He aha ka Alpha-1-Proteinase Inhibitor: Nā hoʻohana, ka nui, nā hopena ʻaoʻao a me nā mea hou aku

ʻO ka Alpha-1-proteinase inhibitor he lāʻau lapaʻau pani protein i hāʻawi ʻia ma o ka IV e kōkua i ka poʻe me kahi maʻi genetic laha ʻole i kapa ʻia ʻo alpha-1 antitrypsin deficiency. Hana kēia lāʻau lapaʻau ma ka pani ʻana i kahi protein nalowale a hewa paha e pale maʻamau i kou mau māmā mai ka pōʻino.

Inā ua ʻike ʻia ʻoe a i ʻole kekahi āu e mālama nei me ka alpha-1 antitrypsin deficiency, hiki iā ʻoe ke manaʻo nui ʻia e nā ʻike lapaʻau a pau. E hele kākou i loko o ka mea e pili ana i kēia lapaʻau a pehea e hiki ai ke kōkua i ka pale ʻana i ke olakino o kou māmā i ka manawa.

He aha ka Alpha-1-Proteinase Inhibitor?

ʻO ka Alpha-1-proteinase inhibitor he lāʻau lapaʻau protein i hoʻomaʻemaʻe ʻia i hana ʻia mai ke koko kanaka i hāʻawi ʻia. Loaʻa iā ia ka protein pale like i hana maoli ʻia e nā kānaka olakino i ko lākou ate, i kapa ʻia ʻo alpha-1 antitrypsin.

ʻO ka poʻe me ka alpha-1 antitrypsin deficiency ʻaʻole lākou e hana i ka nui o kēia protein a i ʻole e hana i kahi mana hewa ʻole e hana pono ʻole. Me ka lawa ʻole o ka protein hana, hiki i nā enzymes i loko o kou mau māmā ke hōʻino i nā ʻeke ea palupalu i ka manawa, e alakaʻi ana i nā pilikia hanu.

Hana kēia lāʻau lapaʻau e like me ka pale no kou mau māmā. Ke hāʻawi ʻia i kēlā me kēia manawa ma o ka IV, kōkua ia i ka hoʻihoʻi ʻana i nā pae protein pale i loko o kou kahe koko a me nā māmā, e hoʻolohi ana i ka holomua o ka pōʻino o ka māmā.

No ke aha i hoʻohana ʻia ai ka Alpha-1-Proteinase Inhibitor?

Ua hoʻolālā ʻia kēia lāʻau lapaʻau e mālama i ka alpha-1 antitrypsin deficiency, kahi maʻi genetic e pili ana i ka 1 i ka 2,500 poʻe. Hoʻohana ʻia ia i ka wā e hōʻike ai nā hoʻokolohua koko he haʻahaʻa loa nā pae o ka protein alpha-1 antitrypsin.

ʻO ka pahuhopu nui ka hoʻolohi ʻana i ka pōʻino o ka māmā a mālama i kāu hana hanu. ʻOiai ʻaʻole hiki iā ia ke hoʻohuli i ka pōʻino i hana mua ʻia, hiki iā ia ke kōkua i ka pale ʻana a i ʻole ka hoʻopaneʻe ʻana i ka hōʻino hou ʻana o kou ʻiʻo māmā.

E ʻōlelo maʻamau kāu kauka i kēia lapaʻau inā loaʻa iā ʻoe ka hemahema genetic a me nā hōʻailona o ka maʻi māmā, e like me ka emphysema a i ʻole ka maʻi pulmonary obstructive maʻi (COPD). ʻAʻole hoʻohana ʻia no nā ʻano pilikia māmā ʻē aʻe ʻaʻole pili i ka hemahema alpha-1 antitrypsin.

Pehea e hana ai ka Alpha-1-Proteinase Inhibitor?

Hana kēia lāʻau lapaʻau ma ka pani ʻana i ka protein pale i nalowale i kou kino. E noʻonoʻo i ka alpha-1 antitrypsin ma ke ʻano he kiaʻi no kou māmā - mālama maʻamau ia i nā enzymes ʻino mai ka hoʻouka ʻana i ka ʻiʻo māmā olakino.

