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Positive Options for Living with LupusSelf-Help and Treatment
By Philippa Pigache
Hunter House Inc., PublishersCopyright © 2006 Philippa Pigache
All right reserved.
Chapter OneRecognizing Lupus
First thing in the morning you peer into the mirror and notice a bright-red rash. From the bridge of your nose it spreads in the shape of a butterfly across both cheeks. If you had spent the previous day unprotected in bright sunlight, you might wonder if you had a sunburn, but most probably you would think, "Heavens! I've caught something. Was it something I ate?"
That's the thing about skin. It is the largest organ of the body and, being on the outside, it is extremely noticeable. As teenagers we subjected every pimple, lump, or blemish to minute examination, and each one filled us with anxiety. As adults we learn to take the odd spot, bruise, or wrinkle in stride, but a rash is different. Perhaps it is our recollection of childhood ailments such as measles or chickenpox; perhaps it is the association between rash and eating or touching something dangerous; perhaps it is even a dim folk memory of historic but fatal epidemics like smallpox or the plague. Either way, we know, almost instinctively, that a rash is a sign of something wrong. If it's on your face you can't ignore it. If it sticks around for some daysyou will almost certainly take it to the doctor.
This is why the oldest recognized symptom of lupus is a rash. Other symptoms may be explained away, ignored, or not recognized as being part of an illness, but a rash on the face merits attention.
The Multiple Personalities of Lupus
Like the butterfly, lupus has more than one incarnation. Suppose that instead of a rash you wake in the morning with inexplicable fever, headache, or fatigue. Suppose every joint and muscle in your body seems to ache. Suppose your eyes are dry and scratchy or your hair comes out in clumps on your comb. Suppose your ankles get puffy, or that you become so depressed you feel life is hardly worth living. Would you conclude that you had something serious? Would you think it worth bothering the doctor about? Or would you decide that you were a bit run down or starting a cold, and take a few days off in the hope that it might all blow over?
This is why the multiple manifestations of lupus took so long to become recognized. Lupus is systemic-that is, it affects many organs throughout the body-and lupus is chronic: It comes and goes. Left untreated it may afflict you for days or weeks but then may inexplicably clear up and leave you in peace for months or even years. At first there is no permanent damage; your body temperature goes down, your skin clears, the aches and pains go away, and your hair grows back in. There may be clues that all is not well-the rash may flare up if you are out in the sun; other symptoms may recur if you eat certain foods or go through a period of stress. You begin to notice that your body has started reacting to things that didn't bother it before. But it is perfectly possible to live with the wolf known as lupus for years without being aware of it.
By describing it as chronic and systemic we explain when and where lupus occurs. By classifying it as an autoimmune disease we indicate which bodily system underlies what goes wrong in lupus. We will explore this in more detail in Chapter 3. Put simply, in lupus, antibodies, which are part of the body's defense system that are normally produced to ward off foreign invaders like bacteria or viruses, start misbehaving and attack the body's own tissues.
There are other diseases in which the body's defense system runs amok; most of these conditions have only recently begun to be understood, and even then imperfectly. The best known is type 1 diabetes, in which part of the immune system destroys the cells that manufacture insulin, a hormone the body needs in order to break down the sugars in food to supply energy. Autoimmune cells are also involved in some cancers, such as leukemia. More significantly for people with lupus, they are implicated in rheumatoid arthritis, a disease closely related to lupus and sometimes mistaken for it.
Relatives of Lupus
Once you know which family lupus comes from, and who some of its close relatives are, it becomes easier to understand why it is so easily mistaken for other, similar diseases. Lupus forms part of a big family called connective tissue diseases: CTD for short. Connective tissue is present all over the body, which is why the symptoms of lupus are so diffuse. It includes skin, as well as the lining or sheath of joints, tendons, ligaments, blood vessels, nerves, and major organs like the heart and lungs. Arthritis, the name for conditions that affect joints (arthros is Greek for "joint"), is the best-known member of the CTD family. There are over one hundred forms of arthritis alone, so they might better be known in the plural as arthrites. Other CTDs include systemic sclerosis (from the Greek word for "hardening"), which affects skin and connective tissue all over the body; polymyositis and dermatomyositis (from the Greek myo, for "muscle," and derma, for "skin"), also called PM-DM, which involves the inflammation of various muscles; plus a host of other rare disorders and syndromes. We will look at them in more detail in Chapter 9. Some CTDs defy any of the established labels; doctors call them "mixed connective tissue diseases" or MCTDs.
