What is Scleroderma?
Scleroderma is classified as an autoimmune disease. This means that a person’s immune system works against itself. The normal immune system protects the body by fighting off foreign invaders such as viruses and infections. In an autoimmune disease, the immune system mistakes a person’s own tissues as foreign invaders and sets up a protective attack that backfires to cause problems.
In scleroderma, cells start making collagen as if there were an injury that needs repairing. The cells do not turn off as they should and end up making too much collagen. The extra collagen in the tissues can prevent the body’s organs from functioning normally.
How will my Scleroderma be treated?
Scleroderma is a chronic illness that will not go away. However, it is manageable. Treatment can make you comfortable. It can help to control the disease and keep it from getting worse. Unfortunately, there is no single treatment for the disease because everyone’s experience with the illness is different.
The severity of Scleroderma varies a great deal from person to person. For some people, it’s a nuisance. For others it can be life threatening. For most people the illness becomes more or less severe over time. Most people experience times when the illness improves and even goes into remission. Your treatment will be based on your symptoms.
Who gets Scleroderma?
Scleroderma has few rules about who gets it and why. It’s a rare disease and fewer than half a million people in the United States are affected. Some experts say that for every 7 people with Scleroderma, 6 of them are women. The most common age to develop Scleroderma is between 35 and 50 years of age. Young children and older adults can also develop Scleroderma.
I’m having a hard time dealing with the fact that I have Scleroderma. What can I do?
Like with any change, it will take time to get used to it. There is a difference between being a victim suffering from an illness and being a person living with a disease. The word victim brings to mind being passive and helpless while the term person living with brings to mind an individual who is influenced by an illness but not defined by it. It denies a sense of acceptance of the illness with the idea that you continues to be the person who you have always been. You cannot let yourself be defined by Scleroderma. This is a hard goal to achieve, but will help you in coping with the unpredictable and uncertain nature of Scleroderma. Your family, your doctor, your priest, minister or rabbi, or a counselor can help you with this goal.
What are the early signs of Scleroderma?
The earliest symptoms of Scleroderma are often fingers that become very sensitive to cold and/or stress and fingers that sometimes change color. These changes in your fingers are known as Raynaud’s and are caused by the excess collagen of Scleroderma that narrows blood vessels and reduces the flow of blood to body tissues and organs.
What is the difference between Limited and Diffuse Scleroderma?
Limited Scleroderma is the milder form of Scleroderma and is more common among Caucasians. Patients with Limited Scleroderma, also known as CREST, usually have only thicknesses of the skin on the fingers. The letters in the word CREST stand for the symptoms of the disease. Everyone is different and has a different pattern of symptoms.
- C is for Calcium deposits in the body tissues
- R stands for Raynaud’s Phenomenon
- E is for Esophageal reflux, commonly referred to as heartburn
- S is for Sclerodactyly or thick skin on the fingers
- T is for Telangiectasias which are enlarged blood vessels that appear as red spots on the face and other areas
Generally CREST does not involve other body organs, but scar tissue in the lungs can build up.
Diffuse Scleroderma is the most involved form of Scleroderma. Thickness of the skin on the arms, legs and trunk occur, and internal organs are more likely to be involved. Tightened skin makes movement of some joints difficult. People may experience trouble bending fingers, hands and joints. Also, they will also have a smaller mouth opening caused by tightness on the face. This makes it difficult to open the mouth wide and makes it hard to close the mouth completely over the teeth.
A person who has Diffuse Scleroderma may have areas of the skin that are lighter and darker. Some will lose hair, have dry skin and sweat less all due to damage in the skin. Diffuse Scleroderma can cause changes to the gastrointestinal tract, heart, lung or kidneys.
Why is it so important for me to take an active role in my care?
You have a chronic illness that won’t go away. Depending on the severity of your Scleroderma you’ll be seeing more and more specialists and need to get the most from each doctor. In order to do this:
- Come to appointments prepared
- Ask questions – Don’t wait for the doctor to ask
- Be very clear about what you want
- Go to appointment with written list of questions and concerns that require answers
- Discuss problems
- If you can’t follow certain advice, tell the doctor and develop an alternative plan
- Don’t be afraid to question your treatment
- Don’t be afraid to ask financial questions
- It’s your right to know the cost of an office visit
- If a treatment is too expensive, ask about alternatives
My doctor speaks in “medical-talk” and often times I don’t understand, what should I do?
