{"id":21184,"date":"2022-09-02T13:03:00","date_gmt":"2022-09-02T13:03:00","guid":{"rendered":"https:\/\/sbpt.org.br\/portal\/?p=21184"},"modified":"2022-09-02T13:03:00","modified_gmt":"2022-09-02T13:03:00","slug":"fibrose-cistica-2022","status":"publish","type":"post","link":"https:\/\/sbpt.org.br\/portal\/fibrose-cistica-2022\/","title":{"rendered":"Setembro: m\u00eas da conscientiza\u00e7\u00e3o sobre a Fibrose C\u00edstica"},"content":{"rendered":"<p style=\"text-align: justify\">A Fibrose C\u00edstica (FC) \u00e9 uma doen\u00e7a gen\u00e9tica, cr\u00f4nica, progressiva, caracterizada por uma muta\u00e7\u00e3o celular na prote\u00edna CFTR.<\/p>\n<p style=\"text-align: justify\">\u00a0A CFTR (prote\u00edna transmembrana da Fibrose C\u00edstica, na sigla em ingl\u00eas) controla o movimento dos \u00edons de cloreto e s\u00f3dio nas membranas celulares. O grau e tipo de mau funcionamento da CFTR depende da muta\u00e7\u00e3o gen\u00e9tica do indiv\u00edduo.<\/p>\n<p style=\"text-align: justify\">O paciente com FC tem produ\u00e7\u00e3o excessiva de secre\u00e7\u00f5es e muco mais espesso, aumentando a suscetibilidade \u00e0s infec\u00e7\u00f5es pulmonares e a bronquiectasias. A doen\u00e7a \u00e9 sist\u00eamica e afeta principalmente os pulm\u00f5es, p\u00e2ncreas, rins, f\u00edgado, aparelho digestivo, intestino e seios da face.<\/p>\n<p style=\"text-align: justify\">Algumas das consequ\u00eancias t\u00edpicas da Fibrose C\u00edstica s\u00e3o suor e saliva \u201csalgados\u201d (com maiores n\u00edveis de cloreto), pneumonia de repeti\u00e7\u00e3o, sinusite frequente, tosse cr\u00f4nica, cansa\u00e7o excessivo, dores abdominais e diarreia, al\u00e9m de dificuldade de ganhar peso e estatura.<\/p>\n<h6 style=\"text-align: justify\"><strong>Diagn\u00f3stico e tratamento<\/strong><\/h6>\n<p style=\"text-align: justify\">A incid\u00eancia da FC \u00e9 de aproximadamente 1 caso para 10.000 nascidos vivos. No Brasil, quase 100% das crian\u00e7as s\u00e3o testadas para Fibrose C\u00edstica por meio do Programa de Triagem Neonatal (\u201cTeste do Pezinho\u201d). Quando positivo, o exame \u00e9 repetido e o diagn\u00f3stico pode ser confirmado pelo Teste Gen\u00e9tico ou pelo Teste do Suor, realizados por centros especializados.<\/p>\n<p style=\"text-align: justify\">O tratamento \u00e9 intenso da inf\u00e2ncia at\u00e9 a idade adulta e exige empenho do paciente, dos familiares e de uma equipe multiprofissional, que muitas vezes envolve a Pediatria, a Pneumologia, a Fisioterapia Respirat\u00f3ria, a Psicoterapia, a Nutri\u00e7\u00e3o, entre outras especialidades. A rotina de reabilita\u00e7\u00e3o pulmonar, medicamentos, exames e interna\u00e7\u00f5es \u00e9 \u00e1rdua e pode afetar muito a qualidade de vida dos pacientes com FC.<\/p>\n<p style=\"text-align: justify\">O m\u00e9dico deve ficar atento aos par\u00e2metros que determinam a gravidade da doen\u00e7a. O exame de Fun\u00e7\u00e3o Pulmonar \u00e9 um dos mais importantes, junto com o estado nutricional, cultura de escarro e o \u00cdndice de Massa Corp\u00f3rea (IMC).