Well, this really does change everything.

Before leaving the apartment for breakfast and then returning for our ride to Lake Oswego, Rachael and I were sitting next to each other on the couch, waiting for the 8AM phone call we've been anticipating ever since returning to Portland.  It's Doctor Greenberg, my rheumatologist, calling to discuss my situation and medication plan.  As soon as we returned home and I had to increase my prednisone dosage back to 40 mg/day because the disease reactivated, he instructed me to remain on that level until this phone call.

The call begins with a quick interrogation and review of how we got here.  What dosage am I currently on, how long has it been since I started taking steroids, what symptoms am I experiencing, how is my vision, and so on.  And of course I'm really doing quite well (crappy knees notwithstanding), as long as I remain at this high steroid dosage.

From there he moves on to tell me that this experience shows that I'm one of those patients who will never be able to taper off the drugs completely without the disease returning and risking ruining the sight in my good eye.  So, drugged for the duration it looks like.

• And that's a problem, because the side effects of long term cortisone usage are very significant, even dire.  It's worth risking that of course if the alternative is total or near total blindness, but there is an alternative: a different drug that is used to treat GCA (and also rheumatoid arthritis): tocilizumab, marketed as Tyenne.  It works through an entirely different mechanism than prednisone, which suppresses your auto immune system so it won't attack the infection and trigger swelling of the temporal arteries that puts pressure on and eventually permanently damages the optic nerves.  Instead, it acts against the specific infection itself. 
  
  The good news here is that replacing prednisone with this alternative will put my autoimmune system back in business to protect me from other scourges such as Kaposi sarcoma, the cancer that torments and kills AIDS patients.  Part of the bad news though is that Tyenne has its own unattractive side effects: notably the risk of serious infections, including TB.  The medical consensus though is that the side effects of Tyenne are much less problematic than those of corticosteroids.  In his opinion, there's no question about it - I need to change drugs, and the sooner the better.  He says that after a half year I'm getting to the point that further steroid usage could be a real threat to my health.

So I'm changing medications, probably right after I've gotten a TB test to make sure I don't have latent tuberculosis already.  Transitioning drugs is a process that will take about two months, with me gradually tapering off prednisone while blood tests confirm that the new drug has successfully taken control of the problem.  It looks like I should be all but off prednisone just about the time we leave for London.

So what else is there to say about Tyenne beyond its side effects, and that I'll be on it for years if not for life?  Well, there's a lot to say; and added up, all of the other considerations mean that the impact is life-changing for me and the team.

For one, there's the cost.  Tyenne is a tier 4 specialty drug (if you're familiar with formularies), meaning it's a controlled drug that's only available to patients with specific conditions - including precisely mine: it's been prescribed by a rheumatologist because I have GCA and can't successfully taper off of corticosteroids without it's symptoms returning.

And, it's a relatively new drug, one that I think has only been prescribed for this use within about the last six years.  So unsurprisingly, it's expensive - very expensive.  Each dose is roughly $430 on the open market, and if I need one every week or two we're looking at a prohibitively expensive cost if I don't get it under my drug plan - roughly $70/day maybe?  At that cost, we're strapped to Kaiser as our source, and to its prescription rules.  I don't know yet what the drug will cost when prescribed by them, or more importantly how much you can purchase at one time - but  most other drugs are limited to 90 days.  One way or another though, this means our future travels will likely be time boxed by the need to periodically return to Oregon to resupply.  At the cost of the drug, it would make sense to fly home for a refill and then return to Europe (for example) if we were trying to stitch together a longer tour.

There's more.  The drug is self-administered as a subcutaneous injection, meaning I'll be shooting myself in the thigh or wherever once every week or so, like folks with Type A diabetes do.  Which sounds unpleasant but something I can get used to of course.  The bigger problem is that the medication needs to remain refrigerated until used, which is a significant constraint on travel.  The model we've used so often of point A to point B tours where we moved to a new town and accommodation every day or few doesn't really work any more, because we don't want to be biking around with a small refrigerator carrying the drug our lives depend on now.

So we're still processing this.  We've got some thoughts about what it means - a much more base-centered approach to travel, where we move from one hub to the next by train or plane or boat and then get a place with a refrigerator that we use as a base until we're ready to move on. Which, when we think about it, sounds very appealing - it's where we've known we were heading anyway, but we're being nudged (well, drop-kicked) there a little more abruptly than we'd expected.

