You'll never walk alone...

And so, the time quickly came for the Liver Life Walk.  Armed with a new attitude, and feeling pretty well, I prepared myself to give it my all.  With the help of many generous friends and family members, we were able to raise a nice sum for the Liver Foundation.  And on the day of the walk, I had several people come out with me to support the effort.  It was their inspiration that allowed me to do my best to walk a full lap of the entire course.

It was a somewhat cool day, which certainly helped, but the sun was shining and I was surrounded by family and friends.  As we walked the course, I did what I could to keep up, but as is typically the case, I soon fell behind the pack.  But that wasn't going to deter me -- I was going to finish that course one way or another!

Well, with my kids around me, I did finish the course...  and went to sit down as I really thought I just may pass out!  But sitting for a while while everyone went back for a second lap helped, and soon I was feeling just a bit stronger...  And certainly began to feel more and more proud of the accomplishment.  I realized quite acutely, however, that there was no way that I could have done it without them.  And that is yet another debt that I realize that I may never be able to fully repay...  But I at least hope to get back on my feet soon so that I can try!

Run, Forrest, Run!

One of the hidden benefits to having a condition like mine is that you soon learn a lot about the people around you.  For the most part, every person that hears the news, will share their concern... and then run.  But it isn't exactly like you might think.

Some folks, whether they are afraid to confront their own mortality by staying by your side, or whether it is for some other reason, will run away.  Some will disappear fast, and others will just slowly let themselves fade from your daily existence.  They will say the right things, and send their best wishes, but that is about all you will ever hear from them.  No more offers of getting together for lunch, or an evening out, or a round of golf.  Sooner or later, they don't even exist in your world.

On the other hand, other people, when learning about the condition, will also run... directly towards you.  When you are sick, they will make the phone call or send the email to check up on you.  They will watch out for your family when you are unable to.  But most importantly, they are simply there for you -- whatever you may need.  And even if you end up never having to rely on them, it does you a wealth of good just knowing that they are there. 

When you're in my condition, it can become very isolating.  With nothing to really do but watch SportsCenter and watch the clock tick away the hours while you are fighting to stay awake, it is extremely easy to give in to those feelings, causing depression and perhaps allowing the disease to progress more freely.  But instead of feeling let down by those who ran away, you need to focus on all of those who ran towards you -- Because you now know who your friends really are.  As far as the others -- Let them go.  They aren't worth your time.

"Life will teach you the lessons, it is up to you to learn them" -- Anonymous

I was soon transferred to a regular room, and as the time went by, felt increasingly strong.  With my medication changed, I felt stronger and stronger.  As a matter of fact, I felt better than I had in months!  With the new energy, and a new focus, I couldn't wait to be released from the hospital and get back home.

One of the insidious parts of liver disease is that, until you get extremely sick, you appear to be in pretty good shape.  I've heard many people describe liver patients as the healthiest looking people in the hospital...  But underneath the appearance can be all of the symptoms, including severe fatigue.  So, as I prepared to go home, with an adrenaline rush going and even a small bit of more energy in reserve, I felt unstoppable.  So much so, that within a few hours after getting home, I was able to kick a soccer ball around with my boys -- We hadn't done anything like that in a long, long time.  And it felt great!

It was then that I learned that adrenaline will only take you so far...  I had bounced back well, and still felt pretty good.  But even the short game with the kids would force me to rest, or even sleep for hours.  However, I certainly wasn't going to complain -- A bit of energy was better than none!

The next few weeks and months began a learning experience for me -- A lesson that I still hate to admit that I am still learning.   I could no longer just do whatever I wanted, whenever I wanted.  Many days, if I went to one of the boy's soccer games, even the walk to the field and back would force me to be so tired that I wouldn't be able to do much else for the day.  Limit some activities, so that I could participate in some others -- There was no option to just do them all...  And, while I am beginning to understand the big picture a bit better, I really, really hate it.

"Get busy living, or get busy dying" -- The Shawshank Redemption (1994)

As I wrote earlier, it is nearly impossible to get any rest in the hospital.  And so, as that morning went by, I became restless.  By this point, my potassium was trending in the right direction, as was my ammonia level.   I knew that I only remained in the ICU as a precaution, so there really wasn't too much to stop me from doing what I did next...

I pulled out my laptop and started working...  Well, as best I could given the circumstances!

Well, needless to say, this was something that they hadn't seen before in the ICU.  While they do have wi-fi, the staff was more used to seeing it being used by family and friends, as opposed to the patients.  I couldn't do a lot, but what little I could do felt good -- At least it kept me busy.  And then I noticed something on an email sent to me...  Something that would change my focus...

The American Liver Foundation was having a Liver Life Walk to raise funds.  It was still a while away, and it gave me a bit of perspective. I would soon set a new short term goal for myself...  I was going to get myself strong enough to participate in the walk -- Didn't know how fast I could go, or how far I was going to get, but I would be there.  And thus began a new way of looking at my situation.  Instead of reacting to each new symptom, I would do my best to be more proactive -- Even if it was only one small event, I was going to take back a bit of control over the situation!

And, soon after I made that decision, as if fate were already beginning to intervene, my nurse walked back into the room.  My latest tests showed that my creatinine level -- the indication of damage or issues with my kidneys, had started to change direction and was, ever so slightly, turning in the right direction.  And while mind still wasn't fully back to "normal", I knew that I had made the right decision...

Do what you love, and you will never work a day in your life...

As I sat in the ICU that day, I realized that there was another "type" of health care worker that I hadn't really thought of.  My nurse in the ICU that came came in and introduced himself, and I soon began to see that this would be a very long day...

He walked in, and introduced himself, telling me that he would be there until that evening.  Very nice person, and from his appearance I surmised that he may have a military background.  "Good to see", I thought, "He'll keep things in order!"  Well, I sure that it was his intent, but I could soon see that, try as he may, his execution could not match his effort.

In the room next to mine was, as I began to gather, an older patient who, whether her condition was acute or chronic, had lost touch with reality.  And, it seemed, every 5 minutes would push the call button for the Nurse, without any real reason to do so.  Most certainly not her fault, but, I can imagine, a stressful situation to all involved.  And while most of the personnel seemed to have developed some pretty strong mechanisms to deal with the stress, this particular nurse had not.

With all of his good intent, he begain to get increasingly frustrated with her.  And while it really didn't affect the level of care that he was giving, the stress was clearly wearing on him.  While others would walk into her room, take care of an issue, and walk out, the next step was key.  The others would, if necessary, stop for just a moment and take a deep breath, or speak with a colleague before seeing their next patient.  And when they did walk into the next room, you couldn't ever tell that they were stressed in the first place.

In the high-paced world of the ICU, it would seem that those who last long term, and who truly enjoy their work, are those who can manage to keep on smiling throughout their day -- No matter what.  For that reason, I most certainly hope that he has since learned the lesson and can deal with his stresses better, or that he has transferred to another type of unit. As I have learned long ago, life is far too short to devote that kind of energy to a job that you don't really enjoy!

Here comes the sun...

Daybreak in the ICU is an extremely perplexing time.  While most workplaces have a certain rhythm that they ramp up with, it seems as if the ICU's rhythm is more like a drag race.  While I am not, personally, a racing fan, I remember a quote once made by a driver involved in some version of the sport -- He said it was akin to "getting hit by a cement truck from behind".  Just when you think that the activity has ceased in the ICU, BOOM -- things are flying again!

