Positive Options for Sjgren's Syndrome

Hunter House Inc., Publishers

Contents

Foreword.....................................................................viiiAcknowledgments..............................................................xIntroduction.................................................................1Chapter 1: What Is Sjgren's Syndrome?.......................................3Sjgren's Syndrome and the Immune SystemSymptomsOther ProblemsSjgren's Syndrome and Lymphoma?Who Gets Sjgren's Syndrome?Preventing Sjgren'sChapter 2: Diagnosing Sjgren's Syndrome.....................................18The Schirmer I TestTests Using DyesMouth ExaminationLip BiopsyBlood TestsOther TestsWhat Happens Next?Chapter 3: Treating Sjgren's Syndrome - Simple Measures.....................25Simple Solutions for a Dry MouthSimple Solutions for Dry EyesSimple Solutions for Aches and PainsSimple Solutions for Skin ProblemsSimple Solutions for Other Problem AreasChapter 4: Treatments for Sjgren's..........................................36More Complex MedicationMedications to Avoid If PossibleSurgeryAcupunctureChapter 5: Supplements.......................................................43Vitamins and MineralsOther SupplementsWhat to Consider when Choosing a SupplementChapter 6: Managing Flare-Ups and Pain.......................................49Managing Flare-UpsManaging PainHelp from Your DoctorPain Clinics and Pain Management ProgramsPain MedicationChapter 7: How to Help Yourself - Lifestyle Issues...........................59FatigueNot Forgetting SexDietDigestive ProblemsSmokingTravelStressChapter 8: Exercise and Physiotherapy........................................76To Rest or Not to Rest?Which Exercise?Don't Overdo ItHow to Get the Best from Your ExerciseHeatDon't Forget to Warm DownChapter 9: Coping With Your Feelings.........................................84GriefReliefAngerDelayed ReactionEmotional Ups and DownsDealing With DepressionDealing With Anger and ResentmentManaging Feelings with Your Partner and FamilyHow to HelpPositive ThinkingHow to Be More PositiveChapter 10: Becoming a Parent................................................96MiscarriageCan the Baby Inherit Sjgren's?PregnancyAfter the Birth and Flare-UpsAdjusting to MotherhoodPostpartum DepressionBaby CareConclusion: The Future.......................................................106Suggested Reading............................................................108Resources....................................................................109OrganizationsWebsitesIndex........................................................................112

Chapter One

Jean Much of the time I feel fine, if a bit less energetic than I used to be. When my Sjgren's is active, I feel vaguely fluey and really tired and I'll often need to take a few hours of complete rest before my energy comes back. My mouth feels hot and dry and I usually drink lots of ice water. My eyes are very sensitive to light, especially sunlight, and I have to wear sunglasses in all sorts of embarrassing places, such as subway stations, church, and concerts! I also have very dry skin and tend to get rashes.

So what is Sjgren's syndrome (pronounced `Sher-gren' or `Showgren')? With its hallmark symptoms of dry eyes and a dry mouth, that are often accompanied by fatigue and generalized aching, this condition has only been recognized relatively recently and certainly isn't yet a household word.

A syndrome is a group of related symptoms or signs that occur together to characterize a particular condition. Sjgren's syndrome (sometimes called SJS for short) was described by the Polish doctor Johann Mikulicz-Radecki in 1898 and was at first called Mikulicz syndrome. However, this name was later applied to other conditions that cause dryness and is therefore no longer used specifically to describe this disease, and so the name of the syndrome was changed to Sjgren's syndrome (SJS) to acknowledge the work of Dr. Henrik Sjgren, a Swedish ophthalmologist who also described the set of symptoms specific to Sjgren's.

In 1933 he noticed that there was a connection in his patients between severely dry eyes, dry mouth, and arthritis. Later on, it was recognized that patients could have the dry eyes and mouth without the arthritis. Sjgren described this syndrome in his doctoral thesis.

There are two kinds of Sjgren's syndrome that have been defined:

1. Primary Sjgren's syndrome occurs on its own with no other associated disease.

2. Secondary Sjgren's syndrome is associated with another autoimmune disease, like rheumatoid arthritis, lupus, or primary biliary cirrhosis.

