Plans and Perseveration

Saturday morning was a lazy one, but I determined to hit the loop inside of St. Jude’s campus before it got too hot for this Yankee girl to even consider it. The sun was already insistently warm before 9 am, but I figured I had enough time to squeak in a reasonable run before it became unbearable to my delicate constitution. The previous 24 hours had been full and draining, leaving me an exhausted heap curled around Colin in the blessedly comfortable bed at St. Jude’s Tri Delta House on Friday night. He had asked me to snuggle with him and I happily obliged, but in the morning I woke up still wearing Friday’s clothes.

Grateful for a friend to stay with the boys at LeBonheur on Friday, we made our way back to St. Jude for our consult with Dr. Merchant regarding craniospinal irradiation (CSI). We had always known that this option was on the table and that he was running a study on reirradiation of ependymoma, but it is the literal atom bomb of treatments with uncertain results and certain risks. The discoveries that came with Thursday’s surgery were really unsurprising, and it wasn’t as bad as it could have been. However, it directed us clearly towards CSI in an effort to treat the entirety of the fluid space in Colin’s nervous system.

For some children, CSI produces durable remission; those with local and metastatic progression have the worst outlook, not accounting for molecular biology (at the time still unknown). When we initially learned about Colin’s recurrence, we believed that we could hope for as much for him, knowing that durable remission becomes harder to achieve with each recurrence or progression. However, also knowing the lasting side effects of that treatment, we had expected to simply hold off that option for as long as possible.

Radiation Consult

Today, we see CSI primarily as a salvage treatment to buy Colin quality time rather than as a curative. Clearly, we welcome durable remission, but we’re simply not counting on it in our management of his situation. With this in mind, we entered the cool halls of the basement at Chili’s Care Center where the radiation oncology department lives. We have been down there many times, both for Colin’s initial radiation treatment and for many follow-up visits in which Dr. Merchant rejoiced over Colin’s improvement from the early days, when the boy made a habit of falling asleep during every consult. It was such a consistent pattern that we accused Dr. Merchant of having a somnolent effect on Colin, and we probably should have recorded him reading bedtime stories.

Many of our concerns around CSI revolved around the possibility of sparing important functions that we knew would be jeopardized by irradiation; we were willing to accept a higher risk of relapse in order to preserve function that we consider important for quality of life. The short answer, in each case, was that this invited risk of relapse and was impractical, as these structures were too close to the ventricular spaces that we absolutely need to treat. I expected to hear this, in part because I knew walking into the consult that Dr. Merchant has a very cure-heavy bias – he does, after all, insist on bandying about that word, even though most oncologists shy away from it, especially when talking about ependymoma.

I was quite dogged in my challenge of the statistics that Dr. Merchant offered (50% “cure” after 3-5 years with CSI) that sounded alluringly hopeful but that I did not feel applied to Colin’s case. I did not even bother to complain that nobody, even the most optimistic, uses the three-year mark to define “cure”; ependymoma cannot be properly assessed on a five-year scale anyway because of the high incidence of late relapses; and that second remissions are often longer than first remissions, so assessment of second remissions must use a different yardstick.

What I did focus on was the particulars of Colin’s case, particularly the 1q chromosomal gain, which is widely recognized as a tremendous risk factor, though the statistical significance related to reirradiation is not known. Your 50% figure certainly does not describe Colin’s realistic statistical profile, I explained; we may lack the data to know what it is, but we’re certain that it’s lower. Given the persistence of the disease and the clear evidence that it has populated the ventricular space, we see indications of an insistent, if not rabid, process.

There is a clear line between the study of a disease and the treatment of patients; while Dr. Merchant takes both missions seriously, he does not allow speculation and conjecture surrounding one individual to bend his interpretation of the existing science and the figures that have emerged from known studies. My intellectual exercise, though focused on the same patient, was different from his. I felt that it was necessary to reconfigure the thinking around Colin’s care around the supposition that quality of life was more important than cure. His response was that, for parents, all of the admittedly troubling long-term side effects pale in comparison against future clear scans. His primary concerns in terms of risk revolve around necrosis and vascular damage, both of which can have fatal results; my observation is that there’s nothing an oncologist hates more, when losing a patient, then losing to something other than the cancer.

