Last year, after a series of illnesses over several months (flu, cough, pneumonia, full-body rash), my husband seemed to be declining in his thinking and ability to find words. Our primary care physician ordered an MRI after she met with him for the rash and saw that he was mostly non-verbal, except for “yes” and “no.” To everyone's surprise, the MRI showed a large tumor in his left frontal lobe. They said, "Glioblastoma." We had no idea what that meant, honestly, but we did hear the words “brain cancer” and “highly aggressive.” Three weeks later, the tumor was removed, and when analyzed in the lab, it was not a glioma but a lymphoma, specifically a primary nervous system lymphoma (PCNSL). We were told that if you had to have brain cancer, a lymphoma was the kind to have because it could be "cured."
My husband came home after the tumor resection, quite verbal and happy. He was scheduled to start chemotherapy two weeks later to clean up the bits of the tumor the neurosurgeon had left purposefully behind (the remnants of the tumor were located in areas that might have been damaging to his brain if the neurosurgeon had tried to remove them). Just days before the scheduled start of chemo, he developed an infection at the surgical site. A second surgery was done to clean up the infection, and the neurosurgeon wasn't hopeful about the outcome because the infection was on the brain. We spent the next month in the ICU, on the medical floor of the hospital, and at a rehabilitation hospital. He came home, and for a few weeks, all was well until he suffered several seizures. Back to the hospital, where they couldn't find anything specifically wrong, but they then decided to start chemo. In the time from the original tumor resection to the start of chemo (about six weeks), the little remaining bits of tumor had grown by 20%.
Chemotherapy was a methotrexate and rituximab cocktail, which is standard treatment for primary central nervous system lymphomas, and after the first three treatments, an MRI showed that the tumor bits were significantly reduced in size. After the final three treatments, the tumor bits had actually grown, not much, but larger, and while on chemotherapy. The next treatment plan was ibrutinib, a twice-a-day pill that my husband started in January of this year. The first month of treatment was simple, and an MRI showed "nearly complete response," or remission, in other words. We were thrilled. But because cancer is cancer, the second MRI, six weeks later, showed that the tumor had radically increased in size! My husband had no symptoms of the rapidly growing lymphoma because it was sitting in the cavity that was left after the much larger tumor was originally removed. However, it was sitting there, growing unbounded.
New treatment plan. Currently, he's taking an infusion of Keytruda® every 21 days, and he's scheduled for the third one soon. An MRI is scheduled after that infusion. Fortunately, or unfortunately, I am now able to understand the radiologist's report of the MRI and often have an idea of what's happening before we meet with the oncologists. And I find myself anticipating a negative result each time, given the past results.
The good news is that my husband has had very few side effects from any of these treatments. He has developed a rash from the ibrutinib (a known side effect), and his balance is a little off, but those are manageable and don't really get in his way. The bad news is that the lymphoma, two surgeries, seizures, and high-dose methotrexate have left his brain a little scrambled. His short-term memory has suffered, and likely, he will not return to his original cognitive baseline. The other potential bad news is that we don't know what the next treatment would be if this one doesn't work, or if there are any. Because of his age (73), the oncologists are hesitant to try other treatments like stem cell transplant or CAR-T cell transplant.
So, we wait. Our lives are now lived in three-week cycles, and waiting for MRI results. My husband is happy, says he feels good, and is generally in good health. Those are the small blessings that keep us/me going.
Through all of this, we've had the most excellent nurse navigator from Blood Cancer United, who has helped identify clinical trials, provided us with reading materials, and offered us advice and a shoulder to cry on when we've needed it (okay, mostly when I've needed it). When we started this journey, we had no idea what resources were out there for us, but Blood Cancer United has been amazing. We have struggled to find information specifically related to PCNSL because it is a rare disease, but the broad information has been invaluable.
Story provided by spouse
Mike
primary nervous system lymphoma (PCNSL) (in memory)
