L\u00e9ka\u0159ka Hana Bu\u010dkov\u00e1 zasv\u011btila nemoci mot\u00fdl\u00edch k\u0159\u00eddel sv\u016fj profesn\u00ed \u017eivot. P\u0159ed patn\u00e1cti lety s kolegy zalo\u017eila v Brn\u011b prvn\u00ed specializovan\u00e9 pracovi\u0161t\u011b a v\u00e1\u017en\u011b nemocn\u00fdm lidem se sna\u017e\u00ed uleh\u010dit a zlep\u0161it \u017eivot.<\/p>\n
D\u00edky n\u00ed i dal\u0161\u00edm l\u00e9ka\u0159\u016fm \u010cesko pat\u0159\u00ed mezi zem\u011b s nejvysp\u011blej\u0161\u00edm syst\u00e9mem l\u00e9\u010dby tohoto vz\u00e1cn\u00e9ho, poeticky naz\u00fdvan\u00e9ho, a p\u0159ece tak krut\u00e9ho onemocn\u011bn\u00ed.<\/p>\n
Hana Bu\u010dkov\u00e1 dlouh\u00e1 l\u00e9ta pracuje v D\u011btsk\u00e9 nemocnici FN Brno na d\u011btsk\u00e9m ko\u017en\u00edm odd\u011blen\u00ed. U\u017e jej\u00ed p\u0159edch\u016fdce koncentroval vz\u00e1cn\u00e9 vrozen\u00e9 vady, setkala se zde tedy i s t\u011b\u017ek\u00fdmi osudy pacient\u016f.<\/p>\n
Popsat nemoc mot\u00fdl\u00edch k\u0159\u00eddel (EB \u2013 epidermolysis bullosa) je pom\u011brn\u011b slo\u017eit\u00e9, kdy\u017e to zjednodu\u0161\u00edme, jde o vrozen\u00e9 d\u011bdi\u010dn\u00e9 puch\u00fd\u0159nat\u00e9 onemocn\u011bn\u00ed zp\u016fsoben\u00e9 mutacemi v genech. D\u011bl\u00ed se na \u010dty\u0159i formy, kter\u00e9 se li\u0161\u00ed m\u00edrou posti\u017een\u00ed k\u016f\u017ee a org\u00e1n\u016f. Tyto z\u00e1kladn\u00ed formy v\u0161ak maj\u00ed t\u00e9m\u011b\u0159 \u010dty\u0159icet podtyp\u016f.<\/p>\n
V\u011bt\u0161ina pacient\u016f m\u00e1 leh\u010d\u00ed formu, kter\u00e1 se projevuje p\u0159edev\u0161\u00edm zvn\u011bj\u0161ku, zbyl\u00e9 jsou t\u011b\u017ek\u00e9 a krom\u011b puch\u00fd\u0159\u016f na k\u016f\u017ei zp\u016fsobuj\u00ed i celou \u0159adu dal\u0161\u00edch probl\u00e9m\u016f. „Kde jsou sliznice, tam v\u0161ude se nemoc m\u016f\u017ee projevit. Pacienti maj\u00ed probl\u00e9my s polyk\u00e1n\u00edm, vyprazd\u0148ov\u00e1n\u00edm, trp\u00ed podv\u00fd\u017eivou, sr\u016fstaj\u00ed jim prsty, maj\u00ed velk\u00e9 defekty v dutin\u011b \u00fastn\u00ed, sklon k zubn\u00edm kaz\u016fm,“ \u0159\u00edk\u00e1 Hana Bu\u010dkov\u00e1. Vysok\u00e9 je i riziko karcinomu k\u016f\u017ee. K poran\u011bn\u00ed p\u0159itom sta\u010d\u00ed jak\u00fdkoli otlak, u t\u011b\u017e\u0161\u00edch forem t\u0159eba i od \u0161v\u016f na oble\u010den\u00ed. Proto pacienti pot\u0159ebuj\u00ed modern\u00ed, kvalitn\u00ed o\u0161et\u0159ovac\u00ed materi\u00e1ly.<\/p>\n
