Posts in the Rebecca Category

Rebecca’s spark has dimmed, and we don’t know why.

On days we go to Pittsburgh, she sleeps the whole way there, is pretty normal at the hospital, and then sleeps some or all the way back home.  When we get home around mid-day, she usually stumbles to the couch and takes a long nap.  She might have a few normal hours of wakefulness starting mid-afternoon, get energized around dinner and bedtime, and then go to sleep for a full night.  Or she might spend those hours not doing much of anything except lie awake.

On days Rebecca goes to kindergarten, she usually walks there and has a normal morning.  And then, right around lunch time, she usually runs out of steam.  We had to go pick her up the other day because she’d fallen asleep at her desk, deeply enough that normal attempts to rouse her got no response.  (Normal, as opposed to “THERE IS A FIRE WAKE UP NOW!”)  We got her home, and she stumbled to the couch and took a long nap.  Once that was done, she didn’t do much for the rest of the day.

Even when she’s awake, at least half the time she seems distant, disconnected.  Much of the time she’ll just lie on a couch or a lap, not moving much, not doing anything but staring off into space.  You can catch her attention and get a warm smile or an air kiss when you do, but that’s it.  She’s there, and yet not there.  At her most energetic now, she’s much more subdued than she has been in the past.

And we don’t know why.

We’ve ruled out seizures.  The EEG showed absolutely no trace of any seizure activity, even when she just sort of stopped and stared off into space, as she does so often these days.  Everything was completely normal, diagnostically.

We think we’ve ruled out pressure due to tumor growth, given the latest CT.  We’ve moved up her next MRI, and expanded it to most of her body, to see if there are tumors elsewhere, or if the CT somehow missed something.  Maybe a thalamic tumor.  Maybe not.

We can’t definitively rule out a virus of some kind.  Her viral panel was negative, but it’s a limited test.  Everyone in the family has had a virus that drained us, as have some of our friends, but most of us recovered in a few days and Rebecca’s been like this for two weeks now.  If it’s a virus, then something is keeping her from recovering from it.  But her blood work is normal, her immune system apparently fine.

We can’t rule out the possibility of side effects from the p28 treatment.  Rebecca is the 38th child ever to receive this treatment, and one of a very few who’ve gotten it at her dosage level.  Maybe this is all just a huge side effect.  Except it’s been almost a week since her most recent treatment, and there doesn’t seem to be much, if any, improvement.

In the absence of certainty, it’s hard not to descend into fear.  Maybe none of these things are what’s wrong, we try not to think.  Maybe she’s just dying.  It’s a little like being deep in the heart of an unknown forest at twilight (or is it sunrise?), unsure which way to turn and what’s really dangerous.  A snake could be in that log.  A rotten tree might fall.  There might be a bear.

I grew up in forests; Kat’s totally a city girl.  But even I can’t help feeling like Rebecca is leaving us slowly, a tiny little bit at a time.  Intellectually, I know that may not be so, but in my gut I feel it.  I look at her lying limply on the sofa, her eyes vaguely focused on something a thousand miles away, and my stomach twists icily.  I want to shake her fully to life, drag her back to us, scream until the spell is somehow broken.

Instead, I take her hand and smile at her.  I sign “I love you”, tapping it to my heart as we always have in our family, to see if she’ll do the same.  Usually, she speaks “I love you” back, because that takes less energy, less movement than signing.  Her eyes crinkle with warm affection when shes says it, looking into my eyes, holding my gaze for a few moments.  Until her gaze slides away and loses its focus on the here and now.

If Rebecca were cranky and irritable from exhaustion, that would actually be easier on us, because that would make sense.  That would be normal.  It’s the quiet inertia that really, deeply scares us.  Whatever our heads may derive from looking at all the maddeningly incomplete data, our hearts are filled with fear.  We’ve tried to push it away, and also tried to accept and acknowledge it, and neither approach seems to help.  The fear persists.  We’re afraid that her light is very slowly going out, that she’s fading from this world, that she’s leaving us.

And we don’t know why.

