Kathy Leutze and Betzi Sheff are both in their 60s. They have only known each other for about a year but they have walked in similar shoes for more than two decades.
When they were in their 30s and early 40s, they began to experience a variety of symptoms that included fatigue, swelling and tightness in their hands, severe heartburn and joint pain.
Their family doctors were dismissive.
“The first doctor I saw told me, ‘You’re fine. You’re in your mid-30s and you’ve got heartburn. What do you want from me?’” Leutze recalled during a recent interview.
She said her chiropractor deduced what might be wrong during a visit when she felt at her lowest – and she had to insist that her primary care doctor confirm the suspicion with a blood test: scleroderma.
Leutze and Sheff, have each lived for more than 20 years with the chronic disease which, when they were first diagnosed, often proved fatal within three to five years. They lead the Scleroderma Foundation Tri-State Chapter Support Group in Western New York, a roughly 30-year-old contingent which meets at 11 a.m. the third Saturday of every other month in the Realty USA building at 4909 Transit Road, Lancaster.
The next meeting is Nov. 21; for more information, click here or call Leutze at 689-8258.
Sheff, who just turned 66, and her husband, Jim, moved from Cooperstown to Lancaster three years ago to be closer to their children and grandchildren. Sheff ran the scleroderma support group in Cooperstown before the move and became active in the Western New York group last year.
Leutze, 60, a child care provider who lives in East Amherst, grew up in the region; like the Sheffs, Leutze and her husband have two children. During the last decade, the skin on her fingers have tightened and her fingers have started to contract. The tip of her right index finger needed to be amputated.
“I can’t do everything with my hands that I used to,” she said, “but I try.”
Q. What is scleroderma?
Leutze: An autoimmune disease, a connective tissue disorder, characterized by an overproduction of collagen. In most cases, it sounds like a wonderful thing because it makes your skin feel smooth and tight. Some people get collagen injections in their face. But I can tell you when you have an overproduction of collagen, it’s not fun. Your skin can tighten to the point where you can’t move your limbs. It can affect your internal tissue. As it crowds into your body, your lungs can’t expand. It can harden your lungs. It can harden your kidneys. It can cause damage in your whole digestive system. It’s very serious and everyone with scleroderma seems to present in a lot of different ways. Another one of the offshoots is Rheumatoid arthritis. I have it. … The quicker you get a diagnosis, the better the chance that it won’t affect the internal organs.
A lot of cases begin with Reynaud’s disease. Your hands will turn blue and red and white. Your fingers will swell. Sometimes people present with extreme GERD, gastroesophageal reflux. Because not everyone presents in the same way, it can be difficult disease to get a diagnosis. The tragic thing is, the quicker you get a diagnosis, the better the chance that it won’t affect the internal organs. It doesn’t always affect the internal organs. Sometimes it just affects the skin. Mostly in juvenile cases, it just affects the skin. It’s drastically emotional for children.
I’ve had it for 22 years. I was 38. I’m sure that I had had it for a while before that. I just don’t think anyone knew what was happening to me. I had Reynaud’s first. Blue skin. I would laugh about it and show everyone and say, “Look at this,” not realizing how much damage was happening to me. Then I started with severe heartburn.
Q. What have you dealt with over time?
Leutze: It progressed in the beginning. Within the first eight to 10 years, I had pneumonia, which is extremely frightening for a scleroderma patient because it’s one of the biggest causes of death. It used to be kidneys, too. But everything has progressed to the point that even though we don’t have drugs specific to the disease, we have a lot of drugs that are cross-over. For instance, I’m taking a drug that’s intended for transplant patients. I’ve never had a transplant but it’s helped me tremendously. (CellCept helps maintain pulmonary health.)
Q. Is that the only medication you need?
Leutze: Gosh no. I take amlodipine for the Raynaud’s – it’s really a blood pressure medication – and I’m on Prilosec for the reflux. I’m on two different diabetes medications. Diabetes came in the last 10 years and it’s something that can come from taking all these medications. I’m also on prednisone (an anti-inflammatory).
There are several types of scleroderma and I have diffuse. That and limited are the two main types.
Diffuse is also known as systemic scleroderma and that involves internal problems. Limited is mostly outward skin tightening and hardening, but it can involve some internal problems.
Q. Betzi, how were you diagnosed?
Sheff: It took three years. My hands were swelling. It got to the point where they swelled so bad I went into a jewelry store and they cut my rings off (including her wedding ring).
I was getting red spots on my hands and my face and a lot of fatigue. I went to many doctors and one said to cut out tuna fish because there’s a lot of sodium in it. I said, “I don’t have edema because if I did, it’d be in my ankles, too.” You don’t want to challenge certain doctors. I finally went to a doctor and she said, “You’ve got scleroderma.” I said, “What’s that,” and she said, “It’s not good. It usually affects your lungs and your kidneys and you go into heart failure.” I got rid of her, too.
I was in my early 40s and had young kids at the time. I went right into menopause. Sometimes women go into early menopause, too.
I had hard calcium deposits in my hands and elsewhere, Reynaud’s, reflux, sclerodactyly (reddening in your nose and other parts of your face.) I have slight calcinosis and pulmonary hypertension but don’t need drugs for those, so far.
