Summer Kick-Started with a Scan

Even on the cusp of summer, Memphis is unforgivingly hot, but the weather is nearly irrelevant when you’re back for check-ups at St. Jude, which we were at the end of June. I packed instead for the climate-controlled stasis of the hospital and the expectation that our days would devolve into a mad dash of hurry up and wait, then hurry up again.

Colin and I hit the ground running, touching down at Memphis International at 12:38 and checking in for his first appointment at 2:00. The visit got off to a good start with an educational sit-down with the endocrinologist. If you just go by the numbers, Colin has shrunk in height because we have begun measuring him standing rather than lying down. Of course, he hasn’t shrunk at all, but the transition was necessary, despite our hyperattentiveness to his growth curve, because the growth chart using height measured lying down runs out at age four, a threshold he crossed between visits.

Between the fake shrinkage and the fact that Colin’s weight has remained exactly the same since January (16.6 kilos on the last three trips), his body mass index remains stable at the upper end of healthy, so there are no concerns that he is failing to thrive. As he gets lankier, he looks more like a boy, a fact that many people commented on during our trip.

The endocrinology visit was primarily my doing, since Colin’s tumor had ventured unusually close (for an ependymoma) to the pituitary gland, the hormonal master gland that signals responses to other glands throughout the body, and I wanted to learn more about what potential signs to look for if the pituitary were damaged. Dr. Merchant once described the plan for radiation as resembling the Statue of Liberty. Using that image, the hand and torch jut into the middle of Colin’s head, with the flames pointing up toward the pituitary.

Beams of radiation, while intended to converge on the original tumor bed, also singed the pituitary and put Colin at higher risk for endocrine complications. Generally speaking, ependymoma kids who receive focal radiation treatments (I’m not sure if this statistic is restricted to the three-quarters of patients with tumors in the lower half of their brain, like Colin) have a 25% chance of an endocrine issue down the road, and Colin’s individual risk is higher than that.

The most common deficiency is of growth hormone, and while the hospital does periodically test these markers, it turns out that lab work alone isn’t especially useful. The primary gauge of whether the hormonal signal for growth is working is growth itself, which seems simultaneously crude and a real no-brainer, no pun intended. I don’t need a yard stick to tell that Colin is getting taller, so we’re safe on that count for the time being. Growth hormone deficiency affects more than stature, so it’s important to keep tabs on.

The pituitary damage can also result in thyroid, adrenal, and other insufficiencies that can cause symptoms ranging from fatigue, diabetes, precocious/delayed puberty, to poor (potentially fatal) stress response. Most of the assessment is based on lab work and clinical symptoms, but the doctor also walked through the door carrying a series of oblong graduated wooden beads on a loop of string. It struck me that she looked more like an occupational therapist than a physician and the purpose of the beads was not obvious, as they spent most of the time untouched on the counter. She consumed most of our time giving me a tutorial on the endocrine system, using the back of a sheet of paper with Colin’s (thankfully normal) lab results to inscribe long arcs and arrows diagramming the path of various signaling hormones from the pituitary.

What a Vas Differens That Makes

The beads didn’t come into play until the discussion on puberty; it turns out doctors use them to measure testicle size. Of course, as any normal mother would, I immediately shrieked in delight, “Oh! That’s what those are for! Ball beads! That’s awesome!” and collapsed in laughter.

Apparently, the teenaged boys don’t respond similarly and dread the appearance of the otherwise innocuous looking wooden beads. In the case of this particular (attractive female) doctor, I’m surmising it’s more likely due to embarrassment than the mortification of unwanted touching.

The three smallest beads are painted a darker shade of blue than the rest and represent pre-pubescent testes. Never one to shy away from a cheap joke, I exclaimed, “Yes, Colin has blue balls!”

Despite my observation, she checked for herself, recording exactly which blue ball represented my future grandchildren. To summarize key points from the rest of our delightful (more so to me than her) conversation on this subject, no, she is never tempted to wear the beads as a necklace and, in a related point, is grossed out when small children play with them or, worse, put them in their mouths. She was unmoved by my observation that these are purely representative wooden models and not actual testes. For less uniformity but greater verisimilitude, they could take the real deal from donated cadavers and inject them with plastic, drill a hole, and put them on a string. Oddly enough, this idea (and possible new business?) didn’t seem to warm her to the wooden beads.

Suffice it to say that Colin’s trip to St. Jude got off to a good start, though he did not share my boundless amusement at our first appointment. Our second appointment (not to worry, I don’t plan to detail every step of our visit) was with Dr. Thompson, our beloved ENT, who gave his blessing to have the trach stoma closed in August. Although it has closed some, it is still fairly large and will never close entirely on its own. In addition to the other benefits we have been focusing on, he added that it will improve Colin’s overall respiratory health by allowing his lungs to better inflate.

Can You Hear Me Now?

