So wo honam ani yɛ kurukuruwa na ɛrepaapae esiane sɛ woka wo ho kakra mpo a? So ebetumi aba sɛ ɛyɛ Epidermolysis Bullosa (EB)?

Ɛtɔ mmere bi a yɛn honam ani yɛ mmerɛw paa, ɛnte saa? Sɛ biribi ketewaa bi bɔ no, anaa atade bi tetew mpo a, ɛnyɛ den sɛ nnipa binom benya akisikuru na wɔn honam ani apira. Titiriw esiane sɛ nkokoaa nkumaa honam ani yɛ mmerɛw yiye nti, sɛ wuhu nneɛma a ɛtete saa a, ɛyɛ hu paa. Enti ɛnnɛ yɛrebɛka yareɛ bi a ɛba honam ani a ɛte saa, a ɛho yɛ na kakra nanso ɛho hia paa sɛ yɛbɛhunu ho asɛm. Ɛno ne epidermolysis bullosa, anaa EB tiawa.

Dɛn ankasa ne epidermolysis bullosa (EB)?

Sɛ yɛbɛka no tiawa a, epidermolysis bullosa (EB) yɛ tebea a wonya fi awo mu . Ɛma wo honam ani yɛ mmerɛw yiye na ɛyɛ mmerɛw na ɛyɛ mmerɛw. Ɛtɔ mmere bi a, sɛ wode ntade petepete wo honam ani anaasɛ wobɔ wo baabi mpo a, wubetumi anya akisikuru ne akuru. Wɔ nnipa binom fam no, ebetumi ayɛ mmerɛw na ɛde akisikuru a ɛyɛ yaw nkutoo na ɛba wɔn nsa, wɔn nsateaa, wɔn nkotodwe, ne wɔn anan so. Nanso, wɔ tebea horow a emu yɛ den mu no, saa akisikuru ne akuru yi betumi apue wɔ nipadua no baabiara. EB ho sɛnkyerɛnne betumi afi nea emu nyɛ den so kosi nea emu yɛ den yiye so .

Kae sɛ ɛtɔ mmere bi a, saa akisikuru yi tumi ba ano, menewa (esophagus), aniwa, ne nipadua no mu akwaa afoforo mu. Sɛ saa akisikuru yi sa a, ebetumi agyaw akuru a ɛyɛ yaw. Wɔ tebea horow a emu yɛ den mu no, saa akisikuru ne akuru yi betumi asɛe nipadua no mu akwaa ahorow, na ebetumi de nkwa ato asiane mu.

Dɛn ne EB ahorow atitiriw?

EB ahorow atitiriw anan na ɛwɔ hɔ, na wɔde honam ani fã a ɛka ho no na ɛkyerɛ.

1. EB Simplex (EBS): Eyi ne EB a wɔtaa de di dwuma . Ebetumi afi nea emu nyɛ den, ɛnyɛ yaw kɛse, kosi nea ɛyɛ yaw kɛse, a emu yɛ den, wɔ nnipa binom mu. Nkuru no ba wɔ honam ani fã a ɛwɔ soro, epidermis . Akuru ntaa mma bere a saa akisikuru yi sa awie no.

2. Junctional EB (JEB): Wɔ JEB mu no, akisikuru ba ano ne mframa kwan no mu. Eyi ho yɛ na kakra , na ebetumi afi nea emu nyɛ den (a ɛnyɛ dɛ kakra, ɛyɛ yaw kakra) kosi nea emu yɛ den so.

3. Dystrophic EB (DEB): Wɔ DEB mu no, akisikuru ba wɔ honam ani mfinimfini fã, dermis . Eyi nso betumi ayɛ soronko afi nea emu nyɛ den so kosi nea emu yɛ den so.

4. Kindler Syndrome: Wɔ Kindler Syndrome mu no, ɛkame ayɛ sɛ akisikuru tumi ba honam ani fã ahorow nyinaa so. Eyi ho yɛ na koraa .

Nnuruyɛfo gyina wo honam ani ntoatoaso a ɛka wo no so kyerɛ nsonsonoe a ɛda EBS, JEB, ne DEB ntam. Wɔ Kindler syndrome mu no, akisikuru betumi apue wɔ wo honam ani mmeae ahorow nyinaa.

