Taking a Dream Walk: Lucille in the Fashion Funds the Cure Event

What do you do when you are a sick kid, and the drug used to treat your tumor was not developed for children and has not been tested in children either?

My daughter, Lucille, is one of these kids. One of a statistic so high, it doesn’t bear writing out. Lucille has been fighting a rare tumor for almost three years, with a drug called Denosumab. For the first year, the drug made her nauseous and made her joints ache. Those side effects were well documented in the adults the drug was developed to treat.

After ten months of treatment with Denosumab, and a five-month drug holiday (a time when a child stops treatment to regain strength or prepare for a surgery or procedure), Lucille became sick with a problem that went undiagnosed for weeks. When we called the oncolocy office with concerns about Lucille’s worsening health–vomiting, extreme weight loss, muscle weakness–the oncology team told us to look elsewhere. These symptoms, they said, could not be caused by her drug therapy, especially after a five-month drug holiday.

By the time her doctors figured out what was wrong, she was so sick, she had to be hospitalized because she was in acude kidney failure, and her heart was at risk. Five days after her oncologist diagnosed the hypercalcemia, he realized the life-threatening condition was a rebound side-effect from her drug therapy on Denosumab.

Lucille’s oncologist had not been monitering her for hypercalcemia, because he did not know he should.

While there were a few case reports of hypercalcemia in children treated with Denosumab, they had not gotten much attention, and her doctor had overlooked them. Only now, years after Denosumab first emerged as a drug therapy to fight childhood tumors, is there a formal study with children who have suffered hypercalcemia as a side effect of Denosumab.

Lucille stopped being able to walk normally when she started having hypercalcemia. That’s also when she started dreaming of being a silks gymnast. I like to dream of her that way too–her body moving through the air instead of resting in her wheelchair, leaning over her walker, or limping in her braces.

Lucille gave voice to this dream last fall during the dream walk in the 2019 Fashion Funds the Cure fashion show for the National Pediatric Cancer Foundation. Then, in the time before COVID, she took the stage with other children like here–other children whose drug therapies made them sick. Other children whose drug therapies were neither developed for nor tested in children.

Like Lucille, all of these children have dreams in which they soar. They want to be dancers, actors, fashion designers, baseball players, heavy equipment operators, and musicians. Some of them were too sick to come to the stage, so their dreams were represented by someone else.

This year, amid COVID, the fashion show will be virtual, but the sick children will still have their “dream walks.” Lucille will be there, speaking her dream.

We hope the event will help raise awareness about the need for more pediatric oncology drug research. We hope Lucille’s dream walk will help another child not get as sick as she has become.

What do you do when drug therapy makes you sick in ways you never imagined? You keep dreaming. If you can, you keep walking.

Join us tonight for the National Pediatric Cancer Foundation’s Fashion Funds the Cure show tonight at 7, or later with the recorded video. Watch these children walk their dream walks.

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Living on the Emergency Line: My child and Drug-induced Hypercalcemia

In the ambulance bay, the paramedics unload Lucille and make their way into a crowded corridor, weaving the stretcher through groups of other paramedics, patients, and police officers. I stay as close to my child as I can, but we get separated, as I knew we would be, when we reach the security guard.  The paramedics want to keep going without me, but the guard assures them my COVID screening will only take a minute.

I say nothing. Lucille is in danger. I want her to get up to the floor where they will treat her, but I also want to stay with her. I look at my daughter on the stretcher. The IV fluids have brought her heart rate down. She is hooked to a heart monitor, and one of the paramedics is a nurse who has been monitoring her since we left the free-standing ER to come here. We are standing in the entrance to the emergency room of the biggest trauma center in Charlotte. Lucille is as safe as she is going to get.

I turn to the security guard and mumble “ok,” expecting him to point me toward the screening station. Instead he leads me back out into the ambulance bay, maneuvering me through the mask-wearing crowd—there is no social distancing in the ambulance bay late on a Friday night.  For some reason, the security guard stays with me. Maybe he is just kind, or maybe I look as lost as I feel, but I’m relieved to have someone stand beside me for a few minutes, showing me what to do.

Somehow the corridor is even more crowded than before. I cannot see Lucille or her stretcher, only a mass of dark uniforms and masked faces. Behind my own mask, I take a breath.

“She’s right there,” the security guard says. “Just walk on through.”  I tell him “thank you,” and bump into people as I make my way toward my daughter. Her eyes are half-open, but she does not see me.

“I’m here,” I say as the paramedics start moving again. Lucille does not answer. I follow Lucille’s stretcher through corridors and into elevators.  We reach the floor on the children’s hospital from one direction, as my husband, Chris, is entering from another. Two nurses and the resident on call are waiting for us.  Lucille rouses as the nurses and paramedics begin the complicated process of transferring her from stretcher to bed, from one set of monitors and IV poles to another.

Lucille has hypercalcemia again. Hypercalcemia is a life-threatening condition when it is moderate or severe. Tonight, Lucille has a severe case. The calcium level in her blood serum is 17, high enough to cause a heart attack. In the nearly three years since Lucille started battling a rare and aggressive tumor, this is the closest we have come to losing her.

This is Lucille’s third episode with hypercalcemia. Her second was two weeks ago. That day, Lucille’s hypercalcemia had been moderate,  a blood serum level of 13, dangerous enough to bring us to the hospital—but not by ambulance. She’d stayed in the hospital two days and gotten an intravenous infusion of Zometa, which brought her calcium back to 9, to a normal level.

Lucille’s nephrologist, Dr. McKay, a kind, careful man whose research credits include publishing a landmark paper about hypercalcemia in children, had been conservative with that first dose of Zometa. Chris and I had appreciated his caution because Zometa is a drug with frightening side-effects, including osteonecrosis—death—of a child’s jaw.

Lucille’s side-effects had been difficult but manageable.  Her jaw stayed healthy, but she spent one week with a high fever feeling so sick she could not leave her room. Three days after the side effects ended, she started getting sick again. Two days after that, a Friday, Lucille was too weak to walk down the steps.