Perhaps posting this publicly will make all the symptoms go away, and I’ll wonder some day what I was so bothered about.  I hope so.  I’ll absolutely take feeling foolish in exchange for having her be herself again.  I’ll even accept never knowing why she’s been like this.  Just as long as we can put this forest behind us.

• Deep in the Forest of Fear was published on Thursday, May 29th, 2014.
• It was assigned to the Cancer and Rebecca categories.
• There have been thirteen replies.

As soon as the doctors came back into the room from looking at the just-completed CT images, I knew the news was not bad.  I didn’t know exactly how not-bad it was, but I could tell just from the way they walked into the room that the worst had not come to pass.  The expressions on their faces simply confirmed it.

And then their words: the primary tumor’s size is essentially unchanged since the MRI that was done three weeks ago.  There are no obvious solid tumors arising from the flare sites.

This is huge.  The rate of growth previous to starting the p28 study was frighteningly rapid.  If unchecked, it should have been at least a third bigger, maybe half again as big, maybe even twice as big, by now.  Instead, it’s basically the same size.  The flare sites have not produced tumors of any notable size, and may have produced none at all.

I can’t say if the primary tumor is exactly the same size, or a little bigger, or what, because today’s image was a CT scan (done in the Pirate Island imaging room, by the way).  As such, it can’t be directly compared to the last MRI.  We won’t get a more precise estimate until another MRI is done, and even then it won’t be ultra-precise.  Medical imaging can only get you so far, precision-wise.

The tumor isn’t being eliminated, because that’s not how this treatment works, but it is being held in check.  The p28 is buying us time — enough time, we can hope, to find a way to shrink or possibly even eliminate the tumor.  That’s where our energies will be directed in the next few weeks and months.

So after all my angst and worry about making a blind choice, there was no opportunity to make it.  In a sense, we had no choice, though of course we always had the option to refuse.  Given the situation, though, we ended up imaging earlier, and differently, than expected, for unexpected reasons.

The reasons were that, starting Friday afternoon, Rebecca became very tired, almost to the point of listlessness between daytime naps.  She hasn’t napped in years, and suddenly she was sleeping twice a day, going to bed early, waking up late, and still having low energy.  There were periods where she’d rally and be her usual self, but they got shorter and less frequent.  Yesterday, there really wasn’t a time where she had her usual energy levels.

Our instincts basically got whipsawed into pulp.  There were a few times over the weekend where I nearly tweeted that she was dying, because that’s how it felt to both me and Kat.  Then she’d rally, and we’d think that maybe it was just a virus.  And then she’d slide into a torpor that felt so much like the days before her seizure last August.

The thing was, it was only fatigue and lethargy.  There wasn’t vomiting, as we would expect with intracranial pressure, nor complaints of headaches.

Then, last night, there was an incident that seemed like a very small seizure.  We talked with the on-call nurses both here and in Pittsburgh, and both agreed that we should get her to Pittsburgh for her p28 infusion and further evaluation with the study doctors.

Kat and I were pretty well convinced that the CT would show a much larger tumor, not driving up pressure so much as disrupting the normal brain tissue and slowly killing her.  We were braced for the news that the drug wasn’t working and that she was out of the study.

As we waited for the CT machine to spin up, Kat broke from our embrace to look me in the eye.  She asked, “Are you ready for the results from this?”

“No, of course not,” I said.  “But whatever they are, we’ll deal with them.”

Less than an hour later, we were asking the doctors to repeat their findings.  No change.  Our intertwined fingers contracted, squeezing as one, disbelief translated into white-knuckle pressure.  We turned inward, fell into each other’s arms, and wept tears of relief and a kind of joy.  For the first time in months, our tears came from something other than fear and sorrow and pain.

Tonight, Rebecca is in yet another hospital bed, this time at the Cleveland Clinic.  She’s still deeply tired all the time, and if the tumor isn’t exerting pressure, then there has to be another cause.  We might have waited it out to see if it’s viral, but there are enough other signs — the incident last night, some subtle physical changes, behaviors that might not be behavioral — to tell us that she’s probably having micro-seizures, and having them pretty often.  The tumor would be the most likely culprit here, considering where it’s located.

