{"id":15009,"date":"2014-03-05T12:38:26","date_gmt":"2014-03-05T11:38:26","guid":{"rendered":"http:\/\/www.invarena.cz\/?p=15009"},"modified":"2014-03-05T13:41:40","modified_gmt":"2014-03-05T12:41:40","slug":"rozhovor-s-hanou-buckovou","status":"publish","type":"post","link":"https:\/\/www.invarena.cz\/?p=15009","title":{"rendered":"Prim\u00e1\u0159ka o nemoci mot\u00fdl\u00edch k\u0159\u00eddel"},"content":{"rendered":"
\n

V Radio\u017eurn\u00e1lu hovo\u0159ila prim\u00e1\u0159ka ko\u017en\u00edho odd\u011blen\u00ed Pediatrick\u00e9 kliniky Fakultn\u00ed nemocnice v Brn\u011b Hana Bu\u010dkov\u00e1\u00a0 o nemoci mot\u00fdl\u00edch k\u0159\u00eddel a o jej\u00ed l\u00e9\u010db\u011b.<\/strong><\/p>\n

 <\/p>\n

Vy jste v roce 2001 zalo\u017eila EB Centrum, kter\u00e9 se v\u011bnuje nemoci mot\u00fdl\u00edch k\u0159\u00eddel. Tak\u00e9 jste zalo\u017eila nadaci Debra. Ale \u0159ekn\u011bte mi, jak\u00e9 bylo to prvn\u00ed, \u00fapln\u011b prvn\u00ed setk\u00e1n\u00ed s va\u0161\u00edm pacientem, kter\u00fd tuhle nemoc m\u011bl? V\u011bd\u011bla jste u\u017e tehdy, co to je?<\/strong><\/p>\n

Ano, to jsem v\u011bd\u011bla, a ono je to je\u0161t\u011b podstatn\u011b d\u0159\u00edve. Kdy\u017e jsem se s t\u00edmto pacientem setkala, a to d\u00edky tomu, \u017ee jsem pracovala na d\u011btsk\u00e9m ko\u017en\u00edm odd\u011blen\u00ed u docenta Rovensk\u00e9ho v Brn\u011b, v d\u011btsk\u00e9 nemocnici. A pr\u00e1v\u011b tam jsem vid\u011bla ty prvn\u00ed tristn\u00ed pohledy na tyto pacienty. I kdy\u017e jsme v\u011bd\u011bli, \u017ee jde o tuhle chorobu, neum\u011bli jsme ji oddiferencovat nebo za\u0159adit, ale ta technika o\u0161et\u0159en\u00ed, ta se diametr\u00e1ln\u011b li\u0161ila. Byly to p\u0159ilepen\u00e9 obvazy prokrv\u00e1cen\u00e9, kter\u00e9 ve van\u00e1ch jsme odstra\u0148ovali, byly to velmi bolestiv\u00e9 p\u0159evazy. Ta technika v\u016fbec medic\u00ednsk\u00e1 tehdy byla opravdu v plenk\u00e1ch.<\/p>\n

V\u011b\u0159\u00edm, \u017ee dneska se ta technika zlep\u0161ila, ale maj\u00ed tito pacienti n\u011bjakou nad\u011bji v podob\u011b t\u0159eba n\u011bjak\u00e9ho l\u00e9ku nebo alespo\u0148 n\u011b\u010deho, co je daleko \u0161etrn\u011bj\u0161\u00ed k jejich o\u0161et\u0159ov\u00e1n\u00ed?<\/strong><\/p>\n

To ur\u010dit\u011b ano. Rozd\u011blila bych to na dv\u011b v\u011bci. My neum\u00edme tu p\u0159\u00ed\u010dinu. My v\u00edme, \u017ee je to chyba v genu a jsou v\u00fdzkumn\u00e9 t\u00fdmy na sv\u011bt\u011b, kter\u00e9 na tom pracuj\u00ed, aby se ten gen opravil. Ale podp\u016frn\u00e1 l\u00e9\u010dba m\u011bla raketov\u00fd start mezi t\u00edm dvac\u00e1t\u00fdm a jednadvac\u00e1t\u00fdm stolet\u00edm. Ty z\u00e1sady techniky o\u0161et\u0159en\u00ed ran, z\u00e1sady v\u00fd\u017eivy, kter\u00e1 je velmi d\u016fle\u017eit\u00e1, aby organizmus nebyl oslaben\u00fd, to jsou v\u011bci, kter\u00e9 se velmi posunuly. A dal\u0161\u00ed, a i n\u00e1\u0161 t\u00fdm na tom bude pracovat, je l\u00e9\u010dba kmenov\u00fdmi bu\u0148kami, ur\u010dit\u00fdm zp\u016fsobem ty bunky oprav\u00edme a sna\u017e\u00edme se je implantovat do ran pacient\u016f.<\/p>\n

