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.

Goodbye 2013!

Well, my trip to Mayo was as wonderful as I could have hoped.  Bottom line is that Dr. Gertz said that I don't meet their criteria for Smoldering Multiple Myeloma (SMM).  Granted, he didn't say I don't have it but that's because there's not a lot of consensus among the hematologists about when someone actually has it.  This table from a Mayo article in the December issue of the journal of the American Society of Hematology shows 9 different sets of criteria to diagnose SMM.  In the article, Mayo proposes new criteria to define SMM.  I'll go with their definition.

When I asked Dr. Gertz about the Revlimid therapy (maintenance chemo) that I'm on, he said that even if I did have SMM I wouldn't be considered high risk and they would not have me on maintenance chemo.  He said something to the effect of "there is no basis in the literature for you to be on Revlimid but I can understand a physician's intellectual leap to think it would be appropriate."  Translating the physician-ese, I think that says, "There aren't any studies that say its effective but your doctor's usage is logical."

The best news was when I asked him if I could expect a durable, long-term remission.  Technically, Amyloidosis is incurable and the best outcome is this durable, long-term remission.  As I've said to many people, I don't need a cure, a 30-year remission is as good as a cure for me.  Dr. Gertz said that it would be reasonable for me to expect that and he didn't see anything to indicate it could not be achieved.  Now, 30 years is beyond what's expected but with the treatment advances that have been made recently and new ones currently in the works, I really believe it's possible.

When I booked the appointment, the Mayo rep told me to plan to spend 2 days there in case they needed additional tests.  I didn't need any additional tests.  That was another confirmation of the expertise of my local physician.  My medical record from Ohio State had all the information Dr. Gertz needed to evaluate my case.

My appointment was at 8am on Monday.  I was back in the exam room shortly after 8 and Dr. Gertz came in around 8:15 wearing a nice pinstripe suit, white shirt and red tie.  It was nice that it was just him, no fellows, no medical entourage.  I love contributing to physician education and training but one on one time with an expert physician is a wonderful luxury.  The exam room looked like it was straight out of the 40s or 50s, in a cool way, not a run-down way.  The exam table was this cool, retro looking wooden table.  There was natural wood wainscoting around the room and one wall featured Dr. Gertz' diplomas, awards and some artwork.  Seemed like it was combo exam room and office for him.

I brought my 400 page medical record from Ohio State as instructed by Mayo.  As I lugged the tome around with me, clutching it like Smeagol clutched his precious ring, I wondered how much of it he would actually review...or would he have some fellow do the data mining.  He came into the room, introduced himself and began reviewing my record as I sat there.  I kid you not, the man reviewed each and every page of this record.

Some pages he just scanned and some he spent a lot of time on.  He circled and underlined items and made lots of notes on another page.  At one point, he pulled up the x-rays from my myeloma survey (basically an x-ray of every bone in my body done when I was first diagnosed) looking at the spots the radiologist identified as possible lesions...a sign of myeloma.  He told me that I didn't have any lesions.

I was impressed with how thorough he was.  I changed into a gown and he examined me doing the usual heart and lung physical exam and then he spent some time on my liver.  He was making his notes with a Mont Blanc ball point pen.  While he was examining my liver he asked if it was ok for him to make some marks on my abdomen and I obliged, of course, so he proceeded to draw some lines with his nice pen.  I kinda wanted to ask him to autograph my belly while he was there.  I also resisted the temptation to take a selfie with him despite my friend's challenge to do it.  I thought it might make the relationship a bit too informal at the start.

He agrees with Dr. Efebera's assessment that my liver may never return to normal size but it will reduce over time. He said my kidneys are showing good recovery and my liver function tests show recovery even if it is still significantly hardened and enlarged.

He spent about 1 hour and 15 minutes with me and answered every question I had.  His calm and confident demeanor was wonderful and he was so thorough in all his answers.

The best part was that I was done after that. I didn't need the 2 days so I called the airline and changed my reservation so I could return that night.  (And its fortunate that I did because my original flight out of Rochester was cancelled so if I had stayed with my original travel plans, I would have had to spend an extra day.)

While I was there, I got to meet Joanne, another Amyloidosis patient who is there for her stem cell transplant.  We met for coffee on Sunday and it was great to meet someone else fighting the same fight.  Her attitude reminds me a lot of my own and it was encouraging to meet another person who fully expects to beat this disease.  On Monday, she was getting her stem cells harvested and I stopped by for a visit.  I was there as they finished the harvesting and her husband took a picture of us posing with the magic potion that will send her into remission.

