Living With Pain

Managing life today can be difficult. Managing life with pain is even more challenging, but it is possible. There are ways to balance your life so that you can live the way you choose, rather than your illness dictating. The key is for you to become an active member of the treatment team. It is important to understand what your responsibilities are to ensure a near normal life style. Your health care team will do all they can to provide the necessary medical care, but you will be responsible for much of the day-to-day routine.

First you must clearly understand what your needs are. Personal needs can range from a balance between getting the proper rest and physical exercise to taking medications and reducing stress. While managing illness and pain are complex issues, the majority of the key components are simply common sense and good living skills. Things such as good nutrition, open communication with family and friends, asserting yourself so that your needs are met, and finding balance between activity and rest are all key components in successfully managing pain.

It is important to recognize your limitation to prevent becoming overly tired or risking increasing pain levels. Staying within your limits can enhance your ability to think clearly and concentrate on important tasks. Understanding personal needs will provide a means to develop a workable plan so that you can incorporate important tasks into your daily routine.

Journaling is an excellent way to ensure necessary tasks are completed while brining you one step closer to independence. Balancing daily activities with necessary rest periods is easier when you have a written overview of your endurance.

Your journal can also provide you with insight into daily stressors. Reducing stress is vital in the fight against pain. Muscles that are already painful will experience increased pain as your stress level increases and your muscles tighten. There are relaxation exercises and audiotapes available to teach ways to recognize stress and reduce tension.

Recognizing and understanding feelings are another important component to successful pain management. When you ignore feelings, they do not go away, but can show up as increased tension, feeling out-of-sorts, or even anger. Dealing with feelings as they occur can greatly reduce both stress levels and pain. Your journal, with its daily entries, can become your road map to wellness and provide you with a sense of empowerment.

Daily exercise should also become a routine activity. Simple stretches can strengthen muscles, improve circulation and improve energy levels. Ask your doctor about an exercise program designed to fit your ability. Invite your family to exercise with you or try exercising to some lively music.

When you plan your day, keep in mind your need to pace activities according to your ability for that particular day. A simple way to remember the importance of pacing is with the word PACE.

P is for prioritizing your tasks to ensure that the most important ones get done first.

A is for planning your actions to ensure the best result.

C is to remind you of your physical comfort. If a task creates increased pain levels, then perhaps you need to ask for help.

E is for energy. Energy levels are never the same from day to day.

You need to consider how much energy you have at the beginning of each day to ensure you are working and playing within your ability.

By combining PACE – Priorities, Actions, Comfort and Energy – with your personal commitment to a near-normal life, you can begin to feel like a person rather than a patient.

By Penny Cowan, Founder and Executive of the American Chronic Pain Association. Reprinted with permission from NewSLEtter, the Bay Area Lupus Foundation newsletter.

=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.

Looking Your Best with Lupus

It's difficult to be confident when you're self-conscious about the way you look. Many people with lupus experience changes in appearance due to the medications they take or the lupus itself, everything from weight gain to hair loss. The following suggestions are not intended to make you feel more self-conscious about yourself; they're just ideas to make you more comfortable with your appearance and boost your self esteem when lupus is wearing you down physically.

It's a Skin Thing

Malar (butterfly) rashes or stretch marks can make you feel pretty self-conscious. The malar rash, which appears in a lot of lupus patients, spreads across the cheeks and the bridge of the nose like a rosy butterfly. There are products out there that you can use to minimize the appearance of this rash. More than just makeup (and a little more pricey than most drugstore brands, too), the products are specifically designed to cover hard-to-hide skin problems. Look for Dermablend or ask a cosmetician to recommend some products. As for stretch marks, you can use these products on those too, but if you have too many to cover, stick to concealing clothes instead. There are anti-stretch creams on the market and eventually those red and purple marks will fade into a paler shade of white. If you're having trouble getting the makeup just right, book yourself a makeover – it might even be fun!

Rapunzel No More

Your hair's falling out! While this can be awful, there are ways to deal with it. First of all, be nicer to the hair you do have. Avoid any products that irritate your scalp or any products that may make you lose more hair, e.g., stick to coated elastic, not rubber, bands. . . on second thought, pony tails can be stressful to your hair. While brushing, be gentle. To cover up thinner hair, experiment with hats or scarves. If hair loss is severe, you can find great synthetic or real hair wigs and they're easy to take care of.

Wait, Now I'm Growing Hair in Other Places!

Strange, isn't it? The hair on your head falls out while hair elsewhere - say, your upper lip – grows like a weed. This is a side effect of certain medications. To get rid of the fuzzy stuff, get your hands on some hair removal products like creams, waxes, natural sugar or honey, bleach, electrolysis – all dedicated to making you fuzz free. If you're not a do-it-yourselfer, visit a salon; they're pros at this kind of thing.

Indulge Yourself

Don't forget the most important part of yourself to cultivate; your inner beauty, of course! All the makeup in the world won't help you if you don't believe in your inner beauty. Don't let lupus (or anything else) make you think otherwise.

Edited for clarity; reprinted from Work Out, the employment newsletter for people living with lupus and other chronic illnesses.

=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.

Young People Beware: Alcohol and Lupus Don't Mix

There's no way to soften the bad news about drinking alcohol. While anyone is at risk from alcohol's negative side-effects, people who take prescription medications (and also some over-the-counter-ones) put themselves at an even greater risk of suffering health troubles.

The problems occur because alcohol can change the way the body uses, or metabolizes, certain medications. Alcohol is absorbed through the intestinal tract and shuttled to the liver, where chemical "knives" called enzymes break it into smaller molecules. Trouble is, alcohol causes the body to make more of these enzymes, especially when someone drinks regularly (even so-called "social" amounts of alcohol). Some of these enzymes are the same "knives" that break down medications so the body can use them. In producing more enzymes, the liver metabolizes medications faster. The bottom line: medications are sent into the bloodstream much faster and to a larger extent than when you don't drink. This can be dangerous, intensifying both the positive and negative side-effects of medications.

It is a myth that you can avoid these alcohol medication side-effects by taking medications while you are not drinking: the liver is still in "over-drive," producing more of these enzymes for some time after you drink. Another myth: "You have to drink hard liquor to suffer dangerous consequences." Beer and wine are just as likely to cause problems.

Of the 50 most frequently proscribed drugs, more than half contain ingredients that react adversely with alcohol. Among the negative effects are seizures, headaches, nausea, vomiting, mental confusion and coma. Don't forget that medications that are available without a prescription can also react adversely with alcohol. People with lupus, for example, often take Tylenol to alleviate pain. Drinking any amount of alcohol can cause Tylenol to be toxic to the liver at much smaller doses. Alcohol mixed with aspirin can lead to bleeding in the stomach. If you are taking methotrexate or other immunosuppressive medications, drinking greatly increases the chance that you will suffer liver damage.

Young bodies demand good nutrition. Teenagers and young adults need relatively more protein and nutrients to support growth and development. In addition, anyone with a chronic disease like lupus needs even better nutrition to also fight the chronic disease. Alcohol interferes with good nutrition in a couple of ways. Alcohol causes the body to waste some nutrients, basically by burning them up at a faster rate. In addition, the body's first priority is to metabolize or use alcohol, rather than the type of calories a person (especially teenagers) needs to grow. Anyone with lupus should avoid alcohol, particularly when taking medications, or restrict their alcohol intake as much as possible.

By Kristine M. Napier, N.P.H., R.D., L.D.

Reprinted, with permission, from Lupus World, Patient Empowerment Through Information, a publication of the University of Massachusetts Medical School, Worcester, MA 01655. Vol.1, No.2. With special thanks to Henrietta Aldjem, Editor.

