Aia kekahi pilikia me nā ʻauwai bile o kou akepaʻa? E aʻo pono kākou e pili ana i ka Primary Biliary Cholangitis (PBC)!

Auē, ke manaʻo nei hoʻi ʻoe ua luhi loa ʻoe, luhi ʻoiai me ka hana ʻole ʻana i kekahi mea? A i ʻole ke manaʻo nei ʻoe i kekahi manawa e like me ke kahakaha ʻana i kou ʻili me ke kumu ʻole? I kekahi manawa, ʻoiai ʻaʻole mākou e nānā nui i kēia mau mea, hiki iā lākou ke lilo i hōʻailona o kahi pilikia me ke akepaʻa, kekahi o nā ʻōkana koʻikoʻi loa i loko o ko mākou kino. I kēia lā e kamaʻilio mākou e pili ana i kēlā maʻi e hoʻopilikia ana i ke akepaʻa, a ʻoiai paha he paʻakikī iki ka inoa, he maʻalahi ke hoʻomaopopo. ʻO ia hoʻi (Primary Biliary Cholangitis) , i ʻike ʻia hoʻi ʻo (PBC) no ka pōkole.

He aha ka Primary Biliary Cholangitis (PBC)? E hoʻomaopopo maʻalahi kākou!

ʻAe, me he mea lā ua lōʻihi iki kēia inoa, ʻeā? E nānā kākou i kona manaʻo.

ʻO "Cholangitis" kahi mumū o kou mau ʻauwai bile . ʻIke ʻoe, ʻo ke wai i kapa ʻia ʻo bile, i hana ʻia i loko o ko kākou ake, e hele ana ma o kahi ʻōnaehana o nā ʻōmole liʻiliʻi. Ua kapa ʻia kēlā mau ʻōmole he mau ʻōmole bile. ʻO "Biliary" kekahi manaʻo e pili ana i kēia mau ʻōmole bile. ʻO "Primary" ke ʻano "kumu" a i ʻole "mua."

A laila, ke hoʻohui ʻoe i kēia mau mea a pau, ʻo ke ʻano o ka Primary Biliary Cholangitis , ʻaʻole ia ma muli o kekahi maʻi ʻē aʻe, ka maʻi, a i ʻole ka pale ʻana, akā ma muli o kēia maʻi, aia kahi mumū o nā ʻauwai bile i loko o kou ake . ʻOiai ʻaʻole kēia mumū e hana ʻia ma ke ʻano he pane i kekahi mea, ʻaʻole ʻoe ʻike i ka wā e pau ai. No laila, he ʻano ia e holomua mālie ana.

Ua kapa ʻia kēia maʻi ma mua ʻo Primary Biliary Cirrhosis no ka mea hiki iā ia ke hoʻopaʻakikī i ke akepaʻa a me ka ʻeha, e alakaʻi ana i kahi maʻi i kapa ʻia ʻo cirrhosis .

Pehea ka hopena o (PBC) i ko mākou kino?

E noʻonoʻo, he aha ka mea e hana ai inā e pehu mau ana kekahi ʻāpana o ko kākou kino? ʻO ka maʻamau, ʻo ka pehu manawa pōkole kekahi hapa o ke kaʻina hana ho'ōla. Akā inā hoʻomau kēia ʻano pehu mau , ma kahi o ka ho'ōla ʻana, hoʻomaka ka wahi e lilo i ʻōpala.

ʻO ia ka mea e hana ʻia i nā ʻauwai bile i loko o kou akepaʻa ma PBC. Hoʻopili ʻia kēia mau ʻauwai a ālai ʻia e ka ʻōpala . A laila, ʻaʻole hiki i ka bile i hana ʻia e ke akepaʻa ke kahe pono. Hoʻomaka kēia bile e hoʻihoʻi i loko o ke akepaʻa. Ke hōʻiliʻili ʻia kēia bile, hōʻino ia i nā hunaola akepaʻa a hoʻoulu i ka mumū i loko o ke akepaʻa. I ka hala ʻana o ka manawa, ua pani ʻia kēia ʻiʻo akepaʻa olakino e ka ʻiʻo ʻōpala. I ka hopena, ʻaʻole hiki i ke akepaʻa ke hana pono, ʻo ia ka mea a mākou i ʻōlelo ai ma mua (cirrhosis) .

