I kekahi manawa, he ʻili koʻikoʻi loa ko kākou ʻili, ʻeā? Inā paha e pā mai ana kekahi mea liʻiliʻi, a i ʻole e haehae ʻia kekahi lole, e loaʻa koke ana i kekahi poʻe nā pehu a me nā ʻeha ʻili. ʻOiai he ʻili palupalu loa ka ʻili o nā pēpē liʻiliʻi, he mea weliweli loa ia ke ʻike ʻoe i ia mau mea. No laila, i kēia lā, e kamaʻilio ana mākou e pili ana i kahi maʻi e kū mai ana i ia ʻili, he mea kakaikahi iki akā he mea nui loa ia e ʻike. ʻO ia hoʻi ka epidermolysis bullosa, a i ʻole EB no ka pōkole.
He aha ka epidermolysis bullosa (EB)?
I ka ʻōlelo maʻalahi, he maʻi hoʻoilina ka epidermolysis bullosa (EB) . Hoʻolilo ia i kou ʻili i mea palupalu loa a pehu a ʻili maʻalahi. I kekahi manawa, inā ʻoe e hamo i kou ʻili me ka lole a i ʻole e pā ʻia i kahi, hiki iā ʻoe ke loaʻa nā pehu a me nā ʻeha. No kekahi poʻe, he akahai paha ia a hoʻoulu wale i nā pehu ʻeha ma nā lima, nā kuʻekuʻe lima, nā kuli, a me nā wāwae. Eia nō naʻe, i nā hihia koʻikoʻi, hiki i kēia mau pehu a me nā ʻeha ke ʻike ʻia ma nā wahi āpau o ke kino. Hiki i nā hōʻailona EB ke ʻano mai ka akahai a i ka koʻikoʻi loa .
E hoʻomanaʻo, i kekahi manawa hiki i kēia mau pehu ke ulu i loko o ka waha, ka ʻāʻī (esophagus), nā maka, a me nā ʻōpū kūloko ʻē aʻe o ke kino. Ke ho'ōla ʻia kēia mau pehu, hiki iā lākou ke waiho i nā ʻeha ʻeha. I nā hihia koʻikoʻi, hiki i kēia mau pehu a me nā ʻeha ke hōʻino i nā ʻōpū kūloko o ke kino, hiki ke hoʻoweliweli i ke ola.
He aha nā ʻano nui o EB?
ʻEhā ʻano nui o EB, i ʻike ʻia e ka papa o ka ʻili i hoʻopilikia ʻia.
1. EB Simplex (EBS): ʻO kēia ke ʻano maʻamau o ka EB. Hiki ke ʻano mai ka ʻoluʻolu, ʻaʻole ʻeha loa, a i ka ʻeha loa, koʻikoʻi, i kekahi poʻe. Hoʻokumu ʻia nā pehu ma ka papa luna o ka ʻili, ʻo ia hoʻi ka epidermis . He mea kākaʻikahi ka ʻeha ma hope o ka ho'ōla ʻana o kēia mau pehu.
2. Junctional EB (JEB): Ma JEB, hana ʻia nā pehu i loko o ka waha a me ke ala ea. He mea kākaʻikahi kēia, a hiki ke ʻano mai ka ʻoluʻolu iki (ʻoluʻolu ʻole iki, ʻeha iki) a i ka nui.
3. Dystrophic EB (DEB): I ka DEB, hoʻokumu ʻia nā pehu ma ka papa waena o ka ʻili, ʻo ia hoʻi ka dermis . Hiki i kēia ke ʻokoʻa mai ka ʻoluʻolu a i ka ʻino.
4. Kindler Syndrome: I ka Kindler Syndrome, hiki ke hana ʻia nā pehu ma aneane nā papa āpau o ka ʻili. He mea kākaʻikahi loa kēia.
Hoʻokaʻawale nā kauka iā EBS, JEB, a me DEB ma muli o nā papa o kou ʻili i hoʻopilikia ʻia. I ka maʻi Kindler, hiki ke ʻike ʻia nā pehu ma nā papa like ʻole o kou ʻili.
ʻO wai ka mea i hoʻopilikia nui ʻia e kēia maʻi? Pehea ka maʻamau o ia mea?
