Understanding the terminology of PH is key for better advocacy
Having trouble grasping the nuances of PH? This explainer can help.
When I was growing up, I noticed that my parents were very specific when identifying my illness. I didn’t just have pulmonary hypertension, I had pulmonary arterial hypertension (PAH). More specifically, I had idiopathic PAH, meaning that the cause was unknown. For a long time, these were just words to me.
It wasn’t until I was older that I began to understand the importance of each label. Through my lived experience, I discovered that these words play an important role in advocacy and education when communicating with both healthcare providers and the world at large.
While various forms of PH are shortened to this simple, all-encompassing title, several clarifying terms help to distinguish one type from another.
Persistent pulmonary hypertension of the newborn
When my peers began having children, one more than one occasion an alarmed friend would reach out to me saying, “I just found out that [so-and-so] had their baby, and it has PH.” The first time this happened, I was caught off guard. It’s such a rare illness, what were the odds, I’d wonder. Then I learned about persistent pulmonary hypertension of the newborn (PPHN). While this is also a serious pulmonary illness that exists parallel to other forms of PH, the cause, treatment, and course of the disease are different.
As I continue to come into contact with cases of PPHN in my personal life, it feels important for me to be able to make this distinction, especially when I’m discussing the topic with concerned friends.
Secondary pulmonary hypertension
PH entails high blood pressure in the vessels of the lungs. In general, it can be broken down into two categories: PAH and secondary PH. Secondary PH is a much broader category of PH with four groups under its umbrella and many subcategories. Ultimately, it includes versions of PH that are caused as a secondary outcome of a different primary disease. Some illnesses that can cause secondary PH include left-sided heart disease and chronic obstructive pulmonary disorder.
Pulmonary arterial hypertension
PAH, denoted by the World Health Organization as Group 1, and secondary PH are very similar in presentation, prognosis, and treatment. Overall, the community is often intertwined, united by many overlapping symptoms and an urgent need for continued research. Still, a distinction does exist.
PAH is a rare and isolated disease process within the blood vessels of the lungs. It results in a narrowing of these vessels, which causes high blood pressure that can eventually lead to right-heart failure. The origin of this disease is often either unknown or genetic. PAH can be discovered at any age, with a small portion of diagnoses, such as mine, being made in children.
Why it matters
More than once, when reviewing my history, a healthcare provider has commented they’d never seen a PH diagnosis at such a young age. (I was diagnosed at 4 years old.) They’d ask me what the cause was. But that question pointed to a misunderstanding of the difference between PAH and secondary PH. Given the rarity of PAH, especially in pediatrics, not all providers are aware of its existence. Instead, they’d assume I had a form of secondary PH. For this reason, creating awareness about the existence of PAH is an important endeavor, which I will always pursue in such cases.
Still, the differences are intricate, and sometimes muddled, making it a difficult topic to navigate. Even during my own health journey, the finer details of my disease classification were occasionally disputed. Often it depended on the provider. This unfortunately is common for a rare disease that is yet to be fully understood. It’s also an indicator of why it’s so important for these classifications to be discussed by patients and providers and to be further defined as progress in the field is made.
When I was in nursing school studying a chart of the different PH classifications, I remember being surprised by how much I didn’t know, despite having PAH. I also was surprised by how little information there was for my category of illness. I finally understood why my parents had been so precise with their language about my disease throughout my childhood. Simply saying that I had PH didn’t cover it; being precise helped further the advocacy and awareness that is so needed in the rare and mighty PAH community.
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