The bottom line though is that we don't know much at this point - about the constraints, or about what will actually work well for us.  In the meantime, we're looking at a lot of white space with only one commitment - our flight to London on August 3rd.  Beyond that we'll learn by doing, and take you along for the ride when we know where we're going next.

The yin and yang of it

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Yes, I know that yin and yang isn't quite the right concept - the last half year has hardly been a harmonious, life-in-balance time of our lives.  It's got that nice black and white, world is in a spin imagery that does fit though.  This feels like a good spot to recap the high and low points that brought us here:

• Nov 26: Shortly after returning from Spain I'm attacked by a shattering headache, by far the worst of my life.  I almost never get even mild headaches, and I can hardly cope with it.  I survive by living from one 400 mg dose of ibuprofen to the next, taking them every four hours round the clock.  I blame it on the recently reelected DJT, but I suspect it's a sinus headache.

• Nov 27: I buy the new Canon and enjoy a couple of days walking through Oaks Bottom and along the Columbia Slough testing it out,  note that I was still reasonably comfortable walking a few miles not that long ago.

• Nov 30: after becoming more or less nonfunctional because of the nonstop headache, I go to the nearby Urgent Care Clinic where I'm prescribed an antibiotic in the hopes it's a sinus infection.

• Dec 1: my birthday passes without celebration, because there's nothing to celebrate at the moment.
• Dec 2: a week on antibiotics changed nothing, so I discuss the headache situation with my PCP during my previously scheduled annual checkup.  He prescribes a longer term antibiotic for me to take with us when we head south and schedules me for an MRI scan of my sinuses, which I return for the next.
• Dec 4: we leave Portland and begin the long drive to Tucson.  Somewhere around Eugene we get a call from my PHP to let me know that the scan was negative, so not the apparent cause.  In a major failing, there is no follow-up to seek out the real cause - he doesn't suggest it, maybe because we're leaving town - but as I'm writing this I realize it was a failure on my part too, because it didn't occur to me to mention that my symptoms were changing - to my relief the headaches have been getting better in the last few days, but for the first time my eyes are bothering me and my vision is a little blurry.  I think it's because of the stress and strain of driving, but if I'd mentioned it he might have told me to turn around and drive home for further tests.  Had that happened, I'd in all likelihood have been completely cured within a few days - someone would have recognized the symptom pattern and put me on intravenous steroids for three days assuming it was GCA.  Darn.

• As usual, we aren't driving directly to Tucson.  We day hop down the coast with stops in Crescent City, and Willets before stopping off for a midday visit our friend Lynn, now living in her delightful new home in Palo Alto.  Afterwards we drive the rest of the way to San Luis Obispo and check ourselves in to an AirBnB we've booked for the next six nights.  Between the headache, the generally foul weather and the developing vision issue it's a difficult, not really enjoyable ride.

• December 7-8: I enjoy as best as I'm able a few days of experimenting with the new camera, but between the vision and headaches I'm not really stable on the bike and quit riding after the short trip to Laguna Lake and back.  And go together to the Urgent Care Clinic - she's there for an ear checkup and walks out with a prescription for flonase.  I'm there for the eyes, and come out with eyedrops.  Very much not to the point, as we'll soon realize, but it's not surprising since she's a generalist.  Still, it seems like an urgent care visit should refer you elsewhere if they can't treat or explain your symptoms.

• Dec 12: I drive to Morro Bay for some bird watching with the new camera.  It's a very good day all around - I see a raft of birds, the headache hardly bothers me at all and my eyes aren't bad either, making this the best day since we returned from Spain.   And in what counts as a small miracle, I reach my goal and see my 300th bird of the year - a Caspian tern - just before it's lights out. 
• Dec 13: (italics are extracts from the journal) I wake up in the middle of the night and realize almost immediately that something is wrong.  Both of my eyes have had some blurriness since the onset of my eye infection two weeks ago.  This is different.  It’s like a ragged grey curtain has been drawn across perhaps the lower third of the field of vision in my right eye.  My first thought is that this might be an adverse reaction to the teardrops I was prescribed when we went to an urgent care clinic two days ago.  I make an appointment for an optometrist, the only eye specialist in town I can find with an appointment available today.  He gives me a standard eye exam, sees nothing wrong, and also sends me away with eyedrops and the instructions to return Monday if I'm not improved.   for me this is the first clear error by a medical professional.  He's an eye guy, he knows he can't explain my symptoms, and there's an ophthalmologist right across the hall he could have had a quick consultation with.  And based on what's happened since then I think there was probably still time to save at least partial vision in the right eye if not restore it completely if they'd sent me back to the emergency room with a diagnosis of possible GCA.  Bu that didn't happen.  Coincidentally, it's Friday the 13th.

