36 Hours in DC

What an incredible trip to DC for a meeting that brought together most of the top Amyloidosis researchers from across the world, patient advocates, caregivers, FDA representatives, and, most importantly, representatives from the Amyloidosis Research Consortium.  The ARC is an organization “committed to building collaborative relationships between patients, academia, industry, foundations, federal funders, and regulators to facilitate and speed new therapies to market.”  Highlights of the meeting included—

• Meeting or seeing again “my people” especially my Amyloidosis sister Kim Tank, who I met in person for the first time
• Confessing my research fan crush on Dr. Ray Comenzo with him…and several of my Amyloiodosis friends (hilarity ensued, FYI)
• Hearing the top researchers in the world share with the FDA why the current drug development methodology in Amyloidosis is killing patients (my words, there)
• Learning of the strength and courage of others who are struggling with this disease and hearing their heartwarming…and gut wrenching…stories.
• Having people share with me how my blog has given them motivation and hope

Basically, the overall theme was HOPE!

Right now, there are no FDA approved therapies for AL Amyloidosis.  Every treatment is an “off-label” use of a drug designed for Multiple Myeloma.  Problem is, we don’t have MM.  As Dr. Morie Gertz noted at the session, “The massive gains in myeloma survival are not translating sufficiently to AL patients.  Unique interventions are needed for amyloid population.  The amyloid population is uniquely fragile.  Typical myeloma regimens have much more profound impact on AL.”  I’m assuming that impact is negative. 

Presenters, including Dr. Giampaolo Merlini from Italy and Dr. Vaishali Sanchorawala from Boston talked about the physical, mental and emotional costs of the disease.  30% of patients diagnosed with AL are dead at 6 months and for those with cardiac involvement, median survival is 18 months.  Not having approved drugs and the slow pace of drug development inherent in diseases with small populations is causing deaths that could be prevented.  The pharma companies have potential drugs but using overall survival as the success measure in clinical trials for small populations makes the approval process too long and not economically sound.  For AL Amyloidosis, it makes sense to use biomarkers that are proven to be predictors of overall survival as success measures in trials.  If the FDA would allow that, it would reduce the time to complete a trial from 6 years to 3….cut in half!

Dr. Sanchorawala shared results showing the impact the disease has on quality of life, another measure the FDA can use in determining whether a drug is shown to be effective.  Her study showed that Amyloidosis patients have a similar reduction in quality of life reported by people with Rheumatoid Arthritis.  And for some groups of patients, it’s at a similar level as chronic lung disease.  The impact to quality of life by those two diseases is pretty well understood.  For many with Amyloidosis, it is hard to measure with a clinical test the impact of the disease.  How do you quantitatively measure the impact that infiltration into the nerves of the mouth and salivary gland has?  I know a woman who’s dealt with this for 13 years.  It’s painful for her to talk, or to smile at her grandchildren, so she feels isolated.   If she were willing, why not let her try a drug that might help improve her quality of life?  But since that’s not an outcome that gets measured, she’s ineligible to participate in any trials, regardless of how willing she is because her outcome doesn't help the pharma company prove the efficacy of the drug to the FDA.

I’m HOPING that the ARC will be able to have the FDA accept biomarkers and quality of life measures as study endpoints for Amyloidosis drugs very soon….like starting next year.  It’s interesting that I was asking on Twitter about this quality of life question over the summer, thinking about the woman with the mouth involvement.  So happy to see it might happen.

It was so wonderful meeting so many of the physicians whose work I’ve read over the past two years.  First and foremost was being able to chat with Dr. Ray Comenzo, who I met at the Amyloidosis Gala in Nashville in October.  I cornered him there for about 20 minutes and told him how much I appreciated his work and how much hope he’s given me.  He was very gracious as I gushed on and talked his ear off. I read this article (“How I treat Amyloidosis”) when I was first diagnosed and the first case was so close to my disease course.  Reading about her recovery gave me so much hope.  When Dr. Comenzo came into the breakfast room, I told my friends at the table he’s my  researcher crush and that while I’ve only met him once, I know him through his writing and I feel a deep connection to him. I said his writing speaks to me more intimately than the most graphic romance novel and, of course, lots of laughter ensued. Right after that, a woman went up to him and they hugged.  I said, quietly so only my table could hear, “Don’t you go hitting on my man.  You ladies see what I’m up against with him.”