Hiki i ka EB ke hoʻopilikia i kekahi. Hiki i nā kānaka o kēlā me kēia lāhui, ʻano lāhui, a i ʻole ke kāne ke loaʻa iā EB. Eia naʻe,Inā loaʻa kekahi o kou mau mākua i ka maʻi, ʻoi aku ka nui o ka loaʻa ʻana o ka EB iā ʻoe.
ʻOiai he mea maʻamau ia, e like me nā helu helu ma ʻAmelika Hui Pū ʻIa, hoʻokahi wale nō i loko o 50,000 poʻe e ʻeha nei i ka EB. ʻO ia hoʻi, he maʻi kakaikahi ia.
He aha nā hopena o ka EB i ke kino?
I nā hihia koʻikoʻi o ka EB, hiki i nā maka ke pehu a hiki ke lilo i ka ʻike . I kekahi manawa, hiki ke loaʻa ka ʻeha nui a me ke ʻano ʻē o ka ʻili a me nā ʻiʻo, e hiki ʻole ai ke hoʻoneʻe pono i nā manamana lima, nā lima, nā wāwae, a me nā hono. Loaʻa i kekahi poʻe me EB kahi pilikia nui o ka hoʻomohala ʻana i kahi ʻano maʻi ʻili i kapa ʻia ʻo squamous cell carcinoma .
I nā hihia koʻikoʻi loa, hiki ke make i kekahi manawa i ka wā kamaliʻi . ʻO kēia ma muli o nā kūlana e like me nā maʻi koʻikoʻi (no ka laʻana , sepsis ), ka paʻakikī o ka hanu ʻana ma muli o ke ālai ʻana o ke ala ea, ka maloʻo ʻana o ka wai, a me ka ʻai ʻole.
Hiki i ka EB ke make?
Aia maoli kēia i ke ʻano o ka EB āu e loaʻa ai. ʻAʻole make nā ʻano EB ʻoluʻolu a ʻaʻole koʻikoʻi. Eia nō naʻe, hiki i ke ola o ka poʻe me nā ʻano EB koʻikoʻi ke piʻi mai ka wā kamaliʻi a hiki i ka 30 mau makahiki. No laila, he ʻano kēia e pono ai ka nānā nui ʻia.
He aha nā hōʻailona o ka EB?
ʻOkoʻa nā hōʻailona o ka EB ma muli o ke ʻano. ʻIke pinepine ʻia kēia mau hōʻailona i ka wā he pēpē a he keiki ʻōpiopio paha ʻoe . He mea maʻamau kekahi mau hōʻailona ma waena o nā ʻano like ʻole. Eia kekahi o nā hōʻailona nui o ka EB:
- Nā pehu ma ka ʻili (lima, wāwae, kuʻekuʻe lima, kuli) a i loko paha o ke kino.
- Ka mānoanoa o ka ʻili ma nā poho lima a me nā kapuwaʻi, e hoʻolilo ana iā lākou e like me nā calluses.
- Ka emi ʻana o nā hunaola koko ʻulaʻula (Anemia).
- Nā manamana lima e pipili ana (nā manamana lima a i ʻole nā manamana wāwae i hui pū ʻia).
- Nā kui lima a me nā kui wāwae i kīnā a/a i ʻole mānoanoa.
- ʻO ke ʻano o nā puʻupuʻu keʻokeʻo liʻiliʻi e like me ka pehu (milia) ma ka ʻili.
- Ka paʻakikī i ka ale ʻana i ka meaʻai (dysphagia).
- Ka nele o ka ulu ʻana i manaʻo ʻia i loko o ke keiki.
- ʻAʻole ulu nā niho e like me ka mea i manaʻo ʻia ( hypoplasia ).
He aha ke kumu o ka EB?
ʻO ke kumu nui o ka EB he mutation a kīnā paha i loko o kekahi o nā genes 18. Loaʻa i ka poʻe me kēia maʻi kahi gene nalowale a hōʻino paha e kōkua ana i ka hana ʻana i kahi protein i kapa ʻia ʻo collagen . E like me kou ʻike, ʻo ka collagen ka mea e hāʻawi i ka ikaika a me ke ʻano i nā ʻiʻo pili e like me ko kākou ʻili. No laila, ma muli o kēia kīnā gene, ʻaʻole pili pono nā papa ʻelua o ka ʻili, ʻo ka epidermis a me ka dermis. ʻO ka hopena, lilo ka ʻili i mea palupalu loa, maʻalahi ka pehu, a me ka ʻeha.
