(music). Frequently under-diagnosed and frequently
under-treated, two phrases to describe a rare life-threatening condition we're about to
discuss in our Behind The Mystery Rare and Genetic Series. Our guests this morning are
Dr. Franck Rahaghi, a Pulmonologist with Cleveland Clinic Florida, and Jessica Armstrong, who
is a patient and a rare disease advocate working with the Pulmonary Hypertension Association.
Good morning to both of you. Good morning.
Doctor, I want to start with you. We're talking about a disease that is called Chronic Thromboembolic
Pulmonary Hypertension, CTEPH for short. What exactly is it?
So CTEPH, or Chronic Thromboembolic Pulmonary Hypertension, happens when perhaps clots form
in the legs, they go into the lung, and sometimes they don't go away. They get stuck, and this
causes patients to feel short of breath, dizzy, having chest tightness. And that's when they
have to really be aware of this, that they're not supposed to keep having these symptoms
after a few months of treatment. And if they are, they should seek help because this could
be CTEPH, meaning the clot is stuck and the pressures are remaining high. Now, about 1
in 25 patients with acute pulmonary embolism, or 3.8%, may develop this condition.
And the symptoms, the shortness of breath, the tightness, the difficulty breathing, they
look like so many other things. Myself, and a lot of other patients that I know, have
been misdiagnosed first with asthma or COPD. So it's so important to ask these questions
and to really keep pushing if you don't think you have the right answer.
And how is it diagnosed because you're giving me some symptoms that I can actually think
of some people that I know that feel that day in and day out? So, how is this one diagnosed?
So there's really two sets of people. There's two ways to get to this diagnosis. The most
important one, and the most prevalent one, is patients with acute pulmonary embolism.
After you had a pulmonary embolism, if you're still having symptoms and it includes shortness
of breath, you should make sure that they're checking the pressures in your heart to see
if there is pulmonary hypertension, if the pressures are high. Now the other set of people
are actually people who do go to their doctors because they're short of breath. They do or
do not know if they have a pulmonary embolism. They're found to have pulmonary hypertension,
but the work-up of the pulmonary hypertension is not complete. So people forget to look
for clots in the correct way, which is with a VQ scan, or a ventilation/perfusion scan.
So even in some centers the work-up is not done completely.
Exactly and then, of course, the patient leaves there not knowing what they have. Now Jessica,
let me bring you in. I know you were diagnosed with CTEPH, and you were diagnosed in a very
far-away place. Tell me what happened, and it was frightening moments for you I think.
Yes. So, I was serving in Afghanistan and I suddenly collapsed.
Oh! I was taken to a hospital and found to have
five pulmonary emboli. So they medically evacuated me from Afghanistan. They took me back to
the U.S. where they were treating the blood clots for a number of months, but I wasn't
improving. I had consistent and increasing shortness of breath.
And you have no idea. Yeah. I couldn't climb a flight of stairs.
I kept passing out. I was dizzy all the time. So, I got orders to go back to Afghanistan
and I decided to seek a second opinion. So I went to see another doctor who actually
did the ventilation/perfusion scan and a right heart catheterization, and diagnosed my pulmonary
hypertension. At that point they told me I had about five months left.
Five months left to live? Five months.
How old were at the time? I was 29-year-old.
Were you petrified? I was. I couldn't even imagine being in that
place. Because obviously before then nothing was
going on. Just suddenly this happens. I know you have a strong message here. Your story
is so powerful about letting viewers out there know that even if you have these symptoms
and you think it's nothing, you want to tell them what?
The first thing is keep asking questions. I made it to a good center that had expertise
specific in my disease. They knew what they were doing and they were able to improve my
life very much. I'm doing wonderfully today. So trust your body, trust your instincts,
and ask questions. If you've had a blood clot and you're not getting better, especially
in your lungs, and you see that progressive shortness of breath. You're tired all the
time. You can't climb a flight of stairs. Ask the question because it might save your
life. And I have more questions for both of you.
So stay right there please because when we come back we're going to discuss more about
what support is available for patients with CTEPH and caregivers as well. So stay right
there. (music).
(music). Welcome back, everyone. We've been discussing
a rare and life-threatening condition called Chronic Thromboembolic Pulmonary Hypertension,
or CTEPH. Rejoining us are Dr. Franck Rahaghi, a Pulmonologist and, of course, Jessica Armstrong,
who is with the Pulmonary Hypertension Association and a patient as well. Jessica, let me bring
you in now. I know it's important to talk about advocacy groups. You are part of one.
Tell me why it's important. The Pulmonary Hypertension Association does
so much to connect patients with resources. We have 245 support groups all over the country.
I can't tell you the impact it has the first time you meet someone else who understands
your story and your experience exactly. Because you feel so alone, right?
Exactly. It's such an isolating experience. What is this thing? I've never heard of it.
Am I dying? What's happening? You need support. We've got educational resources for patients
and medical professionals. We have a lot of events. We do a conference every other year,
and I really encourage anyone who's interested in learning more in visiting our website which
is PHAssociation.Org. And Doctor, you see many patients who, obviously,
are diagnosed with this or maybe they don't know what they have like Jessica is telling
us right now. Advice for patients out there? Yes. Definitely if you have a history of pulmonary
embolism, to make sure you're evaluated for pulmonary hypertension. If you have been diagnosed
with pulmonary hypertension, to make sure that you've been screened with a ventilation/perfusion,
or a VQ, scan that is very sensitive for Chronic Thromboembolic Pulmonary Hypertension. And
lastly, if you finally have a diagnosis of CTEPH, to make sure that you have the advice
of an experienced group to know whether your embolism is operable because that's really
the best way to go about helping the patient. So, Doctor, it's important to note that it's
a manageable and potentially curable disease. Yes.
Jessica, resources out there for patients that maybe want more and need more to understand
more? PHAssociation.Org for sure. CTEPH.com has
some patient information as well. We've got printed materials that we can send you for
free both in English and in Spanish. And we're celebrating CTEPH Awareness Day on November
17th. So there'll be a lot of information out there about that. Please check it out.
I'm on Twitter and Facebook, and I'd love to connect with anyone who has questions.
You really want to spread the word don't you? Absolutely.
Well then you can, let's see, I'm going to read it out loud. You can "Bust CTEPH". There
you go. I like that. Doctor, last-minute thoughts for anybody out
there. Just really inform yourself. Inform yourself
and make sure you're getting help from professionals that are familiar with the disease.
Thank you so much for your time. I do appreciate it. And for viewers out there who would like
to learn more about CTEPH, where do they go Doctor?
They can go to CTEPH.com. That's C-T-E-P-H dot com.
Good to clarify. Thank you. And, of course, for more information on this disease, you
can visit CTEPH.com. That's C-T-E-P-H dot com, or our website, TheBalancingAct.com.
I love my bracelet. I'm going to wear it for you.
(music).