He aha te Odevixibat: Ngā Whakamahinga, te Inenga, ngā Pānga taha me ētahi atu

He rongoā motuhake te Odevixibat e āwhina ana ki te whakahaere i tētahi āhua ate onge e kīia nei ko te progressive familial intrahepatic cholestasis (PFIC). E mahi ana tēnei rongoā whakahau mā te aukati i ētahi kaikawe waikawa pūkohu i roto i ō whekau, ā, ka āwhina tēnei ki te whakaiti i te mōmona me te kino o te ate e pā ana ki tēnei āhua.

Mēnā i whakahaua te odevixibat ki a koe, ki tētahi rānei e arohaina ana e koe, he pātai pea āu mō te āhua o tana mahi me ngā mea e tūmanakohia ana. He tohu nui tēnei rongoā mō ngā whānau e pā ana ki te PFIC, e tuku ana i te tūmanako i ngā wā i iti noa iho ngā kōwhiringa maimoatanga.

He aha te Odevixibat?

He rongoā waha te Odevixibat i hangaia motuhake hei rongoā i te progressive familial intrahepatic cholestasis (PFIC). He mate ira onge te PFIC e pā ana ki te āhua o te tukatuka o tō ate i ngā waikawa pūkohu, e arahi ana ki te mōmona nui me te kino haere o te ate.

E pā ana tēnei rongoā ki tētahi rōpū rongoā e kīia nei ko te ileal bile acid transporter (IBAT) inhibitors. Whakaarohia he aukati whiriwhiri e ārai ana i ō whekau mai i te whakauru anō i te nui rawa o te waikawa pūkohu, koinei te take pūtake o ngā tohumate PFIC.

I whakawhanakehia te rongoā i muri i ngā tau o te rangahau i ngā mate ate onge. I whakawhiwhia ki te whakaaetanga mai i te FDA i te tau 2021, koinei te rongoā tuatahi i whakaaetia motuhake mō te rongoā i te PFIC i ngā tūroro tamariki.

He aha te Odevixibat e whakamahia ana mō?

Ko te Odevixibat te mea nui e whakamahia ana hei rongoā i te progressive familial intrahepatic cholestasis (PFIC) i ngā tūroro e toru marama te pakeke, neke atu rānei. Ka pā te PFIC ki te hanga waikawa pūkohu i tō ate, kaua ki te rere noa ki ō whekau.

Ko ngā tohumate matua e āwhina ana tēnei rongoā ki te whakatika ko te mōmona nui, te mōmona tonu e taea ai te whakapōuri. He maha ngā tūroro e pā ana ki te PFIC e mōmona ana i te kaha e whakararu ana i te moe, te kura, te mahi, me ngā mahi o ia rā.

I tua atu i te whakaiti i te mānukanuka, ka āwhina pea te odevixibat ki te whakaroa i te pūmautanga o te pōrearea ate. Ahakoa ehara i te rongoā mō te PFIC, ka tino pai ake te kounga o te oranga, ā, ka whakaroa pea te hiahia mō te whakawhitinga ate i ētahi tāngata.

Me pēhea te mahi a Odevixibat?

Ka mahi a Odevixibat mā te aukati i tētahi pūmua motuhake e kīia ana ko te kaikawe waikawa pūtaiao (IBAT) i roto i tō whekau iti. Ko tēnei pūmua te tikanga ka hoki anō ngā waikawa pūtaiao ki tō ate, engari i roto i ngā tāngata PFIC, ka whai wāhi tēnei tukanga ki te hanga waikawa pūtaiao.

Mā te aukati i tēnei kaikawe, ka tuku a odevixibat i ētahi atu waikawa pūtaiao ki te wehe i tō tinana mā ngā nekehanga whekau, kaua e hoki ki tō ate. Ka āwhina tēnei ki te whakaiti i te kukume o ngā waikawa pūtaiao i roto i tō toto me te kiko ate.

E whakaarohia ana he kaha te rongoā mō tōna kaupapa motuhake. Ahakoa he tino whai hua ki te aukati i te whakauru anō i ngā waikawa pūtaiao, i hangaia kia mahi haere i te wā, kaua e whai wāhi tonu.

Me pēhea taku tango i te Odevixibat?

Me tango te Odevixibat i te tika i te mea i tohutohutia e tō tākuta, te tikanga kotahi i te rā i te ata. Ka tae mai te rongoā hei pūtau ka taea te horomia katoa, te whakatuwherahia rānei, ka uru ki te kai mō ngā tāngata tamariki kāore e taea te horomia ngā pire.

