Let’s demystify pulmonary hypertension, in all its forms
Like mermaids, conditions like pulmonary hypertension (PH), pulmonary arterial hypertension (PAH), and pulmonary hypertension associated with interstitial lung disease (PH-ILD) are often just beneath the surface, unnoticed and undiagnosed. These rare diseases can make patients feel as though they are trapped underwater, gasping for air.
Together, we can bring their unique experiences into view and ensure they receive the attention they deserve.
Dive into the stories of patients facing high pulmonary blood pressure
Explore the hidden experiences of patients with PH caused by damaged pulmonary arteries
Dive deeper into the realities of patients with PH caused by other chronic lung diseases
What is PH?
Pulmonary hypertension (PH) is a progressive disease that reduces blood flow and increases pressure in the pulmonary arteries1, affecting both the heart and lungs.2
Some forms of PH are rare and can progress quickly, turning every breath into a struggle and making daily life a constant challenge.
There are five different groups of PH based on different causes (WHO classification)1
Pulmonary arterial hypertension (PAH)
Pulmonary hypertension associated with left heart disease
Pulmonary hypertension associated with lung disease and/or hipoxia*
Pulmonary hypertension associated with pulmonary artery obstructions
Pulmonary hypertension with unclear and/or multifactorial mechanisms
*These lung diseases include obstructive lung disease, where the lung airways narrow and make it harder to exhale (e.g. COPD), or emphysema; restrictive lung disease, in which the lungs have a tough time expanding when one inhales (e.g. interstitial lung disease [ILD]); lung disease with mixed restrictive/obstructive patterns (e.g. combined pulmonary fibrosis and emphysema); hypoventilation syndromes, hypoxia caused by living in an area of high altitude, and developmental lung disorders.
What are the symptoms of PH?1,3
Shortness of breath during physical activity or at rest
Shortness of breath when bending forward
Fatigue and rapid exhaustion
Chest pain during exercise
Dizziness during activities such as climbing stairs
Swelling of the ankles, stomach and/or legs
Fainting spells
Bluish colour in the lips, fingernail, and skin
PH is rare but real
Affecting up to
of the global population
(up to 10% aged +65).4
of affected patients live in low- and middle-income countries.4
Left-sided heart and lung diseases have become the most frequent causes of pulmonary hypertension.4
Diagnosis and treatment
Diagnosising PH begins with a clinical suspicion based on symptoms, physical examination and the review of a comprehensive set of tests.1
The good news is that there are several treatments for some of the rare forms of pulmonary hypertension.1 The only way to unlock them is with the correct diagnosis.
- Signs, Symptoms, history suggestive of PH
- Echocardiographic probability of PH
- Referral to a PH centre is strongly advised
- Right heart catheterisation (RHC) is the only method to diagnose PH
What is PAH?
Pulmonary arterial hypertension (PAH) is a progressive form of pulmonary hypertension that occurs when the arteries in the lungs become damaged or narrowed, making it harder for the heart to pump blood through them.5
With only a few effective treatment options out there for PAH, it's harder for patients like me to see better outcomes.
PAH in numbers
high-income countries is
adults.6
arterial hypertension is
adults.6
Hear from others like me
“Before my diagnosis, I was so frustrated because PAH is a rare disease with nonspecific symptoms that can be mistaken for those of other common illnesses”7
“At the time of my diagnosis, I felt anger, confusion, anxiety and a deep worry about what the future held for me.”7
“The diagnosis changed my whole relationship with my family. Suddenly, I had an incurable disease, and my loved ones became full-time caregivers.”7
“Living with PAH meant a decrease in my quality of life, making even simple daily activities more difficult. It also took an emotional toll and negatively affected my social life.”7
With time I have been able to overcome prejudice and show the world that I am capable of anything.7
20% of my friends had to wait more than 2 years after the first symptoms before being diagnosed with PAH.2,8
Many people are affected by pulmonary arterial hypertension:
Newly diagnosed patients
Patient caregivers
Parents caring for children with PAH
Teenagers balancing adolescence and PAH
Long-term survivors of PAH
An early diagnosis can make all the difference.
What is PH-ILD?
Pulmonary hypertension associated with interstitial lung disease (PH-ILD), classified within Group 3 by the World Health Organisation, is a form of pulmonary hypertension that develops due to an interstitial lung disease. Group 3 includes all PH caused by lung diseases.1
PH caused by lung disease deteriorates our exercise capacity and has a significant impact on our lifestyles1
PH-ILD is often associated with:9,10
Reduced functional capacity
Quality of life deterioration
Greater need for oxygen
Increased mortality risk
Some diseases that can be associated with pulmonary hypertension are:9
Chronic obstructive pulmonary disease (COPD)
Pulmonary Langerhans cell histiocytosis
Cystic fibrosis
Lymphangioleiomyomatosis
Lung cancer
Idiopathic interstitial pneumonia (IIP): idiopathic pulmonary fibrosis
Combined pulmonary fibrosis and emphysema
Sarcoidosis
If you don’t look closely, you might not even realise that I have pulmonary hypertension on top of my interstitial lung disease.
Hear from PH-ILD mermaids
Diagnosising PH-ILD can be challenging as its symptoms are nonspecific and can be confused with those of other underlying diseases.1
“The shortness of breath makes it almost impossible for me to catch my breath. Sometimes it feels like an elephant is sitting on my chest because I can't get any air.”10
“There's only 12 feet from my bed to the bathroom. By the time I get there, I'm completely worn out.”10
“I have a dry and unproductive cough, and sometimes the back of my throat itches. When I stay active or do too much, I run out of oxygen and my throat becomes soar.”10
These testimonials highlight the profound impact of PH-ILD on patients, emphasising the need for increased awareness and therapeutic intervention to address both symptoms and the effects on quality of life.10
How much do you know about pulmonary hypertension?
