‘How can you put a price on our daughter’s life?’ - Eight-month-old’s future in the balance in NHS drug pricing row
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To look at little Esme Ives, smiling and inquisitive, you would not anticipate the challenging battle the bubbly eight-month-old has ahead of her.
Like one in every 2,500 babies born in the UK, Esme has cystic fibrosis (CF) - one of the country's most common life-threatening diseases.
But a price has been put on her life as the NHS is currently locked in a fierce funding battle with a major pharmaceutical company over the cost of a life-changing drug.
For parents Claire Wright and Mark Ives, of Bracken Drive, Attleborough, the first hint of something being wrong was when they were still at the Norfolk and Norwich University Hospital after Esme was born in August last year.
Medics noticed problems with Esme's breathing and whisked her away to neonatal intensive care (NICU) while Claire, 33, who had a planned c-section, was left waiting in recovery.
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Initially, it was thought Esme might have digestive problems as she had not passed her meconium (the first stool passed by a baby).
But when the couple were asked to see doctors - and Mark, a horticultural agent, was pulled home from a trade show in Birmingham - they knew it was more serious.
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Claire, a teacher at Thomas Bullock Primary Academy in Shipdham , said: 'We got put in a room, so it's not around the bed so no one else hears. That's where he said he was 99pc sure [it was CF], he seemed quite upset in a way.'
Tests later confirmed Esme did have CF and for Claire the most difficult part was accepting that could mean Esme's life was cut short.
She said: 'I may outlive my daughter, everybody says it will be fine but you have it in the back of your mind.'
The life expectancy for half of all CF patients in the UK was estimated to be 47 years in 2013, but babies born today can expect to live longer because treatment is improving.
But she was also wracked with guilt, as neither she nor Mark had known they were carriers of the faulty CF gene, and they already had one healthy daughter Willow, now two.
She said: 'I did cry, it was that difficult thing of knowing deep down but having someone tell you you're carriers, you do get that guilt in a way, that we've given it to her.'
For the family, the diagnosis opened them up to a world they did not know existed.
Claire said: 'Although I had heard about it people think it's only the lungs. But some will get diabetes and other things, this is a lot bigger than I thought.'
And it also meant altering life plans.
Already they think about saving for medication, which will not be free when Esme turns 18.
And they said they would scrimp and save to enjoy family holidays together instead of putting them off.
The couple had planned to get married eventually, but they brought the wedding forward to August this year to ensure Esme will be there.
Claire said: 'My head knows nothing is going to happen in the next year but I would not want to do it without her.'
She added: 'We do make the most of what we can, we try to do as much as we can with her.
'Willow doesn't understand it but she knows. And it will be about trying to explain it to her when she's old enough to understand because of the whole life expectancy stuff, to cherish every moment with her sister without being dramatic.'
The family hope that as Esme grows, treatment will improve, and a major step towards that would be a drug called Orkambi being made available on the NHS.
Currently it is not offered because it is not considered cost-effective, but has been shown in clinical trials to improve lung function and respiratory symptoms.
The drug costs £104,000 per patient every year.
But campaigners argue the cost is offset with reducing expensive hospital admissions, where CF patients must have their own rooms and specialist care.
If available, Esme could start on the medication when she turns six.
Mark, 30, said: 'We know we are lucky [that Orkambi would help Esme], it would stop her lung damage, it would give her a life, it will stop her taking so much medication.'
Claire added: 'Although it costs a lot, it's my daughter's life.'
What is cystic fibrosis?
Cystic fibrosis (CF) is caused by a defective gene, and one in 25 people carry the faulty DNA.
When two carriers have a child, there is a one in four chance they will have CF.
The condition affects more than 10,400 people in the UK.
The faulty CF gene controls the movement of salt and water in and out of cells, which means those with CF have a build-up of mucus in the lungs, digestive system, and other organs.
Two people with the condition should never meet face-to-face because their lungs carry specific bugs which could cause a serious infection in someone else also affected. This means at CF clinics strict segregation is in place.
People with CF often need to take more than 50 tablets a day to help digest food and keep respiratory symptoms in check, as well as undergoing daily physiotherapy, antibiotics, and taking enzyme tablets with food.
'Lots of work to do' before pricing agreement
The fight over Orkambi is not the first between the cash-strapped health service and pharmaceutical firms - and will not be the last.
Health ministers Steve Brine and Lord O'Shaughnessy have urged Vertex to drop the price of Orkambi and said 'time was of the essence' for CF patients.
They asked Vertex to provide data showing their proposal was cost effective.
The first meeting between the NHS and Vertex was on April 25.
A Vertex spokesman said: 'Both parties recognise there is still some way to go to reach an agreement and Vertex is committed to working together to achieve this.
'We share the cystic fibrosis community's sense of urgency and have agreed to meet again in the coming weeks. There's lots of work to do on both sides ahead of this to progress discussions as quickly as possible.'