Ua ʻike anei ʻoe i kēia maʻi hoʻoilina e hoʻopilikia ai i nā māmā a me ke akepaʻa? (Alpha-1 Antitrypsin Deficiency)

Loaʻa iā ʻoe kekahi ʻū a me ka paʻakikī o ka hanu ʻana i hala no ka manawa lōʻihi? Manaʻo paha ʻoe he mea maʻamau kēia. Akā i kekahi manawa, aia paha kekahi kumu koʻikoʻi ma hope o kēia mau hōʻailona i lohe nui ʻole ʻia e mākou. I kēia lā e kamaʻilio mākou e pili ana i ia ʻano. ʻO ia ka Alpha-1 Antitrypsin Deficiency, a i ʻole (Alpha-1 Antitrypsin Deficiency). Kapa pū kekahi poʻe iā ia ʻo "Alpha-1" no ka pōkole.

I ka ʻōlelo maʻalahi, he aha ka Alpha-1?

He maʻi hoʻoilina hoʻoilina ʻo Alpha-1 i loaʻa iā kākou mai ko kākou mau mākua mai. ʻO ka mea e hana ʻia ana ma kēia, ʻaʻole hiki i ko kākou kino ke hana i ka lawa o ka protein i kapa ʻia ʻo alpha-one antitrypsin (AAT), he protein ia e pale ana i ko kākou mau māmā. E noʻonoʻo i kēia protein AAT ma ke ʻano he kiaʻi no ko kākou mau māmā. Ke nalowale kēia kiaʻi, ʻoi aku ka nui o ka hōʻino ʻia o ko kākou mau māmā.

No laila, ʻoi aku ka nui o ka pilikia o kekahi kanaka me ke kūlana Alpha-1 i ka loaʻa ʻana o nā maʻi māmā, ʻoiai ka emphysema (ka hōʻino ʻana i nā ʻeke ea i loko o nā māmā), cirrhosis (ka ʻeha o ke ake), a me ka panniculitis, kahi maʻi ʻili kakaʻikahi. I kekahi manawa, hiki i kēia mau kūlana ke hoʻoweliweli i ke ola.

No kēia kumu, kapa pū kekahi poʻe i kēia maʻi ʻo "genetic COPD".

Pehea e hiki mai ai kēia maʻi? ʻO wai ka mea i pilikia nui?

No ka hoʻomohala ʻana iā Alpha-1, pono mākou e hoʻoilina i ka gene ʻano ʻē mai ko mākou mau mākua ʻelua. I ka ʻōlelo maʻalahi, ʻo nā genes nā kuhikuhi e hoʻoholo ai i ke ʻano o ka hana ʻana o ko mākou kino. Inā he loli iki i kēia mau kuhikuhi, hiki ke loli ka hana a ke kino.

Ma Alpha-1, ʻo kahi mutation i loko o ka gene i kapa ʻia ʻo SERPINA1 ke kumu o nā pilikia i ka hana ʻana o ka protein AAT. ʻO ka hopena, ʻaʻole i hana ʻia ka protein AAT i nā nui lawa, a i ʻole ke ʻano kūpono ʻole o ka protein i hana ʻia. Ke lawe ʻia ke ʻano kūpono ʻole, ʻaʻole hiki i kēlā protein ke haʻalele i ke akepaʻa a hele ma ke koko i nā māmā. A laila paʻa kēlā protein ʻano ʻē i loko o ke akepaʻa, e hōʻino ana i ke akepaʻa. Eia kekahi, ʻoiai ʻaʻohe protein e hele ai i nā māmā, he palupalu hoʻi nā māmā.

Eia nō naʻe, hoʻoilina kekahi poʻe i kēia gene kīnā mai hoʻokahi makua wale nō (ʻo ko lākou makuahine a i ʻole ko lākou makuakāne). Kāhea mākou iā lākou he "mea lawe." ʻOiai ʻo ko lākou kino e hana maʻamau i ka protein AAT lawa e pale ai i ko lākou mau māmā, aia nō lākou i ka pilikia o ka hōʻino ʻia o ka māmā. He kiʻekiʻe loa kēia pilikia inā puhipaka lākou.