=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.

Substance Abuse in Chronic Illness - A Challenge to Young Adults

Lupus is a disease that usually afflicts women between the ages of 15 and 45. The important word here is "usually" because, as most of you know, lupus can be found in children who are but a few years old up to those who are 15 years of age. Childhood lupus can range from an inconvenient to life-threatening problem.

This is not very different from the adult variety; however, the emotional aspects of it are very different. Children and adolescents suffering from lupus at times may feel severely restricted in what they are allowed to do and in what they are able to do. They may feel suffocated by their parents whose only "crime" is caring too much. Young people with lupus are often required to grow up well before their time. They must possess a certain responsibility that does not allow them to be children, to be teenagers. This leads to a certain sense of frustration and anguish.

However, with appropriate thought, guidance and consideration, such individuals will function quite normally and learn to develop a perspective on how best to view their disease. I think that it is most important that people afflicted with this or any other chronic illness not be "disease oriented" but rather be "life oriented." There are times when their lupus will dominate their day-to-day lives, but there are many times when it should not be so dominant.

There are those, however, who try to take the fact that they must live with a chronic disorder such as lupus and try to forget about it. In these instances, people with this attitude will expose themselves to unnecessary peril.

There are a number of things that go on in a teenager's world that are not necessarily good for anyone, let alone people who have lupus. This includes experimentation with alcohol, smoking and even illegal drugs. No matter who you are, you may be taking chances with your life by using these. Young people with lupus may be in a much more difficult position and fail to realize that the chances they are taking far outweigh those of the average teenager.

Lupus itself is a disease that can affect many organ systems. It is also a disease that can affect the circulation or blood flow to the body. If we look at smoking, for example, the risks taken by someone with lupus are different and more severe than those taken by someone who does not have this disease. One merely has to look at the problems of Raynaud's phenomenon. Smoking aggravates this problem, in which the fingers become white or blue upon exposure to cold.

Cigarettes contain nicotine, a very powerful chemical that causes blood vessels to constrict or clamp down. In people who have problems with circulation to the fingers and toes, clamping down can become so serious that they lose blood supply to the fingers and toes. In extreme cases, they could even lose fingers and toes because of lack of blood supply.

Street drugs can have a similar effect; indeed, their effect on blood flow may be even more severe. Furthermore, street drugs may have very serious effects on the liver and kidneys. In some people living with lupus, these organ systems are already affected, so by taking street drugs a person with lupus adds more poisons and exposes these already damaged organs to further injury. This could lead to loss of organ function to the extent that the liver may deteriorate and put life at risk. In the case of kidneys, the damage may be severe enough to make dialysis necessary.

It is also important to remember that there are various medications that may be necessary for people with lupus to take on a regular basis. Some of these drugs have side effects affecting various organs such as the liver, kidneys and bone marrow. The combination of such drugs as Imuran, Methotrexate or Cyclophosphamide with alcohol may exaggerate the known side effects of these drugs, putting organ systems at greater risk of permanent damage. Such damage could even lead to a life-threatening situation.

For example, it is well known that Methotrexate and alcohol are a very bad combination. Methotrexate is being used with increasing frequency in treating people with lupus. In many individuals the benefits of this drug have been surprisingly good. However, one of the problems with Methotrexate is that it may have some degree of liver toxicity. If an individual who is taking Methotrexate drinks alcohol, that liver toxicity is no longer a "maybe" but is almost a "definite." Persons who have responded extremely well to Methotrexate and possibly are even in remission with lupus may drink themselves into a situation where the Methotrexate must immediately be discontinued. They then may fall out of remission and suffer a lupus flare that could result in hospital admission or worse.

The easy answer to these problems is simply to stop taking the prescribed medications. In this way, I suppose you could say that experimentation with alcohol, cigarettes or street drugs becomes a less risky proposition. Unfortunately, discontinuing your medications makes risks much greater than you can imagine. I agree that it is frustrating, it is annoying and may cause you a tremendous amount of anger that you have lupus. Unfortunately, it is a problem that you may not deserve, but you've got anyway.

I think it is important to make that problem as small an aspect of your life as possible. Exposing yourself to needless risk will suddenly make lupus a problem in your entire life. So, in order for you to live your life, and not let lupus live your life think very carefully before you light that cigarette, drink that drink or try the latest "designer drug."

Dr. C. A. Laskin, a Rheumatologist who works in Toronto.

Minnesota Lupus News, August / September 1998, reprinted with permission from the Taking Life as a Challenge newsletter, Ontario Canada. TLC, geared to young people with chronic illness is supported by the Ontario Lupus Association.

=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.

Explaining Chronic Illness to Your Child

Explaining Chronic Illness to Your Child

As a parent, if you become ill, your illness has a profound impact on the entire family system. In spite of your own increased stress, confusion and anger, your children will look to you to maintain or return to normal family routines as soon as possible. If you, or your spouse, present an image of feeling overwhelmed or being consumed with the illness, your children will feel as if life is spiraling out of control.

As parents, it is often instinctive to want to protect our children from hurt or pain. We want them to be carefree and experience the joy and happiness that only childhood can offer. However, as much as we don't want to acknowledge it, children do experience the same kinds of losses and disappointments that we, as adults, experience.

We, as the nurturers and protectors, must explain and "normalize" these experiences in the best way that we can.

No matter what the age of your child, it is helpful to know and understand the developmental stages and how children at each stage can be expected to respond to stress and change. Developmental refers to the concept that a child's behavior, like his physical growth, develops in patterned and predictable ways. The age and goals of your child at each stage will guide you in how and when you need to intervene when your family goes through a crisis.

The first developmental stage -- infancy up to age two -- is probably the easiest in terms of knowing how to respond. The primary developmental goal is to establish trust. The child is completely dependent on the mother (or primary caretakers) to have basic physical and emotional needs met. With love and nurturing, the infant (and older baby) will thrive and grow.

Adults generally assume that a baby is too young or unaware to notice a change in a parent or family. However, infants and babies are extremely intuitive and can sense when a parent is upset or anxious. Any change in daily routine can throw a baby into a fretful state. When crisis occurs, you may see the following behaviors: increased crying and irritability, changes in appetite and sleep schedules, clinging behaviors and regression. Separation anxiety, which occurs routinely, becomes exaggerated. The baby will develop offensive behaviors. Treat regressive behaviors casually and return to a normal routine as soon as possible.

If your toddler asks a question about your illness, answer openly and honestly. A rule of thumb is never offer more information than the child has requested. Concealing the illness or whispering about what is happening will not help. Children always suffer more from the tension of not knowing than from knowing the truth. Allowing the child to act out fears and frustration through play or art is also an excellent help.

Preschool, ages four to five years, is the age of expansion. Preschoolers are ready to move out of the safety zone of the home and into a broader social arena. There is an increased reliance at this stage on "magical thinking." To a preschooler, anything that happens, good or bad, is related to them and their behavior. If a parent becomes ill during this stage, the preschooler's view will be: "Mommy is sick because I told her she was mean."

In response to stress and change, preschoolers often present with extremes, either being all good or all bad. This is the child's attempt to maintain a sense of control and to feel less frightened. Regressive behaviors are likely to occur, especially an increased reliance on a favorite security object (blanket, teddy bear, thumb-sucking).

To help preschoolers, it is essential to assure them that the illness is not their fault. Returning to a security object should be encouraged, rather than discouraged. Answer all questions honestly, including those about death. This is a good time to rely on books which help you help your child work through complex and often frustrating feelings about illness.