All that morning, Lucille lay on the sofa while I exchanged questions with Dr. McKay, his nurse relaying messages between us. The nurse:  It was not likely a child could have hypercalcemia again so soon after an infusion of Zometa, and Lucille’s calcium was normal in her last set of labs. Me: But her condition was declining as I watched her. Nurse:  Dr. McKay ordered labs—she should come to the clinic to have them drawn—because we couldn’t know what is happening without seeing the calcium level.  Me: I understood. But Lucille was too sick to come to the clinic, which was located inside the main hospital complex—we would never make it past the COVID screeners. Nurse: If she was that sick, she should be evaluated for COVID.

By 1 p.m., Lucille’s eyes were sinking in her face, and she was difficult to rouse. Chris and I drove Lucille to the closest emergency room, where her calcium level was discovered to be life-threatening and where we were loaded the ambulance to come to the children’s hospital.

So now we are here, and Lucille is a child in acute kidney failure.

It is near midnight. Lucille is drowsing in the hospital bed, and the nurse is drawing another set of labs. The resident (who looked impossibly young and well-rested) is standing at the foot of the bed, explaining the plan. Tonight, Dr. McKay wants Lucille to have an infusion of a different drug, Calcitonin, to begin lowering her calcium immediately, and more IV fluids to flush out her kidneys.

I know, without being told, that Lucille has survived the most perilous part of this episode of hypercalcemia. I also know she will not be out of danger until the amount of calcium in her blood drops–a lot. I know that in the morning, Dr. McKay will come to Lucille’s hospital room, and he will order another infusion of Zometa. I know all of this without asking questions, but I ask my questions anyway—what else is there to do? The resident answers them with the measured kindness of someone who has never been as sick as the child in the hospital bed.

The heart monitor shows a normal rate, a steady rising and falling on the screen. Chris kisses me and kisses Lucille then leaves to spend the night with our two young sons at home. I wash my face, store my contacts in two specimen cups, then slide into the hospital bed with Lucille. I watch her heartbeat peek and dip on the screen and run my hands over her chest.

Lucille is fully asleep now and still wearing the graphic tee shirt she had on when we brought her to the emergency room. It reads “Work of art… in progress,” and has blotches of color splashed all over the front. Beneath it, I can feel the electrodes stuck to Lucille’s skin.

Hypercalcemia is rare in children. Sometimes, as is the case with my child, the hypercalcemia is caused by a side effect from another drug therapy.  Sometimes, the doctors do not know how long a child will be at risk, or how many times they will have to treat her before hypercalcemia stops happening. In Lucille’s case, when the hypercalcemia is a side-effect of drug therapy with Denosumab, the problem is too new and the research too limited for anyone to make a well-educated guess.  

Tomorrow, Dr. McKay will come, and Chris and I will agree to another infusion of Zometa. Lucille will be unsteady on her feet, and we will help her to the bathroom, unhooking the heart monitor leads and bumping the IV pole over the threshold each time. Lucille will spend four more days in the hospital before her calcium is low enough to go home. Eight days later, Lucille will be hospitalized with hypercalcemia again, and she will have another IV infusion of Zometa, and we will help her to the to the bathroom, and I will sleep beside her in the hospital bed.

Sometimes, it feels like our family is living on the emergency line. We take Lucille to have her blood drawn once a week.  We call Dr. McKay’s office immediately if she starts to feel nauseous or lethargic. But Lucille has spent the last three years often feeling sick or lethargic, and each infusion of Zometa can make her feel the same way. It’s hard to tell the difference between side-effect sickness and hypercalcemia sickness. The night we sailed through the city in an ambulance, with Lucille’s calcium level so high it was threatening her life, we made the right guess. But if we had figured her symptoms out a day earlier, or even called Dr. McKay, we could have kept our child a little more safe.

We know other families are living on the emergency line. For a few others, the line is hypercalcemia. For others still, the line is a seizure disorder, or a cancer, or an autoimmune disease, or a critically ill parent, or… But living on the emergency line is like the crowded ambulance bay on a Friday night—you are alone even when a crowd of people is standing before the wide emergency room doors.

The part of my child’s hypercalcemia that is hardest for me to come to terms with is this: She could have had a heart attack on the sofa while we were deciding what to do. It’s easy to say her doctor should have known, but a case like Lucille’s does not exist in medical literature. And when we did call—a day and a half after our child started getting sick again—the first thing Dr. McKay did was order labs to check her calcium level. As the parents of a kid struggling with both drug therapy side effects and hypercalcemia, we have to remind ourselves what we have known all along: We are the experts in our own child.  

So we live on the emergency line. We know we can’t treat our child like we are afraid she will collapse at the first inkling of wind. We can’t refuse to let her be alone in a room for five minutes because we’ve developed a sudden fear of childhood heart attacks. But if we think (or wonder if) something is wrong, we need to act. Immediately. On the hypercalcemia emergency line, there is no room to wait and see.

My child has hypercalcemia again. In the three years she has battled this tumor, this is the closest we have come to loosing her.

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Searching for a Diagnosis

Luille wearing another pair of braces, her AFOs. These are not made for walking but designed to stretch her muscles and help correct her foot-drop.

Getting ready to leave for school, Lucille eases her feet into low plastic braces—SMO braces–the kind that wrap around her foot and stick a couple of inches from the tops of her shoes. She pulls the Velcro tight, unzips her Billy shoes, places her braced feet inside them, and zips the shoes all the way around her braces. Then she teeters down the back steps, supporting herself on the railing. This is the new part of Lucille’s morning routine.

Lucille hasn’t been able to walk for nine months. Or at least she hasn’t been able to walk normally. When she is on her own feet, she dips and sways. She walks slowly—about one third as fast as other children her age. When the pain or the muscle weakness become too much, she sits in a wheelchair. On bad days, we carry her up and down stairs. 

The braces have helped, so has putting her Gabapentin, a drug with an intense dosing schedule that works on nerve pain.

