This was something I made a while back. Since then I've learned so much more and I've made a few new friends so they may not be aware of what I have. Unfortunately, my condition has gotten worse since then but there's still hope, especially in the coming years. Now, allow me to educate you about:

Pulmonary Arterial Hypertension (PAH for short)

What is Pulmonary Arterial Hypertension?
Pulmonary Arterial Hypertension is an increase of blood pressure in the pulmonary arteries (the arteries going FROM the heart TO the lungs). Because of this, the right side of the heart (the right ventricle to be exact) has to pump harder against the pressure. As a result, the right ventricle becomes enlarged and overworked. This will eventually lead to cor pulmonale or right side heart failure.


A normal heart-lung vs a PAH heart-lung.

What causes PAH?
One of the causes of PAH is Ventricular Septal Defect or VSD for short. VSD is a birth defect in the heart. People with untreated VSD have a hole between the right and left ventricles of the heart. My theory or guess is that blood coming FROM the lungs TO the left ventricle is overflowing back INTO the right ventricle through the hole. Because of this, the right ventricle now has to pump harder to push all that extra blood into the pulmonary arteries and the pulmonary arteries react by increasing the pressure. It becomes a loop from there, thus creating PAH.
To cure VSD, you'll need an open heart surgery to patch that hole. I had an open heart surgery when I was 8 or 9. So, my VSD is gone and now I have a scar on my chest. But this does not solve that blood pressure in the pulmonary arteries.

Picture of normal vs VSD heart:


What does it feel like living with PAH?
A living physical hell. Well, at least at the later stages. A pretty good analogy normal people can understand is living at a very high altitude. You know how there's less oxygen in higher altitudes and it makes you more tired because you're not getting enough oxygen? It's like that but minus the cold temperatures. Anyways, with PAH, you'd tire out more easily compare to others. Your heart will pound more frequently like after a long run (even if you just ran a short distance). Up the stairs? Call it a mountain hike. It's steeper as PAH gets worse. Around moderate, you'd sometimes feel a random dull thud on the left side of your chest.

In later stages like between moderate and severe, you'd feel a numb spot inside the left side of your upper chest. You'd also feel your left arm being numb sometimes. Sometimes you may feel like passing out faster if you sit down for too long.

In the latest stages, you may need to have oxygen tank and oxygen tubes to live. An example is a singer named Chloe Temtchine (Youtube link). She was diagnosed in 2013 due to a congestive right heart failure and is now living on oxygen to live. Even in not so later stages, oxygen tanks may be needed in air travel due to less oxygen concentration in the airplane.

To help alleviate some of the symptoms is to walk around a little bit (but not too much). Also, rest if you need to. Don't strain yourself too much. Don't lift heavy objects (despite what my doctor said, I still carry 25 lbs/11.3 kg sacks of rice. Hey, if I can carry it, I'll do it. -stares at Haru-). (Unfortunately as of 2019, I can't be as stubborn anymore, though I'm still capable of fighting back when needed).

Treatment
Unfortunately, there's no known cure for PAH other than a heart and lung transplant for the most severe cases. There's only treatment to slow down the worsening until another cure is available. One common treatment is Revatio (Sildenafil) or Adcirca (Tadalafil). You may have heard the phrase, "Call a doctor if IT last for more than 4 hours". Yes, that very same pill is used to treat PAH. It just has a different name for PAH users. How does it work? It increases blood flow to the body by relaxing the blood vessels (or vasodilation in medical terms). Sildenafil is taken 3 times a day while tadalafil is more potent and is taken once a day. One of the side effects of the medication is what the TV ad says about it. Being female of course, this side effect does not apply to me.
Another form of treatment is Letairis (Ambrisentan). Letairis is an endothelin blocker (endothelin is a protein that causes blood pressure). Well, block the protein that causes blood pressure is one treatment. The risk of Letairis is liver failure so as a result, you'll need a blood draw every now and then. As of 2017, the blood draw is optional.
Aspirin is also another treatment. Aspirin is a blood thinner so your blood should flow more easily (and you'd bleed easier too).
In the later stages, Prostacyclin is another drug used as a vasodialator. It's much more powerful than Sildenafil or Tadalafil but Prostacyclin doesn't last very long and decays under a minute (or about 46 seconds to be exact). As a result, some people have Prostacyclin injected into them in their neck vein (gory and painful, I know). Thankfully (as of 2020), I'm not in that stage yet where I need Prostacyclin. There is a pill form but this lowers the effectiveness of Prostacyclin.

