Lupus – The Enemy Within
When Lucy Vodden died at 46, most wouldn’t know this attractive British woman by name or face. She was the inspiration and namesake for one of the Beatles’ most famous hits. Ironically, though the song written about her is well-known, what killed Vodden remains a disease few have even heard of. “Lucy in the Sky with Diamonds” died of systemic lupus erythematosus (SLE).
Lupus, as SLE is often referred, is an autoimmune disorder where the body’s immune system begins attacking its own tissues. This onslaught can lead to debilitating inflammation and pain. Though it can afflict anyone, lupus has a propensity to hit females in their childbearing years. Minority women of African, Latin, Asian, and American Indian heritage are especially prone to getting the disease for reasons that researchers have yet to identify.
There are two primary types of lupus though it can be drug-induced or found in newborns. Systemic lupus erythematosus means that any and all portions of the body can be affected. This can include damage to joints, heart, lungs, skin, blood vessels, kidneys, and brain. In discoid lupus erythematosus, the skin is affected. While discoid lupus does not have the same fatality potential of SLE, it is the most disfiguring. The bumpy red rashes and ridges caused by this form of lupus can disfigure a person’s looks. The singer-songwriter and recording artist Seal has discoid lupus, which left his face scarred.
Hui-Lim Ang, executive director of the local non profit support group, Colors of Lupus, says statistics on lupus are difficult to obtain because lupus is not a widely talked about disease. Research funds are lacking making it implausible to collect and maintain data. However, it is estimated that 5 million people worldwide have the disease, which amounts to approximately 1.5 to 2 million Americans. Roughly 13,500 lupus sufferers reside in Nevada.
Cleveland native and self-described Type A Michelle Stewart got her SLE diagnosis in 2003. “I thought I had a sinus infection,” she says.
Stewart figured she’d get a prescription from her doctor and be on her way. And indeed she was on her way when she received a call from her doctor insisting Stewart head straight to the ER. “She [the doctor] said I had 10 platelets when a normal person has over 300,000. She said she didn’t know how I was still walking around.”
And so Stewart spent the next year in an out of the hospital, getting biopsies, running high fevers, suffering from profound fatigue, losing weight, and going into renal failure. She also saw a number of specialists who didn’t know what the matter was until she saw a rheumatologist who finally diagnosed her with systemic lupus.
The diagnosis rocked her world and resulted in personal upheaval including a divorce, but her optimism for enjoying her life remained undeterred. “I rebuilt my life,” says Stewart. “I only put people in who really get me…and I realize it could be a lot worse.” Subsequently, she also moved to Las Vegas where the weather was more conducive to her overall health.
Lupus has been called a masquerading disease. As Stewart puts it, “Sometimes I just don’t feel good, but you look OK and people can’t understand it.” And that’s the thing with lupus — its raging symptoms are usually internal, so that those affected can feel very isolated, wondering if it’s all in their heads!
According to Dr. John Pixley, associate professor and division chair of the Department of Immunology and Rheumatology for the University of Nevada School of Medicine, lupus follows no traditional disease path — it’s as individual as the people affected. And it’s not at all unusual for a person to look perfectly healthy. The disease can also cause neurologic problems such as seizures and psychosis.
“The best way to understand it is that it’s heterogeneous. It has varied presentations and severity and that includes within individual patients,” says Dr. Pixley. “It’s a group of illnesses with overlap features so a lot of times they’re misdiagnosed. It’s not so important about the overlap as much as how you’re going to treat the patient.”
Lupus therefore falls into the mixed connective tissue disease category.
In addition to causing troubles throughout the body, the unpredictable nature of the disease means that a flare — or exacerbation of symptoms — can happen at anytime. Some are under the belief that a flare can occur due to stress but Dr. Pixley isn’t fully convinced of this association.
According to Ang, there are a total of 11 common symptoms that range from mild to severe. Doctors caution that if four to five symptoms have been present for the past four to five years, testing for lupus is highly advised. In fact, the average lupus patient has suffered for five years before he or she is officially diagnosed with the disease. And even then, people find out that lupus is one of the most difficult diseases to diagnose and treat. This is where a good rheumatologist, or a specialist in connective and autoimmune diseases, is invaluable.
Diagnosing the disease seems to be as challenging as dealing with the disease. “One of the reasons people go into rheumatology is the unpredictable nature of the diseases we treat,” notes Dr. Pixley. He say’s it’s not unusual for a rheumatologist to tell a patient that they may have or may not have the disease during an office encounter and have that ambiguity make total sense.