Lucille has been sick from two years.  But the rare disease she has, a central giant cell granuloma tumor lodged in her jaw, cannot be the reason she has stopped being able to walk. So in the midst of Lucille’s battle with one rare disease, we are searching for another diagnosis.

For months, the general idea was that her drug therapy was somehow the culprit.  Side-effects from Deonsumab had landed her in the hospital ten months ago, even as she’d been on drug holiday, with hypercalcemia and malnutrition. That’s when she stopped being able to walk. At first her doctors thought her muscles would return to normal as she recovered, but they didn’t.

So we took her to an orthopedic surgeon. We took her to a nephrologist. We took her to a neurologist. We took her to another orthopedic surgeon. We took her for an MRI of her spine.  We took her to a geneticist. We took her to a physical medicine and rehabilitation specialist. We took her for labs and more labs.

Searching for a diagnosis is no easy thing, as anyone who has ever done it can tell you. When I read memoirs and essays written by patients—or the families of patients—trying to find a diagnosis, one theme sticks out, the solitude of it. A lot of people find themselves here, but we are always here alone. There are no support groups for patients with undiagnosed diseases. There is not one doctor who can manage a treatment plan. There isn’t a treatment plan to manage. There is a list of referrals, which lead to more referrals, a string of pleasant-enough physicians who shake their heads and send us on. 

So far, all anyone has been able to offer us is an array of increasingly frightening possibilities—and the expensive tests and work-ups to go with them. The latest is Muscular Dystrophy. We’ve been living in the possibility of this new diagnosis since November.  My husband, Chris, and I keep stumbling over the statistics. How could Lucille have another disease almost as rare as the first? Or maybe lightning is striking twice, and it’s striking our daughter.

Luille holding her own genetics lab kit.

Yesterday, her genetics panel came back normal for every known genetic mutation that causes muscular dystrophy or myopathy. Great news, but the normal results don’t rule out muscular dystrophy or another degenerative neuro-muscular disease. There are still plenty of dystrophies and myopathies that have unknown mutations, and Lucille’s history of hypercalcemia and malnutrition complicate the picture.

Each negative or normal test result seems only to lead to other possibilities. There must be a reason Lucille cannot walk—all the doctors agree about that. So each time we get good news from a test result, we exhale, only to draw another breath and start holding it again.

But searching for a diagnosis isn’t just lonely, it’s also uncomfortable, frustrating, and demoralizing.  Friends ask “How is Lucille doing?” But the answers are often too complicated to give and sometimes lead to crying. When we do get good news, we want to say so, but the good news is only ever part of the situation. The possibility of another difficult diagnosis is still hanging around—like a ghost that is always, always staring back at you, when you know there shouldn’t be anyone else in the room.

Now, we are waiting for an appointment in Chapel Hill, a city three hours away that has pediatric neuromuscular neurologist who is willing to do an EMG (a nerve conduction test involving needles and electric shocks) under sedation.

When we took Lucille for her first biopsy over two years ago, we promised her we’d go with her anywhere the tumor took her disease took her.  So as Lucille continues the treatment for her tumor, we are navigating her through the search for a diagnosis. Who knows how long we will travel, or what we will find when we arrive somewhere?  All we can say for certain is that we will continue to go with Lucille wherever this leads.

CT Scans, X-rays, and MRIs : My Child in a Series of Medical Images

I’ve always turned away from disturbing images, in movies, in newspaper photos, online.  Even during the season of Halloween, when haunted houses and yard displays tend more and more toward the violent or macabre, our family sticks with a home-made ghost fashioned from old gauzy curtains and cheese cloth. With the tumor lodged in Lucille’s jaw, though, our family has entered a new era of frightening scenes.

1 & 2: CT, January 2018

Lucille was a few weeks shy of her seventh birthday when she began the process of diagnosing the central giant cell granuloma in her jaw. The first scan, taken in an oral surgeon’s office, showed a bony tumor in her mandible, interrupting what had been the pretty curve of her chin. Her second CT, one day later in a different oral surgeon’s office, confirmed the first. I didn’t like looking at the images from those scans. Bulbous and bulging, the tumor looked like the not-right thing it was. That was back before we understood how much a benign tumor could change a child’s life. When I looked at the images, I looked away quickly and thought, not cancer, not cancer.

3: CT, April 2018

It wasn’t cancer. The tumor was a central giant cell granuloma. Benign and rare, these tumors are often managed with treatment plans that don’t disrupt a child’s entire life. After a two-month course of routine treatment, during which Lucille’s surgeon sedated her and then injected a high-dose steroid directly into the tumor, Lucille had another CT scan. Those images showed a tumor that had grown half a centimeter. In oncology terms, the treatment had failed.

While the surgeon started explaining the bad news, I stared at the screen, trying to come up with words to describe this tumor that had taken over my child’s jaw. The tumor looked not-real. The tumor looked like a mistake. The tumor looked like something that could not fit inside Lucille’s face.

But the surgeon was talking, and I suddenly heard the words “oncology,” and “chemotherapy.” That’s when I wanted to close my eyes.

But this was my child’s jaw bone and my child’s tumor lighting up the screen. The world of pediatric oncology is like that. You have to look.

4: CT, June 2018

By now Lucille was two months into life as a pediatric oncology patient, and her oncologist was the one ordering scans. This CT scan would not happen in one of the friendly little stand-up machines we’d encountered in the offices of oral surgeons. This CT was the kind involving a hospital visit, an IV, and contrast dye. Lucille had to slide into this CT lying down.

She was afraid. This first hospital CT was one of those medical circumstances when you tell your child, “I wish they could do this to me instead of you.” A statement that helps no one. In the CT room, Chris and I stood, cloaked in full lead vests, watching Lucille slide headfirst into the tunnel, wearing only a hospital gown and blanket.

“We’re right here,” we kept telling her. “You’ll be okay.” That’s another thing about pediatric oncology: “okay,” becomes such a relative term.

But the purpose of that CT was to make sure her drug therapy, Denosumab, was working. And it was.

