Stopping Scleroderma

Your skin appears tight. Your face is an expressionless mask. The skin on your hands is incredibly taut. You could have scleroderma.

The term “scleroderma” translates in Greek to “hard skin.” The skin can become so taut that the fingers are bent down to the palms. With this illness, collagen production speeds up. Collagen is the support tissue for skin and internal organs. The overproduced and hastily made collagen binds skin, tightens and hardens it.

Collagen proliferation can take place in the heart, lungs, kidneys and digestive tract, producing a bewildering array of symptoms. With digestive tract involvement, swallowing becomes difficult and heartburn can be a constant torture. Lung involvement can leave patients short of breath. Kidney involvement interferes with the kidneys’ job of cleansing the blood and also leads to high blood pressure.

There are two major forms of scleroderma: localized and systemic. Systemic can be broken down into two categories: diffuse and limited.

Localized scleroderma is the most common form that only affects your skin in certain places. The skin often looks waxy and streakish. This form can go away or stop progressing without treatment.

With diffuse scleroderma, the condition may impact other areas of the body. Not only does it affect the skin but it can affect internal organs as well as digestive and respiratory functions, causing kidney failure. This form can be potentially life-threatening.

Patients with limited scleroderma don’t experience any kidney problems. The skin thickening takes place on the fingers, hands and forearms, sometimes the feet and legs. Digestive involvement is confined to the esophagus.

Pulmonary hypertension can show up later as a complication and can be serious. In pulmonary hypertension, the arteries from the heart to the lungs narrow down and generate high pressure on the right side of the heart, which may lead to right-sided heart failure. Symptoms of pulmonary hypertension include shortness of breath, chest pain and fatigue.

Approximately one in 10,000 individuals is affected by systemic scleroderma. It is more common in women than men and most often develops around age 30 to 50. There are more scleroderma patients than there are patients with multiple sclerosis, muscular dystrophy or cystic fibrosis.

The cause of scleroderma is unknown. It rarely runs in families but most cases show no family history of the illness. Scleroderma is not contagious.

Some symptoms of scleroderma are:

·       Swelling of the hands and feet.

·       Red spots on the skin (telangectasias).

·       Excessive calcium deposition in the skin (calsinosis).

·       Joint contractures (rigidity).

·       Tight, mask-like facial skin.

·       Ulcerations on the fingertips and toes.

·       Pain and stiffness in the joints.

·       Persistent cough.

·       Shortness of breath.

·       Heartburn (acid reflux).

·       Difficulty swallowing.

·       Digestive and gastrointestinal problems.

·       Constipation.

·       Weight loss.

·       Fatigue.

·       Hair loss.

Scleroderma may also be associated with two other conditions, Raynaud’s phenomenon and Sjogren’s syndrome. Primary Raynaud’s phenomenon is common and occurs by itself without any underlying connective tissue disorder.

Sjogren’s syndrome is characterized by dry eyes and mouth. This dryness is derived from lack of secretion of tears and saliva from immune damage and destruction of the moisture-producing glands of the body.

The diagnosis of scleroderma isn’t always easy. Since other parts of the body may be affected—like the joints—scleroderma may be mistaken for lupus or rheumatoid arthritis.

After performing a physical, your doctor may run tests such as blood tests, pulmonary function tests, electrocardiogram, echocardiogram, gastrointestinal tests and kidney function tests.

Since there is no cure, treatment of this disease is about managing and controlling symptoms. A combination of approaches is often needed to manage and treat the disease. Treatments may include:

·       Skin treatments. Moisturizers are used to treat hardened skin and prevent the skin from drying out. Nitrates like nitroglycerin improve blood flow and allow any sores in the fingers to heal. Nitrates work by relaxing the smooth muscle which cause the arteries to dilate (widen). But nitrates may have some side effects such as blurred vision, dizziness, nausea and rapid heartbeat. So it’s important to discuss this with your physician.

·       Digestive remedies. There is a plethora of medications that may be prescribed to help with heartburn or other digestive difficulties. These include over-the-counter and prescribed antacids, proton pump inhibitors (like Prevacid, Protonix, or Nexium) and H 2 receptor blockers (Zantac or Pepcid).

·       Treatment of lung disease. The drug cyclophosphamide has been proven to be useful in treating pulmonary fibrosis in patients with scleroderma.

·       Treatment of pulmonary hypertension. The most successful treatment is continuous intravenous infusion of epoprostenol (Flolan), a prostaglandin, through a pump. Prostaglandins are hormone-like substances found in the body that help relax the smooth muscle and dilate blood vessels.

·       Joint difficulties. Anti-inflammatory drugs may be prescribed for patients with scleroderma who have joint problems. These drugs work by reducing inflammation as well as the pain and swelling. Physical therapy to prevent joints from contracting may also be helpful.

·       Raynaud’s phenomenon. Effective medications include vasodilators (calcium channel blockers), nitroglycerin patches and ointments, alpha blockers and sildenafil.

·       Sjogren’s syndrome. Dry eyes can be relieved with artificial tears and cyclosporine eye drops (Restasis). Dry mouth can be relieved by sipping liquids and chewing gum. For more severe cases of dry mouth, your doctor can prescribe drugs that stimulate the production of saliva like Evoxac or Salagen.

·       Kidney problems. Kidney problems can be treated using medications like Angiotensin Converting Enzyme (ACE) inhibitors and dialysis.

In addition, there are many lifestyle choices you can take to manage scleroderma including: exercising; avoiding lifting heavy objects or putting your joints at risk; skin protection by dressing in layers and using creams or lotions made for dry skin; improving your diet by drinking more water and avoiding foods that give you heartburn; taking care of your teeth (Sjogren syndrome increases the risk for cavities and tooth decay); and practicing stress management.

Scleroderma can be successfully managed and those that have it can lead rewarding lives. It may help to find a support group, so you learn from people who are going through a similar experience. Consult your health care provider about ways to make life easier with this illness.