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Pehea ka hopena o kēia i nā māmā a me ke akepaʻa?

I mea e maopopo ai i kēia, e lawe kākou i kahi laʻana liʻiliʻi.

Hana ʻia kā mākou protein AAT i loko o ke akepaʻa. A laila hele ia ma ke koko a hiki i nā māmā. Loaʻa i ko mākou māmā kahi enzyme i kapa ʻia ʻo Neutrophil Elastase e ​​kōkua iā lākou e hakakā i nā maʻi. E noʻonoʻo i kēia enzyme he koa wiwo ʻole a akamai. Hoʻopau ia i nā maʻi. Akā i ka wā e pau ai, pono kekahi e hoʻōki iā ia. A i ʻole, hoʻomaka ia e hoʻouka i ko mākou mau hunaola māmā olakino.

ʻO kēlā mea "hoʻōki", kēlā hana "hoʻopau", hana ʻia e kā mākou protein AAT. Ua like ia me ke kauoha e ʻōlelo ana i ke koa, "ʻAe, ua lawa kēlā, e kū."

I kēia manawa, i loko o ke kanaka me Alpha-1, ua nele kēia luna AAT i ke kino. A laila, ʻaʻohe mea nāna e hoʻōki i kēlā koa wiwo ʻole (Neutrophil Elastase). Hoʻomau ia i ka hoʻouka ʻana i nā māmā. ʻO kēia ke kumu o ka luku ʻia ʻana o ka protein Elastin i loko o nā māmā. ʻO kēia elastin ka mea e hāʻawi ai i nā ʻeke ea (alveoli) i loko o nā māmā i ko lākou elasticity a me ka ikaika e like me ke kaula laholio. Ke nalowale, lilo nā ʻeke ea i nāwaliwali a palupalu e like me ka balloon i hoʻohaʻahaʻa ʻia. ʻO kēia ka mea a mākou e kapa nei he Emphysema . ʻO kēia ka mea e paʻakikī ai ka hanu ʻana a loaʻa ka oxygen.

He ʻokoʻa iki ka mea e hana ʻia ana i ke akepaʻa. Ma muli o kekahi mau kīnā hoʻoilina, ua hana ʻia ka protein AAT i ke ʻano kūpono ʻole. E like me kahi ʻāpana pepa i pelu pono ʻole ʻia. Ma muli o kēia ʻano kūpono ʻole, ʻaʻole hiki i ka protein ke haʻalele i ke akepaʻa a paʻa i loko o ke akepaʻa. Ke paʻa ka nui o ka protein e like me kēia, ua hōʻino ʻia ke akepaʻa a hoʻomaka i ka ʻōpala. Ua kapa ʻia kēlā he cirrhosis .

He aha nā hōʻailona o ka maʻi Alpha-1?

ʻOkoʻa nā hōʻailona i hoʻokumu ʻia e Alpha-1 ma muli o ke kino i hoʻopilikia ʻia. Ua like pinepine kēia me nā hōʻailona o COPD (Chronic Obstructive Pulmonary Disease).

Pehea e ʻike ai i kēia maʻi?

ʻO ke ala nui e ʻike ai iā Alpha-1 me ka hoʻāʻo koko . No ka mea ua like nā hōʻailona me nā maʻi ʻē aʻe, hiki i kekahi manawa ke lōʻihi ka manawa e loaʻa ai kahi hōʻailona pololei. Inā loaʻa iā ʻoe nā hōʻailona o ke akepaʻa, a inā ua ʻike ʻia ʻoe me COPD a ʻaʻole e hoʻomaikaʻi ana kāu mau hōʻailona me ka mālama ʻana, hiki i kāu kauka ke hoʻoholo e hoʻāʻo iā ʻoe no Alpha-1.

