Hope, tenacity, resiliency

As many of you know, I had a dear friend, Cara, pass away after a 7 month battle with AL Amyloidosis that had attacked her heart.  We met when Tammy, the Nurse Practitioner for our hematologist, Dr. Efebera, introduced us.

One thing I've observed as I talk to patients diagnosed with this disease is that it's normal to go through a grieving process for the life you thought you were going to live.  I wrote about it in this post  "A Time to Mourn."  Tammy thought it would help Cara to meet someone who had gone through that process and treatment, and was returning to a normal life.  I told Cara she was my little sister in this shitty sorority that neither of us wanted to join.  Kappa Lamda Delta--a little Amyloidosis nerd humor, ha ha.

After declining heart function between Thanksgiving and Christmas that required an extended stay in the heart hospital, her cardiac function had started to improve recently and she found out one week before her death that her hematologic response was still good and the disease in her blood was under control  She and I talked for about 30 minutes on the Sunday before she died discussing information I gathered for her at the Amyloidosis Support Group meeting and the good news of her prognosis.

On Thursday, her husband Bill left for work and she was still asleep.  When a friend came to take her to dialysis, she discovered that Cara had died peacefully in her sleep.  It was a real shock to me, almost a blow to the confidence I've had thus far.  As Tammy and I rode together for 3 hours to and from Cara's funeral, we talked about the impact a patient's mental and emotional state has on their disease process.  Tammy asked me if I'd ever thought about the mental and emotional practices that have contributed to my recovey.

Those of you who know me, know the answer to that question is yes.  I think about things A LOT--concepts, ideas, motivators.  As I've thought about this over the last 5 days, the 3 things I've come up with in response to Tammy's question are: hope, tenacity, resiliency.

Hope
You need hope--a vision of what could be and a belief that you'll get there.  The source of my hope is my faith and relationship with God.   Psalm 40 is my story of this disease's impact on my life (you can see a verse from it in the header of the blog--non-mobile version) and I lean on Jeremiah 29:11 a lot--"For I know the plans I have for you,” declares the Lord, “plans to prosper you and not to harm you, plans to give you hope and a future."

My church family, my Body of Christ, supported me every step of the way with encouragement, assistance and, most importantly, prayer support.  This church family wasn't limited to those that attend the physical building of St Patricks Episcopal Church in Dublin, OH or even those who are Christian or religious.  I had support across the country and around the world as friends shared my need for prayers and thoughts.   Knowing this support was there carried me through some really tough times.

One of the first work events I attended after my stem cell transplant was a leadership meeting that featured Shane Lopez.  He's a researcher with the Gallup organization and Gallup calls him "the world's leading expert on hope."  In his talk, Lopez explained the difference between hope and optimism.  Optimism is believing that tomorrow will be better than today.  Hope is believing that tomorrow will be better than today and I have a role in making that happen. Each patient needs to figure out that role he or she plays in making hope happen.   It could be changing to a plant based diet, using supplements, a thorough understanding of the disease (that's mine), a closer relationship with their medical team or any number of things.  I think it's incumbent on the medical team and the patient's support system to understand what that role in making hope happen is for each patient and enable that hope to take root.  Yes, we know medical profession, green tea and/or curcumin (turmeric) supplements have no/few studies proving their efficacy.  But if their use provides hope to a patient and isn't contraindicated, don't put a "road closed" sign on their highway to hope by telling them there's no use taking them.  You can read more about hope in Lopez' book Making Hope Happen.

Tenacity
You need tenacity to go through the physical and emotional challenge of treating and living with the disease.  Sometimes that tenacity requires physical action--work to regain basic functional strength after the SCT, willingness to go through a difficult treatment, training to regain a former performance status, regular dialysis.  Sometimes it requires mental/emotional discipline ie not letting thoughts about the disease overwhelm your thinking thereby preventing you from enjoying the time you have, stopping yourself from playing the "poor me" and "what if" games or trying to ignore debilitating pain  The source of my tenacity is first and foremost my love for my family and friends and the love they have for me.  I will fight to the end so that I am here for them and to make sure my battle honors the sacrifices they have made and the love they have for me.

A second source is my competitiveness.  I've always felt its important for me to "win" if I'm going up against an opponent.  I know a lot of that thinking comes from my brother Rob, who is 16 months older than me, and my dad.  My mom told the story that the first time I stood up as a baby, my brother came over and pushed me down. For the rest of our lives at home, that dynamic played out over and over--putting me in an oil drum and rolling me down a hill; throwing his shoes at me to knock me off my skateboard because I was winning a skateboard race; tying a rope around my waist and hanging me out of a second story window so I could wash them; telling me when I was 5 to touch the tail of a bee he had caught because it "feels neat", throwing a dart high in the air only to have it land in the top of my head. I've always said it prepared me to emotionally survive corporate America, I never thought it would prepare me to survive the battle of my life.  My dad, whenever either of us got a C in school or he sensed any sort of mediocrity, used to inspire us with, "Well, the world needs ditch diggers, too."  I always wanted to prove to my dad that I wasn't going to be a modern day version of a ditch digger.

