This is a story many years in the making. Rae, as I will call her, came into my life before I was an oncology nurse navigator (ONN) and after she had already been on her health journey for most of her life.
Rae’s journey began when she was 3 years old and the first of many desmoid tumors in her brain was diagnosed. Several tumors were surgically removed from her skull. At 10, her oncologist recommended testing for familial adenomatous polyposis (FAP) because of its known relationship with desmoid tumors.
FAP is an inherited cancer syndrome that causes hundreds to thousands of precancerous polyps to form in the colon, and the polyps may develop into cancer if the colon is not removed. Mainly thought of as a disease of the large intestine only, FAP is also associated with extraintestinal manifestations such as precancerous polyps and cancer in other areas of the digestive tract, as well as tumors in the brain, eyes, and thyroid.
When Rae first came to our pediatric gastrointestinal (GI) clinic, I was the nurse who worked with her gastroenterologist. Genetic testing confirmed a diagnosis of FAP. This was the first of many times our paths would cross over the next 14 years. Rae underwent her first colonoscopy in 2004, at age 10, where a few adenomatous polyps were seen. Only 6 months later, her next colonoscopy revealed that her colon wall was carpeted with adenomatous changes. She then underwent a total colectomy with ileostomy. I had gone back to school to become a certified ostomy nurse. Our paths crossed again when she came to see me for ostomy-related issues.
At about the same time I moved on from my position in pediatric GI, Rae moved on to adult care. We lost touch for a couple of years but reconnected during my first year as a complex GI ONN. Rae was being managed with surveillance endoscopies, and she was at the hospital where I work for an endoscopic procedure to follow up on an adenomatous duodenal/ampullary polyp.
Our roles were changing. Rae was preparing to begin nursing school. No longer her nurse, I became her friend and mentor. I was impressed, but not surprised, by her positive attitude in spite of a long life of medical care. She told me how much she wanted to give back and help other people, an admirable quality and a perfect trait for a future nurse.
Rae and I kept in touch while she attended nursing school. In the fall of 2016, her mother contacted me and told me Rae was concerned that she had an abdominal desmoid and she didn’t feel confident that the university’s physician understood her disease. Even though Rae was being seen by adult-practice clinicians — a colorectal surgeon and a gastroenterologist — clinically she was still in between the pediatric and adult worlds. I referred her to the surgical oncology team I work with, something I would not have thought to do before I was an ONN. This turned out to be the best decision because of the precancerous polyp in her ampulla/duodenum. I was able to facilitate the appointment that was the start of the next chapter in her journey.