Ua loaʻa anei kēia pilikia me kou mau māmā? (Pulmonary Alveolar Proteinosis - PAP) E kamaʻilio kākou e pili ana i kēia!

Ke manaʻo nei ʻoe ua pōkole iki ka hanu? A i ʻole ua luhi wale ʻoe? I kekahi manawa ua manaʻo ʻia kēia mau mea he mea maʻamau, akā i nā hihia kakaikahi, hiki iā lākou ke lilo i mau hōʻailona o ka maʻi māmā. I kēia lā e kamaʻilio mākou e pili ana i kahi maʻi māmā kakaikahi loa akā koʻikoʻi e pono e ʻike. Ua kapa ʻia kēia ʻo `(Pulmonary Alveolar Proteinosis)` a i ʻole `(PAP)`. ʻOiai paha he paʻakikī iki ka inoa, e wehewehe maʻalahi kākou.

He aha ka `(Pulmonary Alveolar Proteinosis - PAP)`? I ka ʻōlelo maʻalahi...

ʻAe, e nānā kākou i kēia ʻano i kapa ʻia ʻo `(PAP)`. I ka ʻōlelo maʻalahi, he maʻi laha ʻole ia e kū mai ana i ka wā e hōʻiliʻili ai nā protein, nā momona, a me kekahi mau mea ʻē aʻe i loko o nā ʻeke ea liʻiliʻi (alveoli) i loko o ko kākou mau māmā a hoʻopaʻa iā lākou. E noʻonoʻo, ua hana ʻia ko kākou mau māmā me nā miliona o nā ʻeke ea liʻiliʻi. ʻO kēia mau ʻeke ea liʻiliʻi ka mea a kākou e kapa aku nei he `alveoli` . Ke hanu kākou, lawe ʻia ka oxygen a hoʻokuʻu ʻia i loko o ke koko ma o kēia mau ʻeke ea.

No laila, i ke ʻano o ka `(PAP)`, ua piha kēia mau ʻeke ea i nā mea pono ʻole, e like me kahi ʻauwai i paʻa, e ālai ana i ke ala no ka oxygen e hiki ai i ke koko. He aha ka mea e hana ʻia? ʻAʻole lawa ka oxygen i ko mākou koko. Hiki i kēia ke pilikia i ka hanu ʻana (`dyspnea`) .

He maʻi koʻikoʻi kēia o ka māmā. No laila, he mea maʻamau ke manaʻo iki i ka makaʻu ke loaʻa iā ʻoe kahi hōʻailona e like me kēia. Akā, mai hopohopo. Me ka mālama kūpono, hiki iā ʻoe ke kāohi i ka maʻi a me kāna mau hōʻailona. Inā loaʻa iā ʻoe ka `(PAP)`, he mea nui loa ia e ʻike i ke kauka lapaʻau pulmonary no ka mālama ʻana. E aʻo pū ʻo ia iā ʻoe i nā hoʻololi olakino hiki iā ʻoe ke hana. Hiki i kēia ke hoʻomaikaʻi i kou ʻano ola.

Aia kekahi mau ʻano like ʻole o kēia maʻi `(PAP)`?

ʻAe, ʻekolu mau ʻano nui o ka maʻi `(PAP)`. E nānā kākou i ke ʻano o lākou:

1. Autoimmune PAP: ʻO kēia ke ʻano maʻamau o ka PAP. Ma kahi o 90% o nā pākeke e loaʻa i kēia ʻano. ʻO ka mea e hana ʻia ma ʻaneʻi, ʻo ia ka hoʻouka hewa ʻana o ka ʻōnaehana pale ponoʻī o ko mākou kino i nā cell e kōkua ana e hoʻomaʻemaʻe i nā ala ea. E noʻonoʻo iā ia me ko mākou mau pale ponoʻī e hakakā nei i kā mākou mau cell ponoʻī.

2. PAP Lua: Hana ʻia kēia ʻano ke hoʻopilikia ʻia ka hana o nā pūnaewele i loko o ko kākou ala ea, ma muli paha o kekahi maʻi ʻē aʻe a i ʻole ka ʻike ʻia ʻana i kahi mea ʻawahia (e like me ka lepo, nā kemika). ʻO ia hoʻi, hana ʻia no kekahi kumu ʻē aʻe.

3. Congenital PAP: He kūlana kēia i loaʻa i ka wā hānau. I kekahi manawa hiki ke loaʻa nā hōʻailona i ka wā hānau, a i ʻole e ʻike ʻia paha i nā makahiki like ʻole. ʻO kēia ma muli o nā loli i kekahi mau genes e kaohi ana i ka hana o nā cell i loko o ko kākou mau māmā. Hiki ke hoʻoilina ʻia kēia mai nā mākua a i nā keiki.