The primary development goal of the school-age stage (six to ten years) is achievement. The focus is school, outside activities and developing strong peer relationships. While parents and family are still central, the biggest concern is: "What will happen to me if you are ill?" This self-centeredness is normal.

Although there is still some overlap with magical thinking, by the age of eight children realize that illness may not be their fault. However, they still are not mature enough to remove themselves completely from the situation. The thinking now is: "If I'm good, Mommy will feel better and things will be fine."

School-age children tend to show strong emotions in reaction to change. They may show anger at both parents: "Why did you let this happen?" They tend to have a lot of somatic complaints (headache, stomach pain, fatigue), especially when leaving for school. The child is often fearful to leave the ill parent; he or she often assumes a protective role. Earlier in this stage, children are fearful that if they leave, the parent may die. As such, preoccupation and fear of death may be common.

When attempting to help school-age children, it is important to recognize that angry outbursts are an attempt to grieve or release fearful feelings. The opposite reaction, denial, may also occur as the child hopes that the illness will just disappear. Children will have many more questions and concerns at this stage. However, only the simplest explanations need be given. Information they don't understand will only frighten them and increase anxiety. Questions about death must be answered directly; evasion leads to more fear.

Changes in school performance, either for better or worse, are common. It is essential to let teachers know about the changes at home and to establish a feedback loop.

Our final stage of development is adolescence. The primary goal is to develop a self-identity that is capable of independent action. Adolescents work to achieve separation from parents and to become independent of the family system. This stage is a painful one for both parent and child, as both struggle in this journey toward separation.

Under normal circumstances, adolescents are known for their emotional volatility and moodiness. When a crisis occurs, you may see and hear even more expressions of anger, hurt and confusion. The opposite extreme is also common -- they may withdraw completely and not want to discuss your illness or their feelings about what's happening. There will be ambivalence about helping you. If you have an adolescent who is willing to do his or her part in helping the family, this will not extend to outside the home. Fitting in and acceptance by peers will be much more important than appearing helpful to the family. It is normal for them to be embarrassed by the illness and not want to discuss it with friends or teachers.

This is an essential time for parents to fine tune their communication skills. It is imperative to listen to and understand the volatile outbursts of the adolescent. Accept these feelings without overreacting to their tone. Continue to set limits, rules and boundaries, but keep the task of separation in mind. These outbursts are often fear-based.

At a time when they often feel out of control, teenagers cling to the hope that parents and family will remain structured and safe. Remember, even though he or she appears grown, your adolescent needs as much love and reassurance as your younger children.

In discussions about your illness, be prepared to give much more detailed information, especially all the facts about the illness. A major concern or fear will be: "Will I get it too?"

Some adolescents (the withdrawing ones) may not want to hear about or discuss your illness. They may express anger or disappointment toward the ill parent. These behaviors serve to diffuse their own fears or feelings of inadequacy in controlling the changes that are occurring or may occur in the future. Honest and open discussion of your own feelings may help them to express their own feelings.

One of the most difficult obstacles for parents of the adolescent is to overcome the expectation that he or she will be mature enough to handle the situation and to provide support. In actuality, they are overburdened with their own concerns and too vulnerable to carry the adult concerns.



In conclusion, the following reminders are offered to help you during times of crisis:


- Your children, like mirrors, reflect adult stress and behavior.
Be assured that most children experience times of high stress, confusion and frustration, yet still manage to develop and maintain a healthy sense of personal worth.

- Your children still need limits, rules and structure. Be the same parent you were before the illness.

- Discuss plans and decisions with your children commensurate with age and level of understanding. The more a child is included in planning, the more your relationship will be safeguarded.

- Do not assume the problem behaviors that a child exhibits in times of high stress will be permanent. However, if behaviors persist or worsen over time, seek professional help for the child and the family.


Jan Buxton-Truffer, MS, CEAP, is a counselor with the Sheppard Pratt Health Plan Employee Assistance Program.

Minnesota Lupus News, February / March 1998, reprinted with permission from Lupus Update, Maryland Lupus Foundation Newsletter, LFA, March 1997.

=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.

Heart Problems in Children with Lupus

Introduction
It has been well established that accelerated atherosclerosis, or hardening of the arteries, occurs in adults with lupus, along with its eventual clinical outcomes-myocardial infarction (heart attack) and stroke.

However, atherosclerotic heart disease is emerging as one of the most serious concerns in children and adolescents with lupus. Young, premenopausal women with lupus are up to 50 times more likely to have a heart attack than women of the same age who do not have lupus (1). Our research and that of other investigators indicate that these clinical outcomes are generally related to accelerated atherosclerosis (2,3).

Evidence of heart problems in children with lupus Children with lupus are known to experience myocardial infarction and stroke. Although the frequency of atherosclerosis in these young patients is not fully known, emerging evidence suggests the incidence may be similar to that in premenopausal women with lupus.

It is not unreasonable to suspect atherosclerotic development at young ages, as evidence of fatty streak formation-an early step in plaque formation-has been noted in healthy children as young as two to three years of age.

Atherosclerosis-promoting patterns of cholesterol, triglycerides, and other lipoproteins in children and adolescents with lupus have been documented (4). Coronary blood flow abnormalities were found in 16 percent of children and adolescents with lupus who had no cardiac symptoms. This suggests that there may be a significant percentage of young lupus patients with undiagnosed heart disease.

In addition, evidence of carotid atherosclerotic plaque and abnormal coronary blood flow have been detected two to five years after the onset of lupus, indicating that atherosclerosis may develop very early in the course of disease (5,6).

What causes heart disease in lupus? We do not completely understand the underlying biological cause for accelerated atherosclerosis in young patients with lupus, probably because we do not yet completely understand the underlying biological cause of lupus. One thing we do know is that two key factors are the disease of lupus, itself, and its treatment.

It was initially thought that the increased risk of heart disease in patients with lupus might be due to traditional risk factors, such as:

• hypertension
• diabetes
• obesity
• elevated cholesterol levels.

But recent evidence indicates that the presence of lupus itself, or the treatment for lupus, contribute more than those risk factors alone (2,7). The task now is to identify the biological processes occurring in lupus or resulting from its treatment that promote atherosclerosis.
It was once believed that excess cholesterol built up as plaque inside the blood vessels and obstructed blood flow. Investigators now know that fewer than 20 percent of heart attacks are due to restricted blood flow in progressively narrowed coronary vessels. More commonly, heart attacks occur when an atherosclerotic plaque ruptures and a blood clot forms around the plaque (8).

There are several potential parallels between lupus and the formation and rupture of atherosclerotic plaques (9).

A. For instance, damage to the lining of the blood vessels provokes an inflammatory response, which leads to deposits of immune cells containing fat droplets into the arterial wall. This in turn leads to plaque formation.

B. Inflammation is also responsible for thinning the fibrous cap that covers a plaque, making it more vulnerable to rupture. The inflammatory substances known as cytokines that drive the formation of atherosclerotic plaques and increase the vulnerability of the plaques to rupture are the same substances that play a major role in the inflammatory processes seen in lupus. This may explain why cardiovascular disease is accelerated in lupus.

C. Another factor related to lupus as a prime suspect as sources of damage to the lining of the arterial wall is high levels of circulating immune complexes. Although immune responses are important in the body's normal response to damage and microbial infections, in systemic lupus-and perhaps in atherosclerosis in general-these normally protective immune responses become dysregulated, leading to a high degree of inflammation and tissue damage.

D. A wide variety of autoantibodies may also hold responsibility. Elevated levels of antiphospholipid antibodies, which are often found in people with lupus, have traditionally been linked to an increased risk of blood clotting and may increase the risk of clot formation at the plaque site. More recent evidence suggests that these antibodies may also facilitate the uptake of oxidized low density lipoprotein, the "bad cholesterol," into inflammatory cells in the vessel wall. This is a key step in the formation of atherosclerotic plaque (10).