Sjgren's Syndrome and the Immune System

The fact that Sjgren's is an autoimmune disease may raise fears that you have a weak immune system and are therefore more prone to disease, but with Sjgren's it appears that the immune system is, in fact, overactive.

Almost fifty years ago Sjgren's was defined as a "chronic autoimmune rheumatic disease characterized by the sicca complex (decreased tears and saliva), resulting in keratoconjunctivitis sicca (dry eyes) and xerostomia (dry mouth)." Of course, as many Sjgren's patients know all too well, the symptoms can be much more wide ranging than that and the illness can attack just about any part of the body. It is very common for patients to suffer from fatigue and aching joints, for example, as well as dry airways and skin, and the disorder can cause problems as diverse as numbness, skin rashes, and an underactive thyroid gland. Inflammation is a common problem, and it can affect the joints, muscles, nerves, kidneys, thyroid gland, and indeed just about any area of the body.

In a Sjgren's patient, the body's immune system gets mixed up and starts attacking the body's own healthy cells as if they were invaders, such as germs. Since the body attacks itself, this type of condition is called an autoimmune syndrome, and although this mix-up happens in all autoimmune diseases, in Sjgren's, the body's primary target is the body's moisture-producing glands. The white cells, or lymphocytes, attack and invade the glands, and this gradually reduces the glands' ability to produce fluids-tears and saliva-hence the dry eyes and mouth that are the primary symptoms of Sjgren's.

During this autoimmune process, the immune system produces antibodies, exactly as if the body was fighting off a virus. These are called autoantibodies, and since their presence can be detected through blood tests, testing for them can help doctors to diagnose whether or not a patient is suffering from Sjgren's.

Sjgren's is not by any means the only autoimmune disease. There are a whole host of them. Some, like lupus and Sjgren's, may affect many organs of the body; while others, like thyroid disease or pernicious anemia, may single out just one organ. All are related in some way and sometimes display similar symptoms, which can complicate the doctor's job of diagnosis. You may find that you are, or have been, tested for some of the following:

* Lupus (systemic lupus erythematosus, or SLE)

* Scleroderma

* Rheumatoid arthritis

* Polymyositis

* Dermatomyositis

* Thyroid disease

* Autoimmune liver disease

Patients with secondary Sjgren's syndrome will also suffer from some other kind of autoimmune disorder, like the ones listed on the previous page. Two of the most common include rheumatoid arthritis and lupus.

Rheumatoid Arthritis

Rheumatoid arthritis (RA) is a very common autoimmune disease affecting approximately one in one hundred people, in which the body attacks itself, leading to inflammation and damage to the joints. Because the illness is systemic (it affects the whole body), other organs can also be affected, and additional symptoms include fatigue and depression.

As with Sjgren's, more women than men are affected. For every man who suffers from this disorder, three women get it. Women also tend to develop it sooner and more severely-it typically first manifests itself in women who are in their thirties and in men who are in their forties.

Some of the symptoms of rheumatoid arthritis are the same as those that occur in other forms of arthritis and some are quite distinct. As with other autoimmune diseases, experts believe it originates with part of the body's defense system, which attacks the body's own tissue, mistaking it for foreign invaders, such as bacteria or viruses. There are more than one hundred types of arthritis, not all of which involve inflammation or inflammation alone. Nevertheless, arthritis tends to involve common symptoms, including warmth, pain, soreness, tenderness, or irritation accompanied by swelling and redness in one or more joints; stiffness in the joint, especially on waking or after inactivity; and difficulty or pain in moving the joint that results in loss of movement and flexibility. Treatments consist of management (rest, pain management, lifestyle changes, and sometimes diet), drugs, and, in severe cases, surgery. Research into RA is ongoing, and areas of investigation include genetic inheritance and gene therapy, the role of hormones (given that so many more women than men contract the disease), the nature of the immune system, and new drugs designed to halt the inflammation process.

