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He lāʻau lapaʻau kūikawā ka Odevixibat e kōkua ana i ka mālama ʻana i kahi maʻi ate laha ʻole i kapa ʻia ʻo progressive familial intrahepatic cholestasis (PFIC). Hana kēia lāʻau lapaʻau ma ka pale ʻana i kekahi mau mea lawe waikawa bile i loko o kou ʻōpū, hiki ke kōkua i ka hōʻemi ʻana i ka ʻūlū nui a me ka pōʻino o ke ake e hele pū ana me kēia maʻi.
Inā ua kau ʻia ʻo odevixibat iā ʻoe a i ʻole kekahi mea aloha, he mau nīnau paha kāu e pili ana i ke ʻano o kāna hana a me ka mea e manaʻo ai. He hōʻailona koʻikoʻi kēia lāʻau lapaʻau no nā ʻohana e hana ana me PFIC, e hāʻawi ana i ka manaʻolana i kahi i kaupalena ʻia ai nā koho lapaʻau.
He lāʻau lapaʻau waha ka Odevixibat i hoʻolālā ʻia e mālama i ka progressive familial intrahepatic cholestasis (PFIC). He maʻi genetic laha ʻole ka PFIC e pili ana i ke ʻano o ka hana ʻana o kou ate i nā waikawa bile, e alakaʻi ana i ka ʻūlū nui a me ka pōʻino o ke ake.
No ka papa o nā lāʻau lapaʻau i kapa ʻia ʻo ileal bile acid transporter (IBAT) inhibitors kēia lāʻau lapaʻau. E noʻonoʻo iā ia he mea pale koho e pale ana i kou ʻōpū mai ka hoʻopili hou ʻana i ka nui o ka waikawa bile, ʻo ia ke kumu kumu o nā hōʻailona PFIC.
Ua hoʻomohala ʻia ka lāʻau lapaʻau ma hope o nā makahiki o ka noiʻi ʻana i nā maʻi ate laha ʻole. Ua loaʻa iā ia ka ʻae mai ka FDA i ka makahiki 2021, e lilo ana ia i ka lāʻau lapaʻau mua i ʻae ʻia no ka mālama ʻana i ka PFIC i nā maʻi pediatric.
Hoʻohana nui ʻia ka Odevixibat e mālama i ka progressive familial intrahepatic cholestasis (PFIC) i nā maʻi i ʻekolu mahina a ʻoi aku paha. Hoʻokumu ka PFIC i nā waikawa bile e kūkulu i loko o kou ate ma mua o ka kahe maʻamau ʻana i loko o kou ʻōpū.
ʻO nā hōʻailona nui e kōkua ai kēia lāʻau lapaʻau e pili ana i ka ʻūlū nui a paʻa e hiki ke hoʻopau. Nui nā maʻi me PFIC e ʻike i ka ʻūlū nui e hoʻopilikia i ka hiamoe, ke kula, ka hana, a me nā hana i kēlā me kēia lā.
Ma waho aʻe o ka hoʻomaha ʻana i ka ʻūlū, hiki nō i ka odevixibat ke kōkua i ka hoʻolohi ʻana i ka holomua o ka pōʻino o ke ake. ʻOiai ʻaʻole ia he lāʻau lapaʻau no ka PFIC, hiki iā ia ke hoʻomaikaʻi nui i ka maikaʻi o ke ola a hiki ke hoʻolōʻihi i ka pono no ka hoʻololi ʻana i ke ake i kekahi poʻe maʻi.
Hana ʻo Odevixibat ma ka pale ʻana i kahi protein kikoʻī i kapa ʻia ʻo ka ileal bile acid transporter (IBAT) i loko o kou ʻōpū liʻiliʻi. Hoʻohana maʻamau kēia protein i nā waikawa bile i kou ake, akā i nā maʻi PFIC, kōkua kēia hana i ka hōʻiliʻili ʻana o nā waikawa bile.
Ma ka pale ʻana i kēia mea lawe, hiki i ka odevixibat ke ʻae i nā waikawa bile hou e haʻalele i kou kino ma o nā neʻe ʻana o ka ʻōpū ma mua o ka hoʻi ʻana i kou ake. Kōkua kēia i ka hōʻemi ʻana i ka nui o nā waikawa bile i loko o kou koko a me ka ʻiʻo ake.