If you don’t understand something, don’t be afraid to speak up. Doctors sometimes forget their patients don’t speak the same language.
What can I do if I feel my doctor isn’t listening to me?
You should discuss this with your doctor. It takes some courage, but it will open up communication. Another way to get your doctor to listen is to be brief and to the point.
If need be, how can I get my doctor to spend more time with me?
Ask for more time when you make an appointment. Don’t wait until you arrive for your appointment. You should expect to pay extra because the time you’re using can’t be used by another patient. Also, make the most of your time with the doctor by going to the office prepared. Bring a written list of questions and concerns. Hand the list to your doctor when you arrive and be sure the questions are answered before you leave.
What should I ask about medications that my doctor prescribes?
- Exactly for what reason is the medication being prescribed?
- Do I really need the medication?
- What will it do?
- How and when do I take the medication and for how long?
- What foods, medications or activities should I avoid while taking the medication?
- What are the possible side effects? What should I do if they occur?
- Are any tests required to monitor the medication?
- Can I use an alternative or generic medication that is less expensive?
How do I know if the medication being prescribed is the best one for me?
Unfortunately, there is no way your doctor can know for sure. You made need to try a number of medications before you find the best one. This trial-and-error method can be costly. To keep down costs:
- When starting a new medication, if it’s possible to know in a short time whether or not it will work for you, ask for a prescription for only a week or two with refills
- Ask for samples
- Don’t be discouraged if you have to try several different things
- Call your doctor if you’re having a problem and don’t have an appointment in the near future
How can I feel more comfortable talking to my doctor?
Plan to be dressed. It’s hard to feel comfortable talking in your underwear or an examination gown. Also, sometimes doctor – patient personalities just don’t fit. If you’ve tried to open up communications and it hasn’t worked, it might be time to find a new doctor with whom you are comfortable.
With all the changes I’m experiencing in my body, I feel overwhelmed and depressed. What can I do?
By taking charge of your illness, you will feel more in charge. Having a sense of control and having positive things in your life can help you to cope better with your illness and have a better quality of life. Your illness is only one part of your life. To really take care of yourself you need to balance the more difficult parts of your life with more positive things such as fun activities and physical exercise. These will help you to have a more positive mental outlook which may help you to cope better with your illness,
I feel too tired and sore to exercise, what should I do?
Unfortunately the less active you are, the more your body becomes weak, stiff and out of shape. This can make pain and fatigue even worse. Ask your doctor about what exercises or flexibility and strengthening programs might be good for you. He or she can tell you what level of activity is right for you. Even if your doctor recommends against exercise, think about how you can spend time out of bed doing activities you enjoy.
How often should I exercise?
If your doctor has approved an exercise program for you, you should set aside time for it and exercise several times a week. Start slowly. For the first few times, you might limit your activity to warm-up exercises only.
When I start to exercise, it is very painful. Is there anything I can do to reduce the pain?
Yes, there is. Start slowly with flexibility and strengthening warm-up to get ready for more intense exercise later on.
If I exercise like my doctor recommends, should I take it easier for the rest of the day?
No you should not. You should try to be more physical in your everyday life.
- Get out of the house every day. Spending too much time in bed will make you feel more tired.
- Take a stroll after dinner
- If you can, use stairs rather than an elevator
- Work in the garden or play with your grandchildren
I (a female) am often too tired for sex and experience discomfort during the act. What can I do?
Fatigue is a frequent problem and can seriously alter your sex life. Like any activity that is important to you, you may need to pace yourself and rest up to continue your sex life.
The vagina can become dry with less lubrication during arousal. This can make sex uncomfortable or even painful. Vaginal lubricants can be bought in most drugstores and are very helpful. It’s important to look at other causes of vaginal dryness before blaming Scleroderma. Menopause and the drop in female hormones that goes with it can also cause vaginal dryness. If this is the case, an estrogen replacement in pill form or in a vaginal cream may be helpful.
