Epidermolysis bullosa

ʻO ka Epidermolysis bullosa (EB) kahi hui o nā maʻi kahi e palahu ai ka ʻili ma hope o kahi ʻeha liʻiliʻi. Ua hoʻoili ʻia i loko o nā ʻohana.

ʻEhā ʻano nui o EB. O lākou:

• Dystrophic epidermolysis bullosa
• Epidermolysis bullosa simplex
• Hemidesmosomal epidermolysis bullosa
• ʻO Jointal epidermolysis bullosa

ʻO kekahi ʻano ʻano ʻē aʻe o EB i kapa ʻia ʻo epidermolysis bullosa acquisita. Hoʻolālā kēia palapala ma hope o ka hānau ʻana. He maʻi autoimmune ia, ʻo ia hoʻi ke hoʻouka nei ke kino iā ia iho.

Hiki ke hoʻololi i ka EB mai ka mea liʻiliʻi a i ka make. ʻO ke kumu liʻiliʻi e hoʻonā i ka ʻili o ka ʻili. Hoʻopili ke ʻano make i nā lālā ʻē aʻe. Hoʻomaka ka hapa nui o kēia ʻano i ka wā hānau a i ʻole ma hope koke paha. He paʻakikī paha ke hoʻomaopopo i ke ʻano kikoʻī o ka EB a ke kanaka, ʻoiai ke loaʻa nei nā māka kikoʻī kikoʻī no ka hapanui.

ʻO ka mōʻaukala ʻohana kahi pilikia. ʻOi aku ka kiʻekiʻe o ka makaʻi inā loaʻa kēia ʻano i ka makua.

Kaukaʻi ʻia i ke ʻano o EB, hiki i nā ʻōuli ke hoʻopili pū:

• Alopecia (lauoho lauoho)
• Pā a puni ka maka a me ka ihu
• Pāhū i loko a puni paha ka waha a me ka puʻu, e hoʻoulu ana i nā pilikia i ka hānai ʻana a i ʻole ka ale ʻana i ka paʻakikī
• Pāhili ma ka ʻili ma muli o ka hōʻeha iki a i ʻole ka loli o ka mahana, ʻo ka wāwae hoʻi
• Pākuʻi ʻia i ka wā hānau
• Nā pilikia kahua niho e like me ka pala o ka niho
• Uē nui, uā, a i ʻole nā ​​pilikia hanu ʻē aʻe
• ʻO nā puʻupuʻu keʻokeʻo liʻiliʻi ma ka ʻili i hōʻeha ʻia ma mua
• Nail nalo a i ʻole nā ​​kui kuʻi

E nānā kāu kauka olakino i kou ʻili e hōʻoia iā EB.

Hoʻohana ʻia nā hoʻokolohua e hōʻoia i ke kuhi.

• Ke hoʻāʻo genetic
• ʻ biiwi lāʻau
• Nā hoʻāʻo kūikawā o nā laʻana o ka ʻili ma lalo o kahi microscope

Hiki ke hoʻohana ʻia nā hoʻokolohua ʻili e ʻike ai i ke ʻano o EB.

Nā hoʻokolohua ʻē aʻe i hana ʻia e hoʻopili ʻia:

• Hōʻike koko no ka anemia
• ʻO ka moʻomeheu e nānā ai i ka maʻi maʻi inā hoʻōla maikaʻi ʻole nā ​​ʻeha
• ʻO ka endoscopy luna a i ʻole kahi papa GI kiʻekiʻe inā pili nā ʻōuli i nā pilikia ale

E nānā pinepine ʻia ka helu ulu no kahi pēpē i loaʻa a loaʻa paha iā EB.

ʻO ka pahuhopu o ka mālama ʻana e pale i nā palapū mai ka hana ʻana a hōʻalo i nā pilikia. E pili ka mālama ʻana i ka maikaʻi ʻole o kēia ʻano.

MALAMA HOME

E hāhai i kēia mau kulekele ma ka home:

• Mālama maikaʻi i kou ʻili e pale ai i nā maʻi.
• E hāhai i ka ʻōlelo aʻoaʻo a kāu mea hāʻawi inā ua wīwī a maka maka paha nā wahi i hoʻopili ʻia. Pono paha ʻoe i ka whirlpool therapy maʻamau a hoʻopili i nā hamo antibiotic i nā wahi ʻeha. E haʻi aku kāu mea hāʻawi iā ʻoe inā ʻoe e makemake i kahi kapa a i ʻole ʻaʻahu, a inā pēlā, he aha ke ʻano e hoʻohana ai.
• Pono paha ʻoe e hoʻohana i nā lāʻau lāʻau steroid oral no nā wā pōkole inā loaʻa iā ʻoe nā pilikia ale. Pono paha ʻoe e ʻai i ka lāʻau inā loaʻa ʻoe i ka maʻi candida (hū) i ka waha a i ʻole ka ʻāʻī.
• Mālama maikaʻi i kou olakino waha a kiʻi i nā loiloi niho maʻamau. ʻOi aku ka maikaʻi e ʻike i kahi kauka niho i ʻike i ka mālama ʻana i ka poʻe me EB.
• ʻAi i kahi papaʻai olakino. Ke loaʻa nui ka ʻeha o ka ʻili iā ʻoe, pono ʻoe i nā calorie hou a me nā protein e kōkua i kou ʻili e hoʻōla. Koho i nā meaʻai palupalu a pale i nā nati, nā ʻāpana, a me nā meaʻai crunchy ʻē aʻe inā he ʻeha kou i kou waha. Hiki i ke nutristist ke kōkua iā ʻoe me kāu papaʻai.
• Hana i kahi hoʻomaʻamaʻa kino e hōʻike iā ʻoe e kōkua i ka mālama ʻana i nā hono a me nā mākala.