Ke loaʻa iā ʻoe ka IV infusion, hele ka lāʻau lapaʻau ma kou kahe koko a hiki i kou māmā. Ma laila, hoʻopaʻa ia a hoʻopau i nā enzymes i kapa ʻia ʻo neutrophil elastase, hiki ke wāwahi i nā paia o kāu ʻeke ea inā waiho ʻole ʻia.

Ua manaʻo ʻia he lapaʻau ikaika waena e koi ana i ka hoʻohana mau. ʻAʻole hoʻōla ka lāʻau lapaʻau i ke kūlana genetic ma lalo, akā hāʻawi ia i ka pale paʻa ke hāʻawi ʻia i kēlā me kēia manawa. Pono ka hapa nui o nā kānaka i nā infusions i kēlā me kēia pule e mālama i nā pae protein pale i kā lākou ʻōnaehana.

Pehea wau e lawe ai i ka Alpha-1-Proteinase Inhibitor?

Hāʻawi mau ʻia kēia lāʻau lapaʻau ma ke ʻano he intravenous (IV) infusion i kahi hoʻonohonoho mālama ola. E loaʻa iā ʻoe i hoʻokahi manawa i ka pule, me kēlā me kēia infusion e lawe ana ma kahi o 15 a 30 mau minuke e hoʻopau ai.

Ma mua o kāu infusion, ʻaʻole pono ʻoe e pale i ka ʻai ʻana a i ʻole ka inu ʻana. ʻO ka ʻoiaʻiʻo, ʻo ka loaʻa ʻana o ka ʻai māmā ma mua hiki ke kōkua iā ʻoe e ʻoluʻolu i ka wā o ka lapaʻau. E hōʻoia e noho maikaʻi i ka wai ma ka inu ʻana i ka wai nui i ka lā.

E hāʻawi ʻia ka infusion me ka lohi e hōʻemi i nā hopena. E nānā pono kāu hui mālama ola iā ʻoe i ka wā a ma hope o ka lapaʻau. Manaʻo kekahi poʻe he mea kōkua ke lawe mai i kahi puke a i ʻole papa e noho ai i ka wā o ka manawa infusion.

Pehea ka lōʻihi e lawe ai au i ka Alpha-1-Proteinase Inhibitor?

He hana maʻamau ka Alpha-1-proteinase inhibitor no ke ola. Ma muli o ka maʻi genetic ka alpha-1 antitrypsin deficiency, e pilikia mau ana kou kino i ka hana ʻana i ka nui o kēia protein pale nona iho.

ʻO ka pahuhopu ka mālama ʻana i ka pale mau no kou mau māmā no nā makahiki he nui. ʻO ka hooki ʻana i ka lapaʻau e ʻae i nā enzymes ʻino e hoʻomau i ka hōʻino ʻana i kou ʻiʻo māmā, e alakaʻi ana i ka holomua wikiwiki o ka emphysema a i ʻole nā pilikia māmā ʻē aʻe.

E nānā mau kāu kauka i ka hana o kou māmā a me nā pae protein ma o nā hoʻokolohua koko a me nā hoʻokolohua hanu. Kōkua kēia mau mea e hoʻoholo inā e hana pono ana ka lapaʻau a inā pono e hoʻoponopono i kāu hoʻolālā mālama.

He aha nā hopena ʻaoʻao o Alpha-1-Proteinase Inhibitor?

Hoʻomanawanui ka hapa nui o nā kānaka i kēia lāʻau lapaʻau, akā e like me nā lapaʻau ʻē aʻe, hiki iā ia ke hana i nā hopena ʻaoʻao. ʻO ka hoʻomaopopo ʻana i ka mea e manaʻo ai hiki ke kōkua iā ʻoe e mākaukau a ʻike i ka manawa e hoʻopili aku ai i kāu hui mālama ola.

ʻO nā hopena ʻaoʻao maʻamau e pili ana i ka ʻoi aku o 1 i ka 10 mau kānaka e pili ana i nā hopena maʻalahi ma ke kahua infusion, e like me ka ʻeha liʻiliʻi, ʻulaʻula, a i ʻole ka pehu ʻana ma kahi i waiho ʻia ai ka nila IV. Hiki nō hoʻi iā ʻoe ke ʻike i nā poʻo maʻalahi, ka ʻūlū, a i ʻole ka luhi ma hope o kāu infusion.

