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Pumpless
in Colorado
By
Earl Selvage & Cheryl Switzer
Earl Selvage,
one of our PHfriends and an active volunteer on this web site, shared
his wonderful news with us recently. After many years on the intravenous
drug, Flolan, his pulmonary artery pressures are nearly normal,
he’s feeling rather well and he’s been transitioned to
one of the new oral medications. His pump is history. Earl and our
feature editor Cheryl Switzer had a little chat and it is presented
to you here in interview form.
Cheryl:
Earl, you were diagnosed with PPH in 1992. What made you seek medical
help at that time?
Earl:
I had noticed I was feeling more tired than usual in the two weeks
before my first syncopal episode (fainting spell). I was a smoker
and attributed my weariness to the smoking and the hard work I performed
on a loading dock. It was the fact I fainted that I was taken to
the hospital and so began the endless testing to determine why I
fainted. It took four doctors, two hospitals, and more than a month
to figure it out. I was finally diagnosed at the University of Colorado
Health Sciences Center in Denver.
Cheryl:
What were your treatment options at the time of your dx?
Earl:
I was told I had four years left to live when I was first diagnosed
and treatment options at the time were limited. The main drug proposed
was Diltiazem (a calcium channel blocker). Dr. David Badesch tried
different amounts of this CCB to help alleviate the constant symptoms
of PH. I was short of breath, had chest pains, racing heartbeat,
and couldn't walk up an incline without fainting. Calcium channel
blockers were essentially the only drugs available and Diltiazem
wasn't helping me. My pulmonary arterial pressures were far above
normal. I started getting tested and worked up for transplant when
it was determined I wasn't getting any better. A single lung transplant
was the only remaining option.
Cheryl:
How long did it take for them to determine the CCB was not helping
you?
Earl:
Diltiazem was tried for about a year by itself with little success,
although, I still use it in my combination of drugs. I had two heart
caths performed during that time which revealed Diltiazem was not
helping.
Cheryl:
When were you offered Flolan and what were your expectations?
Earl:
I went into the cath lab in July 1993 to measure the pressures,
which were at their highest, and to test Flolan to see how I would
do with it. During the cath, I had little reaction to Flolan but
decided I needed to try it full time. Flolan was still in its experimental
phase at the time and a placebo was not used, probably because of
the invasive nature of the drug. I had no expectations of Flolan.
It was essentially a last gasp kind of thing to stay off the transplant
table.
Cheryl:
You live in Colorado at a relatively high altitude. Have you always
lived there? Do the doctors think or did they speculate that you
may have developed PH related to the altitude? Did they offer you
any theories about the origins of your elevated pressures?
Earl:
I have lived all 45 years of my life in Colorado. When diagnosed,
I lived at about 5,500 feet. The doctors speculated high altitude
might have had something to do with developing PH. In the cath lab,
I was tested for high altitude. They administered different mixed
gases simulating high altitudes and I didn't have an adverse reaction.
I believe it was determined the altitude didn't have anything to
do with the PH. Many PH patients outside Colorado aren't able to
be in Colorado because of the altitude, but apparently many of us
who live here are able to stay here because we are used to it. As
far as theories about my elevated pressures, there has been no explanation.
Cheryl:
Do you have any other known medical problems?
Earl:
I have absolutely no other known medical problems. I was a weight
lifter and a very healthy person before PH. To this day, PH is the
only medical problem I have. Oh, the life I could be leading if
it weren't for PH!
Cheryl:
What did you know about PH at the time of your diagnosis and what
did you subsequently learn? This was before the Internet was widely
used.
Earl:
I had never heard of PH before diagnosis. Pulmonary Hypertension
was much more rare in 1992, which was before the diet drug problem.
At the time, I wasn't using the Internet. I'm not sure how much
info the Internet would have provided at that time and information
elsewhere was limited. I didn't know any other PH patients in my
area for almost two years. Actually, I felt very alone those two
years. The doctor's office had some literature about PH and I found
little information about it at the library. The information was
nothing like what is available today. Aside from what I learned
from my doctor, I knew little about PH.
Cheryl:
Did you have the support of your family and friends when you received
your diagnosis?
Earl:
My wife was my biggest support of all. She was very active in helping
me cope with the PH. I couldn't have had a better companion than
her. Unfortunately, having to deal with it so much may have helped
lead to her becoming ill herself. She eventually left the family
and divorced me four years after my diagnosis. I raised the kids
by myself after she left. My parents and in-laws were also very
helpful. I had plenty of support and encouragement. My many friends
were also very supportive, although I'm not sure if they really
understood what I was going through.
Cheryl:
How much do your children know about PH? How much have you told
them?
Earl:
I told my children all about my situation after I was diagnosed
and what exactly was wrong with me. My children, two sons 11 and
9 and a daughter 6, were young at the time but knew I had a bad
health problem. I tried to keep everyday family life as normal as
possible to help alleviate any anxiety they may have developed from
my condition. Over the years, they have learned more about PH seeing
what I have gone through first hand. My oldest son was especially
helpful in looking out for me and that I didn't overdo it when I
was being too active.
Cheryl:
PH can run in families. Did you and your doctors discuss this possibility?
Earl:
We discussed the possibility of PH being in my family but it appeared
I was probably the only one affected. When my wife and I went to
the International PH Conference in Atlanta in 1996, we heard more
about familial PH and decided to have all three kids checked for
PH. They had echocardiograms done at Children's Hospital in Denver
and all came out okay. I now know what to look for in my children
knowing this could run in the family.