Lupus

Lupus is an autoimmune disease in which a person's immune system becomes overactive and attacks the body, causing damage and dysfunction. Lupus is called a multisystem disease because it can reach many different parts of the body, sometimes affecting major body organs.

Some patients with lupus have a very mild condition, which can be treated with simple medications, whereas others can have serious, life-threatening complications. Like Sjgren's, lupus is more common in women-in fact, 90 percent of sufferers are women-and tends to occur in those between the ages of fifteen and forty. The reason for this is unknown, as is the cause, though certain factors, such as direct sunlight, can make the condition worse. As with Sjgren's, appearances can be deceptive-the typical "butterfly"-shaped rash on the face gives a false impression of glowing good health, while the unfortunate sufferer may be feeling far from healthy.

Symptoms include extreme fatigue, joint pain, muscle aches, anemia, and general malaise, and lupus may mimic other diseases, such as multiple sclerosis and rheumatoid arthritis, making it difficult to diagnose. It is believed to affect more than 1.5 million people in the United States. Options for lupus sufferers have improved in recent years, with the development of better drug treatments and greater awareness of the condition.

Fibromyalgia (FM)-Another Related Condition

Fibromyalgia (FM), formerly sometimes called fibrositis, is not well understood and is characterized by a group of symptoms involving pain and fatigue. Some estimate that as many as half of all people with Sjgren's also suffer from FM. As with Sjgren's and lupus, FM can be a frustrating condition in that people often don't look ill, but they feel awful. Other symptoms include poor or unrefreshing sleep, depression, forgetfulness, poor circulation, headaches including migraine, an urgent need to pass water, and irritable bowel-type symptoms. Sometimes people with FM experience the same kind of debilitating fatigue as those with Sjgren's-an exhaustion that may come on suddenly and necessitate the sufferer putting her feet up for absolute rest.

The pain affects the muscles and ligaments, but not usually the joints, and is often described as a burning kind of pain. Doctors tend to diagnose it by applying pressure to several tender points around the body. In a person with normal health, such pressure may be uncomfortable but not painful; in someone with FM, it may cause considerable discomfort or pain.

The cause of FM isn't known, although some research has linked the condition with sleep deprivation. It is also thought that a trigger may be needed for FM to develop, such as a virus or any incident that traumatizes the body, like childbirth or a car accident. Treatment includes drugs to control the pain and improve sleep, though lifestyle modifications can be very effective in helping people manage their condition. These include rest, diet, exercise, stress management, and warmth or heat to make the body more comfortable.

Symptoms

Dry mouth and dry eyes are the two key symptoms of Sjgren's syndrome. The absence of salivation and tearing-two seemingly insignificant functions that are largely taken for granted-can have a significant effect on the health of the mouth and the eyes.

Dry Mouth

Julia On bad days, my mouth sometimes feels as if it's full of cotton, and I find it more difficult than usual to swallow and taste. Certain foods make my tongue sore, such as tomato ketchup, vinegar, pineapple, oranges, and apples. My sense of smell has changed, and I have developed a dry cough. I also get occasional mouth infections.

In Sjgren's, the body's immune system gradually attacks and destroys the saliva glands, which are located in the mouth and cheeks and underneath the chin. Most of them are tiny (especially the ones on the inside of your lip), and people don't tend to be aware of them until they stop working properly.

Drinking more liquids helps, but sometimes even doing that isn't enough to replace the natural moisture in the mouth because the body is producing diminishing amounts of saliva. Imagine eating ten crackers in succession without a drink! As the dryness worsens, eating and swallowing become progressively more difficult. Some people may also find that they develop a dry cough or have difficulty in speaking, and it is quite common to undergo changes in smell and taste.

Saliva is more than just water. It contains important substances that are designed to help protect the teeth from decay. Consequently, when not enough saliva is present, the result tends to be more cavities and more scolding from the dentist! Indeed, it is often the dentist who spots the first tell-tale signs and recommends the patient to be tested for Sjgren's.

