This is a long post. If just one person reads this, though, and it helps them to not have to wait 3 years to be diagnosed with Pulmonary Hypertension, then it has been worth it.
The first time I heard about
Pulmonary Hypertension was on August 18th during my first appointment with the
pulmonologist. It was included in a list of other things I hadn't heard of that
might be causing my illness (interstitial lung disease, cardiac asthma,
sarcoidosis and PH; I had heard of pulmonary embolism because that is what
killed my brother in 1990 at the age of 47.) I knew that all of these things
were serious, so I starting learning about them. I'm not sure why it took so
long for my doctor to send me to a "lung doctor" when my main
complaint for the past 2 years had been shortness of breath (SOB), but I was
glad to finally be there.
PH seemed to fit with what
was going on with me. However, a lot of other diagnoses had fit before and I honestly
didn't want to be disappointed yet again when the doctor told me that I didn't
have x,y or z and that he didn't know why my life had changed so drastically
since November of 2011. After doing pulmonary function tests, an exam and
having me walk in the office while my oxygen saturation level was monitored, the
doctor said I would need to have a CT scan to rule out blood clots in my lungs.
That honestly was not scary to me because I've been checked for that so many
times due to my family history. He said that if there were no blood clots, then I would have to have a right and left heart catheterization procedure
to see if I had PH. I had a left heart cath a year ago so I wasn't nervous
about that, and since I had no idea what PH was, I didn't worry about it either.
My pulmonary function tests
the year before showed that I have a mild restrictive process in my lungs but
no obstructive process, so no COPD (which my mom died of in 2000, so I was glad
that was ruled out.) I do have mild Alpha-1 Antitrypsin Deficiency, which can
affect the lungs, so I knew that lung problems could happen to me. The PFTs he
did on 8/18 showed that my lungs were not moving the oxygen in the air I
breathe back into my blood (that's poor diffusion capacity). Mine was about 1/3
of normal. An MRI had already shown that my pulmonary artery is slightly
enlarged, so I knew that something was going on and all signs were pointing to
PH.
Because I really push to get
things scheduled quickly and stay on top of my care, I was able to get the CT
scans scheduled for 8/21, just 3 days after seeing the pulmonologist. The CT
scans showed some mild lung scarring that I already knew about and thankfully
no blood clots.
During these weeks I was
dealing with a lot of body wide pain (unrelated to the PH) and swelling in my
feet, ankles and legs. They basically were just huge stumps, from my toes to my
stomach, with no delineation between the three. They hurt and the stinging in
my feet was unbearable at times. I was also having to keep an eye on my kidney
status because I have Stage 3 Chronic Kidney Disease. Thankfully, there are 5
stages and my disease is mild.
Let me take a second here to
talk about the word "mild". I hate that word now. Everything I
had going on with me was "mild". Mild means that the doctors don't do
much for you and they basically wait until it's not "mild" anymore
before they get serious about talking to you or treating you. At least, that was
my experience. So even though "mild" is good, it sucks. My
cardiologist told me that my heart issues were "mild" and didn't need
to be treated and that I should see if the pulmonologist could help me figure
things out. That just didn't seem right to me. I started asking myself a lot of
questions and realized that I had a lot of questions for the doctors. My life
had changed drastically in the past 2 1/2 years and something was causing that.
There is no way I could go from being able to hike 2-4 hours at 7000-8000 feet
altitude to being SOB and having a racing heart just walking room to room in my
house.
I started believing that PH
was the illness that was causing my problems so I made an appointment with the
PH Specialist in Denver. Neither my cardiologist nor my pulmonologist told me
to do that. I did it because I was tired of not knowing why my life had been
turned upside down and I wasn't going to wait on them to tell me what to do to
get better (since they hadn't done that anyway.)
At the beginning of September, I went back and looked at the sleep study I had done the previous November. I
knew I had sleep apnea and was supposed to wear a CPAP, but it made me feel
claustrophobic and like I was suffocating so I didn't wear it. I was diagnosed
with obstructive sleep apnea in 2006. I got my first CPAP that same year. I had
never used my CPAP consistently in all that time. When asked by my doctors if I
used it, I told them how it made me feel and that I was not using it very often.
No one followed up, no one did any education, no one did any counseling and no
one referred me to a sleep disorders specialist. When I looked at the November
sleep study (that I had requested be done,) I noticed that my oxygen level was
<90% for >99% of the night with and without the CPAP. That didn't seem
right to me. I thought that oxygen levels were supposed to be above 90% all the
time. The pulmonologist had mentioned getting me compliant with the CPAP so I
thought I should look into that. I called the center where I had the study and
no one, to this day, has ever called me back. I just kind of figured that it must not be that big of a deal if no one called me back. I was very wrong about that.
One of the most fortunate
things that happened to me was getting sick around September 7th. I ended up in
urgent care for bronchitis and an upper respiratory infection. They gave me
oxygen and a breathing treatment and sent me home with a ton of meds. I called
the pulmonologists office to let them know about my sudden illness. I spoke with Linda, the doctor's nurse, for 41 minutes on the phone. She was the first
person who seemed concerned about the results of the sleep study and that I was
so sick for so long without a diagnosis. She asked me to come into the office
that afternoon so she could see me. She did a very thorough exam and asked a
lot of questions. I left the office that day with
supplemental oxygen. I could tell right away that adequate oxygen intake is a
good thing. It felt so good to breathe. She took copies of my sleep study and
my other paperwork to discuss with the doctor before my appointment the
following week. Linda really cared, and her taking the time to evaluate me has
made all of the difference in the diagnosis of my illness. Just days later, I was
set up with 24/7 oxygen at home, including using it with my CPAP at night so I
could get adequate oxygenation. On 9/12, when I had my PFTs
done again, my diffusion capacity was 2/3 of normal. That was an improvement. My
lung function wasn't as good, but I had bronchitis and the URI so that kind of
made sense.
