Every year, thousands of patients who are on a successful treatment path for leukemia or other cancers are hit with another deadly obstacle: invasive fungal infections.
It’s a reality that Jessica Gauss lived through with her son Blake, now 7 years old, who defeated leukemia several years ago but continues to navigate the after-effects of a fungal infection that nearly took his life during chemotherapy.
Their story began in May of 2012, just a couple months shy of Blake’s second birthday.
“Blake wasn’t acting like himself and he was looking pale,” recalls Jessica, who lives in in upstate New York. “I took him to the doctor, and was referred to the children’s hospital right away.”
Doctors at the nearby University of Rochester Medical Center’s Golisano Children’s Hospital tested Blake’s blood and provided a near-immediate diagnosis of leukemia. Follow-up tests revealed that he had acute lyphocytic leukemia (ALL), which Jessica was relieved to learn is the most well-studied of childhood leukemia, with a five-year survival rate exceeding 85 percent.
“When you hear about kids with cancer, that’s the type you’ll often hear about,” she says.
The treatment protocol would require six to seven months of heavy chemotherapy, starting right away. The family was in shock, but moved ahead quickly to defeat the disease.
Blake adjusted well to his therapy routine, Jessica said. As long as he had his mom or dad to keep him company, and his toys to play with, he was happy and calm.
But five months into treatment, just as he was entering the most intense phase of chemotherapy, Blake hit a bump.
Doctors Discover a Hidden Threat
As the powerful medicine took hold, Blake’s immune system weakened—a reality for almost everyone who undergoes chemotherapy.
The drug works by obliterating fast-growing cancer cells. But in the process, it indiscriminately hits other fast-growing cells, including the white blood cells that serve as the body’s main defense against infection.
The problem is compounded for the estimated 173,000 U.S. patients every year like Blake, who are diagnosed with leukemia or other blood cancers such as lymphoma or myeloma. These cancers sap the body’s ability to produce immune cells at healthy levels.
When the immune system is down, fungal infections can strike hard. The fungi that exist in patients’ everyday environments—usually with no consequence—can turn deadly. It’s such a serious problem that patients with some types of blood cancers are given preventative doses of a potent antifungal medicine, voriconazole, during their chemotherapy treatment. Blake, however, was not.
In December 2013, Blake had just finished a high-dose regimen of steroids, which are commonly prescribed as part of cancer treatment to make chemotherapy more effective. But one downside of steroids, besides mood disturbances, is that they can mask symptoms of other serious conditions. Such was the case with Blake. The day after he finished his steroid regimen, he presented with a high fever and breathing problems.
Jessica drove him to the hospital, where doctors examined him for a week, attempting to determine the cause of his fever and neutropenia. First, they checked for pneumonia, but didn’t see anything significant. Then they administered a CT scan for a closer look at his lungs. It revealed a large mass that looked a lot like aspergillosis—an invasive and life-threatening infection caused by a mold that’s prevalent in the environment.
Jessica said she was shocked to hear that survival rate for Blake’s infection was a scant 30 percent for patients with blood cancers. It was more deadly than leukemia. “It was terrifying,” she said.
Blake’s clinical team quickly put him on a potent antifungal drug known as voriconazole. It’s one of the only antifungals available to treat invasive aspergillosis, but it has some significant drawbacks, including potential liver damage.
“After Christmas, we met with the surgeon to decide what we were going to do next,” Jessica recalls. “Aspergillus is an aggressive organism, and a drug-only route wouldn’t have been enough. To remove the fungus, we had to remove the entire lower lobe of his left lung—it was the only thing we could do to make sure it was all gone.”
Another Hurdle Emerges
Because the antifungal medicine and surgery was so taxing on his body, Blake had to stop chemotherapy treatment for several weeks. But finally, at the end of January, he was ready to begin again. “We figured he had enough healing time,” Jessica says.
During a routine examination of Blake’s spinal fluid before chemotherapy resumed, the doctors noticed something odd: an abnormally high level of white blood cells. “The first thing we thought is that the leukemia came back after our break in the treatment,” Jessica said. “Or maybe it was meningitis. We started looking for answers.”
CT scans of Blake’s spine and brain pointed to the culprit: another fungal mass, about the size of a plum, tucked up in his cerebellum, extremely close to his brain stem. Until now, all of the scans had focused on his chest and abdomen—yet a fungal mass could have been growing the whole time in his brain, Jessica said. “I’m still baffled that we had no idea,” she said, noting that Blake didn’t exhibit the symptoms you’d expect of someone who has so much pressure on his brain.
In early February, a neurosurgeon removed the fungus without any damage to Blake’s brain or spinal cord. It was a success. But Blake would remain on the strong antifungal medicine for years, as the risk of aspergillosis recurrence was just too high.
Antifungal Medicine Takes Its Toll
Blake continued to visit with clinicians regularly as the months and years progressed after his chemotherapy. He had defeated leukemia. And the fungal infection had not re-emerged. But two years later, he began to suffer from the effects of being on the antifungal drug for so long.
Antifungal drugs, historically, are tough on the body because fungal cells are so similar to human cells. Thus, a drug that targets fungi often will impact healthy human tissue.
“We started seeing a rise in his liver enzymes and bilirubin—above and beyond what was caused by his chemo drugs,” Jessica says. “We didn’t think there was much of a chance he had fungus hiding out anywhere anymore, so we got brave enough to stop the voriconazole. Neither the doctors nor us were comfortable with this decision, but it just got to a point where the drugs were too big of a load for Blake. We were asking so much of his tiny liver.”
Calling for a Less Damaging Antifungal
Today, Blake is a happy 7-year-old kid, and leukemia is a faint memory. But he still has liver and esophageal problems due to his prolonged use of voriconazole—and for these issues, he must make regular visits with doctor who monitors his liver enzymes.
“The drug helped to save his life; I have no doubt about that,” Jessica said. “But it would have been really nice if there was an option that wasn’t so hard on his little body, especially his liver. We really didn’t have a choice.”
Blake’s liver is healing, but he still bruises and bleeds easily due to the damage. And if he ever needs to be on a medication that significantly taxes his liver, he would face irreversible harm.
“Most people aren’t aware that fungal infections can happen to kids who are treated for cancer,” Jessica said. “We need better treatments.”