ʻO wai ka mea i ʻoi aku ka paʻakikī ke loaʻa i kēia maʻi `(PAP)`?

Ma keʻano laulā, ʻo nā ʻano ʻelua i hoʻopilikia nui ʻia ʻo ia ka "Autoimmune PAP" a me ka "Secondary PAP".No nā kānaka ma waena o 30 a me 60 mau makahiki. Eia kekahi, ʻoi aku ka maʻamau o kēia mau ʻano ʻelua i nā kāne . ʻO ka poʻe puhi paka a me ka poʻe i hōʻike ʻia i kekahi ʻano lepo a i ʻole nā ​​​​​​kemika e ʻoi aku ka nui o ka pilikia o ka loaʻa ʻana o kēia maʻi.

ʻO ke ʻano i kapa ʻia ʻo `(Congenital PAP)` ʻike pinepine ʻia i nā keiki ma lalo o 10 mau makahiki. Eia naʻe, e like me ka mea i ʻōlelo ʻia ma mua, hiki ke loaʻa i kēlā me kēia makahiki.

Pehea ka maʻamau o ka maʻi PAP?

ʻO ka ʻoiaʻiʻo, he maʻi laha ʻole ka `(PAP)`. Ma ka honua holoʻokoʻa, hoʻopilikia kēia maʻi i kahi kanahā o ʻekolu miliona mau kānaka. ʻO ke ʻano kēia he kakaikahi loa paha ka poʻe me kēia maʻi ma Sri Lanka.

No ke aha e loaʻa ai kēia maʻi `(PAP)`? He aha ke kumu?

E like me kā mākou i kamaʻilio ai ma mua, he lahilahi loa nā paia o ko kākou mau ʻeke ea (alveoli). Ma o kēia mau paia lahilahi e hele ai ka oxygen mai nā māmā i loko o ke koko. Loaʻa i nā paia o kēia mau ʻeke ea kahi papa ʻaila i kapa ʻia he surfactants . Kōkua kēia mau surfactants i ka mālama ʻana i nā ʻeke ea e wehe me ka ʻole o ka pani ʻana iā lākou. ʻO ia ka manawa e hiki ai i ka oxygen ke komo maʻalahi i loko o ke koko.

I kēia manawa, i loko o ko kākou mau māmā, aia kekahi ʻano cell kūikawā i kapa ʻia ʻo 'alveolar macrophages' . Ua like kēia mau cell me nā limahana hoʻomaʻemaʻe. ʻO kā lākou hana, ʻo ia ka wehe ʻana i kēia mau '(surfactants)' mai nā ʻeke ea i kēlā me kēia manawa, e pale ana iā lākou mai ka paʻa ʻana.

Eia naʻe, ʻo ka mea e hana ʻia ana i loko o ke kanaka me ka PAP, ʻo ia ka loaʻa ʻole o kēia mau cell i kapa ʻia ʻo macrophages i ka hōʻailona e hoʻomaʻemaʻe pono i nā paia o nā ʻeke ea. He aha ka mea e hana ʻia a laila? Hōʻiliʻili kēlā mau surfactants a ālai i nā ʻeke ea. E like me kahi paipu i paʻa i loko o kahi poho holoi. A laila ua ālai ʻia ke ala no ka oxygen e hiki ai i ke koko.

I ka ʻōlelo maʻalahi, ua hoʻokumu ʻia kēia kūlana e ka nāwaliwali o ka "ʻōnaehana hoʻomaʻemaʻe ʻōpala" o nā māmā.

He aha nā hōʻailona o ka maʻi `(PAP)`?

ʻO ka hōʻailona nui a maʻamau o ka PAP ka pōkole o ka hanu (dyspnea) . ʻOiai ka hapa nui o ka poʻe e ʻike nei i ka pōkole o ka hanu i ka wā hoʻoikaika kino, hiki i kekahi poʻe ke ʻike i kēia pilikia ʻoiai ke kū mālie nei lākou.

Eia kekahi, aia kekahi mau hōʻailona ʻē aʻe:

• Ke manaʻo nei i ka ʻeha ma ka umauma.
• Kuke. I kekahi manawa aia paha he koko liʻiliʻi e puka mai ana me ka huʻa.
• ʻO ke kala polū o ka ʻili a me nā kui (cyanosis) (hana kēia ke ʻaʻole lawa ka oxygen i loko o ke koko).
• Manaʻo luhi mau.
• Kuni.
• Nā maʻi pinepine o ka māmā.
• Hoʻonui ʻia nā wēlau o nā manamana lima a lilo i ʻano e like me ka pahu ('clubbed fingers').
• Ka pohō kaumaha me ke kumu ʻole.

