I'm going to have surgery later this month to remove a small area of new tumor growth near the site of my original tumor. I'll report back when I'm through it. Meanwhile, here's more about the situation.
A little over sixteen years ago, a long bout of headaches prompted an MRI —just to be safe. By the time the MRI rolled around, though, the headaches had died down and my mind had moved on. Then — wham! — an earthquake of a result: a tumor in the center of my head.
Not that I had ever given it much thought, but I had always assumed "tumor in the head" meant brain tumor. Turns out, that's not the case. The one in my head was growing from within an obscure (and unneeded) bone called the clivus at the base of my skull. From there, it extended up against my pituitary gland, and bulged back into my brainstem and around key arteries that supply the brain. One neurosurgeon described the area it occupied as "high value real estate."
It still verges on unimaginable to me that a tumor in this location is treatable, let alone treatable in a way that would allow me to go on to live an essentially normal life for all these years since. Just a few decades earlier that would not have been the case. But when this took place medicine had advanced to the point that a team of surgeons at the University of Pittsburgh was able to completely resect the tumor through my nose, repair the hole it had poked in my dura (the membrane that holds your brain and spinal fluid), and leave me no worse for wear.
I am in awe of what they accomplished and have felt immensely grateful for it every day since.
But, while the surgery was the best I could have hoped for, the diagnosis of the tumor as a chordoma was less rosy. That's because chordomas, though typically slow growing, are relentless: even after successful treatment, they eventually tend to come back, often repeatedly, and each time they become more tricky to treat. And, once you've exhausted surgery and radiation, there are no great options to control the tumor.
Without rehashing the whole story here, facing this prospect is what lit a fire under me to attempt to solve the problem in whatever period of good health I have.
Though, fortunately for us, chordoma patients live far longer on average than those afflicted by most other cancers, with chordoma, you're never truly out of the woods: even after long periods without evidence of disease, you never graduate from regular scans, never are declared cured, never are free from the specter of possible recurrence.
So, I've returned ritually to the University of Pittsburgh for checkups. Thankfully, each visit brought a big sigh of relief and another window of time in which to get on with life. Pre-pandemic, our family trips to Pittsburgh even became something we looked forward to, and Rachel and I have many very fond memories from them (our visit with Sam to the Pittsburgh Zoo, for example).
On my most recent MRI, however, what appears to be a small, new tumor showed up.
Though I've always known it's not uncommon for chordomas to recur after ten or fifteen years, admittedly, as my initial diagnosis became more distant, I had let myself imagine that I might be among the lucky few who would skate by without a second brush with chordoma. So, even though I've long been prepared for this news, it's a bummer to be thrust back into patient mode.
Thankfully, I've been able to get invaluable guidance from several of the most experienced chordoma specialists whose perspectives I value greatly. In short, their recommendations all converged on surgery at some point in the not-too-distant future. Luckily, its location and size make achieving a complete resection probable at this point, and better to get it out sooner rather than later when it would be bigger and potentially more complicated to remove. Plus, it's not going to be any easier to undergo surgery when we have a newborn — we're expecting number two at the end of October, by the way :)
So I'm heading back to Pittsburgh later this month for surgery. As was the case last time around, the operation will be performed by a neurosurgeon/otolaryngology (ENT) duo. This time it'll consist of Dr. Paul Gardner, the neurosurgeon who has followed me for the past dozen years and who spotted the new tumor, and Dr. Carl Snyderman, who was one of the surgeons who masterfully performed my original surgery back in 2006. They are among the most experienced chordoma surgeons in the world, I have the utmost admiration for both of them, and I feel confident in their hands.
As with my first surgery, they will once again go through my nose to get the tumor — what's called an endoscopic endonasal approach. I'll most likely come out of surgery with a (temporary) balloon in my nose to keep spinal fluid in and a catheter in my spine to let any excess out in an attempt to give my dura a few days to seal up without too much pressure on it. If all goes well, I'll recover in the hospital for about five days and then will stick around Pittsburgh for another ten days or so for observation and post-op appointments.
Radiation is also typically part of the treatment plan for recurrent chordoma; however, based on the appearance and behavior of my tumor, all of the experts with whom I've consulted suggest holding off for now and keeping it in reserve in case of future recurrence (a great example of why there can be no one-size-fits-all approach to chordoma).
Getting through it
Taking this on is only possible with a lot of support. So I feel incredibly lucky to have a dream team in my corner. Crucially, Rachel will be with me in Pittsburgh the whole time, along with Sam. Additionally, our dear friends ("faux fam" to us), Heather and Steve, will come up from Chapel Hill to be by our side and part of my care team for the first couple weeks. Then, they'll pass the baton for the (hopefully) final week to my cousin Arie and her husband, Jimmy, who will come in from Los Angeles. It's such a relief to know that they'll be there, not only to help watch out for me, but to support Rachel and Sam as well.
Of course, schlepping to a different city for treatment isn't easy, even in normal times, not to mention during a pandemic. But we are getting prepared and things are falling into place: transportation, a place to stay, care for Sam, locals to lean on, etc.