Henanom na tebea yi ka no kɛse? Ɛtaa ba dɛn?

EB betumi aka obiara. Nnipa a wofi abusuakuw, abusuakuw, anaa bɔbeasu biara mu betumi anya EB. Mmom,Sɛ w’awofo mu biako wɔ yare no a, ɛda adi sɛ wubenya EB.

Ɛmfa ho sɛnea abu so no, sɛnea akontaabu ahorow a ɛwɔ United States kyerɛ no, nnipa 50,000 biara mu bɛyɛ biako pɛ na ɔyare EB. Eyi kyerɛ sɛ ɛyɛ tebea a ɛntaa nsi koraa.

Nkɛntɛnso bɛn na EB betumi anya wɔ nipadua no so?

Wɔ EB a emu yɛ den mu no, aniwa no betumi ayɛ kurukuruwa na mpo ama aniwa no ayera . Ɛtɔ mmere bi a, akuru a emu yɛ den ne honam ani ntini a ɛyɛ mmerɛw betumi aba, na ɛmma wontumi ntutu nsateaa, nsa, anan, ne nkwaa no yiye. Nnipa binom a wɔwɔ EB no wɔ asiane kɛse sɛ wobenya honam ani kokoram bi a wɔfrɛ no squamous cell carcinoma .

Wɔ tebea horow a emu yɛ den sen biara mu no, ɛtɔ mmere bi a owu betumi aba wɔ nkokoaabere mu . Eyi fi tebea horow te sɛ ɔyare mmoawa a emu yɛ den (sɛ nhwɛso no , sepsis ), ahome a ɛyɛ den esiane mframa kwan a ɛsiw, nsu a ɛho nhia, ne aduan pa a wonnya nni nti.

So EB betumi akum obi?

Eyi gyina EB ko a wowɔ so ankasa. EB a emu nyɛ den na emu nyɛ den pii no nkum obi. Nanso, nnipa a wɔwɔ EB a emu yɛ den nkwa nna betumi afi nkokoaabere so akosi bɛyɛ mfe 30. Enti eyi yɛ tebea a ɛsɛ sɛ wɔde adwene si so kɛse.

Dɛn ne EB ho sɛnkyerɛnne ahorow?

Ɛsono EB ho sɛnkyerɛnne ahorow a egyina ne su so. Saa sɛnkyerɛnne ahorow yi taa da adi bere a woyɛ akokoaa anaa abofra kumaa . Nsɛnkyerɛnne ahorow bi abu so wɔ ahorow ahorow ntam. EB ho sɛnkyerɛnne atitiriw no bi ni:

• Akisikuru a ɛwɔ honam ani (nsa, nan, nsateaa, nkotodwe) anaa nipadua no mu.
• Wora a ɛyɛ den wɔ nsateaa ne nan ase, na ɛma ɛte sɛ nea ɛyɛ calluses.
• Mogya mu nkwammoaa kɔkɔɔ a ɛso tew (Anemia).
• Nsateaa a ɛbata ho (nsateaa anaa nansoaa a wɔafrafra).
• Nsateaa nnadewa ne nansoaa nnadewa a asɛe ne/anaasɛ ayɛ den.
• Nkuruwankuruwa nketenkete fitaa a ɛte sɛ akisikuru (milia) a ɛda adi wɔ honam ani.
• Aduan a ɛyɛ den sɛ wobɛmene (dysphagia).
• Onyin a wɔhwɛ kwan a enni abofra mu.
• Nsõ a ennyin sɛnea wɔhwɛɛ kwan ( hypoplasia ).

Dɛn na ɛde EB ba?

Nea ɛde EB ba titiriw ne awosu mu nkwaadɔm 18 no biako mu nsakrae anaa sintɔ . Nnipa a wɔwɔ saa yare yi wɔ awosu mu abɔde bi a ayera anaasɛ asɛe a ɛboa ma wɔyɛ protein bi a wɔfrɛ no collagen . Sɛnea wunim no, collagen ne nea ɛma ntini a ɛka bom te sɛ yɛn honam ani ahoɔden ne nhyehyɛe. Enti, esiane saa awosu mu abɔde mu sintɔ yi nti, honam ani fã abien, epidermis ne dermis, ntumi nkɔ bom sɛnea ɛsɛ. Ne saa nti, honam ani yɛ mmerɛw yiye, ɛnyɛ den sɛ ɛbɛbɔ, na apira.