Some women find sex painful because they can’t find a comfortable position. Symptoms of reflux (such as heartburn) can be made worse by lying flat and having the weight of a body on top. Joints may feel stiff and not move as easily. A warm bath may help. Being open to new positions or activities are also important.
I (a male) can’t get and maintain an erection. What causes this and what can I do?
The penis becomes erect when more blood is directed to it than drains from it. The part of the nervous system that is responsible for this is called the parasympathetic nervous system. There is evidence to show this part of the nervous system is damaged in Scleroderma.
Vascular problems may also be responsible. The blood supply to the penis can also be changed.
Once damage to nerves or vessels happens, it may not be possible to reverse it.
Problems with erections can also be caused by medication side effects. Calcium channel blockers are often used to treat Raynaud’s phenomenon. These medications improve the circulation to the fingers but decrease circulation to the penis. Discuss this issue with your doctor before you stop the medication.
Medications such as Viagra can improve the circulation and improve erectile dysfunction. Other treatments such as penile implants may also be helpful. You may want to discuss the alternatives with your doctor or be referred to a urologist.
How can I improve sexual fulfillment?
- Try to set up a calm and relaxed atmosphere
- Find positions that are comfortable for both of you
- Avoid sexual activity when you are really tired
- Avoid sexual activity after a big meal
- Avoid drinking alcohol before sex
- Check with you doctor to see if you’re taking medications which may be interfering
- Rest up and plan for sexual activity
- Stay as active as possible
- If you are having problems with arousal, or loss of interest in sex, it may be due to depression. If treatment for depression doesn’t help, you may want to consult with a professional sex therapist.
I understand the occasional difficulties I have with swallowing, heartburn (reflux), stomach and bowel problems may be due to issues with my gastrointestinal (GI) tract. What is the GI tract, and why am I experiencing these problems?
The GI tract includes:
- Small bowel (intestine)
- Large bowel (colon)
It is made up of smooth muscle, and its purpose is to move food and drink in one direction from the mouth to the rectum. We have no control over these muscles. The GI tract muscles work by reflex and keep food from backing up.
In some Scleroderma patients, the smooth muscle loses its strength and wastes away. It may be replaced by fibrotic scar tissue caused by too much collagen. When this occurs you may experience problems with your esophagus, stomach, small bowel, large bowel and or rectum.
How does the GI tract normally work?
The normally working esophagus moves food and drink down through the esophagus through the lower esophageal sphincter into the stomach. The lower esophageal sphincter stops the stomach contents from going backward up to the esophagus.
In the stomach, food and drink mix with stomach acid which helps to break the food down into small pieces that can be absorbed. The stomach contents are dumped into the small bowel near the common bile duct.
The bile duct adds salts and enzymes that break the food down further. As the food continues to move downward, nutrients (good stuff) are absorbed.
Roughage that can’t be digested passes through the small bowel to the large bowel. The large bowel soaks up water. The rectum holds the rest until you have a bowel movement.
Are there any early signs of gastrointestinal disease?
GI disease often begins with a decrease in appetite. This often means eating less and losing weight.
Why do I have difficulty chewing?
You may experience trouble chewing food because of dry membranes in the mouth. These dry membranes may be caused by Sjogren’s Syndrome. Scleroderma can affect facial; skin around the mouth making it tighter than usual. This can make chewing and opening the mouth fully more difficult.
What are some esophageal problems?
Difficulty swallowing food is called dysphagia. Food gets stuck somewhere in the passage between the mouth and the stomach. There isn’t enough muscle power in the esophagus to move the food to the stomach. Drinking a glass of water can help wash the food down. Taking smaller bits of food and chewing food longer than usual can also help.
Normally dysphagia is not painful, but it can be uncomfortable. If it does hurt, it’s possible you have developed an ulcer on the esophagus or an infection. Ulcers can occur from repeated exposure to stomach acid caused by reflux
Reflux and Heartburn:
Reflux is a “backwards flow” of stomach acid moving back up into the esophagus. Normally stomach acid stays in your stomach because it is blocked from going up by the lower esophageal sphincter. When a person has a reflux problem, the sphincter is too weak to hold the acid back.
Heartburn is a burning sensation in the chest that is caused by stomach acid moving backwards into the esophagus. The pain is in the left side of your chest and can move down into your left arm.
