Orkambi and Cystic Fibrosis

Debate between Paul Scully and Lord Austin of Dudley

Monday 19th March 2018 Westminster Hall

[Paul Scully]

I will come back to that important point in just one second. We were talking about the campaign being brought together, and I want to pay tribute to the hon. Member for Dudley North (Ian Austin), who has done a fantastic job in raising the profile of cystic fibrosis. I am sorry that I was not able to be at the roundtable that he hosted—I was out of the country—but 41 Members were there who were keen to learn more about this. That is because of his efforts and the efforts of the petitioners, and that is absolutely to be welcomed.

[Ian Austin (Dudley North) (Lab)]

That is very generous of the hon. Gentleman, and I am grateful, but the credit is due not to me but to the fantastic campaigning of the Cystic Fibrosis Trust, the army of people around the country who have this condition and their families. Let us be honest: that is why there are so many Members in the Chamber today, just as there were at the roundtable. However, I am grateful for what he says.

[Paul Scully]

Take it while you can—I am joking, of course.

We have talked a little about cystic fibrosis, and we are all here because we know what it is, so I will not talk about that. I know that lots of Members want to speak, so I will finish with the little hint that I have seen of what it must be like to live with cystic fibrosis. It goes to the point about medicines from the hon. Member for South Antrim (Paul Girvan). I saw a fantastic video—it is private at the moment but I hope the Millers will make it public—of Grace Miller, who is 15 and likes to run. What many people do not understand about cystic fibrosis is that many people have to do physio. They actually do more training than a lot of professional athletes. These kids, who may be aged three or four, have to get up and use a nebuliser for an hour or so. I met a mother who talked about her three-year-old daughter who would use a nebuliser with salty water. It would make her cough and make her sick, and she would then have to do her physio. She would have to run on the spot and run around the house for a while, and then she would have to go and eat.

However, cystic fibrosis also suppresses nutrient intake, so sufferers have to eat far more than normal people—50% more in many cases—with some people taking up 3,000 calories or maybe more each day. It is therefore no surprise that sufferers often have a really unhealthy relationship with food. They just do not want to eat. We talked about one girl who filled her pockets with coins because she did not want to go to school and be underweight. That relationship with food is relatively minor in some ways, compared with the shortage of breath and the actual illness. We have not even started to talk about the underlying illness that cystic fibrosis causes.