Mpɛn pii no, EB yɛ yare a wonya fi awo mu , a ɛkyerɛ sɛ sɛ awofo no mu biako wɔ bi a, mmofra no betumi anya bi.

Nanso, ɛntaa nsi koraa sɛ EB betumi aba nso sɛ ɔyare a ɛma nipadua no tumi ko tia nyarewa .

Nea ɛho hia sen biara ne sɛ EB nyɛ yare a ɛde ɔyare mmoawa ba. Mpɛn pii no, efi awosu mu. Enti, sɛ wo ne obi a ɔwɔ EB di nkitaho a, worennya.

Wobɛyɛ dɛn ahu no yiye sɛ wowɔ EB? (Nyare a Wɔahu) .

Nnuruyɛfo betumi ahu EB denam honam ani nipadua mu nhwehwɛmu , a nea ɛka ho ne sɛ oduruyɛfo bi fa honam ani hwɛbea ketewaa bi na ɔde afiri a wɔde hwɛ nneɛma nketenkete hwehwɛ mu no so.

Nea ɛka ho no, awosu mu nhwehwɛmu betumi ahu awosu mu abɔde a asɛe no na wɔasi EB su no so dua. Awofo a wɔreyɛ nhyehyɛe awo ba nso betumi ayɛ awosu mu nhwehwɛmu ansa na wɔawo de ahu sɛ ebia wɔwɔ asiane mu sɛ wɔbɛwo abofra a ɔwɔ EB anaa.

Dɛn ne ayaresa ahorow a wɔde sa EB?

Nea ɛyɛ awerɛhow no, mprempren wonni aduru biara a wɔde sa EB . Nanso, ayaresa ahorow betumi aboa:

• Ɛboa ma wosiw akisikuru ano.
• Sɛ wohwɛ akisikuru ne honam ani yiye a, esiw nsɛnnennen ano.
• Ɛsa aduannuru ho haw ahorow a ebia akisikuru a ɛwɔ ano anaa menewam na ɛde ba.
• Ɛyaw a wobedi so.

Nea ɛbɛyɛ na wɔasiw honam ani asɛe na akisikuru ne akuru a ɛba esiane apereperedi nti ano no, nnuruyɛfo kamfo kyerɛ sɛ:

• Hyɛ ntade a ɛyɛ mmerɛw a ɛnyɛ den a wɔde abɔde mu nhama ayɛ. Ntade a wobɛhyɛ mu akɔ akyi no betumi aboa ma nhama dodow a ɛbɛpete wo honam ani no so atew.
• Kwati sɛ wobɛma wo ho ayɛ hyew dodo; ma adan mu hyew nyɛ nea ɛyɛ fɛ na ɛkɔ so daa.
• Kwati sɛ wobɛkɔ owia mu, anaasɛ hyɛ aduru a ɛbɔ wo ho ban fi owia ho bere a wode ahwehwɛ a wɔde hwɛ owia ne kyɛw redi dwuma no.
• Fa aduru soronko bi di dwuma de bɔ honam ani ho ban – fa aduru a ɛnyɛ nea wɔde kyekyere honam ani ne teyp, gauze a wɔabobɔw, ne nea ɛkeka ho di dwuma.

Sɛ wobɛsa akisikuru a, ebia wo duruyɛfo bɛkamfo nneɛma te sɛ:

• Fa srade gu akuru so da biara da.
• Fa nnuru a wɔde kyekyere nipadua no ho di dwuma na aboa ma akisikuru no asa na asiw ɔyare mmoawa ano.
• Nom nnuru a wode bedi ɛyaw no so.

