D\u011btsk\u00e9 l\u00e9ka\u0159ce nejprve musela maminka dvou nemocn\u00fdch syn\u016f vysv\u011btlit, \u010d\u00edm jej\u00ed d\u011bti trp\u00ed. Vz\u00e1cn\u00e9 onemocn\u011bn\u00ed heredit\u00e1rn\u00ed angioed\u00e9m (HAE), kter\u00e9 Dominikovi a Leo\u0161ovi zp\u016fsobuje na r\u016fzn\u00fdch \u010d\u00e1stech t\u011bla bolestiv\u00e9 a n\u011bkdy i \u017eivot ohro\u017euj\u00edc\u00ed otoky, toti\u017e doktorka dosud znala jen ze studi\u00ed. Nen\u00ed divu. Podle dostupn\u00fdch statistik se choroba vyskytuje zhruba u jednoho \u010dlov\u011bka z 10 tis\u00edc a\u017e 50 tis\u00edc lid\u00ed. Za rok se tedy v \u010cesku narod\u00ed jen n\u011bkolik d\u011bt\u00ed s touto nel\u00e9\u010ditelnou chorobou. Jak se s n\u00ed d\u00e1 \u017e\u00edt? Mezin\u00e1rodn\u00ed den HAE p\u0159ipad\u00e1 na 16. kv\u011bten a m\u00e1 zv\u00fd\u0161it pov\u011bdom\u00ed \u0161irok\u00e9 ve\u0159ejnosti o t\u00e9to vz\u00e1cn\u00e9 celo\u017eivotn\u00ed nemoci.<\/p>\n\n\n\n
\u201eJe to nekone\u010dn\u00fd boj, ale sna\u017e\u00edme se \u017e\u00edt vyrovnan\u011b,\u201c \u0159\u00edk\u00e1 Michaela Kleinov\u00e1, maminka jeden\u00e1ctilet\u00e9ho Dominika a \u010dtrn\u00e1ctilet\u00e9ho Leo\u0161e, kte\u0159\u00ed trp\u00ed onemocn\u011bn\u00edm HAE. Tato zkratka ozna\u010duje vz\u00e1cnou d\u011bdi\u010dnou nevyl\u00e9\u010ditelnou nemoc heredit\u00e1rn\u00ed angioed\u00e9m. Sv\u00e9 nositele tr\u00e1p\u00ed \u010dast\u00fdmi otoky na r\u016fzn\u00fdch \u010d\u00e1stech t\u011bla. Mohou b\u00fdt r\u016fzn\u011b z\u00e1va\u017en\u00e9, z\u00e1le\u017e\u00ed na tom, kde se objev\u00ed.<\/p>\n\n\n\n
\u201eEd\u00e9m m\u016f\u017ee postihovat obli\u010dej, jazyk, hrtan, kon\u010detiny, za\u017e\u00edvac\u00ed nebo mo\u010dov\u00fd trakt. Posti\u017een\u00ed mohou trp\u011bt nap\u0159\u00edklad bolestmi b\u0159icha a nevolnostmi. V z\u00e1va\u017en\u00fdch p\u0159\u00edpadech m\u016f\u017ee otok b\u00fdt i stavem ohro\u017euj\u00edc\u00edm \u017eivot, nap\u0159\u00edklad pokud dojde k z\u00fa\u017een\u00ed \u010di utla\u010den\u00ed d\u00fdchac\u00edch cest, za\u010d\u00edn\u00e1 se posti\u017een\u00fd dusit,\u201c popisuje Miroslav \u0160uta, l\u00e9ka\u0159 z plazmaferetick\u00fdch center Amber Plasma<\/p>\n\n\n\n
Pr\u00e1v\u011b z krevn\u00ed plazmy, kter\u00e1 se v t\u011bchto centrech odeb\u00edr\u00e1 dobrovoln\u00fdm d\u00e1rc\u016fm, se n\u00e1sledn\u011b vyr\u00e1b\u011bj\u00ed l\u00e9ky, je\u017e pacient\u016fm s HAE pom\u00e1haj\u00ed.<\/p>\n\n\n\n
Otoky prozradily d\u011bdictv\u00ed po otci<\/p>\n\n\n\n
Pokud HAE trp\u00ed rodi\u010d, je zde pades\u00e1tiprocent\u00ed \u0161ance genetick\u00e9ho p\u0159enosu na potomky. Toto vz\u00e1cn\u00e9 onemocn\u011bn\u00ed m\u011bl i b\u00fdval\u00fd partner pan\u00ed Michaely. Kdy\u017e se jim narodili synov\u00e9 Dominik a Leo\u0161, doufala, \u017ee d\u011bti nemoc po sv\u00e9m biologick\u00e9m otci nezd\u011bd\u00ed.<\/p>\n\n\n\n
