Systemic lupus erythematosus (SLE or Lupus) has many clinical and laboratory manifestations. This article relates to those manifestations which concern the cells and clotting factors that circulate in the blood. As with other manifestations of lupus, the underlying cause is usually the presence of antibodies, which are proteins that recognise and bind to other proteins in the body However, instead of getting rid of an unwanted foreign protein (which is what normal antibodies do), the antibodies in lupus recognise self-molecules (usually proteins) and interfere with the normal function of our own proteins and the cells containing the proteins.

What is blood?

Blood is made up of cells and a protein rich liquid containing many substances including antibodies (serum). The cellular part is composed of red cells (which contain the oxygen carrying molecule haemoglobin), white cells (which fight infection and can be subdivided into several types, of which the neutrophils and lymphocytes are particularly important) and platelets (which are involved in dotting the process which stops us bleeding when we cut ourselves). Clotting also involves a number of special proteins called clotting factors in addition to the platelets. A full blood count test measures the number of red and white cells, platelets and the amount of haemoglobin circulating in the blood. There are special tests for assessing the clotting properties of blood (see below under ‘how is antiphospholipid syndrome diagnosed?’)

Why do lupus patients have low white cell counts, and what is the significance of this?

In SLE, antibodies directed against white cells are very common. A lower than normal lymphocyte count is found on the full blood count in about 95% of lupus patients. This is due to the presence of antibodies to lymphocytes which results in the destruction of the antibody-coated lymphocytes. Fortunately this rarely causes a clinical problem because more lymphocytes are released from the bone marrow where they are made. This means that there are enough lymphocytes to fight infection, especially those due to viruses like influenza (’flu’). However, high doses of certain drugs can also cause destruction of lymphocytes and, because the bone marrow where these cells are made is also attacked by such drugs, there may not be enough lymphocytes to light infection. So in this case there is an increased risk of infections, especially viral infections. Cyclophosphamide, which is sometimes used to treat more severe forms of lupus such as kidney disease, can have this effect. Consequently the white cell count is regularly checked in people receiving this drug so that the drug dose can be adjusted if necessary.

Low neutrophil counts are less common than low lymphocytes counts as a result of lupus and is more common in people of Afro-Caribbean origin (even in the absence of lupus). Low neutrophil counts can also be the result of drugs such as cyclophosphamide and azathioprine and if severe can be associated with bacterial infections such as pneumococcal pneumonia. Again, regular blood monitoring tests will usually prevent this complication by allowing the drug dose to be adjusted before a problem occurs.

Why do lupus patients become anaemic and what effect does this have?

Anemia means that there are less red cells (and therefore less hemoglobin to carry oxygen) in the blood than there should he. Low red cell counts and the associated low level of hemoglobin in the blood can result from the effect of antibodies attacking the red cells and causing their destruction, a process called haemolytic anemia. It is more common though to have anemia due to poor production of red cells in the hone marrow. This usually occurs as a side-effect of general inflammation

in the body due to lupus. Rarely is anemia caused by drugs but this is possible. Whatever the underlying cause of anemia, the end result is to cause the person to feel tired (fatigue) in proportion to the loss of red cells (hemoglobin), although there are other (often less well understood) causes of fatigue in lupus. In more severe cases, the person may become short of breath even in the absence of lung disease because there is not enough oxygen in the blood.

What are the effects and causes of low platelets in lupus patients?

Low platelets are usually due to antibodies and less commonly the result of drug side-effects. When the count is very low there is an increased risk of bruising and bleeding; fortunately this is a rare manifestation of lupus. Surprisingly there is another type of antibody that can reduce the platelet count (usually only mildly), so there is no bleeding but instead these antibodies can interfere with platelet function and this causes increased clotting, known as thrombosis. This is a painful condition, as not enough blood can get through a blood vessel containing a clot and this causes damage to that part of the body. This can occur, for example, in the calf (deep vein thrombosis), and the clot can spread from a leg vein through the circulation to the lungs (pulmonary embolism). The underlying cause of this thrombotic condition is known as antiphospholipid syndrome, as there is a group of antibodies present which interferes with phospholipids in platelets and other cells and proteins involved in clotting.

What else does the antiphospholipid syndrome cause?

