Ingabe isikhumba sakho siyaqhuma futhi siphuma ngisho noma sithinteke kancane? Ingabe kungaba yi-Epidermolysis Bullosa (EB)?

Ngezinye izikhathi isikhumba sethu siyazwela kakhulu, akunjalo? Ngisho noma into encane isishaya, noma ingubo idabuke, abanye abantu bathola kalula amabhamuza nokulimala kwesikhumba. Ikakhulukazi njengoba isikhumba sezingane ezincane sibucayi kakhulu, kuzwakala kusabeka kakhulu uma ubona izinto ezinjalo. Ngakho-ke namuhla sizokhuluma ngesifo esenzeka esikhumbeni esinjalo, okuyinto engavamile kodwa ebaluleke kakhulu ukusazi. Leso yi-epidermolysis bullosa, noma i-EB ngamafuphi.

Iyini ngempela i-epidermolysis bullosa (EB)?

Kalula nje, i-epidermolysis bullosa (EB) yisimo esizuzwe njengefa . Sibangela ukuba isikhumba sakho sibe buthaka kakhulu futhi sibe namaqhubu futhi sihlubuke kalula. Ngezinye izikhathi, noma ngabe uhlikihla isikhumba sakho ngezingubo noma ushayeka ndawana thize, ungaba namaqhubu nezilonda. Kwabanye abantu, kungaba mnene futhi kubangele amaqhubu abuhlungu ezandleni, ezindololwaneni, emadolweni nasezinyaweni. Kodwa-ke, ezimweni ezimbi kakhulu, la maqhubu nezilonda zingavela noma kuphi emzimbeni. Izimpawu ze-EB zingasukela kwezincane kuya kwezinzima kakhulu .

Khumbula, ngezinye izikhathi la maqhubu angakhula emlonyeni, emphinjeni (emphinjeni), emehlweni, nakwezinye izitho zangaphakathi zomzimba. Uma la maqhubu ephola, angashiya izibazi ezibuhlungu. Ezimweni ezimbi kakhulu, la maqhubu nezibazi kungalimaza izitho zangaphakathi zomzimba, okungaba yingozi empilweni.

Yiziphi izinhlobo eziyinhloko ze-EB?

Kunezinhlobo ezine eziyinhloko ze-EB, ezibonakala ngesendlalelo sesikhumba esithintekile.

1. I-EB Simplex (EBS): Lolu uhlobo oluvame kakhulu lwe-EB. Lungasukela koluncane, olungabuhlungu kakhulu, luye kolubuhlungu kakhulu, olunzima, kwabanye abantu. Ama-blister akheka engxenyeni ephezulu yesikhumba, i-epidermis . Amanxeba awavamile ngemva kokuba la ma-blister epholile.

2. I-Junctional EB (JEB): Ku-JEB, amabhamuza akheka emlonyeni nasemgudwini womoya. Lokhu akuvamile , futhi kungasukela kokuncane (okungakhululeki kancane, okubuhlungu kancane) kuya kokukhulu.

3. I-Dystrophic EB (DEB): Ku-DEB, ama-blister akha ungqimba oluphakathi lwesikhumba, i-dermis . Lokhu kungahluka kusukela kokuncane kuya kokukhulu.

4. I-Kindler Syndrome: Ku-Kindler Syndrome, amabhamuza angakheka cishe kuzo zonke izingqimba zesikhumba. Lokhu akuvamile kakhulu .

Odokotela bahlukanisa i-EBS, i-JEB, ne-DEB ngokusekelwe ezingqimbeni zesikhumba sakho ezithintekile. Ku-Kindler syndrome, ama-blister angavela kuzo zonke izingqimba zesikhumba sakho.

Ubani othinteka kakhulu yilesi simo? Sivame kangakanani?

I-EB ingathinta noma ubani. Abantu banoma yiluphi uhlanga, ubuzwe, noma ubulili bangaba ne-EB. Kodwa-ke,Uma omunye wabazali bakho enalesi sifo, kungenzeka kakhulu ukuthi ube ne-EB.

Nakuba kuvame kangaka, ngokwezibalo e-United States, cishe umuntu oyedwa kwabangu-50,000 unesifo se-EB. Lokhu kusho ukuthi kuyisimo esingavamile.

