Well, here it is.
I've had a huge number of thoughts, hopes, and fears rushing through my mind of late. Finally decided to try to get control of them by organizing and sharing them. Don't know if anyone will take the time to read this, but if even one person gains any hope or can relate to what I'm going through, it was well worth every moment.
Just to introduce myself... I am a 43 year old man from Walled Lake, Michigan (a suburb of Detroit) diagnosed about 2 years ago with Alpha-1 (ZZ). I have a wonderful, loving wife who I couldn't have made it this far without, and two fantastic, smart kids who make it all worthwhile (ages 7 and 11). I own, along with two dedicated friends / business partners, a title insurance agency that does national real estate closings.
For those of you who don't know, I'll give a brief sketch of Alpha-1 Antitrypsin Deficiency (A1AD). Basically, I like to joke that it is a genetic disorder that can cause early onset emphysema and cirrhosis, without the "fun" of smoking or drinking!
When you develop a lung infection, your body releases an enzyme called neutrophil elastase. The purpose of this enzyme is to attack the infection that is, in turn, attacking your lungs. Without this necessary substance, the infection would quickly destroy large amounts of lung tissue.
In a "normal" person, the liver releases a protein called Alpha-1 Antitrypsin. This will attack the neutrophil elastase, and stop any "collateral" damage to the lungs, once the enzyme has served it's purpose. However, in a person with Alpha-1, the A1AT protein produced by the liver is formed incorrectly, and it is therefore not allowed to be released to the lungs. In a lung-affected Alpha, the damage is then caused primarily to the lungs due to the enzyme not being controlled and stopped. In a liver-affected Alpha, the fact that the protein is not released causes the damage to the liver. Hopefully, that gives a bit of an explanation of the story of the disease itself.
I happen to be the more rare variety -- the liver-affected Alpha. And this is my Alpha-1 Story.
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6 comments:
Most of us ZZ's are lung affected. I'm not sure which is worse... Personally, my first thought is that I'd trade with you. :-) I'm at 39% lung function. I've been a lot worse and better. If you have questions, most of us will be glad to try to help. I've been living with this since 1995. Diagnosed in Nov 2001... I'm glad you found Spiderspun. There isn't a better website. Welcome! Michael Crews
Hi Michael,
I certainly didn't mean to imply anything about liver versus long involvement. Please forgive me if it came across that way.
That said, I certainly appreciate the kind welcome and support that you and others have given. It is much appreciated -- I only hope that in some way I can repay yourselves or others like us on down the road.
Thanks again!
Mike
Hi Mike, It's wonderful that you are sharing your journey. It's not often we have the chance to read someone's perspective with the liver-side of Alpha-1 (where it involves an adult). Most of the time it is the children who fall into liver problems and their parents will start an online journel. Adults seems to have more of the lung issues. You will find a wonderful array of support from the Alpha-1 community.
So great to meet you Mike!
Thanks, Noreen!
I'm already beginning to feel the suppor from the community, and it is wonderful. As you said, us liver folks are kind of the rare breed, and if my experience can help them, or anyone else here, in any way at all, then I will certainly consider it time well spent. Perhaps through doing this, a bit of my gratitude will be given back to those such as yourself who have shown such support to me.
Nice to meet you all!
Mike
Thanks a lot for your blog, that is so well described and informative. I am a ZZ lung affected person, known for a year. I will be following your story.
Thanks for your comment -- Much more to come. I wish you the best in your journey. If you need anything that I can do, just contact me!
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