Myofibroblastic Tumor: Family Battles A Terrible Diagnosis Of An 11-Year-Old

You've had a bad feeling all summer, a nagging in your gut that something's wrong. She looks thinner, but she just turned 11 and kids that age get taller, thin out. Yet . . . why is she so pale in July? Why is she tired all the time? Your husband said it was because she'd been staying up too late on her iPad, so you limited her screen time. That didn't help. She keeps falling asleep smack in the middle of bright summer days. You notice she isn't enjoying her summer. She's irritable, picking frequent fights with her younger sister. "It's just hormones," you tell yourself. "Eleven is a difficult age."

You take her to the doctor for a rash and fever, and you learn that she has strep. You're relieved. Maybe that's what it was all along. The doctor looks at your daughter's torso - she looks right at the tiny bulge in her abdomen - but she sees only the rash. "It's scarlet fever," the doctor says. She prescribes an antibiotic and sends you home.

A month later, your daughter returns from a five-day trip with your mother-in-law, who insists she was fine on the trip. "I gave her tea to help her stomach," your mother-in-law says.

That night, your daughter admits that she was in a lot of pain during the entire train ride home. "I'll take you to the emergency clinic if it still hurts in the morning," you tell her. You tuck her in, kiss her forehead and go to bed with a terrible feeling of foreboding.

Maybe, on some level, you suspect this is your last normal night.

The stomach pain doesn't go away. By morning, she's walking hunched over, favoring her right side. You Google "appendicitis" and take her to the emergency clinic, concerned her appendix might burst. The doctor looks at her distended stomach, gently touches it and immediately sends you to the emergency room at your local hospital.

You call your husband and tell him to get backup for your younger daughter, who is 8.

"What do they do for appendicitis?" your daughter asks. Her eyes are wide with fear.

"They take your appendix out," you respond. "It's a really common procedure. You'll be okay." Based on your current level of worry, this feels like a lie.

You get to the hospital, and they take her in quickly.

She gets her first IV. She hates needles, but she's been in pain for days and she's desperate for relief, so she submits. You watch as big tears spill down her cheeks. They wheel in an ultrasound machine and slather goo on her stomach. She giggles. You giggle with her.

They can't get a fix on her appendix.

"Her liver is enlarged and blocking her appendix," the attending emergency physician explains. "She'll need a CT scan."

You Google "enlarged liver in a child" and learn that this can be caused by Epstein-Barr, the virus behind mononucleosis. You briefly relax. This would explain the fatigue and some of her other symptoms. This is something you can grasp. You smile and squeeze her hand. "You're so brave," you say. She smiles back.

Four hours pass from the time she gets the CT scan to the time the results come in. They are planning to transfer her to a hospital with a pediatric unit that is two hours away. You go home to pack a bag for both of you.

The ER attending is waiting when you get back to the hospital. White-faced, he pulls you and your husband out of your daughter's room. Your feeling of dread returns.

"She has an enormous tumor in her abdomen," he says, voice shaking. He avoids making eye contact. "It's obscuring all of her organs. It's at least the size of a cantaloupe."

You feel hot and cold at once. "Is it cancer?" you blurt as your husband begins to cry.

"Yes. It's likely malignant."

Your world tilts sideways.

Your daughter is loaded into an ambulance and given morphine to ease her pain. It was a bright August morning when you brought her to the emergency clinic and now it is dark, after 10 p.m. You ride in the front of the ambulance, making phone calls and texting family members about what's going on.

It's past midnight when you get to the new hospital. They take you into a quiet part of the ER, and a pediatric oncologist soon joins you. She orders labs and explains that they're getting a room ready. A technician draws blood from the existing IV. The results come back in less than 30 minutes: She's profoundly anemic. They give her a blood transfusion right then and there.

"It hurts my arm," your daughter says, wincing.

"I'm sorry, sweetie," you say. You watch the foreign blood snake its way into your daughter's IV. That night, as you lie beside your daughter's hospital bed listening to the beep of her monitors, you wish with all your heart that you could change places with her.

You are bombarded with a litany of physicians within the first few days: two additional oncologists, a general surgeon, a liver surgeon, a hepatologist and a pediatric resident.

It takes nearly three weeks, two biopsies and pathology reports from three different hospitals to get a proper diagnosis. Your daughter has something called inflammatory myofibroblastic tumor. The oncologist has no experience with this rare type of tumor, which usually occurs in children and young adults.

The liver surgeon is urging an immediate liver transplant, but the oncologist wants to try shrinking the tumor with chemotherapy. No one can give you her prognosis.

Days pass and your daughter grows depressed. She continues to lose weight. Each time she steps on the scale, she watches your face. If her weight goes down, she says she's sorry. She says it over and over again. Each apology breaks your heart. Finally, they give her prednisone, and she regains her appetite. After 30 days in the hospital, the doctors still can't decide how to treat her.