Stomach acid can also cause atypical chest pain, coughing, asthma-like wheezing due to aspiration, hoarse voice and a feeling of gagging.
Is reflux painful?
It can be painful and uncomfortable. The stomach’s lining is strong enough to stand the stomach acid but the esophagus is not. Over time the esophagus can be damaged by acid reflux. Simple changes in daily living and eating habits can help to prevent some of the damage. There are also medications that have been proven effective.
What can I do to limit reflux?
- Don’t eat within two hours of bed time.
- Move the main meal towards midday
- Eat more frequent small meals instead of one or two large meals.
- Eat sitting up.
- Eat slowly.
- Chew food thoroughly.
- Drink sips of water between bites and make sure each mouthful is fully swallowed before taking the next bite.
- Avoid carbonated drinks. They will fill your stomach with air and push the contents up.
- Don’t exercise after eating.
- Elevate the head of the bed at least four inches by placing it on wooden blocks. Just propping your head up on pillows won’t work because it doesn’t lift your esophagus above your stomach. Pillows should be wedged from the low back upward to avoid bending the neck or torso.
- Limit actions that increase pressure on your stomach such as bending, vigorous exercise or tight clothing.
- Try to keep your body weight within a healthy range. An overweight abdomen can put pressure on your stomach.
Are there certain foods and/or drink that I should avoid in order to reduce my reflux and heart burn?
- Caffeine, coffee (regular and decaf)
- Acidic foods ( citrus fruits like oranges, tomato sauce)
- Fried foods
- Raw vegetables
- Foods with high fat content ( fast foods, nuts, dairy products)
- Spicy foods
Smoking will also aggravate reflux.
What medications can be taken to reduce reflux and heartburn?
- Over-the Counter Antacids
- H-2 Blockers
- Proton Pump Inhibitors
What stomach problems occur with GI disease?
- Getting full with less food
- This happens because the stomach does not empty as quickly as normal. This may lead to weight loss.
- Nausea or Indigestion(burping)
- These symptoms could be due to the stomach not emptying quickly. It may seem like the stomach is blocked. In extreme cases this can lead to a gastric obstruction which will cause you to vomit your food or gastric paresis which causes problems with bowel movements.
- Erosions or Ulcer Formation
- These can result from the buildup of acids or from the use of certain medications. They cause pain, indigestion and bleeding. There are medications to treat erosions and ulcers.
- Telangiectasias are dilated blood vessels that can bleed. They can occur on the wall on the inside of the stomach. It is called a watermelon stomach because the vessels make the stomach wall look like the green stripes on the surface of a watermelon. It can be treated with laser therapy that removes the vessels during an endoscopy. Rarely the vessels may be closed off by a surgical procedure.
What small and large bowel problems can occur?
- Gas, Cramps and Bloating
- The first symptom of bowel problems may be bloating after eating. People also complain of feeling gassy or cramping. Cramping is caused by the unusually hard muscle contractions as the muscles try to move something through. When this is severe antibiotic therapy can help.
- Some people find a high fiber diet can help to reduce constipation. Others find a high fiber diet worsens their gassiness. Exercise helps promote motion in the bowels. A stool softener can help, but laxatives should be avoided. Miralax is used by some.
- Diarrhea occurs when the normal bacteria in the large bowel spreads backward into the small bowel. Diarrhea can also be caused by medications. Antibiotics and medications are available to treat diarrhea.
- A bowel obstruction is the most serious stomach problem that can occur. Something is blocking the passage of waste. When this happens the stomach can be bloated with pain. In scleroderma this happens without evidence of true blockage or obstruction but rather due to slow movement due to smooth muscle weakness. This is called pseudo-obstruction. The person is not able to move his or her bowels and may feel nausea. This situation is an emergency and needs immediate medical attention. It should be treated without surgery.
- Bowel Incontinence
- When this happens the person has difficulty holding their bowels and may have accidents. This happens because the rectum has two sphincters (muscles that block the stool) that weaken and thus the stools can slip out. This is uncommon in Scleroderma patients but may be seen in the late stages of the disease. Treatment is available.
