Orkambi

Jim Shannon Excerpts

Monday 4th February 2019 Commons Chamber

[Bill Wiggin]

I suggest that the hon. Gentleman hears the rest of the speech before he expects to draw any conclusions.

Cystic fibrosis is a life-limiting genetic disorder. Patients with cystic fibrosis experience a build-up of thick mucus in their lungs. This can have a wide range of effects on their respiratory, digestive and reproductive systems. The disease is widespread in the UK. One person in 25 carries the faulty cystic fibrosis gene. Statistically, that is 26 Members of this House whose future generations could be affected by this cruel disease.

[Jim Shannon (Strangford) (DUP)]

I congratulate the hon. Gentleman on bringing this debate to the House; there is such a good crowd in the Chamber tonight due to the importance of the issue. I also thank him for being an advocate of Orkambi not because I have had any dealings with the company, but because I have many constituents who are affected. One grandmother in particular has asked me to make a plea because the life not only of their grandchild, but the lives of the whole family, have been turned around by this drug. I want this drug to be available for families throughout Northern Ireland and the whole United Kingdom. Does he agree that this Crown licence is a way to get around the stalemate that is preventing CF sufferers throughout the United Kingdom of Great Britain and Northern Ireland from accessing this drug, which is proven to deliver tremendous improvement in quality of life?

[Bill Wiggin]

I am sure that the hon. Gentleman’s constituents will be grateful to him for raising the issue in the House this evening.

Every week, five babies are born with the disease, according to Great Ormond Street Hospital, and every week two young people die as a result of cystic fibrosis. The disease accounts for 9,500 hospital admissions and over 100,000 hospital bed days a year. There are two main ways to treat cystic fibrosis: conventional treatments target the symptoms, and precision medicines such as Orkambi tackle the cause of the condition. For conventional treatment on the NHS, the average waiting time to be admitted to hospital is 45 days.

Orkambi presents a relatively safer, more effective and clinically meaningful alternative. In treating the root causes, it reduces lung damage and cystic fibrosis-related diabetes, and improves pancreatic function. The drug has been approved by the European Medical Association, and the Food and Drug Administration in the United States. It avoids the high risk associated with organ transplants.

Orkambi treats the F508del mutation, which around 50% of people with CF in the UK carry. Essentially, the drug permits more chloride ions to pass into and out of the cells. This helps to keep a balance of salt and water in affected organs. Ivacaftor is one of the active substances in Orkambi. It increases the activity of the defective cystic fibrosis transmembrane conductance regulator protein, thereby making the mucus less thick. Decline in lung function is the most common cause of death for people with cystic fibrosis and, although not a cure, Orkambi has been found to slow the decline in lung function by 42% and reduce hospitalisations by 61%.