Positive Options for Living with Lupus (3 page)

Let us look at lupus symptoms as the patient experiences them: Malaise

“Malaise” is a French word for feeling generally unwell and uncom-fortable. 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.

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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 “post-viral fatigue,” fibromyalgia, or infectious mononucleosis (“glandular fever”).

Skin Rash

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 any-where 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
.
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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.

Arthritis

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 of fice and spent most of her time at the com puter keyboard. She was thir ty-three when she first noticed signs of arthritis—stiffness, swelling, and pain—

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by normal w ear and tear and doesn’t usually sho w 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 o ver a thr ee-day weekend. “On the f irst day we must have covered ten or fifteen miles, a distance we could usually handle easily,” she said. “But the f ollowing morning I jus t 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 pac ks of frozen peas on m y knees and ankles, unable to get up or go to sleep.” Shanon’s husband called the doctor, who thought it was pr obably rheumatoid ar thritis. 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.”

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have pleurisy at some time or another, and about 25 percent may develop pericarditis.

Kidney Problems

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.

Blood Problems

I have already mentioned that the malaise associated with lupus can partly be attributed to a shortage of red blood cells, a condition known as anemia. Supplies of other blood cells may also be affected, including white cells, which fight off disease, and platelets, one of the substances that cause blood to clot. Lupus sufferers are likely to feel tired (due to a shortage of red cells), to keep coming down with minor ailments (due to a shortage of white cells), or to bruise easily and heal slowly (due to a shortage of platelets). Taken on their own, these symptoms may not convince someone they need to see their doctor. They only become significant when part of a larger picture.

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A laboratory test called a complete blood count (CBC) is needed to reveal that significance. One or another of these blood problems affects nearly all lupus patients at some time, though they are not necessarily the first symptoms the patient notices.

◗ ◗ ◗

The symptoms described up to now, some of which people may ignore or put down to the normal ups and downs of life, are the same as the ones the doctor uses to make a diagnosis of systemic lupus erythematosus. Unlike a patient, however, who is limited to his or her own subjective observations, doctors have more sophisticated ways of confirming or excluding various diseases. We will look at these tests when we return to the subject of diagnosis in Chapter 3.

Please note: All the symptoms of lupus listed in this chapter, with the possible exception of hair loss following discoid lesions, clear up with treatment and leave no damage.

Neither lupus, nor any other form of arthritis, is contagious, and having one kind does not predispose you to developing another.

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Chapter 2

Who Develops Lupus,

Where, and Why?

Estimates of noncontagious, nonreportable disease prevalence rely on
some sort of official records such as hospital discharge logs, emergency-room visits, and reasons noted for school absences. It is thus possible to
estimate number of heart attacks, broken legs and children suffering
from severe asthma, for example, with reasonable accuracy.

Sheldon Paul Blau, M.D.

(see details of his book in Further Reading)

These days we like for numbers to be attached to illnesses. The science of who gets what, and where and when they get it, is called
epidemiology.
It is not a very exact science. So how do we get these numbers? If a disease is notifiable (see below), accurate statistics on its
incidence
(number of new cases) can be assembled. A “notifiable”

disease is one that must be reported to the authorities whenever a doctor treats it. In many countries this rule applies to serious or infectious diseases, such as tuberculosis, that have public-health consequences. Likewise, if a disease is fatal or puts a patient in the hospital, it gets recorded as a cause of death or hospitalization, and
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as a result there may be a record of who has succumbed to it. These sorts of records are known as mortality and morbidity (illness) data.

Lupus is not notifiable, and only rarely is it fatal, so we learn little about it from these records. The reason it was rated potentially fatal in the past was because only serious and fatal cases were recorded.

The large number of people who had lupus but didn’t die of it, or didn’t even see a doctor about it, was unknown—like the body of a vast iceberg with only the fatal cases visible above the waterline.

Prevalence and Incidence

In ordinary English we use these words almost interchangeably. In statistics they measure two slightly different things. Prevalence usually refers to the estimated population of people suffering from a disease at any given time. Incidence refers to the number of new cases diagnosed each year. A short-lived disease like flu has a high annual incidence but low prevalence; people get it one after another but then get better. A lifelong disease like diabetes has a low annual incidence but high prevalence; only a few people develop it each year, but once they have it they have it for good. Since lupus is chronic, a person suffering from a new attack may show up more than once in the incidence statistics, but when in remission, they will not appear in the prevalence figures.

Most experts believe that there are still many invisible, un-counted cases of lupus, particularly in underdeveloped countries where there are few doctors and more serious diseases to worry about. Consequently, estimates of the number of cases and of the percentage of the population that develops lupus are constantly being revised upward. The statistics that have been gathered are mostly from developed countries with good health services and a network of medical laboratories and research organizations dedi-cated to the study of diseases and their treatment. In these countries scientists are funded to do population studies to ascertain who suffers from diseases, and statistics for the rest of the world are often extrapolations (scaled-up estimates) based on available data. However, where lupus is concerned not all populations or geographic localities have the same experience.