Positive Options for Living with Lupus (15 page)

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disease. A diagnosis of Sjögren’s is extremely difficult; it has been known to take as long as two or even eight years!

Meanwhile, treatment is necessarily symptomatic. A special low-concentration eyewash containing cyclosporine, a powerful immunosuppressant, may be prescribed, or an oral antimalarial like hydroxychloroquine. For dry mouth, prescription drugs are available that stimulate the production of saliva, for example pilocarpine (Salagen). Regular dental checkups are vital. There are also mechanical topical treatments for the eyes, in the form of drops called

“artificial tears” (which are usually satisfactory), and for the mouth, in the form of “artificial saliva” sprays (which are usually not satisfactory). It is possible to block the tear ducts surgically to retain moisture, and of course sufferers need to avoid smoke, strong winds, and any form of airborne irritant, and to use eye makeup extremely sparingly. Sufferers should beware of proprietary over-the-counter products that promise to cure sore, reddened eyes. These contain
vasoconstrictors
that make blood vessels constrict, aggravating the dryness and discomfort of Sjögren’s. For more information on Sjö-

gren’s syndrome and how to cope with it, I recommend Sue Dyson’s
Positive Options for Sjögren’s Syndrome
(see Further Reading).

Fibromyalgia

Fibromyalgia
, or fibromyalgia syndrome (FMS), was only recognized by the American College of Rheumatology (ACR) and included in the official diagnostic manuals in 1990. The name derives from three Greek root words—
fibro-
(fibrous or connective tissue),
my-

(muscle),
algos
(pain)—but its most prominent symptom is debili-tating fatigue. Some experts believe it is the same condition known as chronic fatigue syndrome, now usually called myalgic encephalitis or ME (more Greek, meaning “brain inflammation”).

Characteristically, the pain of FMS is spread throughout the body, not confined to joints. In addition, people with FMS may experience disturbed sleep patterns, difficulty in concentrating, migraine headaches, anxiety and depression, hearing and seeing problems, and heart-valve abnormalities (see the box “A Hole-in-POL text Q6 good.qxp 8/12/2006 7:39 PM Page 93

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the-Heart Link to Headaches,” below). Things are usually worse in the morning. Some studies suggest that sufferers may also have unusual variations in hormonal or other biochemical patterns, and some also suffer from Raynaud’s phenomenon. Brain-imaging technology confirms that FMS sufferers actually process pain signals differently from most people; their pain is amplified, as though the volume has been turned up. For more information on FMS, I recommend Katrina Berne’s
Chronic Fatigue Syndrome, Fibromyalgia
and Other Invisible Illnesses
(see Further Reading).

A Hole-in-the-Heart Link to Headaches

Thousands of migraine sufferers, including many of those diagnosed with FMS or ME, may have a small hole in the heart that can be corrected by a simple patch. A clinical trial currently in progress is investigating those who suffer from severe migraine accompanied by pins-and-needles and by a visual disturbance known as aura: flashing lights, bright spots, blind spots, or like seeing through a snowstorm. Research suggests that as many as 17 percent of migraine sufferers may fall into this category. Researchers believe that these migraine sufferers may have a common heart defect called patent formen ovale (PFO), shared with up to a quarter of the population, the majority totally unaware. A PFO is a hole, usually harmless, up to a centimeter across, located between the two upper chambers of the heart. The researchers believe that in these cases some blood that should be filtered through the lungs may bypass them through the PFO, allowing chemicals that contribute to migraine to get to the brain.

The connection between holes in the heart and headaches was discovered serendipitously when a number of migraine sufferers who were among stroke victims and deep-sea divers with “the bends” underwent the patch procedure to correct their PFOs and discovered that their headaches also disappeared.

