SW Portland woman with rare disease: Scleroderma ‘feels like your skin is shrinking’

Liz Orem-Bedel knew it wasn’t normal for her fingers to numb and turn blue when she simply touched a chilled steering wheel, no matter how bitter winters were in Philadelphia.

Doctors recognized Raynaud’s phenomenon, a condition in which cold temperature (or strong emotions) can cause spasms in the blood vessels. Scleroderma is a common cause, so when the then-42-year-old moved back to her home in Southwest

Liz Orem-Bedel knew it wasn’t normal for her fingers to numb and turn blue when she simply touched a chilled steering wheel, no matter how bitter winters were in Philadelphia.

Doctors recognized Raynaud’s phenomenon, a condition in which cold temperature (or strong emotions) can cause spasms in the blood vessels. Scleroderma is a common cause, so when the then-42-year-old moved back to her home in Southwest Portland in the early 1990s, a doctor at Oregon Health and Science University checked her for the rare autoimmune disease.

The test was positive. At the time, so was her attitude.

“I thought it would never actually happen,” she said. “My doctor said the disease could kick in at any time, or maybe never. There was no way to know.”

One February morning two years later, she awoke with fingers swollen “like great big fat sausages,” she said. She knew the disease had caught up with her.

Orem-Bedel spent the next several years struggling to dress and eat by herself. She got trapped inside a public bathroom at least once or twice. Hardened skin relentlessly resisted any attempts she made to clench her fingers. Skin on her arms, torso and thighs became leathery and thick.

“It feels like your skin is shrinking, and you’re trapped inside,” she said.

She joined a local support group, but begrudgingly. “I didn’t want anything depressive,” she said. “I can go home and cry by myself, thank you very much,” She said she was frustrated that no one had heard of her disease, there was no cure for it, and little research was being done. Her husband reminded her she had a mouth and suggested she use it to raise awareness.

“I’ve always been a strong woman, and it’s not like me to sit back and do nothing,” she said. “So I got involved.”

Orem-Bedel is 65 now, and finishing up 10 years as the first and so-far only president of the Oregon Chapter of the Scleroderma Foundation. Now she has another project in mind.

Battling the unknown

Scleroderma, like many autoimmune diseases, remains a mystery.

There are two types: Limited, which affects only the skin, and systemic, which affects the whole body. Orem-Bedel battles the latter.

Her version of the disease hardens skin, connective tissue and blood vessels, which can cause a myriad of other problems including potentially fatal conditions in the digestive track, lungs and other organs.

“It’s really hard to come up with one drug because the disease effects so many parts of the body,” said Dr. Lauren Kim, a scleroderma specialist with Legacy Health in Portland.

The cause is unknown. Treatment fights the symptoms — such as acid reflux and pulmonary hypertension — to help keep patients alive and as comfortable as possible. Scleroderma specialists struggle to find research money because, Kim said, the disease is so rare and awareness so low.

Orem-Bedel wants to change that.

Since she joined the support group about 20 years ago, she’s helped organize an annual local seminar and fundraisers, such as the annual spring plant sale in Multnomah Village. She played a key role in connecting the support group to the national Scleroderma Foundation to further their reach. She talks about the disease with anyone who wants to listen. Orem-Bedel’s efforts have been mirrored by leaders at the national foundation, and Kim said the internet has played a huge role in connecting scattered patients.

“It’s been a constant climb to accomplish more and more,” Orem-Bedel said. “We’re just getting to the point where the word ‘scleroderma’ is being recognized. It’s on TV now.”

Orem-Bedel wants more. She wants federal research funding and for people with no connection to the disease to not just vaguely recognize the word ‘scleroderma,’ but know what it means.

She looks to breast cancer advocates for ideas. “Scleroderma kills women, too,” she said. But drawing as much attention as a disease that develops in one out of every eight women in the United States might not be practical, she recognizes??.

A 2008 study from the National Arthritis Data Workgroup estimated there were only 49,000 adult scleroderma cases in the United States. That’s about three-quarters of the population in Southwest Portland, where Orem-Bedel lives with her husband in a home she’s owned 40 years and sings in the Pride of Portland women’s choir.

“The foundation is really good, and they have a pretty strong chapter here,” Kim said. “But you can only do so much. There are so many rare diseases.”

Looking to youth

Orem-Bedel’s next mission is to support children battling scleroderma.

Children haven’t lived yet, she said. Young patients won’t get the chance to raise their own children free of disease. They probably won’t have memories of graceful hands, as she does. They face peers at school who mock and pry.

The disease can be devastating for young children, Kim said, by deforming their faces and skulls or stunting growth in one of their legs.

The national foundation has recently started offering classes for kids at its annual seminar, Orem-Bedel said. She’s been thrilled to see youth attendance climb in the last couple of years. She knows one child in the Portland area with scleroderma, she said, and is suspects there are others. She wants them to know they aren’t alone.

Finding children might also help draw attention to scleroderma. People are more interested in suffering children than suffering middle-aged women, she said, her tone matter of fact. More interest means more dollars, which could mean better treatment for those children later in life.

But the most important thing, she said, is for children and adults alike to know they aren’t the only ones with this condition. On two occasions she’s approached a strangers she believed to have scleroderma, she said. In both cases, the women had never met anyone else with the disease.

“I went up to this lady as she was going out the door, and I said, ‘Excuse me… by chance do you have scleroderma?’” she said. She had recognized the woman’s restricted movement. “Her eyes got wide and she said, ‘How did you know?’

“She broke down in the restaurant and started crying… She had never met anyone with scleroderma. She was just amazed that there were support groups. She didn’t know these things existed.”

Next month, Orem-Bedel will “pass the reins” of the local foundation to someone else. She believes she’s done a good job uniting adults in the area, and that it’s time to refocus her attention and put her “big mouth” to use supporting a group that she feels desperately needs a uniting voice.

— Melissa Binder