Ingaba ulusu lwakho lunamaqhuqhuva kwaye luphuma nokuba kukuchukumisa nje okuncinci? Ngaba yi-Epidermolysis Bullosa (EB)?

Ngamanye amaxesha ulusu lwethu luba buthathaka kakhulu, akunjalo? Nokuba into encinci iyayibetha, okanye impahla ikrazukile, abanye abantu bafumana amadyungudyungu kunye nokwenzakala kolusu. Ingakumbi ekubeni ulusu lweentsana ezincinci lubuthathaka kakhulu, luyoyikisa kakhulu xa ubona izinto ezinjalo. Ngoko ke namhlanje siza kuthetha ngesifo esibakho kolusu olunjalo, esingaqhelekanga kodwa sibaluleke kakhulu ukusazi. Loo nto yi-epidermolysis bullosa, okanye i-EB ngamafutshane.

Yintoni kanye kanye i-epidermolysis bullosa (EB)?

Ngamafutshane, i-epidermolysis bullosa (EB) yimeko ezuzwe njengelifa . Ibangela ukuba ulusu lwakho lube buthathaka kakhulu kwaye lube namaqhubu kwaye luxobuke lula. Ngamanye amaxesha, nokuba uhlikihla ulusu lwakho ngempahla okanye ubetheke ndaweni ithile, unokuba namaqhubu kunye nezilonda. Kwabanye abantu, isenokuba buthathaka kwaye ibangele amaqhubu abuhlungu ezandleni, ezingqinibeni, emadolweni nasezinyaweni. Nangona kunjalo, kwiimeko ezinzima, la maqhubu kunye nezilonda zinokuvela naphi na emzimbeni. Iimpawu ze-EB zinokuqala ukusuka kwezincinci ukuya kwezinzima kakhulu .

Khumbula, ngamanye amaxesha la maqhuqhuva anokuvela emlonyeni, emqaleni (emqaleni), emehlweni, nakwezinye izitho zangaphakathi zomzimba. Xa la maqhuqhuva ephola, anokushiya amanxeba abuhlungu. Kwiimeko ezinzima, la maqhuqhuva kunye namanxeba anokonakalisa izitho zangaphakathi zomzimba, nto leyo enokuba yingozi ebomini.

Ziziphi iintlobo eziphambili ze-EB?

Kukho iintlobo ezine eziphambili ze-EB, ezichongwa ngumaleko wolusu oluchaphazelekileyo.

1. I-EB Simplex (EBS): Olu lolona hlobo luqhelekileyo lwe-EB. Ingahluka ukusuka kwencinci, ingabi buhlungu kakhulu, ukuya kwebuhlungu kakhulu, ibe buhlungu kakhulu, kwabanye abantu. Amaqhuqhuva akheka kumaleko ophezulu wolusu, i-epidermis . Amaqhuqhuva anqabile emva kokuba la maqhuqhuva epholile.

2. I-Junctional EB (JEB): Kwi-JEB, amadyungudyungu akheka emlonyeni nasemphefumlweni. Oku kunqabile , kwaye kunokuhluka ukusuka kokungakhululekiyo (okungakhululekiyo kancinci, okubuhlungu kancinci) ukuya kokuqatha.

3. I-Dystrophic EB (DEB): Kwi-DEB, amadyungudyungu akheka kumaleko ophakathi kwesikhumba, i-dermis . Oku kunokwahluka ukusuka kokuncinci ukuya kokuqatha.

4. I-Kindler Syndrome: Kwi-Kindler Syndrome, amadyungudyungu anokwakheka phantse kuzo zonke iileya zolusu. Oku kunqabile kakhulu .

Oogqirha bahlula i-EBS, i-JEB, kunye ne-DEB ngokusekwe kwiileya zolusu lwakho ezichaphazelekileyo. Kwi-Kindler syndrome, ama-blister anokuvela kwiileya ezahlukeneyo zolusu lwakho.

Ngubani ochaphazeleka kakhulu yile meko? Ixhaphake kangakanani?

I-EB inokuchaphazela nabani na. Abantu balo naluphi na uhlanga, uhlanga, okanye isini banokuba ne-EB. Nangona kunjalo,Ukuba omnye wabazali bakho unesi sifo, unokuba sengozini enkulu yokuba ne-EB.

Nangona ixhaphake kangaka, ngokwezibalo eMelika, malunga nomntu omnye kuphela kwabangama-50,000 onesifo se-EB. Oku kuthetha ukuba yimeko engaqhelekanga.

Ziziphi iziphumo ezinokuba nazo i-EB emzimbeni?

Kwiimeko ezinzima ze-EB, amehlo anokuba namadyungudyungu aze abangele nokulahlekelwa yimbono . Ngamanye amaxesha, ulusu kunye nezihlunu zinokuba namanxeba amaninzi, nto leyo eyenza kube nzima ukuhambisa iminwe, izandla, iinyawo kunye namalungu ngendlela efanelekileyo. Abanye abantu abane-EB banomngcipheko omkhulu wokuba nohlobo lomhlaza wesikhumba olubizwa ngokuba yi -squamous cell carcinoma .

Kwiimeko ezinzima kakhulu, ukufa ngamanye amaxesha kunokwenzeka ebuntwaneni . Oku kungenxa yeemeko ezifana nosulelo olunzima (umzekelo , i-sepsis ), ubunzima bokuphefumla ngenxa yokuvaleka kwendlela yomoya, ukuphelelwa ngamanzi emzimbeni, kunye nokungondleki kakuhle.

Ngaba i-EB ingabulala?

Oku kuxhomekeke ngokwenene kuhlobo lwe-EB onalo. Iintlobo ze-EB ezibuthathaka nezingenamandla kangako azibulali. Nangona kunjalo, ixesha lokuphila kwabantu abaneentlobo ze-EB ezinzima linokususela ebuntwaneni ukuya kwiminyaka engama-30 ubudala. Ngoko ke le yimeko efuna ingqalelo eninzi.

Zithini iimpawu ze-EB?

Iimpawu ze-EB ziyahluka ngokuxhomekeke kuhlobo. Ezi mpawu zihlala zibonakala xa usengumntwana okanye umntwana omncinci . Ezinye iimpawu ziqhelekile phakathi kweentlobo ezahlukeneyo. Nazi ezinye zeempawu eziphambili ze-EB:

• Amaqhuqhuva eluswini (izandla, iinyawo, iingqiniba, amadolo) okanye ngaphakathi emzimbeni.
• Ukuqina kolusu ezintendeni nasezinyaweni, okuzenza zivakale ngathi ziqinile.
• Ukuncipha kweeseli ezibomvu zegazi (i-Anemia).
• Iminwe inamathelene (iminwe okanye iinzwane ezidityanisiweyo).
• Iinzipho zeminwe nezeenyawo ezigoso okanye ezijiyileyo.
• Ukubonakala kwamaqhuqhuva amancinci amhlophe afana namaqhuqhuva (milia) eluswini.
• Ubunzima bokuginya ukutya (dysphagia).
• Ukungabikho kokukhula okulindelekileyo kumntwana.
• Amazinyo angaphuhli njengoko kulindelekile ( hypoplasia ).

Yintoni ebangela i-EB?

Eyona nto ibangela i-EB kukuguquka okanye isiphene kwenye yezakhi zofuzo ezili-18 . Abantu abanesi sifo banezakhi zofuzo ezilahlekileyo okanye ezonakeleyo ezinceda ekwenzeni iproteni ebizwa ngokuba yi-collagen . Njengoko usazi, i-collagen yiyo enika amandla kunye nolwakhiwo kwizicubu ezinxibelelanayo ezifana nolusu lwethu. Ngoko ke, ngenxa yesi siphene sezakhi zofuzo, iileya ezimbini zesikhumba, i-epidermis kunye ne-dermis, azidibani ngokuqhelekileyo. Ngenxa yoko, ulusu luba buthathaka kakhulu, lube namadyungudyungu lula, kwaye lonakale.

I-EB idla ngokuba sisifo esizuzwe njengelifa , oko kuthetha ukuba ukuba omnye wabazali unaso, abantwana banokuyizuza njengelifa.

Nangona kunjalo, kunqabile kakhulu, i-EB inokwenzeka njengesifo sokuzikhusela komzimba esifunyenweyo .

Eyona nto ibalulekileyo kukuba i-EB ayisosifo esosulelayo. Idla ngokuba sisifo sofuzo. Ngoko ke, ukuba udibana nomntu one-EB, awuyi kuba naso.