Sɛ wobɛsa ɔyare mmoawa a, ebia wo duruyɛfo bɛkamfo nea edidi so yi akyerɛ wo:

• Fa nnuru a ekum ɔyare mmoawa a wɔnom anaa fa aduru a wɔde kum ɔyare mmoawa gu so.
• Fa nkataso soronko a wɔde kata akuru so di dwuma ma akuru a ɛnsa.

Nea ɛbɛyɛ na woasiw aduannuru ho haw ne adidi a ɛyɛ den esiane ano anaa menewa mu akisikuru nti ano no, wo duruyɛfo betumi aka nea edidi so yi akyerɛ:

• Sɛ worema wo ba no aduan a, fa akokoaa toa a nufu titiriw bi wom di dwuma .
• Fa aniwa a wɔde tow anaa syringe ma akokoaa no aduan.
• Fa nsu kakra gu aduan a wɔayam no mu na ama ayɛ mmerɛw na ma no aduan. Eyi bɛma ayɛ mmerɛw sɛ wobɛmene.
• Di nnuan a ɛyɛ mmerɛw pii te sɛ soup, nnuan a wɔayam, pudding, ne apɔw-mu-teɛteɛ .
• Wɔde aduan ma wɔ ɔhyew a ɛyɛ hyew (ɛnyɛ hyew dodo) mu.
• Kɔ aduan ho ɔbenfo bi nkyɛn kɔka wo aduannuru pɔtee a wuhia ho asɛm.

Wɔ EB a emu yɛ den mu no, ebia ebehia sɛ wɔyɛ wo oprehyɛn nso . Sɛ akisikuru ne akuru siw esophagus (afiri a ɛde aduan fi ano kɔ yafunu mu) no ano a, wobetumi ayɛ oprehyɛn de atrɛw. Ebia nnipa binom betumi atwa aduan no mu koraa na wɔde afiri a wɔde ma aduan ahyɛ mu ma wɔde aduan akɔ yafunu no mu tẽẽ. Wobetumi ayɛ oprehyɛn nso de atetew nsateaa a abata ho esiane akisikuru no nti.

Ɔkwan bɛn so na obi a ɔwɔ EB hwɛ ne ho?

Sɛ wowɔ EB a, afotu yi bɛboa wo ma woahwɛ wo ho:

• Da ntama ne pillowcases a wɔde abɔde mu nhama a ɛyɛ mmerɛw te sɛ silk anaa satin ayɛ so.
• Hyɛ mpaboa a ɛyɛ fɛ a ɛnyɛ nea ɛtwetwe wo nipadua.
• Kwati sɛ wubegyina hɔ anaa wobɛnantew bere tenten.
• Kwati sɛ wobɛtwetwe anaa wobɛtetew wo were no so. Fa nnuru a ekum akisikuru gu so na ama akisikuru no so atew.
• Hwɛ nneɛma a atwa wo ho ahyia no yiye na kwati sɛ wobɛka wo honam ani anaasɛ wobɛtwetwe wo honam ani wɔ akwanhyia mu.
• Ma wo honam ani nsu bere nyinaa na ama akasakasa so atew.
• Sɛ akisikuru ba a, fa ade a wɔde aduru a wɔde yɛ aduru anaa nkankyee a ɛho tew bɔ no ntɛmntɛm.
• Fa aduru a wɔde kyekyere ade a wɔmfa nkata so di dwuma. Sɛnea ɛbɛyɛ foforo no, fa petroleum jelly (VaselineTM) anaa srade a ɛma honam ani hwɛbea (te sɛ AquaphorTM) gu aduru a wɔde kyekyere honam ani no so na amma ankɔfa honam ani.

Ɔkwan bɛn so na wɔbɛhwɛ abofra a ɔwɔ EB? Afotu bi a wɔde ma awofo

Sɛ wo ba no wɔ EB a, ɛsono wɔn ahiade wɔ afoforo ho. Ebia wɔrentumi nka wɔn haw no ho asɛm. Saa afotu yi betumi aboa ma wo ba no ho atɔ no sɛnea obetumi:

• Hohoro wo nsa yiye ansa na wode wo nsa aka wo ba no were.
• Kwati sɛ wode latex nsateaa bedi dwuma, efisɛ ebetumi ama akasakasa ayɛ kɛse.
• Sɛ woreguare wo ba no a, sɛ́ anka wode nipadua no nyinaa bɛhyɛ ahina a nsu ahyɛ mu ma mu prɛko pɛ no, aguare honam ani fã biara mmiako mmiako.
• Sɛ́ anka wode wo ba a wɔawo no foforo bɛhyɛ mpaboa mu no, susuw ho sɛ wode nneɛma a ɛtwetwe nsu bɛhyɛ so.
• Fa mpaboa a wɔde Velcro® abɔso ayɛ di dwuma. Adhesive straps/tape betumi abata wo ba no honam ani a wunnim.
• Twitwa na yi elastic no wɔ baabi a diaper no nan abata ho no.
• Fa biribi te sɛ silicone gel ntama gu mpaboa no mu na amma were no ankɔfa akisikuru a ayɛ no ho.
• Hwɛ yiye paa bere a worema abofra no so no.Kwati sɛ wobɛma so denam wo nsa a wode bɛhyɛ abasa (axilla) ase no so. Sɛ wo ba no wɔ akisikuru anaa akuru wɔ n’asen ne n’akyi a, fa wo nsa biako ma n’asen na fa wo nsa biako nso gyina n’akyi.
• Hyɛ wo ba nkuran ma ɔnyɛ nnam sɛnea ne EB tebea no ma kwan no. Sɛ obi tra ase dinn a, ebetumi de nsɛnnennen afoforo te sɛ akisikuru ne ntini a ɛyɛ mmerɛw aba . Asuguare yɛ apɔw-mu-teɛteɛ pa a ennya nkɛntɛnso kɛse.
• Mma nhyɛ apɔw-mu-teɛteɛ a emu yɛ den anaa agodie a ɛbɛma wo ba no ho fifiri anaasɛ ne ho ayɛ hyew ho nkuran.

Ɛtɔ mmere bi a, ebetumi ayɛ den sɛ wubedi wo ba no yare no ho sɛnkyerɛnne ahorow ho dwuma. Ebia wobɛte nka sɛ woayɛ basaa na woabrɛ. Bisa wo duruyɛfoɔ ma ɔmma wo afotuo pii na ama wo ba – ne wo – ho atɔ wɔn sɛdeɛ ɛbɛyɛ yie. Afei nso, ɛdenam awofo mmoa akuw a wobɛka ho so no , wubetumi akyɛ wo suahu ahorow na woasua akwan foforo a wobɛfa so adi wo ba no EB tebea no ho dwuma.

So wobetumi asiw EB ano?

Esiane sɛ EB fi awosu mu nti, wontumi nsiw ano . Ebia nnipa a wɔwɔ abusua abakɔsɛm a ɛfa EB ho na wɔreyɛ nhyehyɛe sɛ wɔbɛwo mma no behu sɛ awosu mu afotu bɛboa wɔn kɛse wɔ sɛnea wɔbɛyɛ wɔn abusua ho nhyehyɛe ho gyinaesi mu.

Bio nso, mprempren abenfo nnim nea ɛde EB a wɔanya no ba. Enti, nnuruyɛfo nnim ɔkwan a wɔbɛfa so asiw ano.

Daakye bɛn na obi a ɔwɔ EB betumi ahwɛ kwan?

Nnipa a wɔwɔ EB daakye gyina EB ko a wɔwɔ ne sɛnea emu yɛ den so. Yare no a emu yɛ den betumi ama wate ɛyaw kɛse, adi dɛm, adi dɛm, akuru a ɛnsa da, ne owu ntɛm mpo .

Nanso, nnuruyɛfo betumi aboa wo ma woadi wo yare no ho sɛnkyerɛnne ahorow ho dwuma. Wobetumi de ayaresa a ɛfata na sɛ ɛho hia a, wɔde nnuru a wɔde sa ɛyaw ayɛ eyi. Sɛ wode nnuru a ɛnyɛ nkataso, a ɛbɔ wo ho ban kata honam ani yiye da abien biara, wotaa guare, na wohwɛ akuru no betumi aboa ma EB nkɛntɛnso a ɛwɔ w’asetra so no so atew.