Later in the day, I shared this discussion with Dr. Comenzo and he got quite the laugh.  I thanked him for his research. We talked about the need for more treatments for Amyloidosis and I shared with him my perspective on maintaining a positive mental state.  I said that I pictured a sine wave going up and down across a horizontal axis.  Right now, I’m above the line and wasn’t going to waste a perfectly good remission worrying about relapse.  As long as new treatments are being developed, I can count on one of them being available if I relapse to put me back above the line.  My hope is to keep the wave going as long as possible.  He said, “You may have a crush on me but you inspire me.”  Swoon!

Dr. Sanchorwala came to me during one of the breaks after I’d shared that I was treated at Ohio State.  She asked me if I was treated by Dr. Efebera and when I told her I had, she shared with me how much she enjoyed Dr. E when she trained in Boston. I told Dr Sanchorwala how much I love Dr. E and how my friends and family believe she’s the perfect personality for me.  She mentioned that she recognized me from Twitter from an online Amyloidosis Journal Club that another physician has organized.  It was really cool to talk with her.

Meeting so many patients I’ve interacted with online was a blast.  First and foremost, I got to FINALLY meet my sister in this Amyloidosis sorority, Kim Tank.  Kim and I met through a Facebook group as I was recovering from my stem cell transplant and she was preparing for hers.  She’s a badass runner who completed a 100K run while finishing up her chemo and qualified for the Boston Marathon 9 months after her transplant.  She’s got great faith and great humor and is my sister from another mister.  I’ve felt close to her for over a year, even though she lives in California, and it was great to meet her.  We shared a room and had our Kappa Lambda Delta Amyloidosis Sorority slumber party and then did a whirlwind drive through DC on our way to the airport after the meeting was over.  Lots of love and fun squished into 29 hours.   

I got to reunite with Joanne Campbell who I met when I was at Mayo and visited while she was having her stem cells harvested.  I finally met Carole Harber and her husband David.  He’s a cyclist who had his bike set up on a trainer in their hotel room during his stem cell transplant about a year before mine.  Last year, he was the number one ranked road racer in his age group in the state of Oklahoma.  I saw the actor Michael York again, first met him at the gala in Nashville.  He, his wife and I had a lovely chat.  Sharing a room with more than 100 folks who are “my people” was a blast. The love, energy and support was just phenomenal.  Hearing stories from folks who have had such a tougher road with this disease than I have inspired me and filled me with gratitude. 

I also finally met in person my fellow Amyloidosis Kat that I met on Twitter.  Kat Timpf is a reporter for the National Review and a Fox News Correspondent. Her mom died from cardiac Amyloidosis last year only three weeks after being diagnosed.  We’ve shared some messages on Twitter and tend to re-tweet one another’s amyloidosis tweets.  She got up and shared her mom’s story at the meeting. You can hear an interview she gave about it here.  https://soundcloud.com/ben-kissel/katherine-timpf  It was heart-wrenching to hear her talk about how the family celebrated when the doctors determined that her mom didn’t have cancer.  They didn’t know it at the time, but she had something worse.  Let that sink in.

Josh Lacy shared his story of Amyloidosis.  He was diagnosed with AL Amyloidosis with cardiac involvement in 2012 when he was in his mid-30s.  He was married with a 4 year old daughter and 1 year old son.  You can imagine how devastating this diagnosis would be at that time.  During his talk, he made an impassioned plea so he would be alive to see all the milestones ahead of his children—high school, college, marriage and adult life.  Boy do I know that feeling.  He’s a former college baseball player and talked about how hard it is to know his son will never know his dad as the strong athlete Josh once was.  That really choked me up.   He talked about being too weak to go into school for his daughter’s first day at kindergarten but he was able to go to school in his wheelchair. He also gave me a good laugh when he described how he didn’t have the strength to lift his son when he was sick so if his son didn’t want to do something, he’d lie on the floor and look at Josh like he was saying, “What are you going to do now?”

Later in the day, Josh came up to me and introduced himself.  I told him my name and he said he knew who I was through my blog.  That his wife had read it and used it to motivate him during some of his tough times basically saying, “Look at what that lady is doing.  You can get back, too.”  (My words, not theirs.)  That was my whole reason for starting the blog…to give hope to people that it is possible to have a full and satisfying life with Amyloidosis.  I felt so honored to have served as a tiny bit of motivation for Josh and his wife Adrienne.  I had a great chat with them.  Such a great couple that joined this crappy club.  I am so inspired by their faith and strength.

