{"id":1827,"date":"2023-02-22T15:19:00","date_gmt":"2023-02-22T14:19:00","guid":{"rendered":"https:\/\/niho.sk\/?p=1827"},"modified":"2024-03-30T16:00:39","modified_gmt":"2024-03-30T15:00:39","slug":"22a-liecivo-emicizumab-hemlibra-pri-profylaxii-krvacania-u-pacientov-vo-vsetkych-vekovych-skupinach-s-hemofiliou-a-s-inhibitorom-faktora-viii","status":"publish","type":"post","link":"https:\/\/www.niho.sk\/en\/22a-liecivo-emicizumab-hemlibra-pri-profylaxii-krvacania-u-pacientov-vo-vsetkych-vekovych-skupinach-s-hemofiliou-a-s-inhibitorom-faktora-viii\/","title":{"rendered":"22A: Lie\u010divo emicizumab (Hemlibra) pri profylaxii krv\u00e1cania u pacientov vo v\u0161etk\u00fdch vekov\u00fdch skupin\u00e1ch s hemof\u00edliou A s inhib\u00edtorom faktora VIII"},"content":{"rendered":"
    \n
  1. Ak\u00e1 je z\u00e1\u0165a\u017e ochorenia pre pacienta? Ako z\u00e1sadn\u00e9 je ochorenie?<\/strong><\/li>\n<\/ol>\n\n\n\n

    Hemof\u00edlia typu A je pomerne vz\u00e1cne nevylie\u010dite\u013en\u00e9 dedi\u010dn\u00e9 ochorenie postihuj\u00face predov\u0161etk\u00fdm mu\u017eov, ktor\u00e9 sa prejavuje poruchou zr\u00e1\u017eania krvi. Pacienti s hemof\u00edliou krv\u00e1caj\u00fa po poranen\u00ed alebo pri oper\u00e1cii dlh\u0161ie ako zdrav\u00ed \u013eudia. M\u00f4\u017ee u nich doch\u00e1dza\u0165 aj k spont\u00e1nnemu krv\u00e1caniu do k\u013abov alebo svalov, ktor\u00e9 sa objavuje bez zjavnej pr\u00ed\u010diny, \u010di po malom \u00faraze. <\/p>\n\n\n\n

    V niektor\u00fdch pr\u00edpadoch m\u00f4\u017ee by\u0165 v\u0161ak krv\u00e1canie i \u017eivot ohrozuj\u00face, hlavne vtedy, ke\u010f zasahuje centr\u00e1lny nervov\u00fd syst\u00e9m, tr\u00e1viaci trakt alebo d\u00fdchacie cesty. U \u0165a\u017e\u0161\u00edch hemofilikov \u010dastokr\u00e1t doch\u00e1dza ku krv\u00e1caniu, ktor\u00e9 sa ozna\u010duje ako spont\u00e1nne, bez zjavnej pr\u00ed\u010diny. Spont\u00e1nne krv\u00e1canie sa nikdy ned\u00e1 predv\u00edda\u0165 a ned\u00e1 sa mu pred\u00eds\u0165. V\u0161etci pacienti s potvrdenou hemof\u00edliou A si vy\u017eaduj\u00fa lie\u010dbu. Bez lie\u010dby vedie krv\u00e1canie do k\u013abov a svalov ku vzniku degenerat\u00edvnych zmien v k\u013aboch, k tzv. progres\u00edvnej artropatii a k atrofii svalstva (najm\u00e4 kon\u010datinov\u00e9ho). Aj jedin\u00e9 krv\u00e1canie m\u00f4\u017ee vies\u0165 k \u017eivot ohrozuj\u00facemu stavu alebo trval\u00e9mu oslabuj\u00facemu ochoreniu k\u013abov.<\/p>\n\n\n\n

    Pribli\u017ene u 30% pacientov sa vyvin\u00fa inhib\u00edtory proti faktoru VIII (FVIII), ktor\u00e9 neutralizuj\u00fa pod\u00e1van\u00fd FVIII a robia lie\u010dbu t\u00fdmto faktorom ne\u00fa\u010dinnou.<\/p>\n\n\n\n

      \n
    1. O ak\u00fd liek ide?<\/strong><\/li>\n<\/ol>\n\n\n\n

      Hemlibra obsahuje lie\u010divo \u201eemicizumab\u201c, ktor\u00fd patr\u00ed do skupiny liekov naz\u00fdvan\u00fdch \u201emonoklon\u00e1lne protil\u00e1tky\u201c. Monoklon\u00e1lne protil\u00e1tky s\u00fa typom bielkoviny, ktor\u00e9 rozpoznaj\u00fa \u0161pecifick\u00fd cie\u013e v tele a navia\u017eu sa na\u0148. <\/p>\n\n\n\n

      \u00dalohou Hemlibry je premos\u0165ova\u0165 priestor medzi aktivovan\u00fdm faktorom IX a faktorom X, aby do\u0161lo k obnoveniu funkcie ch\u00fdbaj\u00faceho aktivovan\u00e9ho faktora VIII, ktor\u00fd je potrebn\u00fd na zastavenie krv\u00e1cania. Hemlibra m\u00e1 \u0161trukt\u00faru odli\u0161n\u00fa od \u0161trukt\u00fary faktora VIII, a preto pr\u00edtomnos\u0165 inhib\u00edtora faktora VIII nem\u00e1 vplyv na jej p\u00f4sobenie.<\/p>\n\n\n\n