Now, with a fatal, incurable disease, it's important to have a realistic definition of winning and understand that mediocre results are perfectly acceptable..as long as you have exceptional effort.  For me, winning isn't a cure.  Not to be morbid, but odds are, I'm going to lose this war.  Actually, we all will lose our war against some disease or condition.  As my friend John Rucker told me, who has an inoperable brain tumor secondary to multiple myeloma, "Kid, none of us get out of this alive."  Something's going to win against each one of us.  Is it a blessing or a curse for me to know my opponent?  For my personality, it is somewhat of a blessing.  Knowing my "opponent" gives focus to my competitive spirit.  I think of some of the quotes from Sun Tzu in the Art of War--"If you know the enemy and know yourself you need not fear the results of a hundred battles." and "He who knows when he can fight and when he cannot, will be victorious."

As I told Tammy in a Facebook message (which looking back on it was a pretty crappy way to share something morbid with someone who deals with this and other emotionally and intellectually overwhelming diseases on a daily basis--sorry Tammy) "Have you ever seen the movie 300?  It's about 300 Greeks battling 10,000 Persians. Amyloidosis is like that. You know you're going down, you just want to last as long as possible and inflict as much damage as you can on the enemy."  To be historically accurate, it was 300 Spartans with 700 Thespians and 400 Thebans but really, how can 700 actors help in any battle?!?!? ;)  As Spartan sons would leave for battle, their mothers would reportedly say, "Come back with your shield--or on it."  I'm going to beat this or fight hard to the end if I don't. That saying about it being ok to lose a battle but win the war doesn't apply here.  When I lose the first battle, I've lost the war...I just want the opportunity to engage in as many battles as possible

Resiliency

Finally, you need resiliency so that as you hit bumps and detours on the road to your vision of hope, you can gracefully adjust.  It may require changes in hope--revising that vision of the future due to new developments or changes.  It may require additional tenacity--being willing to go through additional and unexpected physically or emotionally challenging treatments and periods.

It is dangerous to get too firmly attached to any set of expectations because you're dealing with a complex disease in different human bodies that will respond to that disease and treatment differently.  Many of us with this disease read the studies that are published and believe the optimistic results will apply to us and the pessimistic results apply to someone else.  I do that all the time...and that's OK because it drives my hope.  "Sure, I know this disease has a 96% relapse rate but that won't apply to me."  When Urban Meyer was at the (or maybe The) University of Florida, he regularly referred to our academics as being in the top 1%.  Yeah, well that's my hope for my course in AL Amyloidosis, I'll be in the top 1%.

That is my hope, but my plan is to do whatever is required to string together the greatest number and longest duration of "progression free survival" periods. The National Cancer Institute defines progression-free survival as "the length of time during and after the treatment of a disease, such as cancer, that a patient lives with the disease but it does not get worse."  I picture an X-axis of time with a wave representing my disease state rising above and descending below it.  Each time it passes below the axis, into the negative territory, I know that its next ascent, my return to a progression-free state after treatment, will not rise as high, nor span as long as the previous one.  I just need to keep the wave going as long possible 

Hope, tenacity and resiliency are the bricks that make the foundation upon which my mental and emotional state is built.  But you need mortar to connect bricks together to make a structure that is strong.  In my mind, the mortar is a patient's advisors--close family, friends and medical team members whose opinions you trust and who can keep you balanced across the three.  These folks need to operate more as coach than cheerleader.  They need to be able to tell you that you're letting your tenacity overwhelm your hope.  Or you need to be more resilient and recast your vision of hope.  Or you need to increase your tenacity or you'll never achieve your vision of hope.

Understanding how each member of the team balances across those three dimensions can help put their guidance into proper perspective.  No one is perfectly balanced across all three but as patients, I believe our inner circle of trusted advisors needs to collectively span across all three. 

For example, I've learned that Dr. Efebera skews really heavily to the hope side.  That's one of the traits I appreciate the most about her.  I would imagine it's emotionally challenging to be a physician caring for patients whose diseases have such high relapse rates and relatively short overall survival.  Knowing this about her means I need to understand that when she tells me something will improve in 3 months, there's a possibility it may take longer.  In Efebera-speak, that means it could START improving in 3 months.  She's contributing my hope and I appreciate that but I need to make sure my tenacity is in place to do any work required to further that improvement longer than I thought and that my resiliency is strong enough in case that 3 month milestone passes by with little or no improvement.  It's not a time to recast my vision of hope because one milestone wasn't met "on time."  Didn't happen now <> won't happen ever.

My friend, Allyn has also helped with my hope.  Early on, she described the consequences of my disease and treatment as, "A terrible situation with the best possible circumstances."  It made me look more consciously for all those great circumstances that are waaaaay too many to list here.  And as I found them, it reinforced my hope because it was a reminder to me of God's providence.  I remember my friend Cass, who is an Episcopal priest, saying, "What we may call a co-incident is really a God-incident."  It helps reinforce the hope I have.