I thought about my girls as Kat was telling her story.  If I had cardiac involvement, I’m firmly convinced that I wouldn’t be alive today after my delay in diagnosis.  I would have been like Kat’s mom or my friend Cara or any of those other cardiac patients who have a median survival of 18 months after diagnosis.  But, because of some variable portion of the germ line of some gene, my free light chains didn’t effect my heart.  And so I’m here today trying to use the time God has given me as effectively as possible.  

My doctor introduced me to Cara who was diagnosed about 6 months after me.  I told her I'd be her big sister in this crappy sorority we both pledged in to.  She had cardiac involvement and, even though her hematology was starting to respond from chemo, her heart was too damaged and she died about 6 months after being diagnosed.  She left behind a loving husband, three children between the ages of 9 and 14, and scores of loving family members and friends.  I regularly think about the comment one of Cara’s sisters in law said to me at her viewing, “We wanted your story to be her story.”  I vowed then to try to change the story for a Cara in the future. So that someone with cardiac Amyloidosis could have my story.  Yesterday was a HUGE step in that direction.

Ten Thousand Tons

“And on the worst days

When it feels like life weighs ten thousand tons

She's got her cowboy boots and car keys on the bedstand

So she can always run

She can get up, shower, and in half an hour she'll be gone”

As some of you know, I just finished attending the Teradata Partners conference in Anaheim. I call it spring break for nerds but it's always a great time of learning new things and re-connecting with colleagues from across my almost 20 years in the data and analytics field.  Oh and there's a fair amount of fun. I've been on the planning committee for the conference for the last 4 years and this was my last conference as a committee member. It was bittersweet. 

Last year I was the president so I gave a 20-minute keynote in Monday's general session and was emcee for the closing general session on Wednesday. The highlights included telling the story of some good work, meeting football commentator Herm Edwards, and wearing a yellow spandex Kill Bill suit and, later, my cycling kit in front of 3,000 of my closest friends.  I had numerous people stop me this year to tell me how much they enjoyed my time on the stage last year. (I just realized I never wrote about that here...sorry.)

While I wasn't on the big stage this year, I did present with Dean Furness, a colleague who works at Wells Fargo in Des Moines. Dean and I met when I went to speak at a professional development day at Wells Fargo in December 2011. He also presented and we became fast friends at a dinner the night before.  At the time, he was working with a former colleague and volleyball partner, and friend David Brenner.  We talked about the unique experience of being David's friend and colleague.  :)

Seven days after we met, Dean was in an accident at his family farm—an 1,800-pound hay bale rolled off his front loader and crushed him underneath.  It severed his spine and he’s now paralyzed from the chest down.  But, through a lot of faith, support and hard work, he got back to work faster than anyone predicted--including the Social Security admin who came after him to return his disability payments because he obviously couldn’t have been disabled since he came back so quickly.  Ten months later, he presented at the Partners conference in October 2012 and I had the pleasure of introducing him.

Now, those of you who have been around a while may recognize that October 2012 was when I first realized I was sick.  While packing for the Teradata conference that year, I had to go buy a new pair of pants because my jeans wouldn’t button, even though I hadn’t gained weight.  And some of the pictures from the conference show the slight signs of my facial edema.  Hmmmm?

Well, we know how that turned out.  Dean’s triumphant return coincided with the beginning of my decline but, like Dean, through faith, support and hard work I’ve made it back, too.  So Dean and I decided to do a session that talked about how people can use business measurement constructs to help hem “up their average.”  The title was “Curve Busters: Looking for a Dare to be Great Situation.”

One thing Dean noted, as he rolled across the stage in his wheelchair, was that people look at him and automatically know he’s been permanently affected by his experience.  It’s not obvious when someone looks at me.  But I have been permanently affected, too.  And lately, it’s been really weighing on me creating this grating, and at times overwhelming, level of frustration.  I just didn’t know what was frustrating me but it was a consistent feeling. 

On Thursday, after the conference was over, I went up to LA and had dinner with some dear friends.  They’re facing their own health challenges that are impacting their ability to have a life that is as fulfilling as they’d like.  We talked about the struggle of keeping it together through the good days and the bad days.

