It can happen anywhere and at any time: suddenly you can’t breathe anymore, you’re out of breath and you have the sensation of sinking into silence and darkness, without strength. This constant ‘hunger for air’ is the dramatic experience shared by people who live with pulmonary arterial hypertension (PAH), a rare disease that ‘takes your breath away’. A progressive and disabling condition characterized by increased blood pressure in the pulmonary arteries, resulting in heart strain. IAP compromises the patient’s daily life and social, working and emotional life, with a heavy impact on the family and caregiver. If not adequately treated, it degenerates, also involving the heart, which is no longer able to pump blood through the lungs. Increasing knowledge and awareness of the pathology is the goal of ‘At the heart of breathing. Pulmonary arterial hypertension, know it in depth’, a campaign promoted by MSD with the patronage of Amip (Association of pulmonary hypertension patients) and Aipi (Italian Pulmonary Hypertension Association).
To enter the daily reality of those who live with IAP, the campaign – the promoters explain in a note – redefines the narrative of the disease, telling it through a metaphor: the journey of those who free-dive into the depths of the sea, descend into a hostile environment , dark, silent and challenges one’s limits, trying with determination and courage to overcome them every time to slowly rise towards the light and the air. ‘Apnea’, the video of the campaign – available on the website www.alcuoredelrespiro.it – is based on these images, starring the testimonial Alessia Zecchini, world freediving champion. The tools of the campaign are the website, the point of reference for the initiative, and social activities which are developed through an information path with videos, interviews and insights available to patients always on www.alcuoredelrespiro.it.
It is estimated that in Italy there are 3,000-3,500 people affected by PAH and in Europe around 30,000. The symptoms of pulmonary arterial hypertension fade at onset, but then progressively worsen. Non-specific symptoms delay the diagnosis for years, while the time factor is crucial for arriving at an early diagnosis and referring patients to reference centers, equipped with a multidisciplinary team capable of taking care of the patient in all its complexity and administering the best option therapeutic. “The PAH originates directly in the pulmonary arterioles, and causes increased pressure in the small circulation and heart failure due to the involvement of the right side of the heart – describes Michele D’Alto, head of the Pulmonary Hypertension Center, Department of Cardiology, Monaldi Hospital, Naples – E ‘more frequent in women, but has a more severe course in men; it favors the broad age range between 30 and 60 years’, and ‘management is more complex in children and the elderly less specific and more common symptoms are shortness of breath, breathlessness, swelling of the legs, which is why the diagnosis is difficult and often late (more than 2 years) and treatment in an expert center can save many lives”. It is therefore “fundamental the collaboration between clinicians, patient associations, pharmaceutical companies and institutions which, through constant dialog and synergies, can provide answers to the still unsatisfied clinical needs of people affected by PAH”.
People living with pulmonary arterial hypertension have clinical, psychological and social needs and requirements, the note continues. PAH is an invisible disease because air hunger does not cause pain, the symptoms are often overlooked or confused with those of other cardio-respiratory pathologies, and the diagnostic-therapeutic pathways are often uneven at a regional level. There is therefore a strong need for management and psychological support for patient and caregiver. Amip and Aipi are the associations that for years have supported the difficult journey of patients, helping them to inform and deal with therapies, family and work problems, by listening and welcoming and with psychological support.
“The ‘At the heart of breathing’ campaign aims to highlight the problems and difficulties of daily life that the patient and caregiver encounter in managing the disease and in everyday life. And it does so
“We joined immediately with great enthusiasm – says Pisana Ferrari, president of Aipi – because we were struck by at least two elements of the campaign. The first is water: a poetic and delicate way to talk about a pathology that takes your breath away. The second it is precisely the hunger for air that freediving, the chosen sporting metaphor, evokes with great force. In recent years the involvement of patient representatives has become increasingly central in the work of scientific societies, research institutes, European institutions. of the regulatory authorities. Personally I had the pleasure of being able to bring the voice of patients as a patient expert to the EMA, the European Medicines Agency, and as co-author in the drafting of the 2022 guidelines on pulmonary hypertension of the European societies of cardiology and pneumology, virtuous examples of concrete engagement of patients”.
In the video ‘Apnea’ the story of Alessia Zecchini is intertwined with that of a patient with pulmonary arterial hypertension: in their intimate dialogues on the seashore, in a subtle play of parallels, the elements that differentiate their paths delicately emerge, but even traits in common of those who live ‘in apnea’, illustrate the promoters of the campaign. “Heart and breathing are fundamental elements in my sporting discipline – says Zecchini – Breathing is a natural and physiological function, we breathe every second of our life and knowing that there are people who have to face a limitation of this vital function, which too often we give for granted, it made me immediately feel involved as an athlete and as a person in this campaign. Both freedivers and patients have to face an arduous ‘journey’, which first of all is within us to face our fears, our emotions, the our physical limits. But never alone, because every goal can be achieved thanks to the support of a team, whether sports or medical, and obviously, in the case of patients, also thanks to research. For me it was a great pleasure participating in the ’At the heart of breathing’ campaign is an experience that has enriched me as a person and I really hope that it can also help patients.”
In the past, the treatment of PAH consisted of lung or heart-lung transplantation as the only therapeutic option, the note recalls. In the last 10 years the therapeutic path has changed, with the introduction of drugs that extend survival and improve patients’ quality of life. However, there remains a great need for innovative therapeutic strategies. “‘Inventing for life’ has always been MSD’s mission and it is also the case in rare diseases”, highlights the group. “As a company committed to improving and saving people’s lives – states Nicoletta Luppi, president and managing director of MSD Italia – we have a fundamental role both in raising awareness and promoting information on a rare pathology such as pulmonary arterial hypertension, and the campaign ‘At the heart of breathing’ is an excellent example of this, both in advancing our research to find increasingly innovative therapeutic strategies.”