The trip was a great way to close out a crazy 2013.  (You can see more photos from the trip here.)  When I look back on the year, I am amazed at the roller coaster.  Yes, I lost my father and father-in-law; yes, I was diagnosed with and treated for a rare, incurable blood disorder; and yes, at each step of the way felt the love of God and so many friends and family members.  While the diagnosis was shocking, I was on the path to treatment three days after diagnosis and felt the peace and confidence from God the day after I was diagnosed.  I am so fortunate to have expert care available locally and have a physician who is just as caring as she is knowledgeable.  Check out this video of Dr. Efebera for some insight.

Yes, I had three weeks of chemo prior to my month long hospitalization but the timing worked out so I could go on our family ski trip between the chemo and admission.  I still can't believe what a blessing it was that I had the strength (limited as it was) to ski every morning we were in Colorado returning home 9 days before my admission.

I was also able to attend the Fleetwood Mac concert with my daughter and son-in-law, Amy and Sean, and my dear friends Allyn and Jasper on April 4, four days before my admission.  Yes it was a challenge due to my abdominal swelling and edema in my legs but I did it and really enjoyed it.

Yes, I missed all of Claire's freshman lacrosse season in which she was the second leading scorer on her team and made 1st team all-conference and second team all-district.  But, since Alan was off work on Family Medical Leave, he was able to attend all of them which was a rare treat since his work schedule makes it challenging for him to attend her games.  The board for the lacrosse booster association made a photo book for me with lots of pictures and her stats from the season. They gave it to me at the banquet on May 22 which was my first outing after getting out of the hospital. Yes I needed a wheelchair but I made it.

Yes, I had a month-long hospitalization and two months of convalescence but the expressions of love and support from family, friends and coworkers was innumerable.  Visits, gifts, cards, prayer, positive vibes, meals, milkshakes, encouragement....so many expressions of love that I still get teary-eyed when I think about it.  Yes, it was a scary time but it was filled with so much love.

The second half of the year was an ongoing progression toward normalcy.  Even though I was still recovering, I was able to do a lot of "normal things" like:

• travel to Claire's lacrosse tournaments in Virginia and New Jersey in June and July (and I didn't need the wheelchair or walker with seat that the physical therapist thought I might need)
• enjoy visits from several dear friends--Allyn and Jasper who came up from Virginia, my college roomie Deb before she moved to Mexico and my former boss Monica who lives in Australia but was in Ohio visiting family
• attend the wedding of Allyn and Jasper's son in California in August (and eat some Cronuts!  Thanks for the reminder, Jasper)
• go to Cedar Point for 2 days.  We drove up at 7am on Saturday and I stayed at the park until it closed at 10pm (with one little nap in the afternoon--Al took one, too.)  I learned how to pull myself out of a fainting spell during the first drop on this roller coaster when we were in the front row and I wanted to do the entire ride with my hands up. Underestimated the effect of the diuretics I was taking at the time and started to faint but pulled myself out of it.  My new trick I learned from my illness.
• take Claire on a lacrosse recruiting visit to San Diego in September
• attend a few days of the President's Cup golf tournament in October with Allyn and Jasper
• attend the Teradata Partners Conference in Dallas in October for the entire week and was able to meet all my committee obligations
• take Claire and a teammate to a lacrosse tournament in South Carolina in October (without Alan)
• take a business trip to United Healthcare in November to discuss their challenges in using customer data (somewhat ironic since they're my health insurance company.  I did keep the card for their Chief Customer Officer...just in case)
• spend a weekend baking Christmas cookies at Cookiepalooza with my sisters
• hike 10 miles during our Thanksgiving trip to Hocking Hills
• visit Allyn and Jasper in Blacksburg for Claire's annual gingerbread making and drive back in an ice storm (without Alan)
• go to the Mayo Clinic.

As I look to 2014, I've decided that as of 12:00:01 tonight, I am no longer sick.  I'm leaving Amyloidosis in 2013.  I knew going in that I would beat it and I have...at least for a time.  Now, I'm not going rogue...I will stay vigilant about my follow-up care and monitoring for any sign its returning but the sense, the attitude that I have an illness will stay in 2013.

Tomorrow, I will have my usual White Star champagne with strawberries and the black-eye peas that are currently cooking in the crockpot.  The black-eye peas are a southern tradition.  They're supposed to bring good luck but I don't think I'll need it.