Rachel and I feel well-supported and well-prepared to take this on as long as treatment and recovery go largely according to plan. Where we have some concern and may need some additional hands on deck is if there are complications that require a longer stay. We'll cross that bridge when we get there, and — we hope it won't be necessary — will put out a call for help, if needed.
Meanwhile, on a brighter note, we're getting movies and books queued up, scoping out appetizing take-out spots, and lining up fun things for Sam to do (recommendations on all of the above very much welcome!).
What all this means
There are a wide range of potential outcomes from here. Some are very unpleasant; some look much like how things are now. I'm not getting too attached to any of them at this point.
If all goes well, there's every reason to believe that a complete resection could keep me tumor free with great quality of life for quite a few more years, just as my first surgery did. That's clearly the hope.
In the near term, the biggest risk is developing a spinal fluid leak (and associated complications like meningitis). Last time around, I developed leaks twice after my initial surgery which required two subsequent surgeries to fix — and five weeks in the hospital. But the good thing is that it is almost always fixable. The more concerning complications would be vascular or neurologic injury, which are always a relatively small risk of surgery in this part of the body.
I'm hoping we will be able to obtain enough tissue for molecular analysis of the tumor, which could perhaps shed more light on my prognosis and/or clarify the benefit of possibly having radiation after surgery.
But, even absent any further information, the presence of this new tumor puts us on watch for more in the future. I hope that doesn't happen — and there's a chance it won't — but I have to seriously prepare for a future in which this is not in the rearview mirror. After the upcoming surgery, future surgeries would likely be considerably more difficult. Though, assuming I hold off on radiation now, I'll still have it to deploy in case of a future recurrence. And, on the bright side, the way my new tumor has taken its time to show up might indicate that it is intrinsically slow moving, even by chordoma's glacial standards. Of course, cancer evolves and the indolent nature of my tumor could change, but, for now, I'll take it.
Whatever the case may be, finding effective treatments beyond surgery and radiation is now of even greater personal importance.
How I'm doing
I had hoped not to be in this position. But I've have had a long time to get prepared for it. And I've been through something similar before. So it's neither quite the huge jolt nor the complete, scary unknown it was when I was first diagnosed.
I feel much better equipped this time around. I'm better informed. Surgical techniques have advanced. I'm surrounded by love and support and expertise beyond what I could possibly wish for. Most significantly, I have Rachel as my partner in taking this on and there are no words to describe the comfort and confidence that provides.
My main hopes at the moment are to get through surgery without any new deficits and for the time in Pittsburgh to be as positive as it can be for Rachel and Sam.
Looking beyond surgery, the situation does not feel dire. I am deeply encouraged by the research advances that have been made over the past decade and a half, and by the speed with which research can now move, particularly thanks to the recent launch of Chordoma Foundation Labs. With it, we now finally have the ability to make the pace of research match the sense of urgency we feel — urgency that is now only heightened for me.
There's no question that better treatments are coming and I believe more strongly than ever that they can come in time to help those of us dealing with the disease today. I still hope to never need the treatments the Chordoma Foundation and our collaborators are working to bring about, but it sure does feel better knowing how much progress is being made — and to have some agency in pushing it forward.
I feel deeply grateful to everyone who is making that progress possible: the Foundation's amazing team, our dedicated Board and volunteers and advisors, all of the researchers working to find better treatments, and the thousands of supporters across the world who propel our work forward. Nothing gives me more hope about the prospects of outrunning this disease than the people who have rallied behind this cause.
But, more than anything, I'm grateful for the past sixteen years — many more years of good health than I had imagined I would get when I was first diagnosed. Actually, so many that there are now more years I can remember post-chordoma than pre-chordoma. For a long time after being diagnosed, I thought if I could turn back the clock, somehow avoid getting chordoma, and get back to the way things were, I would. It feels strange to type, but I don't feel that way anymore. I treasure how things are now.
Inspired by Heather and Steve's son Justin, our mantra at the Chordoma Foundation is perseverance. In that spirit, I am going to continue fighting doggedly until the problem chordoma poses is solved. I am honored and grateful to be able to do that shoulder to shoulder with many who are in the same fight, and with the support and friendship and encouragement of many more. Thank you for being in this with me — it means a lot.
P.P.S. One of the bright spots from my time in Pittsburgh sixteen years ago was visits from friends and family. In ordinary times, I'd welcome them again; however, because of the ongoing pandemic, with good reason, the hospital has a very restrictive visitation policy, which makes that impossible while I'm in-patient. Plus, once discharged, we're going to be extra COVID-cautious while there to minimize the risk of any added complications with healing and Rachel's pregnancy, not to mention an extra layer of logistical complications. This is a bummer, but, instead, we'll lean on all the forms of digital connectivity that have grown normal in the last few years, and will have many in-person catch ups to look forward to afterwards.
P.P.P.S. If you're a chordoma patient or caregiver and have questions about the disease or treatment options please feel free to get in touch with the Chordoma Foundation's patient navigators at email@example.com.