Having lived with multiple sclerosis for decades, Mary McIndoe was used to the fatigue that overcame her in the summer of 2011.
But when she kept feeling strange, and noticed a scrape on her knee wasn’t healing, her family urged her to see the doctor. Within hours of getting her bloodwork done, she was hospitalized at Medical College of Georgia Hospital and Clinics, then put through immediate aggressive treatment the next day.
McIndoe thinks that treatment saved her life from a rare and often fatal leukemia.
“If I had not been here when all of this happened, I wouldn’t have made it,” she said.
McIndoe had acute promyelocytic leukemia, and implementing a new protocol to treat it is the focus of a $1.67 million, five-year grant for Georgia Regents University Cancer Center to lead a five-center consortium across the Southeast to improve outcomes.
In the disease, genes on two chromosomes become translocated and can cause serious problems in bone marrow. The mutation “gives the cancer cells the proliferative advantage,” over the normal red and white blood cells and platelets that should be produced, said Dr. Anand Jillella, the associate cancer center director for clinical affairs and chief of hematology/oncology and bone marrow transplant.
In fact, “it disrupts the coagulation mechanism” in the body, Jillella said, and patients often hemorrhage, which can be especially bad in the brain and the lungs.
The disease hits very hard, very fast, he said. One recent patient began feeling bad on Thanksgiving weekend “and by Dec. 1 he was in the hospital,” Jillella said. “It is very quick. (The cancer) probably doubles and doubles very, very fast.”
McIndoe, in fact, had clean bloodwork at the end of May 2011 and her disease was life-threatening within a month.
Recently updated data from 2002-08 show the survival rate is 64 percent; Jillella said 30 percent of patients die within a month of diagnosis.
“I think it is unacceptable, totally unacceptable,” he said.
GRU’s cancer center learned that lesson the hard way, when seven of 19 APL patients it treated over four years died, Jillella said. The center decided to study the deaths and the literature, including hiring an independent consultant, and was able to narrow it down to three main causes, with bleeding accounting for seven of 10 deaths overall, he said. It also noticed a pattern to the deaths, with bleeding being the main cause in the first 7-10 days of diagnosis, followed by drug reactions in the next two weeks or so, and then infection after that because of the patient’s weakened immune system.
But the lengthy protocol for treating the rare disease was also a problem, Jillella said.
“It is a 15-page document that few people have the time to read and understand and comprehend, especially if you are seeing one patient every 10 years or never,” he said.
Jillella focused and prioritized the protocol down to an algorithm that is a page and a half, which could speed up not only diagnosis but especially the onset of treatment.
“We actually start the treatment even before we confirm the diagnosis,” he said. “We don’t wait. The worst thing that can happen is you could be wrong and you could stop (treatment) and change it to something else.”
Patients are given the front line drug all-trans-retinoic acid or ATRA and receive transfusions to help prevent the bleeding. Because ATRA can cause fluid buildup in the body, which could cause shortness of breath or worse in the lungs, patients are carefully monitored.
“What we do is we meticulously weigh them every day, in fact, twice a day,” Jillella said. Patients also get antibiotics to help prevent infection, in addition to the chemotherapy.
GRU Cancer Center is teaming up with the cancer centers at Emory University and Medical University of South Carolina, plus Northside Hospital in Atlanta and Upstate Oncology Associates in Greenville, S.C. Between those five centers, they will see about half the APL patients in the region, but more importantly they can be a resource for the outlying physicians who encounter cases.
The cancer affects about one in 250,000 people in the U.S., according to the National Institutes of Health, so consulting with someone who has experience with it is crucial for patient survival, Jillella said.
McIndoe knows firsthand the impact of that personal touch. She was diagnosed and hospitalized July 1, and despite the holiday weekend, Jillella and his team were there to oversee her treatment.
“I have no doubt, if it wasn’t for him, I wouldn’t be here right now,” she said.