Experts note that the scale of the disease is still underestimated.<\/p>\n
On November 26, the annual event dedicated to Russian and European Cystic Fibrosis Day took place at the FSBSI Research Center for Children’s Health. Such festive gatherings of the Center\u2019s young patients with leading specialists in pulmonology, representatives of public organizations, and the media are intended to draw public attention to the issues of diagnosis and timely, appropriate therapy for cystic fibrosis<\/em>.<\/strong><\/p>\n <\/p>\n It has become a good tradition for well-known public figures and stars to take part in Cystic Fibrosis Day \u2014 at various times the campaign has been supported by actors Armen Dzhigarkhanyan and Maria Golubkina, TV presenters Rita Mitrofanova and Olga Shelest, singer Sergey Trofimov, and others. This year, the special guest of the children’s celebration at the Research Center for Children’s Health was The Voice project participant, singer Metodie Bujor. \u201cTaking part in charitable events for children with such serious illnesses is a sacred duty of every artist,\u201d says Metodie. \u201cAfter all, creativity is not only fame, public recognition, and show business. Many great people I met \u2014 Muslim Magomayev, Eldar Ryazanov, Alexander Shirvindt \u2014 said: \u2018When you go on stage, you must leave people a piece of your heart.\u2019 Performing at a celebration for such extraordinary, brave, and courageous kids, you need to take out your heart, give it away, and not regret it.\u201d<\/p>\n Cystic fibrosis (CF)<\/strong> is a severe genetic disease that affects all organs that secrete fluids (digestive juices, sweat, saliva, etc.). Thick mucus accumulates in the bronchi, prevents the lungs from breathing, and blocks the digestive system. It is the most common hereditary disorder in Europe; almost every 15th resident of the Old Continent is a carrier of the CF gene. In total, more than 40,000 adults and children worldwide are battling cystic fibrosis; it affects men and women equally often. In Russia, only a little over 3,000 patients with a confirmed diagnosis are registered, but according to experts, in reality there may be many more. Doctors believe cystic fibrosis is insidious: the course of the disease and the symptoms are individual for each patient and depend on the mutation and the degree of its severity. More than 2,300 gene defect variants are currently known, and with some mild forms of the mutation, patients have no pronounced external symptoms at all.<\/p>\n Another reason the scale of the disease\u2019s prevalence is underestimated is the insufficient level of diagnosis and awareness of CF among Russians. According to specialists, the most important step toward improving the situation was the neonatal screening program for cystic fibrosis, launched in Russia in 2007. In European countries, similar postnatal diagnostics have been carried out for 20 years already, and our specialists and patients are only beginning to reap the first positive results of the program. \u201cNeonatal screening made it possible to detect cystic fibrosis at the earliest stages, when there are not even hints of symptoms,\u201d says Olga Igorevna Simonova, Head of the Department of Pulmonology and Allergology at the Research Center for Children’s Health. \u201cThese children are registered, closely monitored, treated, hardened, and engaged in targeted sports. Of course, their prognosis will be one hundred times more favorable than in any other case.\u201d Doctors are convinced that canceling the program would be a catastrophe and would nullify all the achievements of recent years.<\/p>\n Since 2008, the government program \u201c7 Nosologies\u201d has also been operating in Russia, under which patients with cystic fibrosis are provided free of charge with one of the vital medications \u2014 the enzyme mucolytic dornase alfa. However, experts note that these measures are not enough to provide full-scale care.<\/p>\n Adequate treatment for cystic fibrosis requires antibiotics, pancreatic enzymes, hepatoprotectors, vitamin complexes, etc. At present, how a patient will receive these medications is decided at the regional healthcare level. As a result, it has become common practice for patients to have to buy a significant share of expensive medications at their own expense. Another typical problem is when disability status is removed as the patient’s condition improves, causing them to lose benefits for treatment. \u201cDisability status here is removed with the stroke of a pen. It is granted not because a patient has a congenital incurable disease, but because of the impairment of function that develops as a result of the disease. From the officials\u2019 point of view: if your function is impaired at the moment, then you are disabled; if it is not impaired, then you are healthy,\u201d comments Irina Vladimirovna Myasnikova, Chair of the Board of the Interregional Public Organization \u201cAid to Patients with Cystic Fibrosis.