Yimiphi imiphumela engaba nayo i-EB emzimbeni?

Ezimweni ezimbi kakhulu ze-EB, amehlo angaqhuma futhi abangele nokulahlekelwa umbono . Ngezinye izikhathi, kungaba nezibazi ezinkulu kanye nokukhubazeka kwesikhumba kanye nemisipha, okwenza kube nzima ukuhambisa iminwe, izandla, izinyawo kanye namalunga kahle. Abanye abantu abane-EB banengozi enkulu yokuba nohlobo lomdlavuza wesikhumba olubizwa ngokuthi i-squamous cell carcinoma .

Ezimweni ezimbi kakhulu, ukufa kungenzeka ngezinye izikhathi ebuntwaneni . Lokhu kungenxa yezimo ezifana nokutheleleka okukhulu (isibonelo , i-sepsis ), ubunzima bokuphefumula ngenxa yokuvaleka komoya, ukuphelelwa amanzi emzimbeni, kanye nokungondleki kahle.

Ingabe i-EB ingaba yingozi?

Lokhu kuncike kakhulu ohlotsheni lwe-EB onalo. Izinhlobo ze-EB ezithambile nezingenamandla kangako azibulali. Kodwa-ke, isikhathi sokuphila sabantu abanezinhlobo ze-EB ezinzima singasukela ebuntwaneni kuya eminyakeni engaba ngu-30 ubudala. Ngakho-ke lesi yisimo esidinga ukunakwa okukhulu.

Ziyini izimpawu ze-EB?

Izimpawu ze-EB ziyahlukahluka kuye ngohlobo. Lezi zimpawu zivame ukuvela uma usengumntwana noma usengumntwana omncane . Ezinye izimpawu zivamile phakathi kwezinhlobo ezahlukene. Nazi ezinye zezimpawu eziyinhloko ze-EB:

• Amaqhubu esikhumbeni (ezandleni, ezinyaweni, ezindololwaneni, emadolweni) noma ngaphakathi emzimbeni.
• Ukuqina kwesikhumba ezintendeni nasematheni, kuzwakala sengathi kuyisicaba.
• Ukwehla kwamangqamuzana abomvu egazi (i-Anemia).
• Iminwe inamathelana (iminwe noma izinzwane ezihlangene).
• Izinzipho zeminwe nezinzwane ezigugile noma ezijiyile.
• Ukuvela kwamaqhubu amancane amhlophe afana nama-blister (milia) esikhumbeni.
• Ubunzima bokugwinya ukudla (i-dysphagia).
• Ukuntuleka kokukhula okulindelekile enganeni.
• Amazinyo angakhuli njengoba kulindelekile ( hypoplasia ).

Yini ebangela i-EB?

Imbangela eyinhloko ye-EB ukuguquka kwezakhi zofuzo noma isici kwelinye lama-gene angu-18 . Abantu abanalesi sifo bane-gene engekho noma eyonakele esiza ekwakheni iphrotheni ebizwa ngokuthi i-collagen . Njengoba wazi, i-collagen yiyo enikeza amandla nesakhiwo ezicutshini ezixhumene njengesikhumba sethu. Ngakho-ke, ngenxa yalesi sici sezakhi zofuzo, izendlalelo ezimbili zesikhumba, i-epidermis kanye ne-dermis, azihlangani ndawonye ngokujwayelekile. Ngenxa yalokho, isikhumba siba buthakathaka kakhulu, siphuke kalula, futhi silimale.

I-EB ivame ukuba yisifo esizuzwe njengefa , okusho ukuthi uma omunye wabazali enaso, izingane zingasizuza njengefa.

Kodwa-ke, akuvamile kakhulu, i-EB ingavela njengesifo sokuzivikela komzimba esitholwe .

Into ebaluleke kakhulu ukuthi i-EB ayisona isifo esithathelwanayo. Ngokuvamile siwufuzo. Ngakho-ke, uma uthintana nomuntu one-EB, ngeke uyithole.

Wazi kanjani ukuthi une-EB? (Ukuxilongwa)

Odokotela bangaxilonga i-EB ngokwenza i-skin biopsy , okuhilela udokotela ukuthatha isampula encane yesikhumba bese eyihlola ngaphansi kwe-microscope.