• Nā hopena kahua infusion (ʻeha, ʻulaʻula, pehu)
• Poʻo
• ʻŪlū a i ʻole ka māmā
• Luhi a i ʻole ka manaʻo luhi
• Nausea a i ʻole ka ʻōpū maʻalahi
• ʻEha ʻiʻo

Hoʻomaikaʻi maʻamau kēia mau hopena ʻaoʻao maʻamau i loko o kekahi mau hola ma hope o ka lapaʻau a ʻaʻole pono e hooki i ka lāʻau lapaʻau.

Hiki ke hana ʻia nā hopena ʻaoʻao liʻiliʻi akā ʻoi aku ka koʻikoʻi ma kahi o 1 i ka 100 mau kānaka. Aia kēia mau mea i nā hopena maʻi, e hōʻike ʻia paha e like me ka ʻili, ka ʻū, ka pilikia o ka hanu, a i ʻole ka pehu ʻana o kou maka, nā lehelehe, a i ʻole ka ʻāʻī.

• Nā hopena maʻi (ʻōpala, ʻūlū, pilikia hanu)
• ʻEha umauma a i ʻole ka paʻa
• Puʻuwai wikiwiki
• ʻEha poʻo nui
• Hiva nui a i ʻole nā anu
• Nausea nui a i ʻole ka luaʻi

Inā ʻike ʻoe i kekahi o kēia mau hōʻailona koʻikoʻi, e haʻi koke i kāu hui mālama ola. Hiki iā lākou ke hoʻoponopono i kāu lapaʻau a i ʻole e hāʻawi i ke kākoʻo hou e kōkua i ka mālama ʻana i kēia mau hopena.

I nā manawa kakaʻikahi, hiki i kekahi poʻe ke hoʻomohala i nā ʻōpala koko a i ʻole e ʻike i nā hopena maʻi koʻikoʻi i kapa ʻia ʻo anaphylaxis. Hiki ke loaʻa kēia mau pilikia koʻikoʻi i ka liʻiliʻi ma mua o 1 i ka 1,000 poʻe, akā e mākaukau mau kāu hui lapaʻau e mālama iā lākou inā loaʻa.

ʻO wai ka mea pono ʻole e lawe i ka Alpha-1-Proteinase Inhibitor?

ʻAʻole kūpono kēia lāʻau lapaʻau no nā mea a pau. E nānā pono kāu kauka i kāu moʻolelo olakino e hōʻoia i ka palekana o ka loaʻa ʻana o kēia lapaʻau.

ʻAʻole pono ʻoe e loaʻa i ka alpha-1-proteinase inhibitor inā ua loaʻa iā ʻoe kahi hopena maʻi koʻikoʻi i kēia lāʻau lapaʻau a i ʻole kekahi o kāna mea i hala. Pono paha nā poʻe me kekahi mau maʻi koko a i ʻole ka poʻe e lawe ana i nā lāʻau lahilahi koko e pono ai i nā lapaʻau ʻē aʻe.

Makemake kāu kauka e ʻike e pili ana i kekahi moʻolelo o nā ʻōpala koko, nā pilikia o ka puʻuwai, a i ʻole ka maʻi kīkī. ʻOiai ʻaʻole e kāpae ʻia kēia mau kūlana i ka lapaʻau, pono paha lākou i ka nānā ʻana a i ʻole nā hoʻoponopono ʻana i ka dosis e mālama iā ʻoe.

Inā hāpai ʻoe a i ʻole e hānai ana i ka waiū, e hōʻoia e kūkākūkā i kēia me kāu hui mālama ola. ʻOiai aia ka noiʻi palena ʻia i ka palekana i ka wā hāpai, hiki i kāu kauka ke kōkua i ka loiloi i nā pōmaikaʻi a me nā pilikia no kāu kūlana.

Nā inoa inoa Alpha-1-Proteinase Inhibitor

He nui nā hui lāʻau lapaʻau e hana i nā lāʻau lapaʻau alpha-1-proteinase inhibitor, a loaʻa lākou ma lalo o nā inoa inoa like ʻole. ʻO nā mana i kauoha pinepine ʻia e pili ana iā Prolastin-C, Glassia, a me Zemaira.