Despite much verbal jousting and inquisition on my part (I made Dr. Merchant earn his keep during that meeting), we knew that we had walked into that office to ask for CSI and that there was no other practitioner we would rather have guiding Colin’s treatment and at no other institution. The planning CT scan is scheduled for Monday and we settled on the day after Labor Day to start treatment, leaving a gap for us to return home to prepare for a six-week stay in Memphis.

The feeling, walking away, was a combination of hope, resolve and resignation. Dr. Merchant’s confidence in the efficacy of CSI for relapsed ependymoma was enticing, and we felt the encouraging emotion of it, but I intellectually knew that the fundamental analysis was flawed. I was unable and unwilling to shake the cognitive dissonance of those dichotomies, even as we started to discuss planning around the six weeks of upcoming radiation.

Colin and His Cranium Earn Their Freedom

Returning to LeBonheur, we were soon swept up in the logistics of discharge. This last surgery was purely endoscopic, so Colin never even went to the ICU, nor did he go on a course of steroids to deal with brain swelling, since the endoscopy tube is less disruptive to the brain tissue than cutting would be. Why I don’t really understand; it still seems destructive enough to shove a metal tube through the brain, but I wasn’t going to question it, and I was pointedly instructed that there’s no “shoving” involved.

There is a very small pseudomeningocele (pocket of CSF leaking through the dura) under one of the incisions that nobody is very concerned about, but it’s a sensitive spot that we’ll be watching assiduously. The area around the port is also sore, though the surgeons did a tremendously beautiful job of replacing the port and used the previous site, so it barely looks like he had any kind of surgery. The two spots are glossy with durabond and otherwise only a bit redder than the previous scars.

Colin, being the absolute bad ass that he is, has refused to take any pain medication since surgery, though he does sometimes complain of pain (“later,” he says, when we offer it, but the necessity of later never comes). Aside from some now-resolved constipation, he has been tiring more quickly through the day and is still unsteady on his feet, though we now trust him to amble about on level ground without somebody holding his hand/shoulder.

After discharge, I had to fill various prescriptions at St. Jude. While waiting for the more complicated component of Colin’s follow-up medications, Dr. Gajjar happened to walk out of clinic and we greeted each other cheerily. He knew, of course, about the CSI plans, though we still had a meeting set for Monday afternoon. I explained that we felt that we had no other choice, that gamma knife wasn’t going to give us enough confidence in achieving a remission. We talked about the difference in perspective on the utility of CSI, but he asserted that we have a well informed understanding of the disease, which is reassuring on the intellectual point but also sobering.

Another Option Emerges

We discussed the need to treat the volume, and the dearth of options outside of CSI, and he asked if we had considered intrathecal chemotherapy, specifically topotecan. The injection of chemo directly into the CSF seems to make sense, since the blood-brain barrier keeps so many complex and large molecules (read: chemotherapy) out of the vascular system that serves the brain. However, the fact that the two methods deal with very different fluid systems is important, and we see the results in clinical trials. Tumors, in order to grow, rely upon the blood supply, and they take up chemicals from the blood readily. The CSF, on the other hand, flows freely and inconsistently around tumors and cancer cells, and they are not directly feeding the growth of the tumors, so it is a less effective delivery system for anti-tumor agents.

That said, for a disease that is clearly floating willy nilly within the ventricles, there may be a rationale for intrathecal therapy, though ependymoma is notoriously not very sensitive to chemotherapy.  They haven’t had much experience with this particular regimen at St. Jude, but Dr. Gajjar  promised to get in touch with his contacts elsewhere and find out more about their track record with ependymoma patients. The CSI would have an impact on Colin, but this course might offer very good short-term quality of life while still preserving the CSI option.

As I have said many times before, decisions are temporal, and they change with new information and circumstances; even a plan designed only hours before now suddenly seemed tenuous and up for reconsideration. The possibility dangled in front of us of CSI as our best hope for a longer term remission is tantalizing, but at what cost? Is there a different staging strategy that would allow us to maintain Colin’s current quality of life with some reasonable chance of a nice remission that would allow us to go back to the trough for CSI later?

