My Son Has Been Followed From PH to Heart-lung Transplant to Today

When my son Cullen, 22, was diagnosed with severe pulmonary hypertension (PH) in 2008, he didn’t look sick. People couldn’t see the severely enlarged, overworked heart and sickly lungs struggling to breathe. “He looks so healthy,” we were often told.

There is no cure for PH, but treatments helped improve Cullen’s quality of life for almost six years. Calcium channel blockers, diuretics, and pulmonary vasodilators gave him a constant flush to his freckled cheeks. His rosy complexion distracted people all the time. “Wow, Cullen looks great,” they would exclaim.

Thanks to excellent medical care and Cullen’s brave compliance through hospitalizations, procedures, and multiple complex treatments, things were often better, but never great. Cullen understood that PH was life-threatening, and as his condition worsened over the years, he became increasingly concerned that he might die young.

Sadly, without the heart and double-lung transplant he received in 2014, he might have.

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Since the transplant, Cullen looks healthy, even great, and his new heart and lungs reflect that image. But that’s not to say the struggle is over.

People rarely see what has followed Cullen from PH to transplant in his effort to stay well.

Let me help you picture what he shoulders every day.

Cullen, in a white lab coat and red mask, has fun at a hospital Halloween party in October 2014. Around him are hospital staff members dressed as cavemen. (Photo by Colleen Steele)

Halloween in the hospital

In October 2014, Cullen was almost three months post-transplant, experiencing several big bumps in the road during his recovery and still spending a lot of time in the hospital. But what a fun place it turned out to be for Halloween!

Kudos to all at Lucile Packard Children’s Hospital Stanford, in Palo Alto, California, for going all out to create a fun Halloween party for their pediatric patients, complete with trick-or-treating, games, and costumes, including those worn by hospital staff.

Cullen dressed up as his cardiology and PH specialist, and his PHriend Katy dressed as their pulmonary and lung transplant doctor. Cullen and Katy (not pictured here) were a big hit, but the cavemen costumes worn by doctors, nurses, or hospital staff (we still don’t know who) remain memorable to this day.

This picture of Cullen posing with them continues to make me smile and laugh, but the cavemen in the picture can also represent the shadows that followed Cullen from PH to post-transplant.

Meet Compliance

Standing alone to Cullen’s left is his oldest friend, Compliance, who saw him through his PH days and remains on his side to this day. They are close, but it’s a love-hate relationship.

No matter how tired, frustrated, disappointed, or apprehensive Cullen may feel, Compliance’s club is always ready to proceed with a new treatment, increase a medication dosage, change his diet, and battle so Cullen stays well. Compliance is that friend who tells him not what he wants to hear, but what he needs to hear, and threatens to club him within an inch of his life if he doesn’t listen. So he always does.

Keeping Hope at his side

To the front right of Cullen’s shoulder is Hope. Not quite the ray of sunshine that many people would picture Hope to be, but he’s smiling, his eyes are bright, and instead of a club, he’s holding a bone to throw.

Hope can throw far, but Cullen will only chase aspirations to where he can see they might land. Cullen’s greatest fear is that mortality transplant statistics will catch up to him in the future.

Just as he did through his PH days, Cullen continues to keep Hope close at his side. Together they have reached goals and climbed metaphorical mountains through six years of PH and, currently, eight years post-transplant.

Donor is always at the heart of things

Standing closest behind Cullen is Donor. Every heartbeat and breath that Cullen takes reminds him of Donor’s sacrifice. There are no words powerful enough to describe their unique and powerful relationship.

But the darker side of this figure is the possibility that someday he might need another Donor, if one can be found again for a heart, lungs, or kidneys.

When Cullen wanders deeper into the cave with these thoughts, the big question on his mind is, “Will I want another Donor?”

PHred isn’t dead

Cullen no longer has PH since receiving a transplant, but there is PHred, to the right of Donor in the back. Cullen tries to keep PHred out of sight and out of mind, but post-traumatic stress syndrome has him popping back up now and then.

Dodging Morty’s death stares

It’s difficult for Cullen not to worry about his mortality when remaining healthy post-transplant is challenging and requires great effort. Big Morty, at the far right, is often staring Cullen down with club in hand.

Calling Dr. Cullen

Aptly dressed as a doctor, Cullen is an excellent self-advocate, and as difficult as these thoughts are, they’re a part of the process.

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.