Living with beta-thalassemia takes days off the calendar. It always happens when you live with an illness, but now for thalassemia patients there are very specific numbers that show everyone the harsh truth: transfusions and visits on average take up 3 days a month, 36 in a year. There are many, especially for young people who feel robbed of the possibility of fully living even those days ‘free’ from visits and transfusions. The impact of the disease on the daily lives of patients and their caregivers is measured by a national survey carried out on patients and caregivers, still ongoing, conducted by Elma Research as part of the ‘Parallel Roads: the meaning of time in beta- thalassemia’ promoted by the Italian Society of Thalassemia and Haemoglobinopathies.
Beta-thalassemia, with cell therapy a six-year-old child will no longer need transfusions by Irma D’Aria 09 June 2023
The survey of patients and caregivers
The research, still in progress, was carried out on a sample of 106 participants and highlights how those who live with beta-thalassemia dedicate hours and hours, every week, to the disease and its management: on average there are 4 hours used every week for specialist tests and 19 days each year are dedicated only to transfusions. “The dimension of time is fundamental in the experience of patients with beta-thalassemia, as also emerges from the survey we conducted: the need to dedicate so much time to treatments and checks influences the way in which patients live their lives”, he explains Patrizia Amantinidirector of Elma Research.
One in 4 patients needs a caregiver
Today in Italy there are at least 7 thousand people who have the disease, of which 73% need transfusions. “Thousands of families are called upon to carry out demanding management of care and checks, day after day, year after year”, underlines Raffaella Origa, president of the Italian Society of Thalassemia and Haemoglobinopathies (Site). “Just think that 1 in 4 patients needs to be accompanied by a caregiver every time they go for a transfusion or a visit. The person who accompanies patients on their treatment journey is very often a parent, even over the age of 18. of age”.
Beta-thalassemia, prenatal diagnosis can avoid over 400 cases a year by Tiziana Moriconi 02 May 2023
The burden of the disease on younger people
The perception of being almost robbed of one’s time is greater among younger patients. 46% of those aged between 21 and 40 say that the disease changes their habits, 54% the possibility of travelling, 38% of having a stable relationship or getting married, 42% of having children. “The presence of beta-thalassemia means for many patients having to often ask for help (68%) to manage their children, take care of the house or
be accompanied on visits; it also means having to give up one’s hobbies and interests, holidays and travel and moments of relaxation in general, and for 30% it corresponds to a reset of one’s life in terms of work and family projects”.
It is therefore not surprising that in patients under 20 years old, common feelings are those of incomprehension, confusion and discouragement; in those between 20 and 45, loneliness and stress weigh the most; while for older ones it is above all fatigue that conditions life.
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The loneliness of caregivers
But when an illness enters the family it not only changes the life of those affected: everyone is an active part in some way and those who become caregivers often feel misunderstood, stressed and discouraged. In general, more than 3 out of 10 patients think that the pathology has a major impact on their life, a feeling that is exacerbated for the most active group – between 40 and 50 years old. Over 30% require the intervention of a psychologist
or a psychiatrist. “Beta-thalassemia is a genetic condition with which we can live today, become adults and elderly. Despite improvements in treatment and a life expectancy that was unimaginable until a few decades ago, the need for daily therapies and frequent hospitalizations continue to profoundly impact the lives of patients and their loved ones”, comments Origa.
Symptoms of beta-thalassemia
Beta-thalassemia is a genetic, hereditary disease caused by a defect in the production of hemoglobin, the protein responsible for transporting oxygen throughout the body. If both genes of the beta chains that form hemoglobin are mutated, beta-thalassemia major occurs, which presents a severe clinical picture with severe anemia.
The symptoms of this form of the disease appear already in the first months of life and, if adequate therapies are not intervened, the consequences can be severe anemia, bone deformations, enlargement of the spleen and liver, growth problems, hepatic, endocrine and cardiovascular complications . It is a disease that forces patients, from childhood, to undergo regular blood transfusions at intervals of 2-3 weeks throughout their lives. Not only that: every day they must also take iron chelation therapy, to limit the accumulation of iron in organs such as the heart, liver and pancreas.
Thalassemia, Cutino Foundation: “Summer transfusion emergency: blood collection even on the beaches” by Irma D’Aria 28 June 2023
An awareness campaign
It is difficult to realize the implications of this disease if you don’t know someone who is facing it and this is why Site promotes the ‘Parallel Roads’ campaign. Beta-thalassemia: voices, images, needs’, created with the unconditional contribution of Vertex
Pharmaceuticals, a project that aims to make us reflect on how time is a fundamental element in the life of every individual and how beta-thalassemia can profoundly influence its management. “It is precisely starting from the evaluation of this dimension of subtraction of time – for leisure, for loved ones, for study or for work – well photographed by Elma’s research, that we think it is fundamental to bring out the challenges that patients face, but also how clinicians and institutions must collaborate to find solutions and resolve critical issues on a clinical and organizational level”, states Giovan Battista Ruffo, advisor to the Site Steering Committee.
A clock with one hour less
Not surprisingly, the campaign logo – created by the Doodle Artist Francesco Caporale (aka Fra) – has a clock in the center with 11 hours instead of 12 and parallel streets which represent on the one hand the ‘normal’ life of all people also made up of hobbies such as sport and music and on the other the time spent in hospital for visits and transfusions. In addition to an advertising campaign, a booklet will be created with patient testimonies together with the contribution of the experts who are at their side to help them deal with and manage the pathology. Furthermore, some awareness-raising events are planned which will affect some Italian regions with a greater prevalence of the disease in the coming months. For more information on the pathology and the “Parallel Roads” campaign, the dedicated landing page has been created: www.stradeparallele.it.