So tonight a technician is gluing a couple dozen electrical leads to her scalp so the EEG monitor can tell us if we need to add an anti-seizure medication to her daily routine.  In one way, I hope that it does, because that would be a known, fairly easily-addressed problem.  We’ve done anti-seizure medication in the past, and have a good idea of what works for her.

Of course we don’t want her to be suffering seizures of any kind — she’s suffered enough as it is — but as Kat observed, we’ll take a seizure disorder over an unchecked brain tumor any day.

We’re not out of the woods yet.  Even if the p28 is preventing the tumor from growing, which is all we can realistically expect it to do, the tumor is still there.  We have to find a way to deal with it, because we can’t expect the p28 to be effective forever.  But if this initial effect holds up, we might have several months, possibly even a year or two, in which to search for a stronger, more permanent treatment.  We’re checking into a number of clinical trials, some you’ve heard about in the news and others that were unknown even to our neuro-oncology doctors.  (That’s not a knock on the doctors; there are a lot of studies happening at any one time, and always more being launched.)  We’ll use the time the p28 is giving us as best we can.

A long day, and a very emotional day, but a good day, one of the best in a long time.  Any day you end with more hope than you started with is a day worth celebrating.

• A Good Day was published on Monday, May 19th, 2014.
• It was assigned to the Cancer and Rebecca categories.
• There have been nineteen replies.

Half an hour after we left UPMC this morning, just as we were approaching the entrance ramp for I-76 West, Rebecca threw up with no warning.  She threw up a lot.  We pulled over and scrambled to get her out of the car so we could clean her off and check for any other symptoms.  She stood with her arms held away from her body in the classic “ick” pose, until I peeled her shirt off to get most of the vomit away from her.  “It’s all over my arms and legs!  Why do I have to be like this?” she wailed miserably.

I pulled her to me, wrapped her in my arms, and said over and over, “I know, honey.  I know.”  Keeping my voice as level and calm as I could.  I don’t think I did a very good job of it.

By the time we’d gotten her and her car seat mostly clean, she felt fine, except for the taste in her mouth, which she pronounced “disgusting”.  We found the nearest drug store, bought some wipes and paper towels, completed the cleanup, and got back on the road, a newly fresh set of fears riding with us.

It could be the experimental treatment she’s taking, which is known to induce nausea in some kids, though she’s never been sick from it before.  It could be an aftershock from the GI ailment that went through the house the past few days, which had every single one of us dealing with unpleasant symptoms at one point or another.  It could be that she choked on her juice and triggered the gag reflex.  Or it could be the tumor, finally grown large enough to kill her.

I don’t know.  But the last time Kat and I were cleaning her off by the side of a Pennsylvania highway, she had four days to live.

• Afraid was published on Wednesday, May 14th, 2014.
• It was assigned to the Cancer and Rebecca categories.
• There have been eleven replies.

We lay snuggled together in her bed, the stories read and books put away, the lights turned low, listening to her nighttime music.

“Rebecca, can I tell you a secret?”

“Sure.”

“I love you super a lot.”

“I already knew you were going to say that, Daddy.”

“Oh, so it’s not a secret?”

“Nope.”

“Well then, can I tell you something that’s NOT a secret?”

“Sure.”

“I love you super a lot.”

(exasperated sigh)

I answered with a small chuckle, which earned me an affectionate glare.  Five minutes later, she was asleep, her breath quiet and even and calm and as normal as it had ever been, in the years before the tumors and the months after.

---

We were at the local playground, Rebecca and her sister Carolyn and brother Joshua and some friends and me.  At no apparent prompting, Rebecca came running toward me, then slowed to a walk and beckoned me to lean down closer to her.

“Daddy, I need to tell you something.”

“What?”

“I love you.”

“I love you too.”

“I love you more! I love you to infinity… and BEYOND! No really, I do.”

And she was already headed back to the slides, smiling at me over her shoulder, her eyes squinted in a knowing amusement, before she turned away and rejoined the other kids at play.

---

We lay snuggled together in her bed, the lights turned low, listening to her nighttime music and admiring the rainbow arc projected onto the ceiling by a bedside light.  It fell across the ceiling fan and some of the get-well stars strung in a line from one side of the room to the other.