      Hemlibra sa m\u00e1 pou\u017e\u00edva\u0165 na predch\u00e1dzanie vzniku krv\u00e1can\u00ed (profylaktick\u00e1 lie\u010dba).<\/p>\n\n\n\n

      Liek Hemlibra bol registrovan\u00fd Eur\u00f3pskou liekovou agent\u00farou vo febru\u00e1ri 2018.<\/p>\n\n\n\n

        \n
      1. Ako sa na emicizumab pozeraj\u00fa odborn\u00edci? <\/strong><\/li>\n<\/ol>\n\n\n\n

        Odborn\u00edci, ktor\u00fdch NIHO oslovilo pova\u017euj\u00fa za potrebn\u00e9 ma\u0165 k dispoz\u00edcii \u00fa\u010dinn\u00fd liek pre pacientov s hemof\u00edliou A s inhib\u00edtorom FVIII, ktor\u00fd by mohol v\u00fdrazne zv\u00fd\u0161i\u0165 kvalitu ich \u017eivota.<\/p>\n\n\n\n

        Jeden z odborn\u00edkov tvrd\u00ed, \u017ee dnes je \u0161tandardnou s\u00fa\u010das\u0165ou mana\u017ementu pacientov s inhib\u00edtorom FVIII pr\u00e1ve emicizumab ako jedin\u00fd \u00fa\u010dinn\u00fd liek na prevenciu krv\u00e1can\u00ed po\u010das imunotoleran\u010dnej lie\u010dby, a u pacientov s perzistuj\u00facim inhib\u00edtorom FVIII a z\u00e1rove\u0148 zd\u00f4raz\u0148uje, \u017ee liek mus\u00ed by\u0165 dostupn\u00fd pre v\u0161etk\u00fdch pacientov.<\/p>\n\n\n\n

          \n
        1. \u010co hodnotilo NIHO a ak\u00fd je v\u00fdsledok?<\/strong><\/li>\n<\/ol>\n\n\n\n

          Dr\u017eite\u013e registr\u00e1cie (Roche) po\u017eaduje hradenie lieku z prostriedkov verejn\u00e9ho zdravotn\u00e9ho poistenia pri prevenci\u00ed krv\u00e1cania u pacientov vo v\u0161etk\u00fdch vekov\u00fdch skupin\u00e1ch s hemof\u00edliou A s inhib\u00edtorom faktora VIII.<\/p>\n\n\n\n

          NIHO odpor\u00fa\u010da vyhovie\u0165 <\/strong>\u017eiadosti o kategorizovanie lieku Hemlibra v po\u017eadovanej indik\u00e1cii. <\/p>\n\n\n\n

          Z\u00e1rove\u0148 NIHO odpor\u00fa\u010da zv\u00e1\u017ei\u0165 \u00fapravu indika\u010dn\u00e9ho obmedzenia:<\/p>\n\n\n\n

            \n
          • v s\u00falade s hodnoten\u00edm \u010desk\u00e9ho S\u00daKL z\u00fa\u017ei\u0165 popul\u00e1ciu iba na siln\u00fdch responderov, t.j. pacientov s vysok\u00fdm titrom inhib\u00edtora FVIII (titer inhib\u00edtora >5,0 BU\/ml) z d\u00f4vodu inkl\u00faznych krit\u00e9ri\u00ed \u0161t\u00fadi\u00ed HAVEN 1 a 2, <\/li>\n\n\n\n
          • aby lie\u010dba emizicumabom podliehala predch\u00e1dzaj\u00facemu s\u00fahlasu zdravotnej pois\u0165ovne.<\/li>\n<\/ul>","protected":false},"excerpt":{"rendered":"

            NIHO odpor\u00fa\u010da vyhovie\u0165 \u017eiadosti o kategorizovanie lieku Hemlibra v po\u017eadovanej indik\u00e1cii…<\/p>","protected":false},"author":3,"featured_media":1828,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"inline_featured_image":false,"footnotes":""},"categories":[11],"tags":[80,79],"class_list":["post-1827","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-sumar-hodnotenia","tag-emicizumab","tag-hemlibra"],"_links":{"self":[{"href":"https:\/\/www.niho.sk\/en\/wp-json\/wp\/v2\/posts\/1827","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.niho.sk\/en\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.niho.sk\/en\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.niho.sk\/en\/wp-json\/wp\/v2\/users\/3"}],"replies":[{"embeddable":true,"href":"https:\/\/www.niho.sk\/en\/wp-json\/wp\/v2\/comments?post=1827"}],"version-history":[{"count":0,"href":"https:\/\/www.niho.sk\/en\/wp-json\/wp\/v2\/posts\/1827\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/www.niho.sk\/en\/wp-json\/wp\/v2\/media\/1828"}],"wp:attachment":[{"href":"https:\/\/www.niho.sk\/en\/wp-json\/wp\/v2\/media?parent=1827"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.niho.sk\/en\/wp-json\/wp\/v2\/categories?post=1827"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.niho.sk\/en\/wp-json\/wp\/v2\/tags?post=1827"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}