NA MEA HANA

ʻO ke ʻoki lāʻau e mālama ai i kēia ʻano e komo pū paha:

• ʻO ka hoʻopili ʻana i ka ʻili ma nā wahi hohonu kahi ʻeha
• Hoʻonui (pālahalaha) o ka esophagus inā he heiki
• Hoʻoponopono i nā hemahema lima
• ʻO ka hemo ʻana o kahi carcinoma o ka cell squamous (kahi ʻano maʻi ʻaʻai ʻili) ulu

NA LAAU HOU

Hiki i nā ʻano lāʻau ʻē aʻe no kēia ʻano ke:

• ʻO nā lāʻau lapaʻau e kaomi i ka ʻōnaehana paleʻea e hoʻohana ʻia no ke ʻano autoimmune o kēia ʻano.
• Ke aʻo ʻia nei ka protein a me ka genotherapy a me ka hoʻohana ʻana o ka interferon lāʻau.

Aia ka nānā i ka paʻakikī o ka maʻi.

ʻO ka maʻi maʻi o nā wahi blamed ka mea maʻamau.

Hoʻonui ka maikaʻi o nā ʻano EB me ka makahiki. Loaʻa i nā ʻano koʻikoʻi o EB kahi helu kiʻekiʻe o ka make.

I nā ʻano koʻikoʻi, hoʻopili ʻia ka scarring ma hope o nā ʻano palapū:

• Nā deformities o ka ʻaelike (e laʻa me, ma nā manamana lima, kuʻekuʻe lima, a me nā kuli) a me nā hemahema ʻē aʻe
• ʻO ka ale ʻana i ka pilikia inā pili ka waha a me ka esophagus
• Nā manamana lima a me nā manamana wāwae
• Ka hoʻoneʻe palena ʻia mai ka scarring

Hiki ke hana i kēia mau pilikia:

• Anemia
• Hoemi i ke ola no nā ʻano koʻikoʻi o ke ʻano
• ʻOwingmiʻi ʻo Esophageal
• Nā pilikia o ka maka, me ka makapō hoʻi
• Maʻiʻaʻai, me sepsis (maʻiʻaʻai i loko o ke koko a me nāʻaʻaʻa)
• Loaʻa ka hana ma nā lima a me nā wāwae
• Hoʻoiho muscular
• Maʻi maʻi Periodontal
• Hoʻoikaika kaʻai pono ʻole ma o ka hānai ʻana i ka paʻakikī, e alakaʻi ana i ka holomua ʻole
• Ka maʻi ʻaʻai ʻāpau squamous

Inā loaʻa ka maʻi o kāu pēpē ma hope koke iho o ka hānau ʻana, kāhea i kāu mea lawelawe. Inā loaʻa iā ʻoe ka moʻolelo ʻohana o EB a hoʻolālā e loaʻa nā keiki, makemake paha ʻoe e loaʻa i ka ʻōlelo aʻoaʻo kūlohelohe.

Paipai ʻia ka ʻōlelo aʻo genetic no nā mākua e hiki mai ana i loaʻa ka moʻolelo ʻohana o nā ʻano like ʻole o ka epidermolysis bullosa.

I ka wā hāpai, hiki ke hoʻohana ʻia kahi hoʻokolohua i kapa ʻia ʻo chorionic villus sampling e hoʻāʻo i ka pēpē. No nā kāne a me ka makaʻu nui i ka loaʻa ʻana o kahi keiki me EB, hiki ke hana i ka hoʻokolohua ma mua o ka pule 8 a 10 o ka hāpai keiki. E kamaʻilio i kāu mea hāʻawi.

I mea e pale aku ai i ka ʻili a me ka ʻeha ʻana, e hoʻokomo i nā pale a puni nā wahi ʻeha e like me nā kuʻe lima, nā kuli, nā kuʻekuʻe wāwae, a me nā puʻupuʻu. Hōʻalo i nā haʻuki pili.

Inā loaʻa iā ʻoe ka EB loaʻa a ma nā steroid i ʻoi aku ma mua o 1 mahina, pono paha ʻoe i nā mea hoʻohui kalima a me ka wikamina D. Hiki i kēia mau mea kōkua ke pale aku i ka osteoporosis (nā iwi lahilahi).

EB; Jointal epidermolysis bullosa; Dystrophic epidermolysis bullosa; Hemidesmosomal epidermolysis bullosa; ʻO Weber-Cockayne maʻi maʻi; Epidermolysis bullosa simplex

• Epidermolysis bullosa, ʻoi loa dystrophic
• Epidermolysis bullosa, dystrophic

ʻO Denyer J, Pillay E, Clapham J. Nā Kūlana Kūlana ʻOi Loa no ka Mālama ʻili a me ka ʻeha ma Epidermolysis Bullosa: kahi ʻae kuikahi. Ladana, UK: Wounds International; 2017.

Fine, J-D, Mellerio JE. I: Bolognia JL, Schaffer JV, Cerroni L, eds. Dermatology. ʻEhā ed. Piladelapia, PA: Elsevier Saunders; 2018: chap 32.

ʻO Habif TP. Nā maʻi Vesicular a me bullous. I: Habif TP, hoʻoponopono. Dermatology Lapaʻau. ʻElua huaʻōlelo Piladelapia, PA: Elsevier; 2016: chap 16.