Searching for relief, a fashion executive became desperate over the lack of an explanation
The next morning, Freedman woke up with throbbing pain in her partially numb leg. “I was 100% sure I had a herniated disc,” she recalls. Freedman had spent the previous days “lifting a ton of boxes and carrying” heavy suitcases full of clothing samples to meetings. A few years earlier, she had suffered 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 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 that she had to lift it with her hands.
In December 2019, hours before she was released 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,” Freedman, 54, said. “I did not expect this.”
Alarmed by the intensity of her pain and her difficulty walking, the friend Freedman was staying with drove her to the emergency room of a University Hospital in Los Angeles. Doctors there also suspected sciatica and gave him Dilaudid, a narcotic painkiller. If she wasn’t better in three days, they advised, she should come back.
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Freedman did not improve and fell several times. She returned to the ER and underwent an MRI of her lower spine, which found nothing of concern. Doctors prescribed a stronger opioid, which she said barely touched the pain. Two days later, she flew 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 was wrong, he referred Freedman to a spine specialist. The specialist was perplexed, as was a second spine orthopaedist. The latter gave him the first of two epidural injections of steroids in the lower back; when neither alleviated the pain, he advised her to see a neurologist.
Freedman remembers feeling a creeping sense of despair over his relentless pain and lack of explanation.
The neurologist ordered an electromyography test and a nerve conduction study to check the functioning of the nerves and muscles in Freedman’s leg. The results seemed to indicate that the pain was emanating from the front of his pelvis, not his spine. Fearing she might have a blood clot or peripheral arterial disease, in which the veins narrow due to a buildup of plaque that restricts blood flow, he ordered an ultrasound of her left leg down to the groin. with a CTA, a scanner that inspects blood vessels for abnormalities.
On a Sunday morning several days before the scheduled test date, Freedman headed to the emergency room at Manhattan University Hospital with which 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 she said a young doctor told her ER staff had ‘bigger fish to fry’ and recommended she go home . Freedman isn’t sure what prompted her remarks, but said he seemed to believe she had sciatica and needed to rest.
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“It was the worst time,” she said, crying at the memory. “You are told that you really have nothing wrong and you should just go home. I felt so humiliated.
Soon after, Freedman checked into the emergency room at Mount Sinai, the university hospital where she had been seeing a neurologist for several years for chronic migraines.
The reception was different. Doctors admitted her, and teams from various specialties – oncology, neurology, endocrinology and rheumatology – began ordering tests to try to figure out what was wrong with Freedman, who had earned a nickname: “the lady with weird legs”.
Initially, doctors focused on a mass on her ovary described as “worrying”; it was ultimately ruled benign. A “highly suspicious” thyroid nodule was also ruled out. Doctors noted that Freedman’s leg strength had improved after a course of steroids and she was able to walk, although her pain was persistent and severe. Sciatica was again ruled out; 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 his left femoral nerve, one of the largest nerves in the leg, which controls movement and senses pain.
Doctors suspected that his history of thyroid dysfunction and his improvement on steroids suggested an autoimmune disease in which the body mistakenly attacks itself; they began to exhaust the possibilities. A positive blood test for PR3, which detects anti-neutrophil cytoplasmic antibodies (ANCA), a protein that mistakenly attacks healthy white blood cells, drastically narrowed the options.
On the day of Freedman’s discharge, a team of rheumatologists entered 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 – which can damage organs. The disease often affects the kidneys, lungs and sinuses.
GPA, which mimics an infection, can come on suddenly or develop over weeks or months. Severity and symptoms differ depending on the organ involved. Treatment includes high doses of corticosteroids, usually combined with other powerful drugs to calm the immune system. If treated early, full recovery is possible; without treatment, GPA can be fatal.
An “atypical presentation”
Early in his career in the early 1970s, Anthony S. Fauci, the veteran director of the National Institute of Allergy and Infectious Diseases, and his colleagues described the mechanism of the disease, which at the time killed most people in two years. Fauci also helped design a drug treatment for GPA that was 95% effective.
In Freedman’s case, the disease attacked his femoral nerve. “It was a somewhat atypical presentation,” said Weiwei (Wendy) Chi, the Mount Sinai rheumatologist who treated Freedman shortly after her diagnosis. Freedman also had a history of sinusitis and nosebleeds, which may be early signs of GPA.
She immediately started taking high doses of steroids, which improved her leg’s ability to function but did nothing to alleviate 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 at the moment.”
“The most confusing part of her case is the lingering pain,” which remains severe and unmitigated, Chi observed. “I hate giving people chronic opioids, but we’ve tried so many other things and none of it has really helped.”
“I was just scratching myself.” A retired nurse who kept scratching feared she was facing an organ transplant.
The two and a half years since Freedman’s diagnosis have been difficult. She was hospitalized several times for acute pancreatitis, a severe and painful inflammation of the pancreas often caused by gallstones. She contracted the coronavirus from her roommate in the hospital during a stay. In May 2021, Freedman underwent surgery to remove his gallbladder.
His 20-year-old 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 his life depends on a regimen of drugs that suppress the immune system, the coronavirus poses an increased risk. She received virtually no protection from the first two doses of the vaccine because her body was not making antibodies.
As the omicron wave swept through New York in late 2021, Freedman decided she couldn’t risk living there with her family, which includes a son in high school. She decamped to a small town in California’s Coachella Valley, near her brother, and returned to New York two months ago. (She still caught covid-19 in January.)
In California, she received injections of Evusheld, an experimental drug approved for immunocompromised people. Doctors hope she developed antibodies after a dose of another coronavirus vaccine given in April, after returning to New York.
Before GPA, his health was good. “This disease is scary and really kicked my ass,” she said. “Not many people know much about it, and many of those who have it have very severe cases.”
Freedman “has been relatively stable for the past three years,” said Chi, who rated the severity of his illness as “fair.” She has no life-threatening organic disease like rapid kidney 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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