And so it was that morning.  Just as the sun came out, the noise level increased exponentially.  And the flow of personnel began the steady stream of trips in and out of my room.  As some tried to straighten out my medications, others began to bring me results of the tests being done.  First, the great news was that my cardiac rhythms were again back to normal.  Whew -- One bullet dodged.  Then came the news that my potassium level had stopped rising -- It wasn't near normal yet, but was trending in the right direction.  Yes!  Two for two, and I began to feel like Superman... Faster than a speeding bullet.  Nothing could get me!  I was clearly invincible!

That was the start of one of the most difficult lessons to learn about being ill.  Never get too high about hearing good news, and never get too low after hearing the bad.  And I had taken the bait of the good news, hook, line and sinker.  Of course, the next set of results (which, by this point, I had forgotten even existed), showed that I was still in renal failure, and, although they assured me that they still hadn't seen anything indicating lasting damage, they were going to bring in some specialists -- Just to be sure...

Thanks for visiting us, sir.  Now here is your to-go bag... Full of Kryptonite!  You may want to avoid contact with the high tension power lines on your way down...

So there I sat, still in shock about the sudden turn of events.  I knew that things would probably work out, and yet there I was, like an idiot, unable to believe that I would have let myself get fooled that badly.  Never again, I promised myself.  Never again.

The hospital is no place to get some rest...

They say that, when you really need rest, the worst place to be is in the hospital.  Of course, the first reason is the constant disruptions that only seem to occur just after you have fallen asleep.  Blood pressure, temperature, blood draw, medication... the list goes on and on.  Believe me, I know that there is a reason for them -- I just wish that they weren't absolutely constant!

The second reason is that you are truly out of your typical surroundings.  Not only is the bed different and you are "tied down" with countless amounts of tubing and monitor cables, but you are trapped in a small room with new rules...  What you can and can't do, when to get up and (try) to go to sleep, when you can and can't use the restroom, and having to ask permission or request assistance for every little thing...  The list goes on and on.  As someone who places a high value on my independence, this is especially difficult for me.

The final impediment to sleep, in my opinion, is the noise level. However, it's not necessarily what you think.  Sometimes, the loud noise is unbearable.  People rushing around calling out to each other, procedures being done, other patients in pain or who aren't coherent, and the constant whirring of an infusion pump or other medical equipment.   But at other times, I have found that the exact opposite is true.  The eerie quiet can get to you in the middle of the night just as much as the loud noises.  And that was the case that evening in the ICU...

I know that the ward was busy that evening, but you couldn't have told that from being inside of my room.  As much as I desperately tried to avoid it, I was again alone with my thoughts, and that was not a great place to be that evening.  Thankfully, my nurse soon made her way back into the room.  She was very perceptive, and could see that I was lost in my world of "what-if"...  

She quickly took control of the situation, asking about the transplant, about how I had gotten to this point, and again asking for more details about my disease.  Under most situations, I would have been tremendously annoyed by someone marching into my room at around 2AM and insisting on drawing me into a conversation...  But on that particular night, we sat for what felt like over an hour, and although the conversation involved my health, my family, and my future, the thoughts were kept far more positive than the ones that were running through my mind earlier.  And soon, I began to feel much calmer, and better.. Not necessarily due to any medical procedure, but rather due to a caring individual who, instead of taking a long break when things quieted down, decided to truly watch out for the interest of her patients.  She wasn't just a good technician, nor a good nurse...  She was, without question, a good person.  And I hope that she is aware that others feel that way about her.

And now, a word from our sponsor...

I'm sure that those reading this blog have come to realize that even when I "catch up" my blog to the current day, my story will not yet be fully told.  In "real time", I am still waiting for my transplant.  However, since the idea to put this journal together did not strike me until recently, I am still working on detailing past events, instead of relatively recent ones, as will be the case at some point soon.  And since my blog entries up to this point are not simply random events as the time passes, but more similar to chapters of a story, I would be remiss in not pointing out to those new to reading blogs, that the best way to understand the big picture is to start from the oldest entry, and work forward through each post to the newest.  That may help my ramblings make a bit more sense!

That said, I've got something that I'd really like to say about my current state.  The last few days, for really the first time, I have really been getting a bit low.  Don't know if it is the holidays, if I'm just wearing down a bit, or if it is just the natural progression of this lovely disease.  Regardless of cause, I'm not typically the "woe is me" type.  And needless to say, it's no fun.

It is at this point that I have most noticed an unusual phenomenon that I truly didn't anticipate.  While I certainly hoped for and deeply appreciate all of the support of my wife, family, friends, and neighbors, there is one group whose support I didn't really anticipate -- at least to the extent that it has so far been shown.

I am, hopefully, about to join an extremely select fraternity -- A small group of those who have, through the benefit of a generous donor and a skilled medical staff, received a second chance at life.  And it is those folks who I have significantly misjudged.

You see, I anticipated that, when I came into contact with this group, I would get that gloom and doom attitude that I really am not a fan of.  However, I have found that, to a larger and larger degree, those who have been down this path before me not only are appreciative of the gift of time and life that they have been given, but are quite ready and willing to generously give back their advice, support, and anything else within their power to those who are in the process of "joining the club".

For those who have taken the time to email or post comments here, and for those who I have come into contact with through online and other groups, this is directed to you and those like you.  I owe you each a heartfelt thank you.  I also acknowledge that the debt I have incurred to each of you cannot possibly be paid directly -- and that is why, once I am back on my feet, I intend to do all within my power to raise awareness about, and assist in supporting, those who may be the new members of tomorrow and beyond.

For those who have not yet advanced to the point where I currently am physically, I cannot encourage you enough to open up and listen to the people who have been there before.  Do not let your pessimism about their intent or attitudes cloud your judgment, as it did mine.  This is not to say that all will have good advice for your particular situation -- but rather to encourage you to at least open a discriminating ear to try to separate the advice that may be misguided, or that you do not need or desire, from that which is invaluable.  Do not, as the old saying goes, throw away the baby with the bath water!

OK -- 'Nuff said.  Back to my story...

Perhaps the healthy option wasn't the best option...

Back to the order...  I had ordered up a chicken fruit salad, which had romaine lettuce, grilled chicken, and many types of fruit -- Honeydew, cantaloupe, oranges, and bananas.  Now, in retrospect, I can recognize the commonality between these fruits -- All have HIGH POTASSIUM levels.  But at the time, I wasn't really with it enough to think about it.

So, I began to eat the salad...  And within about 15 minutes, I became dizzy, and started to get extremely nauseous.  Turns out, your body's protection against more potassium when it has too much already, can be sending it back out the way it came in!  And that, my friends, was what was about to happen here.

I stood up to try to reach the bathroom, but was too dizzy, and knew I couldn't make it.  I grabbed a small basin from the table next to the bed (knowing full well that it wasn't going to be big enough, but beggars can't be choosers)!  As I started to return my dinner, the Nurse's assistant that I had given such problems to was walking by the room.  Like a true professional, she ran for the room, grabbed a larger basin, swapped the two with me, and make sure that I didn't fall off of the side of the bed -- All in one quick move.  Truly impressive, especially given the problems that I had inadvertently given to her earlier.

When all was said and done, I gave her my most sincere thanks, but she assured me that it was just her job -- Imagine that?  I then knew immediately that this young person would become one of the great ones -- After feeling startled and upset about my reaction (although not a bit angry or upset with me, only for me), she then did not hesitate to help me at a time when most of us would have turned and walked the other way.  Kudos to you, my friend, and know that your hard work that night was appreciated!