\u201d \u201cToday in pediatrics, losing a child to cystic fibrosis is an emergency. If treatment was available and the family did everything right, pediatricians hand over 18-year-old developed, well-feeling young men and women to internists. Their disability status is removed, they stop receiving medications, and two months later they end up in intensive care. What is the state logic in that?\u201d.<\/p>\n Nevertheless, there are also positive trends in organizing care for adult patients diagnosed with CF, experts are confident: treatment tariffs and the hospitalization procedure have now been approved. Doctors who can monitor these patients have appeared, as well as a new adult department \u2014 15 modern, well-equipped isolation rooms at Moscow City Clinical Hospital No. 57.<\/p>\n However, many issues still need to be resolved <\/em><\/strong>\u2014 there is a shortage of specialist doctors, there is no system of outpatient home care, and there are still few centers and clinics providing care for adult patients.<\/em><\/strong> But the main problem remains the inadequate provision of medicines for patients. <\/em><\/strong>In a number of regions, people have to fight even for the minimum guaranteed to them by the state. \u201cAll drugs for cystic fibrosis are very specific and are distributed by name. Their quantity is clearly defined \u2014 more is not needed for the patient, and less is dangerous,\u201d notes O.I. Simonova. \u201cUnfortunately, in some places the system still works poorly, and families have to spend time fighting bureaucracy, gathering evidence, obtaining certificates, instead of taking care of their child, providing full care and support. Even from the standpoint of economic efficiency, it is much more beneficial for the state to give the child the necessary medicines now and ensure good health at home than to hospitalize them tomorrow in an expensive inpatient facility.\u201d<\/p>\n Access to specialized medical care dramatically improves the quality and length of life of patients with cystic fibrosis.<\/strong> Today in our country, people with this diagnosis live on average about 27 years, whereas in Europe such patients, receiving full modern treatment, live to 50 years and beyond. The goal of Cystic Fibrosis Day is to draw the attention of government officials, healthcare workers, and the public in our country to this problem. Today in Russia, all patients, regardless of where they live, should have access to life-saving medicines and therapy that meet European standards; only then will they have every chance to live a full, long life and benefit society.<\/p>\n","protected":false},"excerpt":{"rendered":" Experts note that the scale of the disease is still underestimated. On November 26, the annual event dedicated to Russian and European Cystic Fibrosis Day took place at the FSBSI Research Center for Children’s Health. Such festive gatherings of the Center\u2019s young patients with leading specialists in pulmonology, representatives of public organizations, and the media […]<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"inline_featured_image":false,"footnotes":""},"categories":[2],"tags":[],"class_list":["post-1063","post","type-post","status-publish","format-standard","hentry","category-news"],"_links":{"self":[{"href":"https:\/\/rovvm.ru\/index.php?rest_route=\/wp\/v2\/posts\/1063","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/rovvm.ru\/index.php?rest_route=\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/rovvm.ru\/index.php?rest_route=\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/rovvm.ru\/index.php?rest_route=\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/rovvm.ru\/index.php?rest_route=%2Fwp%2Fv2%2Fcomments&post=1063"}],"version-history":[{"count":0,"href":"https:\/\/rovvm.ru\/index.php?rest_route=\/wp\/v2\/posts\/1063\/revisions"}],"wp:attachment":[{"href":"https:\/\/rovvm.ru\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=1063"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/rovvm.ru\/index.php?rest_route=%2Fwp%2Fv2%2Fcategories&post=1063"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/rovvm.ru\/index.php?rest_route=%2Fwp%2Fv2%2Ftags&post=1063"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}