Inā loaʻa iā ʻoe kekahi a ʻoi aku paha o kēia mau hōʻailona, ​​​​he mea nui loa e ʻimi i ke aʻo lapaʻau.

Hiki i ka maʻi PAP ke make?

I nā hihia koʻikoʻi, hiki i ka PAP ke hoʻoulu i ka hanu hanu e hoʻoweliweli i ke ola.ʻO ke ʻano kēia, ʻaʻole hiki i nā māmā ke hana pono i kā lākou hana. Eia nō naʻe, ʻokoʻa ke ʻano o kēia maʻi mai kēlā kanaka a kēia kanaka. Hiki i kekahi poʻe ke ho'ōla mai ka maʻi me ka ʻole o ka lāʻau lapaʻau ('spontaneous remission'). No kekahi poʻe, noho mau ka maʻi ma ka pae like. I nā hihia koʻikoʻi, hiki ke make ma muli o ka hāʻule ʻana o ka hanu a i ʻole nā ​​maʻi ʻē aʻe. No laila he mea nui e ʻike mua i ka maʻi a loaʻa ka lāʻau lapaʻau.

Pehea e ʻike ʻia ai ka PAP? He aha nā hoʻokolohua e hana ʻia?

Inā kānalua ke kauka he PAP kou, e nānā mua ʻo ia iā ʻoe a hoʻolohe i kou mau māmā. E nīnau pū ʻo ia iā ʻoe i nā nīnau e pili ana i kou mōʻaukala olakino pilikino a me ka ʻohana a me kou nohona. Inā ʻoe e puhipaka a i ʻole e pili ana i ka lepo a i ʻole nā ​​​​​​kemika, e hōʻoia e haʻi i kāu kauka.

ʻO nā hoʻāʻo e hōʻoia i ka PAP e komo pū me:

• Nā hoʻāʻo koko: E nānā i nā pae oxygen i loko o kou koko.
• He hoʻāʻo koko kūikawā: E nānā i kahi hōʻailona i kapa ʻia ʻo `(granulocyte-macrophage colony-stimulating factor - GM-CSF) autoantibody`. Kōkua kēia i ka ʻike ʻana i ke kumu o ka maʻi `(PAP)`.
• Hoʻāʻo ʻana i ka hana o ka puʻuwai: E ana i ka maikaʻi o ka hana ʻana o kou mau māmā.
• Nā hoʻokolohua kiʻi: Hoʻokomo pū ʻia kēia me kahi X-ray umauma a i ʻole CT scan. Hiki i kēia ke hāʻawi i kahi kiʻi maopopo o ke kūlana o nā māmā.
• Bronchoscopy: Ma kēia, ua hoʻokomo ʻia kahi ʻōmole lahilahi (me kahi kāmela) ma o ka waha i loko o nā ala ea a ua nānā ʻia ka ʻaoʻao o loko o nā māmā.
• ʻO ka biopsy māmā: Lawe ʻia kahi ʻāpana liʻiliʻi o ka ʻiʻo mai ka māmā no ka hoʻokolokolo ʻana, i ka wā o ka bronchoscopy a i ʻole i ka wā o ke ʻoki ʻana.

ʻO kēia mau hoʻokolohua ka mea e hiki ai i nā kauka ke hoʻomaopopo pololei i ka PAP.

Aia kekahi lāʻau lapaʻau piha no ka maʻi `(PAP)`?

A hiki i kēia manawa, ʻaʻohe lāʻau lapaʻau no ka PAP. Akā, mai hopohopo. Nui nā lāʻau lapaʻau e hiki ke kōkua iā ʻoe e kāohi i kāu mau hōʻailona a ola i kahi ola maikaʻi aʻe.

Pehea e mālama ʻia ai ka maʻi PAP?

ʻO ka lāʻau lapaʻau nui a hoʻohana pinepine ʻia no ka PAP ʻo ia ka Whole-Lung Lavage (WLL) . Ua kapa ʻia kēia ʻo "lung lavage." E noʻonoʻo e like me ka holoi ʻana i kou mau māmā.

