Among the many challenges of living with a life-threatening condition are the daily demands it places on both the patient and the caregivers.
A life-threatening illness isn’t something one can push to the back of the mind. Every day, people face the need to assess and address myriad symptoms, side effects, and anything out of the norm. To an outsider, this daily evaluation process, combined with frequent doctors’ appointments, emergency room visits, hospital admissions, and diligent medical research, might seem obsessive.
For my son Cullen and our family, a persistent battle with germs, especially after Cullen’s heart and double-lung transplant, has probably made us appear neurotic and overprotective. We were the hand-washing, sanitizer-toting, stay-away-from-us-if-you’re-sick, mask-covered people long before it became the norm.
Believe it or not, for many years I was blissfully free of medical worry. My children needed a doctor only for wellness checks, immunizations, and occasional ear infections. I vaguely remember the day when a 6-year-old Cullen said, “Mom, I sometimes feel like I can’t breathe.” But at the same time, he was a healthy, rambunctious child who enjoyed roughhousing with his brother and playing sports. Just watching him made me feel out of breath, so I dismissed his comments while assuming he was breathless from being a highly active little boy.
As time went by, it became apparent he was having issues. Cullen’s doctor considered asthma as a possibility, so he prescribed an inhaler and gave Cullen permission to resume his normal activities. And so he did — until he started experiencing chest pains.
We escalated from seeing a pediatrician to seeing a cardiologist. During the family history portion of the appointment, I shared that I have long QT syndrome. The nervous mom in me asked, “Could Cullen possibly have it, too?” Unfortunately, test results confirmed the condition, but Cullen’s was much worse than mine. They placed him on a beta-blocker and told him he should no longer participate in sports.
Though it was difficult, Cullen complied, but his symptoms continued to worsen. Soon, he developed a recurring, barking cough that his doctors believed to be croup. Although he was still young, Cullen started advocating for himself by insisting that he was sicker than we thought. This led to the suggestion that Cullen might struggle with anxiety or hypochondria.
For two years, we rode this asthma/long QT/anxiety/hypochondria roller coaster, but his dad and I suspected Cullen was suffering from something not yet diagnosed. We became “those parents” who insisted on the power of their instincts. Luckily, Cullen had an excellent pediatrician and cardiologist who didn’t feel threatened by our concerns. They recognized that Cullen’s symptoms warranted further testing to find the elusive root cause and finally put our worries to rest.
An echocardiogram and right heart catheterization eventually confirmed our fears and identified the underlying cause as pulmonary hypertension (PH), a rare, incurable, and life-threatening condition.
It was a bittersweet “victory.” After struggling for so long with the unknown and Cullen’s deteriorating condition, finally putting a name to his disease was almost a relief. Sadly, we now became even more focused on Cullen’s health and the health of everyone around him. Exposure to anything contagious, even the common cold, could have led to a serious PH crisis and hospitalization.
At the risk of sounding paranoid, we had to educate others. At the beginning of every school year, we met with Cullen’s principal, teachers, and staff to update them about his condition and address concerns. We also made a point of making sure Cullen’s classmates and their parents were PH-aware, too.
As self-conscious as advocating sometimes made us feel, our efforts were not in vain. People became more understanding of our obsessive attention to Cullen’s health. But I know it didn’t go unnoticed how often Cullen was absent from class or how frequently he visited the emergency room for seemingly minor concerns.
Whether symptoms were big or small, we couldn’t ignore them. A cough, change in appetite, fatigue, insomnia, edema, aches and pains, skin discoloring, and everything in between often were indications of something that needed to be addressed.
It is a common misconception to think Cullen’s transplant meant an end to our obsession about his health. Thanks to a healthy heart and new lungs, concerns eventually became less frequent. But the importance of follow-up has once again intensified, this time because of kidney disease.
My advice to patients and caregivers witnessing worsening symptoms: Ask doctors to consider the medical zebra and not the common horse in the hoofbeats. For the newly diagnosed with PH or another rare condition: Assiduous self-advocacy will help you maintain a better quality of life and hopefully provide you with more time on this earth. Stay obsessed with surviving.
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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