After dinner, I went, by myself, to see one of my new favorite performers, Frank Turner at a small theater with a capacity of about 1,500 people.  It was a great show and a great crowd and well worth the 45-minute drive back to Anaheim at 11:30.  The show was a big sing along and as he played the song “I Am Disappeared” (which I hadn’t listened to before), snippets of the lyrics just struck me and I had moment of catharsis and epiphany.

“Of people wrapped up tight in the things that will kill them  (my disease)

Of being trapped in a lift plunging straight to the bottom (if I relapse)

Of open seas and ways of life we've forgotten (of being able to perform mentally and physically, and not worrying about the disease and it's complications)

I keep having dreams” (of the time before I was sick)

“She woke up screaming in the middle of the night

Terrified of her own insides” (of the chromosomal defect that I have that is the worst factor for long-term survival)

“And on the worst days

When it feels like life weighs ten thousand tons

She's got her cowboy boots and car keys on the bedstand

So she can always run

She can get up, shower, and in half an hour she'll be gone”

“I keep having dreams of things I need to do, And waking up but not following through” (I am so distracted, I think that’s why I stay busy doing so many things)

“And come morning

I am disappeared

Just an imprint on the bedsheets

I'm by the roadside with my thumb out”

http://youtu.be/6t2Hz6KL3uE

I’ve had many days where I felt like I wanted to run away, just get in the car and drive far, far away. There are days when the pressure of living with this disease, dealing with known and possible organ impact, maintaining a career, trying to have a normal life, dealing with challenges at work blah, blah, blah are like a crushing weight on me.  It’s not a lot of overwhelming, attention-grabbing thoughts or even an acute emotional reaction.  Its just there are times where I feel like I’m trudging along wearing a 100-pound backpack.  Maybe not ten thousand tons, not debilitating, just a constant heavy presence.

I feel alone so much of the time, even when I’m around friends, family and colleagues.  It struck me that it wasn't a coincidence that I had this catharsis alone in the middle of a jam packed room.  I’m alone in this struggle no matter how many people I know that love me and care about me.  And it’s made me hyper-sensitive to anything that I think contributes to that sense of separation.  Honestly, there are fleeting moments when I wonder why I'm alive, is what I'm living now worth all the work to get it and, if I relapse, worth all the future work to keep it.  I talk myself out of it pretty quickly, but it happens occasionally.

When I feel like I want to run away, I realize that there’s nowhere to run to get away from this.  Dealing with this disease is the definition of a “fact of life” for me…barring some significant and unexpected medical advances,  I will have to deal with this as long as I’m alive.

From what I read, what I’m dealing with isn’t uncommon for cancer survivors.  I read an article in Psychology Today entitled “The New Cancer Survivors”  noted this—

And as more and more people are living with cancer as a chronic manageable condition, often outlasting the crushing prognosis that the disease will cut their life short, the psychological nature of their situation becomes clearer. Theirs is a hyper-real, intensified state of existence in the liminal space between being terminal and cured. In many cases, after believing that their death was imminent and coming to terms with that fact in whatever way they could, they find themselves instead navigating a new and wildly uncharted reality. Their lives, unnervingly interrupted, are resumed in a form that is somewhat familiar but permanently altered.

So I continue on, permanently altered, navigating this uncharted reality.  I have come to terms with death.  I'm not afraid for me and, as much as I want to be around for Al, Amy, Claire and all my other friends and loved ones, I trust that God will provide for them what I might have.  It's not a fear of death that weighs on me, but a nagging question of what should my life look like and how do I get past this sense of isolation?  I think that's what drives my desire to run away..."If I'm going to feel alone, well then I'll just BE alone."

But, as my buddy Frank Turner says in one of my favorite songs “We can get better, because we’re not dead yet.”  So, I'm not dead...I'll try to get better.

Thanks, Frank!

