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What is Scleroderma?

Scleroderma describes a group of related connective tissue diseases involving skin, joints and internal organs. The term scleroderma literally means hardening (fibrosis) of the skin. The cause of scleroderma is unknown but the disease process is related to a malfunction of the vascular and immune systems. Researchers believe that the immune system, which protects us against infection and cancer, becomes overactive in patients with scleroderma resulting in the overproduction of collagen (connective tissue or "scar" tissue) in the body. The excess collagen is deposited in the skin and in some cases, in and around the organs and that leads to the characteristic hardening and thickening effect. In some people, scleroderma is localized to the skin while in others, it is systemic with excess collagen building up not only in the skin but in and around various organs such as the esophagus, kidneys, lungs, gastrointestinal tract, heart, and peripheral nervous system. Organ involvement leads to many accompanying complications. Many people with scleroderma experience a plateau where the condition stabilizes, which is then followed by a period of improvement and skin softening. The most critical period for systemic scleroderma is usually within the first two to five years of onset when there is the highest risk for rapid progression and the development of serious complications. Most cases of scleroderma are seen initially by a dermatologist and, as the condition progresses, patients often are referred to the care of a rheumatologist. It is important for people diagnosed with scleroderma to be examined and followed by their doctor to evaluate the development of other autoimmune conditions or complications. Fortunately, scleroderma is a rare disease. Data regarding prevalence and incidence varies widely but estimates are that the prevalence (number of cases present in the population) of scleroderma ranges from 50-300 cases per million people and the incidence (number of people per year diagnosed with scleroderma) ranges from 2.3 to 22.8 cases per one million people per year. This means that scleroderma is believed to affect between approximately 40,000 to 300,000 persons in the United States
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and some estimates are even higher. The disorder most commonly occurs in women between the ages of 35 and 55, however, men and children can be affected as well. Women are affected approximately 3-4 times as frequently as men. There is a slightly increased risk for developing scleroderma among African-Americans. African-Americans are also more likely to develop severe lung complications. Native Indians are also at a higher risk than the general population for development of scleroderma. Specifically, Choctaw Indians in Oklahoma are up to 20 times more likely to get scleroderma than the general population. Scleroderma is not contagious. Genetic factors are thought to be involved in scleroderma since there is a high frequency of other autoimmune disease in families of people with scleroderma. However, most people who develop scleroderma do not have relatives with scleroderma and their children do not have a higher risk of developing the disease. Knowledge is Critical when Dealing with a Life-Altering Condition such as Scleroderma If you or a loved one has been diagnosed with scleroderma, it's critical to learn everything you possibly can about this condition so that you can make informed decisions about your treatment. That's why we created the Medifocus Guidebook on Scleroderma, a comprehensive 141 page patient Guidebook that contains vital information about scleroderma that you won't find anywhere in a single source. The Medifocus Guidebook on Scleroderma starts out with a detailed overview of the condition and quickly imparts fundamentally important information about scleroderma, including:
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The underlying causes of scleroderma. The risk factors that can increase a person's chances for developing scleroderma. A detailed overview of the two major types of scleroderma that are recognized, known as localized scleroderma and systemic scleroderma.

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The signs and symptoms that are associated with both localized and systemic scleroderma. The secondary complications that may develop in people with systemic scleroderma, including:
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Pulmonary (lung) complications Cardiac (heart) complications

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Renal (kidney) complications Gastrointestinal complications Vascular complications Oral and dental complications

How scleroderma is diagnosed based on factors such as signs/symptoms, patient history, physical examination, blood tests, and special diagnostic procedures that may be necessary to demonstrate damage to specific organs in people with systemic scleroderma.

Other underlying medical conditions that may be confused with scleroderma and must be considered in the differential diagnosis of scleroderma.

Understanding the Standard Treatments... and the Treatment Options Currently, there is no known cure for scleroderma. The primary goals of treatment for people with scleroderma are as follows:
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Relieve symptoms to the greatest extent possible. Maintain the patient's overall functional capacity. Prevent long-term complications of scleroderma. Manage and treat complications, if they develop. Enable patients to lead a reasonably good quality of life.

