Teenage Cancer: One Girl's Story

Two years ago, 13-year-old central Texas resident Lizzie T. was enjoying the summer between her seventh and eighth grade years. If she seemed anxious for the season to go faster than usual, it was because she had made the cheerleading squad for the Fall.

Then she got that flu-like feeling again.

"My whole life I'd never been sick," she tells me in a phone interview. "But that was the third time I'd felt that way in a pretty short period of time."

Each time before, her doctor diagnosed her with an infection. This time, he sent her to Dell Children's Hospital in Austin for a bone marrow biopsy.

A week later Lizzie, along with her mother and other members of her family, went to the Children's Blood and Cancer Center at Dell (CBCC) for the results. "We all just assumed it was another infection. Nobody was worried."

The doctor entered and spoke squarely.

"I have some bad news. Lizzie, you have leukemia. It's a type of cancer."

At that moment the room froze out, she says. "The doctor started talking again. But I didn't hear a word she said. I was just shocked."

A VIOLENT INCARNATION

She would in due time become all too familiar with her specific diagnosis: acute lymphoblastic leukemia (ALL), one of the most swiftly lethal cancers known to medicine.

Referring to leukemia as "cancer in one of its most explosive, violent incarnations," Siddhartha Mukherjee writes,

[Leukemia's] pace, its acuity, its breathtaking, inexorable arc of growth, forces rapid, often drastic decisions; it is terrifying to experience, terrifying to observe, and terrifying to treat. The body invaded by leukemia is pushed to its brittle physiological limit—every system, heart, lung, blood, working at the knife-edge of its performance.

Children's Oncology Group (COG), the world's largest organization devoted to childhood and adolescent cancers, refers to leukemia as a "liquid" cancer. Instead of forming concentrated tumors, leukemic 'blast' cells—immature blood stem cells that do not develop beyond the blast phase—circulate throughout the body, from the very core where they originate—the bone marrow—all the way to a hideaway behind the blood-brain barrier in the central nervous system. This is a hallmark of leukemia, setting it apart from just about every other cancer type.

This distinction does not mean that ALL does its damage any differently than other cancers. In the case of any malignant cancer, the problem is a ferocity of cancerous cells growing in the body and not dying. Their accumulation eventually crowds out other cells. The consequence is loss of certain functions and, if untreated, death.

Teenage Curiosity

One of the first questions every cancer patient has is also one of the questions no doctor can answer, namely 'How did I get cancer?'

Although she was adopted, Lizzie is close to her biological father. "So I knew there wasn't a history of cancer in my family. This was just totally out of nowhere."

Determining risk factors in adults is somewhat easier since in some instances behavioral issues are at work, but in pediatrics this doesn't fly.

"Most pediatric patients have not had any exposures that should predispose them to any cancer including leukemia," says Dr. Jennifer McNeer, Assistant Professor of Pediatrics, Section of Pediatric Hematology/Oncology and Stem Cell Transplant at the University of Chicago Comer Children's Hospital. "There are chromosome abnormalities, molecular abnormalities that we can find that may be the driving force behind a cancer, but understanding why they happen in the first place is unclear."

BY THE NUMBERS: ACUTE LYMPHOBLASTIC LEUKEMIA

BORN AT THE RIGHT TIME

In 1948, fifty years before Lizzie was born, the survival rate for patients with acute leukemias like hers was zero. Hospitals routinely tucked away leukemia patients—young kids—in quiet corners where nobody had to go if they didn't want to. Here, these patients died by the thousands.

Then, a paper published in the New England Journal of Medicine detailed the otherwise unheardof: kids with acute leukemias were being put into (brief) remission thanks to chemical intervention.

The lead author was a pathologist named Sidney Farber. His paper launched the chemical therapy era, also known as chemotherapy.

Sixty-five years later, Farber's fearless—and broadly unpopular, at the time—work has been reworked and finessed and trialed and tested. The results in blood cancers where chemo has had the most profound effect speak for themselves. For example:

The survival percentage in childhood ALL had been 0 percent. Today, it is 86 percent. In Hodgkin's lymphoma, it had been 0 percent. Today, it is 85.1 percent.

