A TEENAGER with cystic fibrosis made history on Friday when she delivered a desperate plea to an international drug company to “agree a price that the NHS can afford” so that people like her can access orkambi - a drug that could prolong her life.

As she handed over a floral cross to Vertex Pharmaceuticals, to represent all those who have died from the disease, her message was heart wrenching: “No more CF angels.”

Only available on the NHS on compassionate grounds, orkambi currently costs £100,000 per year for each patient and is considered too expensive for general use.

But for 15-year-old Elizabeth Sheahan, it could afford her the time she deserves to enable her to go to Oxford University and fulfil her dreams.

It was an emotional minefield for Elizabeth as, representing the 10,500 people in the UK living with cystic fibrosis, half under the age of 18, she marched through London with protestors from across the country, all wearing yellow and brandishing placards carrying the stark message: ‘Dying for a Decision’.

They were there to support children and young people like Elizabeth who are suffering from cystic fibrosis - a chronic, progressive and frequently fatal genetic disease of the body’s mucus glands which primarily affects the respiratory and digestive systems in children and young adults. On average, individuals with cystic fibrosis have a lifespan of around 30 years.

Elizabeth was the only cystic fibrosis sufferer on the march as people with the disease are not supposed to meet face-to-face due to the risk of cross-infection.

According to East Hampshire Cystic Fibrosis Trust supporter Sue Barnes, the atmosphere on the march was “electric” as they made their way from Paddington station to the London offices of US-based company Vertex Pharmaceuticals, makers of the drug orkambi.

Described as “the second precision medicine” to be licensed in the UK for use by people with cystic fibrosis to slow the decline in lung function, by working on the root cause of the disease and not just the symptoms, orkambi received its European licence in November 2015 and was recognised in June the following year as “an important treatment” by UK Government agency the National Institute of Clinical Excellence. However, it has not been recommended for use by the NHS on grounds of cost-effectiveness, which means it is unavailable to most UK residents suffering with cystic fibrosis.

While acknowledging that the development and testing of a drug like orkambi is expensive, and the lack of long-term data makes it hard for the National Institute of Clinical Excellence to assess its cost-effectiveness, the Cystic Fibrosis Trust still believes that the benefits would far outweigh the cost and supporters are pushing hard for an agreement to be reached which will enable access to this medicine on the NHS.

The Trust has launched a Stopping the Clock campaign, aimed at securing quicker access to life-changing medicines such as orkambi. In January, a petition for access to the drug attracted more than 100,000 signatures in just 10 days, leading to a parliamentary debate at Westminster.

Last Tuesday, Health Secretary Jeremy Hunt added his voice to the campaign by calling on Vertex to be transparent on its pricing for orkambi and agreed to explore avenues for ensuring quicker access to medicine.

On the same day, protesters took their concerns to the doors of Vertex where Rebecca Hunt, vice-president of corporate affairs, appeared visibly moved as she came to talk to the yellow army, telling them that Vertex had put in a new offer to NHS England which it would be discussing at its next meeting on July 4.

Mrs Barnes said: “We, as a community, were grateful for the opportunity to express our anger and frustration face to face.

“We need our young children to have access to orkambi before cystic fibrosis causes irreparable lung damage. We need Vertex and NHS England to understand that some people are just clinging on, hoping that they will get orkambi in time. We don’t have time to wait for them to negotiate or play games, we need orkambi now.”

Marchers went on to petition the NHS Department of Health and Social Care on Victoria Street before moving on to Parliament Square where children performed poignant songs and Elizabeth laid a cross at the feet of the statue of Sir Winston Churchill.

Vertex called on the NHS to “demonstrate flexibility” in the coming negotiations, adding: “We stand alongside the cystic fibrosis community and share their sense of urgency for NHS England to provide access to orkambi in the UK.”

Commenting on behalf of GPs at both Chawton Park Surgery and the Wilson Practice, Dr Andy Fellows said: “As we are all aware the NHS does not have unlimited funds and, much as we would sometimes wish it were different, we have to rely on those who take responsibility for the affordability of all care and treatment under the NHS to reach agreements with the drug companies in the best interests of the population as a whole.”