Understanding the standard treatments - and the treatment options - is critical for successfully achieving the goals of treatment for scleroderma. As you read through the section of the Guidebook that focuses on the treatments for scleroderma, you will specifically learn about:

The treatment options that are currently available for the management and treatment oflocalized scleroderma, including:
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Psoralen photochemotherapy (PUVA) Topical photodynamic therapy Drug therapy

The medications that are prescribed for the management and treatment of systemic sclerosis, including:
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Immunosuppressive agents Antifibrotic agents Anti-infammatory agents Vasodilator agents Angiotensin-converting enzyme (ACE) inhibitors

The management and treatment of secondary complications that may develop in people with scleroderma, including:

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Pulmonary fibrosis Pulmonary hypertension Raynaud's phenomenon Heartburn Joint pain Limb contracture due to stiffness Impaired kidney function Dry mouth and dry eyes in people with scleroderma who also suffer with Sjogren's syndrome

Exercise Can Help!
By Jane Brandenstein, P.T., University of Pittsburgh Medical Center (originally published in "Scleroderma Foundation Newsline," vol. 2, no. 3, Summer/Fall 1999) Tell a friend:
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Editor's note: You can skip directly to the subheads that interest you, by clicking on these links:  Range-of-Motion Exercises o o o  o o o General Warm-up Arm and Hand Exercises Leg and Foot Exercises Stretching Exercises Augmentation Manual
Jane Brandenstein, P.T., speaking at the Scleroderma Foundation's 2001 National Conference in Washington, D.C.

Facial Exercises

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Exercises for the Lips Exercises for the Tongue Exercises for the Palate

As physical therapists, there are some things that we have learned over the years about scleroderma, and there are even more things we wish we understood better and could share with you. But, the information is just not there at this point in time, so as health professionals, we experience a certain amount of frustration, just like you do. What we would like to pass on to you are some of the things that we have found to be helpful and important. One thing we do know is that exercise is imperative in order to maintain the highest level of functional independence possible. We find that you can and should do different things on different days. There are several types of exercise and any or all can be beneficial for you, depending upon your particular needs. These can be range of motion, stretching, strengthening, and/or conditioning/aerobic exercises. Our suggestion is to do range-of-motion exercises to all joints after a warm shower, first thing in the morning to "get things going." Then, later in the day, set aside another period for exercise. It may be helpful to take a pain relieving medication, such as an extrastrength Tyienol one hour before you do your exercise program. Depending on how you feel, you can really stretch those tight areas, strengthen the weaker ones, or do something aerobic, like walking, hiking or even swimming. Basically, the second workout should target the areas that need work. We have found that warm applications are beneficial prior to stretching exercises. Some examples of heat include paraffin baths for hands or feet, electric moist heating pad (for example, the thermophore), microwaveable hot packs, and warm showers or baths. These heat applications should be warm, but not hot as you could bum your skin. Use these for 15 to 20 minutes, no longer than half an hour. If you are having joint aching or stiffness, you can use the heat several times followed by the stretching exercises. When doing range-of-motion and stretching exercises it is very important to go as far as you can, even to the point of pain and hold at this end of the range point for at least ten seconds. Do not bounce with the exercises; a sustained stretch is more beneficial. Sometimes it is helpful to have someone else help by giving some additional pressure at the end range position to get a better stretch. Common areas that experience tightness are the hands, mouth opening, and neck areas, but many other areas can be involved. There are many instances where the skin tightening eases off, but if you have not been stretching you will not be able to take advantage of this. You must seriously think, "use it or lose it" for each motion. It is really important to take each joint through the full range-of-motion each day and to note any changes from the day before. If an area is stiffer, then be a bit more vigorous to get that last degree of motion. Strengthening is another important part of a general exercise routine. Lifting your arm or leg against gravity is actually strengthening and may be the beginning of the program. You could then move to elastic loops (Therabands), cuff weights, or machines for resistance. We do not normally recommend the use ofbarbells this is too much stress on the tiny hand joints. Therabands are available in any Physical Therapy (PT) clinic and work well for strengthening. The best thing when using a Theraband is to make loops in the end to go around your wrist or ankle and tie the other end to something strong. A visit to a physical therapist may be beneficial for instructions. We suggest you do strengthening on an everyotherday schedule. This gives the muscles a chance to recuperate from the activity. Conditioning exercises help with endurance. These exercises can be in the form of walking, riding a bike, or swimming in a heated pool. These activities are good unless you have been told by your doctor to avoid them. The best exercise is something that you want to do. There is no competition; do what you can, but DO IT! If you are unable to tolerate twenty minutes, start with a few minutes and gradually increase. Be pleased with what you are able to accomplish. These types of exercises increase your cardiovascular fitness, which helps combat the fatigue, and also help you to feel better about yourself. If you are going to be swimming, it is important to maintain skin health with moisturizing creams. Those with a high glycerine content seem to be helpful to many folks. Physical Therapists are real fans of group exercise programs, as you derive encouragement from each

