As part of our work at the European Lung Health Group, we are proud to share this powerful story from the pulmonary hypertension community, thanks to our partners at PHA Europe. Originally published in the International Journal of Cardiology: Congenital Heart Disease, the story comes from Hall Skaara, a PH patient from Norway.
In his story, Hall takes us through the emotional and physical challenges of being diagnosed with idiopathic pulmonary hypertension. Nearly two decades later, he has turned this life-changing experience into a source of strength, raising awareness and advocating for others facing similar challenges. Read the full story below.
My journey with pulmonary hypertension: From diagnosis to thriving
I've always believed in living a healthy lifestyle. I exercised regularly, ate well, never smoked, and rarely drank alcohol. For most of my life, I took pride in caring for my body. So, when I started noticing occasional irregular heartbeats around the age of 40, it didn't seem like a big concern. But over time, those irregular heartbeats became worse.
Despite repeated visits to the doctor, the EKGs didn't catch the problem. Eventually, the irregularities became chronic, and finally, they were detected during a routine checkup. That led me to a cardiologist at my local hospital, where I underwent a bicycle test. Despite experiencing constant heart flutters and fibrillations during the test, I managed to perform fairly well.
Afterward, I was sent to a larger hospital for an ablation procedure to address the arrhythmia. My first ablation, targeting the right atrium (isthmus line), was a disappointment. As I was being wheeled back to my hospital room post-operation, the fibrillations started again. I remember feeling crushed by this setback.
The doctors then decided on a more complex procedure: puncturing the septum to perform an ablation around the lung veins in the left atrium. Thankfully, this ablation seemed successful. Although I experienced extra heartbeats occasionally, I no longer had fibrillation attacks. But my journey didn't end there.
Shortly after the ablation, I started feeling short of breath. I assumed this was a side effect of the procedures and tried to push through it, hoping my condition would improve. Several months later, I returned to my cardiologist, who once again put me through a bicycle test. The results were normal for someone my age, and no fibrillation occurred during the test, only extra beats. At this point, they suspected stenosis around the lung veins, but an MR scan showed no signs of it.
However, I was disappointed with my performance. I knew my body, and I felt that I hadn't performed nearly as well as I used to. I expressed this to the nurse, but her response—"You have too high standards"—left a lasting impact on me. It discouraged me from returning to the hospital, even as my breathlessness continued to worsen. I convinced myself that pushing harder and exercising more would eventually improve my condition.
As time went on, it became clear that something was seriously wrong. Two years later, I finally went back to my GP, who referred me to a pulmonologist. After a simple test with an oximeter and a quick walk up several flights of stairs, my oxygen levels plummeted. The pulmonologist knew something was off, though he initially tested for asthma, which came back negative. His next suspicion: pulmonary hypertension (PH).
I was referred to the national hospital for further testing. Over three days, I underwent a battery of tests, including a 6-min walk test in which I covered an impressive 745 m. However, a right heart catheterization confirmed the diagnosis—idiopathic pulmonary hypertension. Although my heart's pressures were substantially elevated (PVR of 8.5 Wood units), my cardiac output was still good.
After receiving the PH diagnosis, my cardiologists reviewed my past EKGs and echocardiograms from when I experienced atrial fibrillation. None of these earlier tests had shown any signs of pulmonary hypertension, suggesting that the fibrillation was likely unrelated to the PH. It seems that the pulmonary hypertension only emerged after the ablations, though it's unclear why. There was even speculation that the procedures may have triggered the condition, but thorough testing ruled out stenosis as a cause. Ultimately, the cause of my PH remains unknown, even today.
Receiving the diagnosis was a blow. I had spent my whole life treating my body well, yet here I was, facing a life-altering illness. My initial reaction was anger - anger at my body for betraying me. But after a few days, my anger transformed into strong determination. I wasn't going to let PH define my life. I was determined to live as long as possible, and with a good quality of life.
I turned to resources from the US Pulmonary Hypertension Association and created my own "survival guide." This guide became my blueprint for living with PH. It contained a long list of lifestyle adjustments and strategies, all designed to help me achieve my goal of thriving despite my condition.
Receiving the diagnosis of pulmonary hypertension was life-changing. My lifestyle before PH was filled with regular exercise, physical activities, and work that I enjoyed. However, when the disease entered my life, everything changed. I had to come to terms with the fact that my old ways were no longer sustainable. Activities that once felt easy, like running or playing squash, became impossible.