ʻO nā hoʻokolohua i hana pinepine ʻia:

• Nā hoʻāʻo koko: E hoʻāʻo ʻia kou koko no ka pae o ka protein AAT. Inā haʻahaʻa ka pae, e hana ʻia ka hoʻāʻo genetic e ʻike inā he kīnā genetic kou e pili ana iā Alpha-1.
• Nā hoʻokolohua kiʻi: Hiki i kahi X-ray umauma a i ʻole CT scan ke hoʻoholo i ka nui a me ka wahi o ka hōʻino ʻana o ka māmā.
• Nā hoʻāʻo hana pulmonary: ʻOiai ʻaʻole hiki i kēia mau mea ke ʻike pololei iā Alpha-1, hiki iā lākou ke hāʻawi i kāu kauka i kahi manaʻo no ka maikaʻi o ka hana ʻana o kou mau māmā.
• ʻO ke Ultrasound o ke akepaʻa a i ʻole FibroScan®: Inā kānalua ʻia ka hōʻino ʻia ʻana o ke akepaʻa, hana ʻia kēia mau scan e nānā no ka ʻeha ma ke akepaʻa.
• Biopsy o ke akepaʻa: Inā koʻikoʻi ka hōʻino ʻana o ke akepaʻa, e lawe ʻia kahi ʻāpana liʻiliʻi o ka ʻiʻo mai ke akepaʻa a nānā ʻia e hoʻoholo ai i ka nui o ka hōʻino.

He aha nā lāʻau lapaʻau no Alpha-1?

ʻOiai ʻaʻohe lāʻau lapaʻau kūikawā no ka maʻi Alpha-1, nui nā lāʻau lapaʻau e hiki ke kaohi i nā hōʻailona, ​​​​hōʻemi i ka hōʻino ʻana o nā kino, a hoʻomaikaʻi i ke ʻano o ke ola.

ʻO ka mea nui loa, ʻo ia ka ʻike mua ʻana i ka maʻi, e hana i nā hoʻololi e pono ai ke ʻano o ka nohona, a e hahai i nā ʻōlelo aʻoaʻo a ke kauka.

ʻOkoʻa nā ʻano lapaʻau ma muli o ke kino i hoʻopilikia ʻia:

Lapaʻau no nā māmā

• Hoʻōla Hoʻonui: He lāʻau lapaʻau kūikawā kēia no Alpha-1. Pili ia i ka hoʻomaʻemaʻe ʻana i ka protein AAT i lawe ʻia mai nā mea hāʻawi koko olakino a hāʻawi iā ia i ke kino ma o kahi aʻa koko, e like me ka saline. ʻOiai ʻaʻole hiki i kēia ke hoʻohuli i ka pōʻino i hana mua ʻia, hiki iā ia ke hoʻōki a kāohi paha i ka pōʻino i ka wā e hiki mai ana i nā māmā.
• Lāʻau lapaʻau: Hāʻawi ʻia nā lāʻau lapaʻau e like me nā inhalers , e like me nā bronchodilators, i nā maʻi me COPD e maʻalahi ai ka hanu ʻana.
• ʻO ka lāʻau lapaʻau oxygen: Inā haʻahaʻa ka pae oxygen i loko o ke koko, hāʻawi ʻia ka oxygen hoʻohui ma o kahi ʻōmole liʻiliʻi i kau ʻia i loko o ka ihu a i ʻole kahi uhi waha.
• Nā papahana hoʻoponopono hou ʻana o ka puʻuwai: Hāʻawi ʻia ke aʻo ʻana e maʻalahi ai ka hanu ʻana ma o nā hoʻomaʻamaʻa hanu a me nā hoʻomaʻamaʻa kino ʻē aʻe.
• Hoʻoili māmā: Inā ua hōʻino nui ʻia nā māmā, pono paha kahi hoʻoili māmā olakino ma ke ʻano he koho hope loa.

Lapaʻau no ke akepaʻa

Hoʻōla ʻia ka hōʻino ʻana o ke akepaʻa ma o nā hōʻailona. Eia nō naʻe, ʻo ke ala wale nō e ho'ōla piha ai i ka maʻi akepaʻa Alpha-1 he hoʻoili akepaʻa . Ke hoʻoili ʻia kahi akepaʻa olakino, hoʻomaka ke akepaʻa e hana i ka protein 'AAT' kūpono.