Ngaphezu kwalokho, ukuhlolwa kwezakhi zofuzo kungahlonza isakhi sofuzo esinephutha futhi kuqinisekise uhlobo lwe-EB. Abazali abahlela ukuba nomntwana bangahlolwa futhi izakhi zofuzo ngaphambi kokubeletha ukuze bathole ukuthi basengozini yini yokuba nomntwana one-EB.

Yiziphi izindlela zokwelapha i-EB?

Ngeshwa, okwamanje akukho ukwelashwa kwe-EB . Kodwa-ke, ukwelashwa kungasiza:

• Kusiza ukuvimbela amabhamuza.
• Ukunakekela kahle amabhamuza nesikhumba kuvimbela izinkinga.
• Yelapha izinkinga zokudla okunempilo ezingase zibangelwe amabhamuza emlonyeni noma emphinjeni.
• Ukulawula ubuhlungu.

Ukuze kuvinjelwe ukulimala kwesikhumba kanye nokwakheka kwama-blister nezilonda ngenxa yokungqubuzana, odokotela batusa okulandelayo:

• Gqoka izingubo ezithambile nezikhululekile ezenziwe ngemicu yemvelo. Ukugqoka izingubo ngaphakathi nangaphandle kungasiza ekunciphiseni inani lemithungo ekhuhla isikhumba sakho.
• Gwema ukushisa ngokweqile; gcina amakamelo ekushiseni okukhululekile nokungaguquguquki.
• Gwema ukuphuma elangeni, noma ugqoke isivikelo selanga ngenkathi usebenzisa izibuko zelanga nezigqoko.
• Sebenzisa izinhlobo ezikhethekile zamabhandeji ukuvikela isikhumba - sebenzisa amabhandeji anganamatheli kanye netheyiphu, i-gauze egoqiwe, njll.

Ukwelapha ama-blister, udokotela wakho angase ancome izinto ezifana nalezi:

• Faka amafutha okugcoba emanxebeni nsuku zonke.
• Sebenzisa amabhandeshi anemithi ukusiza amabhamuza ukuba aphole futhi avimbele ukutheleleka.
• Thatha imithi yokulawula ubuhlungu.

Ukuze welaphe izifo, udokotela wakho angase akuncome okulandelayo:

• Thatha ama-antibiotic ngomlomo noma ufake ukhilimu wama-antibiotic.
• Sebenzisa izembozo ezikhethekile zamanxeba lapho kutholakala khona amanxeba angapholi.

Ukuze uvimbele izinkinga zokudla okunempilo kanye nobunzima bokudla ngenxa yamathumba emlonyeni noma emphinjeni, udokotela wakho angase akuncome okulandelayo:

• Uma uncelisa ingane yakho, sebenzisa ibhodlela lengane eline-nipple ekhethekile .
• Ncedisa ingane nge-eyedropper noma ngesirinji.
• Faka amanzi amancane ekudleni okucoliwe ukuze ukuncibilikise ngaphambi kokukuphakela. Lokhu kuzokwenza kube lula ukugwinya.
• Yidla ukudla okuthambile okwengeziwe njengesobho, ukudla okucoliwe, i-pudding, kanye ne-applesauce .
• Ukudla kuphakelwa ekushiseni okufudumele (hhayi okushisa kakhulu).
• Bonana nochwepheshe wezokudla ukuze uxoxe ngezidingo zakho zokudla okunempilo.

Ezimweni ezimbi kakhulu ze-EB, ungase udinge nokuhlinzwa . Uma i-esophagus (ithubhu elithwala ukudla kusuka emlonyeni kuya esiswini) ivinjiwe amabhamuza nezibazi, ukuhlinzwa kungenziwa ukuze kwandiswe. Abanye abantu bangase bakwazi ukudlula i-esophagus ngokuphelele bese befaka ithubhu lokuncelisa ukuze banike ukudla ngqo esiswini. Ukuhlinzwa kungenziwa futhi ukuze kuhlukaniswe iminwe enamathele ndawonye ngenxa yamabhamuza.

Umuntu one-EB uzinakekela kanjani?

