My Son Wanted More Control Over What He Could Do

Colleen Steele avatar

by Colleen Steele |

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After receiving a heart and double-lung transplant in 2014, my son Cullen no longer has pulmonary hypertension (PH). Since then, he has been focused on protecting those precious organs.

When asked about his PH days, Cullen is direct in his response: “It’s no longer a part of my life. I prefer to keep PH in my past and avoid anything that is a constant reminder of what I went through.”

Thinking about PH is stressful for Cullen, but occasionally, he makes an exception and provides helpful insight for me to share with others.

We recently discussed how frustrated I feel when family and friends don’t take a PH patient’s illness seriously because they don’t look sick. Many patients feel pressured to stay active beyond what their bodies can tolerate. No rest for the weary, so to speak. It is a common discussion in the PH News Forums.

I would feel my ears go hot every time someone declared, “Cullen doesn’t look sick.” Vasodilators flushed Cullen’s cheeks and made him appear vibrant, but echocardiograms and right-heart catheterizations did not reflect a healthy child. I was tempted to carry them around to show people who assumed otherwise.

I thought Cullen might have held on to some of these aggravating PH memories, but he surprised me by saying, “I found the opposite harder to deal with.” Cullen feels resentment for the many times he wanted to do more, but was told no.

Cullen was diagnosed with PH when he was 8, and from the start, he excelled at self-advocacy and self-limitation. He enjoyed activities he knew he could handle and rested when needed. But there were many situations when other people decided what he could and could not do.

“I just wanted people to stop telling me no,” Cullen said.

School was where he felt the most unheard. He said his classmates were great and seldom treated him differently, but the teachers and staff often held him back. Most of all, he feels bitter about not being allowed to play kickball.

“It wasn’t like I was asking to play football or go rock climbing,” he said.

I took a deep breath and broke the news that the teachers were simply following the limitations that his doctors and parents had written out for them. If he was excused from gym because of his PH, it didn’t make sense for him to play kickball during lunch.

Cullen also had long QT syndrome, which is a heart rhythm condition that can cause fast, chaotic heartbeats and sometimes sudden death. It was quite serious, and he had to drop out of all sports activities because of it. This happened two years prior to his PH diagnosis.

I played devil’s advocate a little further and reminded Cullen that he had a central line leading to a pump that administered Flolan (epoprostenol GM). No one wanted to see it get pulled out or damaged, but Cullen stood by his feelings: “I knew how to protect myself. I could have played kickball.”

In hindsight, maybe we could have found an alternative or compromise for Cullen on the school playground. I don’t know what that would have been, but brainstorming with his classmates might have inspired something.

Cullen credits his dad and me for allowing him freedom at home, but when it came to parties and sleepovers at his friends’ houses, it was a different story. He recalls that unless he was going to the home of one of his two best friends, he had to fight tooth and nail for permission to attend.

Although I was happy to return to those homes late at night to change Cullen’s Flolan cartridge, I still remember how awkward it felt, and I constantly worried about not getting to him fast enough if his treatment pump alarm went off or he suddenly experienced a PH crisis.

During one sleepover, his backup cartridge was placed in the refrigerator as we requested, but the crisper froze the Flolan. His friend’s father is a doctor and knew this was problematic, so he informed me. I tossed it and mixed a new one when we got home. Understandably, not every parent would have recognized the concern.

We weren’t so overprotective that we never let Cullen go to sleepovers, but I think it was fair that we considered the distance between our homes and the level of PH awareness of those he was staying with.

Cullen wrapped up our discussion with a memory of attending a haunted house at the hospital for Halloween. “They saw me in a wheelchair and cut the fear factor down to 1. So unnecessary!”

We have learned that creating balance is a monumental challenge when living with PH, and that it’s helpful when everyone involved is mindful of this.

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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.

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