Ok, so am I going to die?

Having Alpha-1 isn't a death sentence. Even if you are PiZZ then you can live a normal life and may well completely escape any major problems. It is likely that unless some developments occur that you may well become SOB in your seventies.

If you do have symptoms whilst young(ish), it probably means that you are a smoker or ex-smoker Don't feel guilty... it's not your fault. You almost certainly wouldn't have developed this without the A1AD. Giving up, even at this stage, is always worthwhile as the rate of decline will slow down. If you do smoke then you know what to do. If you don't stop you'll lose lung function so quickly that it's frightening.

If you work in one of the dusty jobs, or jobs like soldering and welding then you'll have to rethink what you do. Ditto for woodworking, masonry, mining, baking and fibreglass resin work. In fact, anything that makes you cough.

We'll be looking at medication on this page, but obviously making sure that medication is taken properly is also very important in helping to keep things stable.

Liver disease in adults

It's unclear how many PiZZ individuals will escape lung disease but develop liver disease, usually in late middle age.

Erikkson re-examined post mortem results from people who had died of A1AD related COPD.
He found that 39% had liver disease as well. He didn't say how advanced it was or, indeed if it would have gone on to cause real problems. However, we know that A1AD does and can cause cirrhosis and rarely, primary liver cancer. Both of these conditions can be treated by liver transplant.

It's probably a good idea to avoid heavy drinking if you are PiZZ but, in all honesty there doesn't seem to be much of a connection to that in people who have liver problems.