Me tango e koe te odevixibat me te kai hei āwhina i tō tinana ki te ngongo tika. He parakuihi māmā, he paramanawa rānei te nuinga e tika ana. Ko te tango i runga i te puku kau ka whakaiti i tōna whai huatanga.

Mena me whakatuwhera e koe te pūtau, ka taea e koe te tauhiuhi i ngā mea kei roto i te iti o te kai ngawari pēnei i te aporo, te miraka pē rānei. Me mōhio ki te kai tonu i te ranunga katoa, kaua e tiakina ētahi mō muri mai.

Whakamātauria ki te tango i tō horopeta i te wā kotahi i ia rā kia mau tonu ngā taumata i roto i tō pūnaha. Ka āwhina tēnei i te rongoā ki te mahi tino whai hua.

Kia pehea te roa o taku tango i te Odevixibat?

He rongoā wā-roa te Odevixibat mō te PFIC, te tikanga me tango tonu koe i te rongoā i te wā e whai hua ana ki ō tohu. I te mea he mate ira tūturu te PFIC, ko te whakamutu i te rongoā te tikanga ka hoki mai ngā tohu.

Ka aroturukihia e tō tākuta tō urupare ki te rongoā i ngā wā katoa, te tikanga ka tirohia ō tohu me te mahi ate i ia marama. Ko ētahi tāngata ka kite i te whakapai ake i te mōmona i roto i ngā wiki torutoru, engari ko ētahi atu ka roa pea te marama kia kite i ngā painga katoa.

Ko te roa o te rongoā kei runga i te pai o tō urupare ki te rongoā me te kore rānei e pā ki a koe ētahi pānga kino. Ka mahi tahi tō rōpū hauora ki a koe ki te whakatau i te mahere wā-roa pai mō tō āhuatanga motuhake.

He aha ngā pānga kino o te Odevixibat?

Pērā i ngā rongoā katoa, ka taea e te odevixibat te pānga kino, ahakoa he maha ngā tāngata e pai ana ki te tango. Ko ngā pānga kino noa e pā ana ki ngā huringa kūnatu i te mea e pā ana te rongoā ki te āhua o te tukatuka o tō tinana i ngā waikawa pūpū.

Koinei ngā pānga kino e pūrongotia ana e te nuinga ka pā ki a koe:

• Te korere, te tūtae rānei
• Te mamae puku, te kōpū rānei
• Te whakapairuaki
• Te ruaki
• Te ngenge
• Te iti o te hiahia

Ko te nuinga o ēnei pānga kino kūnatu he ngawari ki te āhua, ā, he maha ngā wā ka pai ake i te wā e urutau ana tō tinana ki te rongoā i ngā wiki tuatahi o te rongoā.

Ka pā pea ki ētahi tāngata ngā pānga kino nui engari he iti noa. Kei roto i ēnei ko te korere nui e arahi ana ki te whakawātea wai, te mamae puku nui, ngā tohu rānei o ngā raruraru ate pērā i te kōwhai o te kiri, o ngā kanohi rānei.

Ko ngā pānga kino onge engari nui pea ko ngā urupare pāwera nui, ahakoa he mea onge ēnei. Ko ngā tohu o te urupare pāwera nui ko te uaua ki te hā, te pōro o te mata, o te korokoro rānei, te pupuhi kiri nui rānei.

Whakapā atu ki tō tākuta tonu mēnā ka pā koe i te ruaki tonu, te korere nui, ngā tohu o te maroke, ētahi tohumate rānei e āwangawanga ana koe.

Ko wai kāore e tika kia tango Odevixibat?

Kāore a Odevixibat e tika ana mō te katoa, tae atu ki te hunga whai PFIC. Ka āta arotake tō tākuta mēnā he tika tēnei rongoā mō tō āhuatanga motuhake i mua i te whakatakoto.

Kāore koe e tika kia tango odevixibat mēnā he mōhiotia tō mate pāwera ki te rongoā, ki ētahi o ōna kai. Ko te hunga whai momo mate ate kē atu i te PFIC, kāore pea e tika ana mō tēnei rongoā.

Me āta whakaaro te rongoā mō ngā tūroro whai mate tākihi nui, nā te mea ka pā te mahi tākihi ki te āhua o te tinana e tukatuka ana i te rongoā. Me whakatika pea e tō tākuta tō horopeta, me mātakitaki rānei i a koe i te tata atu mēnā he raruraru tākihi ōu.

