Never Give Up

When we finally arrived in Boston to start proton therapy for Colin, the branches were already bare in Ithaca, but many of the trees along the Charles were still holding their color. The ginkos are just now releasing a carpet of yellow fans on the sidewalk. The night before we left Ithaca, the sky was crystal clear and full of stars; celestial bodies struggle to emerge above the cityscape, though Venus is tenacious in shining through the urban smudge.

Our path to Mass General included a short jaunt for a planning CT and a consultation with our new team. For the first time, we met Dr. Yock, who proved herself to be incredibly intelligent, thoughtful, and compassionate, the trifecta of good doctoring. We left the meeting in a combination of relief and disbelief at how differently radiation oncology can be practiced by different individuals. Given the prior dose to the brain stem (59.4 Gy), she would spare that area, but also reduce the dosage to previously treated areas (quite a large volume in Colin’s case) from 36 Gy to 16 Gy, and she offered to reduce the dose inside the vertebrae to 16 Gy in order to spare the marrow and give us more options for chemotherapy down the road. She and her team understood our concerns about hearing and added their own about vision.

We returned to Boston a week later ready to hunker down for the six weeks of treatment. Our first appointment was an MRI, as Colin hadn’t had one since the pre-op scan in late August. We knew that this could reveal new tumors and, the week before, had expressed our preference to push ahead with treatment and surgically remove new lesions after radiation. The scan took forever, making us nervous, and the next day we got a call asking us to come in for a meeting.

In the world of oncology, one never comes in for a meeting to receive favorable news. On the phone, I didn’t bother asking what kind of meeting it would be but only inquired to see if child life would be available to stay with the boys while we received whatever degree of complicated information was peeking over the horizon. Yes, there would be somebody in clinic. In my mind, the news could range between an adjustment to the radiation plan to “we can’t help Colin, so you need to go home.”

Without mincing words, it was the latter. The disease has exploded within Colin’s ventricles both in number and, relative to what we have seen since January, speed of growth. The largest lesion, which was not at all visible in late August, is 1.1 cm long. Many of the lesions, including fairly bulky ones, are in the area of the original tumor bed.

Arguably Too Numerous to Count

The radiologist characterized the lesions in the fourth ventricle as “arguably to [sic] numerous to count.” I’m guessing that spelling is not a key focus for radiologists, so I will forgive him the grammatical error. Despite this, he did catalogue a list of more than 20 lesions, most between 1-3 mm, that ends up looking like an exam for students challenging them to come up with different ways to describe tumor locations in the brain. Somehow, it wouldn’t surprise me if Colin’s MRI became part of radiology pedagogy, and there’s a part of me that hopes that it will, if only so this otherwise horrifying set of images can become useful to the next generation of medical professionals.

The good news is twofold: Colin is completely asymptomatic; and there are currently no spinal tumors. This last bit is not just a matter of academic importance or a desperate grab at hopefulness but it is about quality of life. It takes a much smaller spinal tumor to cause problems, and the problems can be quite debilitating.

Radiation is off the table because there is no way to give Colin a useful dose (ependymoma is a tough bugger and demands high dosages for any appreciable effect) and the areas of active disease are now tremendous. Even if we had started earlier, it’s unlikely it would have done much good because of the sheer volume of microscopic disease. We knew it was there and suspected it was broadly disseminated but didn’t anticipate the rapidity of growth, though I can’t say that we didn’t know it was a possibility. It was the thing that made us grip our seats when waiting for news from Dr. Boop during the August resection. At the time, he didn’t see massive spread, but we knew we were standing on the precipice – it just wasn’t clear how close.

Our initial reluctance to move ahead with the CSI was that it would not buy Colin enough time to be worth the side effects; it is not a benign treatment and comes with short- and medium-term side effects that concerned us, particularly if his case was one where the tumors would return within six months anyway. Because we knew we were up against a juggernaut of a disease, we were willing to sacrifice some efficacy for the sake of quality of life, but this new revelation made it clear that there was no middle ground where the radiation could be useful. The reality is that the time didn’t change the situation as much as it shed light on it. The delays from our initial verve to start CSI within a week after his August surgery have paid a valuable but sad dividend.