Quiet at last... But was that good or bad?

With my wife and sister gone, and the nurse having stepped out of the room, it became eerily quiet.  My mind wandered over all of the things that I was thinking in the ER -- How did I end up here?  Why me?  Would my heart or kidneys end up damaged, and what could I do if they did?  And, even though I knew it wasn't in everyone's best interest, I really wanted my wife back to go through this with me.

The ER Staff had started an IV, but were not giving me a tremendous amount of fluids.  Therefore, I was just as dehydrated, if not more, than when I came in...  And since hospital rules require 2 lines already set up and in place for each patient,  I knew what was coming next.  And although I'm not a big fan of being poked, I usually don't mind it too much.

However, when I get dehydrated as badly as that, my veins tend to roll and it becomes almost impossible to stick me without considerable pain.  Therefore, when the ER gave it a few tries and failed, they sent me up with only one.  Hence, the new nursing assistant now stood at my door, carrying all of the necessary needles and tubing to get the line in.

"Ready to give this another try?" she asked?  "Sure.  Why not?"  I answered, but first confirmed with her that she knew about the dehydration and the veins.  She had also clearly taken the time to read my chart, so she was well aware and I could tell that she was trying to be as gentle as possible.

"Little poke here...",  followed by "Hmmmm".  A sure-fire indication that the vein had rolled on her.  Words that I have heard many times before by this point.  We laughed, and she admitted that I called it right!  She soon tried again, and again was being very gentle.

And then, it hit me like a ton of bricks...  I know that my arms get very sensitive when they are dehydrated, and I'm not sure if she just missed, if the stress of the whole situation just got to me, or if she hit a nerve, but whatever it was the pain was excruciating.  It startled me, and I jumped and shouted out in pain. The nurse, being very young, was also startled, but managed to quickly calm herself and pull the needle out.  She apologized over and over -- and knowing that it probably wasn't her fault, I told her not to worry about it at all.  However, I could tell that she was a bit shaken by my sudden outburst, and I felt pretty bad about it...  Soon after, the nurse came in, and after a bit of prodding (painful, but nothing like I had just felt), she was able to get a line in, but just barely.. And so that task was given up on for the night.

I hadn't eaten in hours and hours, and the doctor had OK'd me to get some food.  The Henry Ford Hospital near my home is a beautiful new facility, with all new large private rooms, and many innovative features.  One of the features is that the kitchen is open 24 hours -- and the patient can call any time to order a meal from right off of their menu.

I opened the menu looking for something light.  I ordered it up to the room, and they told me it would be there within 30 minutes.  About 20 minutes later, a cart came up to the room with the meal that I had ordered.  Certainly not the only reason to choose a hospital, but a very, very nice feature nonetheless.

And then I started eating the food... But perhaps I should have considered my options a bit better...

Delegate, Delegate, Delegate...

I'm now to a point in my story that I know some of you may disagree with.  You see, after just a few moments of sitting, I threw my wife and sister out of the ICU.

Yes, you read that right.  I didn't want them to hang around there, although I'm sure that they were both more than willing to stay.  Why?  Well, let me explain my reasoning:

My wife and I have two wonderful boys -- Ages 7 and 11.  I like to tell people that the older one is going to be a brain surgeon -- But he will be the most miserable brain surgeon that you've ever met.  He worries about EVERYTHING --   just can't let things go, regardless of what we do.  Of course, until his teachers meet us, I'm certain (many have admitted it) that most think that we must be incredible taskmasters, always hounding him about better grades, not accepting a "B" -- ever, etc...  He just keeps the pressure on himself... Just like his Mom.

My 7 year old could not be any more different.  He is probably going to be a truck driver...  But he will be the happiest, most intelligent truck driver that you will ever come across.  He picks up on things so quickly and easily, but he hates to admit or show just how smart he is.  I'm pretty sure that someday, when I'm gone, he'll be living in his brothers basement, eating his brother's leftover pizza and playing his video games... And having a ball doing it!  In terms of attitude, he takes much more after me -- Pretty laid back unless absolutely necessary.


What does this have to do with kicking them out?  Well, my wife, who also worries like my older boy,  puts so much pressure on herself to be everything for everyone.  And the boys know it, and are quite used to it.  So any disruption in their schedule can drive all three of them up a wall and send our little section of the universe into complete and utter chaos.

So, I have taken to delegating.  When she wants to visit me in the hospital for hours, I refuse.  Although I love seeing her and the boys, I know that, for the time being, their day-to-day well being is more important.  So, although our family, friends and neighbors have all offered numerous times to watch them, I try to keep it to a minimum -- only when we really need them to. While I'm in, I delegate the responsibility of the boys solely to her, and my responsibility is solely to take care of myself -- without expecting  a great amount of assistance from her or others.

There are three reasons...  First, so that all of their schedules remain similar to normal, and so that there is a bit less pressure on my wife because of it.  Second, because quite frankly, I REALLY hate to impose on people.  Again, the less, the better.  And Third, because I know that someday, I will be having an extremely difficult surgery, with a good deal of healing necessary.

My pulmonologist calls the transplant the "failure" option -- Because that means everything else that they have tried hasn't worked.  Knowing all of this, I intend on having my wife by my side 24 hours a day -- I know that I won't make it through without her support.  So if we can minimize all of the disruptions for the kids until then, it certainly will be better -- Because at that point they will have a TON of disruptions, and for as much as I love them, I'm pretty well certain that I'm going to be pretty greedy with her time, and take advantage of ALL of the offers of those who has offered to watch them, drive them to see me, and make sure that they are OK...

So, for now, I figure that, while I'd rather have them here with me, I can put up with all of this alone -- But only because I know that sooner or later, I'm gonna cash all of these markers in and expect payment in full!

Let me know if anyone has a different take on it -- Strictly my opinion!

Uhhhh... Any chance we could get there a bit faster? PLEASE?

So, I was loaded up with everything I had with me, and finally was on my way.  And although I was enjoying being out of the ER, and getting a bit of air, my mind was still pretty preoccupied with the medication that I had just been given, and the inevitable results.  So, in the nicest possible way, I did ask the kind people transporting me to "step it up just a bit".  Well, I think we got there in record time, and out of the stretcher I went -- Into the restroom.

As I emerged, I saw that my new nurse was patiently waiting for me.  "I thought that they had all of the monitoring hooked up on you?  Well, no problem.  We'll get it done now.  But are you supposed to be wandering out of bed?"

I smiled and uttered just one word.  "Kayexalate".

Ahhh... She nodded and began to laugh.  And from this point forward, this kind person was a wonderful source of comfort as I got acclimated to my new reality -- the ICU.  She had already had the opportunity to look over my records hours ago, and although she knew nothing about Alpha-1, she seemed anxious to learn.  The more I explained, the more questions she asked.  Again, my mind was distracted...  to the point that I had almost forgotten about my heart and kidneys, and especially that my wife and sister were patiently waiting outside the room... And (I'm sure) had all kinds of nightmares running through their minds about what was taking so long.

And here's your parting gift, Sir...

Most people who have ever gone through an incident like I was experience (extremely high levels of Potassium in the blood) are going to shudder when I mention this next word...

Kayexalate.

