The fight of her young life

Rachel Kovach and her six girlfriends had a sleepover Thursday night, but sleep wasn't high on their agenda.

The 11-year-olds were up at 5 a.m. to watch royal wedding coverage as they hunkered down in the family room of Rachel's home in Highlands, N.J. Before that, they needed time for decorating mini wedding cakes, pillow fighting, gossiping, and giddy shrieking.

And, way deep down, worrying.

Tuesday, Rachel will undergo a difficult, daylong surgery at Children's Hospital of Philadelphia to remove an ultrarare tumor called Ewing's sarcoma from her right thigh. Surgeons will cut away her femur from above the knee to her hip and replace it with a custom-made cobalt chrome prosthesis.

Combined with seven months of chemotherapy and months of physical rehabilitation, the operation will forever change her life and, everyone hopes, save it.

She will share her "journey," as she mildly calls it, in her new blog, "Onco Girl," which debuts Monday. She hopes to submit an item to Inquirer editors almost every day, but when she's not up to it, her parents, Mari and Kurt Kovach, will pinch hit. (They'll do the postsurgical post Wednesday.)

Although online opiners are as common as "apps" these days, the hope is that "Onco Girl" will reach new audiences in a novel way.

Rachel, an A student in sixth grade at Mother Teresa Regional School and a competitive swimmer with a slew of awards earned since first grade, is an ebullient person who sees almost every glass as half-full. Even her grueling treatment has been "kind of fun in a way," she said Thursday, because it has prompted her to imagine a career in medicine.

"I might like to be a nurse or a doctor," she said. "But not a surgeon. Definitely not a surgeon."

Still, English is not her favorite subject and she could be forgiven if she didn't want to take on a new-media experiment in the midst of fighting a deadly disease. So why did she agree to do the blog when one of her doctors explained that The Inquirer was looking for a young chronicler?

"I'm doing this so I'll have something to do" while stuck in bed, she said. "And maybe to explain how I feel, not ball it up inside."

Mari Kovach, on a sofa across from her only child, took her glasses off and wiped away a tear.

Mari and Kurt met in a college calculus class. Now 51, they are both electrical engineers; she works for the Army, he for a technology consulting firm.

They live in a beautiful house they built on a hill, with a wall of windows providing a panoramic view of the Sandy Hook National Seashore Park and, in clear weather, the Verrazano-Narrows Bridge. Until five months ago, their "leisure" time was full of Rachel's swim practices and meets, not to mention jazz-dancing lessons, figure skating, and basketball.

Then, around Thanksgiving, Rachel began complaining that her right knee hurt. (For the ensuing events that led to the diagnosis of Ewing's sarcoma in January, read her blog at www.philly.com/OncoGirl.)

The cancer is named after James Ewing, an American pathologist who first described it in 1921. It is believed to arise with a primitive stem cell that turns into a renegade instead of producing connective tissue such as bone and muscle.

The malignancy usually develops in bones, or soft tissue near bones, during childhood or adolescence.

Rachel's tumor, originally the size of a cantaloupe and clearly visible in the soft tissue of her right thigh, has shrunk dramatically with chemotherapy, MRI images show. It is now concentrated in the femur, the body's longest bone.

Her diagnosis put her in rare company. About 250 cases occur each year in the United States, or fewer than three per million children and teens.

Even when the disease appears to be limited, or "localized," to one limb - as in Rachel's case - it may have sent microscopic cells to seed the lungs or other organs.

That's why chemotherapy is crucial. Before the cell-killing drugs became available in the 1970s, only one patient with localized disease out of 10 survived five years, said Richard B. Womer, a pediatric oncologist at Children's Hospital.

Now, three of four survive - in no small part because of studies in which Children's has played a big role.

Most recently, Womer led a national study showing that survival rates could be boosted from 68 to 74 percent by giving intravenous chemotherapy every two weeks, instead of every three, for the seven months around surgery.

The five-drug protocol - which is now the standard - is highly toxic. Indeed, two of the drugs are so potent that they are given over five days: two hours a day of the chemicals, eight hours of intravenous fluids to prevent side effects.

"I hate it," Rachel said of those cycles. "You just lay in bed. It's so boring."

Womer's study, published in 2008, proved this intensive regimen can be used without increasing side effects such as infections and bleeding. The key is administering a drug that stimulates the regrowth of crucial immune cells.

So far, Rachel has done remarkably well. She spiked one fever (a sign of infection) that subsided with antibiotics, and had a bout of nausea that required adjusting her antinausea medication. Mostly, her side effects have been fatigue - and, of course, hair loss.

"I just deal with it. I don't even care anymore. I don't wear hats," she said. (On Thursday, however, she wore a Kate Middleton-inspired feathered headpiece that she made at her sleepover party.)

Her father chimed in: "I worried about how other people would respond to her. I didn't want anyone saying things that would upset her. But she was so over it early on."

Which is not to say her parents are over it.