He maʻi hoʻoilina pinepine ka EB, ʻo ia hoʻi inā loaʻa i kekahi o nā mākua, hiki i nā keiki ke hoʻoilina.
Eia nō naʻe, he mea kakaikahi loa, hiki i ka EB ke hana ʻia ma ke ʻano he maʻi autoimmune i loaʻa .
ʻO ka mea nui loa, ʻaʻole he maʻi lele ka EB. He maʻi hoʻoilina ia. No laila, inā ʻoe e launa pū me kekahi mea i loaʻa iā EB, ʻaʻole ʻoe e loaʻa.
Pehea ʻoe e ʻike pono ai inā loaʻa iā ʻoe ʻo EB? (Diagnosis)
Hiki i nā kauka ke ʻike i ka EB ma ka hana ʻana i kahi biopsy ʻili , kahi e pili ana i kahi kauka e lawe ana i kahi ʻāpana liʻiliʻi o ka ʻili a nānā iā ia ma lalo o kahi microscope.
Eia kekahi, hiki i ka hoʻāʻo ʻana i ka genetic ke ʻike i ka gene kīnā a hōʻoia i ke ʻano EB. Hiki i nā mākua e hoʻolālā ana e loaʻa kahi keiki ke hana i ka hoʻāʻo genetic prenatal e ʻike ai inā aia lākou i ka pilikia o ka loaʻa ʻana o kahi keiki me EB.
He aha nā lāʻau lapaʻau no ka EB?
ʻO ka mea pōʻino, ʻaʻohe lāʻau lapaʻau no ka EB i kēia manawa . Eia nō naʻe, hiki i nā lāʻau lapaʻau ke kōkua:
- Kōkua i ka pale ʻana i nā pehu.
- ʻO ka mālama pono ʻana i nā pehu a me ka ʻili e pale aku i nā pilikia.
- Hoʻōla i nā pilikia meaʻai i hiki ke hana ʻia e nā pehu ma ka waha a i ʻole ka puʻu.
- Ke kāohi nei i ka ʻeha.
I mea e pale ai i ka hōʻino ʻana o ka ʻili a me ke kūkulu ʻia ʻana o nā pehu a me nā ʻeha ma muli o ka friction, ʻōlelo nā kauka i kēia mau mea:
- E ʻaʻahu i nā lole palupalu a palupalu i hana ʻia mai nā olonā kūlohelohe. ʻO ke komo ʻana i nā lole i loko a i waho e kōkua i ka hōʻemi ʻana i ka nui o nā humuhumu e hamo ana i kou ʻili.
- E pale i ka wela nui ʻana; e mālama i nā lumi i kahi mahana ʻoluʻolu a kūlike.
- Mai hele i waho i ka lā, a i ʻole e ʻaʻahu i ka sunscreen i ka wā e hoʻohana ana i nā aniani lā a me nā pāpale.
- E hoʻohana i nā ʻano kāʻei kūikawā e pale i ka ʻili - e hoʻohana i nā kāʻei a me ka lipine ʻaʻole e pipili, ka gauze i ʻōwili ʻia, a pēlā aku.
No ka mālama ʻana i nā pehu, hiki i kāu kauka ke ʻōlelo i nā mea e like me:
- E hoʻopili i nā mea poni i nā ʻeha i kēlā me kēia lā.
- E hoʻohana i nā kāʻei lāʻau lapaʻau e kōkua i ka ho'ōla ʻana i nā pehu a pale i ka maʻi.
- E lawe i ka lāʻau lapaʻau e kāohi i ka ʻeha.
No ka mālama ʻana i nā maʻi, hiki i kāu kauka ke ʻōlelo i kēia mau mea:
- E lawe i nā lāʻau antibiotic waha a i ʻole e kau i kahi kirīmi antibiotic.