We also picked up a hearing aid system. The audiologist fitted him for it on our previous visit and his ear is already starting to outgrow the brand new mold – yet another reason why we don’t have to question whether he’s growing. Before, we had been using an FM transmitter that helped him better hear and focus on his teacher and/or aide. The hearing aid goes one step further and enhances sound to compensate for his high frequency hearing loss.

It turns out that Colin is the beneficiary of a number of technological improvements. First, hearing aids used to just amplify sound, but apparently this isn’t very effective in high frequencies. This device actually compresses the frequencies he doesn’t hear well into his audible range, programmed according to his actual hearing loss. Second, he has a CROS piece that is basically a microphone on his right ear that transmits that sound to the hearing aid. In the past, these were connected by a cumbersome wire and poorly tolerated by the kids, but he has a wireless system. Third, electronic miniaturization and tiny batteries allow the manufacture of devices small enough to be used by children. As a bonus, the new hearing aid is compatible with his existing FM system, so he can still use that to better focus at school.

Colin is the first St. Jude patient to use this system in part because of his unusual hearing loss, which combines complete loss on one side with partial loss on the other. The difference in his responsiveness was immediate, and he is sporadically compliant with the devices. We shouldn’t have a problem getting him to use them all the time at school because he’s already used to using the FM system there, but he goes through periods at home where he refuses to use them, even though he knows they improve his hearing.

Although Colin was doing a pretty good job with “s,” “f,” and other similar sounds, we can hear how much more clearly he is enunciating these sounds in particular. Today, the kid who struggled to say “mama” is a veritable chatterbox with all kinds of observations, questions, and jokes. Dr. Thompson said that once the trach stoma is closed, Colin will be twice as loud, so I suspect I’m going to miss the ability to pull the cork (take the Passy-Muir valve off the trach) and control the volume.

The Same Old Brain

Colin’s MRI looked “stable,” something we are pleasantly if warily accustomed to hearing. With the benefit of time, now more than a year from radiation, I figured we might have something meaningful to discern about the mysterious garbage that shows up on the scans (i.e. do you have a thought on what it actually is, and would tumor or scar tissue look different after some period of time). However, the doctors assert that we just need to keep watching (duh) and that they understand the sentiments of a worried mom.

Again, I have to explain the difference between “worry” and “information gathering.” After all, we were told that time would be an important factor in understanding Colin’s disease status, so +time, it seemed worth asking. Dr. Merchant even offered that he is pondering the question of whether doctors should use the term “no evidence of disease” (NED) for long-term survivors who had previously had near-total resections and been classified for years as “stable.”

To me, the words are meaningless and don’t add to the understanding of the situation if there is no other fact that supports a change in terminology. If, after time, there is a difference in outcome between patients deemed stable vs. NED, then that would matter. If, as I suspect, all of these long-term survivors get lumped into the same statistical bundle for recurrence, then a change in terminology hails a false victory.

For Colin’s sake, the size and identity of the residual tumor/garbage were always central to opinions about his prognosis. Of course, we’ve done all we reasonably can at this point, but this falls under the category of gathering additional information. Maybe I am cutting my definitions with too sharp of a blade. At this point, just to get people (by people, I mean oncologists) to stop asserting that I am “hand wringing” or “worried,” I will just shut up about it.

Right now, we are in a very easy place. Colin continues to improve in every way and we have no indications that he is suffering any of the more serious potential side effects of treatment. I had been particularly curious about endocrine issues, and though they can certainly crop up over time, the fact that he has remained unscathed so far is a good sign. While we know plenty of kids with various endocrine problems, and we know that they are all treatable, it’s still a relief to know that this is one thing we don’t have to actively manage. Trips to the hospital are primarily an opportunity for him to show off his new skills and demonstrate what an imp he is.

Brain Tumor Poster Child

Fairly early on in our adventures at St. Jude, ALSAC (the fundraising arm) asked us if we would be willing to have Colin filmed for the one-hour Partner in Hope special. It’s really an infomercial that airs nationally to encourage people to sign up to make regular donations to the hospital. In agreeing to participate, we had nothing to lose and some really awesome home videos to gain. More importantly, of course, we knew that Colin’s story could help the hospital.

The piece is now airing nationally and is called Never Give Up, and Colin is one of the kids featured. In much of the footage, Colin looks very pathetic and we talk about how debilitated he has become. There is an embarrassingly self-pitying clip of me sobbing about my family’s misfortunes, followed mostly by a graphic depiction of perseverance and recovery. I only mention it because I hate seeing it but I’m told it adds to the arc of the story and I accede since it helps the little children with cancer.

As it happens, the team that does the filming was in town during our visit, so they arranged to follow Colin for some of our appointments. In physical therapy, I asked Colin to show Miss Angela how he can walk on his own, and he cooperated without hesitation. The cameras captured both his unaided steps and her visceral reaction, though neither she nor I was thinking about the film crew at that moment.

The people who have seen Colin founder through treatment deserve to see him reclaim his childhood, and in retrospect, I was very happy that we collected even more documentary evidence of his improvement to share with the world. His story has been an amazing one, and I marvel at it every day when he does something normal.

