More Good Bad Luck

These past few years, we have done a poor job in updating on Colin and his progress. Though I can’t completely escape guilt on this point, I am happy to report that the reason is that we have all been busy with normal life and that he has been improving steadily. We moved to Ithaca, the boys have been going to the same elementary school together, and we all enjoy living in a neighborhood that is full of kids and friends.

At 7-1/2, Colin is in second grade and doing great. He hates homework but loves his friends, and his favorite subject is science; he now wants to grow up and be a scientist so he can give people super powers, like flying. The year-plus of treatment alone gave Colin an experiential delay, not to mention the effects of chemo and radiation, but he’s made tremendous progress, learning how to read and do math.

Aidan, now in fifth grade, has found his groove with skiing and soccer. He hasn’t managed to combine the two, but the soccer hasn’t stopped despite the snow. This year, he’s gotten much more involved with racing and is on the cusp of the capstone race for the season.

Drum Roll Please

Amid all this progress at home, we have continued to visit St. Jude every six months for check-ups and all of the after-care that comes with the territory. Unfortunately, during our last trip at the end of January, we discovered that Colin has a new tumor in a new location. I didn’t want to start the update with this news because, while it is obviously a new milestone in his adventure, it shouldn’t overshadow the fact that he has been able to enjoy his childhood and embrace life.

The new tumor is a small spherical blob, about 4-5 mm in size, in the ventricle by the left frontal horn.  Colin isn’t symptomatic at all, and the location isn’t particularly difficult to access. Dr. Gajjar explained that our concern isn’t this particular lesion but what it tells us about the general state of Colin’s central nervous system (CNS).

To take a step back, the original tumor, which was located in the posterior fossa region, was a large, tentacled critter that insinuated itself among the cranial nerves coming out of the brain stem and wound its way up towards the pituitary gland and down through the foramen magnum, the hole at the base of the skull where the spine enters the head. We were able to remove it only through multiple surgeries and after high-dose chemotherapy, which successfully desvascularized the tumor. The pathology report from the last resection showed that the tissue removed was low grade without the markers of aggressive activity, so the chemo presumably did its job.

Though we needed to use chemo to try to loosen the tumor from the delicate areas where it had gotten stuck, the study that Colin was on, SJYC07, was investigating the potential role of chemotherapy in reducing relapse. Standard therapy includes complete removal of the tumor, followed by radiation focused on the original tumor bed. Focal radiation alone leaves the rest of the CNS untreated, so the hope was that chemo would sweep up any additional cells.

In theory, the results from the pathology report tell us that Colin’s tumor was successfully treated (at least for a time being) with the chemotherapy. The reason that chemo isn’t a standard tool used with ependymoma is because it hasn’t been effective in achieving complete control of disease; the chemo only works for so long, and then certain resistant cells rise up and swing the pendulum back in the other direction. The lower the disease load, the greater the chance that chemo will be effective, so one hopes that, on the microscopic level, the chemo is doing its job on things that are too small for us to see, and perhaps that did happen for Colin.

Focal radiation alone is effective about 70% of the time for ependymoma. Oncologists have struggled to improve the long-term survival rate beyond this statistic; thus, the investigations into the role of chemo. Perhaps we will find that it is useful for preventing relapses within the first year or two, but in this case, Colin is more than five years out from his original diagnosis. By some definitions of cancer treatment, Colin has been cured!

We know that there is some small percentage of brain tumor patients that relapse after five years, with a particular concentration 5.5 to 6.5 years out, based on what Dr. Gajjar has told us. I doubt that the cancer cells are aware of the significance of five years in reporting statistics on this disease, but it is a bitter irony that it is an important milestone that often comes with the label “cure” when that is clearly not the case. Knowing this, I requested that we continue six-monthly scans, even though the protocol actually had Colin switch to annual scans. Apparently, it’s common for parents to ask this, and we have had to be in Memphis that often anyway because he is getting growth hormone from the endocrine department.

At any rate, why these late relapses? Our best understanding is that some nascent cells, probably stem cells, are hiding out in the patient’s CNS ready for whatever random event causes them to proliferate and turn into a tumor. Once you have an actively growing tumor, that can itself slough off cells that further seed the CNS, but this is a different issue from the problem of these microscopic time bombs.

These nascent cells, while dormant, aren’t really doing anything, and while that is a good thing, it’s not helpful when you’re trying to eradicate them with chemo. Chemotherapies work on rapidly dividing cells that have certain vulnerabilities when they take up the medications. However, these sleepy little guys are typically unaffected by chemo, so they are able to slip by under the radar. The only hope, and this works in more than two-thirds of ependymoma cases, is that there just aren’t any nascent cells within the CNS.

The appearance of Colin’s new tumor tells us that there was definitely one cell that escaped the initial onslaught of treatment. The tumor is easy to remove and can come out endoscopically in a minimally invasive procedure where he’ll be up on his feet very quickly. The big question is the status of whatever invisible friends may be left behind. This is currently absolutely unknowable. There also may be other tumors proliferating right now that we just can’t see because they don’t show up on the MRI.

