European Cystic Fibrosis Week
19-25 November 2017
What is Cystic Fibrosis
Cystic Fibrosis is the most common inherited disease in the white race and in our country it is estimated that one child is born every week. For a child to be born with Cystic Fibrosis they must inherit a defective gene from each carrier parent. In Greece there are an estimated 500,000 carriers, and it is possible to find out if you are a carrier through prenatal testing. It is a multisystemic disease as it affects and destroys various organs, especially the lungs, resulting in the vast majority of patients ending up with respiratory failure. To date there is no definitive cure.
The situation in Greece according to the recorded data as described by Mrs. Chatziagorou Elpida, Assistant Professor of Medicine. Professor of Pediatrics – Pulmonology, Aristotle University of Thessaloniki, Department of Cystic Fibrosis, Hippocratic Hospital of Thessaloniki.
”As the scientific coordinator and representative of Greece in the European Registry, I am extremely satisfied as the last registration of 2015 includes the centres of Athens and Thessaloniki with 590 registered patients, which we estimate to represent 95% of the patients in Greece. It is worth noting that the data in Greece is similar to the average in Europe: a) the average age of patients in our country is 19.5 years old – similar to the average in Europe (20 years old), b) patients are diagnosed early – the average age of diagnosis in Greece is 2. 85 years, while in Europe it is 4.1 years; c) half of the patients (51%) in Greece are now adults; d) the respiratory function and nutrition of the patients are at excellent levels: the average respiratory function (FEV1) in children is 95% and in adults 66% – similar to European data; e) the average respiratory function (FEV1) in children is 95% and in adults 66% – similar to European data.
The Registry has multiple benefits for patients, as it is a key tool in the hands of the physician who can assess the level of health of patients compared to other countries, check where they excel and improve where they are lagging behind.”
News about innovative drugs and treatments from the Coordinating Director of the Cystic Fibrosis Centre of the Aghia Sophia Children’s Hospital, Ms Ioanna Loukou.
”In recent years, new innovative therapies targeting the causes of the disease are constantly being developed. So far, only two drugs (KALYDEKO and ORKAMBI) have received marketing approval, targeting sufferers carrying specific mutations of the disease (there are more than 2,000 registered) and not all patients. The second is for homozygotes of the most common F508del mutation, aged over 12 years, and very soon this treatment will be extended to younger patients. As an indication, the Cystic Fibrosis Centre of the Aghia Sophia Children’s Hospital – which is the largest centre in Greece with more than 300 patients – is already using the new treatments for about 75 patients.In 2018, the approval of a newer combination of drugs that will target both homozygotes and certain heterozygotes of the F508del mutation is imminent.
It is estimated that by 2020, 90% of patients will have access to treatments of this type.
Gene therapy, which targets all patients regardless of the mutations they carry, is in an earlier phase of development, but with encouraging results so far.”
The situation of adult patients and transplants today is described by Mrs. Filia Diamantea, director of the Adult Cystic Fibrosis Unit at the General Hospital “Sismanoglio”.
”In the Adult Cystic Fibrosis Unit at the Sismanoglio General Hospital 220 patients are currently being monitored and treated. The Unit receives about 20 new patients per year from the corresponding Paediatric Unit at the “Aghia Sophia” Children’s Hospital. As the survival rate of patients with cystic fibrosis has increased significantly in recent years, it has been estimated internationally that the number of patients in the Adult Units is set to increase by 50% over the next 10 years. It is therefore imperative that adult departments be strengthened with the necessary qualified medical and paramedical staff. According to international standards, the monitoring and care of these patients should be carried out by specialised staff from a number of disciplines, coordinated by a specialist pulmonologist. The number of specialist pulmonologists should be 1 for every 100 patients. At the same time, specialised doctors of other specialities (Gastroenterologist – Hepatologist, Endocrinologist – Diabetologist, ENT, Rheumatologist, Thoracic Surgeon and Interventional Radiologist) must accompany the unit, given the fact that many systems of the body are affected by the disease. At present, the adult unit of the Cismanoglio employs 1 permanent and 1 auxiliary pulmonologist , and the recruitment of a 2nd permanent attending is expected.