My husband, Alan, is very focused on tenacity...primarily the work required to get back to some version of normal and to maintain that.  When I got out of the hospital, I had very basic physical therapy exercises to regain my strength, not challenging but tiring--walk backward, walk toe to heel, walk sideways.  Initially when I was discharged, I had trouble even standing up for more than a minute. And I was tired...physically tired, tired of doing things I didn't want to do, just tired after 4 weeks in the hospital getting new bone marrow installed. I was also basking in the glow of surviving the transplant. I had my eyes firmly fixed on that vision of hope of remission and saw the transplant as the road that would get me there.  But a road doesn't get you anywhere solely on its own.  You need velocity to get you to a destination  Alan was very focused on ensuring I was doing the work needed to get me as close as possible to back to normal.  "Drill Sergeant Al's Convalescent Home" is how I described those days.  But I needed a counter-balance to my reliance on hope and I trusted Al (and still do) that he has my best interests at heart and I need to do what he says.  Yes, friends it does happen.

Amy and Claire have each helped with my resiliency.  After the stem cell transplant I went through a period in what I call "no man's land."  I'd just gone through the most physically and emotionally taxing experience of my life and I had to wait another 70-ish days to know whether the transplant had worked.  Sometimes it doesn't. When I got into a state during which I was fixed on having the life I had before the transplant and convinced that I never would Amy told me "To live the life God has given me."  It helped me recast my vision of the future, my expectations, but still maintain my hope.

One night in early August, two days before my appointment that would measure my hematologic response to the stem cell transplant (did all that pain and turmoil accomplish anything?!?!?!?), I was in excruciating pain from my liver.  I was in the kitchen crying, Alan was at work so the comforting fell to Claire.  Now, I've talked about her confidence and strength of spirit before.  When I wanted to make sure she knew how serious this disease is and asked, "You do realize that people die from this?", she responded, "People do but you won't."  That night, she came over to me, put her arm around me and pointed my attention to my vision of hope by saying, "Mom, the doctors keep telling you everything is great.  You've got to believe that's true until they tell you that it's not."  

I am so grateful for the team that has brought me where I am today--God, my family, my friends, my medical team. The second verse of Psalm 40  says, "He drew me up from the desolate pit, out of the miry bog, and set my feet upon a rock, making my steps secure."  God set me on that rock but it was built in partnership with and made secure by my family, friends and medical team.   For that, I will be eternally grateful.

More Milestones

It's been another period of one year milestones, reflecting about where I was and how I was feeling one year ago.  Saturday I went to the Amyloidosis Support Group meeting at Ohio State.  Because there are so few Amyloidosis patients, this meeting covers Ohio and Pennsylvania and is held twice each year.  Last year, fortunately, the meeting was held about 2 weeks after my diagnosis.  I met Sandy during that meeting, a wonderful woman who gave me hope that I could have a normal life with this disease.  Her words of encouragement kept me going through some dark days during and after the transplant.  Sandy and her husband were at the meeting and it was great to see her again and tell her how much her encouragement meant to me.

This period of time between my diagnosis and discharge from the hospital was such a blur.  I was emotionally reeling and trying to mentally process so much that there are many things that happened that I've simply forgotten.  Sandy reminded me that at last year's meeting, Dr. Efebera said about me, "Don't let her appearance fool you.  She looks strong and healthy but she is very sick."  Maybe I blocked that out because I didn't want to accept her assessment.

Last year, I met someone who had Multiple Myeloma and Amyloidosis who told me that he didn't go to the Amyloidosis Support Group meetings because he found them too depressing, that people who attended those meetings didn't seem to have much hope.  He found those that attended the Multiple Myeloma meetings to be much more hopeful.  I decided when he told me that to continue to attend the Amyloidosis meetings to make sure there would be at least one voice of hope in the room.  I remember how much Sandy's message of hope meant to me and I plan to make sure I can share that message of hope with those who may come after me.

Yesterday was Claire's first Lacrosse scrimmage of the season.  She and I have talked several times over the last few weeks about the fact that I have never seen her play Lacrosse in high school.  It seems surreal that I missed an entire season.  She started playing organized sports at age 4 and has played continuously since then. Until last year, I'd probably missed fewer than a dozen games and last year I missed an entire season.

I love taking pictures at her sporting events and have done the year end photo video for her Field Hockey team the last two years.  One of the VPs of the booster club called me to ask if I'd be willing (and able) to take pictures of the lacrosse team this year.  I said, "Of course" not just because I want to help but because it will feel more like normal.  It felt great to be down on the sidelines taking pictures yesterday.  The temperature was in the mid-60s, the sun was shining and I was so thankful to be healthy enough to do it.  Here's a link to the photos....not too bad if I do say so myself. https://plus.google.com/photos/102055039637995406761/albums/5990062683688395537?authkey=CMT7uMar85Dd7gE

Alan and I are planning on taking a weekend ski trip to New York the first weekend of spring break.  Yes, I went skiing at Steamboat for a week last year over spring break but was so weak and I felt so bad it was a real struggle.  Looking back on the trip, I probably shouldn't have gone but I didn't know if I'd ever have the chance to ski with the family again and I was determined not to miss the opportunity.  I was proud that I was able to ski each morning we were out there but it was a real struggle.  I tried to keep up a brave appearance but was really scared and suffering.  I knew things were bad when I would eat a half of a peanut butter sandwich for lunch as if I were a priest distributing communion to myself--rip off one small piece and chew it over a few minutes.  Dinner was also a choice between nourishment and pain.  I knew things were bad for me when I didn't have the energy to ski down the last day.  I'm ashamed to admit I took the gondola down but I knew I didn't have the strength to ski down and was afraid if I tried, I'd end up getting hurt.