Doing Nothing is Something

Our meeting with the Mass General team, which included both attendings (Dr. Yock and Dr. Ebb, the pediatric hematologist/oncologist) and their fellows. We were jammed somewhat comically in a little exam room in the oncology clinic, though Dr. Ebb made no ceremony of his habit of shoving the exam table out of the way and against the wall to give grown-up sized people a chance to converse without sitting knee-to-knee.

On the Wednesday before Colin was to start treatment after the weekend, the team had to completely switch gears and, to boot, had no particularly compelling options to offer us. The several hours’ wait until this moment and our general understanding of Colin’s situation meant that we were not completely blindsided, nor were we expecting a miraculous alternative to slip into its place. However, this is not how things were supposed to go, and in some ways it was harder for Dr. Yock, who reacted as if she were taking something away from Colin, though we didn’t feel that way. The team at Mass General did more for Colin by doing nothing, and we appreciate the thoughtfulness and compassion that drove that advice.

Colin’s ependymoma has declared itself, as we knew it might in the near term and ultimately would. Some kids seem to limp along for many years, moving from one treatment to the next and accumulating scars while also achieving a near-stalemate with the disease. However, kids with the 1q gain, especially those with posterior fossa tumors at a young age, generally follow a much swifter trajectory, a fact that is suggested in the gross data on the molecular biology as well as anecdotal evidence.

The long remission before the first relapse gave us hope that Colin’s ependymoma was quirky in a way that would be beneficial to him, so we had hope: hope that radiation could buy him a few more years, and hope that good temporizing measures would come along in that time to give him more time with good quality of life. We weren’t looking forward to a drawn out game of block and tackle with the disease, but it felt like we could squeeze out some good years from it.

New Decisions

With a tremendous sense of relief that things played out as they did and we escaped treating him without measurable benefit, we took a step back to focus on Colin’s quality of life and contemplate our options. We had another discussion with Dr. Gajjar about intrathecal chemo, which would have few side effects and potentially offer some time, though in that case we have zero data on how any patients have done on it. We have decided to eschew any traditional chemotherapy that is like to have significant side effects, as our focus is to make sure that Colin can have the best quality time now while he’s not otherwise symptomatic.

One of the common go-to drugs for ependymoma is etoposide (VP-16), which is commonly delivered as an IV medication but can also be taken in a lower oral dosage. Although Colin’s never gotten it before, at this point it is not something we’re going to explore because the side effects are likely to be pretty bad for him. We could try it for a cycle, but the chances we would stop are high enough that we need to try other options first, and it’s not proven to be useful enough to be worth the risk.

Going back to the drawing board, there is a trial in Dallas, Texas for everolimus, which targets the mTOR pathway, as a single agent. There are other trials that combine the drug with additional agents, but these other agents typically fall in the category of “stuff we don’t want to do.” The Dallas trial is a Phase II that could lead to the approval of this drug for ependymoma for other children and it gives us rapid access to it; it’s approved for other diseases and some kids have gotten it approved by insurance, but it’s not a slam dunk and we were able to start in less than two weeks. The trial expects to expand sites to NYU, so we would be able to do our monthly travel to New York, which is obviously much more convenient than Dallas and less prone to weather-related travel problems.

A number of children have seen decent remissions (18 months, 14 months) using this or a related drug as a single agent. It is very well tolerated, delivered orally, and the only inconvenience is that we must travel for our monthly supply. The benefit of participating on the trial is that we would have access for up to 24 months without a pitched battle with insurance and it could help other kids get it more easily if it’s approved as well as make it easier to use it in the context of clinical trials combining it with other agents.

The fateful MRI was on 11/17, and Colin starts the everolimus on 11/30. I credit the many wonderful people who have supported our family for making sure that all of the necessary records made their way to Dallas and we were able to make travel arrangements expeditiously. By pushing to start so quickly, we also obviate the need for another scan; there will be monitoring scans every two months.