Teenage Determination

Thus, when Lizzie's 8th grade year got underway in the Fall after her diagnosis, she was not there. When the cheer team performed their routines, she was not among them. The intensity of chemotherapy left her too tired, too sick, and too immunocompromised. And she was losing weight.

"Before I was diagnosed I weighed like 114. But on chemo I didn't want to eat anything. The lowest my weight got down to was I think 86. They [the staff at CBCC] weren't happy about that."

And, she adds, "I was definitely having some problems with chemobrain."

But Lizzie never fell behind in her school work. Thanks to her own determination, thanks to the Home Bound Schooling program, and thanks to her mother, who home-schooled her in a pair of subjects, including Texas History, Lizzie finished the 8th grade on time.

She accomplished this in spite of the grueling therapy regimen for ALL.

THE LONG, LONG CHEMO ROAD

The chemotherapy regimen for childhood ALL is nothing if not relentlessly long and complicated, a multi-disciplinary balancing act of advanced pathology, molecular genetics, pharmaceutical precision, long-term scheduling, and as important as anything—flexibility.

Because ALL is an acute disease it is a medical emergency. Patients like Lizzie tend to began chemotherapy very quickly after diagnosis.

Now 15, Lizzie is two years deep in chemo, and she's not done.

"For me chemotherapy started out basically every week," she says in a mix of weariness and matter-of-fact. "And then the time between appointments started going from a couple weeks to a few weeks to every month. And it's about every month now."

She's loosely describing the three phases of ALL treatment:

• An Induction phase that lasts about a month and for 95 percent of patients puts the cancer into remission by killing as much as 99.9 percent of the leukemia cells.
• Then a more intense consolodation or intensification phase, as long as two months, designed to kill even more of the cells.
• Finally, the maintenance phase, which can last two years and sometimes involves brief flashes of intense chemotherapy.

"I get most of the chemo through a chest port. But every three months—this August should be the last time—they do a lumbar puncture which sends the chemo up my spine and into my brain."

Known as intrathecal chemotherapy, this allows doctors to bypass the blood-brain barrier and kill any leukemia cells camping out in the central nervous system. It is standard treatment, and it is better, long-term, than the former alternative: cranial radiation.

"In patients with overt CNS disease, meaning the spinal fluid is affected at diagnosis, some of those patients do require cranial radiation," adds McNeer, "but we try to avoid it because of the long-term consequences."

If you can wipe out 99.9 percent of the disease in one month, why should it take another 24 or more months to kill the remaining .1 percent? Within the answer lies the key to the rising success rates in patients with ALL over the past few decades.

Teenage Poise

"I wear a wig now," Lizzie tells me, with neither emotional detachment nor even a trace of embarrassment. "My hair had sort of been coming out a little at a time."

In retrospect, the awkwardness of adolescence is hideous and unbearable. We are, wisely, unequipped with this insight at the time, or else few of us would willingly go through it.

And that's if you've got your health. And your hair. While not all chemo causes hair loss, it's pretty consistent in ALL therapy.

"Then I was asked to walk with the cheerleaders in a parade, so my mom and I went shopping and I tried on this turtle neck, which was one size too small. When I pulled it over my head, all of my hair went with it."

CONSOLODATION

"In protocols from a few decades ago, patients would go directly from induction to maintenance, and a good number of them relapsed," says Dr. McNeer. "The protocols pioneered in the 1970s and 1980s introduced the consolodation phase between induction and maintenance, and that's when our survival rates began to go up."

It seems then that killing leukemic blast cells in the body via chemotherapy is not very difficult (relatively speaking). The difficult part is in killing all of them, an assertion that applies not just to ALL but to every cancer.

