In the hours before her life was upended, Megan Freedman had attended a memorable business dinner surrounded by cherished colleagues at a trendy restaurant in Santa Monica, California, overlooking the Pacific Ocean.
“We’d had the most fun,” recalled Freedman, the owner of a New York City fashion showroom who was in Los Angeles in October 2019 to meet with her designers and buyers for national retailers. Perched on a wall outside the restaurant after dinner, Freedman was chatting as she and her friends waited for their cars. When she stood up, her left leg suddenly buckled and she fell. “I wasn’t drunk,” she said. “I just went boink and someone had to lift me up.”
The next morning, Freedman awoke to stabbing pain in her partially numb leg. “I was 100 percent sure I had a herniated disk,” she recalled. Freedman had spent the previous few days “lifting a ton of boxes and schlepping” heavy suitcases heaped with clothing samples to meetings. A few years earlier she had suffered a similar pain in her left arm caused by two herniated disks in her neck.
“I figured I had sciatica,” she said, referring to the pain that radiates down the leg and is often caused by a bone spur or disk that presses on the sciatic nerve.
Freedman would spend the next eight weeks bouncing between doctors’ offices and emergency rooms in Los Angeles and Manhattan in what she described as “ridiculous” pain. Her leg was often so weak she had to hoist it with her hands.
In December 2019, hours before her discharge from a New York hospital after a nine-day stay, Freedman learned she had a serious illness that had developed seemingly without warning.
“I come from a family full of heart disease and cancer,” said Freedman, 54. “I never expected this.”
Crying on the plane
Alarmed by the intensity of her pain and difficulty walking, the friend with whom Freedman was staying drove her to the emergency room of a Los Angeles teaching hospital. Doctors there also suspected sciatica and gave her the narcotic painkiller Dilaudid. If she wasn’t better in three days, they advised, she should come back.
Freedman did not improve and fell several times. She returned to the ER and underwent an MRI scan of her lower spine, which found nothing concerning. Doctors prescribed a stronger opioid, which she said barely touched the pain. Two days later she flew back to New York. “I just sat there crying on the plane,” Freedman recalled.
She consulted the Manhattan orthopedist she had seen for her neck. He reviewed the MRI and took X-rays. Unable to determine what might be wrong, he referred Freedman to a spine specialist. The specialist was perplexed, as was a second spine orthopedist. The latter gave her the first of two epidural steroid injections in her lower back; when neither alleviated the pain, he advised that she see a neurologist.
Freedman remembers feeling a creeping sense of desperation about her unrelenting pain and the absence of an explanation.
The neurologist ordered an electromyography test and a nerve conduction study to check the functioning of nerves and muscles in Freedman’s leg. The results seemed to indicate that the pain was emanating from the front of her pelvis, not her spine. Concerned that she might have a blood clot or peripheral artery disease, in which veins become narrowed because of a buildup of plaque that restricts blood flow, he ordered an ultrasound of her left leg up to the groin along with a CTA, a scan that inspects blood vessels for abnormalities.
On a Sunday morning several days before the tests were scheduled, Freedman headed for the ER of the Manhattan teaching hospital with which the neurologist is affiliated.
“I just couldn’t take the pain any longer,” she recalled. After waiting several hours she was taken to an exam bay where she said a young physician told her the ER staff had “bigger fish to fry” and recommended she head home. Freedman isn’t sure what prompted his remarks, but said he appeared to believe she had sciatica and needed to rest.
“That was the worst moment,” she said, weeping at the memory. “Being told there’s really nothing wrong with you and you should just go home. I felt so humiliated.”
Soon afterward, Freedman went to the ER at Mount Sinai, the teaching hospital where she had been seeing a neurologist for several years to treat chronic migraines.
The reception was different. Doctors admitted her and teams from various specialties — oncology, neurology, endocrinology, and rheumatology — began ordering tests in an attempt to figure out what was wrong with Freedman, who had acquired a nickname: “the weird leg lady.”
