Zero in 50

Zero in 50. Thursday, November, 20, 2008 marked an unfortunate anniversary. It was 50 years ago the U.S. Food and Drug Administration (FDA) last approved a drug to specifically treat lupus.

Side effects of Prednisone

Prednisone is the single most important factor in improving the outlook for lupus patients. It is usually effective in bringing lupus under control and it saves lives. However, there is a price to be paid for this success. If we observe what happens to patients taking high doses of prednisone, there is no doubt this drug can cause a wide variety of side-effects.

Short-term Side-effects:

*Not everyone has the same side effects or same severity of side effects. The longer and the stronger the dosage the more likely sideffects will occur. Side effects are also reversable to a degree when a dosage is lowered or when an individual is taken of the medication.

Side-effects:

- Swelling of the face, often referred to as "moon face" or "chipmunk cheeks"; some patients feel
ugly and say that they do not recognize themselves in the mirror. Remember, these changes
are reversible.
- A hump on the upper part of the back; this hump is made of fat, not bone.
- Bloating or swelling of the abdomen.
- Weight gain; prednisone may cause a great increase in appetite. Weight gain can be controlled
by a low calorie diet, by exercise and by avoidance of salt. Avoid salty foods and do not add
any salt.
- Stomach problems; to ease the burning, try taking prednisone with food. This problem may
require anti-ulcer medication.
- Mood changes; sometimes the change is for the better. However, depression may be made
worse by prednisone.
- Insomnia; patients may have difficulty sleeping at nights.
- Shakiness; patients may have feelings of being "hyper: or that "things are running fast inside
my head".
- Weakness of the thigh muscles; patients may have difficulty in climbing stairs, getting out of
the bath or getting up from a chair or toilet seat.
- Interruption of the menstrual cycle; periods may stop altogether.
- Increased risk of infections; patients may have more infections including some caused by
germs that the body is normally resistant to.

Long-Term Side-effects:

- Easy bruising of the skin; bruising from prednisone use often happens without any obvious
injury and may require that blood clotting ability be checked by the physician. When
prednisone is the cause of the bruising, blood clotting will be normal. This easy bruising
disappears when prednisone is stopped and is not associated with any risk of internal bleeding.
- Stretch marks; these may occur on the upper body, the arms, the abdomen and the thighs.
Some patients have this problem while others do not. Unfortunately, there is no treatment and
the marks are permanent.
-Excessive growth of body hair; this hair growth usually appears on the face and will stop when
prednisone is decreased. The hair that has grown will tend to stay but it can be effectively
removed by using a hair-removal cream.
- Cataracts; these are a cloudiness of the lens of the eyes that cause a decrease in vision.
Occasionally, surgery may be required.
- Osteonecrosis; this condition means "dead bone" and most frequently affects a bone in the hip
joint called the femur (other bones may be involved as well). Osteonecrosis is becoming a more
important cause of pain and disability in lupus patients. If this problem is found early,
worsening of the process may be prevented by performing a surgical procedure. In many
patients, the damage caused by osteonecrosis eventually comes to a stop. In some patients,
where damage to the bone has been severe, an artificial joint may be needed.
- Osteoporosis; this results from a loss of calcium from the bones and often leads to fractures,
particularly in the spine. These may be a major cause of pain and disability. However, this
process is at least partly reversible if prednisone can be stopped. If prednisone must be
continued, this side effect may be decreased by exercise, by eating foods rich in calcium and by
taking extra calcium and vitamin D as prescribed by your physician. These preventive
measures should be started as soon as a high prednisone dose is begun.
- Heart attacks; several factors (including smoking, high blood pressure and high blood sugar)
combined with long term prednisone treatment may lead to a narrowing of the blood vessels of
the heart and early heart attacks. This risk can be decreased by maintaining a reasonable
weight, controlling blood pressure and, most importantly, not smoking.

