Ke puka mai nei kou ʻili i nā pehu ʻoiai inā he liʻiliʻi wale nō? E kamaʻilio kākou e pili ana i ka Epidermolysis Bullosa (EB)

E noʻonoʻo i ka ʻili o kāu pēpē liʻiliʻi e pehu ana a ʻeha maʻalahi i nā lole a lākou e ʻaʻahu ai, ʻoiai inā e kahakaha iki ʻia lākou i kekahi wahi. He kūlana ʻeha a kaumaha loa kēia e lohe ai, ʻeā? Minamina, ke kū nei kekahi mau keiki a me nā pākeke i kēia ʻano kūlana. I kēia lā e kamaʻilio mākou e pili ana i kahi maʻi i kapa ʻia ʻo 'Epidermolysis Bullosa' (EB), ka mea e lilo ai kēlā ʻili i mea palupalu a koʻikoʻi. Kāhea pū kekahi poʻe i kēia mau keiki he "pēpē pepeke" no ka mea he palupalu ko lākou ʻili e like me ka ʻēheu o ka pulelehua.

ʻO ka maʻalahi, he aha ka Epidermolysis Bullosa (EB)?

ʻAʻole he maʻi hoʻokahi ka Epidermolysis Bullosa (EB). He maʻi hoʻoilina ia, ʻo ia hoʻi, ua hoʻoili ʻia i nā hanauna. He palupalu loa ka ʻili o ke kanaka me kēia maʻi. ʻOiai ke kaomi liʻiliʻi a i ʻole ka haʻalulu ʻaʻole e hōʻeha i ke kanaka maʻamau, e like me ka hamo ʻana i ka lole a i ʻole kekahi e hoʻopā ana i ko lākou ʻili, hiki i kekahi manawa ke hana i nā pehu a me nā ʻeha.

Ua hana ʻia ko kākou ʻili me kekahi mau papa nui. Hoʻopaʻa ʻia kēia mau papa e nā protein. I ka pololei, he mea nui loa kahi protein i kapa ʻia ʻo collagen no kēia. Ua like ia me ka 'gum' e pipili ana i nā papa o ka ʻili. Ma muli o kahi kīnā genetic i loko o ka poʻe me ka maʻi EB, ʻaʻole i hana pono ʻia kēia protein. No laila, no ka mea ua nāwaliwali kēlā 'gum', ua maʻalahi ka hoʻokaʻawale ʻana o nā papa o ka ʻili, e hoʻopiha ana iā lākou me ka wai a hana i nā pehu. Hiki i kēia mau pehu ke hana ʻaʻole wale ma nā wahi e like me nā lima, nā wāwae, nā kuʻekuʻe lima, a me nā kuli, akā i loko hoʻi o ka waha, ka ʻāʻī, a i loko o ke kino.

He aha nā ʻano nui o ka maʻi EB?

Ua māhele ʻia ʻo EB i ʻehā ʻano nui, ma muli o ka papa o ka ʻili kahi e hana ʻia ai nā pehu. He mea nui loa kēia no nā kauka e hoʻoholo ai i ka lāʻau lapaʻau.

He aha nā hōʻailona kūpono o ka maʻi EB?

Hiki ke ʻokoʻa nā hōʻailona ma muli o ke ʻano o ka maʻi EB. Hoʻomaka pinepine nā hōʻailona e ʻike ʻia i ka wā kamaliʻi.

• ʻO ka hōʻailona nui, ka pehu ʻana o ka ʻili, ʻoiai ma nā lima, nā wāwae, nā kuʻekuʻe lima, a me nā kuli.
• Ka mānoanoa o ka ʻili ma nā poho lima a me nā kapuwaʻi, me he mea lā e ʻomi ʻia ana.
• Ke lilo nei nā kui i ʻano ʻē, mānoanoa, a i ʻole ka ʻili ʻana.
• ʻO ke ʻano o nā puʻupuʻu keʻokeʻo liʻiliʻi e like me ka pehu (milia) ma ka ʻili.
• Ke paʻakikī i ka ale ʻana i ka meaʻai (dysphagia) ma muli o nā pehu ma ka waha a me ka puʻu.
• ʻO nā ʻeha pinepine e hiki ai ke kahe ke koko mai ke kino, e alakaʻi ai i ka anemia .
• I nā hihia koʻikoʻi, pili pū nā manamana lima.
• Ka nāwaliwali o ka enamel niho a me ka ulu ʻole o nā niho (hypoplasia).
• Ka ulu ʻana o nā keiki me ka ʻole o ka hoʻopulapula.

Pehea e ʻike ʻia ai kēia maʻi a mālama ʻia?

Inā loaʻa i kāu keiki kēia mau hōʻailona, ​​​​he mea nui e ʻike i ke kauka. E hana pinepine ke kauka i kekahi mau hoʻokolohua e ʻike ai i kēia maʻi.

• Biopsy ʻIli: Pili kēia i ka lawe ʻana i kahi ʻāpana ʻili liʻiliʻi loa a nānā iā ia ma lalo o kahi microscope. Hiki i kēia ke kōkua i ka hoʻoholo pololei ʻana i ka papa o ka ʻili i hoʻopilikia ʻia.
• Hoʻāʻo Genetic:Hiki i kēia ke kōkua i ka hoʻoholo pono ʻana i ka gene i hemahema a me ke ʻano o ka EB.

E kamaʻilio kākou e pili ana i ka lāʻau lapaʻau.