- E hoʻohana i nā uhi ʻeha kūikawā no nā ʻeha i ho'ōla ʻole ʻia.
I mea e pale ai i nā pilikia meaʻai a me ka paʻakikī i ka ʻai ʻana ma muli o nā pehu waha a i ʻole ka puʻu, hiki i kāu kauka ke ʻōlelo i kēia mau mea:
- I ka hānai ʻana i kāu pēpē, e hoʻohana i kahi ʻōmole pēpē me kahi nipple kūikawā .
- E hānai i ke pēpē me kahi eyedropper a i ʻole kahi syringe.
- E hoʻohui i kahi wai liʻiliʻi i nā meaʻai i kuʻi ʻia e hoʻoheheʻe iā lākou ma mua o ka hānai ʻana. E maʻalahi kēia i ka ale ʻana.
- E ʻai i nā meaʻai palupalu e like me ka sopa, nā meaʻai i kuʻi ʻia, ka pudding, a me ka applesauce .
- Hāʻawi ʻia ka meaʻai ma kahi mahana mehana (ʻaʻole wela loa).
- E ʻike i kahi meaʻai meaʻai e kūkākūkā i kāu mau pono meaʻai kikoʻī.
I nā hihia koʻikoʻi o ka EB, pono paha ʻoe i ke ʻoki kino . Inā ua ālai ʻia ka esophagus (ka paipu e lawe ana i ka meaʻai mai ka waha a i ka ʻōpū) e nā pehu a me nā ʻōpala, hiki ke hana ʻia ke ʻoki kino e hoʻonui iā ia. Hiki i kekahi poʻe ke kāpae loa i ka esophagus a hoʻokomo ʻia kahi paipu hānai e hānai pololei i ka meaʻai i loko o ka ʻōpū. Hiki ke hana ʻia ke ʻoki kino e hoʻokaʻawale i nā manamana lima i paʻa pū ma muli o nā pehu.
Pehea e mālama ai kekahi me EB iā lākou iho?
Inā loaʻa iā ʻoe ka EB, e kōkua kēia mau ʻōlelo aʻoaʻo iā ʻoe e mālama iā ʻoe iho:
- E hiamoe ma luna o nā pepa moe a me nā uluna i hana ʻia mai nā olonā palupalu a kūlohelohe, e like me ke silika a i ʻole ke satin.
- E ʻaʻahu i nā kāmaʻa ʻoluʻolu ʻaʻole e hoʻopaʻa i kou kino.
- E pale i ke kū ʻana a i ʻole ka hele ʻana no ka manawa lōʻihi.
- Mai ʻōʻō a ʻōʻō paha i ka ʻili. E hoʻopili i nā lāʻau anti-manicule e hōʻemi i ka manicule.
- E makaʻala i kou wahi a puni a pale i ka hoʻopā ʻana a i ʻole ke kahakaha ʻana i kou ʻili.
- E hoʻomaʻū mau i kou ʻili e hōʻemi i ka friction.
- Inā kū mai nā pehu, e hou koke iā lākou me ka nila i hoʻomaʻemaʻe ʻia a i ʻole ka ʻūpā maʻemaʻe.
- E hoʻohana i kahi kāʻei ʻaʻole e pipili. A i ʻole, e hoʻopili i ka petroleum jelly (Vaseline™) a i ʻole kahi poni ʻili (e like me Aquaphor™) i ke kāʻei e pale aku ai i ka pipili ʻana i ka ʻili.
Pehea e mālama ai i kahi keiki me EB? Kekahi mau ʻōlelo aʻoaʻo no nā mākua
Inā loaʻa i kāu keiki ka maʻi EB, ʻokoʻa ko lākou mau pono mai nā poʻe ʻē aʻe. ʻAʻole paha lākou e hiki ke hōʻike i ko lākou ʻeha. Hiki i kēia mau ʻōlelo aʻoaʻo ke kōkua i kāu keiki e ʻoluʻolu e like me ka hiki:
- E holoi pono i kou mau lima ma mua o ka hoʻopā ʻana i ka ʻili o kāu pēpē.
- E hōʻole i ka hoʻohana ʻana i nā mīkina lima latex, no ka mea hiki iā lākou ke hoʻonui i ka friction.