Ungazi njani ngokuqinisekileyo ukuba une-EB? (Ukuxilongwa)

Oogqirha banokuxilonga i-EB ngokwenza i-skin biopsy , equka ugqirha ukuthatha isampuli encinci yolusu aze ayihlole phantsi kwe-microscope.

Ukongeza, uvavanyo lwemfuza lunokuchonga i-gene enesiphene kwaye luqinisekise uhlobo lwe-EB. Abazali abaceba ukuba nomntwana banokuvavanywa i-genetic ngaphambi kokuzalwa ukuze kufunyaniswe ukuba basengozini yokuba nomntwana one-EB.

Ziziphi iindlela zonyango ze-EB?

Ngelishwa, okwangoku akukho nyango lwe-EB . Nangona kunjalo, unyango lunokunceda:

• Inceda ekuthinteleni amadyungudyungu.
• Ukunyamekela kakuhle amaqhuqhuva kunye nolusu kuthintela iingxaki.
• Inyanga iingxaki zesondlo ezinokubangelwa ngamadyungudyungu emlonyeni okanye emqaleni.
• Ukulawula iintlungu.

Ukuthintela ukonakala kolusu kunye nokwenziwa kwamadyungudyungu kunye nezilonda ngenxa yokungqubana, oogqirha bacebisa oku kulandelayo:

• Nxiba iimpahla ezithambileyo nezikhululekileyo ezenziwe ngeefayibha zendalo. Ukunxiba iimpahla ngaphakathi ngaphandle kunokunceda ukunciphisa inani lemithungo ekhuhla ulusu lwakho.
• Kuphephe ukutshisa kakhulu; gcina amagumbi kubushushu obufanelekileyo nobufanayo.
• Kuphephe ukuphuma elangeni, okanye unxibe i-sunscreen xa usebenzisa iiglasi zelanga kunye neminqwazi.
• Sebenzisa iintlobo ezikhethekileyo zeebhandishi ukukhusela ulusu - sebenzisa iibhandishi ezinganamatheliyo kunye neteyipu, i-gauze egoqiweyo, njl.

Ukuze unyange ama-blister, ugqirha wakho unokucebisa izinto ezifana nezi:

• Thambisa amafutha emanxebeni yonke imihla.
• Sebenzisa iibhandishi ezinonyango ukunceda amadyungudyungu aphole kwaye uthintele usulelo.
• Sela amayeza okulawula iintlungu.

Ukuze unyange izifo, ugqirha wakho unokucebisa oku kulandelayo:

• Sela amayeza okubulala iintsholongwane ngomlomo okanye uthambise ikhrimu yokubulala iintsholongwane.
• Sebenzisa izinto zokuvala amanxeba ezikhethekileyo kumanxeba angapholiyo.

Ukuthintela iingxaki zesondlo kunye nobunzima bokutya ngenxa yamadyungudyungu emlonyeni okanye emqaleni, ugqirha wakho unokucebisa oku kulandelayo:

• Xa utyisa umntwana wakho, sebenzisa ibhotile yomntwana ene-nipple ekhethekileyo .
• Mnike umntwana i-eyedropper okanye isirinji.
• Yongeza amanzi amancinci ekutyeni okucoliweyo ukuze ukunyibilikise uze ukuphe ukutya. Oku kuya kwenza kube lula ukuginya.
• Yitya ukutya okuthambileyo ngakumbi okufana nesuphu, ukutya okucoliweyo, ipudingi, kunye ne-applesauce .
• Ukutya kuphakelwa kubushushu obufudumeleyo (hhayi bushushu kakhulu).
• Bona ingcali yokutya ukuze uxoxe ngeemfuno zakho zesondlo.

Kwiimeko ezinzima ze-EB, ungadinga notyando . Ukuba i-esophagus (ityhubhu ethwala ukutya ukusuka emlonyeni ukuya esiswini) ivalwe ngama-blisters kunye namanxeba, utyando lunokwenziwa ukuze lunwenwe. Abanye abantu banokukwazi ukudlula i-esophagus ngokupheleleyo baze bafake ityhubhu yokondla ukuze bondle ukutya ngqo esiswini. Utyando lunokwenziwa ukuze kwahlulwe iminwe enamathelene ngenxa yama-blisters.

Umntu one-EB uzinyamekela njani?

Ukuba une-EB, la macebiso aya kukunceda uzinyamekele:

• Lala phezu kwamashiti kunye neekhabhathi zepillow ezenziwe ngeefayibha ezithambileyo, zendalo, ezifana nesilika okanye i-satin.
• Nxiba izihlangu ezikhululekileyo ezingawuxinanisi umzimba wakho.
• Kuphephe ukuma okanye ukuhamba ixesha elide.
• Kuphephe ukukrwela okanye ukukhaba ulusu. Faka amayeza okunciphisa ukurhawuzelela.
• Lumka kwindawo ekungqongileyo kwaye uphephe ukuchukumisa okanye ukukrwela ulusu lwakho ngempazamo.
• Soloko ufumisa ulusu lwakho ukuze unciphise ukungqubana.
• Ukuba kuvela amadyungudyungu, wafake ngokukhawuleza ngenaliti ecociweyo okanye isikere esicocekileyo.
• Sebenzisa ibhandeji elinganamatheliyo. Okanye, faka ijeli yepetroleum (iVaseline™) okanye ioyile elungele ulusu (efana neAquaphor™) kwibhandeji ukuze inganamatheli eluswini.

Ungamnyamekela njani umntwana one-EB? Amanye amacebiso kubazali

Ukuba umntwana wakho une-EB, iimfuno zakhe zahlukile kwezabanye. Basenokungakwazi ukuchaza ukungakhululeki kwakhe. Ezi ngcebiso zinokunceda umntwana wakho ahlale ekhululekile kangangoko kunokwenzeka:

• Hlamba izandla zakho kakuhle ngaphambi kokuba uchukumise ulusu lomntwana wakho.
• Kuphephe ukusebenzisa iiglavu zelatex, kuba zinokwandisa ukungqubana.
• Xa uhlamba umntwana wakho, endaweni yokufaka umzimba wonke esityeni esizaliswe ngamanzi ngaxeshanye, hlamba lonke ulusu ngalunye ngexesha.
• Endaweni yokufaka umntwana wakho osandul’ ukuzalwa kwiinabukeni, cinga ngokubeka ii-pads zokufunxa phezu kwazo.
• Sebenzisa ii-diapers ezinee-straps zeVelcro®. Ii-straps/iteyipu ezincamathelayo zinokunamathela eluswini lomntwana wakho ngempazamo.
• Sika uze ususe ilastiki apho imilenze ye-napkin incamathele khona.
• Beka into efana namaphepha e-silicone gel ngaphakathi kwi-napkin ukuthintela ulusu ukuba lunganamatheli kwi-blisters esele yenziwe.
• Lumka kakhulu xa uphakamisa umntwana.Kuphephe ukuphakamisa isandla sakho ngokubeka isandla sakho phantsi kwekhwapha (i-axilla). Ukuba umntwana wakho unamaqhubu okanye amanxeba emathangeni nasemqolo, mphakamise ngesandla esinye sijikeleze amathanga akhe kwaye esinye isandla sixhase umqolo wakhe.
• Khuthaza umntwana wakho ukuba asebenze kangangoko imeko yakhe ye-EB ivuma. Ukuhlala ungashukumi kunokubangela ezinye iingxaki, ezinje ngokuqunjelwa kunye nokuwohloka kwemisipha. Ukuqubha ngumthambo olungileyo nongenampembelelo ingako.
• Musa ukukhuthaza imisebenzi enzima yomzimba okanye imidlalo eya kwenza umntwana wakho abile okanye ashushu.

Ukujongana neempawu zomntwana wakho kunokuba nzima ngamanye amaxesha. Ungaziva uxinezelekile kwaye udiniwe. Cela ugqirha wakho ukuba akunike amanye amacebiso okugcina umntwana wakho - kunye nawe - ukhululekile kangangoko kunokwenzeka. Kwakhona, ngokujoyina amaqela enkxaso yabazali , ungabelana ngamava akho kwaye ufunde iindlela ezintsha zokulawula imeko ye-EB yomntwana wakho.

Ngaba i-EB ingathintelwa?

Ngenxa yokuba i-EB ibangelwa lufuzo, ayinakuthintelwa . Abantu abanembali yentsapho ye-EB kwaye baceba ukuba nabantwana banokufumanisa ukuba iingcebiso ngefuzo ziluncedo kakhulu ekugqibeni indlela yokucwangcisa usapho lwabo.

Ukongeza, iingcali azikazi ukuba yintoni ebangela uhlobo lwe-EB olufunyenweyo. Ngenxa yoko, oogqirha abazi ukuba bangayithintela njani.

Luhlobo luni lwekamva umntu one-EB anokulilindela?

Ikamva labantu abane-EB lixhomekeke kuhlobo lwe-EB abanalo kunye nobunzima bayo. Iintlobo ezinzima zesifo zinokubangela iintlungu ezinzima, ukukhubazeka, ukukhubazeka, amanxeba angapholiyo, kwanokufa kwangethuba .

Nangona kunjalo, oogqirha banokukunceda ukulawula iimpawu zakho. Oku kungenziwa ngonyango olufanelekileyo, kwaye ukuba kuyimfuneko, amayeza eentlungu. Ukugcina ulusu lugqunywe kakuhle ngeebhandeji ezinganamathelisiyo, ezikhuselayo yonke imihla, ukuhlamba rhoqo, kunye nokunyamekela amanxeba kunokunceda ukunciphisa impembelelo ye-EB ebomini bakho.

Ukuba une-EB, kufuneka uye kugqirha nini?

Qiniseka ukuba ubona ugqirha wakho kwezi meko zilandelayo:

• Ukuba unengxaki yokuphefumla.
• Ukuba unengxaki yokuginya ukutya.
• Ukuba inxeba lakho libonakala linesifo (ulusu luba bomvu, mfusa, ngwevu, okanye mhlophe; livuvukile okanye lidumbile).
• Ukuba kuvela iimpawu ezintsha.

Yeyiphi imibuzo ebalulekileyo ekufuneka uyibuze ugqirha?

Xa usiya kugqirha, kungcono ukubuza imibuzo efana nale:

• Ungazibona njani ukuba ndine-EB?
• Ukuba andinayo i-EB, sesiphi esinye isifo solusu endinokuba naso?
• Ndingazilawula njani iimpawu zam?
• Ngawaphi amayeza owacebisayo? Ngaba aneziphumo ebezingalindelekanga?
• Zeziphi iindlela zonyango zasekhaya ozicebisayo?
• Yintoni enye endifanele ndiyenze ukuphucula iimpawu zam?
• Ngaba ikho ikhrimu okanye amafutha onokuyinika?
• Ngaba kufuneka ndidibane nogqirha wesikhumba okanye omnye umntu ochwephesha?

Yintoni umahluko phakathi kwe-epidermolysis bullosa (EB) kunye ne-bullous pemphigoid?

I-Bullous pemphigoid sisifo esingaqhelekanga, esihlasela ulusu ngokwasemzimbeni. Sinokubangela ukurhawuzelelwa, ukudumba okufana ne-hive okanye amaqhuqhuva azele lulwelo. I-Bullous pemphigoid idla ngokuchaphazela abantu abangaphezu kweminyaka engama-60 ubudala , kwaye idla ngokunyamalala kwisithuba seminyaka emihlanu. Nangona kunjalo, kwiimeko ezinzima, amaqhuqhuva kunye namaqhuqhuva anokonakalisa amalungu angaphakathi kwaye abulale.

Nangona i-EB ngamanye amaxesha inokuba sisifo esibangelwa kukuzikhusela komzimba, idla ngokubangelwa kukuguquka kwezakhi zofuzo okuchaphazela i-collagen eluswini lwakho. I-EB idla ngokuvela ebuntwaneni okanye ebuntwaneni . Ekubeni kungekho nyango lwe-EB, kunokufuneka uphile neempawu ubomi bakho bonke.

Okokugqibela, izinto ekufuneka uzikhumbule (Umyalezo Wokuya Ekhaya)

I-Epidermolysis bullosa (EB) yimeko enzima ngamanye amaxesha. Nangona kunjalo, ngeengcebiso zonyango ezifanelekileyo, unyango, kunye nenkathalo yothando, unokulawula iimpawu kwaye uphile kakuhle kangangoko unako. Khumbula, awuwedwa. Oogqirha, usapho, kunye namaqela enkxaso bonke bakhona ukukunceda. Ngoko ke, ungoyiki, hlala uqinile, kwaye ujongane nale meko.

Ukuba unemibuzo engaphezulu malunga ne-EB, qiniseka ukuba uthethe nogqirha wesikhumba.

I- Epidermolysis bullosa, i-EB, isifo solusu, amadyungudyungu, isifo sofuzo, isifo solusu lobuntwana, ubuthathaka bolusu