Tis\u00edc obyvatel \u010ceska trp\u00ed hemofili\u00ed, nevyl\u00e9\u010ditelnou d\u011bdi\u010dnou nemoc\u00ed, kter\u00e1 naru\u0161uje p\u0159irozenou sr\u00e1\u017elivost krve. Bez v\u010dasn\u00e9 a spr\u00e1vn\u00e9 l\u00e9\u010dby hroz\u00ed posti\u017een\u00fdm bolesti \u010di trval\u00e1 invalidita.<\/p>\n
Hemofilii se tak\u00e9 n\u011bkdy \u0159\u00edk\u00e1 „kr\u00e1lovsk\u00e1 nemoc“. To proto, \u017ee jej\u00ed nejzn\u00e1m\u011bj\u0161\u00ed d\u011bdi\u010dn\u00e1 linie se odv\u00edj\u00ed od slavn\u00e9 britsk\u00e9 kr\u00e1lovny Viktorie, kter\u00e1 ve druh\u00e9 polovin\u011b 19. stolet\u00ed p\u0159edala ne\u0161\u0165astnou \u0161tafetu synovi Leopoldovi. Jeho dcery Alice a Beatrice ji pak roz\u0161\u00ed\u0159ily d\u00e1l mezi p\u0159\u00edslu\u0161n\u00edky kr\u00e1lovsk\u00fdch rod\u016f po cel\u00e9 Evrop\u011b. Plat\u00ed toti\u017e, \u017ee nositelem defektn\u00edho genu je \u017eena. Sama sice onemocn\u00ed pouze v\u00fdjime\u010dn\u011b, ale nemoc obvykle p\u0159enese na syna, zat\u00edmco dcera se st\u00e1v\u00e1 dal\u0161\u00ed p\u0159ena\u0161e\u010dkou. Dne\u0161n\u00ed \u010dlenov\u00e9 anglick\u00e9 kr\u00e1lovsk\u00e9 rodiny jsou potomci zdrav\u00e9ho syna kr\u00e1lovny Viktorie a hemofilie A ji\u017e nen\u00ed d\u00e1le v tomto rodu p\u0159en\u00e1\u0161ena.<\/p>\n
Alexandra, vnu\u010dka kr\u00e1lovny Viktorie, se po\u010d\u00e1tkem 20. stolet\u00ed vdala za posledn\u00edho rusk\u00e9ho cara Mikol\u00e1\u0161e II. Syn Alexej pak trp\u011bl hemofili\u00ed. Jedin\u00fdm, komu se da\u0159ilo mlad\u00e9mu Alexejovi pomoci, byl mnich Rasputin. Vyu\u017e\u00edval p\u0159edev\u0161\u00edm hypn\u00f3zu, kter\u00e1 Alexejovi pomohla ulevit od siln\u00fdch bolest\u00ed a snad i zm\u00edrnit krv\u00e1cen\u00ed do kloub\u016f.<\/p>\n
Jedin\u00e1 dcera Viktorie Ev\u017eenie „uvedla“ hemofilii na \u0161pan\u011blsk\u00fd kr\u00e1lovsk\u00fd dv\u016fr. Ta tentokr\u00e1t zanechala tragick\u00e9 stopy v podob\u011b p\u0159ed\u010dasn\u00e9ho \u00famrt\u00ed jej\u00edho nejstar\u0161\u00edho a nejmlad\u0161\u00edho syna \u2013 jak Alfonso, tak Gonzalo zem\u0159eli po autonehod\u00e1ch, p\u0159i nich\u017e utrp\u011bli vnit\u0159n\u00ed krv\u00e1cen\u00ed. Gonzalovi b\u011bhem n\u00e1sledn\u00e9 operace selhalo srdce.<\/p>\n
Britsk\u00e1 princezna Alice provdan\u00e1 do velkov\u00e9vodstv\u00ed Hesensk\u00e9ho porodila vedle dvou zdrav\u00fdch syn\u016f tak\u00e9 hemofilika Fridricha, kter\u00e9ho nemoc zabila p\u0159ed t\u0159et\u00edmi narozeninami. Dv\u011b z jej\u00edch dcer, Irena a Alix, pak b\u0159\u00edm\u011b nesly d\u00e1l.<\/p>\n