Apart from causing low platelet counts, deep vein thrombosis and possibly pulmonary embolism, antiphospholipid antibodies can cause blood clots in the major arteries of the body This can result in conditions such as heart attack or stroke. In women of childbearing age, these antibodies can also cause miscarriages or stillbirths due to blood clots forming in the placenta which prevent nutrients from reaching the developing baby during pregnancy.

How is antiphospholipid syndrome diagnosed?

Antiphospholipid syndrome is diagnosed by finding antiphospholipid antibodies in the blood and clinical features of increased blood clotting (for example recurrent deep vein thromboses or miscarriages), The antibodies can be detected by a specific test for the type of antibody, such as anticardiolipin antibodies, or by abnormalities In conventional clotting tests. Antiphospholipid antibodies interfere with many of the conventional clotting tests because these tests depend on phospholipid for the test to work normally. If antiphospholipid antibodies are present, they prevent the blood from clotting, making it look as if there is a shortage of clotting factors, However, in reality the antibody acts as an inhibitor of the clotting test and in real life this is associated with an increased risk of clotting (this paradox is confusing to everyone). If you mix the serum part of the blood containing the antibodies with serum from a healthy person, the antiphospholipid antibodies still interfere with the clotting test, whereas if there Is a clotting factor deficiency (as in haemophilia), the mixing results in correction of the missing factor and the clotting test becomes normal. This is the basis for some special tests of blood clotting used to diagnose antiphospholipid syndrome.

Source: Lupus Site

No one can say for certain that there’s any way to prevent a lupus flare, but the Lupus Foundation of America does provide a number of “healthy habits” that can help patients reduce the potential for triggering a flare.

We’ve taken those habits and gone one step further, by providing some suggestions on how to get into those habits and stay in those habits – thus possibly increasing your chances of staving off a flare.

Take Your Medication as Prescribed:

How can you remember to follow your prescription? The National Heart, Lung and Blood Institute offers these suggestions:

• Take your drugs at the same time every day, linking it with something else you do regularly, like brushing your teeth.
• Write it down – and then use different colors to get your attention.
• Keep a chart or calendar. Use colored pens to track more than one type of medication.
• Use a pillbox.
• Use e-mail reminders to alert you to take your medication.
• Remember to refill your prescription. Make a note on your calendar to order and pick up the next refill one week before your medicine runs out.

Keep Your Doctor’s Appointments, Even If You’re Feeling Fine:

There’s a simple reason for doing this, and that is to keep lines of communication open and to catch any potential illness or complication in its earliest stages. That said, if you’re feeling well, what should you communicate to your doctor or healthcare provider? And how?

Here are some to dos that will help you make the most of your time with your doctor:

• Make a list of questions, symptoms and concerns
• Have a friend or family member come with you to help you remember or to act as your patient advocate.
• Use the time to find out who in your healthcare provider’s office can assist you, and the best times to call.
• Review your treatment plan and ask how the plan will help you improve your health. Find out what might happen if you don’t follow the treatment plan.
• Review your progress on your treatment plan.
• If there are aspects of your treatment plan that seem to not work, share your concerns with your doctor.

Don’t Take Sulfa Drugs (sulfonomides):

Sulfonomides are used to treat infections such as bronchitis and urinary tract infections. Some common sulfonomides are Acetazolamide, Celecoxib, and Xipamide.

Limit Your Exposure to Sunlight:

This is called photosensitivity, and About Lupus includes an article on what it is and ways to avoid sunlight. Basically, it is suspected that UV light causes skin cells to express particular proteins on their surface, attracting antibodies. The antibodies in turn attract white blood cells, which attack skin cells, which leads to inflammation. Apoptosis, or cell death, normally occurs at this point, but it is amplified in lupus patients, which increases inflammation.

Some basic tips:

• Avoid the midday sun
• Wear the correct clothing
• Use sunscreens
• Turn off other sources of UV light
• Take your medicine as directed
• Know what you’re taking
• Watch your make-up (try to buy brands that are also UV protectors, and that are hypoallergenic).

