The memories remain so vivid that Kathy Lang Albright still goes out of her way to avoid driving past the clinic where she received her first chemotherapy treatments. When Ebola was dominating the news, just hearing about the gastrointestinal symptoms of the disease made her weep, as she recalled the hellish weeks when the aftereffects of her treatment seemed worse than the advanced colon cancer it was designed to treat.
Albright, now 65, remembers how her legs shook with fear as she walked into the clinic for her fourth round of chemotherapy in November 2013, anticipating the ceaseless diarrhea that would follow, which made it nearly impossible for her to leave her home on the outskirts of Atlanta. She knew that a new crop of painful mouth sores would make swallowing excruciating. Her hands were sore and sunburned-looking, the skin on her eyelids was peeling and her feet were cracked, spotting her socks with blood. In less than six weeks she had lost 14 pounds.
Why, Albright wondered, was her experience so different from that of a close friend, who had undergone a similar chemo regimen for colon cancer and felt well enough to work through her treatment?
Every time she asked the nurses who administered chemotherapy whether her side effects were normal, their answer was unvarying. “They kept telling me, ‘Well, everyone reacts differently,’ ’’ recalled Albright, a former marketing director for a large national accounting firm.
Several months later, she would learn that there was a biological reason for her suffering, one that had been overlooked for months. Armed with that knowledge and a new oncologist, Albright completed cancer treatment last year, largely spared the toxic side effects that had made her so sick. Now in remission, she says she has made the transition from “cancer victim to one of cancer’s alumni.”
In August 2013 Albright began experiencing searing abdominal pain. Her internist diagnosed diverticulitis, an inflammation of the intestinal wall, and sent her to a gastroenterologist for a colonoscopy. It was her first.
Albright had ignored advice to undergo colon cancer screening, which is recommended starting at age 50. “I just didn’t have time,” she said. “I was taking care of everyone else but me.” She rationalized that no one in her family had had colon cancer. Her parents, both smokers, died of tobacco-related illnesses; Albright was a nonsmoker who had always been healthy.
The results of the colonoscopy, which inspects the lining of the large intestine with an endoscope, were grim. Albright did not have diverticulitis, but rather Stage 4 colon cancer, the most advanced form. The disease had spread to her liver.
“I was actually very calm,” she recalled, “surprised — but not shocked. Other people seemed to be more upset than I was. My focus was on what happens next, what am I supposed to do?”
In late September Albright and her husband met with an oncologist at their HMO to discuss treatment. He recommended a biweekly chemo regimen called FOLFOX for three to four months, followed by surgery to remove the tumor and then more chemo. The FOLFOX regimen, a standard treatment for colon cancer, consists of a cocktail of drugs: leucovorin, fluorouracil (also known as 5-FU) and oxaliplatin. Albright also received infusions of Erbitux, a drug approved to treat metastatic colon cancer.
The oncologist told Albright that she would probably experience some side effects — fatigue, poor circulation in her hands and feet, and acne were mentioned — but that she should remain physically active. Walking her dog, going to her yoga class and working out at the gym would help her feel better, she remembers the doctor telling her.
Albright said she considered getting a second opinion but decided that was unnecessary after the oncologist assured her that he would be on-site when she received infusions, available to monitor her response and deal with any problems that might arise.
But after that meeting, Albright said, she never saw or spoke to the oncologist again, despite repeated attempts to contact him.
Each time she returned for treatment, she said, she dealt with nurses who seemed to be in charge, administering the chemotherapy and fielding questions. She said she left the doctor phone messages and sent him e-mails, which were returned sometimes days later by nurses or the oncology pharmacist. And when she walked past his office in the clinic, it was dark.
Albright received her first dose on Oct. 22. Over the next 10 days her fatigue and nausea worsened. Her second treatment, scheduled for Nov. 5, had to be canceled after Albright was told that her white blood cell count was about 200 — far lower than the 1,500 required for chemo. Maybe, she thought, that explained the profound exhaustion that now required her to rest after walking between the bedroom and the kitchen of her small home.
She told a nurse that her balance was off — she kept tripping over her feet — and that painful sores had erupted on the inside of her mouth, her gums were bleeding and her eyes felt so dry she couldn’t read.
The nurses, she said, told her that “sometimes these things happen.” Albright was given a prescription for a special mouthwash and for shots of Neupogen, a medicine that boosts white blood cell production. She was told she would need to give herself nightly injections in the stomach. When her white count rebounded, chemo would resume.