other and it is not quite as boring. You make a commitment to yourself and your friend that you are not likely to break. There are two programs that are designed for people with any or all types of arthritis. The Arthritis Foundation and health professionals specializing in inflammatory diseases approve both. PACE is People with Arthritis Can Exercise, a land-based group program, and the AFYAP (Arthritis Foundation/YMCA Aquatics Program) is a program of warm water swimming exercises. Both are taught by people specifically trained in the programs and who are aware of the limitations or cautions important to you. The following exercise suggestions may be helpful. They are a good start, but they are not all inclusive. It may be helpful to see a physical therapist for evaluation and recommendations to help with your exercise program. Many times it is helpful to have a professional to ensure that you are working hard enough. There are many therapists who can be very helpful to you, but may not have worked with a patient with scleroderma before. Again, we would like to reinforce the importance of doing some exercises every day. The stretching may be painful, but it is imperative. We would also like to stress that you not start any exercise program without the full approval of your physician(s).

Range-of-Motion Exercises General Instructions
These exercises are to be done daily, preferably early in the morning after heating your stiff joints. Each exercise should be repeated five times. After several days of performing the exercises, try to establish an estimate of your range of motion and the level of pain you have for each joint. On those days that you find either decreased motion or increased pain in any joint or muscle area, you should continue to use heat and repeat exercises three additional times for those areas. In this manner, you should be able to detect limitations and prevent the loss of motion of a joint. You should decrease the number of exercises temporarily for only I those areas where an acute flare-up has occurred (hot, painful, swollen joint), and increase them when the flare subsides. All exercises are designed to limber up muscle and joint stiffness as well as maintain maximal joint motion. These exercises are to be performed in your bed, if firm enough, or on any other flat surface while lying on your back, and without pillows.

General Warm-up
1. Gluteal Setting Pinch or tighten your buttock muscles together tightly, holding for five seconds and relaxing 10 seconds. 2. Quadriceps Setting Place a rolled-up towel under each knee. Tighten the muscles on tops of thighs and hold for five seconds, and then rest 10 seconds. This exercise will pull the kneecaps up toward your hips while you are attempting to straighten the knees and lift your heels off the bed. Your buttocks must remain resting on the bed.

Arm and Hand Exercises
1. Forward Elevations Arms at sides, elbows straight, hips and knees bent. Palms turned toward body, raise both arms back over your head, leading with your thumbs as far as possible in a slow, wide arc of motion. Slowly return to starting position and relax 10 seconds. 2. Sideward Elevation (Abduction/Adduction) Arms at sides, elbows straight, palms upward, hips and knees bent. Slowly slide arms out to the sides and up over your head as far as possible, leading with the thumb. Slowly return to starting position and relax 10 seconds before repeating. Be sure to keep the elbows as straight as possible.

3. Shoulder Rotations Elbows away from body as far as possible, up to level of shoulders with elbows bent 90 degrees and resting on bed with fingers pointing to the ceiling and palms toward the body. Hips and knees bent. Rotate your shoulder while attempting to touch little finger to mattress at hip level. 4. Elbow Flexion and Extension Start with arms straight at your side, with palm resting on bed, and hips and knees bent. Start by turning palm up and bending elbow in an attempt to touch your shoulder Then turn your palm away from your face and attempt to touch your opposite knee while attempting to straighten the elbow as much as possible. Repeat with your other arm. 5. Finger and Wrist Flexion and Extension Arms at sides, fingers pointed toward toes with palms facing body. Start by curling or bending fingers and attempt to touch tips of fingers to palms. Then bend wrist as far as possible away from hips. Slowly straighten fingers as much as possible and bend wrist toward hips as far as possible. Return to start and relax.

Leg and Foot Exercises
1. Hip and Knee Flexion and Extension Legs flat on bed with toes and kneecaps pointing straight up to ceiling and heels four to six inches apart. Start by bending your hip and knee. Slide your foot straight toward your buttocks as far as you can, still keeping some part of the foot resting on the bed. Hold, and then slide back down to the starting point and relax. Repeat same exercise with the other leg. 2. Hip Abduction and Adduction One leg lying flat with toes and kneecap pointing straight toward the ceiling, and the other leg with the hip and knee bent 45 degrees or approximately halfway with foot resting flat on the bed. The exercise consists of sliding the straight leg out to the side as far as possible while keeping the toes and kneecaps pointing straight to the ceiling, and then sliding back to the same starting position. Reverse leg positions and perform the same exercise with the other leg. 3. Internal and External Rotation of Legs Legs out straight with heels four to six inches apart. Roll knees in toward each other. Then roll outward, and then relax. 4. Foot and Ankle Range of Motion Legs flat on bed and heels resting on the bed at all times, start by pointing feet down with toes bent. Then point feet up with toes bent up. Then point feet in with toes relaxed; next point feet out with toes relaxed.

