The management of pulmonary hypertension is a long road

Good news at the doctor's office makes the sacrifices worth it

Karen Schultz avatar

by Karen Schultz |

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It isn’t often that my husband, Tim, and I attend an appointment with his pulmonary arterial hypertension (PAH) physician and hear what we’d like. Many times the news is a gross reminder of the condition and how limiting it can be.

A typical visit goes as follows: Check in, take vital signs, conduct a six-minute walk test (while subconsciously trying to beat the numbers for a previous effort), and undergo an echocardiogram, a chest X-ray, and various labs. The results vary but rarely improve significantly, which would be a dream come true for a PAH patient.

Examples of improvement might be running instead of walking, having endurance instead of stopping to rest, and taking the stairs instead of the elevator. Or perhaps a patient has lost 10 pounds between appointments and receives an “attaboy” from the doctor.

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Important victories

For many patients, just maintaining the status quo is cause for hope. Hearing that cardiac function hasn’t worsened can feel like good news. Oxygen levels remaining greater than 90% during a six-minute walk test can prompt celebration.

A day at the pulmonary clinic can be a lesson in patience. Many people drive for miles, and overnight stays are sometimes necessary depending on where a patient lives. We live about 2.5 hours from the clinic, and each visit can take up to five hours, not counting the drive.

The waiting room can be a brutal reminder of patients who face even more difficult circumstances. Some are in the recovery phase after receiving a lung transplant, so they look and breathe much better than others.

Away from medical facilities, PAH patients don’t often run into others with the same condition. Tim has admitted to feeling out of place at such visits. He has worked hard to maintain his “normalcy.” He equates his health with his ability to maintain a career, suffer through discomforts, and blend in.

Tim’s way of working through his diagnosis has always been to push through, but he doesn’t judge others who cannot. He doesn’t believe that each person’s approach is right or wrong, as each person is unique.

As a caregiver, I support him by being there for him when the news isn’t so great and celebrating when it exceeds Tim’s expectations.

One such moment recently was when Tim’s physician looked over his six-minute walk test and lab results with her usual precision and said, “Well, Tim, it looks like you will die from something other than PAH.” I started tearing up, and years of fear and other feelings I’d suppressed came spilling out. Tim simply smiled at me, winked, and said it was what they’d been working on for 15 long years. It was what we both needed to hear.

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.


Judith Hitchcock avatar

Judith Hitchcock

So well done

Karen Schultz avatar

Karen Schultz

Thank you for your praise, Judith. I so appreciate your words.

Sherri Cole avatar

Sherri Cole

Thanks for sharing this story. I am five years into my PAH journey and my mindset is much like your husband's. Most of the time I feel isolated. I feel okay about my life accomplishments until I return from summer break and compare notes with my "healthy" co-workers, then I feel like so much less. I realize my limitations. I am in my late 50s and struggling to continue to work, but it tires me to the point that I have no energy left for grandchildren and the like. PAH is definitely a unique journey with no tried and true path forward. It's nice to know that I may have so many years ahead of me and that other PAH patients are on similar, yet unique journeys. When you don't personally know anyone else with PAH, it is tough to know if what you are experiencing is shared by anyone else

Karen Schultz avatar

Karen Schultz

Thank you so much for your kind words, Sherri. The most stark reminders of my husband's illness usually occur when his limitations are forced into exposure. He travels to Sweden several times a year for work and dreads it due to the airport scrutiny, being so far from our providers at Duke and the added fatigue from all the WALKING he has to endure while there. The people who he meets with for his company in Sweden are all tall, fit and walk everywhere and he is spent by the time he returns home. I know during these times, I have to allocate him some downtime before and after to ensure he has energy for the things he enjoys. Tim was one of 50 men with Primary Pulmonary HTN in NC when he was diagnosed in 2002. It is hard to go through life with these extra limitations; however good for you for plugging on. I truly think it has been key to keeping him from dwelling on all the things that are "wrong".
Time with grandchildren has to be at the top of your list and I pray your energy lasts to make lots of good memories with them. I hope your journey and choices with time and endurance become clear and that you have years worth of memories to come. Take care. It was good to hear from you.

Allen kosowsky avatar

Allen kosowsky

I am newly diagnosed with PAH and CTEPH. I will have a CATH soon and the next step is likely balloon therapy to open up arteries in the lung. This is all the result of a pulmonary embolism and sepsis in 2020. I would appreciate any suggestions for how to deal with this affliction and am worried about life expectancy. Any thoughts, suggestions or reading material would be greatly appreciated. This is a terrifying combination.


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