He maʻi koʻikoʻi anei ka PBC?

ʻAe, he maʻi mau a holomua ka Primary Biliary Cholangitis. ʻO ke ʻano kēia, ʻaʻole ia e hoʻomaikaʻi wale, hiki ke ʻoi aku ka ʻino i ka hala ʻana o ka manawa. ʻAʻole ia e ʻoi aku ka ʻino i ka manawa hoʻokahi, e ʻoi aku ka ʻino ma o nā pae like ʻole. ʻAʻole paha ʻoe e loaʻa i kekahi mau hōʻailona i ka wā mua.

Eia nō naʻe, ʻo ka mea weliweli loa e pili ana i kēia, ʻo ia ka hiki ke alakaʻi i ka hāʻule piha ʻana o ke akepaʻa a me ka make inā ʻaʻole i hana ʻia kahi hoʻoili akepaʻa .

Akā, ʻo ka mea pōmaikaʻi, aia nā lāʻau lapaʻau e hiki ke hoʻolohi i ka holomua o kēia maʻi . ʻAʻole nā ​​​​mea āpau e hele i kēia pae hope loa. No laila, mai hopohopo.

He aha ka ʻokoʻa ma waena o (PBC) a me (PSC)?

Ua lohe paha ʻoe i kekahi maʻi ʻē aʻe i kapa ʻia ʻo Primary Sclerosing Cholangitis, a i ʻole PSC. Ua like loa nā mea ʻelua, akā aia kekahi mau ʻokoʻa koʻikoʻi.

• I loko o PBC , ʻo nā ʻauwai bile wale nō i loko o kou ake (intrahepatic bile ducts) ka mea i hoʻopilikia ʻia.
• Eia nō naʻe, ma PSC, ua hoʻopilikia ʻia nā ʻauwai bile i loko o ke akepaʻa a ma waho o ke akepaʻa (nā ʻauwai extrahepatic) .
• Aia kekahi ʻokoʻa i waena o nā heluna kanaka kahi i loaʻa pinepine ai kēia mau maʻi ʻelua.
• ʻO ka mea nui, ʻoiai aia nā lāʻau lapaʻau e mālama ai (PBC), ʻaʻohe lāʻau lapaʻau kūleʻa i loaʻa no (PSC) .

ʻO wai ka mea i loaʻa i kēia maʻi (PBC)?

ʻOiai paha he mea kupanaha kēia, ʻoi aku ka nui o ka loaʻa ʻana o ka PBC i nā wahine ma mua o nā kāne (10:1) . Wahi a nā helu helu ma ʻAmelika Hui Pū ʻIa, ma kahi o 60 o 100,000 mau wahine i loaʻa i ka maʻi, hoʻohālikelike ʻia me kahi o 15 o 100,000 mau kāne.

Ua ʻike ʻia ka hapa nui o ka poʻe me ka maʻi ma hope o 40 mau makahiki. ʻOi aku ka maʻi ma Scotland, nā ʻāina Scandinavian, a me ka ʻākau hikina o ʻEnelani.

Eia kekahi, inā loaʻa iā ʻoe a i ʻole kekahi o kou ʻohana kekahi maʻi autoimmune ʻē aʻe, ʻoi aku ka nui o kou loaʻa ʻana o ka PBC . Hōʻike kēia he pilina genetic paha.

He aha nā hōʻailona o ka maʻi (PBC)?

