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Castleman disease, more information and rights for rare patients

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So rare that it is not even on the list of rare diseases. Also for this reason, Castleman’s disease is little known not only among the public but also among doctors. From this lack of knowledge, the idea of ​​founding AMICa, Castleman’s Italian Sick Association, was born, which aims to spread greater knowledge of the disease and support patients who are affected by it, defending their rights and giving them a voice.

What is Castleman’s Disease

Castleman’s disease, first described by US pathologist Benjamin Castleman, and? a rare disease that can affect all ages and includes a heterogeneous group of lymphoproliferative disorders. In affected patients, there is a condition of systemic inflammation, an increase in the size of the lymph nodes and possible damage to various organs. Based on the number of lymph node stations involved, two subtypes of Castleman’s disease are distinguished: the unicentric, that is, localized form, which is characterized by the involvement of only one lymph node station (about 50% of cases); and the rarer and more aggressive multicentric form associated with systemic symptoms and the involvement of multiple lymph node stations (approximately 50% of cases). “Castleman’s disease can present itself in very different forms, but in general it is a systemic inflammatory disease, that is, an inflammation that involves the whole organism,” he says. Simone Ferrero, vice president and coordinator of the AMICa Scientific Committee and researcher at the University Hematology of the Department of Molecular Biotechnologies and Health Sciences, University of Turin. “The initial symptoms can be very general: in addition to swelling of the lymphatic glands, fever, muscle aches and weight loss, patients may have changes in blood tests together with kidney, lung or bone marrow damage. bone”.

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The difficulty of diagnosis

So long as? rare and with symptoms similar to some tumors and autoimmune diseases, Castleman Syndrome can? be difficult to diagnose. Due to its complexity, other infectious, autoimmune and lymphoproliferative conditions must be ruled out in the diagnosis stages before concrete suspicion of Castleman disease exists. For this reason, a transversal and multidisciplinary approach involving various specialist figures is essential to make a correct and early diagnosis. “Castleman’s disease is one of those pathologies in which close collaboration between different professional figures becomes fundamental – he said Pier Luigi Zinzani, member of the AMICa Scientific Committee and full professor of Hematology, Department of Specialized, Diagnostic and Experimental Medicine, University of Bologna. “It often happens, in fact, that patients pass through departments of internal medicine, infectious disease, oncology and rheumatology before arriving in the hematology department; and this not only causes delays in the diagnosis, but also a lesser optimization of the therapeutic approach. AND? therefore, it is essential to promote greater training for specialists and a more significant culture of multidisciplinarity “.

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The therapies

Can this disease be cured? “In the localized forms, the treatment is generally of a surgical type and is almost always resolutive; for the systemic form, however, the therapy is usually pharmacological. To date, fortunately, we have some therapeutic weapons at our disposal that allow us to tackle the disease as a whole, as well as to alleviate symptoms and manage sudden worsening ”, explains Ferrero.

Inclusion in the list of rare diseases

Fundamental for patients suffering from Castleman’s disease is the taking charge by the National Health Service of the entire path of diagnosis and treatment with the recognition of patients suffering from rare disease and the guarantee of free and free access to all forms of treatment, diagnostic examination and medical check-up. “In recent months we have reached very important milestones for rare patients and their families, but we are aware that there is still a lot of work to be done. Castleman’s disease, for example, isn’t it? still included in the list of rare diseases and therefore does not enjoy a specific exemption code that allows patients to undergo visits and examinations for free. – recalled theHon. Fabiola Bologna, Secretary XII Commission for Social Affairs and Health ?, Chamber of Deputies. As a spokesperson for rare patients in Parliament, I reiterate my commitment to give regulatory responses to this type of need as soon as possible, so? to improve the quality of life of patients and their families “.

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The update of the Essential Assistance Levels

The latest update of the Leas in 2017 also provided for the complete reorganization of the list of rare diseases, to allow those who suffer from them to access the exemption more easily. Four years later, however, there are still rare diseases that do not fall on this list, such as Castleman’s disease. “This clearly has important repercussions first and foremost on patients, but also on clinicians who often find themselves having to assign incorrect exemption codes to their patients,” said the Sen. Maria Domenica Castellone, 12th Commission for Hygiene and Health?, Senate of the Republic. “As institutions we must work to ensure that registers and lists are constantly updated and in step with advances in scientific research. Only with careful and assiduous work will we be able to guarantee patients total support from the National Health System and the scientific community in more adequate working conditions ”.

Little information

There are an estimated 21 cases per million people in the United States. Precisely this low incidence and the difficulty in diagnosis have inevitably determined a lack of information as well as a considerable difficulty for patients in finding realities prepared to manage the effects of the disease. “Castleman’s disease is poorly understood among patients, but also within the scientific community; this causes a sense of disorientation in patients who feel isolated and without a guide who can give them certain indications “, declared Claudius Sava?, president of AMICa, during the presentation event organized with the unconditional contribution of Eusapharma.

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The objectives of the Association

AMICa, Castleman’s Italian Sick Association, was created to directly and concretely support the patient, through the constant promotion of information and awareness programs for the population, specialists and institutions. “One of the first objectives of the Association will be to fill this information gap so that there is a more widespread awareness of the disease. Another aspect that we will deal with will be that of taking a census of patients with Castleman’s disease in Italy, precisely because, to date, there is no reliable data on the number of patients in our country. Finally, we will commit ourselves to the protection of patients’ rights in terms of access to treatment and exemptions and we will support the training and information of the scientific and clinical community so that diagnosis, assistance and treatment can be improved ”.

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