Cancer Today - Winter 2017/2018

On Thanksgiving Day 2011, Court Simmons threw on a pair of shorts and jogged toward the hospice center where her mother, Crystal, was a patient.

The energetic 16-year-old loved to cook and had just finished making turkey dinner for her favorite person—her little brother, Reginald, who was 14.

As Court (short for Courtney) ran down the street toward the hospice center, a few blocks away from her Philadelphia home, a throbbing ache beneath her left kneecap forced her to slow her pace to a walk. “I was dealing with a lot, so I ignored the pain. It seemed like the right thing to do at the time,” she says.

Since Court was 9 years old, she had watched her mom, a single parent, deal with a soft tissue sarcoma, a type of cancer that develops in tissue such as muscle or fat. The experience forced Court to grow up fast. When she was 12, her older sister, Christina, who was 17 at the time, died of a brain tumor. And Court was just 16 when her mother died on Christmas Day 2011, at the age of 41.

After her mother’s funeral in January 2012, Court couldn’t wait to get back to school. She and Reginald were staying temporarily with her mother’s friend because they had no legal guardian at the time. School was a refuge—she enjoyed class time, loved reading and longed to laugh with her friends.

On her first day back, Court chased her best friend down the hallway near their lockers. A terrible pain shot up her left leg, similar to what had happened when she ran to her mother’s hospice center on Thanksgiving Day. She sat down on the floor as her leg throbbed, rolled up her pants leg and saw the skin had turned purple. “I thought maybe I had torn a muscle,” she says.

A few days later, she found herself sitting in a dark room at Children’s Hospital of Philadelphia (CHOP) staring in disbelief at an MRI image of an orange-sized mass protruding from the top of her left shin.

A biopsy confirmed that she had osteosarcoma, a form of bone cancer. “The universe wouldn’t do this,” she thought to herself.

A Family History

To say cancer runs in Simmons’ family is an understatement. Court, her mother and her sister all were diagnosed with a condition called Li-Fraumeni syndrome, a rare genetic disorder that predisposes children and adults to several kinds of cancer. (Reginald does not have Li-Fraumeni.) “People with Li-Fraumeni syndrome are born with a mutation in a tumor-suppressing gene or oncogene that predisposes them to certain types of cancer,” says pediatric oncologist Abby Green of CHOP, who treated Court. Cancer striking two young people in one family is a major red flag suggesting Li-Fraumeni syndrome, Green says. In the Simmons family, cancer had hit three family members when they were young: Court’s mom was diagnosed with breast cancer at age 18, while pregnant with Christina, and with soft tissue sarcoma in her mid-30s.

Court is one of about 1,000 people in the United States who are diagnosed each year with osteosarcoma, a type of cancer associated with Li-Fraumeni syndrome. Close to half of those diagnosed with osteosarcoma are children and young adults. The disease tends to develop in the rapidly growing long bones of the legs and arms, especially in teens hitting their growth spurt.

In Court’s case, the cancer had not yet spread beyond her leg bone, and the tumor was in a good location for removal. Her prognosis was favorable, says Green, “but we knew it wouldn’t be easy for her.” She would require 30 weeks of chemotherapy, reconstructive surgery on her leg and rehabilitation to strengthen her leg muscles.