Little Fierce One

Wishes

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On 20th January 2017 Lorcán came into our lives. A wonderful person for us to treasure and nurture.

I asked Dermot, “What happens when all of your wishes come true?” to which he replied “You begin to wish for other things”.

Ten days later Lorcán was diagnosed with Cystic Fibrosis (CF). Ten days later I began to wish for other things.

CF is a genetic condition affecting more than 10,800 people in the UK. There is no cure. Around half of people with CF die before they reach thirty.

One in 25 people carry a faulty CF gene and never know it, it only causes a bother when two come together. Lorcán carries two copies of the most common CF mutation (F508del mutation), one copy inherited from Dermot (I knew he was too good to be true, there’s always a catch!) and one copy from me. The faulty gene causes a build-up of thick, sticky mucus, especially in the lungs and digestive system.

There is no cure for CF, but there is medication that can help and one type can help people who have Lorcán’s mutations in particular. It is called Orkambi.

Orkambi can slow down the decline in lung function experienced by people with cystic fibrosis up to 42%.

Despite having been licensed for use in the UK for people with CF over the age of 12 (it is anticipated that this age will come down) with two copies of the F508del mutation, NICE (the body that appraise these drugs for use in the NHS) rejected its use on the NHS.

Creating and testing drugs like Orkambi is very expensive for the pharmaceutical company Vertex and a lack of long-term data can make it hard for NICE to accurately assess their cost-effectiveness in order to make them available.

However, Orkambi could give Lorcán more years of life.  More years of being able to breathe in and out. More years to laugh and play, to learn and love, and to give so much to this wonderful world. More years to be with me and Dermot, more years in which he could maybe even have a family of his own.

Before you comment, if you feel inclined to, please read this first- https://www.cysticfibrosis.org.uk/…/cfpp_friends_family_gui…

Dermot and I don’t want sympathy, we believe that life is what you make of it. Lorcán is still a wonderful person, he’s just a bit faulty but sure aren’t we all in one way or another? We three have a challenge ahead of us to keep him as healthy as we can, but we’re able for it. We don’t want sympathy but we do want action.

On 26th June there are protests across the UK to call on Vertex and the Government to reach an agreement on Orkambi.

People with CF can’t be in close contact with each other because of cross infection, because of this many people, including kids like Lorcán and their parents who look after them, won’t be able to attend the protests.

If you think this a worthy cause then I am asking you to stand there for those who can’t on Monday 26th June 2016, 1-4pm at Stormont, Holyrood, The Welsh Assembly or Downing Street.

https://www.cysticfibrosis.org.uk/protest-for-orkambi/

Please act to help make one of my wishes come true.

Lots of love,

Jen

#Orkambinow

       

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