The Patient's Role in Controlling Lupus

Lupus can be a serious disease and it is often very difficult to predict what will be the disease course in an individual patient. What is extraordinary is that lupus patients have in their hands some power to keep their disease under control by avoiding certain things. I know of few other illnesses that offer this opportunity.

Protect yourself from the sun

Sun exposure is known to be a cause of troublesome skin rashes in 50% of all patients. Not only do these rashes make patients feel self-conscious about their appearance, they may also ruin their plans, mean extra trips to the doctor and require more tests and treatments (various cortisone ointments or pills). To prevent this problem it is recommended that all patients use a sunscreen on all the exposed parts of the body. If you are not already sun sensitive, the odds may be as high as 50% that you will be eventually.

It is also important to know that sun exposure can cause a flare of internal lupus where the problems can be much more serious. The simple use of a sunscreen can help you avoid this danger. Staying out of direct sunlight does not protect you from the reflection of ultra-violet rays; you still need to use a sunscreen.

Look for a sunscreen with a sun protection factor (SPF) of at least 25, which blocks both UV-A and UV-B rays. This number is written on the front of the container and the higher the number the more protection you get. Paying more does not mean that you will have better protection than with a cheaper brand. You may have to try different brands to find one that feels comfortable and that does not stain your clothing. Remember to apply the sunscreen at least 30 minutes before going out to allow it to penetrate the skin and dry. Apply it again after swimming or a bath, even if you are using a water resistant brand. If you go skiing in the winter, don't forget to apply sunscreen to your face and ears. Sunscreens may also be useful for patients who are sensitive to fluorescent lights.

Do not smoke

Lupus patients must do all they can to ensure their general good health and this is reason enough to emphasize the importance of not smoking. In the past 30 years, the life expectancy has improved considerably for lupus patients, thanks in part to the use of steroids. Now that lupus patients are living much longer, some of them are victims of a long term complication of steroid use, premature coronary disease or heart attacks. While several other factors may contribute to this problem in lupus patients, the association of smoking with heart disease is such that lupus patients should not smoke.

Consult your physician about birth control

Birth control pills containing estrogen do not cause SLE but they are thought to increase the risk of flares in patients who already have the disease. Some birth control pills contain mostly progesterone or low-dose estrogens and these may cause fewer problems. For the lupus patient, barrier methods (condoms or diaphragms) and spermicidal agents are the safest forms of contraception. I advise that you discuss with your physician the use of birth control pills and alternative methods of contraception. If you have to use birth control pills, they should be prescribed by a physician who is familiar not only with contraception but also with SLE.

Choose the best time to become pregnant

If you want to become pregnant, your physician will be able to help you choose the best time. A lupus flare in pregnancy is of great concern because two lives are affected. The ideal time would be when your disease has remained in remission without any medication other than a small dose of prednisone or no prednisone at all (other medications may be harmful to the baby). SLE is also associated with a higher risk of miscarriage, premature delivery and other serious problems. As a result, pregnant lupus patients are best treated in High-Risk Pregnancy Clinics. If you become pregnant by accident, you should see your physician as soon as possible.

Know your antibiotics

Drugs containing sulfonamides (certain antibiotics used in the treatment of infections such as urinary infections) are best avoided as they may mimic a lupus flare by causing fever, arthritis, skin rashes, sunsensitivity and other problems. In general, lupus patients should not take antibiotics (or any drug) unless their physician feels that it is absolutely necessary. Canadians are plagued by colds during the cool season, however, most colds are caused by viruses and viral infections are not helped by antibiotics.

Do not experiment with special diets

It is certainly important that lupus patients maintain balanced and healthy eating habits, however, there is no special diet that can cure lupus. Any advertisement that claims otherwise is quackery. If such a miracle diet existed, I would be the first one to recommend it to my patients as it would make their lives (and mine) so much simpler. So-called "natural" products are today's fad and I used to be accepting whenever a patient told me they were eating these "natural" foods. Recently, however, there have been reports of lupus flares in patients taking alfalfa tablets and I think patients should not experiment with natural products without informing their physician.

I am also asked about the use of vitamins and, if a patient is on high doses of prednisone, I prescribe calcium and vitamin D supplements. Otherwise, there is no vitamin supplement that is scientifically proven to be helpful in SLE.

Say NO to quackery and to miracle cures
Some of the quack treatments being proposed to patients nowadays are so astonishing that I sometimes have the feeling we are back to witchcraft and the Middle Ages. Quackery is disgusting because it is based on lies and exploits the patient's vulnerability as a sick human being, hoping to get better. Unfortunately, there are always some people ready to make a dollar out of this hope.