Like the other disorders described in this chapter, FMS can exist alone or with one or more CTDs. Up to a third of those with a CTD, including lupus, may have it. As with nearly all these conditions, the cause is unknown. There is some evidence of familial, probably genetic predisposition, with the likely trigger being a virus POL text Q6 good.qxp 8/12/2006 7:39 PM Page 94

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or other infection. Treatment is again targeted on symptoms. Unlike lupus, FMS does not respond to steroids, antimalarials, or immunosuppressants, and NSAIDs are usually inadequate in the face of the severe pain, which may demand stronger painkilling drugs called
opiates
. Sometimes antidepressants seem to relieve the pain: either an older tricyclic drug called amitriptyline (Elavil) or one of the newer selective serotonin reuptake inhibitors (SSRIs, e.g., Prozac). These are usually given in lower doses than are required for anxiety or depression. Trials are going on to see if an antiseizure drug, gabapentin (Neurontin), which has been successful in reducing various sorts of nerve pain, might be effective in treating FMS.

To date the most successful ways of treating FMS are through self-help techniques. Sufferers learn to avoid extremes of cold or heat; physical or mental stress; and either too much or, conversely, too little exercise. Living in a climate with warm, dry weather is ideal. And the best physical activities seem to be things like regular, gentle cycling on flat surfaces, t’ai chi, gentle stretching, and swimming in a heated pool. A physiotherapist may be able to devise a routine for FMS sufferers. Professional massage and acupuncture have helped some people.

Libman-Sacks Endocarditis

Two more American physicians bequeathed their names to posterity in 1923 by describing a condition that is estimated to affect from 10

to 20 percent of people with lupus.

In Chapter 1, I explained that the sheaths that line the heart and lungs are made of connective tissue and are therefore vulnerable in lupus and other CTDs. Pericarditis, inflammation of the lining of the heart, is relatively common in lupus.
Endocarditis
involves the interior of the heart (
endo
is Greek for “inside”), in particular, the valves between chambers that regulate the flow of blood and prevent it from going the wrong way. In Libman-Sacks endocarditis (LSE), tiny wartlike growths develop on the valves, causing them to leak. Most people who develop it also have antiphospholipid an-POL text Q6 good.qxp 8/12/2006 7:39 PM Page 95

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tibodies (APS, or Hughes’ syndrome, introduced in Chapter 5 and dealt with in detail in Chapter 10).

Doctors detect the possibility of LSE from listening to the heart (see box “Listening to the Heart,” below). Heart valves affected by LSE cause a distinctive type of murmur behind the sound of the heartbeat. If the doctor thinks he or she can hear it, he or she will probably confirm suspicions by asking the hospital to do an
echocardiograph
. Echocardiography passes sound waves into the chest, where they are reflected back to the instrument from the solid structures inside, providing an “echo” of the structure. The instrument forms a picture of the heart structure from the reflected sound waves; it is the same principle as radar or bats’ sonar.

Listening to the Heart

What does a family doctor, or indeed a cardiologist, hear when he or she sticks the stethoscope on your chest?

The heart is a synchronized pump—or, if you prefer, a pair of pumps, synchronized to pass blood between their chambers and push it through the lungs and around the body. The skilled listener hears the one-way valves opening and shutting and blood being driven from one chamber in the heart to another. There are four distinct sounds in a normal heartbeat, usually described as making a noise rather like “lop-dop.” The explosive consonants in “lop-dop”

are made by the valves opening and closing. A doctor learns to detect the sound of a healthy heart even though there may be slight variations in the patterns of sound. The doctor will note the speed of the beat. Does the heart race, or even gallop? He or she may also hear noises in between the distinct beats of the heart. These are called murmurs, and may indicate that the various valves are not working properly, for example, leaking and allowing some blood to go the wrong way.

These days there are many sophisticated ways of assessing the heart’s performance: by plotting electrical impulses from the heart muscle (electrocardiogram), by constructing images of the blood flow (magnetic resonance imaging or MRI), or by analyzing the sounds (echocardiograph). The stethoscope applied to the medically trained ear dominates front-line diagnosis because it is portable and doesn’t need to be plugged in.

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In itself LSE is not dangerous. Problems arise if the warts growing on the valves become infected. Various things can cause this but the most common is dental treatment. The mouth is a veritable hotbed of bacteria. And bacteria may get into the bloodstream if the body’s internal mucous lining is broken during dental procedures or during medical procedures such as undergoing a cervical smear or a
colonoscopy
(an internal investigation of the colon using a fiber-optic camera).

