Ehlers-Danlos Syndrome patient Ellen Smith serves as advocate for pain relief access

Ellen Smith has Ehlers-Danlos Syndrome, a progressive degenerative connective tissue disorder characterized by joint hypermobility, skin extensibility and tissue fragility. Ellen knew from childhood that she was unusually flexible and highly mobile, but thought nothing of it. She first encountered problems as a master swimmer, when she began to have excessive shoulder pain from overstretching her ligaments. To ease the pain, she would stop swimming for a week. She was once given a steroid shot, but this gave her horrific pain and made her unable to raise her arm for a month. Ellen now knows this course of treatment was the worst thing for EDS, as steroids break down collagen even further.

In 2002, Ellen had surgery for a dropped bladder. When her bladder dropped again two years later, her surgeon referred her to a geneticist for testing, and Ellen was diagnosed with EDS. Although she intuitively knew the diagnosis was right, she was shocked and sought a second and third opinion before she finally accepted it.

Classical EDS affects 1-in-20,000 to 1-in-40,000 people. Ellen's case is severe and has worsened in the past year. She is extremely cautious in public, as the slightest touch, bump, squeeze or hug can easily dislodge her joints. Since her diagnosis, as the deterioration of connective tissue in her joints has progressed, she has undergone 22 major surgeries to help her maintain mobility. Her pain increases significantly throughout the day, and on bad days, her mind becomes foggy. With compromised oxygen levels reaching as low as 45%, Ellen relies on a BiPAP SIT (i.e., a home respirator) to keep her oxygen levels within a normal range.

Maggie, Ellen's black Labrador service dog, is her protector. Maggie helps stabilize Ellen so she can stand and walk, and brings her medicine from the refrigerator. Just six days after receiving Maggie, Ellen stopped breathing in the middle of the night, and Maggie woke her, saving her life.

Ellen has had to stop teaching, swimming and coaching. She can no longer help her husband with small home improvement projects, a pastime she once enjoyed. To the outside world it appears that EDS robbed Ellen of her life, and yet Ellen would disagree. Although she recognizes that her life has shifted course, she refuses to become emotionally paralyzed. She and her husband, Stu, live by the motto that you must enjoy what you have while you have it. She has passion for life and continues to live with hope and love.

Ellen is proactive in her therapy program. She follows an exercise regime on her bed to strengthen core muscles and uses pool therapy to jog. Because she also has a rare form of celiac sprue, a chronic digestive react disease that interferes with the digestion and absorption of nutrients from food, she follows a strict diet that prohibits dairy, soy and gluten.

Although Ellen has had severe reactions to most medications, she has found pain relief from medical marijuana, which is legal in her home state of Rhode Island. She is proud of her state for passing legislation enabling its distribution, and is an active supporter of the Gifting Program, which allows people to share limited amounts with others.

Ellen works as an advocate leader for the U.S. Pain Foundation and as a state ambassador for the Arthritis Foundation. Another organization that is particularly close to her heart is the Ehlers-Danlos Syndrome Network CARES Foundation, a group dedicated to creating public awareness and funding research.

While working with these organizations gives Ellen a sense of purpose, she finds the greatest fulfillment in connecting with other EDS patients. She understands the speculation and criticism they experience, and urges them to have hope and courage. Ellen is still a compassionate educator. She teaches people to be proactive, to be their own best advocate and to find their own answers, believing that if something cannot hurt us, it is worth trying.

With the support of her four sons, the devotion of Maggie and the unconditional love of her husband, Ellen moves forward. She lives each moment as it comes and is happy to be alive.

"I don't want to be remembered as someone who pitied herself. I want to be remembered as person who tried, a person who made a difference, lived life and found joy."



Resources

INvisible Project
Ehlers-Danlos National Foundation
Ehlers-Danlos Syndrome Network C.A.R.E.S., Inc.

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