Common Symptoms Can Deceive

By Sandra G. Boodman
Washington Post Staff Writer
Tuesday, December 11, 2007

All her life, Jamie Fear's gut had been her proverbial Achilles' heel. When other people contracted colds or other respiratory infections, she got stomach viruses. She tried to baby her digestive system and learned to live with her sensitive stomach.

But when her symptoms increased in the mid-1990s and she noticed a persistently tender spot on the lower right side of her abdomen, Fear began a series of visits to her HMO. Each time, she recalled, doctors and nurses had the same answer: irritable bowel syndrome (IBS), a common, catchall diagnosis for digestive problems characterized by bouts of bloating, pain, constipation and diarrhea. There is no specific test for IBS, which is diagnosed on the basis of symptoms.

But as Fear later learned, her problem was anything but common -- and it wasn't IBS. She is convinced that her life might have been different had her repeated complaints that something was wrong been investigated more thoroughly; in fact, she recalled, her HMO did not refer her to a gastroenterologist.

"Back then I was the type of person, 'Okay, doctor, whatever you say,' " said Fear, 49, who lives in Woodbridge. "I didn't know how to demand things."

In November 1998, alarmed by worsening bloating, she called an advice nurse to see about getting a CA-125 test, a tumor marker used to screen for ovarian cancer; bloating and abdominal pain are common symptoms of the malignancy. The nurse, she said, pooh-poohed her request, saying, "Honey, you'd be in extreme pain if you had ovarian cancer." Fear didn't pursue it.

Although her problem was not ovarian cancer, Fear's CA-125 level turned out to be significantly elevated when it was tested later. Such a finding would have spurred doctors to look further and might have led to an earlier diagnosis.

Bloating so noticeable that her husband once asked Fear if she was pregnant was not her only symptom. Food went "right through me," Fear recalled, yet she had gained 20 pounds in the preceding year and was eating less. And sex had suddenly become painful.

Blood tests, X-rays, a pelvic exam and a barium enema revealed nothing; a CT scan or ultrasound, which would have detected the unusual problem, were not ordered.

"I felt like a hypochondriac searching for a disease," said Fear, then an editor at the National Academy of Sciences. "Nothing was ever wrong."

In March 1999, she went back to her primary care doctor and announced that her digestive problems "were affecting every aspect of my life." Maybe, she thought, that would get his attention.

By then her distended stomach was so tender that she felt a jolt of pain if her husband's arm grazed it while they were sleeping. The rim of her navel felt hard.

Fear recalled that her physician sat across the room scribbling on her chart and reading off a checklist of IBS symptoms, a problem he told her he had, too.

What about the hard belly button? she asked.

"That's nothing to worry about," she recalled him saying, without palpating it. It was just a tiny umbilical hernia, he confidently pronounced.

Feeling uneasy, Fear decided to tough it out. Another new symptom -- fatigue -- was probably a consequence of commuting, working full time, going to night school in preparation for a career change and turning 40, she surmised.

Two months later Fear was lying in bed feeling her belly button when she touched something else that "made me so scared I felt like I would pass out." It was a hard lump the size of a small banana near the long-tender spot on her right side. At that moment, she knew instinctively that her life would be forever divided into two phases: before she discovered the lump -- and after.

A few days later, a radiologist told her that CT scans and other tests revealed that her abdomen and pelvis were full of fluid. Benign fibroid tumors were an unlikely possibility. Doctors suspected she had advanced ovarian cancer or metastatic colon cancer. Her CA-125 level was nearly 254: A reading under 35 is typically considered normal. A colon cancer tumor marker was significantly elevated as well.

After an emergency colonoscopy ruled out colon cancer, Fear underwent a total hysterectomy at Inova Fairfax Hospital. The surgeon told her he thought he had gotten most of the tumor -- she would later learn it was more like 50 percent -- and had also removed her appendix for further testing. She probably had stage III ovarian cancer, the surgeon said, although there was a 5 percent chance she had a rare malignancy that originated in the appendix.

And that's what the pathologist found: a poorly understood condition called pseudomyxoma peritonei (PMP), a form of abdominal cancer so uncommon many doctors see it only in a textbook.

Known colloquially as "jelly belly," PMP is caused by the accumulation of mucus-secreting tissue that forms gelatinous tumors that stick like Velcro in the abdomen and pelvis, according to Fear's longtime surgical oncologist, Brian Loggie. It occurs after a polyp bursts through the wall of the appendix, spreading tumors that cause swelling and impede digestion. Without treatment, which often involves aggressive surgery and chemotherapy, PMP is fatal.

Loggie, who is now chief of surgical oncology at Creighton University in Omaha, said he has rarely seen a case as advanced as Fear's: she had tumors that extended to her diaphragm.

In the early 1990s while at Wake Forest University in Winston-Salem, N.C., Loggie had pioneered a PMP treatment that involves surgery followed by heated chemotherapy drugs infused directly into the abdominal cavity, a regimen that has improved survival and quality of life for some patients.

Fear arrived at Loggie's North Carolina office several weeks after her hysterectomy, desperate for options. She said her HMO oncologist had told her that although he had never seen PMP, he planned to treat it with a colon cancer protocol Fear said she had learned was ineffective for malignancies like hers. Her life expectancy, the HMO oncologist predicted, was about two years.

"That was eight years ago," she noted. Since then Fear has undergone seven surgeries performed by Loggie, whom she followed from Winston-Salem to Dallas to Omaha.

By the time I get patients, "the ball may or may not be fumbled" by doctors unfamiliar with PMP, Loggie said. He sees one to two new cases each week and has treated well over 200 PMP patients, some from abroad.

"I get a lot of patients who've had experiences like Jamie," he added.

Fear knows she has beaten the odds but says the battle has sometimes felt overwhelming. She has had part of her liver and colon removed, along with her spleen and umbilicus. So far she has avoided a colostomy.

Her husband, Don Fear, a photographer, has documented her illness in a multimedia exhibit titled "Healing Steps: Jamie's Journey," which has been displayed at the National Institutes of Health, the Smith Farm Center for Healing and the Arts, and the Office of the Secretary of Health and Human Services, as well as in hospitals in Fairfax and Charlottesville.

"I guess you could call me a success story," Fear said, adding that she doesn't dwell on the "what-ifs."

"I've struggled many days and have enjoyed many days. In the end, who on this Earth hasn't done the same?"

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