ʻAʻohe hōʻailona o ka nui o ka poʻe me PBC i nā pae mua. I ka holomua ʻana o ka maʻi, hoʻomaka nā hōʻailona e pili ana i nā ʻauwai bile e ʻike ʻia. ʻO nā hōʻailona mua a maʻamau i ʻike ʻia i ka poʻe me PBC:

• Luhi nui (ma kahi o 65% o nā kānaka)
• ʻIli ʻāʻī (ma kahi o 55% o nā kānaka)

Hoʻopilikia kēia mau hōʻailona i kēlā me kēia kanaka i kekahi ʻano like ʻole. Loaʻa paha i kekahi poʻe iā lākou i ka wā mua o ka maʻi, aʻo kekahi poʻe e loaʻa iā lākou ma hope. Hiki iā lākou ke ʻoi aku ka koʻikoʻi a emi mai paha i kēlā me kēia pae o ka maʻi. ʻAʻole like me he pilina nui ma waena o ke koʻikoʻi o kāu mau hōʻailona a me ke ʻano o ka holomua o ka maʻi. Eia nō naʻe, hōʻike kekahi mau noiʻi e hiki i ka poʻe i loaʻa nā hōʻailona koʻikoʻi i ka wā mua ke loaʻa ka wikiwiki o ka holomua o ka maʻi .

He aha nā pilikia o nā maʻi o ka ʻōpū bile?

I nā pae mua, ʻaʻole paha ʻoe e ʻike he pilikia kou me kāu mau ʻauwai bile. Akā i ka holomua ʻana o ka maʻi, hoʻomaka nā pilikia like ʻole. ʻO ka bile i hiki ʻole ke kahe pono, ʻo kāuHoʻomaka ia e kahe i loko o ke koko, kahi e hoʻoulu ai i nā maʻi. Eia kekahi, ke hiki ʻole i ka bile ke hiki pono i ka ʻōnaehana hoʻoheheʻe, ua pilikia pū ka hoʻoheheʻe ʻana . No ka mea, he mea nui ka bile no ka hoʻoheheʻe ʻana i ka meaʻai.

Ma hope mai, hiki i ka ʻeha o ke akepaʻa ke hoʻopilikia i nā kīʻaha koko e hele ana ma o ke akepaʻa, e alakaʻi ana i kahi kūlana i kapa ʻia ʻo portal hypertension , ʻo ia hoʻi ke koko kiʻekiʻe i loko o kēlā mau aa koko.

1. Ka malabsorption momona

Inā ʻaʻole lawa ka bile i loko o kāu ʻōnaehana hoʻoheheʻe ʻana, ʻaʻole hiki iā ia ke wāwahi pono a omo i nā momona i loko o kāu meaʻai. Hiki i kēia emi ʻana o ka omo ʻana o nā momona ke hana i kēia mau mea:

• ʻO ke kolesterol koko kiʻekiʻe .
• Nā waihona momona ma lalo o ka ʻili .
• ʻŌpū momona a i ʻole ka maʻi pākī .
• Ka emi ʻana o ke kaumaha .
• Ua emi ka nui o nā wikamina momona-hoʻoheheʻe ʻia A, D, E, a me K.
• ʻO ka Osteoporosis kahi maʻi lahilahi o ka iwi i hoʻokumu ʻia e ka emi ʻana o ka omo ʻana o ka huaora D hiki ke hoʻoheheʻe ʻia i ka momona.

2. Ke koko kiʻekiʻe o ka puka

Ke ālai ʻia nā kīʻaha koko e hele ana ma ke akepaʻa ma muli o ka ʻeha, hiki mai ke ʻano o ka hypertension portal i ʻōlelo ʻia ma luna, ʻo ia hoʻi, piʻi ke koko i loko o kēlā mau aa koko a me nā aa koko ʻē aʻe e pili ana iā lākou. Hiki i kēia hypertension portal ke hana i kēia mau kūlana:

• Ake i hoʻonui ʻia .
• ʻŌpū nui .
• Anuanu pinepine a me nā maʻi ʻē aʻe (hoʻemi ʻia ka palekana).
• ʻO ka maʻalahi o ka ʻeha a me ke kahe ʻana o ke koko (nā platelets koko haʻahaʻa - (thrombocytopenia) ).
• ʻO ke ʻano o nā kīʻaha koko ʻulaʻula e like me ka nananana (spider angiomas) ma ka ʻili.
• Nā aa koko pehu a i ʻole nā ​​​​​​ua i nakinaki ʻia i loko o ka esophagus a me ka ʻōpū (nā aa koko pehu - varices) .
• Ke kahe koko ʻana o ka ʻōpū .
• Ka hōʻiliʻili ʻana o ka wai i loko o ka ʻōpū (ascites) .
• Ka pehu ʻana (edema) o ke kino haʻahaʻa.
• I kekahi manawa, kū mai ka huikau o ka noʻonoʻo ma muli o ka hōʻiliʻili ʻana o nā mea ʻawahia i loko o ke koko.