“Daddy, did someone put a rainbow on that star?”

“No, it’s from the rainbow light, sweetie.”

“Oh.”

“What do you think when you look at your stars?”

“I think that my friends are always cheering for me.  Always.”

“You bet they are.  Always.”

“I love you, Daddy.”

“I love you too.”

“I will love you forever.”

“I will love you forever and ever.”

“Daddy, why are you copying me?”

“Well, because your words are beautiful, and they’re all true.”

“Oh.  Okay.”

“I love you to the moon and the stars and back again.  I love you to infinity and beyond.”

“Okay, okay, Daddy, we get it already.”

“Good.”

She smiled at me like we shared a gentle secret, as normal as ever.  I kissed her forehead, and within two minutes, she had fallen asleep.  I listened to her slow, steady breathing, my arm still curled around her — not to protect her, but just to hold her close — and looked up at the stars and watched the rainbow until its timer reached zero and it faded away.

• Three Interludes was published on Monday, May 5th, 2014.
• It was assigned to the Parenting and Rebecca categories.
• There have been seven replies.

This morning, I walked Rebecca and her best friend to kindergarten, all of us enjoying the crisp spring sunshine after the long, cold winter.  The girls ran ahead of me to see if the playground had been re-flooded by last night’s rains (it hadn’t) and then balance-walked a low retaining wall.  Once inside the school doors, I hugged and kissed Rebecca and told her to have a good day, collecting a hug and kiss and a “Love you, Daddy“ in return.  I watched as she tromped down the hallway in her sparkly new Bella Ballerina shoes and pajamas (today is a special Pajama Day at school) and rounded the corner out of sight.  And then I handed her principal a Do Not Resuscitate order.

She’s still so alive, so very vital, but we know that could change at any moment.  We’ve lived through it once already, last August, when she went from playing on the beach to the literal brink of death in just three days.

We carry DNR cards with us, and have given the school a DNR form sealed into a manila envelope with our names and phone numbers written on the outside, because if she suddenly seizes, our overriding goal is to make her as comfortable as possible while she dies.  The EMTs or hospice or we ourselves will give her medication to take away the pain and, if at all possible, the fear.  As much as she needs.

Because we know what will happen if the tumor induces seizure and she’s forced back to life.  We know that once it’s reached that stage, there are mere hours left, even if we permit life-prolonging measures.  Heroic measures, they’re called.  Hours, possibly days, spent in misery and pain and fear.

We can’t do that to her.  If there were a reasonable chance of her suffering leading to a cure, yes.  We did that last August, submitting her to multiple surgeries and the difficult recovery afterward, because there was reason to think that doing so would save her life.

Now we know better.  We know that when the cancer overwhelms her, there is nothing that can stop it.  We know that the best we can do is make what’s left of her life as normal and happy and full of love as possible, and minimize any horrors as it ends.

We’ve thought about pulling her from school entirely.  That would ensure that if she does have a sudden seizure, she’ll do so with one of us.  By sending her to school, we risk it happening when she’s not with us, and inflicting that experience on her schoolmates instead.

We send her to school because she loves it there, however much she may complain about having to get up in the morning and get dressed and put on a coat to walk to school.  Try as she may to hide it, she loves to learn.  She loves her teacher, her classmates, and her friends, and they love her in return.  It would be selfish of us to take that away, despite the risks, despite the hours of separation.  It would shift some of our burden onto her shoulders, force her to pay the cost of our sorrow and fear.

There are so few things we can do for her now, so very few things, but we can do this: we can give her her life, as whole and unbroken as we can manage, and an unspoken promise to fiercely guard it from even ourselves.  We can give her this.  Our last gift.

• Heroic Measures was published on Thursday, May 1st, 2014.
• It was assigned to the Cancer, Parenting, and Rebecca categories.
• There have been fourteen replies.

Very early this morning, we hit the road for Pittsburgh.  We plan to be back home around lunchtime.  We plan to do this three times a week for the next four weeks.  Twelve days of two and a half hours in the car, an hour or more in the hospital, and then another 2.5 hours in the car, all on a slim hope of buying some time.

