G is for Goals

Of course, given it is world cup time, that should probably be:

GOOOOOOOOOOAAAAAAAAAALLLL!!!!!

How do you have goals with a terminal disease?  The same way you have goals with the fact that you are going to die sometime, just with a shorter time line.  12.5 years ago, I thought I had a roughly 50/50 chance of making it to 10 and I set my goals appropriately.

I wanted my kids to know me and know what is important to me.  Riley was just 4, River was 3, and Forrest was 6 months.  Permanent memories begin to form around the age of 4 and that meant that I had to at least live 4 years for Forrest to know me.  I wanted him to know me as someone who did not stop just because I had been dealt a bitter pill.

People get dealt bitter pills all the time.  Rejection from someone you care for deeply;  Rejection from job applications;  Rejection at sports; Lack of recognition at work.  All of these things have happened to me and they hurt.  I wanted my kids to see someone who rose up in spite of hardship, who rises up in spite of getting knocked down.

I had a goal of seeing my kids into high school and have achieved that for all but Forrest.  He is just a year off and I don't have much worry about seeing him there and will cheer for him loudly when he competes in his first high school soccer game.  I have seen my daughter come out as a beautiful young woman and blow away everyone in her math class as well as to start swimming competitively this year.  My oldest, Riley, is going to be a Senior.  A SENIOR!  I will see him graduate in a year and head off to college to start training for his career in engineering.  I have seen him perform well in soccer, making varsity his first year and I will get to see him lead the varsity team this next year.

I have met my goals of seeing my wife reach levels that I never imagined.  I got to see her complete the Pikes Peak Ascent and earn a medal.  I saw her after completing her first marathon and the rode with her on her first metric century.  I have gotten to hike, kayak (once, don't like the water), ski, see her waterski for the first time and so much more.  My goals of seeing her grow into the person that she wants to be.

I have made my goal of trying out for ski patrol, missing the cutoff by 1.6 points at Winter Park (don't think I will ever be in that good of shape again).  I have completed the triple bypass which I will NEVER do again.  I have excelled at work and been able to perform at the level I want.  I have skied almost 40 days in a year and only have three more ski resorts remaining to ski in Colorado to bag them all.

What do I do now?  What do you do for goals at this point?  I don't think I have 10 years left in me.  My disease is advancing and we have used almost everything in the bag of tricks for my cancer.  Even if I were to wake up tomorrow with no cancer, my deteriorating kidneys do not bode well for a long life.

5 years?  I think that is possible.  I think my chances of another 5 years are probably in the 50/50 range now.  I don't have anything immediately wrong that can kill me but there are a large number of risks in my future.

• My every 6 month ureter swap could throw me into carcinoid crisis and I could die on the OR table.  Low probability here as we have a routine that seems to avoid invoking crisis
• Dialysis will be starting sometime, within the next 6 months to a year I imagine.  That carries a low risk of blood infections and other issues that could cause death at some point but they are also low probability
• Abdominal obstruction is always at the front of my mind.  I am 1.5 years out from the last laparotomy so that frees me from the concern of the surgery causing another obstruction but the tumors will continue to grow and there is a moderate probability of it forcing a laparotomy with all the risks of crisis along with major surgery
• My liver seems to be doing OK for now but those tumors also are growing and we may need to deal with them at some point through another procedure which carries some risk.
• The unknown.  I never expected to lose my kidneys and the unknown factor scares me.

So, what are my goals?

• I want to see all my kids graduate from high school.  Yes, that is right at 5 years but my goal is to see Forrest walk down the aisle, diploma in hand.
• I want to recover some of my strength and weight.  I am almost scarily skinny right now,  6'3", weighing in at 170 pounds and much of that weight might be fluid retention from my ailing kidneys.
• I want to be able to walk up 5 flights of stairs again without having to stop half way to rest.
• I want to walk the quarter mile into and from work every day without wishing I waited and took the bus.
• I want to ride again.  My legs are weak and there is no way I could do the academy loop at all, let alone in the 40 minutes it used to take me.  I want to ride the loop in under an hour without having to stop.
• I want to climb a 14er.  I have been to the top of two and rode my bike to the top of one but I have never hiked to the top of any.
• I want to ski outhouse without stopping again
• I want to have a shutout again.  They are rare as hen's teeth for me but I want another one. Achieved, 8/11/2014
• I want new adventures with my wife.  I want to see things we have never seen and go places we have only dreamed of.

