I was eight years old, unafraid, full of energy and curiosity. My mother told me to wear sandals whenever I played outside. Like most children, I disobeyed now and then. That bright summer day was one of those times when I ventured outside without shoes.
I was running through a field of long grass playing with some friends unaware of the debris in my path. I stepped on a board with an old rusty nail sticking out of it. The nail went in and out of my foot. I ran to the house and ran water from a water hose over the wound as blood gushed out. Afraid of getting in trouble, I found a rag in the garage and wrapped my foot until the bleeding stopped. I did not tell my mother.
A couple of days later, I woke up with my knee the size of a grapefruit. It was full of liquid and I could not straighten it out without extreme pain. My mother rushed me to the doctor's office. They drained the fluid with a big syringe. My mother almost passed out. I was admitted to the hospital with a massive infection. I was given three shots three times a day of antibiotics. I'll never forget how I dreaded those injections. I stayed there for a week or so and was sent home on crutches and more antibiotics. I recovered quickly and soon returned to my normal schedule.
A few months went by and then I started to develop liquid-filled bumps on my wrists. The doctor removed the liquid by syringe once or twice a month. I had to take aspirin for inflammation. That passed.
A month or two later, I started to get little white patches of dry skin on my left upper arm. I notice them and showed them to my mother. She applied creams including pure aloe vera, but it did not seem to work. The patches started to connect and harden. We went to our family doctor who thought it was a fungus. We applied anti-fungal medication. It did not help. The patches spread down the inside of the arm toward the hand. My family doctor suspected an arthritis related illness. He said I should take aspirin.
My parents weren't content with that answer so they took me to the Kelsey-Sebold Clinic in Houston. There, I underwent lots of tests including a barium x-ray test and a biopsy of my arm. They diagnosed me with Localized scleroderma and said there was no treatment as the disease had to run its course. They said I should take massive doses of aspirin and Vitamin E.
Meanwhile, the hardening spread to my left thumb and started to spread up toward my neck as well. I exercised with a rubber ball to keep my thumb from atrophying. It did not work. My left thumb was left small and disabled. The creeping hardening of the skin continued it's crawl up my shoulder and then it stopped. The white patches now had an odd brownish pigment and the muscle underneath was clearly disfigured. I was twelve years old. For the next twenty five years, I lived my life with this scar from my shoulder to my hand. Though it was hard growing up with this 'en coupe de sabre', I knew I was lucky. I was healthy otherwise and did all the things young women do. I was active in school, had boyfriends, went to college, had two children, got married and had a successful career as a journalist.
Then around the age of thirty-six, I started noticing that I was having shortness of breath when I exercised. Though I was always about fifteen to twenty pounds overweight, I could always exercise and get down to my 'fighting' weight of one hundred and twenty pounds. But this was different, I would exercise and feel worse afterwards. I could not get through long exercise tapes without feeling breathless and extra tired. I chocked it up to getting older.
Every year, during my checkups, I would mention it to the doctor. He would listen to my lungs and heart and say it might be stress from work. I started trying to reduce my stress but it did not seem to help. I started feeling more and more fatigued. So when I went in for my yearly checkup on my thirty-seventh birthday, I again told my doctor about the shortness of breath and fatigue. He listened to my heart and this time he heard something. He brought in an EKG machine and saw the telltale signs of the right side of my heart working extra hard.
That's when I first heard the words pulmonary hypertension , (PH). He said that people with scleroderma can get PH, a rare cardiovascular disorder caused my high blood pressure in the pulmonary arteries. "One out of a million," he said. But people with my type of scleroderma, Linear Scleroderma, aren't supposed to get PH. So he sent me to a lung specialist, a rheumatologist and a cardiologist.
After several more tests, the new lung doctor said my lungs were the healthiest he'd seen in a long time. "No PH," he declared.
The rheumatologist tested my blood for scleroderma, lupus or other autoimmune antibodies. I tested negative for scleroderma or Lupus, but came up positive for Sjögren's, an autoimmune disease that effects the glands in the eyes and mouth. This made sense, because I had been having trouble with eye dryness. I went to see an eye doctor who told me to use a different type of eye drop that can be purchased over the counter. The drops helped, but my tongue began to develop cracks and hurt when I ate certain foods. This is also a symptom of Sjögren's.