Why do my fingers get numb and turn white, then blue or purple when they’re exposed to the cold?
This is a result of Raynaud’s Phenomenon. Raynaud’s is a change in your fingers caused by the excess collagen of Scleroderma that narrows blood vessels and reduces the flow of blood to body tissues and organs. Raynaud’s phenomenon is often times the earliest symptom of Scleroderma.
The color change is an exaggeration of what normally happens when hands are exposed to the cold. However, the normal cold response is a blotchy red and white pattern while the color changes of a Raynaud’s attack— white, then blue or purple are very different. At the end of the attack, the fingers turn red which is the result of blood flowing back to the fingers in a rush. This may be painful.
Can I develop Raynaud’s anywhere else on my body?
Yes, many people have Raynaud’s attacks in their toes?
Can Raynaud’s attacks be prevented?
Yes they can.
- Keep your entire body warm, not just your hands and feet
- Bring extra layers of clothing when you’re going somewhere that might be cold
- Wear hats in cold weather
- Use a space heater in the bathroom or at your office at work if it gets too cold
- Warm up your bed with an electric blanket or hot water bottle; Use flannel sheets
- Warm up your car before you leave the house; Use a remote car starter; Cover your steering wheel with a lambskin wheel cover
- Wear mittens (not gloves) and warm socks when the temperature drops below 65 degrees. Use chemical or battery operated hand and foot warmers
- Take extra precautions to protect your fingers and toes from the cold
- Keep gloves everywhere
- In kitchen for reaching into the refrigerator or freezer
- In office desk for when air conditioning is high
- In purse or pockets to be used in frozen food areas in grocery store
- Try to avoid sudden changes in temperature
- Wear comfortable shoes and socks that allow for good circulation
- Avoid stressful situations
- Other things can trigger a Raynaud’s attack:
- Air conditioning
- Holding a cold drink
- Washing something in cold water
Are there any medications that can help to prevent Raynaud’s attacks?
The most common medications are called vasodilators. Calcium channel blockers are the most effective. They stop the spasm of arteries in Raynaud’s phenomenon. A topical nitroglycerin cream can also be used.
For more severe Raynaud’s Phenomenon with digital ulcers intravenous prostaglandins are given.
Are there any other methods of treating Raynaud’s phenomenon?
Biofeedback has been shown to be helpful. Biofeedback uses a machine to give you information on the temperature of your hands. A biofeedback therapist can teach you skills for increasing the blood flow and warming the temperature of your hands.
Why is my skin hard (thick and tight)?
Your body has produced extra collagen in your skin. Collagen is what makes your skin firm and also what your body makes to help heal wounds. In Scleroderma, the cells start making collagen as if there were an injury that needs to be fixed. The excess collagen gets into the dermis layer of the skin and causes it to thicken and feel tight and hard.
My fingers are tight and swollen. Will this spread elsewhere on my body?
The degree of skin involvement varies from patient to patient a great deal. Most have “limited scleroderma” with only the fingers and mild face involvement. Others have “diffuse scleroderma” with many areas of the body affected. Skin thickening usually begins on the fingers. In the diffuse form it can, over time, be on the back of the hands, the forearms, the upper arms, the face, the neck, the trunk, the legs or the feet. Similar to thickening of the skin, fibrosis or excess collagen can even occur in the internal organs.
When can I expect to get some relief from the skin thickening?
In general, without treatment, the skin thickening will reach its peak within 1-2 years after it starts and will then begin to loosen. How much it will loosen will vary from person to person.
Will there be any other visible changes in my skin?
Some areas may get darker (hyperpigmentation) while other areas may develop a patchy loss of skin color (hypopigmentation). Large patches of pigment loss can also happen. The thickened skin can look shiny and scaly from being dry. The pigment changes get better over time and the skin tone usually returns to its normal appearance after several years. You may also develop red spots caused by the widening of small blood vessels in the surface of the skin. These changes are not painful but may be a cosmetic problem.
What can I do to reduce the visibility of the changes in my skin tone?
Generally it is best not to try to change the color with drugs or creams but heavy cosmetics that cover better are the most helpful. Avoidance of sun will prevent increased pigment and stopping any scratching of the skin will reduce damage and prevent loss of pigment. Creams and lotions that moisten the skin can prevent further skin damage. Prescription creams with vitamin A are sometimes given.
