Where Do You Fit Within the PH Community?

Jen Cueva avatar

by Jen Cueva |

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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.

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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.


Johan E. avatar

Johan E.

I was diagnosed way back in 2010, with no caregiver and no after care. The cardiologist just basically said in a cold voice that I have pulmonary hypertension. I did not even know what it meant! So my wife did some research, and also did not tell me about it (she was too scared about what she read), until I listened to a radio show one morning where they spoke about PH. And I realised exactly what was said and what the prognosis is! Needless to say I was shocked. But since then I can say that my PH has improved. I believe mainly due to CPAP treatment for apnea, and not taking the disease lying down. 12 years down the line and I am still going strong, with no slowing down, no medication, and hopefully many years left...

Jen Cueva avatar

Jen Cueva

Hi Johan, I'm grateful that you mention that your PH has improved with your BIPAP. Hopefully, that and any other treatments your PH team has you on will offer relief from PH symptoms. It certainly can be a scary diagnosis, but we can see improvements with proper care, as you mention.

Keep on going strong and PHighting PH as you have been. If you haven't yet joined us, we would love to join the PH News forums. I am a co-moderator there and enjoy the support that everyone offers.

Thanks for reading and offering your feedback. Take care and stay safe.

Sally Hoffman avatar

Sally Hoffman

In Oct of 2018, I climbed a flight of stairs and collapsed on the top step. I quickly saw my PCP who immediately recognized that I might have PAH. The testing began at once and by January a RHC confirmed the diagnosis. During the Cath I learned that I was among a very small lucky group of people who responded well to Nitric Oxide. I was placed on Adcirca. My educational background, and career, as well as a personal family history always had me closely connected to the medical community so I researched every word about PAH & PH. After about a year where I felt like I was just treading water and my pressure numbers had crept up a little, I went to my doctors and asked if we could do more. It was now the height of COVD. I had nothing else to do ao I researched drugs. When I "discovered" Uptravi I asked if we could tryout. They said okay and that led me to a brutal and extended titration, complicated by an acute kidney injury caused by a very early case of COVID which had gone unnoticed and untreated. In the middle of my Uptravi titration I had kidney surgery. That was when my doctors noticed that I had "always" been on diuretics. They tried to blame the deteriorating kidneys on my long term use of diuretics; however, after placing a stent in my renal artery my kidneys began to improve. I completed my Uptravi titration, but we already knew that a mid level dosage had completely reversed things and my pressures dropped from the mid 60s to the id 40s. At that time my Pulmonologist said I probably would not end up dying from PAH. A setback of SOB brought a second Cath where it was discovered I was suffering from fluid overload. More diuretics were added. I recovered. Another year went by and I learned about some current research looking at the drug Spironolactone as a possible treatment for PAH. Diuretics are known to treat PH but this was new. We decided to try it if my kidneys held stable. My functional ability improved. Right around this point Nov 2021 a new Pulmonologist was added to my team. She said, that based on my reaction to the increased diuretics that I had PH not PAH. This caused a bit of consternation between the Pulmonologist, Cardiologist and the PCP and a third Cath was scheduled to determine if I had PPH and was it caused by my heart valves. Well, the Cath showed that I had both mild PAH and mild PH but it wasn't caused by the valves. So what suddenly caused me to develop PH. When no answers were forthcoming I took my questions to the Accredo pharmacist. She asked my many seemingly unrelated questions about my complete medical history focusing on my hormonal experience. She then told me she was going to rock the very foundations of my being. We all know the old timeline of 3 - 5 years. We also know that many of our fellow Peers are surviving into the 20 years. She told me I have had PH since my early 20w and had accidentally survived so far almost 60 years because of the diuretics was on. Se explained that here were hormonal drivers of PH and my history pointed to that as my cause. Currently there is no classification for this cause but that I might be the person to bring this to the community. She even told me mu PAH had probably occurred as a result of my stopping my HRT. This morning I took all this to my Pulmonologist who agreed with all of it and we will be contacting a professor at Vanderbuilt University Medical Center to present my story.l You are the first to know.

Jen Cueva avatar

Jen Cueva

Wow, Sally, what an interesting bit of information you have now been given. I have heard about PH being related to certain hormones but not too many specifics. This would also make an excellent topic in the forums if you want to share it there. I also would love to hear what the professor at Vanderbilt says about all of this information. You certainly have been through the wringers perse for the past few years.

I am and have been taking spironolactone (Aldactone) since 2005. so that helps me with my other diuretics.

Hopefully, soon, you will get some better answers from this professor. But the pharmacists at Accredo, etc., are excellent sources of information. I think we often forget about them and discount them as credible sources.

Thanks for reading while offering your valuable feedback.

Take care and stay safe, my PHriend.

Aunt Lizzie avatar

Aunt Lizzie

Johan, I know what it's like to be told in a cold clinical way that you have something you've never heard of before. My diagnosis from the cardiologist in 2012 was that I had severe PH and heart failure. There was no explanation only to go back to my GP for her to look after me. I had been trying to find out what was wrong for almost 20 years, the last 10 I was dying, so any diagnosis was a sort of relief. I am lucky to have been directed by my GP to the correct specialists and am now monitored by them and a PH Clinic at the local public hospital here in Australia. I receive my medication through a government sponsored scheme so costs are minimal, except for the occasional specialist visit when something doesn't look right. I also Googled PH and was shocked at the 3 - 5 year survival story, but here I am, 10 years later and 80 years old. If COVID doesn't get me, I expect to be around for a few more years. Being my own advocate and studying all the pros and cons of medications and treatments has been invaluable for my survival. I have had some medications prescribed which had the opposite effects than they were meant to. Steroidal puffers, diuretics - no thank you. My little pink pill every morning has thankfully kept me stable these 10 years. I wish all PH-ers good health.

Jen Cueva avatar

Jen Cueva

Hi Aunt Lizzie, what a horrible way to be told about such a rare diagnosis like PH. I appreciate the support you offer Johan but sad that you both went through that. I'm grateful that you both are doing well as you can under the care of trustworthy PH teams.

You bring up an excellent point about being your best advocate; that's an important reminder. Keep on doing what you can and thinking ahead as you continue to kick PH and whatever else comes in your way, butts!

I'm sending you best wishes for many more years and positive thoughts, My PHriend. Thank you for taking the time to read my column and offer your perspectives.

Take care and stay safe.


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A Conversation With Rare Disease Advocates