Pulmonary Hypertension (PH)

Pulmonary hypertension (PH) is increased pressure in the pulmonary arteries. The arteries carry blood from your heart to your lungs to pick up oxygen. PH isn’t a disease by itself, but rather a result of an underlying condition.

Nicole has a rare form of PH called pulmonary arterial hypertension or PAH. PAH affects about 140,000 people worldwide. I believe it is probably more than this. The National Organization of Rare Diseases (NORD) has classified PH as rare. The criteria I believe for this is under 250,000 but I’m not certain of this.

In PAH the pulmonary arteries will become too narrow. The process puts added pressure on the right side of the heart. Over time, this can cause the right side of the heart to become larger and the walls of the heart to thicken. In a normal healthy person your pulmonary pressure should be under 25 when the pressure becomes over 30 you are diagnosed with PH.

With less blood flowing into the lungs and less oxygen making it to the rest of the body symptoms will include dizziness, shortness of breath, persistent cough and fatigue. Other symptoms can include chest pain and swelling of the ankles and legs.

The disease can develop slowly so you can have it for years and not know it. This makes it hard for doctors to diagnose. Many people may go through years of testing to rule out other conditions before getting diagnosed. Misdiagnosis is also very common.

There’s two common classification systems for PH. One is to distinguish PAH from other types of PH and the other is to describe the severity of the disease.

The World Health Organization (WHO) divides PH up into five groups: PAH makes up the first group. This is the group that Nicole falls in. PAH which has no known cause is called primary or idiopathic PAH. PAH which has an underlying disease or cause is called associated PAH or APAH. PAH which is inherited is called familial or genetic PAH. Nicole has associated PAH as hers was caused by her congenital heart defect.
 
The New York Heart Association (NYHA) created a system to classify the diseases severity. This is used to monitor the disease progression.

Class I: no symptoms with physical activity.
Class II: some symptoms with ordinary activity and slight limitation of physical activity (example would be shortness of breath caused by two flights of stairs, walking uphill, walking fast, gardening)
Class III: symptoms with less than ordinary activity and increased limitation of physical activity (example would be shortness of breath caused by one flight of stairs, light cleaning, golf, power push lawn mower)
Class IV: symptoms with any activity, possibly even at rest (example would be shortness of breath caused by rest, dressing, showering, walking at home).

I know that they classify these as mild, moderate and severe but I don’t know how they divide them up. Nicole is somewhere between Class III and Class IV and it is considered severe.

When I spoke in January to our monthly homeschool group meeting about Nicole and her illness I had the ladies do an experiment to illustrate what it feels like for Nicole to breathe. Feel free to try this at home.

Get a regular drinking straw and put it in your mouth, plug your nose and breathe through the straw for a minute. This illustrates what it is like for Nicole to breathe normally. Now get a coffee stirrer and do the same thing. This illustrates what it is like for Nicole to breathe after physical activity.