THE BAD
I started smoking my cap pistol when I was 9 years old in Virginia, but I didn’t inhale! About 7 years later, in 10th grade, I went on a weekend trip from Huntsville to Birmingham, Alabama, for the annual State Key Club Convention, a great place to sneak in a couple of Swisher Sweet cigars with my 3 best buddies, that is when we weren’t dropping water balloons from our hotel room 12 stories up! And, of course, we DID occasionally attend the Key Club meetings in the big ballroom, so the school’s money wouldn’t be totally wasted. Even though these same buddies found various smoking pleasures throughout high school, I was on the football team, which included, among other ‘mixed’ benefits, a huge ‘microscope’ watching our personal lives, pretty much 24/7, during season! To get caught smoking resulted in darn-near capital punishment. I remember one player named Elmer who got caught behind the lunchroom with a Camel (one without the humps). The next day at practice, Coach told him to run 500 laps around our football field… after the 2,000 pushups, or he could quit. Last I heard, he joined the Varsity Tiddledewinks Team, and eventually flipped out!
I started college at Georgia Tech in 1971, still playing football, but without a scholarship, but still under the ‘microscope’. This will be hard to believe, so brace yourselves. In 1971 anyone could smoke during classes and often our professors puffed their pipes during their lectures. Talk about 2nd hand smoke in a small area! And 3rd and 4th and 5th hand….you got the idea.
Due to a series of beyond-my-control events, I was released from the Georgia Tech Football Team along with 5 other TV-Star-Wannabees, walk-on players. After several days of sulking, I picked up where I left off at the Key Club Convention, now 3 years later, and grabbed a handful of cigars down at the 7-11 store. Only this time I tried the wooden tips…still sweet…Hav-A-Tampa Jewels. They were, of course, ideal for chewing away tension, which most smokers and college freshman, like me, carried in abundance. This cigar habit continued with rare occurrences until I discovered a pipe was much more relaxing, and somewhat intoxicating!
Now before I lead you into my gateway of pipes, let me say something about drinking; alcohol that is. To give you a crystal clear view of my virtual abstinence, I attended a BIG yard party at a rich kid’s home the winter between my sophomore and junior years in high school, off-season from my football commitments, that’s extremely important to know! Beer, at this party, was flowing like gas at truck stops. You guessed it, his parents were somewhere far, far away. Now there were about 100 of us at this future-fraternity-brothers-of-America event. The lead party-boy, I’ll call him “Fonzie”, kept ribbing me saying something like “beer is good for you, lots of natural ingredients-like vitamins”, in his slurry speech sort of way. I smiled. He then said, “oh come on, Traci will have one with you.” Now I had no idea who Traci was, but I have always believed that “misery loves company”, and Traci sounded like an improvement in my “come as I was…lonely” condition. So…Fonzie grabbed a mug, loaded it with mostly beer and a little foam, then proudly slipped it into my hand. When I turned that mug up and started slowly chugging those vitamins, there must have been at least 25, maybe 30 stunned chums surrounding me with their pure astonishment revved in high gear, some smiling, some laughing, but most probably thought they were already drunk, and therefore delusional. Allen Russell was drinking a beer! But, where was Traci? Dang Fonzie lured me with his own delusion!! That, scouts honor, was the only alcohol I drank until years later.
Before I go on, let me pause at this point to say….I had NO IDEA until 29 years later that I had alpha-1 antitrypsin deficiency, and now I know, I was poking a sleeping bear with my lifestyle.
The summer after my freshman year, I was taking a trip from Huntsville to Nashville to see a new girlfriend. Traveling solo in my Dad’s ’67 Mustang, I was feeling VERY adult. I started seeing images of those Ga. Tech professors in class and said to myself, “SELF…you need a pipe!”. Within 5 miles, there was a drug store and I remember Dr. Grabow’s brand was always stocked on the wall behind the cash register with a wide assortment of essentially the same thing…a little fire-proof bowl with a tube connected for inhaling smoke. Of course tobacco was essential to make all this work, and I had no idea which one to buy. Well heck, there were only about 20 choices! I wonder which one the professors bought? It must have been foreign since they all seemed to know a little Latin, so I looked for anything with the name Rome or Caesar or Hamlet on it. No luck. But I did find one from Sweden, due north of Italy, and much colder, which probably made the tobacco much fresher anyway! Borkum Riff offered two flavors, “whiskey” and “cherry”. Well “cherry” sounded too adolescent, so I picked the “whiskey”. Pipe cleaners and a lighter rounded out my bill.
I HAD to pack the Borkum Riff into that little fire-proof bowl before starting the Mustang, then off I went northbound, puffing away. Around the Tennessee state line, I needed some gas. I pulled up next to the self-service pump, hopped out and nearly hit the asphalt in this stupor-sort-of-feeling. A lot like when I drank those vitamins to entertain Fonzie and the chums! My first thought was, “Wow, this stuff is LEGAL?” No wonder those professors were so laid back! Well, you would think I would puff and puff right into Nashville, but honestly, I didn’t want to arrive drunk when I met my new girlfriend’s parents. Her Dad was a famous lawyer and I wasn’t sure whether he prosecuted or defended. I decided right then to play it safe, assuming prosecution was his passion. So, I stuffed the pipe in the trunk as I entered Tennessee, about 2 hours south of Nashville.