Symptoms which may Indicate a Lupus Flare

It is important to note that this list of symptoms applies only to patients with a diagnosis of systemic lupus erythematosus. In addition, remember that lupus is often a disease that repeats itself. Be on the lookout for a return of symptoms that were experienced at the beginning or onset of your disease


- Persistent (continuing) fatigue which is out of proportion to what you consider your normal
- fatigue
- Persistent weakness
- Aching all over without any obvious reason
- Persistent fever (In order to establish that you have fever, take your temperature every six
hours and write down the results.)
- Persistent loss of appetite
- Involuntary weight loss
- Excessive and persistent hair loss
- Recurrent (repeated) nose bleeds
- Sores on the roof of the mouth which burn when spicy foods are eaten
- Unexplained rash anywhere on the body
- Persistent hives
- Skin ulcers
- Recurrent pain in the joints
- Swelling of one or several joints
- Persistent joint stiffness upon wakening in the morning
- Chest pain which increases when breathing in
- Unusual shortness of breath
- Coughing up blood
-Persistent, unusual headache
- Persistent nausea and vomiting
- Recurrent or persistent pain in the abdomen
- Persistent and worsening swelling of the feet and legs
-Persistent swelling of the eyelids
- Blood in the urine or in the stools

Flare for Fashion

‘Flare’ for Fashion - Event and Ticket Information
Event Information
DATE : Wednesday October 15, 2008PLACE : Liberty GrandADDRESS : 25 British Columbia Rd., Exhibition Place

For the purchase of a table or more information please email events@lupusflarefoundation.ca or call 905.278.6286 for further details.

Coping with Lupus

Lupus patients must learn to control their lupus, rather than be controlled or "invaded" by it. I believe that lupus patients can learn to fight back and join with their physician to gain control over their illness.

The twin burdens of lupus
The burden of having to live with lupus is two-fold; on the one hand, there is the physical burden of the illness and its potential seriousness and, on the other hand, there is the intense fear that so often accompanies the illness, even in patients who are doing quite well. Through education, the patient with lupus can learn to fight back on both fronts.

Coping with the illness
Learning to live with lupus is like anything else in life; like becoming a parent, starting a new job, making an investment or buying a car. One has to learn how to do it and this learning does not happen overnight. Life is made up of pleasures and of obligations. Clearly, lupus can be quite an obligation but, depending on the person, the burden of this obligation may be lighter or heavier.
One key to coping with the illness is to get organized. Managing the day to day aspects of your lupus will help you to be in better control. Below is a list of some practical tips that will help you take care of your lupus responsibilities.


- See your doctor regularly and do not postpone your visit because you "feel so well". Make a list of all your questions and bring it to your next appointment.

-Make a list of all your pills or medications to each appointment. This helps your doctor identify "that little yellow pill" that was prescribed by "that other physician". Knowing all your medications, your doctor may be able to cut down on the number you are taking, which is nice and saves money too.

-Buy a multi-compartment pill box in any pharmacy so that you can lay out your pills for each day or week. This makes it easy to check if you have forgotten to take your medications.

-If your doctor orders a urine collection over 24 hours, collect your urine from Sunday to Monday morning and bring it into the hospital on your way to work. Carrying around a jug of urine may be embarassing but do your best not to lose a drop. Important decisions about the management of your disease will be made, in part, based on the results of this collection. (Urine collection should not be done during menstruation.)

-If your blood pressure is checked by your family doctor, the nurse at work or by yourself, don't forget to bring these readings to your next appointment. (Readings done at the Lupus Clinic are often higher and this can cause unnecessary concern.)

-Purchase a thermometer. If you feel that you are running a fever, take your temperature every six hours and wrrite down the results. If the problem continues and is not explained by a cold or the flu, call your physician.

-Be prepared for blood tests to be taken regularly, even when everything seems fine. SLE is unpredictable and the sooner a flare is detected, the better.

The physical burden of lupus
Over the past several years, there has been an extraordinary change in the concept or thinking about lupus. Lupus is no longer seen as a disease of crises but as a chronic illness. Many years ago, when the treatments for lupus were so limited and the death rate so high, lupus was a disease of catastrophes or crises. Patients would often go from one major physical problem to another, losing more and more ground with each flare. The physician's main concern was to get the patient out of crisis, if at all possible. What happened to the patient in-between was thought to be much less important.

With the use of prednisone and because of better knowledge, lupus has become a different illness, often with a two-phase pattern. The first phase is that of the serious illness or the period of increasingly severe symptoms that lead to diagnosis and treatment. In the second phase, the lupus patient is no longer seriously ill but rather chronically ill. In this phase, some patients have long remissions while other patients continue to have bothersome symptoms. The symptoms, though not life-threatening, will interfere with their ability to lead a normal life and will require continuing treatment, often in the form of a low dose of prednisone.