Now, I thought that living on my own as a bachelor at school exposed me to just about any type of horrible, demonic, vile substance known to man.  How many times did I hear "Dude -- just eat it.  It's not THAT old...", as I tried to search through my sofa cushions to find enough change to get something else for dinner -- Even if it was Ramen Noodles.  ANYTHING to avoid the month old "mystery plate" in a college student's fridge.  But unfortunately, too many times necessity became the mother of substances that used to be referred to as food, and I caved in.  Not sure how I survived, but I did!

Now, with this kind of background, you would think that no kind of medication can possibly scare me.  Kayexalate (along with one other, which I will discuss later), are the true exceptions to the rule.  To those who have taken Lactulose, the syrupy substance that I discussed earlier, Kayexalate is just like Lactulose -- on steroids!

Yep -- Same side effects (it binds with the Potassium in your intestinal tract and helps escort it out!), with a far, far, worse taste.  If this wasn't originally discovered as being used by some third world culture as a torture device, I would be really, really surprised.

And, just as I'm getting ready for the trip to the ICU, in they come with this evil brew.  I somehow choke it down, and almost immediately I begin to feel the churning and rumbling in my stomach.  Within 5 minutes, I begin to realize just how quickly and viciously this substance works.  And that, of course, is when the nurse finally comes back in to get me so that I can be transferred.

And the only benefit to this caring and wonderful parting gift from the ER?  At least it took my mind off of what was going on with my heart and kidneys...  Even if only temporarily!

“Don't be reckless with other people's hearts, don't put up with those who are reckless with yours.” -- Mary Schmich

I sat in the ER with my diagnosis, and knew that they had already run a cardiac test.  They again assured me that the reason for me going to the ICU instead of a regular room was in order for them to monitor my heart on a continuous basis -- The sooner that they knew about any potential damage, the more that they could do.

And so, I waited...  And waited...  And waited...  And as I sat there waiting, I realized that there was something wrong with this entire picture.  We've all seen the movies and TV shows where the patient gets thrown onto a stretcher and the transportation staff run all the way to the ICU.  Now, I know that my condition wasn't good -- But as there was no damage yet, this was really a precaution in case it happened before they could get the Potassium level down.

So I didn't really expect any extraordinary rush, with people screaming for items STAT and pushing each other out of the way to get their procedure done.  However, I did expect, as I think is fair, a reasonable level of care and concern.  After all, this was my heart we were talking about, and again, this could have significant effects on the transplant.  So I began to wonder...  If I am going to the ICU for constant heart monitoring, and I already have the contacts on from the first time that they ran the ER heart test, why were the contacts not connected to the ER monitoring (which is very similar to the ICU)?  We asked a few times, and no one knew the answer, but promised that they would "get the nurse right away"!

In retrospect, we SHOULD have demanded an answer.  Over and over again.  It was my right.  But fool me once, shame on you.  fool me twice, shame on me.  I will not make the same mistake again.

SIX hour later, without a single further check of the condition of my heart, I got the news -- Transportation was taking me to the ICU.  And now, although I am not a tremendously religious man, I began to pray that no damage had occurred during that long, long wait...

"Tears are the safety valve of the heart, when too much pressure is laid on it." -- Albert Smith

However, it turns out that the Potassium wasn't the other medication causing issues.  Apparently, as I had gone through each step of the process, medications were added, without careful checking of those that I was already one.  I had ended up with multiple medications for the same symptoms, and despite having the list in their internal system, as well as with me at all times, no one had noticed.  AAARRGGGHHHH.....

So, as I sat there, I began to think about the implications of all of this.  Remember, they were just doing the evaluation to get me on the transplant list, and then... all of this.  If there was lasting damage to my heart or kidneys, regardless of the prognosis for them, would that disqualify me from the transplant list?  Because, without an eventual transplant, I am quite aware of my prognosis -- and it is not a good one.

Realizing the potential of either the lasting damage or disqualification from the list, I soon pretty much fell apart.  A diseased liver, you see, can also lower your testosterone levels (perhaps this is why I now seem to fall apart and get emotional at every little thing!).  So there I sat in an ER bed, a fully grown, reasonably well spoken 43 year old man, blubbering like a three year old, and not being able to pull myself back together. 

Soon the hepatologist came over next to me and put her hand on my shoulder.  Since I was still trying to pull myself together, my wife explained my concerns.  She assured me that they would take good care of me in the ICU, and that they weren't noticing any evidence of lasting damage to my heart or kidneys yet.  She promised that they would watch closely, and I managed to calm down a bit...  And so they left the room, leaving me in the hands of the ER staff to get me safely to the ICU...

"It's not your fault" -- Words without much consolation, at the time...

And so, the surprises began to come.  First, the two hepatologists came into the ER.  They came in my room with a look of concern on their faces, and a chart in their hands.  The Doctor who was clearly in charge introduced themselves and began asking questions about my medications.  In my current condition, I was not a tremendous help in providing information about the medicines, so my wife was kind enough to take over.

The hepatologist was clearly upset over the chart as she reviewed it.  She would point with her pen, point again at another area, and then look over at her colleague, shaking her head.  "Why is he taking THIS medicine along with THIS one?"  She would ask.  Finally, my wife spoke up -- "The list is in your system -- it's what he is supposed to be taking".  She nodded and looked back up...  "I know", she said "It's not your fault.  He's doing what he is supposed to be doing.  We just need to make some major changes to these meds."

The first thing that went through my mind was one med in particular -- The Potassium.  As you may recall from an earlier post, the ER Doctor had prescribed it on a regular basis because she felt the level was low.  And now, the cause of all of the new symptoms and concern was... Potassium.  Even in my state, that all was coming together, and my favorite ER Doctor had made her mark once again -- and not in a good way, nor had she affected my treatment for the last time...

There were a few lessons learned that day that will not soon be forgotten...

My head certainly wasn't totally clear after receiving this news.  However, I was clear enough to know exactly what was happening around me, and, in retrospect, what I learned that night (almost) made going through it worthwhile.

First, I was fortunate enough to have my wife and her sister with me there.  While she is not "technically" my sister, I am proud to claim her as such.  There were many others, including my wife's parents (who again, I claim as my own, whether they like it or not) and friends who sent support and offers of "anything at all that we needed".

If you are a patient, you probably understand, as I had been reminded that day, how everything that we take for granted can be taken away from us so quickly.  As the saying goes, "Man plans... and God laughs".  Do not -- I repeat, DO NOT -- take this for granted.  Tell your family and friends how you feel about them.  Hug your kids... over and over again.  I know it sounds ridiculous, but participate in a "random act of kindness".  In short, PAY IT FORWARD.  You won't regret any of it.  You have my guarantee.

Second, remember this -- Any health care worker who cares at all about what they do does not intend to do you harm, or endanger you in any way intentionally.  These are the folks that truly want to go home after their day of work, doing things that most of us others couldn't imagine doing, and take comfort in a job well done -- even if it meant just making a small difference in one patient's day.

That is why it is YOUR job as the patient to be involved in your care.  Have your most important medical records organized, and bring them to the hospital if you need to go.  Smile and say "thank you" to the Doctors and nurses when they go out of their way for you...  It makes them feel good also.  And finally, and certainly most importantly, be informed about your care.  ASK QUESTIONS when you don't understand.  There certainly isn't any place to be rude or inconsiderate to them, but be firm, fair, and direct about your concerns.  If you aren't comfortable with the answer, ask again -- or get a second opinion.  Remember, it is your care, your health, your body -- and your life.