It's all very confusing. That's because connective tissue is ubiquitous-found all over the body-which means not only that it can give rise to a vast range of symptoms, but also that there is a wide overlap of symptoms among CTDs. North American and European medical institutions have made some progress toward inscribing diagnostic criteria in stone, but ultimately the naming of diseases is an imperfect science. In many cases uncertainty prevails, a situation that proves frustrating for both patient and doctor.
It may seem pointless or frivolous to waste time and effort over precise medical labeling (what doctors refer to as establishing a differential diagnosis). But it plays a vital role in deciding what treatment is likely to work and what outcome to expect.
Presenting Symptoms: The Patient's View
There are two ways of considering symptoms: the patient's view and the doctor's. They obviously overlap, but their significance is viewed differently. The patient has the intimate, day-to-day experience of living in his or her body. Some days feel better than others, and there are all sorts of minor aches, pains, bumps, or blemishes that come and go, signifying nothing. How a person reacts to them depends somewhat on whether he or she is a bit of a hypochondriac or tends to keep a stiff upper lip about things.
Doctors know this and do their best to make allowances for it. They figure out that Mrs. Smith thinks she has developed an allergy every time she has a touch of indigestion, whereas they don't see poor old Mr. Thomas until he has taken to his bed with pneumonia. But the patient's variable response to illness is a particular problem for doctors when the disease in question slips in and out of the forest every now and then leaving almost no trail, like lupus.
Janet's Story Janet discovered she had lupus in 1978, when she became pregnant. She was in her late twenties. "You name a complication of pregnancy, and I had it," she says. "Blood clots, raised blood pressure, edema [puffiness caused by fluid retention] up to my knees. And then a blood clot started blocking my heart. My obstetrician said I was lucky to have survived the pregnancy and that I should not risk another one." Fortunately he also asked Janet several probing questions and discovered that as a teenager she had suffered from a short attack of painfully swollen hands. Her doctor had put it down to rheumatoid arthritis. She also told her obstetrician that she had developed curious lumps in her legs when she had taken the birth control pill. The obstetrician said she might have "collagen disease" and recommended that she see a rheumatologist. Janet was fortunate that by then laboratory tests had been developed that provided a more conclusive diagnosis than the one offered by the shifting kaleidoscope of symptoms found with CTDs. Systemic lupus was diagnosed, and by the time Janet was ready to undertake another pregnancy her doctors made sure she received treatment that prevented all the previous complications.
Let us look at lupus symptoms as the patient experiences them:
"Malaise" is a French word for feeling generally unwell and uncomfortable. It has that "can't-put-your-finger-on-it" character that makes it likely to be overlooked as a significant symptom of real illness. It may be accompanied by a slightly raised temperature or a headache. Malaise, with or without fever, is the most common feature of lupus and probably, when the patient looks back, the first he or she experienced, though the patient didn't identify it at the time. It is most likely caused by the disseminated (widespread or systemic) nature of lupus. Connective tissues all over the body may be inflamed-some in the joints, others in the brain-which can lead to headaches or depression. Furthermore, a lupus sufferer may be anemic, meaning that the supply of important oxygen- and glucose-carrying red cells in the blood is depleted, a condition that causes feelings of malaise. If a person goes to the doctor with these symptoms, lupus as a cause can be easily missed or mislabeled as "postviral fatigue," fibromyalgia, or infectious mononucleosis ("glandular fever").
Only about a fifth of those with lupus experience the classic butterfly rash as their first symptom. Ultimately about half have it. Nevertheless, the skin is one of the organs most commonly involved in the illness. The rash doesn't hurt or itch, though it may burn slightly if exposed to sunlight. In fact, the lupus rash is a bit of a werewolf. It takes several forms and appears in diverse places. It is sometimes faint and rosy and, because it often follows exposure to ultraviolet light, can occasionally be mistaken for sunburn. Other times it takes the form of disc-shaped, scaly, red patches, which can appear anywhere on the body and can leave scars when they clear up. They can occur in the scalp, causing hair loss that may be permanent. (Hair loss, which strictly speaking is another skin symptom, may occur even in the absence of a rash. On those occasions it invariably regrows.) These raised plaques are the discoid lesions that occur in about 15 percent of lupus sufferers, the vast majority of whom have none of the other lupus symptoms. For this reason, in the past, this skin condition was often classified as a separate illness, discoid lupus erythematosus. Because their symptoms are so mild, it is likely that patients with discoid lesions slip between the population statistics of lupus; in many parts of the world they may not even consult a doctor for their symptoms.
