Kodi khungu lanu likutupa ndipo likutuluka ngakhale mutakhudza pang'ono? Kodi ndi Epidermolysis Bullosa (EB)?

Nthawi zina khungu lathu limakhala lofewa kwambiri, sichoncho? Ngakhale chinthu chaching'ono chikagunda, kapena chovala chikang'ambika, anthu ena amadwala matuza ndi kuvulala pakhungu mosavuta. Makamaka popeza khungu la makanda aang'ono ndi lofewa kwambiri, zimawawa kwambiri mukaona zinthu zotere. Kotero lero tikambirana za matenda omwe amapezeka pakhungu lotere, omwe ndi osowa pang'ono koma ofunikira kwambiri kudziwa. Umenewo ndi epidermolysis bullosa, kapena EB mwachidule.

Kodi epidermolysis bullosa (EB) ndi chiyani kwenikweni?

Mwachidule, epidermolysis bullosa (EB) ndi matenda obadwa nawo . Amapangitsa kuti khungu lanu likhale lofooka kwambiri komanso lotupa komanso losauka mosavuta. Nthawi zina, ngakhale mutapaka khungu lanu ndi zovala kapena kuvulala kwinakwake, mutha kukhala ndi matuza ndi zilonda. Kwa anthu ena, zitha kukhala zofewa ndipo zimangoyambitsa matuza opweteka m'manja, m'zigongono, m'mawondo, ndi m'mapazi. Komabe, pazochitika zazikulu, matuza ndi zilondazi zimatha kuwoneka kulikonse pathupi. Zizindikiro za EB zimatha kuyambira zofatsa mpaka zowopsa kwambiri .

Kumbukirani, nthawi zina matuza awa amatha kumera mkamwa, pakhosi (m'mero), m'maso, ndi m'ziwalo zina zamkati mwa thupi. Matuza awa akachira, amatha kusiya mabala opweteka. Pa milandu yoopsa, matuza ndi mabala awa amatha kuwononga ziwalo zamkati mwa thupi, zomwe zingakhale zoopsa kwa moyo.

Kodi mitundu ikuluikulu ya EB ndi iti?

Pali mitundu inayi ikuluikulu ya EB, yomwe imadziwika ndi khungu lomwe lakhudzidwa.

1. EB Simplex (EBS): Uwu ndi mtundu wofala kwambiri wa EB. Ukhoza kukhala wofatsa, osati wopweteka kwambiri, mpaka wopweteka kwambiri, komanso woopsa, mwa anthu ena. Matuza amapangika pamwamba pa khungu, khungu la khungu . Zilonda sizimachitika kawirikawiri matuza awa akachira.

2. Junctional EB (JEB): Mu JEB, matuza amapangika mkamwa ndi m'njira yopumira. Izi sizichitika kawirikawiri , ndipo zimatha kuyambira zofatsa (zosasangalatsa pang'ono, zopweteka pang'ono) mpaka zowopsa.

3. Dystrophic EB (DEB): Mu DEB, matuza amapangika pakati pa khungu, dermis . Izi zimathanso kusiyana kuyambira pang'ono mpaka koopsa.

4. Kindler Syndrome: Mu Kindler Syndrome, matuza amatha kupangika pafupifupi pakhungu lonse. Izi zimachitika kawirikawiri .

Madokotala amasiyanitsa EBS, JEB, ndi DEB kutengera zigawo za khungu lanu zomwe zakhudzidwa. Mu Kindler syndrome, matuza amatha kuwonekera m'magawo osiyanasiyana a khungu lanu.

Ndani amene amakhudzidwa kwambiri ndi vutoli? Kodi ndi lofala bwanji?

Matenda a EB angakhudze aliyense. Anthu a fuko lililonse, fuko lililonse, kapena jenda lililonse akhoza kukhala ndi matenda a EB. Komabe,Ngati m'modzi mwa makolo anu ali ndi matendawa, muli ndi mwayi waukulu wopeza EB.

Ngakhale kuti matendawa ndi ofala kwambiri, malinga ndi ziwerengero ku United States, munthu m'modzi yekha pa anthu 50,000 ali ndi vuto la EB. Izi zikutanthauza kuti ndi vuto losowa kwambiri.

