Positive Options for Living with Lupus (16 page)

Hopefully, early intervention will prevent necrosis from progressing to this point. The first-line treatment, once the problem is detected, is with drugs. In fact, patients identified as being at high risk are usually offered prophylactic (preventive) treatment to head off even the possibility of joint damage. Some risk factors can be reduced by modifying the lifestyle (cutting out cigarettes, alcohol, and fatty junk foods), and others, like high blood pressure, anemia, and elevated cholesterol, can be treated with relatively side effect–free drugs. If bone damage is detected early, a surgical procedure called
core decompression
will be recommended
.
Under anesthetic, a small core of tissue is withdrawn from inside the blood-deprived area of bone to relieve the pressure and also to encourage the formation of new, fine blood vessels and healthy bone. This procedure has been in use for more than thirty years and has a good record of helping patients avoid more radical surgical treatment.

Once osteonecrosis reaches the stage at which it shows up on X-ray images, core decompression may be too late. Replacing the affected joint may then be necessary, especially in the case of a load-bearing joint like the hip or knee. This may sound radical, but it has become an almost routine procedure for many people with osteoarthritis (the common wear-and-tear kind). Artificial knee and hip replacements have a very satisfactory history of restored move-POL text Q6 good.qxp 8/12/2006 7:39 PM Page 99

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ment and reduced pain. Ruptured tendons also have to be repaired by surgery.

Drug-Induced Lupus (DIL)

This last lupus-like condition is not so much a lamb dressed up as a wolf as it is a completely human-made wolf. To be precise, this is an
iatrogenic
form of lupus, meaning it is caused by doctors or medical treatment (
iatros
is Greek for “physician”). As one doctor puts it,

“Iatrogenic basically means it’s our fault.”

Mitch’s Story

Like a number of men who had worked hard, lived well, and not gotten enough exercise, Mitch’s blood pressure crept up in middle age. High blood pressure increases the chances of heart disease and stroke, and a good doct or always insists on correcting it. In Mitch’s case modifying the lif estyle—cutting out salt, sof t-pedalling the booze, and getting out onto the golf course more—

didn’t achieve adequate results. What’s mor e, at one of his regular checkups, his physician discovered that he had an irregular heartbeat, one of the early signs of hear t disease. She pr escribed a drug to bring down the blood pressure and another to stabilize the hear tbeat. At first, things im proved, and so did Mitch’s handicap.

Some months later Mitc h began to suffer from extreme fatigue. The cardiologist whom he was seeing told him to get more exercise. “How can I ge t more exercise when I f eel exhausted from the moment I wak e up,” he complained to his wife. And then one morning he f ell over at the f irst tee. His leg jus t gave way beneath him. He got up and tried to go on, but a few yards farther down the fairway his other leg gave out. He went to his family doctor. She did a thor ough examination and discovered that he was suf fering from muscle weakness in both legs. His muscles were wasting away. She took blood and told Mitch she thought he had dev eloped DIL. She s topped the drugs he had been taking and prescribed known, safe alternatives. She also POL text Q6 good.qxp 8/12/2006 7:39 PM Page 100

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sent Mitch off to a physiotherapist for a cour se of exercise to build up his wasted muscles.

More than one hundred different drugs have been reported as causing lupus-like conditions (see box “Some Drugs That Induce Lupus Symptoms,” below). The phenomenon was first noted in the 1940s.

We know that some drugs (antibiotics, for example) can cause a flare in someone who already has lupus, but these drugs cause it in otherwise lupus-free people. The two drugs Mitch reacted to are those most often implicated, though they are not prescribed frequently these days.

Some Drugs That Induce Lupus Symptoms

◗ Hydralazine (blood-pressure lowering agent)

◗ Procainamide, quinidine bisulfate (for irregular heart rhythm)

◗ Sulfasalazine (anti-inflammatory used for colitis and rheumatoid arthritis)

◗ Minocycline (antibiotic used for acne)

◗ Penicillamine (antibiotic)

◗ Isoniazid (antibiotic used for tuberculosis)

◗ Chlorpromazine (used for serious mental illness and severe nausea)

◗ Methyldopa (used for Parkinson’s disease)

◗ Phenytoin (an anticonvulsant, used for epilepsy) Some of the very latest drugs, for example biological agents developed to treat rheumatoid arthritis, have also been implicated in drug-induced lupus.

What’s the difference between drug-induced lupus (DIL) and the genuine article? Symptoms are usually, though not invariably, less severe; there may be fatigue, arthritis, widespread rashes, swollen lymph glands, pleurisy, or pericarditis, but it is rare for the condition to cause kidney damage. Blood tests do not reveal the characteristic pattern of antibodies, but reveal some similarities and some differ-POL text Q6 good.qxp 8/12/2006 7:39 PM Page 101

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ences, depending on the drug culprit causing the trouble. The principle difference is that all symptoms disappear soon after the drugs are withdrawn, leaving no lasting damage.

In the United States, as many as fifty thousand people are thought to suffer from DIL, though Graham Hughes says that in his experience it is “rare” in the U.K. Clearly the incidence of any iatrogenic condition is not a natural phenomenon. If the at-risk people and culprit drugs can be positively identified, there should ideally be zero cases to report. However, it is not absolutely clear why some people develop DIL with certain drugs. One theory holds that some people metabolize drugs more slowly and may therefore be more vulnerable. It seems likely, as with lupus itself, that some genetic factor contributes to lupus being triggered by drugs.

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Chapter 10

Lupus and Pregnancy

History provides us with a dramatic example of the effect lupus can have upon childbearing.

