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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.

Echo

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.

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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.

PH

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.