I was diagnosed with Alpha-1 Antitrypsin Deficiency in November 2002. I am lung-affected with a ZZ phenotype. I have 2 siblings;sister is SZ and brother is ZZ. Neither one are lung or liver affected and in fact, my brother jogs 3- 5 miles almost daily. I've been affected with this bug since 1995. That is when I first began to notice that things were not quite right...either that or I was getting old very quick for 37. I've always been into sports. Tennis and softball began to really get to me and I had a chest xray to find out why. Extreme emphysema the doctor said. Wow, smoking stops today and it did. Didn't get better though and didn't know why until my first trip to a pulmonologist in November 2002. He took the time to do the tests necessary to know why. A1AD and all of the definition he knew. I've had ups and downs, but I'm not going down without a big fight. Golf is a religious hobby for me now. When asked how my lungs affect my game, I usually just say "you can hold your breath long enough to hit a golf ball!" :-)

My FEV1 was 27% in 1997. That was my first breathing test and the doctors definition was that my lungs were about 160 years old. I didn't understand the 27%, but I did the 160 years old! With meds after diagnosis my FEV1 was 38% in early 2003. I went to the gym, did the treadmill and some weights and got up to 62% by March 2005. All was going great and then in February 2007, I hit a brick wall. I was diagnosed with Staph in my lungs and spent a week in the hospital in April 2007. Today I'm doing better, but no where near where I was. My SOB is back to what I call severe with exercise, but at least I'm back to where I can do a mile in about 30 minutes on the treadmill.

I'm on all the meds everyone else is; Albuterol & Provental by Nebulizer X4, Spiriva, Symbicort 2X2, Zemaira 90mg per kg 1/week. All but the Zemaira helps you breathe. The Zemaira keeps you well. I have been on Prolastin, Aralast and Zemaira. I have had the least sinus infections with the Zemaira. I also had the Staph while on Zemaira, but I had a Port and I believe after 5 years, the Port is how I got the Staph. I go for veins in my hands now. There are good and bad points to not having the port. The bad is that I can't infuse myself. The good point of not having the port is that Staph will be next to impossible to get by infusion. Hope my veins don't wear out. I haven't had a sinus infection, cold, etc... since 2006.

My advice for what it is worth: DO NOT give up. DO NOT sit down because you think you can't do it anymore. Do what you can until you become a little short of breath, rest and begin again. Next week you will be going twice as far. Doc says 24/7 oxygen. I say, not yet Doc. Lungs are weak, but heart and mind are strong. :-)

Aug 08 update. My oxygen concentrator malfunctioned and blew its contents all over my house. The contents "zeolite" remove the water from the air. Zeolite is not something you want to breath. Didn't know this until it was too late. This occurred while I was asleep and connected. I breathed the zeolite for maybe hours. Didn't know there was a problem until I woke up with the alarm. 4 days later I went to the hospital with pleurisy. Now, a month later, I'm on the mend again and about as bad as when I had staph in my lungs. I wish I knew what to tell everyone that is on a concentrator, but at least I can pass on the info that the zeolite is not a good thing to have outside the concentrator and especially not in your lungs. I haven't made it that far yet, but it seems to me that this has to be a manufacturer defect. If there is anything you don't want to happen to a respiratory patient with a concentrator, it would have to be this.

Good luck everyone!