Rebecca has qualified for a Phase 1 clinical trial of an experimental drug that might — it might, maybe, possibly, if we’re very lucky — slow or even halt the growth of her tumors for a while.  Not shrink them nor eradicate them; this is not a cure.  It’s a new type of treatment, not chemotherapy, but genetic trickery.  Our research had led us to be very interested in this study, and thankfully we were able to secure a spot and qualify for inclusion.

The fact that it’s not chemotherapy means there should be no toxic or other negative physical side effects, which is a very high priority for us.  Furthermore, for people with Rebecca’s specific genetic mutation, this drug has shown a fair amount of promise.  In an earlier Phase 1 trial of the same drug as applied to adults, one patient’s tumors stopped growing for a period of years.  But then, another’s tumors barely stopped growing at all.  (And we don’t know which kinds of tumors responded in which ways.)

This clinical trial is the first test of the drug in children, to see if it works the same, or better, or worse, as compared to adults.  It’s being managed through the Pediatric Brain Tumor Consortium.  The closest PBTC site is the Children’s Hospital of Pittsburgh, where we were able to be sited.  So off we go.

And we keep asking ourselves: Are we doing the right thing?  Is it worth it?

It might seem like an easy choice.  What’s three mornings a week against a child’s life?  It’s 20-25 hours, is what it is, out of something like a hundred waking hours every week.  That’s almost a quarter of her waking time spent sitting in cars and hospital rooms, instead of being at school or running around playgrounds or coloring or playing with friends and siblings.

Sitting in a car isn’t 100% wasted time, of course; it’s not like she’ll be confined to a blank beige box for the trip.  She can sleep on the way there, and watch videos or play games on the way back — thank you, tablet computing industry! — or vice versa, I suppose; but it’s still a lot of time that could otherwise be invested in other, more interesting activities.  So while there may be no pharmacological side effects, there are serious side effects nonetheless.

And thanks to the MRI that was done Thursday, in order to establish a baseline for the study, we know that she is quickly running out of time.  The primary tumor is noticeably larger than it was five weeks ago, and the flare sites haven’t gone away.  How she’s managed to avoid neurological damage, nobody is quite sure.

Given how little time she has left if untreated, if this drug adds a year to her life, then yes, it’s absolutely worth it, especially if that gives us time to qualify for a study that somehow leads to tumor reduction.  But what if the drug only adds a couple of weeks?  Is it worth it to extend her remaining time by fifteen percent, if a quarter of her life is spent away from friends and home?

On the other hand, what if the drug doubles her time left to live?

On the other other hand, what if it doesn’t add any time at all?

We don’t know.  We can’t know.  We can only guess, and try to be as rational about it as we can be even though we are, essentially, blindly choosing how quickly our child will die, and what her life will be like along the way.

• A New Trial was published on Monday, April 28th, 2014.
• It was assigned to the Cancer, Parenting, and Rebecca categories.
• There have been twenty-four replies.

Our first day here at Disney World, we tried to go on Big Thunder Mountain Railroad, but it was closed “for refurbishment”, so we decided to try Splash Mountain instead.  In addition to being a ride that was right there where we were, it promised to cool us off a bit.  Going from 30ºF to 90ºF in the space of a day was a bit rough on everyone.

Carolyn and Uncle Jim sat in the front seat of the log boat, and Rebecca sat between me and Kat in the next seat back.  Things started well enough as we splooshed and bobbed around bends, but fairly near the beginning of the ride, you get a glimpse of its (sort-of) end: a fifty-foot drop down to a splash pool.  Rebecca, seeing a log full of people fly past us down the ramp, the screams of its riders trailing in its wake, wasn’t at all happy.  She switched instantly from being amused at the water occasionally splashing her parents to scared of what was going to happen.

The various animatronic displays along the way helped distract her, but she kept returning back to her fear of the big hill.  Kat and I soothed her as best we could, telling her it would be all right.  She generally accepted this, calming down until the next time she remembered the big hill that lay ahead.  She never cried, exactly, but the fear was still there, an almost physical thing at times.

“I don’t wanna go down the big hill!”