E. The amino acid homocysteine is another agent that is often elevated in lupus patients and is a likely source of arterial injury. Elevated levels of homocysteine have been linked to thrombosis in lupus patients (11) and to coronary heart disease and stroke in non-lupus patients. The reasons for elevated homocysteine in lupus are not entirely known, but may be related to kidney disease, diet, or treatment.

Is there a link between corticosteroids and atherosclerosis?With the advent of glucocorticoids (prednisone) in the 1950s, there has been a significant improvement in the lifespan of young people with lupus. Yet there is concern that these agents may actually contribute to the development of atherosclerosis, either directly by promoting plaque formation or indirectly by intensifying risk factors such as:

• weight gain
• hypertension
• elevated serum glucose and lipid levels.

In contrast, some evidence indicates that the anti-inflammatory effects of glucocorticoids may actually provide protection against atherosclerosis, suggesting that poorly controlled lupus activity may contribute to cardiac disease, with corticosteroid treatment providing a degree of protection.

Perhaps with the use of newer biologic therapies with similar anti-inflammatory and immunomodulatory effects as corticosteroids, but fewer adverse side effects, we will begin to see a reduction in heart disease.

How can atherosclerosis be managed in young people? It is of critical importance that physicians and patients be aware of the increased risk of cardiovascular complications in lupus. Young people generally view their risk of heart disease as negligible, yet cardiovascular disease intervention and prevention has the potential to significantly lengthen and improve the quality of their lives over many years.

Chest Pain.

Any physician treating a young person with lupus, regardless of the patient's age or sex, should be suspicious of chest pain. Because the patients are young and because chest pain in lupus may be attributable to other causes, physicians may overlook conditions, such as angina (chest pain due to myocardial ischemia). Yet often there are no warning signs for an impending heart attack. For these reasons, a major focus on management strategies should rest on preventing the development of atherosclerosis.

Diet.

There have been reports on the potential benefits of diet modification in controlling abnormal lipid levels in children with lupus, but diet alone is not always sufficient, and pharmacologic therapy may be necessary. However, the type, timing, and dosage of such therapy in children have not been well established by large studies.

Blood clots.

Measures to reduce potential blood clots, such as anticoagulation or antiplatelet therapy, should be considered in patients at increased risk, such as those with kidney disease, antiphospholipid antibodies, and other coronary disorders.

There are few clinical data on the effects of non-steroidal anti-inflammatory drugs (NSAIDs) on atherosclerosis. However, some evidence suggests that the selective inhibitors of the prostaglandin-producing enzyme COX-2 might actually enhance blood clot formation in some non-lupus populations. Further investigation in this area is currently underway.
Aspirin. There is strong evidence from clinical trials to support the use of low-dose aspirin therapy in preventing heart attacks in the general population. At low doses, aspirin is probably reducing the clotting risk but not reducing inflammation.

Dietary supplements.

There is some evidence that the antioxidant vitamins E and C may improve arterial dilatation in children with familial hypercholesterolemia or combined hyperlipoproteinemia. However, the long-term benefit of antioxidant therapies in reducing cardiovascular risks in lupus is unknown. Measures to reduce homocysteine levels with folate supplementation may be beneficial; again, however, the effects on prevention of coronary events are unproven.
Steroids. Based on the possible opposing effects of corticosteroids-increasing traditional risk factors and controlling inflammation-there are no established recommendations about the use of corticosteroids concerning cardiovascular risk in lupus. In general, judicious use of these agents to control the underlying disease and to minimize the proven long-term side effects is recommended.

Table 1 (below) illustrates suggested strategies to manage and/or prevent atherosclerosis in young patients with lupus. These strategies are targeted at both traditional cardiovascular risk factors and at potential lupus-specific factors. Physicians should communicate these potential risks to patients and their parents, and provide relevant information and resources for patient education.

Table 1. Strategies For Managing Cardiovascular Disease Risk In Children With Lupus

Step 1: Physician awareness
Recognize increased risk in young population.
Conduct a thorough cardiac evaluation if there is any suspicion of heart disease.

Step 2: Patient education
Make patients and parents aware of increased risk.

Step 3: Minimize traditional cardiovascular risk factors
Encourage a regular aerobic exercise program.
Establish guidelines for a heart-healthy diet.
Assist with weight loss program, if necessary.
Start a smoking cessation program.
Control hypertension and diabetes, if present.
Treat hyperlipidemia.

Step 4: Address potential lupus-specific risk factors
Use corticosteroids judiciously.
Reduce homocysteine levels (folate supplementation).
Consider aspirin or anticoagulant therapy for patients at high risk for blood clotting.

Physicians also should work together with the patients and parents to encourage a heart-healthy diet, a regular exercise program that involves aerobic activity, and weight loss, if necessary. Patients should be advised not to start smoking and to quit if they have already started. Hypertension and diabetes should be managed aggressively.

Bottom line

It is clear that premature atherosclerosis in children, adolescents, and young, premenopausal women with lupus is a substantial medical concern. The reasons that atherosclerosis is accelerated in lupus patients likely involve the inflammatory and immune-mediated mechanisms shared by these two disease processes.
Until new biologic therapies are available that can halt the immune dysregulation and resulting inflammation and vascular damage in lupus, we must promote aggressive approaches to reducing traditional cardiovascular risk factors.

Noninvasive methods for specifically identifying vulnerable plaques might also pinpoint those lupus patients at greatest risk for heart attack and those most likely to benefit from intervention. Investigations into the pathways that lead to premature heart disease in lupus may provide an ideal model for examining the role of inflammation in all populations with cardiovascular disease.

About the Authors Susan Manzi, MD, M.P.H., is an Associate Professor of Medicine and Epidemiology at the University of Pittsburgh School of Medicine in Pennsylvania.
Janice M. Sabatine, Ph.D. is a medical writer and editor.
Laura E. Schanberg, MD is an Assistant Professor of Pediatric Rheumatology at Duke University Medical Center in Durham, NC.

=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.

References
1. Manzi S, Meilahn EN, Rairie JE, Conte CG, Medsger TA, Jr., Jansen-McWilliams L et al. Age-specific incidence rates of myocardial infarction and angina in women with systemic lupus erythematosus: comparison with the Framingham Study. Am J Epidemiol 1997;145:408-415.2. Manzi S, Selzer F, Sutton-Tyrrell K, Fitzgerald SG, Rairie JE, Tracy RP et al. Prevalence and risk factors of carotid plaque in women with systemic lupus erythematosus. Arthritis Rheum 1999;42:51-60.3. Selzer F, Sutton-Tyrrell K, Fitzgerald S, Tracy R, Kuller L, Manzi S. Vascular stiffness in women with systemic lupus erythematosus. Hypertension 2001;37:1075-1082.4. Ilowite NT. Premature atherosclerosis in systemic lupus erythematosus. J Rheumatol 2000;27 Suppl 58:15-19.5. Gazarian M, Feldman BM, Benson LN, Gilday DL, Laxer RM, Silverman ED. Assessment of myocardial perfusion and function in childhood systemic lupus erythematosus. J Pediatr 1998;132:109-116.6. Falaschi F, Ravelli A, Martignoni A, Migliavacca D, Sartori M, Pistorio A et al. Nephrotic-range proteinuria, the major risk factor for early atherosclerosis in juvenile-onset systemic lupus erythematosus. Arthritis Rheum 2000;43:1405-1409.7. Esdaile JM, Abrahamowicz M, Grodzicky T, Li Y, Panaritis C, du BR et al. Traditional Framingham risk factors fail to fully account for accelerated atherosclerosis in systemic lupus erythematosus. Arthritis Rheum 2001;44:2331-2337.8. Libby P. What have we learned about the biology of atherosclerosis? The role of inflammation. Am J Cardiol 2001;88:3J-6J.9. Manzi S. Systemic lupus erythematosus: a model for atherogenesis? Rheumatology (Oxford) 2000;39:353-359.10. Vaarala O. Autoantibodies to modified LDLs and other phospholipid-protein complexes as markers of cardiovascular diseases. J Intern Med 2000;247:381-384.11. Petri M, Roubenoff R, Dallal GE, Nadeau MR, Selhub J, Rosenberg IH. Plasma homocysteine as a risk factor for atherothrombotic events in systemic lupus erythematosus. Lancet 1996;348:1120-1124.

