Epidermolysis Bullosa Ni Iki? Ibimenyetso, Impamvu, n’Uko Ivurwa

Epidermolysis bullosa (EB) ni indwara idasanzwe iterwa na gène, ituma uruhu rwawe ruba rworoshye cyane kandi rugatinda gukira. No gukoraho gake, gukorakorana, cyangwa ikibazo gito gishobora gutera ibisebe bibabaza n’ibikomere ku ruhu rwawe, rimwe na rimwe no mu mubiri wawe imbere.

Tekereza ko uruhu rwawe rumeze nk’impapuro zoroheje. Nubwo iyi ndwara igira ingaruka zitandukanye ku bantu, ikintu gikomeye ni uko uruhu rwawe rutagira imyunyu ngugu ikomeye ikeneye kugirango ibice byarwo bihuze neza. Ibi bituma ibikorwa bya buri munsi bigorana, ariko ubufasha bukwiye n’ubuvuzi, abantu benshi bafite EB babona ubuzima buhebuje.

Ibimenyetso bya Epidermolysis Bullosa ni ibihe?

Ikimenyetso nyamukuru ni ibisebe byoroshye cyane kurusha uko bikwiye. Ibi bisebe bishobora kugaragara ahantu hose ku mubiri wawe, akenshi ahantu imyenda ikorakora cyangwa aho uhuye n’ubukorakorana bwa buri munsi.

Dore ibimenyetso bisanzwe ushobora kubona:

• Ibisebe ku ntoki, amaguru, amavi, n’amavi biturutse ku bikorwa bisanzwe
• Uruhu rworoshye rudashobora kwihanganira gukorakora
• Ibisebe mu kanwa, mu muhogo, cyangwa mu mara
• Uruhu rukomeye ku kuboko n’ibirenge
• Ibironda nyuma y’aho ibisebe bikize
• Imisumari y’intoki n’ibirenge ibura cyangwa yangiritse
• Ibibazo by’amenyo nko guhumeka cyangwa ibibazo by’enamel

Mu bihe bikomeye, ushobora kugira ibisebe mu munwa bigorana kugira ngo umenyere, cyangwa ibibazo by’amaso bigira ingaruka ku kubona kwawe. Bamwe mu bantu barwara anémie kubera ibikomere bidakira n’uburyo bwo gukira.

Ibimenyetso bikunze kugaragara mu buto cyangwa mu bwana, nubwo ubwoko buke butoroshye bushobora kutagaragara kugeza mu myaka y’ubukure. Uburambe bwa buri muntu ni bwihariye, kandi ibimenyetso bishobora kuva ku bisebe bito kugeza ku bibazo bikomeye bya buri munsi.

Ubwoko bwa Epidermolysis Bullosa ni ubuhe?

Hari ubwoko bune nyamukuru bwa EB, buri bwoko bugira ingaruka ku bice bitandukanye by’uruhu rwawe. Gusobanukirwa ubwoko ufite bifasha abaganga gutanga ubufasha bukwiye na gahunda y’ubuvuzi.

Epidermolysis Bullosa Simplex (EBS) ni ubwoko busanzwe kandi busanzwe buke. Ibisebe bikunze kugaragara ku ruhu rwo hejuru kandi bikira bidahariye. Ushobora kubona ibisebe byinshi mu gihe cy’izuba cyangwa mu gihe cy’imikino.

Dystrophic Epidermolysis Bullosa (DEB) igira ingaruka ku ruhu rwo hasi kandi ikunze gutera ibironda. Ubu bwoko bushobora gutuma intoki n’ibirenge bihuza hamwe igihe kirekire, kandi bishobora kugira ingaruka ku bice by’imbere by’umubiri nko mu munwa.

Junctional Epidermolysis Bullosa (JEB) itera mu gice gihuza ibice byo hejuru n’ibyo hasi by’uruhu rwawe. Ubu bwoko bushobora kuva ku bworoshye kugeza ku bukomeye, ubwoko bumwe na bumwe bukaba ubwo bugira ingaruka ku buzima mu buto.

Kindler Syndrome ni ubwoko buke cyane, buhuza ibimenyetso by’ubundi bwoko. Abantu bafite ubu bwoko bakunze kugira uburyohe bwinshi ku zuba kandi bashobora kugira impinduka z’uruhu zisa n’iz’ubukure.

Impamvu za Epidermolysis Bullosa ni izihe?

EB iterwa n’impinduka (mutations) muri gène zikora imyunyu ngugu ishinzwe guhuza ibice by’uruhu rwawe. Iyi myunyu ngugu ikora nk’ikiboko cyangwa igikoresho cyo gufata, kandi iyo idakora neza, uruhu rwawe ruraba rworoshye.

Iyi ni indwara ikomoka ku miryango, bivuze ko iherwa abana n’ababyeyi binyuze muri gène. Ariko, uburyo bwo guherwa butandukanye bitewe n’ubwoko bwa EB ufite.

Ubwoko bwinshi bukurikira icyo abaganga bita “autosomal recessive” inheritance. Ibi bivuze ko ababyeyi bombi bagomba kuba bafite gène yahindutse kugira ngo umwana wabo arware EB. Ababyeyi bafite kopi imwe ntibagira ibimenyetso ariko bashobora guherwa abana babo iyi ndwara.

Ubwoko bumwe bukurikira “autosomal dominant” inheritance, aho umubyeyi umwe gusa akeneye kugira gène yahindutse. Mu bihe bidafite akarusho, EB ishobora kuba impinduka nshya ya gène, bivuze ko nta mubyeyi ufite iyo ndwara cyangwa ufite iyo gène.

Ni ryari ukwiye kujya kwa muganga kubera Epidermolysis Bullosa?

Ukwiye kujya kwa muganga niba wowe cyangwa umwana wawe mugize ibisebe byoroshye cyane cyangwa bidakomoka ku kintu runaka. Ibi ni ingenzi cyane niba ibisebe bigaragara iyo ukoresheje imbaraga nke cyangwa mu bikorwa bisanzwe bya buri munsi.

Shaka ubufasha bwa muganga vuba niba ubona ibisebe mu kanwa cyangwa mu muhogo bigorana kurya cyangwa kunywa. Banza urebe ibimenyetso by’indwara ku bikomere, nko gutukura, ubushyuhe, kubyimba, cyangwa ibyuya.

Niba ufite amateka y’umuryango wa EB kandi uteganya kubyara, inama y’abaganga ku birebana na gène ishobora kugufasha gusobanukirwa ibyago n’amahirwe uboneye.

Ntugatege amatwi niba ubona ko ibisebe bikira buhoro, bikunze kwandura, cyangwa niba iyo ndwara igira ingaruka ku bushobozi bwawe bwo kurya, kunywa, cyangwa gukora ibikorwa bya buri munsi. Ubufasha bwihariye bushobora kugufasha kwirinda ingaruka mbi no kunoza ubuzima.

Ibyago bya Epidermolysis Bullosa ni ibihe?

Ibyago nyamukuru ni ukugira ababyeyi bafite impinduka za gène ziterwa na EB. Kubera ko iyi ari indwara ikomoka ku miryango, amateka y’umuryango wawe agira uruhare runini mu kumenya ibyago byawe.

Niba ababyeyi bombi bafite gène ya EB, buri gihe cyo gutwita gifite amahirwe 25% yo kubyara umwana ufite iyo ndwara. Iyo umubyeyi umwe afite ubwoko bwa EB, buri mwana afite amahirwe 50% yo kuherwa iyo ndwara.

Kugira abavandimwe cyangwa abandi bantu ba hafi bafite EB byongera amahirwe yo kuba ufite iyo ndwara. Ariko, birakwiye kuzirikana ko EB ishobora rimwe na rimwe kuba impinduka nshya ya gène, ndetse no mu miryango idafite amateka yayo.

Uruhererekane rw’amoko runaka rushobora kugira umubare munini w’ubwoko bwa EB, ariko iyi ndwara ishobora kugira ingaruka ku bantu b’ubwoko ubwo aribwo bwose. Uburemere n’ubwoko bwa EB uherwa biterwa n’impinduka za gène zohererezwa n’ababyeyi bawe.

Ingaruka zishoboka za Epidermolysis Bullosa ni izihe?

Nubwo abantu benshi bafite EB babasha kuyigenzura neza, hari ingaruka zimwe na zimwe zishobora kuza igihe kirekire. Gusobanukirwa ibyo bishoboka bigufasha gukorana n’itsinda ryawe ry’abaganga kugira ngo ubirinde cyangwa ubiganireho hakiri kare.

Dore ingaruka zishobora kubaho:

• Ibikomere bidakira vuba kandi bikunze kwandura
• Ibironda bishobora kugabanya ubushobozi bwo kugenda, cyane cyane mu biungo
• Guhuza kw’intoki cyangwa ibirenge (pseudosyndactyly)
• Kugabanyuka kw’umunwa, bigorana kugira ngo umenyere
• Anémie iterwa no kubura amaraso n’imirire mibi
• Ibibazo by’impyiko mu bwoko bumwe bukomeye
• Ibibazo by’amaso harimo n’ibikomere bya cornea
• Ibyago byiyongereye bya kanseri y’uruhu mu bice bikomeye

Ibibazo by’imirire bikunze kubaho kuko kurya bishobora kubabaza iyo ibisebe bigaragara mu kanwa cyangwa mu muhogo. Ibi bishobora gutera igihombo cy’uburemere, gutinda gukura mu bana, no kubura vitamine n’imyunyu ngugu ikenewe.

Mu bihe bidafite akarusho, ubwoko bukomeye bwa EB bushobora gutera ingaruka zikomeye ku buzima mu buto. Ariko, ubufasha bukwiye bwa muganga n’ubufasha, ingaruka nyinshi zishobora kwirindwa cyangwa zigacungwa neza.

Epidermolysis Bullosa imenyekanwa ite?

Abaganga bakunze kumenya EB binyuze mu gusuzuma uruhu rwawe no kubona amateka yawe y’ubuzima. Bazareba uburyo ibisebe bigaragara kandi babuze igihe ibimenyetso byagaragaye bwa mbere n’icyo bibatera.

Ubusanzwe, gusuzuma uruhu ni ngombwa kugira ngo hamenyekane neza indwara n’ubwoko bwa EB ufite. Muri ubu buryo, muganga wawe akuramo igice gito cy’uruhu akagisuzuma munsi y’imashini zihariye kugira ngo arebe aho uruhu rwatandukaniye.

Isuzuma rya gène rishobora kumenya impinduka za gène ziterwa na EB. Aya makuru afasha kumenya uko iyo ndwara ishobora gutera imbere kandi ayobora imyanzuro y’ubuvuzi. Ni ingenzi kandi mu gutegura umuryango no kugira inama ku birebana na gène.

Muganga wawe ashobora kandi gusaba ibizamini by’amaraso kugira ngo arebe anémie cyangwa kubura imirire. Niba ugira ibibazo byo kugira ngo umenyere, ashobora kugusaba gusuzuma amaso kugira ngo arebe umunwa wawe n’ibice by’imbere by’umubiri.

Uko Epidermolysis Bullosa ivurwa

Nubwo nta muti urakora kuri EB, uburyo bwo kuvura bugamije kurinda uruhu rwawe, gucunga ibimenyetso, no kwirinda ingaruka. Intego ni ugufasha kubaho neza kandi utekanye.

Kwita ku bikomere ni shingiro ry’uburyo bwo kuvura EB. Ibi birimo gukuraho ibisebe neza, gushyiraho imyenda yihariye, no gukoresha uburyo bwo gukira no kwirinda indwara.

Dore uburyo nyamukuru bwo kuvura:

• Imyenda yihariye y’ibikomere idakora ku ruhu rworoshye
• Ibikoresho byo kwita ku ruhu n’uburyo bworoshye
• Gucunga ububabare hakoreshejwe imiti ikwiye
• Ubufasha mu mirire kugira ngo ugume ukura neza kandi ukire
• Ubufasha bwo kugenda kugira ngo ugume ugendagenda kandi wirinde ibironda
• Antibiotique iyo indwara ibayeho
• Ubutabire ku ngaruka zikomeye nko kugabanyuka kw’umunwa

Itsinda ryawe ry’abaganga rishobora kuba ririmo abaganga b’uruhu, abahanga mu kuvura ibikomere, abahanga mu mirire, n’abahanga mu kuvura. Bazakorana kugira ngo bagufashe gushyiraho gahunda y’ubuvuzi ikubereye.

Uburyo bushya bwo kuvura burakorwaho, harimo uburyo bwo kuvura gène n’uburyo bwo kuvura imyunyu ngugu. Nubwo bitaraboneka, bitanga icyizere cy’uburyo bwo kuvura bukorwa neza mu gihe kizaza.

Uko wita kuri Epidermolysis Bullosa mu rugo

Kwita ku buzima bwa buri munsi mu rugo bigira uruhare rukomeye mu gucunga EB neza. Ukoresheje uburyo n’ibikoresho bikwiye, ushobora kugabanya ibisebe no gufasha ibikomere gukira vuba.

Kwita ku ruhu rworoshye ni ingenzi. Koresha amazi ashyushye yo koga kandi usukure uruhu rwawe aho kurukorakorana. Hitamo imyenda yoroshye kandi idakomeye kandi wirinde ibintu bishobora gutera ubukorakorana cyangwa kubabaza.

Dore uko ushobora kwita kuri wowe cyangwa umuntu ukunda ufite EB mu rugo:

• Gabanya imisumari y’intoki cyane kandi uyisukure kugira ngo wirinde gukorakorana
• Koresha ibikoresho byihariye cyangwa ibintu byoroshye ahantu hakunda kuba ibisebe
• Shyiraho amavuta yo gusukura uruhu kugira ngo uruhu rugume rworoshye kandi rudakora
• Hindura imyenda y’ibikomere buri gihe ukoresheje uburyo buzira umwanda
• Kora isuzuma ku bikomere kugira ngo urebe ibimenyetso by’indwara nko gutukura cyangwa ibyuya
• Komeza imirire myiza kugira ngo ufashe gukira
• Komeza kunywa amazi kugira ngo uruhu rwawe rugume rukomeye

Kurema ahantu heza mu rugo bigufasha kwirinda imvune zitari ngombwa. Kuraho ibintu biremereye, koresha ibintu byoroshye, kandi ube ufite umucyo uhagije kugira ngo wirinde kugwa bishobora gutera ibisebe bishya.

Ntuzuyaze kuvugana n’itsinda ryawe ry’abaganga iyo ufite ibibazo cyangwa impungenge ku bijyanye no kwita ku bikomere. Bashobora kugufasha no guhindura gahunda yawe y’ubuvuzi uko bikenewe.

Uko wakwitegura kujya kwa muganga

Kwita ku buzima bwawe mbere yo kujya kwa muganga bigufasha kubona ibyiza byinshi mu gihe cyawe n’itsinda ry’abaganga. Zana urutonde rw’ibimenyetso uheruka kugira, imiti, n’ibibazo byose ushaka kuganiraho.

Komeza kwandika ibimenyetso byawe, uko ibisebe bigaragara, icyo bishobora kuba byarateye, n’uko bikira. Fata amafoto y’ibikomere bishishikaje cyangwa impinduka ku ruhu rwawe kugira ngo ubigaragarize muganga.

Andika imiti n’uburyo bwo kuvura ukoresha ubu, harimo ibintu ugura udafite ubufasha bwa muganga n’uburyo bwo kuvura mu rugo. Ibi bifasha muganga wawe gusobanukirwa icyakoraga n’icyo gikwiye guhinduka.

Tegura ibibazo ku bijyanye no kwita ku buzima bwa buri munsi, ibikorwa ugomba kwirinda, n’igihe ukwiye gushaka ubufasha bwihuse. Baza ibibazo ku bijyanye n’ibikoresho, amatsinda y’ubufasha, n’abahanga bashobora kugufasha mu mimerere yawe.

Uko Epidermolysis Bullosa ishobora kwirindwa

Kubera ko EB ari indwara ikomoka ku miryango, ntishobora kwirindwa mu buryo busanzwe. Ariko, inama y’abaganga ku birebana na gène ishobora gufasha imiryango gusobanukirwa ibyago byabo no gufata ibyemezo byiza ku bijyanye no kubyara.

Niba ufite EB cyangwa ufite gène yayo, abaganga bagufasha gusobanukirwa amahirwe yo guherwa iyo ndwara abana bawe. Bashobora kandi kuganira ku buryo nko gusuzuma abana bataravuka cyangwa uburyo bwo kubyara bufashwa.

Ku bantu basanzwe bafite EB, kwirinda bigamije kwirinda ibisebe bishya n’ingaruka. Ibi bivuze kurinda uruhu rwawe imvune, kugira imirire myiza, no gukurikiza gahunda yawe y’ubuvuzi buri gihe.

Gutabara hakiri kare no kwita ku bikomere neza bishobora kwirinda ingaruka nyinshi zijyana na EB. Gusuzuma buri gihe n’itsinda ryawe ry’abaganga bigufasha kubona no gukemura ibibazo mbere y’uko biba bikomeye.

Icyingenzi cyo kuzirikana kuri Epidermolysis Bullosa

EB ni indwara igorana isaba kwitabwaho buri gihe, ariko abantu benshi bafite EB babona ubuzima buhebuje. Ikintu nyamukuru ni ugukorana n’abaganga b’abahanga no kumenya uburyo bwo kwita ku buzima bwawe.

Nubwo nta muti urakora, ubushakashatsi burakomeza gutera imbere ubumenyi bwacu n’uburyo bwo kuvura. Uburyo bwo kuvura buriho bushobora kunoza ubuzima kandi bugakemura ingaruka nyinshi iyo bukoreshejwe buri gihe.

Zirikana ko EB igira ingaruka zitandukanye ku bantu. Icyakorera umuntu kimwe gishobora kudashoboka ku wundi, rero komeza kuvugana n’itsinda ryawe ry’abaganga kugira ngo unoze gahunda yawe y’ubuvuzi.

Huza n’imiryango ishyigikira EB n’andi matsinda y’imiryango ifite iyo ndwara. Bashobora gutanga inama zifatika, ubufasha bwo mu mutima, n’amakuru mashya ku bijyanye n’ubushakashatsi n’uburyo bushya bwo kuvura bishobora kugufasha.

Ibibazo bikunze kubaho kuri Epidermolysis Bullosa

Epidermolysis Bullosa yandura?

Oya, EB ntiyandura. Ni indwara ikomoka ku miryango uvuka ufite, si ikintu ushobora kwandura cyangwa guherwa abandi bantu. Ibisebe n’ibikomere biterwa n’uruhu rworoshye, si mikorobe cyangwa virusi zishobora guherwa abandi.

Abantu bafite Epidermolysis Bullosa bashobora kubyara?

Yego, abantu benshi bafite EB bashobora kubyara. Ariko, hari ibyago byo guherwa abana babo iyo ndwara bitewe n’ubwoko bwa EB n’ubuzima bwa gène bw’uwo bashakanye. Inyunganizi y’abaganga mbere yo gutwita ishobora kugufasha gusobanukirwa ibyo byago no gusuzuma amahitamo yawe.

Epidermolysis Bullosa izakomeza kuba mbi uko igihe kigenda?

EB igira ingaruka zitandukanye ku bantu mu buzima bwabo. Ubwoko bumwe buguma buhagaze, ibindi bishobora gutera ibironda cyangwa ingaruka mbi uko igihe kigenda. Ariko, ubufasha bukwiye n’ubuvuzi, ingaruka nyinshi zishobora kwirindwa cyangwa zigabanuka. Gutabara hakiri kare no kwita ku bikomere buri gihe bigira uruhare rukomeye mu bizava mu gihe kirekire.

Abantu bakuru bashobora kurwara Epidermolysis Bullosa mu myaka y’ubukure?

EB nyayo ikomoka ku miryango ibaho kuva umuntu avutse, nubwo ubwoko buke butoroshye bushobora kutamenyekana kugeza mu myaka y’ubukure. Ariko, hari indwara idasanzwe yitwa epidermolysis bullosa acquisita ishobora kugaragara mu bantu bakuru kubera ibibazo by’ubudahangarwa aho kuba impamvu zikomoka ku miryango. Ibi bisaba uburyo butandukanye bwo kuvura ugereranije na EB ikomoka ku miryango.

Hari ibikorwa abantu bafite EB bakwiye kwirinda burundu?

Nubwo abantu bafite EB bagomba kwitondera ibikorwa bitera ubukorakorana cyangwa imvune, benshi bashobora kwitabira ibikorwa by’imikino n’imyidagaduro bakunda. Koga akenshi bihanganirwa neza, mu gihe imikino ikorakora ishobora kuba igorana. Korana n’itsinda ryawe ry’abaganga kugira ngo ubone uburyo bwo kuguma ukora imyitozo ngororamubiri n’ibikorwa bikubereye.