5, 6, & 7: CT, October 2018- January 2019

After eight months of drug therapy, Lucille was back in the surgeon’s office, where a new CT showed a thin line of bone reclaiming the tumor. Lucille, her jaw, and her tumor were ready for surgery. Or would be after a three-month drug holiday.

After her “holiday,” Lucille’s pre-op CT showed a stable tumor. In the operating room: nothing but success. The radical surgery broke medical ground and reshaped Lucille’s face. The post-op CT was the most beautiful bit of radiology I’d ever seen. Lucille’s jaw looked right again.

8: CT, April 2019

Sometimes tumors come back. Lucille’s central giant cell granuloma recurred three months after her radical surgery. Because Lucille was in the room when her surgeon showed us the scan, none of us said the f-word. But on the way home, I told Lucille she could shout any swear word she wanted to, as long as she stopped before we got out of the car. Another pediatric oncology observation: It’s hard to know when you are making good parenting decisions.

Lucille’s surgeon resected the tumor in another surgery, three days later. But neither He nor the oncology team thought the surgery would be enough. As soon as her jaw had safely recovered from this surgery, Lucille needed to go back on drug therapy.

8-18, 19-?: X-ray, May 2019

As any parent of a critically ill child knows, at some point, you lose count. My point came when Lucille lost enough weight to become malnourished and started limping. During a frightening office visit, her oncologist ordered labs and an x-ray study of her left hand, both of her feet, and both of her knees. After the phlebotomist drew labs on Lucille, who had become too sick to care much about the needle, we took the elevator four floors down to outpatient pediatric radiology. Ten images. I stood on the other side of a door and watched the x-ray sign blink off and on.

What’s ten more x-rays, I thought. Maybe they’ll figure something out.

Lucille’s oncologist called before we made it home. Her labs were back, and she needed to go to the hospital. Lucille had a dangerous condition called hypercalcemia, a side effect of her drug therapy. By the time she made it into the hospital room, Lucille was in acute kidney failure and could barely walk. The doctors on call ordered IV fluids for Lucille’s kidneys a full skeletal x-ray study, for reasons no one wanted to elaborate.

By now my husband, Chris, was with us, and only one parent could go into the radiology room. So Chris donned the leaden vest, and I sat on the other side of the wall, while a technician x-rayed each bone in Lucille’s body.

They were looking for evidence of other tumors. They were looking for evidence that her growth plates were freezing up. On the other side of the wall, I sat with these two possibilities and thought, let it be neither. Let it be nothing.

The results: Lucille’s bones looked fine. So did her growth plates.

Something else was happening. But the happening could not be named. Which is one more observation about pediatric oncology: There will come a time when something is wrong, but no one knows what it is.

?-?: X-ray, June 2019

There will also come a time when you can’t remember the order in which things happened to your sick child. According to my iPhone, Lucille went back for another set of x-rays one month after she was discharged. I would have confused these x-rays with those she had before going into the hospital, were it not for the iPhone’s impeccable memory.

That day, I insisted on walking through the door. I sat behind a screen, trying to fold my body enough to protect it, and asked for as much lead as they could drape over Lucille, which wasn’t enough. Even as she recovered from the hypercalcemia and malnutrition, she Lucille was walking less and less, and we spent most evenings carrying her around the house. But those x-rays looked like the others. Nothing, nothing, nothing.

 ?: CT, August 2019

Four months into a second regime of Denosumab drug therapy to treat the tumor recurrence and nineteen months into her tumor battle: a check up with her surgeon. The image of her mandible confirmed the drug therapy was working, again. But Chris had to carry Lucille into the surgeon’s office because sometimes she could not walk at all.

Two weeks later, her oncologist handed me a study noting briefly that some patients on long-term denosumab developed a neurological condition called mild peripheral neuropathy.

Two weeks after that, Lucille went to a neurologist for the first time. There, we learned of a new concern: the possibility of lesions on Lucille’s spine.

?: MRI, Two Days Away

Today Lucille is twenty-two months and two surgeries into her fight with this tumor. I don’t know how many images of her body radiologists have studied. Nor do I know why she cannot walk. I can say she spends some of each day in a wheelchair, and her physical therapist has become worried about permanent damage to her feet. I can also say she has spent a total of fourteen months on Denosumab and is due for her next round in two weeks.

Lucille has acquired medical specialists like she acquires Beads of Courage. Sometimes I imagine them clinking together on a string—the oncologists, the radiologists, the nephrologist, the oral surgeon, the orthopedic surgeon, the neurologist, the physical therapist… clink, clink, clink. But no answers resonate with that sound. 

Today is Wednesday. On Friday, Lucille will have an MRI of her spine. The radiologist and the neurologist will be looking for lesions or anything else that might explain whatever is happening to Lucille’s legs and feet. On Friday, Chris and I will ask to enter the MRI room with Lucille. When she slides into the chamber, we will wait without speaking. We will wish the MRI, the trouble walking, the drug therapy, and the tumor were happening to our bodies, instead of hers. We will long to reach into the chamber and place our hands on hers. Instead, we will hope for answers without knowing which to hope for.

Being Different from Everyone Else: Living Every Day with a Childhood Tumor

Lucille writes encouraging notes to herself. I found this one tacked to her bulletin board.

This morning Lucille went to school twenty minutes late and limping. After talking her through the nausea, feeding her a toaster waffle along with some homeopathic tablets that sometimes help, and asking her to down her other meds with a cup of water, I watched Lucille support herself on the railing to make it down our back steps.  

Watching her struggle out the door to go to school, I had the same thought I do every time I let myself question what is happening to my daughter.  How did we get here?

Lucille is not late for school every day, but she does spend every day limping, and some days, she rides the school wheel chair to cross long distances.  To get from the third-grade hallway in the back of the school to, say, the art room in a trailer behind the school is more than she can pull off most days.

The wheel chair is new this year, but being fundamentally different from everyone else is not. Battling a childhood tumor is like that. The child with the tumor and the family she belongs to become somehow other from everyone else.