Bohu\u017eel krevn\u00ed testy mezi druh\u00fdm a t\u0159et\u00edm rokem prok\u00e1zaly, \u017ee i oni jsou nositeli nemoci. \u201eV t\u00e9 dob\u011b u\u017e se jim za\u010d\u00ednaly d\u011blat men\u0161\u00ed otoky na ruce a v puse, u star\u0161\u00edho syna se objevovaly bolesti b\u0159\u00ed\u0161ka. V\u011bd\u011bli jsme tedy, \u017ee to za\u010d\u00edn\u00e1,\u201c vzpom\u00edn\u00e1 dnes \u010dty\u0159icetilet\u00e1 Michaela.<\/p>\n\n\n\n
Kluci se narodili v Ho\u0159ovic\u00edch, ale v sou\u010dasnosti \u017eij\u00ed v Jiho\u010desk\u00e9m kraji. Jsou zde jedin\u00ed, kdo t\u00edmto vz\u00e1cn\u00fdm onemocn\u011bn\u00edm trp\u00ed.<\/p>\n\n\n\n
\u201eDnes u\u017e n\u00e1s na pohotovosti v \u010cesk\u00fdch Bud\u011bjovic\u00edch dob\u0159e znaj\u00ed. Kdy\u017e p\u0159ijde ataka a mus\u00edme do nemocnice, sta\u010d\u00ed nahl\u00e1sit jm\u00e9no a l\u00e9ka\u0159i u\u017e v\u011bd\u00ed, co maj\u00ed d\u011blat. Za\u010d\u00e1tky byly ale t\u011b\u017ek\u00e9 \u2013 museli jsme v\u0161ude vysv\u011btlovat, co tato nemoc obn\u00e1\u0161\u00ed. Dokonce ani d\u011btsk\u00e1 l\u00e9ka\u0159ka se s n\u00ed nikdy ve sv\u00e9 praxi nesetkala. Znala ji jen ze studi\u00ed, ale ne\u017e poznala n\u00e1s, nikoho s HAE na\u017eivo nevid\u011bla,\u201c vysv\u011btluje pan\u00ed Michaela.<\/p>\n\n\n\n
Michaela se znovu se vdala a se sv\u00fdm sou\u010dasn\u00fdm man\u017eelem m\u00e1 je\u0161t\u011b \u0161estiletou dcerku. Tak\u00e9 jej\u00ed mu\u017e se musel nau\u010dit, jak \u0159e\u0161it situace, kdy se nebezpe\u010dn\u00fd otok u kluk\u016f objev\u00ed.<\/p>\n\n\n\n
Spole\u010dn\u00fdmi silami se rodina sna\u017e\u00ed situaci co nejl\u00e9pe zvl\u00e1dat.<\/p>\n\n\n\n
Na z\u00e1pas s injekc\u00ed<\/p>\n\n\n\n
Dnes je Dominikovi 11 a Leo\u0161ovi 14 let. Kv\u016fli heredit\u00e1rn\u00edmu angioed\u00e9mu jsou \u010dasto nemocn\u00ed a musej\u00ed doh\u00e1n\u011bt zame\u0161kan\u00e9 u\u010divo. Riziko vzniku otok\u016f je limituje i v n\u011bkter\u00fdch aktivit\u00e1ch, nap\u0159\u00edklad nemohou jet na ly\u017ea\u0159sk\u00fd v\u00fdcvik.<\/p>\n\n\n\n
\u201eMlad\u0161\u00ed syn cht\u011bl t\u0159eba stra\u0161n\u011b hr\u00e1t fotbal a hokej, ale nebylo to mo\u017en\u00e9. Alespo\u0148 tedy za\u010dal hr\u00e1t baseball a chytlo ho to. Jsou tam sice tak\u00e9 rizika, ale je hodn\u011b aktivn\u00ed a sport pot\u0159ebuje. Samoz\u0159ejm\u011b v odd\u00edle o nemoci tak\u00e9 v\u011bd\u00ed, ale vych\u00e1zej\u00ed n\u00e1m vst\u0159\u00edc,\u201c vypr\u00e1v\u00ed Michaela.<\/p>\n\n\n\n
Kdy\u017e Dominik jede na z\u00e1pas, m\u00e1 s sebou injekce a aktu\u00e1ln\u00ed l\u00e9ka\u0159skou zpr\u00e1vu \u2013 kdyby se objevily otoky, u\u017e v odd\u00edle v\u011bd\u00ed, co d\u011blat. \u201eStejn\u00e9 je to ve \u0161kole, kde byli ze za\u010d\u00e1tku u\u010ditel\u00e9 zasko\u010den\u00ed. Jsou ale super, u\u017e p\u0159ed n\u00e1stupem jsme se domluvili, co nemoc obn\u00e1\u0161\u00ed, maj\u00ed k dispozici pot\u0159ebnou injekci, a kdyby kluk\u016fm za\u010dal ot\u00e9kat krk nebo lev\u00e1 ruka, v\u011bd\u00ed, \u017ee maj\u00ed okam\u017eit\u011b volat rychlou z\u00e1chrannou slu\u017ebu,\u201c vysv\u011btluje Michaela.<\/p>\n\n\n\n