Uma une-EB, lawa macebiso azokusiza ukuthi uzinakekele:

• Lala emashidini nasezicabheni zemicamelo ezenziwe ngemicu ethambile, yemvelo, njengesilika noma i-satin.
• Gqoka izicathulo ezikhululekile ezingakuvimbeli umzimba wakho.
• Gwema ukuma noma ukuhamba isikhathi eside.
• Gwema ukuklwebha noma ukukhahlela isikhumba. Faka imithi yokulwa nokuklwebheka ukuze unciphise ukuklwebheka.
• Qaphela indawo okuyo futhi ugweme ukuthinta noma ukuklwebha isikhumba sakho ngengozi.
• Njalo faka umswakama esikhumbeni sakho ukuze unciphise ukukhuhlana.
• Uma kuvela amabhamuza, wafake ngokushesha ngenaliti ehlanziwe noma isikele esihlanzekile.
• Sebenzisa ibhandishi elinganamatheli. Ngaphandle kwalokho, faka i-petroleum jelly (i-Vaseline™) noma amafutha alungele isikhumba (njenge-Aquaphor™) ebhandishini ukuze lingayinamatheli esikhumbeni.

Ungayinakekela kanjani ingane ene-EB? Amanye amathiphu kubazali

Uma ingane yakho ine-EB, izidingo zayo zihlukile kwezabanye. Kungenzeka ingakwazi ukuveza ukungakhululeki kwayo. Lawa macebiso angasiza ingane yakho ihlale ikhululekile ngangokunokwenzeka:

• Geza izandla zakho kahle ngaphambi kokuthinta isikhumba somntwana wakho.
• Gwema ukusebenzisa amagilavu ​​​​e-latex, ngoba angandisa ukungqubuzana.
• Uma ugeza ingane yakho, esikhundleni sokufaka umzimba wonke esitsheni esigcwele amanzi ngesikhathi esisodwa, geza ingxenye ngayinye yesikhumba ngasinye ngesikhathi.
• Esikhundleni sokufaka usana lwakho olusanda kuzalwa kuma-nappies, cabanga ngokubeka ama-pad okumunca kuwo.
• Sebenzisa amanabukeni anezintambo zeVelcro®. Izintambo/itheyiphu enamathelayo inganamathela esikhumbeni somntwana wakho ngengozi.
• Sika bese ususa i-elastic lapho imilenze ye-napkin inamathele khona.
• Beka okuthile okufana namashidi ejeli e-silicone ngaphakathi kwe-napkin ukuvimbela isikhumba ukuthi singanamatheli emaqabungeni asele ekhona.
• Qaphela kakhulu lapho uphakamisa ingane.Gwema ukuphakamisa ngokubeka izandla zakho ngaphansi kwamakhwapha (i-axilla). Uma ingane yakho inamaqhubu noma amanxeba emathangeni nasemhlane, yiphakamise ngesandla esisodwa sizungeze amathanga ayo kanti esinye isandla sisekele umhlane wayo.
• Khuthaza ingane yakho ukuba isebenze ngangokunokwenzeka njengoba isimo sayo se-EB sivuma. Ukuhlala uthule kungaholela kwezinye izinkinga, njengokuqunjelwa kanye nokuwohloka kwemisipha. Ukubhukuda kuyindlela enhle nengakhiphi mthelela.
• Ungakhuthazi imisebenzi enzima yomzimba noma imidlalo ezokwenza ingane yakho ijuluke noma ishise.

Ukubhekana nezimpawu zengane yakho kungaba nzima ngezinye izikhathi. Ungase uzizwe ukhungathekile futhi ukhathele. Cela udokotela wakho amathiphu engeziwe okugcina ingane yakho - kanye nawe - nikhululekile ngangokunokwenzeka. Futhi, ngokujoyina amaqembu okusekela abazali , ungabelana ngolwazi lwakho futhi ufunde izindlela ezintsha zokuphatha isimo se-EB sengane yakho.

Ingabe i-EB ingavinjelwa?

Ngenxa yokuthi i-EB iwufuzo, ayinakuvinjelwa . Abantu abanomlando womndeni we-EB futhi abahlela ukuba nezingane bangathola ukwelulekwa ngofuzo kuwusizo kakhulu ekunqumeni ukuthi bangawuhlela kanjani umndeni wabo.

Ngaphezu kwalokho, ochwepheshe okwamanje abazi ukuthi yini ebangela uhlobo lwe-EB olutholakele. Ngakho-ke, odokotela abazi ukuthi bangaluvimbela kanjani.

Hlobo luni lwekusasa umuntu one-EB angalulindela?

Ikusasa labantu abane-EB lincike ohlotsheni lwe-EB abanalo kanye nobunzima balo. Izinhlobo ezinzima zalesi sifo zingabangela ubuhlungu obukhulu, ukukhubazeka, ukukhubazeka, amanxeba angapheli, ngisho nokufa kusenesikhathi .

Kodwa-ke, odokotela bangakusiza ukuphatha izimpawu zakho. Lokhu kungenziwa ngokwelashwa okufanele, futhi uma kudingeka, ngemithi yobuhlungu. Ukugcina isikhumba simbozwe kahle ngamabhandeshi angenamathelisi, avikelayo njalo ngemva kosuku olulodwa, ukugeza njalo, nokunakekelwa kwamanxeba kungasiza ekunciphiseni umthelela we-EB empilweni yakho.

Uma une-EB, kufanele ubonane nodokotela nini?

Qiniseka ukuthi ubona udokotela wakho kulezi zimo:

• Uma unenkinga yokuphefumula.
• Uma unenkinga yokugwinya ukudla.
• Uma inxeba lakho libonakala lithelelekile (isikhumba siphenduka sibe bomvu, sibe nsomi, sibe mpunga, noma sibe mhlophe; sivuvukele noma sivuvukele).
• Uma kuvela izimpawu ezintsha.

Yimiphi imibuzo ebalulekile okufanele uyibuze udokotela?

Uma uya kudokotela, kuwumqondo omuhle ukubuza imibuzo efana nale:

• Ungabona kanjani ukuthi ngine-EB?
• Uma ngingenayo i-EB, yisiphi esinye isifo sesikhumba engingaba naso?
• Ngingazilawula kanjani izimpawu zami?
• Yimiphi imithi oyincomayo? Ingabe inemiphumela emibi?
• Yikuphi ukwelashwa kwasekhaya okuncomayo?
• Yini enye okufanele ngiyenze ukuze ngithuthukise izimpawu zami?
• Ingabe kukhona ukhilimu noma amafutha ongawasebenzisa?
• Ingabe kufanele ngibone udokotela wesikhumba noma omunye uchwepheshe?

Uyini umehluko phakathi kwe-epidermolysis bullosa (EB) ne-bullous pemphigoid?

I-bullous pemphigoid yisifo sesikhumba esingavamile, esingelapheki. Singabangela ukulunywa, ukuqubuka okufana ne-hive noma ama-blister agcwele uketshezi. I-bullous pemphigoid ivame ukuhlasela abantu abangaphezu kweminyaka engama-60 , futhi ivame ukuphela phakathi neminyaka emihlanu. Kodwa-ke, ezimweni ezimbi kakhulu, ama-blister kanye nezibazi kungalimaza izitho zangaphakathi futhi kubulale.

Nakuba i-EB ngezinye izikhathi ingaba yisifo sokuzivikela komzimba, imvamisa ibangelwa ukuguquka kwezakhi zofuzo okuthinta i-collagen esikhumbeni sakho. I-EB ivame ukuvela ebuntwaneni noma ebuntwaneni . Njengoba kungekho ikhambi le-EB, kungase kudingeke uphile nezimpawu impilo yakho yonke.

Okokugcina, izinto okufanele uzikhumbule (Umyalezo Wokuya Ekhaya)

I-Epidermolysis bullosa (EB) iyisimo esiyinselele ngezinye izikhathi. Kodwa-ke, ngeseluleko esifanele sezokwelapha, ukwelashwa, kanye nokunakekelwa ngothando, ungakwazi ukulawula izimpawu futhi uphile kahle ngangokunokwenzeka. Khumbula, awuwedwa. Odokotela, imindeni, kanye namaqembu okusekelana bonke bakhona ukukusiza. Ngakho-ke, ungesabi, hlala uqinile, futhi ubhekane nalesi simo.

Uma uneminye imibuzo mayelana ne-EB, qiniseka ukuthi ukhuluma nodokotela wesikhumba.

I- Epidermolysis bullosa, i-EB, isifo sesikhumba, amabhamuza, isifo sofuzo, isifo sesikhumba sobuntwana, ubuthakathaka besikhumba