Alan will be gone over Mothers Day weekend this year which is the first anniversary of my discharge from the hospital.  But, I'm glad he'll be gone, not because I don't want him around but because he'll be riding the 200-mile Tour of the Scioto River Valley bike ride.  He's done this ride several times since we moved up here and it feels very normal to know that's what he'll be doingh...not at home with me cajoling me to do my physical therapy and installing the shower hand rail and bench I needed when I first arrived home.

I'm so thankful that I can enjoy this normal that's pretty close to the old normal.  How different things look this year.

A Time To Mourn

February will always be the month of mourning for me.  Many of you know the challenging February I had last year—the deaths of my father and father-in-law, and my diagnosis with AL Amyloidosis (and eventually Smoldering Multiple Myeloma) all in the same month.

There’s the traditional mourning that accompanies the death of a loved one and that was part of my February.  But my diagnosis cut short that mourning and introduced me to mourning the passage of something else.

Before my diagnosis, I considered myself a pretty strong Christian who recognized that God was ultimately in control of my life.  At the end of the Confession of Sin in the Episcopal service we say, “For the sake of your Son Jesus Christ, have mercy on us and forgive us;  that we may delight in your will,
 and walk in your ways, to the glory of your Name.”  I’ve always appreciated that word “delight.”  I don’t ask God to help me “accept” His will but to delight in it.  That’s a pretty high standard but I’d always felt I was doing a pretty good job at it.

I have found that its really easy to delight in God’s will when God’s will is relatively close to my design for my life—happy, healthy, faithful family; financial security; loving friends.  It’s challenging to delight in a diagnosis of AL Amyloidosis with Smoldering Multiple Myeloma and the health problems and likely reduced lifespan that comes with it.

That sense of security, the expected guarantee that my life design will continue, is lost with the diagnosis of a fatal, incurable disease that has a 96% relapse rate.  Yes, I know, that guarantee was never really there in the first place.  But an illusion that persists long enough starts to feel like reality.  And, so, in the midst of mourning the loss of my father and father-in-law, I started mourning the loss of the life I thought I was going to have.

From talking to others with this disease, many of us progress through the five stages of grief after diagnosis—denial, anger, bargaining, depression and acceptance.  I think one of the reasons I’ve done so well with this emotionally is because I was able to move through those stages pretty quickly.  I don’t think you can be “all in” in the fight until you get to acceptance.

One year ago today, my phone call from Dr. Levin as I drove home on I-270 went something like this, “Mrs. Koontz, I have a diagnosis for you.  It’s a rare blood disorder called Primary Amyloidosis.  Your next steps will be to have biopsies of your liver and kidney and an echocardiogram of your heart to see if and how much they’ve been affected by the disease.  I will need to refer you to a specialist but I don’t know who treats it here at OSU.  I have some phone calls and emails out and will call you tomorrow with the name of someone to whom I can refer you.  Have a great night!”  Ok, he didn’t say “Have a great night” but I can’t really remember what he said, if anything, to close the conversation.

After I got off the phone with Dr. Levin, I called Alan.  I remember I was passing the I-270 exit for Tuttle Crossing when I was on the phone with Dr. Levin and was passing the 33 exit for Avery-Muirfield when I was on the phone with Alan.  I got home, jumped on the internet and was shocked at the prognoses I saw.

Yes, everyone says “don’t go on the internet there’s a lot of misleading information out there.”  But I knew how to find the good information….I work in data and analytics and understand statistics; I did Media Relations for a teaching hospital, writing general media news releases and pitching story ideas about clinical trials going on at the hospital; my husband is an RN with a career of experience in the ER and ICU.  I know how to find the valid information….and the valid information was the scary stuff.  There’s not a lot of false information floating around the interwebs about a rare disease.  Not too many sensational news reports exaggerating the seriousness of the diagnosis....no exaggeration needed.

I remember seeing average survival time of 4 years and thinking I wouldn’t even be around to see Claire graduate from college or know any grandchildren that might come along.  As I reeled from the shock, I kicked my meta-analysis into high gear searching for some hope in the medical literature I was reading.  At the time, I wasn’t aware of the progress that has been made against this disease in the last 10 years and was giving equal consideration to information from 1999 that I was to that from 2012.

But as I read more, I saw such variation in the overall survival estimates and decided I was going to be one of the people on the long tail of the survival curve.  I saw on the Amyloidosis Foundation website that high dose chemo with stem cell transplant “is the most aggressive treatment to date for those who can qualify for it. It is showing a great deal of promise.” I decided the phrase “aggressive with a great deal of promise” sounded like what I wanted.