He aha kaʻu e hana ai e ola olakino me Alpha-1?

ʻOiai inā ua ʻike ʻia ʻoe me Alpha-1, ʻaʻole ia ka hopena o kou ola. Nui nā mea hiki iā ʻoe ke hana e hōʻemi i ka hōʻino ʻia i kou mau kino.

ʻO ka mea nui loa a me ka mea mua loa, ʻo ia ka pale loa ʻana i ka puhipaka. ʻO ka puhipaka no kekahi me Alpha-1 ua like ia me ka hoʻohui ʻana i ka wahie i ke ahi. Hoʻonui nui ia i ka nui o ka hōʻino ʻana o ka māmā.

Ma waho aʻe o kēlā, e makaʻala hoʻi i kēia mau mea:

• E noho mamao aku mai nā wahi e puhipaka ai ka poʻe. (E pale i ka uahi lua)
• Mai hanu i nā mea e hōʻino ai i kou mau māmā, e like me ka lepo a me nā kemika. Inā pili ʻoe i ia mau mea ma ka hana, e ʻaʻahu i kahi pale maka pale.
• E hoʻōki loa i ka inu ʻana i ka waiʻona a i ʻole e kaupalena iā ia i ka nui e like me ka hiki. Hiki i ka waiʻona ke hoʻonui i ka hōʻino ʻana o ke ake.
• E hōʻalo i ka hoʻohana ʻana i nā lāʻau ʻeha (e like me ka lawe ʻana i ka paracetamol nui loa) a i ʻole nā ​​​​​​mea kanu ʻē aʻe e hiki ke hoʻopilikia i ke akepaʻa me ka ʻole o ke kūkākūkā ʻana me kāu kauka.
• E kiʻi i nā lāʻau pale āpau e pono ai. He mea nui loa ka loaʻa ʻana o ka lāʻau pale no nā maʻi e like me ka niumonia, ka maʻi palū (flu), COVID-19, a me ka hepatitis A a me B, nā mea e hoʻopilikia ai i ke akepaʻa.
• E holoi pinepine i kou mau lima, e noho maʻemaʻe, a e pale iā ʻoe iho mai nā maʻi.
• Inā loaʻa i kekahi o kou ʻohana ʻo Alpha-1, he manaʻo maikaʻi e hoʻāʻo ʻoe iho. E kamaʻilio me kāu kauka e pili ana i ia mea.
• Inā loaʻa iā ʻoe ʻo Alpha-1 a i ʻole he mea lawe ʻoe, he mea nui loa ia e kamaʻilio me kahi kākāʻōlelo genetic inā ʻoe e hoʻolālā ana e loaʻa nā keiki.

Āhea ʻoe e ʻike ai i ke kauka?

• Inā loaʻa iā ʻoe nā hōʻailona o ka māmā a i ʻole ke akepaʻa a mākou i kamaʻilio ai ma mua.
• Inā ua ʻike ʻia kekahi o kou ʻohana me Alpha-1.
• Inā ua ʻike ʻia a ua mālama ʻia ʻoe no ka COPD a i ʻole ka hānō, akā ʻaʻole e hoʻomaikaʻi ana kāu mau hōʻailona, ​​​​e kamaʻilio me kāu kauka e pili ana i ka loaʻa ʻana o kahi hoʻāʻo Alpha-1.
• Inā loaʻa iā ʻoe ʻo Alpha-1, e ʻike koke i kāu kauka inā ʻike ʻia nā hōʻailona hou a i ʻole e ʻoi aʻe ka ʻino o nā hōʻailona e kū nei.

Hiki ke paʻakikī ke noho me Alpha-1. Akā me ka ʻike kūpono, ka lāʻau lapaʻau, a me ke ʻano o ka nohona, hiki iā ʻoe ke ola i kahi ola olakino a hana. ʻO ke ala maikaʻi loa e hana ai i kēlā, ʻo ia ke kamaʻilio ākea me kāu kauka a hana i kahi hoʻolālā e kūpono iā ʻoe.