E aʻo wale kākou e pili ana i ka Pulmonary Alveolar Proteinosis (PAP), kahi maʻi e hoʻopaʻa ai i nā ʻeke ea i loko o nā māmā.

Ke manaʻo nei ʻoe i kekahi manawa ua pilikia ʻoe i ka hanu ʻana, me he mea lā ua paʻa kou umauma? Ke luhi nei ʻoe ma hope o ka hele wāwae ʻana i kahi mamao pōkole? Hoʻopoina pinepine mākou i kēia mau mea, me ka manaʻo he luhi maʻamau wale nō ia. Eia naʻe, i kēia lā e kamaʻilio mākou e pili ana i kahi maʻi koʻikoʻi hiki ke kumu o kēia mau hōʻailona, ​​​​ʻoiai he mea kakaikahi loa. ʻO ia ka Pulmonary Alveolar Proteinosis, a i ʻole PAP no ka pōkole.

I ka ʻōlelo maʻalahi, he aha ka Pulmonary Alveolar Proteinosis (PAP)?

E noʻonoʻo ua piha ko kākou mau māmā i nā miliona o nā ʻeke ea liʻiliʻi. Kāhea mākou i kēia mau ʻeke ea liʻiliʻi he alveoli . Ke hanu mākou, ma o kēia mau ʻeke ea mākou e lawe ai i ka oxygen.

I kēia manawa, ma ka PAP, ʻo ka mea e hana ʻia ana, ʻo ia ka hōʻiliʻili ʻana o nā protein, nā momona, a me nā mea ʻē aʻe i loko o kēia mau ʻeke ea liʻiliʻi, e like me ka ʻōpala. ʻO ke kumu o kēia, ʻo nā cell e hoʻomaʻemaʻe i ka mea like me ka ʻaila i kapa ʻia ʻo surfactant, ka mea i loaʻa maoli i loko o nā paia o kēia mau ʻeke ea, ʻaʻole hana pono. A laila hōʻiliʻili kēia surfactant a ālai i nā ʻeke ea. No laila, ʻaʻole hiki i ka ea ke komo pono, no laila ʻaʻole loaʻa i ke koko ka nui o ka oxygen e pono ai. Hoʻopilikia kēia i ka hanu ʻana.

Aia kekahi mau ʻano nui o ka PAP?

ʻAe, ua māhele nui ʻia ka PAP i ʻekolu ʻano. He ʻokoʻa ke kumu o kēlā me kēia ʻano.

ʻO wai ka mea i ʻoi aku ka pilikia o ka loaʻa ʻana o kēia maʻi?

Hoʻopilikia pinepine ka maʻi autoimmune a me ka PAP lua i nā kānaka ma waena o 30 a me 60 mau makahiki. ʻOi aku ka maʻamau o kēia mau ʻano ʻelua i nā kāne . ʻOi aku ka nui o ka pilikia o ka poʻe puhipaka a me ka poʻe i pili i ka lepo a i ʻole nā ​​​​​​kemika .

ʻO ka PAP Congenital ka mea maʻamau i nā keiki ma lalo o 10 mau makahiki, akā hiki ke loaʻa i kēlā me kēia makahiki.

He aha nā hōʻailona o ka maʻi PAP?

ʻO ka hōʻailona nui a maʻamau o kēia maʻi, ʻo ia ka pōkole o ka hanu (dyspnea) . Loaʻa i ka hapa nui o ka poʻe ka pōkole o ka hanu i ka wā hoʻoikaika kino a hana paha. Eia nō naʻe, hiki i kekahi poʻe ke ʻike i kēia pilikia ʻoiai ke kū mālie nei lākou.

Eia kekahi, hiki iā ʻoe ke ʻike i kēia mau hōʻailona:

• ʻEha umauma
• ʻŪhū (malia paha me ka phlegm a i ʻole ke koko)
• ʻO ke kala polū o ka ʻili a me nā kui (cyanosis) - Hana ʻia kēia ke lawa ʻole ka oxygen i loko o ke koko.
• Luhi mau
• kuni
• Nā maʻi pinepine o ka māmā
• Nā manamana lima i hoʻopaʻa ʻia
• ʻO ka pohō kaumaha me ke kumu ʻole

Pehea e ʻike ai ke kauka i kēia maʻi?

Inā kānalua ʻoe he maʻi PAP kou, e nānā mua kāu kauka iā ʻoe a hoʻolohe i kou mau māmā me kahi stethoscope. A laila e nīnau lākou iā ʻoe e pili ana i ka mōʻaukala olakino o kou ʻohana a me kou nohona. Inā ʻoe e puhipaka, ua pili i ka lepo a i ʻole nā ​​​​​​kemika, pono ʻoe e haʻi i kāu kauka .

