email forum - by joining the group e-mail forum you will be in contact with Alpha patients, their families, carers and friends living throughout the UK and overseas. We are a friendly group, and between us we have a wealth of knowledge and experience to share. There is always someone to help with any worries or queries you might have.

What is Alpha-1 Antitrypsin Deficiency?

Alpha-1 Antitrypsin Deficiency also known as Alpha-1, A1AD or AATD is an inherited, genetic condition that is passed on from generation to generation. As the name suggests it is a deficiency of alpha-1 antitrypsin (AAT) in the bloodstream. AAT is an enzyme produced in the liver to help protect the tissues of the body during infections. The low level of AAT in the blood occurs because the AAT is abnormal and cannot be released from the liver at the normal rate. This leads to a build up of abnormal AAT in the liver that can cause liver disease and a decrease of AAT in the blood that can lead to lung disease.

Top Story

CSL Behring Receives Marketing Authorization for Respreeza® in Europe

Respreeza® is a maintenance treatment for severe Alpha-1 Antitrypsin Deficiency patients and has been shown to slow the progression of emphysema

CSL Behring announced today that the European Commission (EC) has granted marketing authorization in all European Union (EU) member states for Respreeza® to treat patients with Alpha-1 antitrypsin deficiency (AATD). Respreeza®, a highly purified Alpha-1 protein derived from human plasma, is the only Alpha-1 proteinase inhibitor that has been proven in a prospective double blind, placebo controlled trial (the RAPID study) to significantly reduce the loss of lung tissue, slowing the progression of emphysema due to AATD. AATD is a hereditary condition marked by a lack of the Alpha-1 antitrypsin protein, whose main function is to protect the lungs from inflammation.

“AATD is a potentially debilitating disease and many affected individuals suffer from serious lung disease," said Professor Helmut Teschler, MD, Director of the West German Lung Centre at the University of Essen. "With the approval of Respreeza®, healthcare professionals can now provide patients with severe AATD in Europe with a next generation Alpha1-proteinase inhibitor (A1-PI) that provided additional evidence that this augmentation therapy can slow the accelerated loss of lung tissue."

Frank Willersinn, M.D., Alpha-1 Global Steering Committee Chair and patient representative in Europe added, “We are so glad that Respreeza® has been approved by the European Medicines Agency. We commend CSL Behring for their long-lasting commitment to the Alpha-1 community, now bringing their established Alpha 1-antitrypsin product to Europe, allowing it to be a cornerstone for treatment of AATD patients in the near future.”

Source: CSL Behring 25/08/2015

Alpha-1 UK Support Group
14th Annual Social Gathering/Information Day

Bentley Hotel Leisure Club and Spa Lincoln
Saturday 12th September 2015

Due to popular demand this year’s Alpha-1 UK Support Group 14th Annual Social Gathering will again be in the historic city of Lincoln on Saturday 12th September 2015 at the Bentley Hotel Leisure Club and Spa. The event is one of the largest gatherings of Alphas and their families in the UK where you can meet and socialise with fellow Alphas and be updated
by leading experts in the field of Alpha-1 Antitrypsin Deficiency.


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Latest News

CSL Behring Receives Marketing Authorization for R...
26 Aug 2015 07:26

Respreeza® is a maintenance treatment for severe Alpha-1 Antitrypsin Deficiency patients and has been shown to slow the progression of emphysema CSL [ ... ]

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One step closer to the first UK drug license for augmentation therapy for patients with severe alpha-1 antitrypsin deficiency CSL Behring has annou [ ... ]

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