From the Archives: Fall 2006 Issue of Lupus Now Magazine
Here Comes Trouble: Learn to take control when a lupus flare strikes
by Jaclyn Law
Lupus is an extremely unpredictable disease. It can be relatively quiet, but tolerable, for months at a time. Then, within days, it returns and leaves you physically and emotionally drained. And the worst part? You don't know when or how to stop it. Dealing with flares is a reality of living with lupus, and it is one of the biggest concerns of the 1.5 million Americans who have the disease.
"A lupus flare is when an individual's lupus has been quiet or in remission and then becomes active again, either with new symptoms or a recurrence of old ones," explains Gary Gilkeson, M.D., chair of the Lupus Foundation of America (LFA) Medical-Scientific Advisory Council and a professor at the Medical University of South Carolina. "The body's immune system is active, inappropriately releasing a variety of factors that lead to the inflammation that causes the patient's symptoms."
Unfortunately, flares are all too common. "Fifty to 60 percent of lupus patients experience a flare in a given year, with 25 percent having a severe flare," says Gilkeson. "How often they experience a flare ranges widely. Flares can last anywhere from a couple of days to a couple of months."
When a flare erupts, patients often ask why, as in "Why now? What did I do wrong?" While there is no concrete answer to this question, doctors do know that a lupus flare can be triggered by an infection, medications such as a sulfa drug (sulfonomide), or exposure to the sun. Although it is often mentioned by patients as a possible cause of their flares, "stress is a less proven trigger," says Gilkeson. He adds that a flare can also indicate that a patient's therapy is no longer effective. "Often, however, we really do not know what triggered the flare," he says.
Learn Your Symptoms
Flares often creep up slowly. Most often you'll see signs of prior symptoms -- the more common include increased fatigue, fever, and joint pain -- as well as anything new or unusual.
"Normally, patients experience the same symptoms they had before, but they can also have new symptoms develop," explains Gilkeson.
Kim Pridemore, 47, president-elect of the South Central Texas Chapter of the LFA, was diagnosed with lupus in 1984, six months after her second child was born. She had experienced baffling symptoms from a very young age, including the joint pain, weight loss, and exhaustion that led to her diagnosis at age 26. She has had numerous flares, sometimes lasting for months.
"When a flare is beginning, my rash always seems to become worse and I run a low-grade fever," says Pridemore. "Then, depending on the area affected, the symptoms would present themselves."
Although flares usually mean a return of prior symptoms, Pridemore was recently surprised by a new complication. "I had severe chest pains last December," she says. "Prednisone didn't give me relief, and I ended up taking cortisone shots in my chest. I finally had total relief around May."
Mary Goodman, 62, of Brandon, FL, was diagnosed with lupus in 1997. Like Pridemore, she has had symptoms including painful joints, Sjögren's syndrome, and Raynaud's phenomenon for most of her life.
"My flares last several days to a week, after which it takes me a good bit of time to re-energize," she says.
Goodman also developed an unexpected symptom during a flare -- an optical neuropathy that caused blindness in one eye. "My doctor was out of town, but luckily returned and gave me the needed prednisone before permanent vision loss occurred."
"I urge lupus patients to know their own bodies well enough that if new symptoms happen, they know they should talk to their doctor," says Robin L. Brey, M.D., a neurologist and associate dean for research at the School of Medicine at the University of Texas Health Science Center at San Antonio.
Pridemore agrees. "Pay close attention to your body. Not everything is a cold or a virus. See your doctor and ask questions, and mention anything that worries you."
The Road To Recovery
Your doctor is your ally in resolving a flare. "We almost always ask the patient to come to the clinic to be evaluated, and will see them more frequently until the flare subsides," says Gilkeson.
In addition to a physical exam, your doctor will probably order laboratory tests. "It is likely that blood work would be done to check for signs of inflammation and lupus activity," adds Brey.