Do I have other goals?  Maybe but these are the ones I see right now.  5 years seems like a long time but just achieving these will be some significant accomplishments for me.

F is for Fight

Cancer patients are expected to be warriors, fighters, tearing into their disease like Beowulf ripping the arms off of Grendel.  You see this in the books about how one should fight their disease and anecdotal stories of how a positive attitude will extend their life (hint, it doesn't).  I feel like Charlie Brown, trying to kick that football one more time but only every couple of months...

I don't fight my cancer.  I tolerate it.  Most of the time, we have an uneasy truce where I ignore it and it leaves me alone.  Most of the time this works.  I am not one of those people who spends time concentrating on chemo and focusing on the radiation attacking the tumors.

That doesn't mean that I pretend the disease doesn't exist and avoid treating it.  I do explore new treatments.  I do take the medicines that have a possibility of working and I do follow the progress of my disease.  I take the steps that are necessary and look for potentials in an analytic fashion but I never think of myself as fighting the disease.

I am proud of what I have been able to achieve in spite of my disease but I struggle with the image of being a fighter.  I have had it easy.  My cancer has been slow growing and I have not had the debilitating side affects that many other types of cancer get.

Please be respectful of those who are struggling with their disease.  Don't tell them that they just need to fight it because that puts the fault of their failure to survive on them rather than something that is beyond their control.  Support them in their struggle.  Sit with them and listen.  Don't say things that imply they are simply not working hard enough.  Be respectful of what treatments they choose because they know what they can tolerate and what they can handle.

E is for Enlightenment

There is something about terminal diseases or near death health experiences that give you a special view into yourself and how you approach your life.   This is true of people who, like me, have carcinoid cancer that will eventually take their lives and it is true of people who have heart attacks, other types of cancer and are either cured or not cured.

These experiences do a funny thing to your attitudes.  You start to prioritize things differently.  You start to think more about what the long (and short) term consequences are for the decisions you make today.  I was always one that was willing to take a day off of work when sufficient snow fell but I also frequently worked like a madman, putting in multiple 80 hour weeks to achieve a deadline.

Work was fun and rewarding but I missed things.  I went on a business trip once over my daughter's birthday.  Yeah, I know she will have many, many more but I missed one for work and the trip wasn't even that important.  I still love many aspects of my job and am blessed with quite a bit of latitude in the projects that I work on and get to work on so many cool things but work is no longer a priority.

20 years from now, no one is going to recall whether or not I worked a particular day.  Even when I am at my best, receiving accolades and kudos for my performance at work, no one will remember that 5 years from now.  On the other hand, being a coach for my son's or daughter's soccer team will be remembered for all their lives.  Taking a day off to play hooky with my son on a bluebird powder day at Copper Mountain?  That is something that will make a difference.

This disease has blessed me with the ability to see what is really important and to focus on those things.  Work, though highly rewarding and satisfying, is no longer a priority.  Family, friends, and memories are what I want.  That is where I want to spend my time.  Coaching and encouraging my wife through her first metric century was one of my proudest accomplishments of 2013.  21 ski days with my family and friends in the 2013-2014 season was outstanding.  Watching my youngest son come within inches of a hat-trick, seeing my daughter excel academically and begin to compete as a swimmer, and watching my oldest son manage the defensive line on his premier team are all things I value now.

Family, friends, and memories.  That is what I want for my legacy.  That is what I want people to remember about me.  That is what will last long after I have become worm food.

You don't need to wait for your near death experience or life changing event to see what is really important.  Love those around you and let them know how important they are to you.  Show them where they rank by spending your most valuable asset on them:  Your time.

