Like the Weather, My Son’s PH Journey Brought Storm Clouds and Sunshine

A popular phrase people like to say when discussing the climate is, “If you don’t like the weather, wait a minute and it’ll change.”

Living with a serious illness often can feel this way, too. You might wake in the morning without issue, and by early afternoon experience an onslaught of symptoms.

Disease can take you by surprise at the doctor’s office when a routine checkup turns into a tornado of new concerns. Even if you think you are doing well, test results might not always reflect your self-diagnosis. And out of the blue, a doctor can recommend testing to prepare for the possibility of future stormy conditions.

When my son Cullen had pulmonary hypertension (PH), we were always ready for a change in weather. We kept a bag of essentials in the car in case of an unexpected hospital admission. Cullen always carried important lists of medical information on his person, and we stowed duplicates in the glove compartment. We kept at least a week’s worth of medications and medical supplies organized and ready for unplanned trips from our home in Washington state to Cullen’s PH specialist in California.

A routine right-heart catheterization was one of the best ways to measure the calm before a storm. Sometimes we were pleasantly surprised by the results, and other times they alerted us to worrisome conditions not yet visible to Cullen and his medical team. Although the forecasts were not always ideal, right-heart catheterizations were essential in helping the doctor treat Cullen’s PH and adjust dosages as needed.

Life outside clinic doors was a confusing time as Cullen and our family adjusted to the first year of his PH diagnosis. We were challenged with spreading awareness and advocating for our healthy-looking son who had an impending storm brewing inside of him.

PH treatments helped keep the worst of it at bay, but there were days when there was no way for him to run for cover. Literally, there was no way for him to run or sometimes even walk, climb steps, or participate in normal childhood activities, such as attending school. It was up to us to educate people about why Cullen could tolerate PH one day and sometimes not the next. We defended making the most out of his good days and resting on the bad.

Supplemental oxygen, PH medications including Flolan (epoprostenol GM), and milrinone for right-heart failure were umbrellas that provided a better quality of life for almost six years. Unfortunately, the PH storm became too much for them to bear, and the only option left was a heart and double-lung transplant.

Life post-transplant, especially in the first few years, had Cullen enjoying beams of sunlight through threatening clouds. He has battled rejection, posterior reversible encephalopathy syndrome, osteoporosis, high blood pressure, and kidney disease. But he also dodged a few threats such as post-transplant lymphoproliferative disease and a parasitic infection.

As the years pass by, Cullen has enjoyed more sunny days and starry nights. He knows he is fortunate and does not take for granted the six years and counting of post-transplant. He also doesn’t let his guard down, because even now, the winds can change.

For example, Cullen recently made an unexpected trip to the emergency room when his bloodwork indicated a dramatic rise in potassium levels. Concerned that this could cause heart arrhythmia and other complications, we immediately headed to the hospital per the request of his doctor. As it turned out, Cullen dodged another storm. Redrawn labs showed normal potassium levels — it was a false alarm.

What I have repeatedly said throughout Cullen’s PH and transplant journey is that it’s better to be safe than sorry. This is Cullen’s life, not the weather we are dealing with, so I would much rather prepare for a medical storm that never comes than face one without warning.

I often hear recently diagnosed PH patients express concern about day-to-day changes in their health. I share Cullen’s experiences to help others understand their new normal. Yes, it is common to feel fine one day, awful the next, then well again. I recommend enjoying the good days in moderation and encourage you not to feel guilty on days when you need to rest. Only you and your doctors should judge your health, not others.

I wish you the strength to weather every storm and smooth sailing on days when PH clouds are few.

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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.