Koj daim tawv nqaij puas ua pob khaus thiab tawg tawm txawm tias kov me ntsis xwb los? Puas yog nws yog Epidermolysis Bullosa (EB)?

Qee zaum peb daim tawv nqaij rhiab heev, puas yog? Txawm tias muaj ib yam me me ntaus nws, lossis ib daim khaub ncaws raug dua, qee tus neeg yooj yim raug mob thiab raug mob ntawm daim tawv nqaij. Tshwj xeeb tshaj yog vim daim tawv nqaij ntawm cov menyuam yaus me me yog qhov mos heev, nws zoo li ntshai heev thaum koj pom tej yam zoo li no. Yog li hnub no peb yuav tham txog ib qho kab mob uas tshwm sim rau ntawm daim tawv nqaij zoo li no, uas yog qhov tsis tshua muaj tab sis tseem ceeb heev uas yuav tsum paub. Ntawd yog epidermolysis bullosa, lossis EB luv.

Dab tsi yog epidermolysis bullosa (EB)?

Hais yooj yim xwb, epidermolysis bullosa (EB) yog ib yam mob uas tau txais los ntawm noob caj noob ces. Nws ua rau koj daim tawv nqaij tsis muaj zog thiab ua pob khaus thiab yooj yim tev tawm. Qee zaum, txawm tias koj txhuam koj daim tawv nqaij nrog khaub ncaws lossis raug ntaus qhov twg los xij, koj tuaj yeem tsim cov pob khaus thiab mob. Rau qee tus neeg, nws yuav tsis hnyav heev thiab tsuas yog ua rau mob pob khaus ntawm tes, lub luj tshib, lub hauv caug, thiab ko taw xwb. Txawm li cas los xij, thaum muaj mob hnyav, cov pob khaus thiab mob no tuaj yeem tshwm sim txhua qhov chaw ntawm lub cev. Cov tsos mob ntawm EB tuaj yeem sib txawv ntawm qhov tsis hnyav heev mus rau qhov hnyav heev .

Nco ntsoov tias, qee zaum cov hlwv no tuaj yeem tshwm sim hauv qhov ncauj, caj pas (esophagus), qhov muag, thiab lwm yam hauv lub cev. Thaum cov hlwv no zoo lawm, lawv tuaj yeem tso cov qhov txhab mob. Hauv cov xwm txheej hnyav, cov hlwv thiab cov qhov txhab no tuaj yeem ua rau cov hlwv hauv lub cev puas tsuaj, uas tuaj yeem ua rau tuag taus.

Cov hom EB tseem ceeb yog dab tsi?

Muaj plaub hom EB tseem ceeb, uas raug txheeb xyuas los ntawm txheej tawv nqaij uas cuam tshuam.

1. EB Simplex (EBS): Qhov no yog hom EB uas feem ntau pom . Nws tuaj yeem mob me me xwb, tsis mob heev, mus txog rau mob heev, hnyav heev, rau qee tus neeg. Cov pob khaus tsim nyob rau hauv txheej sab saud ntawm daim tawv nqaij, epidermis . Qhov caws pliav tsis tshua muaj tom qab cov pob khaus no zoo lawm.

2. Kab mob sib txuas (JEB): Hauv JEB, cov pob khaus tsim sab hauv lub qhov ncauj thiab txoj hlab pa. Qhov no tsis tshua muaj , thiab tuaj yeem ua rau mob me ntsis (me ntsis tsis xis nyob, mob me ntsis) mus rau mob hnyav.

3. Dystrophic EB (DEB): Hauv DEB, cov pob khaus tsim nyob rau hauv txheej nruab nrab ntawm daim tawv nqaij, dermis . Qhov no kuj tuaj yeem sib txawv ntawm qhov me me mus rau qhov hnyav.

4. Kindler Syndrome: Hauv Kindler Syndrome, cov hlwv tuaj yeem tsim rau yuav luag txhua txheej ntawm daim tawv nqaij. Qhov no tsis tshua muaj .

Cov kws kho mob cais qhov EBS, JEB, thiab DEB raws li cov txheej ntawm koj daim tawv nqaij uas raug cuam tshuam. Hauv Kindler syndrome, cov hlwv tuaj yeem tshwm sim thoob plaws ntau txheej ntawm koj daim tawv nqaij.