Ty v\u0161ak p\u0159ed dvaceti lety nebyly a ani se tolik ne\u0159e\u0161ily komplikace, kter\u00e9 nast\u00e1vaj\u00ed u pacient\u016f s t\u011b\u017ekou formou. „Kdy\u017e jsem vid\u011bla, jak jsou obvazy na d\u011btech p\u0159ilepen\u00e9, obt\u00ed\u017en\u011b se sund\u00e1vaj\u00ed p\u0159i koupeli, d\u011bti trp\u00ed podv\u00fd\u017eivou, chudokrevnost\u00ed, za\u010dala jsem se v\u00edc o nemoc zaj\u00edmat. V devades\u00e1t\u00fdch letech za\u010dala fungovat prvn\u00ed specializovan\u00e1 centra odborn\u00edk\u016f a pacientsk\u00e1 centra v Evrop\u011b, ve vysp\u011bl\u00fdch st\u00e1tech \u2013 Rakousku, Nizozemsku, Anglii a Francii. To mi p\u0159i\u0161lo skv\u011bl\u00e9. Nav\u00edc jsem m\u011bla i doma dobr\u00e9ho spojence, m\u016fj man\u017eel byl histopatolog, tak\u017ee od za\u010d\u00e1tku jsme m\u011bli modern\u00ed diagnostiku.<\/p>\n
V t\u00e9 dob\u011b jsme pe\u010dovali asi o 38 pacient\u016f.<\/p>\n
Kdy\u017e jsem dermatologick\u00e9 spole\u010dnosti p\u0159ednesla z\u00e1m\u011br vytvo\u0159it pro pacienty specializovan\u00e9 centrum s t\u00fdmem odborn\u00edk\u016f, byli nad\u0161eni a sou\u010dasn\u011b p\u0159ekvapeni, kolik pacient\u016f \u2013 mot\u00fdlk\u016f \u2013 m\u00e1me,“ vypr\u00e1v\u00ed Hana Bu\u010dkov\u00e1.<\/p>\n
EB centrum<\/p>\n
Sestavila t\u00fdm EB Centra, ve kter\u00e9m zpo\u010d\u00e1tku byla ona \u2013 dermatolo\u017eka, jej\u00ed man\u017eel \u2013 histopatolog, oslovila i rehabilita\u010dn\u00edho l\u00e9ka\u0159e, nebo\u0165 klouby nemocn\u00fdm lidem tuhnou, a plastick\u00e9ho chirurga. Ten odstra\u0148uje lo\u017eiska rakoviny k\u016f\u017ee, zachov\u00e1v\u00e1 pacient\u016fm \u00fachop ruky, kdy\u017e doch\u00e1z\u00ed k sr\u016fst\u016fm prst\u016f. „Postupn\u011b jsme se rozrostli na 25 odborn\u00edk\u016f, kte\u0159\u00ed pe\u010duj\u00ed o d\u011bti i dosp\u011bl\u00e9 pacienty s nemoc\u00ed m o -t\u00fdl\u00edch k\u0159\u00eddel. Z\u00e1rove\u0148 jsem cht\u011bla, aby s t\u00fdmem specialist\u016f \u0161la ruku v ruce pacientsk\u00e1 organizace,“ \u0159\u00edk\u00e1. Tak vznikla \u010desk\u00e1 DEBRA. Za odborn\u00edky v EB Centru se sj\u00ed\u017ed\u011bj\u00ed pacienti z cel\u00e9 republiky a sdru\u017een\u00ed DEBRA jim zaji\u0161\u0165uje \u0161irokou s\u00ed\u0165 slu\u017eeb a podpory. „Minul\u00fd m\u011bs\u00edc DEBRA v Pra\u017esk\u00e9 k\u0159i\u017eovatce pok\u0159tila charitativn\u00ed kalend\u00e1\u0159 na rok 2017. Cel\u00fd v\u00fdt\u011b\u017eek z prodeje jde na podporu pacient\u016f s nemoc\u00ed mot\u00fdl\u00edch k\u0159\u00eddel,“ \u0159\u00edk\u00e1 Hana Bu\u010dkov\u00e1.<\/p>\n
Lid\u00e9, kte\u0159\u00ed s nemocn\u00fdmi a jejich bl\u00edzk\u00fdmi pracuj\u00ed, mus\u00ed b\u00fdt velmi empati\u010dt\u00ed. Nejen ti ze sdru\u017een\u00ed DEBRA, ale i l\u00e9ka\u0159i. \u00dad\u011bl rodiny, kde je chronicky nemocn\u00fd \u010dlov\u011bk, je velice t\u011b\u017ek\u00fd. „P\u0159ijdou k n\u00e1m a my jim nem\u016f\u017eeme d\u00e1t nad\u011bji, \u017ee je nemoci \u00fapln\u011b zbav\u00edme. Jejich denn\u00ed starosti jsou tak velk\u00e9 a tak naru\u0161\u00ed a obr\u00e1t\u00ed \u017eivot vzh\u016fru nohama, \u017ee se rodina n\u011bkdy rozpadne,“ \u0159\u00edk\u00e1 l\u00e9ka\u0159ka.<\/p>\n