Many quack remedies require that patients stop taking their medications so as to "cleanse the body". When drugs, such as prednisone, are needed to keep lupus under control, it is very dangerous to stop taking them. These miracle cures are made even more dangerous because patients often have to travel to another city, province or country to get them. If a serious flare should result, patients may well be far away from the medical team that can best help them and be unable to travel. The fact that some patients are prepared to take these risks shows how profoundly their lives have been affected by lupus.

The only way for SLE patients to maintain the same rapid pace of improvement is to invest their money in scientific research, not quack remedies.

As you have seen, there are many things that you can do, or avoid, to help keep your lupus under control. However successful the treatments or however careful patients are to avoid flares, lupus remains a chronic disease that will not always be controlled. In the next chapter, I will discuss how patients can learn to cope with the physical and emotional effects of living, day after day, with SLE.

Treating Lupus with Medications

The vast majority of SLE patients can be treated successfully. Some patients have mild SLE that does not require any treatment although regular follow-up is still necessary. Even SLE patients who have required treatment can reasonably hope that a remission will occur when little or no treatment will be needed.

Ideally there should be only one physician in charge of supervising your illness and treatment and this approach is generally followed in the University Hospital Lupus Clinics across Canada. In these clinics, most patients are treated by physicians who have specialized in rheumatology (called rheumatologists). These physicians have received a particularly specialized training in lupus. As needed, consultation with other specialists is sought and many patients are also followed by physicians specialized in skin care (dermatologists), blood diseases (hematologists), kidney diseases (nephrologists) or immunologic diseases (immunologists).

In dealing with lupus, two approaches are used. First, the symptoms are treated if necessary and secondly, people with SLE are advised to avoid certain things that are known to cause a flare in some patients.

Medications to Treat the Symptoms of SLE

Drugs for Arthritis

Arthritis is probably the most common symptom of SLE and when it is mild no treatment may be needed. If the pain is more severe, many patients are satisfied with a basic pain medication such as acetaminophen (for instance, Tylenol or Atasol). Non-steroidal anti-inflammatory drugs (called NSAIDS for short) are often used when acetaminophen doesn't control the pain of arthritis. There are over 20 different drugs in this family of medications, including aspirin. Although some of the NSAIDS are available over the counter, it is best left to your doctor to decide which one you should use. Not every drug works well for every patient and you may have to try several NSAIDS before finding one that relieves your pain.

The NSAIDS may cause many side-effects, from stomach upset to changes in kidney function. Several of these side-effects can cause trouble as they may imitate lupus problems or complicate lupus problems that already exist. For this reason, NSAIDS are often prescribed to be taken for a short time only, with instructions to decrease the dose as the arthritis improves.

Hydroxychloroquine and chloroquine

The drugs hydroxychloroquine (Plaquenil ®)and chloroquine (Aralen ®) are very useful in treating sun sensitive skin rashes such as the discoid or the subacute types of lupus rashes. In many cases, rheumatologists prefer to use hydroxychloroquine because it is considered to be less toxic. However, dermatologists may prefer to use chloroquine to treat lupus rashes. This drug may also be tried if hydroxychloroquine is ineffective. While a sunscreen may often provide sufficient protection, some patients do get skin rashes in spite of the proper use of a good sunscreen. In these patients, hydroxychloroquine may be started in the spring and continued until the late fall.

Hydroxychloroquine is also used to treat lupus fatigue, arthritis and other milder symptoms of SLE. A recent study in several Canadian University Hospital Lupus Clinics has shown that hydroxychloroquine can help prevent flares in patients who have taken this medication continuously over a six month period.

At the relatively low dosage used in SLE (the dose is calculated based on the patient's weight), hydroxychloroquine seems to cause few problems. Apart from the pill's bad taste, the most common unwanted effect is some stomach upset. However, if hydroxychloroquine and chloroquine are taken in a high dose and over a long period of time, they may accumulate in the background of the eye (retina) and cause a loss of vision. In rare cases, blindness may occur. In over ten years of supervising patients on hydroxychloroquine, I have never seen a single case of damage to the eye but my more senior colleagues tell me that I probably will, eventually.

A series of precautions are taken to make certain that damage to the eye does not occur. Dosage is based on the body weight and, if possible, hydroxychloroquine is only used from spring to fall when the sun is the strongest. (However, many patients must take hydroxychloroquine for much longer periods.) Before hydroxychloroquine or chloroquine is started, patients must have their eyes examined by an eye doctor (ophthalmologist) and this examination should be repeated every six months. As well, patients started on these drugs should receive an Amsler grid and be told how to use it. The Amsler grid is a sheet of paper with many small squares (like a piece of graphing paper). Patients should inspect the squares regularly and if the lines are not clear, straight and square, they should see their eye doctor immediately. In my experience with hydroxychloroquine, eye damage will not get worse and sight will not be threatened if the drug is stopped at this early stage.

