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.

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.

It's Complicated

I decided to go to the Mayo Clinic for a second opinion on my prognosis and treatment plan.  Mayo is one of the top Amyloidosis centers in the world and with such a rare and poorly understood disease, I thought it was a good idea to have them weigh in on my case.  As someone said on the online Amyloidosis Support Group, "I quickly learned that the doctors that wrote the articles and books do know a lot more about Amyloidosis than the local doctors that read their articles and books."

Granted, my doctor is much more informed than the average local doctor but her primary research focus is Myeloma...related but a separate disease.  She's presenting 6 papers at the American Society of Hematology meeting next month--4 on Myeloma, 2 on complications from stem cell transplants, 0 on Amyloidosis.

Given that I have met my out of pocket maximum for the year and Mayo is considered in-network for my insurance, it was important to get the appointment done before the end of the year.  So, I fly from Columbus to Rochester, Minnesota on Sunday, December 29th for my appointments on Monday, December 30 and Tuesday, December 31.

To prepare for the appointment, I needed to get a copy of my entire medical record from Ohio State, copies of all my radiology studies (3 ultrasounds, two CTs, two MRIs and an echocardiography) and the actual glass slides from my biopsies. I was worried how long that would take so I went on Monday and put in my request.  They pulled together the copy of my medical record--all 350 pages of it--while I waited.

I have access to lots of test results and other information on-line but it doesn't include any of the info from when I was an inpatient.  Reading through the inpatient notes was really interesting.  Some of the info I remembered, some of it brought back memories and some of it I had no recollection of at all.

It was interesting to read through it, especially the doctor's notes.  During one of my appointments in June, Dr. Levin described me as "a thin chronically ill appearing but animated middle aged woman."  Even at my lowest point physically, I still had some life in me.  Can't keep me down!

I found two things in the medical record that caused me some concern.  I always thought, based on my reading and guidance from folks in the support group who've had this disease lots longer than me, I had achieved a Complete hematologic response.  But my doctor's notes only list a Very Good Partial hematologic response.   At first I was a little concerned because of this graph--

But, the article that goes along with the picture says "Patients achieving a CR or VGPR after HDM/SCT, as defined by the new criteria, had indistinguishable OS (overall survival) and EFS (event free survival), with median OS exceeding 9 years."  Ok, so I'll take that.

The other thing I found that was concerning was a diagnosis of Primary Amyloidosis with Smoldering Multiple Myeloma (SMM).  So it's not enough for me to get a rare disease that is only diagnosed 3,000 times per year in North America, I also get an add-on bonus feature of the possibility of developing Multiple Myeloma.  That's what is complicated.

But, one of the people I met through the online support group said that he had the same diagnosis from his local doctors.  He went to Mayo and Dr. Gertz, who is one of the world leaders in Amyloidosis research and treatment, cured him of the SMM in a few minutes.  He said Dr. Gertz looked at all his test results and determined the other doctors were incorrect and the only issue he had was Amyloidosis.  I sure hope that's what happens with me.  I never thought I'd look forward to getting another diagnosis of Amyloidosis but given this complication, I'm hoping for it now.

My flight back from Mayo lands at 11:59 PM on December 31.  Getting the Amyloidosis only diagnosis would be a great way to close out 2013 and move into 2014 with even more hope than before.

One of the songs I've been listening to a lot lately is "Come Thou Fount of Every Blessing" by Sufjan Stevens.   It's a classic hymn with the words written in 1757.   One of my favorite lines is "Here I raise my Ebenezer;  Hither by Thy help I'm come;".  What's so great about raising an Ebenezer?  This information I found online seems to explain my basic understanding of the line--

In 1 Samuel 7, the prophet Samuel and the Israelites found themselves under attack by the Philistines. Fearing for their lives, the Israelites begged Samuel to pray for them in their impending battle against the Philistines. Samuel offered a sacrifice to God and prayed for His protection. God listened to Samuel, causing the Philistines to lose the battle and retreat back to their own territory. After the Israelite victory, the Bible records: “Then Samuel took a stone and set it up between Mizpah and Shen, and called its name Ebenezer, saying, ‘Thus far the Lord has helped us’ ” (1 Samuel 7:12). 

The word Ebenezer comes from the Hebrew words ’Eben hà-ezer (eh’-ben haw-e’-zer), which simply mean “stone of help” (see Enhanced…, 1995). When Robinson wrote his lyrics, he followed the word Ebenezer with the phrase, “Here by Thy great help I’ve come.” An Ebenezer, then, is simply a monumental stone set up to signify the great help that God granted the one raising the stone. In Robinson’s poem, it figuratively meant that the writer—and all who subsequently sing the song—acknowledge God’s bountiful blessings and help in their lives. 

The next time you sing about raising your Ebenezer, you will be able to “sing with the understanding” that you are acknowledging God’s help in your life (1 Corinthians 14:15).

So, regardless of what happens I'll raise my Ebenezer--acknowledging God's bountiful blessings and help in my life.  I guess I need to find a big rock!