ʻOiai aia i loko o kēia mau huahana ka mea hana like, ʻokoʻa paha lākou i ke ʻano o ka hana ʻana a i ʻole ka hoʻopaʻa ʻana. E koho kāu kauka i ke koho kūpono loa e pili ana i kāu mau pono kikoʻī a me ka mōʻaukala olakino.

Hana like nā lāʻau lapaʻau a pau a loaʻa ka pono like. Inā pono ʻoe e hoʻololi ma waena o nā hōʻailona no kekahi kumu, e alakaʻi kāu hui mālama ola iā ʻoe ma o ka hoʻololi ʻana me ka palekana.

Nā Koho ʻē aʻe o ka Alpha-1-Proteinase Inhibitor

I kēia manawa, ʻo ka IV alpha-1-proteinase inhibitor ka lāʻau lapaʻau mua no ka hemahema o ka alpha-1 antitrypsin. Eia naʻe, ke hana nei nā mea noiʻi i ka hoʻomohala ʻana i nā ʻano hana hāʻawi ʻē aʻe i hiki ke ʻoluʻolu i ka wā e hiki mai ana.

Ke aʻo ʻia nei kahi ʻano inhaled o ka lāʻau lapaʻau i nā hoʻokolohua lapaʻau. Hiki i kēia ke ʻae iā ʻoe e lawe i ka lāʻau lapaʻau ma ka home ma mua o ka hele ʻana i kahi hale mālama ola i kēlā me kēia pule. Eia naʻe, ʻaʻole i loaʻa kēia koho no ka hoʻohana maʻamau.

No kekahi poʻe, ʻo nā lāʻau lapaʻau kākoʻo e like me nā bronchodilators, oxygen therapy, a i ʻole ka hoʻoponopono ʻana i ka pulmonary e hiki ke hoʻopili i ka lāʻau lapaʻau alpha-1-proteinase inhibitor. ʻAʻole lākou e pani i ka pono no ka hoʻololi ʻana i ka protein akā hiki ke kōkua i ka mālama ʻana i nā hōʻailona a hoʻomaikaʻi i ka maikaʻi o ke ola.

ʻOi aku ka maikaʻi o ka Alpha-1-Proteinase Inhibitor ma mua o nā lāʻau lapaʻau ʻē aʻe?

ʻO ka alpha-1-proteinase inhibitor i kēia manawa wale nō ka lāʻau lapaʻau e kamaʻilio pololei i ka hemahema protein i ka hemahema o ka alpha-1 antitrypsin. Hiki i nā lāʻau lapaʻau ʻē aʻe ke kōkua i ka mālama ʻana i nā hōʻailona akā ʻaʻole e hāʻawi i nā pōmaikaʻi pale like no kou mau māmā.

Ke hoʻohālikelike ʻia me ka hoʻohana ʻana i nā bronchodilators a i ʻole nā lāʻau lapaʻau COPD ʻē aʻe wale nō, ua hōʻike ʻia ka lāʻau lapaʻau hoʻololi protein e lohi i ka holomua ʻana o ka pōʻino o ka māmā. Hōʻike nā haʻawina hiki iā ia ke kōkua i ka mālama ʻana i ka hana māmā i ka manawa, ʻoi aku ka maikaʻi ke hoʻomaka ʻia ma mua o ka hiki ʻana mai o ka pōʻino nui.

ʻO ka pōmaikaʻi nui, ua kuhikuhi ʻia i ke kumu kumu o ka pilikia ma mua o ka mālama ʻana i nā hōʻailona. ʻOiai he mea nui nā lāʻau lapaʻau ʻē aʻe no ka mālama ʻana i nā pilikia hanu i kēlā me kēia lā, hāʻawi ka alpha-1-proteinase inhibitor i ka pale lōʻihi kū hoʻokahi ʻaʻole hiki i nā koho ʻē aʻe ke hāʻawi.

Nā Nīnau i Nīnau Pinepine ʻia e pili ana i ka Alpha-1-Proteinase Inhibitor

He Palekana anei ka Alpha-1-Proteinase Inhibitor no ka poʻe me ka maʻi puʻuwai?