Stepping down from my soap box, now...  As I said, there were more surprises to come that day.

"What we've got here is failure to communicate" -- Cool Hand Luke (1967)

Another episode with confusion, another visit to the ER.  But this one turned out a bit differently...

This time, the local ER was busier, so I certainly would have understood if things moved a bit slowly.  However, I was in for a real surprise.  The staff took me back, and I gave my short, concise, and to the point explanation of my condition, as I described in an earlier post (or at least as best as I could do given what was going on with me at that point).  They started an IV, ran a quick heart test, and took blood for testing.  About 2 hours later, the results came back...

We knew right away that something was going on, given the look on the Doctor's face.  They explained that my Potassium levels were extremely high (6.9), and they were causing other complications.  These were:

1. Dehydration -- Turned out to be the least of my concerns.
2. Acute renal failure -- That's right, my kidneys were shutting down...
3. Irregular cardiac rhythms -- Needless to say, the most disconcerting of them all.

I was told  that they were going to have the hepatologists come right away to get their opinion, and that they were very concerned about lasting damage to the kidneys or heart.  As such, I was being admitted to the Intensive Care Unit to watch the levels until they stabilized.  But that wouldn't happen for a while.  Meanwhile, there were more surprises to be revealed in the ER. 

It was at this point that the communication issues began to show themselves -- and it really began to get ugly... 

Is the treatment for the symptoms worse than the symptoms themselves?

OK...  I'm just kidding about the post title.  But I did soon come to discover the treatment for the ammonia buildup / encephalopathy -- And let's just say that it is NO fun.  For those not interested in WAY TMI, go to the next post immediately!

You see, as I posted earlier, the simple explanation is that your system gets backed up, and can't get rid of the ammonia.  The treatment?  In my case, the start is Lactulose, a medication that has two functions:  First, it inhibits the bacteria from your intestines from producing ammonia.  Secondly, it "loosens" you up, pushing everything through your system much more quickly.  MUCH more quickly.

I'm going to avoid the obvious disruptions that this can cause in your day for now.  Before that point, you have to be able to choke down this overly sweet, syrupy liquid multiple times every day.  Even for those with a sweet tooth, this stuff can be nearly unbearable to take!

Next... And how do I put this nicely...  They have to begin the process of adjusting the level given to you so that they can create trips to the restroom three times per day.  With someone with a sensitive system like myself, this eventually meant numerous hospitalizations to finally get to the dosages that would not occupy my entire day, but would still avoid the "back-ups" to my system.  More on those hospitalizations as my story proceeds...

Whew...  Hopefully that was helpful to read for those with questions about this topic, but not too in depth for others!  And so, the battle against the medications continued... Until my next hospital visit, when things got much, much worse...

WHERE IS MY RAZOR?????

My wife is a teacher, so she is home all day during the summer. On that particular Monday, she knew that I really needed the rest, so when I slept right through my alarm, she let me sleep -- The office could wait -- This was my health, right?

I, however, didn't see it the same way. When I woke up late and saw the time, I was immediately agitated. "Why did you let me sleep? I have to go to the office", I said. I tried to hurry to get myself ready to go, and that's where the problem really showed itself.

You see, I have an electric razor that has been in the same spot in our bathroom for about 8 years -- 2nd Drawer down, next to my hairbrush. But that morning, as I spent 20 minutes searching for the razor, including in the drawer, it was nowhere to be found. I walked out of our bedroom, and made it halfway down the stairs to the main level of the house. "Judy -- WHERE IS MY RAZOR?" I shouted, and she went upstairs to help me out and look for it. And, as you can probably imagine, 10 seconds later she emerged from the bedroom, after finding my razor in the 2nd Drawer down, next to my hairbrush.

"Where was it?" I asked. She quietly explained that it was right where it always was -- An answer that, at the time, I didn't believe. I was convinced (wrongly so) that she had hidden the razor to stop me from going to work, so that I could rest. I took the razor, went upstairs, got ready, and went to the office.

Oh yeah -- One more thing... Even after all of that, I FORGOT to shave. Sitting at the office, as that fact finally sunk in, I was convinced that something was going on with me. A quick call to my transplant coordinator convinced me to get back to the ER, which I did. And soon, I was admitted for my first inpatient stay...

Feeling like you are losing your mind? You aren't alone!

As I mentioned in one of the earlier posts, I was getting a bit more "loopy". The confusion and memory loss is, for me, one of the most terrifying parts of the disease. Not only could it progress to a more permanent status, but it really can affect just about everything that you do -- And between my business and my young kids, there is no greater nightmare to me than to be losing any of my mental abilities -- Especially since I haven't got much to work with!

In addition, in my case, it would sneak up on me slowly, and by the time that I may have realized that something was going on, it was already well underway. Thankfully, I now have my lovely wife, my family, my friends, and my business partners all up to speed on it -- And when I start acting that way, they let me know!

I know what you must be thinking -- How does your LIVER affect your BRAIN? The first time that I heard this, before the explanation, I thought that the Doctors and Nurses must have lost it. Once I heard the cause, it became more clear... The liver is part of the digestive system. To put it simply, if your liver is not functioning properly, than you can get "backed up". Since some of the items that the liver gets rid of are ammonia and bacteria, and the fact that they are staying in your body longer can allow them to get to the bloodstream. The ammonia can get to your brain, and cause what is called "hepatic encephalopathy". Again, referred to by us as "getting loopy".

The first time that this got really bad was before my first admission to the hospital. I had worked all week, and Friday I was extremely tired (much more so than usual). I slept a lot of the weekend, and just wasn't myself. A bit confused -- just not really with it. I would struggle to remember specific words that I was trying to use, and then would lose my train of thought. But the worst happened on Monday morning...

"Treat 'em and street 'em", AKA "GOMER" in room 12.

The first thing you need to know as an Alpha going to an ER is that most of the time, they have NO clue about your disease. Therefore, it is up to you to try to describe your disease, symptoms, history, and the reasons for the ER visit to them -- in 25 words or less.

OK, I'm joking about the 25 words... But for the most part, my experience has been that if an ER Doctor doesn't sense something that they fully understand and have a specific plan that they can follow for treating you (eg. Broken bones, bleeding, heart issues), then they want no part of your care. Hence, the mantra "Treat 'em and street 'em". Or better yet, the nickname of GOMER (Short for Get Out of My Emergency Room). These terms typically refer to older patients that continue to "bounce back" to the ER, but my experience shows that they are also being directed at those of us with medical conditions that they just don't understand...

On my first visit to the local Henry Ford ER, the ER doc had no clue about the disease, so I tried as best I could to describe everything. Main issues to be accomplished were: Checking my liver function tests, confirming no serious changes, checking for dehydration, and addressing the cramps, if possible.

On a night where the ER was NOT really that busy, I waited nearly 10 hours before finally telling the staff that I was leaving -- one way or another. During those hours, they had managed to take my blood... And that was the extent of it. Well, then the Doctor was able to leave the break area long enough to give me the following info: She had finally reviewed my blood work, told me that I was, indeed, dehydrated, and noticed that my Potassium level was low, advised me to add Potassium to my daily regimen, drink plenty of fluids, and check back with my primary care Doctor. Unfortunately, this was not my last experience with this particular ER Doctor -- As a matter of fact, if you can believe it, 10 hours of waiting without treatment for the dehydration and finally advice to "take some Potassium" was probably the best experience that I have had with her to date! More on this Doctor as my story continues...

