Patty Nixon, who worked as a registered nurse at Holy Family Hospital, of Spokane, for 35 years, loved to work with her hands.


Those hands once played the violin in the Spokane Symphony, plucked harp strings, planted vegetables in her garden, cradled her grandchildren, and comforted critically-ill patients.


Late last year, when Nixons nimble fingers became stiff and swollen, she didnt know they were showing the first signs of a diseasecalled sclerodermathat would take her life just two months later.


An estimated 300,000 Americans suffer from scleroderma, a rare autoimmune disease that affects roughly three times more women than men, says the Danvers, Mass.-based Scleroderma Foundations Web site. In an autoimmune disease, the immune system attacks or destroys the bodys own tissue.


There are about 1,650 diagnosed cases of scleroderma in Washington state, and about 150 new cases are diagnosed here each year, says foundation spokesman Chris Underation.


Scleroderma, which literally means, hard skin, is an arthritic condition that leads to hardening and tightening of the skin and connective tissues, and in some cases affects the blood vessels and internal organs, says the Rochester, Minn.-based Mayo Clinics Web site. It results from an overproduction and accumulation in body tissues of a protein called collagen, but researchers dont know what prompts that abnormal production, and they dont know how to stop or reverse it.


The life expectancy for patients who have been diagnosed with scleroderma can range from a few months to more than 20 years. For Nixon, who died on Feb. 1, just before her 59th birthday, it turned out to be about 50 days.


This disease progressed more rapidly than the doctors could imagine, says her husband, Dan Nixon, who is a nurse and case manager at Holy Family. I kind of knew I wasnt going to have her for a long time, but I thought Id have her for a lot longer than I did.


Nixons symptoms started subtly. Last November, her stomach started bothering her more than usual, and her acid reflux condition grew worse, causing her to lose weight, Dan Nixon says. She also started feeling stiffer when she got up in the morning to exercise at a local gym before work. Her hands became weaker, to the point she couldnt open jar lids, he says. Nixon thought, though, that those were just typical signs of aging and side effects of her hypothyroidism, or deficient activity of the thyroid gland, which was diagnosed about six months prior.


In December, Nixon became short of breath, so her husband took her to the hospital, where doctors discovered she had pneumonia in both her lungs, he says. Fluid also was building up in her lungs because her esophagus had become paralyzed and wasnt keeping down fluid. It was then that Nixons primary-care physician, Dr. Kim Badger, diagnosed her with scleroderma, he says.


In January, Nixon began to experience more joint and muscle pain and was increasingly short of breath, he says. She was being fed intravenously, since she couldnt ingest food, and was breathing with the assistance of an oxygen machine. Her white blood cell count continued to climb, and doctors couldnt figure out why. Yet, the Nixons tried to maintain a positive attitude, he says.


We dealt with things as they came up, and said, Were going to fix this, Dan Nixon says.


Nixons skin became increasingly dark and stiff. It was so tough that hospital workers couldnt get a tube into her stomach to drain it, and couldnt puncture the skin on her arm with a needle to insert an artery line for a heart catheter, he says.


By the end of January, Dan Nixon was lifting his wife in and out of bed and pushing her around the house in her wheelchair. He says his occupation at Holy Family took a backseat to his role as husband and caregiver. He watched as the disease attacked Pattys skin, lungs, and esophagus, but focused more on meeting day-to-day challenges rather than looking at the larger picture.


I just kept telling myself that we could beat this, he says. I guess I would have done anything to keep her, but I dont think she would have wanted to be stuck in a wheelchair and in pain.


Dan Nixon says he learned about a group of physicians at Virginia Mason Medical Center, in Seattle, who conduct research on the disease, and talked with those physicians about airlifting her there.


We hoped there was a magic bullet, Nixon says. We were just hoping they could come up with something, anything to help.


On the night of Jan. 31, a few days before her scheduled appointment, Nixon became extremely short of breath. As her husband drove her to the hospital, he could hear fluid in her lungs, and in retrospect, he wonders if she was undergoing congestive heart failure.