The rheumatologist concluded, though, that I did not have any of the signs of generalized scleroderma. Although I would struggled with heartburn most of life, testing showed that I had no signs of scleroderma there either.
During that time, I also saw Dr. Maureen Mayes, an internationally known expert on scleroderma, to get her opinion. She was still in Detroit at the time and concluded that I did not have active scleroderma either.
The cardiologist, however, said that I did have PH and the prognosis was not good. I had mean pulmonary pressures of about 78, which indicates moderate to severe pulmonary hypertension, and my pulse oxygen rate reduced to 85 when I stood up.
I decided to get a second opinion with a local expert in PH, Dr. Mel Rubenfire, a cardiologist who runs the Pulmonary Hypertension Clinic at the University of Michigan (U-M). I met Dr. Rubenfire a few months later, in the fall of 1998. He confirmed the PH but gave me hope. He also did not think that it was related to the scleroderma but he could not rule it out. Either I had a rare case of childhood linear scleroderma developing internal involvement years later or I was struck by lightning twice. Either way, it was a serious condition and we had to do something about it soon.
I underwent a right heart catherization to get an exact measurement and to see if I responded to another type of medication. I did not respond to that oral type of treatment so they started me on Flolan. Flolan is considered the strongest blood vessel dilator on the market and the treatment requires 24-hour IV administration of the medicine. I was set up with a Hickman catheter in my chest, shown how to mix my medicine daily, how to keep from getting infections and how to deal with side effects of the medication.
At first, it was scary, but the medicine really helped with my quality of life. I was already at the point where I couldn't walk up stairs without being winded. I was sleeping all weekend and I could not think very straight. Once the Flolan started going, it was like a new day for me. I had not felt that good in such a long time that it made the side effects easy to handle. There was nausea, migraine headaches, diarrhea, jaw pain, leg pain and acid reflux. But again, the pay off was so great, I dealt with it. I was still working full time and juggling the boys with the help of my husband.
Though Flolan helped me and had the potential to restore some of damage in the blood vessels, I was not getting better. A second heart catherization a year later revealed that though I was feeling better, the pressure in the pulmonary arteries was getting worse. They started talking to me about heart and lung transplants. I was devastated.
I had to return to the rheumatologist for a redo on blood work as a first step to ensure that I did not have scleroderma. The transplant team at the University of Michigan wanted assurances that I did not have it. When I retested more than a year later, the results were reversed. I tested positive for the scleroderma antibodies and negative for Sjögren's.
The rheumatologist and Dr. Mayes both thought that the blood work had been incorrectly processed.
At this point, I was pretty desperate. I did not want to have a transplant. So I called up Dr. Rubenfire and we talked about other options. He said we could push the Flolan as much as I could handle and see what happens. I agreed. I also decided to pull out all the stops and try just about everything I had heard about.
I called a doctor I had interviewed for a story I had written a few months earlier; an obstetrician-gynecologist (OB-GYN), who was doing some groundbreaking work with men and Type 2 diabetes. He also was involved with anti-aging medicine and had successfully treated his own lupus-afflicted wife with DHEA, a naturally occurring enzyme thought to regulate cell function and hormone levels.
This doctor, Dr. Edward Lichten, did some blood work and put me on DHEA. He also said I should take 1600mg of ginkgo biloba to aid in circulation, 800mg of vitamin E, 1000mg of vitamin C and weekly B-complex and B-12 injections for stress. Also, I started taking pharmaceutical grade fish oil tablets for inflammation. Then he referred me to a homeopathic practitioner and medical intuitive, Lynn Nichols. She had apparently cured Dr. Lichten of a long term stomach ailment and he sent her all his desperate cases. That sure did describe me.
I started seeing Lynn in March 2002. A certified practitioner, she discovered that I had several metals in my system, a couple of parasites including hook worms and a couple of viruses. I started a cleansing process to get rid of the metals and viruses first. She also gave me minerals and herbal supplements to help me remove these things from my body. I took calcium and potassium, fiber, herbal teas, and other natural cleansers. The homeopathic tablets looked and tasted like sugar pills. The overall goal was to balance the immune system.
I did not know if I believed it but I knew it would not hurt me so I tried it. I felt like I had a slight flu, body aches most of the time. Then I would have a strong urge to go to the bathroom and I would immediately feel better afterward. I started to taste the metals coming out through my mouth. She said all this was normal.
