Ese uruhu rwawe rurimo uduheri kandi rukavuka no mu gukoraho gato? Ese byaba ari Epidermolysis Bullosa (EB)?

Hari igihe uruhu rwacu rugira ubworoherane cyane, si byo se? Nubwo ikintu gito cyarukubita, cyangwa umwenda wacitse, hari abantu barwara uduheri n'ibisebe byoroshye ku ruhu. Cyane cyane ko uruhu rw'abana bato ari rworoshye cyane, biteye ubwoba cyane iyo ubonye ibintu nk'ibyo. Uyu munsi rero tugiye kuvuga ku ndwara igaragara muri bene urwo ruhu, idasanzwe ariko ni ingenzi cyane kubimenya. Iyo ni epidermolysis bullosa, cyangwa EB mu magambo ahinnye.

Icyorezo cya epidermolysis bullosa (EB) ni iki?

Mu magambo make, epidermolysis bullosa (EB) ni indwara umuntu avukana . Itera uruhu rwawe koroha cyane kandi rukavunguka byoroshye. Hari igihe, nubwo wakwisiga imyenda cyangwa ukagwa ahantu runaka, ushobora kugira udusebe n'ibisebe. Kuri bamwe, bishobora kuba byoroshye kandi bigatera udusebe tubabaza ku biganza, ku nkokora, ku mavi no ku birenge. Ariko, mu bihe bikomeye, utu dusebe n'ibisebe bishobora kugaragara ahantu hose ku mubiri. Ibimenyetso bya EB bishobora kuva ku byoroheje kugeza ku bikomeye cyane .

Wibuke ko hari igihe ibi bisebe bishobora kumera mu kanwa, mu muhogo (mu ruhu), mu maso, no mu zindi ngingo z'umubiri. Iyo ibi bisebe bikize, bishobora gusiga inkovu zibabaza. Mu bihe bikomeye, ibi bisebe n'inkovu bishobora kwangiza ingingo z'umubiri, bishobora gushyira ubuzima mu kaga.

Ni ubuhe bwoko bw'ingenzi bwa EB?

Hari ubwoko bune bw'ingenzi bwa EB, bugaragazwa n'urwego rw'uruhu rwagizweho ingaruka.

1. EB Simplex (EBS): Ubu ni bwo bwoko bwa EB bukunze kugaragara cyane . Bushobora kuva ku bworoheje, butababaza cyane, kugeza ku bubabare bukabije, ku bantu bamwe na bamwe. Udusebe twibasira uruhu rwo hejuru, uruhu rwo mu nda . Inkovu ni nke cyane nyuma y’uko utwo dusebe dukize.

2. Indwara yo mu gice cy'amagufwa (JEB): Muri JEB, uduheri tuba mu kanwa no mu myanya y'ubuhumekero. Ibi ni gake cyane , kandi bishobora kuva ku byoroheje (bidatuje gato, bibabaza gato) kugeza ku bikomeye cyane.

3. Dystrophic EB (DEB): Muri DEB, udusebe tuba mu gice cyo hagati cy'uruhu, dermis . Ibi bishobora kandi gutandukana kuva ku byoroheje kugeza ku bikomeye.

4. Kindler Syndrome: Muri Kindler Syndrome, uduheri dushobora kugaragara ku ruhu hafi ya rwose. Ibi ni gake cyane .

Abaganga batandukanya EBS, JEB, na DEB bashingiye ku bice by'uruhu rwawe byafashwe. Muri Kindler syndrome, uduheri dushobora kugaragara mu bice bitandukanye by'uruhu rwawe.

Ni nde wibasirwa cyane n'iki kibazo? Ni iki gikunze kugaragara?

EB ishobora kugira ingaruka ku muntu uwo ari we wese. Abantu b'ubwoko ubwo aribwo bwose, ubwoko ubwo aribwo bwose, cyangwa igitsina bashobora kugira EB. Ariko,Niba umwe mu babyeyi bawe afite iyi ndwara, uba ufite amahirwe menshi yo kurwara indwara ya EB.