Prolet\u011bl se vrtuln\u00edkem<\/p>\n\n\n\n
\u201eMen\u0161\u00ed otoky trvaj\u00ed obvykle jeden a\u017e t\u0159i dny a v\u011bt\u0161inou vy\u010dk\u00e1v\u00e1me, u t\u011bch v\u011bt\u0161\u00edch je to i t\u00fdden. Nejhor\u0161\u00ed je lev\u00e1 ruka, kter\u00e1 jde k srdci, anebo krk, kdy v n\u011bm za\u010dnou c\u00edtit knedl\u00edk a nem\u016f\u017eou d\u00fdchat. To u\u017e je na erzetu,\u201c popisuje Michaela.<\/p>\n\n\n\n
Vzpom\u00edn\u00e1 na nejvypjat\u011bj\u0161\u00ed situaci, kter\u00e1 se odehr\u00e1la v roce 2016. Tehdy syna, kter\u00fd je nav\u00edc alergik, p\u00edchla v\u010dela, otekla mu lev\u00e1 ruka a musel b\u00fdt letecky transportov\u00e1n do nemocnice, proto\u017ee se jednalo o \u017eivot ohro\u017euj\u00edc\u00ed stav.<\/p>\n\n\n\n
\u201eOn byl ale nakonec r\u00e1d, \u017ee se prolet\u011bl vrtuln\u00edkem. Sna\u017e\u00edme se to br\u00e1t sportovn\u011b,\u201c dod\u00e1v\u00e1 Michaela.<\/p>\n\n\n\n
D\u016fle\u017eit\u00e9 je, aby byla v p\u0159\u00edpad\u011b vzniku v\u00e1\u017en\u00e9ho otoku pacientovi co nejrychleji pod\u00e1na l\u00e9\u010dba prvn\u00ed pomoci. U syn\u016f pan\u00ed Michaely jde o injek\u010dn\u00ed nitro\u017eiln\u00ed l\u00e9k vyr\u00e1b\u011bn\u00fd z lidsk\u00e9 krevn\u00ed plazmy. Proto\u017ee krevn\u00ed plazmu nelze vyrobit um\u011ble, je nutn\u00e9 pou\u017e\u00edt \u017eivotod\u00e1rnou tekutinu d\u00e1rc\u016f.<\/p>\n\n\n\n
Krevn\u00ed plazma nad zlato<\/p>\n\n\n\n
Zn\u00e1m\u00e1 poh\u00e1dka tvrd\u00ed, \u017ee s\u016fl je nad zlato. Pro mnoh\u00e9 nemocn\u00e9 m\u00e1 ov\u0161em je\u0161t\u011b vy\u0161\u0161\u00ed hodnotu vz\u00e1cn\u00e1 \u017elut\u00e1 tekutina \u2013 krevn\u00ed plazma.<\/p>\n\n\n\n
\u201ePlazma je v\u00fdchoz\u00ed surovinou pro v\u00fdrobu mnoha r\u016fzn\u00fdch \u017eivot zachra\u0148uj\u00edc\u00edch l\u00e9k\u016f. Tyto l\u00e9ky nelze vyrobit jin\u00fdm zp\u016fsobem a jejich celosv\u011btov\u00e1 spot\u0159eba rok od roku stoup\u00e1. Darov\u00e1n\u00edm plazmy pom\u00e1haj\u00ed d\u00e1rci zachr\u00e1nit nebo vylep\u0161it lidsk\u00e9 \u017eivoty,\u201c vysv\u011btluje doktor \u0160uta.<\/p>\n\n\n\n
Postupu, p\u0159i n\u011bm\u017e se z krve odd\u011bluje pouze plazma a zbytek je vracen zp\u011bt do ob\u011bhu d\u00e1rce, se \u0159\u00edk\u00e1 plazmafer\u00e9za. Oproti odb\u011bru pln\u00e9 krve je v\u00fdhodou, \u017ee se v t\u011ble plazma obnov\u00ed b\u011bhem 48 hodin. Proto m\u016f\u017ee d\u00e1rce darovat plazmu ve specializovan\u00e9m centru ka\u017ed\u00e9 dva t\u00fddny. Ne\u017e se ale z odebran\u00e9 plazmy vyrob\u00ed pot\u0159ebn\u00fd l\u00e9k, m\u016f\u017ee to trvat a\u017e dvan\u00e1ct m\u011bs\u00edc\u016f.<\/p>\n\n\n\n
