Scleroderma: Support, Inform, Advocate, Educate
I’ve been off the blog for a few weeks trying to get my own body issues under control, so I can keep on living, and frantically typing and writing. That’s the goal, anyway. I’ve been riddled with auto immune disease for years now, and finally have begun a course of action to take back control of my scar tissue covered lungs, and keep some function in my body. One thing that I’ve learned is that no one knows ANYthing about Scleroderma!! And even after I’ve explained, they’re still looking at me with confusion, curiosity, and wonder. So, this blog post will have lots of information about scleroderma in it. An important thing to remember–auto immune diseases are different for everyone, and no one’s symptoms are exactly the same. And they can affect ANYone, of ANY age, from infancy to elders.
My issues: Scleroderma, with underlying SLE Lupus, Raynaud’s Phenomenon, Thyroid issues (fluctuating)…and a few other, special extras. My scleroderma affects my Cardio-Pulmonary System, Gastrointestinal System, and Respiratory System. So far, my respiratory system has had the most damage…and that’s what I’m on the *strong* meds for. My biological mother, and the females in her family all also seem to have different types of auto immune issues. Thusly making me think that there is a certain amount of heredity, and a predisposition to certain autoimmune diseases. Anyway, time for the facts!~
Scleroderma (skleer-oh-DUR-muh) is a group of rare, progressive diseases that involve the hardening and tightening of the skin and connective tissues — the fibers that provide the framework and support for your body.
Localized scleroderma affects only the skin. Systemic scleroderma also harms internal organs, such as the heart, lungs, kidneys and digestive tract.
Scleroderma can happen to anyone at any time in any geographical area. However, the disease affects women more often than men and most commonly occurs between the ages of 30 and 50. Scleroderma can run in families, but in most cases it occurs without any known family tendency for the disease. Scleroderma isn’t considered contagious, but it can greatly affect self-esteem and the ability to accomplish everyday tasks.
What scleroderma is not:
Scleroderma is not contagious, infectious, cancerous or malignant.
How serious is scleroderma?
Any chronic disease can be serious. The symptoms of scleroderma vary greatly for each person, and the effects of scleroderma can range from very mild to life threatening. The seriousness will depend on the parts of the body, which are affected, and the extent to which they are affected. A mild case can become more serious if not properly treated. Prompt and proper diagnosis and treatment by qualified physicians may minimize the symptoms of scleroderma and lessen the chance for irreversible damage.
Scleroderma symptoms vary, depending on which organ systems are involved. Diagnosis can be difficult because some of the early symptoms are common in the general population and aren’t always associated with scleroderma. The most prevalent signs and symptoms of scleroderma include:
Raynaud’s phenomenon. An exaggerated response to cold temperatures or emotional distress, this condition constricts the small blood vessels in the hands and feet and causes numbness, pain or color changes in the fingers or toes.
Gastroesophageal reflux disease (GERD). In addition to acid reflux, which can damage the section of esophagus nearest your stomach, you may also have problems absorbing nutrients if your intestinal muscles aren’t moving food properly through your intestines.
Skin changes. These changes may include swollen fingers and hands; thickened patches of skin, particularly on the fingers; and tight skin around the hands, face or mouth. Skin can appear shiny because it’s so tight, and movement of the affected area may be restricted.
The two main varieties of localized scleroderma, which affects only the skin, are distinguished by very distinctive signs and symptoms:
Morphea. This variety features oval-shaped thickened patches of skin that are white in the middle, with a purple border. Skin patches could appear in only a few spots on your body or be more widespread, appearing all over your body. Morphea generally fades out after three to five years, but you may retain areas of darkened skin where the areas of thickened skin appeared.
Linear scleroderma. This variety, which is more common in children, features bands or streaks of hardened skin on one or both arms or legs, or on the forehead. Linear scleroderma often appears mostly on one side of the body.
With localized scleroderma, the first signs of disease may be the presence of Raynaud’s phenomenon and may develop several years before you notice any other symptoms.
This type of scleroderma, also called systemic sclerosis, affects not only your skin but also your blood vessels and internal organs. Subcategories are defined by what portions of your body are affected. One variation is known as limited scleroderma, or CREST syndrome.
With systemic scleroderma, skin changes may occur suddenly and progressively worsen during the first one to two years of the disease. After that, changes level off or subside, and sometimes even resolve on their own without treatment.
Scleroderma results from an overproduction and accumulation of collagen in body tissues. Collagen is a fibrous type of protein that makes up your body’s connective tissues, including your skin.
Although doctors aren’t sure what prompts this abnormal collagen production, the body’s immune system appears to play a role. For unknown reasons, the immune system turns against the body, producing inflammation and the overproduction of collagen.