Eating More Fish – The Answer To Lupus?

Eating More Fish – The Answer To Lupus?
11th March 2003

Press Release from the University of Ulster , Ireland

New research from the University of Ulster today offered hope to millions of lupus sufferers worldwide.

Dr Emeir Duffy, from the School of Biomedical Sciences, and Dr. Gary Meenagh, from Musgrave Park Hospital , have discovered new evidence to suggest that fish oil can greatly reduce the symptoms of the disease.

Systemic Lupus Erythematosus (SLE) or Lupus is a disorder of the Immune System, where the body harms its own healthy cells and tissues. The body tissues become damaged causing painful or swollen joints, unexplained fever, skin rashes, kidney problems, complications to the cardiovascular system and extreme fatigue.

There are approximately 500 diagnosed cases of SLE in Northern Ireland and it is most common in women of child-bearing age.

At present there is no cure but a key to managing lupus is to understand the disease and its impact. Steroids are the main drug used in the treatment of lupus and they should be administered for the shortest period possible to reduce side-effects.

But recently researchers have been looking specifically at its management through diet. Fish oils contain long-chained polyunsaturated fatty acids which are essential for normal growth and development but also have anti-inflammatory and anti-autoimmune properties.

Dr Duffy said: “We have been investigating how fish oil can improve the quality of life for lupus sufferers.

“In lupus, the body's immune system does not work as it should. Antibodies, which help fight viruses, bacteria and other foreign substances, are not produced effectively. The immune system actually produces antibodies against the body's own healthy cells and tissues. These auto-antibodies contribute to inflammation and other symptoms of the disease.

“Participants in the study who were taking fish oil supplements, three times per day for twenty-four weeks, saw a reduction in disease activity, an improvement in quality of life and reported an overall feeling of improved health by the end of the study compared to those taking a placebo supplement. Participants taking the fish oil also showed a reduction in fatigue severity, the most debilitating symptom for lupus sufferers.

“From our study and from other work, there is evidence that increasing dietary intake of the polyunsaturated fats found in fatty fish can have beneficial effects for lupus sufferers. Good examples of fatty fish include mackerel, lake trout, herring, sardines, tuna and salmon”.

=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.

New Research Shows Certain Vegetables May Delay the Onset and Reduce the Severity of Lupus

New Research Shows Certain Vegetables May Delay the Onset and Reduce the Severity of Lupus:

Scientists find that a compound abundant in broccoli, cauliflower and cabbage may help fight disease that affects 1.5 million Americans

A team of scientists from the North Shore-Long Island Jewish (LIJ) Research Institute have discovered that a compound found in abundance in the cruciferous family of vegetables delays the onset of systemic lupus erythematosus (SLE) in mice and reduces the severity of the disease once it has developed. This could be good news for individuals at risk for lupus, especially women, as well as those already affected by it.

Published today in the Journal of Nutrition, the study led by Karen Auborn, PhD, evaluated the effect of a supplement of indole-3-carbinol (I3C) - the compound found in broccoli, cauliflower, cabbage and similar vegetables - on the outcome of SLE in mice that are bred to develop the disease genetically. The results showed that mice with lupus lived significantly longer when fed the supplemented diet than did diseased mice fed the normal diet. Some even lived the normal lifespan.

Whether the mice were started on the I3C diet before or after the onset of the disease, the result was the same: there were fewer kidney problems (kidney disease is one of main complications of SLE) and they lived much longer than the control group.

"Mice are not people, of course, but the implication is that a diet rich in cruciferous vegetables could do much to ameliorate the disease," said Dr. Auborn. The findings support the view that I3C may benefit people at risk for SLE as well as those in the early stages of the disease. SLE is often treated with immunosuppressive drugs, which can have serious toxic side effects. By reducing the severity of the disease, I3C may allow a decrease in the dose of immunosuppressive drugs required, thereby reducing toxicity. It may even help prevent the recurrence of the disease.

A person could get the human equivalent dose of I3C tested in the study from about a third to a half of a head of cabbage. So getting the proper amount of I3C from food is realistic, although I3C also is available on the market as a dietary supplement.

According to the Lupus Foundation of America, approximately 1.5 million Americans suffer from some form of lupus, of which SLE is the most common. While it is unknown why women are nine times more likely to develop the disease than men, it is suspected that estrogen plays a role. Research has shown that women with SLE have abnormal estrogen metabolism. In many cancer prevention studies, I3C has been shown to exhibit antiestrogenic activity in the body. The North Shore-LIJ investigators had theorized that because I3C is an antiestrogen, it may prevent, delay, or even represent an adjunct treatment for lupus. They were right, at least for mice that genetically develop lupus.

The North Shore-LIJ Research Institute is planning a human study of the effects of I3C on SLE. Nicholas Chiorazzi, MD, a world-renowned rheumatologist and member of the study team, is working closely with Richard A. Furie, MD, chief of rheumatology at North Shore University Hospital in Manhasset, to make this a reality.

"It will be essential to determine if I3C can have similar effects in patients with SLE. Such studies are more difficult in humans because individual patients with lupus differ in their genetic backgrounds and also because the effects of lupus vary greatly from person to person, at least as far as the organs targeted by the problem," said Dr. Chiorazzi, who is also director and CEO of the North Shore-LIJ Research Institute.

This research was supported by grants from the Ryan Caulfield Foundation, the Willa and Robert Bernhard Fund, and the National Institutes of Health.

About the North Shore-Long Island Jewish Research Institute The North Shore-LIJ Research Institute is among the top seven percent of institutions nationally that receive funding from the National Institutes of Health. Building on its strengths in immunology and inflammation, oncology and cell biology, human genetics, and neurodegenerative and psychiatric disorders, its goal is to understand the biological processes that underlie various diseases and translate this knowledge into new tools for diagnosis and treatment.

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This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.

Lupus: Its Impact on Young People
By Robert H. Phillips, Ph.D.

Introduction
Most of the books and articles written about lupus are targeted for adults with lupus. There is a lot of information about the disease, such as its symptoms, treatment and lifestyle changes necessitated, all of which is very valuable. But there is a unique population that is not addressed often enough in this written material: the young person with lupus.

Lupus is most commonly diagnosed in women of childbearing age, and men are diagnosed as well, although in smaller numbers. But it is important to remember that boys and girls before childbearing years also can be diagnosed with lupus. And although they may experience many of the same problems that adults do (such as pain, other physical symptoms and medication side effects), certain problems are more “exclusively theirs.”

This article will address a sampling of the problems that may affect young people with lupus. (It is not the scope of this article to discuss medical facts. Rather, it focuses on the psychosocial issues that may appear). Rarely will a young person with lupus experience every psychological problem due to lupus. However, it's important for everyone involved – the young person, other family members, friends, educators and healthcare professionals – to be aware of, and sensitive to, these difficulties.

