Don Brown remembers the extreme fatigue he felt on a ski trip 17 years ago, just before a physician diagnosed him with Waldenstrom's macroglobulinemia, a rare B-cell, non-Hodgkin's lymphoma for which there is no cure.
The 75-year-old from suburban Chicago told Medscape Medical News that an exam shortly after that trip showed that his blood viscosity was at least three times what it should be.
"Blood was pumping like ketchup through my veins," he said. "I could have had a stroke and died on the mountain."
Brown had felt worn down for 2 years before he consulted a sleep specialist, who referred him to a hematologist–oncologist. A bone marrow biopsy in 2002 confirmed that he had Waldenstrom's, and that the slowly progressive disease — which only affects around 1500 new patients a year in the United States — was already at an advanced stage. He did not expect to live out the year after his diagnosis.
But 17 years later, after "162 injections of five major drugs in 11 rounds of chemotherapy," he's still here. His last infusion of the chemotherapy drug bendamustine was a year and a half ago, he reported.
Brown and his wife, Mary, will discuss their experience with his cancer at the upcoming International Waldenstrom's Macroglobulinemia Foundation (IWMF) 2019 Forum in Philadelphia.
The avid skier and tennis player said he has rarely missed a tennis match since his diagnosis and he has not let his disease affect his love of skiing. In April, he checked off an item on his bucket list when he skied the black-diamond trails in Steamboat Springs, Colorado, with 11 members of his family.
The disease has meant changes, he acknowledged. He wears a mask on airplanes and fiercely protects his compromised immune system in public places. Family members know to stay away when they are ill.
But, more important, it has made him more grateful for his life and his family, Brown explained. And it has deepened his Christian faith, an experience he says is inextricable from his medical journey.
"Good medical care probably saved my life. But my great healer is the Lord," Brown said. "It's like having an orchestra leader. You need all the first violinists, but the orchestra conductor brings it all together."
Carl Harrington, from Philadelphia, was diagnosed after a retinal exam found bulging capillaries in his eyes when he was 56 years old. This led to an eventual referral to a hematologist–oncologist and a bone marrow biopsy that identified cells signaling Waldenstrom's macroglobulinemia.
Because he didn't have symptoms and the only markers were blood tests that indicated abnormal immunoglobulin M levels, his doctors advised that he not start treatment until he was symptomatic.
This is tough news to hear after a cancer diagnosis, Harrington told Medscape Medical News, especially because his father had died from lung cancer.
Waiting for symptoms feels like the "sword of Damocles hanging over my head," but he said he is glad that both treatments and life expectancy are improving while he waits.
"There may be some time in the future when the treatments are benign enough that we won't wait," he said.
Now, at 69, Harrington is president of the IWMF, a professional organization that has grown from 21 members in 1994 to 21,000 members, with affiliates in 18 countries. There are no dues and the organization is run by volunteers.
Harrington wants people newly diagnosed to know they are not alone and that connecting with the IWMF can lead them to experts, support groups, and the most comprehensive and up-to-date information on the disease.
"We all remember what it was like to get the news that you've got cancer," he explained, and especially that "it's incurable and it's rare."
Pain in Hip Led to Unexpected Diagnosis
Lisa Wise, also from Philadelphia, was diagnosed in 2010 after she went to see a rheumatologist for hip pain. The pain was resolved with a cortisone shot but bloodwork showed something her rheumatologist never expected to find: signs pointing to Waldenstrom's macroglobulinemia. The rheumatologist repeated the bloodwork to make sure.
The now 53-year-old Wise is a third-generation B-cell lymphoma patient. Her mother and her maternal grandfather both died at age 59 from B-cell lymphoma, so she was familiar with the type of cancer but had never heard of Waldenstrom's macroglobulinemia.
"The only symptom I had had was anemia and I didn't know a woman who didn't have that at that time of life," she told Medscape Medical News.
Like Harrington, Wise was on a watch-and-wait strategy for 7 years. Then her spleen enlarged and nodes developed that generated fluid in her lungs, indicating that it was time to start treatment. She finished six cycles of chemotherapy administered over 8 months in 2017.
Now her energy levels are "off the charts" positive, she said.
Wise currently chairs 35 support groups across the nation and coordinates 45 lifeline volunteers — patients and caregivers who volunteer to talk to others. She is trying to build a national community, a tall order because people with Waldenstrom's macroglobulinemia often do not know any other people with the disease.
She herself talks to patients every day, often trying to help them reverse the belief that they have to fight the disease aggressively.
The goal is "to do as little as you can as long as you can," she explained. "You want to keep your magic bullets in your pocket until you need them." She also wants to make sure that patients understand that there is hope.
"Getting a Waldenstrom's diagnosis is not a death sentence," said Wise. "The disease manifests itself differently in every single person, and the important thing is to connect with a Waldenstrom-expert physician who will be able to work with you to figure out what is best for you."
Brown, Harrington, and Wise have disclosed no relevant financial relationships.
International Waldenstrom's Macroglobulinemia Foundation (IWMF) 2019 Forum. To be presented June 6 (Wise) and June 7 (Brown and Harrington), 2019.
Medscape Medical News © 2019
Cite this: Patients With Rare Lymphoma Describe Divergent Experiences - Medscape - May 30, 2019.