"At work, I've got my 'client' face on," her father said. "But inside, I'm dying because I'm always thinking, 'I've got to get home to Rachel and Mari.' Because I know that at any moment, Rachel could spike a fever. Or the oncologist could call and say she needs to come in" because her white blood-cell counts are low.

Surgery, as well as chemotherapy, has come a long way. Amputation used to be standard for Ewing's sarcoma - and still is, in undeveloped countries. Now, even children such as Rachel can expect to get back on their feet. Both feet.

"The odds are 98 percent that this will go smoothly and she will eventually walk without crutches or a brace," said John P. Dormans, chairman of orthopedic surgery at Children's. At worst, "she might have a slightly unbalanced gait."

Dormans said he and his team spent many hours last week studying MRI images, figuring out where to cut Rachel's femur. They concluded they could save the area near her knee where the bone is still growing. (The reaction from Rachel, who at 5 feet 3 inches is closing in on her mom's height: "Yeah! I always wanted to be taller.")

However, the surgeons won't know precisely how much bone and soft tissue to cut - or how long the prosthesis has to be - until they open her leg and evaluate it.

"We asked the endoprosthesis manufacturer to have all the options covered, so we'll have contingencies in the operating room," said Dormans, a leading expert on pediatric musculoskeletal tumors. "Every case is a little different. They're all like snowflakes.

Dorman said he hasn't yet gotten to know his latest snowflake, but he will.

"We develop a very special bond. Kids are kids, and I don't think they fully understand what this entails. It's incredibly stressful."

Rachel already understands one thing that becoming bionic entails, and it reduced her to tears: No swimming for many months. She will also have to permanently quit her specialty, the breaststroke. It requires a frog-like kick that would be treacherous for her new femur and its delicate mechanics in her hip.

"Me without swimming is like pizza without cheese," said the girl who, after two rounds of chemo in February, swam her personal best time in a 100-yard mixed-stroke race.

One small consolation: Dancing is not verboten.

"That surprised me," she said. "I thought the only dancing I'd be doing is chair dancing."

In the coming months, Rachel may have some bad days and bad moods. But clearly, she will not have a bad attitude.

Asked what one word best describes her, she didn't hesitate.

"Optimistic." she said.

Hello, my name is Rachel. I am 11 years old. I am a competitive swimmer, a figure skater, a jazz dancer, and now I am also a cancer patient at Children's Hospital Of Philadelphia (CHOP). I have a rare bone cancer called Ewing's Sarcoma. My parents brought me to CHOP, even though we live two hours away in Highlands, NJ, because they wanted me to have the best possible care there is.

Around Thanksgiving, I was having pain in my right knee. My parents took me to an orthopaedic physician - a bone specialist - who initially thought this was tendonitis from training with my swim team. He told me not to train with fins for a few days, take a pain reliever, and things should be fine - and they were for a few weeks.

Just after Christmas, my knee started to hurt again and my parents made an appointment to go back to the bone doctor. This time my right thigh was swelling up and the pain was getting so bad that I had trouble sleeping. When we went to the doctor, he took more x-rays and really didn't see anything. But after seeing the swelling in my thigh, he said that I needed an MRI to get a better idea what was going on in my leg.

My MRI was scheduled for Thursday, Jan. 20. The next morning, while I was at school, the orthopaedist called my parents and told them there was a "mass" in my leg and he thought it could be Ewing's sarcoma, a rare form of bone cancer that strikes children and teenagers. My parents rushed to the doctor's office. He showed them the MRI films and pointed out what he thought was the tumor.

My parents later told me that this was a very difficult thing to hear. But when I came home from school that day, my mother didn't say anything until I said, "So, did you hear anything about the MRI?" We talked for a few minutes and she was obviously trying not to alarm me. Finally, I asked whether it might be cancer. She admitted it might be.

Fortunately, my bone specialist used to work with Dr. John Dormans, the head of orthopaedic surgery at CHOP. I was able to get in to see Dr. Dormans first thing on Monday. I later came to know this was a really good thing because every day counts when you're fighting a cancer that grows as fast as mine.

Dr. Dormans told me that I would need a biopsy to get a good diagnosis of whether it was cancer in my leg. I felt like my heart was going to sink right out of my body and into the center of the earth. Even though I knew it might be cancer, when I heard it from the doctor, it became real. I was crying too much to listen carefully to everything he said.

Dr. Dormans did the biopsy on my leg later that week and confirmed that what I have is Ewing's sarcoma. I had lots of other tests to see whether the cancer had spread. Even though it was only in my leg, my doctors said I would still need chemotherapy to kill any cancer cells they couldn't see. So from February until two weeks ago, I had chemotherapy every other week. Chemo makes me much more tired than usual, and it made my hair fall out, but otherwise, the side effects haven't been too bad.

I feel really good about the team working with me at CHOP. The oncology team actually wrote the treatment protocol that's used around the world to treat Ewing's. The team developed a roadmap of my treatment plan that lays out every step and schedules every test, every medication, every treatment.

My surgery is tomorrow. I'm scared, but optimistic.

Over the next few months, I plan to share the rest of this journey with you as I see it happen.