Denial
It is interesting that adolescents, more than virtually every other age group, have a common, non-verbalized (and sometimes loudly verbalized) belief about their medical status: “I'm fine!” They don't want to feel sick; they don't want to be sick; and they don't want to be different. But lupus may throw a monkey wrench into that.

What makes it especially difficult, though, is that unless lupus is affecting the young person so aggressively that they are virtually unable to move, in many cases the young person's attitude will continue to be “I'm fine.” This can be frustrating for other family members, who are trying to be protective and helpful, and for healthcare professionals, who have more difficulty treating a young person if the answers to questions about symptoms are evasive or denying.

School
Adults who work generally have a clear sense of their responsibilities and obligations at their jobs; they know that if they don't work, changes will result – many of which can be difficult and unpleasant. Young people, whose primary job is to go to school, may go because they want to and because they know they are expected to. Yet they may not be aware of the far-reaching implications of inconsistent school attendance.

For young people with lupus, the attitude about school varies. Some are upset and frustrated if lupus interferes with consistent school attendance and schoolwork performance. Others see school as less important and may have few or no qualms about missing excessive time in school.

Young people with lupus may have a more difficult time in school if they have to deal with the cruelty of other children (“What is that ugly rash on your face?”) or the ignorance of teachers (“You've missed too much school work; either get with the program or you're going to fail.”) In addition, because of lupus, young people may find themselves ostracized and even excluded from activities that were once within their physical capabilities.

Peer pressure
Peer pressure may also affect young people with lupus. The need to “fit in” – at its strongest during childhood and adolescence – can be devastating to someone who has a chronic illness with noticeable physical effects (e.g., rashes, bloating, etc.) and behavioral effects (slower, more painful movements, etc.).

It is heartwarming to hear stories of young people with lupus whose friendships continue despite their illness. Yet it is sad to hear of other stories in which the person with lupus is ridiculed and even abandoned by former friends.

Parents
Young people with lupus may have difficulties with parents being overprotective (“Stay inside, the sun is out”), not protective enough (“You want to go to the beach? Do whatever you want”), or insensitive to their needs (“Stop complaining about your pain, already. Get up and finish your school work”).

Parents may be concerned about the effects their child's lupus will have on the family, such as financial issues, problems with or neglect of other children, or even feeling like their independence is being inhibited. Any of these concerns can likewise affect the young person with lupus. Already unhappy because of having lupus, but feeling responsible for problems within the family or with the parents, a young person may feel guilty to the extent that it interferes with their physical and emotional health.
For example, the young person may not tell parents about a serious lupus symptom, knowing it might mean another trip to the doctor or even the possibility of hospitalization.

Siblings
Brothers and sisters of a young person with lupus may be very resentful. Being less able to understand the physical impact of the disease, they may dislike the added “attention” being directed at their sick sibling. They also may not like getting less attention, and may act out in an attempt to regain their “share” of parental interactions. Their resentment toward their sick sibling may be manifested in many hurtful ways, such as anger, ignoring instructions, spiteful behavior, concealing important information from their parents, etc.

A final note
It is difficult enough for anyone to live with lupus, but the young person with lupus has added, age-related problems. Being aware of the potential impact of lupus in young people does not eliminate these problems. But increased awareness can pave the way to a better understanding of the unique needs of young people, and can lead to methods for better alleviating the problems that may occur.

Some Helpful Suggestions for Parents and Other Adults Dealing with Young People
Be Sensitive to the young Person's unique needs. Lupus can be a difficult disease to live with, especially for a child who has fewer “coping strategies” in place. Don't assume that the young person has the emotional strength or the social support network to handle lupus-related problems successfully.

Communicate appropriately. Try to look at any lupus-related issues through the eyes of the young person. See what they see. Feel what they feel. Using anger and aggressiveness in “forcing issues” is rarely productive. Calm, constructive discussion is a much more positive way to address lupus-related issues.

As much as possible, treat the young person like an adult. Plan together the appropriate ways to treat, and live with, lupus. Demonstrating adult-like behavior in interactions with young people is more likely to generate adult-like behavior in return.

Educate significant others. Any individuals who are not familiar with lupus, including family members, friends and teachers, can be obstacles to successful living with lupus. This is especially important in school, since the young person is going to spend a good number of hours there each day. Provide pamphlets and other information to teachers, guidance counselors and even classmates, so that school can truly be a “home away from home.”

Reprinted with permission of the Lupus Foundation of America, ©2001. Dr. Robert H. Phillips is founder and director of the Center for Coping in Long Island, NY ( www.coping.com) . He has been in private practice as a licensed psychologist since 1975 and has published and spoken widely on coping with physical ailments and other psychological topics. He has appeared on dozens of television and radio programs and currently is the host of “Coping Conversations, “ a weekly radio talk show on WKJY-FM (98.3) on Long Island .

Dr. Phillips is the published author of more than 20 books, including the highly popular Coping With Lupus (now completely revised and updated for its third edition); Lupus: Everything You Need to Know (with co-author Dr. Robert Lahita); and his new Successful Living With Lupus: An Action Workbook, published last year. These books, as well as several others, are available for sale from the Lupus Foundation of Minnesota (952-746-5151).

Dr. Phillips has served on the National Board of Directors of the Lupus Foundation of America and currently is a member of the Lupus News Advisory Board.


=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.

Kids Adapting to Lupus

Kids Adapting to Lupus

About 25 percent of lupus begins in childhood and adolescence, posing special problems. Pain, fatigue, the interruption of school and sports activities, limitations of mobility, change in appearance, and the feeling of being different are all particularly difficult for kids.

Lupus in childhood threatens the normal development process of gaining social and academic skills, the formation of a solid identity, and the development of independence and separation from parents. Very young children have rather concrete notions of disease, essentially that it is a result of accidents or catching germs from someone else. The more abstract reasoning required to understand lupus as an autoimmune disease does not usually develop fully before adolescence. In addition, children are focused on the immediate aspects and consequences of their disease, such as whether or not they can go to school or spend time with their friends or engage in any particular activity that day or that week. It's not until adolescence that children begin to understand the way in which their disease might interfere with their future goals. Depression stemming from this perception of future loss may be more of an issue for adolescents.

Lupus in children is essentially the same disease as that which occurs in adults. Some newborn infants of mothers with SLE experience abnormally slow heart rhythms and temporary skin rashes, the so-called "neonatal lupus syndrome", this is caused by antibodies that originate in the mother, but affect the newborn. This is not true lupus. In childhood, as in adulthood, true lupus can cause joint pain, fever, fatigue and butterfly rash as well as serious organ dysfunction, such as kidney or central nervous system involvement. Test results are similar, and treatment recommendations for childhood lupus are essentially the same as that for adults.

In most parts of this country, children with lupus are treated by pediatricians and often by a rheumatologist with special training in pediatric lupus. Generally, by the time patients reach adolescence, their care is transferred to an adult rheumatologist. However, the timing of this transfer may vary considerably and should be managed sensitively. Another significant difference with the treatment of lupus in children has to do with the effects of prednisone and other corticosteroids; calcium and vitamin D supplements are used to avoid or delay future problems with osteoporosis.

Over time, the adolescent patient should assume an increasingly autonomous role in her or his own care. They should be expected to do the talking in an appointment and have answers directed to them rather than to their parents. They should gradually gain responsibility for handling medications and scheduling their appointments while parents monitor the situation. They also have a right to confidentiality that should be respected.

