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.
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.