Defektn\u00ed gen<\/strong> zp\u016fsobuje nedostate\u010dnou tvorbu b\u00edlkovin d\u016fle\u017eit\u00fdch pro sr\u00e1\u017elivost krve. Pokud se dot\u00fdk\u00e1 takzvan\u00e9ho faktoru VIII, hovo\u0159\u00ed l\u00e9ka\u0159i o hemofilii typu A, j\u00ed\u017e trp\u00ed 85 procent<\/strong> pacient\u016f, u faktoru IX pak o hemofilii typu B. A n\u00e1sledky? Jsou to zejm\u00e9na st\u00e1le siln\u011bj\u0161\u00ed bolesti a postupn\u00e9 znehyb\u0148ov\u00e1n\u00ed kloub\u016f<\/strong>, posl\u00e9ze cel\u00e9ho pohybov\u00e9ho apar\u00e1tu.<\/p>\n Sou\u010dasn\u00e1 medic\u00edna pova\u017euje za zlat\u00fd standard l\u00e9\u010dby d\u011bt\u00ed posti\u017een\u00fdch t\u011b\u017ekou formou hemofilie takzvanou profylaxi<\/strong>. Hemofilik dost\u00e1v\u00e1 obvykle dvakr\u00e1t a\u017e t\u0159ikr\u00e1t t\u00fddn\u011b nitro\u017eiln\u011b koncentr\u00e1t obsahuj\u00edc\u00ed onen faktor<\/strong>, kter\u00fd mu chyb\u00ed. Jeho hladina v krvi se tak zv\u00fd\u0161\u00ed, \u010d\u00edm\u017e se p\u0159edejde spont\u00e1nn\u00edmu krv\u00e1cen\u00ed. Tato l\u00e9\u010dba pacientovi zaru\u010duje jak v d\u011btstv\u00ed, tak pozd\u011bji v dosp\u011blosti, kvalitn\u00ed ka\u017edodenn\u00ed \u017eivot bez stresu z komplikac\u00ed v budoucnosti. Jedin\u00e9 nepohodl\u00ed p\u0159edstavuje nutnost pravideln\u00e9 aplikace injekce, kterou si ale pacient v\u011bt\u0161inou pod\u00e1 s\u00e1m doma.“<\/p>\n V\u00fdrazn\u011b hor\u0161\u00ed je situace u dosp\u011bl\u00fdch hemofilik\u016f<\/strong>, z nich\u017e v\u00edce ne\u017e dv\u011b t\u0159etiny jsou v sou\u010dasn\u00e9 dob\u011b l\u00e9\u010deni l\u00e9\u010dbou OnDemand<\/strong>, p\u0159i kter\u00e9 si aplikuj\u00ed \u00fa\u010dinnou l\u00e1tku teprve ve chv\u00edli, kdy za\u010dnou krv\u00e1cet. Takto l\u00e9\u010deni jsou v\u011bt\u0161inou hemofilici narozeni p\u0159ed za\u010d\u00e1tkem devades\u00e1t\u00fdch let minul\u00e9ho stolet\u00ed, a n\u011bkdy se k n\u00ed uchyluj\u00ed i mlad\u00ed dosp\u011bl\u00ed, nebo\u0165 ji ch\u00e1pou jako m\u00e9n\u011b obt\u011b\u017euj\u00edc\u00ed. Velk\u00e1 \u010d\u00e1st z nich se v\u0161ak k profylaxi vrac\u00ed<\/strong>.<\/p>\n L\u00e9\u010dba OnDemand<\/p>\n Tato l\u00e9\u010dba u t\u011b\u017ek\u00e9 formy hemofilie p\u0159in\u00e1\u0161\u00ed komplikace v podob\u011b nevratn\u00e9ho po\u0161kozov\u00e1n\u00ed kloub\u016f. Profylaktick\u00e1 l\u00e9\u010dba nasazen\u00e1 v t\u00e9to f\u00e1zi stav po\u0161kozen\u00e9ho kloubu sice ji\u017e v\u00fdrazn\u011b nezlep\u0161\u00ed, p\u0159esto ale v\u00fdznamn\u011b zpomal\u00ed dal\u0161\u00ed zhor\u0161ov\u00e1n\u00ed stavu. Z\u00e1rove\u0148 v\u00fdrazn\u011b sni\u017euje po\u010det dal\u0161\u00edch kloubn\u00edch krv\u00e1cen\u00ed a bolest. Hemofilik tak m\u016f\u017ee v\u00e9st kvalitn\u00ed \u017eivot. Proto i u takov\u00fdchto pacient\u016f m\u00e1 profylaxe sv\u00e9 opodstatn\u011bn\u00ed. Nav\u00edc profylaxe v dosp\u011blosti nemus\u00ed znamenat v\u00fdznamn\u00e9 zv\u00fd\u0161en\u00ed n\u00e1klad\u016f, nebo\u0165 centra, kter\u00e1 l\u00e9\u010d\u00ed OnDemand, spot\u0159ebuj\u00ed \u010dasto stejn\u00e9 mno\u017estv\u00ed faktoru jako p\u0159i profylaxi, ale s daleko hor\u0161\u00edmi v\u00fdsledky.