I vun hi tlemte chauh ni mahse blisters-ah a chhuak em? Epidermolysis Bullosa (EB) chungchang hi han sawi ila

I naute tê vun chu a thawmhnaw hak chuan a hring chhuak a, awlsam takin a hliam a, khawi emaw laiah tlem a hrual pawh ni se, han mitthla teh. Hei hi thil awmdan hrehawm leh lungchhiatthlak tak a ni a, a ni lawm ni? Vanduaithlak takin naupang leh puitling thenkhat chuan hetiang dinhmun hi an tawk thin. Vawiin hian ‘Epidermolysis Bullosa’ (EB) tih natna pakhat kan sawi dawn a, chutiang vun chu a nalh em em a, a sensitive hle bawk. Mi thenkhat chuan heng naupangte hi hmangaih takin "butterfly babies" an ti bawk a, a chhan chu an vun hi butterfly thla ang maia nalh a nih vang a ni.

A awlsam zawngin sawi ila, Epidermolysis Bullosa (EB) hi eng nge ni?

Epidermolysis Bullosa (EB) hi natna pakhat chauh a ni lo. Genetic condition a ni a, thlah kal zelah a kal zel tihna a ni. He natna vei vun hi a chhe hle. Mi pangngai tichhe lo tur pressure tenau emaw, inhnawh tawnna emaw pawh ni se, thawmhnaw chunga hnawih emaw, mi pakhatin an vun a khawih emaw pawh hian a châng chuan vun leh hliam a thlen thei a ni.

Kan vun hi main layer engemaw zat atanga siam a ni. Heng layer te hi protein te hian an vawng tlat a ni. A dik tak chuan hei atan hian protein pakhat collagen an tih hi a pawimawh hle. Vun layer-a innghat ‘gum’ ang mai a ni. EB natna vei te zingah genetic defect a awm avangin he protein hi a siam tha lo hle. Chuvangin, chu ‘gum’ chu a chak lo avangin vun layer te chu awlsam takin a inthen a, tuiin a khat a, blisters a siam thin. Heng blisters te hi kut, ke, elbow, leh khup ang hmunah chauh ni lovin, ka chhung, kawr chhung leh taksa chhungah pawh a lo awm thei a ni.

EB natna chi hrang hrangte chu engte nge ni?

EB hi chi paliah then a ni a, chu chu vun layer a blisters siamna hmun a zirin a ni. Hei hi doctor-te tan chuan enkawl dan tur rel turin a pawimawh hle.

EB natna lan chhuahna awm thei te chu engte nge ni?

EB natna chi hrang hrang a zirin symptoms a inang lo thei. Nausen lai atang hian symptoms hi a lang tan tlangpui.

• A lan chhuah dan ber chu vunah a hring (blistering) a ni a, a bik takin kut, ke, kekawrte leh khup-ah te a hring chhuak thin.
• Kutphah leh kephah vun a thim, a hrual ang maia tihthianghlim.
• Nail a deformed, thickened, a peeling.
• Vunah hian blister ang chi bumps (milia) dum te tak te lo lang.
• Chaw ei harsa (dysphagia) chu ka leh kawr a hrual vang a ni.
• Hliam fo hian taksa atanga thisen a hloh thei a, chu chuan thisen tlakchhamna a thlen thei a ni .
• A nasat chuan kut zungtang chu a inzawm khawm thin.
• Ha enamel chak lo leh ha lo thanglian lo (hypoplasia).
• Naupangte thanlenna tlahniam.

Engtin nge he natna hi hriatchhuah leh enkawl a nih?

I fa hian heng symptoms te hi a nei a nih chuan Doctor pan a pawimawh. Doctor chuan he natna hi a hriat theih nan test eng emaw zat a ti tlangpui ang.

• Skin Biopsy: Hei hian vun te tak te tak tak lak a, microscope hmanga enfiah a ni. Hei hian vun layer eng nge a nghawng tih chiang taka hriat theih nan a pui thei a ni.
• Genetic Testing neih dan tur: 1.1.Hei hian eng gene nge a chhia tih leh eng chi EB nge a nih tih chiang taka hriat theih nan a pui thei a ni.

Enkawlna chungchang hi han sawi ila.

Sawi hmasak ber tur chu vanduaithlak takin EB hi damdawi a la awm lo. Mahse, symptoms te chu control theih a ni a, complication te chu ven theih a ni a, damlo chu a nuam thei ang bera nun a hmang thei bawk. Enkawlna tum berte chu:

• A theih ang anga blistering tih tlem.
• Blisters leh hliam te a kai loh nan fimkhur.
• Natna enkawl dan.
• Ei leh in lama harsatna awm thei venna.

Hemi atana doctor ten tips an pek thenkhat chu hengte hi a ni.

• Thawmhnaw nêm, hrual hrual: Cotton ang chi natural fiber a\anga siam, tight lo, i taksaa hnawih lo tur thawmhnaw i ha tur a ni.
• I vun vawt reng rawh: Khua lum leh thawmhnaw hian blister a tipung thei a, chuvangin room chu temperature nuam takah dah a pawimawh.
• Bandage bik hman dan: Hliam tan chuan special bandage leh gauze vunah a innghah loh hman tur a ni.
• Nitin hliam enkawl dan: Doctor-in a pek hriak hriak hnawih la, a hliamte chu thianghlim takin vawng reng ang che.
• Diet: Kawng ulcer i neih chuan chaw lum, spicy, leh hard foods ei loh tur a ni a, soup, mashed foods, leh puddings ang chi ei tur nêm tak tak ei tur a ni.
• Surgery: Thil na tak tak thenkhatah chuan chaw kawng chu a zim a nih chuan zau zawka siam emaw, kut zungtangte chu a inzawm tlat chuan then hran nan emaw operation neih a ngai mai thei.

EB vei naupang enkawltu nu leh pate tan thurawn

Hetiang naupang enkawl hi nu leh pate tan chuan harsatna lian tak a ni. Taksa leh rilru hahthlak tak a ni thei. Mahse i hmangaihna leh i ngaihsakna chu naupang tan chuan chakna ropui tak a ni.

• I naute vun i khawih hmain i kut chu uluk takin silfai hmasa phawt ang che .
• Naute i chawi sang dawnin, a hnungzang hnuaiah chuan man suh . Chutianga tih chuan vun a tichhe thei a ni. Naute hnungzang leh kekawr hnuaiah i kut dah la, uluk takin chawi sang rawh.
• Diaper i hman chuan adhesive tape hmang lovin Velcro nei hmang zawk ang che. Ke chhehvela elastic band awmte chu tan la, friction i tihziaawm thei.
• Naute i sil dawnin a taksa peng tin chu tui basin-a thun ai chuan zawi zawia tihfai a tha ber.
• I fa thiltih hi khap suh. Rough play hi a dik lo nain, tui chawi ang chi exercise nêm tak chuan i fa taksa ruh a tichak thei a ni.
• Nang pawh rilru lama chau tawh anga i inhriat chuan, i doctor, chhungte, a nih loh leh chutiang thil tawn tawh nu leh pate hnen aṭangin ṭanpuina zawng hreh suh.

Engtikah nge Doctor i hmuh nghal tur?

EB enkawl hian a chang chuan damdawi lam pan vat a pawimawh thin. A hnuaia symptoms te hi fimkhur hle ang che.

• Thâwk harsa i neih chuan. (Chutiang hunah chuan , damdawi in hnai ber Emergency Department (ETU) ah kal nghal tur a ni. )
• Chaw emaw tui emaw i ei theih loh chuan .
• Hliam chu hri kai anga lang (chu chu hliam chhehvel chu a sen, a hring, a lum, a nih loh leh a hring chhuak tihna a ni).
• Naupangin khawsik a neih chuan.
• I hriatthiam theih loh symptom thar a lo lang a nih chuan.

Hetiang natna nena nun hi thil harsa tak ni thei mah se, damdawi lam thurawn leh enkawlna dik tak i neih chuan a natna chu i thunun thei a, nun ṭha tak i nei thei bawk. A pawimawh ber chu doctor nen hnaih taka thawh dun reng a, heng thilte chauh hi hmachhawn tum lo tur a ni.