I was diagnosed with lupus in 1989, but looking back, I am sure I had lupus in high school. I became very ill at the end of my senior year and had to miss my senior prom. I was released from the hospital just in time for graduation. The physicians never could understand why I became ill. In the fall I started attending nursing school. Life seemed to be going well. However, I remember being so exhausted all the time. At the time I related my exhaustion to school and work, but now I can see that it really was the lupus.
I married shortly after graduating from nursing school. The time while planning a wedding and waiting for my state board results to come in the mail was very stressful. I finally found out that I passed the exam just a week before the wedding. That helped reduce some of the stress. I found a job and life went on, again.
During the first few years of the marriage, I had two children, Ben and Ashley. My health began to deteriorate even more when Ashley was two, I was exhausted constantly and had a hard time keeping up with work and being a mom. I started to lose weight and couldn’t understand why. I was worried about losing my job because I wasn’t working up to my ability.
After years of seeing gastroenterologists, dermatologists, neurologists, oncologists, psychiatrists, and hematologists for various symptoms which included rashes, intestinal problems, seizures, and low blood counts, I was finally diagnosed with lupus. I was actually relieved that something really was wrong with me. Now people could understand that I was not crazy and depressed.
My journey with lupus has been a long one. Having to be on prednisone daily was a real struggle. My dose kept getting higher and I hated the side effects. I gained a lot of weight and blew up like a balloon. For years I had noticed extreme fatigue and shortness of breath. I just related it to my lupus and being out of shape. I was diagnosed with pneumonia several times at the Urgent Care after experiencing symptoms of shortness of breath and chest discomfort. I was becoming increasingly more short of breath and I had a hard time just walking up a flight of stairs. Then one day I had a fever, I wasn’t too concerned because this happens when I have a flare-up with my lupus. Just to be sure, I decided to go into Urgent Care. As the physician listened to my heart and lungs, she looked a little concerned. She called in another physician. They thought I was having a heart attack. I was admitted to the coronary intensive care unit. I remember being told something about my pulmonary pressures being high.
The next day the cardiologist came into my room and informed me that I had secondary Pulmonary Hypertension (PH). I was already living with lupus and wasn’t prepared to take on another chronic disease. I remember asking what it was. I learned it is high blood pressure in my lungs that causes the right side of my heart to enlarge. Is there a cure? I remember being told that there isn’t a cure but there is a treatment to help maintain the symptoms. I was told that I would not be able to work anymore and that I needed to file for disability. I remember saying to the staff I am not disabled and I still want to work and I am not going to give up.
I spent about a month in the intensive care unit. It was a long struggle. The drug Flolan was in the process of being approved for secondary PH at about the time I was diagnosed. Flolan works like a vasodilator. Unfortunately the method of delivery was a continuous flow through an I.V. A catheter is inserted surgically through the subclavian vein and threaded into the right atrium of the heart. I was scared. I went through the procedure to have the catheter placed.
Then I was attached permanently to this pump and could never disconnect it except when I was changing cassettes of the medication. For me, it became a normal routine. I couldn’t leave the house without it.
The central lines were supposed to be pretty permanent; however, I had the unfortunate experience of my lines falling out four different times. This was a big deal because you could only be off of the Flolan for 3-6 minutes or I could go into rebound and possibly die. My lines fell out when I least expected it. When I got out of the shower, I noticed my line had fallen out. I called 911 so they could re-start the I.V. in my arm. Then I went by ambulance to have another central line put in.
I was on the Flolan for about three years when I found out there was a new drug being approved for PH called Tracleer. I was so excited to try this new drug even though one of the major side effects is liver toxicity. I was told that I needed my liver function tested once a month. I had my blood checked after being on the drug for one month. Two days later I received a phone call from my cardiologist informing me that I needed to stop the medication because my liver function tests were four-times the normal.
I was devastated. He told me that I needed to start the Flolan again. Because I did not want to give up, I asked the cardiologist about Viagra. I had heard that it was being used in PH patients. The drug wasn’t approved so the cardiologist was very reluctant. I pleaded with him to just let me try it and if it didn’t work I would go back on the Flolan.
Because I had to be monitored during this process, I went back to the coronary intensive care unit. I was in there for two days and believe it or not, my pulmonary pressures remained the same on the Viagra as they were on the Flolan. I was ecstatic—I couldn’t wait to have my central line removed forever, thinking that now I only would have to take pills—Viagra at that. It was like a dream come true. Viagra is also a vasodilator and it works in many ways. Can imagine the look on the pharmacist’s face when I dropped off my prescription for Viagra? I needed 250 pills for one month. I had to come back the next day because they didn’t have enough in stock.
It has been almost four years now that I have been taking the Viagra. It was a process of getting insurance to cover it since it wasn’t approved to treat PH. It certainly was a lot cheaper than the Flolan which cost about $4,000 a month. A year ago Viagra was approved for the Treatment of PH. I feel very fortunate that I was able to be on it before it was approved.
I have come a long way from the grim prognosis of possibly dying to having to quit work and be on disability insurance. Now I work part-time and I am able to walk three miles a day without becoming short of breath.
It is exciting to know that there is on-going research regarding Pulmonary Hypertension. More new treatments are becoming available for this rare disease.
The 7th International Pulmonary Hypertension Conference PHA’s 7th International PH Conference and Scientific Sessions: Roadmap to a Cure will be held June 23 – 25, 2006 in Minneapolis, Minnesota. It is a wonderful opportunity to bring together patients, caregivers and medical professionals. Please contact PHA’s offices at 301-565-3004 or [email protected]