He aha nā kumu o (PBC)?

I loko o ka PBC, hoʻouka kāu ʻōnaehana pale ponoʻī i nā cell e hoʻopuni ana i nā ʻauwai bile o kou ake . Me he mea lā e ʻike ana ka ʻōnaehana pale i kēia mau cell me he poʻe komo hewa lā. ʻO kēia ka mea a mākou e kapa nei he maʻi autoimmune., a i ʻole nā ​​maʻi autoimmune. No ke kumu ʻike ʻole ʻia, hoʻopau ka ʻōnaehana pale i nā hunaola olakino, a ʻaʻohe mana e hoʻōki iā ia. ʻO kēia mumū e hoʻomau nei ke kumu o nā ʻeha.

ʻAʻole maopopo iā mākou ke kumu o ka ulu ʻana o kēia mau maʻi autoimmune. Eia nō naʻe, me he mea lā he kumu hoʻoilina . He nui ka poʻe i loaʻa i nā maʻi autoimmune he moʻolelo ʻohana o ka maʻi, a hiki iā lākou ke loaʻa i nā ʻano maʻi autoimmune he nui.

Me he mea lā aia kekahi kumu pili i ke kaiapuni . ʻO ia hoʻi, malia paha he mea i loko o ke kaiapuni e hoʻoulu ai i kēia maʻi. He kemika paha ia āu e hōʻike nei, a he maʻi paha.

Pehea e ʻike ʻia ai ka PBC?

E nīnau mua kāu kauka iā ʻoe e pili ana i kou mōʻaukala olakino a me nā hōʻailona, ​​​​e hana i kahi hoʻokolokolo kino, a laila e lawe i kahi hāpana koko e nānā no PBC.

ʻO ka mea nui, ʻimi kēia mau hoʻāʻo koko i nā antibodies kikoʻī e pili ana me PBC, ʻo ia hoʻi kahi antibody i kapa ʻia ʻo antimitochondrial antibody (AMA) . Ke nānā pū nei lākou i nā enzymes ate kiʻekiʻe, ʻo ia hoʻi ka alkaline phosphatase (ALP), e hōʻike ana i ka hōʻino ʻia o ke ake.

Inā maikaʻi nā hopena o kāu hoʻāʻo no PBC, e makemake ana kāu kauka e nānā i nā kiʻi o kou ake a me ka ʻōnaehana biliary . Hiki i kēia ke kōkua i ka hoʻopau ʻana i nā kumu ʻē aʻe o kāu mau hōʻailona a ʻike i ke ʻano o ka maʻi. ʻO ka maʻamau, hana mua ʻia kahi mea maʻalahi e like me ka ultrasound ʻōpū. Akā i kekahi manawa, pono paha kahi hoʻāʻo e like me ka MRI (magnetic resonance imaging) e loaʻa ai nā kiʻi maopopo.

Ma kahi o 5% o ka poʻe me PBC e loaʻa paha kahi hoʻāʻo AMA maikaʻi ʻole, akā loaʻa paha iā lākou nā hōʻailona ʻē aʻe. Inā hana ʻia kēia, pono paha kāu kauka e lawe i kahi ʻāpana liʻiliʻi o ka ʻiʻo mai kou ake (he biopsy ake) e hōʻoia i ka PBC. Hana pinepine ʻia kēia ma ka ʻaoʻao moe ma ke kau ʻana i kahi nila i loko o kou ake. Hoʻouna ʻia ka hāpana liʻiliʻi o ka ʻiʻo i lawe ʻia mai ka nila i kahi lab e nānā ʻia ma lalo o kahi microscope.

He aha nā lāʻau lapaʻau no ka Biliary Cholangitis mua?

