Pitani ku nkhani

Epidermolysis bullosa (EB): Zizindikiro, chithandizo, ndi chisamaliro

EB ndi chiyani?

EB ndi chidule cha epidermolysis bullosa.

EB Yobadwa ndi gulu la matenda osowa komanso opweteka kwambiri akhungu omwe amachititsa kuti khungu lichite matuza ndikung'ambika mukangokhudza pang'ono. Ndi khungu losalimba ngati mapiko agulugufe, EB imatchedwa 'khungu la butterfly'.

  • Zikuganiziridwa kuti zimakhudza anthu osachepera 5,000 ku UK ndi 500,000 padziko lonse lapansi. Ziwerengerozi zitha kukhala zochulukirachulukira chifukwa nthawi zambiri zimakhala zosazindikirika. Panopa palibe mankhwala a EB.
  • Ndi chikhalidwe cha chibadwa; mumabadwa nacho ngakhale sichingawonekere mpaka mtsogolo mwa moyo.
  • Mtundu wa EB womwe muli nawo susintha pakapita nthawi ndipo EB sipatsirana kapena kupatsirana.

EB yopezeka imadziwika kuti EB acquisita ndipo ndi mtundu wosowa, wowopsa wa EB womwe umayambitsidwa ndi matenda a autoimmune.

 

nkhani;

Kodi EB imayambitsa chiyani?

Munthu aliyense ali ndi makope awiri a jini iliyonse, imodzi yochokera kwa kholo lililonse. Jini ndi gawo la DNA lomwe limayang'anira gawo la chemistry ya cell - makamaka kupanga mapuloteni. Jini lililonse limapangidwa ndi DNA, yomwe ili ndi malangizo opangira mapuloteni ofunikira, kuphatikizapo omwe amathandiza kuti khungu likhale limodzi. 

Ndi anthu omwe adatengera EB, jini yolakwika kapena yosinthika yomwe imadutsa m'banja imatanthawuza kuti madera okhudzidwa a thupi akusowa mapuloteni ofunikira omwe amamangiriza khungu pamodzi, zomwe zikutanthauza kuti khungu limatha kusweka mosavuta ndi kukangana. 

Mwana, wachinyamata, kapena wamkulu yemwe ali ndi EB angakhale adatengera jini yolakwika kuchokera kwa kholo lomwe lili ndi EB, kapena angakhale atatengera jini yolakwika kuchokera kwa makolo onse omwe ali "onyamula" koma alibe EB okha. Kusintha kwa jini kumathanso kuchitika mwangozi ngati palibe kholo lomwe limakhala lonyamula, koma jiniyo imasinthasintha yokha mu umuna kapena dzira musanatenge mimba.

Komanso kawirikawiri, mtundu woopsa wa EB ukhoza kupezeka chifukwa cha matenda a autoimmune, kumene thupi limapanga ma antibodies kuti awononge mapuloteni ake a minofu.

EB ikhoza kutengedwa ngati yolamulira, pomwe jini imodzi yokha ili yolakwika, kapena yochulukirapo, pomwe makope onse a jini ali ndi vuto. Makolo ali ndi mwayi wa 50% wopatsira mwana wawo mtundu waukulu wa EB, pamene mwayi wodutsa mawonekedwe a EB umatsika kufika 25%. Makolo onse awiri akhoza kunyamula jini popanda kudziwa ndi kusonyeza zizindikiro zilizonse. 

Jini lolakwika ndi puloteni yosowa imatha kuchitika pazigawo zosiyanasiyana pakhungu, zomwe zimapangitsa mtundu wa EB.

Kodi pali mitundu yosiyanasiyana ya EB? 

Amaganiziridwa kuti pali mitundu yopitilira 30 ya EB yotengera, yomwe ili m'magulu anayi akuluakulu omwe alembedwa pansipa. Mtundu uliwonse wa EB umadziwika molingana ndi momwe khungu limakhudzidwira ndi jini yolakwika ndi mapuloteni osowa.  

Dziwani zambiri za mitundu yosiyanasiyana ya EB pansipa.

izi ndi zofala kwambiri mtundu wa EB kuwerengera 70% ya milandu yonse. EBS zizindikiro zimasiyana in kukhwima. Ndi EBS kusowa kwa mapuloteni ndi fragility kumachitika mkati mwa pamwamba wosanjikiza wa khungu lotchedwa epidermis. 

 

Dziwani zambiri za EB simplex

Zitha kukhala zochepa kwambiri kapena kwambiri (kutengera ngati izo ziri wolamulira kapena wopondereza). The kusowa mapuloteni ndi fragility zimachitika pansi pa membrane yapansi, chomwe chiri kagawo kakang'ono kamene kamadutsa minofu yambiri ya anthu. 25% ya milandu yonse ya EB ndi Dystrophic EB. 

 

Dziwani zambiri za Dystrophic eb

Mtundu wosowa wa EB zomwe zimawerengera basi 5% ya milandu yonse. Zizindikiro za JEB zimasiyana molimba ndipo zimayambitsidwa ndi a kusowa mapuloteni pakhungu. Fkuwonongeka kumachitikas ndiin kapangidwe kamene kamasunga epidermis ndi dermis (wosanjikiza wamkati wa ziwiri zazikulu zigawo khungu) pamodzi - nembanemba chapansi.

 

Dziwani zambiri za junctional eb

KEB. A osowa kwambiri EB (osakwana 1% ya milandu). Amatchedwa chifukwa jini yolakwika kukhala ndi udindo zambiri chofunika kupanga mapuloteni a Kindlin1. Ndi KEB, fragility imatha kuchitika pamagulu angapo akhungu. 

 

Dziwani zambiri za KINDLER eb

Kodi EB imadziwika bwanji? 

EB nthawi zambiri imapezeka mwa makanda ndi ana aang'ono pamene zizindikirozo zikhoza kuwonekera kuyambira kubadwa.  

Komabe, mitundu ina ya EB, monga EBS, sangadziwike mpaka munthu wamkulu, ndipo nthawi zina sangadziwike nkomwe, popeza akatswiri azachipatala sazindikira nthawi zonse zizindikiro kapena nthawi zambiri amatha kuzizindikira ngati vuto lina lakhungu lotupa monga. psoriasis kapena atopic dermatitis (chikanga chachikulu). 

Ngati akukayikira kuti mwana wanu ali ndi EB, adzatumizidwa kwa katswiri wapakhungu (dermatologist) yemwe angayezetse (ndi chilolezo chanu) kuti adziwe mtundu wa EB ndi dongosolo lothandizira lothandizira kuthetsa zizindikirozo. Atha kutenga kachikopa kakang'ono (biopsy) kuti atumize kukayezetsa kapena kuwunika kudzera mu kuyezetsa magazi. 

Nthawi zina, pamene pali mbiri ya banja la EB, zingakhale zotheka kuyesa mwana wosabadwa kwa EB pambuyo pa sabata la 11 la mimba. 

