Coping with the ‘Great Imitator’ – Adapting to Life with Lupus Requires Stress-free Control

A good day for Skochii Bennett Polchlopek, administrative assistant at the Springfield Ronald McDonald House, is one in which she has no stiffness, no aches or sharp pains in her joints, and no fogginess in her brain.

A bad day is one when all of the above occur — and much more.

“I might not have the use of one of my hands, or my wrist, I’m exhausted the whole day, and the fogginess … just the little things I can’t remember; it makes life very inconvenient.”

She adjusts her day and ekes out what she can do to make it through the many physical issues that have became a way of coping with a disease known as ‘the Great Imitator.’

Bennett Polchlopek has systemic lupus erythematosus (SLE), an autoimmune disease that currently has no cure. “It attacks everything,” she told HCN before amending that comment in a poignant way. “Actually, your body is attacking itself.”

Like many diseases that are difficult to diagnose because they involve overlapping physical symptoms, lupus is referred to as an imitator because its symptoms are very similar to rheumatoid arthritis, various blood disorders, fibromyalgia, diabetes, thyroid problems, Lyme disease, and a host of heart, lung, muscle, and bone diseases. Lupus causes the human immune system to become hyperactive and go literally into overdrive, attacking not only infectious foreign organisms that invade the body, but healthy tissue as well.

Diagnosed at the age of 41 in August 2010, Bennett Polchlopek knew of the disease because her younger sister, Toki, had been struggling with it for 15 years.

Toki had ‘presented’ — a term Bennett Polchlopek used many times to describe the combination of physical anomalies that lead to a diagnosis — with pleuritis, an inflammation of the membrane around the lungs.

“They gave her this really horrible, long list of things it could possibly be — AIDS, cancer, lupus — and lupus was the best of the list, and that’s what she had,” Bennett Polchlopek remembered.

Her own form of lupus probably presented much earlier than when she was officially diagnosed by her primary-care physician (PCP) and then sent to Dr. James Schumacher, a rheumatologist at Riverbend Medical Group. His sub-specialty in internal medicine deals mainly with clinical problems involving joints, soft tissues, autoimmune diseases, vasculitis, and heritable connective-tissue disorders.

But before her diagnosis, her symptoms acted like a strange form of arthritis — hands, wrists, shoulders, elbows, and toes were all affected with severe pain in the joints — and she knew something was not right.

“The pain kept weirdly moving around, from one hand to the other, from shoulders to toes,” she said. “And my hands would be so swollen I couldn’t bend them. My shoulder was frozen, and I couldn’t dress myself for a while; there was pain consistently in one place, and I’m thinking, ‘I’ve got a problem with just that part,’ but it was migrating, and that was just too much.”

Ibuprofen was prescribed, but the pain became worse. Strong meds, like Percocet, lessened the pain, but she couldn’t function, and not knowing when or how the pain was going to stop led her PCP to perform a diagnostic antibody test called an ANA panel (more on this later).

“I already knew what lupus was, because of my sister, so I kind of held out for Lyme disease. It sounds horrible to be hoping for some other disease, but it came back as lupus,” said Bennett Polchlope as she remembered reluctantly accepting her fate.

Officially her form of lupus is the most serious and common form, systemic lupus erythematosus, or SLE, as opposed to the other three forms: discoid, a chronic skin condition of inflamed sores and scarring of the face and other body areas; drug-induced, caused by chronic use of 38 known medications that bring about an autoimmune response (the body attacks its own cells); and neonatal, lupus in an infant born from a mother with SLE.

Of the lupus patients that Schumacher has seen over his 30-year career, he’s noticed that once the severity and specific anomalies of a lupus patient are identified, it’s a sort of ‘brand’ that they alone have. In Bennett Polchlopek’s case, her brand of SLE involves severe photosensitivity, multiple areas of joint pain and inflammation, kidney disease, and possible ocular toxicity, a result of her daily lupus drug, Plaquenil. That round of afflictions, whether mild to severe, or what Bennett Polchlopek called a ‘flare’ — severe affliction to the point of debilitation  — is what she can expect for the rest of her life.

For this issue’s focus on disease and infection, HCN spoke with Bennett Polchlopek regarding her personal and associative experiences with lupus, and also with Schumacher about the many nuances of a incurable disease that is hard to diagnose and hard to live with, unless a lifestyle of adaptive control is in constant overdrive.

With her lupus, Bennett Polchlopek is in the company of celebrities, including singers Toni Braxton, Seal (his facial scars are the result of discoid lupus, involving only the skin), and Lady Gaga, who is ‘borderline positive,’ a designation that demonstrates the difficulty in diagnosing this disease.

According to the Lupus Foundation of America, the term lupus, which is Latin for ‘wolf,’ is attributed to the 13th-century physician Rogerius, who used it to describe erosive facial lesions that were reminiscent of a wolf’s bite.

The actual diagnosis of lupus is the most difficult hurdle; according to the Lupus Foundation, the ANA panel that was used to diagnose Bennett Polchlopek is a sensitive blood test to examine antinuclear antibodies (ANAs), which bind to the nucleus of the cell. This abnormal process damages, and can destroy, the cells. Since these antibodies are found in 97{06cf2b9696b159f874511d23dbc893eb1ac83014175ed30550cfff22781411e5} of people with the disease, in the presence of three or more typical features of lupus — such as involvement of the skin, joints, kidneys, lungs, heart, blood, or nervous system — a positive ANA test will usually confirm a diagnosis of lupus.