A laila, hiki ke hana i kekahi mau hoʻokolohua e hōʻoia i ka maʻi:

• Nā hoʻāʻo koko: E nānā i ka pae oxygen i loko o ke koko. Eia kekahi, hiki ke hana ʻia nā hoʻāʻo kikoʻī e like me ka GM-CSF autoantibody e ʻike ai i ke kumu o ka maʻi PAP.
• Hoʻāʻo ʻana i ka hana o ka puʻuwai: Ke ana i ka maikaʻi o ka hana ʻana o kou mau māmā.
• ʻO ke kiʻi X-ray o ka umauma a i ʻole ka CT scan: No ka ʻike pono ʻana i ke kūlana o nā māmā.
• Bronchoscope:He kaʻina hana e hoʻokomo ʻia ai kahi ʻōmole lahilahi me kahi kāmela i hoʻopili ʻia ma o ka waha ma lalo o ka anesthesia e nānā i loko o ke ala ea a me nā māmā.
• ʻO ka biopsy māmā: Lawe ʻia kahi ʻāpana liʻiliʻi o ka ʻiʻo mai ka māmā i ka wā o ka bronchoscopy a i ʻole i ka wā o ke ʻoki ʻana no ka hoʻokolokolo ʻana.

He aha nā lāʻau lapaʻau no ka PAP?

ʻAʻohe lāʻau lapaʻau no ka PAP i kēia manawa, akā aia nā lāʻau lapaʻau kūpono loa e hiki ke kōkua iā ʻoe e mālama i kāu mau hōʻailona a ola i kahi ola maikaʻi aʻe.

ʻO ka lāʻau lapaʻau nui a hoʻohana pinepine ʻia no ka PAP ʻo "Whole-Lung Lavage" (WLL) .

He aha kēia holoi ʻana i ka māmā?

ʻO ka mea e hana ʻia ma kēia, ʻo ia ka hoʻohana ʻana o ke kauka i kahi bronchoscope (kahi ʻōmole kāmela) e holoi i nā māmā pākahi me ka saline (wai paʻakai i hoʻomaʻemaʻe ʻia) e wehe i nā protein a me nā momona i paʻa. Ma hope o kēia, ua lilo i mea maʻalahi ka hanu ʻana.

I ka wā e hana ai i kēia lāʻau lapaʻau:

• E hoʻopau loa ʻia ʻoe i ka anesthesia, no laila ʻaʻole ʻoe e manaʻo i kekahi mea.
• Hoʻokomo ke kauka i ka bronchoscope ma ka waha a kuhikuhi iā ia i kekahi māmā.
• Hoʻolako ʻia ka oxygen i kekahi māmā e kahi mīkini.
• I kekahi manawa, e ʻaʻahu ʻoe i kahi ʻaʻahu haʻalulu a puni kou umauma. Hoʻokuʻu kēia haʻalulu i nā mea i paʻa i loko o kāu mau ʻeke ea.
• A laila hoʻokomo ʻia ka wai saline i loko o nā māmā, a i ka wā e huki ʻia ai, puka mai ia me nā mea i paʻa.
• Malia paha e holoi ʻia kekahi māmā i ka manawa like, a i ʻole ma hope o kekahi mau lā.

Nā lāʻau lapaʻau ʻē aʻe

• Nā Bronchodilators: Hoʻomaha kēia mau lāʻau lapaʻau i nā ʻiʻo a puni nā alaloa, e maʻalahi ai ka hanu ʻana.
• Lapaʻau pani GM-CSF: Hoʻoheheʻe ʻia kēia ma o kahi nebulizer a i ʻole hāʻawi ʻia ma ke ʻano he inikini. Hoʻomaikaʻi ia i ka hana o nā cell e hoʻomaʻemaʻe i nā ala ea.
• Hoʻoili māmā: ʻO ka poʻe nona ka maʻi koʻikoʻi loa a ua hōʻino ʻia ko lākou mau māmā e pono paha i kahi hoʻoili māmā olakino ma ke ʻano he koho hope loa.
• Plasmapheresis a me ka hoʻololi plasma: Pili kēia i ka wehe ʻana i ka ʻāpana wai o ke koko (plasma) a hoʻololi iā ia me ka plasma mai kahi kanaka olakino. I loko o ka PAP autoimmune, kōkua kēia i ka pale ʻana i ka ʻōnaehana pale mai ka hoʻouka ʻana i nā cell olakino.
• ʻOkikene hoʻohui: Hāʻawi ʻia ka oxygen ma o kahi pale maka a i ʻole kahi ʻōmole liʻiliʻi i kau ʻia i loko o ka ihu e maʻalahi ai ka hanu ʻana.

He aha kaʻu e hana ai ma ke ʻano he kanaka me PAP?

Inā loaʻa iā ʻoe ka PAP, he mea nui loa ia e hahai i ka lāʻau a kāu kauka e kuhikuhi ai a mālama pū i kou nohona.

• E pale loa i ka puhipaka a pale i nā wahi e puhipaka ai kekahi poʻe.
• E kiʻi i nā lāʻau pale no ka maʻi palū (flu) a me ka niumonia e like me ka ʻōlelo a kāu kauka.
• E noho mamao aku mai ka poʻe maʻi.
• E ʻai i ka meaʻai momona a kaohi i kou kaumaha.
• E holoi pono i kou mau lima ma hope o ka hoʻohana ʻana i ka lua a ma mua a ma hope o ka ʻai ʻana.
• E kamaʻilio me kāu kauka a hoʻomohala i kahi hoʻolālā hoʻoikaika kino palekana e kūpono iā ʻoe.