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Exciting update!!

The following is written by a Cf patient who participated in a recent study designed to treat the underlying cause of cystic fibrosis. 
Enjoy the update!!

The result of a trial by Vertex Pharmaceuticals for a drug that treats the underlying cause of Cystic Fibrosis was released. I read the data in The New York Times over and over until it sunk it.
 

The study showed the drug combination of Ivacaftor – also known as Kalydeco – and the new drug Lumacaftor has a groundbreaking effect on people with Cystic Fibrosis who were carriers of the gene combination double delta F508. It’s the most common genetic mutation in just under 80pc of the world’s population with CF.

The results narrated perfectly how and why I had been feeling certain ways for the past 12 months.

I was one of the lucky 1,100 people deemed suitable and selected for the trial, which began late last summer.

It enlisted people with Cystic Fibrosis with double DeltaF508 from across EuropeNorth America and Australia. All participants signed confidentiality forms.

Any major deviation from this would jeopardise not just one’s own place but the place of all other participants in this never before witnessed treatment effort, which has been 22 years in the making. 

I sat on my hospital bed shaking in the knowledge that this was a historic day for people with CF, their families, their friends and their medical workers worldwide.

This is the first time in the history of the illness, since the gene was discovered in 1989, that there is a treatment that treats the origins of the illness for the larger population and not just the symptoms.

Much of the finances to keep these trials going were borne by the CF Foundation in the USA who worked hard to keep research going.

There were two divisions of the trial, which were each then divided in three. Patients participating took one drug - ivacaftor and either a low or high dose of another drug -lumacaftor.

Patients in the third group received a placebo. After 24 weeks lung function had improved in all four groups taking the combination drugs. Lung function fell a little in the placebo group.

The study found the drugs reduced infections from 30-40pc meaning less time in hospital and a better quality of life. 

It means more time for living, for love, for work, for school, for chasing dreams, for all those things that any person wants. 

I started the trial in August and immediately felt a difference. I cleared more mucus from my lungs and buzzed around with unflappable positivity. I believe now this is probably what they call ‘the placebo effect’. 

I wanted so badly to be on the drug I believed that I was. While tidying my room a month later I found myself unable to stand up after leaning to pick something off the ground. 

A trip to the hospital registered a lung collapse and back on intravenous treatment I went. The support of solid friends at this time really kept me steady.

One of two things was happening. Either this drug would just not work enough for me to see a real difference – because this was a possibility. Or, maybe, I was on the placebo. I still don’t know for sure. But what I do know is this.

In January I got a severe flu which was complicated by CF and I was hospitalised. While recovering the next stage of the trial started. Every participant now started some dosage of the drug combination.

By my birthday in March I was still out of hospital. I was still well. When May came around I had defeated two postnasal drips that usually would cause at least two weeks in hospital on intravenous antibiotics. 

My sputum was thinner.  It came up easier. My energy was higher. I could follow through on so many things socially and professionally. This was an invigorating concept.

In recent prolonged periods in over 20 degree heat instead of finding breathing difficult I felt normal.

Instead of trying to deal with breathlessness after a day or two in extreme heat I was comfortable. I barely noticed the difference. This made me cry a little at the sheer magic of what was happening. My body had really changed.

It’s June now and I’m in hospital for the first time since January. The last time I had that length of freedom between admissions I was maybe five or six years old. This treatment is not a cure for CF, but to see the real change in my own body is a miracle.

Of course I must still do treatments as normal and my CF has not gone away but it has changed.

It’s not so much a drastic change, it’s a gradual one that I give thanks for every day.

I am thankful every time I realise I am still out of hospital. Or when I can plan a week or two in advance to go to a gig or a birthday party, or meet a friend and not have to rest on the idea that I might not actually make it despite my best intentions. 

The transformative feeling the past few months have brought is overwhelming at times - like it’s almost too good to be true.

My weight has increased too, which is a big deal in CF as it often drops when unwell and leaves less ammunition to fight infection.

But there is a more substantial thing too, something completely unexplainable or immeasurable by science. It’s best described as substance, or a sturdiness, in the body that was not there before.

It is something full of energy and vitality that allows me to look towards the future with the thought that I might actually make it. I feel stronger. The cycle of infection that was once so predictable has seriously reduced and in many ways my body feels like my own again. I have a new quality of life.

The next step for the drug combination is application to and approval by the Food and Drugs Administration and the European Medicines Agency so that negotiations can start.

Typically this should take eight months. However this must be watched carefully and will be by campaigners and people with CF worldwide. It has been estimated by analysts the drug will cost between 160k and 200k a year per person which is significantly less that Ivacaftor alone which was approved in 2012 and  treats a much smaller population.

The struggle to get Ivacaftor (Kalydeco) to people who need it must not be a repeated one.

When the combination eventually arrives it will change some much of the worlds population with CF forever which will in turn be more economically beneficial to health services everywhere.

Futures that were not possible, families that were never considered and adventures that may not have otherwise happened will become possibilities. In many ways, life will begin again and we all deserve a chance to live it.


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