Queen Anne’s Story

Queen Anne, of England, died in 1714, tormented as much by her physicians’ misguided efforts at treatment as by her own agonizing illness. Her short life had been plagued by ill health, not least by an exhausting succession of miscarriages. In the first eighteen years of her marriage she had seventeen pregnancies, including eleven miscarriages, and only one child survived infancy. Since one of her primary aims in life, as the last of the Stuart line, a dynasty plagued by religious strife for nearly a hundred years, was to produce a Protes-tant heir, this was a bitter failure.

In his book
The Sickly Stuarts,
Professor Fredrick Holmes, of the University of Kansas Medical Center, writes, “Systemic lupus erythematosus remains the best explanation for Anne’s ill-starred obstetric history and the disabling rheumatic disease she suffered in the last decade or so of her life . . . which led to her premature death from a cerebrovascular event—a stroke—common among sufferers of this disease.”

In the views of both Professor Holmes and Graham Hughes, Anne had lupus with antiphospholipid antibody syndrome (APS), a
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blood condition that, unchecked, causes pregnancies to fail at between three and five months because of thrombosis: blood clots blocking the small blood vessels to the placenta, which feeds the fetus. In modern times, if the antibody is identified, a single aspirin a day can prevent miscarriage. Reflecting on this remedy Professor Holmes writes, “In all likelihood in the early eighteenth century the equivalent was actually available as salicylic acid in herbal prepara-tions containing willow bark, although its efficacy in Anne’s condition could not have been known at the time. . . . [C]learly Anne had the antiphospholipid antibody.”

If Queen Anne’s doctors had known what we know today, history might have followed a different course; the house of Hanover might not have inherited the throne of England, and George III might not have lost the American colonies!

As recently as twenty-five years ago, doctors usually advised women with lupus not to get pregnant because recurrent miscarriage was a known symptom of the disease. But with a better understanding of the reasons behind these miscarriages the picture has changed. Studies of lupus pregnancies reveal that, whereas forty years ago less than half of them resulted in live births, these days between two-thirds and three-quarters are successful. And these figures are averages; in some hospitals, what is known as the “take-home-baby” rate is even higher, although in about a quarter of lupus pregnancies there remains a risk of premature birth.

Facing Up to the Risks

Among the barrage of laboratory tests lupus patients undergo during diagnosis is one that is central to the outcome of pregnancy: the test for APS, an illness we discussed in Chapter 5. APS is now recognized as a distinct autoimmune disease in its own right, but whereas only about 5 percent of the general population exhibit it, a very high percentage of lupus sufferers do. It is associated with increased risk of the formation of obstructive blood clots, which in turn increase the danger of heart attack or stroke and, when they POL text Q6 good.qxp 8/12/2006 7:39 PM Page 104

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obstruct the blood supply to the placenta, which nourishes the fetus, starve it of oxygen and cause miscarriage. The syndrome was first identified by Graham Hughes, of St. Thomas’ Hospital, London, and is also known as Hughes’ syndrome. (See Graham Hughes’

book on lupus or Triona Holden’s
Positive Options for Living with Antiphospholipid Syndrome (APS)
in “Further Reading.”) People with APS often have a wide range of other symptoms—

seizures, migraine, joint pain and inflammation, avascular necrosis (see Chapter 9), leg ulcers, anemia—most of them traceable to problems with circulation and thrombosis. All APS-related difficulties are made worse by smoking, high blood pressure, diabetes, and high levels of cholesterol and other
lipids
(soluble fats) in the bloodstream.

Two further antibodies can cause trouble in pregnancy. One is called
anticardiolipin
(ACL), and the other is lupus anticoagulant (LAC). These two work in different ways to increase problems with circulation and heart function, and to increase the risk of miscarriage.

A diagnosis of APS requires both clinical symptoms—thrombosis or a history of miscarriage—and positive laboratory tests for ACL or LAC. As with other autoimmune diseases, the cause of APS is unknown, though studies support the idea of a genetic susceptibility triggered by a viral infection.

Any woman who is undertaking a pregnancy and is diagnosed with APS, including those who also have lupus, will be put on an aggressive pharmaceutical regime to correct the condition: drugs to lower high blood pressure, high cholesterol, and other blood lipids, and also to control diabetes. The risk of blood clots can be reduced by prescribing aspirin or more powerful “blood-thinning” drugs such as heparin.

It goes without saying that she will also be adjured to strictly follow rigid pregnancy health behaviors
.
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medication to the letter, and, above all, stay in close contact with her rheumatologist as well as her obstetrician. Keeping lupus and APS under control during the pregnancy is absolutely central.

The medical team will inevitably rule out a home delivery.

Women with lupus—or women with any chronic condition that poses a risk to either mother or child—need to be in a good hospital under a specialist’s care when they give birth. There is an ever-present risk of premature birth with a lupus pregnancy, and that dictates having access to a unit equipped to care for the premature, or otherwise distressed, newborn.

Lupus Drugs During Pregnancy

It goes against instinct to be taking powerful drugs during pregnancy. And some drugs prescribed for lupus are indeed contraindi-cated, but surprisingly few. Steroids, probably prednisolone, even in quite large doses, do not appear to do harm. The mother’s body breaks down this drug in the placenta in such a way that limits the amount reaching the fetus, though it is important that taking it doesn’t lead to the mother’s gaining excessive weight. Some steroids do cross the placental barrier and may be used deliberately when an effect on the baby is intended. If a premature birth looks likely, a steroid called dextramethasone may be given to accelerate the development of the baby’s lungs and reduce the breathing problems babies suffer when born early.