However, as much as kids want to become independent from their parents, they are anxious about taking these steps, whether it be going away to college, or communication directly with their doctors, making appointments, or generally assuming responsibility for this aspect of their lives. They may forget their medications, appointments, and their need for rest and sleep. These are opportunities to provide additional guidance, reminders, and encouragement and not to usurp control of their healthcare. These are also good opportunities for open dialogue about the issues of independence and expectations of support. Studies of other chronic childhood diseases, such as asthma and diabetes, suggest that kids often fail to take their medications in the prescribed dosage. It is very important to involve children as active participants in their own health care.

Within the family, it is important to not treat the child with lupus differently than other children. For example, kids should not be over-sheltered or protected from the usual expectations or developmental challenges. Parents commonly feel guilty about their children with lupus because of the influence of genetic factors in its onset. However, they need to manage that guilt in such a way as not to become over-involved in the life of their ill child to the point of suspending their lives or neglecting the need of their other children.

Kids benefit from having some contact with adults who have had lupus and yet managed to live full and gratifying lives. One patient, Vicki Croke, now a journalist for the Boston Globe, has written about the importance of discovering the example of Henrietta Aladjem. When Vicki was quite ill she met "Hennie", whose disease by then had been in remission for many years, Vicki's hope was restored. Kids above all need a vision for themselves in the future and adult role models to help provide it. They also benefit from having contact with other kids with illness, either in support groups or in chat groups on the Internet. One nine year-old with lupus, Jessica, has a web site in which she described her illness, the disruption in her activities, the teasing about prednisone-related weight gain and her recovery. The teasing improved when her mom, a nurse, came to school and explained the illness to her class.

Fortunately, children and adolescents are extremely resilient. With the support of their family, friends, and doctors, most manage to negotiate the extra challenges imposed by illness. Sometimes counseling or psychotherapy can be helpful as well. At times parents need counseling even more than their children. If more difficulties or anxiety persist, psychiatric intervention and / or referral are recommended.

By Malcom P. Rogers, MD.

=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.

The Genetic Basis of Lupus

The Genetic Basis of Lupus

Lupus is an illness in which the immune system appears to have gotten confused. Instead of attacking viruses, bacteria or cancer cells – which is what it's there to do – the immune system attacks the person's own body.

The evidence that lupus is a genetic disease is quite clear. Lupus runs in families. If you have lupus, there's a five percent chance that one of your siblings will get the disease.

If you are a non-identical twin, the chances are about the same as for a sister, suggesting that nothing major happens in the womb to cause lupus. But if you are an identical twin – and your twin has lupus – the chances go up to 57 percent that you will develop the disease. Thus, there is strong evidence for a genetic basis, but it is clearly not a simple genetic basis.

If just one gene you could inherit always caused lupus, at least one out of four siblings should get lupus, considering the classic laws of genetics. Some people who are related to lupus patients have various lupus-like symptoms, suggesting that they have acquired “incomplete lupus” without developing the full-blown disease.

So how do we explain that? And, since identical twins are genetically identical, why does lupus affect only 57 percent of identical twins? We're just beginning to understand that there are several possible explanations.

Multiple Genes Involved
The first important concept is that more than one gene may be involved. One-fourth of your siblings should inherit any one gene, but if three, four or more different genes need to be inherited together, this doesn't explain why both identical twins don't get the disease, since they inherit the same genes.

A concept that could explain why some identical twins of lupus patients are spared is called gene penetrance. This means that you can have a gene – and that gene may cause you to be susceptible to a disease – but the illness still doesn't show up. This is possible because something in the environment is needed to get the disease started. This environment can be either outside your body or inside your body, which is greatly influenced by infections or toxins, or even your other genes. The idea that a certain gene could be there – but not expressing itself completely – is called “incomplete penetrance.”

A third concept, developed in recent years, is that there are cases where genes actually change or rearrange themselves in the body after the first cell divides at the moment of creation. So even identical twins can end up with somewhat different genes that develop later, after they have separated from each other in the womb, or after birth. This can be true for certain genes that have to do with the immune system.

The Role of Genes
What do we know about lupus that helps us to understand the roles of genes? First, there is the predominance of women to men who get the disease. It seems likely that this has to do with a direct effect of hormones on the immune system. Female hormones may help create the environment that allows a lupus gene to penetrate.

Much research is being done to identify the genes that lupus patients share. The ones that are best understood are a series of genes which regulate how the immune system works and where and when it might attack.

These genes are part of the network that is involved in tissue typing. They allow the immune system to recognize and attack foreign invasions from viruses, bacteria, or cancer cells, and to distinguish them from things that should not be attacked, such as parts of the body or tissue-typed organ transplants.

If you were looking for a defective gene that causes lupus, major histocompatibility complex (MHC) genes, whose normal function is to regulate immune responsiveness, would be good candidates since the immune system in lupus gets confused into attacking a person's own body. And, in fact, MHC genes are shared by many lupus patients.

How Genes Work
How do these genes work? They make proteins that act very much like the Lord Chamberlain to a very paranoid queen. Imagine a world in your bloodstream that is rather like medieval times with lots of small castles, each inhabited by a different queen, each queen waited on by a special court made up of one Lord Chamberlain and a lot of little soldiers.

If an infection enters the bloodstream, an invading particle will be picked up by a special Lord Chamberlain, who is genetically programmed to recognize it. He takes the particle back to his own castle and formally presents it to the queen as if it were the ambassador from a foreign country. The queen takes one look at the particle, shouts, “Off with its head!” – and all sorts of things start to happen.

First, immune-fighting cells get made. Then little proteins called antibodies get made. These are like little soldiers who specifically know how to recognize that original invading particle. So they leave cells and run around the blood stream attacking anything that looks to them like the particle.

If they get confused and think your kidney or joints look like an invading particle, then you might develop lupus. But the antibodies would never have started attacking if the Queen hadn't started shouting. And the Queen would have kept quiet if the Lord Chamberlain hadn't brought the particle into her castle.

The Lord Chamberlain (who is an MHC molecule) may be shared by many lupus patients and may be one of primary genes that put people at risk. The Queen (who is called the I Cell Receptor) and the antibodies are examples of immune genes that can rearrange themselves after the beginning of life, so they may or may not be shared exactly in families, even by identical twins. This could explain why an identical twin of a lupus patient might have more risk of developing lupus than another sister, but not a 100 percent risk.

Self-Attacking Genes
But why do lupus patients carry these genes that can start an attack on their own bodies – and what prevents other people from doing the same thing? In order to have a diverse ability to recognize and protect the body from diverse infections over a lifetime, everybody has some genes capable of making proteins that attack their own organs.

However, immune cells go through a complex educational process early in life in the thymus gland, where they are taught to recognize the difference between “us” and “them” – and what sorts of invaders it is appropriate to attack. Those that misbehave and threaten to attack parts of their own bodies are usually simply killed off by a process called apoptosis. But the well-behaved immune cells “graduate” from the thymus and are allowed to enter the bloodstream.

A gene which regulates the system that eliminates self-attacking cells in the thymus gland is defective in some mice with a lupus-like illness. The jury is still out on whether this is the fact in human lupus – but many researchers are now considering the possibility.

I cannot go into all the other genes that seem to put people at increased risk for lupus. But one important set of genes gives rise to special inflammatory proteins called complement proteins. These proteins act something like the artillery used by the antibody soldiers when they attack, and defects to some of the complement genes have been described in lupus and lupus-like illnesses.

Continued Research Needed
In summary, lupus is a complicated disease with a complicated genetic basis. It involves several genes we know of, and probably more not yet identified, that are important to regulating appropriate immune activity.