"I Am Disappeared"

I keep having dreams

Of pioneers and pirate ships and Bob Dylan

Of people wrapped up tight in the things that will kill them

Of being trapped in a lift plunging straight to the bottom

Of open seas and ways of life we've forgotten

I keep having dreams

Amy worked in a bar in Exeter

I went back to her house and I slept beside her

She woke up screaming in the middle of the night

Terrified of her own insides

Dreams of pirate ships and Patty Hearst

Breaking through a life over-rehearsed

She can't remember which came first

The house, the home, or the terrible thirst

She keeps having dreams

And on the worst days

When it feels like life weighs ten thousand tons

She's got her cowboy boots and car keys on the bedstand

So she can always run

She can get up, shower, and in half an hour she'll be gone

I keep having dreams of things I need to do

And waking up but not following through

But it feels like I haven't slept at all

When I wake to a silence and she's facing the wall

Posters of Dylan and of Hemingway

An antique compass for a sailor's escape

She says you just can't live this way

And I close my eyes and I never say

I'm still having dreams

And on the worst days

When it feels like life weighs ten thousand tons

I sleep with my passport

One eye on the back door

So I can always run

I can get up, shower, and in half an hour I'll be gone

And come morning

I am disappeared

Just an imprint on the bedsheets

I'm by the roadside with my thumb out

A car pulls up, and Bob's driving

So I climb in

We don't say a word

As we pull off into the sunrise

And these rivers of tarmac are like arteries across the country

We are blood cells alive in the bloodstream

The beating heart of the country

We are electric pulses

In the pathways of the sleeping soul of the country

We are electric pulses

In the pathways of the sleeping soul of the country

We are electric pulses

The sleeping soul of the country

The sleeping soul of the country

The sleeping soul of the country

Kath's Cath Update

I've had lots of discussions with my Medical Advisory Board (MAB) about the need to have the cardiac catheterization to evaluate my possible Constrictive Pericarditis.  Remember, my cardiologist recommended this test so that he could either rule out the condition or give him a better set of data from which to manage the condition over time.  It would not result in any treatment or recommended lifestyle change for me.

My MAB consists of:

• Husband, ICU Nurse
• Daughter, Nurse Practitioner
• Brother in law, Internal Medicine Physician
• Oncologist, Oncologist :)
• Oncologist's Nurse Practitioner (you get the drift)
• Sisters in law, 3 nurses including a CVICU nurse

After lots of discussion, I've decided not to have the cath done.  It's an expensive, invasive and risky procedure and the results wouldn't change anything.  As good ole Dr. Tom Bishop would say, you collect data so it can serve as a rational basis for action.

They're going to monitor my heart via MRI every 12 months and IF they notice a change in the structure, then have a cath and determine if surgery is needed.  That surgery would require cutting a "window" in my pericardium so, needless to day, I'd prefer to delay that as long as possible.

Bottom line, no cath for Kath.  In the meantime, he said I can keep doing what I'm doing.  He said the condition is "self-limiting" which means if I stress my heart too much, I just faint.  That's not bad.  My stupid human trick I learned when recovering from my stem cll transplant is how to recognize I'm starting to faint and prevent it.  I did it on the Millenium Force rollercoaster, I'm sure I can do it on the bike or in my workout classes.

All that other stuff I listed in my last post, I'm going to get busy doing THAT!

Just when you thought it was safe....

So, as you can tell from my various activities--riding strong in Pelotonia, hiking  and white water rafting in the Cascades, racing in cyclocross--my health has been pretty good lately.  But....there's been this issue that has caused me some concern and been a source of worry for my husband, who's an ICU nurse.  When I exercise, my heart rate has been about 20 beats per minute higher on average and at max compared to when I got sick.  On long, hard bike rides, I've had extended periods of time during which my heart rate was above my max HR.  And, I've had some near fainting spells during particularly difficult times.

I did the responsible thing (shocking, yes?) and consulted with my hematologist.  Yes, it seems odd to consult with a hematologist on a cardiac issue but I assume all issues are due to the effects of the amyloidosis or treatment so I start with Dr. Efebera.  She referred me to a cardiologist at Ohio State Ragavendra Baliga, MD.  I met with him September 17 and, like every other physician I've encountered at Ohio State, I was impressed with his knowledge, friendliness and balanced perspective.  I loved his icebreaker question.  He asked me, "What are you famous for?"  I answered, "Kicking Amyloidosis' butt and being a data geek."  (Confirmation came that same week when Pelotonia tweeted out a link to my story in The James magazine-- https://twitter.com/Pelotonia/status/647018183652737024 and Teradata Partners posted an interview with me talking about how we select sessions for the conference-- http://www.teradata-partners.com/blog/post/global-compelling-stories-value ) After reviewing my history, he ordered some tests--a cardiac MRI and a VO2 stress test.

