What is Scleroderma?

By now, you're probably wondering just what exactly is Scleroderma. Well, in simple terms (and in a cute typo-graphic)

 

So, basically, my body has decided that it doesn't like the healthy body tissues in my body, and tries to get rid of them. So much for trying to get in shape and be healthy right? In more in depth terms, this is what eMedTV says about it:

Scleroderma is derived from the Greek words "sklerosis," meaning hardness, and "derma," meaning skin. Therefore, it literally means "hard skin." Although it is often referred to as a single disease, it is really a symptom of a group of diseases. This group involves the abnormal growth of connective tissue, which supports the skin and internal organs. Therefore, scleroderma is sometimes used as an umbrella term for these disorders.

In some forms of scleroderma, hard, tight skin is the extent of this abnormal process. However, in other forms, the problem goes much deeper, affecting blood vessels and internal organs, such as the heart, lungs, and kidneys.

Scleroderma is considered a rheumatic disease and a connective tissue disease. A rheumatic disease refers to a group of conditions characterized by inflammation and/or pain in the muscles, joints, or fibrous tissue. A connective tissue disease is one that affects the major substances in the skin, tendons, and bones.

Here is a list of symptoms of scleroderma with the ones I've experienced in bold.

Skin symptoms of scleroderma may include:

• Fingers or toes that turn blue or white in response to hot and cold temperatures (Raynaud's phenomenon)
• Hair loss
• Skin hardness and thickening
• Skin that is darker or lighter than normal
• Stiffness, and tightness skin of fingers, hands, and forearm
• Small white lumps beneath the skin that sometimes ooze a white substance that looks like toothpaste
• Sores (ulcers) on the fingertips or toes
• Tight and mask-like skin on the face

Bone and muscle symptoms may include:

• Joint pain
• Numbness and pain in the feet
• Pain, stiffness, and swelling of fingers and joints
• Wrist pain

Breathing problems may result from scarring in the lungs and can include:

• Dry cough
• Shortness of breath
• Wheezing

Digestive tract problems may include:

• Bloating after meals
• Constipation
• Diarrhea
• Difficulty swallowing
• Esophageal reflux or heartburn
• Problems controlling stools

Awesome right? Okay, so it's broken into two main forms of Scleroderma, {localized} & {systemic}. Here's the difference between the two. (click on image to see it bigger).

Got that? So naturally I have the worse of the two. Systemic. A lot of the symptoms for both are the same, however systemic covers more of the body. Some of those wonderful symptoms are known as CREST.

• Calcinosis. The formation of calcium deposits in the connective tissues, which can be detected by an x-ray. They are typically found on the fingers, hands, face, and trunk and on the skin above the elbows and knees. Painful ulcers may appear if the deposits break through the skin.

• Raynaud's phenomenon. A condition in which the small blood vessels of the hands and/or feet contract in response to cold or anxiety. As the vessels contract, the hands or feet turn white and cold, then blue. As blood flow returns, they become red. Fingertip tissues may suffer damage, leading to ulcers, scars, or gangrene.

• Esophageal dysfunction. Impaired function of the esophagus (the tube connecting the throat and the stomach) that occurs when the smooth muscles in the esophagus lose normal movement. In the upper esophagus, the result can be swallowing difficulties; in the lower esophagus, the problem can cause chronic heartburn or inflammation.

• Sclerodactyly. Thick and tight skin on the fingers, resulting from deposits of excess collagen within skin layers. This condition makes it harder to bend or straighten the fingers, and may also cause skin to appear shiny and darkened, with hair loss.

• Telangiectasias. Small red spots on the hands and face that are caused by the swelling of tiny blood vessels. While these spots are not painful, they can create cosmetic problems.

  The main ones I suffer from are the Raynaud's, Esophageal dysfunction, and sclerodactyly. Love it!

Now, as mentioned above, Systemic scleroderma is broken down into Limited or Diffuse.

Whereas Diffuse is a bit more complicated.

Guess which one I've got. (And no cheating, since I told you in my last post.) But yes, I've got Diffuse Systemic Scleroderma, due to it affecting so much of my body, as well as having all of those awesome symptoms listed.

The damage caused by diffuse scleroderma typically occurs over a few years. After the first three to five years, people with diffuse scleroderma often enter a stable phase lasting for varying lengths of time. During this phase, skin thickness and appearance stay about the same and damage to internal organs progresses little, if at all. Symptoms of scleroderma also subside, which means that joint pain eases, fatigue lessens, and appetite returns.

The skin will gradually begin to change. Less collagen will be made, and the body will get rid of excess collagen. This process, called "softening," tends to occur in reverse order of the thickening process: the last areas thickened are the first to begin softening. For some people, skin returns to normal, while other people are left with thin, fragile skin without hair or sweat glands. More serious damage to the heart, lungs, or kidneys is unlikely to occur, unless previous damage leads to more advanced deterioration.

People with diffuse scleroderma face the most serious long-term outlook if they develop severe kidney, lung, digestive, or heart problems. Fortunately, less than one-third of patients with diffuse scleroderma develop these problems. Early diagnosis and continual and careful monitoring are important factors in this.

So, I guess there is hope that maybe this disease with become stable. It progressed really rapidly from about June-September, and since being on the medication, it has slowed down. Because of the kind I have, they have to monitor my internal organs pretty regularly to make sure nothing goes wrong there.

There is not a known cause for what brings this disease on. Scientists suspect that it comes from several factors, which may include:

• Abnormal immune or inflammatory activity
• Genetics
• Environment (I blame it on work! haha!)
• Hormones

There is also no known cure for it, though there are methods to prevent the disease from progressing.

And last but not least, the prognosis.

Individuals with morphea or limited scleroderma have a relatively positive outlook. Those with very widespread skin and organ involvement (systemic) have a negative prognosis. More women have scleroderma, but the disease kills more men. Following diagnosis, two-thirds of patients live at least 11 years. The higher the patient's age at diagnosis, the more likely he or she is to die from the disease.

People with scleroderma have very different life expectancies. Some — for example, those with limited or mild diffuse disease — can expect to live 20 to 50 years after diagnosis. Others with severe, rapidly progressive disease — a group which makes up less than 10% of the total number of patients with diffuse scleroderma — might have a 50% chance of a five-year survival.

Encouraging right? So, I'll either live 5-50 years. But it's okay. It's really hard to know with these types of diseases, because they affect everyone so differently. Let's just hope I'm on the longer living end of things.

Well, I think that about sums it up. If you have any questions, feel free to ask. I'm open to answering them the best that I know how.

Info gathered are from these two websites:

• eMedTV
• PubMed Health