Lep\u0161\u00ed, ne\u017e kdy\u017e tu nemoc budete popisovat vy, bude, kdy\u017e to, co za\u017e\u00edv\u00e1 denn\u011b, pop\u00ed\u0161e jedna z va\u0161ich pacientek.<\/strong><\/p>\n

pacientka:
\nTe\u010f t\u0159eba, kdy\u017e sed\u00edm na voz\u00edku a i kdy\u017e m\u00e1m podlo\u017eky, tak c\u00edt\u00edm bolest, proto\u017ee po cel\u00e9m t\u011ble m\u00e1m puch\u00fd\u0159e, r\u00e1ny a tak\u00e9 jizvy, kter\u00e9 z\u016fst\u00e1vaj\u00ed po t\u011bch ran\u00e1ch a puch\u00fd\u0159\u00edch. Ty jizvy se d\u011blaj\u00ed t\u0159eba i v j\u00edcnu, tak\u017ee mus\u00edm j\u00edst mixovanou stravu, d\u011blaj\u00ed se mi i na plosk\u00e1ch nohou, tak\u017ee m\u011b bol\u00ed ka\u017ed\u00fd krok. Sr\u016fstaj\u00ed mi prsty na rukou, tak\u017ee se nem\u016f\u017eu sama o sebe postarat. Nem\u00e1m skoro \u017e\u00e1dn\u00e9 vlasy, rozpadly se mi zuby, nem\u00e1m nehty. S tou nemoc\u00ed souvis\u00ed i podv\u00fd\u017eiva, tak\u017ee jsem 14 dn\u016f byla te\u010f na kapa\u010dk\u00e1ch v bohnick\u00e9 nemocnici. T\u011bch v\u011bc\u00ed, co bych mohla \u0159\u00edct, je stra\u0161n\u011b moc.<\/p>\n

To je pacientka pan\u00ed prim\u00e1\u0159ky, jmenuje se Olinka a podle m\u00e9ho na to, jak vlastn\u011b ona sama popsala, co j\u00ed je, p\u016fsobila nesm\u00edrn\u011b vesele. Jak\u00e1 je?<\/strong><\/p>\n

BU\u010cKOV\u00c1, prim\u00e1\u0159ka
\nAno, p\u0159esn\u011b toto jsem v\u00e1m cht\u011bla \u0159\u00edct. A ona pat\u0159\u00ed do skupiny t\u011bch 40%, t\u011bch velmi t\u011b\u017ek\u00fdch pacient\u016f, kde krom\u011b k\u016f\u017ee jsou posti\u017eeny i vnit\u0159n\u00ed org\u00e1ny. A j\u00e1 se hluboce p\u0159ed t\u011bmi pacienty skl\u00e1n\u00edm, oni jsou p\u0159ev\u00e1\u017en\u011b pozitivn\u00ed a velmi siln\u00ed. N\u00e1s skol\u00ed leckter\u00e1 bol\u00edstka, ale je neuv\u011b\u0159iteln\u00e9, s \u010d\u00edm oni \u017eij\u00ed a jak bojuj\u00ed.<\/p>\n

Tak my jsme jim i na t\u00e9hle t\u011b\u017ek\u00e9 cest\u011b \u017eivotem pomohli. Tak jak Olinka vzpom\u00ednala, te\u010f chod\u00ed a m\u00e1 specieln\u00ed v\u00fd\u017eivu. Ta v\u00fd\u017eiva mus\u00ed b\u00fdt na 140, 150% oproti zdrav\u00e9mu jedinci, ano, aby ona dostala do organismu pot\u0159ebn\u00e9 l\u00e1tky.\u00a0 proto\u017ee Olinka pat\u0159\u00ed mezi velmi t\u011b\u017ek\u00e9 pacienty, tak jej\u00ed \u017eivot je velmi slo\u017eit\u00fd.To znamen\u00e1, p\u0159edstavovat si, \u017ee si d\u00e1 kol\u00e1\u010d t\u0159eba nebo n\u011bco takov\u00e9ho, je v podstat\u011b nemo\u017en\u00e9?<\/strong><\/p>\n

