What is Pulmonary Arterial Hypertension (PAH)?
“Zebra is the American medical slang for arriving at a surprising, often exotic, medical diagnosis when a more commonplace explanation is more likely.”
Have you ever heard of Pulmonary Arterial Hypertension? Or more commonly PAH?
PAH is high blood pressure in the lungs and this causes extra stress on your heart. It is often called a medical zebra — this is something that looks and sounds like something more common but is actually something quite rare and unique. Each PHer (a name for someone with PAH or PH) has a different journey and outcome — much like the stripes of a zebra.
When I was initially having problems (you can read about that here), I brushed it off as being out of shape… but when I was having chest pain and what I thought was a panic attack, my symptoms were brushed off as anxiety.
This goes back to being a zebra — why would someone who looks healthy have a rare, complex life-threatening illness??
This picture was taken about 10 days before I was admitted to the hospital. A 32 year old wife, mother and new puppy owner. I did not look sick but I couldn’t go up the stairs in our home without nearly passing out.
In PAH, the pulmonary arteries become stiffened and narrow over time. As a result, the right side of the heart, a normally low pressure system, has to work hard to pump blood through. Eventually, this compensation leads heart failure.
Below you can see the difference between a normal heart (specifically looking at the right ventricle) and a heart with PH. You can also see the difference in the arteries. The diagram with PAH has constricted arteries, this is what causes the heart to enlarge.
The most common symptoms — like I said earlier – can often be mistaken for other more common medical problems. Common signs and symptoms include:
Lightheadedness or dizziness
Fatigue
Chest pain
Shortness of breath
Leg edema (or swelling)
Blue lips and/or fingernails
Early detection is difficult and challenging. It is easily mistaken for typical illnesses like anxiety or asthma. PAH can take up to 2 YEARS to diagnosis!
How are you diagnosed?
The gold standard for a PAH diagnosis is a right heart catheterization. It is the accurate tests to get a definitive diagnosis for PAH. A small catheter is inserted through a large vein in either the groin or the neck. The cath is then passed up through to the heart to measure the blood pressure in the lungs and right side of the heart.
Other tests include blood draws, chest x-rays, ECG, echocardiograms, pulmonary function tests, exercise tolerant tests and nuclear scans.
This picture was taken during my pulmonary function test
What does treatment look like?
Thankfully I am currently ONLY on oral meds. As of today, this is my current pill doses. There are combinations of pills for the PAH and the side effects.
There are other treatments available — those include inhaled, subcutaneous (infusion), intravenous, stem cell therapy, research and clinical trials and lung transplantation.
New treatments are coming out all the time! I can only hope I continue to improve!
Now — going and trying to research this on Dr. Google is NOT recommended. Instead — your best bet is to look up information from the Pulmonary Hypertension Association.
Advocate for yourself, if something doesn’t feel right, even if you think you’re otherwise healthy, push for answers!
Currently there is no cure for this progressive disease. I hope to one day see a cure for this terrible disease — but until then I’ll keep phighting. Phighting for myself, my family and phriends who have become like family!
Help me raise awareness by sharing my story!
—Chels