Kodi zotsatira za EB zingakhale zotani pa thupi?

Pa milandu yoopsa ya EB, maso amatha kutulutsa matuza komanso kupangitsa kuti munthu asaone bwino . Nthawi zina, khungu ndi minofu zimatha kusokonekera kwambiri, zomwe zimapangitsa kuti zikhale zovuta kusuntha zala, manja, mapazi, ndi malo olumikizirana mafupa bwino. Anthu ena omwe ali ndi EB ali pachiwopsezo chachikulu chotenga khansa ya pakhungu yotchedwa squamous cell carcinoma .

Pa milandu yoopsa kwambiri, imfa nthawi zina imachitika ali akhanda . Izi zimachitika chifukwa cha matenda monga matenda oopsa (monga sepsis ), kuvutika kupuma chifukwa cha kutsekeka kwa mpweya, kusowa madzi m'thupi, komanso kusowa zakudya m'thupi.

Kodi EB ikhoza kupha?

Izi zimadalira kwambiri mtundu wa EB womwe muli nawo. Mitundu yochepa komanso yocheperako ya EB siipha. Komabe, nthawi yoti anthu omwe ali ndi mitundu yoopsa ya EB akhale ndi moyo imatha kuyambira ali ana mpaka zaka 30. Chifukwa chake iyi ndi vuto lomwe limafunika chisamaliro chambiri.

Kodi zizindikiro za EB ndi ziti?

Zizindikiro za EB zimasiyana malinga ndi mtundu wake. Zizindikirozi nthawi zambiri zimawonekera mukadali khanda kapena mwana wamng'ono . Zizindikiro zina zimakhala zofala pakati pa mitundu yosiyanasiyana. Nazi zina mwa zizindikiro zazikulu za EB:

• Matuza pakhungu (manja, mapazi, zigongono, mawondo) kapena mkati mwa thupi.
• Khungu la m'manja ndi m'mapazi limakhuthala, zomwe zimapangitsa kuti zikhale ngati zotupa.
• Kuchepa kwa maselo ofiira a magazi (Anemia).
• Zala zolumikizana (zala kapena zala zolumikizana).
• Zikhadabo za zala ndi zala zakumapazi zopindika komanso/kapena zokhuthala.
• Kuoneka kwa matuza ang'onoang'ono oyera ngati matuza (milia) pakhungu.
• Kuvuta kumeza chakudya (dysphagia).
• Kusakula bwino mwa mwana.
• Mano osakula monga momwe amayembekezera ( hypoplasia ).

Kodi chimayambitsa EB ndi chiyani?

Chifukwa chachikulu cha EB ndi kusintha kwa majini kapena chilema mu imodzi mwa majini 18. Anthu omwe ali ndi matendawa ali ndi jini yomwe yasowa kapena yowonongeka yomwe imathandiza kupanga puloteni yotchedwa collagen . Monga mukudziwa, collagen ndiyo imapatsa mphamvu ndi kapangidwe ka minofu yolumikizana monga khungu lathu. Chifukwa cha chilema ichi cha majini, zigawo ziwiri za khungu, epidermis ndi dermis, sizimalumikizana bwino. Zotsatira zake, khungu limakhala lofooka kwambiri, limatupa mosavuta, komanso limavulala.

Matenda a EB nthawi zambiri ndi matenda obadwa nawo , zomwe zikutanthauza kuti ngati kholo limodzi lili nawo, anawo akhoza kuwatenga.

Komabe, kawirikawiri, EB imathanso kuchitika ngati matenda obwera chifukwa cha autoimmune .

Chofunika kwambiri ndichakuti matenda a EB si matenda opatsirana. Nthawi zambiri amakhala obadwa nawo. Choncho, ngati mutakumana ndi munthu amene ali ndi matenda a EB, simudzawatenga.

Kodi mungadziwe bwanji ngati muli ndi EB? (Kuzindikira)

Madokotala amatha kuzindikira EB mwa kuchita biopsy ya khungu , yomwe imaphatikizapo dokotala kutenga chitsanzo cha khungu ndikuyang'ana pansi pa maikulosikopu.

