My Adcirca “Review.”

After giving much in-depth explaining on what PH is, and how we find it, I thought I would now talk about my treatment. Pulmonary Hypertension is usually first treated by a very generic version of Viagra called Revatio if Doctors think it’s even a possibility. A lot of patients actually stop reacting to it very quickly, so most doctors just skip over the whole “Revatio” option to begin with. That brings us to more high-tech, crazy strong vasodilators and other medications to keep the blood vessels dilated so we can breathe. If that doesn’t work, they might try an inhalable solution, or finally be put on a IV Remodulin Pump that is comparable to a diabetic’s pump. If the Remodulin doesn’t change things then heart and lung transplant options are discussed.

So, because my body miraculously reacted to medication, I don’t know much about experiences with the other treatments. Therefore, I would love to give a little medication “review” on what my treatment does for me.

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I was put on Adcirca and Norvasc almost six months after my diagnosis. We tried Revatio at first, which failed, and then they discovered I reacted to the others beautifully. These are very high-powered vasodilators allowing me to breathe, but causing all sorts of other issues throughout the body. My first year on Adcirca and Norvasc was absolute hell. The swelling was the first to hit. I began to notice that my feet would ache, and feel “tight.” When I looked down I didn’t even recognize my feet at the start of this swelling “adventure.” Over the next couple of months it just “grew” to be worse. My feet would swell around my shoes only permitting me to wear these open type of moccasins. The pitting edema was so bad that you could push into my feet like memory foam, and the fingerprint would remain. Not only was this extremely ugly (for an eighteen year old I thought it was the end of the world), but this was very painful. Finally, like magic, I woke up with skinny ankles, and the swelling initiation disappeared.

…Until the warm red patches started. It started in my knee’s with an extremely hot sensation. When I took my pants off, I noticed that I had a straight red line right above my knee’s, and continued right below them. It literally look like someone took a red marker to my legs, and on top of that I, myself, wasn’t warm, but my knee’s were probably a thousand degrees. I would seriously make my co-workers feel them just to make sure I wasn’t going insane. From my knees, the red sensation moved to the back of my arms, and my face which is now where it currently sits from time to time. What’s so strange is that I am physically cold, and want to cover up, but my skin is on fire. If I’m in the sun, or if my body temperature elevates, then the red patches strike! On top of the red insanity – I already have rosacea in my face. When you put this Adcirca rash over it, I look INSANE. This is why my makeup is so sacred, and is like an art to me. I have a lot of uncomfortable, and not so cute things to cover up due to medication, my natural face, and skin damage.

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For those of you suffering from “Adcirca face” I recommend a green concealer that eliminates redness! LA Girl Pro Conceal has been my absolute favorite to use so far, and yes, it’s very affordable at only $5. Use a sponge, or a brush to even the green out over the red area and you will notice how it “tones” it down. No, this isn’t a magic wand so you will still have some red. On a bad day, I recommend using it underneath foundation, and again over the foundation if need be.

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On top of the redness, Adcirca, for me, causes a lot of pain. If I ever skip a dose, then start again it’s almost like having the flu. Adcirca dilates all the blood vessels in the body – not just the lungs (kind of like how chemo kills everything – not just the cancer) therefore the skin turns red, and the vessels in even your eyes, and head are dilated too. When that happens an extreme headache follows, and for me body aches and chills. Also, my eye doctor told me that Adcirca has pretty much destroyed my vision. Every year I go we have to check on my eye pressures, and anyone around me knows I can’t see shit.

Overall, Adcirca does the job, and as long as I’m consistent the troubles will be few depending on how it wants to act up. I however wish we could find something that could cooperate with our bodies just a little more, and maybe just maybe an actual cure. 😉 Have a lovely Sunday! Check out the “Song of the Week” to flashback to a very uplifting tune, and also check out the Facebook for the NEW T-SHIRTS!!

-haley.

Textbook Breakdown Part 2: Diagnosis.

So, yesterday we covered what Pulmonary Hypertension is in itself. Now today we’re moving onto how we find PH, and who has it. If you missed yesterdays post, then click here to share or read it.

So, since Pulmonary Hypertension is happening in the lungs itself affecting the heart and internal organs, it’s not affecting much of what happens on the outside of this person. In fact, most Pulmonary Hypertension patients are young; kids, babies, teenagers, young adults, you name it. Anyone could be born with Pulmonary Hypertension. Basically, Pulmonary Hypertension hides behind a mask of youth, or “normal.”