The symptoms begin to get worse...

So, I was on my way through the appointments and testing, and I noticed some disturbing changes going on -- The edema (swelling) in my legs was getting worse and worse, the fatigue was nearly unbearable, and, perhaps most distressing, I was having trouble remembering things and keeping my thoughts straight. As someone who handles large financial transactions, business decisions, and transactions with significant legal ramifications, having my mind getting cloudy (or as my wife lovingly refers to it), getting "loopy" -- more on that later -- is clearly not a good thing!

The first real symptoms that needed immediate treatment came from the edema. My Doctors were walking a fine line to balance the discomfort and issues related to the edema, with the treatment for them (diuretics) to remove the excess fluid. Overuse of the diuretics could cause kidney problems (very bad for a liver patient!), but there came a time when some diuretics were necessary. However, it seemed like no one was certain of all of the medications that I was on, regardless of their computer systems and the lists that I carried with me.

Therefore, the levels of the Lasix that I was taking (or Vitamin "P", as it is lovingly referred to by those who have made all of those trips to the restroom because of it!) quickly became too high, leaving me dehydrated. The first sign of it was muscle cramps throughout my body (but mostly in my feet, legs, and abdomen) that were excruciatingly painful and just wouldn't go away. And soon came my first trip to the Emergency Room since my diagnosis...

Chaos is the score upon which reality is written.

So, you've had your appointments, left seemingly gallons of blood for testing, and lost countless hours of sleep. And then, your transplant team (surgeon, social worker, psychologist, specialty Doctors -- eg. hepatologist, pulmonologist, etc..., and your transplant coordinator) have a meeting, and then you are voted on. To list, or not to list? With any "luck", you soon get the good news -- you are being placed on the transplant list!

At this point, your transplant coordinator will contact you and input your information into UNOS (the website mentioned in an earlier post). The staff there is then responsible to store your information, gather information about donated organs, and find an appropriate match. But what if there are numerous matches? Who gets the organ?

This is where the "score" comes in. In the case of a liver transplant, your score is called a MELD or PELD score. According to UNOS, "The Model for End-Stage Liver Disease (MELD) and Pediatric End-Stage Liver Disease (PELD) are numerical scales that are currently used for liver allocation. The MELD and PELD scores are based on a patient's risk of dying while waiting for a liver transplant, and are based on objective and verifiable medical data."

Translation: These scores are an objective indication of how sick you are. In theory, this is intended to prevent a certain billionaire (who shall remain nameless) from receiving a liver when he is not the most deserving based on purely medical reasons. And after simply writing that, I can already feel the operating system on my laptop crashing and destroying all of my stored information... Just to be on the safe side, please disregard the theoretical reference to any billionaire computer moguls above!

Making a list... Checking it twice...

So, you've made the Dean's list. You've watched a Letterman Top 10 list, written a shopping list, and perhaps, after your diagnosis, you've made yourself a "bucket list" (more about that later). You will soon find out that every list you have made or seen COMBINED all pale in comparison to getting onto the transplant list. So how do you do it?

First, you find a Transplant Center. My choice was Henry Ford. At some point, your Doctor will decide that it is time to "get you listed". This means what seems like an unending stream of tests, meetings, and appointments. Most go into this process thinking that the purpose of all of these is to determine how sick you are, and where you go on the list. For the most part, that is absolutely wrong.

Remember, most Doctors want to heal us and return us to our lives -- every single patient that they see. However, this is where reality rears it's ugly head. While some of the tests will determine how sick you are and how vital it is to get you an organ sooner rather than later, the vast majority are for totally different reasons. First, remember that a hospital is, at it's core, a business. Although the Doctors may not like it, they have to make sure that if they do this procedure, that you will have the ability to pay for it (typically through insurance). Like it or not, those without the means to pay or appropriate insurance will have a much more difficult time getting on the list, if indeed they ever do...

Second, the Transplant Centers are rated on one thing -- Patient survival. Plain and simple -- Was the surgery a success 3, 5, 10 or more years down the road? Hence, they look at things like your heart (can you survive an extremely invasive and difficult procedure?), your support system (do you have appropriate help with your medications and care after the procedure, as well as drivers to take you to appointments?), and your psychological history and status (to be frank, this is a procedure that can drive the most sane person to the brink... But it would clearly be more difficult for someone with these types of issues).

And finally... There is a CRITICAL shortage of organs. While the transplant committees appear to be trying to "play god" with the organs, the real truth is that they have an ethical responsibility to give the organ to someone who has a better chance of a full recovery and a meaningful life. These are extremely difficult decisions -- For example, do they list an 80 year old person who is otherwise reasonably healthy, or do they list a 13 year old who needs an organ but has a vast array of other maladies? Or both, or neither? Having to make these types of decisions are one part of their job that I truly do not envy.

Learning a new language...

So, we began the next phase of my journey, and I began the process of learning an entirely new language -- Transplantese. As a person who had never spent a night as an inpatient in the hospital in my entire life, it was quite overwhelming. But having some documentation from the Transplant Center at Henry Ford, as well as having learned a bit more along the way, I turned back to my old friend, the internet, for advice.

First things first -- What is a MELD score? and how does this mysterious "list" work, anyway? As my education on the web went, these were some of the easier questions to get answered, once I found the right place: http://www.unos.org/

Kudos to the folks that designed this site. For a Pre or Post Transplant patient, or for families, friends or caregivers, this is a well laid out site that provides links for the processes for specific transplants ( http://www.transplantliving.org/ ), a listing of the "rules" that determine who get organs first ( http://optn.transplant.hrsa.gov/ ), as well as huge amounts of data with regard to survival outcomes, waiting lists, and the like ( http://www.unos.org/data/ ). I won't bore you with all of the details, but I will absolutely encourage anyone who is going through this, or has a family member who is, to take a look!

That site will give you some idea as to what the "list" is (they have separate ones depending on what organ or organs you need). But first, you have to get on the list...

Remember watching Charlie Brown cartoons?

As a kid growing up in the 70's, we didn't have the 24 hour access to Cartoon Network or Nickelodeon that kids today have. Instead, when we got to watch a cartoon at any other time than a Saturday Morning, it was a real treat. That was the case whenever they would air one of the Peanuts specials.

Although my personal favorite (and probably that of most 6 year olds of that time frame) was Snoopy, one of the most memorable characters on the show was one that you never even got to see. Their teacher would make appearances on the show, but only with her voice. And, as we remember, it wasn't really a voice. It was more of a "wah waaaaah waaaah wah" sound, but we always could recognize it, and it was extremely funny at the time and in the context.

As my hepatologist left the exam room, he brought in a person that he introduced as my transplant coordinator. Although I'm sure that much of the information that was given to me by her and the staff for the rest of the meeting was informative and helpful, all I could hear was "wah waaah waah wah wah". This was the first time that I had this experience, but later bouts with encephalopathy caused it more and more often (although for physiological rather than emotional causes)! Needless to say, these times weren't nearly as entertaining as hearing it for the first time as a 6 year old. I'll give more details of what led to more and more of those moments as my story progresses...

Please keep your hands and feet inside the ride at all times.

I'm sure that most of us can remember that feeling from the first time we rode a roller coaster. For one reason or another, we felt that it was something we needed to do -- Whether it be showing off your bravery for friends, siblings, parents, the opposite sex, or just to prove something to yourself. But when you got to the top of that first really big hill, you could only think of one thing... "What in the world am I doing here????"