Hoʻohana ke kauka i kahi bronchoscope (ka ʻōmole lahilahi me kahi kāmela) a me ka hopena saline (wai paʻakai sterile) e hoʻomaʻemaʻe i hoʻokahi māmā i ka manawa. E maʻalahi kēia iā ʻoe ke hanu. Hiki i kēia kaʻina hana ke hala ma kahi o ʻehā a ʻelima mau hola. Ma muli o ke koʻikoʻi o kāu mau hōʻailona, ​​​​pono paha ʻoe e loaʻa iā WLL i kēlā me kēia mau mahina a i ʻole hoʻokahi manawa i ka makahiki. Eia nō naʻe, ʻaʻole pono kekahi mau maʻi PAP iā WLL. Aia kēia i ke koʻikoʻi o kou kūlana.

He aha ka mea e hana ʻia ke hana ʻoe i ka `(Whole-Lung Lavage - WLL)`?

• E hāʻawi ʻia ʻoe i ka anesthesia laulā a i ʻole nā ​​​​lāʻau hoʻomaha.
• Hoʻokomo ke kauka i kahi bronchoscope ma kou waha i loko o kou ala ea a kuhikuhi iā ia i loko o kekahi o kou mau māmā.
• Hāʻawi ka mīkini i ka oxygen i kekahi māmā ʻē aʻe.
• ʻAʻahu ʻoe i kahi ʻaʻahu haʻalulu a puni kou umauma. Hoʻokuʻu kēia haʻalulu i nā surfactants i hoʻopaʻa ʻia i loko o nā ʻeke ea.
• A laila e hoʻokomo ke kauka i ka hopena saline i loko o nā māmā a huki hou i waho (suctions) me nā surfactants.
• I kekahi manawa hiki ke holoi ʻia kekahi māmā i ka manawa like, a i ʻole hiki ke holoi ʻia i loko o kekahi mau lā.

Ma waho aʻe o kēia lāʻau lapaʻau `(WLL)`, aia kekahi mau ʻano lapaʻau ʻē aʻe:

• Nā Bronchodilators: Hoʻomaha kēia mau lāʻau lapaʻau i nā ʻiʻo a puni kou mau alaloa, e maʻalahi ai iā ʻoe ke hanu.
• ʻO ka lāʻau lapaʻau hoʻololi Granulocyte-macrophage colony-stimulating factor (GM-CSF): Hāʻawi ʻia kēia ma o ka hanu ʻana ma o kahi nebulizer (kahi mea e hoʻohuli ai i ka lāʻau lapaʻau wai i mahu) a i ʻole ma ke ʻano he inikini. Hana kēia lāʻau lapaʻau ma ka hoʻomaikaʻi ʻana i ka hana o nā cell e hoʻomaʻemaʻe i nā ala ea (macrophages).
• Hoʻoili māmā: ʻO ka poʻe me ka maʻi PAP koʻikoʻi a me ka hōʻino ʻana o ka māmā e pono ai i kekahi manawa kahi hoʻoili māmā. Ma kēia kaʻina hana, hoʻokahi a i ʻole ʻelua o kou mau māmā maʻi e wehe ʻia a hoʻololi ʻia me kahi māmā mai kahi mea hāʻawi olakino.
• Plasmapheresis a me ka hoʻololi plasma: Pili kēia i ka wehe ʻana i ka ʻāpana wai o kou koko, i kapa ʻia ʻo plasma, a hoʻololi iā ia me ka plasma mai kahi mea hāʻawi olakino. No ka poʻe me ka PAP autoimmune, kōkua kēia lāʻau i ka pale ʻana i ka ʻōnaehana pale mai ka hoʻouka ʻana i nā cell olakino.
• ʻOkikene hoʻohui: Hiki i ka lāʻau lapaʻau oxygen ke kōkua iā ʻoe e hanu maʻalahi. Hiki ke hāʻawi ʻia ma o kahi pale maka ma kou maka a i ʻole kahi ʻōmole liʻiliʻi i kau ʻia i loko o kou ihu.
• Nā hoʻokolohua lapaʻau: Loaʻa paha iā ʻoe ka manawa kūpono e komo i ka noiʻi ʻana i nā lāʻau lapaʻau hou. E kamaʻilio me kāu kauka e pili ana i kēia.

Hiki ke pale ʻia ka maʻi PAP?

E like me kā mākou i kūkākūkā ai ma mua, aia nā ʻano PAP like ʻole. No laila, ʻaʻole hiki ke pale ʻia ka Autoimmune PAP a me ka Congenital PAP no ka mea pili lākou i nā kaʻina hana kūloko i loko o ko mākou kino.

Eia nō naʻe, hiki ke pale aku i ka `(Secondary PAP)` i kekahi ʻano. Pehea ʻoe e hana ai i kēlā? Pono ʻoe e hōʻemi i kou ʻike ʻana i nā mea ʻawahia i ka hiki . Inā ʻaʻole hiki kēlā, pono ʻoe e hoʻohana i kahi pale maka maikaʻi (`respirator` a i ʻole kahi pale maka i kūpono maikaʻi ʻia) e uhi pono ana i kou waha a me kou ihu.