Will there be other changes to my physical appearance?
The most common visible change is tight skin on the fingers causing bending of the fingertips toward the palm. Tight skin on the face may reduce the size of a person’s mouth opening, create a few small vertical lines around the lips and in some cases interfere with drawing the lips over the teeth. You may experience hair loss on the arms and legs, sweat less or develop skin sores where the skin is injured by trauma. Some patients will develop small calcium deposits in the tissues of the fingers, in the bursa (sac) over the tip of the elbows, rarely over the kneecaps or in other pressure areas. If the deposits open, they may become infected. You should contact your doctor if this happens.
Why does my skin itch?
Dry skin is caused by several things. First the extra layer of collagen in the inner layer of skin destroys normal sweat and oil glands. Second, the outer layer of the skin usually has fatty substances that moisten the skin. In scleroderma, this outer layer often gets thinner so there is less oil. Finally, in the early phase of scleroderma inflammation in the skin releases histamine and other chemicals that cause the skin to itch.
How long will the itching last?
Without treatment, itching from inflammation normally occurs in the first 6 months to 2 years of the disease and then gets better as the inflammation disappears. However, when the skin is thickened and damaged it may have the problem of itching for many years.
What can I do to prevent the itching?
- Scratchy fabrics such as wool
- Rubbing alcohol or other products containing alcohol (perfume)
- Harsh soaps, detergents and household cleaners such as: Wisk, Lestoil, Arm and Hammer, Woolite, Cheer, Tide, Liquid soaps, detergents with extra power or brighteners, store brand soaps.
- All fabric softeners’
- Hot baths
- Excess cold exposure
- Vigorous exercise
- Active or passive cigarette smoke
- Fatigue and anxiety
- All Powder, Das Powder, Ivory Snow and Safeskin
What can I do to relieve the itching?
- Turn down the heat.
- Use mild, unscented or moisturizing soaps such as: Alpha Kerry Dove, Aveeno Bar (dry skin formula), Lever 2000, Neutrogena (dry skin formula).
Do not use: Ivory, Dial, Zest, Safeguard, Jergens, Lava, Lifebuoy, Camay, Coast, Cuticura or Irish Spring soaps.
- Limit the use of soap to areas that are likely to have body odor. Rinse well.
- Use mild, non-drying shampoos such as: Neutrogena Regular, DHS Clear, Duplex or Progaine.
- Use warm, not hot water.
- Take soothing baths. Add ½ cup baby oil to your bath. Bathe with Aveeno Oatmeal, Oilated Aveeno, Cornstarch, Mineral Oil or Cottonseed Oil.
- Put moisturizers over your entire body immediately after bathing or showering. Use such products as: Eucerin Cream or Lotion, Neutroderm, Lubriderm, Complex-15, Alpha-Keri Lotion, Eutra Lotion, Neutrogena Hand Cream, Candermyl Cream, Moisture, Aquaphor Ointment, Lacticare Lotion, U-Lactin Lotion, Theraplex Lotion and Emollient.
- Moisturize often.
- Use cooling agents such as menthol or camphor (Sarna Lotions).
- Use gloves when using any substances that are rough on the hands.
- Avoid excessive cold or heat exposure.
- Do not use topical numbing agents or antihistamines since they tend to be drying.
- Avoid electric blankets except to warm the bed. Their constant heat will cause you to lose body moisture.
Why do I sometimes develop sores on my fingers, toes and elbows?
These sores are called digital ulcers and are caused when excess collagen collects on the inside of blood vessels causing them to narrow and restrict blood flow. They occur most often on the fingertips but can develop anywhere on the finger or toe when the skin in stretched too tight. Sores can also appear at pressure points, like elbows. Sores may be very painful and can make it difficult to use your hands or other parts of your body.
How can I prevent digital ulcers?
- Avoid stress and cold
- Keep as warm as possible
- Wear layers of clothing
- Wear hats in cold weather
- Wear shoes and socks that provide room for circulation
- Use Band-Aids
How do I treat digital ulcers?
- See a doctor
- Keep ulcers clean
- Use medication if infection develops