Hla mus rau cov ntsiab lus

Epidermolysis bullosa (EB): cov tsos mob, kev kho mob, thiab kev saib xyuas

EB yog dab tsi?

EB yog luv rau epidermolysis bullosa.

Inherited EB yog ib pab pawg ntawm cov kab mob uas tsis tshua muaj thiab mob tsis txaus ntseeg ntawm daim tawv nqaij mob uas ua rau daim tawv nqaij hlwv thiab tsim kua muag ntawm qhov kov me ntsis. Nrog daim tawv nqaij tsis yooj yim li npauj npaim tis, EB feem ntau hu ua 'butterfly daim tawv nqaij'.

  • Nws tau xav tias yuav cuam tshuam tsawg kawg 5,000 tus neeg hauv tebchaws Askiv thiab 500,000 thoob ntiaj teb. Cov nuj nqis no tuaj yeem siab dua txawm tias nws feem ntau mus tsis tau kuaj pom. Tam sim no tsis muaj kev kho mob rau EB.
  • Nws yog ib yam kab mob caj ces; koj tau yug los nrog nws txawm tias nws yuav tsis tshwm sim kom txog thaum lub neej tom ntej.
  • Hom EB koj muaj tsis hloov tom qab hauv lub neej thiab EB tsis kis tau lossis kis tau.

Tau txais EB yog lub npe hu ua EB acquisita thiab yog ib qho tsis tshua muaj, hom EB uas yog tshwm sim los ntawm tus kab mob autoimmune.

 

txheem:

Dab tsi ua rau EB?

Txhua tus neeg muaj ob daim ntawv luam ntawm txhua tus noob, ib qho dhau los ntawm txhua tus niam txiv. Ib lub noob yog ib feem ntawm DNA uas tswj hwm ib feem ntawm lub cell chemistry - tshwj xeeb tshaj yog cov protein ntau. Txhua lub noob yog tsim los ntawm DNA, uas muaj cov lus qhia ua cov protein tseem ceeb, suav nrog cov uas pab khi cov txheej txheem ntawm daim tawv nqaij ua ke. 

Nrog cov neeg uas tau txais EB, ib qho tsis raug lossis hloov pauv tau dhau los ntawm tsev neeg txhais tau hais tias thaj chaw cuam tshuam ntawm lub cev tau ploj lawm cov proteins tseem ceeb hauv lub luag haujlwm rau khi daim tawv nqaij ua ke, uas txhais tau hais tias daim tawv nqaij tuaj yeem tawg yooj yim nrog kev sib txhuam. 

Ib tug menyuam yaus, cov hluas, lossis cov neeg laus nrog EB tej zaum yuav tau txais cov noob tsis raug los ntawm niam txiv uas muaj EB, lossis lawv yuav tau txais cov noob tsis raug los ntawm ob niam txiv uas tsuas yog "cov neeg nqa khoom" tab sis tsis muaj EB lawv tus kheej. Qhov kev hloov pauv rau cov noob kuj tuaj yeem tshwm sim los ntawm lub sijhawm thaum tsis muaj leej niam leej txiv yog cov neeg nqa khoom, tab sis cov noob hloov pauv hloov pauv ntawm cov phev lossis qe ua ntej xeeb.

Tsis tshua muaj, daim ntawv hnyav ntawm EB tuaj yeem kis tau los ntawm cov kab mob autoimmune, qhov twg lub cev tsim cov tshuaj tiv thaiv los tua nws tus kheej cov ntaub so ntswg.

EB tuaj yeem tau txais txiaj ntsig los ntawm qhov tseem ceeb, qhov twg tsuas yog ib daim qauv ntawm cov noob tsis raug, lossis rov ua dua, qhov twg ob daim ntawv theej ntawm cov noob tsis raug. Cov niam txiv muaj 50% txoj hauv kev dhau ntawm daim ntawv tseem ceeb ntawm EB rau lawv tus menyuam, thaum lub caij nyoog dhau ntawm daim ntawv EB poob rau 25%. Ob leeg niam txiv tuaj yeem nqa cov noob yam tsis paub thiab pom cov tsos mob. 

Cov noob tsis raug thiab cov protein uas ploj lawm tuaj yeem tshwm sim ntawm cov txheej txheem sib txawv ntawm daim tawv nqaij, uas yog qhov txiav txim rau hom EB.

Yog xav paub ntxiv txog EB, thov mus saib cov ntawv txuas hauv qab no.

Molnlycke

NHS

Puas muaj ntau hom EB? 

Muaj kev xav tias muaj ntau dua 30 subtypes ntawm EB qub txeeg qub teg, muab faib ua plaub hom tseem ceeb hauv qab no. Txhua hom EB raug txheeb xyuas raws li cov txheej txheem ntawm daim tawv nqaij cuam tshuam los ntawm cov noob tsis raug thiab cov protein uas ploj lawm.  

Xav paub ntau ntxiv txog ntau hom EB hauv qab no.

Qhov no yog lub feem ntau cov type EB accounting rau 70% ntawm tag nrho cov mob. EBS cov tsos mob txawv in hnyav. Nrog EBS cov protein uas ploj lawm thiab fragility tshwm sim nyob rau hauv lub sab saum toj txheej ntawm daim tawv nqaij lub npe hu ua lub epidermis. 

 

Xav paub ntau ntxiv txog EB simplex

Yuav yog tsawg heev los yog mob hnyav (nyob ntawm seb nws yog dominant los yog recessive). Qhov tsis muaj protein ntau thiab fragility tshwm sim hauv qab ntawm lub hauv paus membrane, uas yog ib txheej nyias, ntom uas kab feem ntau tib neeg cov ntaub so ntswg. 25% ntawm tag nrho cov mob EB yog Dystrophic EB. 

 

Xav paub ntau ntxiv txog Dystrophic eb

Ib daim ntawv tsis tshua muaj EB uas tso npe rau cia li 5% ntawm tag nrho cov mob. JEB cov tsos mob sib txawv hauv qhov hnyav thiab tshwm sim los ntawm a tsis muaj protein ntau hauv daim tawv. Fragility tshwm sims nrogin cov qauv uas khaws cov epidermis thiab dermis (lub puab txheej ntawm ob lub ntsiab Txheej ntawm daim tawv nqaij) ua ke - hauv qab daus membrane.

 

Xav paub ntau ntxiv txog junctional eb

KEV. Ib qho tsawg heev daim ntawv ntawm EB (tsawg dua 1% ntawm cov neeg mob). npe vim cov noob caj noob ces ua lub luag haujlwm rau cov ntaub ntawv yuav tsum tau los tsim cov protein ntau Kindlin1. Nrog KEB, fragility tuaj yeem tshwm sim ntawm ntau theem ntawm daim tawv nqaij. 

 

Xav paub ntau ntxiv txog KINDLER eb

EB kuaj tau li cas? 

EB feem ntau yuav kuaj tau hauv cov menyuam mos thiab menyuam yaus thaum cov tsos mob tuaj yeem pom tseeb thaum yug los.  

Txawm li cas los xij, qee hom EB, xws li EBS, tsis tuaj yeem kuaj pom txog thaum muaj hnub nyoog laus, thiab qee zaum tsis tuaj yeem kuaj pom tag nrho, vim tias cov kws kho mob tsis nco qab paub cov tsos mob lossis feem ntau tuaj yeem kuaj pom tsis zoo raws li lwm yam mob ntawm daim tawv nqaij xws li psoriasis los yog atopic dermatitis (mob eczema). 

Yog tias nws xav tias koj tus menyuam muaj EB, lawv yuav raug xa mus rau tus kws kho mob tshwj xeeb ntawm daim tawv nqaij (dermatologist) uas tuaj yeem ua qhov kev sim (nrog koj qhov kev tso cai) los txiav txim seb hom EB thiab cov phiaj xwm txhawb nqa uas xav tau los pab tswj cov tsos mob. Lawv tuaj yeem nqa cov qauv me me ntawm daim tawv nqaij (biopsy) xa mus kuaj lossis kuaj ntshav los ntawm kev kuaj ntshav. 

Qee zaum, qhov twg muaj tsev neeg keeb kwm ntawm EB, nws tuaj yeem kuaj tus menyuam tsis tau yug los rau EB tom qab lub lim tiam 11 ntawm cev xeeb tub. 

Kev kuaj cev xeeb tub muaj xws li amniocentesis thiab chorionic villus sampling. Cov kev ntsuam xyuas no yuav raug muab yog tias koj lossis koj tus khub paub tias yog tus neeg nqa khoom ntawm cov noob tsis raug lossis puas tsuaj cuam tshuam nrog EB thiab muaj kev pheej hmoo ntawm kev muaj menyuam nrog EB. Qhov no tsis yog ib qho kev sim uas koj yuav tau them rau yog tias koj raug xa mus rau ib qho. Nws yog ib qho kev xaiv xaiv, qee cov tsev neeg yuav xaiv qhov kev sim kom pom tias lawv tus menyuam hauv plab puas raug cuam tshuam, lwm tus yuav xaiv tsis ua. Feem ntau, GP yuav ua tus xa mus, thiab tus nqi yog them los ntawm NHS. Pab neeg saib xyuas kev noj qab haus huv tshwj xeeb EB yuav sib tham txog txoj hauv kev uas yuav dhau mus. Cov tsev neeg kuj tuaj yeem raug xa mus rau kev sab laj txog caj ces yog tias lawv xav tau. 

Yog tias qhov kev ntsuam xyuas pom tau tias koj tus menyuam yuav muaj EB, koj yuav tau txais kev sab laj thiab kev tawm tswv yim los ntawm NHS uas khiav plaub lub chaw kho mob EB ntawm kev ua tau zoo hauv kev koom tes nrog DEBRA UK.  

Yog tias koj lossis ib tus neeg hauv tsev neeg tsis ntev los no tau kuaj pom tus mob EB, peb tuaj yeem pab tau koj qhov kev pabcuam koj xav tau, thov hu rau peb EB Community Support Team. Koj tuaj yeem nkag mus rau kev pab nyiaj txiag los ntawm lub NHS kev thauj mus los  yog tias koj ua tau raws li cov txheej txheem thiab tau txais cov txiaj ntsig tshwj xeeb. Xwb, koj ua tau thov rau DEBRA UK rau cov nyiaj pab txhawb nqa.

EB cuam tshuam rau lub cev li cas? 

Cov tsos mob yuav txawv nyob ntawm seb hom thiab qhov hnyav ntawm EB tab sis qhov kev sib tw tseem ceeb rau cov neeg uas muaj EB ntsib txhua hnub yog qhov mob thiab khaus uas tshwm sim vim qhov hlwv. Hauv qee hom EB suav nrog EBS, hlwv tuaj yeem nyob hauv thaj tsam ntawm tes thiab ko taw lossis dav dav thoob plaws lub cev, txawm li cas los xij hauv hom EB hnyav nws tuaj yeem cuam tshuam rau ib feem ntawm lub cev nrog rau cov mucosal linings, uas yog cov moist, sab hauv hauv ob sab phlu. cov kab mob hauv nruab nrog cev thiab lub cev xws li lub qhov ntswg, qhov ncauj, lub ntsws, thiab lub plab. Blistering kuj tuaj yeem tshwm sim ntawm lub qhov muag thiab ntawm cov kab mob hauv nruab nrog cev nrog rau lub caj pas thiab oesophagus.  

Xav paub ntau ntxiv txog seb EB cuam tshuam rau lub cev li cas hauv qab no.

  • kov los yog kev sib txhuam tuaj yeem ua rau shearing ntawm daim tawv nqaij thiab hlwv rau daim ntawv, hlwv tsis yog tus kheej txwv, yog li yuav tsum tau lanced tsis tu ncua kom tsis txhob loj tuaj.  
  • kho cov hlwv tuaj yeem ua rau mob, khaus hnyav, thiab caws pliav. 
  • Hauv qee hom EB, blistering feem ntau tshwm sim ntawm ob txhais tes thiab ko taw uas ua rau muaj teeb meem nrog kev taug kev / txav mus los, thiab lwm yam dej num txhua hnub. 
  • Nyob rau hauv cov ntaub ntawv hnyav ntawm EB, hlwv sab hauv xws li hauv lub qhov ncauj tuaj yeem ua rau nws nqos tau nyuaj thiab tuaj yeem ua rau txoj hlab pas nqaim (caj pas) thiab cov hlab pa uas yuav tsum tau muaj kev kho mob. 
  • blistering ntau thiab qhov txhab tuaj yeem ua rau daim tawv nqaij kis tau yog tias tswj tsis tau zoo. 
  • Qee hom EB tuaj yeem muaj qhov caws pliav ntau, hloov xim ntawm daim tawv nqaij thaum lub sij hawm, thiab muaj kev pheej hmoo ntau dua ntawm kev mob qog noj ntshav ntawm daim tawv nqaij.  
  • qhov tsim ntawm cov caws pliav tuaj yeem ua rau cov ntiv tes thiab cov ntiv taw sib txuas ua ke uas tuaj yeem xav tau kev kho mob. 
  • EB tuaj yeem cuam tshuam rau lwm yam kabmob hauv lub cev ntxiv rau ntawm daim tawv nqaij nrog rau cov pob txha thiab cov hnyuv, nws kuj tuaj yeem ua rau lwm yam teebmeem kev kho mob, suav nrog cem quav, tshwj xeeb tshaj yog rau cov menyuam yaus vim muaj hlwv nyob ib puag ncig hauv qab, thiab vim tias nws yog ib qho kev mob tshwm sim ntawm qee qhov. hom tshuaj kho mob. EB kuj tuaj yeem ua rau mob ntshav qab zib. Cov teebmeem ntawm EB muaj ntau lub cev thiab hauv hom EB pob txha hnyav tuaj yeem cuam tshuam loj heev. 

Ob ntawm cov tsos mob feem ntau cuam tshuam nrog EB yog mob thiab khaus. Cov tsos mob no tshwm sim los ntawm qhov tshwm sim ntau zaus thiab qee zaum blistering uas tuaj yeem tshwm sim thoob plaws lub cev thiab sab hauv vim ploj lawm lossis txawv txav los ntawm cov noob tsis raug lossis hloov pauv, uas txhais tau tias daim tawv nqaij tsis khi ua ke raws li nws yuav tsum tau ua. .  

Tam sim no tsis muaj kev kho mob rau EB tab sis muaj kev txhawb nqa kev ntsuas / tshuaj uas pab kho mob thiab khaus thiab lwm yam tsos mob. Koj tus kws kho mob tshwj xeeb EB yuav tuaj yeem qhia cov kev kho mob twg tsim nyog rau koj, tab sis hauv qab no yog cov ntsiab lus dav dav ntawm cov laj thawj thiab kev kho mob rau ob qho kev mob tshwm sim no.

Muaj ntau qhov laj thawj nyuaj vim li cas cov neeg uas muaj EB ntsib kev mob thiab txheeb xyuas qhov ua tau yog qhov tseem ceeb kom muaj kev qhia txog kev txo qhov mob. Yog tias koj tab tom ntsib kev mob thiab xav tau kev txhawb nqa, pab pawg kws kho mob tshwj xeeb EB yuav tuaj yeem qhia thiab txhawb koj nrog kev tswj xyuas qhov mob. Cov DEBRA EB Community Support Team tseem tuaj yeem muab kev txhawb nqa rau koj thiab kev xav nrog rau cov nyiaj pab rau cov khoom uas yuav pab tau qhov mob thiab khaus.  

Feem ntau ua rau mob nrog EB muaj xws li: 

  • blisters / hlwv kho. 
  • thaj chaw ntawm daim tawv nqaij poob thiab qhib qhov txhab. 
  • Cov kab mob (qhov chaw ntawm qhov txawv txav los yog cov ntaub so ntswg puas) ntawm cov mucous daim nyias nyias, uas yog cov ntaub so ntswg uas secretes mucus thiab kab kab noj hniav thiab kab noj hniav, cov no muaj xws li lub qhov ncauj, daim tawv muag, lub plab, thiab lub cornea ( pem hauv ntej ib feem ntawm lub qhov muag). 
  • kis tau tus mob. 
  • sab hauv blistering. 
  • kev raug mob ntawm daim tawv nqaij zoo li rub los yog bang. 
  • ua kom sov 
  • tsis paub ua rau lossis muaj teeb meem tsis cuam tshuam rau ntawm daim tawv nqaij. 
  • thov kev hnav khaub ncaws tsis raug lossis tshuaj pleev xim.  
  • hnav khaub ncaws hloov. 
  • rhiab heev rau cov khoom xws li ntxhua khaub ncaws thiab deodorants. 
  • cov khoom siv khaub ncaws. 

Thaum koj paub tias yog vim li cas koj thiaj raug mob (txawm tias tsis paub tias yog vim li cas), koj tuaj yeem ua haujlwm nrog koj tus kws kho mob EB ntawm txoj kev npaj txo qhov mob. Hauv qab no yog qee cov lus qhia dav dav txog kev txo qhov mob rau cov neeg nyob nrog EB, txawm li cas los xij, qhov ua haujlwm rau ib tus neeg yuav tsis ua haujlwm rau lwm tus yog li koj yuav tsum tau nrhiav tswv yim los ntawm koj tus kws kho mob EB rau koj tus kheej. 

Khaus khaus yog ib qho kev xav tsis zoo uas provokes khawb. Rau cov neeg nyob nrog EB, khaus tuaj yeem mob heev. Kev khawb tuaj yeem nyuaj rau kev tiv thaiv thiab tuaj yeem ua rau muaj kev puas tsuaj ntawm daim tawv nqaij ntxiv thiab ua rau tawg ntawm qhov txhab uas yuav luag zoo. Kev khawb kuj tuaj yeem ua rau muaj cov tshuaj tiv thaiv kab mob, uas ntxiv dag zog rau qhov khaus khaus. 

Feem ntau ua rau khaus hauv cov neeg mob EB 

  • kho cov pob khaus.  
  • daim tawv nqaij qhuav. 
  • ua kom sov 
  • o. 
  • tsis tu ncua ntawm daim tawv nqaij puas vim yog rov tshwm sim ntawm hlwv nyob rau hauv tib cheeb tsam.  
  • qee cov tshuaj opiates/opioids (cov tshuaj kho mob) tuaj yeem ua rau khaus khaus. 
  • rhiab heev rau cov khoom xws li ntxhua khaub ncaws, deodorants, thiab lwm yam khoom uas ua tau raws li daim tawv nqaij. 
  • kev ntxhov siab tuaj yeem ua rau khaus - saib cov kev sib txuas uas muaj txiaj ntsig rau cov ntaub ntawv thiab cov peev txheej los pab koj daws kev ntxhov siab. 
  • anemia uas tuaj yeem yog qhov tshwm sim ntawm EB uas ua rau khaus. 
  • tsis paub, los yog ua ke ntawm qhov ua rau. 

Hauv qhov xwm txheej hnyav tshaj plaws EB tuaj yeem pom tau zoo heev thiab tuaj yeem cuam tshuam ntau qhov chaw ntawm lub cev, txawm li cas los xij, hauv lwm qhov xwm txheej, piv txwv li EB Simplex, uas suav txog 70% ntawm tag nrho cov mob EB, nws tuaj yeem pom tsawg dua thiab cuam tshuam rau qee qhov chaw ntawm EB. lub cev zoo li ko taw. EB kuj tseem tuaj yeem yog qhov kev xiam oob khab uas txhais tau tias cov teebmeem ntawm tus neeg mob tuaj yeem hloov pauv tau. Piv txwv li, ib tus neeg uas muaj EB yuav tsis xav tau kev txhawb nqa kev mus los thiab hloov kho qhov tsim nyog los pab tswj lossis tiv thaiv lawv cov tsos mob, txawm li cas los xij lwm tus neeg uas muaj EB yuav xav tau kev pab txav mus los, thiab rau lwm tus lawv yuav muaj. ib txwm xav tau kev txhawb nqa kev mus los.

Peb cov tswvcuab tau hais rau peb tias EB tuaj yeem xav zoo li qhov kev tsis taus zais zais uas tuaj yeem tsim teeb meem ntxiv vim tias EB, hauv txhua yam, tuaj yeem ua neej nyob nrog ob lub cev thiab lub hlwb yam tsis tas yuav raug nug lossis raug ua kom xav zoo li koj yuav tsum piav qhia. nws yog dab tsi. Qhov no yog vim li cas nws yog ib qho tseem ceeb uas ntau tus neeg paub thiab nkag siab EB.

 

Nrhiav kom paub ntau ntxiv txog EB raws li kev tsis taus zais cia

Pab kev sib txuas lus

EB cov lus qhia tus neeg mob - DEBRA International

EB Treatment - NHS

Cov lus qhia me nyuam mos - Great Ormond Street

Action on Pain

Mob Kev Nyuaj Siab

Tswj qhov mob ntev - NHS

Kev tswj kev mob - Medtainment

Dawb coj kev xav rau qhov mob

Kev them nyiaj yug pw tsaug zog - Lub siab

Cov txheej txheem pw tsaug zog - NHS

Puas muaj tus kws kho mob tshwj xeeb EB? 

DEBRA UK koom tes nrog NHS txhawm rau xa cov kev pabcuam kho mob EB txhim kho uas yog qhov tseem ceeb rau cov neeg nyob nrog txhua hom EB vim nws lub hom phiaj txo qis kev pheej hmoo ntawm daim tawv nqaij ntxiv thiab teeb meem, thiab tswj cov tsos mob xws li mob thiab khaus. 

Muaj plaub lub chaw saib xyuas kev noj qab haus huv EB uas zoo tshaj plaws hauv tebchaws Askiv uas muab cov kws tshaj lij tshwj xeeb EB kev kho mob thiab kev txhawb nqa, nrog rau lwm qhov chaw hauv tsev kho mob thiab cov chaw kho mob tsis tu ncua uas lub hom phiaj muab kev pabcuam EB rau cov neeg nyob txhua qhov chaw lawv nyob. Cov pab pawg muaj xws li DEBRA EB Community Support Managers, cov kws pab tswv yim, EB cov thawj coj, cov kws saib xyuas neeg mob, thiab lwm cov kws kho mob tshwj xeeb yuav ua hauj lwm ua ke los txiav txim siab txog kev tswj cov tsos mob uas zoo tshaj rau koj, koj tus menyuam, lossis tus neeg koj saib xyuas.

Qee cov tsev neeg uas muaj EB tuaj yeem xaiv xa cov ntaub ntawv thiab cov lus qhia rau cov tub ntxhais hluas los pab lawv daws cov tsos mob, tshwj xeeb tshaj yog tias lawv tsis tuaj yeem kuaj xyuas lossis kev txhawb nqa uas lawv xav tau los ntawm lawv tus GP. Qhov no yog to taub tag nrho tab sis thov kom paub tseeb tias tus kws kho mob tshwj xeeb EB muaj los ntawm NHS kuj muaj rau koj thiab. Qhov kev pabcuam yog nyob ntawd los txhawb nqa tag nrho EB zej zog, cov neeg ntawm txhua lub hnub nyoog nyob nrog txhua hom EB.  

Peb pom zoo kom koj hu rau peb raws li peb tuaj yeem pab koj xa mus rau EB cov kws kho mob tshwj xeeb kom koj tuaj yeem tau txais cov lus qhia thiab cov ntaub ntawv tshiab txog cov kev pabcuam hauv zos. Los yog koj xav kom tsis txhob raug xa mus, peb tuaj yeem pab txhawb koj rau lwm txoj hauv kev. Txawm li cas los xij, kom paub tseeb tias koj paub txog cov kev pabcuam tshwj xeeb yog qhov tseem ceeb vim nws pab txhawb rau kev nce qib ntawm EB kev kho mob. Peb muaj daim ntawv xa mus rau tus qauv uas koj tuaj yeem siv yog tias xav tau los muab rau koj tus GP thaum thov kev xa mus. Thov tiv tauj peb kom paub ntau ntxiv.

Txhawm rau nkag mus rau EB tus kws kho mob tshwj xeeb los ntawm NHS ib txwm xav tau kev xa mus. Yog tias koj xav tias koj muaj daim ntawv EB, koj tuaj yeem mus ntsib koj tus kws kho mob, yog tias lawv tseem xav tias koj yuav muaj EB, lawv tuaj yeem xa koj mus rau ib qho ntawm EB cov chaw uas tus kws kho mob tshwj xeeb ntawm daim tawv nqaij (dermatologist) tuaj yeem siv tshuaj biopsy xa rau. kuaj lossis kuaj ntshav thiab ib zaug hauv pab pawg kws tshaj lij lawv yuav ua haujlwm nrog koj los muab kev saib xyuas zoo tshaj plaws rau koj tus menyuam. 

Txhawm rau txhawb koj tus GP hauv kev txheeb xyuas seb koj puas muaj EB lossis tsis muaj, thiab kom ntseeg tau tias lawv xa koj mus rau qhov chaw kho mob tshwj xeeb EB uas raug, peb EB Pawg Pabcuam Hauv Zej Zog tuaj yeem muab tsab ntawv uas koj tuaj yeem qhia rau koj tus GP. Thov ib tug thov hu rau peb.

Thaum koj raug kuaj pom tias muaj EB, thov thov rau ua ib tug tswv cuab ntawm DEBRA UK yog li koj tuaj yeem tau txais txiaj ntsig los ntawm cov ntaub ntawv pub dawb, cov peev txheej, thiab kev txhawb nqa uas peb muab rau txhua tus neeg hauv UK nyob nrog lossis cuam tshuam ncaj qha los ntawm EB.

Koj tuaj yeem nrhiav cov ntaub ntawv tiv tauj rau plaub lub chaw kho mob EB ntawm kev ua tau zoo tau teev tseg hauv qab no thiab lub Lwm lub tsev kho mob uas cov kws kho mob tshwj xeeb EB nyob nyob. Yog tias koj xav tau kev pab hu rau pab pawg kho mob EB lossis yog tias koj muaj lus nug txog EB kev kho mob, thov hu rau peb.

Cov pab pawg kws kho mob tshwj xeeb EB rau cov menyuam yaus thiab cov tub ntxhais hluas yog nyob ntawm Birmingham Women's and Children's Hospital, Great Ormond Street Hospital hauv London, thiab Glasgow Royal Tsev Kho Mob rau Cov Me Nyuam.  

 

Birmingham Women's and Children's Hospital 

Cov ntaub ntawv rau yuav mus tsev kho mob li cas.

Hu rau cov ntsiab lus: 

  • Hu rau - 0121 333 8757 lossis 0121 333 8224 (hais tias tus menyuam muaj EB) 
  • Email – eb.team@nhs.net 

 

Zoo Ormond Txoj Kev Tsev Kho Mob 

Cov ntaub ntawv rau yuav mus tsev kho mob li cas.

Hu rau cov ntsiab lus: 

  • Hu rau - 0207 829 7808 (EB team) los yog 0207 405 9200 (tseem ceeb switchboard) 
  • Email - ib.nurses@gosh.nhs.uk  

 

Glasgow Royal Tsev Kho Mob rau Cov Me Nyuam 

Cov ntaub ntawv rau yuav mus tsev kho mob li cas.

Hu rau cov ntsiab lus: 

 

Sharon Fisher – EB Pediatric Clinical Nurse 

 

Kirsty Walker - Tus Kws Kho Mob Dermatology 

 

Dr Catherine Drury - Tus Kws Pab Tswv Yim Dermatology  

  • Hu rau - 0141  

 

Main switchboard 

  • Hu rau - 0141  

Cov pab pawg kws kho mob tshwj xeeb EB rau cov neeg laus yog nyob ntawm Solihull Tsev Kho Mob, Guys & St.Thomas' Tsev Kho Mob hauv London, thiab Glasgow Royal Infirmary.

 

Solihull Tsev Kho Mob 

Cov ntaub ntawv hais txog yuav mus rau tsev kho mob li cas

Hu rau cov ntsiab lus: 

  • Hu rau - 0121 424 5232 los yog 0121 424 2000 (tseem ceeb switchboard)  

 

Guys & St. Thomas' Hospital  

Pab neeg laus EB saib xyuas kev noj qab haus huv ntawm Guys & St.Thomas 'Tsev Kho Mob yog nyob rau hauv Lub Chaw Tsis Muaj Kab Mob: 

Rare Diseases Center, 1st pem teb, South Wing, St Thomas' Hospital, Westminster Choj Road, London, SE1 7 EH 

Cov ntaub ntawv hais txog yuav mus rau tsev kho mob li cas

 

Hu rau cov ntsiab lus: 

  • Hu rau - EB Administrator ntawm 020 7188 0843 lossis Lub Chaw Pabcuam Tsis Muaj Kabmob txais tos ntawm 020 7188 7188 txuas ntxiv 55070 
  • Email - gst-tr.dermatologyreferralsEB@nhs.net 

 

Glasgow Royal Tsev Kho Mob 

Cov ntaub ntawv rau yuav mus tsev kho mob li cas

Hu rau cov ntsiab lus: 

Maria Avarl – EB Adult Clinical Nurse Specialist 

 

Dr Catherine Drury - Tus Kws Pab Tswv Yim Dermatology  

  • Hu rau - 0141  

 

Susan Herron - EB Kev Pabcuam Kev Lag Luam  

  • Hu rau - 0141  

 

Switchboard (A&E) 

  • Hu rau - 0141 

Yuav kho EB li cas? 

Tsis muaj kev kho mob rau EB, tab sis muaj cov kev kho mob uas tsom mus txhim kho lub neej zoo los ntawm kev tswj cov tsos mob kom zoo tshaj plaws. 

Hauv seem no koj yuav pom cov ntaub ntawv ntxiv thiab cov lus qhia txog kev kho mob thiab tswj cov tsos mob ntawm EB.  

Rau cov neeg feem coob nyob nrog EB, lossis saib xyuas ib tus neeg nrog EB, kev saib xyuas qhov txhab yog qhov tseem ceeb ntawm lub neej niaj hnub. Paub txog kev tswj cov hlwv thiab ntau hom mob, kho qhov mob thiab khaus, tiv thaiv, thiab kho tus kab mob thiab thaum twg los nrhiav kev kho mob yog txhua yam tseem ceeb hauv EB qhov txhab tu. 

Muaj ntau yam kev txhawb nqa los pab tib neeg thiab tsev neeg daws cov teeb meem ntawm kev nyob nrog EB suav nrog EB Healthcare Centers of Excellence qhov twg cov neeg mob nyob hauv ib qho ntawm plaub lub tebchaws tuaj yeem raug xa mus rau kev kho mob thiab kev txhawb nqa. EB tus kws kho mob Eb hauv cov chaw tshwj xeeb no, qee qhov uas yog ib feem ntawm cov tawv nqaij UK, suav nrog kev kho cov tawv nqaij thiab cov kev kho mob uas muaj los daws cov tsos mob. Koj tuaj yeem thov kom raug xa mus ib lub chaw los ntawm koj tus GP. Yog tias koj tus GP tsis paub meej txog kev xa mus rau koj lossis koj tsis paub meej txog qhov yuav thov, thov hu rau peb EB Community Support Team leej twg yuav tuaj yeem pab koj thiab tuaj yeem muab daim ntawv qauv qhia rau koj tus GP. 

Hauv qab no koj tuaj yeem nrhiav cov ntaub ntawv los pab koj hauv kev saib xyuas cov hlwv thiab txo qis ntawm daim tawv nqaij, nrog rau kev txuas mus rau lwm cov kev pabcuam. 

Ib qho tseem ceeb ntawm txhua txoj kev npaj kho mob rau cov neeg nyob nrog EB yog tiv thaiv kev raug mob los yog kev sib txhuam ntawm daim tawv nqaij kom txo tau qhov tshwm sim ntawm hlwv thiab yog li txo qhov mob, khaus, thiab caws pliav. Txhua tus kev paub nrog EB yog qhov txawv me ntsis thiab cov lus qhia yuav txawv nyob ntawm qhov hnyav thiab hom EB. Yog li nws tau pom zoo kom koj nrhiav kev txhawb nqa los ntawm EB tus kws kho mob tshwj xeeb rau koj tus kheej tab sis raws li kev qhia nws raug pom zoo rau: 

  • txo kev taug kev mus ntev thiab sim txuag koj cov tawv nqaij / ko taw rau kev taug kev tseem ceeb yog qhov ua tau. 
  • sim thiab tsis txhob ua pob thiab khawb thiab sim kom tsis txhob rub daim tawv nqaij - cov niam txiv yuav tsum tau hloov kho lawv li cas lawv nqa cov menyuam mos thiab menyuam yaus. 
  • sim nrhiav cov khaub ncaws zoo uas tsis rub tawm ntawm daim tawv nqaij thiab qhov twg koj tuaj yeem ua tau, zam cov seams loj, qhov twg ua tau, hnav cov fibers zoo xws li txhob lo lo ntxhuav, xyoob thiab paj rwb, vim qhov no tuaj yeem pab txo qhov khaus. 
  • khaws cov tawv nqaij kom txias li sai tau. 
  • xaiv cov khau khau uas tsis muaj tawv seams sab hauv. Xav paub ntau ntxiv nyob rau hauv khau taw qhia.
  • siv cov kev pab thiab kev hloov kho uas tau hais los ntawm koj pab neeg saib xyuas kev noj qab haus huv uas tuaj yeem yog cov kev daws teeb meem yooj yim xws li insoles lossis cov quav quav, lossis cov khoom siv txav mus los xws li lub rooj zaum log lossis rub cov hlua hauv chav dej. Nco ntsoov nug koj tus kws kho mob tshwj xeeb EB vim qee yam khoom siv lossis kev pab cuam txav mus los yuav tsis tsim nyog rau koj EB. 
  • Hais kom lwm tus xav txog koj qhov kev xav tau. 

Cov neeg nyob nrog EB feem ntau piav qhia qhov mob los ntawm lawv cov tawv nqaij puas xws li qhov kub hnyiab thib peb, thiab qee zaum, tej zaum yuav muaj qhov poob ntawm daim tawv nqaij ntau dua. Kev saib xyuas tshwj xeeb yog yuav tsum tau txwv qhov mob, khaus thiab lwm yam tsos mob cuam tshuam nrog blistering. Yog tias koj tsis paub meej tias yuav tu koj cov tawv nqaij li cas, lossis ntawm ib tus neeg hauv koj qhov kev saib xyuas, nco ntsoov xa mus rau tus kws kho mob tshwj xeeb. Koj nrhiav tau tiv tauj cov ntsiab lus rau cov chaw tshwj xeeb zoo li yuav ua li cas thaum muaj xwm ceev no.

cov DEBRA EB Community Support Team kuj tseem tuaj yeem muab cov lus qhia siv tau suav nrog kev paub txog koj cov cai thiab cov luag haujlwm ntawm cov kws qhia ntawv thiab cov tswv ntiav ua haujlwm kom paub tseeb tias kev kho mob tuaj yeem tswj tau raws sijhawm. Tham nrog cov neeg nyob ib puag ncig koj txog EB kuj tseem tuaj yeem pab koj daws cov teeb meem kev noj qab haus huv ntawm lub cev thiab lub hlwb ntawm kev nyob nrog EB.  

Yuav kom tswj tau cov tsos mob ntawm EB koj yuav xav tau qee yam khoom thiab khoom siv. Yam koj xav tau yuav nyob ntawm seb hom thiab qhov hnyav ntawm koj li EB, thiab koj tus kws kho mob EB yuav tuaj yeem qhia koj tab sis hauv qab no yog qhov qhia txog cov khoom siv thiab cov khoom siv uas koj xav tau: 

  • Txiab. Cov txiab ntse yuav tsum tau txiav thiab txiav cov ntaub qhwv, cov txiab tsis tu ncua kuj ua haujlwm rau kev txiav cov khaub ncaws. Xyuas kom koj ntxuav thiab ntxuav txiab tom qab siv txhua zaus. 
  • Hnav khaub ncaws. Muaj ntau hom kev hnav khaub ncaws muaj rau ntau hom EB thiab koj tus kws kho mob EB yuav tuaj yeem qhia koj txog kev hnav khaub ncaws zoo tshaj plaws rau koj lossis tus neeg koj saib xyuas. Nyob rau hauv txhua rooj plaub, nws yog ib qho tseem ceeb uas yuav tsum tau siv cov khoom tsis nplaum uas tsis lo rau ntawm daim tawv nqaij kom txo qis kev puas tsuaj ntxiv. 
  • ntaub nplaum. Cov ntaub qhwv yuav tsum tau ua kom ntseeg tau tias cov khaub ncaws nyob hauv qhov chaw vim yog hnav khaub ncaws plam, lawv tuaj yeem rhuav tshem cov tawv nqaij tsis zoo los yog ua rau cov qhov txhab lo rau khaub ncaws lossis txaj. Kev tuav ntaub qhwv tuaj yeem pab xyuas kom hnav khaub ncaws nyob hauv qhov chaw.  
  • Moisturizers. Kev khaus tuaj yeem yog qhov teeb meem loj hauv txhua hom EB. Raws li qhov txhab kho, lossis thaum kis kab mob tshwm sim, khaus tuaj yeem ua teeb meem tab sis ua kom daim tawv nqaij zoo moisturized tuaj yeem pab tau tiag tiag. 
  • Antimicrobial cleansers. Muaj kev pheej hmoo ntawm kev kis kab mob nrog txhua hom EB vim feem ntau thaj chaw loj ntawm qhov txhab qhib thiab yog li cov tshuaj tua kab mob tua kab mob, moisturizers thiab tshuaj pleev ib ce feem ntau tsim nyog los txo qhov kev pheej hmoo no. Kev kho tshuaj pleev ib ce yog cov tshuaj siv rau ib qho chaw ntawm lub cev nrog lub hom phiaj yog tias nws kho cov ntaub so ntswg uas nws tau thov yam tsis muaj kev cuam tshuam tseem ceeb ntawm lwm qhov chaw. 

Muaj cov chaw muag khoom tshwj xeeb uas tuaj yeem xa cov khoom xws li cov no ncaj qha mus rau koj lub tsev, thiab ntau lub tsev muag tshuaj uas muab kev pabcuam xa zaub mov yog li kos nrog koj lub tsev muag tshuaj. Ib tug ntawm cov muab kev pab no yog Bullen Healthcare uas muaj kev paub dhau los txhawb EB Community. Lawv yeej ib txwm tuav cov khoom lag luam loj thiab cov khoom siv uas feem ntau tau sau rau cov neeg nyob nrog EB thiab tseem tau tsim ib pab neeg mob siab rau pab nrog txhua EB queries thiab xaj. Yog xav paub ntxiv txog kev nkag mus rau cov khoom siv kho mob thiab tau txais cov tshuaj uas tsim nyog, thov hu rau DEBRA EB Pab Pawg Pabcuam Zej Zog.

Txawm hais tias koj tuaj yeem txo qhov kev pheej hmoo ntawm daim tawv nqaij raug mob, hlwv yog qhov tsis tuaj yeem thiab qee zaum tshwm sim, tshwm sim yam tsis muaj qhov pom tseeb. Cov hlwv kuj tsis txwv tus kheej thiab tuaj yeem loj dua yog tias nyob ib leeg. Cov hlwv loj dua = qhov txhab loj dua, thiab yog li kev tswj cov hlwv yog ib feem tseem ceeb ntawm koj txoj kev tu tawv nqaij thiab lancing lawv sai li sai tau yog qhov tseem ceeb. Thov xa mus rau seem kev pab cuam (Txuas mus rau 'RESOURCES') yog xav paub ntxiv txog kev tu tawv nqaij. 

Lub hom phiaj yog txhawm rau tiv thaiv lub hlwv kom loj dua los ntawm kev tso tawm cov kua dej, tawm hauv qhov qhib kom txaus kom tsis txhob muaj cov hlwv thiab rov ua dua, thaum tiv thaiv cov tawv nqaij nyoos hauv qab. 

Hauv qab no yog qee cov lus qhia los pab koj tswj koj cov hlwv:  

  1. Lancing los yog 'popping' hlwv nrog ib rab koob hypodermic kom tso cai rau cov kua dej tawm. Qhov no tiv thaiv lub hlwv los ntawm kev loj hlob thiab tsim kom muaj thaj tsam loj ntawm cov tawv nqaij puas. 
  2. Siv rab koob tsis muaj menyuam - qhov loj me yog qhov tseem ceeb yog li tham nrog koj tus kws kho mob kom paub tseeb tias koj siv qhov loj me. Koj tuaj yeem nug koj tus kws kho mob GP lossis EB qhov chaw tshwj xeeb rau kev muab cov koob tsis muaj menyuam. Thov nco ntsoov tias koj yuav xav tau ib lub thawv ntse thiab ib qho kev pabcuam sau rau kev pov tseg cov koob. Cov ntaub ntawv hais txog kev pov tseg cov koob.
  3. Lance ntawm qhov qis tshaj ntawm lub hlwv kom lub ntiajteb txawj nqus tuaj yeem pab tshem tawm cov kua dej tawm. 
  4. Maj mam siv cov ntaub qhwv los yog cov ntaub huv si los pab cov kua dej - qee cov neeg nyiam siv cov tshuaj ntxuav tes kom tshem tawm cov kua dej. 
  5. Tawm ntawm lub ru tsev ntawm lub hlwv kom zoo los tiv thaiv cov tawv nqaij nyoos hauv qab thiab txo qhov muaj peev xwm kis tau. 
  6. Tshem tawm cov tawv nqaij tuag los yog cov khib nyiab los ntawm ib ncig ntawm lub hlwv, thaum tawm hauv lub ru tsev ntawm lub hlwv kom zoo - dab tsis txhob rub kom txwv cov tawv nqaij puas. 
  7. Yog tias ib feem ntawm cov tawv nqaij nyoos hauv qab yog sab laug zoo li lub qhov txhab qhib, koj tuaj yeem xav hnav cov khaub ncaws uas tsis yog-sticks qhia los ntawm koj tus kws kho mob. Tsis txhob siv cov nplaum nplaum li qub vim tias cov no tuaj yeem ua rau muaj kev puas tsuaj ntxiv rau ntawm daim tawv nqaij. Yog tias siv cov nplaum nplaum ua yuam kev, muaj cov khoom siv tshem tawm cov nplaum nrog rau cov tshuaj tsuag thiab cov ntaub so ntswg uas tuaj yeem pab txo qis kev puas tsuaj rau ntawm daim tawv nqaij, cov no tuaj yeem muaj rau koj ntawm daim ntawv tshuaj los ntawm koj tus kws kho mob lossis hauv khw muag tshuaj. Nws kuj tseem yuav muaj txiaj ntsig zoo los muab cov khoom no rau koj tus menyuam lub tsev kawm ntawv, chaw zov menyuam, lossis nqa nrog koj mus rau kev teem sijhawm, xws li thaum muab ntshav. 
  8. Tej zaum nws yuav pab tau kom lub qhov txhab moist vim qhov qhuav tuaj yeem ua rau khaus zuj zus. Muaj cov cream muaj los pab nrog qhov no. 

Koj pab neeg saib xyuas kev noj qab haus huv yuav pab qhia koj txog kev kho hlwv thiab tuaj yeem pom zoo hnav khaub ncaws thiab cov khoom tsim nyog rau koj cov tawv nqaij thiab hom EB. 

Cov hlwv kuj tuaj yeem tshwm sim sab hauv - hauv qhov ncauj, qhov quav, thiab lwm yam mucous daim nyias nyias (qhov ntswg, qhov ncauj, lub ntsws plab), uas tuaj yeem ntxhov siab. Cov protein uas ploj lawm uas muab cov tawv nqaij nws lub zog yog qhia nyob rau hauv ntau cov ntaub so ntswg nyob rau hauv lub cev, nrog rau daim nyias nyias uas npog lub qhov muag thiab cov ntaub so ntswg nyob rau hauv lub qhov ncauj thiab oesophagus. Thov tham nrog koj pab neeg saib xyuas kev noj qab haus huv EB kom tau txais kev txhawb nqa txog kev kho cov hlwv hom no. 

Feem ntau, ib zaug ib lub hlwv yuav kho tau thiab tsis ua rau mob ntxiv lawm, tiam sis qee tus neeg tseem tuaj yeem hnov ​​​​mob thiab khaus yam tsis muaj hlwv.  

Ib cheeb tsam ntawm daim tawv nqaij uas tau hlwv tuaj yeem ua rau tsis yooj yim dua, tshwj xeeb tshaj yog tom qab rov tshwm sim ntawm hlwv hauv tib cheeb tsam. 

Qee lub sij hawm lub qhov txhab tsis zoo, lossis kho tau tab sis tom qab ntawd tawg dua, uas tuaj yeem ua rau mob, thiab nws tuaj yeem ua rau lub qhov txhab kis tau yooj yim dua. Qhov no yog hu ua lub qhov txhab mob. Hauv cov xwm txheej no, tham nrog koj tus kws kho mob EB txhawm rau txheeb xyuas seb vim li cas lub qhov txhab tsis kho kom lawv tuaj yeem pab tau, piv txwv li, los ntawm kev qhia lwm hom kev hnav khaub ncaws, los ntawm kev siv cov nplaum nplaum, lossis hnav khaub ncaws nrog cov tshuaj tiv thaiv kab mob / tshuaj tiv thaiv kab mob. cov khoom, lossis los ntawm kev sau ib yam dab tsi los tshem tawm tus kab mob. Kuj tseem muaj lwm yam uas tuaj yeem cuam tshuam koj qhov txhab zoo li cas nrog rau kev noj haus, pw tsaug zog thiab txo kev ntxhov siab. Thov hu rau koj tus kws kho mob tshwj xeeb EB lossis tus DEBRA EB Community Support Team rau kev noj qab haus huv. 

Kev hloov khaub ncaws ntau zaus uas yuav tsum tau tswj hwm EB tuaj yeem ua rau muaj kev ntxhov siab thiab mob heev, tab sis lawv yog ib feem ntawm lub neej ntev thiab tseem ceeb ntawm kev saib xyuas txhua hnub ntawm daim tawv nqaij, qhov txhab thiab kev tswj hlwv. 

Cov hlwv yuav tsum tau lanced kom sai li sai tau los tiv thaiv lawv ua rau mob ntxiv thiab puas. 

Lub sij hawm coj los ua kom tiav kev hnav khaub ncaws tuaj yeem sib txawv heev tab sis kom txo tau qhov mob kom zoo, nws raug nquahu kom ua kom tiav kev hnav khaub ncaws hauv lub sijhawm tsawg kawg nkaus. Muaj kev hnav khaub ncaws sib txawv, thiab xyuas kom koj muaj cov khaub ncaws uas tsim nyog tshaj plaws yog qhov tseem ceeb, xws li cov menyuam yug tshiab uas muaj kev cuam tshuam loj heev yuav tsum tau tuav tsawg kawg nkaus thiab yuav tsum tau hnav khaub ncaws uas tuaj yeem nyob twj ywm rau ob peb hnub.  

Kev hnav khaub ncaws tsis nplaum yog qhov tseem ceeb los txo qis kev puas tsuaj ntxiv thiab mob. Yog tias kev hnav khaub ncaws nplaum raug siv ua yuam kev, muaj cov khoom tshem tawm cov nplaum muaj rau koj ntawm daim ntawv xaj los ntawm koj tus GP lossis los ntawm cov khw muag tshuaj. Silicone raws li hnav khaub ncaws feem ntau yooj yim rau kev thov thiab tshem tawm dua li cov tsoos tsho, nplaum nplaum. Koj pab neeg saib xyuas kev noj qab haus huv tshwj xeeb ntawm EB yuav raug muab tso ua ke zoo tshaj plaws los qhia. 

Cov pab pawg kws kho mob tshwj xeeb EB ntawm EB cov chaw kho mob zoo tshaj plaws muaj kev txawj ntse hauv kev tswj qhov txhab thiab yuav tuaj yeem qhia txog txoj kev kho mob zoo rau koj. Thov hu rau koj tus kws kho mob tshwj xeeb EB lossis yog tias koj tsis muaj kev nkag mus rau ib tus kws tshaj lij, DEBRA EB Pab Pawg Pabcuam Zej Zog tuaj yeem pab koj nrog kev xa mus. 

Nws tuaj yeem nyuaj heev los daws qhov mob uas cuam tshuam nrog qhov txhab thiab hlwv ntawm daim tawv nqaij tshwm sim los ntawm EB txawm li cas los xij muaj cov kev xaiv sib txawv rau kev kho mob nyob ntawm qhov mob hnyav ntawm cov tsos mob thiab qhov mob tshwm sim, cov no suav nrog cream, gels, thiab qhov ncauj. tshuaj. 

Rau qee hom EB, xws li EBS, cov tshuaj tua kab mob tom khw tuaj yeem pab tau tab sis thov nco ntsoov tias cov menyuam yaus hnub nyoog qis dua 16 xyoo yuav tsum tsis txhob muab tshuaj aspirin vim muaj qhov pheej hmoo me me uas tuaj yeem ua rau mob hnyav hu ua Reye's Syndrome. , qhov no tau qhia los ntawm NHS. 

Cov kev xaiv muaj zog rau kev tswj qhov mob kuj tseem muaj nyob ntawm koj tus kws kho mob EB tshwj xeeb ntawm cov tshuaj xws li morphine, uas feem ntau siv ua ntej hloov khaub ncaws, thiab qhov ncauj sucrose tov rau cov me nyuam mos, qhov twg me me ntawm cov tshuaj qab zib (qhov ncauj sucrose) muab tso rau ntawm tus nplaig kom txo tau cov txheej txheem mob. Qhov no tau pom tias muaj txiaj ntsig ua ntej thiab thaum cov txheej txheem hauv cov menyuam yug tshiab. 

Thov sib tham txog kev siv cov tshuaj tua kab mob, txawm tias tom khw muag khoom nrog koj tus kws kho mob EB. 

Txo lub sij hawm nws yuav siv sij hawm los hloov kev hnav khaub ncaws, piv txwv li los ntawm kev siv cov qauv los txiav cov khaub ncaws ua ntej yuav txo tau lub sij hawm uas tus neeg muaj kev ntxhov siab thiab pab txo lawv qhov mob.  

Qee cov neeg nyob nrog EB pom tias ua tej yam uas lawv nyiam, xws li mloog nkauj, siv sijhawm nrog lwm tus, tawm sab nraud, ua si, lossis saib TV muab kev cuam tshuam zoo. Siv cov kev nco qab thiab kev ua pa ntawm lwm yam kev noj qab haus huv tuaj yeem pab tau. Pab pawg saib xyuas kev noj qab haus huv ntawm EB cov chaw tshwj xeeb muaj kev paub ntau hauv kev tswj kev mob thiab tuaj yeem pab txhawb koj, txawm li cas los xij me me lossis hnyav koj qhov mob yog, nrog rau cov tswv yim tswj kev mob lossis phiaj xwm kev kub ntxhov. 

Cov kev sib txuas muaj txiaj ntsig ntu muaj cov kev txuas mus rau cov koom haum uas muaj kev txhawb nqa ntawm kev tswj kev mob.  

Koj tseem tuaj yeem nkag mus rau cov lus qhia thiab cov lus qhia txog tswj kev mob ntev ntawm NHS lub vev xaib.

Qhib qhov txhab lossis daim tawv nqaij nyoos tuaj yeem kis tus kab mob uas yuav tsum tau kho sai kom tsis txhob mob ntxiv thiab puas tsuaj. Ntau yam kab mob tuaj yeem tiv thaiv tau nrog kev ntxuav tes kom huv thiab cov cuab yeej huv si yog qhov tseem ceeb thaum lancing hlwv thiab hloov khaub ncaws. 

Cov hauv qab no yuav qhia tau tias muaj tus kab mob. 

Yog tias koj ntsib ib qho ntawm cov tsos mob no lossis koj txhawj xeeb tias koj lub qhov txhab yuav kis tau, koj yuav tsum nrhiav kev tshuaj xyuas tim ntsej tim muag nrog koj tus kws kho mob hauv zos lossis tus kws kho mob. 

  • redness thiab kub nyob ib ncig ntawm daim tawv nqaij. 
  • thaj tsam ntawm daim tawv nqaij tawm cov kua paug los yog dej tawm. 
  • crusting ntawm qhov chaw ntawm qhov txhab. 
  • ib lub qhov txhab uas tsis kho. 
  • liab streak los yog kab kis tawm ntawm lub hlwv, los yog ib tug sau ntawm hlwv (tej zaum yuav nyuaj rau pom ntawm cov tawv nqaij dub los yog xim av). 
  • kub siab (ua npaws) ntawm 38C (100.4F) lossis siab dua. 
  • tus ntxhiab tsw txawv txawv. 
  • mob ntxiv. 

Thaum thawj qhov kev mob tshwm sim, thov hu rau koj tus kws kho mob lossis tus kws kho mob uas yuav muaj peev xwm muab kev kho mob tsim nyog uas yuav suav nrog tshuaj tua kab mob, tshuaj tua kab mob, gels, lossis cov kws kho mob tshwj xeeb. 

Rau kev txhawb nqa ntev thiab pab kho qhov txhab koj tuaj yeem txhawb koj txoj kev tiv thaiv kab mob los ntawm kev noj zaub mov thiab kev noj haus. Tham nrog koj tus kws kho mob tshwj xeeb EB los tham txog txoj kev npaj zaub mov zoo tshaj plaws rau koj, thiab mus saib peb cov khoom noj khoom haus thiab khoom noj khoom haus rau cov zaub mov qab los ntawm peb cov kws kho mob tshwj xeeb EB thiab cov tswv cuab, tawg nrog cov khoom xyaw noj qab haus huv los muab cov protein ntau, cov zaub mov muaj txiaj ntsig thiab, rau qee hom EB, cov zaub mov muaj calorie ntau, paj npleg, lossis khoom noj txom ncauj.

Kev tu tawv nqaij zoo yog qhov tseem ceeb los pab txo qhov khaus. Txawm hais tias qhov kev xav kom khawb tuaj yeem nyuaj rau kev tiv thaiv, qee cov neeg pom patting thaj chaw maj mam nrog ib daim ntaub ntub dej txias, los yog da dej txias muab qee qhov kev pab. Yog tias qhov khaus khaus hnyav heev, nws muaj cov tshuaj muaj xws li cov tshuaj pleev ib ce thiab tshuaj pleev los pab txo qhov khaus. 

Xyuas kom koj hydrated, tsis txhob overheating, thiab nco ntsoov cov khoom uas tiv tauj koj cov tawv nqaij, kuj yuav pab tau. 

Kev so, kev ua pa thiab kev nco qab tuaj yeem ua rau muaj kev ntxhov siab, feem ntau ua ke nrog kev kho mob thiab lwm yam kev coj cwj pwm. Cov kev daws teeb meem sib txawv ua haujlwm rau cov neeg sib txawv yog li nws yog qhov zoo tshaj los tham txog koj tus kheej cov kev xav tau nrog koj tus kws kho mob EB. 

Xav paub ntau ntxiv thiab cov lus qhia yuav ua li cas kho cov tawv nqaij khaus.

EB qhov txhab thiab hlwv tuaj yeem kho nrog caws pliav. Scarring yog ib feem ntawm lub cev cov txheej txheem kho kom zoo thaum cov ntaub so ntswg puas thiab tuaj yeem ua rau me me, sab nrauv, thiab ib ntus, lossis dav thiab ruaj khov. Feem ntau, qhov caws pliav ntau dua, qhov chaw tawg yooj yim dua tuaj yeem ua tau. Padding rau cov chaw tsis muaj zog ntawm daim tawv nqaij tuaj yeem pab txo qis thiab qeeb ntxiv kev puas tsuaj. 

Kev caws pliav ntau tuaj yeem ua rau muaj teeb meem uas yuav xav tau kev phais, koj tus kws kho mob EB yuav tham nrog koj seb cov kev kho mob twg muaj. 

Yog tias koj muaj kev txhawj xeeb txog kev caws pliav lossis xav tau kev txhawb nqa, EB pawg kws kho mob tshwj xeeb muaj kev paub dhau los hauv cheeb tsam no thiab tuaj yeem sib tham txog koj cov kev txhawj xeeb nrog rau kev txhawb nqa kev xav uas koj xav tau. 

Kev ntxhov siab thiab pw tsis tsaug zog tuaj yeem ua rau tsis zoo kev cuam tshuam kho qhov txhab thiab muaj peev xwm daws tau qhov mob. Txawm li cas los xij, cov tsos mob no tuaj yeem txhim kho los ntawm kev tswj hwm kev ntxhov siab, kev noj zaub mov zoo, tshuaj noj, kev xav, kev nco qab, thiab lwm yam kev pabcuam kev noj qab haus huv. Koj pab neeg saib xyuas kev noj qab haus huv EB yuav tuaj yeem pab koj nrhiav kev kho mob zoo rau koj, koj tuaj yeem nkag mus ntxiv cov peev txheej ntawm no Lossis tuaj xyuas no rau cov lus qhia ntxiv. 

Nyob nrog EB tuaj yeem nyuaj, tab sis DEBRA EB Community Support Team nyob ntawm no rau txhua tus neeg nyob nrog lossis cuam tshuam ncaj qha los ntawm txhua hom kev tau txais txiaj ntsig thiab tau txais EB. Pab neeg no tuaj yeem muab kev xav, kev xav, thiab cov ntaub ntawv nyiaj txiag thiab kev txhawb nqa ntawm txhua theem ntawm lub neej. 

Koom nrog DEBRA UK ua tus tswv cuab yog dawb kiag li, thiab kev ua tswv cuab muab rau koj nkag mus rau tag nrho cov kev pab cuam muab los ntawm DEBRA EB Community Support Team, ntxiv rau lwm yam kev pab zoo nrog rau cov xwm txheej, uas koj muaj peev xwm txuas nrog cov tswv cuab ntawm EB zej zog nyob rau hauv-tus neeg los yog on-line, luv nqi hnub so kev so, kev tawm tswv yim, thiab cov ntaub ntawv hais txog nyiaj txiag, kev txhawb nqa, thiab nyiaj pab. 

Kev ua tswv cuab kuj muab lub suab rau koj thiab lub cib fim los ua qhov peb ua; cov haujlwm tshawb fawb peb tau nqis peev hauv, thiab cov kev pabcuam peb muab rau tag nrho EB zej zog. Tsis tas li los ntawm kev koom ua ib tus tswv cuab koj yuav ua qhov sib txawv vim tias peb muaj cov tswv cuab ntau, cov ntaub ntawv ntau dua peb muaj, uas yog qhov tseem ceeb los txhawb EB kev tshawb fawb, thiab ntau tus tswv cuab muab peb lub suab nrov los pab txhawb tsoomfwv, NHS, thiab lwm lub koom haum rau kev txhawb nqa uas xav tau los txhim kho cov kev pabcuam kom tau txais txiaj ntsig ntawm tag nrho EB zej zog. 

Epidermolysis bullosa acquisita (EBA) 

Epidermolysis bullosa acquisita (EBA) yog hom kab mob tsawg tshaj plaws ntawm EB thiab raug cais raws li tus kab mob autoimmune, uas yog qhov chaw tiv thaiv kab mob pib tua cov nqaij mos ntawm lub cev. Nws tsis paub meej tias yog vim li cas qhov no. 

EBA ua rau cov tawv nqaij fragility zoo li lwm hom EB tab sis qhov tseem ceeb ntawm plaub hom EB yog cov kab mob caj ces tshwm sim los ntawm cov noob tsis raug lossis hloov pauv, EBA yog hom EB tau txais. 

Ib yam li lwm hom EB, EBA tuaj yeem cuam tshuam rau lub qhov ncauj, caj pas, thiab plab hnyuv. Txawm li cas los xij, tsis zoo li qee yam ntawm lwm hom EB, EBA cov tsos mob feem ntau tsis tshwm sim txog thaum lub neej tom ntej; nws feem ntau cuam tshuam rau cov neeg muaj hnub nyoog tshaj 40 xyoo. 

Qhov laj thawj tshwj xeeb ntawm EBA tsis paub tab sis nws tau xav tias lub cev tiv thaiv kab mob (cov protein nyob hauv lub cev uas yog ib feem ntawm lub cev tiv thaiv kab mob) ua yuam kev tawm tsam collagen noj qab haus huv - cov protein ntawm daim tawv nqaij uas khi cov tawv nqaij ua ke. Yog li ntawd, lub cev pib tawm tsam nws tus kheej cov ntaub so ntswg noj qab haus huv thiab ua rau blistering ntawm daim tawv nqaij thiab cov hauv ob sab phlu ntawm lub cev.   

EBA feem ntau tshwm sim rau cov neeg uas muaj lwm yam kab mob autoimmune xws li Crohn's thiab Lupus. 

Cov kws tshawb fawb tau txheeb xyuas 3 yam sib txawv ntawm EBA:   

  • Generalized inflammatory EBA - blistering thoob plaws, liab, thiab khaus, kho nrog tsawg caws pliav.  
  • Mucous membrane inflammatory EBA - blistering ntawm mucous daim nyias nyias (ib cheeb tsam ntawm lub cev lined nrog ib daim nyias nyias xws li qhov ncauj, caj pas, ob lub qhov muag, thiab plab) nrog rau tej zaum tseem ceeb caws pliav.  
  • Classic lossis tsis-inflammatory EBA - ua rau daim tawv nqaij hlwv feem ntau ntawm ob txhais tes, hauv caug, pob taws, lub luj tshib, pob taws, thiab cov chaw mucous. Scarring tuaj yeem tshwm sim lossis pob dawb (milia) tuaj yeem tsim.  

Cov tsos mob ntawm EBA tuaj yeem zoo li cov tsos mob ntawm lwm hom EB thiab tuaj yeem muaj qhov hnyav ntawm me me mus rau nruab nrab. Cov tsos mob tshwm sim muaj xws li hlwv ntawm tes, hauv caug, pob taws, lub luj tshib, thiab pob taws.  

Qhov cuam tshuam ntawm kev muaj EBA yog txiav txim siab los ntawm ib qho hauv qab lossis cuam tshuam txog kev noj qab haus huv thiab nrog rau lwm hom EB, ntau yam ntawm kev kho mob  muaj los pab txo cov tsos mob. 

Raws li nrog EB, tam sim no tsis muaj kev kho rau EBA tab sis muaj kev kho mob los txo cov tsos mob xws li mob thiab khaus. 

Ua kom muaj kev saib xyuas lub qhov txhab kom raug thiab tswj xyuas cov khoom noj kom zoo yog qhov tseem ceeb heev kom txo tau txoj kev pheej hmoo ntawm cov teeb meem. 

EBA kuj tuaj yeem kho nrog kev siv tshuaj tiv thaiv kab mob, uas yog tsim los tiv thaiv lossis txo qis kev siv tshuaj tiv thaiv kab mob hauv lub cev, thiab cov tshuaj tiv thaiv kab mob.  

Kuj tseem muaj cov piv txwv ntawm kev kho tshuaj uas tau lees tias muaj qee qhov kev vam meej hauv alleviating cov tsos mob ntawm EBA.

GPs tuaj yeem xa cov neeg mob uas muaj EBA mus rau tus kws kho mob dermatologist lossis mus rau lub tsev kho mob autoimmune los txiav txim siab txog kev kho mob uas tsim nyog. 

Yog tias koj, koj tsev neeg, lossis ib tus neeg koj saib xyuas tau kuaj pom tias muaj EBA, koj tuaj yeem tiv tauj lub DEBRA EB Community Support Team  rau kev txhawb nqa ntxiv. Peb pab neeg no nyob ntawm no los txhawb tag nrho EB zej zog hauv UK, suav nrog cov neeg nyob nrog lossis

Epidermolysis bullosa acquisita | DermNet (dermnetnz.org) 

Epidermolysis bullosa acquisita: Kev kuaj mob tam sim no thiab kho - PMC (nih.gov) 

Orphanet: Epidermolysis bullosa acquisita 

Epidermolysis bullosa acquisita (pcds.org.uk) 

Yuav ua li cas kuv yuav tsum ua li cas Kuv xav tias kuv muaj EB?

Yog tias koj xav tias koj muaj ib daim ntawv ntawm EB, koj tuaj yeem mus ntsib koj tus kws kho mob hauv zos, yog lawv xav tias koj yuav muaj daim ntawv EB ces lawv yuav xa koj mus rau ib qho ntawm cov EB specialist centers. Pab neeg kho mob ntawm EB center yuav kuaj xyuas koj cov tawv nqaij thiab tom qab ntawd lawv yuav npaj (nrog koj qhov kev tso cai) rau kev kuaj caj ces kom paub meej tias koj puas muaj daim ntawv EB. Yog tias EB raug lees paub, EB pab neeg kho mob yuav ua haujlwm nrog koj txiav txim ib txoj kev kho mob. Koj tseem yuav tau txais kev txhawb nqa los ntawm cov DEBRA EB Community Support Team.