Ua manaʻo ʻia ka lāʻau lapaʻau he mea ikaika no kāna kumu kikoʻī. ʻOiai he mea maikaʻi loa ia i ka pale ʻana i ka reabsorption waikawa bile, ua hoʻolālā ʻia e hana mālie i ka manawa ma mua o ka hāʻawi ʻana i ka hoʻomaha koke.
Pono e lawe ʻia ʻo Odevixibat e like me ke kauoha a kāu kauka, maʻamau hoʻokahi manawa i ka lā i ke kakahiaka. Hele mai ka lāʻau lapaʻau ma ke ʻano o nā capsules hiki ke ale ʻia a i ʻole e wehe ʻia a hui pū ʻia me ka meaʻai no nā maʻi ʻōpio ʻaʻole hiki ke ale i nā pila.
Pono ʻoe e lawe i ka odevixibat me ka meaʻai e kōkua i kou kino e komo pono. He lawa ka ʻaina kakahiaka māmā a i ʻole ka ʻai. ʻO ka lawe ʻana ma ka ʻōpū ʻole e hōʻemi i kona pono.
Inā pono ʻoe e wehe i ka capsule, hiki iā ʻoe ke kāpīpī i nā mea i loko ma kahi meaʻai palupalu liʻiliʻi e like me ka ʻāpala a i ʻole ka yogurt. E hōʻoia e ʻai koke i ka hui holoʻokoʻa a mai mālama i kekahi mea no ka wā e hiki mai ana.
E hoʻāʻo e lawe i kāu mahele i ka manawa like i kēlā me kēia lā e mālama i nā pae kūlike i loko o kāu ʻōnaehana. Kōkua kēia i ka lāʻau lapaʻau e hana pono.
He lāʻau lōʻihi ʻo Odevixibat no PFIC, ʻo ia hoʻi, pono paha ʻoe e lawe mau ia mea i ka wā e kōkua ana i kāu mau hōʻailona. No ka mea he maʻi genetic maʻi ʻo PFIC, ʻo ka hoʻōki ʻana i ka lāʻau lapaʻau, ʻo ia ka mea e hoʻi mai ai nā hōʻailona.
E nānā mau kāu kauka i kāu pane ʻana i ka lāʻau lapaʻau, e nānā ana i kāu mau hōʻailona a me ka hana o ke ake i kēlā me kēia mahina. ʻIke kekahi poʻe maʻi i ka hoʻomaikaʻi ʻana i ka ʻūlū i loko o kekahi mau pule, aʻo nā mea ʻē aʻe e lawe i kekahi mau mahina e ʻike i nā pōmaikaʻi piha.
Aia ka lōʻihi o ka mālama ʻana i ka maikaʻi o kāu pane ʻana i ka lāʻau lapaʻau a inā paha ʻoe e ʻike i nā hopena ʻaoʻao pilikia. E hana pū kāu hui mālama ola me ʻoe e hoʻoholo i ka hoʻolālā lōʻihi maikaʻi loa no kāu kūlana.
E like me nā lāʻau lapaʻau a pau, hiki i ka odevixibat ke hoʻoulu i nā hopena ʻaoʻao, ʻoiai he nui ka poʻe e ʻae maikaʻi iā ia. Pili ka hapa nui o nā hopena ʻaoʻao maʻamau i nā loli digestive no ka mea e pili ana ka lāʻau lapaʻau i ke ʻano o kou kino e hana ai i nā waikawa bile.
Eia nā hopena ʻaoʻao i hōʻike pinepine ʻia e ʻike ai ʻoe:
ʻO ka hapa nui o kēia mau hopena ʻaoʻao digestive e maʻalahi a i ka waena a pinepine e hoʻomaikaʻi i ka wā e hoʻoponopono ai kou kino i ka lāʻau lapaʻau i nā pule mua o ka mālama ʻana.
Hiki i kekahi poʻe maʻi ke ʻike i nā hopena ʻaoʻao koʻikoʻi akā ʻaʻole maʻamau. Hiki i kēia mau mea ke komo i ka maʻi maʻi nui e alakaʻi ai i ka dehydration, ʻeha ʻōpū nui, a i ʻole nā hōʻailona o nā pilikia o ke ake e like me ka melemele o ka ʻili a i ʻole nā maka.