If an infection does occur in the heart valves the symptoms are fever, irregular heartbeat, difficulty breathing, and, if not treated, heart failure. To avoid this risk, LSE sufferers are prescribed antibiotics as a precautionary measure in advance of any risky dental or medical procedure.

Avascular Necrosis

Avascular necrosis
is the exception to the general rule that lupus, unlike rheumatoid arthritis, is not progressive and does not do permanent damage to joints. (See box “Cell Death and Recycling,” on the facing page.)

Joint pain is a feature of lupus for many. A small number also suffer actual damage to some part of the joint. The condition is known by a variety of names, most of which describe what it is
not.

It is not damage caused by trauma (atraumatic); it is not damage caused by infection (aseptic); what it is caused by is reduced blood supply, hence the name
avascular
necrosis. (Diminished blood supply leads to shortage of red blood cells, hence disruption to the delivery of their oxygen payload, and hence tissue damage.) There is uncertainty about how many lupus sufferers experience joint necrosis; it may be as many as 40 percent, or it may be as few as 5 percent.

Two parts of the joint may be damaged by avascular necrosis.

First, bone, particularly the head of the bone in load-bearing joints like the hips, knees, and shoulders. (These are frequently the sites of other forms of arthritis, both wear-and-tear osteoarthritis and rheumatoid arthritis.) When bone is affected the condition may be POL text Q6 good.qxp 8/12/2006 7:39 PM Page 97

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Cell Death and Recycling

For animals, death is the end. If it comes during sleep, in the full-ness of years, it is reckoned slightly better than if the animal is cut off in its prime, but it is still the end. It is a little different with cells.

Cells are what living organisms are composed of, whether a tree, a goldfish, or your old Uncle Harry, and they come and go in a constant cycle throughout the lifetime of the organism. It’s as though Uncle Harry were a waterfall. (Don’t be difficult; just try to imagine it.) The water in the waterfall is always changing, but the waterfall itself is still the same waterfall. That’s how it is with the cells of the body. They are continuously produced (give or take a few exceptions like the corneal surface of the eye and some brain cells), go about their business, and then die off in an orderly and preprogrammed manner to get reabsorbed into the body—a sort of cell recycling.

This end is known in medicine as apoptosis. You have met it in connection with the cells of the immune system, some of which fail to do this in autoimmune diseases.

Necrosis is a Greek word for “death.” (You may recognize this root in words like necropolis, a place where the dead are buried, or necrophilia, a disturbed erotic obsession with a dead person or people.) The term “necrosis” is used in medicine to describe unscheduled and unhealthy death of cells or some part of the body—cell death as decay.

called
osteonecrosis,
adding the Greek term for “bone” to “necrosis.”

Second, many more lupus patients suffer damage to tendons (the cable-like structures that tether muscle to bone) caused by disrupted blood supply. This is known as tendon rupture, but is also described as “fraying” or “tearing.” The affected tendon does not need to be load-bearing, and the most common site is the fingers.

The rupture causes a sudden collapse of the bone supported by the tendon and can be quite alarming. If it is one of the bones of the hands, the person may drop something; if it is a leg tendon, he or she may fall down. One patient described it by saying, “I’ve severed tendons like they were spaghetti, including the tendons in both of my thumbs—one of them twice. The latest was a
patellar tendon
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of my thumbs I was just picking up a bag of oranges! I’ve had more surgery than anybody I know.”

Lupus patients with a history of arthritis, blood disorders like anemia, circulatory problems, high blood pressure, elevated cholesterol, diabetes, heavy drinking or (perish the thought) smoking are more at risk for avascular necrosis. If bone is affected (the previously mentioned osteonecrosis) the first symptom is likely to be pain in the joint itself, or possibly pain referred to a nearby area. If the necrosis is not checked, there may also be pain at rest. Without treatment the necrosis causes actual loss of tissue in the joint, leading to collapse and fracture. At first, the damage does not show up on X rays, though it does on more sensitive imaging technologies.