So, at 10 pm, about 5 hours after I first heard the phrase “primary amyloidosis” from Dr. Levin, I posted on my blog (Relaxin in the Amyloid Oasis) that I had seen a calendar for a stem cell transplant and noted, “It says the process takes 5 weeks but can take up to 8 depending on how well the patient tolerates the treatment.  I told Al, "My goal is to be done in 4!"  :)”

I moved through those grief stages pretty quickly that night and got to a point where I was ready to fight once I had a sense that there was a path to success.  Now, I’m not saying I didn’t cycle back through some stages over the coming weeks and at different points in my journey (because I did), but that night and over the next few days, God gave me the strength to want to fight this.  And the very next morning, He gave me the peace to know that I would be OK. ("It Is Well With My Soul")

Over the summer, as I recovered from the stem cell transplant and wondered if it was successful, I still went through periods during which I would mourn the life I had, or I thought I had, before my diagnosis.  But once I learned I had the best response possible to the transplant, I haven’t done the before and after thing.  Words of wisdom from two beloved believers helped me with that.  First, my daughter Amy admonished me to “live the life God has given me.”  And my friend Jim told me that “my after is now a new before.”  I have a life, there is more to come and I need to look to the future thankful for what I now have, not disgruntled about losing what I thought might have been.

Another period of mourning I’m now experiencing is related to work.  In January, we had a new leader named for my group.  He has reorganized the group and my team of three directors and 25 people has been split up into other teams within his organization so I can take on a new role.  While I’m excited about the new role, there is a sense of mourning for a team and a function that I’ve been working to build over the last 3+ years.  I’ve developed some great relationships with folks on the team and, while the relationships will continue, there’s no escaping that they will change.  You take a job for the work, you stay in a job for the relationships and I’ll miss working closely with the team.

But, if this last year has taught me anything, it’s that change will come and even when I can’t see where the path will lead, I can trust in God’s love and care as I walk the path.  Lately, I’ve been thinking about the passage from Ecclesiastes 3 (There is a time for everything, and a season for every activity under the heavens:  a time to be born and a time to die…) and humming "Turn, Turn, Turn", The Byrds song that is based on the passage.  I was pretty familiar with the section of the passage that lists all the activities for which there is a time but I hadn’t read the whole chapter recently.  I was struck by the verse that comes shortly after—“I know that there is nothing better for people than to be happy and to do good while they live.”

Yes, as I move through this period of mourning and past these one year anniversaries, I will hold fast to that…to be happy and do good while I live.

The Real Slim Shady & Multiple Myeloma

May I have your attention please?

May I have your attention please?

Will the real Slim Shady please stand up?

I repeat, will the real Slim Shady please stand up?

We're gonna have a problem here..

The request at the gala on Saturday night seemed simple enough....."Will everyone with Multiple Myeloma please stand up?" Hmmmm, I wonder if I should stand up, I pondered, as the words from Eminmen's "The Real Slim Shady" crept into my head.  According to my OSU medical record, I have Primary Amyloidosis diagnosed 3/11/2013, I have Multiple Myeloma diagnosed 3/11/2013 and I have Smoldering Multiple Myeloma diagnosed 5/11/2013.

According to Dr. Gertz at the Mayo Clinic, I "do not meet their criteria for Smoldering Multiple Myeloma."  Notice he didn't say that I don't have it.  I've watched several webcasts and videos from The International Myeloma Foundation in which they will have physicians with different perspectives review a set of diagnostic information and discuss what treatment is warranted.  I call these the myeloma battle of the bands.  Mayo is always on the conservative end of the spectrum--don't treat, cost doesn't warrant the outcome improvement, etc.  (Meanwhile, Antonio Palumbo, MD in Italy is almost always advocating to treat.  Those impulsive Italians!)  

So, when Dr. Gertz says I don't meet their criteria, I take it with a grain of salt, knowing that Mayo is very conservative.  It seems there's not a lot of clear cut, agreed upon criteria that separates Primary Amyloidosis (AL), Smoldering Multiple Myeloma (SMM) and Multiple Myeloma (MM).  At the point I started to get fixated on labeling what I have, Dr. Efebera explained that the name doesn't really matter.  She will look at all the clinical information available to her and develop the most effective treatment plan for me, not based on the combination of letters for my disease.

I trust her expertise and judgement and, in theory, her approach sounds great.  The problem with it is the difference in prognosis for someone with the different combinations of the alphabet soup--AL, AL with SMM, AL with MM.  She told me I'm "in a gray area" between AL and AL with SMM.  

Just this week I saw a study from Mayo that said AL patients with greater than 10% bone marrow plasma cells really have AL with MM.  Well, what do you know, my aspirate sample (the liquidy part of the marrow) was 7% but the core sample (from the solid piece of bone marrow) was smack dab on 10%.  And, according to Dr. Efebera, the core sample is the more accurate measurement.  Did the pathologist round up or round down?

I recently read an article in the New York Times entitled "How Long Have I Got Left?"  It chronicles the quest to get a definitive life expectancy figure by a Neurosurgical resident recently diagnosed with lung cancer.

