During a two-week rafting trip through the Grand Canyon, Steve Kramer’s neck and feet became stiff, making it hard to take in panoramic views of majestic cliffs and hike to campsites.
After he returned home to New Jersey, the normally healthy 55-year-old started to feel pain and swelling in his shoulders and hands. Rolling over in bed hurt so much, he could barely sleep. At a hotel, tightly tucked sheets made even going to bed a challenge.
“I couldn’t untuck them,” he said. “I didn’t have the strength.”
He began to lose weight, and purple blotches appeared on his ankles. His doctor tested him for Lyme disease, hepatitis and other infections that might explain his symptoms, but the results were negative.
A rheumatologist — a doctor who specializes in autoimmune disorders, when the body mistakenly attacks its own healthy tissue — suspected Kramer had rheumatoid arthritis. But he didn’t have two key blood markers for the disease, and he didn’t respond to treatments for it.
Subtle changes in an often-overlooked part of the body eventually led to the diagnosis of a life-threatening condition. But Kramer’s symptoms were atypical, puzzling specialists who saw him, until one doctor made an additional diagnosis of a rare syndrome that helped explain what was going on.
“When symptoms or signs arise that don’t fit the diagnosis you’re treating … you really need to reconsider your assumptions,” said Jason Liebowitz, the rheumatologist who treated Kramer.
Stiff joints
Kramer, now 59, has long stayed active and fit — running, playing tennis and softball, going scuba diving and breeding a little-known type of sled dog called the Chinook. He had never been hospitalized.
In June 2022, some of his friends planned a rafting trip. When someone else canceled at the last minute, Kramer decided to join them. The Grand Canyon scenery was magnificent, just as when he went on a similar trip in 1997. But this time his body began to ache. He figured his neck was stiff from sleeping under the stars. Maybe the rigidity in his feet was plantar fasciitis, or inflammation on the bottom of the foot that runners often face.
The next month, he contracted covid-19, despite having been vaccinated twice the year before. After recovering, he saw his primary care provider in New Jersey for the joint pain that now affected his whole body. Blood tests revealed no culprit, and the provider referred him to Liebowitz, the rheumatologist.
At their first visit in September 2022, Liebowitz recalls, Kramer wore Teva sandals with straps. “His feet were overflowing,” said the doctor, who now works with Columbia University in New York. “The swelling was really pronounced.”
Liebowitz ordered blood tests, which ruled out autoimmune conditions such as lupus and scleroderma. Kramer’s symptoms seemed typical of rheumatoid arthritis, which irritates joints and eats away at bone, and he had high levels of two inflammatory markers associated with the condition. However, his tests for two key antibodies were negative.
Liebowitz figured Kramer had what’s known as seronegative rheumatoid arthritis, which accounts for up to 30 percent of rheumatoid arthritis cases. He started him on prednisone, a steroid that reduces inflammation and tamps down the immune system. It eased Kramer’s swelling a bit.
But a month later, when Liebowitz tapered the prednisone and started Kramer on methotrexate, a first-line drug for rheumatoid arthritis, his symptoms got worse. On a business trip, walking became so agonizing that Kramer had to use a wheelchair to navigate the airport. Liebowitz switched Kramer to newer drugs for rheumatoid arthritis — Humira and then Actemra — but he still didn’t improve.
“I was missing something,” Liebowitz said.
Clue to a new diagnosis
At their next visit, in January 2023, Liebowitz examined Kramer thoroughly and discovered something he hadn’t noticed before: tiny black lines in the beds of his fingernails and toenails. The marks were splinter hemorrhages, evidence of bleeding that can be caused by certain infections, drug reactions and abnormal proteins seen in some cancers.
Kramer has a family history of cancer. His mother died, at age 60, of non-Hodgkin’s lymphoma, a type of blood cancer, in 1996. Lung cancer took the life of his father, a nonsmoker, at age 70, in 2007. Liebowitz ordered more blood tests, which showed a protein pattern suggesting blood cancer.
Liebowitz referred Kramer to a hematologist-oncologist, or blood cancer specialist. That doctor did a bone marrow biopsy, collecting spongy tissue from Kramer’s hip through a needle. The test showed that Kramer had a high concentration of identically abnormal plasma cells, white blood cells that fight infection.