Most patients, like Stewart, are diagnosed with the disease when an acute condition transpires. Often these people are hospitalized with a particularly bad case of pneumonia, anemia, heart attack, or kidney failure. Rarely, patients are actually seen in psychiatric hospitals with psychosis caused by the inflammatory conditions associated with lupus. Only an alert clinician who observes secondary characteristics such as fever, elevated white counts, or rash might request blood testing of these patients to determine if their mental state is really a symptom of something else.
Blood testing is helpful but not definitive for diagnosis and includes anti-nuclear antibody testing (ANA), etractable nuclear antigens (ENA), anti-cardiolipin, anti-DNA, and rheumatoid factor. There is typically a pattern of abnormality in these laboratory values that point to lupus. Blood work in conjunction with active symptoms and physical manifestations such as the classic butterfly rash across the nose and face also help a specialist determine lupus.
The U.S. Department of Health and Human Services reports that more than 16,000 Americans develop lupus annually. Cascading symptoms can range from dry eyes and mouth (often caused by another autoimmune condition called Sjogren’s Syndrome) to an increasing fatigue or ache in the joints and muscles. Heart disease is the major cause of death in lupus sufferers and a major complication of the disease. It causes inflammation that can cause cardiac enlargement or damage the heart’s pumping efficiency. Scarring in the lungs is also common. However, seemingly vague and “routine” symptoms can include frequent urinary tract infections, anemia, hair loss, shortness of breath, vision problems, and depression.
The primary way to treat lupus is medication therapy, namely corticosteroids to reduce the inflammation. This inflammation is caused by the body’s immune system attacking its own tissues and organs.
“You can control lupus with steroids but you can’t sustain high doses for long,” says Dr. Pixley. Other medications used to battle the disease include immunosuppressives, antimetabolites, and various chemotherapy drugs.
Perhaps pharmaceutical research and development teams are undertaking one of the more exciting prospects on the war with lupus. Dr. Pixley explains that the use of biologic medications including systemic interferon inhibitors and B-cell ablators are showing great promise for treating lupus. They’ve been used for rheumatoid arthritis for years, but testing for lupus has been difficult. As Dr. Pixley says, “It’s very expensive to bring drugs to market and it requires a uniform patient population.” Lupus sufferers are anything but uniform.
In one week alone, Dr. Pixley indicates he saw two newly diagnosed lupus patients, both men, one of whom was 60 years old. However, his patients are mostly female and the chronic nature of the disease can put a strain on finances as well.
He recalls a story involving two teen girls who, because of inadequate health insurance coverage, ended up on kidney dialysis because they couldn’t afford care. As Dr. Pixley emphasizes, “Maybe 15 to 20 thousand dollars of medical coverage [for care], and the state could have saved millions of dollars.” He also notes that while the girls had some kidney damage from lupus, they would have surely done far better had they had access to regular medical care. Because the girls didn’t receive this preventative care, the disease escalated into a crisis where both are now awaiting kidney transplants. It adds insult to injury and this is not an unusual situation say many health care providers.
One of the world’s foremost programs for autoimmune research with an emphasis on lupus is at the University of Pittsburgh. Its Lupus Center for Excellence is designed to centralize lupus research. It also provides world-class care to lupus patients in a facility staffed with internationally renowned physicians and researchers who have access to the latest treatments and clinical trials. It has grown to support patients from 27 states and 10 countries.
Contending with lupus can also be a challenge when dealing with having a family or pregnancy. Before conceiving, specialists caution women who ha ve had significant lupus activity to be aware that they’re at higher risk of miscarriage, premature births, or pre-eclampsia or toxemia. Many of the medications used to treat the disease can also cause birth defects or compromise a person’s fertility.
Emotional support is key to dealing with a chronic condition, particularly one with such an erratic nature. Groups like Colors of Lupus allow participants to share experiences and offer support to sufferers and their loved ones.
Ang says, “We provide members an opportunity to reclaim the quality of their life. We do so by providing immediate physical services and support to those afflicted with lupus. We also help with transportation to and from doctor appointments, deliver food, and provide referrals to an exclusive physician network specializing in alterative therapies. The idea behind providing these services is to alleviate stress and allow for an expedient recovery.”
While lupus is definitely life-changing, patients should have hope. Both Dr. Pixley and Stewart advocate for a well-informed patient that remains committed to making the best of the situation. Stewart takes her advocacy one step further. She believes that a patient needs to be fearless and ask questions.
As Stewart explains it, it’s not enough to simply know the medications you’re on. “You have got to know what you’re talking about. Why do you take the meds? What are your doctor's plans for you? You have to be able to speak that language.”
