This year too, World Thalassemia Day will be celebrated on 8 May, a precious opportunity to turn the spotlight on this hereditary pathology, which is quite widespread in Italy and in particular in Sicily and Sardinia but equally in our territory of the Po Delta, raising awareness among the population. , healthcare workers and institutions, especially to support thalassemia patients from all over the world. “Empowering human lives, embracing progress: for fair and accessible treatment of thalassemia for all”: this is the theme chosen for the 2024 edition to underline the fact that in different parts of the world there are still profound differences in terms of prevention, diagnosis and treatment of Thalassemia.
In fact, the evolution and prognosis of the disease strongly depend on the availability of blood and the quality of treatment. Unfortunately, thousands of thalassemia patients still die every year in the poorest regions of the world due to the lack of blood, drugs and appropriate therapies. Fundamental, dtherefore, on the occasion of this anniversary, raise awareness among the population of the importance of blood donation on which patients’ lives depend.
THE THALASSEMIA DH OF CONA HOSPITAL. In the Ferrara area, one of the areas of Italy with the highest presence of healthy carriers, the topic of Thalassemia has been addressed since the 1950s thanks to the creation of a Center dedicated to the treatment of this pathology, which was previously located in Savonarola at the former Pediatric Institute (IPI) under the guidance of prof. Marino Ortolani, then with the transfer within Sant’Anna, under the guidance respectively of prof. Calogero Vullo, by Dr. Vincenzo De Sanctis and Dr. Maria Rita Gamberini. Now the DHTE (Day Hospital for Thalassemia and Hemoglobinopathies) has its headquarters in Cona and is the HUB Center of a Regional network created in Emilia Romagna in 2005.
“The Thalassemia and Hemoglobinopathies (DHTE) Day Hospital of the Cona hospital – highlights Dr. Filomena Longo, Director of the Operational Unit – provides highly complex treatments to patients of all ages coming not only from our province but from the whole of Emilia-Romagna (as a Hub center of the regional “Hub & spoke Network”), as well as to patients who come for consultation from other regions. In 2023, over 400 patients with thalassemia and hemoglobinopathies referred to the DHTE of Ferrara for a path of diagnosis, therapy and follow-up of the disease. The reference medical team includes the paediatrician, the internist and the haematologist who have the important task of maintaining a global vision of the patient and his problems and of coordinating a multidisciplinary approach which involves many players including specialists of the Sant’Anna hospital (endocrinologist, cardiologist, hepatologist, ophthalmologist, transfusionist, radiologist, etc.) to address the complexity of these patients and manage the numerous possible complications. Precisely to cope with the growing number and complexity of cases, the treatment team has recently been strengthened with the arrival of a new internal medicine doctor and, shortly, another paediatrician. The Center also carries out clinical research as part of international studies on iron chelating drugs and agents that modulate hemoglobin production. Furthermore, several observational studies are underway on highly topical topics such as atrial fibrillation, pregnancy and monitoring of innovative therapies, in collaboration with the Italian Society of Thalassemia and Hemoglobinopathies (SITE)”.
Great importance is also given to the topic of prevention: thanks to the possibility of identifying, through simple tests, healthy carriers of Thalassemia, couples at risk are referred to the Medical Genetics service for counseling, with the possibility of accessing prenatal diagnosis. The teaching activity has also grown. The Level II University Master in Management of Thalassemia and Hemoglobinopathies, promoted by the University of Cagliari, which involved 7 Italian reference centers and trained 20 doctors in the field, has just ended. Since the current academic year, the University of Ferrara has established a specific course on Thalassemia and Hemoglobinopathies for medical students, which has been entrusted to Dr. Longo.
“Only with the active involvement of all the actors (hospital doctors, general practitioners, paediatricians, gynaecologists, consultants, etc.) and with the support of the institutions – concludes Dr. Longo – will it be possible to increase knowledge of such an important pathology in our territory.”
THE ROLE OF THE ASSOCIATION “Association for the Fight against Thalassemia – RINO VULLO” OF FERRARA. “I would like to extend a “huge thank you” to all blood donors and the associations that represent and organize them”. Valentino Orlandi, President of the “ALT Rino Vullo” Association of Ferrara and National President of UNITED (National Federation of associations of patients affected by this pathology), expresses himself in these terms.
“There are around 7,000 sick people in our country – continues Orlandi -, a thousand in Emilia-Romagna. The one in Ferrara is one of the centers that has contributed most to the scientific and clinical progress of Thalassemia, both at a national and international level. The synergies that have contributed to this result are many: collaboration with blood donor associations, with clinicians, hospital and regional health institutions, researchers who study and prepare drugs and diagnostics. Within this context there is also the contribution of the “Rino Vullo” Thalassemia Fight Association of Ferrara, founded in 1974, and which is still constantly engaged alongside patients and their families. This year will be 50 years since the birth of the Ferrara Association, one of the first in Italy. On May 10th, a meeting with the hospital authorities and the city’s institutions will be held in the Arengo Hall of the Municipality to remember and celebrate this important anniversary, coinciding with World Thalassemia Day. We are constantly working to collaborate with Donor Associations to increase the number of donations, even among young people, and meeting new segments of the population such as immigrants from countries that are carriers of Haemoglobinopathies (Nigeria, Morocco, Albania) to raise awareness among new residents in our area to become regular donors”.
For information on the work of the associations you can consult the websites www.altferrara.it and www.unitedonlus.org.
THE ILLNESS. Thalassemia is a hereditary hemoglobinopathy, very widespread in the world, and especially in the Mediterranean area, Asia, Africa and the Middle East. Countries like China, Iran, Egypt have tens of thousands of sick people. In recent years, following migration phenomena and the increase in multi-ethnic marriages, Thalassemia has spread to almost all countries in the world. In industrialized countries, the number of newborns with Thalassemia has been drastically reduced thanks to prenatal screening programs that have been active since the 1970s.
Italy has more than three million healthy carriers of Thalassemia and over seven thousand sufferers of the most serious form of this disease. Two parents who are healthy carriers of Thalassemia have a 25% chance, with each pregnancy, of transmitting the genetic error to their child which will prevent the normal production of hemoglobin and will make transfusion therapy necessary for life. The accumulation of iron resulting from chronic transfusions requires early and continuous therapy with chelating drugs to prevent numerous organ complications, especially affecting the heart, liver and glands.
The objective is very important, both at a national and above all international level, to offer a homogeneous level of care to patients, even in the most remote areas of the world. Without forgetting the war zones where patients with thalassemia and sickle cell disease suffer doubly from the inhuman condition of war and from the lack of care that this brings with it.
(by the Press Office of the S.Anna University Hospital of Ferrara)