Nubwo ari ibintu bisanzwe, nk'uko imibare ibivuga muri Leta Zunze Ubumwe za Amerika, umuntu umwe gusa mu bantu 50.000 ni we urwaye indwara ya EB. Ibi bivuze ko ari indwara idasanzwe.

Ni izihe ngaruka EB ishobora kugira ku mubiri?

Mu bihe bikomeye bya EB, amaso ashobora guturika ndetse akanatuma umuntu atabona neza . Hari igihe hashobora kubaho inkovu zikomeye n'ubusembwa ku ruhu no ku mitsi, bigatuma intoki, amaboko, ibirenge n'ingingo bidakora neza. Bamwe mu bantu bafite EB bafite ibyago byinshi byo kurwara kanseri y'uruhu yitwa squamous cell carcinoma .

Mu bihe bikomeye cyane, hari igihe urupfu rushobora kubaho mu bwana . Ibi biterwa n'indwara nk'indwara zikomeye (urugero , sepsis ), kugorwa no guhumeka bitewe no kuziba kw'imiyoboro y'ubuhumekero, umwuma, ndetse n'imirire mibi.

Ese EB ishobora kwica?

Ibi biterwa cyane n'ubwoko bwa EB ufite. Ubwoko bworoheje kandi budakabije bwa EB ntabwo bwica. Ariko, igihe abantu bafite ubwoko bukomeye bwa EB bashobora kubaho kuva mu bwana kugeza ku myaka 30. Iyi ni indwara ikeneye kwitabwaho cyane.

Ni ibihe bimenyetso bya EB?

Ibimenyetso bya EB biratandukanye bitewe n'ubwoko. Ibi bimenyetso bikunze kugaragara iyo uri uruhinja cyangwa umwana muto . Ibimenyetso bimwe na bimwe bikunze kugaragara hagati y'ubwoko butandukanye. Dore bimwe mu bimenyetso by'ingenzi bya EB:

• Uduheri ku ruhu (amaboko, ibirenge, inkokora, amavi) cyangwa imbere mu mubiri.
• Uruhu rwo mu biganza no mu birenge binini, bigatuma rusa n'aho rufite uruhu rudakomeye.
• Kugabanuka k'uturemangingo dutukura tw'amaraso (Anemia).
• Intoki zifatanye (intoki cyangwa amano bifatanye).
• Inzara z'intoki n'iz'ibirenge zifite imiterere mibi cyangwa ubugari.
• Kugaragara kw'utubyimba duto tw'umweru tumeze nk'udusebe (milia) ku ruhu.
• Kugorwa no kumira ibiryo (dysphagia).
• Kubura imikurire iteganijwe ku mwana.
• Amenyo ntakura uko byari byitezwe ( hypoplasia ).

Ni iki gitera EB?

Impamvu nyamukuru ya EB ni impinduka cyangwa inenge muri imwe muri gene 18. Abantu bafite iyi ndwara bafite gene ibura cyangwa yangiritse ifasha gukora poroteyine yitwa collagen . Nkuko mubizi, collagen ni yo itanga imbaraga n'imiterere ku ngingo zihuza uruhu nk'uruhu rwacu. Kubera iyi gene defect, ibice bibiri by'uruhu, epidermis na dermis, ntibihuza neza. Ingaruka zabyo ni uko uruhu rurushaho koroha cyane, rukagira udusebe tworoshye, kandi rugakomereka.

Akenshi indwara ya EB ni indwara umuntu avukana , bivuze ko iyo umwe mu babyeyi ayirwaye, abana bashobora kuyiragwa.

Ariko, ni gake cyane, EB ishobora no kuba indwara y’ubwiyongere bw’umubiri .

Igikuru ni uko indwara ya EB atari indwara yandura. Akenshi iba mu murage. Rero, niba uhuye n'umuntu ufite EB, ntuzayirwara.

Wamenya ute neza ko ufite EB? (Isuzuma)

Abaganga bashobora gusuzuma EB bakora biopsy y'uruhu , aho umuganga afata agace gato k'uruhu akagasuzuma akoresheje mikorosikopi.