Lupus flares can affect different organs in the body, so people with lupus should be aware of the symptoms that suggest this is occurring.
"If the flare seemed to be in one particular organ, imaging of that organ might be done to look for damage," says Brey.
To make the distinction between ongoing disease activity and a new flare, your doctor will use lab values, including levels of anti-double-stranded DNA antibodies and complement in the blood, to gauge how active your lupus has become. Not all signs of lupus activity are obvious. For example, excess protein in the urine could mean the kidneys have been affected, even if you haven't noticed any symptoms. To treat a flare, your doctor may adjust your medications, such as increasing prednisone or adding other immunosuppressant drugs. Your doctor might also add medications to relieve your symptoms.
Feelings and Family
Not surprisingly, flares can take an emotional toll. "It is obviously upsetting for patients to have their disease return, and it leads to enhanced stress on them and their families," says Gilkeson. "The monetary expense of being ill and the inability to function normally are often hard to manage."
"Being ill always has adverse effects on emotions and relationships," Brey agrees. "Because lupus flares are usually unexpected and come out of the blue, it makes the emotional aspect even more problematic."
Goodman says that coping with her flares, the new symptoms, and the "pain in the butt" factor of lupus definitely affects her emotionally. "I wonder, why me? When will this end?"
Pridemore also gets frustrated, especially when flares force her to change her plans. "Flares make me irritated because I can't do what I want. I get discouraged, especially when I've been doing really well and then it happens."
Family activities are often put on hold when a flare strikes. Both Goodman and Pridemore recall how lupus interfered with family life when their children were young.
"I always felt guilty that I couldn't do this or that for the kids because of a flare; couldn't go on field trips or had to cancel when I made a commitment to be there," says Goodman. "My husband had to pick up the majority of the parental responsibilities."
Relatives and friends can play important roles in helping people with lupus handle the effects of a flare. Just offering to help with household chores is an example of what can be tremendous relief for someone in the midst of a flare. Goodman suggests letting people close to you be part of the recovery process. "Let your family in, without being a 'burden.' Kids don't mind helping if it is just one more thing expected of them."
Getting Through It
Flares can happen at the worst times -- say, right before your sister's wedding or when you're trying to meet a deadline at work. You may be tempted to push on despite the mounting symptoms, but you do so at your own risk. It's a lesson that Goodman has learned over the years.
"I don't fight the flares as hard as I used to, and they are shorter because of that. I used to feel that I had to be superwoman, and that price was too high to pay," says Goodman. "So now, I am older and more balanced in my response. I rest at an earlier point in the flare, and no longer beat myself up for having a flare."
To recover from flares and prevent others, Goodman is learning to pace herself-part of her doctor's orders. "That is something I have not mastered," she admits. "When I was 'well,' I had two speeds: very fast or not at all. It has been exceedingly difficult for me to learn to pace myself, regardless of the consequences."
Goodman meditates, swims, or walks to reduce stress. "I try to deal with those things I can deal with, and say 'Oh, well,' to things I have no control over. It doesn't always work, but it is a good goal."
Pridemore has also found helpful coping strategies. "I sleep more. I keep myself busy with things that don't take physical strength to accomplish." She also eats healthfully, exercises, spends time with friends, and volunteers with the local LFA. "Since I had to stop working, this gives me a sense of purpose."
Pridemore believes her efforts have helped reduce the occurrence and severity of her flares. "I know my body now and what I'm capable of doing. I know when I have to slow down and when I have to stop altogether."
Gilkeson encourages patients to be vigilant about their health. "The most important thing is to keep taking your medication and keep your doctor's appointments even if you are feeling well."
Goodman also has sage advice. "Learn all you can, find things that make it easier for you to cope, and don't let the flare interfere with taking care of yourself." She is also a big believer in laughing away the pain. "Humor has restorative powers, so use it as much as possible!"
Keeping a Flare At Bay
Doctors have identified healthy habits that can help you avoid triggering a lupus flare:
- Always take your medication as prescribed.