*Risk Factors…..(although these can mean a lot, or a little with auto immune diseases)
Several factors appear to increase the risk of certain types of scleroderma:
Race and ethnicity
Certain groups of people are more likely to develop scleroderma than are others:
Native Americans. Choctaw Native Americans in Oklahoma are at least 20 times as likely as is the general population to develop systemic scleroderma. Oddly enough, this increased risk doesn’t apply to Choctaws living in Mississippi.
African-Americans. Systemic scleroderma is more common in African-Americans than it is in Americans of European descent. And the African-Americans who have systemic scleroderma are more likely to develop severe lung complications.
-Silica dust, which is common in coal mines and rock quarries
-Some industrial solvents, such as paint thinners
-Certain chemotherapy drugs
Who develops scleroderma, and when?
It’s estimated that about 300,000 Americans have scleroderma. About one third of those people have the systemic form of scleroderma. Since scleroderma presents with symptoms similar to other autoimmune diseases, diagnosis is difficult. There may be many misdiagnosed or undiagnosed cases.
Localized scleroderma is more common in children, whereas systemic scleroderma is more common in adults. Overall, female patients outnumber male patients about 4-to-1. Factors other than a person’s gender, such as race and ethnic background, may influence the risk of getting scleroderma, the age of onset, and the pattern or severity of internal organ involvement. The reasons for this are not clear. Although scleroderma is not directly inherited, some scientists feel there is a slight predisposition to it in families with a history of rheumatic diseases.
However, scleroderma can develop in every age group from infants to the elderly, but its onset is most frequent between the ages of 25 to 55. When doctors say “usually” or “for the most part,” the reader should understand that variations frequently occur. Many patients get alarmed when they read medical information that seems to contradict their own experiences, and conclude that what has happened to them is not supposed to happen. There are many exceptions to the rules in scleroderma, perhaps more so than in other diseases. Each case is different, and information should be discussed with your own doctor.
*Complications…. (and this is a small list)
Scleroderma complications range from mild to severe. Some may even become life-threatening.
The variety of Raynaud’s phenomenon that occurs with scleroderma can be so severe that the restricted blood flow permanently damages the tissue at the fingertips, causing pits or skin sores (ulcers). In some cases, gangrene and amputation may follow.
Scarring of lung tissue (pulmonary fibrosis) can result in reduced lung function, reduced ability to breathe and reduced tolerance for exercise. You may also develop high blood pressure in the arteries to your lungs (pulmonary hypertension).
When scleroderma affects your kidneys, you can develop an elevated blood pressure and an increased level of protein in your urine. More serious effects of kidney complications may include renal crisis, which involves a sudden increase in blood pressure and rapid kidney failure.
Scarring of heart tissue increases your risk of abnormal heartbeats (arrhythmias) and congestive heart failure, and can cause inflammation of the membranous sac surrounding your heart (pericarditis).
Severe tightening of facial skin can cause your mouth to become smaller and narrower, which may make it hard to brush your teeth or to even have them professionally cleaned. People who have scleroderma often don’t produce normal amounts of saliva, so the risk of decay increases even more. In addition, acid reflux can destroy tooth enamel, and changes in gum tissue may cause your teeth to become loose or even fall out.
Scleroderma can affect your upper and lower digestive tracts. Digestive problems associated with scleroderma can lead to acid reflux and difficulty swallowing — some describe feeling as if food gets stuck midway down the esophagus — as well as bouts of constipation alternating with episodes of diarrhea.
Men who have scleroderma often experience erectile dysfunction. Scleroderma may also affect the sexual function of women, by decreasing sexual lubrication and constricting the vaginal opening.
*Tests & Diagnostic
Your doctor may conduct the following tests:
Blood tests. People with scleroderma usually have elevated blood levels of certain antibodies produced by the immune system.
Tissue sample. Your doctor may remove a small tissue sample (biopsy) of your affected skin to be examined in the laboratory for abnormalities.
Skin assessment. Your doctor ideally evaluates the condition of your skin in 17 specific areas to determine the level of skin involvement, which provides a clue to the likelihood that you also have life-threatening changes happening to internal organs.
Based on the outcome of your initial evaluation, your doctor may also recommend other diagnostic tests to identify any lung, heart, kidney or gastrointestinal complications accompanying scleroderma.
*Types of Scleroderma
The changes, which occur in localized scleroderma, are usually found in only a few places on the skin or muscles, and rarely spread elsewhere. Generally, localized scleroderma is relatively mild. The internal organs are usually not affected, and persons with localized scleroderma rarely develop systemic scleroderma. Some laboratory abnormalities commonly seen in systemic scleroderma are frequently absent in the localized form.
