Positively Battling Scleroderma
The Kao Autoimmunity Institute and the Cedars-Sinai Scleroderma Program Mark First Anniversary During Scleroderma Awareness Month
Scleroderma has taken both of Lovette Twyman Russell's lungs and the tips of three of her fingers, but not one ounce of her joy in life. For the 58-year-old former triathlete, getting the right treatment for this rare autoimmune disease – which causes abnormal growth of connective tissue and can permanently damage the joints, skin, cartilage and internal organs – has made all the difference.
Russell travels regularly from her home in Atlanta to the Kao Autoimmunity Institute and Scleroderma Program at Cedars-Sinai, where she is a patient of program director Francesco Boin, MD.
In the Beginning
Russell was an active wife and mom, raising two sons and competing in triathlons when, at 40, she first noticed a common early scleroderma symptom: chronically cold hands. "When I went to hold something cold to drink, my hands would feel like they were on fire," Russell said. "That worried me."
To protect her sensitive fingers, she wore gloves all the time. "I have maybe 200 or 300 pair, and they just became a fashion statement for me. They match everything that I wear," said Russell. It was when she began losing her mane of curly hair, however, that Russell knew she needed to take more serious action.
After two years and visits to several doctors, who diagnosed everything from "stress, to it's hormonal, to it was practically in my head," Russell finally learned she had scleroderma, a condition she had never heard of.
A friend referred her to Boin, then director of the Translational Research program at the Johns Hopkins Scleroderma Center. She flew regularly to see him, and when Boin joined Cedars-Sinai in 2020 to establish the Scleroderma Program, Russell began traveling to Los Angeles.
"I am willing to travel to a place that I know understands my illness better than any other place," Russell said.
One of the reasons Boin was drawn to scleroderma as a specialty was the chance to connect with patients like Russell.
“We can really do a great job helping the patient, treating them, making them better," Boin said. "But we still don't have a cure for autoimmune disease. So, these become lifelong patients. I always enjoy this unique bond with the patient, the fact that we become companions in paths that sometimes can be very challenging.”
Not many medical centers in the U.S. have the resources to create the type of comprehensive program these patients need. Symptoms of scleroderma, which can be fatal, vary from person to person, and get worse over time.
“It takes a village, literally, to take care of these patients," said Boin. "They can face the challenges this disease represents only in a program that brings together the necessary clinical and scientific expertise." This means rheumatologists, pulmonologists, cardiologists, plastic surgeons, and dermatologists.
The tips of three fingers on Russell's right hand developed ulcers and would eventually have to be removed. Scleroderma began to affect her lungs as well. "I had just finished doing the Honolulu Marathon, and my timing for that race, even though I had been training for nine months, was not what I anticipated and hoped it would be," she said. Russell gave up racing, and when even walking left her short of breath, she was placed on oxygen fulltime.
Prescription: Positive Attitude
Through it all, Russell was determined to maintain a good attitude. "I would be pulling an oxygen tank," she said. "I would have on heels, a dress, and gloves. There was no shame in my game. It just became a part of life, and I tried to stay as positive as I could."
In 2019, Russell received a double-lung transplant. She still takes 57 pills a day to stave off rejection and manage her scleroderma symptoms, but is back in training and plans to be competing as a triathlete again in about nine months.
Meanwhile, Russell continues traveling regularly to Cedars-Sinai.
"Dr. Boin is now in a space that is dedicating money and energy and effort into making it comfortable for patients like me to come and visit, and not feel some kind of way because my fingers are a little weird, or my mouth looks weird or my skin looks weird," said Russell. "All the nurses understand what you have and what you're going through. The doctors understand and know exactly what you're going through, and how to help you through this journey medically and emotionally."
Along with patient care, an important component of the Cedars-Sinai Scleroderma Program is translational research.
"This research starts in the examination room with the patient, " said Boin, "and translates into more sophisticated research in the lab. We have been bringing on physician scientists who have the ability to assess patients from a clinical standpoint, and also to help us answer important questions such as why people develop scleroderma and how we can stop the process that damages internal organs.”
Until these questions are answered, Russell has a word of advice for her fellow scleroderma patients.
"What I've learned to do is really to look at all the positive things in my life," she said. "I have really come to rely on my faith and my positivity to keep me in this life, enjoying everything I do."
Read more on the Cedars-Sinai blog: Advances in Scleroderma Treatment: FAQ