Father Time Isn’t Very Paternal

Right now, our focus as a family is on capitalizing on Colin’s current condition and doing as much as we possibly can. We have taken advantage of our time in Boston for a little tourism, including the Duck Boat Tours, which turned out to be a tremendous success. Thanksgiving, we were able to visit our family in CT and round up the cousins. While we’re hopeful that the everolimus will be helpful for Colin, we certainly can’t count on it, and we see the writing on the wall that this could very well be his last everything: Thanksgiving, Christmas, and birthday, if that’s even in the cards at all. Furthermore, we can’t count on him maintaining a good quality of life into the spring.

After so many wonderful years of remission, time is not on our side. We always knew this was a possibility and I held the intellectual fact that relapse lurked in the mist while not letting it infest my daily thoughts. Our hope was that it was inspiration, the bullet dodged, and not the ultimate trajectory. However, we’ve opened the box and we know the fate of Schrodinger’s cat. I can feel the vertigo that that we have passed the apex, that gravity has its grips on us, and we are on the wrong side of the parabola.

Math and physics have a tendency of prettying up life, but they can make poor metaphors for life, since we organic creatures don’t scurry within the confines of strict lines of force and motion. Yet again, we must live a dual existence, one in which we hope that the next two months are the start of a lovely and long relationship with everolimus and another in which we are back to the drawing board and ever more frantically preparing to organize our lives around an invisible disease process going on inside the confines of Colin’s skull.

We have started discussing hospice, plans to accelerate the long-discussed Make-a-Wish, and talk about the inevitable set of events we conveniently encapsulate within the sterile phrase “final planning.” All of these horrifying realities seem much easier to stomach while Colin is feeling well and we can labor under the illusion of his transient health rather than breathlessly staying only one step in front of the curve. There’s a pastoral sustainable cemetery near us that we’ll be visiting next weekend that fits nicely with Colin’s penchant for nature.

People have been asking a lot about how much Colin understands. He has certainly understood that he’s received more active medical care in the last year than he is able to clearly recall; his memories of his initial treatment at St. Jude are vague at best. We were already concerned before the trip to Boston, but matters came to a head when we were down there, though not immediately when he received the news about the change in the treatment plan. He knows that his cancer is back yet again and that this has taken the radiation plan off the table. He also knows that we are in Texas to get some pills he will take every day.

Colin knows that he is mortal and he has started expressing fears that he will die of cancer. He is scared that he will never be rid of it. He is worried that death will be painful. It’s hard to hear these words but important for him to articulate them and have us there to guide him through this process as best we can. We are not blunt about the prognosis, but we also don’t offer him fabricated reassurance. His ideas of his own mortality seem focused on a time in the distant future. I think it’s like Santa, which he finally strong armed me into fessing up about this summer. His reply: “I thought he wasn’t real, but I hoped that he was.” It was clear that I hadn’t crushed him, that he was ready for the truth, but also heart wrenching that I had to immolate the surviving bits of that ho-ho-hoing hallmark of childhood. Colin will ask the questions when he is ready, but by then he will also probably know the answer.

Over the past several months, and before the progression was diagnosed in August, he’s retreated much more into a world that is predictable and comforting to him, which consists in very large part of watching videos on YouTube (primarily Stumpy Longnose and Dan TDM; most people in possession of a similarly aged child are familiar with these geeky, kind hearted video game reviewers that have become stars of the early Elementary school set). We are trying to find the balance between allowing him this escape and forcing him to engage with a world that he finds increasingly treacherous. The Duck Boat Tour was terrifying to him before we started (it’s dangerous; I’ll get seasick; I’ll get wet) but ended up being the highlight of the day, and he asked to do it again.

Colin is always, if this is even possible, even more loving than he has ever been. He often looks up at me lilting charmingly in his little helium voice, “I love you, mom.” In the moments when he is happy like this, he beams angelically and I feel the compression of large volumes of love into a brief moment that will be harvested for many years to come. He is extremely attached to all of us, even though his relationship with Aidan is complicated by fraternal competitiveness.