Along with dryness and dental decay comes soreness. A dry mouth easily becomes damaged, cracked, and inflamed. Mucous membranes can feel as if they are burning. Infections can get into the gums, and thrush may become a constant problem. In severe cases, the parotid glands on either side of the face (the ones that swell up when someone has the mumps) can puff up and become inflamed or infected.

Another complication can be a sore throat and problems with hoarseness in the voice, which can be serious if you have a job like singing or teaching, where you need a good, strong, clear voice. Fortunately, you can help protect your voice, and the measures you can take are discussed in Chapter 3.

Dry Eyes

Mark

At first I kept running to the mirror to see if I had an eyelash stuck in my left eye, in particular, but there was never anything there. I feel as though there's a bit of sand all the time in my eyes-on a windy day it's as though specks of grit are constantly being blown into my eyes. They feel hot and very sensitive.

Feeling like you have a bit of grit in your eye but being unable to see it is one of the classic signs of Sjgren's. As the immune system attacks the lachrymal glands (the ones that produce tears), the glands produce less and less of the liquid that is vital for lubricating the eyeball. Doctors call this aqueous tear deficiency, because the amount of water in the tears is reduced. Lack of moisture makes the eyes more sensitive to both chemical and physical environmental irritants, producing the gritty sensation. Without tears, the eye's delicate surface is vulnerable to damage every time the eyelid flicks over it or if a speck of dirt gets in. There may also be an abnormal sensitivity to light, and/or little trails of mucus on the cornea, and the white of the eye may become red. In severe cases, infection and corneal erosions may occur.

Needless to say, a dry eye is also extremely uncomfortable, so most people will seek medical help and advice a long time before any serious damage has been done. I, for example, used to wear soft, disposable contact lenses occasionally for acting or singing on stage. Gradually I became aware of the fact that they just weren't comfortable any more. They felt gritty, and when I took them out, my eyes felt sore. A visit to the optician confirmed that I was not producing enough tears, and I was prescribed eye-drops that replace the moisture.

Is the Dryness Really Due to Sjgren's?

It is important to realize that a number of other conditions can mimic the pattern of the dryness of Sjgren's syndrome, particularly in the early phase. A combination of fatigue, a feeling of dryness, and widespread aching may, for example, present as part of depression, FM, or hypothyroidism.

Other diseases influence the brain centers that control tears and saliva. People with multiple sclerosis and diabetes may have dryness of the eyes and mouth, as these diseases affect the brain processes involved with the control of certain sensory and motor functions.

In addition to problems with the neural activation of the glands, other medical conditions can cause the glands to be dry or to become enlarged. People with FM may suffer fatigue, memory loss, aching muscles and, occasionally, depression. They very frequently have dryness of the eyes and mouth. These symptoms may be very disabling but, unlike in Sjgren's, the gland itself is not damaged. It is important to distinguish these symptoms from Sjgren's syndrome itself, which is an autoimmune process that does destroy the gland, because the treatments are different.

Decreased tear and salivary production also occurs as part of the normal aging process, and a variety of commonly used drugs interfere with the neurogenic stimulus to the tear and salivary glands-the so-called anticholinergic drugs. Examples of these are antidepressants, antihistamines, and some drugs used to treat Parkinson's disease.

Other Problems

Mandy

I blamed our old mattress for my discomfort and made my husband go out and buy an expensive new one-the aches and pains were just as bad! I sometimes get so tired that I fall asleep on the sofa watching TV with the kids, and a few times we've missed their after-school activities, which I feel very guilty about. I've also got dreadful teeth and have had to have one removed!

Unfortunately, both primary and secondary Sjgren's syndrome can affect other parts of the body as well, causing problems in the skin, joints, lungs, kidneys, blood vessels, and nervous system-though usually not all at once. The symptoms are all linked to the immune system's attack on the body's moisture-producing glands. In other words, the autoimmune response that causes dry eyes and mouth can cause inflammation throughout the body.

(Continues...)

Excerpted from Positive Options for Sjgren's Syndromeby Sue Dyson Copyright © 2005 by Sheldon Press. Excerpted by permission.
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