Because in adult patients the most severe complications of the disease involve the respiratory system, lung transplantation is the only viable option in the final stages of respiratory failure. Since unfortunately in our country there is no transplant center performing lung transplantation, the adult unit at Sismanoglio has been connected to the University Hospital of Vienna where 40 successful transplants have been performed in our patients in the last years. Because the cost of the operation and the subsequent monitoring and care of the patients is exorbitant, there is an immediate need to organize, at least in the first phase, a system of monitoring of patients after the first year of transplantation in our country where, in cooperation with the Vienna Transplant Centre, the supervision of the patients will be undertaken. In a second phase, a well-organised transplant centre specialising in lung transplantation should also be established in our country.”
Goals, concerns and challenges for 2018 are analyzed by the President of the Hellenic Cystic Fibrosis Association Dimitris Kontopidis
”The news for the bright European Cystic Fibrosis Week is more than welcome. New medicines for more and more patients who with persistent struggles we managed to get reimbursed in our country. More and more adult patients are becoming numerically more numerous, and life expectancy is increasing significantly. Transplants in the last 4 years are close to reaching the number of all lung transplants performed on our patients since 1983.
It would not be an exaggeration to say that Cystic Fibrosis as a serious condition will soon be a thing of the past, as children born today are estimated to have a life expectancy of over 60 years – without the use of innovative drugs.
Indeed, in the last few years of the unprecedented economic crisis in our country we have managed to meet decades-old demands. We have managed to radically change the tragic situation in our country as far as our disease is concerned, increasing life expectancy, which until a few years ago was perhaps a decade less.
By turning ”complaining” into realistic proposals and partnerships we have achieved tremendous improvements in specialist health care for our patients. Collaborations with scientific institutions, physicians, health care providers in Greece and abroad have been a catalyst for this development. Perhaps the most important achievement is the awareness and knowledge about the disease and keeping the needs of Cystic Fibrosis patients ”high on the agenda” of state priorities.
Of course, we are far from our targets in relation to European standards, which will remain as a guide in our demands.The biggest problem is the understaffed adult CF units of the ‘Sismanoglio’ in Athens and the ‘Papanikolaou’ in Thessaloniki, which operate thanks to the self-sacrifice of doctors and minimal staff, where if a solution is not found immediately there will be a serious problem. The ‘hospitality’ of our patients for transplants in Austria, apart from the exorbitant costs, also has an expiry date. If we do not proceed in time with a follow-up programme for our transplanted patients in our country and then develop it into a full transplant programme in Greece, it will be another tragic end to the loss of our patients’ acquisition of a second chance at life. The National Transplantation Organization (NTO), which we are in constant contact and cooperation, has repeatedly analyzed the plan of action that should be supported in time by the State.
As responsibly informed patients we will continue to contribute to finding realistic solutions in constant contact with the European Cystic Fibrosis community. Our successful collaborations on transplantation issues with Austria, the linking of the C.F. unit of Sismanoglio with the corresponding specialized adult C.F. unit of Southampton at no cost to the Greek state and the effective claiming of reimbursement of innovative drugs for patients are the result of seeking and implementing solutions through best practices abroad. The expectation of approval of clinical trials of innovative drugs for heterozygous patients with the df508 mutation in our country in early 2018, the inclusion in the reference centers of the C.F. units, home care, the start of transplantation programs in our country and finally the inclusion of exercise and physiotherapy programs on a daily basis that will give a longer life expectancy and quality of life to our patients, are our main goals for 2018.”
Unlimited breath for life without limitations!
”BreathUnlimited – Lifeunlimited”
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* Watch the TV spot (45”) of the Hellenic Cystic Fibrosis Association about prenatal screening here. (our patients themselves participate in the video)
** See the TV spot ( 2.30”) ”Living with Cystic Fibrosis” – ”LivingwithCF” by the Hellenic Cystic Fibrosis Association about life here. (our patients themselves participate in the video)
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LEARN MORE
about the disease from the Hellenic Cystic Fibrosis Association at www.cysticfibrosis.gr
SUPPORT CYSTIC FIBROSIS PATIENTS
for unrestricted breathing and unrestricted life: National Bank of Greece, IBAN: GR 7401101780000017829600521
WE_ARE_WAITING_FOR_YOU
At 25 & 26 November at The Mall Athens to get your own ”BreathUnlimited” , apparel & accessories dedicated to the empowerment of patients with Cystic Fibrosis
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