ʻO nā hopena ʻaoʻao kakaʻikahi akā koʻikoʻi paha e komo i nā hopena maʻi maʻi koʻikoʻi, ʻoiai ʻaʻole maʻamau kēia mau mea. ʻO nā hōʻailona o kahi hopena maʻi maʻi koʻikoʻi e komo i ka pilikia o ka hanu ʻana, ka pehu ʻana o ka maka a i ʻole ka ʻāʻī, a i ʻole ka ʻili ʻili nui.
E kāhea koke i kāu kauka inā loaʻa iā ʻoe ka luaʻi mau, ka maʻi maʻi nui, nā hōʻailona o ka maloʻo ʻana o ke kino, a i ʻole nā hōʻailona e hopohopo ai ʻoe.
ʻAʻole kūpono ʻo Odevixibat no nā mea a pau, ʻoiai ʻo ka poʻe me PFIC. E loiloi pono kāu kauka inā kūpono kēia lāʻau lapaʻau no kāu kūlana kikoʻī ma mua o ke kau ʻana.
ʻAʻole pono ʻoe e lawe iā odevixibat inā ʻike ʻoe i ka maʻi maʻi i ka lāʻau lapaʻau a i ʻole kekahi o kāna meaʻai. ʻAʻole paha he moho maikaʻi nā kānaka me kekahi mau ʻano maʻi ate ʻē aʻe ma mua o PFIC no kēia lapaʻau.
Pono ka lāʻau lapaʻau i ka noʻonoʻo pono ʻana i nā maʻi me ka maʻi kīkī koʻikoʻi, no ka mea, pili ka hana kīkī i ke ʻano o ka hana ʻana o kou kino i ka lāʻau. Pono paha kāu kauka e hoʻoponopono i kāu mahele a i ʻole e nānā pono iā ʻoe inā he pilikia kou kīkī.
Pono nā wahine hāpai a hānai paha e kūkākūkā i nā pilikia a me nā pōmaikaʻi me kā lākou mea mālama ola. ʻOiai ua kaupalena ʻia nā haʻawina i nā wahine hāpai, pono paha ka lāʻau lapaʻau inā ʻoi aku ka maikaʻi ma mua o nā pilikia.
ʻAʻole pono nā keiki ma lalo o ʻekolu mahina e loaʻa iā odevixibat, no ka mea, ʻaʻole i hoʻokumu ʻia ka palekana a me ka pono i kēia pūʻulu makahiki ʻōpio.
Kūʻai ʻia ʻo Odevixibat ma lalo o ka inoa inoa ʻo Bylvay ma ʻAmelika Hui Pū ʻIa a me nā ʻāina ʻē aʻe. Hana ʻia ʻo Bylvay e Albireo Pharma a ʻo ia wale nō ke ʻano o ka odevixibat i loaʻa ma ke kālepa.
Loaʻa ka lāʻau lapaʻau i nā ikaika capsule like ʻole e hoʻokō i nā pono dosing like ʻole, ʻoi aku ka mea nui no ka mea e hoʻohana ʻia ana i nā keiki a me nā mākua. E hāʻawi kāu hale kūʻai lāʻau i ka ikaika kikoʻī i kauoha ʻia e kāu kauka.
No ka mea he lāʻau lapaʻau kūikawā kēia no kahi maʻi laha, ʻaʻole paha e loaʻa ʻo Bylvay ma nā hale kūʻai lāʻau lapaʻau a pau. Hiki i kāu kauka a i ʻole ka mea kūʻai lāʻau lapaʻau ke kōkua e hoʻonohonoho iā ʻoe e loaʻa i ka lāʻau lapaʻau ma o nā lawelawe hale kūʻai lāʻau kūikawā inā pono.
He palena nā koho lapaʻau no PFIC, ʻo ia ke kumu e hōʻike ai ʻo odevixibat i kahi holomua nui. Ma mua o ka loaʻa ʻana o kēia lāʻau, ua kālele ka lapaʻau i ka mālama ʻana i nā hōʻailona a me nā pilikia.