Mayesero oyembekezera amaphatikizapo amniocentesis ndi chorionic villus sampling. Mayesowa angaperekedwe ngati inu kapena mnzanuyo mukudziwika kuti ndinu onyamula jini yolakwika kapena yowonongeka yokhudzana ndi EB ndipo pali chiopsezo chokhala ndi mwana wa EB. Awa si mayeso omwe mungafunikire kulipira ngati mwatumizidwa kuti mukhale nawo. Ndi mayeso osankha, mabanja ena amatha kusankha kuyezetsa kuti awone ngati mwana wawo wobadwayo wakhudzidwa, ena angasankhe kusatero. Nthawi zambiri, GP ndi amene amatumiza anthu, ndipo mtengo wake umalipiridwa ndi a NHS. Gulu la akatswiri azachipatala la EB lidzakambirana za mwayi wopatsirana. Mabanja angathenso kutumizidwa kukalandira uphungu wa majini ngati angafune. 

Ngati kuyezetsa kutsimikizira kuti mwana wanu adzakhala ndi EB, mudzapatsidwa uphungu ndi uphungu kudzera mwa a NHS omwe amayendetsa zipatala zinayi za EB zochitira bwino mogwirizana ndi DEBRA UK.  

Ngati inu kapena wachibale mwapezeka ndi EB posachedwa, titha kukuthandizani kuti muthandizidwe, chonde lumikizanani ndi Gulu lathu la EB Community Support Team. Mutha kupeza thandizo lazachuma kudzera pa Ntchito zoyendera za NHS  ngati mukwaniritsa zofunikirazo ndipo mukulandira phindu linalake. Kapena, mungathe lembani ku DEBRA UK kuti mupeze thandizo.

Kodi EB imakhudza bwanji thupi? 

Zizindikiro zimasiyana malinga ndi mtundu ndi kuuma kwa EB koma vuto lalikulu lomwe anthu omwe ali ndi EB amakumana nawo tsiku ndi tsiku ndi ululu ndi kuyabwa komwe kumachitika chifukwa cha matuza. M'mitundu ina ya EB kuphatikiza EBS, matuza amatha kukhala m'manja ndi kumapazi kapena kufalikira thupi lonse, komabe mumitundu yowopsa ya EB imatha kukhudza gawo lililonse la thupi kuphatikiza minyewa yamkati, yomwe imakhala yonyowa, mkati mwa ena. ziwalo ndi zibowo za thupi monga mphuno, pakamwa, mapapo, ndi m'mimba. Matuza amathanso kuchitika m'maso ndi m'ziwalo zamkati kuphatikiza pakhosi ndi kum'mero.  

Dziwani zambiri za momwe EB imakhudzira thupi pansipa.

  • kukhudza kapena kukangana kungayambitse kumeta ubweya wa khungu ndi matuza kupanga, matuza sadziletsa okha kotero amayenera kuwongolera pafupipafupi kuti asakule.  
  • kuchira kwa matuza kungayambitse kupweteka, kuyabwa kwambiri, ndi zipsera. 
  • mu mitundu ina ya EB, matuza amatha kuchitika makamaka m'manja ndi kumapazi zomwe zimayambitsa mavuto ndi kuyenda / kuyenda, ndi ntchito zina za tsiku ndi tsiku. 
  • mu mitundu yoopsa ya EB, matuza amkati monga mkati mwa pakamwa amatha kukhala ovuta kumeza ndipo pangakhale kuchepa kwa mphuno (pakhosi) ndi mpweya womwe ungafune thandizo lachipatala. 
  • kufalikira kwa matuza ndi mabala kungapangitse khungu kukhala ndi kachilombo ngati silinasamalidwe bwino. 
  • mitundu ina ya EB imatha kukhala ndi zipsera zazikulu, kusintha kwa mtundu wa khungu pakapita nthawi, komanso chiopsezo chotenga khansa yapakhungu.  
  • kupangika kwa minofu ya zipsera kungayambitse zala ndi zala kuti zigwirizane pamodzi zomwe zingafunike thandizo lachipatala. 
  • EB ikhoza kukhudza ziwalo zina mkati mwa thupi kuphatikizapo khungu kuphatikizapo mafupa ndi matumbo, zingayambitsenso zovuta zina zachipatala, kuphatikizapo kudzimbidwa, makamaka kwa ana chifukwa cha matuza pansi, komanso chifukwa ndi zotsatira za ena. mitundu ya painkiller. EB ingayambitsenso kuchepa kwa magazi m'thupi. Zotsatira za EB ndi multisystemic ndipo mumitundu yoopsa ya EB kachulukidwe ka mafupa imatha kukhudzidwa kwambiri. 

Zizindikiro ziwiri zomwe zimagwirizanitsidwa ndi EB ndi ululu ndi kuyabwa. Zizindikirozi zimachitika chifukwa cha matuza pafupipafupi komanso nthawi zina ochulukirapo omwe amatha kupezeka mthupi lonse komanso mkati chifukwa chosowa kapena ma protein achilendo omwe amayamba chifukwa cha jini yolakwika kapena yosinthika, zomwe zikutanthauza kuti khungu silimangirira pamodzi momwe liyenera kukhalira. .  

Pakalipano palibe mankhwala a EB koma pali njira zothandizira / mankhwala omwe amathandiza ndi ululu ndi kuyabwa komanso zizindikiro zina. Katswiri wanu wa zaumoyo wa EB adzatha kukulangizani mankhwala omwe ali oyenera kwa inu, koma pansipa pali mwachidule zomwe zimayambitsa ndi mankhwala a zizindikiro ziwirizi.

Pali zifukwa zambiri zovuta zomwe anthu omwe ali ndi EB amamva ululu ndikuzindikira chifukwa chake n'kofunika kuti uphungu wochepetsera ululu uperekedwe. Ngati mukumva zowawa ndipo mukufuna thandizo, akatswiri a EB azachipatala azitha kukulangizani ndikukuthandizani pakuwongolera ululu. The DEBRA EB Community Support Team imathanso kukupatsirani chithandizo chothandiza komanso chamalingaliro kuphatikiza ndalama zothandizira kulipirira zinthu zomwe zingathandize kupweteka ndi kuyabwa.  

Zomwe zimayambitsa kupweteka ndi EB ndizo: 

  • kuchiritsa matuza / matuza. 
  • madera otayika khungu ndi mabala otseguka. 
  • zilonda (malo a minofu yachilendo kapena yowonongeka) pa mucous nembanemba, yomwe ndi minofu yomwe imatulutsa ntchofu ndi mizere mabowo ndi ziwalo, zomwe zimaphatikizapo pakamwa, m'zikope, m'mimba, ndi cornea (mbali ya kutsogolo kwa diso). 
  • matenda. 
  • matuza amkati. 
  • kuvulala pakhungu ngati kupukuta kapena kuphulika. 
  • kutentha kwambiri. 
  • zifukwa zosadziwika kapena zovuta zosagwirizana ndi khungu. 
  • kugwiritsa ntchito mavalidwe olakwika kapena mankhwala apamutu.  
  • kusintha kusintha. 
  • kukhudzika ndi zinthu monga zotsukira zovala ndi zochotsera fungo. 
  • zipangizo zobvala. 

