When Nancy Garza was diagnosed with scleroderma in 2015 at 60, she felt lost, depressed and was in denial.
“I had had never heard of scleroderma,” Garza said.
Scleroderma is a chronic autoimmune disease with no cure and can be life-threatening. As many as 10,000 people die annually from the serious forms of scleroderma.
June is Scleroderma Awareness Month and; more importantly, June 29 is World Scleroderma Day, founded with the intent to educate people about the disease and how it affects people living with it.
The word “scleroderma” comes from two Greek words, “sclero” meaning hard and “derma” meaning skin. The disease stems from the over-production of collagen in the body that creates hardening of the skin and internal organs such as the lungs, kidneys, heart and gastrointestinal tract.
One of the most visible traits of the disease is skin hardening, but it can take several forms and varies from person-to-person. Scleroderma can be hard to diagnose because other diseases can have similar symptoms. Eighty percent of those afflicted with the disease are women.
The good news, according to the Scleroderma Foundation, is the disease is not contagious, infectious, cancerous or malignant.
“I come from a Mexican-American heritage known for a legacy of longevity,” Garza said. “The women in my family live to be 100 years old. Great-grandmother died at 106; grandmother died at 103; and my mother will be 90 this year.”
Reports estimate that 300,000 Americans have scleroderma; however, the average person has never heard of it.
“The average age for scleroderma is between 35 to 55 years old, but it can affect much younger and much older people,” said Dr. Elizabeth Volkmann, who is an assistant professor and co-director of the connective tissue disease program with the UCLA Division of Rheumatology. “Older age is associated with higher mortality rate.”
Volkmann added: “No prevention is known because we don’t know who would be affected and when.”
Garza was not taking any medication and had no health issues other than a little extra weight gain. She did not have symptoms that reflected that she could have a disease.
At a typical dental visit, Garza was having difficulty opening her mouth for her examination.
“I felt something wasn’t right because the skin around my mouth was very tight and it also seemed the size of my mouth had shrunk,” Garza said. “I also noticed that I would be out of breath mid-sentence when I was talking.”
The Scleroderma Research Foundation explains how several different complications of scleroderma converge to make mouth and dental care challenging.
Tight, thick skin and a gradual reduction in the size of the mouth, and excessive dryness of the mouth from damage to the salivary glands are the two principal conditions that make oral hygiene and dental health difficult to maintain. Eating and speaking can also be difficult.
“During the time of these strange changes in my skin and breath, I was under a lot of stress: the loss of my husband of 38 years, being a caregiver for him during his illness, and overworked at a full-time job may have contributed to be affected with this disorder,” Garza said.
“No one in my family has scleroderma. I have one niece who has Lupus, so this was baffling,” she said. “I found out that I had low energy because of this disease and lack of oxygen.”
Garza had no idea that she was lacking oxygen because she didn’t feel dizzy or lightheaded.
Turns out, her disorder affected her lungs. The Scleroderma Foundation cites that pulmonary hypertension is not the only type of lung disease that can occur with scleroderma. Interstitial lung disease, also called pulmonary fibrosis, is another serious complication.
“Scleroderma can affect the internal organs but not every patient is affected that way,” Volkmann said. “Lung involvement — shortness of breath; GI tract involvement — reflux/heartburn, hard to swallow, constipation; kidney involvement — high blood pressure, nausea or vomiting, headaches; and heart — shortness of breath, heart racing/palpitation, and chest pain” are some of the symptoms.
“I have to be on oxygen 24 hours, 7 days a week,” Garza said. “Without a constant flow of oxygen, my oxygen saturation drops immediately to as low as the high 70s.”
The doctors advise Garza to keep her level at 92. The average level for people with no ailments is 98.
“I take six chemo pills a day to prevent scarring because it will block the oxygen to get to my lungs,” Garza said. “I take two more pills to treat hypertension that affected the right side of my heart which developed because of the disease.”
“Patients with scleroderma are at increased risk for developing pulmonary hypertension, also known as high blood pressure in the blood vessels of the lungs,” said Volkmann, whose clinical interests include systemic sclerosis, interstitial lung disease, pulmonary hypertension and systemic lupus erythematosus. “This reduces the blood oxygen level, which in turn, may cause a reflex increase in blood pressure in the pulmonary arteries.”
The Scleroderma Foundation further notes that pulmonary hypertension patients may also notice unusual chest pains and symptoms of right-sided heart failure, such as worsening shortness of breath and swelling of the feet and legs. Other symptoms that patients may experience include cough, light-headedness or fainting, palpitations (heart racing or fluttering) and swelling.
“Hand swelling and puffy, hardening/thickening of the skin can be limited to arms, hands, lower legs, feet and face or can be diffused can also happen in chest and torso,” Volkmann said. “All patients have Raynaud phenomenon and some degree of thickening of the skin.”
Garza has the redness and tightness on her face that is a typical trait of the disorder.
“It felt as though I was given a life sentence,” Garza said. “The most difficult part about scleroderma is the unknown, so I attend the scleroderma support group meetings to help me understand the disorder and to know how to live with the limitations.”
Scleroderma affects her quality of life.
“I no longer have the freedom of choice,” Garza said. “I have to be sure I have enough oxygen and charged batteries for the oxygen tank when I leave home.”
At home, she uses a condenser (the size of a carry-on suitcase) that operates around the clock.
“I have to wear a nasal cannula, which is connected to about 70 feet of tubing to allow me to basically walk throughout my home and stay connected to the oxygen.”
It’s people like Tina Burger, a patient service manager at the Scleroderma Foundation, who helps to educate people about the disease and make it easier for patients to understand the disease by providing them with the tools they need to cope and thrive.
“After volunteering for 17 years, I knew it was time to provide patient support, outreach and education on a full-time basis,” Burger said. “I was in awe of the strength and endurance of these patients living with the disease.”
Burger provides a variety of resources to help patients through the difficult time in their lives and help them see the light at the end of the tunnel.
“We are there to help them on many levels, that is, to deal with the confusing news of the diagnosis on an emotional level, provide information and tools needed to help navigate their treatment, and to offer a community of resources with options for their care,” she said.
Garza is committed to educating others about scleroderma.
“I plan to start a support group in my new adopted city – Las Vegas,” she said. “I want people to know they are not alone, educate others about what people living with scleroderma experience on a daily basis, and to help fund efforts to find a cure.”
Scleroderma Foundation – www.scleroderma.org
World Scleroderma Day – www.worldsclerodermaday.org
Scleroderma Research Foundation – http://www.srfcure.org/
Marie Y. Lemelle, MBA, a public relations consultant, is the owner of Platinum Star PR and can be reached on Twitter @PlatinumStar or Instagram @PlatinumStarPR. Send “Health Matters” related questions to firstname.lastname@example.org and look for her column in The Wave.