Leej twg raug tus mob no ntau tshaj? Nws tshwm sim ntau npaum li cas?

Kab mob EB tuaj yeem cuam tshuam rau txhua tus neeg. Cov neeg txawm yog haiv neeg twg los xij, pawg neeg twg los xij, lossis poj niam txiv neej twg los xij tuaj yeem muaj kab mob EB. Txawm li cas los xij,Yog tias ib tug ntawm koj niam koj txiv muaj tus kab mob no, koj yuav muaj feem ntau dua rau tus kab mob EB.

Txawm hais tias nws yog ib qho mob uas tshwm sim ntau npaum li cas los xij, raws li cov ntaub ntawv hauv Tebchaws Meskas, tsuas yog kwv yees li ib tus neeg ntawm 50,000 tus neeg raug mob los ntawm EB. Qhov no txhais tau tias nws yog ib qho mob tsis tshua muaj.

EB muaj tej yam cuam tshuam li cas rau lub cev?

Hauv cov mob hnyav ntawm EB, lub qhov muag tuaj yeem ua pob thiab ua rau pom tsis meej . Qee zaum, cov qhov txhab loj heev thiab cov leeg nqaij tuaj yeem tshwm sim, ua rau nws tsis tuaj yeem txav cov ntiv tes, tes, taw, thiab pob qij txha kom raug. Qee tus neeg uas muaj EB muaj kev pheej hmoo ntau ntxiv ntawm kev mob qog nqaij hlav hu ua squamous cell carcinoma .

Hauv cov mob hnyav tshaj plaws, qee zaum kev tuag tuaj yeem tshwm sim thaum tseem yog menyuam yaus . Qhov no yog vim muaj cov mob xws li kev kis kab mob hnyav (piv txwv li , sepsis ), ua pa nyuaj vim yog kev thaiv txoj hlab pa, lub cev qhuav dej, thiab kev tsis noj qab haus huv.

Kab mob EB puas tuaj yeem ua rau tuag taus?

Qhov no yeej nyob ntawm seb koj muaj hom kab mob EB twg. Cov kab mob EB me me, tsis hnyav heev tsis ua rau tuag taus. Txawm li cas los xij, lub neej ntev ntawm cov neeg uas muaj cov kab mob EB hnyav tuaj yeem ntev li ntawm cov menyuam yaus mus txog li 30 xyoo. Yog li qhov no yog ib qho mob uas xav tau kev saib xyuas ntau.

Cov tsos mob ntawm EB yog dab tsi?

Cov tsos mob ntawm EB sib txawv nyob ntawm hom. Cov tsos mob no feem ntau tshwm sim thaum koj tseem yog menyuam mos lossis menyuam yaus . Qee cov tsos mob yog qhov tshwm sim ntawm ntau hom. Nov yog qee cov tsos mob tseem ceeb ntawm EB:

• Cov pob khaus rau ntawm daim tawv nqaij (tes, taw, lub luj tshib, lub hauv caug) lossis sab hauv lub cev.
• Daim tawv nqaij ntawm xib teg thiab hauv qab taw tuab zuj zus, ua rau lawv zoo li cov tawv nqaij tawv.
• Txo cov qe ntshav liab (Anemia).
• Cov ntiv tes lo ua ke (cov ntiv tes lossis cov ntiv taw sib xyaws).
• Cov rau tes thiab rau taw uas tsis zoo thiab/lossis tuab.
• Muaj cov pob me me dawb zoo li cov hlwv (milia) tshwm sim rau ntawm daim tawv nqaij.
• Nyuaj nqos zaub mov (dysphagia).
• Tus menyuam tsis loj hlob raws li qhov xav tau.
• Cov hniav tsis loj hlob raws li qhov xav tau ( hypoplasia ).

Dab tsi ua rau muaj EB?