- Keep your doctor's appointments even if you are feeling well.
- Avoid taking sulfa drugs (sulfonomides) that are used to treat infections such as bronchitis and urinary tract infections.
- Limit your exposure to sunlight; apply sunscreen before going outside during the day, and wear sun-protective clothing and a wide-brimmed hat.
- Reduce your risk of infections.
- Get plenty of rest.
- Do not smoke.
Where Flares Strike
A lupus flare can occur in different organs and cause serious damage to your health if left untreated. It's very important to be aware of the signs, as a flare may require special medical attention.
Where: The Kidneys
Signs and symptoms: As many as half of the people with lupus have kidney inflammation, also known as lupus nephritis. There are very few symptoms of lupus nephritis, so it is important to have regular blood and urine tests. Excess protein in the urine often means the kidneys are not functioning properly. Weight gain and swelling in the feet, ankles, and legs due to water retention can be caused by excessive protein loss. Other signs include foamy, frothy urine and getting up several times during the night to urinate. If lupus causes a flare in the kidneys that is left untreated, the inflammation can lead to kidney damage that may in time require dialysis or a transplant.
What your doctor will do: Treatment for lupus nephritis varies according to the extent of the damage. Your doctor may order a kidney biopsy to check the type and extent of damage. Your doctor may also make changes in your diet to control your salt and calorie intake, as well as prescribe any of the following medications: diuretics (to eliminate excess fluid), anti-hypertensive drugs (for high blood pressure), anticoagulants (in case of blood clots), corticosteroids (to control inflammation), and immunosuppressive drugs (to suppress the activity of the immune system).
Where: The Heart
Signs and symptoms: In the heart, a lupus flare can attack the sac surrounding the heart (resulting in the condition called pericarditis), the muscle layer of the heart (myocarditis), the lining of the inside of the heart (endocarditis), and the coronary arteries. Pericarditis is the most common condition associated with lupus and can include sharp chest pains that may feel like a heart attack. Myocarditis symptoms include an unexplained rapid or irregular heart beat. Endocarditis causes the surface of heart valves to thicken or develop growths that can cause heart murmurs. This can become very serious if infection occurs, and can result in bacterial endocarditis.
What your doctor will do: To diagnose heart problems, your doctor may order a chest X-ray, echocardiogram, electrocardiogram, and blood tests. Treatment for pericarditis is usually anti-inflammatory medications. With myocarditis, your doctor will most likely prescribe corticosteroids, such as prednisone, or immunosuppressive drugs.
Where: The Nervous System
Signs and symptoms: The nervous system consists of the brain, the spinal cord, and the nerves throughout the body. When lupus strikes the nervous system, it is the most serious form of the disease and can cause headaches, high fevers, seizures, strokes, confusion, altered behavior, visual changes, and memory loss.
What your doctor will do: For many nervous system symptoms, your doctor may do a brain imaging study, such as an MRI scan. For seizures, an electroencephalogram may be done. If your doctor suspects that there is an infection, he or she may order a lumbar puncture. To treat memory loss or confusion, antimalarials and/or steroids may be used. Anti-convulsant medications are used to prevent seizures, and headaches may be treated with corticosteroids.
Where: The Skin
Signs and symptoms: Lupus can affect the skin in different ways. The first sign of a flare is often the malar or "butterfly" rash that appears on the cheeks and across the bridge of the nose. The rash usually heals within weeks without scarring. Another type of skin involvement appears as disk-shaped lesions, often after sun exposure. These lesions develop slowly, may take months to heal, and may leave scars. A third kind of damage, also related to sun sensitivity, appears as red circles on the arms, chest, and back. This rash, which may look scaly, like psoriasis, can take weeks or months to heal and may reoccur with repeated sun exposure.
What your doctor will do: To treat your skin rashes, you may be prescribed antimalarial or immunosuppressive medication, including pills and topical creams.