Let’s find out how much you already know!
Take the quiz to find out!Seeing is believing
What is it like to live with PAH?
“During adolescence I was breathless playing sports, it was too much effort. I felt like I was not getting enough oxygen. Also when skiing, or in the cableway. People use to tell me: “You are not sporty, you are not courageous”. I never consulted a doctor for that because I also told myself that I was not sporty, not courageous.”
What is the biggest frustration patients have as they embark on their journey?
“Everyone tells the patient something different. Sometimes it’s allergic asthma other times a myocardial insufficiency or he’s just fat or old. Some tell us that they saw a psychologist because they thought it was depression. There is a broad range of, sometimes absurd, different diagnostics. Not everyone will get through it, some just give up eventually.”
How do patients cope with the disease in the long-term?
“I would like to work, but I am too weak for it. I am exhausted all the time. I have no energy, I have no strength, I feel decrepit, I cannot sleep, I feel cold all the time. Five years ago, I could easily work for 12 hours, and now after 4 hours of computer work, I feel like I have been working for 15.”
Join us in
#DemystifyingRareDiseases
At Ferrer, our purpose is to use business to fight for social justice. We aim is to provide significant and differential value to people suffering from severe conditions. For that reason, we have defined a clear global strategy to develop a portfolio of holistic solutions and innovative products capable of transforming the lives of people living with serious and debilitating diseases, especially in the field of pulmonary vascular and interstitial lung diseases.
In order to raise awareness about Pulmonary Hypertension, we launched the Phantasticals to reaffirm our commitment to people living with PH.
Because we know they exist, we can identify them, care for them and put all our efforts into helping them.
May 5th
World Pulmonary Hypertension Day
PH frequently asked questions
Pulmonary hypertension or PH is high blood pressure in the arteries of the lungs that are connected to the heart to facilitate blood oxygenation. This high blood pressure hampers proper blood flow, leading to health problems in both the heart and lungs.1,2
What are the symptoms of pulmonary hypertension?
Explore the symptomsAre there different types of pulmonary disease?
See the variationsHypertension refers generally to high blood pressure in the arteries of the body, whereas pulmonary hypertension (PH) is specifically related to high blood pressure in the arteries of the lungs. PH encompasses five different classifications depending on the cause.1 While hypertension is usually related to lifestyle, pulmonary hypertension is more often a secondary complication related to other health issues.11
Meet the PH mermaid for more infoPulmonary arterial hypertension (PAH) is the first of the five classifications of PH. It occurs when lung arteries are damaged, making it harder for the heart to pump blood through them. Over time, this strain can lead to heart issues.1
Meet the PAH mermaid for more infoThe third classification of PH includes cases that develop as secondary complications from lung diseases such as chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD).
- COPD is a lung disease that causes airway obstruction, leading to reduced airflow and difficulty breathing
- ILD refers to a group of lung diseases that cause scarring (fibrosis) in the lungs, leading to stiffness and breathing difficulties.
Both conditions affect lung function and can increase blood pressure in the pulmonary arteries, leading to PH.1
Meet the PH-ILD mermaid to learn moreLung transplant is indicated for patients with PAH (group 1) or PH associated with a lung disease (group 3) that do not respond to medical treatment, as the main cause resides in the lung.1 Although it can be a potential “cure” for PH and improve patient survival and quality of life, these patients may face other health issues associated with the transplant acceptance. Therefore, rather than a “cure”, a lung transplant should be considered as a transition from PH to another medical condition with a better prognosis but that will still require medication and medical follow-up.12
References
1. Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;43(38):3618-731. 2. Armstrong I, Billings C, Kiely DG, et al. The patient experience of pulmonary hypertension: a large cross-sectional study of UK patients. BMC Pulm Med. 2019;19(1):67. 3. PHA Europe. About PH. Symptoms. Available at: https://www.phaeurope.org/about-ph/symptoms/. Last access: january 2025. 4. Hoeper MM, Humbert M, Souza R, et al. A global view of pulmonary hypertension. Lancet Respir Med. 2016;4(4):306-22. 5. PHA Europe. About PH. What is PAH? Available at: https://www.phaeurope.org/about-ph/what-is-pah/. Last access: january 2025. 6. Leber L, Beaudet A, Muller A. Epidemiology of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: identification of the most accurate estimates from a systematic literature review. Pulm Circ. 2021;11(1):2045894020977300. 7. Ferrari P, Skåra H. My life with pulmonary arterial hypertension: a patient perspective. Eur Heart J Suppl. 2019;21(Suppl K):K54-k9. 8. Strange G, Gabbay E, Kermeen F, et al. Time from symptoms to definitive diagnosis of idiopathic pulmonary arterial hypertension: the delay study. Pulm Circ. 2013;3(1):89-94. 9. Thomas CA, Anderson RJ, Condon DF, et al. Diagnosis and management of pulmonary hypertension in the modern era: insights from the 6th World Symposium. Pulm Ther. 2020;6(1):9-22. 10. DuBrock HM, Nathan SD, Reeve BB, et al. Pulmonary hypertension due to interstitial lung disease or chronic obstructive pulmonary disease: a patient experience study of symptoms and their impact on quality of life. Pulm Circ. 2021;11(2):20458940211005641. 11. HealthCentral. What’s the difference between high blood pressure and pulmonary hypertension? Available at: https://www.healthcentral.com/slideshow/difference-between-high-blood-pressure-and-pulmonary-hypertension. Last access: january 2025. 12. Pulmonary Hypertension Association (PHA). Answers about lung transplantation for pulmonary hypertension. Available at: https://phassociation.org/patients/treatments/transplant/finding-answers/. Last access: january 2025.
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