D is for Depression

No matter how strong a person is, depression will set in at some point in time.  The first 10 years of my disease, I was able to avoid any sort of significant depression.  Other than the occasional major surgery, the monthly shots, carcinoid syndrome, diarrhea, and other symptoms, I lived a pretty normal life.  I knew I had cancer and that it had a good chance of getting me at some point but this did not affect my day to day living and depression never really entered into my life.

This changed in 2012 when I hit a major bowel obstruction and went on TPN for a couple of months prior to the first of two surgeries, one of which was several days.  I had procedures to address stents in my biliary duct and had two major carcinoid crisis that put me within inches of death.  I lost 50 pounds, looked like death warmed over and still haven't been able to return to a more normal weight.  The colostomy from my second major surgery has played hell with my body image although I am starting to get used to it two years later.

The tumors impinged on my ureters and that has caused my kidneys to begin to fail.  Their slow decline will be leading to my going on dialysis in the near future and this is yet another hit on body image and on my general life and outlook.

All of these things have led to occasional bouts of depression that  have lasted for mere hours to as long as a week.  Sometimes, I just get overwhelmed with everything that has happened to me and everything that I know is going to happen.  I get overwhelmed by the thoughts of things I will miss out on because the cancer is going to eventually get me.  I get overwhelmed by the pressure to be strong and to keep a smiling face.

I hear from so many that they are proud of the way I have handled the disease and how strong I am in the face of the future but sometimes I just need to be weak.  For those times, I am extremely thankful for my wife who lets me lean on her when I am weak, who lets me cry as she holds me and comforts me when the thoughts get to be too much, who I know will always be there for me.  Being strong is an outward face that most everyone else sees and Stephanie helps me stay strong by giving me a safe place to be weak..

This disease will kill me and of that I have no doubt.  I am many years past the point of a medicine finding a miracle cure and have to learn to incorporate that knowledge in my life.  Depression will come again.  Depression will come close to overwhelming me and my thoughts again.  Depression is just another of the battles that I face in my dealing with this disease.

Of course, I am not the only one that gets depressed.  Stephanie has a hard time thinking of the things I will miss, the things I can't do, and how all our vacations seem to be to New Orleans for my treatment.  My kids never admit to it but I am sure that they have the same bouts of depression that I have but their bouts are on a different level.

Nor is depression unique to me and my disease.  I am sure all with chronic or to be fatal diseases have the same issues that I do.  Know that they are struggling the same way that I am with the public face of strength and their private battles with what is inevitable.  Life goes on whether or not we want it to and the fact of or lack of a disease is nothing that affects the ticking of the clock.  Be willing to be there for your friends.  Know that they have struggles with happiness and be willing to stand there for them when they do lose control of that public persona.

C is for Carcinoid Crisis

Carcinoid tumors are odd birds.  They are of a group of cells in your body that are known as the neuroendocrine system.  Neuroendocrine cells receive some sort of signal from your nervous signal and, in response, they release some sort of hormone into your blood system.  Those hormones then stimulate another part of your body to perform some task.

For example, if someone sneaks up behind you in the parking lot and screams loudly, your adrenaline gland releases a huge amount of adrenaline into your blood stream, enabling the fight or flight mechanism.  Insulin is another example.  If you consume a large amount of sugar, your pancreas releases insulin to bring help bring the level of sugar down in your blood to a manageable level.

The neuroendocrine cells from which carcinoid tumors grow are cells that generate serotonin.  Serotonin serves several purposes in body but one of the primary jobs is to regulate intestinal movements.  The tumors don't forget their previous life as neuroendocrine cells but now there are a much greater number of these cells.  What that means is that they still attempt to respond to the signals to regulate intestinal movements.

This results in things getting out of balance.  The tumors will get the signal that food has entered the digestive tract and then decide to start telling the rest of the digestive tract to begin processing the food.  Normally, this is just fine but with all the extra cells, way too much serotonin gets released and the digestive system goes into overdrive, leading to, often, explosive diarrhea.