Mukadziwa chifukwa chake mukumva ululu (ngakhale pali zifukwa zosadziwika), mukhoza kugwira ntchito ndi katswiri wa zaumoyo wa EB pa ndondomeko yochepetsera ululu. M'munsimu pali malangizo ena ochepetsera ululu kwa anthu omwe ali ndi EB, komabe, zomwe zimagwira ntchito kwa munthu m'modzi sizingagwire ntchito kwa wina kotero muyenera kufunafuna malangizo kwa katswiri wa zaumoyo wa EB pazochitika zanu. 

Kuyabwa ndi kumverera kosasangalatsa komwe kumayambitsa kukanda. Kwa anthu omwe ali ndi EB, kuyabwa kungakhale kowawa kwambiri. Kukanda kumakhala kovuta kukana ndipo kungayambitsenso kuvulala kwapakhungu ndikupangitsa kuwonongeka kwa mabala omwe atsala pang'ono kuchira. Kukanda kungayambitsenso kutupa, komwe kumalimbitsanso kuyabwa. 

Zomwe zimayambitsa kuyabwa kwa odwala omwe ali ndi EB 

  • kuchiritsa matuza.  
  • khungu lowuma. 
  • kutentha kwambiri. 
  • kutupa. 
  • kuwonongeka kwa khungu kosalekeza chifukwa cha kuyambiranso kwa matuza m'dera lomwelo.  
  • ma opiates/opioids (mankhwala ochepetsa ululu) amatha kukulitsa kuyabwa. 
  • kukhudzika ndi zinthu monga zotsukira, zochotsera fungo, ndi zinthu zina zomwe zimakumana ndi khungu. 
  • kupsinjika kumatha kukulitsa kuyabwa - onani maulalo othandiza kuti mudziwe zambiri ndi zinthu zomwe zingakuthandizeni kuthana ndi nkhawa. 
  • kuperewera kwa magazi zomwe zingakhale zotsatira za EB zomwe zimabweretsa kuyabwa. 
  • osadziwika, kapena kuphatikiza zifukwa. 

Pazovuta kwambiri EB ikhoza kuwonekera kwambiri ndipo ingakhudze madera ambiri a thupi, komabe nthawi zina, mwachitsanzo EB Simplex, yomwe imakhala ndi 70% ya milandu yonse ya EB, ikhoza kukhala yocheperako komanso imakhudza madera ena okha. thupi monga mapazi. EB ingakhalenso chilema champhamvu chomwe chimatanthawuza kuti zotsatira za chikhalidwe pa munthuyo zikhoza kusintha. Mwachitsanzo, munthu m'modzi yemwe ali ndi EB sangafunike chithandizo chilichonse choyenda ndipo m'malo mwake apanga kusintha kofunikira kuti athe kuthana kapena kupewa matenda awo, komabe munthu wina yemwe ali ndi EB angafunikire chithandizo chakuyenda nthawi zina, ndipo wina akhoza kukhala nacho. kusowa pafupipafupi kwa chithandizo chakuyenda.

Mamembala athu adatiuza kuti EB ikhoza kumva ngati chilema chobisika chomwe chingapangitse zovuta zina chifukwa EB, mwa mitundu yake yonse, ikhoza kukhala yovuta kukhala ndi thupi komanso m'maganizo popanda kufunsidwa kapena kupangidwa kuti mumve ngati muyenera kufotokoza. chomwe chiri. Ichi ndichifukwa chake ndikofunikira kuti anthu ambiri adziwe ndikumvetsetsa EB.

 

Dziwani zambiri za EB ngati kulumala kobisika

Kodi pali katswiri wazachipatala wa EB? 

DEBRA UK amagwirizana ndi NHS kuti apereke chithandizo chamankhwala chowonjezereka cha EB chomwe chili chofunikira kwa anthu omwe ali ndi mitundu yonse ya EB chifukwa cholinga chake ndi kuchepetsa chiopsezo cha kuwonongeka kwa khungu ndi mavuto, ndikuwongolera zizindikiro monga kupweteka ndi kuyabwa. 

Pali malo anayi osankhidwa a EB ochita bwino ku UK omwe amapereka katswiri wa EB chithandizo chamankhwala ndi chithandizo, komanso malo ena achipatala ndi zipatala zomwe zimafuna kupereka chithandizo cha EB kwa anthu kulikonse kumene ali. Magulu opangidwa ndi DEBRA EB Community Support Managers, alangizi, EB otsogolera, anamwino, ndi akatswiri ena azachipatala apadera adzagwira ntchito limodzi kuti adziwe ndondomeko yoyendetsera zizindikiro yomwe ili yabwino kwa inu, mwana wanu, kapena munthu amene mumamusamalira.

Mabanja ena omwe ali ndi EB amatha kusankha kupereka zambiri ndi upangiri kwa achichepere kuti athe kupirira zizindikiro, makamaka ngati sanathe kupeza matenda kapena chithandizo chomwe angafunikire kudzera kwa GP wawo. Izi ndizomveka koma chonde dziwani kuti katswiri wazachipatala wa EB yemwe akupezeka kudzera ku NHS ali ndi inunso. Ntchitoyi ilipo kuti ithandizire gulu lonse la EB, anthu azaka zonse okhala ndi mitundu yonse ya EB.  

Tikukulangizani kuti mulumikizane nafe chifukwa titha kukuthandizani kuti mutumizidwe kwa akatswiri azachipatala a EB kuti mupeze upangiri waposachedwa komanso zambiri zokhudzana ndi chithandizo chapafupi. Kapena ngati mukufuna kuti musatumizidwe, titha kukuthandizani m'njira zina. Komabe, kuwonetsetsa kuti mukudziwika ndi ntchito zapadera ndikofunikira chifukwa zimathandizira kupititsa patsogolo chithandizo cha EB. Tili ndi template ya kalata yotumizira yomwe mungagwiritse ntchito ngati ikufunika kuti mupereke kwa GP wanu popempha kuti akutumizireni. Chonde tiuzeni kuti mudziwe zambiri.

Kuti mupeze chithandizo chamankhwala cha EB kudzera mu NHS nthawi zambiri pamafunika kutumiza. Ngati mukuganiza kuti muli ndi mtundu wa EB, mukhoza kupita kwa GP wanu, ngati akukayikira kuti muli ndi EB, akhoza kukulozerani ku malo amodzi a EB kumene katswiri wa khungu (dermatologist) angatenge biopsy kuti atumize. kuyezetsa kapena kuyezetsa magazi ndipo kamodzi pansi pa gulu la akatswiri adzagwira ntchito nanu kuti mupereke chisamaliro chabwino kwa mwana wanu. 

Kuti muthandize dokotala wanu kudziwa ngati muli ndi EB kapena ayi, ndikuwonetsetsa kuti akutumizani ku chipatala choyenera cha EB, Gulu lathu la EB Community Support Team lingakupatseni kalata yomwe mungagawane ndi GP wanu. Kuti ndipemphe imodzi chonde Lumikizanani nafe.

Mukapezeka ndi EB, chonde lembani ku kukhala membala wa DEBRA UK kotero kuti mutha kupindula ndi zambiri zaulere, zothandizira, ndi chithandizo chomwe timapereka kwa aliyense ku UK yemwe akukhala kapena kukhudzidwa mwachindunji ndi EB.

Mutha kupeza zidziwitso zamalo anayi azachipatala a EB omwe alembedwa pansipa komanso ndi zipatala zina komwe akatswiri a EB ali ilipo. Ngati mungafune thandizo kulumikizana ndi gulu lachipatala la EB kapena ngati muli ndi mafunso okhudza chithandizo chamankhwala cha EB, lemberani.

Magulu apadera a zaumoyo a EB a ana ndi achinyamata amakhala ku Birmingham Women's and Children's Hospital, Great Ormond Street Hospital ku London, ndi Glasgow Royal Hospital for Children.  

 

Chipatala cha Amayi ndi Ana cha Birmingham 

Zambiri pa bwanji kupita kuchipatala.

Mauthenga othandizira: 

  • Imbani - 0121 333 8757 kapena 0121 333 8224 (tchulani kuti mwanayo ali ndi EB) 
  • Imelo – eb.team@nhs.net 

 

Chipatala chachikulu cha Ormond Street 

Zambiri pa bwanji kupita kuchipatala.

Mauthenga othandizira: 

  • Imbani - 0207 829 7808 (gulu la EB) kapena 0207 405 9200 (switchboard yayikulu) 
  • Imelo - eb.nurses@gosh.nhs.uk  

 

Glasgow Royal Hospital for Children 

Zambiri pa bwanji kupita kuchipatala.

Mauthenga othandizira: 

 

Sharon Fisher - EB Pediatric Clinical Namwino 

 

Kirsty Walker - Namwino wa Dermatology 

 

Dr Catherine Drury - Wothandizira Dermatology  

  • Imbani - 0141 451 6596  

 

Main switchboard 

  • Imbani - 0141 201 0000  

Magulu apadera a zaumoyo a EB a akuluakulu ali ku Solihull Hospital, Guys & St.Thomas' Hospital ku London, ndi Glasgow Royal Infirmary.

 

Chipatala cha Solihull 

Zambiri za momwe mungapitire kuchipatala

Mauthenga othandizira: 

  • Imbani - 0121 424 5232 kapena 0121 424 2000 (switchboard yayikulu)  

 

Guys & St. Thomas' Hospital  

Gulu la achikulire la EB lachipatala ku Guys & St.Thomas' Hospital lili mkati mwa Rare Diseases Center: 

Rare Diseases Center, 1st floor, South Wing, Chipatala cha St Thomas, Westminster Bridge Road, London, SE1 7EH 

Zambiri za momwe mungapitire kuchipatala

 

Mauthenga othandizira: 

 

Glasgow Royal Chipatala 

Zambiri pa bwanji kupita kuchipatala

Mauthenga othandizira: 

Maria Avarl - Katswiri wa Namwino Wachikulire wa EB 

 

Dr Catherine Drury - Wothandizira Dermatology  

  • Imbani - 0141 201 6454  

 

Susan Herron - Wothandizira EB Business Support  

  • Imbani - 0141 201 6447  

 

Switchboard (A&E) 

  • Imbani - 0141 414 6528 

Kodi EB imathandizidwa bwanji? 

Palibe machiritso a EB, koma pali mankhwala omwe akupezeka omwe cholinga chake ndi kukonza moyo wabwino pothana ndi zizindikiro zake bwino momwe angathere. 

Mu gawoli mupeza zambiri ndi upangiri wokhuza machiritso ndi kasamalidwe ka zizindikiro za EB.  

Kwa anthu ambiri omwe ali ndi EB, kapena kusamalira munthu yemwe ali ndi EB, chisamaliro cha bala ndi gawo lalikulu la moyo watsiku ndi tsiku. Kudziwa momwe mungasamalire matuza ndi mitundu yosiyanasiyana ya mabala, kuchiza ululu ndi kuyabwa, kupewa, ndi kuchiza matenda komanso nthawi yofunsira upangiri wamankhwala ndizofunikira pakusamalira mabala a EB. 

Pali chithandizo chosiyanasiyana chothandizira anthu ndi mabanja kuthana ndi zovuta zakukhala ndi EB kuphatikiza EB malo azaumoyo abwino kwambiri kumene odwala okhala m’maiko anayiwo angatumizidwe kukalandira chithandizo chamankhwala nthaŵi zonse. Akatswiri azachipatala a EB m'malo awa, ena omwe amathandizidwa ndi DEBRA UK, ndi odziwa zambiri komanso odziwa bwino momwe angasamalire khungu, kuphatikiza momwe angatulutsire matuza ndi mankhwala omwe amapezeka kuti athetse zizindikiro. Mutha kupempha kuti mutumizidwe imodzi mwa malo kudzera kwa GP wanu. Ngati dokotala wanu sakudziwa zoti akulozeni kapena simukudziwa chomwe mungapemphe, chonde lumikizanani ndi Gulu lathu la EB Community Support Team amene angakuthandizeni ndipo akhoza kukupatsani template ya kalata kuti mugawane ndi GP wanu. 

M'munsimu mungapeze zambiri zokuthandizani kusamalira matuza ndi kuchepetsa kuwonongeka kwa khungu, komanso maulalo azinthu zina zothandiza. 

Mbali yofunikira ya dongosolo lililonse lamankhwala kwa anthu omwe ali ndi EB ndikupewa kuvulala kapena kukangana kwa khungu kuti muchepetse kuchuluka kwa matuza motero kuchepetsa ululu, kuyabwa, ndi zipsera. Zomwe aliyense amakumana nazo ndi EB ndizosiyana pang'ono ndipo upangiri umasiyana malinga ndi kuuma ndi mtundu wa EB. Chifukwa chake tikulimbikitsidwa kuti mupeze chithandizo kuchokera kwa katswiri wazachipatala wa EB pazochitika zanu koma monga chitsogozo ndikulimbikitsidwa kuti: 

  • chepetsani kuyenda mtunda wautali ndikuyesera kupulumutsa khungu/mapazi anu pamaulendo ofunikira ngati kuli kotheka. 
  • yesetsani kupewa misozi ndi kukanda ndikupewa kupukuta khungu - makolo angafunikire kusintha momwe amanyamulira makanda ndi ana. 
  • yesetsani kupeza zovala zabwino zomwe sizikupaka pakhungu ndi komwe mungathe, pewani misomali yambiri, ngati n'kotheka, valani ulusi wosalala wachilengedwe monga silika, nsungwi ndi thonje chifukwa izi zingathandize kuchepetsa kupsa mtima. 
  • sungani khungu kuti likhale lozizira momwe mungathere. 
  • sankhani nsapato zabwino zomwe zilibe zolimba mkati. Dziwani zambiri mu kalozera wa nsapato.
  • gwiritsani ntchito zithandizo zilizonse ndi zosinthika zomwe gulu lanu lazaumoyo litha kukhala njira zosavuta monga ma insoles kapena chopondapo, kapena zothandizira kuyenda monga chikuku kapena njanji zogwirizira m'bafa. Nthawi zonse funsani katswiri wanu wa EB chifukwa zida zina kapena zothandizira kuyenda sizingakhale zoyenera kwa EB yanu. 
  • funsani ena kuti aziganizira zosowa zanu. 

Anthu omwe amakhala ndi EB nthawi zambiri amafotokoza zowawa za kuwonongeka kwa khungu lawo ngati kutentha kwa digiri yachitatu, ndipo nthawi zina, pangakhale kuwonongeka kwakukulu kwa khungu pamadera akuluakulu. Chisamaliro chapadera chimafunika kuchepetsa ululu, kuyabwa ndi zizindikiro zina zokhudzana ndi matuza. Ngati simukudziwa momwe mungasamalire khungu lanu, kapena la munthu amene mukumusamalira, nthawi zonse tchulani katswiri. Mutha kupeza zolumikizana ndi akatswiri komanso chochita pakagwa mwadzidzidzi Pano.

The DEBRA EB Community Support Team angaperekenso uphungu wothandiza kuphatikizapo kudziwa ufulu wanu ndi udindo wa opereka maphunziro ndi olemba ntchito kuti atsimikizire kuti mpumulo wa ululu ukhoza kuperekedwa panthawi yoyenera. Kulankhula ndi omwe ali pafupi nanu za EB kungakuthandizeninso kuthana ndi zovuta zakuthupi ndi zamaganizidwe zomwe zimakhala ndi EB.  

Kuti muthe kuthana ndi zizindikiro za EB mudzafunika zinthu zina ndi zinthu zina. Zomwe mukufunikira zimadalira mtundu ndi kuopsa kwa EB yanu, ndipo katswiri wa zaumoyo wa EB adzatha kukulangizani koma pansipa pali chisonyezero cha zipangizo ndi zinthu zomwe mungafunike: 

  • lumo. Malumo akuthwa adzafunika kudula ndi kudula mabandeji, lumo lokhazikika limagwiranso ntchito podula mavalidwe. Onetsetsani kuti mwatsuka ndi kuyeretsa lumo mukatha kugwiritsa ntchito. 
  • Zovala zamabala. Pali mitundu yosiyanasiyana ya zovala zomwe zilipo zamitundu yosiyanasiyana ya EB ndipo katswiri wanu wa zaumoyo wa EB adzatha kukulangizani za zovala zoyenera kwambiri kwa inu kapena munthu amene mumamusamalira. Nthawi zonse ndikofunika kugwiritsa ntchito zovala zosamata zomwe sizimamatira pakhungu kuti muchepetse kuwonongeka kwina. 
  • Mabandeji. Ma bandeji angafunike kuonetsetsa kuti zovalazo zizikhalabe bwino chifukwa zovala zikaterereka zimatha kung’amba khungu losalimba kapena kupangitsa mabala kumamatira ku zovala kapena pa zofunda. Bandeji yosungira imatha kuthandizira kuonetsetsa kuti zovalazo zizikhala bwino.  
  • Moisturizers. Kuyabwa kungakhale vuto lalikulu mumitundu yonse ya EB. Pamene mabala akuchira, kapena matenda akamakula, kuyabwa kumatha kukhala kovuta koma kusunga khungu lonyowa kungathandizedi. 
  • Antimicrobial oyeretsa. Pali chiopsezo chotenga matenda ndi mitundu yonse ya EB chifukwa cha nthawi zambiri madera akuluakulu a mabala otseguka ndipo motero oyeretsa antimicrobial, moisturisers ndi mankhwala apamutu nthawi zambiri amafunika kuchepetsa ngoziyi. Chithandizo chapamutu ndi mankhwala omwe amagwiritsidwa ntchito pamalo enaake pathupi ndi cholinga chake ndikuchiza minyewa yomwe idagwiritsidwa ntchito popanda zotsatira zazikulu pamasamba ena. 

Pali akatswiri othandizira omwe amatha kubweretsa zinthu ngati izi kunyumba kwanu, komanso ma pharmacies ambiri omwe amapereka chithandizo chamankhwala kotero fufuzani ndi malo omwe muli nawo. Mmodzi mwa opereka awa ndi Bullen Healthcare omwe ali ndi chidziwitso chothandizira EB Community. Nthawi zonse amakhala ndi katundu wambiri komanso katundu omwe amaperekedwa kwa anthu omwe ali ndi EB ndipo apanga gulu lodzipereka kuti lithandizire pa mafunso ndi maoda onse a EB. Kuti mudziwe zambiri zokhudza kupeza chithandizo chamankhwala ndikupeza mankhwala oyenera, chonde lumikizanani ndi gulu la DEBRA EB Community Support Team.

Ngakhale mutha kuchepetsa chiwopsezo cha kuvulala pakhungu, matuza ndi osapeweka ndipo nthawi zina amangochitika zokha, amawonekera popanda chifukwa chilichonse. Matuza nawonso sadziletsa okha ndipo amatha kukula ngati atasiyidwa okha. Matuza akulu = mabala akulu, kotero kuyang'anira matuza ndi gawo lofunikira lachizoloŵezi chanu cha chisamaliro cha khungu ndikuwadula mwachangu ndikofunikira. Chonde onani gawo lazothandizira (LUMIKIZANI KWA 'ZOTHANDIZA') kuti mudziwe zambiri za chisamaliro cha khungu. 

Cholinga chake ndi kuteteza kuti chithuza chisakule potulutsa madzi aliwonse, ndikusiya kutsegula kwakukulu kokwanira kuti chithuzacho chisatseke ndi kupanganso, ndikuteteza khungu lamkati. 

