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BWhat do celebrities like Cindy Frey, Toni Braxton, Lady Gaga, Nick Cannon and Seal have one thing in common? Lupus, the disease that President Ferdinand E. Marcos reportedly died from complications of. Just recently, the disease was in the spotlight again when Kris Aquino posted in her social media that she may have it or a very similar condition.
“Lupus is a disease that is hard to diagnose and hard to describe,” said professor Pao Hsii Feng, a clinical professor at the National University of Singapore. “It is not contagious and its cause is still unknown.”
With those words, Feng has brought us to a disease that exhibit symptoms so vague that doctors are left scratching their heads as test after test fails to detect anything amiss. Reader’s Digest singled out lupus as one of the 10 diseases most doctors miss to diagnose. “What is wrong with my patient?” a doctor may ask himself.
But one thing is certain: Lupus generally affects more women than men—10 females to 1 male. “Lupus is predominantly a disease of women,” said Dr. Jose Paul Lorenzo, head of Makati Medical Center’s section of rheumatology. “It is eight to 15 times more common in women than in age-matched men.” Feng added: “Women who are most likely to get lupus are those between the ages of 18 and 50 or those within the reproductive age.”
Medical science classifies lupus as an autoimmune disease. “In autoimmune diseases,” explained Lorenzo, “our immune system does not efficiently recognize the self or our body, versus nonself, such as infection. It therefore attacks our body tissues, causing disease.”
Although there are three types of lupus (systemic lupus erythematosus or SLE, discoid lupus and drug-induced lupus), SLE is the most serious form and that’s what most people are referring to when they say lupus.
The auto-immune disease was given its name by a 19th-century French doctor who thought that the facial rash of some people with lupus looked like the bite or scratch of a wolf. The term “lupus” comes from word, which means “wolf” while “erythematosus” is Latin for “red.” Systemic means that it may affect many parts of the body, such as the joints, skin, kidneys, lungs, heart, or the brain.
“Discoid lupus is descriptive of a characteristic skin lesion, which may be found in SLE or may exist independently without any systemic features,” explained Dr. Sandra V. Navarra, cofounder and adviser of the Lupus Foundation of the Philippines. “Drug-induced lupus can simulate clinical SLE in many ways—but this is triggered by drugs.”
More often than not, people don’t know they have the disease because they don’t know so much about it. As such, actual figures of people with lupus, particularly SLE, are hard to come by. But the Geneva-based World Health Organization (WHO) contends that incidence of lupus has nearly tripled over the past four decades. That’s the bad news.
But the good news is that 10-year survival rates are now up by 80 percent to 90 percent, compared to 50 percent in the 1950s. A major factor for this is earlier diagnosis, resulting in treatment during less severe stages. “This has positive impact on the prognosis and outcome of the disease,” said Feng.
Still, lupus is potentially fatal, especially when misdiagnosed and inadequately treated, said Dr. Ratanavadee Nanagara, associate professor in medicine of the Department of Medicine at the Khon Kaen University in Thailand.
“Early diagnosis and prompt management of the various disease manifestations are of utmost importance for prevention of worsening disease or complications,” said Lorenzo.
“Lupus is dangerous when it involves the kidneys, brain, heart and the blood elements,” warned Feng. “If you have lupus involving brain, you may develop fits, depression and other psychiatric symptoms.”
Oftentimes, patients with severe lupus die. “They often die from kidney failure or infection,” says Dr. Alberto Santos-Ocampo, a Filipino rheumatologist who works at the Straub Clinic and Hospital in Honolulu, Hawaii. “Patients with SLE are prone to infections, because of their abnormal immune system, and because of the immune suppressing properties of the drugs they are given to control their disease activity.”
The disease course can range from very calm to very stormy. “Many lupus patients do not experience bad complications and are able to perform their daily activities quite efficiently,” said Navarra, “except for some ‘bad days.’”
Lupus has been called as “the disease with a thousand faces.” The reason: “Many of its signs and symptoms are commonly seen in other illnesses, including infections and malignancies,” said Santos-Ocampo. “In addition, lupus tends to evolve during its initial stages, and may not present with enough features for the clinician to ‘clinch’ the diagnosis.”