He aha ka manaʻolana no kekahi me PAP?

Hiki i nā lāʻau lapaʻau maʻamau e like me ka `(Whole-Lung Lavage - WLL)` ke kōkua iā ʻoe e hanu maʻalahi a hōʻoluʻolu i nā hōʻailona ʻē aʻe. Hoʻōla kahi poʻe liʻiliʻi mai ka `(PAP)` me ka ʻole o ka lāʻau lapaʻau. Eia nō naʻe, inā ʻaʻole mālama ʻia, hiki i nā kūlana `(PAP)` koʻikoʻi ke alakaʻi i ka hāʻule ʻana o ka hanu a i ʻole ka make. No laila, he mea nui loa ia e hahai i nā ʻōlelo a kāu kauka.

Inā loaʻa iaʻu ka PAP, he aha kaʻu e hana ai noʻu iho?

Inā loaʻa iā ʻoe ka PAP, aia kekahi mau mea hiki iā ʻoe ke hana e mālama i kou olakino:

• E nīnau i kāu kauka e pili ana i nā hoʻokolohua lapaʻau no ka mālama ʻana iā PAP.
• Mai puhi paka. Eia kekahi, mai hanu i ka uahi lua i ka wā e puhi paka ai kekahi poʻe .
• E hahai i nā ʻōlelo a kāu kauka e pili ana i ka loaʻa ʻana o nā lāʻau lapaʻau no ka maʻi palū a me ka maʻi pneumonia.
• E noho mamao aku mai ka poʻe maʻi.
• E ʻai i ka meaʻai momona. E kāohi i ke kaumaha o kou kino.
• E holoi pono i kou mau lima ma hope o ka hoʻohana ʻana i ka lua, ma mua a ma hope o ka ʻai ʻana.
• E hana pū me kāu kauka e hoʻomohala i kahi hoʻolālā hoʻoikaika kino palekana e kūpono iā ʻoe.

E hoʻomanaʻo, ʻo ka Pulmonary Alveolar Proteinosis (PAP) kahi maʻi māmā laha ʻole e hiki ai ke pilikia i ka hanu ʻana a me nā hōʻailona ʻē aʻe ma ke kumu ʻana i nā ʻeke ea i loko o nā māmā e ālai ʻia. Eia nō naʻe, aia kekahi mau lāʻau lapaʻau kūpono e hiki ke kōkua i ka kaohi ʻana i nā hopena o ka PAP. Inā loaʻa iā ʻoe kēia ʻano, e noʻonoʻo e hana pū me kahi hui multispecialty. I ka hui pū ʻana, hiki iā lākou ke kōkua i ka hoʻomaikaʻi ʻana i kou ʻano ola.

Leka Lawe-Home (ʻO ka mea nui e hoʻomanaʻo)

ʻAe, ua kamaʻilio nui mākou e pili ana i ka `(Pulmonary Alveolar Proteinosis - PAP)`. ʻOiai he mea paʻakikī iki kēia, ʻo ka mea nui e hoʻomanaʻo ai , ʻoiai he maʻi laha ʻole kēia, he mea nui e pale aku i ka hopohopo a loaʻa ka ʻōlelo aʻoaʻo a me ka mālama kūpono.

• Inā loaʻa iā ʻoe nā hōʻailona e like me ka paʻakikī o ka hanu ʻana, ka ʻū mau, a i ʻole ka luhi pinepine, e ʻike i ke kauka.
• Inā ua ʻike ʻia ʻoe me ka `(PAP)`, e ʻimi i ka lāʻau lapaʻau ma lalo o ka nānā ʻana a kahi kauka pulmonologist .
• Aia nā lāʻau lapaʻau, no laila mai hoʻopau i ka manaʻolana. Hiki i nā lāʻau lapaʻau e like me `(Whole-Lung Lavage - WLL)` ke hāʻawi i ka nui o ka hōʻoluʻolu.
• ʻO ka hahai pono ʻana i nā ʻōlelo aʻoaʻo lapaʻau a me ke ola ʻana i kahi nohona olakino e kōkua nui i ka kaohi ʻana i ka maʻi.

Inā he mau nīnau hou aku kāu e pili ana i kēia, mai kānalua e kamaʻilio me kāu kauka. E noho olakino!

ʻO ka Proteinosis Alveolar Pulmonary, PAP, Maʻi Mama, Paʻakikī Hanu, Alveoli, Surfactants, Holoi ʻana i ka Mama Holoʻokoʻa