Hoʻohana nā kauka i kēia mau mea e mālama ai iā PBC:

Lāʻau lapaʻau

ʻAʻole hiki ke ho'ōla loa ʻia ka PBC. Eia nō naʻe, hiki i nā lāʻau lapaʻau ke kāohi i ka holomua o ka maʻi a hoʻomaikaʻi i kou kūlana .

(ʻAkika Ursodeoxycholic - UDCA)He ʻano waikawa bile ia. Kōkua ia i ka wehe ʻana i ka bile mai ke akepaʻa a hōʻemi i ka hōʻino ʻana o ke akepaʻa. Hana maikaʻi kēia lāʻau lapaʻau no ka hapalua o ka poʻe me PBC, ʻoi aku hoʻi i nā pae mua o ka maʻi. No ka poʻe i pane ʻole i ka UDCA, paipai pinepine nā kauka i kahi ʻano waikawa bile ʻē aʻe i kapa ʻia ʻo obeticholic acid .

Eia kekahi, hiki ke mālama ʻia kāu mau hōʻailona pilikino me nā lāʻau lapaʻau like ʻole.

• No ka ʻili maneʻo: hiki ke hāʻawi ʻia nā antihistamines (e like me diphenhydramine - e like me Benadryl®), ultraviolet light therapy, a i ʻole nā ​​​​bile acid sequestrants e like me cholestyramine.
• E pale i nā hopena ʻaoʻao e like me ka nele o nā huaora a me ka osteoporosis: Hiki i nā mea hoʻohui huaora ke kōkua.
• Hiki i kekahi poʻe me ka luhi ke pōmaikaʻi mai nā mea hoʻoulu e like me ka modafinil.

ʻoki kino

Inā ʻaʻole e hoʻomaikaʻi kou kūlana me ka lāʻau lapaʻau, a ke hoʻomau nei ka hana o kou akepaʻa i ka emi ʻana, hiki i kāu kauka ke kau iā ʻoe ma ka papa inoa kali no kahi hoʻoili akepaʻa . Loaʻa i ka poʻe me PBC nā hopena maikaʻi loa me ke ʻoki hoʻoili akepaʻa.

E like me nā maʻi autoimmune ʻē aʻe, hiki i ka PBC ke hoʻi hou ma hope o ke kāpili ʻana o ke akepaʻa, akā ʻoi aku ka lohi o ka holomua o ka maʻi i ka lua o ka manawa. He mea maʻamau ka manaʻolana o ke ola ma hope o ke kāpili ʻana o ke akepaʻa.

He aha kāu e manaʻo ai i ka wā e noho ai me kēia maʻi?

I ka hapanui o nā hihia, holomua mālie ka Primary Biliary Cholangitis. Hiki i ka ʻike mua ʻana a me ka mālama koke ʻana ke pale a i ʻole e hoʻopaneʻe i nā pae hope o ka maʻi a me kona mau pilikia.

Hiki i ka hapa nui o ka poʻe ke kāohi maikaʻi i ko lākou mau hōʻailona me ka lāʻau lapaʻau. Eia nō naʻe, hiki ke lilo ka mālama ʻana i ka luhi e kū mai ana me PBC i mea paʻakikī. Noho ka nui o ka poʻe no nā makahiki me ka ʻole o ka hoʻopilikia nui ʻana i ko lākou ola o kēlā me kēia lā ma muli o PBC.

Eia naʻe, ʻaʻole i ʻike ʻia nā kānaka a pau me ka PBC i kona mau wā mua. A loaʻa i kekahi poʻe kahi ʻano ʻoi aku ka ikaika o ka maʻi. Hiki i nā hōʻailona e like me ka luhi nui a me nā pae bilirubin kiʻekiʻe i loko o ke koko ke hōʻike e hiki i ka maʻi ke holomua koke i ka hāʻule ʻana o ke akepaʻa. Inā hiki ʻoe i kēlā pae, pono ʻoe i kahi hoʻoili akepaʻa e ola ai. Akā no ka poʻe me PBC i loaʻa kahi hoʻoili akepaʻa holomua, maikaʻi loa ka prognosis.

ʻEhia ka lōʻihi e hiki ai iā ʻoe ke ola me (PBC)?

ʻO ka maʻamau, ma waena o 15 a me 20 mau makahiki e hiki ai i ka maʻi i ke kahua hope loa.

