Updated 08/07/2012 10:18 AM
Scleroderma damages skin, vital organs
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Scleroderma is a condition where the body produces too much collagen. It can cause blotchy red spots on the face, cause the hands to be deformed, and in some cases, affect vital organs.
"It's a disease not just of the skin, it's a disease in which there can be fibrosis internally, the heart, the lungs and any part of the GI track," explained rheumatologist Dr. Lee Shapiro.
"My hemoglobin was at very dangerous levels and after many tests it was determined I was bleeding internally from scleroderma," said Suzy Ballantyne, a scleroderma patient.
Scleroderma caused Ballantyne's intestinal tract to bleed. It caused Nancy Sokil's hands to deform and her lungs to suffer. In 20-percent of scleroderma cases, patients can develop pulmonary arterial hypertension, which is high blood pressure in the artery that supplies blood to the lungs and can be deadly.
"If the pressure becomes significantly elevated with the lung artery, on the right side of the heart can enlarge and can fail and patients can die of the disease because of that," said cardiologist Dr. J.D. Filippone.
A common symptom of scleroderma is a condition called Raynaud’s. Symptoms that may indicate the heart and or lungs are involved could be fatigue and shortness of breath.
Patients who are on medication for pulmonary arterial hypertension may be on that medication for their entire life. And if the heart is damaged, it may be permanent.
“The unfortunate things is that often patients are not diagnosed until they are very far along in their disease state and not diagnosed until they have pulmonary hypertension for a long time and at that point, the right heart can already be quite sick and the disease is difficult to treat and hard to reverse the heart disease that already exists," said Dr. Filippone.
Treatment for scleroderma comes in many forms.
The disease takes on many forms and progresses at different speeds. Some people who have scleroderma suffer blotchy patches on their skin and cause deformities like Nancy Sokil's hands.
"My knees and some of my other joints began to feel achy," said Nancy Sokil, a scleroderma patient.
The disease caused Suzy Ballantyne’s intestinal track to bleed.
'It's mostly internal digestive issues that I have," Ballantyne explained.
The more severe symptoms involve the heart, called pulmonary arterial hypertension.
"The pressure build up leading to heart dysfunction or failure would cause fatigability and shortness of breath because of the inability to pump as much body as it needs to the lungs to pick up oxygen," said cardiologist Dr. Michael Marmulstein.
There is no cure, but treatment helps the symptoms. If the heart is affected, there are eight FDA approved drugs for treatment. And for the lungs…
"There are pretty powerful drugs. Cytoxin…also used for chemotherapy and for a variety of cancers and the other called CellCept, which is commonly used to prevent rejection of kidney transplant and both of those medications are used early have a very high success rate of at least arresting the disease if not improving the lung function," said rheumatologist Dr. Lee Shapiro.
In addition to medication, keeping your heart and lungs in good shape is critical.
"I think that maintaining normal body weight, maintaining muscular skeletal fitness through appropriate exercise through a physical guided program is very helpful good for all people with all diseases, including scleroderma," said Dr. Marmulstein.