Cortisone and Prednisone

The single most important factor explaining the remarkable improvement in life expectancy of SLE patients has been the discovery of the steroid drug cortisone in 1948 and, shortly after, the related drug prednisone. Although the term cortisone continues to be used by patients and the public, SLE patients are now treated with the much more effective drug prednisone. This drug is the best inflammation-fighting drug available at the moment. Prednilosone is also used in Canada and, for some SLE patients, is the best drug available. These drugs are sometimes referred to as steroids, but they are not related to testosterone and the other anabolic steroids used by some athletes to build muscle.

The decision to prescribe prednisone is never made lightly by your physician. Prednisone is considered if the symptoms are not being well controlled by other treatment(s), if there is a concern that SLE may quickly get out of control unless prednisone is used or if SLE is severe and perhaps life-threatening.

On the bright side, prednisone is necessary for the successful treatment of many lupus patients. For some, it is a lifesaving drug and the single best drug available. However, this powerful drug has a dark side too. There is no doubt that prednisone can cause a wide variety of side-effects in both the short and the long term. If you are taking prednisone, it is important for you to know what these problems are. This information is included in Appendix Two at the back of the booklet, "Side-effects of prednisone". Patients will be happy to know that research is underway to find steroid drugs which would have the powerful inflammation-fighting action of prednisone with fewer unwanted side-effects.

At times, patients may be tempted to stop taking prednisone or to reduce or increase their dose. Perhaps they are fed up with the side-effects or perhaps they are feeling better or worse. It is important that patients taking cortisone understand how cortisone works and why stopping or changing the medication without the supervision of the physician is dangerous.

Cortisone is a chemical that is normally produced by the body, not a foreign substance. In fact, the body has a vital need for cortisone. When prednisone treatment is started, the body responds by stopping its own production of cortisone and the adrenal glands that produce it get sluggish and lazy. If prednisone is used for a period of time and then suddenly stopped, the body may not be able to start producing enough cortisone for some time. The result could be a life-threatening lack of cortisone (adrenal insufficiency). So prednisone should never be stopped suddenly. If you are tempted to stop taking your prednisone, do not do it. Rather, discuss the situation with your physician.

Other patients decrease or increase their dose on their own. This is unacceptable. If you feel well enough that you think prednisone could be decreased or if you feel worse and think that it should be increased, call your doctor. Never forget that both you and your doctor have worked very hard to improve your health. You should not put your improvement at risk by making any changes to your therapy.

Cyclophosphamide and Azathioprine

Cyclophosphamide (Procytox®) and azathioprine (Imuran®) are powerful drugs which are used in the treatment of certain SLE patients. One of these medications may be used when prednisone alone is unable to control SLE, when prednisone causes too many serious side-effects or when it is thought that combining the drug with prednisone may be better than using prednisone alone. These drugs work in SLE by changing the body's immune response and they are referred to as "immunosuppressive", "immunomodulatory" or "cytotoxic" drugs. Cyclophosphamide and azathioprine are usually given as pills and are generally not used together. As with other kinds of treatment, the decision to use one of these drugs is made on the basis of the symptoms and the condition of the patient. The dosage is based on the patient's weight.

Like all powerful drugs, the immunosuppressives have potential serious side-effects. The three most serious unwanted effects are changes to the bone marrow (where blood cells are produced) which may lead to a decrease in the number of red or white blood cells or platelets, increased risk of infections including infections to which we normally have resistance and a slightly increased risk of developing certain types of cancer. The risk of infection is particularly high when cyclophosphamide or azathioprine are combined with high doses of prednisone. Cyclophosphamide can also be associated with bleeding from the bladder (haemorrhagic cystitis) and failure of the ovaries to produce their normal output of hormones (ovarian failure). In practice, azathioprine is considered less toxic than cyclophosphamide. Obviously, these are very serious problems and patients taking these medications are watched very carefully by their physicians. Periodic blood tests are mandatory throughout the course of this therapy.

These problems have led to a recent report from the American National Institutes of Health on the use of intravenous cyclophosphamide in patients with severe kidney involvement in SLE. In this form of treatment, a much larger dose is given by vein (approximately 10 to 15 times more than the daily dose when given in pill form). By repeating this treatment every 4 to 12 weeks, kidney inflammation was very much reduced as were the serious side-effects (including risk of cancer). Many University Hospital Lupus Clinics in Canada now use this treatment for serious kidney involvement as well as for certain types of serious SLE problems. The procedure can be done on an outpatient basis or during a short stay in hospital. It should be kept in mind that although this form of treatment is better, it is still far from harmless, is used only for SLE patients with very serious disease and that our experience with it is short.