Me kōrero ngā wāhine hapū, ngā wāhine whāngai ū rānei i ngā tūponotanga me ngā painga ki tō rātou kaiwhakarato hauora. Ahakoa he iti ngā rangahau i ngā wāhine hapū, me tika pea te rongoā mēnā ka nui ake ngā painga i ngā tūponotanga.

Kāore ngā tamariki kei raro i te toru marama te pakeke e tika kia whiwhi odevixibat, nā te mea kāore anō i whakatūria te haumaru me te whai hua i tēnei rōpū tau tamariki rawa.

Ingoa Waitohu Odevixibat

Ka hokona a Odevixibat i raro i te ingoa waitohu Bylvay i te United States me ētahi atu whenua. Ko Bylvay te mea i hangaia e Albireo Pharma, ā, koinei anake te āhua o te odevixibat e wātea ana i te hokohoko.

E wātea ana te rongoā i ngā kaha pūtau rerekē hei whakatutuki i ngā hiahia horopeta rerekē, he mea nui rawa atu nā te mea e whakamahia ana i ngā tamariki me ngā pakeke. Ka tukuna e tō whare rongoā te kaha motuhake i whakatakotohia e tō tākuta.

Nā te mea he rongoā motuhake tēnei mō te āhuatanga onge, kāore pea a Bylvay e wātea ana i ngā whare rongoā katoa. Ka āwhina tō tākuta, tō kai rongoā rānei i a koe ki te tiki i te rongoā mā ngā ratonga whare rongoā motuhake mēnā e tika ana.

Ngā Kōwhiringa kē mō Odevixibat

He iti noa ngā kōwhiringa maimoatanga mō te PFIC, koinei te take he mea nui te odevixibat. I mua i te wātea mai o tēnei rongoā, i te tuatahi ka arotahi te maimoatanga ki te whakahaere i ngā tohumate me ngā raruraru.

Ko ngā maimoatanga tuku iho ka whakamahia tonutia e ngā tākuta i te taha, i te wāhi rānei o te odevixibat ko ngā mea e here ana i te waikawa pūkohu pērā i te cholestyramine. He rerekē te mahi a ēnei rongoā mā te here i ngā waikawa pūkohu i roto i ō whekau, engari he iti ake te whai hua, ā, he uaua ake te aro ki a rātou.

Mō ngā keehi nui kāore e aro ki te maimoatanga rongoā, ko te whakawhitinga ate tonu te kōwhiringa maimoatanga tino pai. Heoi, ka āwhina pea te odevixibat ki te whakaroa i te hiahia mō te whakawhitinga i ētahi tūroro.

Ka whai hua ētahi tūroro mai i ngā maimoatanga tautoko pērā i ngā antihistamines mō te mōmiti, ngā tāpiringa kai mō ngā huaora wairewa ngako, me te aroturuki tupato i te mahi ate. E whakatika ana ēnei maimoatanga i ngā tohumate engari kāore i te aro ki te take i raro i te mata pērā i te odevixibat.

He Pai Ake Te Odevixibat I Te Cholestyramine?

He rerekē ngā tikanga mahi a te Odevixibat me te cholestyramine, e uaua ana te whakataurite tika. Heoi, e whakaatu ana ngā rangahau haumanu he pai ake pea te odevixibat mō te maha o ngā tūroro PFIC.

E hiahia ana te Cholestyramine kia maha ngā horopeta i ia rā, ā, he uaua pea te tango, ina koa mō ngā tamariki. He maha ngā wā ka pā mai te pāpāpā, ā, ka raru pea te urunga o ētahi atu rongoā me ngā matūkai.

E whakarato ana te Odevixibat i te wātea o te horopeta kotahi i ia rā, ā, he pai ake te aro ki a ia e te nuinga o ngā tūroro. I whakaatu ngā whakamātau haumanu he pai ake i te placebo i te whakaiti i te mōmiti i roto i ngā tūroro PFIC.

Ka whakaaro tō tākuta ki ngā āhuatanga pērā i tō tau, ō tohumate, ētahi atu rongoā, me te pai o tō urupare ki ngā maimoatanga o mua i te whakatau i waenga i ēnei kōwhiringa. Ka whakamahi pea ētahi tūroro i ngā rongoā e rua i te taha tahi ki te hiahiatia.

Ngā Pātai Auau Mō Te Odevixibat

He Haumaru Te Odevixibat Mō Ngā Tamariki?

Ae, kua whakaaetia te odevixibat hei whakamahi i roto i nga tamariki e toru marama te pakeke. I akohia te rongoa i roto i nga turoro tamariki mai i te mea he maha nga wa ka pa te PFIC ki nga tamariki.