While Kramer was driving home from work one day in March 2023, the doctor called to tell him he had multiple myeloma, a cancer of plasma cells that affects about 36,000 Americans a year. Kramer thought about his wife’s mother, who died of the disease in 2015, at age 72, nine months after she was diagnosed. About 62 percent of people with multiple myeloma are alive five years after diagnosis, according to the Cleveland Clinic. That means 38 percent don’t live that long.
“Everyone says, ‘Don’t look up the survival statistics,’” Kramer said. “So, of course, that’s the first thing you do.”
But Kramer didn’t have the main symptoms of multiple myeloma: calcium elevation in the blood; renal failure, or kidney problems; anemia, or low red blood cell counts; and bone disease, such as fractures or lesions, which typically cause deep bone pain. He only had mild anemia, and initial scans showed no bone lesions. He wasn’t experiencing unusual infections, nausea, thirst or confusion, other symptoms associated with the disease.
Still, the hematologist-oncologist started him on a four-drug chemotherapy regimen for multiple myeloma and said he should see a myeloma specialist to guide further treatment.
A rare syndrome
In April 2023, Kramer visited Cesar Rodriguez, a specialist in myeloma and other plasma cell disorders at Mount Sinai Hospital in New York. Rodriguez said he was struck by two things about Kramer: he had swelling that was unusual in multiple myeloma, and a new PET scan showed bone lesions marked by bone overgrowth, not bone loss as is typical with the disease.
“That caught my attention,” Rodriguez said. He ordered a blood test that showed Kramer had a high level of VEGF, a growth factor involved in forming blood vessels.
Based on those findings, Rodriguez determined that Kramer, in addition to having multiple myeloma, had a syndrome known as POEMS, another rare plasma cell disorder. The acronym stands for five features:
- Polyneuropathy, or joint pain and weakness.
- Organomegaly, an enlarged spleen, liver or lymph nodes.
- Endocrinopathy, or abnormal hormone levels.
- Monoclonal protein, from abnormal plasma cells.
- Skin changes.
Kramer didn’t have the O or the E, but he had the other features, plus the high VEGF level, qualifying him for the diagnosis, Rodriguez said. POEMS and regular multiple myeloma are treated in a similar way: with chemotherapy and a stem cell transplant. But the POEMS diagnosis helped explain why Kramer’s cancer had been hard to catch and assisted doctors in monitoring side effects of the cancer treatments, Rodriguez said.
The day after his first chemotherapy treatment, Kramer felt better. Three weeks later, he was walking 10,000 steps a day, though with some limping, while attending the French Quarter Festival in New Orleans.
By that summer, tests showed his abnormal plasma cells and skin changes were mostly gone. He and his wife, Kim, went on a cruise in Greece that September, swimming and hiking around the Cyclades islands. “I felt normal again,” he said.
In October 2023, Kramer underwent a stem cell transplant at Mount Sinai. Rodriguez gave him a high dose of melphalan, a potent chemo drug, to kill any cancerous plasma cells that remained. The treatment wipes out nearly all bone marrow, including healthy blood cells needed to replenish the immune system. As a rescue, Kramer received an infusion of his own stem cells, which had been collected in late July. Over two weeks in the hospital, amid nausea and hair loss from the melphalan, Kramer’s immune system gradually returned, and he began to feel better again.
It’s possible some of Kramer’s abnormal plasma cells were collected with his stem cells. But plasma cells rarely survive the freezing and thawing process before the collected cells are returned to the patient in the transplant, Rodriguez said.
Today, Kramer is in remission and grateful that doctors caught his unusual cancer early enough to keep it in check. He still takes one of the four chemo drugs, a daily pill, but is expected to go off it late this year. His left ankle still stiffens up sometimes when he exercises, but he doesn’t focus much on that.
“Is that from the disease, the treatment or old age? It’s likely all three,” he said. “But I went through something pretty severe here and came out on the other side. I celebrate that.”
David Wahlberg has been a medical reporter for 30 years, including at the Atlanta Journal-Constitution and the Wisconsin State Journal in Madison.
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