"Enough, enough now"

I've been thinking a lot about my trip to Mayo and what I'm hoping to get out of it.  Of course, I have a document with a list of questions...that I keep on my Google drive so I can add to it whenever I think of a question...but I want to be prepared with a well-thought out answer if Dr. Gertz, or someone else asks me, why I'm there.  I think my answer will be...

1. Confirm the diagnosis of Smoldering Multiple Myeloma (SMM).  If it is confirmed, I'd like to know find out my risk for progression to Multiple Myeloma.  If its not confirmed, I want to discuss the pros and cons of the Revlimid maintenance.
2. Hear his perspective on long-term prognosis, risk for relapse and how to identify a relapse has occurred.
3. Discuss the liver and kidney organ response.

I'll go with those 3 because his answer will most likely cover a lot of the random questions I have (What is the prognostic significance of my t(11;14) translocation and 13q deletion found on the cytogenic study?) plus people like things to be in groups of 3.  Those of us who grew up on Schoolhouse Rock know its the magic number.

I'm sure I'll have many more questions but those are the big conversation starters.

My real goal is to leave Minnesota...or when I have my final consult with Dr. Gertz...with the information and confidence I need to fully enjoy this current period of remission and recovery.  Don't get me wrong, I'm pretty happy and surprisingly not worried about my disease.  When people ask me whether Alan is going with me and I tell them he's not, many people seem a bit incensed by that.  But, this isn't a trip where I'm fearful or facing an emotional situation.  It's almost like a business trip...just gathering those last few pieces of information so my Strategic and Self-Assurance strengths I discussed in the last post are fully functioning.  

Yes, I know my ultimate assurance is not from Self but from God and I run the risk of creating myself, my doctors or my knowledge as a false idol that occupies a place that is rightfully God's.  My peace about my disease, my acceptance of either diagnosis (with or without the SMM) and, my belief that God is in control and whatever I do or my doctors do cannot undo God's dominion over this give me confidence that I have God in His rightful place as I go through this.

I was chatting with a colleague at work today who serves on the board of a local Multiple Myeloma fundraising group.  She's been a wonderful supporter, confidante and guide as I've gone through my treatment.  When I told her about my trip to Mayo, she said that Dr. Michael Caligiuri, CEO of the James Cancer Hospital, will tell you to get treated by an expert and always get a second opinion. 

The second opinion part is pretty clear criteria but how do you define "an expert"?  Is it the best in your town, state. region, country, the world?  I know that Dr. Efebera is an expert...but Dr. Gertz is a more experienced expert...or is he more of an expert?  As I wrestled with this idea, the question of "How much is enough?" came into my mind.  When is my information enough, when is the expertise of my medical team enough?  It just feels like its a big brain teaser I'm trying to figure out.  At some point, I hope its enough.

These thoughts of "enough" made me think of one of my favorite scenes from my on of my favorite movies.  It's a scene about knowing when you've done enough and knowing its time to move on.

(Click here to see the video on mobile-- http://youtu.be/dNJe7LmPbvU .)

I'm hoping I leave Mayo with a sense of "enough."  Now, I don't think Dr. Gertz and I actually will re-enact the scene (mostly because he'd have to be Keira Knightley and I'd have to be Andrew Lincoln and that's just weird.)   But, I am hoping to walk away with a sense of "enough, enough now."

Send your prayers and positive vibes that I can achieve that.  It would be a great feeling with which to start 2014.

Liam or Morie

For the longest time, my celebrity crush has been Liam Neeson.

Whether its his work as an opportunist  turned Jewish rescuer Oskar Schindler in Schindler's List, the kick-ass dad in Taken or the sensitive widower in Love Actually, his acting combined with his looks has put him at the top of my celebrity crush list.

But now, he's been replaced by this guy....

Yes, perhaps not as attractive and maybe not as famous as Neeson to the uninformed but to folks knowledgable about research and treatment of Amyloidosis, this guy is a rock star.  This is Dr. Morie Gertz, Chair of the Department of Internal Medicine at the Mayo Clinic and one of the top physicians and researchers in Amyloidosis.  When I called to make my appointment with Mayo, they didn't tell me who my appointment was with.  When I got the letter with the details of my appointment and saw that it was with Dr. Gertz, I felt like I won the lotto.

So many of the studies I've been reading over the last few months have been authored by him.  He's the editor of the book that is considered the gold standard on diagnosing and treating Amyloidosis and he's one of the top physicians in the world at treating Amyloidosis.  From everything I've heard from people treated by him and who have met him, I will love him.  

If he's unseated Liam, I already do.