Reduce Your Risk of Infections:

Since lupus is an automimmune disease, the risk of getting infections is greater for lupus patients. Here are some simple tips on how to reduce your risk of getting an infection:

• Take care of yourself. Eat a balanced diet, exercise regularly and get plenty of rest.
• Work and play safe. Consider your risk for infection based on where you work (hospital? clinic? daycare? vet?).
• Pets provide many benefits to owners. To avoid infection from your pet, make sure your pet is up to date on all its shots and vaccinations to prevent disease. And always wash your hands after petting your pet, especially before you eat.
• Avoid eating raw or undercooked eggs
• Wash fruits and vegetables carefully before you eat them.
• Wash your hands, cutting boards, counters and knives with soap and water.

Don’t Smoke:

This one is self-explanatory. And if you needed any more encouragement, consider that smoking is responsible for 87% of lung cancers in the United States.

_Sources:

_{Flares and What Triggers, Lupus. Lupus Foundation of America. May 2008}

A little girl stood near a small church from which she had been turned away because it was “too crowded.”

“I can’t go to Sunday School,” she sobbed to the pastor as he walked by.

Seeing her shabby, unkempt appearance, the pastor guessed the reason and, taking her by the hand, took her inside and found a place for her in the Sunday school class. The child was so happy that they found room for her, and she went to bed that night thinking of the children who have no place to worship Jesus.

Some two years later, this child lay dead in one of the poor tenement buildings. Her parents called for the kindhearted pastor who had befriended their daughter to handle the final arrangements.

As her poor little body was being moved, a worn and crumpled red purse was found which seemed to have been rummaged from some trash dump.

Inside was found 57 cents and a note, scribbled in childish handwriting, which read: “This is to help build the little church bigger so more children can go to Sunday School.”

For two years she had saved for this offering of love.

When the pastor tearfully read that note, he knew instantly what he would do. Carrying this note and the cracked, red pocketbook to the pulpit, he told the story of her unselfish love and devotion.

He challenged his deacons to get busy and raise enough money for the larger building.

But the story does not end there…

A newspaper learned of the story and published It. It was read by a wealthy realtor who offered them a parcel of land worth many thousands.

When told that the church could not pay so much, he offered to sell it to the little church for 57 cents.

Church members made large donations. Checks came from far and wide.
Within five years the little girl’s gift had increased to $250,000.00- -a huge sum for that time (near the turn of the century).. Her unselfish love had paid large dividends.

When you are in the city of Philadelphia , look up Temple Baptist Church , with a seating capacity of 3,300. And be sure to visit TempleUniversity, where thousands of students are educated.

Have a look, too, at the Good Samaritan Hospital and at a Sunday School building which houses hundreds of beautiful children, built so that no child in the area will ver need to be left outside during Sunday school time.

In one of the rooms of this building may be seen the picture of the sweet face of the little girl whose 57 cents, so sacrificially saved, made such remarkable history. Alongside of it is a portrait of her kind pastor, Dr. Russell H. Conwell, author of the book, “Acres of Diamonds”.

This is a true story, which goes to show WHAT GOD CAN DO WITH 57 CENTS.

I came across this article from a Lupus website (I’m sorry I forgot the link) and it has helped me in many ways. Every word in that article spoke right through me, it is exactly what I am feeling. One of the reasons I started blogging is to write all the feelings that I have been keeping to myself, emotions that I cannot reveal to my family and even to my closest friends. I have always been strong and I want them to see that I have enough courage to face everything but I am not always strong, many times I just break down in tears.

Feelings I Keep To Myself
A beautiful article written by Mark Flapan, Ph.D.

When family members or friends ask, “How are you?”, they usually want to know if you’re in any particular pain or discomfort or if you’re any worse or better off physically than before. Seldom do they have in mind your emotional state. Yet at times, your emotional reactions to your illness are more stressful than are the physical effects.

While you and your family are doing everything you can to treat and cope with your physical ailments, there may be more you and they can do to relieve your emotional distress. You can learn to better understand and accept your feelings–to understand without shame, self-blame, guilt, or recrimination.

To promote this understanding, I’ll describe the commonly experienced emotions of persons with a chronic illness, recognizing, of course, that not everyone experiences all the reactions I’ll describe.* In describing these emotional reactions, I may be giving voice to thoughts and feelings you’ve never expressed or possibly even acknowledged. This may upset those of you who are able to maintain a positive attitude by putting aside disturbing thoughts and feelings. This article is for those of you who need the emotional relief of a shared understanding.