In the same vein, kallikrene (yet another hormone but an odd one) is released at times which lead to the blood vessels increasing in size.  This affect people notice is that the face and chest will often turn quite red and there is often a feeling of light-headedness that accompanies this along with a drop in blood pressure.  I can usually cause this to happen by walking up 5 flights of stairs and then watch my face turn red 45 seconds after reaching the 5th floor.

Carcinoid crisis is when this happens to the extreme and it usually happens at one of the single most scary times possible.  General anesthesia is the most complicated portion of a surgery and it is one of the single biggest causes of carcinoid crisis.  Under general anesthesia, my entire upper body will turn almost purple, my heart rate drops and my blood pressure hits the floor.

I typically have a fairly low blood pressure (~110/75) but the last time crisis occurred during surgery, I dropped to 75/40 and lower.  Heart rate (usually around 65 for me) dropped into the 40s and I was standing at the precipice of death.  For this reason, I always try to avoid general anesthesia for any procedures and it appears that low levels of propofol are usually sufficient for the procedures that I have been receiving lately.

Sometime, I will need general anesthesia and we have a plan for that as well.  Sandostatin is a drug that seems to inhibit  carcinoid syndrome and is generally given in small doses.  Whenever I do need a major surgery, I will be admitted the night before and then put on a continual high-dose drip of sandostatin.  Just prior to anesthesia being given, I will receive another bolus shot of sandostatin and then the drip will continue throughout the procedure.  This worked for me in my second major surgery of 2012 and, if I have to go under again, we hope it will work again.

If I still go into crisis, the key is to then just start giving massive doses of sandostatin and hang with me because I have always come out of it.  It takes as long as an hour but as long as my heart continues to pump, I will survive the crisis because the body will simply run out of kallikrene.  Whenever I do go in for procedures, even with propofol, I still try my best to scare the anesthesiologist as much as possible and ensure that they have sufficient sandostatin ON HAND in the operating room.

B is for Bowels

Carcinoid Cancer is frequently all about the bowels.  Most carcinoids are detected in the bowels with about 40% being found (like mine), in the terminal illium (end of the small intestine).  Many are found in the rectum, some are in the pancreas and a surprising number of appendectomies are actually caused by the seeds of a carcinoid tumor.

Once the cancer starts, it grows very slowly, sometimes for ten or twenty years before it actually begins to cause any side affects and I have no idea how long mine had been hidden.  What I do know is that it was found in 2001 due to a complete small bowel obstruction and had grown to 1.7cm at that time which about twice the size at which they generally begin to metastasize.  My best guess is that the cancer started growing about the same time I started grad school in 1990 and has been growing very slowly since that point.

Once the cancer metastasizes, it usually stays in the abdomen, preferring to affect any nearby tissues.  It frequently goes to the liver, and often ends up clustering itself around the mesenteric lymph nodes which is exactly the course my cancer took.  When it metastasizes, that is when it actually begins to grow much faster and bigger.  No one really knows why (yet) but it may be that the soil where the cancer first sprouts is not very fertile and the places it goes to are much more accommodating.  This is why I have had 3-4cm tumors throughout my abdomen, even though the primary was quite small and likely would have stayed that way.

Another reason B is for Bowels is the diarrhea that carcinoid syndrome brings with it.  The tumors act like the neuro-endocrine cells from which they were formed and generate all the signals that those types of cells generate.  However, there are now so many more of them and they often act in concert, flooding your body with signals such as "Open the blood vessels" or "Empty the Bowels NOW" and in such volume that your body behaves oddly.  These actions can be somewhat controlled by some drugs such as Sandostatin but there is only so much you can do.

Finally, B is for Bowels because the tumors regularly cause obstruction and ischemia in the bowels.  For me, this has happened four times so far.  The tumors have bound up the small and large intestines to the point that nothing can pass and this has lead to dramatic weight loss.  The tumors have further impinged on the superior mesenteric vein, preventing blood from getting to the intestines so that they can do their work of digestion.

