Roads Less Traveled

I’ve just finished one year of eculizumab.  Yes, it is still the most expensive drug in the world.  Yes, insurance is currently covering the cost, once the deductible is met (which this year would have been on January 1st–ha–take that, no copay for the rest of the year!).  But the biggest “yes” is that it is working.  And that is a huge YES!

The infusions are very easy.  I arrive at the clinic every other Monday promptly at 8 AM, I go through all the COVID precautions (including mask, temperature, hand-washing), get my IV, infuse for about 30 minutes, wait for an hour to be sure nothing catastrophic happens, then head back home.  I am a little bit tired for the rest of the day, but that’s it.  At least so far.  And I’ve got 26 or so treatments under my belt by this point.

All of my numbers are better.  My proteinuria is back within the stratosphere.  My creatinin number is lowering, and that shows improvement in the kidney function.  I haven’t seen Dr. Zand since January because of COVID.  I had a little run to the ER on March 12 due to a fever (and here’s the really annoying thing about being immuno-suppressed: if you get a “fever” over 100.4, you are supposed to go to the ER, which is a major pain).  Then I got sent home with orders to stay home due to COVID.  I talked to Dr. Zand by phone in July.  She’s very pleased with how things are going.  The one thing I did not like in our conversation is that she said sometimes eculizumab stops working and is itself deposited in the kidneys.  What I think that means is that I will be on eculizumab in one form or another until I’m not.  And I have no idea what that means in terms of time.  Good news:  there is another drug (a variant of eculizumab) that can be given once every eight weeks, instead of once every other week.  We are going to go for that one when it’s time to reauthorize in November.  I have no idea of the cost of this drug, but surely it’s not more than the one I’m currently on.  (Which means that I have no idea and of course it could cost more money because it’s a drug for orphan diseases and apparently there’s no limit to the cost of those drugs.)

Oh, and see how adorable Olive is now.  ZilkerBark took these photos.  Olive has everyone’s number, check out the wink.

. . . Or what happened between November and now.  I got sick.  Lots of sick.  Apparently prograf + cellcept + steroids + daratumumab-still-in-your-system = petri dish for anything contagious.  So I’m going to go through this like the lightning round on a game show because it was un-fun (and frequently involved illnesses of an, shall we say, intestinal nature).

January was “Fever of Unknown Origin” month.  When you are immuno-suppressed, Fever of Unknown Origin (FUO) is cause for concern when your temp is over 100.4, because the doctors have to try to figure out what is causing it.  In my case, one Austin hospital visit and one Mayo clinic visit later, we still don’t know.  The speculation is: “a virus.”  To that, I say FU, FUO!

February was my regular check-in and check-up at Mayo.  It turned into “C Diff and See Your Way to the Mayo Hospital” month.  I got there on a Sunday and got sick by Sunday night.  Thankfully, Delaney Ozmun was in Rochester with me, so she could get me everywhere I needed to go.  We enjoyed the truly arctic weather together and shared a lovely view from my “in isolation” hospital room for a few days.

And so on and so forth until now.  I am feeling better.  Almost done with antibiotics for whatever landed in March.

The end result of the sick stuff is that Dr. Zand decided that I needed to be off all immunosuppressants.  They aren’t really helping the kidney disease, and they are really making me susceptible to any old germ lurking out there and waiting to land on someone.  I’m down to 5 mg of steroids a day, soon to be to 2.5 mg, then soon to be (gasp) without kidney medication.  For the first time since 2006.  Wowser.

The next road should begin in June, with eculuzimab infusions.  This is a new treatment that Dr. Zand has recommended.  And wouldn’t you know that eculuzimab is currently one of the most expensive drugs in the United States?  We are dealing now with the insurer, who says that eculuzimab has not been demonstrated to be medically necessary for my disease.  What a surprise.  Dr. Zand goes to battle with them on Tuesday in a peer-to-peer review.  She’s tough.  I’d bet on her any day.  But fingers and toes are crossed for good luck!  More to come . . .

Delaney Ozmun met me in Rochester on November 8 for my physical exam and full lab work-up on November 9, to be followed by the last daratumumab infusion #16, on November 12.  What a treat to have Delaney with me in really chilly Minnesota–snow and the whole works!  As it happens, I apparently do not own a “true” winter coat.  Kendall realized this while helping me pack for the trip, and she kindly supplemented my luggage with a warm knit hat, ski gloves, sock liners, and other cold weather gear that she had collected when she lived in Chicago.