- I ka ʻauʻau ʻana i kāu pēpē, ma kahi o ke kau ʻana i ke kino holoʻokoʻa i loko o kahi pola i piha i ka wai i ka manawa hoʻokahi, e ʻauʻau i kēlā me kēia ʻāpana o ka ʻili hoʻokahi i ka manawa.
- Ma kahi o ke kau ʻana i kāu keiki hānau hou i loko o nā diapers, e noʻonoʻo e kau i nā pads absorbent ma luna o lākou.
- E hoʻohana i nā diaper me nā kaula Velcro®. Hiki i nā kaula/lipine pipili ke pipili me ka manaʻo ʻole i ka ʻili o kāu pēpē.
- E ʻoki a wehe i ka elastic kahi i hoʻopili ʻia ai nā wāwae o ka diaper.
- E kau i kahi mea e like me nā pepa gel silicone ma loko o ka diaper e pale ai i ka pipili ʻana o ka ʻili i nā pehu i hana ʻia.
- E akahele loa i ka hāpai ʻana i ke keiki.E pale i ka hāpai ʻana ma ke kau ʻana i kou mau lima ma lalo o nā ʻāʻī (axilla). Inā he mau pehu a ʻeha paha kāu keiki ma ko lākou mau ʻūhā a me ke kua, e hāpai iā lākou me kekahi lima a puni ko lākou mau ʻūhā a me kekahi lima e kākoʻo ana i ko lākou kua.
- E paipai i kāu keiki e hana ikaika e like me ka mea e ʻae ai ko lākou maʻi EB. Hiki i ka noho mālie ke alakaʻi i nā pilikia ʻē aʻe, e like me ka paʻa ʻana o ka ʻōpū a me ka atrophy o nā ʻiʻo. He hoʻoikaika kino maikaʻi ka ʻauʻau ʻana, he haʻahaʻa ka hopena.
- Mai paipai i nā hana kino ikaika a i ʻole nā pāʻani e hoʻohou ai i kāu keiki a wela paha.
Hiki ke paʻakikī ke hoʻoponopono ʻana i nā hōʻailona o kāu keiki i kekahi manawa. Hiki iā ʻoe ke manaʻo ua kaumaha a luhi hoʻi. E noi i kāu kauka no nā ʻōlelo aʻoaʻo hou aku e mālama i kāu keiki - a me ʻoe - i ka ʻoluʻolu e like me ka hiki. Eia kekahi, ma ke komo ʻana i nā hui kākoʻo makua , hiki iā ʻoe ke kaʻana like i kāu mau ʻike a aʻo i nā ala hou e hoʻokele ai i ke kūlana EB o kāu keiki.
Hiki ke pale ʻia ka EB?
No ka mea he genetic ka EB, ʻaʻole hiki ke pale ʻia . ʻO ka poʻe i loaʻa ka mōʻaukala ʻohana o EB a ke hoʻolālā nei e loaʻa nā keiki e ʻike paha he mea kōkua nui ka ʻōlelo aʻoaʻo genetic i ka hoʻoholo ʻana pehea e hoʻolālā ai i ko lākou ʻohana.
Eia kekahi, ʻaʻole ʻike ka poʻe loea i kēia manawa i ke kumu o ke ʻano o ka EB i loaʻa. No laila, ʻaʻole ʻike nā kauka pehea e pale aku ai.
He aha ke ʻano o ka wā e hiki mai ana e hiki i kekahi me EB ke manaʻo?
ʻO ka wā e hiki mai ana o ka poʻe me EB e pili ana i ke ʻano o ka EB i loaʻa iā lākou a me kona koʻikoʻi. Hiki i nā ʻano koʻikoʻi o ka maʻi ke hana i ka ʻeha nui, nā kīnā, nā kīnā, nā ʻeha e ho'ōla ʻole ai, a me ka make koke .
Eia nō naʻe, hiki i nā kauka ke kōkua iā ʻoe e hoʻokele i kāu mau hōʻailona. Hiki ke hana ʻia kēia me ka mālama kūpono a, inā pono, nā lāʻau ʻeha. ʻO ka mālama ʻana i ka ʻili i uhi pono ʻia me nā ʻaʻahu pale ʻaʻole e hoʻopili i kēlā me kēia lā ʻē aʻe, ka ʻauʻau pinepine ʻana, a me ka mālama ʻana i nā ʻeha e hiki ke kōkua i ka hōʻemi ʻana i ka hopena o EB i kou ola.