• Dec 14: My day begins early and very badly, not long past midnight.  I wake up to go to the bathroom and instantly realize my bad right eye has gotten much worse in the last few hours so that perhaps two thirds of my field of vision has now clouded over with a darker, less penetrable shade of grey.  I can still see a clear, raggedly edged narrow window in the center through the glass, but darkly.  And then I realize that my ‘good’ eye has gotten blurrier too. My overall vision using both eyes is misleading, being better than either eye alone because it’s learning to optimize from the best information available.  I go back to back to bed but when I wake up four hours later the right eye is out completely.  In a late-coming flash of insight I connect all the recent dots and wonder if I've had a stroke.  I reach for the IPad, do some research and discover that eye stroke is a rare but real condition, and it's a medical emergency.  We treat it as such.  I wake up Rachael even though it's 4 AM and she drives me to French Hospital, which is fortunately only a half mile away.  They immediately take me in, examine me, and it's not long before I'm being wheeled down to the MRI room for an eye scan to test for evidence of a brain stroke.

• The eye scan turns up negative.  Afterwards I'm interviewed by a young Asian doctor, a woman who impresses us both - a female Doctor House, we both come away thinking.  She asks all the right questions, including whether I have jaw pain, a key symptom of GCA - and I do - it starts when I'm chewing and gets progressively worse quickly, to the point that I can never finish more than a half bowl of cereal and have to quit because of the pain.  So she knows, and it's not until I'm writing this that I realize this was another missed opportunity, a spot when it might have still been possible to retain some vision in the bad eye.  She knows the disease but she doesn't know the right response, the one that's been the default for at least fifty years - if you suspect GCA, don't wait for a biopsy to confirm it because time is so critical.  Start the intravenous steroids immediately, and verify later.
• But she didn't do that, because although she's definitely a cut above but not the valedictorian in her class perhaps.  Instead she says it's urgent to see an ophthalmologist, and I'm in luck - there is exactly one working in the region on this Saturday, and he has a single vacancy at noon., less than two hours from now down in Santa Maria.  It's 30 miles to the south straight down the freeway, and I drive - half blind, half blinded by the glare of the sun, with Rachael navigating and watching lanes and accesses and entrances from the shotgun seat.  it's by far the most harrowing drive of my life, one I'd never repeat.  We're lucky we didn't kill ourselves or someone else either.
• This ophthalmologist is no Doctor House either, far from it.  He's a former military doctor, just going through the motions while he's waiting for his golf game I imagine.  He examines me, sends me off with a blister pack of 5mg prednisone pills, and tells me to check back early next week.  Nice job, guy.  If it would change anything and we needed the money, I'm sure there's a case for a lawsuit here.  And I'm sure the company knows, because they never sent a bill for the service.
• Dec 16: It's Sunday, and we're just marking time.  Rachael takes a walk and I slow-walk a few errands downtown.  They're a mixed bag: I pick up some new CDs that we anticipate listening to on the drive back to Portland (plans for Tucson were already scrapped by this point), but I lose my driver's license and the credit card I used on my shopping spree.  Wondering whether any birding at all will be in my future, I optimistically begin the 2025 bird count early when I'm unexpectedly able to see a large bird atop a snag above the creek - a red-shouldered hawk.
• Dec 16: the eye clinic calls and says to drive down for a follow up ophthalmology appointment, which we do.  We're less rushed and Rachael's had a bit more practice by now and drives this time, which is stressful too but much safer.  This time we get the real female Doctor House - another young Asian woman, but yet another cut above.   I've since read her profile on the website and she's a recognized expert in the field - makes presentations at international conferences, that sort of person.  She asks all the same symptom questions, but starts with collecting a complete event chronology all the way back to when we left Spain.  We see her flinch and cross her eyes before quickly composing herself when she hears that an optometrist sent me home with eyedrops, and asks the name of the clinic.  And then it happens again, when she hears the that just two days ago her colleague across the hall sent me home with a blister pack and the instructions to check back in a couple of days.
• And then she tells us to drive back north to the emergency room as fast as we safely can, and they'll be waiting for me.  When we arrive I'm ushered nearly immediately into a room where I'm put on an intravenous steroid drip.   I'll be eternally grateful to that woman, who saved our lives. 
• Dec 16-17: a lot happens during my two night stay in the hospital.  I'm administered steroids for three straight days, and there is at least some improvement - I'm thrilled when I realize the orange streak on the wall ten feet in front of me is a row of faces - the pain scale - though all I can distinguish is that they're a row of separated circles on the yellow/red spectrum.  I can see Rachael's face, but barely - shes blurry, seen through a ragged oval that covers only part of her face.  It's a painful time with us talking about how we'll function if I go totally blind - I'll be the stoker on a tandem, she'll read out loud from our old journals and describe photographs I can remember but no longer see.  I have a whole brain MRI - an unnerving, shattering experience that lasts an unbearable half hour.  I experience the most intense, bizarre hallucinations I've ever experienced that accompany me for days, hallucinations I could never have imagined and wouldn't believe now if I hadn't described them in such great detail at the time.
• Rachael takes the sad, lonely walk back to the apartment in the  evening where she's comforted by our host Alicia  - she's left flowers, a card and a gift basket in the apartment, and they share a weepy conversation about the turn our world has taken.  