Aidan, who traps everything inside his affable demeanor, has been assiduously avoiding the truth for months, but that dichotomy eats away at him. If we hadn’t already made headway before the meeting in Boston, he certainly understood what the doctors explained then. The tears he shed were a mixture of stale and fresh emotion and I don’t know if he fully tapped his own well, but he touched his own sadness in a way that was unstoppable and necessary.

One of the frustrating elements of this process is that we had just come off of high-fiving each other at what a great job we did settling into our temporary home in Beantown when we had to reverse the process and simultaneously formulate a new treatment strategy for Colin. We were so damned prepared for the holiday season that we were fully stocked with winter gear, including skis and skates. The return trip required a rental car in addition to our own, but once we started packing in earnest, we realized how homesick we were and left a day sooner than we had planned.

All of us were happy to go home early, though not as happy about the implications. Were we shocked? Not really. The writing was on the wall, and we were apprehensive enough about Colin’s situation to doubt whether this course would be right for him. We still felt the abrupt and numbing stun of being thrown into the sea of uncertainty when we thought we had landed on an island, but we have adjusted to the shifts and leaps that are necessary as hope morphs from one definition to another. Right now, we are consumed by the joyful hope of providing Colin with as many wonderful experiences as we can and fulfilling the wishes of a childhood that will never fully unfold.

Never Give Up

I know that some people find my blunt expectations of Colin’s death to be morbid and unoptimistic. Don’t lose hope. Never give up. I don’t and I never will give up on either of my children. The issue is that “giving up,” like “hope,” is a fluid construct that adjusts to the situation – or at least it can be, if one allows it. Many years ago, I wrote about the father who wailed, “Don’t stop!” at the ICU team that had been struggling to resuscitate his daughter for what seemed like forever. As much compassion as I have for that man and his deep despair in that moment, it is not how we interpret not giving up.

We cannot afford (Colin cannot afford for us) to define hope narrowly by a certain desired result that has, now, slipped through our fingers. Early on, I recognized the folly of restricting my idea of success to a cure because that perspective would not give proper deference to whatever amount and kind of life Colin would have, whether measured in months or years. Though we moved into territory where we could reasonably assume that Colin was actually destined for long-term survival, we always knew that we were only a scan away from a new road map of likely outcomes.

When that scan came, we all – Dr. Gajjar included – held on to the hope that Colin could end up on the favorable side of statistics, but that was not the case. When we optimistically removed that lone well encapsulated tumor from his left frontal horn, we were inadvertently planting seeds. Along the tract of that resection, where Dr. Boop entered and exited to remove the tumor and then scope the ventricle, one of the tumors from the recent scan sprang up. That lesion is a clock of sorts because that pathway wasn’t there until the March surgery. The tumor that took root along that channel took seven-and-a-half months to sprout and was not visible on MRI only three months before. Regardless, we put it there when we laid a pathway and the cancer-seeded CSF flowed into the new opening.

Colin’s brain is bathed in cancer cells of a disease that has resisted the most aggressive treatments we know of in a location that is difficult to access, so it shouldn’t be hard to understand why we don’t dwell on even the remotest hope of a cure. Such a vision is irrelevant and unproductive in the face of the reality of tumorlets studding the fourth ventricle, finding their way back to a home we forcefully evicted their progenitor from six years ago. To add to the picture, there is suspected new growth in the tumor remnants in that area, the sleeping lion that we always thought would be the culprit of a recurrence yet has been remarkably silent these past months.

One always has to choose how to focus one’s energy, and we serve Colin best by adopting a fluid definition of hope and not expending energy clinging to an unrealistic worldview. The process has been gradual since last January, though it is then that we had to face the fact that the statistics were no longer in Colin’s favor. We placated ourselves with positives laid on top of those more dire facts. The single tiny lesion in an easy place seemed so unthreatening, and all of us used that inoffensive image to skirt around the dismal reality: relapsed metastatic disease with ventricular seeding.