These statements really captured many of my feelings as I've researched my disease and prognosis--

Before my cancer was diagnosed, I knew that someday I would die, but I didn’t know when. After the diagnosis, I knew that someday I would die, but I didn’t know when. But now I knew it acutely.

But the range of what is reasonably possible is just so wide. Based on today’s therapies, I might die within two years, or I might make it to 10. If you add in the uncertainty based on new therapies available in two or three years, that range may be completely different. Faced with mortality, scientific knowledge can provide only an ounce of certainty: Yes, you will die. But one wants a full pound of certainty, and that is not on offer. 

What patients seek is not scientific knowledge doctors hide, but existential authenticity each must find on her own. Getting too deep into statistics is like trying to quench a thirst with salty water. The angst of facing mortality has no remedy in probability. 

The writer describes his quest for a life expectancy number.  But before I can even get to a number, I need a name and there's not a lot of consensus in the medical community about the name of my disease, my unique combination of letters.

I know that one of the spiritual lessons from this journey has been trust.  God brings that message of trust before me on a regular occurrence...bordering on annoying almost (sorry, God, but I call it like I see it.)  This mystery about the name of what I have, much less the amount of time that diagnosis is expected to afford me, is just another opportunity to trust God.

I'm doing pretty good about not worrying about my combination of letters.  I will continue to read and study because I am intellectually curious about this.  I'll keep working to regain my strength and stamina so I can feel that I've left the effects of the disease in the past.  My recent trip to Colorado with my sister in law and her sisters (my adopted sisters) was a big step.  I went backcountry skiing for at least 2 hours each morning and kept up just fine.  I went downhill skiing at Steamboat Springs for one day and successfully made it down my favorite black diamond run "Huevos".  You can see the photographic evidence in my Google photo album.  That's really good progress less than 9 months after my stem cell transplant.  Crossing the finish line on my 100-mile Pelotonia ride will be the ultimate expression that I've moved past the impact the disease and treatment have had on me.

I'll continue to live my life one day at a time trusting that, as it was before my diagnosis, my time on earth is in God's hands.  But I do need someone to tell me whether or not I should stand up.

Relatively Easy

The last two weeks have been a continuous stream of good news and confirmation of how well I've made it through the treatment of and recovery from this disease.  My nephrologist telling me last week that I "breezed through the transplant," my gastroenterologist telling me that not only is my liver size reducing, its also softening which means its getting closer to normal.

The latest good news that I've shared on Facebook that I had my abdominal ultrasound on Wednesday for a post-transplant re-staging of my disease. It showed that in the 9 months since the transplant, my liver has reduced 5.4 cm in size from 24 cm to 18.6 cm. That meets the defined criteria for organ response. My kidneys were also effected by the Amyloidosis and I've achieved organ response there, also.

Hematologic response--check complete
Liver response--check greater than 2 cm reduction
Kidney response--check greater than 50% reduction in 24-hour urine protein

Here's the picture from an article in the journal of the American Society of Hematology entitled "What do I need to know about immunoglobulin light chain (AL) amyloidosis?" authored by three Mayo physicians including Dr. Gertz who I saw for my second opinion.

So, I've made it across the entire response continuum and for that I am so thankful--thankful to God for His guidance and faithfulness, thankful I pushed for a diagnosis, thankful to my medical team and thankful for the support of my family and friends.  I know of so many amyloidosis patients whose disease went undiagnosed and untreated/ineffectively treated for an extended period of time and suffered irreparable damage to a combination of their heart, kidneys, liver, nerves and/or skin.  They are facing limited strength and endurance due to heart damage, regular dialysis and edema due to kidney damage; ongoing abdominal bloating and digestive problems due to liver damage; painful neuropathy, loss of balance, dangerously low blood pressure due to nerve damage; and/or debilitating pain when doing something as simple as talking or smiling due to amyloids in their soft tissue.  I don't have any of that.  Yes, I have some clinical signs from the damage caused by the disease but they don't impact my ability to live the life I had before.  Yes, I have a ~96% chance of relapse but overall survival in complete response SCT patients is averaging 10 years now and getting longer every day.  I am healthy and disease free until Dr. Efebera presents me with test results and her assessment that I have relapsed.

I've been listening to a new to me artist named Jason Isbell that a friend (music identifier extraordinaire) recommended to me.  He knows I like progressive rock and alt country artists who write poetic lyrics wrapped in simple solid music--Ryan Adams, Josh Ritter, Delta Spirit, Old 97s, The Avett Brothers (still mad I missed their show in May due to my SCT recovery).  Andy Staples, a sportswriter I follow on Twitter and fellow UF Journalism school grad, called Isbell, "A storyteller who follows Strunk and White's rule 17.  That rule says, "Omit needless words." 

Isbell has a song called "Relatively Easy" which has become my favorite song recently.  I know one of the main reasons is because of all the good news I've received compared to others I know fighting Amyloidosis.  I almost feel a little guilty...like that person who survives a plane crash and wonders "Why me?"

