What are the priorities that, after a year of pandemic, haemophilia patients would like to see realized? The Federation of Hemophilia Associations (FedEmo) asked them on the occasion of the World Hemophilia Day 2021. Well 63% of patients believe that the pandemic could be an opportunity to increase home care and 75% ask to extend territorial services already existing and functioning for other pathologies, in order not to always have to go to the hospital, especially in emergency periods like this one, and thus lighten the burden of the Haemophilia Centers. Over half of hemophilia patients (57%) said of not having carried out, during the pandemic emergency, physical activity which instead it is an integral part of the care to prevent joint damage and micro-bleeding, which are not always evident. From the same survey emerge the services that they would like to see provided at home and / or on the territory: physiotherapy (30% of patients); blood test and home infusion (14%); distribution of drugs (11%); pediatric hemophilia surgeries (6%).
Difficult to access the Hemophilia Centers
Fortunately, haemophilic patients, unlike what happened with other rare diseases, have not encountered, due to Covid-19, problems in the supply of drugs and they have not been forced to modify the treatments in use. Confirming the needs highlighted, but also the need to make use of innovative tools, such as telemedicine, another survey conducted between November 2019 and June 2020 from the University of Milan Bicocca, in collaboration with FedEmo, highlighted that 15% of the interviewees found it difficult to access the Hemophilia Center even before the pandemic, because, for many, it is not easily accessible. Over 50% said they have difficulty walking, in 35% of cases to feel pain, while 16% was due refer to specialists outside the Center. “We ask the competent institutions to listen to the needs of patients – he declares Cristina Cassone FedEmo president, representing over 10,000 Italian patients – and promote the application of the Agreement on Congenital Hemorrhagic Diseases (MEC), thus ensuring greater efficiency and autonomy in therapies. We also wish to draw the attention of AIFA, hoping that the principle of therapeutic non-equivalence between drugs in haemophilia is soon sanctioned once and for all “.
La campagna social #MakeYourChoice
Hemophilia is one particularly delicate rare disease, being characterized by the deficiency, at various levels of severity, of a clotting factor following a genetic defect. A condition that can become limiting, if the person with haemophilia does not find the courage and support to accept their disease and live with it. There are many daily choices that those living with haemophilia – and with a rare disease, in general – are forced to make, but no one is alone and thanks also to the example of other patients you can live a free and rewarding life. Bring to the attention of the public and patients the importance of the decisions that each of them makes for themselves and for those close to them, without ever giving up on your dreams, is the goal of the social campaign #MakeYourChoice (“Make your choice”), launched on the occasion of the 17th World Hemophilia Day, which invites you to “wear” a rare disease and make your own life choice promoted by Uniamo FIMR Onlus and Uno Sguardo Raro with the support of Sobi. Through social media and a dedicated website, the campaign tells the point of view of patients, their stories, testimonials and offers a number of useful information to deepen the knowledge of rare diseases, starting with haemophilia. «In Italy – he explains Annalisa Scopinaro, president of Uniamo – there they are between 7,000 and 8,000 different rare diseases: we are talking about almost two million people, of which 1 in 5 is a child. These are very consistent numbers, worthy of great attention. In all these care settings, it is always the choices that determine life. The video #MakeYourChoice underlines, in a sort of “sliding-doors”, the importance of making the right choice when it comes to adherence to therapies. Only in this way can the person’s quality of life be improved ».
Pain management
Also on April 17, on the occasion of World Hemophilia Day, the meeting will be held in Palermo (online on the Zoom platform, 11 am https://us02web.zoom.us/j/81690215170#success, registration is not required) ” Hemophilia and joint pain in the life cycle: the multidisciplinary approach ”, promoted by the Associazione degli Amici dell’Emofilia Onlus, as part of the Articoliamo in tour campaign. The different aspects of the pain management: from the psychological aspect, to adherence to drug therapy, to the role of physical activity and physiotherapy. Target: raise awareness of patients and caregivers on the importance of prophylaxis factors with a prolonged half-life and the key role of ultrasound, to identify damage to the joints early and avoid severe arthropathies. “The Association of the Friends of Hemophilia of Palermo, founded in 1979 to support patients with coagulation problems, has worked over the years to improve the quality of life. Always committed to helping patients plan paths and build solid relationships with healthcare professionals, the association welcomed the “Articulate in Tour” campaign, whose goal is to connect specialists and patients, making them understand their weaknesses and how to best deal with them, ”he explains Maria Adelaide Celestino, president of the Friends of Hemophilia Onlus.
What is hemophilia
According to the website of the Higher Institute of Health, hemophilia is a rare disease caused by a protein deficiency involved in the coagulation process. Two of these, factor VIII and factor IX, are missing or not very present in people with haemophilia. Therefore, if in a healthy person a bleeding stops quickly thanks to the action of a series of plasma proteins, in a haemophilic subject, the lack of the aforementioned proteins it can turn a small lesion into a hemorrhage and provoke frequent, even spontaneous ones. Only in Italy about 5,000 people suffer from it, over 32,000 in Europe, almost all male. There are two types of haemophilia: “A” is the most common form and is due to a lack of coagulation factor VIII, present in one case in every 10,000 males; “B” is caused by a deficiency in coagulation factor IX and affects one in every 30,000 males. In both cases, the severity of the disease depends on the percentage of clotting factor present in the person’s blood. Treatment of the pathology provides the administration, via infusions, of the drug, for some time now no longer blood-derived but recombinant (ie produced in the laboratory with genetic engineering techniques), containing the deficient coagulation factor. The ISS was established National Register of Congenital Coagulopathies which collects data relating to the number and distribution of patients affected by Congenital Hemorrhagic Diseases (MEC), with particular attention to epidemiological surveillance of complications and the evaluation of the quantity of drugs prescribed for the therapies.
April 16, 2021 (change April 16, 2021 | 20:06)
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