Cheryl:
Do you think PH has compromised your ability to parent in the way
you would like?
Earl:
Very much so! I was always very active with all three kids, especially
my younger son. I kept them physically active all the time. When
I was diagnosed, most of the physical activity came to a screeching
halt. I was still able to do things with them, but not like I would
have preferred. I was still able to help them with homework like
always.
Cheryl:
You've recently been weaned off Flolan. How long were you on Flolan
before you started to see some improvement in your pressures / cardiac
output?
Earl:
I was on Flolan a few weeks when I started feeling a slight improvement
and some of the symptoms of PH started lessening. After about six
months on Flolan, I had gone from a high pressure of around 100
down to a very surprising 35. I had occasional echocardiograms for
the next few years to check on my heart. I continued to feel better
over the years but the most surprising result was yet to come. In
my last heart cath which was performed recently, my resting pressure
was 19 and cardiac output was 7.5 liters. The normal range for resting
pulmonary arterial pressure is 20-25 and normal for cardiac output
is 5-10 liters. In other words, I am practically normal.
Cheryl:
Many of us wonder why we don't feel better when our pressures drop.
Was there a lag time between seeing the numbers drop and feeling
an improvement in your level of energy / stamina?
Earl:
It's hard to say how long it took to start feeling like I could
do many of the things I did prior to PH after the pressures dropped.
I was more able to be active after about a year on Flolan. It was
a slow process but in the nine years I have been on Flolan, I have
eventually built up my energy and stamina and been able to do much
more in my life.
Cheryl:
Did your doctors tell you Flolan was a lifelong commitment or did
you have the expectation that you'd be pumpless someday?
Earl:
Considering how wonderful Flolan was working for me, I felt in my
heart I would live a long time and someday I would be off Flolan.
At first, the doctors were telling me Flolan was a bridge to transplant
but it worked so well, it became part of my life. I have heard of
many patients being weaned off Flolan. Doctors in different parts
of the country have used different methods of weaning patients onto
other drugs. Dr. Badesch is conservative in his methods and brought
my Flolan dose down very slowly. I think it was good to go slow
so we could see the affects of the new drug I started taking to
replace Flolan. I should also point out a major reason to transition
to an oral drug was because of the many line infections I endured
during the nine years I was on Flolan. The infections mostly occurred
at the beginning of the treatment, but the danger was still there.
Some people seem to be more prone to infections than others.
Cheryl:
Can you tell us if you're on Tracleer now and how long that course
of treatment might last.
Earl:
Yes, I am on Tracleer now along with Diltiazem, Coumadin and oxygen
at night. Tracleer has been great for me and I expect to remain
on it indefinitely or until any problem might arise with my liver
tests. When I was transitioning from Flolan to Tracleer, I was always
prepared to stay on Flolan if I had a problem with my liver from
Tracleer. If a problem does come up, I would be prepared to return
to Flolan. Flolan was and is a miracle drug for me and I will always
remember that.
Cheryl:
Do you still and perhaps will you always have a diagnosis of PH?
Earl:
Even though my pressures and cardiac output are normal, I still
have PH and will always have PH until a cure is discovered.
Cheryl:
And today, how do you feel physically? Do you have normal energy
and stamina for a guy your age?
Earl:
I still cannot exercise or exert very much because I get tired after
a while. If I overdo in one day, I am very tired the next day. Otherwise,
I feel great and am able to do many things I couldn't in recent
years. The symptoms I had in the beginning, such as chest pain,
shortness of breath, heart palpitations, racing heartbeat, and not
being able to walk up inclines are mostly gone but I still feel
weary from exertion at times, enough to remind me I am still sick.
Cheryl:
You must know it's every PH'ers dream to have normal pressures again.
You're one lucky guy. Is there a new Earl? Have you changed a lot
over the years because of your PH experience?
Earl:
The most noticeable way I have changed since I developed PH is how
I have become more relaxed and serene. Stress was a very bad thing
when I was first diagnosed and in a way still is. Over the years,
I have learned to relax much more. If there is something good that
has come from having PH, it is that I am more at peace with myself.
Cheryl:
What would you like to say to others aspiring to be pumpless someday?
Earl:
Having to be attached to that pump every minute of every day, having
to mix medicine every single day, straining to keep that site clean,
keeping the tubing tucked away, explaining to others what it is
all about, along with so many other inconveniences like jaw pain,
leg pain, and diarrhea, Flolan can be a real pain sometimes. It
was a dream of mine to be off Flolan so I could feel that much more
normal again, but not once did I take my Flolan for granted. If
you are on Flolan and wanting to transition to another drug, take
your time! It must be done right and it needs to be the right thing
to do. As hard as it is to diagnose PH, getting the medications
correct can be just as difficult. Sometimes, it can also be hard
to tell the difference between side effects of meds and symptoms
of PH. Going from one drug to another, especially a vasodilator
to an endothelian receptor blocker, makes it that much more tricky.
With all that said, be careful if you decide to transition off Flolan.
I will always be a fan of Flolan because it has been the best medicine
available for me. I am now hoping Tracleer will continue keeping
me as healthy as Flolan did. Being able to be pumpless has been
a blessing. If Tracleer ever fails me, it would be a blessing to
be able to go back to the pump, jaw pain and all.
October 1, 2002
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