• Dec 18: earlier than expected, the anesthesiologist comes in, describes what's about to happen, and I'm wheeled down to to surgery for the biopsy that will confirm the condition.  Rachael's there to meet me when I wake up, and we spend the morning planning for our escape.  We're not driving home, because it's too slow - they want me to see my ophthalmologist tomorrow, so we're flying.  We call in the friends to bail us out - Liz and George will store our car until Bruce and Andrea fly down afte the first of the year to fly it home - and George will find for Roddy, the bike I love but expect never to ride again.  And then we're discharged mid-afternoon, I'm sent off with 60 mg prednisone doses, and head back to the apartment to start packing.
• Dec 19: We load the car and drive over to Liz and George's to drop it off in their garage and share a lunch before they drive us to the airport.  After I unload the Rodriguez I realize I need a last photo for the blog so I go into the house to get the camera.  On the way back I start crying - really, for the first time in this entire month-long nightmare.  A real, cathartic cry, sobbing loudly the whole way back - and I realize just in time that there's no need to make a decision about the bike now.  He goes back in the Raven to be returned to Salem, and we drive off.  Along the way I'm pleased to be ble to just barely make out the unique blue of a scrub-jay flying across the road, probably the smallest bird figure I can make out.
• When we reach the airport we discover we're missing something - my rucksack, left back in the kitchen from lunch.  That would be the rucksack with the new camera in it, and both iPads, and my passport.  It's a good thing we arrived with an abundance of time to spare while our friends make another round trip home to bail us out.  In the meantime Rachael gets herself checked in, and after they return I stand in the front of the line waiting for an agent to free up.  I have company though - real passengers waiting behind , but also a queue of imaginary people lined up just behind my right, blind-side shoulder.  Imaginary passengers who are real enough to me that I keep double-checking because I'm concerned I might be farting right into someone's face.
• We're met at the airport by Bruce and Andrea who drop us off at our new, tiny apartment we'll be staying in for the next five weeks.  And then we walk the three blocks over to Safeway, where I'm refused the purchase of a six pack of NA beer because I don't have any ID.  I didn't think to bring my passport with me and my drivers license is lost somewhere in California.  A pretty irksome experience if you're half blind and pushing 80 to be told you can't buy a nonalcoholic beer without ID.  Fortunately Rachael's got hers handy and saves the day.
• Dec 19: Jason the surgeon calls, confirming a positive diagnosis for GCA.  I thank him and then go for a short walk along the river, gratified that I'm starting to see a few somewhat smaller birds - a mallard, a rock pigeon, even a house sparrow if it's right in front of my face.
• In the afternoon Bruce drives us south to my ophthalmology appointment with Doctor Torres.  Before meeting him I'm given a field of vision test and the standard Snelling eye chart test - and I can barley make out a blurry large E on the first tier of the pyramid, but below that everything is an unreadable blur.  We walk away with the appointment with an even stronger prescription: 80 mg/day now - and the dreadful news that by the time patients enter treatment at this stage there's seldom improvement and the goal is to preserve whatever functionality remains.  As bleak as the entire month has been, this is the darkest hour.
• Dec 20-29: in what counts in our book as a real Christmas miracle and for me as close as I'll ever come to a religious experience, my visions starts improving noticeably every day over the course of the next week.  One day I can make out the faces of the servers at the coffee shop; the next I can make out the shapes of cormorants a few blocks away on the river walk; and when Elizabeth and I go to a piano recital toward the end of this period I can clearly see the pianist's hands reflected off the cover of the grand piano.  By the end I'm still sightless on the right side of course, but it feels like vision in my left eye has been completely restored.  Faces, music, birding have all been restored.  It feels like I've been handed my life back.  There have been some momentous times in my life, but this is probably the best single week I've ever experienced.
• Dec 30: My Xmas miracle nearly turns back to a nightmare again when I go to the optometrist for an eye exam.  I'm called in by the technician, who asks how I'm getting home afterwards and if we're doing one eye or two today - and then prepares to inject something into the back of my good eye.  For an eye exam!  Fortunately I squawk in time that something looks very wrong with this picture, so he asks who I am.  In a major protocol failure, he never asked my name or date of birth (a fact that I later reported to my ophthalmologist so he could follow up).  The sap doesn't even apologize as he walks me down the hall to another office where someone's been calling my name for awhile thinking I'm a no show. On the way out of the lobby we pass an even sadder looking old guy in a wheelchair, the other Scott waiting for his name to come up.
• Jan 6: the Raven Returns!  It's wonderful to see the car and the Rodriguez (and our friends here and down south, who have our everlasting gratitude for helping us out).  The kicker though is that Andrea found first my drivers license and then my credit card, where it had fallen down into the slot beside the passenger's side of the car.