Here's a link to the song-- http://youtu.be/NIQ1NHa0g6A

Here are the lyrics that really impact me--

You should know compared
To people on a global scale
Our kind has had it relatively easy
And here with you there's always
Something to look forward to
Our angry heart beats relatively easy 

I've always felt this way about life in America.  My work with Food For The Poor and my travels to work with the destitute poor in Haiti and Jamaica gave me a broader perspective of what prosperity really is.  I met a parent whose child died for want of $2 in antibiotics.  If I lived in any country other than a highly-developed first world country--US, Canada, Western Europe, Japan--I'd be looking at a much more grave prognosis.  Because of the care I've received and the care that will be available to me in the future, I have a lot to look forward to.  I think about all the support from Al and his work and dedication to get me back to normal.  So yes, here with Al, there are lots of things to look forward.  Our hearts that were angry when I was first diagnosed now beat relatively easy.

I lost a good friend
Christmas time when folks go off the deep end
His woman took the kids and he took klonopin
Enough to kill a man of twice his size 

Not for me to understand
Remember him when he was still a proud man
A vandals smile a baseball in his right hand
Nothing but the blue sky in his eye

 While I never even considered suicide, I can understand the despair over the future that could make a reasonably emotionally healthy person consider it.  You can become hopeless when who you are seems so far from the person you've always considered yourself to be and you don't see a clear path to become the person you believe you are.  That's what those two verses say to me.  I talked about that feeling in this post--Before and After Part 2  This was in July when I wasn't sure about the hematologic response and hadn't seen any organ response either.  I looked back at the person I was at Amy's wedding--not just the physical state, but also the emotional state--and didn't think I'd ever get back to a reasonable facsimile of that person.

Still compared to those
A stones throw away from you
Our lives have both been relatively easy
Take the year and make a break
There ain't that much at stake
The answers could be relatively easy

I think about the people I know who are fighting much harder battles with this disease than I am and face a much longer road to normal, if that's even possible.  I think about my friend Robyn who died in September after a four-year battle with Pancreatic Cancer.  I'm coming up on my one-year anniversary of my diagnosis.  I did take a year, there was a lot at stake, but, yes the answers were relatively easy.  As Dr. Parikh said I've breezed through this.

So, I'll sit here enjoy a post-workweek glass of red wine and bask in all the good news I've received and contemplate Relatively Easy.

Good Progress

I had appointments with my gastroenterologist and nephrologist on Wednesday.  I call it my day with the gists.  I really like both of these physicians and they're about as different as possible on the surface.  But what they do share is a deep knowledge of their medical science with equal portions of compassion, a bias for action and a willingness to educate me...which are very important to me.

Dr. Levin, my gastroenterologist, is about 70 and a focused scientist who seems to have a gruff exterior but has a heart of gold and a mind that can quickly organize data.  He reminds me of that grumpy uncle who is somewhat intimidating when you first encounter him but is quietly caring and figures out all the brain teasers.  When I was continuing to have issues with my fluid retention in the hospital and felt like the transplant physician, Dr. Levin and my nephrologist couldn't develop an approach, I asked Dr. Levin to step in and get things moving and he did.  He was also the one to diagnose me.  For that, I will be forever grateful.  During my first appointment with him, he asked if I was related to a physician named David Koontz.  I told him David is my brother in law and Dr. Levin mentioned that he trained David when David was a resident at Riverside Hospital about 25 years ago.  It shows me he's a man who cares enough about people to commit them to memory.

Dr. Parikh, my nephrologist, is probably in his early to mid 30s and is very eager and engaging.  I wrote about my first encounter with Dr. Parikh in this post.  He answers all of my questions and provides context and the reasons why lab values may be changing.  When I notice a change and express concern, he doesn't just tell me "that's normal, don't worry about it"  he tells me why its normal, what is driving the change and why I don't need to worry about it.  So often when questioning any technical expert, I'm reminded of the quote by Einstein that says "If you can't explain it simply enough, you don't understand it."  Dr. Parikh always explains things simply enough so I can understand them.   When we were talking about changing some medication levels over the summer and I wasn't ready to change them as much as he was, he listened to my concerns and said we would try my course of action first.  To me, that shows a great deal of confidence in his ability to manage my condition since he didn't insist on his course as the only course.

On Wednesday, Dr. Levin told me that he could feel that my liver size has reduced.  I felt that but his assessment validated that it wasn't just wishful thinking on my part.  He also said my liver is no longer rock hard.  Liver hardness < Rock  That's a nice equation.  I was reading the proceedings of the International Symposium on Amyloidosis and read this interesting information in the history section.

Thomas Bartholin, discoverer of the lymphatic system in humans, described in his Historiarum Anatomicarum Rariorum the autopsy of a female whose spleen was so hard that it could scarcely be cut with a knife.  He stated that incision of the spleen produced a sound like that of the cutting of spongy timbers. This autopsy report was included among the 3000 collected in Theophili Boneti’s Sepulchretum Sive Anatomia Practica which was published in 1679.

That type of organ hardness is what I've imagined my liver is like.  Nice to know Dr. Levin feels that its softening up.  Goodbye spongy timbers!  (When I read "spongy timbers" I thought that would be a good name for a pirate ship captained by Spongebob Squarepants.)