In May 2001, I returned to the University of Michigan for another right heart catherization. My pressures had reduced to a mean of 55! I was so excited. The doctor at U-M said that the increased Flolan might have had something to do with it. Also, they discovered something new during this cath: I had a large atrial septal defect; a hole in my heart. The doctor isn't sure whether the hole caused the PH or if it opened up to relieve the pressure. Either way, it explained why my oxygen levels dropped so significantly when I walked. It was also a better explanation than the scleroderma which did not make sense to anyone. With the reduced pressures, I became a good candidate for a new oral drug for PH called Tracleer. This is an endothelial antagonist that prevents inflammation in the blood vessels. I started taking it in June 2001 but it made me feel so bad at first that I stopped taking it. I felt dizzy and weird in my chest.
I continued with the homeopathic remedies and the vitamin therapies through the summer. Overall, I felt about the same though I was able to do yoga and walk on my treadmill.
In Sept. 2002, I saw my U-M doctor again and my six minute walk was the best I would ever done. I did not need oxygen and I walked farther. The echocardiogram estimated my pressures at about the same, mid-50s. I was experiencing a lot of trouble with acid reflux as a result of the increased Flolan as well. So the doctor suggested I give Tracleer another try. I started taking it at a lower dose. I felt the same sensations, but they subsided after a few days. We increased the dose and I have been on it for about five months now. In this time, I have been able to reduce my Flolan to where I was when I started.
I returned for support to my anti-aging doctor who tested my saliva and urine to check my adrenal glands. As expected, they're completely depleted with chronic illness, which is why I have very little energy some days.
I mentioned this to my homeopathic person and she suggested that I try small amounts of licorice root extract to help with my energy levels. The licorice root has been a godsend. It's helped me peel back some of the layers of fatigue I have been living with for so long. But the licorice root is contraindicated with digoxin and warfarin, both of which I am taking. So you have to be very careful and listen to your body.
I am almost through my homeopathic regimen. I am working to get rid of my last parasite, toxoplasmosis which effects muscle. Once I have gone through this, then I am going to see another local healer, a nutritionist and chiropractor who is going to help me build my body back up.
I have had to be careful and I have kept my PH doctor informed of what I am doing. His attitude about it is that it probably won't hurt me so he's not opposed to it. I am also working to develop a daily meditation practice and am attending workshops on energy medicine. I am going to receive my first acupuncture treatment soon and I have even been documenting my dreams for ethereal healing direction.
Okay, I know it's a little out there for some, but I have decided to leave no stone unturned.
I have quit my job and reduced my stress a hundred percent. I am doting on my children and my husband. I am calling my parents more often and talking to my sisters and brother at least once a week. I am also seeking out long lost friends.
There's no more putting off for tomorrow in my world. That's for sure. I am writing a screenplay, something I have always wanted to do, and working with some nonprofit organizations to help the poor. I have learned time is indeed fleeting and that we all have only limited time to learn and do good. Then it's time to move on and go back to God. No matter what the outcome, I have learned to take control and I am not afraid anymore. That's a lot to learn in three years, all before my 40th birthday.
And that's the beauty of it.
Story Artist: Ione Bridgman
Story Editor: Judith Devlin
Dr. Maureen Mayes
'en coupe de sabre'
Pulmonary Hypertension Stories
ISN Artist Ione Bridgman created original artwork to illustrate this page. She is 90 years old, and lives in New Zealand. Her lovely paintings illustrate many of our pages and the covers of our Voices of Scleroderma Book Series.
Many thanks to our recent donors, Jack and Elizabeth Lewis, Network for Good, Shirley Miller, Reata Pharmaceuticals, and United Way of Snohomish County! See ISN News for more donors. Click Here to Donate.
TOLL FREE HOTLINE, U.S. and Canada: 1-800-564-7099
Ask for our Free Info Packet by email or postal mail!
SCLERO.ORG is the world's leading nonprofit for trustworthy research, support, education and awareness for scleroderma and related illnesses.
We are a service of the nonprofit International Scleroderma Network (ISN), which is a 501(c)(3) U.S.-based public charitable foundation, established in 2002. Meet Our Team, or Volunteer. Donations may also be mailed to: