Alpha-1 Antitrypsin Deficiency

Tonia Antoniazzi Excerpts

Wednesday 31st October 2018 Westminster Hall

[Mark Pawsey]

The hon. Gentleman anticipates two of the asks that I will come to later; I thank him for that helpful contribution.

Going back to Stephen’s condition, he suffered very seriously from chest infections. In fact, at one point he suffered with his lungs collapsing, and Birmingham Children’s Hospital, which was treating him at the time, took the decision to staple his lungs to his chest wall. As I say, it was eight years after first presenting with the issues that Stephen’s own general practitioner tested him for alpha-1 and that was found to be the condition he was suffering from.

The British Lung Foundation estimates that approximately 25,000 people in the UK suffer from the disease, and while many can live relatively normal and healthy lives, others such as Stephen suffer from the condition. Interestingly, I was contacted only today by a colleague here in Parliament, who told me that they suffer from the condition and that, as one of three siblings in a family of six, they were involved in a study based at University College London in the 1970s. That was someone we would not recognise as being a sufferer, which exemplifies the fact that not everybody shows the symptoms that so many people have.

It is rarer for children to suffer, which explains why the doctors who treated Stephen did not test for alpha-1 initially, but we can be grateful that by 2012 he was finally correctly diagnosed. Stephen’s mother Sarah was very concerned about the lack of awareness of the condition and became involved with the Alpha-1 Alliance, a charity formed in 1997 to support those suffering from the condition and their families, carers and friends. We are joined today by members of that group and affiliated organisations, as well as Professor David Parr, who is head of medicine and clinical director for cardiothoracic services and a consultant respiratory physician at University Hospitals Coventry and Warwickshire.

Both Sarah and Stephen, who suffers from alpha-1, have told me that they found the support from the Alpha-1 Alliance and the alpha-1 support group invaluable. When I met them back in 2012, they asked me to get more involved, learn and understand more about the condition, and do what I might as their Member of Parliament to raise awareness here in Westminster. To that end, I raised the condition with the Leader of the House in business questions in December 2012; I understand that was the first time that the condition alpha-1 had been mentioned in the House. We then arranged a seminar and a report was brought to Parliament, and there I met Professor Parr, whose hospital, UHCW, happens to serve the residents of my Rugby constituency.

At that time, there was a sense that the work of the Alpha-1 Alliance was gaining traction and starting to raise awareness. We had a meeting with the then Secretary of State, my right hon. Friend the Member for South West Surrey (Mr Hunt), and Karen North and Margaret Millar of the alpha-1 support group came along to explain a little about the condition and how the treatment for it could be improved.

Testing for this condition is a relatively straightforward process, requiring a simple blood test. Many organisations, including the World Health Organisation, have made recommendations on who should be tested for the condition, such as anyone who suffers from emphysema, COPD or chronic bronchitis; people with a family history of liver disease; and certainly blood relatives of a person diagnosed with alpha-1. Perhaps also newborns and children with unexplained liver diseases should be considered as potential sufferers from alpha-1.

[Tonia Antoniazzi (Gower) (Lab)]

I welcome today’s debate, as I also have a constituent, Tina Walker, who has alpha-1. She travels from Swansea to the clinic in Coventry twice a year. Last night, I also had an email from an ex-pupil who I used to teach in Wigan, whose daughter—she must be just a small child—also has the condition. I feel strongly that we parliamentarians should be working together. Does the hon. Gentleman agree that it would be wise of us to urge the Government to run a UK-wide campaign to raise awareness? As he says, this involves just a simple blood test.

[Mark Pawsey]

Part and parcel of our efforts today is to achieve precisely that. I am delighted that the hon. Lady’s constituent comes to Coventry and is being treated by the excellent services at University Hospitals Coventry and Warwickshire.

Unfortunately, alpha-1 is not yet curable, and no specific treatment for the disease is freely available in the UK; it is a matter of treating only the symptoms with the appropriate therapeutic methods. The hon. Member for Strangford (Jim Shannon) referred to intravenous AAT protein augmentation therapy, which involves replacing the missing AAT protein. That treatment is available in the United States, Spain, Germany and Italy, for example, but it is not yet available in the UK.

The National Institute for Health and Care Excellence—the Government body that produces guidelines on which treatments to make available—only last month published draft guidelines that rejected the use of the only licensed augmentation therapy product in the UK, Respreeza. It has had a UK licence since 2015, but was unfortunately deemed by NICE to be too expensive to be made available on the NHS. We acknowledge that it is expensive; lifelong therapy costs around £60,000 per patient per year. NICE continues to evaluate that and will make its final recommendations next year. The entire alpha-1 community has been heavily involved in pressing the case for patients across the country to be prescribed the treatment.

Only the symptoms of alpha-1 sufferers are treated, often by inhaled medications developed for people suffering from asthma and COPD, rather than specific treatments for the lung damage caused by alpha-1. The other issue is that those who suffer from alpha-1 become susceptible to chest infections, which was certainly the case for Stephen Leadbetter, and it is vital that they are treated quickly with antibiotics at the first sign of infection and are vaccinated every year against flu.