Lack of Vasculitis Expertise Leads to Devastating End

Karen Miller has joined her local hospital’s patient advisory board after her mother, Lynn Johnston, died from cascading effects of her delayed diagnosis for vasculitis. Her hope is that her role on the advisory board will lead to changes in the way patients and families are heard and that it will encourage greater cooperation among health care providers.

It is also why she supports the Vasculitis Foundation’s efforts to make vasculitis centers more accessible, encourage doctors to collaborate, to offer fellowships to increase the number of highly qualified physicians and researchers who can diagnose, treat, and improve the lives of people living with vasculitis, and to offer more education for patients and family members.

Lynn, a vital woman whose retirement consisted of Crossfit three days a week, long walks and chasing after a toddler as well as after-school pick-up for her grandchildren, died in a few short months when an expert vasculitis treatment team didn’t come into place and there were no vasculitis centers nearby to offer the treatment she needed.

“She was the healthiest 72-year-old around,” Karen said. “Her blood pressure was always low and she didn’t even have wrinkles.”

At a regular check-up before her first hospital stay all of her bloodwork was normal, except for her thyroid, for which she was taking medication that had recently been switched to a generic. The next day she had a routine dental cleaning and she complained about severe pain afterward. Her mother had always been meticulous about brushing and flossing and went to the dentist regularly, so this was far from ordinary.

“She was consumed with the post dental cleaning,” Karen said.

Since her mother never complained, Karen said she knew something was seriously wrong. She thought at the time that it could be a bacterial infection and took her to the hospital.

It turned out Lynn had nodules in her lungs and inflammation. The doctor ruled out pulmonary embolisms. Her first physician was confident it was GPA because he had seen it 20 times in his career, Karen said, but the hospital failed to run further tests for GPA. The team also neglected to consult a Rheumatologist until they ruled out lung cancer saying that only her lungs were affected, so they didn’t agree with the diagnosis. Then the hospital had delays in scheduling the bronchoscopy for her lungs and Johnston’s symptoms got worse.

Searching for Answers

Karen, who owns a CPA firm, wanted to be a strong advocate for Lynn, but she had taken on the full-time responsibility of caring for her while trying to research vasculitis. Karen’s daughter and sister are nurses, but even they didn’t know much about the rare autoimmune disease. Still they offered good advice on patient care.

Karen was at the hospital 12-14 hours a day to make sure her mother got proper care. Lynn’s husband also was there. But many times they were frustrated because they couldn’t get the necessary care and tests were often delayed.

“I was an active participant,” Karen said. “I am not quiet, passive or ignorant to question how and why things are done.”

Karen listened to the doctors when they opted for more tests rather than vasculitis treatment in the beginning. She said the doctors should have been seeking other opinions and advice because the disease can present in so many different ways. The doctors never brought a Rheumatologist into the hospital where Lynn was an inpatient.

“The Physicians needed to be more active in seeking guidance and direction. Me and my sisters found the Vasculitis Foundation,” she said. “There needs to be more awareness for doctors.”

When Lynn was released from the hospital, Karen took her to see a Rheumatologist, but they had to wait a week for an appointment, more tests, and two weeks for the results. The Rheumatologist prescribed 1,000 mg of prednisone intravenously and then a 60 mg maintenance dose while they waited.

Her mother didn’t want to be on steroids, but she felt better after the first day. And then she started having side effects from the prednisone, including an increase in her blood pressure.

At the follow-up two weeks later, the Rheumatologist said he didn’t believe it was GPA because the kidneys weren’t affected and she wasn’t coughing up blood. He suspected blood cancer and he sent her to an oncologist, where she had more tests. While she waited for the test results, Johnston collapsed. She was taken to the ER and again the tests showed vasculitis – GPA or MPA – and this time there was kidney and intestinal presentation.

Delayed Treatment

Before treating Lynn for vasculitis, the hospital began prepping for a colonoscopy and in the middle of the night she told the nurses she couldn’t do the prep and her body went limp. They believed she had a stroke and gave her Plavix, preventing her from having a scheduled kidney biopsy to check for vasculitis.

To complicate things further, Lynn’s brother had been diagnosed with APS, an autoimmune blood-clotting disorder, so the doctors did lab work for APS and put her on heparin. They eventually consulted with a vasculitis specialist at the University of South Florida, who told them they didn’t need the biopsy results to start Rituxan. 

When Lynn finally got the kidney biopsy, it came back positive for PAN vasculitis. The doctors put her on Rituxan and she immediately responded to the treatment. They also added Cytoxan.

After a month of treatment, Lynn complained about fatigue and lethargy again. She also had a hard time explaining what was wrong because she would mix up her words and mispronounce them.  The doctors prescribed Wellbutrin, but her blood pressure had been over 180 for three consecutive days. The next day she had a stroke – what they believed was her second stroke.

The hospital got Lynn well enough to transfer to a rehabilitation facility, which was even worse, Karen said. After 10 days Karen brought her mother home. By then Lynn had lost all mobility, she couldn’t sit up or get out of bed. Lynn’s family hired a private nurse for around-the-clock care.

She Didn’t Trust the Treatments

They also got her an outpatient appointment with a Nephrologist because her kidneys were failing. Her kidney numbers started to come down, but her blood pressure was still an issue and the family never saw improvement in Lynn’s health after she came home.

She was home for a month when she started coughing. Her lungs started to fill and she had edema. An ambulance brought her back to the hospital where they gave her Lasix.

“At that point they start talking about treating her GPA,” Karen said.  “They started the prednisone again and that triggered psychosis.”

At that point, Lynn, who had a living will, said she was done with all the treatments.

All of the back-and-forth left Karen frustrated and confused about what was best for her mother. Lynn, who had been so healthy, gave up hope that the right treatment could be found.

“Maybe the standard course of treatment is good,” Karen said “But they need to develop systems where physicians can give confidence to the patients and families to help them understand what recovery looks and feels like. What are the things we should look for to know that the medications were effective.”

Karen tried getting her mother into the Mayo Clinic, but her mother’s insurance wasn’t accepted there because it was “too good,” she said. She also searched for a nearby vasculitis center, but couldn’t find a specialized care center.

Karen wanted to find the right care for her mother, but it was impossible while they were always in crisis mode. She also felt the hospital and the treating physicians didn’t seek advice from vasculitis experts, something she strongly encouraged.

“We would have protected her kidneys, brain and intestines if we had started the treatments right away,” Karen said. “She completely lost her mobility because of those strokes.”

“The Physicians needed to be more active in seeking guidance and direction,” she added.

Karen is joining her local hospital’s Patient Advisory Board in an effort to advocate for other patients and prevent another family from going through what she and her family lived. It is also why she supports the Vasculitis Foundation.

Karen used the VF’s resources to advocate for her mother. There needed to be a care coordinator in the hospital, Karen said, who made sure the experts were available to advise and treat. She would like doctors and hospitals connected to vasculitis experts and resources. The Vasculitis Foundation offers patient education and advocates for creating more connections among experts, doctors and hospitals. Will you help the foundation achieve these goals so other families don’t have to watch as someone they love struggle? Please donate today.