Qhov ua rau muaj EB yog kev hloov pauv lossis qhov tsis zoo hauv ib qho ntawm 18 lub noob caj noob ces . Cov neeg uas muaj tus kab mob no muaj ib lub noob caj noob ces uas ploj lawm lossis puas lawm uas pab ua cov protein hu ua collagen . Raws li koj paub, collagen yog qhov muab lub zog thiab cov qauv rau cov ntaub so ntswg sib txuas zoo li peb cov tawv nqaij. Yog li, vim yog qhov tsis zoo ntawm cov noob caj noob ces no, ob txheej ntawm daim tawv nqaij, epidermis thiab dermis, tsis txuas ua ke li qub. Yog li ntawd, daim tawv nqaij ua rau tsis muaj zog heev, yooj yim ua pob, thiab raug mob.

EB feem ntau yog ib yam kab mob uas tau txais los ntawm noob caj noob ces , uas txhais tau tias yog ib tug ntawm cov niam txiv muaj tus kab mob no, cov menyuam tuaj yeem tau txais tus kab mob no los ntawm noob caj noob ces.

Txawm li cas los xij, tsis tshua muaj, EB kuj tuaj yeem tshwm sim ua tus kab mob autoimmune uas tau txais .

Qhov tseem ceeb tshaj plaws yog tias tus kab mob EB tsis yog ib yam kab mob sib kis tau. Feem ntau nws yog los ntawm noob caj noob ces. Yog li ntawd, yog tias koj tau sib cuag nrog ib tus neeg uas muaj tus kab mob EB, koj yuav tsis tau tus kab mob no.

Koj paub tseeb li cas tias koj muaj EB? (Kev kuaj mob)

Cov kws kho mob tuaj yeem kuaj mob EB los ntawm kev ua qhov biopsy ntawm daim tawv nqaij , uas yog tus kws kho mob coj ib qho qauv me me ntawm daim tawv nqaij thiab kuaj nws hauv qab lub tshuab microscope.

Ntxiv mus, kev kuaj noob caj ces tuaj yeem txheeb xyuas cov noob caj ces uas puas lawm thiab lees paub hom EB. Cov niam txiv uas tab tom npaj yuav muaj menyuam kuj tuaj yeem kuaj noob caj ces ua ntej yug menyuam kom paub seb lawv puas muaj feem yuav muaj menyuam uas muaj EB.

Cov kev kho mob rau EB yog dab tsi?

Tu siab kawg li, tam sim no tsis muaj tshuaj kho tau tus kab mob EB . Txawm li cas los xij, kev kho mob tuaj yeem pab tau:

• Pab tiv thaiv kom tsis txhob muaj cov pob liab.
• Kev saib xyuas cov hlwv thiab daim tawv nqaij kom zoo yuav tiv thaiv tau cov teeb meem.
• Kho cov teeb meem kev noj haus uas yuav tshwm sim los ntawm cov hlwv hauv qhov ncauj lossis caj pas.
• Tswj qhov mob.

Yuav kom tiv thaiv kev puas tsuaj ntawm daim tawv nqaij thiab kev tsim cov hlwv thiab cov qhov txhab vim kev sib txhuam, cov kws kho mob pom zoo kom ua raws li cov hauv qab no:

• Hnav cov khaub ncaws mos mos thiab xoob uas ua los ntawm cov xov ntuj. Hnav cov khaub ncaws sab hauv thiab sab nraud tuaj yeem pab txo qhov sib txhuam ntawm cov xov rau koj daim tawv nqaij.
• Tsis txhob kub dhau; khaws cov chav kom sov so thiab sib xws.
• Tsis txhob tawm mus rau hauv lub hnub, lossis hnav tshuaj pleev thaiv hnub thaum hnav tsom iav dub thiab kaus mom.
• Siv cov ntaub qhwv tshwj xeeb los tiv thaiv daim tawv nqaij - siv cov ntaub qhwv thiab daim kab xev uas tsis nplaum, cov ntaub qhwv uas dov tau, thiab lwm yam.

Yuav kho cov hlwv, koj tus kws kho mob yuav pom zoo cov yam li no:

• Siv cov tshuaj pleev rau cov qhov txhab txhua hnub.
• Siv cov ntaub qhwv uas muaj tshuaj los pab cov hlwv kho thiab tiv thaiv kev kis kab mob.
• Noj tshuaj kom tswj tau qhov mob.

Yuav kho cov kab mob, koj tus kws kho mob yuav pom zoo cov hauv qab no:

• Noj tshuaj tua kab mob hauv qhov ncauj los yog pleev tshuaj pleev tua kab mob.
• Siv cov khoom siv tshwj xeeb los npog qhov txhab rau cov qhov txhab uas tsis kho.

Yuav kom tiv thaiv tau cov teeb meem kev noj haus thiab kev nyuaj siab noj mov vim yog qhov ncauj lossis caj pas hlwv, koj tus kws kho mob yuav pom zoo cov hauv qab no:

• Thaum pub mis rau koj tus menyuam, siv lub raj mis menyuam yaus uas muaj lub txiv mis tshwj xeeb .
• Pub tus menyuam noj nrog ib lub raj mis tshuaj qhov muag los yog ib lub koob txhaj tshuaj.
• Ntxiv me ntsis dej rau cov khoom noj uas zom lawm kom nws yaj ua ntej pub rau noj. Qhov no yuav ua rau nws yooj yim dua rau nqos.
• Noj cov khoom noj mos mos ntau dua xws li kua zaub, cov khoom noj zom, pudding, thiab kua txiv applesauce .
• Cov khoom noj muab rau ntawm qhov kub thiab txias (tsis kub dhau).
• Mus ntsib kws noj zaub mov kom tham txog koj cov kev xav tau tshwj xeeb rau kev noj haus.

Hauv cov mob hnyav ntawm EB, koj kuj tseem yuav tsum tau phais . Yog tias lub caj pas (lub raj uas nqa zaub mov los ntawm lub qhov ncauj mus rau lub plab) raug thaiv los ntawm cov pob khaus thiab cov caws pliav, kev phais yuav ua tau kom nws dav dua. Ib txhia neeg yuav muaj peev xwm hla lub caj pas tag nrho thiab muaj lub raj pub zaub mov ntxig rau hauv lub plab kom pub zaub mov ncaj qha rau hauv lub plab. Kev phais kuj tseem yuav ua tau kom cais cov ntiv tes uas lo ua ke vim yog cov pob khaus.

Ib tug neeg uas muaj EB yuav saib xyuas lawv tus kheej li cas?

Yog koj muaj EB, cov lus qhia no yuav pab koj saib xyuas koj tus kheej:

• Pw tsaug zog rau ntawm cov ntaub pua chaw pw thiab cov hauv ncoo ua los ntawm cov xov mos mos, ntuj, xws li txhob lo lo ntxhuav lossis satin.
• Hnav cov khau xis uas tsis ua rau koj lub cev nruj.
• Tsis txhob sawv lossis taug kev ntev.
• Tsis txhob khawb los yog chwv daim tawv nqaij. Siv tshuaj tiv thaiv kev khaus kom txo qhov khaus.
• Ceev faj txog koj ib puag ncig thiab tsis txhob kov lossis khawb koj daim tawv nqaij yam tsis tau xav txog.
• Ib txwm ua kom koj daim tawv nqaij noo kom txo tau kev sib txhuam.
• Yog tias muaj cov hlwv tawm, muab rab koob uas tau ntxuav kom huv si los yog txiab huv si los tsoo kom tawg sai sai.
• Siv daim ntaub qhwv uas tsis nplaum. Los yog, muab roj petroleum jelly (Vaseline™) los yog tshuaj pleev rau daim tawv nqaij (xws li Aquaphor™) rau ntawm daim ntaub qhwv kom tiv thaiv nws kom tsis txhob lo rau daim tawv nqaij.

Yuav ua li cas saib xyuas ib tug me nyuam uas muaj EB? Qee cov lus qhia rau cov niam txiv

Yog koj tus menyuam muaj EB, lawv cov kev xav tau txawv ntawm lwm tus. Lawv yuav tsis tuaj yeem qhia lawv qhov tsis xis nyob. Cov lus qhia no tuaj yeem pab kom koj tus menyuam xis nyob li sai tau:

• Ntxuav koj txhais tes kom huv si ua ntej kov koj tus menyuam daim tawv nqaij.
• Tsis txhob siv cov hnab looj tes latex, vim tias lawv tuaj yeem ua rau muaj kev sib txhuam ntau ntxiv.
• Thaum koj tus menyuam da dej, es tsis txhob muab tag nrho lub cev tso rau hauv lub tais uas muaj dej puv nkaus ib zaug, tab sis muab txhua daim tawv nqaij ib daim zuj zus da dej.
• Es tsis txhob muab koj tus menyuam mos tso rau hauv cov ntaub qhwv menyuam, xav txog kev muab cov ntaub nqus dej rau lawv.
• Siv cov ntaub qhwv menyuam yaus uas muaj cov hlua Velcro®. Cov hlua/daim kab xev nplaum yuav lo rau ntawm daim tawv nqaij ntawm koj tus menyuam yam tsis tau xav tseg.
• Txiav thiab tshem cov roj hmab uas txuas ob txhais ceg ntawm daim pam vov.
• Muab ib yam dab tsi zoo li cov ntawv silicone gel rau sab hauv ntawm daim ntaub pam kom tiv thaiv daim tawv nqaij kom tsis txhob lo rau cov hlwv uas tau tsim.
• Ua tib zoo thaum tsa tus me nyuam.Tsis txhob tsa koj tus menyuam los ntawm kev muab koj txhais tes tso rau hauv qab caj npab (axilla). Yog tias koj tus menyuam muaj cov pob liab lossis cov qhov txhab ntawm lawv ob txhais ceg thiab nraub qaum, tsa lawv nrog ib txhais tes ncig lawv ob txhais ceg thiab lwm sab tes txhawb lawv nraub qaum.
• Txhawb koj tus menyuam kom ua ub ua no ntau li ntau tau raws li lawv tus mob EB tso cai. Kev zaum twj ywm tuaj yeem ua rau muaj lwm yam teeb meem, xws li cem quav thiab cov leeg nqaij qaug zog . Kev ua luam dej yog ib qho kev tawm dag zog zoo thiab tsis muaj kev cuam tshuam ntau.
• Tsis txhob txhawb kom ua ub ua no lossis ua si uas yuav ua rau koj tus menyuam hws lossis kub.

Kev daws cov tsos mob ntawm koj tus menyuam tej zaum yuav nyuaj rau qee lub sijhawm. Tej zaum koj yuav xav tias koj ntxhov siab thiab qaug zog. Nug koj tus kws kho mob kom paub ntau ntxiv txog kev ua kom koj tus menyuam - thiab koj - xis nyob li sai tau. Tsis tas li ntawd, los ntawm kev koom nrog cov pab pawg txhawb nqa niam txiv , koj tuaj yeem qhia koj cov kev paub dhau los thiab kawm cov hauv kev tshiab los tswj koj tus menyuam tus mob EB.

Puas tiv thaiv tau tus kab mob EB?

Vim tias EB yog los ntawm noob caj ces, nws tsis tuaj yeem tiv thaiv tau . Cov neeg uas muaj keeb kwm tsev neeg ntawm EB thiab tab tom npaj yuav muaj menyuam yuav pom tias kev sab laj txog noob caj ces yuav pab tau zoo heev rau kev txiav txim siab yuav npaj lawv tsev neeg li cas.

Tsis tas li ntawd xwb, cov kws tshaj lij tam sim no tsis paub tias yog vim li cas thiaj ua rau muaj tus kab mob EB no. Yog li ntawd, cov kws kho mob tsis paub yuav ua li cas thiaj tiv thaiv tau.

Ib tug neeg uas muaj EB yuav cia siab tau yav tom ntej zoo li cas?

Yav tom ntej ntawm cov neeg uas muaj tus kab mob EB nyob ntawm seb lawv muaj hom kab mob EB li cas thiab nws qhov hnyav npaum li cas. Cov kab mob hnyav heev tuaj yeem ua rau mob hnyav, puas qhov tsis zoo, tsis muaj peev xwm ua haujlwm, cov qhov txhab uas tsis kho tau, thiab txawm tias tuag ntxov .

Txawm li cas los xij, cov kws kho mob tuaj yeem pab koj tswj koj cov tsos mob. Qhov no tuaj yeem ua tiav nrog kev kho mob kom zoo thiab, yog tias tsim nyog, tshuaj loog mob. Khaws daim tawv nqaij kom zoo nrog cov ntaub qhwv tsis nplaum, tiv thaiv txhua ob hnub, da dej ntau zaus, thiab kev saib xyuas qhov txhab tuaj yeem pab txo qhov cuam tshuam ntawm EB rau koj lub neej.

Yog koj muaj tus kab mob EB, koj yuav tsum mus ntsib kws kho mob thaum twg?

Nco ntsoov mus ntsib koj tus kws kho mob hauv cov xwm txheej no:

• Yog koj ua pa nyuaj.
• Yog koj muaj teeb meem nqos zaub mov.
• Yog tias koj qhov txhab zoo li muaj kab mob (tawv nqaij tig liab, ntshav, grey, lossis dawb; o lossis o).
• Yog tias muaj cov tsos mob tshiab tshwm sim.

Cov lus nug tseem ceeb uas yuav nug tus kws kho mob yog dab tsi?

Thaum koj mus ntsib kws kho mob, nws yog ib lub tswv yim zoo los nug cov lus nug zoo li no:

• Koj yuav paub li cas yog tias kuv muaj EB?
• Yog tias kuv tsis muaj EB, kuv yuav muaj lwm yam kab mob ntawm daim tawv nqaij dab tsi ntxiv?
• Kuv yuav tswj kuv cov tsos mob li cas?
• Koj pom zoo kom siv cov tshuaj twg? Puas muaj tej yam tshwm sim tsis zoo?
• Koj pom zoo kom kho mob dab tsi hauv tsev?
• Kuv yuav tsum ua dab tsi ntxiv kom kuv cov tsos mob zoo dua?
• Puas muaj tshuaj pleev los yog tshuaj pleev uas koj sau tau?
• Kuv puas yuav tsum mus ntsib kws kho mob tawv nqaij lossis lwm tus kws tshwj xeeb?

Dab tsi yog qhov txawv ntawm epidermolysis bullosa (EB) thiab bullous pemphigoid?

Kab mob Bullous pemphigoid yog ib yam kab mob tawv nqaij uas tsis tshua muaj. Nws tuaj yeem ua rau khaus, zoo li cov pob khaus lossis cov kua dej puv. Kab mob Bullous pemphigoid feem ntau cuam tshuam rau cov neeg hnub nyoog tshaj 60 xyoo , thiab feem ntau nws ploj mus hauv tsib xyoos. Txawm li cas los xij, thaum muaj mob hnyav, cov pob khaus thiab cov qhov txhab tuaj yeem ua rau cov kab mob sab hauv puas tsuaj thiab ua rau tuag taus.

Txawm hais tias EB qee zaum tuaj yeem yog tus kab mob autoimmune, nws feem ntau yog tshwm sim los ntawm kev hloov pauv caj ces uas cuam tshuam rau collagen hauv koj daim tawv nqaij. EB feem ntau tshwm sim thaum yau lossis thaum yau . Vim tias tsis muaj kev kho rau EB, koj yuav tsum nyob nrog cov tsos mob rau tas koj lub neej.

Thaum kawg, tej yam yuav tsum nco ntsoov (Cov Lus Coj Mus Tsev)

Kab mob Epidermolysis bullosa (EB) yog ib qho mob nyuaj rau qee lub sijhawm. Txawm li cas los xij, nrog kev qhia kho mob, kev kho mob, thiab kev saib xyuas zoo, koj tuaj yeem tswj tau cov tsos mob thiab ua neej tau zoo li sai tau. Nco ntsoov tias, koj tsis nyob ib leeg. Cov kws kho mob, tsev neeg, thiab cov pab pawg txhawb nqa txhua tus nyob ntawd los pab koj. Yog li, tsis txhob ntshai, nyob ruaj khov, thiab ntsib qhov xwm txheej no.

Yog tias koj muaj lus nug ntxiv txog EB, nco ntsoov nrog kws kho mob tawv nqaij tham.

Epidermolysis bullosa, EB, kab mob tawv nqaij, hlwv, kab mob caj ces, kab mob tawv nqaij thaum yau, tawv nqaij tsis khov