Epidermolysis bullosa

I-Epidermolysis bullosa (i-EB) liqela lokuphazamiseka apho i-blisters yesikhumba ifom emva kokulimala okuncinci. Iyadluliselwa kwiintsapho.

Zine iindidi eziphambili ze-EB. Bazi:

• I-Dystrophic epidermolysis bullosa
• Epidermolysis bullosa simplex
• IHemidesmosomal epidermolysis bullosa
• Ukudibana kwe-epidermolysis bullosa

Olunye uhlobo olunqabileyo lwe-EB lubizwa ngokuba yi-epidermolysis bullosa acquisita. Le fomu ikhula emva kokuzalwa. Sisifo esingumzimba, esenza ukuba umzimba uhlasele ngokwawo.

I-EB ingahluka ukusuka kwincinci ukuya ekubulaleni. Ifom encinci ibangela ukudumba kolusu. Ifom ebulalayo ichaphazela amanye amalungu. Uninzi lweentlobo zale meko ziqala ekuzalweni okanye kungekudala emva koko. Kunganzima ukubona uhlobo oluchanekileyo lwe-EB umntu anayo, nangona iimpawu ezithile zemfuza ngoku ziyafumaneka uninzi.

Imbali yosapho ibubungozi. Umngcipheko uphezulu ukuba umzali unale meko.

Ngokuxhomekeke kwifom ye-EB, iimpawu zingabandakanya:

• I-Alopecia (ilahleko yeenwele)
• Amadyungudyungu ajikeleze amehlo kunye nempumlo
• Amadyungudyungu ngaphakathi okanye ngeenxa zonke emlonyeni nasemqaleni, ebangela iingxaki zokondla okanye ukugwinya ubunzima
• Amadyungudyungu kulusu ngenxa yokulimala kancinci okanye utshintsho lobushushu, ngakumbi iinyawo
• Ukuqhaqhaza okukhoyo ekuzalweni
• Iingxaki zamazinyo ezinjengokubola kwamazinyo
• Ukukhala, ukukhwehlela, okanye ezinye iingxaki zokuphefumla
• Amabhampa amhlophe amancinci kulusu olonzakeleyo ngaphambili
• Ukuphulukana nesipikili okanye iinzipho ezonakeleyo

Umboneleli wakho wezempilo uya kujonga ulusu lwakho ukufumanisa i-EB.

Uvavanyo olusetyenziselwa ukuqinisekisa ukuxilongwa lubandakanya:

• Uvavanyo lwemfuzo
• Ulusu lwe-biopsy
• Uvavanyo olukhethekileyo lweesampulu zolusu phantsi kwemicroscope

Uvavanyo lolusu lungasetyenziselwa ukuchonga uhlobo lwe-EB.

Olunye uvavanyo olunokwenziwa lunokubandakanya:

• Uvavanyo lwegazi lwe-anemia
• Inkcubeko yokujonga ukuba asosulelanga na intsholongwane
• I-endoscopy ephezulu okanye uthotho oluphezulu lwe-GI ukuba iimpawu zibandakanya ukugwinya iingxaki

Izinga lokukhula liya kuhlolisiswa rhoqo kwintsana ene-EB okanye ingaba nayo.

Injongo yonyango kukuthintela i-blisters ekwakheni nasekuphepheni iingxaki. Olunye unyango luya kuxhomekeka ekubeni imeko imbi kangakanani.

UKUNAKEKELWA KWASEKHAYA

Landela le migaqo ekhaya:

• Khathalela kakuhle ulusu lwakho ukuthintela usulelo.
• Landela ingcebiso yomboneleli wakho ukuba iindawo ezineeblisti ziyaqhekeka okanye ziluhlaza. Unokufuna unyango lwe-whirlpool rhoqo kwaye usebenzise i-ointotic ointments kwiindawo ezinjengezilonda. Umboneleli wakho uya kukwazisa ukuba ufuna i-bandage okanye unxibe, kwaye ukuba kunjalo, loluphi uhlobo onokulusebenzisa.
• Kuya kufuneka usebenzise amayeza e-steroid yomlomo ixesha elifutshane ukuba uneengxaki zokuginya. Kuya kufuneka uthathe amayeza ukuba ufumana usulelo lwe-candida (igwele) emlonyeni okanye emqaleni.
• Khathalela impilo yakho yomlomo kwaye ufumane ukuhlolwa kwamazinyo rhoqo. Kungcono ukubona ugqirha wamazinyo onamava okunyanga abantu abane-EB.
• Yitya ukutya okusempilweni. Xa unokwenzakala okuninzi kolusu, unokufuna iikhalori ezingaphezulu kunye neeproteni zokunceda ulusu lwakho luphole. Khetha ukutya okuthambileyo kwaye uphephe amandongomane, iitshiphusi, kunye nokunye ukutya okune crunchy ukuba unezilonda emlonyeni. Ingcali yesondlo inokukunceda ekutyeni okutyayo.
• Ngaba ukwenza umthambo i-Therapical Therapist ikubonisa ukuba uncede ukugcina amalungu akho kunye nezihlunu ezihambayo.

UKUGQIBELA

Utyando lokunyanga le meko lunokubandakanya:

• Ukumiliselwa kwesikhumba kwiindawo apho zinzulu izilonda
• Ukudityaniswa (ukwandiswa) komqala ukuba kukho ukuncipha
• Ukulungiswa kweziphene zezandla
• Ukususwa kwayo nayiphi na squamous cell carcinoma (uhlobo lomhlaza wolusu) okhulayo

OLUNYE UNYANGO

Olunye unyango lwale meko lunokubandakanya:

• Amayeza acinezela amajoni omzimba anokusetyenziselwa uhlobo oluzimeleyo lwale meko.
• Iiproteni kunye nonyango lwe-gene kunye nokusetyenziswa kwe-interferon yeziyobisi kuyafundwa.

Imbonakalo ixhomekeke kubuzaza besi sifo.

Usulelo lweendawo ezinamadyungu liqhelekile.

Iifom ezinobumnene ze-EB ziphucula kunye nobudala. Iifom ezinzulu kakhulu ze-EB zinezinga eliphezulu kakhulu lokufa.

Kwiifom ezinzima, ukuqhekeka emva kwefom blisters kunokubangela:

• Ukukhubazeka kwekhontrakthi (umzekelo, kwiminwe, iingqiniba, kunye namadolo) kunye nezinye iziphene
• Ukuginya iingxaki ukuba umlomo kunye nomqala uchaphazelekile
• Iminwe kunye neenzwane ezifakiweyo
• Ukuhamba okunomda kunqabile

Ezi ngxaki zinokwenzeka:

• Ukunqongophala kwegazi
• Ukuncitshiswa kwexesha lokuphila ngenxa yeendlela ezinzima zemo
• Ukunciphisa i-Esophageal
• Iingxaki zamehlo, kubandakanya ukungaboni
• Usulelo, kubandakanya i-sepsis (usulelo egazini okanye kwizicubu)
• Ukuphulukana nomsebenzi ezandleni nasezinyaweni
• Isihlunu semisipha
• Isifo sePeriodontal
• Ukungondleki okumandla okubangelwa kukondla ubunzima, kukhokelela ekusileleni ukukhula
• Umhlaza wesikhumba esine squamous

Ukuba usana lwakho lunokuphazamiseka kungekudala emva kokuzalwa, tsalela umnikezeli wakho. Ukuba unembali yosapho ye-EB kwaye ucwangcise ukuba nabantwana, unokufuna ukuba neengcebiso ngemfuzo.

Ukucebisa ngemfuza kuyacetyiswa kubazali abanokubakho abanembali yosapho yalo naluphi na uhlobo lwe-epidermolysis bullosa.

Ngexesha lokukhulelwa, uvavanyo olubizwa ngokuba yi-chorionic villus sampling lungasetyenziselwa ukuvavanya umntwana. Kwababini abasemngciphekweni omkhulu wokuba nomntwana one-EB, uvavanyo lunokwenziwa kwiveki yesi-8 ukuya kwe-10 yokukhulelwa. Thetha nomboneleli wakho.

Ukuthintela ukonakala kolusu kunye namadyungudyungu, nxiba i-padding ejikeleze indawo ezinobungozi ezinje ngeengqiniba, amadolo, amaqatha kunye neempundu. Kulumkele ukudlala imidlalo.

Ukuba unayo i-EB kunye ne-steroids ixesha elide ngaphezu kwenyanga enye, unokufuna i-calcium kunye ne-vitamin D. Ezi zongezelelo zinokunceda ukuthintela i-osteoporosis (ukuncipha kwamathambo).

EB; Ukudibana kwe-epidermolysis bullosa; Dystrophic epidermolysis bullosa; Hemidesmosomal epidermolysis bullosa; Isifo seWeber-Cockayne; Epidermolysis bullosa simplex

• I-Epidermolysis bullosa, eyona nto ibalulekileyo kwi-dystrophic
• Epidermolysis bullosa, idystrophic

UDenyer J, uPillay E, uClapham J. Esona sikhokelo sokuziqhelanisa nolusu kunye noKhathalelo lweNxeba kwi-Epidermolysis Bullosa: Isivumelwano seHlabathi. ILondon, e-UK: Amanxeba aMazwe ngaMazwe; Ngo-2017.

Kulungile, JD, Mellerio JE. Ku: I-Bolognia JL, uSchaffer JV, uCerroni L, ii-eds. Dermatology. Ngomhla we-4. IPhiladelphia, PA: Elsevier Saunders; I-2018: isahluko 32.

IHabif TP. Izifo ezingaqhelekanga kunye nezibi. Ku: Habif TP, ed. Zonyango lwezonyango. Umhla wesi-6. IPhiladelphia, PA: Elsevier; 2016: isahluko 16.