Here is an example: Right before the end of the school year in June, a friend called to tell me what a great time she had volunteering for a PTA event. That’s a pretty reasonable thing to say to a friend, but I hung up the phone and cried. Lucille had just gotten out of the hospital, after a frightening 5-day stay brought on by side-effects from her drug therapy. I’d been focused on making sure Lucille drank enough fluids, ate enough calories, and took enough footsteps each day.  A PTA event? Volunteering? I could not make sense of it.

But sometimes, I forget how different we are.  A childhood tumor is part of the ebb and flow our lives now. But we are still a family of five with all the attendant housekeeping, school attending, and activity managing.  Lucille is still the older sister to her two little brothers. She is still our smart, sassy girl. And yet. And yet…

I can’t speak for other families with kids battling childhood tumors, but I see them enough to be pretty sure it is like this for most of us. Your child gets a diagnosis, you enter the unsettling world of pediatric oncology.  You manage medications and nausea and appointments. Your family gets a new normal, but the rest of your life doesn’t stop. You know how different you and your child are, but most of the time, you don’t think about it in those terms.

Then, in the midst of a busy morning trying to get everyone off to work and school, these moments of startle. Seeing your child struggle through the tumor battle.  Seeing the way it has changed your family, almost is if looking in from the window. Look: what they are doing? There is vomit on the floor. Prescription bottles falling from the cabinet. The toddler has climbed up the table again. The kindergartener is trying to find his left shoe. It’s time to leave for school. Where are the parents? Oh, they are in the turquoise bedroom with the sick one.

First, how did we get here? Then, here we are. In this place of being different. “How are you?,” our kind friends ask. I don’t know how we are. Only that we are here.

It has been two years since Lucille’s face started to change as the tumor grew and almost twenty months since her diagnosis.  I don’t know how long it has been since we realized we were different.

I can say I wrote my first post about Lucille’s changed life one year ago.  Mutha Magazine is running that post as the first in a serial of adapted posts from The Face of Bravery.  The post as it appears on Mutha is expanded from the post that originally appeared here.  I hope you will read it on Mutha.  While you are there, take a look at some of the other real-life parenting stories written by moms, mothers and muthas!  Some of them are about other families who, for whatever reason, find themselves in a place few others seem to be.

When the Doctor has Bad News (Again)

I was holding my daughter’s hand—again—reaching down to her as she sat in the exam chair in the surgeon’s office—again. Chris, my husband, was there too, as he always is.  And—again—the surgeon was not coming right back. Chris and I looked at each other.  What we did not need to say out loud was that we already knew the surgeon would have bad news. 

Each time the surgeon does not come right back, the news is bad. It had been so for a year and a half, and now, we told each other with our eyes, we knew what to expect. So we took turns holding Lucille’s hand, and we waited.

We know we aren’t the only family to sit in an exam room waiting for more bad news—though that isn’t what I was thinking while were sitting there. The truth is that even while were waiting, countless other families were sitting in their own limbo, waiting for doctors who shouldn’t have been taking so long.

I can’t say exactly what it’s like for everyone, but here is what it’s like for us.

Something Feels Wrong

Lucille was three months past her radical surgery, six months out of drug therapy, and two months into getting back to normal. Everything should have been fine.  But it wasn’t.  She started feeling pain in her jaw.  The pain was intense enough to need Motrin and ice packs, and it was in her mandible—her lower jaw bone—right where the tumor had grown.

We let it go for a few days, telling our daughter and ourselves that everything should be fine.  When we called her surgeon, he advised us to watch her for a few more days.  She had been sick; maybe her jaw was sore from throwing up, or from crushed nerves healing enough to feel again, or from adult teeth coming in.  After all, the pathology that came back after the surgery was perfect—no tumor cells anywhere. But after a few more days, he asked to see her—just to be on the safe side.

So Lucille had a CT scan as soon as we walked into the office. It was April again, and now we were waiting for the surgeon to look at a CT scan, just like we had one year ago, when the surgeon had come in and apologized for keeping us waiting before telling us the tumor had grown, and Lucille would need to start treatment with an oncologist.

Now, as the surgeon finally came back into the room, he said, “Sorry to keep you waiting.  I wanted to take my time looking at the CT.”

So he had bad news for us again.

I wanted to drop the f-bomb but didn’t, not with Lucille sitting in the chair.  In a minute, Lucille’s scan illuminated the screen.  We could see something there, in her jaw, in the middle of the newly-grown bone.

The surgeon went over the options: It could be nothing, just bone growing more densely than the bone around it. It could be bone infection. It could be tumor.  She needed a biopsy. And she needed it soon.

I’ve already written a post about the biopsy waiting game. Each day is an eternity. Other parents have been there.  First, you wait for the day of the biopsy, then you wait through the procedure, then you wait for the results. You Google it just to make sure you aren’t the only family who has waited this long for a biopsy. You recite the possibilities like prayers—dense bone, bone infection, tumor recurrence.

The Morning of the Biopsy

We pulled Lucille out of bed when the dawn light was still grey. She knew this routine; we all did. She brushed her teeth, and we left the house with Lucille still in her pajamas—pink plaid bottoms this time, paired with the pink 1984 Detroit Tigers tee shirt I got when I was her age.

Like her first biopsy fifteen months ago, this one was happening in the small operating room inside the surgeon’s office. Unlike the first one, we knew her surgeon well now, so he let us stay while he slipped the needle into Lucille’s hand to sedate her. Chris and I stood beside our daughter, holding her hands until her pupils dilated, and the surgeon told us what we already know. She was out, and we needed to leave the room.

I’d have stay if he would have let me. I’d always rather see what is happening to her. But parents aren’t allowed in operating rooms.

In the waiting room, we think things are taking too long. Each time the surgeon does not come right back…

“He must be doing more than the biopsy,” I said.

“I know,” Chris answered.  The surgeon had warned us that if he got inside her bone and saw what was clearly either tumor or infection, he’d widen the incision and remove all he could.