ʻO ka mea mua e ʻōlelo ai, ʻo ka mea pōʻino, ʻaʻohe lāʻau lapaʻau no ka EB i kēia manawa. Eia nō naʻe, hiki ke kāohi ʻia nā hōʻailona, ​​​​hiki ke pale ʻia nā pilikia, a hiki i ka mea maʻi ke ola ʻoluʻolu e like me ka hiki. ʻO nā pahuhopu nui o ka mālama ʻana:

• E ho'ēmi i ka pehu ʻana i ka hiki.
• E mālama pono ana i ka pale ʻana i nā pehu a me nā ʻeha mai ka lilo ʻana i maʻi.
• Ka mālama ʻana i ka ʻeha.
• Ke pale ʻana i nā pilikia meaʻai.

Eia kekahi o nā ʻōlelo aʻoaʻo i hāʻawi ʻia e nā kauka no kēia.

• E ʻaʻahu i nā lole palupalu a palupalu : Pono ʻoe e ʻaʻahu i nā lole i hana ʻia mai nā olonā kūlohelohe e like me ka pulupulu, ʻaʻole paʻa, a ʻaʻole hoʻi e hamo i kou kino.
• E mālama i kou ʻili i ka anu: Hiki i ka wela a me ka hou ke hoʻonui i nā pehu, no laila he mea nui e mālama i ka lumi i kahi mahana ʻoluʻolu.
• Ke hoʻohana ʻana i nā kāʻei kūikawā: Pono e hoʻohana ʻia nā kāʻei kūikawā a me ka gauze i ʻole e pipili i ka ʻili no nā ʻeha.
• Ka mālama ʻeha i kēlā me kēia lā: E kau i nā mea poni i kuhikuhi ʻia e ke kauka a mālama i nā ʻeha i maʻemaʻe.
• ʻAi: Inā he ʻeha waha kou, pono ʻoe e pale i nā meaʻai wela, ʻala, a paʻakikī a ʻai i nā meaʻai palupalu e like me nā sopa, nā meaʻai i kuʻi ʻia, a me nā puddings.
• ʻO ke ʻoki kino: I kekahi mau hihia koʻikoʻi, pono paha ke ʻoki kino e hoʻonui i ka esophagus inā ua ʻūmiʻi ʻia a i ʻole e hoʻokaʻawale i nā manamana lima inā pili pū lākou.

Nā ʻōlelo aʻoaʻo no nā mākua e mālama ana i kahi keiki me EB

He pilikia nui ka mālama ʻana i kahi keiki e like me kēia no nā mākua. Hiki ke luhi i ke kino a me ka noʻonoʻo. Akā, ʻo kou aloha a me kou mālama ʻana he ikaika nui ia no ke keiki.

• E holoi mau i kou mau lima ma mua o ka hoʻopā ʻana i ka ʻili o kāu pēpē.
• I ka hāpai ʻana i ka pēpē, mai hopu iā ia ma lalo o nā ʻāʻī . Hiki i ka hana ʻana pēlā ke hōʻino i ka ʻili. E kau i kou mau lima ma lalo o ke kua a me nā ʻūhā o ke pēpē a hāpai mālie.
• Inā ʻoe e hoʻohana i nā diapers, e hoʻohana i nā mea me Velcro ma kahi o ka lipine hoʻopili. Hiki iā ʻoe ke hōʻemi i ka friction ma ka ʻoki ʻana i nā kaula elastic a puni nā wāwae.
• I ka ʻauʻau ʻana i kāu pēpē, ʻoi aku ka maikaʻi o ka hoʻomaʻemaʻe mālie ʻana i kēlā me kēia ʻāpana o ke kino ma mua o ka hoʻoluʻu ʻana iā ia i loko o kahi kīʻaha wai.
• Mai kaupalena i nā hana a kāu keiki. ʻOiai ʻaʻole kūpono ka pāʻani ʻino, hiki i ka hoʻoikaika kino mālie e like me ka ʻauʻau ke kōkua i ka hoʻoikaika ʻana i nā ʻiʻo o kāu keiki.
• Inā ʻoe kekahi e luhi nei i ka noʻonoʻo, mai kānalua e ʻimi i ke kākoʻo mai kāu kauka, ʻohana, a i ʻole nā ​​mākua i loaʻa nā ʻike like.

Āhea ʻoe e ʻike koke ai i ke kauka?

I ka mālama ʻana iā EB, he mea nui i kekahi manawa ke ʻimi koke i ke kōkua lapaʻau. E makaʻala i nā hōʻailona ma lalo nei.

• Inā pilikia ʻoe i ka hanu ʻana. (I kēlā mau hihia , e hele koke i ka ʻOihana Pōpilikia (ETU) o ka haukapila kokoke loa. )
• Inā ʻaʻole hiki iā ʻoe ke ale i ka meaʻai a i ʻole nā ​​​​wai.
• Inā ʻike ʻia ka ʻeha ua maʻi ʻia (ʻo ia hoʻi, ua ʻulaʻula ka ʻāpana a puni ka ʻeha, pehu, wela, a i ʻole he pehu e puka mai ana).
• Inā loaʻa ke kuni i ke keiki.
• Inā ʻike ʻia kahi hōʻailona hou hiki ʻole iā ʻoe ke hoʻomaopopo.

ʻOiai hiki ke paʻakikī ke noho me kēia ʻano maʻi, me ke aʻo lapaʻau kūpono a me ka mālama ʻana, hiki iā ʻoe ke kāohi i ka ʻeha a ola i kahi ola maikaʻi. ʻO ka mea nui loa, ʻo ia ke hana pū me kāu kauka a ʻaʻole e hoʻāʻo e lawelawe wale i kēia mau mea.