I've had a cardiac MRI before.  You get an IV to deliver the contrast, you get loaded into the MRI machine and you spend about an hour following instructions of when to breathe and when not to, and listening to noises that sound to me what an apoclyptic robot war would sound like.  The VO2 stress test is the usual stress test--walking then running on a treadmill with ever increasing pace and incline--WITH a mask over your face and your nose pinched shut so they can measure how your body uses oxygen.

First, the good news....the VO2 test shows that I'm in pretty good shape.  I performed at 138% of what is expected for someone of my age and gender.  Dr. Baliga says he never sees anyone over 100%...well, he's a cardiologist treating advanced and complex cases.  Logic says his patient population won't be filled with folks who have high cardiovascular function.  He told me my VO2 max was 36...and then he told me that Michael Phelps' was 71.  My rose-colored glasses helped me see that halfway to Michael Phelps for a 53-year-old woman wasn't too bad.

The bad news is that the MRI showed some thickening of the pericardium, that's the fibrous sac that contains the heart.  It also showed some enlargement of the atria, the top chambers of the heart.  These are not the chambers that pump the blood out, they're the ones that receive blood from the body and the lungs.  Their walls are thinner than the lower chambers, the ventricles, that do the pumping.  

The MRI report said there was evidence of "constrictive physiology" which means the thickened pericardium, the sac that holds the heart, could be pressing in on the heart and constricting it's function.  Like a balloon, when you squeeze on one part, it causes another part to enlarge.  That may be why my atria are enlarged.

So, Dr. Baliga wants me to undergo a cardiac catheterization to measure the pressure in my chambers....to know whether my heart is constricted.  Initially, I wasn't a big fan of the idea.  When I asked my standard question regarding any major test, "How will the results change my treatment or management of my disease?"  The answer, as so many things when managing a complex disease, wasn't as straightforward as I would have liked.  Bottom line is, it could rule out this constrictive physiology and show the heart isn't constricted and this isn't something we need to monitor and manage over time.  Or it could show that this is a problem that we need to monitor and this would give us a very thorough set of baseline data so we could pick up changes and know when to start treating it....through surgery by cutting a window in the pericardium.  Eeek! 

Dr. Baliga called Amyloidosis an outlier disease because there's not a lot of proven treatment approaches once you get beyond the basics of the disease.  And he said I'm an outlier within the Amyloidosis patient population so there's no literature on how to deal with my situation...everything is anecdotal.  The good thing is, he trained at Boston which is one of the centers of excellence in Amyloidosis and has a colleague there who he will consult on my case after he gets the results from the catheterization.  

As I was texting with my nurse practitioner Tammy about whether to have the cath, she strongly recommended it saying, "This is your heart, we don't want it broken."  I said, "A ruptured atrium sounds kinda serious" and she said, in her usual understated manner, "Ya think?"

So, on October 28, I'll have a cardiac catheterization done by a cardiologist specifically chosen by Dr. Baliga because, as his nurse comfortingly (?) noted, he has a lot of experience with patients with heart failure.  And then we'll see if I have an additional problem to monitor or not.  I was thinking about how well things had been going over the last year and it made me think of the tag line from Jaws 2....

But, I'm going to "stay in the water."  Between now and then I have two cyclocross races, a trip to Nashville for an Amyloidosis Gala and patient meeting, a trip to Anaheim for the Teradata Partners conference including a doing a presentation called "Curve Busters: Looking for a 'Dare to Be Great' Situation" (with my friend Dean Furness who became a paraplegic from a farm accident a year before I was diagnosed), a Frank Turner concert in LA the night the conference ends and dinner with my friends Reid and Bethy, a party with my Pelotonia team, leading my church's pledge drive as chair of the Stewardship Committee, organizing weekend food kits my church is providing for 75 students on free lunch at a nearby elementary, starting a new mentoring relationship with a 6th grader at Champion Middle School, watching some Gator football, doing some exercising and yoga, keeping the day job, wife-ing a little for Al and Mom-ing a little for Claire.  

No time to worry.  God has given me the grace to deal with what is, and not worry about what might be.  

Keep on keepin' on my friends!!