So, at 54 years of age, I was
on supplemental oxygen 24/7 and taking diuretics to reduce the fluid overload
in my body (hypervolemia). I was feeling older than I am.
On 9/18, one month after
first hearing about PH, I was in the cath lab getting my pulmonary artery
pressure looked at. The cardiologist told me that I had PH (the pressure in my
pulmonary artery was higher than it should be), that my body was overloaded
with fluid and that I probably have hypoventilation syndrome (I breathe too
shallow) due to being overweight. It was a bit overwhelming. The pulmonologist
confirmed the diagnosis on 10/6. The PH Specialist confirmed the diagnosis on
10/7. They both told me that I needed to treat my sleep apnea, continue using
oxygen 24/7 indefinitely, lose a significant amount of weight, consider moving
to a lower altitude (I live at 6,700 feet) and definitely not travel to
higher altitudes and keep my sodium intake to <2000mg/day and my fluid
intake to <1.5L/day to combat the fluid overload. That was VERY
overwhelming.
Thankfully, I was seeing
a counselor to help me deal with being chronically ill even before I got the
diagnosis of PH. She has been wonderful and extremely helpful. I also was
dealing with the possibility of having lymphedema (causing the swelling in my
legs) and had to work through the process of getting that diagnosis ruled out.
I had to have a nuclear scan done where they inject the radioactive dye between
your toes! Thankfully, it sounds a lot worse than it is. No lymphedema, so that's one
less thing to worry about.
I have had a lot of questions
for all of my doctors since being diagnosed with PH. There are 5 different groups
of PH. It looks like I am in groups 2 (PH due to heart issues) & 3 (PH due
to lung issues, which are sleep apnea and hypoventilation syndrome). Group 1
is the only type of PH that has specific medications to treat it. It is also
the most rare and dangerous type of PH. Every single patient's
experience with PH is different than every other patient's experience with PH.
There are similarities, but everyone has something unique to their illness. That
makes it difficult to treat PH. In my case, since my PH is due to underlying
conditions, those underlying conditions have to be treated to make the PH get
better. I have to treat my sleep apnea, my obesity, my fluid overload and my
oxygenation issues. I'm hoping that if I treat those things, that the PH will
get better and/or go away completely. I may have to deal with PH the rest of my
life. To what degree, I don't know.
I have spent a lot of time on
the phone with the PH Specialist's nurse. I wasn't supposed to see him again
until April, but they want to follow up with me. So, I am going back up to Denver
next week. The difficulty with having PH due to other illnesses is that no one
is quite sure who is supposed to treat and follow up with the patient. It is a
heart disease and it is a lung disease. It is complicated. Thankfully, the
specialist is going to make sure that I get my questions answered and that I
understand my PH as best as I can.
David and I are feeling
overwhelmed right now. We are considering moving to a lower altitude if it
would be the best thing to do. We still have so many questions. I am hoping
that my appointment with the specialist next week sorts a lot of this out. I am
looking at doing a Pulmonary Rehab Program 3x/week for 3 months to get me
active again. I have basically been sedentary for the last 3 years. I'm hoping
to get a portable oxygen concentrator (a machine that makes its own oxygen) so
I don't have to bring oxygen cylinders with me everywhere and have to
constantly be refilling them. I want to be mobile and get out again. I want to
understand what my flavor of PH means to me. What can I do to feel better? What
can I do, if anything, to halt/reverse/eliminate PH in my life?
I am going to see the
ENT/Allergy doctor this afternoon to help with the constant nasal congestion I
have. That keeps me from wanting to wear my CPAP mask at night. Wearing oxygen
full time affects your nose and sinuses. What can I do to make sure I comply
with wearing oxygen 24/7? I am going to see the sleep disorders specialist
tomorrow. I know that being compliant with my CPAP, treating the sleep apnea
and reducing the amount of time that my oxygen level is below 91% is crucial. I
need to know if I do have the hypoventilation syndrome. I'm not sure if he
would diagnose that or not but I'll ask him. And I definitely need to sleep
better. Being rested is important, too.
I have a lot of huge
lifestyle changes to make. I'm working on making those changes every day. I am
looking at every option from surgical weight loss to moving from my beloved
Colorado back to sea level (or as close as I can get). Being diagnosed with PH
has changed my life. It has changed David's life, too. I am sad knowing that I
cannot go back to Waldo Canyon to hike. My goal since I got sick 3 years ago
was to get back to Waldo Canyon to hike again. It's at 7000 feet, though, so that
is not an option right now. I can't go up to the summit of Pikes Peak, 14,114
feet, and get a "high altitude" donut (they just taste different up
there!). Life is different now. I'm relieved to finally have a diagnosis so
that I can move forward with my life, but it's slow going right now. I know
that, quite honestly, one of my options is to do nothing and just get worse or
at least, no better. I don't think I will do that, though. My pulmonary rehab
program evaluation is next Thursday. I may be able to start the program on
Friday, Monday at the latest. It will feel good to be active again.
The PHA website has been an
invaluable resource since hearing about PH. Not only do they provide lots of
information but they also provide a lot of support through online chats,
telephone support groups, one on one email and telephone support and
educational resources. I can't thank the people and other patients on the PHA
website enough for being there for me and helping me to navigate through all
this since being diagnosed with PH.
Thank you for reading this
very lengthy post. I hope it helps you to understand the journey to a PH
diagnosis and the affect it has had on my life. I hope it helps someone who
needs to be diagnosed or has been recently diagnosed with PH.
Written by Samantha-Anne Wagoner
Written by Samantha-Anne Wagoner
