Where Do You Fit Within the PH Community?
The words I wasn’t prepared to hear went something like this: “Mrs. Cueva, it seems that you have a rare and life-threatening illness.”
My mind immediately flashed back to months earlier, when a local doctor had told me that if I had pulmonary hypertension (PH), it would be “a death sentence.” My heart broke, and I cried as my husband, Manny, held my hand beside me. Thank God the local doctor was uneducated in PH, because 17 years later, I’m still here.
Anyone familiar with PH knows it isn’t a one-size-fits-all disease. I’ve worked with three highly educated PH specialists who agree that my PH certainly doesn’t progress like how they had learned it would in medical school.
But despite where we are in our journeys with the disease, all of us patients fit right in among the PH community. I certainly do. I’m considered a long-term survivor but continue to learn and find inspiration from others within our close-knit community.
Congestive heart failure, particularly on the right side of the heart, may be indicative of PH progression. I had congestive heart failure when I was diagnosed with PH, which is common. Following multiple treatments and various ups and downs, I’d say that I’ve improved since my diagnosis. But others may progress differently.
Because PH has no cure, treatment goals involve symptom management. Stability is a positive thing in our world. But just as in life, I write my own PH journey, and I bet you do, too.
Rare Disease Day on Feb. 28 is a virtual global event. During leap years, it’s observed on Feb. 29, the rarest day of the year. This unique event has played a critical role in building an international rare disease community that is global, diverse, and composed of multiple diseases, yet united in purpose.
This year, I decided to dive into the greater PH community to ask others to share their thoughts about the topic. I interviewed two patients and one caregiver via phone and email to learn more about their perspectives. I asked them the following questions: “If your symptoms don’t fit the ‘typical’ progression for PH, where do you feel like you fit into the PH community? How does it make you feel to be a rarity among rarities?”
Their responses follow.
Mary, who has a functional classification of PH
“Since doctors caught my PH early, I have a much milder case than most. This is not the norm for most [people who have] PH. For this reason, I do feel like I am a rarity in this rare disease community. Hopefully, my PH will remain stable, and I won’t progress, thanks to ongoing research.”
Carol, who has pulmonary arterial hypertension and scleroderma
“For several years, I was misdiagnosed. When I was finally diagnosed with PAH [pulmonary arterial hypertension], I was told I had a very rare and expensive disease, and 3-5 years to live. PAH was bad enough, then I was diagnosed with scleroderma. I was sure that PAH was the disease that would take me out. Come to realize, it probably won’t — I have been pretty stable with PAH meds for over 17 years.
“I would say yes, I feel I fit within this community. There are so many of us, and many with more complications. What’s rare is that so many doctors and [others in] the medical field still know so little about PH and related diseases. If you Google PH or PAH today, you’ll still find life expectancy listed at 3-5 years. It’s frustrating that there is so much inaccurate information out there.”
Cheryl, a caregiver
“Because my daughter’s PH progression was quick, I would say we fit into the community just fine. Since diagnosis, her PH has worsened, requiring more extensive treatments. But I am grateful to the PH community for its support. As a community, I think of us all as rare, like the zebra that often represents PH.”
Cheryl makes an excellent point — PH is often known as a medical zebra. That’s because of the disease’s complexity. Its rarity means PH often is overlooked or misdiagnosed by members of the medical community.
In honor of Rare Disease Day 2022, let’s work together to increase awareness and education about PH. Although rare, like a zebra, together we are stronger.
Note: Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Hypertension News or its parent company, BioNews, and are intended to spark discussion about issues pertaining to pulmonary hypertension.