Delaney and I met with Dr. Zand on November 9 to review my lab tests.  Dr. Zand works with Dr. Fervenza, and she is a co-investigator on the daratumumab study.  She’s been out for three months with her new baby, and her first week back to work coincided with my appointment.  Which was lucky, since Dr. Fervenza was called away to a family emergency in Brazil.

I wish I had good news to report.  Unfortunately, none of my lab numbers have gone the right direction.  Proteinuria, which is the chief measure of this disease at least so far as I know, has never been quite so high.  Other bad stuff–blood pressure issues and edema–all indicate progression of dense deposit disease.

We were not expecting this information, so Dr. Zand did some additional testing, and Delaney and I stayed in Rochester until November 13 to meet with her again.  And I actually completed daratumumab infusion #16 on November 12.  Unfortunately, the additional testing was still the pits, so the daratumumab trial gets a failing grade.  We are all still processing the information–the short version is that the daratumumab did not work and did not target what is actually causing this disease.  It targeted the MGUS, but for me, at least, the MGUS is not what causes my disease.  Sigh.  Also, the lab numbers show that things are moving in the wrong direction, so it is time for a new treatment route.  Thus, the end of the daratumumab road.  I  had an IV steriod infusion on Tuesday at Mayo while sitting in a heated massage chair, and it was quite the set-up!

This is not all bad news.  My kidneys continue to function, for which I am more grateful than I can possibly say.  I am back on immuno-suppressant therapy, plus a hefty dose of steroids (this is a new addition).  So if I am jumpy, anxious, and unpleasant, I now have the steroid excuse, and I will ask you to forgive me up front for bad behavior.  I will probably also become a germophobe and carry Clorox wipes everywhere I go.  I’ll keep you posted if anything interesting happens along the way.  Thanks for the love you keep sending my way–and I’m sending it back in spades.

Did the fly-in, fly-out trick over the weekend–flew into Minneapolis on Sunday, out of Minneapolis on Monday, right after #15.  We’ve decided that it is unwise to try to fly in and out of Rochester in the winter, so Minneapolis (+ 1 hour, 15 minute drive to Rochester) gets our vote.  Speaking of votes, I hope anyone who reads this has already voted!  If not, get thee to early voting!

I was delighted to have Chelsea as my nurse for #15.  She was my first nurse and is a hoot!  We compared notes on our favorite Netflix shows for binge-watching.  She was horrified to “nick” one of my veins on the first try–she’s very proud of her reputation as one of the best stickers in the research unit.  So she switched arms, and the second time around was a breeze.  I think veins get tired of being poked and decide not to cooperate over time.  She’s on the schedule for my next (AND LAST) infusion, and she’s going to see if she can have me assigned to her, which would be an appropriate end to this tale.

I’ll be in Rochester starting November 8 for a boring several days of testing and doctor visits and such.  My great hope is that Dr. Fervenza will tell me on November 9 that the daratumumab is making a difference.  Delaney plans to meet me there for a fun time, and we will try to hit our favorite Rochester restaurants.  I have recently eaten at only the Courtyard restaurant or at the Canadian Honkers restaurant.  My devotion to the Honkers is unceasing.  But their vegetable soup is therapeutic.  I am convinced.  And all is still well.

Canadian Honkers veggie soup (and yes, that is a grilled tuna melt right next to it!)

It’s officially cold in Rochester now.  I flew in on Sunday, October 14, to a snowstorm that lasted the rest of the day.  Snowstorms in Rochester make dining decisions very easy–eat at the hotel!  Monday was infusion #14, which was not at all exciting.  To liven things up, I decided to post a photo of daratumumab.  The medication is sensitive to light, so you get this nice brown bag over your medication.  It’s kind of a trick–you don’t really know when the infusion will end, because you can’t see into the brown bag, and the pharmacy routinely overfills it.  So it’s a guessing game, and some of the nurses are really quite competitive with me as to who will correctly guess the time of the ending of the infusion.

See?  Daratumumab.  Not too pretty, kind of blah, matches the wallpaper.

