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Bouncing back

Halfway man participated in scleroderma research study

Halfway man participated in scleroderma research study

November 06, 2006|by JULIE E. GREENE

HALFWAY - In July 2003, Joe Dill thought he'd be dead within a year.

He prepaid for his funeral package, wrote his obituary and organized his belongings.

Dill had been diagnosed in March 2003 with diffuse scleroderma - an autoimmune disease with no known cause or cure - and was about to embark as a test subject for a stem cell transplant with chemotherapy treatment. He didn't know whether it would help him or kill him.

Three years later, Dill, 62, is still living with the effects of scleroderma, but he no longer has any diseased scleroderma cells and is living an active life in Halfway, volunteering for Big Brothers Big Sisters, swimming, fishing and visiting friends and family.

Scleroderma affects the skin and other organs, including joints, muscles and some internal organs such as the heart, lungs, kidneys and intestinal tract. Symptoms can include tightening of the skin, ulcers on the fingers, joint pain, stiffness, deformity, and hardening of organs leading to their dysfunction, says Dr. Thomas Medsger Jr., director of the Scleroderma Research Program in Pittsburgh.

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Scleroderma is uncommon, with about 300,000 people in the U.S. estimated to have the disease, according to the Scleroderma Foundation. This includes 80,000 to 100,000 people with the diffuse, or systemic, form, which can affect the whole body, as opposed to the local form.

Like most other immune system diseases, scleroderma tends to occur more often in women than men, Medsger says.

Before the transplant, tightness in Dill's hands prevented him from turning his car key to start the engine, so he would use a homemade device to help him. Now he still has some pain in his hands, but no longer needs to use the device.

Dill describes the stiffness and soreness he felt from scleroderma as 10 times what many people experience when they first wake up in the morning.

Stem cell transplant helped

The stem cell transplant research study Dill participated in through the University of Pittsburgh School of Medicine, where Medsger is a professor in the Division of Rheumatology, greatly relieved his pain and symptoms, though Dill faced some problems afterward.

Dill says he underwent the research study in hopes it would help other scleroderma patients and was disappointed when the study was canceled in May 2005.

Worldwide, more than 200 stem-cell transplants have been done in research studies for scleroderma patients with two-thirds of patients showing improvement in their skin thickening and no progression in other aspects of the disease, Medsger says.

The small pilot study Dill participated in ran out of funding, Medsger says.

However, the division is participating in a larger clinical trial that treats qualified scleroderma patients with either a stem cell transplant similar to Dill's or monthly treatments of high-dose intravenous Cytoxan for a year to study the ability of both to control the disease, Medsger says.

Transplant is successful

In the National Institutes of Health study Dill participated in, he was given a chemotherapy treatment with Cytoxan to help the stem cells in his bone marrow exit into the bloodstream so they could be harvested through a chest tube.

A higher-dose of Cytoxan chemotherapy treatment was given to each patient to eliminate the abnormal immune system, Medsger says. In all, Dill received six chemotherapy treatments. His stem cells - harvested by drawing blood - were purified using a mesh that held proteins that bonded to unwanted cells, leaving healthy stem cells.

Two days after his last chemotherapy treatment, Dill's cleaned stem cells were injected into his bloodstream to repopulate the bone marrow and mature over months into a healthy immune system.

The research study had seven test subjects. Three of them, including Dill, had sustained improvement for more than two years after their transplants, Medsger says.

Dill celebrated his third anniversary for the transplant in August.

Two test subjects died from disease-related complications after their transplants, Medsger says. One patient improved, but then the scleroderma returned. The other patient had a severe case of scleroderma that had not improved after the transplant.

The research study was for scleroderma patients with severe and rapidly progressing and severe skin thickening, because they were at the highest risk to develop involvement with internal organs and potentially die, Medsger says. The current larger study is for patients with the same symptoms.

Some damage from scleroderma cannot be fixed, but study officials hoped the transplant would result in some improvement and long-term remission, he says.

It's still unclear whether the remission experienced by patients, like Dill, is temporary or permanent, because the transplants were recent, Medsger says.

Dill is optimistic that his remission will last. He says he's kept a positive attitude, especially knowing he's helping future research.

Study was a lifesaver



Dill says he was feeling great in the summer of 2004, even biking 12 to 14 miles a day and climbing trees to trim branches.

"Most of the pain was gone," he says.

Then, in January 2005, he developed cold symptoms and went on antibiotics. Further tests were done in Pittsburgh, revealing he had polymyositis, another autoimmune disease that inflames muscles and causes weakness.

Dill says prednisone took care of many of polymyositis' effects, though the conditions after effects and a thyroid problem cause him to get fatigued easily.

Medsger says it's unknown what caused the polymyositis.

Dill says the study was worth it, despite some lingering effects from both diseases and the pain he experienced from the chest tube and chemotherapy that caused him to lose his hair temporarily.

"If I hadn't gone through the clinical trial, I'd be dead or in a nursing home," Dill says.

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