Ano, u t\u011bch t\u011b\u017ek\u00fdch forem ne, stravu jim maminky musej\u00ed mixovat a u n\u011bkter\u00fdch ta slo\u017eitost se vystup\u0148uje tak, \u017ee ten j\u00edcen \u00fapln\u011b se z\u00fa\u017e\u00ed a sroste na jeden dva milimetry, ji\u017e se ned\u00e1 technicky dilatovat na gastroenterologick\u00e9 klinice a \u017ee jim je zavedena v\u00fd\u017eiva do \u017ealudku nebo do st\u0159ev.<\/p>\n

Vy jste skute\u010dn\u011b v EB Centru ud\u011blali obrovsk\u00fd kus pr\u00e1ce v\u010detn\u011b toho, \u017ee vlastn\u011b nepracujete jenom s pacienty samotn\u00fdmi, ale s jejich rodi\u010di. Pochopila jsem, \u017ee tam m\u00e1te pro rodi\u010de t\u011bch mal\u00fdch pacient\u016f, u kter\u00fdch se teprve to onemocn\u011bn\u00ed objev\u00ed, n\u011bjakou speci\u00e1ln\u00ed \u0161kolku, \u0161kolu, co se tam u\u010d\u00ed?<\/strong><\/p>\n

V\u00edte, ta pou\u0165 u tohohle t\u011b\u017ek\u00e9ho onemocn\u011bn\u00ed za\u010d\u00edn\u00e1 ji\u017e v tom novorozeneck\u00e9m v\u011bku, kdy v podv\u011bdom\u00ed l\u00e9ka\u0159\u016f v \u010cesk\u00e9 republice to\u00a0 ji\u017e je a pos\u00edlaj\u00ed novorozence s podez\u0159en\u00edm na toto onemocn\u011bn\u00ed na na\u0161i pediatrickou kliniku, na jednotku intenzivn\u00ed p\u00e9\u010de, kdy d\u00edt\u011b je p\u0159ijato i s maminkou a za\u010d\u00edn\u00e1 se diagnostika a technika o\u0161et\u0159en\u00ed. Maminka je sou\u010dasn\u011b zacvi\u010dov\u00e1na. A kdy\u017e si vemete toho t\u0159\u00edkilov\u00e9ho tvore\u010dka, na n\u011bm my, j\u00e1 s tou EB sestrou pracuji i 2 hodiny, o\u0161et\u0159ujeme.<\/p>\n

J\u00e1 si jako maminka neum\u00edm v\u016fbec p\u0159edstavit, \u017ee bych o\u0161et\u0159ovala t\u0159eba hodinu, dv\u011b hodin svoje d\u011b\u0165\u00e1tko, kter\u00e9 by mi u toho plakalo.\u00a0<\/strong><\/p>\n

V tom t\u00fdmu m\u00e1me i anesteziologa, kter\u00fd tlum\u00ed bolest, ale zaj\u00edmav\u00e9 je u t\u011bch star\u0161\u00edch, \u017ee slu\u017eby anesteziologa, t\u00e9 poradny pro tlumen\u00ed nebo l\u00e9\u010den\u00ed bolesti vyu\u017e\u00edvaj\u00ed zcela minim\u00e1ln\u011b a oni neuv\u011b\u0159iteln\u011b sn\u00e1\u0161ej\u00ed tu bolest. To je pro n\u00e1s a\u017e nepochopiteln\u00e9.<\/p>\n

Vy mluv\u00edte o t\u011bch star\u0161\u00edch, to jsou jak star\u00e9 d\u011bti?<\/strong><\/p>\n

V\u00edte, my pe\u010dujeme o d\u011bti, ale i dosp\u011bl\u00e9.<\/p>\n

vy ty pacienty neum\u00edte vyl\u00e9\u010dit, ale jistou dobrou zpr\u00e1vou pro jejich rodiny a pro jejich budoucnost m\u00e1te.<\/strong><\/p>\n