* (The emotional reactions described pertain primarily to chronic disorders acquired after childhood.)

DISTRESSING EMOTIONS

ANGER–You’re angry for having an illness–but whom should you be angry at?– God, fate, the whole world? You’re angry at doctors because they have no cure for your disease. You thought doctors knew so much, but now you realize they don’t. What’s more, they don’t seem particularly interested in you except as a “case.” When you go for a visit, they’re either rushed and don’t explain enough or say things that frighten and upset you.

You’re also angry at family members and friends who at times are unavailable when you need them and who expect more of you than you can do. You wish they could live in your body for a day or even an hour so they could understand what life is like for you. Then maybe they wouldn’t say or do things that hurt your feelings. You’re also upset by the thought that they might resent all they have to do for you–and you feel hurt and resentful in return.

SELF-BLAME–You may blame yourself for your illness. Maybe you brought on your condition by not taking proper care of yourself. Or maybe God’s punishing you for something you did wrong–but you don’t know what. You just feel it’s your fault.

SHAME–If you take your illness as a sign of weakness or as reflection of a flawed character, you’re ashamed to be ill. If you pride yourself on being independent, or on doing things for others, you’re especially ashamed if you need others to do things for you. Moreover, if you have a visible disfigurement or deformity you may be painfully self-conscious and ashamed.

FRUSTRATION–If you have a disability that requires you to rely on others for your daily needs, you’re constantly frustrated. You’re frustrated because you can’t do these things for yourself and you’re frustrated because others don’t do them promptly enough or exactly as you would like. Also frustrating and depressing as well is to be unable to engage in activities you once enjoyed, or to use abilities in which you once took pride.

SELF-DEVALUATION–If you’re unable to do what you used to do, you not only feel frustrated but inadequate too. And, if in addition, you’re a perfectionist with expectations you can no longer meet, you may discourage or even hate yourself.

SELF-PITY–You feel sorry for yourself if you can’t lead a normal life like everyone else. You feel cheated and unfairly treated if you’re unable to finish your education, get married, have children, earn a living, or pursue a career. And you can’t help envying and resenting others who can do all these things.

GUILT–You feel guilty if you can’t fulfill your responsibilities as a wife or husband. Guilt may be unbearable if you’re a mother unable to do all you think you should for your children. Guilt is intensified if you feel you’re a burden on others–especially if you need help in your personal care and hygiene. And you should sense resentment on the part of family members on whom you depend, you not only feel guilty but hurt as well.

FEAR–If your disorder is potentially progressive, you live in dread of the future and are alarmed by any actual or imagined change in your condition. If your condition is life-threatening a cloud hangs over your head. You’re also plagued with fears relating to your family. If you have young children–you worry–what will happen to them if something happens to you? If you’re dependent on your parents or partner for personal care, you worry–what will happen to you if anything happens to them? Although you know it’s unlikely, the thought has occurred to you–what if your partner gets tired of taking care of you and leaves? What would you do then?

EMOTIONAL RELIEF

Even though your emotional reactions may be commonplace, if you criticize yourself for your feelings, you may be suffering more than need be. If that’s the case, you gain both understanding and self-acceptance by sharing your feelings with a sympathetic family member or friend who is sensitive to your feelings and knows how to listen. You can even use this article as a basis for talking about your feelings.

You can also lighten your emotional burden by sharing your feelings in a support group or with others individually through NORD’s “networking” program. If these sources are not sufficiently helpful, you can benefit from professional counseling. Counseling may not only relieve the pains of guilt, apprehension, anxiety, self-disparagement, and depression, but may provide new perspective for living and coping with your disorder.

It’s important to keep in mind–while you’re not responsible for your physical disorder–you are responsible for what you do or don’t do to help yourself live with it. I hope you’re a good helper.

Five Ways God Uses Problems
By David Langerfeld

The problems you face will either defeat you or develop you - depending on how you respond to them. Unfortunately, most people fail to see how God wants to use problems for good in their lives. They react foolishly and resent their problems rather than pausing to consider what benefit they might bring. Here are five ways God wants to use the problems in your life:

1. God uses problems to DIRECT you. Sometimes God must light a fire under you to get you moving. Problems often point us in a new direction and motivate us to change. Is God trying to get your attention?