ʻO ke kahua mua, ʻo ia hoʻi, inā ua hoʻāʻo ʻia ʻoe a loaʻa iā ʻoe ka PBC, hiki nō iā ʻoe ke noho asymptomatic no ka manawa lōʻihi. Ma kahi o ka hapalua o ka poʻe e hoʻomaka e hoʻomohala i nā hōʻailona i loko o nā makahiki ʻelima a ʻumi e hiki mai ana.Ke loaʻa iā ʻoe nā hōʻailona, ​​​​ʻo ka awelika o ke ola ma kahi o 10 mau makahiki. 65% o ka poʻe i loaʻa kahi hoʻoili ate kūleʻa e ola ana no 10 mau makahiki.

Pehea wau e mālama ai iaʻu iho ke loaʻa iaʻu (PBC)?

ʻO ka mālama ʻana i ka ʻai a me ke ʻano o ka nohona e kōkua i ka mālama ʻana i kou akepaʻa no ka lōʻihi loa.

ʻo kahi laʻana:

• E ho'ōki i ka puhi paka, ka inu ʻona, a me ka hoʻohana ʻana i nā lāʻau lapaʻau a me nā lāʻau lapaʻau ma ke ʻano kūpono ʻole a i ʻole no ka leʻaleʻa.
• E ʻai iki i nā meaʻai i hana ʻia a me nā meaʻai i hoʻopili ʻia, a e ʻai i nā meaʻai holoʻokoʻa.
• E hōʻemi i nā momona momona a ʻai i nā momona unsaturated olakino.
• E hele wāwae wikiwiki i kēlā me kēia lā. Hiki i ka hoʻoikaika kino hāpai kaumaha ke kōkua i ka mālama ʻana i ke olakino o ka iwi.

Hiki i nā poʻe he nui me ka Primary Biliary Cholangitis ke ola no nā makahiki me ka ʻole o nā pilikia nui. Eia nō naʻe, ʻo ka loaʻa ʻana o kahi maʻi holomua, i kekahi manawa make, hiki ke hoʻopilikia iā ʻoe. E kamaʻilio me kāu kauka e pili ana i nā ala e hōʻemi ai i kou kaumaha kino, noʻonoʻo, a me ka naʻau . Eia kekahi, e nīnau e pili ana i nā pūnaewele kākoʻo e kōkua i nā poʻe e noho ana me PBC. E hoʻomanaʻo, ʻoi aku ka nui o kou olakino holoʻokoʻa ma mua o ka maʻi wale nō.

Leka Lawe-Home

ʻO ka Primary Biliary Cholangitis (PBC) kahi maʻi mau loa e hoʻopilikia ana i nā ʻauwai bile o ke ake. Ua manaʻo ʻia ua hana ʻia e kahi maʻi autoimmune . ʻOiai ʻaʻole paha e ʻike ʻia nā hōʻailona i ka wā mua, hiki ke ulu aʻe nā hōʻailona e like me ka luhi a me ka ʻili ʻili i ka hala ʻana o ka manawa. I ka holomua ʻana o ka maʻi , hiki iā ia ke alakaʻi i ka cirrhosis a me ka hāʻule ʻana o ke ake.

Eia nō naʻe, me ka ʻike mua ʻana a me ka hoʻohana ʻana i nā lāʻau lapaʻau e like me (UDCA), hiki ke kāohi ʻia ka maʻi . Pono paha kekahi poʻe i kahi hoʻoili ake.

ʻO ka mea nui loa, ʻo ia ke ʻimi i ke kōkua lapaʻau a hoʻohana i kahi nohona olakino inā loaʻa iā ʻoe kēia mau hōʻailona. ʻAʻole ʻoe hoʻokahi, mai makaʻu e kamaʻilio e pili ana i kēia a loaʻa ke kōkua.

ʻO ka Cholangitis Biliary Mua, PBC, maʻi akepaʻa, nā ʻauwai bile, cirrhosis, autoimmune, luhi, ʻulaʻula ʻili, maʻi akepaʻa, nā ʻauwai bile, cirrhosis, autoimmune