M'munsimu muli malangizo okuthandizani kusamalira matuza anu:  

  1. Kuthamanga kapena 'kutuluka' matuza okhala ndi singano ya hypodermic kuti madzi a chithuza atha. Izi zimalepheretsa chithuza kuti chisakule ndikupanga malo ochulukirapo akhungu lowonongeka. 
  2. Gwiritsani ntchito singano yosabala - kukula kwake ndikofunika kotero lankhulani ndi wothandizira zaumoyo wanu kuti muwonetsetse kuti mukugwiritsa ntchito kukula koyenera. Mutha kufunsa GP kapena EB katswiri wazachipatala kuti akupatseni singano zosabala. Chonde dziwani kuti mudzafunika bokosi lakuthwa ndi ntchito yotolera kuti mutaya singano. Zokhudza kutaya singano.
  3. Lance pamunsi kwambiri pa chithuza kuti mphamvu yokoka ithandizire kukhetsa madzi. 
  4. Panikizani pang'onopang'ono ndi chopyapyala kapena nsalu yoyera kuti muchepetse madzi - anthu ena amakonda kugwiritsa ntchito syringe yoyera kuchotsa madzimadzi. 
  5. Siyani denga la chithuza lolimba kuti muteteze khungu lakuda pansi ndikuchepetsa kuthekera kwa matenda. 
  6. Chotsani khungu lakufa kapena zinyalala kuzungulira chithuza, ndikusiya denga la chithuzalo liri bwino - dab osapaka kuti muchepetse kuwonongeka kwa khungu. 
  7. Ngati mbali ina ya khungu laiwisi pansi yasiyidwa ngati bala lotseguka, mungafunike kuvala malowo pogwiritsa ntchito mavalidwe osakhala ndi ndodo molangizidwa ndi dokotala wanu. Osagwiritsa ntchito pulasitala wamba wamba chifukwa izi zitha kuwononga kwambiri khungu. Ngati pulasitala womata agwiritsidwa ntchito molakwika, pali zinthu zochotsa zomatira kuphatikiza zopopera ndi zopukuta zomwe zingathandize kuchepetsa kuwonongeka kulikonse pakhungu, izi zitha kupezeka kwa inu pamankhwala kudzera kwa GP wanu kapena kudzera m'ma pharmacies. Zingakhalenso zothandiza kupereka mankhwalawa ku sukulu ya mwana wanu, wothandizira ana, kapena kunyamula kuti mugwiritse ntchito pa nthawi yokumana, mwachitsanzo popereka magazi. 
  8. Zingakhale zothandiza kuti chilonda chikhale chonyowa chifukwa kuuma kungapangitse kuyabwa kwambiri. Pali ma creams omwe amathandizira pa izi. 

Gulu lanu lazaumoyo lidzakuthandizani kukulangizani za chisamaliro cha matuza ndipo lingakulimbikitseni kuvala ndi mankhwala omwe ali oyenera khungu lanu ndi mtundu wa EB. 

Matuza amathanso kuwonekera mkati - m'kamwa, m'dera lakumbuyo, ndi zina za mucous (mphuno, pakamwa, m'mimba m'mapapo), zomwe zingakhale zokhumudwitsa. Mapuloteni omwe akusowa omwe amapatsa khungu mphamvu zake amawonekera m'magulu osiyanasiyana m'thupi, kuphatikizapo nembanemba yomwe imaphimba diso ndi minofu yomwe ili m'kamwa ndi kum'mero. Chonde lankhulani ndi gulu lanu lazaumoyo la EB kuti akuthandizireni momwe mungachitire matuza amtunduwu. 

Nthawi zambiri, chithuza chikang'ambika chimachira ndipo sichidzapwetekanso, komabe anthu ena amatha kumva ululu ndi kuyabwa popanda matuza.  

Khungu lomwe lili ndi matuza likhoza kukhala losalimba kwambiri, makamaka potsatira kubweranso kwa matuza pamalo omwewo. 

Nthawi zina chilonda sichipola, kapena kuchira koma kenako chimasweka, zomwe zimakhala zowawa, ndipo zimatha kupangitsa kuti chilondacho chitengeke mosavuta ndi matenda. Izi zimatchedwa chilonda chosatha. Zikatere, lankhulani ndi katswiri wa zachipatala wa EB kuti adziwe chifukwa chake bala silipola kuti athe kukuthandizani, mwachitsanzo, pokupatsani malingaliro amtundu wina wamavalidwe, kugwiritsa ntchito zonona, kapena kuvala ndi anti-fungal/anti-bacterial. katundu, kapena popereka mankhwala kuti athetse matenda. Palinso zinthu zina zomwe zingakhudze momwe mabala anu amachiritsira monga zakudya, kugona komanso kuchepetsa nkhawa. Chonde funsani katswiri wazachipatala wa EB kapena a DEBRA EB Community Support Team kwa chithandizo chabwino. 

Kusintha kwa kavalidwe pafupipafupi komwe kumafunikira kuti muzitha kuyang'anira EB kumatha kukhala kovutitsa komanso kowawa kwambiri, komabe ndi gawo la moyo wonse komanso lofunikira pakusamalira khungu tsiku ndi tsiku, kuwongolera mabala ndi matuza. 

Matuza ayenera kudulidwa mwachangu momwe angathere kuti asabweretse ululu komanso kuwonongeka. 

Nthawi yomwe imatengedwa kuti mutsirize kusintha kwa mavalidwe imatha kusiyana kwambiri, koma kuti muchepetse ululu ndikulangizidwa kuti mumalize kusintha kavalidwe munthawi yochepa kwambiri. Pali mavalidwe osiyanasiyana omwe alipo, ndipo kuonetsetsa kuti mwavala moyenera ndikofunikira, mwachitsanzo, ana obadwa kumene omwe akhudzidwa kwambiri amafunikira kusamalidwa pang'ono ndipo pangafunike kuvala komwe kumatha masiku angapo.  

Zovala zosamata ndizofunikira kuti muchepetse kuwonongeka ndi kupweteka kwina. Ngati zomatira zagwiritsidwa ntchito molakwika, pali mankhwala ochotsa zomatira omwe akupezeka kwa inu pamankhwala kudzera kwa GP wanu kapena kudzera m'ma pharmacies. Zovala zokhala ndi silika nthawi zambiri zimakhala zosavuta kuziyika ndikuchotsa kusiyana ndi zovala zachikhalidwe, zomata. Gulu lanu la akatswiri azachipatala la EB lidzakhala loyenera kulangiza. 

Magulu odziwa zachipatala a EB omwe ali m'malo opangira chithandizo chamankhwala a EB ali ndi ukadaulo wambiri pakuwongolera mabala ndipo azitha kukulangizani za dongosolo loyenera la chithandizo chanu. Chonde funsani katswiri wa zaumoyo wa EB kapena ngati mulibe mwayi wopeza katswiri, gulu la DEBRA EB Community Support Team lingakuthandizeni potumiza. 

Zingakhale zovuta kwambiri kuthana ndi ululu wokhudzana ndi zilonda ndi kuphulika kwa khungu chifukwa cha EB komabe pali njira zosiyanasiyana zochepetsera ululu malingana ndi kuopsa kwa zizindikiro zomwe zimakhudzidwa ndi ululu wokhudzana ndi ululu, izi zimaphatikizapo zonona, ma gels, ndi pakamwa. mankhwala. 

Kwa mitundu ina ya EB, monga EBS, mankhwala ochepetsa ululu angapereke mpumulo koma chonde dziwani kuti ana osapitirira zaka 16 sayenera kupatsidwa aspirin chifukwa pali chiopsezo chochepa chomwe chingayambitse matenda aakulu otchedwa Reye's Syndrome. , izi zikulangizidwa ndi NHS. 