“Our commitment – continues Luppi – will be maximum alongside institutions, associations and the scientific community, so that our therapeutic innovation reaches the people who urgently need it in a timely and effective way. And for this to happen – he specifies – it is necessary to act to eliminate the regulatory and bureaucratic brakes that hinder it, guaranteeing equal access to care and, at the same time, the attractiveness of our market with the introduction of new rules to manage innovation and define new mechanisms for early access to medicines innovative, we can really change the patient care journey with Iap. I am proud to see that this is the common objective that guides all of us ’actors’ of the health system, gathered in an important event like today’s, which sees us converge our efforts to guarantee the right to health of patients too with pulmonary arterial hypertension”. Says Louise Houson, President Core Europe & Canada Region, MSD International: “Our commitment to making a meaningful difference for patients extends to rare diseases such as pulmonary arterial hypertension, which remains a high-need public health challenge. dissatisfied doctor. I am convinced that the collaboration and partnerships between all the sectors involved, united today for the launch of the campaign, will generate the awareness and action necessary to help save and improve many lives in Italy”.
#life #apnea #campaign #heart #breathing #tells #story #Iap
What are the symptoms and challenges faced by patients with pulmonary arterial hypertension (PAH)?
Interview between Time.news Editor and Michele D’Alto, Expert in Pulmonary Arterial Hypertension
Time.news Editor (E): Good afternoon, Dr. D’Alto, and thank you for joining us today. Your expertise in pulmonary arterial hypertension (PAH) is crucial, especially with the recent ‘At the heart of breathing’ campaign gaining attention. Can you start by explaining what PAH is and its impact on patients?
Michele D’Alto (D): Good afternoon, and thank you for having me. PAH is a rare but serious condition characterized by elevated blood pressure in the pulmonary arteries, which can lead to heart strain and progressive disability. Patients experience shortness of breath, a feeling of suffocation, and other debilitating symptoms that significantly affect their daily activities and emotional wellbeing.
E: That sounds incredibly challenging. I understand that the campaign uses the metaphor of freediving to illustrate the struggles faced by those with PAH. Could you elaborate on that metaphor?
D: Absolutely. The metaphor of freediving is quite poignant. Just as freedivers dive deep into an environment that can feel suffocating and dark, PAH patients face a daily struggle with their breath and energy levels. The journey upwards, towards the light and air, symbolizes the fight against the limitations imposed by their condition. It’s a powerful reminder of both the physical and emotional battles they endure.
E: The campaign aims to raise awareness about the disease, but what do you believe are the most critical issues that need addressing in PAH management?
D: One of the most pressing issues is the delayed diagnosis. Symptoms are often non-specific and can mimic other respiratory conditions, leading to years of misdiagnosis. Early intervention can save lives, so increasing awareness among both the general public and healthcare professionals is essential. Additionally, there’s a need for coordinated care through specialized centers that offer a multidisciplinary approach to treatment.
E: It’s staggering to think that many patients go undiagnosed for so long. How does this lack of awareness affect the psychological and social aspects of their lives?
D: The repercussions are significant. PAH is often referred to as an ‘invisible disease’ since its impact isn’t readily apparent. This can lead to misunderstandings among family, friends, and employers. Patients may feel isolated as they struggle with anxiety and depression due to their condition. Hence, support from patient associations like Amip and Aipi is invaluable as they provide not only information but also psychological support.
E: Speaking of support, how crucial is collaboration among clinicians, pharmaceutical companies, and patient advocate groups in improving outcomes for PAH patients?
D: Collaboration is vital. It allows for the sharing of knowledge and resources, which leads to better treatment strategies and support systems. Having patient representatives involved in discussions and decision-making processes is also essential for ensuring that the needs of patients are prioritized in research and policy-making.
E: The ‘At the heart of breathing’ campaign enlists prominent figures, like the world freediving champion Alessia Zecchini, to help spread the message. How do you think the involvement of such influential individuals enhances awareness?
D: Having a high-profile advocate like Alessia Zecchini brings attention to the campaign in a unique way. It humanizes the condition and fosters a connection between the audience and the struggles faced by PAH patients. Her involvement also signifies the importance of breath—an intrinsic part of both freediving and living with PAH—thereby emphasizing the urgency of the message.
E: What advances have been made in the treatment of PAH in recent years?
D: Fortunately, there have been significant advancements. A decade ago, treatment options were limited primarily to lung transplants. Now, a variety of medications have been developed that not only prolong life but also improve the quality of life for patients. However, ongoing research and development of innovative therapies are crucial to address the unmet needs of these patients.
E: Lastly, what advice would you give to individuals who suspect they may have PAH or are navigating a diagnosis?
D: My foremost advice is to seek medical attention promptly. If you’re experiencing symptoms like breathlessness or fatigue, don’t ignore them. Advocate for yourself within the healthcare system. Seek out specialists in PAH who can provide comprehensive care. Support groups and organizations like Amip and Aipi are also invaluable resources for information and encouragement.
E: Thank you, Dr. D’Alto, for sharing your insights. Your work and the ongoing campaigns like ‘At the heart of breathing’ play a crucial role in advancing awareness and improving the lives of those affected by PAH.
D: Thank you for this opportunity to discuss such an important topic. Together, through education and support, we can make a difference in the lives of PAH patients.