Morphea is a form of localized scleroderma characterized by waxy patches on the skin of varying sizes, shapes and color. The skin under the patches may thicken. The patches may enlarge or shrink, and often may disappear spontaneously. Morphea usually appears between the ages of 20 and 50, but is often seen in young children.
Linear scleroderma is a form of localized scleroderma which frequently starts as a streak or line of hardened, waxy skin on an arm or leg or on the forehead. Sometimes it forms a long crease on the head or neck, referred to as en coup de sabre because it resembles a saber or sword wound. Linear scleroderma tends to involve deeper layers of the skin as well as the surface layers, and sometimes affects the motion of the joints, which lie underneath. Linear scleroderma usually develops in childhood. In children, the growth of involved limbs may be affected.
Systemic scleroderma (systemic sclerosis)
The changes occurring in systemic scleroderma may affect the connective tissue in many parts of the body. Systemic scleroderma can involve the skin, esophagus, gastrointestinal tract (stomach and bowels), lungs, kidneys, heart and other internal organs. It can also affect blood vessels, muscles and joints. The tissues of involved organs become hard and fibrous, causing them to function less efficiently. The term systemic sclerosis indicates that “sclerosis” (hardening) may occur in the internal systems of the body. There are two major recognized patterns that the illness can take – diffuse or limited disease. In diffuse scleroderma, skin thickening occurs more rapidly and involves more skin areas than in limited disease. In addition, people with diffuse scleroderma have a higher risk of developing “sclerosis” or fibrous hardening of the internal organs.
About 50 percent of patients have a slower and more benign illness called limited scleroderma. In limited scleroderma, skin thickening is less widespread, typically confined to the fingers, hands and face, and develops slowly over years. Although internal problems occur, they are less frequent and tend to be less severe than in diffuse scleroderma, and are usually delayed in onset for several years. However, persons with limited scleroderma, and occasionally those with diffuse scleroderma,
can develop pulmonary hypertension, a condition in which the lung’s blood vessels become narrow, leading to impaired blood flow through the lungs resulting in shortness of breath.
Limited scleroderma is sometimes called CREST syndrome. CREST stands for the initial letters of five common features:
To further complicate the terminology, some people with diffuse disease will go on to develop calcinosis and telangiectasias so that they also have the features of CREST.
Although most patients can be classified as having diffuse or limited disease, different people may have different symptoms and different combination of symptoms of the illness.
*Treatments & Meds
Scleroderma has no known cure — no drug will stop the overproduction of collagen. But the localized variety of scleroderma sometimes resolves on its own. And a variety of medications can help control the symptoms of scleroderma or help prevent complications.
Dilating the blood vessels. Blood pressure medications that dilate blood vessels may help prevent lung and kidney problems and may help treat Raynaud’s disease.
Suppressing the immune system. Drugs that suppress the immune system, such as those taken after organ transplants, may help reduce scleroderma symptoms.
Physical or occupational therapy. Therapists can help you to manage pain, improve your strength and mobility, and work on performing essential daily tasks to maintain your independence.
Cosmetic procedures. The appearance of skin lesions associated with scleroderma may be helped by exposure to ultraviolet light. Laser surgery also may help camouflage or eliminate these lesions.
Amputation. If finger ulcers caused by severe Raynaud’s disease have developed gangrene, amputation may be necessary.
Lung transplants. People who have developed high blood pressure in the arteries to their lungs (pulmonary hypertension) may be candidates for lung transplants.
*Alternative Medications or Therapies
None known. Meditation has been noted to help with symptoms.
*Coping & Support
As is true with other chronic diseases, living with scleroderma can place you on a roller coaster of emotions. Here are some suggestions to help you even out the ups and downs:
Maintain normal daily activities as best you can.
Pace yourself and be sure to get the rest that you need.
Stay connected with friends and family.
Continue to pursue hobbies that you enjoy and are able to do.
If scleroderma makes it difficult for you to do things you enjoy, ask your doctor about ways to get around the obstacles.
Keep in mind that your physical health can have a direct impact on your mental health. Denial, anger and frustration are common with chronic illnesses.
At times, you may need additional tools to deal with your emotions. Professionals, such as therapists or behavior psychologists, may be able to help you put things in perspective. They can also help you develop coping skills, including relaxation techniques.
Joining a support group, where you can share experiences and feelings with other people, is often a good approach. Ask your doctor what support groups are available in your community.
www.scleroderma.org check it out, and donate to the cause, today!!!