In January, we held out hope that it was otherwise and we were able to live in a hopeful place suspended within a matrix of uncertainty until the threads started to snap in August. On top of everything else, we only then became aware of the story told by the tumor testing from March and the pall cast by the chromosome 1q gain. Each step has brought us closer to the current moment and our ability to accept what is happening now and to embrace Colin’s life fully as it is right now in this moment.

Hope, Redefined

For our family, not giving up means not losing sight of life itself, which is not just the fact of being alive but the spirit of the endeavor. I will never give up on providing Colin with the best balance of quality and length of life, of always bearing in mind what is important to him and what enjoyment he will derive from loving and being loved with whatever circumstances surround that. These are, I will fully admit, impossible decisions. There is no knowing what constitutes good quality of life for another person, a lesson I learned when dealing with my elderly father’s health crises.

Atul Gawande, who writes insightfully about the medical profession, most recently wrote the book Being Mortal, which is about the way we handle aging and the prospect of death in our medicalized culture (succinctly: not well). Some of his own experiences resonated deeply with me, as we had just gone through the struggle of deciding on CSI for Colin. Knowing the reality of the prognosis and expectations of the effectiveness of treatment as well as side effects were critical data points in that process as well as every decision moving forward. This isn’t to say that we wouldn’t embrace a miraculous outcome, but we don’t seek it and we have to be careful about what we would sacrifice for any goal we pursue with treatment.

In his book, Gawande brings up the important exercise of asking patients what constitutes reasonable quality of life and what would satisfy them. This answer is often surprising, even to intimate family members, and he gives a few examples to this point. He also demonstrates how goals morph over time and patients are able to adapt, expanding and contracting their view of a fulfilling life to suit their physical circumstances and expected lifespans.

How does one sit in the shoes of a child who is only barely able to understand the concept of death itself and whom we are trying to spare the most frightening aspects of this experience? Gawande makes no attempt to answer that question, but his book certainly underscores the importance of having a clear mind about the cost-benefit analysis of medical decision making and incorporating discussions of death as an active subject rather than the elephant in the room.

We live brimming with hope, but if you want to know precisely what that means in a given moment, you will have to ask, as it can change on a dime. Today, at this moment, hope comes in the form of a bottle with a month’s supply of pills that may give Colin protracted stability with great quality of life. Hope comes in knowing that Colin’s participation in a trial may help other children and it may be part of a better way of providing up-front treatment for ependymoma. Hope comes in knowing that we will do our best to give Colin an awesome ride, however things play out for him.

The White Squirrel

One day when I was walking back to our Boston apartment at Christopher’s Haven, I noticed a splash of white fur in the bushes, a white squirrel going about its business. I hesitate to call it an albino because its eyes weren’t obviously pink, but it doesn’t matter. I’ve never seen a white squirrel before, and it immediately stood out. I watched it for a while and thought to take a picture so the boys could see, but photographic evidence of this sort usually is disappointing.

During our stay, I saw the squirrel two other times, the last when Colin was with us and we were able to point it out to him. As a creature, squirrels are commonplace and I have had a particular dislike of them for decades, finding them to be quite hostile and insufficiently cute to compensate for their poor temperament and the general nuisance they represent. Even I, the long avowed squirrel hater, can recall each occasion I saw this particular animal and do so with fondness.

We easily latch onto things that are remarkable in some way. So, too, children like Colin stand out. He is easy to remember, whether you notice the zombie gait or the devilish cackle; a warrior’s collection of scars or boundless imagination. Colin is a white squirrel, the kind that makes you stop and focus your attention on something outside of the mundane flow of life. White squirrels have the ability to jar us loose from the dangerously mesmerizing patterns of the mundane and we are lucky to have one so close at hand.

I want our little white squirrel to collect nuts and scamper up a tree along with his brethren. We will do everything in our power to keep him busy with his bushy tailed business as we push aside the melancholy of a future that cannot be indefinitely forestalled but that does not need to be lived today. A time will come when all we hold is a memory, but that time is not now. Today, I stand on the Thoreau Path that wends among the West End apartments in Boston and I watch a white squirrel in the bushes doing nothing special at all that is, all the same, acutely precious to me.