Extract
When I was first diagnosed with pulmonary arterial hypertension (PAH) in 2009, I had no idea how much my life would change. The fatigue, the dizziness and the chest pain were all challenging, but nothing prepared me for the terrifying experience of dyspnoea crises. It is not just about struggling to catch your breath – it is a visceral feeling that your body is being strangled from the inside out, a constant reminder that your lungs and heart are in a battle against a relentless disease.
Shareable abstract
A patient with pulmonary arterial hypertension shares their experiences, from diagnosis to daily life, including the physical and psychological battle of breathlessness in management of chronic lung disease https://bit.ly/4gxgIBC
Introduction
When I was first diagnosed with pulmonary arterial hypertension (PAH) in 2009, I had no idea how much my life would change. The fatigue, the dizziness and the chest pain were all challenging, but nothing prepared me for the terrifying experience of dyspnoea crises. It is not just about struggling to catch your breath – it is a visceral feeling that your body is being strangled from the inside out, a constant reminder that your lungs and heart are in a battle against a relentless disease.
The first crisis: a wake-up call
The first time I experienced a dyspnoea crisis was unforgettable. I was walking up a short flight of stairs, something I had done countless times before without a second thought. But that day was different. Halfway up, I felt a tightness in my chest, as if an invisible hand was gripping my lungs. My breaths became shallow, each one more laboured than the last. Panic set in as I realised I could not draw in enough air. My vision blurred, and a cold sweat broke out across my body. I leaned against the railing, trying to stay upright as I gasped for air. The world seemed to close in on me, my heart racing as if it was about to explode.
I was fortunate to have someone with me who quickly recognised what was happening. They helped me sit down, and as I focused on slowing my breathing, the immediate crisis passed. But the memory of that moment stayed with me, a haunting reminder that PAH was not something I could ever ignore.
Understanding dyspnoea: it is more than just breathlessness
Before my diagnosis, I had heard the term “dyspnoea” before, but I never really understood what it meant. After that first crisis, I learned that dyspnoea is not just about feeling short of breath – it can also be a sensation of suffocation, where no matter how hard you try, you cannot get enough air into your lungs. For someone with PAH, this happens because the blood vessels in the lungs are narrowed, making it hard for blood to flow through and for oxygen to reach the rest of the body. This constant lack of oxygen leads to the feeling of breathlessness that can quickly escalate into a full-blown crisis.
The emotional toll: living in fear
The fear of another dyspnoea crisis is always lurking in the back of my mind. It changes the way you live your life. Before PAH, I was active and independent. Now, every action is calculated. I avoid stairs, long walks, and even standing for too long. I have to think twice before going out, worrying about whether I will have access to a place where I can sit and catch my breath if needed. The uncertainty is exhausting – never knowing when the next crisis will hit, or how severe it will be.
This fear is not just physical but deeply emotional. There is a constant sense of vulnerability, a feeling that my body could betray me at any moment. Social events become a source of anxiety rather than enjoyment. I am always afraid that I will have an episode in front of others, that I will have to explain my condition, or worse, that someone will panic because they do not know how to help. The result is isolation – pulling back from the world because it is easier to stay home where I feel safe.
Managing crises: finding a way to breathe again
Over time, I have learned to manage dyspnoea crises better, but that does not make them any less frightening. The first step is recognising the early signs: the slight tightness in my chest, the subtle change in my breathing pattern, the faint dizziness. When I notice these, I immediately stop whatever I am doing and focus on controlling my breathing. I sit down, lean forward slightly, and try to take slow, deep breaths. Sometimes, I use a fan to blow air on my face, which somehow tricks my brain into thinking I am getting more oxygen than I actually am.
Medication helps to some extent. I am on a regimen of drugs to manage my PAH, including vasodilators that help open the blood vessels in my lungs, making it easier for blood to flow. These medications have reduced the frequency of my dyspnoea crises, but they have not eliminated them. Nonetheless, every episode feels like walking a tightrope – I am never quite sure if I will make it to the other side without falling.
When you suffer from PAH and you are in World Health Organization (WHO) functional class III, you realise the cost of every word you speak. Sometimes, you have just enough oxygen in your lungs just to say the most important word. During a crisis, that word was usually “oxygen”, “water” or “ambulance”.
The impact on daily life: redefining normal
Living with PAH and the constant threat of dyspnoea crises means I have had to redefine what “normal” is for me. I used to take my health for granted, but now every breath is a reminder of my condition. Simple tasks like grocery shopping or cleaning the house have become monumental challenges. I have had to learn to pace myself, to do things in small increments, and to rest often. I have had to accept that my life is different now – that I cannot do everything I used to do, and that is okay.
One of the hardest things to accept has been the loss of spontaneity. Everything I do requires planning and preparation. I carry a portable oxygen concentrator with me wherever I go for 2 years before my double lung transplantation. For Bulgarian patients to have a portable oxygen concentrator is still a luxury. Patient organisations often organise donation campaigns to support those in need. I avoid crowded places where the air might be thin or where I might have to walk long distances. I keep my medication with me at all times, and I have taught my friends and family what to do if I have a crisis.
But despite these challenges, I have found new ways to enjoy life. I have taken up activities that are less physically demanding, like reading, writing and painting. I have become more mindful of the small joys in life – the warmth of the sun on my face, the sound of birds in the morning, the comfort of a good book. PAH may have changed my life, but it has not taken away my ability to find happiness.
Looking forward: living with hope
Living with PAH and the constant threat of dyspnoea crises is not easy, but it is something I have learned to manage. I have accepted that my life will never be the same, but that does not mean it cannot be fulfilling. I have become more resilient, more patient and more appreciative of the things I can do, rather than focusing on what I have lost.
Most importantly, I have learned to live with hope. Hope that medical advancements will continue to improve the quality of life for people with PAH. Hope that one day, there will be a cure. And hope that, in the meantime, I can continue to live a meaningful and joyful life, one breath at a time.
Footnotes
Conflict of interest: N. Maeva reports support for the present manuscript from the European Lung Foundation (ELF), Pulmonary Hypertension Association Europe (PHA) and Bulgarian Society of Pulmonary Hypertension Patients Association (BSPPH). L. Robinson is an employee of ELF, which was founded by the European Respiratory Society (ERS), who also provides core funding for ELF activities.