<\/p>\n<h6 style=\"text-align: justify\"><strong>Avan\u00e7os<\/strong><\/h6>\n<p style=\"text-align: justify\">Na \u00faltima d\u00e9cada, foram desenvolvidos medicamentos que atuam na corre\u00e7\u00e3o do defeito celular que leva a doen\u00e7a. \u201cTemos potencializadores e corretores da fun\u00e7\u00e3o deste canal de cloro. Por\u00e9m, um medicamento s\u00f3 funciona para uma muta\u00e7\u00e3o espec\u00edfica ou, pelo menos, para uma classe espec\u00edfica de muta\u00e7\u00e3o no gene acometido\u201d, explica o dr. Marcelo Bicalho de Fuccio, coordenador da Comiss\u00e3o Cient\u00edfica de Fibrose C\u00edstica da SBPT.<\/p>\n<p style=\"text-align: justify\">\u201cEsses medicamentos est\u00e3o se mostrando revolucion\u00e1rios na estabilidade cl\u00ednica e ganho de qualidade de vida dos pacientes e, tudo leva a crer, t\u00eam um grande impacto na sobrevida\u201d, complementa o especialista.<\/p>\n<p style=\"text-align: justify\">Outro avan\u00e7o no tratamento foi a descoberta da efic\u00e1cia da azitromicina como anti-inflamat\u00f3rio imunomodulador na Fibrose C\u00edstica, em 2004, al\u00e9m dos esquemas de nebuliza\u00e7\u00e3o com efeito mucol\u00edtico (alfa-dornase).<\/p>\n<h6 style=\"text-align: justify\"><strong>Perspectivas futuras da Fibrose C\u00edstica<\/strong><\/h6>\n<p style=\"text-align: justify\">A partir de 2011, surgiram os moduladores de CFTR, medicamentos que atuam no defeito celular da fibrose c\u00edstica (canal de cloro), resolvendo o problema da secre\u00e7\u00e3o mais espessa e conservando os pulm\u00f5es para grande parte dos pacientes.<\/p>\n<p>Esses medicamentos s\u00e3o indicados de acordo com a muta\u00e7\u00e3o gen\u00e9tica, por isso, ainda n\u00e3o beneficiam todos os pacientes.<\/p>\n<p style=\"text-align: justify\"><strong>Kalydeco (ivacaftor)<\/strong>: j\u00e1 incorporado ao SUS. Tem indica\u00e7\u00e3o restrita a poucas muta\u00e7\u00f5es, mas, com excelente resposta nesses pacientes.<\/p>\n<p style=\"text-align: justify\"><strong>Trikafta (combina\u00e7\u00e3o de elexacaftor, tezacaftor e ivacaftor)<\/strong>: aprovado pela Ag\u00eancia Nacional de Vigil\u00e2ncia Sanit\u00e1ria (Anvisa) para uso no Brasil em mar\u00e7o de 2022. Cerca de 50% dos pacientes no Brasil s\u00e3o eleg\u00edveis a esse tratamento.<\/p>\n<p style=\"text-align: justify\">A tentativa de incorpora\u00e7\u00e3o do Trikafta ao SUS \u00e9 o nosso pr\u00f3ximo desafio. Lembrando que o alto custo ainda \u00e9 um entrave para o acesso.<\/p>\n<p style=\"text-align: justify\">Pesquisas preliminares sobre terapias g\u00eanicas, que corrigem o DNA em vez de atuar na prote\u00edna, tamb\u00e9m trazem otimismo com rela\u00e7\u00e3o ao tratamento da Fibrose C\u00edstica no futuro.<\/p>\n<p><a href=\"https:\/\/d1xe7tfg0uwul9.cloudfront.net\/sbpt.org.br\/portal\/wp-content\/uploads\/2021\/09\/Fibrose-c\u00edstica-UU-v3.png\"><img loading=\"lazy\" decoding=\"async\" class=\"aligncenter size-medium wp-image-21185\" src=\"https:\/\/d1xe7tfg0uwul9.cloudfront.net\/sbpt.org.br\/portal\/wp-content\/uploads\/2021\/09\/Fibrose-c\u00edstica-UU-v3-300x300.png\" alt=\"\" width=\"300\" height=\"300\" \/><\/a><\/p>\n<h6 style=\"text-align: justify\"><strong>Fibrose C\u00edstica no 40\u00ba Congresso Brasileiro de Pneumologia (<a href=\"https:\/\/sbpt.org.br\/sbpt2022\/\" target=\"_blank\" rel=\"noopener noreferrer\">SBPT 2022<\/a>) &#8211; 12 a 16 de outubro &#8211; Campinas (SP)<br \/>\n<\/strong><\/h6>\n<p>\u2022 Confer\u00eancia &#8211; Processo da constru\u00e7\u00e3o da diretriz de fibrose c\u00edstica.