<\/p>\n Ke stanoven\u00ed v\u010dasn\u00e9 diagn\u00f3zy sta\u010d\u00ed laboratorn\u00ed vy\u0161et\u0159en\u00ed krve.<\/p>\n","protected":false},"excerpt":{"rendered":" Tis\u00edc obyvatel \u010ceska trp\u00ed hemofili\u00ed, nevyl\u00e9\u010ditelnou d\u011bdi\u010dnou nemoc\u00ed, kter\u00e1 naru\u0161uje p\u0159irozenou sr\u00e1\u017elivost krve. Bez v\u010dasn\u00e9 a spr\u00e1vn\u00e9 l\u00e9\u010dby hroz\u00ed posti\u017een\u00fdm bolesti \u010di trval\u00e1 invalidita. Hemofilii […]<\/a><\/p>\n<\/div>","protected":false},"author":1,"featured_media":27915,"comment_status":"open","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[11],"tags":[],"class_list":["post-14616","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-zdravi"],"_links":{"self":[{"href":"https:\/\/www.invarena.cz\/index.php?rest_route=\/wp\/v2\/posts\/14616","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.invarena.cz\/index.php?rest_route=\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.invarena.cz\/index.php?rest_route=\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=%2Fwp%2Fv2%2Fcomments&post=14616"}],"version-history":[{"count":0,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=\/wp\/v2\/posts\/14616\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=\/wp\/v2\/media\/27915"}],"wp:attachment":[{"href":"https:\/\/www.invarena.cz\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=14616"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=%2Fwp%2Fv2%2Fcategories&post=14616"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=%2Fwp%2Fv2%2Ftags&post=14616"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}
\nN\u00e1sledkem toho se krev v t\u011ble sr\u00e1\u017e\u00ed m\u00e9n\u011b ne\u017e u zdrav\u00e9ho \u010dlov\u011bka, a doch\u00e1z\u00ed k nadm\u011brn\u00e9mu krv\u00e1cen\u00ed. Zat\u00edmco p\u0159i lehk\u00e9<\/strong> hemofilii hroz\u00ed komplikace v\u011bt\u0161inou jen p\u0159i poran\u011bn\u00ed nebo operaci, p\u0159i t\u011b\u017ek\u00e9<\/strong> hemofilii sta\u010d\u00ed i b\u011b\u017en\u00e9 uhozen\u00ed a krv\u00e1cen\u00ed se \u010dasto spou\u0161t\u00ed dokonce spont\u00e1nn\u011b. Nebezpe\u010d\u00ed je t\u00edm v\u011bt\u0161\u00ed, \u017ee jde o neviditeln\u00e9 krv\u00e1cen\u00ed do kloub\u016f, sval\u016f p\u0159\u00edpadn\u011b i vnit\u0159n\u00edch org\u00e1n\u016f.<\/p>\n