Chris and I have gotten into the habit of not touching each other in waiting rooms.  Having a critically ill child is hard on a marriage.  You forget you are the kind of people who like holding hands. You forget you are the kind of couple who can do anything with each other except hold your family together.

The wait continued, and we didn’t say much else.  After a time—who knew how long?—we looked at each other and spoke enough to agree that the news was definitely bad. And just then the surgeon’s assistant, still smocked, came to bring us to a consultation room.

When the surgeon came in, he wasn’t smiling. (After her radical surgery three months ago, his grin had been the first thing we noticed).

Now, he uttered some preemptive word, like “well.” But I beat him to the punch and said, “You had to do more than a biopsy.”

The surgeon began explaining. The bone looked great.  It killed him (this was the phrase he actually used) to put a hole in the beautiful new bone. But drill a hole he did. And he came to a hollow space.  And it was filled with what looked like mononucleated giant cells. The cells that, in Lucille’s case, make a central giant cell granuloma.

“It looks like tumor recurrence or multiple recurrences,” the surgeon said and went on to explain that it was possible the cells were infection, not tumor. Either way, the news would be bad.

Under the table, I reached for Chris’ hand. His fingers folded around mine.

The Bad News Comes

Knowing the news will be bad, either way, we wondered which we should hope for:  Bone infection or tumor. This time, the wait seemed longer than Lucille’s first two biopsies.  Though it ended up being the shortest by over a week.  Lucille was still recovering from the surgery when the news came in.

Lucille’s surgeon is a nice person, and Lucille is a special case, so the phone call lasted for nearly half an hour.  He told me she would have been more likely to lose her jaw if the cells had been a bone infection. He told me he removed the tumor during the surgery, but the surgery won’t be enough: Tumor cells were certainly still there. He assured me we caught the recurrence early. But still, Lucille would need more drug therapy.

Facing the Immediate Future

Lucille bringing her new dog home from the pound. We hope pet ownership will help her face the tumor recurrence. 

In the days to follow we will face the complex web of consequences and decisions brought on by this re-diagnosis. We will talk about oncology options and a feeding tube. We will calculate weight loss. We will hand our daughter antibiotics and probiotics as her body fights a post-surgery infection. We will adopt a dog.  And through all of this, our brave girl and her brave face will absorb the news.

 

The tumor is back. The biopsy shows central giant cell granuloma tumor cells, a recurrence. Lucille is going back to pediatric oncology. Her beautiful, brave face will continue to fight.

The Biopsy Waiting Game

First, you wait. As anyone who has become an oncology patient knows, the biopsy comes first. You have the biopsy—or your child has the biopsy—and then you spend your hours thinking not so much about what the pathologist is doing, as what the results will mean for your life.

When Lucille had her first biopsy, a year ago, we knew she had a solid tumor in her face. We were waiting to find out if the tumor was cancer or what it turned out to be, a central giant cell granuloma. I don’t remember much about what I was thinking that day. Our third child was four days old—I was exhausted and still leaking from everywhere. But I do remember wearing the baby in his sling, kissing Lucille before they sedated her, and thinking everything would be okay as long as it wasn’t cancer.

That day, everyone, even the surgeon, was still referring to Lucille’s tumor as a “mass,” and we had not realized how fundamentally a benign tumor could change our lives. We had not imagined how large the tumor would grow or how disfigured our daughter’s face would become. We did not know that some people with benign tumors still became oncology patients. We thought that if a tumor was benign, most of its effect would be benign, too.

—-

One year later, we got it. This time, as we waited for pathology results, what was on the line was the oncology: the need—or not—for more drug therapy. Lucille had spent eight months on a drug called denosumab, still experimental in its use for this kind of tumor, risky, but not chemo. If she needed more drug therapy now, chemotherapy was looming as one possibility.

This time, we weren’t waiting for a biopsy, exactly. This time, the surgeon had cut most of the bony tumor from Lucille’s chin and had sent every chunk to the pathologist. This time, everyone was hoping the drug therapy had turned the giant cell tumor tissue into bone tissue, at least around the parts that had been touching the tissue the surgeon had left behind to form her new jaw.

This pathology wait began with Lucille’s recovery from a radical surgery. Helping Lucille through the beginning of her recovery buoyed us through the first week of waiting. This time, I did think about what the pathologist was doing. So did Lucille’s surgeon, who told us at her five day follow-up and her eight day follow-up that it takes a long time to slice up so much bony tumor into readable sections.

So Lucille continued a difficult recovery, and we waited. And we waited. Another thing anyone who has waited for a biopsy—or any kind of pathology report—knows is that the rest of your life keeps going. The rest of your life doesn’t care what the pathologist is doing. The kids—including the one recovering from a radical surgery—still need to be fed. The important emails from work still need your response.  The fight you are having with your health insurance company still needs to be fought.

So we waited. My husband, Chris, and I went back to work. I won one skirmish with the insurance company. Lucille progressed from a liquid diet to soft foods. We prayed, and we hoped.  We tried not to get irritable with each other. The surgeon emailed the pathologist to check in. The surgeon promised he would call me as soon as he got word. I carried my phone to class and apologized to my students when I had to look to see who was calling.

Lucille read and colored, took a lot of selfies, and wrote a story on her Chromebook. Nicolas went to preschool. Wade teethed a lot. The kids and I went for walks and played a lot of snorffle-tickle-monster. Friends and strangers sent cards and meals and gifts and flowers. We read and ate and opened and appreciated. And we waited.

And then, like most of the other times in my life when I’ve been waiting for news, the news came at the exact moment I’d forgotten to think of it. I’d just gotten back from class, where I had forgotten to bring my phone. I sat down in my office, and heard my phone vibrating from inside my desk. It was Lucille’s surgeon.

He had just that instant gotten off the phone with the pathologist. I could tell from the sound of his voice that the news was good. I did not get up to close my office door. I sat and listened. The news was better than good. There were no giant cells anywhere, not anywhere at all. Not in the border tissue, not in the tissue deeper into the tumor. Not even in the small, snowman-shaped declivity everyone thought would probably still hold some tumor cells inside.