<br \/>\n\u2022 Mesa redonda &#8211; Tratamento da fibrose c\u00edstica: indica\u00e7\u00f5es dos antibi\u00f3ticos inalat\u00f3rios; peculiaridades do tratamento da exacerba\u00e7\u00e3o; situa\u00e7\u00e3o das terap\u00eauticas moduladoras do CFTR.<br \/>\n\u2022 Confer\u00eancia &#8211; O crescimento da popula\u00e7\u00e3o adulta com fibrose c\u00edstica no Brasil: demandas e oportunidades.<br \/>\n\u2022 Confer\u00eancia &#8211; O horizonte promissor da fibrose c\u00edstica e a realidade brasileira.<\/p>\n<hr \/>\n<p style=\"text-align: justify\"><strong>Refer\u00eancias<\/strong><\/p>\n<p style=\"text-align: justify\">Sociedade Brasileira de Pneumologia e Tisiologia. <strong>Encontros Virtuais SBPT &#8211; Bronquiectasias<\/strong>. 22 out. 2020. Dispon\u00edvel em: https:\/\/youtu.be\/vsEFO_mXTkE<\/p>\n<p style=\"text-align: justify\">Sociedade Brasileira de Pneumologia e Tisiologia. <strong>Direto ao Ponto &#8211; Fibrose C\u00edstica: conquistas e perspectivas<\/strong>. 25 set. 2020. Dispon\u00edvel em: <a href=\"https:\/\/anchor.fm\/sbpt\/episodes\/Direto-ao-Ponto-Fibrose-Cstica-conquistas-e-perspectivas-ek44im\/a-a3b6gum\">https:\/\/anchor.fm\/sbpt\/episodes\/Direto-ao-Ponto-Fibrose-Cstica-conquistas-e-perspectivas-ek44im\/a-a3b6gum<\/a><\/p>\n<p style=\"text-align: justify\">Sociedade Brasileira de Pneumologia e Tisiologia. <strong>Setembro: m\u00eas de conscientiza\u00e7\u00e3o sobre a fibrose c\u00edstica<\/strong>. 5 set. 2020. Dispon\u00edvel em: https:\/\/sbpt.org.br\/portal\/\/fibrose-cistica-2020\/<\/p>\n","protected":false},"excerpt":{"rendered":"A Fibrose C\u00edstica (FC) \u00e9 uma doen\u00e7a gen\u00e9tica, cr\u00f4nica, progressiva, caracterizada por uma muta\u00e7\u00e3o celular na prote\u00edna CFTR. \u00a0A CFTR (prote\u00edna transmembrana da Fibrose C\u00edstica, na sigla em ingl\u00eas) controla o movimento dos \u00edons de cloreto e s\u00f3dio nas membranas celulares. O grau e tipo de mau funcionamento da CFTR depende da muta\u00e7\u00e3o gen\u00e9tica do [\u2026]","protected":false},"author":3,"featured_media":21188,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"ngg_post_thumbnail":0,"footnotes":""},"categories":[68],"tags":[164],"class_list":["post-21184","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-noticias","tag-fibrose-cistica"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v28.7 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>8 de setembro: dia mundial de conscientiza\u00e7\u00e3o sobre a Fibrose C\u00edstica<\/title>\n<meta name=\"description\" content=\"A Fibrose C\u00edstica (FC) \u00e9 uma doen\u00e7a gen\u00e9tica, cr\u00f4nica, progressiva, caracterizada por uma muta\u00e7\u00e3o celular na prote\u00edna CFTR.\" \/>\n<meta name=\"robots\" content=\"index, follow, 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