Why is it important that lupus patients be aware of this? Because lupus flares can still happen in this chronic phase and because nowadays patients can help their physician in identifying when a flare is starting. How is this possible? Because lupus is a disease that repeats itself, the symptoms that the disease has caused in the past are often the symptoms that tend to recur.

Today, most lupus flares do not happen overnight. Rather, patients will notice a slow but steady decrease in their well-being, often over a period of several days, weeks or months. Since the major problems of lupus often follow the milder symptoms such as increasing fatigue, pain in the joints, increasing hair loss, etc., the watchful patient will know that something is going on. Nobody knows his/her body better than the patient. It is the patient, not the physician, who will feel if the disease is flaring.

Knowing about lupus is important
It is important for the patient to be educated about the symptoms of lupus in order to identify when flares are beginning. When I have seen a patient for some time after diagnosis and the disease has been brought under control, we talk over their illness and make a list of the symptoms that were experienced at the onset or beginning of SLE. We are usually able to trace those symptoms back to a period several months before the diagnosis was made. Often these are symptoms that the physician cannot see such as increasing fatigue and loss of appetite.

While lupus is a disease that tends to repeat itself, patients may, during the course of their illness, develop new symptoms or warning signs. This makes it important for them to know the most common problems so they can recognize them and know they are important. I have included a list of these symptoms or warning signs at the back of this booklet in Appendix One called "Symptoms which may indicate a Lupus flare".

In the chronic phase of lupus, these symptoms may show up again and signal the start of another flare. The patient who notices these signs can bring them to the attention of the physician who will do a careful examination and order tests to check for other evidence. When caught at this stage, a small increase in the dosage of medication may be all that is necessary. If the physician is uncertain that a flare is occurring, and false alarms are frequent, the physician will want to see the patient more frequently in the weeks to come. At any rate, telling your physician will have made possible the early identification of a possible flare. Action will have been taken and a crisis will have been avoided.

Symptoms that don't go away
Part of the burden of lupus are those symptoms that hang on for several months after a flare is over or seem to never go away. I am thinking, in particular, of chronic fatigue, the lack of drive, the inability to do as much as one could before. These symptoms are frustrating and a major feature of lupus as a chronic illness. After the more severe part of a flare is over, these symptoms are often the hardest to combat.

often tell my patients that it is easier to treat the severe symptoms of lupus than to correct the fatigue or, as I call it, "the last 10 or 20% of well-being that is missing in order for you to feel perfect". This is a hidden disability resulting from the disease. You will have to learn to live with these symptoms and to adjust your activities to a less busy schedule that takes your limitations into account. Changes to medication can be made, such as increasing slightly the dose of prednisone or starting the drug hydroxychloroquine, to try and treat these symptoms. In many cases, the patient just has to learn to live with it.

Things that make you feel good or bad
Another way for lupus patients to lessen the burden of lupus is to look for things or activities that make them feel good. Patients should not feel guilty for doing exercises, eating a favorite food or, by all means, avoiding some activities they don't like. Throughout a lifetime of chronic illness, lupus patients will always find some things that make them feel better and they should feel at ease, not guilty, in indulging. And if, sooner or later, patients find a particular activity makes them feel worse, why not avoid it? Listen to your body.

The burden of fear in lupus
Lupus sufferers will experience the disease for the rest of their lives. It will modify their ability to work, will change their marital and family life, and will also change their social life. It may change the way they look and it will change the way they feel. New and strong emotions will surface and these have to be dealt with. Patients may feel sad, worried, angry and, perhaps, haunted by thoughts of dying even when the disease is under control. The fear of "what will come next", of the dreaded fatigue, of not being able to function, of the side-effects of the therapy, of going into a flare, of death - these are the burdens of the patient with SLE.

I believe that what eases these fears also eases a part of lupus and that is why I feel that patient education is so important. If I was not absolutely convinced of this, I would not have accepted the task of writing this booklet! The fact that you are reading it indicates your willingness to be educated about your illness. Ignorance of lupus generates fear but knowing about lupus generates confidence and will help you cope. So let us look at some of the more common fears experienced by lupus patients.