 Schumacher said that among the approximately 80 patients he sees each week, two are lupus patients. And while that’s not a large percentage of his patient base, lupus is not a rarity. In fact, as an African-American woman, Bennett Polchlopek was a prime candidate.

 “About 90{06cf2b9696b159f874511d23dbc893eb1ac83014175ed30550cfff22781411e5} of lupus patients are women,” said Schumacher. “And there is a higher incidence and more severity in the African-American population.”

The reason why is unknown, he added, and while it could be due to U.S. environmental influences or the interracial mix of Caucasian and African-American genes, the incidence of lupus in Africa is not as high as in the U.S., which could be a result of Africa’s limited medical care and the fact that it is not well-diagnosed.

“There is a wide spectrum of the disease. For instance if you have ALS [amyotrophic lateral sclerosis, or Lou Gehrig’s disease, a progressive neurodegenerative disease affecting brain and spinal cord nerve cells], in five years, you’ll be dead,” explained Schumacher. “And with lupus, depending on the severity, you may be sick at times or could die, but you’ll probably live, and why is really unclear.”

Bennett Polchlopek’s sister, who suffers from a more debilitating brand of the disease, was close to needing a kidney transplant, a dangerous and almost futile exercise because the lupus would probably attack the new kidney.

“She was in the last stages of glomerulonephritis [a stage-five kidney disease inflaming and damaging the kidneys], but she was put on a class of chemo drugs to bring her kidneys back,” said Bennett Polchlopek, adding that she, too, has stage-three kidney disease, very possibly due to the early large-dose use of ibuprofen. Her focal segmental glomerulosclerosis (FSGS) disease interferes with kidney function, producing internal scars.

She undergoes blood and urine testing, eye exams, and other procedures on a six-month rotation, unless, of course, she has a ‘lupus flare,’ which is sometimes caused by acute stress.

“Stress can send you into a lupus flare, and you can experience all the horrible negative effects of the disease once that happens,” she explained.

Like two-thirds of lupus sufferers, Bennett Polchlopek also has severe photosensitivity, making a sunny day outside a laborious task of preparation.  A combination of high-block lotion, a shirt with sleeves, a hat, and socks is necessary when outdoors.

“Every day I check the UVA ray levels on an app on my phone, and once it hits ‘4’ I start lotioning up, wearing hats, and watch my exposure time in the sun.”

While surfing the Internet for arthritis-related information, Bennett Polchlopek found an old remedy that she admits sounds a bit crazy, but says it has shown a definite improvement for her and two other friends who have lupus.

Laughing and rolling her eyes, Bennett Polchlopek explains the concoction is golden raisins soaked in gin.

“It’s the worst-tasting thing … I mean, I like gin, but not with raisins,” she said, adding that she’s been taking a teaspoon of the gummy substance every morning, and her pain has decreased significantly, letting her pitch her cane for almost a year. Her doctor is monitoring her potassium levels, due to the amount of potassium in raisins, but so far, so good, with no adverse effects in her blood work.

Schumacher said the impact of any disease depends on how severe it is and how the individual deals with it.  “What’s hard about lupus is that we just don’t have that great a treatment for it.”

Traditionally, treatment for lupus has been pretty much the same since the 1960s. Schumacher said the anti-malarial Plaquenil, which is the marketing name for hydroxychloroquine, is the key drug. According to the Lupus Foundation, Plaquenil’s origin dates back to World War II when anti-malarials were first used to treat parasitic infections like malaria, but by the 1960s it was being used to treat joint pain that occurs with rheumatoid arthritis, and soon after, similar beneficial effects were seen in the treatment of skin issues and joint pain associated with SLE.

Three other recently introduced drugs are showing promise: Benlysta, which targets specific immune cells and is the first drug approved to treat lupus in more than 50 years; CellCept, an immunosuppressant for those with lupus-related kidney disease; and the more established Cytoxan, a chemotherapy agent. Another drug, prednisone, has been used for years to treat certain inflammatory diseases and some types of cancer, but has significant adverse effects.

“Prednisone scares me the most; I’ve had two rounds of it during flares, and I don’t want to do that again,” said Bennett Polchlopek, noting that some patients, like her sister, aren’t as lucky and are on prednisone long-term.

 “The yin and yang of treating autoimmune diseases is that we’re always giving drugs to try to blunt the immune response to make the patient not have the symptoms of the autoimmune disease,” said Schumacher, “but then infections become more likely.”

While this treatment process is an invitation of sorts for other infections because the body’s natural defenses have been muted, the afflictions are not dire most of the time. But it heightens the patient’s need for education about infectious contamination in their normal day-to-day routine, which means constantly keeping hands washed and staying away from others that are sick.

A cure for lupus is something Schumacher and Bennett Polchlopek may not see in their lifetimes, but Schumacher feels that research involving other, far more common diseases will afford more beneficial solutions.

“For multiple sclerosis MS and arthritis, there’s a bigger bang for research dollars, but a lot of autoimmune diseases feed off of other research because we have drugs coming out of those fields that may be helpful for lupus,” Schumacher told HCN. “We have a lot of experience now with AIDS; how you treat an infection in an AIDS patient may be similar to an infection in a patient on an immunosuppressant drug; some of that knowledge is transferable.”

Bennett Polchlopek considers herself lucky. “My doctors have kept it under control,” she said. “I’m keeping myself as stress-free as I possibly can, and I just need to be smart about my choices.”

Which will hopefully offer her far more good days than bad for the rest of her life with lupus.