Amy Sullivan was a 19-year-old college student in 1993, taking the first steps towards what would become a successful career in commodities trading. When she wasn’t hitting the books, she was hitting the trails and slopes in Colorado, by foot, bike and ski.
That December, Sullivan took a trip to Mexico with her family, then returned for the new semester at Fort Lewis College in Durango. She soon met an opponent far more formidable than the toughest course or exam. And the challenge came from within.
Sullivan had been getting physical therapy after a car accident, but during a session the exertion suddenly overcame her.
“I was in physical therapy and I didn’t have the strength to get up off the floor,” she said. “I couldn’t even walk up the stairs.”
Lupus diagnosis for mysterious symptoms
The symptoms worsened and included extreme fatigue, shortness of breath, severe body aches and pleuritis, which is inflammation of the membranes around the lungs. The problems cost Sullivan the fall semester of her next year in college.
After a series of hospitalizations and tests, Sullivan finally learned the source of her misery. It was lupus, an autoimmune disease that causes the body to attack itself with chronic inflammation. Lupus can cause a host of symptoms like those Sullivan suffered as well as organ damage. It affects some 1.5 million people in the United States, about nine in 10 of them women.
The most common signs of lupus are fatigue, joint pain and swelling, skin rashes and hair loss, but there is no easy formula for making a diagnosis, said Dr. Susan Boackle, a rheumatologist at UCHealth University of Colorado Hospital on the Anschutz Medical Campus and associate professor of Medicine-Rheumatology at the University of Colorado School of Medicine. Boackle treats patients in the multidisciplinary Lupus Clinic at UCH and collaborates with other specialists to manage the various symptoms and problems caused by the disease (see box).
“Some lupus patients have really classic symptoms and some don’t so it can be really difficult to make a diagnosis,” Boackle said. Some patients suffer only mild effects; others, like Sullivan, battle progressively debilitating problems. In addition, some patients with symptoms that suggest lupus may instead have other conditions, such as fibromyalgia, rheumatoid arthritis or hyperthyroidism, Boackle noted.
The need for multidisciplinary care and study to address Lupus
Pinning down the elusive disease requires an extensive workup, she said. A visit to the Lupus Clinic includes a review of symptoms, a physical exam and lab work. Depending on their situation, patients may also need X-rays and other imaging, pulmonary function tests, bone density tests, echocardiograms and referrals to the appropriate specialists.
“The disease can affect every organ in the body,” Boackle said. “A multidisciplinary approach is helpful with complex diseases like lupus because coordinated care from specialists is beneficial for patients.”
Boackle is also involved in several clinical trials of treatments for lupus, including one, dubbed SMILE, that tests the ability of hydroxychloroquine, long used to treat lupus symptoms, to slow or prevent the progression of the disease in at-risk individuals. Boackle is the principal investigator at CU for the multi-site SMILE study.
In addition to the trial studies, Boackle conducts her own lupus research. That includes identifying a genetic sequence change that protects people from developing the disease. A better understanding of the mechanisms behind this natural protection could lead to new therapies, Boackle said.
She does all she can to treat and study lupus, but her commitment runs deeper. “I really want to cure this disease,” Boackle said. “It’s my passion and my mission.”
The heavy toll of lupus
The example of Amy Sullivan, who has been Boackle’s patient for about a decade, vividly illustrates that desire. Lupus continues to take a heavy toll on Sullivan’s life. Just 46 years old, Sullivan had to stop working several years ago. After her initial diagnosis, she suffered through a bewildering array of health complications that demonstrate the power of lupus to launch multi-system attacks on the body.
Early on, Sullivan says she controlled her Lupus symptoms with hydroxychloroquine and prednisone. She earned her college degree, began her career in finance and stayed active, despite periodic “flare-ups” of her symptoms. After about five years, though, the bouts grew more frequent and lasted longer. Sullivan also developed optic neuritis, or inflammation of the optic nerve in the eye, and trigeminal neuralgia, which causes facial pain. Both conditions are linked in relatively rare cases to lupus.
The immunosuppressant drug Imuran, added to her other medications, “worked very well” to help Sullivan manage her growing number of Lupus symptoms, and she worked 50- to 60-hour weeks, with enough energy left for an active social life.