The fear of failing
The fear of inadequacy is a common trap for the lupus patient. A typical example is the young mother with lupus who is unable to look after her child and her household chores because she feels so tired and worn out. Because she cannot do these things, she feels guilty and inadequate as a wife and mother. Then, the feelings of guilt may become feelings of sadness and depression. If you feel inadequate and guilty, have your spouse read this paragraph and perhaps you could both discuss this with your physician.

Fear and depression
In some patients, these feelings of guilt and depression can become a very heavy burden and the physician may become concerned. Lupus is so complex that, at times, it is not clear if the emotions of the patient are related to the disease or to the treatment or to the normal difficulty in adapting to a serious illness. It could also be a combination of all these things or, perhaps, something completely unrelated. In this setting, I tell my patients that I need the opinion of a psychiatrist to help me sort things out and provide them with the best treatment.

Asking for the advice of a psychiatrist is no different from asking for the opinion of a dermatologist or any other specialist. However, many patients become upset, thinking that they are mentally disturbed. It is an unfortunate product of our society's attitude that while it is acceptable to be extremely sick physically, nothing should ever go wrong with the mind. Remember that, if ever needed, the advice of a psychiatrist can be extremely useful to the lupus patient.

The fear of a flare
Another aspect of the fear burden is the question "Is my disease getting worse? Am I becoming sick again?". Patients naturally look to their physician to answer this question. While their physician can often reassure them, the patient must learn to give the physician time to make a decision or come to a conclusion. Lupus is not a disease of absolute "yes" or "no". I do not hesitate to tell my patients that I need a little bit more time to know in what direction the wind is blowing and the patient must learn to live with this necessity. So give your physician a chance; lupus is a difficult disease to treat and physicians are constantly concerned about either undertreating or overtreating it.

The fear of a crisis
Many patients are afraid that their lupus will suddenly get worse, and this fear can leave them with a feeling of impending disaster. I think that most patients can now be reassured about this as, contrary to the situation of 30 years ago, physicians are better able to keep the disease under control. I believe that with regular follow-up, most patients need not fear becoming unexpectedly and severely ill with lupus.

The fear of death
It is not surprising that lupus patients in the middle of a serious flare or a crisis would think about death. Sometimes these thoughts linger on even after the crisis has passed and patients wonder how close to death they have been. Indeed, having a serious chronic illness (or dealing with it as a close family member or physician) does remind us that some day we will die. The lupus patient may wonder if lupus will make this happen sooner rather than later.

This fear is normal and there is no magic button that will make it go away. Like so many things about lupus, it has to be faced and dealt with. Often, an honest discussion with the physician will help the patient to remember that, even when things seem blackest, there is room for hope. Remember too that the survival rate is now over 90% and that each year the outlook is improving for lupus patients.

The patient's choice
What can patients do to alleviate or ease these fears? Patients should discuss their fears with their physician. Of course, this is easier said than done. No one likes to think or talk about fear or death. Patients are told to have confidence in their doctor. Again, this is easier said than done. However, a good, trusting relationship can develop, provided that the physician and the patient give each other time. I see lupus not only as a "chronic illness" to which the patient must learn to adapt but also as a "chronic doctor" to whom the patient must learn to adapt and vice versa. With time, patients can benefit tremendously by being honest and open with their doctor.

Often, patients hold back from asking a question fearing what the answer might be. In fact, that answer could prove reassuring. Don't keep your questions locked up inside, just ask! By doing this, you will not only be dealing with your fears in a positive, healthy way, but you will also be helping your physician to make decisions about treating your illness. Indeed, one of the major challenges of treating lupus is to separate lupus symptoms that need treatment from symptoms that do not. The physician must be constantly deciding what is dangerous to the patient and what is just troublesome. By asking questions and talking about their fears and concerns, patients can help their physician in making those important decisions.

What else can a patient do?
As you have seen, SLE is a complex disease. It is not always easy for your physician to answer all of your questions. Becoming a member of a support group of lupus patients can be extremely helpful in finding support and additional information, not only for yourself, but for your spouse and family as well. Talk to other patients. You will be amazed and reassured to find other people who have gone through what you are now experiencing. Through public meetings with health care professionals, newsletters, telephone networks and informal gatherings, lupus support groups will help you cope.

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.