Njira zamphamvu zothanirana ndi ululu zitha kupezekanso kudzera mwa katswiri wanu wamankhwala a EB pamankhwala omwe akuphatikizapo morphine, omwe amagwiritsidwa ntchito nthawi zambiri asanasinthe mavalidwe, ndi njira yapakamwa ya sucrose ya makanda, pomwe zotsekemera zotsekemera (oral sucrose) zimayikidwa pa lilime. kuchepetsa kupweteka kwa ndondomeko. Izi zasonyezedwa kuti n’zopindulitsa makanda obadwa kumene asanayambe komanso akamayamba. 

Chonde kambiranani za kugwiritsa ntchito mankhwala opha ululu, ngakhale omwe ali m'kauntala ndi dokotala wanu wa EB. 

Kuchepetsa nthawi yosintha mavalidwe, mwachitsanzo pogwiritsa ntchito ma templates kuti mudule zovala pasadakhale kumachepetsa nthawi yomwe munthu amakumana ndi zovuta komanso kumathandiza kuchepetsa ululu.  

Anthu ena okhala ndi EB amapeza kuti kuchita zinthu zomwe amasangalala nazo, monga kumvetsera nyimbo, kukhala ndi nthawi yocheza ndi ena, kutuluka panja, kusewera masewera, kapena kuonera TV kumapereka zosokoneza zothandiza. Kugwiritsa ntchito malingaliro ndi njira zopumira pakati pa njira zina zaumoyo zingathandizenso. Magulu a zaumoyo ku malo odziwa bwino a EB ali ndi chidziwitso chochuluka pa kayendetsedwe ka ululu ndipo akhoza kukuthandizani, ngakhale kupweteka kwanu kumakhala kochepa kapena koopsa, ndi njira zothandizira kupweteka kapena ndondomeko ya mavuto. 

Maulalo othandiza gawoli lili ndi maulalo ku mabungwe omwe amapereka chithandizo panjira zowongolera ululu.  

Mukhozanso kupeza malangizo ndi malangizo za Kusamalira ululu wosaneneka patsamba la NHS.

Mabala otseguka kapena khungu laiwisi limatha kutenga kachilombo komwe kumafunikira chithandizo chachangu kuti mupewe kupweteka komanso kuwonongeka. Matenda ambiri amatha kupewedwa posamba m'manja bwino komanso zida zoyera ndizofunikira potupa matuza ndikusintha mavalidwe. 

Zotsatirazi zingasonyeze kukhalapo kwa matenda. 

Ngati mukukumana ndi zizindikiro izi kapena mukudandaula kuti mabala anu akhoza kutenga kachilomboka, muyenera kuwonana ndi dokotala wanu kapena wothandizira zaumoyo wanu. 

  • kufiira ndi kutentha kuzungulira dera la khungu. 
  • malo omwe khungu likutuluka mafinya kapena kutuluka kwamadzi. 
  • kutundika pamwamba pa bala. 
  • bala losapola. 
  • mzere wofiira kapena mzere wofalikira kutali ndi chithuza, kapena kusonkhanitsa kwa matuza (zingakhale zovuta kuziwona pakhungu lakuda kapena lofiirira). 
  • kutentha kwakukulu (kutentha thupi) kwa 38C (100.4F) kapena pamwamba. 
  • fungo losazolowereka. 
  • kuchuluka ululu. 

Mukangoyamba kuona ngati muli ndi matenda, chonde funsani dokotala wanu kapena wopereka chithandizo chamankhwala kuti akupatseni chithandizo choyenera chomwe chingaphatikizepo mankhwala opha tizilombo toyambitsa matenda, maantibayotiki, ma gelisi, kapena mavalidwe apadera. 

Kuti mukhale ndi chithandizo chanthawi yayitali komanso kuthandizira kuchira kwa chilonda mutha kulimbikitsa chitetezo chanu chamthupi pogwiritsa ntchito zakudya ndi zakudya zowonjezera. Lankhulani ndi katswiri wazachipatala wa EB kuti mukambirane zazakudya zabwino kwambiri kwa inu, nanunso pitani ku gawo lathu lazakudya ndi zakudya maphikidwe okoma ochokera kwa akatswiri azakudya ndi mamembala athu a EB, okhala ndi zosakaniza zathanzi kuti apereke mapuloteni ambiri, opatsa thanzi komanso, pamitundu ina ya EB, zakudya zama calorie ambiri, ma puddings, kapena zokhwasula-khwasula.

Kusamalira bwino khungu ndikofunikira kuti muchepetse kuyabwa. Ngakhale kuti chilakolako chofuna kukanda chimakhala chovuta kuchikana, anthu ena amapeza kuti akusisita ndi nsalu yonyowa pang'onopang'ono, kapena kusamba madzi ozizira kumapereka mpumulo. Ngati kuopsa kwa kuyabwa kukufunika, pali mankhwala omwe amapezeka monga mafuta odzola komanso odzola kuti achepetse kuyabwa. 

Kuonetsetsa kuti muli ndi hydrated, kupewa kutenthedwa, komanso kukumbukira zinthu zomwe zimagwirizana ndi khungu lanu, zidzakuthandizaninso. 

Njira zopumula, kupuma ndi kulingalira zingaperekenso mpumulo, nthawi zambiri kuphatikizapo chithandizo chamankhwala ndi makhalidwe ena. Mayankho osiyanasiyana amagwira ntchito kwa anthu osiyanasiyana kotero ndi bwino kukambirana zosowa zanu payekha ndi katswiri wa zaumoyo wa EB. 

Zambiri ndi malangizo amomwe mungachitire ndi kuyabwa khungu.

Mabala a EB ndi matuza amatha kuchira ndi chipsera. Zilonda ndi mbali ya machiritso achilengedwe a thupi pamene minofu yawonongeka ndipo ikhoza kukhala yofatsa, yowonekera, ndi yanthawi yochepa, kapena yowonjezereka komanso yosatha. Nthawi zambiri, pamene zipsera zimachuluka, malowo amakhala osalimba kwambiri. Kupaka malo omwe ali pachiwopsezo pakhungu kungathandize kuchepetsa ndikuchepetsa kuwonongeka kwina. 

Kupweteka kwakukulu kungayambitse zovuta zomwe zingafunike opaleshoni, katswiri wanu wa zachipatala wa EB adzakambirana nanu njira zothandizira zomwe zilipo. 

Ngati muli ndi nkhawa zokhuza mabala kapena mukufuna thandizo, magulu azachipatala a EB ali ndi zokumana nazo zambiri mderali ndipo atha kukambirana zakukhosi kwanu ndi chithandizo chilichonse chomwe mungafune. 

Kupsinjika maganizo ndi kusowa tulo kungawononge zotsatira machiritso a chilonda komanso kuthekera kothana ndi ululu. Komabe, zizindikilozi zitha kuwongoleredwa pogwiritsa ntchito njira zowongolera kupsinjika, zopatsa thanzi, mankhwala, kusinkhasinkha, kulingalira, ndi njira zina zaumoyo. Gulu lanu lachipatala la EB lizitha kukuthandizani kuti mupeze chithandizo choyenera, mutha kupezanso Zina zothandizira pano Kapena pitani Pano Kuti mudziwe zambiri. 