“Honey, it’s all right.  It’s going to be okay.  We’re right here.  Mommy and I will keep you safe.”

“No, I don’t wanna, it’s too scary.”

“I know, sweetie.  I know you don’t.  But there’s no need to be afraid.  It’s coming and it will happen and then it will be over.  Try to enjoy all the little shows before.”

“Daddy, please let me get off the ride.”

“I can’t, honey.  If there was a way to get off, we’d take it, but there isn’t.  The ride goes where it goes, and we’ll go with it, together.”

“I wanna get off this ride!”

“I wish we could get off, Rebecca, but we’re on the ride now and we have to take it all the way to the end.  I know you’re scared, but we’re right here with you.  We’ll keep you safe, sweetie.  You don’t have to be afraid.”

We’d arrived at the bottom of the last climb, the one that would take us to the big drop.  She looked up the ascending tunnel into the bright, bright light streaming down, and shrank back, quivering, her eyes wide with fear.  She knew the moment was close.  I leaned in next to her, keeping my voice level and light.

“I know, Rebecca.  I know.  I know you’re scared, but it’s going to be all right.  It’s going to be all right.  It’ll be all done very soon.  Mommy’s here, and I’m here, and we’re with you until it’s over.  We’ll stay with you all the way to the end, all of us together.  We’ll be right here with you.  Always.”

The log was drawn up the hill, the drop coming closer and closer, relentlessly.  My arm around her shoulders, Kat’s arm around her shoulders, both of us hunched toward her tiny, trembling body pressed against our sides; and as we came to the precipice, she shut her eyes and squeezed our hands tight.

• All the Way to the End, All of Us Together was published on Tuesday, April 8th, 2014.
• It was assigned to the Cancer, Parenting, and Rebecca categories.
• There have been twenty-four replies.

Our situation, and my posts, have been the cause of sleepless nights and fallen tears for a great many people.  In some ways I feel bad about that; it occasionally feels like I’m forcing our pain onto other people, which isn’t exactly a friendly thing to do.  But I know you’re here because you want to be here for us, and here, words are how we commune.

But why, I am occasionally asked and occasionally ask myself, am I writing about Rebecca’s cancer instead of doing other things?  There are a number of reasons.

Part of it is that I’m creating a precisely timestamped chronicle for later, the historian in me asserting itself.  This is where a lot of my tweets come from, as well: the desire to record something at the moment, so that later I’ll be able to say whether X happened before or after Y or how many days apart two events actually were.

But it’s also for Joshua, if he wants to know more about his sister and what happened to her, when he’s older; and for Carolyn, if she ever wants to revisit this time or see it from my perspective, to compare against her memories.  And perhaps for others, if I ever decide to collect these fragments into some sort of longer work.

More importantly, writing about what’s happening and how I feel about it allows me to organize my thoughts and give some structure to what’s happening.  In a situation where so much is beyond our ability to do anything at all, this is something I can shape directly.  It allows me to feel some small measure of influence.  It lets me face my fears by naming them.  It helps me get a handle on a few shards of this overwhelming thing that defies any real understanding.

And of course I’m grieving online.  I do that here so that I can put it away elsewhere, so to speak.  When I’m with the kids, I can be there for them as the father I’ve always been and hope to keep being, rather than the hollowed-out ruin I sometimes feel like.  Grieving here, through the words that come to me, makes that easier to do.  So I write and tweet.  A little bit of pressure release.

But most of all, I am sharing Rebecca with you, with anyone who will listen.  We’ve always felt it’s up to our kids to become themselves and then bring themselves to the world in their own way, to meaningfully affect it and be affected by it.  In the words of Khalil Gibran:

We’ve always meant for our children to fly free of their own accord, on the arc of their choosing, when they were ready.  Rebecca will almost certainly not have that opportunity.

So now we are her archers.  In the Web, I have a bow that can send her arrow all the way around the world.  If her flight is to be short, then let it be far, a trail of purest fire etched across every sky, more beautiful and wondrous than any comet could ever hope to be.

• On Writing was published on Friday, April 4th, 2014.
• It was assigned to the Cancer, Parenting, Rebecca, and Writing categories.
• There have been forty-six replies.