Hiki ke hoʻohana palekana ʻia ka alpha-1-proteinase inhibitor i ka poʻe me ka maʻi puʻuwai, akā he mea nui ka nānā ʻana. Hāʻawi ʻia ka lāʻau lapaʻau me ka lohi e hōʻemi i ka pilikia o ka wai nui a i ʻole ke koʻikoʻi cardiovascular.

E nānā pono kāu kauka i kou kūlana puʻuwai ma mua o ka hoʻomaka ʻana i ka lāʻau lapaʻau. Hiki iā lākou ke hoʻoponopono i ka helu infusion a i ʻole e nānā pono iā ʻoe i ka wā o ka lāʻau lapaʻau e hōʻoia i ka mālama maikaʻi ʻana o kou puʻuwai i ka wai hou.

He aha kaʻu e hana ai inā loaʻa iaʻu ka nui o ka Alpha-1-Proteinase Inhibitor?

No ka mea, hāʻawi ʻia kēia lāʻau lapaʻau e nā poʻe loea mālama ola i nā wahi i mālama ʻia, he mea maʻamau loa nā overdoses ulia pōpilikia. Hoʻohālikelike ʻia ka dosing me ka akahele e pili ana i kou kaumaha a nānā ʻia i ka wā o ke kaʻina infusion.

Inā hopohopo ʻoe e pili ana i kāu mahele a i ʻole e ʻike i nā hōʻailona maʻamau i ka wā a i ʻole ma hope o ka lāʻau lapaʻau, e kamaʻilio koke me kāu hui mālama ola. Hiki iā lākou ke loiloi inā pono ka nānā ʻana a i ʻole ka lāʻau lapaʻau.

He aha kaʻu e hana ai inā hala iaʻu kahi mahele o ka Alpha-1-Proteinase Inhibitor?

Inā hala ʻoe i kāu infusion i hoʻonohonoho ʻia i kēlā me kēia pule, e hoʻokaʻaʻike i kāu mea mālama ola i ka hiki wawe e hoʻonohonoho hou. He mea nui e mālama i nā pae protein paʻa i loko o kāu ʻōnaehana no ka pale ʻana i ka māmā.

E hoʻāʻo ʻaʻole e hele ma mua o 7-10 mau lā ma waena o nā lāʻau lapaʻau inā hiki. Hiki i kāu hui lapaʻau ke kōkua iā ʻoe e hoʻi i ke ala me kāu papa manawa maʻamau a hiki ke nānā pono i kāu mau pae protein ma hope o ka mahele i hala.

I ka manawa hea e hiki ai iaʻu ke hooki i ka lawe ʻana i ka Alpha-1-Proteinase Inhibitor?

Hoʻomau mau ʻia ka alpha-1-proteinase inhibitor inā olakino ʻoe e loaʻa ai a ke hāʻawi nei i nā pōmaikaʻi. ʻAʻole e pau ka maʻi genetic, no laila pono ka pale mau.

E loiloi mau kāu kauka inā kūpono ka lapaʻau iā ʻoe. ʻO nā mea e like me ke olakino holoʻokoʻa, ka hana o ka māmā, a me ka maikaʻi o ke ola e pāʻani i ke koho ʻana inā e hoʻomau i ka therapy.

Hiki iaʻu ke hele i ka wā e lawe ana i ka Alpha-1-Proteinase Inhibitor?

ʻAe, hiki iā ʻoe ke hele i ka wā e loaʻa ana kēia lapaʻau, akā pono ia i kekahi hoʻolālā. Pono ʻoe e hoʻopili me nā keʻena mālama ola ma kāu wahi e hoʻonohonoho ai i nā infusions, a i ʻole e hoʻoponopono i kāu papa huakaʻi e pili ana i kāu mau lā lapaʻau.

Hiki i nā kikowaena lapaʻau he nui ke kōkua i ka hoʻonohonoho ʻana i ka mālama ʻana me nā keʻena ma nā kūlanakauhale ʻē aʻe. ʻOi aku ka maikaʻi e hoʻomaka i ka hoʻolālā ʻana i kekahi mau pule ma mua o kāu huakaʻi e hōʻoia i ka hoʻomau ʻana o ka mālama ʻana i ka wā e hele ana ʻoe.