Kukhala ndi EB kungakhale kovuta, koma DEBRA EB Community Support Team ili pano kwa aliyense amene akukhala kapena kukhudzidwa mwachindunji ndi mtundu uliwonse wa EB wotengera ndi wopeza. Gulu likhoza kupereka chidziwitso chamalingaliro, chothandiza, komanso chandalama ndi chithandizo pagawo lililonse la moyo. 

Kujowina DEBRA UK ngati membala ndi zaulere, ndipo umembala umakupatsani mwayi wopeza ntchito zonse zoperekedwa ndi Gulu Lothandizira la Gulu la DEBRA EB, kuphatikiza maubwino ena kuphatikiza zochitika zaposachedwa, pomwe mutha kulumikizana ndi mamembala ammudzi wa EB panokha kapena pa intaneti, tchuthi chotsika. kupumula, kulengeza, komanso chidziwitso chazachuma, chithandizo, ndi thandizo. 

Umembala umakupatsaninso mawu komanso mwayi wopanga zomwe timachita; ntchito zofufuza zomwe timayikamo, ndi ntchito zomwe timapereka kwa gulu lonse la EB. Komanso kungolowa nawo ngati membala mupanga kusintha chifukwa tikamakhala ndi mamembala ambiri, timakhala ndi data yambiri, zomwe ndizofunikira kwambiri pothandizira pulogalamu ya kafukufuku ya EB, ndipo mamembala ambiri amatipatsa mawu okweza kuti tithandizire kukopa boma, NHS, ndi mabungwe ena kuti athandizidwe kuti apititse patsogolo ntchito zopindulitsa gulu lonse la EB. 

Epidermolysis bullosa acquisita (EBA) 

Epidermolysis bullosa acquisita (EBA) ndi mtundu wosowa kwambiri wa EB ndipo umatchulidwa ngati matenda a autoimmune, pomwe chitetezo chamthupi chimayamba kuukira minofu yathanzi yathupi. Sizikudziwika chomwe chimayambitsa izi. 

EBA imayambitsa kufooka kwa khungu monga mitundu ina ya EB koma mitundu inayi ikuluikulu ya EB Ndi ma genetic omwe amayamba chifukwa cha zolakwika kapena zosinthika, EBA ndi mtundu wopezedwa wa EB. 

Mofanana ndi mitundu ina ya EB, EBA imatha kukhudzanso pakamwa, pakhosi, ndi m'mimba. Komabe, mosiyana ndi mitundu ina ya EB, zizindikiro za EBA sizimawonekera pakapita nthawi; nthawi zambiri zimakhudza anthu azaka zopitilira 40. 

Zomwe zimayambitsa EBA sizidziwika koma zimaganiziridwa kuti mapuloteni oteteza thupi (mapuloteni m'thupi omwe ali mbali ya chitetezo cha mthupi) amaukira molakwika collagen wathanzi - mapuloteni a khungu omwe amamanga khungu pamodzi. Chifukwa chake, thupi limayamba kuwononga minofu yake yathanzi ndipo izi zimayambitsa matuza akhungu ndi minyewa yamkati mwa ziwalozo.   

EBA imakonda kukhala yofala kwambiri mwa anthu omwe ali ndi matenda ena a autoimmune monga Crohn's ndi Lupus. 

Ofufuza apeza mitundu itatu ya EBA:   

  • Generalized yotupa EBA- kufalikira kwa matuza, kuyabwa, kuyabwa, kuchira ndi zipsera zochepa.  
  • Kutupa kwa mucous membrane wa EBA- matuza a mucous nembanemba (gawo la thupi lokhala ndi nembanemba monga pakamwa, pakhosi, maso, ndi m'mimba) komwe kungathe kukhala ndi zipsera zazikulu.  
  • Classic kapena non-inflammatory EBA- kuchititsa matuza pakhungu makamaka m'manja, m'mawondo, m'miyendo, m'zigongono, mu akakolo, ndi m'mitsempha. Zipsera zimatha kuchitika kapena mawanga oyera (milia) amatha kupanga.  

Zizindikiro za EBA zitha kukhala ngati zizindikilo za mitundu ina ya EB ndipo zimatha kukhala zolimba kuyambira pang'ono mpaka pang'ono. Zizindikiro za Commons zingaphatikizepo matuza m'manja, mawondo, ma knuckles, elbows, ndi akakolo.  

Zotsatira za kukhala ndi EBA zimatsimikiziridwa kwambiri ndi zovuta zilizonse zokhudzana ndi thanzi komanso momwe zimakhalira ndi mitundu ina ya EB, osiyanasiyanamankhwala zilipo kuti zithandize kuchepetsa zizindikiro. 

Mofanana ndi EB, panopa palibe mankhwala a EBA koma pali mankhwala ochepetsera zizindikiro monga kupweteka ndi kuyabwa. 

Kuwonetsetsa kusamalidwa koyenera komanso kukhala ndi zakudya zopatsa thanzi ndikofunikira kwambiri kuti muchepetse zovuta zomwe zingachitike. 

EBA ingathenso kuchiritsidwa pogwiritsa ntchito mankhwala oletsa chitetezo cha mthupi, omwe amapangidwa kuti aletse kapena kuchepetsa mphamvu ya chitetezo cha mthupi, komanso anti-inflammatory agents.  

Palinso zitsanzo za chithandizo chamankhwala chomwe chimati chinachita bwino kuchepetsa zizindikiro za EBA.

Madokotala amatha kutumiza odwala omwe ali ndi EBA kwa dermatologist kapena chipatala cha autoimmune kuti adziwe njira yoyenera yamankhwala. 

Ngati inu, wachibale wanu, kapena wina amene mumamusamalira wapezeka ndi EBA, mutha kulumikizananso ndi a DEBRA EB Community Support Team kwa chithandizo chowonjezera. Gulu lathu lili pano kuti lithandizire gulu lonse la EB ku UK, kuphatikiza anthu okhala kapena

Chani ndichite ngati Ndikuganiza kuti ine ndi EB?

Ngati mukukayikira kuti muli ndi mtundu uliwonse wa EB, mutha kukaonana ndi GP wakudera lanu, ngati akuganizanso kuti mutha kukhala ndi mawonekedwe a EB ndiye akulozerani kwa mmodzi wa EB akatswiri malo. Gulu lachipatala ku EB center lidzazindikira momwe khungu lanu lilili ndipo adzakonza (ndi chilolezo chanu) kuti ayese chibadwa kuti atsimikizire ngati muli ndi mtundu uliwonse wa EB. Ngati EB yatsimikiziridwa, gulu lachipatala la EB lidzagwira ntchito nanu anatsimikiza dongosolo laumoyo. Mukhozanso kupeza thandizo kuchokera ku DEBRA EB Community Support Team.