“Do we need a second pair of eyes?” I asked, wondering if another pathologist should give a second opinion. Funny how sometimes good news can be just as unbelievable as bad.

But an entire pathology team had studied her tumor and with special interest. (She was, after all, a very special case.) No, we did not need to get another opinion. We did not need to make another difficult drug therapy choice. We did not need to do anything but stop waiting.

Parenting a Child Recovering from Surgery

Today we ate breakfast with Lucille at La Peep, the same restaurant we’d taken her to nine months ago. On that morning, her surgeon had taken a CT scan and given us the disturbing news that her tumor, a central giant cell granuloma, had grown half a centimeter, despite eight weeks of intra-tumor injections of a high-dose steroid. Our next stop would be pediatric oncology, but first, we would eat pancakes. That morning, we did not realize what lay ahead for Lucille. (“It’s a benign tumor,” we’d whispered to each other more than once. “How bad can this really get?”)

But now, nine months later, we have just finished Lucille’s first post-op appointment with her surgeon, and we are sitting in another booth at La Peep. We are still parenting a child with a rare disease, but now she is recovering from a radical and successful surgery. So much of her battle against this tumor is suddenly, miraculously, behind us.

“Pancakes?” we ask. Today we are working on eating solid foods and drinking with a straw instead of a syringe.

“Yes,” Lucille says. She wants the funny face pancakes with fruit and whipped cream, which is what she ordered nine months ago.

She wants a chocolate shake to drink. We are still in the get-as-many-calories-into-our-kid-as-possible phase of Lucille’s recovery. Two days ago, we were feeding her Pediasure, with a syringe, milliliter by milliliter. Sugar and carbohydrates for breakfast? No problem, as long as she can eat them herself.

Lucille talks to us from behind a hospital mask as we wait for our coffees and her milkshake. It’s hard to understand what she is saying. Her bottom lip is still swollen and numb.  She is wearing the mask because she is embarrassed by the way her face looks. “Swollen” is the only word for it, but it doesn’t really articulate what is happening here.

The day we left the hospital, her bottom lip looked like a balloon, the long kind balloon artists twist into flowers or unicorns. The rest of her face was just as big. The swelling goes down a little every day, but not enough to make her feel normal, even by her pre-surgery, disfigured-face standards.

Then there is the drooling, which happens because she can neither close her lips nor feel them. And, until today, blood was still leaking from the incision inside her mouth and falling down onto her swollen chin. And the sutures, which are also iniside her mouth, are still clearly visible with all the open-lipped swelling.

We keep telling Lucille how beautiful she is and how swelling after a surgery is normal. But this doesn’t feel normal to Lucille. Nothing about a rare disease is “normal.” Neither, really, was the surgery itself.

But Lucille is a special girl in more ways than her disease. She’s smart too, and she came up with the idea of covering her mouth with a hospital mask, so she can feel normal when we go out in public, or when visitors come to our house. We complemented her on solving a problem for herself, and went to a drug store to buy a box of masks.

The rest of her recovery is beginning to feel normal, too, or at least we’ve gotten into a routine. At night, we prop her up on pillows and tuck a towel under her chin to catch the blood. One of us sleeps beside her, just in case. We give her the meds. During the day, we make sure she is hydrated and nourished. We keep her masks and her syringes and her meds on a Winnie the Pooh tray. We squirt water into her mouth. We teach her to use the syringes herself. We encourage her to try a straw, to try a sip, to try taking a bite.

I rub coconut oil on across her lips to keep them from cracking. I rub it onto her chin to keep the skin from peeling. When food and chocolate milk and Pediasure and blood collect above the soft stretch of tissue and incision between her teeth and her lip, we flush her mouth with water and mint-flavored chlorhexidine. We clean gently with oral swabs. We help her brush her teeth.

Under all the swelling is a chin shaped like a normal one. And above her mask, Lucille’s eyes spend time smiling. Parenting a child recovering from a radical surgery is exhausting. And surprising. Ditto for parenting a child with a rare disease. But when is parenting not these things?

So we’ll take it. And like we have every day for the last 380 days, we will celebrate the gains, even the tiny ones. We will blend this part of life with all the rest of it.

There may still be giant cells—tumor cells—to deal with. There is the high recurrence rate—over 20 percent—to be reckoned with. There may be other surgeries to finish reshaping the bone. There will be orthodontia not—as her surgeon puts it—for the faint of heart. There is still extra tissue growing on her chin and inside her lip. But for now, she is recovering. And we will take it day by day.

The Only One/ The Lucky One

The 3-D printed model of Lucille’s tumor, a central giant cell granuloma

Yesterday, Lucille became the first person with a central giant cell granuloma to have surgery after an eight-month course of a drug called denosumab. Ground-breaking things were happening in the operating room yesterday. Things that will open up new worlds of treatment for other children suffering from this rare, aggressive, and disfiguring disease.

When your daughter is the only one—the one on which the medical ground is being broken–it’s hard to keep a wide perspective. For you, the opening ground is the ground your family stands on, and it’s been shaky now for one year and nine days.

Your alarm goes off at 4:00 a.m., waking the baby before it wakes you. While you nurse the baby back to sleep, you think of your daughter, of her face, and you pray, or you meditate, or you say, “please.” Or that’s what you try to do.  Instead you keep wondering how her surgeon will even know what her face was supposed to look like. And then you wonder if the news will be good enough for him to follow his plan A, or if this will be a Plan B surgery.  Then you wonder if he is still feeling as optimistic as he was at your daughter’s last pre-op visit.  Then you realize you have spent more time in the last three days thinking about this surgeon, than you have your own husband. Then you wonder—again–how he will even know what her face was supposed to look like.

Of course, the surgeon has told you how he will know. He has looked at data sets of hundreds of jaws of girls your daughter’s age. But data sets don’t mean much to you. Words do. So you try to frame some that will get your daughter from here to the recovery room. Then you ease away from the baby and get dressed in the dark.

