Positive Options for Living with Lupus (2 page)

For the moment forget about the wolf. Think instead of the butterfly, a word used to describe a rash that appears on the faces of many people with lupus. It fans out from the bridge of the nose across the cheeks and ranges in color from rose pink to angry red.

The fact that lupus affected other organs besides the skin was not understood until the end of the nineteenth century, when it was discovered that lupus involved inflammation of the joints
(arthritis),
fatigue, and a number of other physical symptoms, including potentially fatal kidney damage. Once the link between the skin rash and
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fatal kidney disease had been established, lupus got very bad press.

Medical textbooks printed in the first half of the twentieth century spoke of it with gloom and despondency. Ninety percent of sufferers are women of childbearing age, so female lupus sufferers were warned against getting pregnant or going out in the sun (lupus can increase the skin’s sensitivity to sunlight). Sadly, a few medical professionals still cleave to this opinion. If you or someone you love has lupus, I am pleased to inform you that this is old-fashioned rubbish.

Before refined laboratory tests were developed to identify the condition, the only recognized cases were people who had a severe case of the disease and had remained untreated for many years. Now that lupus is more readily diagnosed and treated, it has been established that many people have it, but only quite mildly. In fact the current belief is that there are thousands of “sleeping” lupus sufferers who go peacefully to their graves for some quite unrelated cause, unaware that they ever had the illness.

Those whose condition is diagnosed cannot yet be offered a cure, but increased understanding of what goes on in the disease, combined with modern treatment, takes the bite out of lupus. The wolf may not be dying out, but it is certainly a much less threatening creature than thought to be in the past.

Nevertheless, lupus is still a mysterious illness. Like its name-sake, it lurks in the shadows of the forest and comes out at night to leave unexplained damage and devastation. And, like the animal in the fable wearing sheep’s clothing, it is difficult to recognize and often gets mistaken for something else. My hope is that this book will explain some of the mysteries surrounding lupus.

Hints for the Reader

You can read this book from beginning to end, or simply dip in and out, picking out what interests or concerns you. Only the first two chapters, which explain the key features of lupus, are essential to understanding the rest of the book. Chapters 4 and 5, on diagnosis, are important if you think that you, or someone close to you, may POL text Q6 good.qxp 8/12/2006 7:39 PM Page 3

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be a sufferer. Chapters 6, 7, and 8, on treatment and management, are important for someone already confirmed with lupus. I have tried to use titles and subheads that will help you find what you are in search of. You can also refer to the Index.

Difficult words are explained in the text. Those printed in
italic
type
when they first appear are also listed in the Glossary, located at the end of the book. Some interesting information on the general story of lupus is separated out in boxes.

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Chapter 1

Recognizing 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 days you 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.

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5

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

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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 arthrit
es.

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 ubiq-uitous—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.

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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.

Medical Specialists Who Treat Lupus

Because lupus affects so many different parts of the body, a patient can come into contact with a vast array of medical specialists. Im-munologists specialize in the immune system. Dermatologists treat skin diseases. Rheumatologists specialize in diseases that cause inflammation (lupus is a form of arthritis caused by inflammation).

An ophthalmologist, a specialist in conditions of the eye, may be consulted if the eyes are affected. A nephrologist, a kidney specialist, may also be consulted, because in its worst manifestations lupus can damage the kidneys. If the disease affects the membranes that enclose the heart or lungs, a cardiologist may be consulted. If a lupus sufferer becomes pregnant she may develop a blood disorder and need to consult a hematologist (hem- is from the Greek for

“blood”). Many lucky lupus sufferers never get referred to specialists but instead remain in the care of their family doctor; even they will need the services of a pathologist, who specializes in analyzing the sophisticated laboratory tests that tell you what’s wrong and how treatment is progressing.

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.

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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 sa ys. “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 luc ky to have survived the pregnancy and that I should not risk another one.”

Fortunately he also ask ed Janet several probing questions and discovered that as a teenager she had suf fered from a short attack of painfully sw ollen hands. Her doct or had put it do wn to rheumatoid arthritis. She also t old 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 of fered by the shifting kaleidoscope of symptoms found with CTDs. Systemic lupus was diagnosed, and b y the time Jane t was ready to undertake another pregnancy her doct ors made sur e she r eceived treatment that prevented all the previous complications.