aggressive-treatment-scleroderma-03-afMichael George feels so good he can pinch himself after curing the dangerous scleroderma disease with adult stem cells.

And today, after undergoing an autologous adult stem-cell transplant 14 months ago to stop his advanced case of scleroderma, an incurable autoimmune disease that causes excess collagen to make skin tighten and feel hard and thick, George is able to pinch his pliable skin. He’s also able to open his mouth real wide.

“I feel really good,” said George, a retired William C. McGinnis School principal, who in April was taken off all medications, including antibiotics, related to his transplant.

“I feel I was reborn,” George said. “It’s great to be alive.”

It was at his one-year check-up in April doctors told George, an avid fisherman, that he can now do anything he wants.

What is Scleroderma?

Scleroderma is an autoimmune disease of the connective tissue. Autoimmune diseases are illnesses which occur when the body’s tissues are attacked by its own immune system. Scleroderma is characterized by the formation of scar tissue (fibrosis) in the skin and organs of the body. This leads to thickness and firmness of involved areas. Scleroderma, when it’s diffuse or widespread over the body, is also referred to as systemic sclerosis.

The cause of scleroderma is not known. Researchers have found some evidence that genes are important factors, but the environment seems to also play a role. The result is activation of the immune system, causing injury to tissues that result in injury similar to scar tissue formation. The fact that genes seem to cause a predisposition to developing scleroderma means that inheritance at least plays a partial role. It is not unusual to find other autoimmune diseases in families of scleroderma patients. Some evidence for the role genes may play in leading to the development of scleroderma comes from the study of Choctaw Native Americans who are the group with the highest reported prevalence of the disease. The disease is more frequent in females than in males.

What are the symptoms of Scleroderma?

The symptoms of scleroderma depend on the type of scleroderma present and the extent of external and internal involvement in the individual affected. Because scleroderma can affect the skin, esophagus, blood vessels, kidneys, lungs, blood pressure and bowels, the symptoms it causes can involve many areas of the body.

Scleroderma affects the skin to cause local or widespread signs of inflammation (redness, swelling, tenderness, itching, and pain) that can lead to skin tightness or hardening. These skin changes can be widespread, but it’s most common for them to affect the fingers, feet, face, and neck. This can lead to decreased range of motion of the fingers, toes, and jaw. Tiny areas of calcification (calcinosis), while not common, can sometimes be noticed as hard nodules at the tips of the elbows or in the fingers.

Scleroderma affecting the esophagus leads to heartburn. This is directly a result of stomach acid flowing back up into the esophagus. Sometimes this can lead to scarring of the esophagus with difficulty swallowing and/or localized pain in the central chest.

Blood vessels that can be affected include the tiny arterioles of the finger tips, toes, and elsewhere. These vessels can have a tendency to spasm when the areas are exposed to cold, leading to blueness, whiteness, and redness of involved fingers, toes, and sometimes nose or ears. These color changes are referred to as Raynaud’s phenomenon. Raynaud’s phenomenon can cause inadequate supply of oxygen to the involve tips of fingers or toes, causing tiny ulcers or blackened (dead) skin. Sometimes Raynaud’s phenomenon is also associated with tingling. Other blood vessels that can be involved in scleroderma are the tiny capillaries of the face, lips, mouth, or fingers. These capillaries widen (dilate) forming tiny, red blanching spots, called telangiectasias.

Elevated blood pressure is potentially serious and can lead to kidney damage. Symptoms include headache, fatigue, and in severe cases, stroke.

Inflammation of the lungs in scleroderma can cause scarring, resulting in shortness of breath, especially with physical exertion. Elevated pressure in the arteries to the lungs (pulmonary hypertension) can also cause shortness of breath and difficulty getting an adequate breath with activity.

Scleroderma affecting the large bowel (colon) most often causes constipation but can also lead to cramping and diarrhea. When this is severe, it complete stool blockage (fecal impaction) can result.

How Scleroderma diagnosed?

The diagnosis of the scleroderma syndrome is based on the finding of the clinical features of the illnesses. Nearly all patients with scleroderma have blood tests which suggest autoimmunity, antinuclear antibodies (ANAs). A particular antibody, the anticentromere antibody, is found almost exclusively in the limited, or CREST, form of scleroderma. Anti-Scl 70 antibody (antitopoisomerase I antibody) is most often seen in patients with the diffuse form of scleroderma.

adultstemcellsOther tests are used to evaluate the presence or extent of any internal disease. These may include upper and lower gastrointestinal tests to evaluate the bowels, chest x-rays, lung function testing, and CAT scanning to examine the lungs, EKG and echocardiograms, and sometimes heart catheterization to evaluate the pressure in the arteries of the heart and lungs.

George recently returned from a five-day trip to Martha’s Vineyard, where he went fishing with friends. And soon George will travel to Spain with his wife, Alice, brother and sister and their families during which he will celebrate his 40th wedding anniversary.

“We’re looking forward to it,” he said.

And he’s spending lots of time this summer on his 22-foot fishing boat, which he affectionately refers to as “my baby” and along the city’s marina.

“This is my passion,” said George while sitting along the marina. “This is my kind of weather.”

Just before the March 13, 2008, adult stem-cell transplant, George’s skin was stiff and his face was tight. He had a hard time swallowing. Doctors were concerned his condition was spreading internally to the point it might close his esophagus and damage his heart and lungs.

The procedure George underwent at Northwestern Memorial Hospital in Chicago involved taking stem cells from his bone marrow, cleaning and freezing them while he received large doses of chemotherapy to cleanse his immune system before the stem cells were put back into his body to grow.

He said a stainless steel vat with a long cylinder contained the stem cells that were put into a syringe and his body.

He was asked to identify any distinct smells.

“It smelled like cream corn. It filled the whole hall,” George said. “They couldn’t explain it. It was so strange.”

George said when he returned to the hospital for visits he knew if he smelled cream corn that someone had undergone a stem-cell transplant.

“I eat a lot of corn now,” he joked.

George stayed in the hospital for nearly three weeks and remained in Chicago for about three months staying in a hotel affiliated with the hospital which offered apartment-like accommodations for transplant patients.

The night he returned to Perth Amboy, George remembers being able to bend down to pick up the luggage from the trunk of the cab. These days his knees are giving him some trouble when he bends due to arthritis.

George said the procedure didn’t cure him, but it stopped the disease and reverses it to a certain extent.

“In April the doctor said “are you sure I didn’t give you a lung transplant, instead of a stem cell transplant,” he said, noting both his lungs and heart are now in good condition.

Following the transplant, George said his body has a low tolerance for cold weather and he has to really bundle up. He may spend part of the upcoming winter visiting a friend in California and his son, who lives on the island of St. Vincent.

George said he really appreciates life and everyone at his church, the school district and city who wrote or e-mailed him and contributed toward his medical expenses, which were more than $200,000.

He now wants to pay it forward by helping others facing adult stem-cell transplants.

“I’m like an advocate,” said George, who sees his role as offering a helping hand, just as others who had been through the procedure had helped him. “We advocate among each other.

It helps them, and it helps me too. All my life I wanted to help people. Helping kids was my forte. Now to help someone in need who doesn’t know what to expect, it raises it to a whole different level.”

Source: MyCentralJersey. com