There are two reasons why continued research into the causes and genetic basis of lupus is very important – first and foremost, to improve the care of lupus patients. Although treatments are better than 20 years ago, and we encourage lupus patients to have optimism for a fairly normal life, people still die from lupus or from side effects of the medications. And others become very ill and suffer major organ damage. Patient care could be better if we knew more.

Second, what we learn by studying lupus has profound implications for better understanding and treatment for many other diseases. This point should not be forgotten when writing to Congresspersons or fundraising for the Lupus Foundation.

Lupus research provides a large picture window into the mysteries of the immune system and will very likely contribute to the understanding and treatment of AIDS, heart disease, cancer, diabetes and many other diseases. This research includes the one disease that costs more than any other – and affects everyone on earth – the process of aging itself.

By Joan T. Merrill, MD, St. Luke's-Roosevelt Hospital Center, New York City. Reprinted with permission from the newsletter of the SLE Foundation, New York.

=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.

Difficult-to-Manage Lupus

Difficult-to-Manage Lupus

Having treated over 2,000 lupus patients over the last 20 years, I was intrigued when the editor of Lupus Update asked me to write about “Difficult to Manage Lupus.” After all, isn't all lupus difficult to manage? However, there are certain situations that are more difficult to manage than others. Ten patient cases are presented here:

When not to overtreat? Resistant non-organ-threatening disease?
A patient with Systemic Lupus Erythematosus (SLE) has persistently active rashes, fevers, fatigue and pain on taking a deep breath despite nonsteroidals and Plaquenil. There is no evidence for heart, lung, liver, central nervous system, hematologic or renal involvement. There are circumstances when certain treatments often do more harm than good if oral steroids or methotrexate are added in this situation, so I might try a few other things first. Consider increasing Plaquenil, consider DHEA, use very high dose nonsteroids or a local one-time only steroid boost.

Cyclophosphamide (Cytoxan) resistant lupus nephritis
Fifteen to twenty percent of all individuals with SLE have lupus in their kidneys and biopsies showing proliferative disease. With no treatment, they will be on dialysis within 2-3 years. Although Cytoxan is the treatment of choice for them, this toxic therapy does not work or wears off 30-40 percent of the time. What do I do? My options include: consider adding azathioprine (Imuran) to the Cytoxan and continue treatments; add plasmapheresis or pulse-dose steroids; or substitute nitrogen mustard for Cytoxan. Consider rebiopsying the patient and make sure that the renal lesion is reversible. Sometimes, it's better to let a patient go on to dialysis and transplant them than treat them further. Newer treatments may be utilized: cyclosporin, mycophenolate mofetil (CellCept) or tacrillimus (Prograf, FK 506). Drug trials with LJP394 and Biogen's antiCD40 ligand are available at selected medical centers.

Refractory chronic cutaneous lupus with no systemic disease
A patient has lupus rashes covering 70 percent of the body, but their ANA is negative and all blood work is normal. Plaquenil has not helped. I have had some success with: switching from Plaquenil to Chloroquine and adding quinacrine, retinoids such as Accutante or Soriatene; antileprosy drugs including thalidomide, clofazimine or dapsone; topical nitrogen mustard or BCNU.

Lifestyle-altering central nervous system (CNS) symptoms with a normal MRI scan of the brain and blood tests showing slight activity
When my patients complain about not thinking clearly, severe headaches and profound fatigue, the issue is raised as to whether it could be vasculitis of the CNS. True CNS vasculitis in SLE is usually obvious (e.g., fevers, meningitis-like picture, psychosis, seizures), and responds to high dose steroids. But could the patient have “subclinical vasculitis?” In fact, this is rare and most often the symptoms are due to abnormal blood flow to the brain due to a dysfunction of the autonomic nervous system and / or the dysfunction of chemicals known as cytokines (interleukins, interferons, etc.). I frequently order a SPECT scan (which might include antineural antibodies) and antiribosomal P antibodies and a spinal tap. Make sure that your doctor obtains LE cells, oligoclonal bands, antineuronal antibodies and IgG synthesis rate in addition to the usual determinations.

Cognitive impairment in patients without CNS vasculitis
How do we treat the patient in number 4 (above) who does not have vasculitis? Interventions are useful that improve the blood flow to the brain regulated by the autonomic nervous system (which controls the dilation or constriction of blood vessels, thus regulating our pulse and blood pressure), such as: biofeedback, relaxation techniques, cognitive therapy and counseling. Additionally, serotonin boosters (Prozac, Zoloft, Paxil) may help give a patient more energy and clarity. Antimalarials (Plaquenil, quinacrine) and DHEA can be useful. Steroids may seem to help at first but make things worse in the long run and should be avoided unless there is evidence for inflammation.

More than one miscarriage in a patient without anticardiolipin antibodies
Antiphospholipid antibodies and the circulating lupus anticoagulant can cause miscarriages. Most primary care doctors stop the workup after obtaining a negative anticardiolipin antibody and circulating anticoagulant test and don't treat the patient. On the other hand, aggressive reproductive immunologists unnecessarily treat patients with expensive and toxic approaches such as heparin, prednisone and intravenous gamma globulin for subsequent pregnancies which would be successful in any case. Active lupus by itself can cause miscarriages. I'm in the middle. I check for three to four different phospholipid antibodies, Protein C, Protein S, antithrombin III, Factor V Leiden mutation, BDRL and kaolin PTTs.

Is the muscle and joint aching a lupus flare or fibromyalgia?
Fibromyalgia can be as discomforting as lupus-associated inflammation but is made worse by corticosteroids. It is important not to inappropriately treat. I generally only treat symptoms of lupus with higher doses of anti-inflammatory medicine when there is objective evidence of joint swelling (synovitis), a high CPK (muscle enzyme), a high sed rate or CRP (blood tests for inflammation), low C3 complement or high anti-DNA. Lacking this, sometimes I have had to resort to obtaining a bone scan to assess if somebody with profound musculoskeletal discomfort and SLE is inflamed or experiencing a flare or fibromyalgia. The latter is treated with tricyclics, serotonin boosters and muscle relaxants and is seen in 25 percent of lupus patients.

The patients with non-organ threatening disease who want to treat their disease “naturally”
Twenty percent of patients with non-organ threatening disease will evolve organ threatening disease over five years, but this percentage decreases to five percent with two years of Plaquenil. No herb or spice has been shown to be specifically effective for SLE, and studies are sorely needed. None of these preparations, marketed as nutritional supplements to avoid FDA regulation, are standardized. Avoid believing testimonials and only rely on peer-reviewed published controlled studies. Let the buyer beware.

My eight-year-old daughter has aches and a positive ANA. Should I be worried?
Ten percent of women with SLE will have a daughter with the disease, and two percent a son with it. Twenty percent with SLE will have an offspring with an autoimmune disorder (most commonly autoimmune thyroid disease). Fifty percent of children of lupus patients have a positive ANA. Girls who are prepubertal develop lupus very rarely. Their positive ANA is inherited and their aches are usually due to growing pains. We usually advise against ANA or antibody testing unless there is objective evidence for a problem such as a fever, swollen joints or rash.

The 70-year-old woman with a diagnosis of new-onset lupus
Ten percent of the population develops ANAs as they age. When senior citizens are found to have a positive ANA and have a high sedimentation rate and joint aches, they often come to rheumatologists with a diagnosis of lupus. In reality, the overwhelming majority do not have the disease. Polymyalgia rheumatica, fibromyalgia, rheumatoid arthritis and particularly Sjogren's syndrome need to be ruled out.

By Daniel J. Wallace, MD, Clinical Professor of Medicine, UCLA School of Medicine. Reprinted with permission from the Maryland Lupus Foundation. Dr. Wallace is the author of “The Lupus Book: A Guide for Patients and their Families”.

=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.