I thought about a selfie in the hospital bed.  I actually took one just to see how bad it would be, and it was beyond ugly.  So no selfies in the bed.  All is still well–it’s just cold now for the visits.  AND . . . there are only two infusions left!

As promised, some photos of the September trip to Roquebrune-Cap-Martin and St-Paul-de-Vence.  Various views from a window and of a window:

Beautiful Daughter

Not a Cat Nap

Not your usual toy boat

Beautiful Mother

Some outdoor shots from the garden that Princess Grace loved:

Some family viewing:

In Dolceaqua (okay, this is technically in Italy):

Apparently Monet frequently painted this bridge.  I have no idea who is walking across it.

Many, many, many steps in Dolceaqua:

This is a mean trick–a fountain that purports to spit out red wine.  Except that there’s a sign that says it’s not drinkable.  Where is the fun in that???

St-Paul-de-Vence:

St-Paul-de-Vence, a beautiful walled city

And enjoying a few of the luxuries to be found there–lunching at the famous La Colombe d’Or (thank you, Michael, our most gracious host) and viewing St-Paul-de-Vence courtesy of Le Mas de Pierre, our lovely hotel.

Okay, so there.  Proof that the trip happened, that it was gorgeous, and that it was a welcome respite during the Infusion Days.  All’s still well.

So France was lovely–Roquebrune-Cap-Martin and St.-Paul-de-Vence are now beautiful memories, and I have failed once again to blog about these far away places and their strange-sounding names.  Perhaps later, when I am remembering them through photos.

The “Even Dozen” infusion #12 on September 12 was essentially non-eventful, with the notable exception of travel problems and a Bactrim allergy.  On the allergy front, I’d been taking one Bactrim a day since the beginning of the daratumumab infusions with the goal of Not Getting Sick while my immune system is down.  Unfortunately, my body decided that Bactrim was an enemy and let me know by a pretty significant rash (hives?) on my arms, hands, chest, back–all areas touched by the sun despite copious use of sunscreen.  This was not a good look for me.  So on the Even Dozen day, Dr. Fervenza decided to take me off Bactrim while he did some serious thinking on alternatives.

As for the travel, we hurried away from the hotel in time for our 3:45 flight, only to watch the arrival of far too many seriously dark clouds over the Rochester airport.  Then rain.  Our flight was initially delayed because, per the Delta gate agent, flights were suspended from landing in Minneapolis, our only hop skip and jump of a stop.  The flight was delayed and delayed still further, but so was the flight from Minneapolis to Austin.  So we had hope.

We watched another plane leave the airport successfully and were greatly cheered.  We actually boarded the plane and were still cheered, since the Delta gate agent told us it was really only about a 17-minute flight to Minneapolis.  Then the pilot welcomed us on board and told us that the plane had accidentally been over-fueled by something like 14,000 gallons and that we would have to fly around for one hour and forty minutes until we could land.  Hard to get any more hosed than that one.

After one thrilling night at the Country Inn close to the Minneapolis airport–physically attached to a TGI Friday’s, which does not make a bad margarita–we made it back to Austin.  And thus ended the Even Dozen Infusion.

I tell you this story because here I am after infusion #13, sitting in the Courtyard by Marriott lobby, waiting on my taxi to the airport, and watching the rain.  Really?!  Again?!  And this time it is 43 degrees, rather than the previous 85 degrees we experienced during the Even Dozen.

Perhaps there is some basis for triskaidekaphobia, a morbid and somewhat irrational fear of the number 13.  All I can say at this point is that I would almost never select a plane seat in Row #13.  I would not pick #13 as my lucky number.  And I would not be surprised if Infusion #13 results in yet another travel delay.

The infusion itself went like clockwork.  However, Dr. Fervenza has decided to start me on another antibiotic, which is given in liquid form and which the study coordinator describes as “distasteful.”  I’m thinking that may be an understatement.  I shall ask the pharmacist whether it can be followed with a tequila chaser or something like that.

My new nurse Patty is 62 years old, and her husband was on the floor below the Clinical Research floor, where he is battling cancer and other things due to Agent Orange (this all from Nurse Patty).  Patty can put an IV in your arm so quickly and painlessly that you forget that there were ever any other bad IV experiences.  She is one of seven girls in her family, and she has five other brothers.  She is the only one of the seven sisters to have curly hair.  All of Patty’s sisters went into the medical field in some form or another.  Her sister Judy just got back from Uganda, where she delivered 400 pairs of expandable shoes (what a great idea!) to 400 orphans.  Patty thought about becoming a nun for a long time–lucky for my veins that she decided to go the nursing route.  When she retires (which she thinks will be sooner rather than later because of the Epic system now used by Mayo), she may make a trip to Uganda with Judy.  But she worries that she would bring 400 orphans home with her.   If I had a glass of wine right now, I’d certainly toast Patty.

