And then, it all became clear... Well, kind of clear.

There it was. On, no less, the VERY FIRST PAGE of my chart. Written in pen at the VERY TOP of the page. In my doctor's handwriting. In large print.

"Alpha-1 level???"

You may recall that a number of years earlier, he mentioned a level that he assured me not to worry about. Trust me... Just lose some weight, and you'll be fine. This couldn't possibly be the same thing that was causing an issue now, could it?

"Jim... Did you look at this?" I could tell by the tone of his voice -- It was not a question to which he anticipated an appropriate answer. My primary doctor looked at it, and his face grew pale. For the first time in five years, I witnessed this educated, well-spoken man being rendered speechless. It was clear that not only did he not recall writing the note, but he had no real idea what it meant. My heart sank as I waited for some sort of explanation or treatment suggestion that could put my mind at ease. Needless to say, none was forthcoming.

The hepatologist, at this point clearly uncomfortable and not wishing to throw my primary care doctor under the proverbial bus, glossed over the issue with a brief, cursory explanation and quickly left the room. My primary care doctor told me that he would get back with me with details. I anxiously awaited the details from him, hoping for the best but expecting the worst.

Jim... Did you look at this?

I presented the records to my doctor, and soon learned some rather disturbing news. My doctor called in a hepatologist that worked in the office with him. The hepatologist met with me, ran some blood tests, reviewed my records, and asked me to follow up with my regular doctor shortly. I scheduled a time when they were both in the office, just in case. It would turn out to be a choice that was extremely enlightening.

During that appointment, my doctor still didn't have a clue as to what was causing the cirrhosis. He called in the hepatologist, who began to review the chart held by my primary care doctor. It didn't take very long -- only a minute or two before he uttered words I don't think I'll ever forget.

"Jim... Did you look at this?"

Who could have seen this coming?

As you may have already surmised, there never really was a "Non-A, Non-B Hepatitis." Hepatitis C was ruled out in my case, and so I soon turned into "that patient" that my doctor had no real clue as to how to classify. So, when he found that low liver enzyme level in my blood, he hadn't any idea what it meant. Instead of doing any research, it was simply chalked up to my weight and I was told that I simply had a "fatty liver."

Five years later, I began to have issues. First, Kidney Stones (no fun at all)! Then, gallstones. My doctor referred me to a surgeon to remove my gallbladder. All went reasonably well, until I received a call from my doctor a few weeks afterwards. He regretted to inform me that I had an issue with my liver.

So, I called the surgeon, who apparently had taken a photo of the liver when he was doing the surgery. His nurse, who didn't really want to be bothered with my request for MY medical records, at first denied that there was any pictures of my liver in the file. "Only one picture, here", she said, "and that can't be your liver. You see, the outside of a liver is smooth and the only picture here shows something that is all shriveled up and bumpy." Well, then. Thanks for your concern.

Let's just watch it for now... Famous last words!

So... Where do I start?

Many years ago, when I was just a teenager, I had some routine blood work drawn. Turns out that my liver enzymes were high. "What does that mean?", I asked. After ruling out Hepatitis A and Hepatitis B (the only two they knew about), I was told that I had "Non-A, Non-B Hepatitis".

When I asked my doctor what that meant, he assured me that it was nothing to worry about. "No big deal - Just follow up with blood work every few years and we'll watch it."

So that is what I did. For the next 16 or so years, I simply "watched" the numbers. Finally, not really trusting the analysis of the situation, I scheduled an appointment with my wife's doctor. He mentioned some blood level, but again, said that it was nothing to worry about.

The start of my long, strange, journey...

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.