I-Epidermolysis bullosa

I-Epidermolysis bullosa (EB) iyiqembu lezinkinga lapho amabhamuza esikhumba akha ngemuva kokulimala okuncane. Idluliselwa emindenini.

Kunezinhlobo ezine eziyinhloko ze-EB. Kunjalo:

• I-Dystrophic epidermolysis bullosa
• I-Epidermolysis bullosa simplex
• I-Hemidesmosomal epidermolysis bullosa
• Ukuhlangana kwe-epidermolysis bullosa

Olunye uhlobo olungavamile lwe-EB lubizwa nge- epidermolysis bullosa acquisita. Leli fomu likhula ngemuva kokuzalwa. Kuyisifo sokuzilimaza komzimba, okusho ukuthi umzimba uyazihlasela.

I-EB ingahluka kusuka kokuncane kuya ekubulaleni. Ifomu elincane libangela ukuqhuma kwesikhumba. Ifomu elibulalayo lithinta ezinye izitho. Izinhlobo eziningi zalesi simo ziqala lapho kuzalwa noma ngemuva nje kwalokho. Kungaba nzima ukukhomba uhlobo ngqo lwe-EB umuntu analo, yize izimpawu ezithile zofuzo sezitholakalela iningi.

Umlando womndeni uyingozi. Ingozi iphezulu uma umzali analesi simo.

Ngokuya ngesimo se-EB, izimpawu zingabandakanya:

• I-Alopecia (ukulahleka kwezinwele)
• Amabhamuza azungeze amehlo namakhala
• Amabhamuza emlonyeni noma emphinjeni noma ngaphakathi, abangela izinkinga zokudla noma ubunzima bokugwinya
• Amabhamuza esikhunjeni ngenxa yokulimala okuncane noma ukushintsha kwezinga lokushisa, ikakhulukazi ezinyaweni
• Ukuqhuma okukhona lapho kuzalwa
• Izinkinga zamazinyo ezinjengokubola kwamazinyo
• Ukukhala, ukukhwehlela, noma ezinye izinkinga zokuphefumula
• Amabhampa amhlophe amancane esikhunjeni esalimala phambilini
• Ukulahleka kwesipikili noma izipikili ezikhubazekile

Umhlinzeki wakho wokunakekelwa kwezempilo uzobheka isikhumba sakho ukuthola i-EB.

Ukuhlolwa okusetshenziselwa ukuqinisekisa ukuxilongwa kufaka:

• Ukuhlolwa kofuzo
• Isikhumba biopsy
• Ukuhlolwa okukhethekile kwamasampuli esikhumba ngaphansi kwesibonakhulu

Ukuhlolwa kwesikhumba kungasetshenziselwa ukukhomba uhlobo lwe-EB.

Olunye uvivinyo olungenziwa lubandakanya:

• Ukuhlolwa kwegazi kwe-anemia
• Isiko lokuhlola ukutheleleka kwamagciwane uma amanxeba ephola kabi
• I-endoscopy ephezulu noma uchungechunge oluphezulu lwe-GI uma izimpawu zifaka phakathi izinkinga zokugwinya

Izinga lokukhula lizohlolwa kaningi enganeni ene-EB noma engaba nayo.

Inhloso yokwelashwa ukuvimbela amabhalidi ekwakheni nasekugwemeni izinkinga. Olunye ukwelashwa kuzoya ngokuthi isimo sibi kangakanani.

UKUNAKEKELWA KWEKHAYA

Landela le mihlahlandlela ekhaya:

• Nakekela kahle isikhumba sakho ukuvikela izifo.
• Landela iseluleko somhlinzeki wakho uma izindawo ezinamakhasi ziba nesiga noma zibe luhlaza. Ungadinga ukwelashwa okuvamile kwe-whirlpool futhi usebenzise amafutha okulwa namagciwane ezindaweni ezinjengezilonda. Umhlinzeki wakho uzokwazisa uma udinga ibhandeshi noma ukugqoka, futhi uma kunjalo, hlobo luni okufanele ulusebenzise.
• Ungahle udinge ukusebenzisa imishanguzo yomlomo ye-steroid isikhathi esifushane uma unezinkinga zokugwinya. Ungadinga futhi ukuphuza umuthi uma uthola i-candida (imvubelo) ukutheleleka emlonyeni noma emphinjeni.
• Nakekela impilo yakho yomlomo bese uthola ukuhlolwa kwamazinyo njalo. Kungcono ukubona udokotela wamazinyo onolwazi lokwelapha abantu abane-EB.
• Yidla ukudla okunempilo. Uma unokulimala okuningi kwesikhumba, ungadinga ama-calories amaningi namaprotheni ukusiza isikhumba sakho siphole. Khetha ukudla okuthambile futhi ugweme amantongomane, ama-chip, nokunye ukudla okumanzi uma unezilonda emlonyeni wakho. Isazi sokudla okunempilo singakusiza ekudleni kwakho.
• Ingabe ukuzivocavoca okwelapha ngokomzimba kukubonisa ukusiza ukugcina amalunga nemisipha yakho iselula.

UKUHLINZA

Ukuhlinzwa ukwelapha lesi simo kungafaka:

• Ukuxhunyelelwa kwesikhumba ezindaweni lapho izilonda zijule khona
• Ukwehliswa (ukunwetshwa) kwesisu uma kukhona ukuncipha
• Ukulungiswa kokukhubazeka kwesandla
• Ukususwa kwanoma iyiphi i-squamous cell carcinoma (uhlobo lomdlavuza wesikhumba) okhulayo

EZINYE UKWELASHWA

Ezinye izindlela zokwelashwa zalesi simo zingafaka:

• Imithi ecindezela amasosha omzimba ingasetshenziselwa ukwakheka komzimba walesi simo.
• Amaphrotheni nokwelashwa kofuzo nokusetshenziswa kwe-interferon yezidakamizwa kuyacwaningwa.

Umbono uncike ebucayi bokugula.

Ukutheleleka kwezindawo ezinamakhasi kuyinto evamile.

Izinhlobo ezimnene ze-EB ziyathuthuka ngobudala. Izinhlobo ezimbi kakhulu ze-EB zinezinga eliphakeme kakhulu lokufa.

Ngamafomu abucayi, ukusikwa ngemuva kwefomu lamabhaladi kungadala:

• Ukukhubazeka kwenkontileka (ngokwesibonelo, eminweni, endololwaneni nasemadolweni) nokunye ukukhubazeka
• Izinkinga zokugwinya uma umlomo nomphimbo kuthintekile
• Iminwe nezinzwane ezifakiwe
• Ukuhamba okunomkhawulo kusuka ekulimaleni

Lezi zinkinga zingenzeka:

• Ukushoda kwegazi
• Isikhathi esinciphile sokuphila sezinhlobo ezinzima zalesi simo
• Ukuncipha kwe-Esophageal
• Izinkinga zamehlo, kufaka phakathi ukungaboni
• Ukutheleleka, kufaka phakathi i-sepsis (ukutheleleka egazini noma kwizicubu)
• Ukulahlekelwa ngumsebenzi ezandleni nasezinyaweni
• Ukukhubazeka kwemisipha
• Isifo se-Periodontal
• Ukungondleki okukhulu okubangelwa ubunzima bokudla, okuholela ekwehlulekeni ukuchuma
• Umdlavuza wesikhumba ogcwele squamous

Uma usana lwakho lunokuqubuka ngemuva nje kokuzalwa, shayela umhlinzeki wakho. Uma unomlando womndeni we-EB futhi uhlela ukuba nezingane, ungahle uthande ukuthola ukwelulekwa ngezofuzo.

Ukwelulekwa ngofuzo kunconyelwa abazali abazoba nomlando womndeni wanoma yiluphi uhlobo lwe-epidermolysis bullosa.

Ngesikhathi sokukhulelwa, isivivinyo esibizwa nge-chorionic villus sampling singasetshenziswa ukuhlola ingane. Emibhangqwaneni esengozini enkulu yokuba nengane ene-EB, ukuhlolwa kungenziwa ekuqaleni kweviki lesi-8 kuya kwele-10 lokukhulelwa. Khuluma nomhlinzeki wakho.

Ukuvimbela ukulimala kwesikhumba nokugqabhuka, gqoka okugoqwayo ezizungeze izindawo ezingahle zilimale njengezindololwane, amadolo, amaqakala nezinqe. Gwema imidlalo yokuxhumana.

Uma une-EB acquisita futhi usebenzisa ama-steroids isikhathi esingaphezu kwenyanga engu-1, ungadinga izithako ze-calcium nevithamini D. Lezi zithasiselo zingasiza ekuvikeleni i-osteoporosis (ukuncipha kwamathambo).

EB; Ukuhlangana kwe-epidermolysis bullosa; I-Dystrophic epidermolysis bullosa; I-hemidesmosomal epidermolysis bullosa; Isifo se-Weber-Cockayne; I-Epidermolysis bullosa simplex

• I-Epidermolysis bullosa, i-dystrophic evelele
• I-Epidermolysis bullosa, i-dystrophic

UDenyer J, uPillay E, uClapham J. Imihlahlandlela Engcono Kakhulu Yokuzijwayeza Yokunakekelwa Kwesikhumba Nesilonda e-Epidermolysis Bullosa: Ukuvumelana Komhlaba Wonke. ILondon, e-UK: Amanxeba Amazwe Ngamazwe; 2017.

Kuhle, JD, Mellerio JE. Ku: Bolognia JL, Schaffer JV, Cerroni L, ama-eds. Isikhumba. Umhla wesi-4. IPhiladelphia, PA: Elsevier Saunders; 2018: isahluko 32.

IHabif TP. Izifo ezibangelwa i-veesicular and bullous. Ku: Habif TP, ed. Ukwelashwa Kwemithi Yezokwelapha. Umhlaka 6. IPhiladelphia, PA: Elsevier; 2016: isahluko 16.