Inā loaʻa iā ʻoe ʻo EB, i ka manawa hea ʻoe e ʻike ai i ke kauka?
E ʻike pono i kāu kauka i kēia mau hihia:
- Inā pilikia ʻoe i ka hanu ʻana.
- Inā pilikia ʻoe i ka ale ʻana i ka meaʻai.
- Inā ʻike ʻia kou ʻeha he maʻi (e ʻulaʻula ana ka ʻili, poni, hina, a keʻokeʻo paha; pehu a pehu paha).
- Inā ʻike ʻia nā hōʻailona hou.
He aha nā nīnau koʻikoʻi e nīnau aku ai i ke kauka?
Ke hele ʻoe e ʻike i ke kauka, he mea maikaʻi ke nīnau i nā nīnau e like me kēia:
- Pehea e hiki ai iā ʻoe ke haʻi inā loaʻa iaʻu ka EB?
- Inā ʻaʻohe oʻu EB, he aha nā maʻi ʻili ʻē aʻe e loaʻa iaʻu?
- Pehea e hiki ai iaʻu ke mālama i kaʻu mau hōʻailona?
- He aha nā lāʻau lapaʻau āu e ʻōlelo nei? Loaʻa iā lākou nā hopena ʻaoʻao?
- He aha nā lāʻau lapaʻau home āu e ʻōlelo nei?
- He aha hou kaʻu e hana ai e hoʻomaikaʻi ai i kaʻu mau hōʻailona?
- Aia kekahi kirīmi a ʻaila paha e hiki iā ʻoe ke kuhikuhi?
- E ʻike anei au i ke kauka ʻili a i ʻole kekahi loea ʻē aʻe?
He aha ka ʻokoʻa ma waena o ka epidermolysis bullosa (EB) a me ka pemphigoid bullous?
He maʻi ʻili kakaʻikahi ka Bullous pemphigoid , he maʻi ʻili autoimmune. Hiki iā ia ke hana i nā ʻūlū ʻili, nā ʻūlū e like me ka hive a i ʻole nā pehu i piha i ka wai. Hoʻopilikia pinepine ka Bullous pemphigoid i nā poʻe ma luna o 60 mau makahiki , a nalo pinepine ia i loko o ʻelima mau makahiki. Eia nō naʻe, i nā hihia koʻikoʻi, hiki i nā pehu a me nā ʻōpala ke hōʻino i nā ʻōpū o loko a make.
ʻOiai hiki i ka EB ke lilo i maʻi autoimmune i kekahi manawa, ʻo ia ka mea maʻamau i hoʻokumu ʻia e kahi loli genetic e hoʻopilikia ai i ka collagen i loko o kou ʻili. ʻIke pinepine ʻia ka EB i ka wā kamaliʻi a i ʻole ka wā kamaliʻi . ʻOiai ʻaʻohe lāʻau lapaʻau no EB, pono paha ʻoe e noho me nā hōʻailona no ke koena o kou ola.
ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai (Leka Lawe-Home)
He kūlana paʻakikī ka Epidermolysis bullosa (EB) i kekahi manawa. Eia nō naʻe, me ke aʻo lapaʻau kūpono, ka mālama ʻana, a me ka mālama aloha, hiki iā ʻoe ke hoʻokele i nā hōʻailona a ola maikaʻi loa. E hoʻomanaʻo, ʻaʻole ʻoe hoʻokahi. Aia nā kauka, ka ʻohana, a me nā hui kākoʻo e kōkua iā ʻoe. No laila, mai makaʻu, e noho ikaika, a e kū i kēia kūlana.
Inā he mau nīnau hou aku kāu e pili ana iā EB, e ʻoluʻolu e kamaʻilio me ke kauka ʻili.
ʻEpidermolysis bullosa, EB, maʻi ʻili, pehu, maʻi hoʻoilina, maʻi ʻili o nā kamaliʻi, nāwaliwali o ka ʻili
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