I love watching Colin sit or stand and play with unselfconscious intensity. I marvel at how erect his body is and how he just doesn’t look sick any more. He has a full thatch of dark hair, marred only a little by the various scars that curve along the back of his head. Maybe the most abnormal thing about him is how happy he is. I have no way of knowing what a brain tumor-free Colin would have been like, but he seems to appreciate the new freedoms that his recovering body gives him as well as many of life’s basic pleasures.

Eating Machine

One of the biggest changes over the last six months is the recovery of Colin’s swallow function to the extent that he is taking in all of his food by mouth. This happened somewhat by accident and without the benefit of a new swallow study to confirm that he can swallow effectively. All testing aside, we’re not about to stop him now.

My personal expectation was that it would take about a year to get Colin eating completely by mouth, in part because he had to regain strength and endurance in his tongue and mouth. Our early experience was that he could eat, but only very slowly and certainly not enough to sustain himself, so his marginal pass on solids on the previous swallow study was not a great concern. After all, it was important for the sake of rehab to have him eating a little bit, and that is exactly what we were cleared to do.

Before I knew it, Colin was eating everything and I had to stop giving him canned feeds because they only caused problems. He is still more limited on textures, and by this, I mean that broccoli and carrots have to be cut small in order for him to eat a nutritionally significant amount of them, but he can certainly eat them. He has a pretty much normal diet for a four-year-old, with the exception that the g-tube allows me to give him pureed vegetables if he doesn’t cooperate, and I also give him V-8.

Of all the miracles related to Colin’s recovery, this is the one that is most clearly driven by him as a person. I recently asked him if he could remember when he wasn’t able to eat and he said no. If he can’t remember when he couldn’t eat, which is not long ago, how could he remember that he used to eat when he couldn’t, which was well over a year? Only recently has he relearned the vocabulary of meals, thirst, and satiety (“I don’t want lunch, I want a snack,” or “Me full”).

I know that saw others engaged in the act of eating and that we reassured him that he would be able to eat again, even as we withheld food from him, but there must be something more fundamental that drove his desire to eat. For months, he would cry when he saw anyone eat, so we hid (in the hospital) or ate in shifts (at home) so as to distract him during meal times. Aidan knew to eat his snacks in private or else his brother would cry.

On the one hand, I wanted that suffering to stop, but on the other, I knew that it was linked to his desire to eat, and his determination has served him well. If you talk to the parents of most kids who have had to stop taking in food by mouth for long periods, even when the physical ability returns, it takes longer for them to get over the oral aversion and actually eat enough to sustain themselves.

I am overjoyed about Colin’s eating, but I am also relieved. I often think back to the difficult decisions we had to make in the course of Colin’s treatment, and one was exposing him to the risk that he would lose some basic functions temporarily or even permanently. Obviously, he is getting back most of what he lost (his hearing will not recover until somebody comes up with effective nerve regeneration technology), but I also swoon to think of the possibility that the tumor could have gained momentum before he had the opportunity to improve.

Some parents decide not to pursue aggressive surgery, and we have seen many kids with residual tumor recur. The statistics support the fact that survival is linked to successful removal of the entire mass, and our observations have unfortunately supported that – neither new chemo regimens nor radiation techniques seem to alter that base fact. That said, even a gross total resection does not guarantee short- or long-term survival. Colin could have ended up among the population of patients who endured significant deficits and still weren’t able to outrun the disease.

When I look at Colin, I see a joyful (and stubborn, and cantankerous) four-year-old, but I also freshly feel the wind and whizz of a bullet passing by my ear. At the same time that I am grateful for his life, I am selfishly grateful that I do not have to forgive myself for the decisions that we made.

Good Bad Luck

We are so lucky. We are lucky that Colin is happy and alive, that he is getting better, and that he is living a healthy and able life beyond treatment. We are lucky that Aidan is a wonderful, normal kid who loves/hates his charming/annoying brother. We are luckier than you know.

July always seems to be a rocky month for the ependymoma community, which we are part of through an international online group. This year, five parents lost their children during the month and many more are dealing with new recurrances.

When people look at our family (well, really just Colin) with something ranging from horror to pity, I always want to scream, “You have no idea! We’re the lucky ones!”

If you start looking, you can find good luck and bad luck around every corner. Numbers don’t drive results; they only describe them. Statistics can hold despair or the allure of false comfort at the same time that they can provide a needed framework for reality or hope.

In my experience, when you live with a miracle, you feel humbled rather than impervious to bad odds. I find myself marveling at how well Colin does normal things, like climb up on the bed with a book, and I think about how I didn’t know I would get the gift of watching these moments. I don’t believe that there is a Great Statistician that will even out our odds, nor is there positive mojo at work that will improve our fortunes at the casino or lottery.

I do know that Colin’s luck can turn, as can anybody’s. The only thing that matters is that I appreciate the small moments. I remember that being an exercise that required constant vigilance, but these days, it’s just a component of daily life.