Ano. Ono se to t\u00fdk\u00e1 t\u011bch modern\u00edch metod, a to jsou metody molekul\u00e1rn\u00ed biologie, my um\u00edme naj\u00edt ten nemocn\u00fd gen, kter\u00fd zp\u016fsob\u00ed tu nemoc. A pr\u00e1v\u011b tam vlastn\u011b hled\u00e1me tu p\u0159\u00ed\u010dinu u t\u011bch rodi\u010d\u016f. to znamen\u00e1, \u017ee rodi\u010de t\u011bchto pacient\u016f jsou \u010dasto zdrav\u00ed, jsou takzvan\u011b zdrav\u00fdmi p\u0159ena\u0161e\u010di, jak tat\u00ednek, tak maminka. Oni netu\u0161\u00ed, \u017ee nesou ten mutovan\u00fd gen a jejich d\u011b\u0165\u00e1tko onemocn\u00ed touhle chorobou. A my dok\u00e1\u017eeme d\u00edky tomu pak v rodin\u011b hledat, jestli tam nejsou dal\u0161\u00ed skryt\u00ed p\u0159ena\u0161e\u010di. A v p\u0159\u00edpad\u011b pl\u00e1nov\u00e1n\u00ed rodiny t\u011bchto mlad\u00fdch jsme schopni jim pomoct tak, aby se jim narodilo zdrav\u00e9 d\u011b\u0165\u00e1tko.<\/p>\n

Znamen\u00e1 to, \u017ee m\u00e1te tak\u00e9 pacienty s touhle nemoc\u00ed, kte\u0159\u00ed sami maj\u00ed d\u011bti?<\/strong><\/p>\n

Ano, je tomu tak, a to i ti velmi t\u011b\u017ec\u00ed. J\u00e1 bych se dostala k tomu z\u00e1kladn\u00edmu d\u011blen\u00ed a v\u016fbec jak tato nemoc ty pacienty postihuje. Mohu \u0159\u00edct u t\u011bch 60% t\u00e9m\u011b\u0159 nepozn\u00e1me je. Nen\u00ed to tak vid\u011bt, m\u011b bol\u00ed jeden puch\u00fd\u0159, kter\u00fd se mi v nov\u00e9 bot\u011b ud\u011bl\u00e1 na pat\u011b, ano, oni nemaj\u00ed jednoduch\u00fd \u017eivot. Ale je to takov\u00e1 \u00fanosn\u011bj\u0161\u00ed forma. A t\u011bch 40% je t\u011b\u017ek\u00fdch. A pacient\u016f je toho \u010dasu p\u0159es 226, a i z t\u011bch 40% d\u00edky tomu, \u017ee spolupracuj\u00ed s na\u0161\u00edm centrem, tak m\u00e1me maminky, kter\u00e9 maj\u00ed dystrofickou formu, tu nejt\u011b\u017e\u0161\u00ed EB a maj\u00ed zdrav\u00e9 d\u011b\u0165\u00e1tko. A samoz\u0159ejm\u011b m\u00e1me plno gravidit, t\u011bhotenstv\u00ed, kde prvn\u00ed d\u011b\u0165\u00e1tko t\u0159eba se narodilo s touhle chorobou a mlad\u00ed rodi\u010de s n\u00e1mi pak spolupracovali a dal\u0161\u00ed d\u011b\u0165\u00e1tka maj\u00ed zdrav\u00e9.<\/p>\n

kdy\u017e jsem tak \u010detla n\u011bkter\u00e9 p\u0159\u00edb\u011bhy va\u0161ich pacient\u016f, tak jsem si \u0159\u00edkala, \u017ee jsou chv\u00edle, kdy na n\u011b mus\u00edte b\u00fdt hrozn\u011b py\u0161n\u00e1.<\/strong><\/p>\n

Je tomu tak. Zd\u016fraz\u0148uji, tito pacienti nemaj\u00ed posti\u017een\u00fd intelekt. \u0158ada m\u00e1 odborn\u00e9 vzd\u011bl\u00e1n\u00ed, maj\u00ed vysok\u00e9 \u0161koly a na druh\u00e9 stran\u011b zase m\u011b sou\u017e\u00ed, \u017ee maj\u00ed trochu probl\u00e9m na trhu pr\u00e1ce z\u00edskat to povol\u00e1n\u00ed. A to m\u011b za n\u011b sou\u017e\u00ed a v tom pr\u00e1v\u011b my a Debra, pacientsk\u00e1 organizace, je velmi podporujeme.<\/p>\n