• Jan 7-12: Life continues to improve and normalize.  I get prescription glasses, I take my first bike ride, I take my first turn at the wheel of the Raven.  Best of all, at my final visit for now with the ophthalmologist he gives his blessing for us to fly to Tucson where I can continue learning my new life and get some exercise.
• Jan 13-Mar 2: Tucson happens.  Kelly meets us at the airport, we have lunch, and then she drops us off at the AirBnB that will be our home for the next six weeks  There are challenges, but once we've adapted reasonably well we experience a nearly normal winter escape: lots of CycleBlaze meetups, I bike my age in miles, I rent a car for a few weeks so we can get beyond what's within reach on a day ride, there are plenty of dinner and lunchtime meals at our favorite meals, I manage getting my blood drawn and faxed back to Portland so the rheumatologist can monitor my state, I add nearly 70 birds to the year's list, I bike nearly 1,000 miles.  The trip far exceeds hopes and expectations.
• Mar 3 - Apr 8: we fly back to Portland where our new friend Jonathan meets us at the airport and drops us off at our new AirBnB apartment across the street from our storage unit.  Life in Portland feels nearly normal.  I finally meet with the rheumatologist for the first time, and he agrees we can travel to Europe for 9 months as long as I follow the rules: regular blood draws, prednisone reduction on schedule, check myself into an emergency room if the original symptoms return.
• Mar 9 - Italy and France.  The planned tour proceeds more or less as planned, except it gets cut short not because of my eyes but because my knees have gotten so much worse.  We gradually shorten our plan: first we lop off Spain, then the second half of England, then all of England, then finally the tour of northern Italy.  Countless bookings get cancelled, tentative bookings in America get made as we try to game out when we might be in country getting knee replacement surgery.  Interesting logistics result when we have to get our suitcases shipped north from Bari so we can fly home from Lyon.
• June 18: the meeting with the ophthalmologist ushers in a new, different, ultimately prednisone-free life.  Come what may, it'll be great and worth living for.