At 7am, I wake up and, while most people are starting their days, having breakfast and making their way to work, I – like the 10,400 people in the UK with cystic fibrosis – am beginning my daily treatment regime. For me, this involves a concoction of 30 tablets to fuel my morning, each with their own variety of debilitating side-effects, from sickness to dizziness and fatigue. The morning calm is disturbed by my noisy inhalation machine as I breathe in medications, while a positive pressure machine helps me cough up thick, sticky mucus from my lungs, which takes another hour. All in all, it’s a glamorous way to start the day.
And it doesn’t stop there. Every time I eat, I have to take enzymes to help me digest my food, and I take a regular dose of painkillers to help me get through the aches and pains. It’s a constant reminder of my disability and all the limitations it has on me living a “normal” life. After a long and exhausting day, my medication routine is repeated in the evening, with more oral antibiotics and the same gruelling therapy process. I do this every day, morning and evening, in order to try to stay as healthy as possible and in an attempt to maintain my lung function.
Cystic fibrosis is a life-shortening condition caused by a defective gene. It results in passageways in the lungs and digestive system becoming blocked with secretions, causing fatal lung damage and a range of other health problems. The life expectancy is about 47 years for half of those born with cystic fibrosis, compared with the average UK life expectancy of about 80 years.
While cystic fibrosis is known to be a lung disorder, it comes with an array of other health problems, such as pancreatitis and osteoporosis, as well as liver and fertility issues. Despite these daily treatments, people with cystic fibrosis must be regularly admitted to hospital when flare-ups and lung infections occur. All the while, when I’m lying in a hospital bed for weeks, feeling weak and sick from the side-effects of intravenous antibiotics, drug companies are making millions on the back of my suffering, which could be vastly alleviated if I had access to their drugs.
The Scottish Medicines Consortium has recently decided the drugs Orkambi and Symkevi are not cost-effective, following on from the same decision made by the National Institute of Health and Care Excellence in England in 2016. These drugs, developed by the American pharmaceutical company Vertex, aim to prolong the lives of people with cystic fibrosis and have increased some patients’ lung function by up to 20%. They are transformative for those taking them. The core of the dispute lies in the price Vertex is asking of the NHS for these life-changing medications – £104,000 per patient, per year. It has been estimated that the company will make a profit of $21bn (£17bn) over the drugs’ lifetime.
As someone with cystic fibrosis who carries the specific mutation that Orkambi treats, this drug would change my life. So it is distressing to see this large, profitable pharmaceutical company holding so much power to drive up the price of the drug, thus forcing the NHS to put a monetary value on my life. There is no doubt the drug could, and should, be produced more cheaply. Knowing there is a medication that could dramatically improve my life, and those of so many other people with cystic fibrosis, is cruel. These life-changing drugs are priced to be unavailable to the vast majority of those who need them, the value of our lives left in the hands of market forces. A simple pill could open up the possibility of a more fulfilling, longer life, yet the high cost leaves me burdened by this illness, unable to complete many simple daily tasks.
The development of this drug brought hope to people with cystic fibrosis, significantly reducing treatment times and the number of hospital admissions, hopefully pushing the need for lung transplants back and taking us one step closer to a cure. Priced correctly, it should save the NHS money in the long term. These drugs should be available to all those who need them, rather than feeding the profits of companies that hold in their hands the power to change lives. What gives Vertex the right to dictate the value of my life?
• Isabelle Jani-Friend is a recent graduate of the University of Southampton