Someone may be born with Pulmonary Hypertension, and take years to finally show it. They could be like me, attending public school, participating in physical activities, then one day feel a very unusual and horrid pain all over, and lose consciousness. Most people would either ignore it, or think they had an asthma attack. Even if this young patient was attended to by medical care in a hospital, or doctors office they would never do an EKG (checking heart rhythms) an oxygen test, or chest x-ray…why? Because why in the world would we check a very young person’s heart when they just got tired, and collapsed? When people ask why we collapsed, we tend to only be able to answer that we couldn’t breathe. People generalize those symptoms down into something like asthma. PH doesn’t have a disease appearance because it’s all internal therefore just about every person does not take the person’s symptoms seriously. A PH patient begins to think they might be crazy, are out of shape, and need to learn to keep up. Maybe they’re told that they have an anxiety disorder, or something. Basically, it is very very very rare for anyone to listen to someone suffering from PH because they can’t “see it.”

So what happens after being ignored for so long? The pressure in the heart, and lungs begins to mount and the patient begins to get worse. Not being able to walk, fainting with any physical exertion, and the downfall of their quality of life. Yes, this will even happen to kids. Pulmonary Hypertension patients can die at any time for a variety of things; blood clots, an arteries bursting, their heart rhythm becoming too chaotic, and finally their heart just stopping. If a PH patient is not taken seriously by medical professionals in time, their chances of dying increase every day.

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So what happens when a patient is actually taken seriously, and what does it take for a doctor to find PH? An EKG, and a simple oxygen saturation test should at least show some concern with a weird rhythm presenting a conduction delay, or showing the right side of the heart is working too hard. The oxygen levels should also be low. A chest x-ray, or echo should show that the heart is over sized which a normal physician would immediately want a heart catheterization done. A heart catheterization is where they insert a catheter in the femoral artery (or a vein in the neck) and move it all the way into the heart. From there using fluoroscopy (a moving xray) they can view the actual heart itself to make sure it was formed right. Then they can measure the pressures, and actually see how the heart is pumping so they get an idea of what is wrong. If the heart is formed normally, but is still working so hard to the point of failure then that tells the doctor that the pressure is coming from the lungs…Pulmonary Hypertension.

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At this point the doctor would finish the surgery, and would contact the closest Pulmonary Hypertension Specialist, or Pulmonologist to start treating the patient immediately. Depending on how high pressures are, and what shape the patient’s organs are in, the doctor would now notify the family of the terminal diagnosis, and then the patient with a very grim life expectancy.

What now? Tomorrow we will dive into treatment.

-haley.

Textbook Breakdown Series. Part One: PH Itself.

Okay loves this first week in November for Pulmonary Hypertension awareness month I wanted to do an entire cohesive series on the first steps to understanding PH to share with your loved ones,and to help everyone understand just a little better! Each day I will post information about PH breaking down the disease, diagnoses, treatment, and other things about PH that make it hard for others to understand. Here we go!

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So, to start with PH, we have to start from the very beginning; the disease itself. What is Pulmonary Hypertension? Not all the other things that come with it, but the actual disease itself in our bodies. Pulmonary Hypertension is first off, a lung disease that affects many many other things. Where is the “disease” or problem at? In the arteries of the lungs (the pulmonary arteries.) What is wrong with these arteries? Well, they are extremely small, and not large and open like they should be. Why is this a bad thing? Because these arteries carry blood, and oxygenated blood to a major part of our body (the heart) to be pumped out to the rest of our body. Think about trying to move an entire high school class down a very narrow hallway. Imagine that you have to get them through super fast, and in the exact same amount of time that it would take them to move through a very large hallway. Is that possible? Absolutely not, because this class is cramped; they simply cannot move as fast as they originally could in a large hallway. So, these arteries in our lungs cannot get blood, and oxygenated blood where they need to in time, because basically, its small, and cramped in there.

IMG_2467“IPAH” meaning Idiopathic Pulmonary Arterial Hypertension AKA fancy words for PH.

So, here is where it gets a little complicated. If oxygen can’t get where it needs to in time, how does it affect the body? We need oxygen to stay alive, and to literally do everything. If our brain doesn’t receive oxygen then it can’t send out signals to the rest of the body. It literally can’t even stay on, so it would shut itself off (AKA Black out.) Also, guess what else is not receiving oxygen? Your whole body; therefore your arms, legs, muscles anywhere feel like they’re cramping up. A person would literally hurt all over, especially in the chest, would eventually lose consciousness, and stop breathing if the oxygen supply continues to be cut off.

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So, I think we all understand that we have to have oxygen. Now because the heart is the one doing all this pumping, Pulmonary Hypertension directly affects the heart. This organ is the one responsible getting this blood through a very “crowded hallway” or the pulmonary arteries that are tiny. It can’t possibly get this oxygenated blood how fast that the body needs the blood, therefore its receiving signals saying that the whole body is hurting, the brain feels dizzy, and even the heart begins to suffer. What does it do with all this pressure mounting? It picks up the crappy lung’s slack, and works even harder hoping the blood will get through faster. This may get the body by for a while, but what happens when you start working a muscle hard? It grows…just like your biceps would. Is it good for your heart to grow? That may be good for the Grinch, but it’s not okay with anyone else. With your heart growing bigger, it gets tired, the pressure in the heart itself gets higher and higher. Eventually, the heart would start slacking on all its jobs, and then one day just stop; that is heart failure.