Nixon died the next day at the hospital where she had worked for more than three decades. Dan Nixon says he thinks the disease probably had attacked her heart, causing the tissues to become too fibrous to function.


Nixon says he wants others to learn about scleroderma.


The more everybody knows, if nothing else, the quicker theyll go to see the doctor, he says.


There are two types of scleroderma. Localized scleroderma only affects the skin and the deep tissues below the skin, while systemic scleroderma affects the skin as well as blood vessels and major organs, the Mayo Clinic Web site says. Symptoms of the disease include stiffness and pain in joints and curling of fingers; sores over joints; numbness or color changes in fingers, toes, nose, and ears, puffy hands and feet; and digestive problems. Complications can arise with the gastrointestinal system, the lungs, kidneys, and heart as scarring of tissues in those organs occurs.


Scleroderma is one of a number of so-called connective tissue diseases. It affects more women than men because it involves estrogen and its effects on the immune system, says Dr. Jeffrey Carlin, a rheumatologist at Virginia Mason, which he claims has one of the largest referral bases for scleroderma patients in the country. Most connective tissue diseases, such as rheumatoid arthritis, are more common among women in their child-bearing years and during menopause, when estrogen levels are fluctuating, he says.


Researchers also are examining whether pregnancy might play a role in scleroderma, Carlin says. If a person undergoes a bone transplant and the body rejects the bone graft, inflammation occurs that looks similar to scleroderma, he says. Researchers are developing a hypothesis that during pregnancy, if a womens body begins to reject the fetal cells that are entering her bloodstream, scleroderma might begin to develop.


Although researchers are exploring various theories, little is known about sclerodermas origins, or about how to treat it, Carlin says.


We use a lot of medications, but we dont know what triggers it, he says. The most frustrating thing is that treatment options arent very good.


While theres not a specific treatment for scleroderma, and theres no cure, some patients have done very well with certain types of treatments, says Dr. Jerry Molitor, a rheumatologist at Virginia Mason. For the most seriously ill patients, stem cell transplants and chemotherapy drugs have proven somewhat successful, although there are significant risks involved with them, he says. Less aggressive, but also less effective, medicines, such as immunosuppressants commonly used for arthritis and Lupus, also have produced positive results. Most treatments dont counter the disease as a whole, but they do address certain symptoms of it, he says.


I dont like to hear when patients have been told theres nothing that can be done, because that is not true, he says. Its worth seeing a physician that has experience with this and can discuss different treatment options.


As more research is done and awareness is raised about the disease, doctors are getting better at diagnosing it earlier, Carlin says. He says that even though theres no cure, its important to catch sclerdoderma early, so that related complications such as kidney disease can be treated before they become life-threatening.


Unfortunately in Patty Nixons case, her scleroderma progressed too rapidly for anything to be done, Dan Nixon says as he walks through the couples house and points out different home improvement projects she had been working on before she got sick. Nixon had planned to learn how to knit, and had bought knitting materials about a month before she died. A ball of yarn now sits unused on a table with her quilting supplies. Pictures hanging on the walls track the progression of her disease, showing how her face began looking more drawn as her skin tightened.


Also hanging on the walls are various awards Nixon won for her work at the hospital, including Employee of the Year for Competence in 2000. In addition to her job as a nurse, Nixon earned a masters degree in counseling at Whitworth College in the early 1990s, and counseled youth at the YMCA here, as well as families who otherwise couldnt afford such services.


In honor of her service to Holy Family Hospital and the surrounding community, the hospital has renamed its annual Nursing Excellence Award the Patricia Nixon Nursing Excellence Award. Dan Nixon and others have donated money to the hospitals employee benevolence fund in her honor.


I want Patty to be remembered, he says. If people learn about this disease, and about Patty, it means shes not forgotten, and she was too special a person to forget.


Contact Emily Brandler at (509) 344-1265 or via e-mail at emilyb@spokanejournal.com.