8/9/2022

World Cystic Fibrosis Day

“Winning back our lost breath”

September 8, World Cystic Fibrosis Day, the most common inherited disease, affecting more than 80,000 patients worldwide with a low life expectancy. Cystic fibrosis affects many organs and systems of the human body, mainly the lungs and pancreas, and is usually diagnosed in infancy. The main characteristic of the disease is the appearance of highly viscous and dehydrated secretions and mucus in various organs and glands of the body, which gradually clog and destroy the organs. The complications of the respiratory system, which over time destroy the lungs, are considered critical to the course of the patients, having the overwhelmingly highest proportion among the causes of mortality from the disease.

Every year 1,000 new cases of Cystic Fibrosis patients are diagnosed worldwide and there are more than 2,100 gene mutations of the disease. In Greece there are more than 720 registered patients suffering from Cystic Fibrosis, and it is estimated that more than half a million of the country’s population are carriers of the disease. It is essential for new couples who want to have children to undergo molecular testing to determine whether they are carriers of the disease.

On the occasion of the celebration of World Cystic Fibrosis Day, the Vice President of the Hellenic Cystic Fibrosis Association, Irini Katsini, states the following:

“This year’s World Cystic Fibrosis Day finds us with medicine for all Cystic Fibrosis patients in Greece. This summer marks 2 years since the day the first critically ill Greek patients started the revolutionary innovative treatment that ‘freezes’ the disease. In continuous collaboration with the Ministry of Health and the relevant authorities, after the coordinated struggle of our Association, for which we were awarded the Prix Galien Greece 2021, currently all eligible adult patients and children over 12 years old have access to the innovative treatment. In fact, with the recent approval by the European Medicines Agency for administration at younger ages, our young patients 6-12 years old are gradually starting the treatment, which will help them to manage the symptoms of the disease very early in their lives.

At this year’s ECFS European Cystic Fibrosis Congress, the positive results of long-term treatment were presented, which not only improved patients’ lung function, but reduced the risk of exacerbations by 77%, the risk of lung transplantation by 87% and the risk of death by 74%

Today we are in the final stage of completing the reimbursement procedures for treatment in our country with the recommendation of the Drug Price Negotiation Committee in the hands of the Minister of Health to be signed. At the same time it is very encouraging that our Greek patients are participating in the clinical trials of the next innovative treatment, which is expected to have even more positive results.

However, for a small number of our patients who are not eligible for the breakthrough therapy due to their rare gene mutations and are in end-stage respiratory failure, the only survival option is lung transplantation. With the initiatives of our Association, a transnational agreement had been reached between the National Transplantation Agency (EOM) and a transplantation centre in Austria, resulting in saving the lives of about 20 of our patients who underwent lung transplantation in Austria from 2015 to 2019.

Since 2019, the National Lung Transplant Program was launched in Greece with our country having a low rate of organ donation. In one year we lost 3 of our patients who did not manage to gain a 2nd chance for life, among them our Association’s Secretary General, Vassilis Palios, who fought for transplants until his last breath. But last month, the first successful lung transplantation of a Cystic Fibrosis patient in Greece took place, giving all of us smiles of optimism and hope to those patients who are waiting for the gift of transplantation.

In addition to the actions to raise awareness of the society for organ donation through our campaign “Breathe Unlimited Breath- Be a Life Donor”, our Association’s priorities are to ensure that all the necessary standards and procedures for the follow-up of our transplant patients from Vienna who are followed in our country are met, as lung transplants are delicate and often present various complications and rejection by the body.”

Continuing, the President of the Hellenic Cystic Fibrosis Association, Anna Spinou, states:

“Early diagnosis of Cystic Fibrosis and innovative treatments that act on the causes of the disease have contributed to a significant increase in the survival of our patients, with 50% of patients worldwide now being adults, compared to the 1980s when only 30% reached adulthood. In fact, it is estimated that by 2025, 75% of Cystic Fibrosis patients will be adults. This creates a huge pressure to strengthen and adequately staff our country’s adult Cystic Fibrosis Units to adequately meet the needs of our patients.

In our country there are 2 adult units: the Adult Cystic Fibrosis Unit of the Sismanogleio Hospital in Athens and the Cystic Fibrosis Centre of the Aristotle University of Thessaloniki at Papanikolaou in Thessaloniki, which are covered by only one specialised resident pulmonologist in each unit, creating significant shortages in the follow-up of adult patients. Our Association has requested from the Ministry of Health the immediate appointment of a second permanent pulmonologist for the Cystic Fibrosis Centre in Papanikolaou, for the adequate monitoring of our patients from Northern Greece, while we expect soon the completion of the procedures for the appointment of the second permanent pulmonologist in the Cystic Fibrosis Unit of Sismanoglio.

As I presented as a speaker at the European Lung Foundation’s annual event a few days ago, very important for patients with Cystic Fibrosis is the development of programmes and tools for self-management of the disease. Since the advent of the coronavirus pandemic in our country, at the initiative of our Association, we have so far donated 300 individual spirometers to our patients across the country so that they can do the necessary regular spirometry test themselves remotely from their homes.

Also this year for the first time our Association started a pilot program of psychological support groups for patients and their caregivers, which is a voluntary service of Dr. Dipl.-Psych. Stavroula Rakitzi, with positive first results.

The Hellenic Cystic Fibrosis Association is one of the longest-standing patient associations in Greece, and in a few months we will be celebrating 40 years of operation since its foundation in 1983. Through a long and difficult journey of four decades, parents and patients from all over Greece have joined forces and have fought hard to ensure “Unlimited Breath” for all Cystic Fibrosis patients in our country. The 40 years of teamwork has paid off thanks to the tireless contribution of all our Boards of Directors and members of our Association, the development of trusting relationships with institutions, collaborations and our participation in institutional representation.

With the motto “Unlimited Breath” the Cystic Fibrosis community of our country managed to change the map of Cystic Fibrosis in Greece, proving that only through the team we can make our dreams come true. But our actions and our work continue, as there is still much more to be done.

With early diagnosis and innovative treatments, the disease is gradually transforming from a life-threatening to a chronic treatable disease. The difficult days are over, and in the coming years we will also have treatments for patients with rare mutations and gene therapy, for which clinical trials have already begun. So this year we celebrate World Cystic Fibrosis Day with optimism and hope that very soon all Cystic Fibrosis patients will be able to regain our lost breath!”