I quickly realized that it was essential to accept the changes and adapt to a new lifestyle. Insisting on maintaining the same level of physical activity or work as before would not only be unrealistic but could also lead to deterioration in my health. Many PH patients cannot work as hard as they used to because it risks worsening their condition, and I was no exception.
At first, it was difficult to face these limitations. But over time, I discovered that the changes weren't necessarily bad. While I could no longer run, I found that I could walk. I couldn't bike like I used to, but with an electric bike, I could still enjoy cycling. I had to let go of competitive squash, but I found a new passion in backgammon, which gave me an outlet for my competitive spirit.
I took this new hobby seriously, and after six years of dedication and practice, I became the Norwegian champion in backgammon. A year later, I won my first international tournament, the London Open. Backgammon became more than just a pastime for me—it was a way to shift my focus away from the illness and enjoy a sport that didn't require physical exertion. It's a great reminder that, even with PH, you can find joy and fulfilment in activities that suit your new lifestyle. I even have a specially designed backgammon board from Turkey with the PHA Europe logo and the date of my diagnosis. It's a conversation starter, and people often ask about it, giving me the opportunity to share my journey with pulmonary hypertension (PH) and create awareness for the disease.
The first thing backgammon players in Norway—or their family members—will think of if they ever experience unexplainable shortness of breath is PH. By sharing my experience and creating awareness for PH through this hobby, I hope to help others recognize the symptoms early. Early diagnosis is crucial for improving quality of life and potentially increasing survival rates for those affected by PH.
One of the most important things I tell newly diagnosed PH patients is to accept their illness as soon as possible and adjust their lives accordingly. It's crucial not to dwell on the things you can no longer do. Instead, focus on what you can still achieve. Life is far from over because of a serious illness. By adapting, you can still lead a high-quality life, full of meaning and joy. The key is to embrace the changes, find new passions, and take care of your physical and mental well-being.
More than 10 years after my diagnosis, I went through a divorce. I often wonder if the disease might be affecting me mentally in ways I'm not fully aware of. I sometimes think that I may be making decisions differently than I would have if I didn't have this illness, similar to how marriages can face crises after experiencing something traumatic, like losing a child. It's important to understand that living with a serious disease doesn't only affect you physically - it can have significant emotional and psychological effects as well. Being prepared for these mental challenges is just as important as managing the physical symptoms.
One key moment in my journey was in 2012 when I read about a rehabilitation program for PH patients in Heidelberg, Germany, led by Prof. Grünig [1]. I had always been physically active, and my doctor in Norway had cautioned me that exercise wouldn't cure my condition. However, the program in Heidelberg introduced me to the concept of low-intensity exercise as a way to manage PH. Over the course of the three-week program, my VO2 max increased by nearly 20 %. It was an eye-opener.
Since then, I've incorporated regular exercise into my life, focusing on low-intensity activities like walking. I pay close attention to my body's signals, always mindful not to overexert myself. But when I feel strong, I push myself a little - walking a bit farther or climbing a hill a little faster. Over time, my physical condition has improved significantly. In fact, 19 years after my diagnosis, my physical capacity is nearly double what it was when I was first diagnosed. My VO2 max even exceeds that of a healthy person my age, despite living with PH.
I'm still on mono-therapy today (I'm not a calcium blocker responder), 19 years after being diagnosed with serious PH. My daily routine is grounded in maintaining a healthy and active lifestyle, which has helped me sustain my physical well-being and mental positivity. I've also found immense satisfaction in helping others. Supporting fellow PH patients is not only rewarding for them but also a way to keep myself grounded and positive.
When I was diagnosed with pulmonary hypertension (PH) in 2005, there was no PH association in Norway. The feeling of isolation was profound, as I knew no other PH patients at the time. Determined to create a community, I took the initiative to establish the first Norwegian PH association (Here is a link to the original webpages.) I didn't have a roadmap, but I found guidance from a knowledgeable patient, Ralf Schmiedel, a long-term survivor of PH based in Germany. Ralf was incredibly well-informed, reading scientific papers weekly and offering me practical advice on living with PH. His insights not only helped me but gave my family hope, showing them that a future with PH was possible. This was a critical moment because living with PH does not only affect the patient - it profoundly impacts the entire family. My wife and children found immense comfort in seeing someone thriving with the condition. At a time when the internet was filled with misleading and often frightening information about PH, Ralf's experience and knowledge became a lifeline for us.