You forget to brush your daughter’s hair and your daughter’s teeth. You pull her coat on over her pink snowflake pajamas and sit beside her in the back seat while your husband drives to the hospital. You get lost trying to find patient registration. Your daughter seems calm—remarkably calm. Back in the car, she was even excited.  You all are, in a way.  This surgery is going to get her closer. This surgery is a definite step after all the maybes, no’s, try this’s, and if’s.

Here is today’s if. If the still-experimental drug therapy ossified the tumor into bone, or at least most of the tumor into bone, the surgeon will slice most of it off and re-shape her face.  If not, he will take out as much of the tumor as he can. She won’t look any different, but she will still be moving forward.

Walking up to the surgery floor, you feel the weight of the year your daughter has spent fighting this tumor. But it’s not the kind of weigh that holds you down. It’s the kind you push through, like swimming through water, to move.  The surgeon has been waiting too, through one treatment then another, to get enough bone to do this surgery.

In pre-op, you step into a tiny bathroom to help your daughter change into a gown the color of Caribbean water. It falls to her toes, and with its gaping sleeves and strings, it reminds you of the angel costume she wore for the Christmas pageant.

When you come out, the surgeon is standing in the tiny room, and your daughter starts crying. She cries while you smooth the numbing cream over the veins on the back of her hand. She cries as your husband reminds her why this surgery is a good thing. She cries while the anesthesia nurse starts the IV and slips in the drug to make her relax.

By now, you are lying in the bed with her. You stay there, your arms wrapped around her while your youth minister comes in to say a prayer, while the nurse anesthetist explains what will happen next, while they slip more medication into her IV. You decide you should get out of the bed before they say you have to. So when they open the curtain and the wide glass door, you kiss your daughter. You tell her you will see her as soon as she wakes up.  Then you walk, one hand on the bed rail, one hand on your daughter’s chest, until it is time for you to go one way and her to go another.

Then you wait. You wait with your husband, and your cousin who has taken off work for the day, and your brother who has flown in from Michigan, and your husband’s sister who has flown in from Colorado, and your mother-in-law and her husband, and the youth minister. You wait, and you watch a screen that lists the status of patients in surgery.

The surgeon has promised to send word when he knows which way the surgery is going. You have reminded him of this promise three times. You wait for word. When word comes, the news is confusing. “Plan 2,” the receptionist says.  You watch your husband’s face crumble.

“No,” you say. “Plan 2 was not an option. There was only Plan A or Plan B. Call the OR back, and tell us if he is doing Plan A or Plan B.”

You wait again. The word comes back again: Plan A.

You are sitting opposite your husband in a row of chairs. You look at each other, but you do not hug. Neither of you can risk the other’s touch now. But soon all of the people who have come to sit with you are laughing. And you and your husband are laughing too.

The surgery goes on for almost three more hours. The other names on the screen change status. Your daughter says at “surgery in progress.” You drink tea gone cold, and go use your breast pump, and talk to the people surrounding you. Then, miraculously, the surgeon is standing in the waiting room, smiling.

In a small conference room, he tells you the surgery went better than he hoped. He was able to remove enough to give her a normal jaw. He saved the nerves in her chin, put tissue back where it was supposed to go, reconnected the ligaments, put the crease back into her chin. He tells you how much bony tumor he removed from her mandible. The carved-out pieces measured 5 centimeter, 6 centimeters, 7 centimeters. He pulls out his phone to show you a picture from the OR, after he closed the incision.

Your daughter’s face looks like it did before the tumor.

Later, in the recovery room, you climb into bed with her again. Rolled gauze is sticking out of her mouth like two tusks. Blood is dried on her face and on the pressure dressing wrapped around her head. You lay your face against hers, you stare at her chin.  You know there may still be tumor cells to deal with. You know the surgeon is sending every piece he cut from her mandible out to pathology.  You know the results are two weeks away.

But for now, you lay beside her, feeling the warmth of her face on yours, your heart filling with gratitude. You remember your daughter is the only one to have this treatment plan. You remember the other kids you’ve seen in the pediatric oncology clinic. You know your daughter is a lucky one.

 

30 Things You Should Know About Lucille

1. She is seven.
2. She loves to dance.
3. At Christmas, she danced as a Gumdrop in the Nutcracker.
4. She listens Johnny Cash, especially Ring of Fire and Folsom Prison Blues.
5. She has a rare disease. A central giant cell granuloma has taken over her mandible.
6. She has two little brothers, five and one.
7. Her wavy, dirty-blond hair curls into ringlets when it gets humid.
8. Now, she has a disfigured face, but her brown eyes still look like mine.
9. She has my husband’s lips.
10. Tomorrow, she is having a radical surgery.
11. Before the tumor, she had my mother’s chin.
12. Her favorite meal is spanakopita.
13. She delights in chocolate–in all its forms.
14. She believes in Heaven but isn’t sure it sounds very fun.
15. To make this surgery a viable possibility, she spent eight months on a drug called deonsumab.
16. In second grade, her favorite specials are P.E., science, and art.
17. She does not like learning Spanish.
18. She reads two and a half years ahead of her grade, but she likes to read for pleasure, not school.
19. At the pediatric oncology clinic, the child life specialist gives her Beads of Courage. Her strand is growing long.
20. A Poopsie Surprise Unicorn and a Chrome Book were her favorite Christmas presents.
21. Her favorite restaurants are Chick-fil-A and Yafo.
22. She likes staying in hotels, especially the ones that serve breakfast.
23. She’s not bad with a hula hoop.
24. She makes beaded jewelry, and her favorite colors are hot pink, purple, and turquoise.
25. She has become one of the bravest people I know.
26. Most days, if you saw her when she wasn’t nauseous and didn’t look at her face, you’d think she was a normal kid.
27. She wishes people would stop pointing.
28. Her surgeon has been slicing up 3-D printed models of her jaw: He has a Plan A and a Plan B.
29. We are all hoping for Plan A: complete removal of the tumor and reshaping of her jaw.
30. When she wakes up tomorrow afternoon, she may have a different face.