Byongeye kandi, ibizamini bya gene bishobora kumenya gene ifite inenge no kwemeza ubwoko bwa EB. Ababyeyi bateganya kubyara bashobora no gupimwa gene batwite kugira ngo bamenye niba bafite ibyago byo kubyara umwana ufite EB.

Ni ubuhe buryo bwo kuvura EB?

Ikibabaje ni uko nta muti uhari wa EB . Ariko, uburyo bwo kuvura bushobora gufasha:

• Bifasha kwirinda uduheri.
• Kwita ku duheri n'uruhu neza birinda ingorane.
• Ivura ibibazo by'imirire bishobora guterwa n'uduheri mu kanwa cyangwa mu muhogo.
• Kugenzura ububabare.

Kugira ngo hirindwe kwangirika k'uruhu no kubyara udusebe n'ibisebe bitewe no gukururana, abaganga batanga inama zikurikira:

• Ambara imyenda yoroshye kandi idafite ishingiro ikozwe mu migozi karemano. Kwambara imyenda imbere inyuma bishobora kugabanya ingano y'imigozi ku ruhu rwawe.
• Irinde gushyuha cyane; komeza ibyumba bifite ubushyuhe busanzwe kandi butuje.
• Irinde kujya hanze ku zuba, cyangwa kwambara amavuta yo kwisiga izuba mu gihe uri gukoresha indorerwamo z'izuba n'ingofero.
• Koresha ubwoko bwihariye bw'udupfundikizo kugira ngo urinde uruhu - koresha udupfundikizo tudafata neza n'ikaseti, agatambaro kazinze, nibindi.

Kugira ngo uvure uduheri, muganga wawe ashobora kukugira inama y'ibintu nk'ibi:

• Siga amavuta ku bikomere buri munsi.
• Koresha ibipfuko byabugenewe kugira ngo ufashe uduheri gukira no kwirinda kwandura.
• Fata imiti yo kugenzura ububabare.

Kugira ngo uvure indwara zandurira mu mubiri, muganga wawe ashobora kukugira inama ikurikira:

• Fata imiti igabanya ubukana cyangwa usige amavuta yo mu bwoko bwa antibiyotike.
• Koresha udupfundikizo twihariye tw'ibikomere ku bikomere bidakira.

Kugira ngo wirinde ibibazo by'imirire no kugorwa no kurya bitewe n'udusebe two mu kanwa cyangwa mu muhogo, muganga wawe ashobora kukugira inama ibi bikurikira:

• Mu gihe wonsa umwana wawe, koresha icupa ry'umwana ririmo agapfukamunwa kadasanzwe .
• Gaburira umwana ukoresheje agakoresho ko mu maso cyangwa seringe.
• Shyiramo amazi make ku biryo byaseye kugira ngo ubivange neza kandi ubihe. Ibi bizatuma byoroha kumira.
• Kurya ibiryo byoroshye cyane nk'isupu, ibiryo bivanze, pudingi, na applesauce .
• Ibiryo bitangwa ku bushyuhe bushyushye (butari bushyushye cyane).
• Reba inzobere mu mirire kugira ngo aganire ku byo ukeneye mu mirire.

Mu bihe bikomeye bya EB, ushobora no gukenera kubagwa . Niba umwobo (umuyoboro utwara ibiryo mu kanwa ujya mu gifu) ufunze kubera udusebe n'inkovu, kubagwa bishobora gukorwa kugira ngo biguke. Hari abantu bashobora kurenga umwobo wose hanyuma bagashyiramo umuyoboro wo kugaburira kugira ngo bashyire ibiryo mu gifu. Kubagwa bishobora kandi gukorwa kugira ngo intoki zifatanye hamwe kubera udusebe.

Ni gute umuntu urwaye EB yiyitaho?

Niba ufite EB, izi nama zizagufasha kwiyitaho:

• Kuryama ku mpapuro n'udufuka tw'imisego bikozwe mu migozi yoroshye kandi karemano, nka silk cyangwa satin.
• Ambara inkweto nziza zitakubangamira umubiri wawe.
• Irinde guhagarara cyangwa kugenda igihe kirekire.
• Irinde gushwanyaguza cyangwa gufata ku ruhu. Siga imiti igabanya ubushyuhe kugira ngo ugabanye ubushyuhe.
• Menya neza ibikukikije kandi wirinde gukorakora cyangwa gukanda uruhu rwawe utabishaka.
• Buri gihe uruhu rwawe ruhumeke kugira ngo ugabanye gucikagurika.
• Iyo udusebe tutangiye kugaragara, tubyinjize vuba ukoresheje urushinge rwasukuwe cyangwa imikasi isukuye.
• Koresha agapfundikizo kadafata. Ubundi buryo, shyiramo amavuta ya peteroli (Vaseline™) cyangwa amavuta yo kwisiga afasha uruhu (nka Aquaphor™) ku gipfundikizo kugira ngo kidafata ku ruhu.

Ni gute wakwita ku mwana ufite EB? Inama zimwe na zimwe ku babyeyi

Niba umwana wawe afite EB, ibyo akeneye bitandukanye n'iby'abandi. Bashobora kutabasha kugaragaza ko bamubangamiye. Izi nama zishobora gufasha umwana wawe kuguma amerewe neza uko bishoboka kose:

• Karaba intoki zawe neza mbere yo gukora ku ruhu rw'umwana wawe.
• Irinde gukoresha uturindantoki twa latex, kuko bishobora kongera ukubiri.
• Mu gihe woga umwana wawe, aho gushyira umubiri wose mu gikombe cyuzuye amazi icyarimwe, oga buri gice cy'uruhu kimwe kimwe.
• Aho gushyira umwana wawe mu matwi, tekereza kubishyiramo udutambaro duto two kumwotsa.
• Koresha udupira tw'ijisho dufite imishumi ya Velcro®. Imishumi/ikaseti ishobora gufata ku ruhu rw'umwana wawe mu buryo butaziguye.
• Kata hanyuma ukureho elasitiki aho amaguru y'ijipo afatanye.
• Shyira ikintu kimeze nk'impapuro za silikoni za gel imbere mu gitambaro kugira ngo uruhu rutaguma ku dusebe twavutse.
• Witondere cyane mu gihe uterura umwana.Irinde guterura ushyira ikiganza cyawe munsi y'aho afungiye (axilla). Niba umwana wawe afite udusebe cyangwa ibikomere ku bibero bye no mu mugongo, muzamure ukuboko kumwe ku bibero bye ukundi kuboko gushyigikiye umugongo we.
• Shishikariza umwana wawe gukora cyane uko uburwayi bwe bwa EB bubimwemerera. Kwicara utekanye bishobora gutera izindi ngorane, nko kubura impatwe no kugabanuka kw'imitsi. Koga ni imyitozo ngororamubiri myiza kandi idatera ingaruka mbi.
• Ntugashishikarize umwana wawe gukora imyitozo ngororamubiri iremereye cyangwa gukina imikino ishobora gutuma abira ibyuya cyangwa agashyuha.

Guhangana n'ibimenyetso by'umwana wawe rimwe na rimwe bishobora kugorana. Ushobora kumva uremerewe cyane kandi unaniwe. Saba muganga wawe inama nyinshi zo gutuma umwana wawe - nawe - uhora wumva umerewe neza uko bishoboka kose. Nanone, winjira mu matsinda y'abaterankunga b'ababyeyi , ushobora gusangira ubunararibonye bwawe no kwiga uburyo bushya bwo gucunga indwara ya EB y'umwana wawe.

Ese EB ishobora kwirindwa?

Kubera ko EB ari indwara y’uturemangingo, ntabwo ishobora kwirindwa . Abantu bafite amateka y’umuryango wabo ya EB kandi bateganya kubyara bashobora gusanga ubujyanama mu by’uturemangingo ari ingirakamaro cyane mu gufata icyemezo cy’uko bateganya umuryango wabo.

Byongeye kandi, impuguke muri iki gihe ntabwo ziramenya impamvu ya EB yabonetse. Kubwibyo, abaganga ntibazi uburyo bwo kuyirinda.

Ni ubuhe buryo bw'ejo hazaza umuntu ufite EB ashobora kwitega?

Ahazaza h'abantu bafite EB haterwa n'ubwoko bwa EB bafite n'ubukana bwayo. Ubwoko bukomeye bw'indwara bushobora gutera ububabare bukomeye, ubumuga, ubumuga, ibikomere bidakira, ndetse n'urupfu rukiri ruke .

Ariko, abaganga bashobora kugufasha guhangana n'ibimenyetso byawe. Ibi bishobora gukorwa hakoreshejwe uburyo bukwiye bwo kuvura, ndetse nibiba ngombwa, n'imiti igabanya ububabare. Gupfuka uruhu neza ukoresheje imyenda idafata, irinda buri munsi, kwiyuhagira kenshi, no kwita ku bikomere bishobora kugabanya ingaruka za EB ku buzima bwawe.

Niba ufite EB, ni ryari wagombye kujya kwa muganga?

Menya neza ko ugiye kwa muganga wawe muri ibi bihe:

• Niba ufite ikibazo cyo guhumeka.
• Niba ufite ikibazo cyo kumira ibiryo.
• Niba igisebe cyawe gisa n'icyanduye (uruhu ruhinduka umutuku, umuhengeri, imvi, cyangwa umweru; cyabyimbye cyangwa cyabyimbye).
• Iyo ibimenyetso bishya bigaragaye.

Ni ibihe bibazo by'ingenzi ugomba kubaza muganga?

Iyo ugiye kwa muganga, ni byiza kubaza ibibazo nk'ibi:

• Wamenya ute ko mfite EB?
• Niba ntarwaye EB, ni iyihe ndwara yindi y'uruhu narwara?
• Ni gute nakwitwara mu gihe mfite ibimenyetso?
• Ni iyihe miti usaba? Ese ifite ingaruka mbi?
• Ni ubuhe buryo bwo kuvura mu rugo usaba?
• Ni iki kindi nakora kugira ngo nongere ibimenyetso byanjye?
• Ese hari amavuta cyangwa amavuta ushobora kwandika?
• Ese nkwiye kubonana n'umuganga w'uruhu cyangwa undi muganga w'inzobere?

Ni irihe tandukaniro riri hagati ya epidermolysis bullosa (EB) na pemphigoid?

Indwara ya Bullous pemphigoid ni indwara idakunze kugaragara, itera uruhu kwibasira, nk'uduheri cyangwa uduheri twuzuye amazi. Ikunze kwibasira abantu barengeje imyaka 60 , kandi akenshi ishira mu myaka itanu. Ariko, mu bihe bikomeye, uduheri n'inkovu bishobora kwangiza ingingo z'imbere no kwica.

Nubwo rimwe na rimwe EB ishobora kuba indwara iterwa n’ubwiyongere bw’umubiri, akenshi iterwa n’impinduka mu miterere y’uturemangingo igira ingaruka kuri kolajeni mu ruhu rwawe. EB ikunze kugaragara mu bwana cyangwa mu bwana . Kubera ko nta muti wa EB uhari, ushobora kuba ufite ibimenyetso ubuzima bwawe bwose.

Amaherezo, ibintu byo kwibuka (Ubutumwa bwo kujyana mu rugo)

Epidermolysis bullosa (EB) rimwe na rimwe ni ikibazo kigoye. Ariko, iyo ugiriwe inama n'abaganga, ukavurwa, kandi ukabaho neza uko bishoboka kose, ushobora kwihanganira ibimenyetso no kubaho neza uko bishoboka kose. Wibuke ko utari wenyine. Abaganga, imiryango, n'amatsinda y'abaterankunga bose barahari kugira ngo bagufashe. Rero, ntutinye, komera, kandi uhangane n'iki kibazo.

Niba ufite ibindi bibazo kuri EB, menya neza ko wavugana n'umuganga w'uruhu.

Epidermolysis bullosa, EB, indwara y'uruhu, uduheri, indwara y'uturemangingo, indwara y'uruhu rw'abana, uruhu rudakora neza