Increasing Awareness about
Pulmonary Hypertension
By Paula Swanson
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 conference@phassociation.org