Sɛ wowɔ EB a, bere bɛn na ɛsɛ sɛ wokɔ oduruyɛfo nkyɛn?

Hwɛ hu sɛ wobɛkɔ wo duruyɛfo nkyɛn wɔ nsɛm yi mu:

• Sɛ ɛyɛ den ma wo sɛ wobɛhome a.
• Sɛ ɛyɛ den ma wo sɛ wobɛmene aduan a.
• Sɛ ɛte sɛ nea wo kuru no anya ɔyare mmoawa (honam ani yɛ kɔkɔɔ, kɔkɔɔ, fitaa, anaa fitaa; ɛho ayɛ hyew anaasɛ ɛho ahonhon).
• Sɛ sɛnkyerɛnne foforo da adi a.

Nsɛmmisa a ɛho hia bɛn na ɛsɛ sɛ wobisa oduruyɛfo no?

Sɛ wokɔ oduruyɛfo nkyɛn a, ɛbɛyɛ papa sɛ wubebisa nsɛm te sɛ eyinom:

• Wobɛyɛ dɛn ahu sɛ mewɔ EB anaa?
• Sɛ minni EB a, honam ani yare foforo bɛn na metumi anya?
• Mɛyɛ dɛn atumi adi me yare no ho sɛnkyerɛnne ahorow ho dwuma?
• Nnuru bɛn na wokamfo kyerɛ? So wɔwɔ nsunsuanso bɔne?
• Ofie ayaresa bɛn na wokamfo kyerɛ?
• Dɛn bio na ɛsɛ sɛ meyɛ na ama me yare no ho sɛnkyerɛnne atu mpɔn?
• So cream anaa ngo bi wɔ hɔ a wubetumi akyerɛw ama obi?
• So ɛsɛ sɛ mekɔ honam ani yare ho oduruyɛfo anaa ɔbenfo foforo nkyɛn?

Nsonsonoe bɛn na ɛda epidermolysis bullosa (EB) ne bullous pemphigoid ntam?

Bullous pemphigoid yɛ honam ani yare a ɛntaa nsi a nipadua no tumi ko tia nyarewa. Ebetumi ama akisikuru a ɛyɛ mmerɛw a ɛte sɛ akutu anaasɛ akisikuru a nsu ahyɛ mu ma aba. Bullous pemphigoid taa ka nnipa a wɔadi boro mfe 60 , na ɛtaa kɔ wɔ mfe anum ntam. Nanso, wɔ tebea horow a emu yɛ den mu no, akisikuru ne akuru no betumi asɛe nipadua no mu akwaa ahorow na akum obi.

Ɛwom sɛ ɛtɔ mmere bi a EB betumi ayɛ yare a nipadua no tumi ko tia nyarewa de, nanso mpɛn pii no, awosu mu nsakrae a ɛka collagen a ɛwɔ wo honam ani no na ɛde ba. EB taa pue wɔ nkokoaa anaa mmofraase . Esiane sɛ wonni aduru biara a wɔde sa EB nti, ebia ɛsɛ sɛ wotra ase ne sɛnkyerɛnne ahorow no wo nkwa nna nyinaa.

Nea etwa to no, nneɛma a ɛsɛ sɛ wokae (Take-Home Message) .

Epidermolysis bullosa (EB) yɛ tebea a ɛyɛ den wɔ mmere bi mu. Nanso, sɛ wunya aduruyɛ mu afotu a ɛfata, sa wo yare, na wofi ɔdɔ mu hwɛ wo a, wubetumi adi yare no ho sɛnkyerɛnne no ho dwuma na woatra ase yiye sɛnea wubetumi biara. Kae sɛ ɛnyɛ wo nko ara. Nnuruyɛfo, abusua, ne akuw a wɔboa wo nyinaa wɔ hɔ sɛ wɔbɛboa wo. Enti, nsuro, kɔ so yɛ den, na gyina tebea yi ano.

Sɛ wowɔ nsɛmmisa foforo bi fa EB ho a, hwɛ hu sɛ wo ne honam ani yare ho oduruyɛfo bɛkasa.

` Epidermolysis bullosa, EB, honam ani yare, akisikuru, awosu mu yare, mmofra honam ani yare, honam ani yare