You have to go through a battery of tests every year: an ecocardiagram, pulmonary function test, blood work. I get a CAT scan on my lungs, too.
Leutze: You also need a urine test to make sure the drugs aren’t destroying your liver.
Q. Talk about the extent of the disease.
Leutze: There are as many people in the United States with scleroderma as with MS – about 400,000 – and yet nobody has heard of scleroderma. It happens about four times as much to women as men. Nobody knows why.
Sheff: When men get it, it’s usually more severe. It’s slightly more common and severe among African Americans. Ages of onset usually are 30 to 50s, but kids get it.
Leutze: Most of the first-timers who come to the support group are scared.
Sheff: We’re trying to help those people get through their first year, maybe, get them where they need to go. We can’t recommend medications. That’s not our role. Our role is to get them resources and listen to their stories. I think the more we can help each other, the more we can help ourselves.
Q. How many folks tend to attend your meetings?
Leutze: We’ve had as many as 28 and as few as seven or eight. There are 30 to 35 on our email list.
Sheff: We’re still trying to get the word out.
Leutze: Why it’s sometimes such an unknown disease is that sometimes patients don’t want to be seen. We have disfigurements…
Sheff: We adapt. People say, “you look pretty good for having it.” What am I supposed to look like? We’re all unique. What you see isn’t always what it is, and I never judge people by their appearances. But some people come into meetings and have a lot of issues. It’s an emotional thing. It’s scary. You think you’re going to die. When I got it – and the Internet wasn’t around – I went to the library at the hospital where I worked and everything said scleroderma is usually fatal. That’s not the case anymore. Doctors have come a long way and do a lot of research.
Leutze: If they could just find a cause.
Sheff: Autoimmune means basically your body turns against itself. … Your body doesn’t work the way it’s supposed to. Something’s wrong. There’s a disconnect.
Leutze: I don’t mean to sound brutal on doctors because it is hard to diagnosis. My primary doctor says, “When I get a student, I check to see if you’re on my docket because I want them to meet you.”
Sheff: My doctor, too asks if he can schedule a resident or an intern. If they never meet you, how are they going to learn? Anytime I have the opportunity to educate anybody on it, it’s a good day.
Q. It sounds like you both have had to put together a medical support team. What members are really important?
Leutze: A rheumatologist.
Sheff: That’s your number one doc, who directs the show.
Leutze: A pulmonologist. Cardiologist. Gastroenterologist. And a primary care doctor with understanding.
Sheff: Your primary care doc may not always be able to keep up with it. You have to keep going until you find someone you can work with. It’s very hard on your family, too.
A lot of having this disease is educating regular health practitioners, because they don’t see it often. I’m a veteran of this, so when you go and see the specialists and they say everything’s remaining the same, that’s a really good thing.
Q. What has changed to make this more of a chronic disease?
Leutze: Research and experimenting with new drugs. I wish they could figure out the cause, so they could refine the drugs.
Sheff: The University of Michigan is really doing a lot and we’re privy to the research because we give a lot of money. Administrative costs are very low in Tri-State.
Q. Do exercise or good nutrition make an impact?
Leutze: I’m sure they do.
Sheff: I’ve been doing Weight Watchers so I’m eating better, cleaner. … I swim a lot and I find it helps my joints. There’s a lot of people with bad backs, bad knees, and of course I always educate them on scleroderma.
Q. Wintertime must be a challenge considering the circulatory challenges.
Sheff: Everybody’s bundled up. Anytime I take something out of the freezer, when I walk through the frozen food aisle, my hands turn blue. Air conditioning is tough.
Q. What happens during support meetings?
Leutze: Most people we talked to have systemic scleroderma but that’s because they’re hurting a little bit more. We try to have speakers in the various specialties. We’ve had nutritionists in the past. We have DVDs available through the foundation from various conferences around the country and those featured will speak on a wide range of dental issues. Our mouth openings are smaller, so it’s a nightmare for us to go visit the dentist. (With a laugh) You’d never know it because I’m a big talker. It’s hard to find someone who knows what they’re doing dental-wise...
At the beginning of a meeting, we say we have privacy policies, so what you say doesn’t go any farther. We go around and introduce ourselves. I say, “Your story’s going to change every time you tell it.” We need to hear it all. There are an awful lot of emotions when people hear others are going through the same things.
Sheff: It really makes a difference. With any support group, you’re all in the same boat.
Q. What are good resources for scleroderma support and how can someone reach you?
Leutze: They can call me (at 689-8258). I have brochures at my house and I can send them. I encourage people to come to the meetings. One of the big things I like to say is “you are not alone.”
At the beginning, I felt very alone. I didn’t know anyone who had it.
Q. What would you say to somebody who’s just been diagnosed?
Leutze: I had one person who I met in the parking lot. I said, “Hi, welcome to the meeting,” and she burst into tears. I gave her a big hug and said, “It’s going to be OK. We’re going to help get you through this.”
Sheff: A lot of this is fear of the unknown.
Leutze: We can say, “I’ve had it for 22 years and you’re going to be OK.”
Sheff: We’ve had a good run.
Twitter: @BNrefresh, @ScottBScanlon