ʻO nā lapaʻau kuʻuna e hiki i nā kauka ke hoʻohana pū a i ʻole ma kahi o odevixibat, e komo pū ana me nā mea hoʻopaʻa waikawa bile e like me cholestyramine. Hana ʻokoʻa kēia mau lāʻau lapaʻau ma ka hoʻopaʻa ʻana i nā waikawa bile i loko o kou ʻōpū, akā ʻaʻole pinepine lākou i ka maikaʻi a paʻakikī ke ʻae.
No nā hihia koʻikoʻi ʻaʻole e pane i ka lapaʻau lapaʻau, ʻo ka hoʻololi ʻana i ke ake ka koho lapaʻau maopopo. Eia naʻe, hiki i ka odevixibat ke kōkua i ka hoʻopaneʻe ʻana i ka pono no ka hoʻololi ʻana i kekahi mau maʻi.
Pōmaikaʻi kekahi mau maʻi mai nā lapaʻau kākoʻo e like me nā antihistamines no ka ʻūlū, nā mea hoʻohui meaʻai no nā huaora hiki ke hoʻoheheʻe ʻia i ka momona, a me ka nānā pono ʻana i ka hana o ke ake. Hoʻoponopono kēia mau lapaʻau i nā hōʻailona akā ʻaʻole i ka pahuhopu kumu e like me ka odevixibat.
Hana ʻo Odevixibat a me cholestyramine ma o nā ʻano hana like ʻole, e paʻakikī ana i nā hoʻohālikelike pololei. Eia naʻe, hōʻike nā haʻawina lapaʻau e ʻoi aku ka maikaʻi o odevixibat no nā maʻi PFIC he nui.
Pono ʻo Cholestyramine i nā lāʻau he nui i kēlā me kēia lā a paʻakikī ke lawe, ʻoiai no nā keiki. Hoʻoulu pinepine ia i ka paʻa ʻana a hiki ke hoʻopilikia i ka lawe ʻana i nā lāʻau lapaʻau a me nā meaʻai ʻē aʻe.
Hāʻawi ʻo Odevixibat i ka maʻalahi o ka hoʻohana ʻana i hoʻokahi manawa i ka lā a ʻoi aku ka maikaʻi o ka ʻae ʻia e ka hapa nui o nā maʻi. Ua hōʻike nā hoʻokolohua lapaʻau he ʻoi aku ka maikaʻi ma mua o ka placebo i ka hōʻemi ʻana i ka ʻūlū i nā maʻi PFIC.
E noʻonoʻo kāu kauka i nā mea e like me kou makahiki, nā hōʻailona, nā lāʻau lapaʻau ʻē aʻe, a me ka maikaʻi o kāu pane ʻana i nā lapaʻau mua i ka wā e hoʻoholo ai ma waena o kēia mau koho. Hiki i kekahi mau maʻi ke hoʻohana i nā lāʻau lapaʻau ʻelua inā pono.
ʻAe, ua ʻae ʻia ka odevixibat no ka hoʻohana ʻana i nā keiki mai ʻekolu mahina. Ua aʻo kūikawā ʻia ka lāʻau lapaʻau i nā maʻi pediatric no ka mea e pili pinepine ana ka PFIC i nā keiki.
Ua komo nā hoʻokolohua lapaʻau i nā maʻi mai nā pēpē a i nā mākua, me ka nānā pono ʻana i ka hoʻohana ʻana a me ka palekana i nā pūʻulu makahiki ʻōpio. Ua like ke ʻano o nā hopena ʻaoʻao ma nā pūʻulu makahiki like ʻole, akā ʻoi aku paha ka naʻau o nā keiki i nā hopena digestive.
E helu ke kauka o kāu keiki i ka mahele kūpono e pili ana i ko lākou kaumaha a e nānā pono iā lākou no ka pono a me nā hopena ʻaoʻao. He mea nui nā manawa e hahai ai i ka maʻamau e hōʻoia i ka palekana a me ke kōkua ʻana o ka lāʻau lapaʻau.
Inā ua lawe hewa ʻoe i ka nui o ka odevixibat ma mua o ka mea i kauoha ʻia, e hoʻokaʻaʻike koke i kāu kauka a i ʻole ke kikowaena mana ʻawa. ʻO ka lawe ʻana i ka nui hiki ke hoʻonui i kou pilikia o nā hopena ʻaoʻao koʻikoʻi, ʻo ia hoʻi nā pilikia digestive.
