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Sisters battling cystic fibrosis told only one can have drug to fight it

Two sisters battling cystic fibrosis have been told that only one of them can have a breakthrough £100,000-a-year drug.

Kirsty Young, 29, is one of the 1,000 cystic fibrosis sufferers with serious lung damage in Britain who have been given the drug free.

But her sister Shona, 24, has been told by health bosses that she isn’t sick enough to be given Symkevi, which reduces the build-up of sticky mucus in the lungs and digestive system.

Cystic fibrosis is a genetic disorder that affects mostly the lungs, but also the pancreas, liver, kidneys, and intestine.

Patients have an average life expectancy of 37 years.

Manufactuers Vertex are making the £100,000-a-year treatment available to 1,000 sufferers across Britain under a compassionate access scheme.

Kirsty, a music teacher from Carron, near Falkirk, qualified for the free drug after her lung capacity dropped to 40 per cent.

She said: ‘I am grateful to receive the drug but distressed that Shona is not getting it.’

Shona – whose lung capacity currently veers between 60 per cent and 86 per cent – fears she will not receive the drug until her lungs have deteriorated to a similar level.

Kirsty said: ‘We have to watch Shona cope with serious lung infections caused by cystic fibrosis.

‘I began to deteriorate after 24 and fear this may happen to her too. It took me years to qualify to become a teacher – seven years to complete my teachers’ probationary year.

‘I was in and out of hospital with serious lung infections.

‘I appreciate all the dilemmas faced, the huge amount invested in making the drug breakthrough and the cost to the NHS.

‘But I will do everything I can to support Shona and others fighting to get a drug which stops their lungs being destroyed.’

Shona, who works as a classroom assistant, said: ‘My lungs and digestive system are affected and I desperately need the drug now. I am young and would like to get married and have a family.

‘My hospital consultants were so hopeful that the drug would be approved by the Scottish Medicines Consortium that they said I should be getting it by next month.

‘I was hoping to one of the first in Scotland but the drug was not approved.’

Since starting her job, Shona has been forced to move from primary ones to primary fives to spare her lungs.

She said: ‘I was catching everything that the little ones get when they start school and needed to move to an older group.

‘My doctors advised me to do it to spare my lungs and my school have been happy to make it happen.’

Vertex said: ‘We provide free medicines to patients in the UK who are the most seriously ill, based on objective clinical criteria.

‘Over 65 patients have got Vertex medicines through a peer approved clinical system with the Scottish Government this year.’

The Scottish Government said: ‘We understand the great disappointment that people with cystic fibrosis, together with their parents, families and friends, will feel about the Scottish Medicines Consortium’s (SMC) decision not to recommend Orkambi and Symkevi for routine availability on the NHS in Scotland.

‘We have been working with the SMC, the manufacturer and with other interested parties to develop the principles of a solution that can make possible the widest availability of these medicines, wherever clinically appropriate.

‘This work continues now, as a matter of urgency.

‘The government hopes that all parties continue to work together to achieve a positive outcome for all the children and adults with cystic fibrosis in Scotland as quickly as is practicable.’

NHS England has been negotiating with Vertex for years now over the drugs, with the manufacturer refusing a £500 million offer for a five year period this year.

The company has argued that it is not cost effective and have claimed they have invested £9.65 billion in the research and development of cystic fibrosis drugs.

However, experts estimate the firm will make more than £17 billion in profit over the lifetime of Orkambi and an earlier drug called Kalydeco.

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