That was what happened to Marianne, a 16-year-old high-school student. One morning, she experienced a terrible headache and was feeling drowsy. Her teachers told her to go home and take a rest. At home, she had an unexplained high fever, which went on for more than four days. When she went back to school, she was no longer the Marianne her friends knew: she was losing weight.
Marianne’s parents didn’t know what was going on with their daughter. Coming from a poor family, she was not given proper medical treatment. She was brought to the public health hospital and was treated for few days. Then, she went back to the school again.
Days passed and the teachers noticed that her condition was getting worse. Marianne was experiencing achy and painful joints and having sores in her mouth. At one time, while attending a class, she felt very sleepy.
“There must be something wrong with her,” one of the teachers said. So, they decided to bring her to a hospital and hoped that the student’s life would be saved. “She was very drowsy when I first saw her,” the doctor who treated Marianne recalled. “There was very low platelet count on her blood test and as such it was very risky to have spontaneous bleeding anywhere in her body. If the teachers had not brought her to the hospital, Marianne would have been dead from massive bleeding.”
Over half of the patients with lupus develop a characteristic red, flat facial rash over the bridge of the nose. Because of its shape, it is frequently referred to as the “butterfly rash” of lupus. The rash is painless and does not itch. The facial rash, along with inflammation in other organs, can be precipitated or worsened by exposure to sunlight.
“Sunlight exposure, especially those between 9 a.m. and 4 p.m., triggers the expression of lupus as well as aggravate the disease,” said Navarra. She cited the case of the 21-year-old patient, who developed an unexplained fever for almost a week and unusually bad sunburn after a beach holiday in Boracay. Then, she experienced persistent, extreme fatigue and weakness for days even after six to eight hours of restful night-time sleep.
Most lupus patients, however, develop arthritis at some time during the course of their illness. Arthritis in systemic lupus commonly involves swelling, pain and stiffness of the small joints of the hands, wrists and feet. Sometimes, lupus arthritis can mimic that of rheumatoid arthritis (another autoimmune disease), but is not as destructive.
Elsa, a 26-year-old female executive, did not only experience joint pains, but she was also having some breathing difficulty and was losing her hair. Likewise, her legs were swelling. “These symptoms are not unusual for a person having lupus,” Navarra said.
Since patients with SLE can have a wide variety of symptoms and different combinations of organ involvement, no single test establishes the diagnosis of lupus. This is the reason lupus is hard to diagnose.
“A combination of symptoms is suggestive of lupus if other causes, including infection and malignancy, have been ruled out,” Santos-Ocampo said. “Diagnosis is usually done by taking a history of the patient and doing a full physical examination. The diagnosis is then confirmed by taking blood tests,” said Feng.
Since it takes time to diagnose someone having lupus, it may be too late to treat the disease once diagnosed. “We can control lupus, but not cure it,” explained Santos-Ocampo. Feng also added, “In its early stages, lupus is a treatable disease. However, if proper treatment is delayed then it can be fatal.”
As such, Feng recommended, “It is important for patients with lupus to see a doctor experienced in the treatment of the disease. Since lupus is a chronic condition characterized by exacerbation and remission, patient compliance is very important. This means patients must be well educated with regard to the disease.” “Once diagnosed with lupus, I determine any internal organ involvement and the severity of involvement,” said Dr. Navarra. “These will influence the form of individualized therapy the patient will need.”
A variety of drugs are used, depending on how lupus manifests itself. “Controlling lupus is a more realistic goal than curing it,” said Santos-Ocampo.
“Never try untested remedies,” reminds Dr. Keith Lim, a Malaysian consultant rheumatologist with the Subang Jaya Medical Center in Kuala Lumpur. “I have seen many patients who come to see us too late because they were using alternative forms of treatment. By that time, the disease has already done its worst course in the body.”
For those with lupus, Feng suggested: “Learn as much as you can about the disease. Learn as much as you can about your medications, too—both its good effects and side effects. Be an active partner with your doctor in managing the disease.”