My personal treatment is to get plenty of exercise (but not too much) everyday to keep your heart strong and help keep your blood flowing. I did learn that lack of oxygen (hypoxia) does speed up the worsening of pulmonary hypertension so it's good to get some fresh air everyday.
Avoid salts and sodium. Salt's bad for you. It increases blood pressure. (Thankfully, I'm taking a break from Overwatch).
Garlic. It taste and smells bad but it lowers cholesterol and blood pressure. Put a clove of garlic in your water. It'll taste bad but it's good for you.
Another treatment that could work is to eat anti-inflammatory foods like tomatoes, olive oil, green leafy vegetables (spinach, kale, and collards), nuts , fatty fish (fresh ones, not the canned ones. The canned ones have sodium), and fruits such as (strawberries, blueberries, cherries, and oranges). Here's the source of the anti-inflammation foods: Anti-inflammatory foods. The reason why anti-inflammatory food is down below.


Possible cures and new treatments

Every now and then, I look up look and research any potential cures for pulmonary (arterial) hypertension. Even though I'm not a doctor or real researcher, I believe that if more people knew how exactly PAH worked, we can have more people and brain power to look for the cure. That's why I made this blog; to simplify (or dumb down) and explain how PAH works. Now, before I introduce you to one of the things I found, allow me to explain some technical stuff. Oh, don't worry. I'll make this simple as possible and I have pictures.

Take a look at these pictures:


The picture above is a cross-section of a healthy artery. However, this is what a pulmonary hypertension artery looks like:


Gruesome, right? Allow me to explain. As I've mentioned earlier in the blog, the right ventricle is working extra hard to pump against the pressure in the pulmonary arteries. I left out one detail. Do you see that layer called the endothelium in the picture above? In pulmonary hypertension, the endothelium gets eroded away by the very high pressure in the pulmonary arteries. So, what's the big deal? Well, the endothelium produces a chemical called nitric oxide or NO. Nitric Oxide is a signaling chemical that is sent to the smooth muscle cell layer. In the smooth muscle cells, nitric oxide gets converted into another chemical called cGMP. cGMP tells the smooth muscle cells to relax and open up the blood vessels more (or vasodialate). This keeps going until another chemical called PDE5 stops the cGMP. This obviously tells the smooth muscle cells to stop relaxing and close up the blood vessels (or vasoconstrict).
Let me summarize all that. Endothelium makes nitric oxide, nitric oxide turns into cGMP, cGMP tells the smooth muscle cells to relax and open up, PDE5 deletes cGMP, which cancels out the entire process.
Now, as you may have noticed, the endothelium gets worn away in pulmonary hypertension. So, doesn't that mean there's less nitric oxide to start all that process? Correct. This is one of the fundamental causes of pulmonary hypetension. One of the other fundamental problems is the smooth muscle cells. Without the endothelium layer, the high pressures are now trying to erode the smooth muscle cell layer away but the smooth muscle cells respond by inflaming/become swollen and adding more smooth muscle cells (proliferation), further increasing the pressure in the arteries. This vicious cycle is what makes pulmonary hypertension nearly impossible to cure (that and it's deep in the heart and lung area). There is hope.

In late 2018, I learned about a gene called BMPR2. BMPR2 stops this vicious cycle by limiting the growth/proliferation of smooth muscle cells and help the endothelium layer grow back. For some odd reason (I don't fully understand yet), this gene is inhibited/deactivated in some people with pulmonary hypertension.

Around Autumn 2019, I learned about a clinical trial drug called Sotatercept. Sotatercept is a ligand-trapper, or in other words, a chemical signal interceptor. Having a broken BMPR2 gene causes a bunch of signaling chemicals (called ligands) to tell the smooth muscle cells to become inflamed/swollem and proliferate. Sotatercept stops those ligands before they reach the smooth muscle cells and cause all that mess. Sotatercept also helps repair the endothelium layer. Unlike the drugs I mentioned in the Treatments section, Sotatercept targets at one of the fundamental causes. In other words, Sotatercept isn't just a simple bandage for pulmonary hypertension. It has the potential to cure it. I have talked to a few people in the trial and they said they felt their condition improve so there is that strong promise. In fact, I have such high hopes for Sotatercept that I made a 5-7 year plan around it. I do hope I can survive long enough to have the drug given to me.

If you've managed to keep up with everything I've said here, congrats. You know more about pulmonary hypertension than my pathetic (Baby) Boomer "Godmother" of a trauma nurse, who has a medical background, somehow cannot comprehend the severity of my pulmonary hypertension and instead says I'm faking it to avoid working while also insulting my mother at the same time a group of "relatives" in my family.
------


Well, that's all I can share. Don't give me any special treatments because I'm still strong enough keep up with normal people. Strong enough to walk a mile without rest and can carry rice sacks. I've managed to survive on my own for 2 years with only my wits and without help from family. Even at this point, I'm still capable and not a pushover.

For more information and resources for Pulmonary Hypertension, check out http://www.phassociation.org.