Your daughter wants to have the transplant.

"Why?" you ask her.

"I don't want to lose my hair."

An oncologist at one of the top cancer centers in the world weighs in on your daughter's treatment. He has little experience with this tumor, but he is an expert on solid tumors. He urges you to try chemotherapy. "I think we can save her liver," he says, but he makes no promises.

This is when it dawns on you that doctors can't fix everything. They're groping in the dark, just like the rest of us.

You decide to try chemotherapy. Your daughter is inconsolable.

About three weeks after the first dose of chemotherapy, your daughter loses most of her waist-length hair in one awful, agonizing night. You sit down to brush it, and it falls out in clumps. When you're done brushing, there's a pile of hair on her bed. Your daughter puts on a knit cap, lays her head in your lap, and sobs.

The chemotherapy fails. After a brief period of dormancy, the tumor starts growing again. You know this even before the CT scan confirms it because her abdomen has begun to swell. She will need a liver transplant after all.

You transfer your daughter's care to a state-of-the art facility in New York City, a two-hour drive from home. She is added to the transplant waiting list in December, and you hold your breath through the holidays, hoping and not hoping that you get the call that a liver is available. The tumor is so large now that your 11-year-old daughter looks six months' pregnant.

On a cold day in February, you finally get the call. A man has been shot. His blood type is a match for your daughter's. After dropping your younger daughter at a relative's house, you, your husband and your older daughter rush to the hospital, terrified and exhilarated. The awful nightmare will be over soon.

The 10-hour surgery is successful, but your daughter needs to be rushed back to the operating room a few hours after her transplant to clear a blood clot. You are sure you're going to lose her. The surgeon has left for the evening, but his surgical fellow is here. You look this young person in the eye and say, as calmly as possible, that he must not let your daughter die.

He doesn't let her die. She makes it through this second, two-hour procedure and is returned to the pediatric intensive care unit.

Your daughter spends 10 days as an inpatient. When they finally discharge her, she is given a cocktail of 23 pills to take daily.

She recovers. She heals. She begins to grow again. She spends her 12th birthday in the hospital because her body tries to reject the liver, but she is dosed with steroids and comes through this event unscathed.

You are blessed. She's in remission.

You feel as if you've gone through hell and back, but your daughter is okay. You take her for her six-month MRI. They find two new spots near the transplanted liver and possible spots near her lungs.

The cancer is back, and it has spread.

You switch her care to one of the best pediatric cancer facilities on the planet. Over the next three years, she manages mostly to stay out of the hospital. She has three more surgeries to remove tumors in her abdomen, pelvis and bowel. She undergoes radiation to shrink tumors in her lungs. She tries multiple targeted chemotherapy drugs - pills that she can take at home.

One of the drugs turns her hair white. After an initial tantrum, she shrugs it off and learns to pencil in her eyebrows.

You scour the Internet for anyone who knows anything about your daughter's rare cancer. There are no experts, no clear protocols for treating it. You get another pathologist to look at her biopsy, paying the $500 fee yourself, but the diagnosis is the same. Every doctor you reach out to says the same thing: Surgery is the most effective treatment. You shake your head at this. It seems so barbaric to keep cutting off different pieces of your child to save her.

She turns 13, then 14, then 15 and then . . . the oral chemotherapy begins wearing her down. She gets sores in her mouth and throat, and the drugs must be stopped until she heals. She has one last major surgery to clean her entire abdomen and pelvis of tumors, but they grow back in four months. The lung tumors begin snowballing. Soon, they threaten to collapse her left lung.

It's been four years since her diagnosis in 2012. She is 15 and in 10th grade now. She knows what she's losing. She writes about it in a journal that she makes you promise to read after she dies.

Nearly four years to the day she was diagnosed, her oncologist tells you there's nothing more he can do.

You take her home and let her live her life. You put your own life on hold so you can drive her to school, to parties and to the performances she loves: She's a musician, and you live to hear her beautiful voice.

She has one final scan. She's been struggling for breath. She's been extremely pale. She's been getting fevers every night that spike as high as 103. You learn that the tumor near her left lung is now the size of a grapefruit. It's close to her heart. The oncologist says he's sorry. He does not schedule any more scans. He does not schedule any more follow-ups.

She lives for three months longer. With the help of hospice and palliative care, she is able to stay home. She goes to a final birthday party, meets her friends for a final lunch date. She texts her best friend the night before she dies: "I'll see you this weekend."

It is March 22, 2017. The outside world is gray and cold, covered with snow. You and your husband sit beside her in her bedroom and listen to her labored breathing. You tell her you love her. You tell her you're proud of her. You tell her you're sorry you couldn't save her. You tell her it's okay to go.

She opens her eyes - those big blue eyes you know better than your own - and sighs one last time. Then she's gone.