“It felt as though an alien had invaded my body,” said Kennedy, formerly a manager at the National Geospatial-Intelligence Agency. “I felt like I was in doctors’ offices all the time.”
Tests for possible ailments — including blood disorders, cancer, multiple sclerosis and rheumatoid arthritis — were negative. For seven years. Kennedy and her primary-care physician, who said she felt as though she sent Kennedy to “every specialist that walked,” had no clear idea what might be wrong.
But during a physical in January 2012, her doctor, Melissa Johnson, struck by Kennedy’s trouble walking and her accelerating deterioration, decided to check for a condition not previously considered.
“I’m not sure what made me order the test,” recalled Johnson, who practices in a St. Louis suburb. “But at this point I had known her for seven years and I really felt that something just wasn’t right.”
Growing up in Wise County, in Virginia’s remote, mountainous coal-mining region, Kennedy said that her robust immune system was often remarked upon by her relatives, who contracted the usual, mostly routine, illnesses.
“I volunteered in schools and at a nursing home, and I almost never got sick — not even a cold,” Kennedy recalled. As an adult, she was strong and enjoyed chopping wood.
Days after moving to Missouri, she met Johnson, a family physician who became her doctor after she sought treatment for a skin infection. Johnson flagged Kennedy’s elevated white blood cell count, which remained slightly too high even after the infection had cleared. An elevated white count has a multitude of causes, ranging from stress to cancer.
Johnson decided to watch Kennedy’s counts over the next six months. “They were never really high enough” to become alarming, the doctor said. But in 2006, noting Kennedy’s new symptoms — a dry feeling in her throat, sporadic heart palpitations and intestinal problems — she sent Kennedy to a hematologist for a closer look.
The blood specialist ordered sophisticated blood tests, which ruled out myelodysplastic syndrome, a rare condition that often precedes leukemia, as well as lymphoma, another cancer that affects blood cells, and found nothing amiss. The specialist said, “ ‘Yes, the white blood count is elevated, but we’re not going to worry about it,’ ” Johnson recalled.
Kennedy was relieved, but her symptoms proliferated. She was given a diagnosis of adult-onset asthma and began experiencing odd events she called “spells,” lasting several minutes, in which she was temporarily unable to focus or function.
An MRI scan of Kennedy’s brain suggested a possible ominous cause of the spells: white matter lesions that might indicate multiple sclerosis, a neurological disease that affects the brain and spinal cord. Such lesions can also indicate high blood pressure, which Kennedy had, or migraine headaches, which had not been a problem.
In 2007, Kennedy saw three neurologists, each of whom ruled out MS. One suggested she had migraines.Another told her she needed to see a psychologist after Kennedy handed him a piece of paper detailing her increasingly complicated and confusing medical history.
“You’re just looking for a diagnosis,” she recalled him saying accusingly.
“Right,” Kennedy responded. “That’s what I’m paying you for.”
A third neurologist ultimately determined she had epilepsy and prescribed medication to control the spells, which were actually seizures. Johnson said she expected that once the seizure drugs were fine-tuned, her patient would start to feel better.
But after the seizures were under control, Kennedy developed recurrent sinus infections.
Johnson at first attributed them to Missouri’s climate. “Her symptoms — ear pain, headaches, sinus pain and pressure — well, a lot of people have that,” Johnson noted. “But her infections were difficult to treat and wouldn’t respond to antibiotics.”
An ear, nose and throat specialist prescribed nasal sprays, which didn’t help. And Kennedy noted that she never had a runny nose. “Everything just felt very dry,” she said.
Johnson said she didn’t doubt that Kennedy’s problems were genuine, but wasn’t sure what was causing some of them or whether they were connected. “She wasn’t a patient who was coming to me with 30 problems and wanted 30 pills,” she said. Kennedy’s mushrooming list of symptoms continued to muddy the diagnostic picture.