Dod\u00e1v\u00e1, \u017ee nemocn\u00ed a jejich bl\u00edzc\u00ed jsou nesm\u00edrn\u011b state\u010dn\u00ed a obdivuhodn\u00ed lid\u00e9. Pro\u017e\u00edvaj\u00ed to, co si zdrav\u00fd \u010dlov\u011bk nedok\u00e1\u017ee p\u0159edstavit, a p\u0159itom se v\u011bnuj\u00ed sv\u00fdm kon\u00ed\u010dk\u016fm, sna\u017e\u00ed se udr\u017eovat si optimismus, neklesat na mysli, studuj\u00ed, cht\u011bj\u00ed pracovat.<\/p>\n
Nyn\u00ed se v \u010cesku s nemoc\u00ed mot\u00fdl\u00edch k\u0159\u00eddel l\u00e9\u010d\u00ed na 300 lid\u00ed. Z nich m\u00e1 56 % tzv. leh\u010d\u00ed formu, kdy na pohled jejich onemocn\u011bn\u00ed nepozn\u00e1te. „Trp\u00ed drobn\u00fdmi puch\u00fd\u0159ky na rukou, na nohou. Ale maj\u00ed b\u011b\u017enou kvalitu a d\u00e9lku \u017eivota. Pak jsou typy, kter\u00e9 bohu\u017eel zem\u0159ou v prvn\u00edm roce \u017eivota, a pak existuje t\u011b\u017ek\u00e1 forma \u2013 dystrofick\u00e1 a junk\u010dn\u00ed. Lid\u00e9 s t\u00edmto typem maj\u00ed velk\u00e9 posti\u017een\u00ed a \u010dasto je brzy v dosp\u011blosti ohro\u017euje rakovina k\u016f\u017ee, tzv. spinocelul\u00e1rn\u00ed karcinom, kter\u00fd je \u010dasto zahub\u00ed. P\u0159e\u017eit\u00ed je tak p\u011bt let,“ vysv\u011btluje Hana Bu\u010dkov\u00e1.<\/p>\n
diagnostika a prevence<\/p>\n
\u010cesk\u00e9 EB centrum je velice dob\u0159e hodnoceno i ve sv\u011bt\u011b. „\u010cesko m\u00e1 velice dobr\u00e9 jm\u00e9no, m\u00e1 velmi dobrou diagnostiku a propracovanou preventivn\u00ed p\u00e9\u010di. DEBRA m\u00e1 tak\u00e9 velmi dobr\u00fd zvuk,“ \u0159\u00edk\u00e1 l\u00e9ka\u0159ka. „Pro m\u011b je velk\u00e1 \u010dest, \u017ee jsme partnerem pro Salzburg, kter\u00fd je v\u00fdborn\u00fd, pro Groningen, kde je \u0161pi\u010dkov\u00fd odborn\u00edk profesor Jongmann, kde si v\u00e1\u017e\u00ed kvality p\u00e9\u010de, kterou pacient\u016fm poskytujeme. Je zaj\u00edmav\u00e9, \u017ee N\u011bmecko m\u00e1 v\u00fdproto, bornou v\u011bdeckou z\u00e1kladnu ve Freiburgu, ale nem\u00e1 klinickou z\u00e1kladnu pro pacienty, ti jezd\u00ed do Salzburgu. Kdy\u017e to vezmu kolem n\u00e1s, tak z b\u00fdval\u00e9ho socialistick\u00e9ho bloku jsme jedin\u00ed, kdo m\u00e1 specializovan\u00e9 EB Centrum. Jezd\u00ed k n\u00e1m i cizinci. A to v\u0161e se d\u011bje \u017ee na za\u010d\u00e1tku bylo nad\u0161en\u00ed.