It was almost exactly that feeling that I was experiencing the morning of the appointment. I knew that something simply had to be going on, I just didn't really know what it was. But one thing was certain... There was no turning back now. All I could do was follow the safety instructions given by the 16 year old kid operating the coaster, and simply try to hang on without losing my lunch.

The hepatologist soon walked into the examination room, introduced himself, and sat down to discuss things with me. How was I feeling? What brought me to Henry Ford? And before long, the answer to the question that was running through my mind since the prior day was finally answered.

"I've looked at your history, and calculated your MELD score. We think that it is about time to list you for a transplant."

This brought to mind several things. First, what the hell is a MELD score? And second, what is the "list" all about? But these two questions paled in comparison to my immediate reaction to the news... Was it the "cure" that I had hoped for? Or was it, as my lung Doctor refers to it, simply trading one chronic condition for another? Should I be happy, upset, angry, or all of the above? As it turns out, I just ended up simply feeling numb. It would be quite some time until I could truly process the information that he had just given me, and that his staff was about to walk me through. Call it denial, or whatever you like. I just knew that I couldn't really feel anything.

Can you meet the Doctor downtown instead?

I knew that I could not sever the relationship that I had built with my lung Doctor -- I was certain that I would never find anyone that was a better fit, no matter how long and hard I searched. However, I also knew that I had to begin the process of meeting a new hepatologist, and searching out a new primary care Doctor that could work within Henry Ford's system.

The day for the first meeting with the hepatologist was fast approaching, and now sat just two days away. Although his practice was based out of their main campus location downtown, I had scheduled the meeting at his GI practice location in Novi, a suburb near my home and office. As I pondered what the future held for this new system, I received the phone call that would permanently change the course of my treatment. It was his nurse calling, from the Novi location. After introducing herself, she asked me the following:

"Can you meet the Doctor downtown instead?"

Again, my heart began to race, and my mind was swimming with all of the possible causes for the change of location. As I began to regain my composure, I asked her why the meeting was to be moved. Her answer was "just because he has more staff available downtown than here."

Was that all there was to it? Simply a personnel issue? Or was there more to the story that they wanted to tell me in person? Although I hadn't been sleeping well up until this point, it was even worse that night, as I awaited the meeting that was now scheduled for the following day.

Lung Doc? Check. Liver Doc? Check. Now what?

So, with a diagnosis confirmed by specialists, I brought the results back to my primary care Doctor. You remember him, right? The Doctor with the heart of gold, but no real understanding of the disease. After much conversation with my wife, we finally decided that I would continue to see him as a primary care Doctor. We knew that down deep, he had my best interests at heart. Now, with a team in place for him to delegate those issues toward, I knew that I had to give him a chance.

One of the funny parts about being diagnosed with a disease like this is that your mind can really begin to wreak havoc on your life. Not in any physical way (at least not at that point), but rather the emotional side of things. Any small ache or pain would leave my head and heart racing: Did this have anything to do with my liver? Is it getting worse? Even the most normal, temporary muscle pain that I wouldn't have given 5 seconds to a few months earlier, suddenly seemed like the end of my world.

It is for this reason that I began to have issues with the team that I had assembled. Blood draws done by my primary care Doctor were entered into his system (he practices out of a different hospital), but never made it out of his office and to where they needed to be at U-M. Using a football analogy, what I really needed was a head coach. He would be responsible for the big picture, every day type items. Diagnosing small issues and recognizing when they were really larger issues that had to be dealt with by a position coach (in this case, the lung or liver specialist). Basically, treat what you are capable of doing, but when it goes beyond your level of specific expertise, recognize it and, above all else, DELEGATE!

When it became clear that my primary care Doctor could not play that sort of role, I knew that I had to make a change. My wife and I went back and forth with the staff at U-M, looking for any local primary care Doctor that they trusted to do what I needed, and was within their system, so that they could view test results without unneccesary delays. After several attempts, lost time, wasted effort, and significant frustration, I was at the end of my rope. It was then that I reconsidered my original choice, and scheduled a meeting with a hepatologist from Henry Ford.

Enough with the genetics lesson. What about my kids?

Well, now that we fully understood the genetic makeup, we awaited the results from MUSC. It turns out (thankfully) that my wife was a MM (the "normal" genotype), so the kids are automatically MZ -- No chance of them being anything else. Therefore, I have less to worry about in terms of the testing being part of their medical record. However, we have had a hypothetical conversation with their doctor, and asked her to help with the same types of precautions that any lung or liver affected alpha may take -- more aggressively approaching lung infections, vaccinations, smoke avoidance, etc...

However, the genotype is only half the battle. Given that it is a Co-Dominant gene, you can be an MZ and still have levels of A1AT in your bloodstream that are well within normal ranges. Or, you could have levels that come close to those with the ZZ genotype. Therefore, it is vital to have family members tested for the following:

1. Genotype (eg. MM, MZ, SZ -- This one contains the even more rare allele, the "S" Allele, ZZ)

2. If the genotype is anything other than MM, A1AT levels should be tested.

3. Again, if the genotype is anything other than MM, Liver function tests should be tested and monitored.

Unfortunately, as my experience has shown, there are many primary care Doctors that have no idea as to the existence of A1AT, much less the need for you and your family to be tested. In this regard, we are our own best advocate when it comes to health care. Do not be afraid to ask questions, or suggest possibilities if you suspect something. It could very well save your life, or the life of someone around you!

In the words of Lee Corso... Not so fast, my friend!

Turns out that there was a real flaw to our logic.

As we later learned from my pulmonologist, the genetic structure of A1AT was not the simple dominant-recessive relationship that we all recall. It is instead, what is referred to as a Co-Dominant gene.

What does that mean? Well, let's take the example of brown eyes or blue eyes. If the genetics were a simple dominant-recessive relationship, and A is brown eyes (dominant) and B is blue eyes (recessive), then two parents, one with AA, and one with BB, will each contribute one allele, leaving their offspring to be AB. Since the A is the dominant allele, the offsprings eyes will be brown. They will be a carrier of the B allele, meaning that the offspring's children, or further down the family tree, still have a chance to inherit the two recessive alleles necessary to have blue eyes (BB).

On the other hand, A1AT is Co-dominant. That means that BOTH alleles contribute to the makeup of the offspring. It isn't necessarily a 50-50 split (sometimes 60-40, sometimes 40-60, etc...) but a combination of the two regardless. Hence, using our example, the AB offspring would have some combination of the blue and brown color in their eyes.

Hold everything... I haven't even thought about my family yet!

The entire procedure thus far had been such a whirlwind, that I hadn't really given as much thought as I should have to the genetics involved. As I now realize, the gene that causes A1AD is even more common than the one that causes cystic fibrosis... And yet very, very few of us have heard of it.

When I started thinking about the genetics of the disease, I was drawn back to the basic high school genetics lesson -- the dominant gene and the recessive gene. Since I was a ZZ, it was clear that I would give my two kids the Z allele. But what would they have received from my wife? And how did I get them tested without harming their ability to obtain health insurance, life insurance, or perhaps even employment when they grew up?

