Carrying Hope from One Decade to the Next

Colleen Steele avatar

by Colleen Steele |

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Over the past decade, my son and our family have been through an intense journey from pulmonary hypertension (PH) to transplant. Most of the big, scary, sad, joyful, and incredible moments were squeezed into the past 10 years.

Cullen was two years into his diagnosis when the world ushered in 2010. Life before PH had become a faded memory. Pictures of Cullen playing sports were stored away upon his request. Reminders of what he could no longer do were too painful. At 10, the young adult in him was more prominent than the child.

He started the decade sore from the placement of a new central line and nervous about an upcoming trip to Lucile Packard Children’s Hospital Stanford for his PH checkup.

He had good reason to feel nervous. The first two years of PH treatments had provided an improved quality of life, but he was starting to experience signs of decline. This became obvious when he tried to complete a six-minute walk. During this test the patient walks at a normal pace for six minutes to help doctors evaluate lung function, fitness, and how well the body is reacting to treatments.

Cullen was doing well until he was asked to take his hands out of his pockets and walk a little faster. He tried, but abruptly stopped short, turned pale, clutched his chest, and dropped to his knees.

He recovered quickly, but this brief episode was an indication that it was time for adjustments to his treatments. Cullen was proud to learn that he had grown taller, but not thrilled to have his Flolan (epoprostenol GM) concentration and rate increased to accommodate that growth. Titrations gave him severe migraines.

So, 2010 started out with a big headache. Cullen’s body eventually adjusted to the higher doses, but we couldn’t shake how quickly and unexpectedly decline could happen.

Motivated by this scary reality, that February we set up a fund with the Children’s Organ Transplant Association to prepare for the inevitability of a future heart and double-lung transplant. For two years, we were surrounded by community and were guests of honor at fundraisers that were both heartwarming and successful.

There were other serendipitous PH experiences. A favorite was when our family attended Camp Taylor, a camp for children with heart disease. It was there that Cullen enjoyed swimming for the first time since diagnosis thanks to the invaluable purchase of a dry suit. Fellow PHer Kathleen Sheffer introduced herself to Cullen, and to this day, they have a special friendship. Cullen’s brother, Aidan, bonded with other healthy siblings who related to his fears and experiences. Camp Taylor provided all of us — Mom and Dad included — a chance to let our guard down and have fun.

As years passed, PH friendships transitioned into a PHamily that supported one another through laughter and tears. Together we proceeded further into the decade and closer to the next leg of the shared journey: transplant.

Cullen and his comrades fought hard. He battled through more titrations, eventually supplemental oxygen, and the placement of a double Broviac-Hickman central line. He even underwent an atrial septostomy, but fate still led him to transplant.

Cullen and five of his Stanford PHriends became donor recipients. Together they forged on, celebrating a second chance at life but battling new challenges. In this second half of the decade, Cullen was once again pained with migraines. This time, a symptom of posterior reversible encephalopathy syndrome, caused by anti-rejection medication. He has also faced rejection and kidney disease. But despite it all, life moved forward with promise.

We took a family trip to Utah. Cullen jet-skied and drove a boat during summer vacations in Eastern Washington. He graduated from high school, started college, and by 2019 was finally feeling optimistic enough to set goals for his future.

This new decade started out differently than the previous one. Being five years post-transplant and feeling healthy was the highlight. He is shorter than what he anticipated he would be by 20. PH treatments stunted his growth, but that holds little importance to him now. His heart is heavy, no longer with disease but from loss. Four of his transplant PHriends passed away, and celebrating the new decade without them was hard.

But as they would have wanted, he held onto hope and carried it into 2020.

In the words of Richelle E. Goodrich, author of “Slaying Dragons,” “Past and Present I know well; each is a friend and sometimes an enemy to me. But it is the quiet, beckoning Future, an absolute stranger, with whom I have fallen madly in love.”


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 Services, and are intended to spark discussion about issues pertaining to pulmonary hypertension.


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