“ U n\u00e1s je centrum pro \u010ceskou republiku ve Fakultn\u00ed nemocnici Brno. „B\u011b\u017en\u011b u\u017e to funguje tak, \u017ee kdy\u017e se narod\u00ed takto nemocn\u00e9 d\u011b\u0165\u00e1tko, p\u0159ivezou ho na novorozeneckou JIP Pediatrick\u00e9 kliniky FN Brno, kde dermatolog a EB sestra zau\u010duj\u00ed maminku v o\u0161et\u0159ovac\u00ed technice. Ta je velice n\u00e1ro\u010dn\u00e1, p\u0159evaz trv\u00e1 dv\u011b t\u0159i hodiny,“ popisuje Hana Bu\u010dkov\u00e1. Nyn\u00ed u\u017e existuje na p\u0159evazy speci\u00e1ln\u00ed materi\u00e1l, kter\u00fd na r\u00e1nu lehce p\u0159ilehne, z\u016fstane na n\u00ed t\u0159i \u010dty\u0159i dny a pak se s\u00e1m sekrec\u00ed uvol\u0148uje, a je\u0161t\u011b podporuje hojen\u00ed.<\/p>\n
Vnemocnici se ihned d\u011bl\u00e1 diagnostika histologick\u00e1 a na molekul\u00e1rn\u00ed \u00farovni. „\u010casto, ne\u017e je d\u00edt\u011b z nemocnice propu\u0161t\u011bno, co\u017e je tak za dva t\u0159i t\u00fddny, u\u017e zn\u00e1me mutaci, kter\u00e1 ur\u010dit\u00fd typ EB zp\u016fsobila. V novorozeneck\u00e9m v\u011bku je zaj\u00edmav\u00e9, \u017ee n\u011bkdy projevy nemoci mohou vypadat velice dramaticky, ale v pr\u016fb\u011bhu let se zlep\u0161\u00ed a nejsou t\u00e9m\u011b\u0159 poznateln\u00e9.<\/p>\n
My jim tedy m\u016f\u017eeme p\u0159i odchodu z nemocnice \u0159\u00edci konkr\u00e9tn\u00ed poznatky. Nastoup\u00ed gastroenterolog, nutri\u010dn\u00ed specialista, aby jim vysv\u011btlil, jak se maj\u00ed stravovat, d\u00e1le psycholog a soci\u00e1ln\u00ed pracovnice. R\u00e1ny se l\u00e9pe hoj\u00ed, je-li strava vyv\u00e1\u017een\u00e1 a kaloricky vydatn\u00e1. Do p\u00e9\u010de se zapojuj\u00ed dal\u0161\u00ed specialist\u00e9, podle v\u00fdvoje pacientov\u00fdch p\u0159\u00edznak\u016f. D\u016fle\u017eitou sou\u010d\u00e1st\u00ed komplexn\u00ed p\u00e9\u010de o pacienta a jeho rodinu je genetick\u00e9 poradenstv\u00ed. Um\u00edme zabr\u00e1nit d\u011bd\u011bn\u00ed nejz\u00e1va\u017en\u011bj\u0161\u00edch forem z generace na generaci, prov\u011b\u0159it, zda rodi\u010de, sourozenci \u010di n\u011bkdo v \u0161ir\u0161\u00edm p\u0159\u00edbuzenstvu nejsou zdrav\u00ed p\u0159ena\u0161e\u010di mutovan\u00e9ho genu pro EB. Ale nezabr\u00e1n\u00edme prvov\u00fdskytu onemocn\u011bn\u00ed. To znamen\u00e1, \u017ee neum\u00edme dop\u0159edu diagnostikovat EB v t\u011bhotenstv\u00ed, kdy\u017e jde o prvn\u00ed v\u00fdskyt v rodin\u011b. V dne\u0161n\u00ed dob\u011b se v\u00ed, \u017ee nemoc zp\u016fsobuje 18 gen\u016f a existuje kolem 40 jej\u00edch klinick\u00fdch typ\u016f. Z\u00e1ludnost nejt\u011b\u017e\u0161\u00edch, recesivn\u00edch forem EB tkv\u00ed v tom, \u017ee nikdo z n\u00e1s nev\u00ed, jestli p\u0159\u00edpadn\u011b nenese jeden mutovan\u00fd gen t\u00e9to nemoci. U v\u011bt\u0161iny t\u011b\u017ek\u00fdch pacient\u016f jsou jejich rodi\u010de zdrav\u00fdmi p\u0159ena\u0161e\u010di mutovan\u00e9ho genu. Z krve jsme schopni rozk\u00f3dovat, jestli tomu tak je.“<\/p>\n