Onemocn\u011bn\u00ed nen\u00ed v\u00e1zan\u00e9 na pohlav\u00ed \u2013 postihuje chlapce i d\u00edvky a je d\u011bdi\u010dn\u00e9. Pokud ale rodi\u010d heredit\u00e1rn\u00edm angioed\u00e9mem trp\u00ed, nemus\u00ed to v\u017edy znamenat, \u017ee jej bude m\u00edt i potomek. Pravd\u011bpodobnost p\u0159enosu posti\u017een\u00ed z rodi\u010d\u016f na d\u011bti z\u00e1vis\u00ed na tom, v jak\u00e9 podob\u011b nesou mutovan\u00e9 geny. Pokud d\u00edt\u011b nemoc zd\u011bd\u00ed, znamen\u00e1 to pro n\u011bj obvykle celo\u017eivotn\u00ed komplikace. Genetickou mutaci, kter\u00e1 nemoc zp\u016fsobuje, zat\u00edm medic\u00edna neum\u00ed vyl\u00e9\u010dit. P\u0159esto dok\u00e1\u017ee pacient\u016fm pomoci \u017e\u00edt kvalitn\u011bj\u0161\u00ed \u017eivot.<\/p>\n\n\n\n
16. kv\u011bten pat\u0159\u00ed nemoci HAE<\/p>\n\n\n\n
Mezin\u00e1rodn\u00ed den HAE p\u0159ipad\u00e1 na 16. kv\u011bten a m\u00e1 zv\u00fd\u0161it pov\u011bdom\u00ed \u0161irok\u00e9 ve\u0159ejnosti o t\u00e9to vz\u00e1cn\u00e9 celo\u017eivotn\u00ed nemoci. C\u00edlem je, aby pacienti s heredit\u00e1rn\u00edm angioed\u00e9mem mohli navzdory chorob\u011b v\u00e9st kvalitn\u00ed \u017eivot \u2013 d\u00edky lep\u0161\u00ed l\u00e9\u010db\u011b, v\u010dasn\u00e9 diagn\u00f3ze i p\u0159\u00edstupu okol\u00ed, kter\u00fd je pro n\u011b velmi d\u016fle\u017eit\u00fd. Jde o vrozenou poruchu, ale n\u011bkdy nemoc nemus\u00ed b\u00fdt odhalena hned p\u0159i narozen\u00ed, proto\u017ee jej\u00ed projevy se mohou objevit a\u017e po n\u011bkolika letech, \u010dasto u d\u011bt\u00ed ve v\u011bku \u0161est a\u017e sedm let. <\/p>\n\n\n\n
Zdena Lackov\u00e1<\/p>\n\n\n\n
Den\u00edk.cz<\/p>\n\n\n\n
https:\/\/www.denik.cz\/<\/a><\/p>\n","protected":false},"excerpt":{"rendered":" D\u011btsk\u00e9 l\u00e9ka\u0159ce nejprve musela maminka dvou nemocn\u00fdch syn\u016f vysv\u011btlit, \u010d\u00edm jej\u00ed d\u011bti trp\u00ed. Vz\u00e1cn\u00e9 onemocn\u011bn\u00ed heredit\u00e1rn\u00ed angioed\u00e9m (HAE), kter\u00e9 Dominikovi a Leo\u0161ovi zp\u016fsobuje na r\u016fzn\u00fdch […]<\/a><\/p>\n<\/div>","protected":false},"author":1,"featured_media":9102,"comment_status":"open","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[9],"tags":[],"class_list":["post-39671","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-zpravy"],"_links":{"self":[{"href":"https:\/\/www.invarena.cz\/index.php?rest_route=\/wp\/v2\/posts\/39671","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=39671"}],"version-history":[{"count":0,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=\/wp\/v2\/posts\/39671\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=\/wp\/v2\/media\/9102"}],"wp:attachment":[{"href":"https:\/\/www.invarena.cz\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=39671"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=%2Fwp%2Fv2%2Fcategories&post=39671"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=%2Fwp%2Fv2%2Ftags&post=39671"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}