By the end of 2011, Kennedy said, she felt increasingly desperate. At times she was worried she might be dying because “so much seemed wrong” and no doctor could pinpoint why she felt so crummy. Although she looked fairly healthy, she had missed days of work and her co-workers and boss kept saying pointedly, “Didn’t you just
go to the doctor?”
In January 2012, as part of a New Year’s resolution to improve her health, she made an appointment for an annual physical with Johnson.
At that point, Kennedy was having serious trouble walking because of joint pain. Several years earlier, she had developed osteoarthritis, a common and slowly progressive joint disease that typically surfaces in middle age, but her mobility problems had worsened dramatically in the previous few months. “I was so stiff it would take me 90 minutes to get ready in the morning, where it used to take me 30.”
She told Johnson her body hurt “from the top of my head to the tips of my toes.” Her mouth and eyes, she said, felt constantly dry, and she was exhausted.
During an hour-long appointment, Kennedy described her pain, and she and Johnson reviewed her family history of arthritis: An aunt has rheumatoid arthritis (RA), and her father has severe osteoarthritis. Johnson, who had suspected Kennedy might be suffering from an autoimmune disorder, a constellation of diseases in which the body mistakenly attacks its own tissues, ordered another test for RA, although previous screens had been negative. This time, she added tests for an autoimmune disorder that often accompanies RA, Sjogren’s syndrome, for which Kennedy had never been checked.
The results of those tests provided the answer that had eluded Johnson and Kennedy. Her joint pain, dryness, chronic sinus infections and fatigue were the result of primary Sjogren’s syndrome; tests for RA were negative.
A chronic and uncommon disorder, Sjogren’s typically develops in women after 40, when white blood cells attack moisture-producing glands in the eyes and mouth, causing eye infections and dryness that can lead to difficulty swallowing. Chronic sinusitis and joint pain are also common, according to the Bethesda-based Sjogren’s Syndrome Foundation, an education and advocacy group.
Between 400,000 and 3 million American adults are estimated to have Sjogren’s; among them is tennis champion Venus Williams, who received a diagnosis in 2011. Sjogren’s may be triggered by an infection; genetic factors are also believed to play a role. Because the symptoms of Sjogren’s mimic other diseases, including MS, delayed diagnosis is common; the foundation reports that the average delay between the onset of symptoms and accurate diagnosis is more than 41
There is no cure for the disorder, which is treated with various drugs to combat dryness and other symptoms. Patients must closely monitor the health of their eyes and mouth to prevent damage from the disease, which can also affect the liver, kidneys and lungs. Among Sjogren’s patients, the rate of lymphoma is higher than among the general population.
Johnson is not sure what prompted her to order the Sjogren’s tests. “I was really thinking more of RA, given her family history,” she said. “And in her case, nearly every symptom could be attributed to something else.” To complicate matters, some of Kennedy’s medical problems, including seizures and hypertension, were unrelated to Sjogren’s.
Johnson referred Kennedy to a rheumatologist, whom she sees regularly. He prescribed Plaquenil, an antimalarial drug that is also often used to treat Sjogren’s, which helps modulate an overactive immune system. “She’s doing much, much better,” Johnson said.
Kennedy, now 54, retired early from her federal job. Although her health has improved, the past year has been marked by “good days and bad days.” She is chair of her neighborhood community association, and volunteers at a horse farm and for the Sjogren’s Syndrome Foundation, helping shepherd other patients through the throes of diagnosis and treatment. On good days, she enjoys riding her John Deere tractor around her 14-acre spread, hauling logs and sometimes chopping wood. On bad days, she stays home and rests.
Kennedy said she is relieved to have a diagnosis and is especially grateful to Johnson, whom she says “worked diligently on my behalf. . . . I hear many stories of patients whose doctors have given up on them.”
Living with a chronic condition has required certain adjustments. “I have to think through things I never thought I’d be having to think through,” she said, such as whether she feels well enough to travel to see her family in Virginia. “But I realized, ‘Okay, this is only going to get fixed so much.’ It’s important to be grateful for what you have and to make the most of the good moments.”
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