Since you’re probably real tired of all this detail, let me get to my point. I was a smoker, and I drank some, never knowing I had been born with alpha-1. And a “ZZ” phenotype at that! My blood chemistry showed a measure of only 19 mg/DL in the “quantitation & phenotyping” test performed in 1999. This level was in the “null-null” range. That’s the worst. That meant I had an extremely LOW amount of antitrysin in my blood, and an extremely HIGH chance of severe lung and/or liver disease. Emphysema and Cirrhosis were the sleeping bears I had been poking all these years. ‘Deadly bears at that, unless I did something SOON and WITH RIGOR. This was…THE BAD.
THE UGLY
Those years of intense exercise through football and track and basketball meant nothing when I started gasping for air about 7 months before I learned I had alpha-1. My new allergist told me no doubt about it, I had asthma and he gave me two powerful inhalants and detailed instructions. My family doctor said stop smoking and stop drinking, or they would stop me, very soon. Both ended September 4th, 1998, before dark, at a romantic resort, sponsored by my wife in the north Georgia mountains.
The ‘asthma’ got worse, and morning-nausea set in. Again, my family doctor intervened. He knew a really good gastroenterologist named Dr.Alan Sunshine in north Atlanta. I figured he must be a warm guy, so I signed up! His answer…a biopsy of my liver. MY LIVER ?? That had nothing to do with breathing gasps, but he had a hunch. Yep, when my wife and I sat in his office a week after the biopsy he told us about the alpha-1. I was his very first patient with this disorder, and he had been wearing a lab coat and stethoscope for over 20 years! Lucky me, huh?
The next level of expertise was my referral to an Englishman-hepatologist, just 3 months later. He studied all the tests, added some of his own, then later said he was certain I would need a liver transplant in 3 – 10 years. The day I received that “F” on my report card in English, back in 11th grade, flashed within my mind. “How, why …..I don’t deserve this, do I?” He said something like “Allen, you were born with this rare liver disease, that can often lead to overwhelming lung impairment. What we need now is finding ways to maintain your best quality of life. For you, a liver transplant is one of those ways.”
Over the next three years, I continued working at BellSouth at a fast clip. In fact, I was actually promoted to the next level of management during this period, and my boss was well-aware of my looming fate. He made me focus on my health, my job responsibilities and my quality of life. We worked through the many hours I spent in doctors’ offices, blood labs and breathing chambers to be sure my work got done. Keeping me focused and positive was essential to my self-confidence.
Among the ‘speed bumps and barbed-wire fences’ along the way, my nausea escalated to cramps. The breathing required new and more powerful inhalants. My spleen ballooned painfully into my rib cage. My abdomen became bloated with a weird kind of water. Because of my declining muscle mass, my golf game became a joke, and my jogging landed me face forward, caught by my stung and bleeding hands, on the street’s asphalt…near my house. My esophagus started bleeding internally and rubber bands were used to prevent further bleeding from killing me. My blood-test indicators got further and further out-of-ranges and in several categories. And worst of all these ‘sharp arrows’ was the day I began literally losing my mind…short-term memory, gone, recognition of familiar places & people, gone, and a loss of sound judgment. It was then I let my boss know, as agreed long before, I could no longer make the contribution to BellSouth that he expected. I went home and rested. I had been on the liver transplant waiting list for only 3 days before leaving my job. At home, I found the memory and judgment losses were extremely frustrating, but not as critical to my functioning…as at the office. This was tiring, this was scary, and this was…THE UGLY.
THE GOOD
Thirty-five days crawled by, after retreating to my blurry castle. I thought it was going to be 400 more days, give or take a few months, as was the norm before being called for surgery. I would get a pager soon, to be found whenever and wherever. Then suddenly, the morning of the 36th day after being at home, the phone rang. It was a nurse from Vanderbilt. My ‘new’ liver would be waiting that afternoon. It was the right blood type, the right size and the right moment…wow, what a moment!
My wife and sons were gathering enough of their clothes and other necessities to settle into Nashville for several days. My parents, just 17 miles from our home, picked me up and off we went onto the highways to Nashville. Nearly 300 miles zooming north, I kept thinking about that dreaded IV (intravenous conduit for speeding along the effect of drugs), poked into my clammy arm for hours, even days at a time!
I had always hated needles. Little concern for the 13-inch inverted smile about to be incised into my watery belly, with it all held together for nearly three weeks by 42 jumbo staples. It was the thought of that invasive IV poking through my skin to deliver a staggering array of drugs…that really terrified me!
We arrived at Vanderbilt in mid-afternoon and I was RUSHED through admissions, highly encouraged to disrobe rapidly, sling on the flappy gown, and hop backside-down on the rolling table with the really big wheels. One of the reasons I was rushed through admissions was due to the plan of my signing a clipboard-full-of- forms as I was rolling down the hall toward the operating room. Hey, this must be how executives feel conducting business in their limos!
When I woke up from the 8 hours of surgery, I felt only a tightness in the area of all the action. In fact, and I’m not making this up, I thought I was still waiting for the last drug to really put me under! I think the nurse forgot the part about counting backwards from 100. I just soared into that painless state of solid nothing. And next thing I know, I’m looking up at lots of men and women in lab coats smiling like they had just passed their medical boards! And all I could say was “AMAZING”…about 8 times, once for each hour.
Recovering from all this was nothing short of miraculous. I was eating steak within hours, walking by the 2nd day, and back home to Peachtree City, Georgia, in less than 3 weeks, and it was the same wonderful place I remembered before my hepatic encephalopathy, before my loss of memory and judgment.
Instead of being a “ZZ” in the frightening fog of alpha-1, I was an “MM”. My new liver was perfectly healthy. My antitrypsin, like gas at a truck stop, was flowing upward as it NEVER had before. Remember, I was born with very little of it flowing as intended. And now, I had it all. Yes, and this was…THE GOOD!
Allen Russell
Fall, 2007
