Scleroderma 101

Scleroderma is an autoimmune rheumatic disease that affects connective tissues. Derived from the Greek words sclero meaning hard, and derma meaning skin, scleroderma is a fairly rare condition affecting about two in 10,000 people in Canada.

There are many different manifestations of the disease – one of the most common is hardening of the skin. Dr. Janet Pope, one of the Rheumatologists at St. Joseph’s Hospital in London has been working with scleroderma patients for 16 years, says scleroderma has three main facets. “Scleroderma can result in blood vessel issues, leading to people’s hands turning white or blue when cold. It can also cause autoimmune issues where the immune system can attack the body. And fibrosis, where the production of too much collagen leads to scaring of the skin and possibly internal organs which can lead to severe problems with the heart, kidneys, lungs and even the conduction of the swallowing tube. This form of scleroderma is called diffuse scleroderma and can cause pulmonary, gastrointestinal and renal complications.”

Kandy Kurkcuyan has scleroderma and has experienced some of these difficult outcomes. Kandy has been dealing with scleroderma for almost 30 years, a rarity, as she was diagnosed at the age of 11 and scleroderma predominantly affects people in their 40s. Kandy was 11 when she began to experience stiffness and joint pain while visiting her cousins. “I was sitting on the floor playing and I started to feel a lot of pain, to the point of crying, shortly thereafter I was diagnosed with scleroderma, developed renal failure, and was in the hospital for 3 months.”

Humourously crediting herself for the invention of plastic IV bags, Kandy would have fun in the hospital by riding her IV pole down the hallways, but honestly admits growing up with scleroderma was anything but fun. As a child she developed Raynaud’s phenomenon, a skin discoloration caused by vascular issues, as well as small red dots on her face, which was a result of blood vessels becoming more visible. “It was hard. Kids wouldn’t play with me and in the beginning even my siblings didn’t know how to react.”

Kandy credits the care she received as a child, and the support of her family for helping her through the difficult beginning stages of dealing with scleroderma. Today she credits her personal strength, her family’s support and the care she receives from Dr. Pope and the Rheumatology department at St. Joseph’s.

Scleroderma Awareness Month has just passed, which was fitting for Kandy as she completed the scleroderma day program at St. Joseph’s in early June. “I called it Scleroderma 101. I can’t believe how much this program offered me – how much it taught me. I have lived with scleroderma for 29 years, I thought I knew everything until I left the program at the end of the day with new knowledge.”

The day program emphasizes well being inside and out, including education on skin care, body image, range of motion exercises, nutrition, and how to use assistive devices including household gadgets. “We offer a lot of different day programs in the Rheumatology department,” says Warren Nielson, director of the Fibromyalgia day program at St. Joseph’s. I don’t think anyone offers such a comprehensive scleroderma day program anywhere else in the county.”

Kandy says she completely enjoyed the 2-week program. “It felt wonderful to learn something new everyday and to talk to others who had similar experiences; we all shared stories and tips on dealing with scleroderma. The program truly gave us the tools for a better quality of life.”

Dr. Pope says there have been many great strides in the care of scleroderma. “There are many new treatments in screening and early diagnosis. More has been done in the last two years than in the past 50. Not only has there been quite a change in the treatment of Scleroderma but also in the area of research. There is a lot more hope for patients, there are proven therapies and much more to offer them.”

Kandy was so impressed with the care she received through the day program that she surprised everyone with a platter of sandwiches on the last day. “I am so appreciative of the program and the care I receive from Dr. Pope. I feel like I don’t have to worry anymore. I’m not concerned about what the future holds, I know how to handle it, and I know I have the support I need from my family and my doctor. I feel like the world is my oyster again.”