Kuphatikiza apo, kuyezetsa majini kumatha kuzindikira jini lolakwika ndikutsimikizira mtundu wa EB. Makolo omwe akukonzekera kukhala ndi mwana amathanso kuyezetsa majini a amayi apakati kuti adziwe ngati ali pachiwopsezo chokhala ndi mwana wokhala ndi EB.

Kodi mankhwala a EB ndi otani?

Mwatsoka, pakadali pano palibe mankhwala a EB . Komabe, mankhwala angathandize:

• Zimathandiza kupewa matuza.
• Kusamalira bwino matuza ndi khungu kumateteza mavuto.
• Amachiritsa mavuto azakudya omwe angayambitsidwe ndi matuza mkamwa kapena pakhosi.
• Kulamulira ululu.

Pofuna kupewa kuwonongeka kwa khungu komanso kupanga matuza ndi zilonda chifukwa cha kukangana, madokotala amalimbikitsa izi:

• Valani zovala zofewa, zomasuka zopangidwa ndi ulusi wachilengedwe. Kuvala zovala mkati ndi kunja kungathandize kuchepetsa kuchuluka kwa misoko yomwe imakukuta pakhungu lanu.
• Pewani kutentha kwambiri; sungani zipinda pamalo abwino komanso otentha mofanana.
• Pewani kutuluka padzuwa, kapena valani mafuta oteteza ku dzuwa mukamavala magalasi ndi zipewa.
• Gwiritsani ntchito mitundu yapadera ya mabandeji kuti muteteze khungu - gwiritsani ntchito mabandeji osamatirira ndi tepi, gauze wopindidwa, ndi zina zotero.

Pofuna kuchiza matuza, dokotala wanu angakulangizeni zinthu monga:

• Pakani mafuta odzola pa mabala tsiku lililonse.
• Gwiritsani ntchito mabandeji opangidwa ndi mankhwala kuti muthandize matuza kuchira ndikuletsa matenda.
• Imwani mankhwala kuti muchepetse ululu.

Pofuna kuchiza matenda, dokotala wanu angakulangizeni izi:

• Imwani mankhwala opha tizilombo toyambitsa matenda kapena pakani kirimu wothira mankhwala opha tizilombo toyambitsa matenda.
• Gwiritsani ntchito zophimba mabala zapadera pa mabala omwe sachira.

Pofuna kupewa mavuto okhudzana ndi zakudya komanso kuvutika kudya chifukwa cha matuza a pakamwa kapena pakhosi, dokotala wanu angakulangizeni izi:

• Mukadyetsa mwana wanu, gwiritsani ntchito botolo la mwana lokhala ndi nipple yapadera .
• Perekani mwana mankhwala ochotsera maso kapena syringe.
• Onjezani madzi pang'ono ku chakudya chophwanyika kuti muchepetse kuuma ndikuchidyetsa. Izi zipangitsa kuti chikhale chosavuta kumeza.
• Idyani zakudya zofewa kwambiri monga supu, zakudya zosenda, pudding, ndi applesauce .
• Chakudya chimaperekedwa kutentha kotentha (osati kotentha kwambiri).
• Pitani kwa katswiri wa zakudya kuti akuuzeni zomwe mukufuna pa zakudya zanu.

Pa milandu yoopsa ya EB, mungafunikenso opaleshoni . Ngati m'mero ​​(chubu chomwe chimanyamula chakudya kuchokera pakamwa kupita m'mimba) chatsekedwa ndi matuza ndi zipsera, opaleshoni ingachitike kuti ikule. Anthu ena akhoza kupitirira m'mero ​​wonse ndikuyika chubu chodyetsera kuti chakudya chilowe m'mimba mwachindunji. Opaleshoni ingachitikenso kuti zala zomwe zamamatirana pamodzi chifukwa cha matuza.

Kodi munthu amene ali ndi EB amadzisamalira bwanji?

Ngati muli ndi EB, malangizo awa adzakuthandizani kudzisamalira nokha:

• Gonani pa mapepala ndi mapilo opangidwa ndi ulusi wofewa, wachilengedwe, monga silika kapena satin.
• Valani nsapato zabwino zomwe sizikuchepetsa thupi lanu.
• Pewani kuyimirira kapena kuyenda kwa nthawi yayitali.
• Pewani kukanda kapena kutola pakhungu. Pakani mankhwala oletsa kuyabwa kuti muchepetse kuyabwa.
• Samalani ndi malo omwe muli ndipo pewani kukhudza kapena kukanda khungu lanu mwangozi.
• Nthawi zonse nyowetsani khungu lanu kuti muchepetse kukangana.
• Ngati matuza ayamba, aduleni mwachangu ndi singano yoyeretsera kapena lumo loyera.
• Gwiritsani ntchito bandeji yosamatirira. Kapena, pakani mafuta odzola (Vaseline™) kapena mafuta odzola khungu (monga Aquaphor™) pa bandejiyo kuti isamamatire pakhungu.

Kodi mungasamalire bwanji mwana yemwe ali ndi EB? Malangizo ena kwa makolo

Ngati mwana wanu ali ndi EB, zosowa zake zimakhala zosiyana ndi za ena. Mwina sangafotokoze kusasangalala kwake. Malangizo awa angathandize mwana wanu kukhala womasuka momwe angathere:

• Sambani m'manja mwanu bwino musanakhudze khungu la mwana wanu.
• Pewani kugwiritsa ntchito magolovesi a latex, chifukwa amatha kukulitsa kukangana.
• Mukasamba mwana wanu, m'malo moika thupi lonse m'beseni lodzaza ndi madzi nthawi imodzi, sambani khungu lililonse limodzi ndi limodzi.
• M'malo moika matewera anu akhanda m'matewera, ganizirani kuwayika ma pad onyowa.
• Gwiritsani ntchito matewera okhala ndi zingwe za Velcro®. Zingwe/tepi yomatira imatha kumamatira pakhungu la mwana wanu mosadziwa.
• Dulani ndi kuchotsa chotanuka chomwe miyendo ya thewera imalumikizidwa.
• Ikani zinthu ngati mapepala a silicone gel mkati mwa thewera kuti khungu lisamamatire ku matuza omwe apangidwa.
• Samalani kwambiri mukamanyamula mwanayo.Pewani kunyamula manja anu pansi pa makwapa (axilla). Ngati mwana wanu ali ndi matuza kapena mabala pa ntchafu ndi kumbuyo, mukwezeni ndi dzanja limodzi mozungulira ntchafu zake ndipo dzanja lina likuchirikiza msana wake.
• Limbikitsani mwana wanu kuti akhale wochita masewera olimbitsa thupi momwe matenda ake a EB amalolera. Kukhala chete kungayambitse mavuto ena, monga kudzimbidwa ndi kufooka kwa minofu. Kusambira ndi masewera olimbitsa thupi abwino komanso osakhudza thupi.
• Musalimbikitse mwana wanu kuchita masewera olimbitsa thupi kapena kuchita masewera olimbitsa thupi omwe angamupangitse kutuluka thukuta kapena kutentha.

Kuthana ndi zizindikiro za mwana wanu kungakhale kovuta nthawi zina. Mungamve kuti mwatopa kwambiri. Funsani dokotala wanu kuti akupatseni malangizo ena kuti mwana wanu - komanso inu - mukhale omasuka momwe mungathere. Komanso, polowa nawo m'magulu othandizira makolo , mutha kugawana zomwe mwakumana nazo ndikuphunzira njira zatsopano zothanirana ndi vuto la EB la mwana wanu.

Kodi EB ingapewedwe?

Popeza EB ndi matenda a majini, sangathe kupewedwa . Anthu omwe ali ndi mbiri ya banja la EB ndipo akukonzekera kukhala ndi ana angaone kuti uphungu wa majini ndi wothandiza kwambiri posankha momwe angakonzekerere banja lawo.

Kuphatikiza apo, akatswiri pakadali pano sakudziwa chomwe chimayambitsa mtundu wa EB womwe wapezeka. Chifukwa chake, madokotala sakudziwa momwe angapewere izi.

Kodi munthu amene ali ndi EB angayembekezere tsogolo lotani?

Tsogolo la anthu omwe ali ndi EB limadalira mtundu wa EB womwe ali nawo komanso kuopsa kwake. Mitundu yoopsa ya matendawa ingayambitse ululu waukulu, zilema, kulumala, mabala omwe sachira, komanso imfa yoyambirira .

Komabe, madokotala angakuthandizeni kuthana ndi zizindikiro zanu. Izi zitha kuchitika ndi chithandizo choyenera komanso, ngati pakufunika, mankhwala opweteka. Kusunga khungu lophimbidwa bwino ndi ma dressing osamatirira, oteteza tsiku lililonse, kusamba pafupipafupi, komanso kusamalira mabala kungathandize kuchepetsa zotsatira za EB pa moyo wanu.

Ngati muli ndi EB, kodi muyenera kupita kwa dokotala liti?

Onetsetsani kuti mwaonana ndi dokotala wanu pazochitika izi:

• Ngati mukuvutika kupuma.
• Ngati mukuvutika kumeza chakudya.
• Ngati bala lanu likuoneka kuti lili ndi kachilombo (khungu likufiira, lofiirira, lotuwa, kapena loyera; lotupa kapena lotupa).
• Ngati zizindikiro zatsopano zikuwonekera.

Kodi ndi mafunso otani ofunika kufunsa dokotala?

Mukapita kwa dokotala, ndi bwino kufunsa mafunso ngati awa:

• Kodi mungadziwe bwanji ngati ndili ndi EB?
• Ngati ndilibe EB, ndi matenda ena ati a pakhungu omwe ndingakhale nawo?
• Kodi ndingathe bwanji kuthana ndi zizindikiro zanga?
• Kodi ndi mankhwala ati omwe mumalimbikitsa? Kodi ali ndi zotsatirapo zake?
• Kodi ndi mankhwala ati a kunyumba omwe mumalimbikitsa?
• Ndiyenera kuchita chiyani china kuti ndichepetse zizindikiro zanga?
• Kodi pali kirimu kapena mafuta odzola omwe mungamupatse?
• Kodi ndiyenera kupita kwa dokotala wa khungu kapena katswiri wina?

Kodi pali kusiyana kotani pakati pa epidermolysis bullosa (EB) ndi bullous pemphigoid?

Bullous pemphigoid ndi matenda osowa kwambiri pakhungu omwe amayambitsa chitetezo chamthupi. Angayambitse kuyabwa, kutupa ngati ming'oma kapena matuza odzaza ndi madzi. Bullous pemphigoid nthawi zambiri imakhudza anthu azaka zopitilira 60 , ndipo nthawi zambiri imatha mkati mwa zaka zisanu. Komabe, pazochitika zazikulu, matuza ndi zipsera zimatha kuwononga ziwalo zamkati ndikupha.

Ngakhale kuti nthawi zina EB ingakhale matenda odziteteza ku matenda enaake, nthawi zambiri imayamba chifukwa cha kusintha kwa majini komwe kumakhudza collagen pakhungu lanu. EB nthawi zambiri imawonekera kuyambira ali mwana kapena ali mwana . Popeza palibe mankhwala a EB, mungafunike kukhala ndi zizindikiro kwa moyo wanu wonse.

Pomaliza, zinthu zoti muzikumbukira (Uthenga Wopita Nawo Kunyumba)

Epidermolysis bullosa (EB) nthawi zina imakhala vuto lalikulu. Komabe, ndi upangiri woyenera wachipatala, chithandizo, ndi chisamaliro chachikondi, mutha kuthana ndi zizindikirozo ndikukhala ndi moyo wabwino momwe mungathere. Kumbukirani, simuli nokha. Madokotala, mabanja, ndi magulu othandizira onse alipo kuti akuthandizeni. Chifukwa chake, musachite mantha, khalani olimba mtima, ndipo muthane ndi vutoli.

Ngati muli ndi mafunso ena okhudza EB, onetsetsani kuti mwalankhula ndi dokotala wa khungu.

Epidermolysis bullosa, EB, matenda a pakhungu, matuza, matenda a majini, matenda a pakhungu la ana, kufooka kwa khungu