“Trust in the Lord with all your heart; and lean not unto your own understanding. In all thy ways acknowledge him, and he will direct your paths.”

Proverbs 3:5-6

2. God uses problems to INSPECT you. People are like tea bags… if you want to know what’s inside them, just drop them into hot ever water! Has God tested your faith with a problem? What do problems reveal about you?

“When you have many kinds of troubles, you should be full of joy, because you know that these troubles test your faith, and this will give you patience.”

James 1:2-3

3. God uses problems to CORRECT you. Some lessons we learn only through pain and failure. It’s likely that as a child your parents told you not to touch a hot stove. But you probably learned by being burned. Sometimes we only learn the value of something by losing it.

“It is good for me that I have been afflicted; that I might learn thy statutes.”

Psalm 119:72

4. God uses problems to PROTECT you. A problem can be a blessing in disguise if it prevents you from being harmed by something more serious. Last year a friend was fired for refusing to do something unethical that his boss had asked him to do. His unemployment was a problem - but it saved him from being convicted and sent to prison a year later when management’s actions were eventually discovered.

“You intended to harm me, but God intended it for good”

Genesis 50:20

5. God uses problems to PERFECT you. Problems, when responded to correctly, are character builders. God is far more interested in your character than your comfort. Your relationship to God and your character are the only two things you’re going to take with you into eternity.

“We can rejoice when we run into problems… they help us learn to be patient. And patience develops strength of character in us and helps us trust God more each time we use it until finally our hope and faith are strong and steady.”

Romans 5:3-4

God is at work in your life - even when you don’t recognize it or understand it. But it’s so much easier when you surrender to his plan for your life.

What causes lupus?

Researchers are hunting for clues to this mystery. Most likely, lupus develops when a person inherits certain genes from their parents, and then factors in the environment such as infection from a virus, exposure to sunlight, extreme stress, or hormone surges trigger the disease. Lupus is not infectious, meaning that a person can’t “catch” lupus from someone else.

Who gets lupus?

While lupus can develop in men or women, it is much more common in women. Most get the disease during their childbearing years. A person of color (African American) is three times more likely to get lupus than a Caucasian individual, and is also more likely to have disease that is severe. Other groups that get lupus more frequently are Asian-American, Latina/Hispanic, and Native American women.

How is lupus diagnosed?

Many people suffer from lupus for months or years without a diagnosis. Not only do the symptoms of lupus come and go and often look like those of other illnesses, but at this point, no single test can prove that a person has lupus. To make a diagnosis, a doctor needs to ask detailed questions about medical history, do a careful physical examination, and take blood and urine for testing to get a picture of level of inflammation in the body, and how the immune system is working.

What can be done to treat lupus?

There is no cure for lupus, and everyone has a different experience with it. But an early diagnosis and the right treatment can help to lessen pain and other symptoms, and lower the risk that organs or tissues will get damaged. Treatments are given based on how active and widespread the lupus is—which is why it’s so important to get tested regularly for blood or urine changes that can signal a flare. Treatments include medicines, stress-relief strategies, healthy diet, physical and emotional rest, psychotherapy, and avoidance (or protection) from direct sunlight. Some of these approaches can make a very big difference in how a person with lupus feels from day to day.

What medicines are used to treat lupus?

Many people with lupus at some point take prescription corticosteroids, anti-malaria drugs, or other medicines that lessen the immune system’s attack on itself. These powerful drugs can really improve lupus and even protect organs during a flare, but some also have unpleasant side effects. Many people also take over-the-counter non-steroidal anti-inflammatory drugs (NSAIDs) and acetaminophen, which can make a dramatic difference in lessening stiffness, joint pain, and other discomforts.

What is the outlook for people with lupus?

There isn’t a cure yet, but every year researchers get better insights into lupus and come closer to uncovering less toxic and more specific treatments. In 1955, only 50 percent of people newly diagnosed with lupus were expected to live more than four years. Today most people with lupus can look forward to a normal lifespan.

Reviewer: Gary Zagon, M.D.
Spring 2006

Source: