Yu skin de blist ɛn brok if yu tɔch yu ivin smɔl? Yu tink se na Epidermolysis Bullosa (EB)?

Sɔntɛnde, wi skin kin rili sɛnsitiv, nɔto so? Ilɛksɛf smɔl tin nak am, ɔ wan klos rɔtin, i kin izi fɔ mek sɔm pipul dɛn gɛt blista ɛn wund na dɛn skin. Ɛspɛshali bikɔs di smɔl pikin dɛn skin kin rili dilik, i kin rili fred we yu si dɛn kayn tin ya. So tide wi go tɔk bɔt wan sik we kin apin na dis kayn skin, we nɔ kin apin smɔl bɔt we rili impɔtant fɔ no. Dat na epidermolysis bullosa, ɔ EB fɔ shɔt tɛm.

Wetin na di ɛksaktɔli epidamɔlaysis bulɔsa (EB)?

Fɔ tɔk am simpul wan, epidermolysis bullosa (EB) na wan sik we dɛn kin gɛt frɔm dɛn mama ɛn papa . I kin mek yu skin rili fraylayz ɛn blista ɛn peel izi wan. Sɔntɛnde, ivin if yu rɔb yu skin wit klos ɔ yu gɛt hit sɔmsay, yu kin gɛt blista ɛn sos. Fɔ sɔm pipul dɛn, i kin smɔl ɛn i kin jɔs mek dɛn gɛt blista dɛn we de mek dɛn fil pen na dɛn an, ɛlbow, ni, ɛn fut. Bɔt if i rili bad, dɛn blista ɛn sɔri ya kin apia ɛnisay na di bɔdi. EB simptom kin kɔmɔt frɔm smɔl to rili siriɔs .

Mɛmba se sɔntɛnde dɛn blista ya kin kam insay di mɔt, trot (ɛsophagus), yay, ɛn ɔda pat dɛn we de insay di bɔdi. We dɛn blista ya wɛl, dɛn kin lɛf skata we de mek pɔsin fil pen. If i siriɔs, dɛn blista ɛn skata ya kin pwɛl di insay pat dɛn na di bɔdi, ɛn dis kin mek pɔsin in layf de pan denja.

Wetin na di men kayn EB?

fכ men tכp dεm de fכ EB, we dεn kin no bay di layεr fכ di skin we dεn afekt.

1. EB Simplex (EBS): Dis na di kayn EB we kɔmɔn pas ɔl . E kin kɔmɔt frɔm wae nɔr de fil bad, nɔr de fil pen pasmak, to wae de mek pɔrsin fil pen pasmak, wae de pasmak, pan sɔm pipul dɛm. di blista dεm de fכm na di tכp layεr na di skin, di εpidamis . Ska nɔ kin bɔku afta dɛn blista ya dɔn wɛl.

2. Junctional EB (JEB): Insay JEB, blista de fכm insay di mכt εn di aywe. Dis nɔ kin apin sote , ɛn kin kɔmɔt frɔm smɔl (smɔl nɔr kin fil fayn, nɔr kin fil pen) to wae i kin tranga.

3. Dystrophic EB (DEB): Insay DEB, blista de fכm na di midul layεr na di skin, di dεmis . Dis kin difrɛn bak frɔm wae nɔr kin pasmak to wae i kin tranga.

4. Kindler Syndrome: Insay Kindler Syndrome, blista kin fכm pan kכlכsכl כl di layεr dεm na di skin. Dis nɔ kin apin so ɔltɛm .

Dɔktɔ dɛn kin difrɛns bitwin EBS, JEB, ɛn DEB bay di layers na yu skin we dɛn afɛkt. Insay Kindler syndrome, blista kin apia ɔlsay na difrɛn layers na yu skin.

Udat dɛn kin afɛkt mɔ pan dis sik? Aw i kɔmɔn?

EB kin afɛkt ɛnibɔdi. Pipul dɛn we kɔmɔt na ɛni trayb, trayb, ɔ man ɔ uman kin gɛt EB. Bɔt,If wan pan yu mama ɛn papa gɛt dis sik, yu go gɛt EB.

Pan ɔl we i kɔmɔn, akɔdin to sɔm statystik dɛn na Amɛrika, na lɛk wan pan ɛvri 50,000 pipul dɛn nɔmɔ de sɔfa wit EB. Dis min se na wan sik wae nɔr kin bɔrku.

Us ifɛkt EB kin gɛt pan di bɔdi?

If EB siriɔs, di yay kin blista ɛn ivin mek i nɔ ebul fɔ si fayn igen . Sɔntɛnde, di skin ɛn mɔsul dɛn kin gɛt bad bad skata ɛn nɔ fayn, ɛn dis kin mek i nɔ ebul fɔ muv di finga dɛn, an dɛn, fut dɛn, ɛn di jɔyn dɛn fayn fayn wan. Sɔm pipul dɛn we gɛt EB kin gɛt wan kayn kansa na dɛn skin we dɛn kɔl squamous cell carcinoma .

We i kin rili bad, sɔntɛnde i kin day we i smɔl . Dis kin bi bikɔs ɔf tin dɛn lɛk bad bad infɛkshɔn (fɔ ɛgzampul , sɛpsis ), i nɔ kin izi fɔ blo bikɔs ɔf di we aw di briz de blo, di wata we nɔ de na di bɔdi, ɛn we i nɔ de it fayn.

Yu tink se EB kin kil pɔsin?

Dis rili dipen pan di kayn EB we yu gɛt. Di kayn EB wae nɔr kin pasmak, wae nɔr kin pasmak nɔr kin kil am. Bɔt di layf we pipul dɛn we gɛt siriɔs EB kin liv kin kɔmɔt frɔm we dɛn smɔl te to lɛk 30 ia so. So dis na kɔndishɔn we nid fɔ pe atɛnshɔn to bɔku pipul dɛn.

Wetin na di sayn dɛm fɔ EB?

Di sayn dɛm fɔ EB kin difrɛn difrɛn wan bay di kayn. Dɛn sayn ya kin apin we yu na bebi ɔ yɔŋ pikin . Sɔm sayn dɛm kin kɔmɔn bitwin difrɛn kayn sik dɛm. Na sɔm pan di men sayn dɛm fɔ EB:

• Blista dɛn na di skin (an, fut, ɛlb, ni) ɔ insay di bɔdi.
• di skin we de tik na di palm dεm εn di sכl dεm na di fut dεm, we de mek dεn fil lεk kכlכs.
• di rεd bכdi sεl dεm de dכn (Anemia).
• di finga dεm we de stik togeda (fכs fכ finga dεm כ di fut dεm).
• di finga nεl dεm εn di fut nεl dεm we difכm εn/כ tik.
• di apinεns fכ sכm sכm wayt blista lεk bכmp dεm (milia) na di skin.
• I nɔ kin izi fɔ swɛla it (dysphagia).
• Lak fɔ mek pikin gro we dɛn de ɛkspɛkt.
• Tit dεm we nכ de divεlכp lεk aw dεn bin de εkspεkt ( hypoplasia ).

Wetin kin mek pɔsin gɛt EB?

Di men tin we kin mek pɔsin gɛt EB na we wan pan di 18 jin dɛn chenj ɔ we nɔ fayn . Pipul dɛn we gɛt dis sik gɛt wan jin we nɔ de ɔ we dɔn pwɛl we de ɛp fɔ mek wan prɔtin we dɛn kɔl kolagen . as yu no, kolagen na di tin we de gi trεnk εn strכkchכ to di kכnektiv tisu dεm lεk wi skin. so, biכs fכ dis jin dεfεkt, di tu layεr dεm na di skin, di εpidamis εn di dεmis, nכ de kכnekt tכgeda nכmal wan. Dis kin mek di skin kin rili brok, i kin izi fɔ mek i gɛt blista, ɛn i kin wund.

Bɔku tɛm, EB na sik we pɔsin kin gɛt frɔm in mama ɛn papa , we min se if wan pan di mama ɛn papa gɛt am, di pikin dɛn kin gɛt am.

Bɔt, na smɔl tɛm nɔmɔ, EB kin apin bak as akwyayz ɔtoimyun disɔda .

Di tin we impɔtant pas ɔl na dat EB nɔto sik we pɔsin kin gɛt. Bɔku tɛm, i kin kɔmɔt frɔm dɛn mama ɛn papa. So, if yu de in kɔntakt wit pɔsin we gɛt EB, yu nɔ go gɛt am.

Aw yu go no fɔ tru if yu gɛt EB? (Diagnosis) .

Dɔktɔ dɛn kin no if i gɛt EB bay we dɛn du wan skin biopsy , we min se dɔktɔ tek smɔl sampul pan di skin ɛn chɛk am ɔnda maykroskɔp.

apat frכm dat, jεnεtik tεst kin fכ no di jin we nכ de wok fayn εn kכnfכm di EB tכp. Mama ɛn papa dɛn we de plan fɔ bɔn pikin kin du dɛn jenɛtik tɛst bak bifo dɛn bɔn fɔ no if dɛn de pan denja fɔ bɔn pikin wit EB.

Wetin na di tritmɛnt dɛm fɔ EB?

Bɔt i sɔri fɔ no se naw, no mɛrɛsin nɔ de fɔ EB . Bɔt, di tritmɛnt dɛn kin ɛp:

• I de ɛp fɔ mek yu nɔ gɛt blista.
• We yu tek kia gud wan pan di blista ɛn di skin, dat kin mek yu nɔ gɛt prɔblɛm dɛn.
• I de trit prɔblɛm dɛn we gɛt fɔ du wit it we kin kam bikɔs ɔf blista na yu mɔt ɔ yu trot.
• Fɔ kɔntrol di pen.

Fɔ mek di skin nɔ pwɛl ɛn fɔ mek blista ɛn sos nɔ kam bikɔs ɔf frikshɔn, dɔktɔ dɛn kin se dɛn tin ya:

• Wear klos we saf ɛn we nɔ de fit we dɛn mek wit natura fayv. If yu wɛr klos insay yu skin, dat kin ɛp fɔ mek yu nɔ gɛt bɔku tin dɛn we de rɔb yu skin.
• Nɔ mek yu wam pasmak; kip di rum dɛn na fayn ples ɛn di tɛmpracha we nɔ de chenj.
• Nɔ go na do na di san, ɔ wɛr sanskrin we yu de yuz sanglas ɛn hat.
• Yuz spɛshal kayn bandej fɔ protɛkt di skin – yuz nɔ-adhesive bandej ɛn tep, rol goz, ɛn ɔda tin dɛn.

Fɔ trit blista, yu dɔktɔ kin tɛl yu fɔ du tin dɛn lɛk:

• Put ɔntmɛnt pan wund ɛvride.
• Yuz bandej we gɛt mɛrɛsin fɔ ɛp di blista dɛn fɔ wɛl ɛn fɔ mek dɛn nɔ gɛt infekshɔn.
• Tek mɛrɛsin fɔ kɔntrol di pen.

Fɔ trit infɛkshɔn, yu dɔktɔ kin tɛl yu fɔ du dɛn tin ya:

• Tek antibayɔtik we yu de it ɔ put antibayɔtik krim.
• Yuz spɛshal wund kɔva fɔ wund dɛn we nɔ de wɛl.

Fɔ mek yu nɔ gɛt prɔblɛm wit yu it ɛn i nɔ izi fɔ it bikɔs yu gɛt blista na yu mɔt ɔ yu trot, yu dɔktɔ kin tɛl yu fɔ du dɛn tin ya:

• We yu de gi yu pikin tin fɔ it, yuz pikin bɔtul we gɛt spɛshal nipple .
• Fid di pikin wit aydrop ɔ sirinj.
• Ad smɔl wata to mash it fɔ mek dɛn dil bifo yu gi dɛn. Dis go mek i izi fɔ swɛla dɛn.
• It mɔ saf it dɛn lɛk sup, mash it, pudin, ɛn apul sos .
• Dɛn kin gi it we wam (nɔto tu ɔt) tɛmpracha.
• Si to pɔsin we sabi bɔt it fɔ tɔk bɔt di patikyula tin dɛn we yu nid fɔ it.

If EB siriɔs, yu kin nid ɔpreshɔn bak . If di εsophagus (di tiub we de kɛr it frɔm di mɔt to di bɛlɛ) blok bikɔs ɔf blista ɛn skata, dɛn kin du ɔpreshɔn fɔ mek i big. sכm pipul dεn kin ebul fכ baypas di εsophagus כlsay εn dεn kin put wan fכ it tyub fכ fכ it dεn wan dεm dairekt insay di bεlε. Dɛn kin du ɔpreshɔn bak fɔ separet di finga dɛn we dɔn stɔp togɛda bikɔs ɔf di blista dɛn.

Aw pɔsin we gɛt EB kin tek kia ɔf insɛf?

If yu gɛt EB, dɛn advays ya go ɛp yu fɔ kia fɔ yusɛf:

• Slip pan sheet ɛn pilo kes dɛn we dɛn mek wit sɔft, natura fayv, lɛk silk ɔ satin.
• Wear kɔmfyut sus we nɔ de kɔnstrikt yu bɔdi.
• Nɔ tinap ɔ waka fɔ lɔng tɛm.
• Nɔ skrach ɔ pik di skin. Put mɛrɛsin dɛn we de mek yu nɔ it it fɔ mek yu nɔ it bɛtɛ.
• Yu fɔ no di tin dɛn we de arawnd yu ɛn nɔ tɔch ɔ skrach yu skin bay aksidɛnt.
• Ɔltɛm, yu skin wet fɔ mek yu nɔ gɛt frikshɔn.
• If blista dɛn bigin, pop dɛn kwik kwik wan wit nidul we dɛn dɔn stɛrilayz ɔ klin siza.
• Yuz wan bandej we nɔ de adhesive. Ɔda we de fɔ put petrolium jeli (VaselineTM) ɔ ɔntmɛnt we fayn fɔ di skin (lɛk AquaphorTM) pan di bandej fɔ mek i nɔ stik na di skin.

Aw fɔ kia fɔ pikin we gɛt EB? Sɔm advays dɛn fɔ mama ɛn papa dɛn

If yu pikin gɛt EB, wetin dɛn nid difrɛn frɔm ɔda pipul dɛn. Dɛn nɔr kin ebul fɔ tɔk bɔt aw dɛn nɔr kin fil fayn. Dɛn advays ya kin ɛp fɔ mek yu pikin fil fayn as i pɔsibul:

• Wash yu an fayn fayn wan bifo yu tɔch yu pikin in skin.
• Nɔ yuz latɛks glɔv, bikɔs i kin mek yu gɛt mɔ frikshɔn.
• We yu de was yu pikin, bifo yu put di wan ol bɔdi insay wan bɛsin we ful-ɔp wit wata wan tɛm, was ɛni pat pan di skin wan bay wan.
• Bifo yu put yu nyu bɔbɔ insay dayapɛt, tink bɔt fɔ put pad we de absɔb pan am.
• Yuz dayapɛt wit Velcro® strap. Adhesive straps/tape kin stik pan yu pikin in skin we yu nɔ no.
• Kɔt ɛn pul di ɛlastik usay di dayapɛt in leg dɛn tay.
• Put sɔntin lɛk silikon jel sheet dɛn insay di dayapɛt fɔ mek di skin nɔ stik to di blista dɛn we dɔn fɔm.
• Tek tɛm bad bad wan we yu de es di pikin.Nɔ es ɔp bay we yu put yu an dɛn ɔnda di armpit (axilla). If yu pikin gɛt blista ɔ wund na in shɔl ɛn bak, es am wit wan an rawnd in shɔl ɛn di ɔda an fɔ sɔpɔt in bak.
• Ɛnkɔrej yu pikin fɔ bi aktif as in EB kɔndishɔn alaw. If yu sidɔm wansay, dat kin mek yu gɛt ɔda prɔblɛm dɛn lɛk we yu gɛt kɔnstipɛshɔn ɛn we yu mɔsul dɛn dɔn pwɛl . Swim na fayn ɛksesaiz we nɔ de ambɔg bɔku.
• Nɔ ɛnkɔrej yu fɔ du bɔku bɔku tin dɛn ɔ fɔ ple gem dɛn we go mek yu pikin swet ɔ wam.

Sɔntɛnde, i nɔ kin izi fɔ dil wit yu pikin in sik. Yu kin fil se yu at pwɛl ɛn yu taya. Aks yu dɔktɔ fɔ mɔ advays fɔ mek yu pikin – ɛn yu – fil fayn as yu ebul. Dɔn bak, bay we yu jɔyn mama ɛn papa sɔpɔt grup , yu kin sheb yu ɛkspiriɛns ɛn lan nyu we fɔ manej yu pikin in EB kɔndishɔn.

Yu tink se dɛn kin ebul fɔ stɔp EB?

Bikɔs EB na jenɛtik, dɛn nɔ go ebul fɔ stɔp am . Pipul dɛn we gɛt famili histri bɔt EB ɛn we de plan fɔ bɔn pikin kin si se di kɔyl fɔ dɛn jɛnɛtiks kin rili ɛp fɔ disayd aw fɔ plan dɛn famili.

Apat frɔm dat, masta sabi pipul dɛn naw nɔr no wetin kin mek dɛn gɛt di kayn we aw dɛn kin gɛt EB. So, dɔktɔ dɛn nɔ no aw fɔ mek i nɔ gɛt am.

Us kayn fiuja pɔsin we gɛt EB kin ɛkspɛkt?

De fiuja fɔ pipul dɛm wae gɛt EB de dipen pan di kayn EB wae dɛn gɛt ɛn aw i siriɔs. Bɔrku kayn sik kin mek pɔrsin fil bad bad wan, kin gɛt pwɛl hat, disabled, wund wae nɔr kin ɛva wɛl, ɛn ivin day kwik .

Bɔt, dɔktɔ dɛn kin ɛp yu fɔ kɔntrol di sayn dɛn we yu gɛt. Dɛn kin du dis wit di rayt tritmɛnt ɛn if nid de, dɛn kin tek mɛrɛsin fɔ mek pɔsin fil pen. Fɔ kip di skin kɔba fayn wit dresin we nɔ de adhesive, we de protɛkt yu ɛvri ɔda de, fɔ was ɔltɛm, ɛn fɔ kia fɔ wund, dat kin ɛp fɔ ridyus di impak we EB gɛt pan yu layf.

If yu gɛt EB, ustɛm yu fɔ go to dɔktɔ?

Mek shɔ se yu go to yu dɔktɔ pan dɛn kayn tin ya:

• If yu gɛt prɔblɛm fɔ blo.
• If i nɔ izi fɔ yu fɔ swɛla it.
• If yu wund luk lɛk se i gɛt infɛkshɔn (skin de tɔn rɛd, pepul, grey, ɔ wayt; inflamed ɔ swel).
• If nyu sayn dɛn de sho.

Wetin na di impɔtant kwɛstyɔn dɛn we wi fɔ aks di dɔktɔ?

We yu go si dɔktɔ, i go fayn fɔ aks kwɛstyɔn dɛn lɛk dɛn wan ya:

• Aw yu go no if a gɛt EB?
• If a nɔ gɛt EB, us ɔda sik na mi skin a go gɛt?
• Aw a go ebul fɔ kɔntrol mi sik dɛn?
• Us mɛrɛsin dɛn yu kin advays? Dɛn gɛt bad bad tin dɛn we kin apin to dɛn?
• Us tritmɛnt dɛn na os yu kin advays?
• Wetin bak a fɔ du fɔ mek a gɛt bɛtɛ sik?
• Yu tink se krim ɔ ɔntmɛnt de we yu kin gi pɔsin?
• A fɔ go to dɔktɔ we de mɛn di bɔdi ɔ ɔda pɔsin we sabi du in wok?

Wetin na di difrɛns bitwin di epidamolysis bullosa (EB) ɛn bullous pemphigoid?

Bullous pemphigoid na wan sik we nɔ kin apin so ɔltɛm, we kin mek pɔsin nɔ ebul fɔ fɛt insɛf. I kin mek yu it, welt lɛk hayv ɔ blista we ful-ɔp wit wata. Bullous pemphigoid kin afɛkt pipul dɛm wae dɔn pas 60 ia , ɛn i kin dɔn insay fayv ia. Bɔt if i siriɔs, di blista ɛn skata kin pwɛl di insay pat dɛn ɛn i kin kil pɔsin.

Pan ɔl we sɔntɛnde EB kin bi wan sik we pɔsin kin gɛt we i de fɛt insɛf, bɔku tɛm na bikɔs ɔf wan jenɛtik chenj we kin afɛkt di kolagen na yu skin. EB kin apin we i smɔl ɔ we i smɔl . Bikɔs no mɛrɛsin nɔr de fɔ EB, yu kin gɛt fɔ liv wit di sayn dɛm fɔ di res ɔf yu layf.

Fɔ dɔn, tin dɛn fɔ mɛmba (Take-Home Message) .

Epidermolysis bullosa (EB) na wan kayn tin we kin tranga sɔm tɛm. Bɔt if yu gi yu di rayt dɔktɔ, tritmɛnt, ɛn kia fɔ yu wit lɔv, yu go ebul fɔ kɔntrol di sik dɛn ɛn liv fayn fayn wan. Mɛmba se nɔto yu wangren de. Dɔktɔ dɛn, famili, ɛn sɔpɔt grup dɛn ɔl de fɔ ɛp yu. So, nɔ fred, kɔntinyu fɔ strɔng, ɛn fes dis sityueshɔn.

If yu gɛt ɛni ɔda kwɛstyɔn bɔt EB, mek shɔ se yu tɔk to dɔktɔ we de mɛn yu skin.

` Epidermolysis bullosa, EB, skin sik, blista, jεnεtik sik, pikin skin sik, skin fragility