Dr. Parikh said that my kidney function is normal except for an excessive amount of protein in my urine.  The amyloids have pried open the filters in my kidneys.  Substances that are too large to pass from the blood into the urine through the normal size filter will go through in an amyloid damaged kidney.  Protein that should stay in my blood leaks into my urine through the filters that have been pried open.  He said that's improved thus far and he expects it to continue improving.

In Amyloidosis, they measure treatment response in hematologic response (are you no longer producing the substances that create the amyloids) and organ response (are the organs damaged by the amyloids recovering.)  My doctor said that organ response can take up to three years to complete and in the studies I read, that seems to be the standard period of time they look for changes.

I had a complete hematologic response and as long as my immunoglobulin free light chains are normal, that response is continuing.  There are defined standards to determine organ response.

For the liver, that criteria is ≥50% decrease in an initially elevated alkaline phosphatase level, or decrease in liver size by at least 2 cm.  My alk phos level has reduced by 33% in 9 months and I still have 27 months within which improvement could occur.  I go next week for an ultrasound to measure my liver.  Last time they measured it, it was 24cm.  22cm next week shows organ response in the liver.  Now, bear in mind that normal for someone my height is about 8cm. It reminds me of that scene in the Grinch where his heart grew 3 sizes that day...except for me its my liver and it wasn't undersized to begin with, as far as I know.  But I sure got the 3 sizes thing.

For the kidneys, response criteria is 50% reduction in 24-hour urine protein excretion (at least 0.5 g/day) without worsening of creatinine or creatinine clearance by 25% over baseline.  At my appointment in December, my 24-hour protein had reduced by 48%.  So close but I'm going to round up.  I'm sure I'll be officially in the response criteria when they measure it again, probably in April.

So, more good news and good progress.  The bad thing about doing so well is that I'm now on 6 month follow-up with Dr. Levin and Dr. Parikh.  I'm really going to miss seeing them but since it means that I'm getting better, then sorry guys, we're going to have to get comfortable with a long distance relationship (time-wise, at least.)  It sure beats the alternative of being in a health state where they are managing an active disease process.

So, docs, see you in July.  Hope you don't miss me too much!

Hospital Memories

This benediction showed up on my Facebook feed last week:

In the quiet of this place, in the dark of the night, I wait and watch. The senses of my heart are awake to you. In the stillness of my soul, I search in the silence of my spirit, O God. (Celtic Benediction)

It reminded me of some of the lonely, scary nights I had in the hospital.  The days were fine because they were filled with a lot of people and activity.  Alan was with me most days from 8 am until 2 pm and then back with Claire between 7 and 9 pm.  Amy came for a few hours every night, as well. What a loving sacrifice from her.  She had only been married for 6 months and would spend her days at her job at Grant Hospital and her evenings with me at The James or Dodd Hall.   But, after 11pm when things slowed down in the hospital, it was me, my thoughts, my music, my prayers and God.

I would stream music from my phone to a bluetooth speaker I brought with me and during some of the nights when I couldn't sleep well, which was most nights between about April 15 and April 28.  I had a playlist of my favorite hymns that was my go to choice.

• It Is Well With My Soul
• A Mighty Fortress Is Our God
• Come Thou Fount of Every Blessing
• Great Is Thy Faithfulness
• The Old Rugged Cross
• How Great Thou Art

These are songs I remember my grandfather singing and have sung a lot myself in church and at Cursillos.  They always took me to a happier time and reminded me who was still in charge.

"It Is Well With My Soul" was one of the first hymns that came into my mind after my diagnosis.  As I explained in this blog post, Jesus told me during my morning prayer the very next morning after my diagnosis that "He's got this" and that promise has held true since that moment.  I've learned that faith isn't digital ie it's on or it's off.  It's much more variable than that.  Sometimes it was full power, total faith, no room for doubt.  But sometimes it was a really weak signal with lots of doubt and fear mixed in.  And, I think God's OK with that as long as I keep seeking Him, no matter how frail my efforts may be.

Many nights I would think of the story of the Cherokee right of passage where the young man is taken into the forest at sundown by his father, blindfolded and left sitting on a stump until the sun rises.  He experiences many fears through the night and when he removes the blindfold in the morning, he realizes his father has been sitting next to him all night.  You can read more about it here.  I felt that way during many scary nights when I was in so much pain and fearful about my recovery.  I knew God was sitting there next to me.  But one night, I started thinking about everyone who was praying for me, thinking about me and sending me positive vibes.  I pictured them as a ring around me on that stump helping to keep anything negative or harmful away from me.  I held on to that image many times through my hospitalization and recovery.

Another memory I have is from a line in "Come Thou Fount of Every Blessing".  There's a line that says--"here's my heart, O take and seal it, seal it for thy courts above."  I remember one night I was pretty doped up on morphine but still in a lot of pain.  When I heard that line I said, "Hey God, while you're in there doing that heart sealing thing, could you maybe work on my liver, kidneys and abdominal swelling?  You know, since you'll have the hood up."  That made me laugh at myself and gave me a little glimmer of hope that I could make it through this with my unique sense of humor still in tact.

Chemo and pain can alter many things but I'm glad my spirit..and sense of humor...have recovered well.