Small: Life and Death on the Front Lines of Pediatric Surgery

8 | Something to Celebrate

something to celebrate I couldn’t help but notice. I hadn’t seen them in several months, maybe six or so, but the last time I saw Rachel’s mother, she had had a full head of hair. “Rachel shaved it,” she told me. “It’s cute,” I said. I glanced at her daughter’s own hairless head. Only a few wisps of solitary hairs remained at her neckline and around her ears. Her mother’s appearance needed no further explanation. I knew it was one of those things that mothers do for daughters, especially five-year-old daughters who have lost all their hair to chemotherapy. “I was a movie star for Halloween,” Rachel blurted out. Her mother proudly whipped out a hefty stack of glossy photos . Rachel was posing boldly for the camera, her hands held high above a full head of teased red hair. She sported a shimmering evening gown, comically risqué for a child. A wide ribbon of lipstick blotted her mouth, and she had eyebrows again, drawn in by her mother, for the first time since her treatment began. Rachel was being treated for rhabdomyosarcoma, a highly malignant tumor that originated in the muscle tissue of her back. In the continuum of cancer that extends from favorable to unfavorable to just plain horrible, Rachel’s type would fall into the not-sogreat end of the spectrum. At the first operation the tumor had been large and fixed, spreading diffusely throughout the tissues, so only a biopsy was performed. Sometimes tumors, particularly something to celebrate ( 121 ) large ones, may be too close to vital organs or other structures that, if removed, would cause long-term disability. In such cases a surgeon may opt to biopsy the tumor, have the pathologist identify exactly what type of tumor it is, and then determine the optimum treatment plan. To shrink the margins of the tumor and control the spread of it throughout her body would require several rounds of some of the strongest chemotherapy available, her only hope for cure. So Rachel underwent another operation just to put in a Broviac catheter , a long white tube that was tunneled beneath the skin of her chest, under her clavicle, and guided into a large vein, where it ultimately rested just inside the heart. This catheter would minimize the number of sticks Rachel had to endure for countless blood draws and ensure that caustic chemotherapy drugs were delivered directly into the bloodstream. Most of us think of cancer as a disease of adults, a devastating diagnosis our friends, neighbors, and coworkers get hit with in the prime of life. We are jarred when someone we know in their thirties, forties, or even fifties receives the news of what could be a terminal diagnosis. The idea of a child having cancer is even more unsettling, but approximately 12,000 children in the United States are diagnosed with it each year.1 The peak incidence of childhood cancers is in patients under two years of age. When compared with adults, childhood cancer only comprises 2 percent of all cancer cases; however, it accounts for 10 percent of all deaths among children and is the leading cause of death from disease in children.2 While we never fail to be shocked and saddened when a child is diagnosed with cancer, there is some comfort in the fact that we expect many, up to 80 percent, to be cured, compared to only 65 percent of adults. Why do kids with cancer fare better than adults? The number one factor is the fact that pediatric cancers are bi- ( 122 ) s m a l l ologically different from adult cancers. While childhood cancer shares some similar characteristics to adult cancer—it can spread, come back months to years later, and be fatal—in many respects it is almost a different disease. The most common adult cancers— skin, prostate, lung, breast, and colon—are frequently linked to long-term exposure to carcinogens such as the sun or tobacco and are seldom seen in children. Adult cancers are derived from cells that line cavities and glands, known as epithelial-type cancers. Childhood cancers are derived from embryonal, or immature, tissue types originating from nerves, brain, bone marrow, bone, and muscle. As is true for any cell type undergoing relatively rapid cell growth, childhood cancer types are in general more responsive to chemotherapy and radiation than adult types...