Independent.ie that she was born with Alpha-1 but it didn't surface until she was in her 30s, when she started to feel breathless.
Her condition went rapidly downhill and she had to give up her job as a hairdresser.
Marion said she was offered a new lease of life 11 years ago when she was chosen to participate in the trail of Repreeza.
She explained: "The trial was given to 21 of us and we were able to take it for as long as the trial continued.
"It was fantastic, I could go back to things like shopping independently, gardening and doing things for myself.
"I had a new lease of life, it felt like a miracle.
"Before that I would have been in and out of hospital six or seven times a year but with Respreeza I think I was in hospital just once over three years so I felt like I had my freedom back."
The group were informed 22 months ago that the trial was coming to an end but had to wait month-to-month to find out when.
In September the drug trial was finished but a further supply has been made available to those on trial but there has been confusion over who will administer it recently.
"It's licenced in other countries and while the HSE and Health Minister might claim it's not worth it then how did I feel so much better for those eleven years?," Marion said.
"I've only been off Respreeza for six weeks and already I can feel a massive difference in my health.
"They're putting a price on our lives, if we don't get this drug we will die younger, they are denying us the chance to have a fairly healthy life."
Heartbreakingly, Marion said she wanted to watch her granddaughter Mia (six months) grow up.
She said: "I'm already having trouble lifting Mia, I've always pushed myself to do things and not let this hold me back but one night it really upset me that I'm only 53 and there's so many things I can't do."
She continued to say: "Every chest infection takes away a little piece of my lungs that won't recover.
"When Orkambi (a drug for people with Cystic Fibrosis) was approved earlier this year it did give me hope, I'm not giving up this fight.
"I feel there's a price on my head but I think my life is worth more than €80,000."
Previously the National Centre of Pharmacoeconomics said the drug "wasn't value for money" after it carried out a drug assessment.
Professor Michael Barry, the centre’s clinical director, said that the decision not to reimburse the drug was not solely based on its cost however, saying the centre primarily found the evidence from the clinical trial was not strong enough.
"While there was a benefit in terms of lung density on CT scanning, unfortunately clinical outcomes in terms of lung function tests, quality of life, exacerbations and indeed survival; there was no impact of this drug on any of those parameters," he said.
"That in conjunction with the high cost of the drug - it can be anything over €80,000 per patient per year, we felt that it wasn't value for money."
A HSE spokeswoman said: "The HSE has agreed to a temporary funding arrangement of six months for the administration of Respreeza to those affected by manufacturer’s recent decision to modify its compassionate access programme.
"This decision is to allow the patients remain on the drug while they transition to an alternative care pathway."
Prior to her death Marion had set campaigned for Resprezza to be made available here, for more information please visit here.