Medical Mystery: She was overcome by searing leg pain that struck without warning

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Medical Mystery: She was overcome by searing leg pain that struck without warning

Seeking relief, one fashion executive despaired that there was no explanation

(Cam Cottrill for the Washington Post)

In the hours before her life was turned upside down, Megan Freedman had attended a memorable business lunch at a trendy restaurant in Santa Monica, California overlooking the Pacific Ocean, surrounded by esteemed colleagues.

“We had the most fun,” recalled Freedman, the owner of a fashion showroom in New York City, who was in Los Angeles in October 2019 to meet with her designers and buyers for national retailers. Freedman sat against a wall outside the restaurant after dinner and chatted while she and her friends waited for their cars. As she stood up, her left leg suddenly buckled and she fell. “I wasn’t drunk,” she said. “I just ran away and someone had to pick me up.”

The next morning, Freedman awoke with shooting pains in her partially numb leg. “I was 100 percent sure I had a herniated disc,” she recalls. Freedman had spent the last few days “lifting a ton of boxes and” lugging heavy suitcases full of clothing samples to meetings. A few years earlier she had similar pain in her left arm caused by two herniated discs in her neck.

“I thought I had sciatica,” she said, referring to the pain that radiates down the leg and is often caused by a bone spur or disc pressing on the sciatic nerve.

Freedman spent the next eight weeks bouncing between doctor’s offices and emergency rooms in Los Angeles and Manhattan in what she described as “ridiculous” pain. Her leg was often so weak that she had to pull it up with her hands.

In December 2019, hours before she was discharged from a New York City hospital after a nine-day stay, Freedman learned she was suffering from a serious illness that had seemingly developed without warning.

“I come from a family of heart disease and cancer,” said Freedman, 54. “I never expected it.”

Concerned about 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 doesn’t feel better in three days, they advised her to come back.

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Freedman did not improve and fell several times. She returned to the emergency room and underwent an MRI scan of her lower spine, which revealed nothing of concern. 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 and cried on the plane,” Freedman recalled.

She consulted the orthopedist in Manhattan she had been to about her neck. He checked the MRI and took x-rays. Unable to determine what might be wrong, he referred Freedman to a spine specialist. The specialist was at a loss, as was a second spinal orthopedist. The latter gave her the first of two epidural steroid injections in her lower back; When neither of them eased the pain, he advised her to see a neurologist.

Freedman recalls feeling a creeping sense of despair at her unrelenting pain and lack of explanation.

The neurologist ordered an electromyography test and nerve conduction study to check nerve and muscle function in Freedman’s leg. The results seemed to indicate that the pain originated in the front of her pelvis, not her spine. Concerned that she might have a blood clot or peripheral artery disease, in which the veins narrow due to a buildup of plaque restricting blood flow, he ordered an ultrasound of her left leg up to the groin, along with a CTA, a scan who examines blood vessels for abnormalities.

On a Sunday morning, a few days before the tests were scheduled, Freedman went to the emergency room at the Manhattan Teaching Hospital, where the neurologist is affiliated.

“I just couldn’t take the pain anymore,” she recalls. After waiting several hours, she was taken to an examination bay, where a young doctor told her the ER staff had “bigger fish to fry” and advised her to go home. Freedman isn’t sure what prompted his remarks, but said he appears to think she has sciatica and needs to rest.

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“That was the worst moment,” she said, crying at the memory. “You were told that you really are fine and that you should just go home. I felt so humiliated.”

Soon after, Freedman went to the emergency room 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 different specialties — oncology, neurology, endocrinology, and rheumatology — began ordering tests to find out what was wrong with Freedman, who’d earned a nickname: “the odd leg lady.”

Initially, doctors focused on a mass on her ovary that was described as “worrying”; it was ultimately considered benign. A “highly suspicious” thyroid nodule was also dismissed. Doctors found 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 ruled out again; Scans showed only mild spinal degeneration.

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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 that controls movement and senses pain.

Doctors suspected that her history of thyroid dysfunction and improvement on steroids suggested an autoimmune disorder, in which the body mistakenly attacks itself; they began to assess the possibilities. A positive blood test for PR3, which detects antineutrophil cytoplasmic antibodies (ANCA), a protein that mistakenly attacks healthy white blood cells, severely narrowed the options.

On the day of Freedman’s discharge, a team of rheumatologists rushed to 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 blood vessels – that can damage organs. The disease commonly affects the kidneys, lungs, and sinuses.

GPA, which mimics an infection, can appear suddenly or develop over weeks or months. Severity and symptoms differ depending on which organ is affected. Treatment involves high doses of corticosteroids, usually along with other strong drugs to calm the immune system. With early treatment, full recovery is possible; without treatment, GPA can be fatal.

An “atypical presentation”

Early in his career in the early 1970s, Anthony S. Fauci, longtime director of the National Institute of Allergy and Infectious Diseases, and his colleagues described the mechanism of the disease, which was then killing most people within two years. Fauci also helped develop a drug treatment for GPA that is 95 percent effective.

In Freedman’s case, the disease attacked her femoral nerve. “It was a somewhat atypical presentation,” said Weiwei (Wendy) Chi, the rheumatologist at Mount Sinai who treated Freedman 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 her leg’s ability to function but did not relieve the pain; The damage to her femoral nerve is likely permanent. None of the medications typically used to treat her pain have worked, Chi said, so “she’s on opiates for now.”

“The most confusing part of her case is the ongoing pain,” which remains severe and unabated, Chi noted. “I hate giving people chronic opioids, but we’ve tried so many other things and none of them really helped.”

“I was just scratching myself.” A retired nurse who couldn’t stop scratching feared she was facing an organ transplant.

The 2 1/2 years after Freedman’s diagnosis were tough. She has been hospitalized several times for acute pancreatitis, a severe and painful inflammation of the pancreas often caused by gallstones. She contracted the coronavirus while staying with her roommate in the hospital. In May 2021, Freedman underwent surgery to remove her gallbladder.

Their 20-year business imploded as a result of the pandemic. Freedman said she was forced to close her showroom and lay off her five employees; She now works from home.

Because her life depends on treatment with drugs that suppress the immune system, the coronavirus poses an increased risk. The first two doses of the vaccine offered her virtually no protection because her body was not producing antibodies.

As the Omicron wave swept through New York City in late 2021, Freedman decided she couldn’t risk living there with her family, which includes a son in high school. She moved to a small town in California’s Coachella Valley near her brother and returned to New York two months ago. (She got Covid-19 in January anyway.)

In California, she received Evusheld injections, an experimental drug approved for use in immunocompromised people. Doctors hope she developed antibodies after a dose of another coronavirus vaccine given in April after she returned to New York.

Before GPA, her health had been good. “This disease is scary and it really kicked my ass,” 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 past three years,” said Chi, who described the severity of her illness as “intermediate.” She has no life-threatening organ disease like rapid renal failure.”

“In the most severe forms, people can be perfectly healthy one day and in intensive care the next,” added the rheumatologist. “It’s such an unpredictable disease.”

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