Sometimes the rash takes the form of small blisters (vesicles), which are caused by inflammation in the small blood vessels. These may appear on the face, neck, elbows, palms, tips of the fingers, or soles of the feet. Blisters on the feet can easily be missed unless there are accompanying symptoms. Vesicles can also crop up on the mouth in the form of painless ulcers, and occasionally in the vagina. About one in eight lupus patients has these blisters at some time or other.
Faced with any of these types of rashes, a doctor is on much firmer ground than with malaise. Lupus will definitely be among his or her likely suspects.
Before we leave the topic of the skin manifestations of lupus, it is worth mentioning a very common symptom: About a third of lupus patients are photosensitive-that is, they react in an extreme way to ultraviolet light, with inflammation, burning, and blistering. Some also react to fluorescent lights. In terms of diagnosis, this symptom has the advantage of being closely associated with lupus but with few other CTDs.
As we saw in Janet's case, lupus is easily mistaken for rheumatoid arthritis. In both illnesses the joints most commonly affected are those of the hands, arms, feet, and legs. Joint pain is the first-noted symptom in about three-quarters of lupus cases, and over 90 percent of diagnosed cases experience arthritis at some time. The most common pattern is to experience stiffness, tenderness, and swelling of the fingers and wrists upon waking. Unlike the joint pain in rheumatoid arthritis, which in many cases is unremitting unless treated, lupus arthritis usually comes and goes and varies in intensity.
Shannon's Story Shannon worked in a busy insurance office and spent most of her time at the computer keyboard. She was thirty-three when she first noticed signs of arthritis-stiffness, swelling, and pain-in her hands. "It's a bit early for osteoarthritis," she said to herself. (Osteoarthritis is not caused by inflammation but mostly by normal wear and tear and doesn't usually show up until someone is in their fifties.) She also wondered about repetitive strain injury or carpal tunnel syndrome, two conditions that often bother people who type a lot. She took acetominophen and tried to ignore it. But then she and her husband went on a walking vacation over a three-day weekend. "On the first day we must have covered ten or fifteen miles, a distance we could usually handle easily," she said. "But the following morning I just couldn't get out of bed. The weekend was ruined. The pain continued into the week. I couldn't go to work and ended up lying in bed with packs of frozen peas on my knees and ankles, unable to get up or go to sleep." Shanon's husband called the doctor, who thought it was probably rheumatoid arthritis. But when the doctor asked about Shannon's medical history Shannon remembered that she'd had mouth ulcers a few years back. The doctor sent samples of Shannon's blood to the laboratory. The results confirmed that it was lupus.
Heart and Lung Problems
The heart and lungs are both surrounded by membranes made of connective tissue. The heart membrane is called the pericardium; inflammation of the pericardium is called pericarditis. The membranes enclosing the lungs are called the pleura; inflammation of the pleura is pleurisy. When these membranes become inflamed the patient experiences pain, especially during breathing. These symptoms are unlikely to be the first ones experienced by anyone with lupus, but if a patient has fever and malaise that he or she attributes to flu or a bad cold, the condition may escalate to bronchitis, pneumonia, and then pleurisy. If things get to this stage the person will almost certainly be admitted to the hospital. Lots of tests will be done to identify the problem, resulting ultimately in an accurate diagnosis, even if a number of others are considered first. As one patient put it, "There isn't a single test that says unequivocally, 'Yes, you have lupus.' You sort of back into it after visiting several other possibilities." Studies show that between 33 and 45 percent of lupus sufferers have pleurisy at some time or another, and about 25 percent may develop pericarditis.
Half of lupus sufferers develop some degree of kidney involvement. These symptoms, although unlikely to be among the first encountered by someone with lupus, can have a potentially fatal outcome. Prior to the 1940s, before modern understanding of the disease and modern drugs to treat it, it was kidney failure that gave lupus such a bad name.
Kidney problems first manifest in the form of puffy ankles and possibly puffy fingers and knees. When the kidneys are unable to adequately filter waste products from the body, fluid builds up, starting in the ankles and working its way up (due to gravity). When fluid remains pooled in the tissues it causes swelling and discomfort called edema. Edema is easy to identify because if you press a finger into the swelling the indentation does not fade for some minutes. Edema is a signal that the kidneys are not functioning. It is quite common in the latter stages of pregnancy, even for women without lupus. It is confirmed by a simple urine test that detects the presence of protein fragments normally filtered out of the urine by healthy kidneys.
Excerpted from Positive Options for Living with Lupus by Philippa Pigache Copyright © 2006 by Philippa Pigache. Excerpted by permission.
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