Of course these last two reasons mean I am continually worried about my bowels.  If I am having too much diarrhea, I am frustrated at the bathroom time required and the interruptions to normal life.  If I am not having much diarrhea, I begin to worry that I am starting to obstruct again and that I will be back on the OR table for another extended laparotomy.  Right now, I am in the later camp and am stressed that things are starting to obstruct and don't know how I will deal with yet another 12hrs of surgery and 8 weeks of recovery.

Bowel obstruction is difficult to diagnose in some respects.  There are some standard symptoms but those symptoms could be any of a number of other things as well.

• You will generally have a loss of appetite (Hmmm, that could be my kidney failure).
• You will have abdominal pain (have had that for 13 years from the diarrhea and other).
• You will sometimes throw up indigested food (not having that currently but it sounds like just being sick).
• CT scans work well as long as you can use contrast (can't due to my kidney failure).

ARGH!

Of course, once it does obstruct, what do you do?  Usually it requires some fairly urgent surgery but there aren't many surgeons willing to go into an abdomen with as many adhesions as I have.  If I wait too long, I get weak and surviving/recovery from surgery will be all the more strenuous.  And, of course, there is the voice in the back of your head saying that maybe if you ignore it, it will just go away.

October is 13 years since my first laprarotomy and I have had them again in 10/2003, 1/2010, 6/2012, and 11/2012.  I would really like to get another year or more before having a surgery that takes me out for months.

My ABCs of Cancer: A is for anxiety

Madhulika Sikka wrote a book on the ABCs of breast cancer.  Stephanie has encouraged me to write my own ABCs of cancer.  If nothing else, it gives me topics for quite a while if I am able to keep up the writing.

So, let's start off with 'A is for Anxiety'.  Having or having had cancer puts one in a continual state of anxiety and your life is never the same again.  You are continually ruled by the latest lab reports, scans, or blood tests.  Your life comes to a complete halt in those days before the test and in the days afterwards as you wait for the results.

I have several friends who have been diagnosed with prostate cancer and their big test is the PSA test.  Men begin getting this test later in life and it almost always comes back normal (less than 4) but for all too many people, it crosses that magic line and their life with cancer begins.  The funny thing about these tests is that they are not precise.  Someone can have a PSA of 3.5 and be in full blown metestatic disease while someone else has a PSA of 6 with only the early stages of cancer.  You just never know.

Of course, that is where the major anxiety kicks in.  You have the cancer removed by some means and then you watch the PSA level and it hopefully begins to drop.  You get more and more confidence as that number continues to drop but your stress level goes through the roof when the time for your next blood test comes around.

My cancer has an extra annoyance that comes with it.  One of the standard markers is the Chromogranin-a and there is not a consensus amongst labs about how to perform it.  Some labs have a 'good' range of 0-35 with their marker while other labs have 0-5.  Furthermore, this blood test is thrown off by commonly used protein pump inhibitors (Nexium, Protonix, Prevacid) that are prescribed for acid reflux and similar disorders.


Other markers are becoming available for me that are less variable and reduce the anxiety some but it is never gone.  Even for those friends who have dealt with their cancers and reached the magic 5 year mark, you never know for sure.  Another friend passed her 4 year mark in the clear but then the 5 year test showed that she is not through the storm.  That is Anxiety with a capitol A.

I know everyone worries about mortal illnesses from time to time but when you have or have had cancer, this worry coms with extra intensity.  You almost become a hypochondriac.  Every twinge, headache, cramp, upset stomach, or cold makes you worry that the cancer is attacking with a vengeance.  It takes days to get a couple of days off of the cancer rollercoaster just to get back in line for the next ride.

I have been on that ride for 12.5 years now and it seems to never stop.  The good thing about it is that as long as I am on the ride, I am alive and able to enjoy the view some.  There is a thrill to the minor successes when you get off the ride for a while and get to wander around the amusement park, taking in the sunshine and the smells.  Something about surviving that last ride can make everything else just a little bit brighter.