On Nov. 12, Albright returned to the clinic. The Neupogen had boosted her white count but was difficult to tolerate: The shots, administered by her husband, made her anxious and jittery and caused bone pain that she said “hurt like hell.” The sores on the inside of her mouth bled when she tried to eat, and the diarrhea was unremitting.
Upset by the oncologist’s constant absence, Albright said she was not sure what more to do. “I was such a nice person [that] I didn’t like other people to feel uncomfortable,” she said. “I thought, ‘Well, I’ll have another chance to ask this question.’ ”
By early December Albright was so sick she could barely stand up and went to the HMO’s urgent care center twice in one week. The first time, a doctor diagnosed her with flu. The second time, another physician performed several tests, then called an ambulance. “He told me that my potassium and electrolytes were so low I was at risk for a heart attack and that there was so much going on in my system I needed a complete workup in a hospital,” Albright remembers being told.
Initially, doctors who treated her during her five-day hospitalization suspected that Albright had contracted an infection, a consequence of an immune system weakened by chemotherapy. A CT scan revealed that her colon was severely inflamed. Late one night shortly after her admission, Albright said she began weeping and told a nurse, who comforted her, that she felt her “guts were disintegrating.”
The hospitalist treating Albright told her that the elusive oncologist was no longer affiliated with the HMO. “Now I had no doctor,” said Albright, who insisted that a gastroenterologist see her. The GI specialist told her that there was no sign of an infection and that her problem was most likely a bad reaction to chemo.
A week after her discharge, Albright, feeling “weak, woozy and scared,” and her husband met with a new oncologist. The specialist mentioned that some patients have a sensitivity to certain chemotherapy drugs. “She said something about D-something deficiency,” Albright recalled. “She said the side effects [in such a patient ] would be 10 times worse” than normal.
Galvanized by her ordeal, Albright began researching sensitivity to chemotherapy. She stumbled across a description of DPD deficiency, short for dihydropyrimidine dehydrogenase deficiency, which described many of the problems she had suffered. “The more I read, the more alarmed I got,” she said.
DPD deficiency is an inherited metabolic disorder caused by mutations in the DPDY gene, which provides instructions for making an enzyme involved in breaking down two molecules when the body does not need them.
Mutations that result in complete deficiency are rare and are apparent in infancy; they are characterized by repeated seizures, small head size, delayed motor skills and intellectual disability. Cases of partial deficiency, believed to affect 2 to 8 percent of the population, show no symptoms — unless such a person is given certain chemotherapy drugs, including 5-FU, one of the drugs Albright received.
In these cases, 5-FU is not metabolized normally but instead accumulates to toxic levels, causing ulceration of the lining of the gastrointestinal tract, severe diarrhea, mouth sores called mucositis, low white blood cell counts and peeling skin on the palms and soles of the feet — all of which Albright experienced. If left untreated, death can result.
“It’s a horribly toxic event, and when you see it, it’s just unbelievably striking,” said Gary K. Schwartz, chief of hematology/oncology at Columbia University Medical Center and a spokesman for the American Society of Clinical Oncologists. An estimated 3 to 5 percent of cancer patients are believed to have some degree of DPD deficiency, which was first identified about 20 years ago.
“We don’t routinely screen for it because it’s so rare,” Schwartz said, and because people who don’t have a DPD deficiency can have toxic reactions to cancer drugs. But “most medical oncologists are aware of this genetic variant and they would think about it — or should” in a patient with severe side effects.
Albright, whose trust in her HMO was shattered, now got a lucky break. Her husband had taken a new job, and she was covered by a second health insurance policy, which enabled her to see new doctors.
“We just walked away” from the HMO, she said.
In late December 2013, Albright began seeing Robert Allen, the Atlanta oncologist who had successfully treated her friend for colon cancer. Albright said that at their first meeting Allen reviewed her records, then told her that based on his calculations, she had received an overdose of chemo: 30 percent too much for her weight and height.
Allen, in an interview, said he could not recall the entirety of their conversation and, in any case, would not discuss another doctor’s treatment. He ordered a genetic test that revealed that Albright had a partial DPD deficiency.
In early 2014, Albright underwent surgery to remove tumors from her colon and liver, followed by three months of chemotherapy, which included a much lower dose of 5-FU. “She did real well with it,” said Allen, who adjusted the dose several times to minimize side effects. Her last treatment occurred in June 2014.
“Right now it looks good,” Allen said. Follow-up scans have shown no evidence of cancer anywhere in her body. Albright says she has recovered her most of her stamina and hopes to remain a cancer alumna for as long as possible.
The experience, she said, taught her to advocate for herself, something she found difficult. “I was surprised to discover how intimidated I was by the white coats and the [medical] language,” she said.