Final Reminder
These exercises should be done daily, slowly, and without causing increased pain.

Facial Exercises
The following list of facial exercises has been prepared by the Physical Therapy Department of PresbyterianUniversity Hospital of Pittsburgh. The exercises are designed to:    maintain or improve mouth and jaw opening improve movement and tone of the face prevent further stiffness

Do the stretches gently but firmly. Hold each position five to ten seconds before relaxing.

Stretching Exercises
1. Raise your eyebrows as high as possible, then relax.

2. Frown as hard as possible, wrinkle your nose, and bring your eyebrows together. 3. Open your mouth as wide as you can, and stretch it as much as possible. 4. Smile as widely as you can while keeping your lips closed. 5. Puff out your cheeks and stretch them as much as possible. 6. While keeping your mouth closed, tilt your head back as far as you can to stretch your throat area. 7. Pucker up your lips as if giving a kiss. 8. Make exaggerated vowel sounds (aaaaaaa, eeeeeee, iiiiiii, ooooooo, uuuuuuu), stretching as much as possible. 9. Stick your tongue out as far as possible. Then, move your tongue to the right, to the left, up toward your nose, and down toward your chin.

Insert a stack of tongue depressors, bound by a rubber band, into your mouth from your front middle teeth to your back molars on one side. Use only as many tongue depressors as necessary to stretch your jaw. Repeat to the opposite molars.

Manual Stretch
1. Place your right thumb into your left cheek and push outward firmly. 2. Place your left thumb into your right cheek and push outward firmly. 3. With both thumbs, push outward firmly on opposite cheeks at the same time.

Exercises for the Lips
1. Open your mouth as wide as possible and hold it for two seconds; close mouth, making sure your lips are closed. Practice slowly 10 times. 2. Pucker your lips and hold for two seconds, then relax. Practice slowly 10 times. 3. Spread your lips into a smile and hold for two seconds, then relax. Practice slowly 10 times. 4. Alternately pucker lips then spread lips as though saying, oooo-eeee-oooo-eeee. Practice slowly, repeating each series 10 times. 5. Open your mouth wide, then try to pucker lips as though saying "oh" for two seconds, then relax. Practice slowly 10 times. 6. Pucker your lips and make a kissing sound. Practice slowly 10 times. 7. Suck your lips into your mouth, then release in a loud smacking noise. Practice slowly 10 times. 8. Keeping teeth together, say these sounds: "ba bi bu," exaggerating the lip movements. Practice slowly 10 times.

Exercises for the Tongue
1. Open your mouth and protrude your tongue. Be sure your tongue is straight out, not resting on your lips or pointing to one side. Maintain this position for two seconds. Practice slowly 10 times. 2. Protrude your tongue and move it slowly from corner to corner of your lips. 3. Repeat step 2, as rapidly as possible, still touching each corner of your lips. Practice repeating each series 10 times. 4. Protrude tongue and point it downward toward chin; hold for two seconds, then relax. Practice slowly 10 times. 5. Protrude tongue and point it upward toward nose; hold for two seconds, then relax. Practice slowly 10 times.

6. Protrude and point your tongue to touch the top, bottom, and corners of your lips. Practice slowly 10 times. 7. Repeat step 4, as rapidly as possible. Practice repeating each series 10 times. 8. Protrude tongue and move it down then up as though licking an ice cream cone; relax. Practice slowly 10 times. 9. Move tongue around your lips in a circle, touching all of upper lip, corners, and lower lip; relax. Practice slowly 10 times. 10. Raise the tip of the tongue and touch the upper lip, the upper teeth, and the ridge behind the teeth and the hard palate. Practice slowly 10 times. 11. With your mouth open, suck up hard on your tongue, drawing in the sides of the tongue as hard as possible to the hard palate. Hold for five seconds and relax. Practice slowly 10 times.

Exercises for the Palate
1. Puff out the cheeks and press the fingers against one cheek without allowing the air to escape through the mouth or nose. Practice slowly 10 times. 2. Blow on a straw keeping a finger on one end and maintain the air in the straw for 5 to 10 seconds. Practice five times. 3. Say the (s) sound "ssss" without allowing the air to come through the nose. Practice slowly 10 times. 4. Repeat "ka" 20 times, "ga" 20 times, and "sss" 20 times.