"Ideally we like to have patients in remission by the end of the first month, and now we have tests that are so sensitive they can detect [leukemia] cells in the bone marrow down to .01 percent. But we know that if we stop there, the cancer will come back. In ALL we want to see minimal residual disease (MRD) of less than .01 percent."

In the majority of ALL patients, McNeer says that "it's the duration of the therapy that ultimately eradicates every last [cancer] cell."

However, even two or three years of chemotherapy doesn't guarentee a cure for each and every patient, since there are still those who relapse—either after treatment has finished or sometimes during treatment, when the presumptions are either that some leukemia cells are somehow still dodging detection, or they have become resistant to the drugs.

Teenage Grace

One of youth's most magical gifts is the illusion of immortality. Life normally reserves the first confrontation with one's own mortality for a much later time than the early teens.

"There was this other kid I knew from the Blood and Cancer Center who had the same cancer as me," says Lizzie. "I knew him pretty well. And he didn't um— he didn't make it through, or— finish the program."

To this point in the conversation euphemisms weren't Lizzie's thing; she was a straight shooter. Although it was obvious what she meant, I decided not to let her get around it.

"Can you rephrase that?"

"I'm sorry, I mean he died," she responded gracefully. "It was scary, it was unbelievable. Here's a kid with the same illness as me, exact same, and he's dead from it, you know?"

RESEARCH PAST, PRESENT AND FUTURE

Between 1975 and 2006, deaths from childhood cancer declined by over 50 percent, with the most notable decline seen in ALL. In fact, in that 32 year time frame, experts estimate that advances in therapy saved the lives of about 38,000 kids with cancer.

The current trend in medical oncology (adult cancers) is towards developing therapies that target specific cell surface antigens or cancer-causing chromosomal translocations. When you read about newly FDA-approved drugs that cost tens of thousands of dollars per month, you're reading about developments for treating adult cancers.

In pediatric oncology, the chemotherapy drugs are almost uniformly old work horses with well-understood activity and toxicity profiles, like cytarabine, methotrexate and doxorubicin.

To that end, there is an extraordinary atmosphere of collaboration in this field. Standard treatment guidelines are so well-established that second opinions are practically redundant. Meanwhile, clinical trials continue in all phases across several research consortiums, including Children's Oncology Group (in which The Children's Blood and Cancer Center participates), as well as smaller consortiums who run their own clinical trials, such as St. Jude's Children's Hospital, and Dana-Farber/Boston Children's Cancer and Blood Disorders Center.

Teenage Living

In the fall, Lizzie is scheduled to start her sophomore year, in step with her class like any other kid. She will also fulfill her achievement from the summer of her diagnosis: Lizzie tried out and made the cheerleading squad for the fall—deferred, but not deterred, by two years and a cancer diagnosis.

"So the last couple years you've had leukemia," I said, beginning one of the dumbest questions I've ever asked, "you've been to the Blood and Cancer Center so many times, multiple procedures, lost your hair [which is growing back], lost 30 pounds; can you describe what that's been like?"

"No. I can't."

Oh my God that's a good answer, I thought. "I guess that might be kinda hard."

"You know what, everyone [at CBCC] was so incredible. Especially when I just didn't want to go through another infusion, another procedure, they were just so supportive and so amazing. Still," she said, her tone of voice, languid in that very indolent teenage way, suddenly assumed its first hint of strain. "It's been … rough. That's the only word I can think of to describe it, rough."

* * *

"Having leukemia has definitely changed my life, my perspective, on everything," she told me as our phone call wound down. "You never know what can happen."

Then, with an almost comical edge she tacked on a final statement that sounded like she'd said it a hundred times before, and like it should have been followed by a yada yada yada or a blah blah blah, "You know, life is precious ..."

Despite the frivolity, I think Lizzie not only believes that life is precious but knows it first-hand.

I also think she's tired of thinking and talking about life, and ready to start living it again.

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This is the third installment in my series on the Children's Blood and Cancer Center at Dell Children's Hospital in Austin, Texas. The first two are below:

• Introduction: Uncommonly Ordinary
• I: Not Your Average Prom