Initially, doctors zeroed in on a mass on her ovary described as “concerning”; it was ultimately deemed benign. A “highly suspicious” thyroid nodule was similarly dismissed. Doctors noted that Freedman’s leg strength improved after a course of steroids and that she was able to walk, although her pain was persistent and severe. Sciatica was again ruled out; scans showed only mild spinal degeneration.
But Freedman’s EMG and nerve conduction studies were abnormal, and an MRI showed inflammation in her left femoral nerve, one of the largest nerves in the leg, which controls movement and senses pain.
Doctors suspected that her history of thyroid dysfunction and her improvement on steroids suggested an autoimmune disease in which the body mistakenly attacks itself; they began running down the possibilities. A positive blood test for PR3, which detects antineutrophil cytoplasmic antibodies (ANCA), a protein that mistakenly attacks healthy white blood cells, greatly narrowed the options.
On the day of Freedman’s discharge, a team of rheumatologists trooped into her room to tell her they believed she had granulomatosis with polyangiitis (GPA), formerly known as Wegener’s disease. GPA is a form of vasculitis — inflammation of the blood vessels — that can damage organs. The disease often affects the kidneys, lungs, and sinuses.
GPA, which mimics an infection, can occur suddenly or develop over weeks or months. Severity and symptoms differ depending on which organ is involved. Treatment includes high doses of corticosteroids, usually along with other powerful medications to calm the immune system. If treated early, full recovery is possible; without treatment, GPA can be fatal.
An ‘atypical presentation’
At the beginning of his career in the early 1970s, Anthony Fauci, the veteran director of the National Institute of Allergy and Infectious Diseases, and his colleagues delineated the mechanism of the illness, which at the time killed most people within two years. Fauci also helped devise drug treatment for GPA that is 95% effective.
In Freedman’s case, the disease attacked her femoral nerve. “It was a somewhat atypical presentation,” said Weiwei (Wendy) Chi, the Mount Sinai rheumatologist who has treated Freedman since shortly after her diagnosis. Freedman also had a history of sinusitis and nosebleeds, which can be early signs of GPA.
She immediately began taking high doses of steroids, which improved the ability of her leg to function but did nothing to blunt the pain; the damage to her femoral nerve is probably permanent. None of the medications typically used to treat her pain have worked, Chi said, so “she’s on opiates for the time being.”
“The most confusing part of her case is the persistent pain,” which remains severe and undiminished, Chi observed. “I hate giving people chronic opioids but we’ve tried so much else and none of it really helped.”
The 2 1/2 years following Freedman’s diagnosis have been rough. She was hospitalized several times for acute pancreatitis, a serious and painful inflammation of the pancreas often caused by gallstones. She contracted the coronavirus from her hospital roommate during one stay. In May 2021, Freedman underwent surgery to remove her gallbladder.
Her business of 20 years imploded as a result of the pandemic. Freedman said that she was forced to shutter her showroom and lay off her five employees; she now works from home.
Because her life depends on a regimen of drugs that suppress the immune system, the coronavirus poses a heightened risk. She received virtually no protection from the first two vaccine doses because her body did not make antibodies.
As the omicron wave swept through New York City in late 2021, Freedman decided she could not risk living there with her family, which includes a son in high school. She decamped to a small city in California’s Coachella Valley near her brother, returning to New York two months ago. (She got COVID-19 anyway in January.)
In California, she received injections of Evusheld, an experimental medication approved for immunocompromised people. Doctors hope that she developed antibodies after a dose of a different coronavirus vaccine administered in April, after her return to New York.
Before GPA, her health had been good. “This disease is frightening and has really kicked my butt,” she said. “Not many people know much about it, and many who have it have very severe cases.”
Freedman “has been relatively stable for the last three years,” said Chi, who characterized the severity of her illness as “in the middle. She doesn’t have life-threatening organ disease like rapid kidney failure.”
“In the most severe forms people can be totally healthy one day and in the ICU the next,” the rheumatologist added. “It’s such an unpredictable disease.”