Increasing Awareness about Pulmonary Hypertension

I was diagnosed with lupus in 1989, but looking back, I am sure I had lupus in high school. I […]

Generic Article graphic with Access Press emblem

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]

  • Wash your hands! Hands that look can still have icky germs!
  • Work with your care provider to stay healthy. Protect yourself. Vaccines are your best protection against being sick.


Health plans as unique as you. Learn more. UCARE.
Go beyond your gambling habit. No judgement. Only help. GetGamblingHelp.com