There are so many layers to this decision, as part of the rationale for doing the radiation now is that he’s in good condition and therefore better able to handle it. This affects both short-term and long-term side effects. Furthermore, in the process of delaying by trying something else, we take the risk that the disease itself flares out of control to the point where the CSI as a control is less effective. This is a very real concern, especially given the new proliferation. There is the added X factor of the immunotherapy, which may still be serving to control the disease to some extent and concealing the virulence of this resurgence of Colin’s ependymoma. There is also the X factor, more properly the C factor, of Colin himself, who has proven himself to be unusually resilient and, as his brother says, “if
anybody can get through it, Colin can.”

Really, we were lucky to catch things when we did; those tiny tumors, grown, would not result in a single lesion hanging in the ventricle but multiple spots. At the moment, we have minimum residual disease, with nothing evincing on an MRI, but logic tells us that there must be other incipient tumors waiting to peek their heads up for the camera.

Phrased that way, the CSI plan still feels nearly unavoidable, yet I find it impossible to not consider the intrathecal chemo option. Adding to the complexity of the decision, Ian had talked to Aidan about his perspective on our approach to treatment. When I questioned Aidan further about his thoughts on Colin, his response was the single most heartbreaking thing I’ve heard: “I can’t live without him.”

Catharsis

It is no wonder that I curled up around Colin’s familiar solid warmth and, after long minutes of contemplating the impossibility of this decision, of ever knowing what was right, I felt tears seep between my lashes. It was the first I have cried since the first news of the relapse, though it didn’t precipitate a violent outpouring of emotion, as one might expect; I couldn’t have evoked that if I had tried, partly because I have accepted my child’s mortality and his suffering in the name of a cure in bits and pieces over the course of six years. I am not much of a crier, and this was a gentle catharsis that left me steeped in a combination of warmth and exhaustion that carried me into the morning.

The morning run in emerging heat forced some kind of clarity as I made my rounds of the sinuous loop around campus. The lantana, formerly brilliant in the early light of day, today looked muted under the full sun of the morning, its colors submerged under its dark green leaves. I could not help but think of that boy, all he has gone through and overcome, and how difficult it is for me to strip that identity away from him.

Colin has fought hard to get where he is, and it feels like he’s hanging on the edge of mountain by his fingernails; I fear that CSI would sweep him off the face into a ravine. He already expressed frustration over a modest increase in his facial palsy that was affecting his face and his speech – this was for a short period before coming to Memphis and has since resolved, but the sight of that child staring into a mirror, contorting his face and exclaiming in frustration, gives me a glimpse into the future. How would he respond to more of that or, worse, the gradual failure of his mind to hold thoughts? Children do notice these cognitive changes.

Under the pressure of the heat, energy compressed into the basic elements of movement and thought, midway through the last circuit, I was convinced that CSI was absolutely unpalatable. Late in the night, suffused with coffee and pushing my mind to a different form of exhaustion, it seems unavoidable. I am everywhere and nowhere, a position that may externally appear to be unenviable, but in reality, it’s the best seat in the house.

Before rounding Chili’s Care Center and emerging from the shade into the dense early heat that foretold an oppressive day, I was overcome with the gratitude and empowerment that come from the tremendous fortune that modern technology has afforded us. It is easy to lament that we are still lacking the tools to cure these children and do so without marking and even contorting them for life. I do yearn for treatments that improve efficacy with reduced long-term impact, but I can just as easily look backwards and consider Colin’s plight 20 years ago, when he would not have made it to kindergarten, much less third grade.

Colin continues to be the luckiest bad luck kid on the planet. We are caught in the dilemma of decision making because we currently have at our disposal the broadest arsenal of tools that have ever been available, including an immunotherapy regimen that he is the only child to ever receive. We have the luxury of a decision to make, even if is an impossible one with unknowable consequences. Tonight, he sleeps in peace and luxuriates in the love of his family and friends. It is all a boy could ask for, and the contented smile on his face and satisfied high-pitched hum as he settles into his spot are all we could ever hope to see.