Pan\u00ed Mart\u00e9nkov\u00e1 p\u00ed\u0161e: „Moc zdrav\u00edm pan\u00ed prim\u00e1\u0159ku a chci pod\u011bkovat za v\u0161echno, co d\u011bl\u00e1 pro pacienty s nemoc\u00ed mot\u00fdl\u00edch k\u0159\u00eddel. Moc si v\u00e1\u017e\u00edm za \u00fasil\u00ed, kter\u00e9 vynalo\u017eila, aby pomohla moj\u00ed dce\u0159i.“ Tat\u00ednek Honz\u00edka, pan Tarcala, se v\u00e1s pt\u00e1, jak jste si kdysi d\u00e1vno p\u0159edstavovala tu dne\u0161n\u00ed situaci v l\u00e9\u010db\u011b EB a jak vlastn\u011b moc se ta va\u0161e tehdej\u0161\u00ed p\u0159edstava li\u0161ila od toho, co te\u010f d\u011bl\u00e1te a co te\u010f v\u0161echno m\u00e1te k dispozici.<\/strong>J\u00e1 d\u011bkuji za ty dotazy a zdrav\u00edm ob\u011b dv\u011b rodiny. Pan\u00ed Mart\u00e9nkovou, to mn\u011b vh\u00e1n\u00ed a\u017e slzy do o\u010d\u00ed. A moc ji zdrav\u00edm a d\u011bkuji za tohle uzn\u00e1n\u00ed, proto\u017ee my jsme pro\u0161li velmi t\u011b\u017ekou cestu spolu a Leni\u010dka u\u017e s n\u00e1mi nen\u00ed, jej\u00ed dcera. A to si velmi toho v\u00e1\u017e\u00edm, jej\u00edho hodnocen\u00ed, proto\u017ee prost\u011b tak to je. A k panu Tarcalovi, j\u00e1 mysl\u00edm, rodi\u010de u\u017e m\u011b znaj\u00ed, j\u00e1 jsem takov\u00fd ten \u00faporn\u00fd tvor, kter\u00fd kdy\u017e se zakousne a chce nebo vid\u00ed ur\u010ditou vidinu pomoci,. Samoz\u0159ejm\u011b to nen\u00ed jenom m\u00e1 z\u00e1sluha, k tomu pot\u0159ebujete cel\u00fd ten t\u00fdm a m\u011bla jsem to \u0161t\u011bst\u00ed v dal\u0161\u00edch odborn\u00edc\u00edch, kter\u00e9 jsem oslovila.<\/p>\n

kdybyste cht\u011bli pomoci Deb\u0159e, tak se pod\u00edvejte na jej\u00ed webov\u00e9 str\u00e1nky www.debra.cz. Pan\u00ed prim\u00e1\u0159ko, d\u00edky za v\u0161echny va\u0161e pacienty a a\u0165 se v\u00e1m da\u0159\u00ed.<\/strong><\/p>\n

ilustra\u010dn\u00ed foto: luisrock62 MorgueFile<\/p>\n<\/div>\n","protected":false},"excerpt":{"rendered":"

V Radio\u017eurn\u00e1lu hovo\u0159ila prim\u00e1\u0159ka ko\u017en\u00edho odd\u011blen\u00ed Pediatrick\u00e9 kliniky Fakultn\u00ed nemocnice v Brn\u011b Hana Bu\u010dkov\u00e1\u00a0 o nemoci mot\u00fdl\u00edch k\u0159\u00eddel a o jej\u00ed l\u00e9\u010db\u011b.<\/p>\n<\/div>","protected":false},"author":1,"featured_media":14151,"comment_status":"open","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[12],"tags":[],"class_list":["post-15009","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-rozhovory"],"_links":{"self":[{"href":"https:\/\/www.invarena.cz\/index.php?rest_route=\/wp\/v2\/posts\/15009","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=15009"}],"version-history":[{"count":0,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=\/wp\/v2\/posts\/15009\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=\/wp\/v2\/media\/14151"}],"wp:attachment":[{"href":"https:\/\/www.invarena.cz\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=15009"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=%2Fwp%2Fv2%2Fcategories&post=15009"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.invarena.cz\/index.php?rest_route=%2Fwp%2Fv2%2Ftags&post=15009"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}