At this point, with metastatic disease (spread outside of the initial tumor area), we’re more concerned about what we can’t see than what we can. The problem is that the only effective treatment for ependymoma remains radiation, but this time, Colin would have to receive craniospinal irradiation (CSI) that would have much more severe effects on his cognition and stature.

If we were to pursue CSI for Colin, we would have a chance of never seeing another tumor again, but we would definitively and permanently change Colin himself. Especially in light of the uphill battle he’s gone through to get where he is today, we can’t do this to him, and a single, small tumor doesn’t force our hand in the matter.

This Isn’t our First Rodeo

In a sense, we’ve been through this process twice now. The second time around has been easier by a long shot. For one thing, we weren’t dropped in the middle of the ocean with no clue of how to survive in open water. This time, we know the drill: inflate the life raft, tally our resources, and figure out our best chance of making landfall.

From the get-go, the advice we have gotten has been specific not just to Colin’s case but to our entire family, and we have established relationships with the practitioners we’re dealing with. On top of that, we have a much better understanding of the factors that go into that advice, so we’re able to process and synthesize that into a decision on our own end.

That summary is a bit of hindsight, but I can go back to that moment when Dr. Gajjar called to say, “It’s not good news,” and my own visceral response. There have been two moments where doctors’ proclamations caused vertigo, the utter sense that my life had pivoted completely out of its axis. The first was the initial diagnosis in the emergency room of Danbury Hospital. The second was when Dr. Wisoff, the neurosurgeon who did the second surgery that removed the largest volume of tumor, called to let us know that the pre-op MRI of the spine revealed metastatic tumors (nope; just motion artifact), so he would be somewhat less aggressive in his approach (thank goodness).

Standing in the hallway next to the bathrooms of a noisy Memphis restaurant, those words, “It’s not good news,” did not send me reeling. I instantly knew what this meant (long-term survival went from 70% to 30%), but these are not the worst odds that Colin has faced. It was not the worst news we have ever heard, nor was it the worst day of my life – not by a long shot.

Out, Out, Damned Spot!

Here, I’ll distill several weeks of research, discussion, and consultation into a short summary of the treatment plan. Phase One is removal of the tumor, through endoscopy with Dr. Boop at LeBonheur in Memphis on March 6th. We’ll have to assess the spine and perform a lumbar puncture to verify that the disease isn’t on the spine, but since Colin doesn’t have any spine-related symptoms, we’re hopeful in this regard.

I have perhaps unrealistic expectations of how easy this is going to be and had for some reason thought, after Dr. Boop said he’d get Colin out of the hospital in a day, that we could have surgery on a Friday and go back to school on a Monday. Even I will admit that this was a bit optimistic, and probably the result of a long habit of me pushing things to the edge. It still feels a little soft to let him get out of school for a whole three extra days after brain surgery. Kids these days.

We do have a treatment plan for what follows, but I’m going to be vague about it because we’re still waiting for things to be finalized, and it requires various approvals. Suffice it to say that, at a minimum, we are hoping to delay a subsequent relapse as long as possible (on the order of years) so that CSI will not be as devastating.

In the meantime, Colin is happy and healthy. He knows about the cancer and his upcoming treatment in a way that is completely new to all of us. For the most part, he’s been focusing on the benefits of getting a port so he doesn’t have to have needle sticks and he’s looking forward to making new friends. We’ve had to quell his fears that this surgery will cause deficits like the first triad of procedures, which was easily done.

The single biggest driver in our decisions around Colin’s next steps is Colin himself. We see a curious, loving child who enjoys people and has a fascinating mind. His quality of life is very important to us, and we are more conscious than ever of the need to balance the pursuit of durable remission with his ability to enjoy the life he has today.

Good Bad Luck

Anybody who’s familiar with Colin’s story also knows his propensity for good bad luck, and we’re going to keep riding on that wave. Having a relapse was certainly bad luck, but the location and size of this lesion are very good luck. The word metastasis is enough to chill the blood, but in his case it suggests that his disease is more susceptible to the primary tool to deal with this disease, radiation.

Relying on Colin’s good fortunes is not a very sound strategy, but it is strangely reassuring. There are so many crazy twists and turns in his case that could have ended badly but didn’t. He managed to squeeze through the eye of the needle on the path for durable remission; it feels like we were so close, and maybe we still are. This disease doesn’t leave a lot of room for do-overs, but we’re still in the zone.

Needless to say, we will be providing more regular updates on Colin’s status and treatment. I will also backtrack a bit with some of the thoughts from this pre-relapse period. Despite our long silence, we have felt the support of the many people who have followed Colin’s story through the years. He is taking us on another adventure and we appreciate the company. Thanks for holding our hands as we enter a brave new world.