“Slow Down.”

Having a rare disease where no one knows what’s wrong in the first place is frustrating. A word in correlation with my diagnosis is “idiopathic” meaning of no known origin. No top medical specialist in the world can tell me why the arteries in my lungs decided to shrink. Do you know how many times I have had to explain my condition to even doctors hoping to god they wouldn’t kill me by accident? Do you know how many people think I’m lying about how serious my condition is? So many patients are misdiagnosed, ignored, and not taken seriously for years often escaping a chance to be early diagnosed providing them with options, and yes, some people lose their life instead. Knowing what’s happening inside of a Pulmonary Hypertension patient’s body even during an “episode” could not only help you truly understand what they feel, it also could potentially save their life.

SOO, WHAT IS IT?

Pulmonary Hypertension is not high blood pressure in this whole person’s body. No, I do not relate to your mother’s, sister’s, cousin’s, brother’s friend…that would be regular hypertension. Pulmonary means lungs; I have high blood pressure in my lungs only. How? The pulmonary arteries themselves are very very small therefore oxygenated blood cannot move through them at a regular speed like blood would through a regular very open artery. If you asked an entire high school class to walk down a huge hallway then asked them to all walk down a tiny corridor – which one would they make it through faster?

pulmonary_hypertension

When the blood gets backed up in these tiny arteries (the tiny corridor) oxygenated blood is not getting to vital organs in time. The body panics, and the heart picks up the slack by pumping harder and faster thinking it can make up for this problem. Why is this bad? After a while, the heart pumping harder and faster is not a great thing. It’s only one of our most vital organs – why would it be good to over work it? As any muscle would when you work it, it grows. This may have been acceptable for the Grinch, but I’m a human and a growing heart causes heart failure. So now my lung disease is starting to affect other organs. My heart was formed normally, and was just fine until it decided to overcompensate for my deformed lungs.

WHAT IS AN EPISODE?

A lot of people might see me “slow down”, start breathing hard, or hear me tell them to “wait”. This is the beginning of an “episode” – something I have always called the very painful and terrifying moments when my body is receiving literally no oxygenated blood. Just walking around like a normal person, a Pulmonary Hypertension patient’s heart beats like we’re running a marathon. It takes A LOT of effort to pump through these tiny arteries, and get the blood to vital organs. Certain activities that you don’t even think about can upset our heart, and take it by surprise. It differs from every patient – what treatment they are on, what their activity level is, what stage of PH or heart failure they are in, how long they have been living with it…all of this plays into their daily life function.

When I decide to jump up after laying down a while (first thing in the morning, after a nap, after being a vegetable at all) my heart freaks out. My activity level just went from zero to full blast. When walking on a slight incline, or walking long distance, I can feel a dull throbbing in my head and I start to gasp. When climbing stairs, I usually can do about four to five before I have to take a deep breather. On my absolute worst days, washing my hair and applying my makeup can take hours because lifting my arms above my head is nearly impossible. So what is happening during all of these what seem like regular activities? Why am I slowing down?

As stated before, the blood is backed up in the lungs and is not moving through fast enough. My heart starts beating uncontrollably hard to push faster to start sending this oxygenated blood out to all of my vital organs. The first place where I am NOT receiving oxygen is my brain which is why the dull throbbing headache starts. My vision blurs, along with my hearing, I can’t communicate very well, and yes I’ve been known to become combative out of pain and confusion. Having no oxygenated blood in the organ that keeps you awake is kind of terrifyingly painful. If I am even still awake at this point, I usually cannot move my legs, and definitely can’t feel my arms. None of my muscles feel refreshed because they haven’t received blood flow either. This for me leads to extreme cramping, and zero movement. By this time usually I have lost consciousness. When your brain doesn’t receive oxygenated blood, no power source, it shuts off. Hopefully around five minutes later I wake up feeling shaky, and exhausted. My heart just beat so erratically out of sheer panic, and my body didn’t receive the thing that keeps us alive – oxygenated blood. Yes yes yes – we can die from this. What’s even scarier is that it does NOT take a hike to do this. It takes a flight of stairs, a walk across the parking lot, getting out of bed, lifting our arms, getting dressed…it’s just that easy.

Understanding exactly what is happening in a Pulmonary Hypertension patient’s body saves lives. Understanding our bodies helps us feel a little less like an alien too. What can you do around a PH patient who might be having an episode? Call an ambulance, provide oxygen if you have it, and provide the knowledge about this rare disease to medical professionals so they don’t accidentally kill us too. 😉

It’s not dramatic, it’s a life or death situation in the most casual atmosphere of just walking around, or getting out of bed.

Do you remember where you were, or what you were doing when you had your first episode? Do you remember the pain?

-haley.

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5 thoughts on ““Slow Down.”

  1. Beautifully written Haley. Yes…I can relate, the episodes, that feeling, you can’t stop it once it starts. It happened prior to dx in 2000, dxd 2004, still happened until 2012. I am blessed it has not happened since. I think it took that long to get better. Thank you

  2. Thank you so much Haley for writing this article on what it’s like living with PH, and you took the words right out of my mouth as I too, share this journey with you and the many others…2013 before I was finally DX. My family,friends and as you mentioned even our own DRS find it hard to believe unless you show them your cath findings. So, I’ve shared your article with them hopefully they’ll all understand me better. Keep fighting my friend.

  3. Thank you so much Haley. I wish more people understood this disease. I lost my daughter in May 2016 from PPH. Maybe if more doctors and people understood this disease I could have had more time with her. Keep fighting!

  4. very well explained. I am also idopethic pah patient. But u know what is amazng i never ever passed out. I have to bear the pain which is very difficult. My brain remain active even though saturation drop down to 60 n heart beat reaches abov 160. My current pressures are 140.z

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