PH Heart

A Pulmonary Hypertension patient’s heart most of the time is formed perfectly. There is nothing wrong with the heart, until the lungs come along causing pressure in the lungs, therefore putting pressure on the heart. That by definition is Pulmonary Hypertension (Aka high pressure in the lungs.) Pulmonary Hypertension is a domino game of disease when it comes to the body because eventually all organs become affected, and fall victim to this disease.

There are different types of PH? Yes. Idiopathic, and secondary. Idiopathic means “of no known origin.” There is not something causing the Pulmonary Hypertension in this patient’s body other than they were born with it. A secondary PH patient means they have this disease, or developed PH because of something else.

Haley

Soo, who is PH affecting, and where do we go from here? Then check back tomorrow with my diagnosis breakdown. 😉 I hope this breakdown of Pulmonary Hypertension helps you understand this disease a little better!

-haley.

I Just Might Be Done Talking.

So today marks both Pulmonary Hypertension Awareness Month AND Native American Heritage month. If you’re me then you know November already sounds exhausting. Living with a disease can wear you out seeing as how it sucks the life out of your body. However, I think what is more exhausting are the people who refuse to learn, expand their minds, and actually start acknowledging such invisible diseases. Like, truly acknowledge.

A lot of you know that I somewhat retired from writing about PH simply because I felt I had fulfilled my duty. I literally had nothing else, and no inspiration left when it came to writing. When I accepted the PHA’s Young Adult Citizen award it all just somewhat felt complete, and I wanted to begin tackling other issues. To this day when I get inspired to write about PH I feel as though just the keyboard gets heavier. I honestly just feel as though when I talk about my disease to others that I’m holding conversation with a mannequin.

In today’s world we exist on terms with a sense of entitlement. If anyone gets offended by anything then it’s in our Newsfeed Facebook Trending Topics section, and everyone in the comments is participating in a keyboard war. I also feel that because of dangerous things that keep unfolding in America because of times progressing that we are all in a state of mind called “survival” and “worry about me, and my point of views only.” At least it seems that way. Most people are not shocked to find out that I am extremely democratic, and liberal. However when you say that you get called a “liberal dumbass, needy bitch, living off the government” and what ever else people can throw in your face. I get it, its political views which you are not supposed to discuss. While I may support liberal standpoints, I overall just kind of want to start a campaign that’s called, “Educate people, and let them make their own life decisions.” I guess you could say that’s what I stand for.

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So, why am I talking about politics when I started off with Pulmonary Hypertension, and Native American Heritage Awareness? Because these months in my eyes are almost useless. Don’t get offended, I still do everything I can. But so many people do not listen, and do not want to listen these days. When we say, “I have a rare disease, and I almost died because there wasn’t enough awareness” people will respond with, “Oh, wow.” You really can’t shock people with the facts anymore. But they kind of don’t want your story clogging their newsfeed, instagram, and don’t want to make time for you on published articles. When you try to educate people on why you believe in Pro-choice for the safety of Pulmonary Hypertension patients who are women who cannot carry a child, or be on birth control, they literally don’t listen. Their logic’s are firmly implanted, and they type back about how despite you wanting to adopt, or do surrogacy that you are a murderer. These days I feel like everyone sits on their own sort of pedestal…and they are not willing to open their mind. To listen to someone, to truly want to understand what they are saying, to truly want to feel what they are feeling you not only have to open your ears, but every part of your mind that has the preconceived notion that they are wrong. Actually listening to people is a wonderful, and eye opening thing.

Basically, I think when it comes to raising awareness about a disease we are not only trying to give our disease a voice, but also fighting against a set mentality among most people. I’m not saying it’s one political group, or certain types, but literally everyone. I know (seriously, I fucking know) all of you get so mad about how much I post about abortion, and politics. But we have to understand that government decisions affect how patients live…or whether they don’t. Think about a woman with Pulmonary Hypertension living in a country where abortion is outlawed, and she were to become pregnant? How fair, and safe is that? Exactly. When we fight for awareness, we are not just trying to get into people’s ears, but their minds. We truly have to completely educate them. It’s exhausting, and it feels almost impossible.

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I will continue to write a few articles this month on Pulmonary Hypertension. And I really hope that instead of just liking the post, that you are reading it, understanding it, and contemplating major things now. That’s what awareness has to do; it has to move mountains in people’s minds so that they are made aware, and help us fight. We have a lot to overcome. I guess I’m not trying to be negative; I’m just unloading some very heavy, and prevalent thoughts when it comes to November.

Song of the Week is Heart Shaped Box by Nirvana. Anyone else feel like screaming this?

-haley.