Vp\u0159\u00edpad\u011b, \u017ee jsou p\u0159ena\u0161e\u010di oba partne\u0159i a cht\u011bj\u00ed miminko, nab\u00edz\u00ed se pl\u00e1novan\u00e9 t\u011bhotenstv\u00ed, kdy se d\u00e1 ve 14.\u201316. t\u00fddnu gravidity zjistit, zda plod je zdrav\u00fd. Mnohem \u0161etrn\u011bj\u0161\u00ed je ale preimplanta\u010dn\u00ed metoda, tedy tzv. d\u00edt\u011b ze zkumavky. Tak rod\u00ed i nemocn\u00e9 maminky s EB zdrav\u00e9 d\u011bti.<\/p>\n
L\u00e9ka\u0159ka Hana Bu\u010dkov\u00e1 velice oce\u0148uje sv\u00e9 pacienty a jej\u00edch bl\u00edzk\u00e9. Sama p\u016fsob\u00ed nesm\u00edrn\u011b klidn\u011b a optimisticky. „N\u011bkdy v\u00e1s to docela sraz\u00ed, proto\u017ee utrpen\u00ed pacient\u016f je velk\u00e9. Na druh\u00e9 stran\u011b si mus\u00edte naj\u00edt kompenzaci, j\u00e1 ji m\u00e1m v hudb\u011b, v\u00fdstav\u00e1ch, m\u00e1m r\u00e1da hory. Vn\u00edm\u00e1m v\u011bci oby\u010dejn\u00e9, p\u0159itom kr\u00e1sn\u00e9. A m\u00e1m \u0161est vnou\u010d\u00e1tek, jsou to moje zlat\u00ed\u010dka.<\/p>\n
Pozitivn\u00ed lad\u011bn\u00ed jsem asi dostala do v\u00ednku.<\/p>\n
Velmi mne pot\u011b\u0161ilo, \u017ee letos EB Centrum a DEBRA z\u00edskaly presti\u017en\u00ed ocen\u011bn\u00ed \u010cesk\u00fd Goodwill 2016 pr\u00e1v\u011b za pomoc, kterou pacient\u016fm s nemoc\u00ed mot\u00fdl\u00edch k\u0159\u00eddel poskytuj\u00ed. \u010clov\u011bk si toho v\u00e1\u017e\u00ed,“ \u0159\u00edk\u00e1 Hana Bu\u010dkov\u00e1.<\/p>\n
Mus\u00edte si naj\u00edt kompenzaci, j\u00e1 ji m\u00e1m v hudb\u011b, v\u00fdstav\u00e1ch, m\u00e1m r\u00e1da hory. Vn\u00edm\u00e1m v\u011bci oby\u010dejn\u00e9, p\u0159itom kr\u00e1sn\u00e9<\/p>\n
Andrea Zunov\u00e1<\/p>\n
Pr\u00e1vo<\/p>\n","protected":false},"excerpt":{"rendered":"
L\u00e9ka\u0159ka Hana Bu\u010dkov\u00e1 zasv\u011btila nemoci mot\u00fdl\u00edch k\u0159\u00eddel sv\u016fj profesn\u00ed \u017eivot. P\u0159ed patn\u00e1cti lety s kolegy zalo\u017eila v Brn\u011b prvn\u00ed specializovan\u00e9 pracovi\u0161t\u011b a v\u00e1\u017en\u011b nemocn\u00fdm lidem […]<\/a><\/p>\n<\/div>","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[9],"tags":[],"class_list":["post-26824","post","type-post","status-publish","format-standard","hentry","category-zpravy"],"_links":{"self":[{"href":"https:\/\/www.invarena.cz\/index.php?rest_route=\/wp\/v2\/posts\/26824","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=26824"}],"version-history":[{"count":0,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=\/wp\/v2\/posts\/26824\/revisions"}],"wp:attachment":[{"href":"https:\/\/www.invarena.cz\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=26824"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=%2Fwp%2Fv2%2Fcategories&post=26824"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=%2Fwp%2Fv2%2Ftags&post=26824"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}