I fear that this blog post will bore you beyond words.  With that in mind, my next entry will include a few photos from the beautiful trip to France–perhaps they will inspire me to blog more about beautiful travel than medical travel.  All is still well.

Short update:  August 17 was infusion #10, three days after Day 77 of the daratumumab trial (the Go/No Go day), and four days after David and I arrived in Rochester.  Although my response has not yet been dramatic, nephrologist Dr. Fervenza said “go.”  So we did.  Another quick infusion and back home on August 18.  The saddest news:  it’s the end of the season for the Honkers, so no game to see.  Boo.

August 30 was infusion #11–not the usual two-week delay, but I’ll explain in a minute.  I flew in on August 29 and out on August 30 for this one.  I did not want to mess up the protocol, and I had been cleared this one time . . . because Mom, Kendall, and I left on September 1 for France.  You bet.  We aren’t talking Rochester this time.

This journey was a planes, trains, and automobile trip from Austin to Philadelphia to the Gare de Lyon in Paris to the fast (?) train to Nice to the van in the rain to the town of Roquebrune-Cap-Martin, with an incredible view of the Mediterranean Sea.

Kendall very comfy on the plane

Mom, Kendall, and I have joined Michael and Ryan at L’Aumonerie (“The Alms House”), a beautiful old former monastery with an incredible view of Monte Carlo and a stunning garden.  From Princess Grace’s My Book of Flowers:  “… a garden, small but precious, that I like to visit on a scorching summer day is the translucent green one called L’Aumonerie.”  More to come on this slice of heaven.

Room with a view–of Monte Carlo!!

So Infusion #8 happened as scheduled on July 23–in at 6, out at 11, then David and Olive met me at the hospital–fully packed and ready for the drive home.  After spending one night at the Holiday Inn Express in Emporia, Kansas, we made it home on July 24.  Great to be home!  Huge thanks to Lynn and Elaine Herron for their careful tending of Indy and the house while we were gone for so many weeks.

Infusion #9 was my first in/out to Rochester.  I flew in on Sunday, August 5, and stayed right across the street from the Mayo St. Mary’s campus.  Another 6 AM arrival, done by 11, then off to the airport for my flight home.  It went perfectly, which will no doubt never repeat itself!

I headed to Portland, OR, with my friend Lisa for a long-scheduled celebration of her birthday and a visit to sweet Delaney on August 9, home on August 14.  We had a wonderful time–visited a few wineries in the Willamette Valley, spa day with Lisa and Delaney, Portland tour with Delaney, dinner with Delaney and Noah.  Lucky us!!  Portland is beautiful, and I can now understand more fully how much Delaney and Noah love living there.

Pretty Portland, Pretty Delaney

Just because it was there . . .

Lucky #7!

Had Lucky Infusion #7 on Monday–and today is Thursday.  In at 6 AM, out by 11:30 AM.  We hope that this cadence holds, since I will soon be flying in and out of Rochester for the infusions.

I should have known that #7 would be a lucky infusion, based on some Sunday pre-infusion events.  David won yet another prize at the Honkers’ ball game–a cow bell inscribed with a perfect message for him (milk is his beverage of choice):

Cow Bell Victory

The lucky program page

Speaking of luck, I am also happy to report (finally) that the Honkers actually won the game on Sunday!  I know–what a shock.  Score: 13–3.  I’m not sure we will make another game before we head for home–it will be good to go out with a (brief) memory of a win for the poor Honkers.

I am feeling good–I managed a short business trip to Seattle on Tuesday and Wednesday, and sweet daughter Delaney drove from Portland to meet me.  Long drive for her–very nice treat for me!

Delaney and a friend at the hotel

Infusion #8 is on Monday, July 23, and we will start the drive home that very day!  After #8, I will have one infusion every two weeks for the next four months, so there will be plenty of trips to and from Rochester in the future.  All’s well.