You see, our medical community is reaching new levels of understanding our bodies every day, and our society and legislators can't really keep up. So the fear is this: If a person is pre-disposed to having a genetic disease (for example, a ZZ Alpha), but have no symptoms, can a life insurance company deny them coverage? After years of work, the Congress had passed (and the President had signed) a new law referred to as GINA (The Genetic Information Non-disclosure Act). However, until many years pass, and the inevitable challenges of the law are decided on by the courts, nothing is really set. Because of this, I knew that the kids had to be tested... But I still didn't know how?

I received information regarding a confidential testing program done at the Medical University of South Carolina (MUSC). They would test for the disease anonymously and give us the information back directly. So, we sent away for a kit, and my wife sent her test back in to them. Our thought was this: If, by some miracle, my wife was an MM (the "normal" alleles), then our kids would be MZs. By the lessons that we recalled above, an MZ would make them carriers of the disease, but without symptoms, right?

Of course, it can't be quite that easy!

Next stop: The hepatologist!

With an extremely positive experience, as well as a great prognosis, in hand from my lung Doctor, I knew my next step. The U-M hepatologist awaited... And I certainly had a feeling that the news would not be quite as positive from him.

Meeting the hepatologist was, in some ways, less stressful than my pulmonologist. Clearly, he had a tough act to follow in terms of both bedside manner as well as demonstrating his capabilities and awareness of the ins and outs of A1AT. Another Doctor once described giving a speech after my pulmonologist as akin to "coming to the plate right after Lou Gehrig."

With all of that being said, the U-M hepatologist certainly had a good working knowledge of the disease, and came across as very caring and compassionate. He scheduled me for a liver biopsy to confirm the diagnosis, and gave me his best estimate on the progression of the disease. However, even he admitted that this disease is pretty unpredictable, and that the best thing to do was watch my weight and carefully follow the blood work. The only "cure" at this point was a likely liver transplant -- the question was simply when?

While this news was not all of that different from what I had received in the past, I felt much better receiving it from someone who seemed to know much more about the disease. In addition, there were several other steps that I could take: Flu shots, Pneumonia vaccination, total avoidance of alcohol, and, when truly necessary to have any pain relievers, Tylenol instead of Motrin.

Then came the discussion that I hadn't really thought about yet, but that hasn't left my mind since -- And it probably never will.

"You aren't going to die from your lung disease"

As we spoke, he began asking questions... How was I sleeping, was I confused first thing in the morning, did I snore. Questions that didn't really seem directly related to Alpha-1, but being new to the game, I wasn't really sure.

Once we had asked all of the questions that we could think of, and he patiently and happily answered every one, he gave us his prognosis. As long as I continued to follow up with him, and as long as I avoided irritants (smoke, workplace chemicals, etc...) than he was extremely confident in this:

"You aren't going to die from your lung disease"

It took a moment for the news to digest. I knew that my major symptoms were from the liver, but after seeing all of the lung information, I was certainly unsure of how this would play out. His news, however, did come with one caveat: Although I had come to see him about the Alpha-1, he was always trained to spot symptoms of any other issues that he could address and heal. He did highly suspect Sleep Apnea, in my case (hence the "strange" questions that he had asked). He scheduled me for a sleep study, where his suspicions were proven correct. A small hiccup in the healing process, compared to what I had feared.

I have continued to see this Doctor for the last two years, and would HIGHLY recommend him to anyone in the area who needs a pulmonary Doctor, or even a second opinion. I recently heard him speak at an Alpha-1 Education day, and, even after doing two years of research on Alpha-1, he still managed to impress me with his knowledge and presentation skills, as well has his pure humanity. If anyone needs his information, please just contact me!

Finally. A positive experience!

I can still see, in my mind's eye, exactly what I expected. An older male, probably a charter member of the "good old boys network". He would walk in the room, review my file, make his determination as to my condition, and answer only as many questions as he was forced to sit through before leaving to see the next patient.

And then, the door opened, and in walked the Doctor. I could hardly believe what I was seeing... A young man, about as far from the "old boys network" as I could imagine. He closed the door, gave my wife and I a warm smile and a firm handshake, and began to speak with us. Not "to" us, or "at" us, but truly speak with us.

Suddenly, this wasn't "one of the Deans at the Med School" or "the prolific researcher and publisher". He sat down, and asked how I was doing and what brought me there, even though he had already taken the time to review my file. He was truly the neighbor and friend that you would trust, even with your very life.

The physicians that could determine my fate -- How do I choose?

So, I then began the work of trying to decide on a hepatologist and a pulmonary Doctor. My first meeting was with the lung doctor, and I was in for a tremendous surprise!

I had done the research, and, given that I did not have any personal referrals to either specialist, I chose by reviewing each Doctor's online profile on the University website. One lung Doctor in particular stood out, as he had significant research work, and he also had achieved a prominent role within the University system -- Among other titles, he was serving as one of the Deans of admission for the U-M Medical School.

I scheduled the appointment, and my wife and I showed up at the lung clinic. After a long series of tests, I had my results in hand and was ready to see the Doctor. And at almost exactly the appointed time (not 20-30 minutes late, like most every other Doctor I had ever seen), the examination room door opened. I was terrified... How do you introduce yourself to one of the people who could determine your fate, and perhaps those of your wife, children, friends, family, business associates and others. I could only stand there and wait, and hope that I had chosen wisely...

And thus began the REAL search.

I soon received a call from my primary care Doctor. Clearly, he had done some basic research, but still was set on the opinion that Alpha-1 could not be the true cause of my liver issues. Again, lose some weight and things will be better. If it wasn't, than perhpas a transplant was in my future. No real way of telling anything.

And oh... By the way. The blood work showed that I was a ZZ. "What in the world is that?" I asked myself. But I knew better than to really pursue it. As good and caring of a person as he was, I could tell that he, as a Doctor, was in over his head on this one.

So off I went on my quest to find specialists that knew more about this disease, and that could help me manage the symptoms, and perhaps have a bit of control over the eventual course of the disease. I soon discovered that there were two local hospitals that had staff with the knowledge to understand the disease, the experience from having dealt with other patients with it, and the capability to carry the treatment through to the conclusion, if necessary... A liver transplant. It was down to Henry Ford Hospital and the University of Michigan.

In my searching, I couldn't find any real benefits or flaws that would allow me to easily choose one of the programs. They both seemed to have updated facilities in their transplant areas, extremely competent staff, and produces similar outcomes for their patients. Although it isn't the best means to choosing a hospital, since all other items appeared equal, I chose U-M -- First, because the campus is located in Ann Arbor (a much safer area than Henry Ford, which is in the New Center area of the city of Detroit). Secondly, and on a much lesser level, because I got my undergraduate degree there, knew the town, and loved the area.

Alpha what????

It was at that moment that the internet temporarily became both my best friend and worst enemy with regard to the disease. For every helpful post or article that I would find, there seemed to be at least 3-4 written by people who appeared well intentioned, but, in retrospect, knew little or nothing about the topic. I'm not sure which was more distressing... Finding out that I had this mysterious disease, or not being able to trust most of the information that I was discovering about it.

The single, most frustrating part, from my viewpoint, was the fact that nearly all of the information that I found discussed the disease as only a lung disease. Knowing that I had little to no lung symptoms, I couldn't understand how this could possibly be the cause of my cirrhosis. As one educated hepatologist said to me months and months later, while I sat in an emergency room, "Guess you didn't read the textbook, huh?" To this day, that is probably the most succinct, heartfelt, and honest explanation of my symptoms that I have heard. Thank you, Dr. Silverman.

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.