Original date: November 5, 2009
Okay boys and girls, all you faithful blog readers, get ready for a lesson in PH…
First of all, with the heart there is left side and right side involvement. This is about right side involvement – the kind I am most familiar with.
PH is known as “the other high blood pressure” – a totally misleading term. It is indeed high blood pressure , but treating it is no where as simple as popping a pill, watching your sodium, and loosing some weight. The blood pressure problem is within the pulmonary artery of the lungs. Simply stated, the heart has to work really hard against this extra pressure. It tends to enlarge (as mine has) and eventually it can lose its ability to pump (which I’m hoping mine won’t!).
PH knows no age, race, or gender. ANYONE can get it. However, it primarily strikes women in their childbearing years. It is not unusual for symptoms to emerge with or just after pregnancy, as was the case with me. Pregnancy doesn’t cause it, but if you’re going to have it anyway, something about pregnancy seems to kickstart it a bit.
Symptoms of PH include quite a list. I have experienced the following common symptoms:
Chest pain, shortness of breath on “easy” exertion like climbing a flight of stairs or carrying groceries, low energy/fatigue, dizziness and fainting, and heart palpitations.
In addition, a patient might have: swollen ankles and legs and bluish lips and skin.
This list, unfortunately, looks like a whole lot of other symptoms lists for other diseases. That is, in part, why so many people go through many doctor visits, misdiagnosis, and even years of suffering before a correct diagnosis is given. At that point, for many, the disease is at an advanced state and treatment has to be swift and intense. (More on that later)
There are some preliminary screenings for PH. It might be suspected from a chest x-ray showing the enlarged heart and/or an abnormal EKG (that’s me again!). An echo cardiogram gives estimates of pressures and you can measure cardiac output and heart size as well… but diagnosis is best done via a Right Heart Catheter (RHC). Oh yeah, those are as fun as they sound. A thin tube is inserted into the body via the groin or the neck and then they move it around and take a look at your pulmonary system. The best part – the patient is awake for the whole thing. I have seen the inside of my heart up on a screen while laying on a gurney in a freeeeeeezzzzziinnnnnggg room, being worked on by a tech who resembled Harry Connick Jr. (That wasn’t so bad!). Valium is a beautiful thing in these situations, but not always offered. After two such procedures, one drugged and one not, I assure you, offered or not, it will be politely requested demanded for the next round!
Once there is a definitive diagnosis, the fun really begins. First, you have to just wrap your head around the fact that you have an “incurrable and often fatal disease”. I think that is another post for another time…
You have to look for and/or rule out all possible causes, such as: autoimmune disorders (such as Scleroderma/CREST or Lupus – two common culprits), an Atrial Septal Defect (an ASD, a birth defect of the heart – a “hole”), and blood clots in the lungs (Pulmonary Embolism). It can also be caused by other underlying health issues like liver disease and drug use – specifically diet drugs like Phen-phen and “recreational drugs” like cocain. (Yes, they really call them recreational – it took me a while to figure out what I was being asked when they screened me. And the answer was “NO!”).
These tests are not a lot of fun. You give up vials and vials of blood. Screening for blood clots in the lungs involves a procedure where you are actually radioactive for a while after (a fact not mentioned to me before the test was just about in progress!) This also means that while driving home, you may be pulled over for suspected terrorist activity since you are emmitting radioactive waves for a while. That didn’t happen to me, but I kind of wish it had. It would have been fun to explain!
“Oh, no, officer, I’m not carrying illegal chemical explosives… that’s me your nifty little machine caught. I’m radioactive, you see…”
Back to the topic at hand, I have NONE of the underlying causes. This makes me a lucky one or two in a million who have what is often referred to as Primary or Idiopathic Pulmonary Arterial Hypertension. Yup, one in a million. How do you like those odds? I knew I should have played the lottery more…
After diagnosis, comes the treatment regime, but if you’ve stuck with me this far, you’ve probably read enough for one day! Thank you.
Treatments will come some other time in the near future… next post will be a little bit more about what it is really like living with PH. I hope you stay tuned.
(In the interest of proper citations practice, I have to give credit to www.phassociation.org for the fact in this post. This stuff is all in my head now, but most of it came at the beginning from the really good folks there)