As I created a webpage and online forum for PH patients, I slowly began to connect with others facing the same challenges. After about a year, I organized the first patient meeting. It was a two-day event, which not only included a scientific program led by a doctor and a rehabilitation representative but also a social aspect, with a dinner that allowed us to bond. The relationships we built during that time were critical in forming the foundation of the organization. Soon, more people joined, and the association became a collective effort rather than something I carried alone.
One of the fortunate aspects of living in Norway is the high quality of our healthcare system, thanks to universal coverage. Access to medication and psychosocial support, including rehabilitation, has generally not been a significant barrier. Therefore, much of our early association work focused on raising public awareness about PH. One particularly impactful moment was when my older son, a top-level basketball player, and his entire team painted their lips blue during a televised match to raise awareness for PH. The opposing team, upon hearing about the cause, joined in, and the headlines read, "Son plays for his seriously ill father." This led to an invitation for us to appear on TV, where I shared my experiences living with PH, my son spoke about what it was like to have a father with the condition, and my doctor discussed the medical aspects of PH.
The impact of that appearance became evident at the next patient meeting when a woman stood up and shared her story. After seeing us on TV, she had recognized the symptoms and asked her doctor to test her for PH, ultimately leading to her diagnosis. Realizing that I had helped even one person find the right diagnosis made all the awareness efforts feel truly worthwhile. Of course, we know that our campaigns have likely reached many more people, but that individual story reinforced the power of patient advocacy.
In 2012, our association joined the European umbrella organization, PHA Europe. As the Norwegian group grew stronger and more autonomous, I became increasingly involved in the European PH community. Handing over the leadership of the Norwegian association was bittersweet, like letting go of a child I had nurtured and raised. But seeing it flourish without me filled me with pride and allowed me to take on new responsibilities.
At PHA Europe, I took on various roles, including leading the “White Spot Program,” which aimed to support the establishment of PH associations in countries where none existed. We developed guides and templates to help local groups start their own organizations. It was incredibly rewarding to watch these groups form and join our community at PHA Europe. However, it was also a sobering experience to realize that not all countries offer the same level of care and access to medication that I had in Norway. Many Eastern European countries, in particular, struggle with access to basic treatments. Through PHA Europe, we have worked to support and mature these associations, empowering them to advocate for better care for their patients.
Another project I've been closely involved with is PHA Europe's Bel Air Center, an innovative virtual PH community. The Bel Air Center is a free, online platform where users can create accounts at www.belaircenter.info. The virtual space is designed to bring together the global PH community. It features stands where PH associations from around the world can display information about their organizations, including documents, brochures, and videos. PH pharmaceutical companies can also participate, sharing educational materials about their products and research. The platform includes a social area where users can meet, chat, or even video conference with one another, fostering a sense of connection. Additionally, there's an art gallery, offering patients, caregivers, and healthcare professionals the opportunity to showcase their artwork. Furthermore, the presentation area hosts a variety of PH-related webinars, and PHA Europe regularly organizes live events in the virtual space, further enhancing engagement and education within the PH community.
I also had the privilege of serving as president of PHA Europe during a challenging period. Although it was a demanding role, it was deeply meaningful to assist the organization and, in turn, help thousands of PH patients across Europe. Living with PH creates a unique bond between patients, almost like we are part of a family. This sense of responsibility to support others living with PH has been a driving force in my journey.
Over the years, I've become deeply involved in the international PH community. I've attended numerous conferences, met countless patients and doctors, and have been fortunate enough to contribute to the 7th World PH Symposium as part of the patient task force [2]. It was both humbling and exhilarating to present in front of 1700 specialists. While they are experts in the disease, I've come to see myself as a specialist in living with the disease. Together, we make a powerful team.
Together with another PAH patient, I co-authored a paper [3] in 2019 highlighting the patient experience and the growing role of patient voices in shaping treatment strategies for pulmonary arterial hypertension. The paper emphasizes the importance of early diagnosis, holistic management, and patient advocacy in improving quality of life and outcomes for those living with PAH. By sharing our personal journeys and insights, we aimed to raise awareness among healthcare professionals and contribute to the development of more patient-centered care models and clinical guidelines.
I'm heartened to see that patients are increasingly involved in decision-making, both at the individual level and in areas like PH guidelines and regulatory affairs. It's encouraging to witness this shift toward patient-centered care.
Looking back, my life with pulmonary hypertension has been full of challenges, but it has also been incredibly fulfilling. In many ways, my quality of life is better now than it might have been if I had never gotten sick. Living with PH has taught me resilience, determination, and the importance of a healthy mindset. And for that, I am grateful.
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