Hiki i ka overdose ke kumu i ka maʻi maʻi koʻikoʻi, ka dehydration, nā kaulike electrolyte, a i ʻole ka ʻeha o ka ʻōpū. Hiki ke koʻikoʻi kēia mau hopena, ʻoiai i nā keiki a i ʻole nā kānaka me nā maʻi olakino ʻē aʻe.
Mai hoʻāʻo e hoʻōki iā ʻoe iho ke ʻole i kuhikuhi ʻia e kahi loea mālama ola. E mālama i ka hue lāʻau me ʻoe i ka wā e ʻimi ai i ka lāʻau lapaʻau i ʻike pono nā mea mālama ola i ka mea a me ka nui i lawe ʻia.
Inā ua hala ʻoe i kahi mahele o ka odevixibat, e lawe koke iā ia ke hoʻomanaʻo ʻoe, inā ʻaʻole kokoke i ka manawa no kāu mahele aʻe. Inā kokoke i ka manawa no kāu mahele i hoʻonohonoho ʻia, e lele i ka mahele i hala a e hoʻomau me kāu papa manawa maʻamau.
Mai lawe i ʻelua mahele i ka manawa hoʻokahi e hoʻoponopono ai i kahi mahele i hala, no ka mea, hiki i kēia ke hoʻonui i kou pilikia o nā hopena ʻaoʻao. ʻAʻole e hāʻawi ka lawe ʻana i nā mahele pālua i ka pōmaikaʻi hou a hiki ke hōʻino.
Inā poina pinepine ʻoe i nā lāʻau, e hoʻāʻo e hoʻonohonoho i ka puʻupuʻu i kēlā me kēia lā a i ʻole e hoʻohana i ka mea hoʻonohonoho pila e kōkua iā ʻoe e hoʻomanaʻo. ʻO ka lawe ʻana i ka lāʻau i kēlā me kēia lā e kōkua i ka mālama ʻana i nā pae paʻa o ka lāʻau i loko o kou kino.
Pono ʻoe e hooki i ka lawe ʻana iā odevixibat ma lalo o ke alakaʻi a kāu kauka. No ka mea he maʻi maʻi ʻo PFIC, ʻo ka hooki ʻana i ka lāʻau lapaʻau, ʻo ia hoʻi, e hoʻi hou ana kāu mau hōʻailona.
Hiki i kāu kauka ke ʻōlelo e hooki a hoʻololi paha i kāu lapaʻau inā loaʻa iā ʻoe nā hopena ʻaoʻao koʻikoʻi, inā ʻaʻole e hana pono ka lāʻau lapaʻau, a i ʻole inā loli nui kou kūlana.
Ma mua o ka hana ʻana i nā loli i kāu hoʻolālā lapaʻau, e kūkākūkā i kāu mau hopohopo me kāu hui mālama ola. Hiki iā lākou ke kōkua iā ʻoe e kaupaona i nā pōmaikaʻi a me nā pilikia o ka hoʻomau ʻana i ka lawe ʻana i ka lāʻau.
Hiki i ka Odevixibat ke launa pū me kekahi mau lāʻau lapaʻau, no laila he mea nui e haʻi i kāu kauka e pili ana i nā lāʻau lapaʻau a pau, nā mea hoʻohui, a me nā huahana lāʻau lapaʻau āu e lawe nei. Aia kēia i nā lāʻau lapaʻau i kuhikuhi ʻia, nā lāʻau lapaʻau ma luna o ka pāʻālua, a me nā huaora.
Hiki i ka lāʻau lapaʻau ke hoʻopilikia i ke ʻano o kou kino e komo ai i nā huaora hiki ke hoʻoheheʻe ʻia i ka momona (A, D, E, a me K), no laila e ʻōlelo aku kāu kauka i nā mea hoʻohui huaora a i ʻole e nānā pono i kāu mau pae.
ʻO kekahi mau lāʻau lapaʻau i komo i loko o ka ʻāpana like o kou ʻōpū me odevixibat e loaʻa paha ka hopena i hoʻololi ʻia. Pono paha kāu kauka e hoʻoponopono i ka manawa a i ʻole ke ana ʻana o nā lāʻau ʻē aʻe e pale ai i nā pilina.