I uru nga whakamatautau haumanu ki nga turoro mai i nga kohungahunga ki nga pakeke, me te aro nui ki te horopeta me te haumaru i roto i nga roopu reanga iti. He rite tonu te ahua o nga paanga taha puta noa i nga roopu reanga, ahakoa he nui ake te tairongo o nga tamariki ki nga paanga o te nakunaku.

Ka tatau to taakuta tamaiti i te horopeta tika i runga i to ratou taumaha, ka ata tirotiro i a ratou mo te whai hua me nga paanga taha. He mea nui nga wa whakarite whai muri kia mohio kei te haumaru tonu te rongoa me te awhina.

Me aha ahau ki te whakamahi pohehe au i te nui rawa o te Odevixibat?

Mena ka nui ake te odevixibat i te mea i tohua, whakapā atu ki to taakuta, ki te pokapū whakahaere paihana ranei i te wa tonu. Ko te nui rawa o te tango ka piki ake to tupono ki nga paanga taha kino, ina koa nga raruraru o te nakunaku.

Ka puta pea te nui o te horopeta i te korere kino, te matewai, te kore taurite o te hiko, te mamae o te puku ranei. He kino pea enei paanga, ina koa ki nga tamariki, ki nga tangata ranei e pa ana ki etahi atu mate hauora.

Kaua e ngana ki te ruaki i a koe ano ki te kore e tohutohuhia e te tohunga hauora. Kia mau ki te pounamu rongoa i a koe e rapu ana i te rongoa kia mohio ai nga kaiwhakarato hauora ki te aha me te nui i tangohia.

Me aha ahau ki te ngaro ahau i te horopeta o te Odevixibat?

Mena ka ngaro koe i te horopeta o te odevixibat, tangohia i te wa e mahara ana koe, mena kaore i te tata ki te wa mo to horopeta e whai ake nei. Mena kua tata te wa mo to horopeta kua whakaritea e whai ake nei, waiho te horopeta i ngaro, ka haere tonu me to waarangi auau.

Kaua e tango i nga horopeta e rua i te wa kotahi hei utu mo te horopeta i ngaro, na te mea ka piki ake to tupono ki nga paanga taha. Ko te tango i nga horopeta takirua e kore e whai hua atu, ka kino pea.

Mēnā he maha ō wā e wareware ana ki ngā horopeta, ngana ki te whakatū i tētahi pūoho ia rā, ki te whakamahi rānei i tētahi kaiwhakarite pire hei āwhina i a koe ki te mahara. Ko te horopeta i ia rā ka āwhina i te pupuri i ngā taumata pūmau o te rongoā i roto i tō pūnaha.

Āhea Ānei Ka Taea e Au te Kati te Tango Odevixibat?

Me kati anake te tango odevixibat i raro i te ārahitanga a tō tākuta. I te mea he āhuatanga mau tonu te PFIC, ko te kati i te rongoā te tikanga ka hoki mai ō tohumate.

Ka tūtohu pea tō tākuta kia kati, kia huri rānei i tō maimoatanga mēnā ka pā ki a koe ngā pānga taha kino, mēnā ka mutu te mahi whai hua o te rongoā, mēnā rānei ka tino huri tō āhuatanga.

I mua i te whakatutuki i ngā huringa ki tō mahere maimoatanga, kōrerohia ō āwangawanga ki tō rōpū hauora. Ka taea e rātou te āwhina i a koe ki te whakatau i ngā painga me ngā tūponotanga o te haere tonu ki te kati rānei i te rongoā.

Ka Taea e Au te Tango Odevixibat me Ētahi Atu Rongoā?

Ka taea e Odevixibat te pā ki ētahi rongoā, nō reira he mea nui kia kōrero ki tō tākuta mō ngā rongoā katoa, ngā tāpiringa, me ngā hua otaota e tango ana koe. Kei roto i tēnei ngā rongoā whakahau, ngā rongoā kore-whakahau, me ngā huaora.

Ka pā pea te rongoā ki te āhua o te tango a tō tinana i ngā huaora wairewa ngako (A, D, E, me K), nō reira ka tūtohu pea tō tākuta i ngā tāpiringa huaora, i te aroturuki rānei i ō taumata kia tata ake.

Ko ētahi rongoā e uru ana ki te wāhi kotahi o tō whekau me odevixibat ka huri pea te whai huatanga. Me whakatika pea tō tākuta i te wā, i te horopeta rānei o ētahi atu rongoā hei karo i ngā pānga.