“I had a tolerance for adapting,” Sullivan said. “I still had flares but did well overall.”
A serious Lupus symptom exacerbation
Her health, however, began to spiral downward 10 years ago after a complicated pregnancy and premature birth of daughter Sienna – now a healthy youngster. About three months after the birth, Sullivan had a serious symptom exacerbation that included the return of the trigeminal neuralgia and nerve pain in her feet and legs that she likened to electrocution and burning. She received medication that helped ease that pain.
Physicians at UCHealth University of Colorado Hospital who provide specialized lupus care:
- Dr. Joshua Thurman (specializes in glomerulonephritis, including lupus)
- Dr. Sarah Young (combined Rheumatology/Renal clinic with Drs. Zell and Harley)
Dr. Elizabeth Wallace (Dermatology/Rheumatology clinic with Dr. Zell)
However, Sullivan battled other stresses that she believes contributed to her worsening symptoms. She continued to work full-time while caring for the infant Sienna and went through a divorce two years after the birth. While staying with her parents, her blood pressure crashed, she was rushed to the hospital and wound up in intensive care for two weeks. After she transferred to UCH for another two weeks of care, Sullivan met with Dr. Sterling West, a rheumatologist and lupus specialist who suggested she receive IVIg (intravenous immunoglobulin) infusions.
IVIg treatment delivers antibodies – immunoglobulin – that have been isolated from the purified blood of healthy donors. The antibodies guard against infection, boost red blood cell supplies and protect against inflammation. The treatments, which she still receives, stabilized Sullivan sufficiently to leave the hospital. However, she was too weak to walk on her own and needed months of home therapy to inch her way from her bed to a wheelchair, to a walker to a cane to unassisted walking.
The arduous episode forced Sullivan to quit working and go on disability. She realizes that lupus contributed to a gathering storm of physical and mental stress that eventually swamped her after Sienna’s birth.
“I was on autopilot, then things slowed and I had a huge crash,” Sullivan said.
Sullivan said her monthly IVIg infusion has helped her greatly, but lupus caused significant damage to her body. She has had colon issues, a pacemaker implanted, facial paralysis, and continued nerve pain, in addition to other problems. She walks with a cane outside and needs help on the stairs.
A helping hand for lupus care
But there are bright spots too. Sullivan remarried and lives happily in Arvada with Sienna, husband Dave, and stepdaughter Olivia. And she is grateful to have Susan Boackle to manage her lupus.
“She’s been a savior,” Sullivan said. “My history is complicated, and she knows it and advocates for me.”
Sullivan said she particularly values Boackle’s role as not only rheumatologist but also as a point person in coordinating her care with other specialists, ordering tests and helping her find treatments.
“She sees me as a patient as a whole instead of treating one piece of me,” Sullivan said. “She’s always trying to get the whole picture. Without her, my life would be totally different.”
In Sullivan’s view, Boackle’s stated “passion and mission” to cure lupus are more than words. For example, at one point she told Boackle that Sienna expressed a desire to be a doctor so she could cure lupus. Boackle invited Sullivan, her parents and Sienna to a lunch, where she talked to them about lupus, new treatment developments and research into the disease.
“She goes above and beyond and is so passionate about the work she does,” Sullivan said.
An ongoing search for a medical solution
For her part, Boackle said her desire at the start of her medical and scientific career was simply to help people. She later focused on immunology and the mystery of autoimmune disease, which causes “the body to respond incorrectly to itself.”
In lupus, Boackle found a formidable challenge, but one that inspires her to help people live better lives and perhaps one day be free of their disease. And lupus may just be the start: “I hope this work will have broader implications for other autoimmune diseases as well.”
Sullivan, meanwhile, continues to cope with the disease. It’s taken much from her, but she appreciates the simple pleasures of short walks and spending time with family and friends. She wants people to know that lupus resists simple explanations.
“There are many misunderstandings about it,” Sullivan said. “People may think it just makes you tired and a little achy. Lupus is different in each person. Just because you know what lupus looks like in one person, it doesn’t mean it’s everybody’s case.”
She accepts the uncertainty that is part of having lupus.
“It can be very hard to predict and plan, but lupus can be managed,” Sullivan said. “It’s possible to lead a happy, productive, fulfilling life.”