I vun chu khawih tlemte atang pawhin a hring chhuak a, a chhuak chhuak em? Epidermolysis Bullosa (EB) a ni thei ang em?

A chang chuan kan vun hi a sensitive hle a ni lawm ni? Thil tenau takin a rawn deng a nih pawhin, a nih loh leh thawmhnaw pakhat a phelh a nih pawhin, mi ṭhenkhat chuan awlsam takin vun an nei a, vun hliam an nei bawk. A bik takin naute te vun hi a nalh em em a, chutiang thil i hmuh chuan a hlauhawm hlein a lang. Chuvangin vawiinah hian chutiang vunah natna lo awm, tlem a tlem hle mahse hriat a pawimawh em em chu kan sawi dawn a ni. Chu chu epidermolysis bullosa, a tawi zawngin EB a ni.

Epidermolysis bullosa (EB) hi eng nge ni tak tak?

A awlsam zawngin, epidermolysis bullosa (EB) hi rochun natna a ni . I vun chu a ti chhe hle a, blister leh peel awlsam takin a siam bawk. A châng chuan i vun chu thawmhnaw hmanga i hnawih emaw, khawi emaw laiah i vuak emaw pawh ni se, blisters leh sores i nei thei. Mi thenkhat tan chuan a na lo thei hle a, kut, kekawrte, khup leh keah chauh hliam na tak a thlen thei. Amaherawhchu, a nasat chuan heng blisters leh sores te hi taksa khawi hmunah pawh a lang thei. EB symptoms hi a na lo atanga na tak thlengin a awm thei .

Hriat reng tur chu, a chang chuan heng blisters te hi ka chhungah, kawr chhungah (esophagus), mit chhungah leh taksa chhungril dang dangah te a lo awm thei a ni. Heng blisters te hi a dam chuan hliam na tak tak a hnutchhiah thei a ni. A nasat chuan heng blisters leh scars te hian taksa chhungril lam a tichhe thei a, chu chuan nunna a tichhe thei a ni.

EB chi hrang hrangte chu engte nge ni?

EB hi chi pali a awm a, chungte chu vun layer a nghawng dan atanga hriat theih a ni.

1. EB Simplex (EBS): Hei hi EB chi hrang hrang zingah chuan a tam ber a ni. Mi thenkhatah chuan a na lo, a na lutuk lo, a na lutuk, na tak thlengin a awm thei. Chu blisters chu vun chunglam layer, epidermis -ah a lo awm a. Heng blisters te hi a dam hnuah hian scarring hi a awm tlem hle.

2. Junctional EB (JEB): JEB-ah chuan ka chhung leh thawkna kawng chhungah blister a lo awm thin. This is somewhat rare , leh a na lo (a nuam lo deuh, a na lo deuh) atanga na tak thlengin a awm thei.

3. Dystrophic EB (DEB): DEB ah hian vun layer lai tak, dermis ah hian blisters a lo awm thin. Hei hi a na lo atanga na tak thlengin a danglam thei bawk.

4. Kindler Syndrome: Kindler Syndrome-ah chuan vun layer zawng zawng deuhthaw-ah blister a awm thei. Hei hi a tlem hle .

Doctor-te chuan EBS, JEB leh DEB te hi i vun layer a nghawng dan a zirin an thliar hrang thin. Kindler syndrome-ah chuan i vun layer hrang hrangah blisters a lo lang thei a.

He natna hian tute nge a nghawng ber? Engtiang chiahin nge a hluar?

EB hian tu pawh a nghawng thei. Hnam, chi, mipa leh hmeichhia emaw pawhin EB an vei thei a ni. Engpawhnise,I nu leh pa zinga pakhatin he natna hi a neih chuan EB i vei theihna a sang zawk.

A awm fo ang bawkin, United States-a statistics-a a lan dan chuan mi 50,000 zinga pakhat vel chauhvin EB hi an vei a ni. Hei hian a awmzia chu a tlem hle tihna a ni.

EB hian taksaah eng nghawng nge a neih theih?

EB natna khirh takah chuan mit a hring thei a, mit hmuhna pawh a hloh thei bawk . A châng chuan vun leh ruhteah hliam na tak leh piansualna a awm thei a, chu chuan kut zungtang, kut, ke leh ruhkawrte chu mumal taka chelh theih loh a ni. EB vei thenkhat chuan vun cancer chi khat squamous cell carcinoma an tih chu an vei theihna a sang hle .

A nasat bernaah chuan nausen laiin thihna a thleng thei . Hei hi natna khirh tak tak (entir nan , sepsis ), thawkna kawng khar vanga thawk harsatna, tui tlakchhamna, leh ei leh in tha lo ang chi dinhmun avang a ni.

EB hi a thihpui thei em?

Hei hi EB i neih chi hrang hrangah a innghat tak zet a ni. EB natna chi hrang hrang, a na lo zawkte hi a thihpui lo. Mahse, EB natna khirh tak nei te dam rei zawng hi nausen atanga kum 30 vel thleng a ni thei. Chuvangin hei hi ngaihven hlawh tak condition a ni.

EB natna lan chhuah dan chu engte nge ni?

EB natna lan chhuah dan hi a chi hrang hrangah a inang lo. Heng symptoms te hi naute emaw naupang te emaw i nih laiin a lang tlangpui . Symptom thenkhat chu chi hrang hrang inkarah a awm fo thin. EB natna lan chhuah dan tlangpui thenkhat chu hetiang hi a ni:

• Vun (kut, ke, elbow, khup) emaw taksa chhungah emaw blister awm.
• Kutphah leh kephah vun a thim a, callus ang maiin a awm.
• Thisen sen (Red blood cells) tlahniam (Anemia).
• Kut zungtang inzawm khawm (fused fingers or toes).
• Fingernail leh toenail deformed leh/or thickened.
• Vunah hian blister ang chi bumps (milia) dum te tak te lo lang.
• Chaw ei harsa (dysphagia).
• Naupang pakhata thanlenna beisei awm lohna.
• Ha beisei angin a lo thang lian lo ( hypoplasia ).

Eng thilin nge EB hi a thlen?

EB thlentu ber chu gene 18 zinga pakhata mutation emaw defect emaw vang a ni . He natna vei te hian gene bo emaw chhia emaw an nei a , chu chuan protein collagen an tih siam a pui a ni . I hriat angin collagen hi kan vun ang chi connective tissue te chakna leh structure petu a ni. Chuvangin, he gene defect avang hian vun layer pahnih, epidermis leh dermis te hi a pangngaiin an inzawm khawm lo. Chuvang chuan vun chu a chhe hle a, awlsam takin a hring a, a hliam bawk.

EB hi inherited disease a ni fo thin , chu chu nu leh pa zinga pakhatin a neih chuan fate pawhin an rochun thei tihna a ni.

Mahse, a tlem hle a, EB hi acquired autoimmune disorder angin a awm thei bawk .

A pawimawh ber chu EB hi natna hrik kai theih a ni lo tih hi a ni. A tlangpuiin rochun atanga lo chhuak a ni. Chuvangin, EB nei mi nen in inzawm a nih chuan i hmu lovang.

Engtin nge EB i nei tih chiang taka i hriat theih ang? (Diagnosis) tih a ni.

Doctor-te chuan EB hi skin biopsy hmangin an hmu thei a, chu chu doctor-in vun sample tlemte a la a, microscope hmangin a enfiah a ni.

Hei bakah hian genetic testing hmangin gene chhia chu hriatchhuah theih a ni a, EB type chu a dik tih finfiah theih a ni bawk. Nu leh pa fa neih tumte pawhin nau neih hmaa genetic testing an nei thei bawk a, EB vei fa neih theihna tur hlauhawm an nih leh nih loh hriat theih a ni bawk.

EB enkawlna tur engte nge ni?

Vanduaithlak takin tunah hian EB damdawi hi a la awm lo . Mahse, enkawlnate hian a pui thei a ni:

• Blisters venna atan a pui.
• Blisters leh vun enkawl that hian harsatna a veng thei a ni.
• Ka emaw, kawr emawa blisters avanga lo awm thei nutritional problems te a enkawl thin.
• Natna thunun.

Vun chhiatna leh inhnawh vanga hliam leh hliam lo awm lo turin doctor-te chuan a hnuaia mi ang hian an rawt a ni.

• Natural fiber atanga siam thawmhnaw nêm, loose-fitting ha rawh. Thawmhnaw chhung lam atanga pawn lam hak hian i vunah seams rubbing zat a ti tlem thei a ni.
• A lum lutuk loh tur; room-te chu a nuamin, a lumna inang lo takah dah rawh.
• Ni chhuahna hmuna chhuah loh tur, a nih loh leh sunglass leh lukhum i hman laiin sunscreen hmang la.
• Vun venhim nan bandage chi hrang hrang hmang rawh – nonadhesive bandage leh tape, rolled gauze, etc. hmang rawh.

Blisters enkawl nan chuan i doctor chuan hetiang hi a rawn rawt thei ang:

• Hliam ah nitin hriak hnawih thin ang che.
• Blisters dam leh infection venna atan damdawi hmanga siam bandage hmang thin ang che.
• Natna thunun nan damdawi ei thin ang che.

Infection enkawl nan i doctor chuan a hnuaia mi ang hian a rawn rawt thei ang:

• Oral antibiotics ei emaw, antibiotic cream hnawih emaw la.
• Hliam dam lo tan chuan hliam khuhna bik hmang thin ang che.

Kawng emaw, kawr emaw avanga ei leh in lama harsatna leh ei leh in lama harsatna awm lo turin doctor chuan a hnuaia mi ang hian a rawt thei ang:

• I naute chaw i pek dawnin, naute bottle, a hnute bik nei hmang la .
• Naute chu eyedropper emaw syringe emaw hmangin chaw pe rawh.
• Mashed food ah hian tui tlem te te dah la, a dilute la, feed rawh. Hei hian ei a awlsam phah ang.
• Chaw nêm tak tak, soup, mashed foods, pudding, leh applesauce te ei tam zawk rawh .
• Chaw chu a lum (a lum lutuk lo)-ah an pe thin.
• I ei leh in mamawh bikte sawipui turin dietitian hmu rawh.

EB natna khirh takah chuan operation i mamawh thei bawk . Esophagus (ka atanga chaw ei tur phurtu tube) chu blisters leh scarring in a block a nih chuan surgery hmanga zau zawka siam theih a ni. Mi thenkhat chuan chaw kawng (esophagus) chu an bypass vek thei a, chaw ei tur (feeding tube) an dah a, chu chuan chaw ei tur chu an pum chhungah direct-in an pe thei a ni. Kut zungtang zung (blisters) avanga inzawm khawm te pawh hi operation tih theih a ni bawk.

EB vei hian engtin nge a in enkawl?

EB i neih chuan heng tips te hian i in enkawl dan tur a pui ang che:

• Silk emaw satin emaw ang chi, natural fiber nêm tak aṭanga siam sheet leh pillowcase-ah mu rawh.
• I taksa tikhawlo thei lo kekawr nuam tak ha rawh.
• Hun rei tak ding emaw, kal emaw loh tur.
• Vun chu hrual emaw, picking emaw loh tur. Itch tihziaawm nan anti-itch damdawi hnawih thin ang che.
• I chhehvel fimkhur la, i vun chu accident-a khawih emaw, i hrual emaw loh tur a ni.
• Friction tihziaawm nan i vun hi moisturize fo ang che.
• Blisters a lo awm chuan sterilized needle emaw scissors fai emaw hmangin pop vat rawh.
• Nonadhesive bandage hmang rawh. A dang pawhin petroleum jelly (VaselineTM) emaw vun tana tha tak (AquaphorTM ang chi) emaw chu bandage-ah hnawih la, vunah a innghah loh nan.

EB vei naupang enkawl dan tur? Nu leh pa tan tips thenkhat

I fain EB a neih chuan an mamawh chu midang aiin a danglam a ni. An lungawi lohna chu an sawi chhuak thei lo mai thei. Heng tips te hian i fa chu a nuam thei ang bera awm tir turin a pui thei a ni:

• I naute vun i khawih hmain i kut te uluk takin sil hmasa phawt ang che.
• Latex gloves hman loh tur a ni a, friction a tipung thei a ni.
• I naute i sil dawnin a taksa pum pui chu vawi khatah tui khata dah ai chuan a vun section tin chu pakhat khatin silfai thin ang che.
• I nau piang thar chu diaper-ah dah ai chuan, a chungah absorbent pad dah hi ngaihtuah rawh.
• Velcro® strap nei diaper hmang rawh. Adhesive strap/tape hi hriat lohvin i naute vunah a innghat thei.
• Diaper ke awmna hmuna elastic chu cut la, la chhuak rawh.
• Diaper chhungah silicone gel sheet ang chi thil dah la, vun chu a hring chhuak tawhah a innghat lo ang.
• Naupang chawi lai hian fimkhur hle ang che.I kut chu armpits (axilla) hnuaiah dahin chawi chhuah loh tur. I fain a kekawrte leh a hnungzang-ah blisters emaw hliam emaw a neih chuan kut pakhatin a kawrfual velah leh kut dangin a hnungzang thlawpin chawi sang rawh.
• I fate chu an EB condition-in a phal angin active turin fuih rawh. Thut reng hian harsatna dang a thlen thei a, chu chu constipation leh muscle atrophy . Swimming hi exercise tha tak, low-impact a ni.
• Taksa tihchakna nasa tak emaw, i fate thawk hah emaw, a lum emaw ti thei tur game emaw chu fuih suh.

I fa chhinchhiahnate hmachhawn chu a châng chuan a harsa thei hle. I rilru a buai a, i chau hle mai thei. I fa – leh nangmah – a nuam thei ang bera awm theih nan i doctor hnenah tips tam zawk zawt rawh. Tin, nu leh pa puihna group-a telna hmang hian i thil tawnte i sawi thei a, i fate EB dinhmun enkawl dan thar i zir thei bawk.

EB hi ven theih a ni em?

EB hi genetic a nih avangin a ven theih loh . Chhungkaw history-a EB vei tawh leh fa neih tumte chuan an chhungkaw ruahmanna siam dan tur relnaah genetic counseling hi a \angkai hle mai thei.

Tin, tunah hian mithiamte chuan EB acquired form hi eng thilin nge a thlen tih an hre lo. Chuvang chuan Doctor ten a ven dan tur an hre lo.

EB vei hian eng ang hmalam hun nge a beisei theih ang?

EB vei te hmalam hun chu EB an neih chi leh a nasat danah a innghat a ni. Natna chi hrang hrang na tak chuan natna nasa tak a thlen thei a, taksa pianphung a tichhe thei a, chak lohna a thlen thei a, hliam dam ngai lo a awm thei a, thih hma pawh a thlen thei bawk .

Mahse, doctor-te chuan i natna lan chhuah dan tur an enkawl thei che a ni. Hei hi enkawl dan dik leh a tul chuan natna damdawi hmanga tih theih a ni. Ni hnih danah vun chu nonadhesive, protective dressing hmanga khuh tha taka dah te, inbual fo te, hliam enkawl te hian EB hian i nun a nghawng dan a ti tlem thei a ni.

EB i neih chuan engtikah nge doctor i hmuh ang?

Heng thil thlengah hian doctor hmu ngei ang che:

• Thâwk harsa i neih chuan.
• Chaw ei harsatna i neih chuan.
• I hliam chu natna vei anga lang (vun chu a sen, a sen, a sen, a var; a hring emaw a hring emaw a nih chuan).
• Symptom thar a lo lang a nih chuan.

Doctor hnena zawh tur pawimawhte chu engte nge ni?

Doctor hmu tura i kal dawnin hetiang zawhna hi zawh a tha ang:

• Engtin nge EB ka nei tih i hriat theih ang?
• EB ka neih loh chuan eng vun natna dang nge ka neih theih ang?
• Engtin nge ka symptoms te hi ka enkawl theih ang?
• Eng damdawi nge i rawt? Side effect an nei em?
• Eng in lama enkawlna nge i rawt?
• Ka natna lan chhuah dan tihchangtlun nan eng nge ka tih belh ang?
• Cream emaw ointment emaw i pek theih tur a awm em?
• Dermatologist emaw, specialist dang emaw ka pan tur em ni?

Epidermolysis bullosa (EB) leh bullous pemphigoid te hi eng nge an danglamna?

Bullous pemphigoid hi vun natna hmuh tur awm lo, autoimmune skin disease a ni. Itchy, hive ang chi welts emaw fluid-filled blisters emaw a thlen thei. Bullous pemphigoid hian kum 60 chunglam a vei tam ber a, kum nga chhungin a bo tlangpui. Mahse, a nasat chuan a blisters leh scarring hian chhungril lam a tichhe thei a, thihna a thlen thei bawk.

EB hi a chang chuan autoimmune disease ni thei mahse, a tlangpuiin i vunah collagen tichhe thei genetic mutation avanga lo awm a ni. EB hi nausen emaw naupan lai emaw ah a lang tlangpui . EB damdawi a awm loh avangin i dam chhung zawng symptoms nen i nunpui a ngai mai thei.

A tawp berah chuan thil hriat reng tur (Take-Home Message) .

Epidermolysis bullosa (EB) hi a chang chuan harsatna a awm thin. Mahse, damdawi lam thurawn dik tak, enkawlna dik tak, leh hmangaihna nena enkawlna i neih chuan a lan chhuah dante chu i thunun thei a, a theih ang anga ṭha takin i nung thei bawk. Nangmah chauh i ni lo tih hre reng ang che. Doctor, chhungte, leh support group te chu tanpui turin an awm vek a ni. Chuvangin, hlau suh, chak takin awm la, he dinhmun hi hmachhawn rawh.

EB chungchanga zawhna dang i neih chuan dermatologist nen inbia ngei ang che.

` Epidermolysis bullosa, EB, vun natna, blisters, genetic disease, naupan lai vun natna, vun chhe thei lo