He aha te Primary Sclerosing Cholangitis? Tohutohu, Take, me te Maimoatanga

Ko te primary sclerosing cholangitis (PSC) he mate ate roa e pā ana ki te mumura me te toka o ngā ngongo wai-mate i roto me waho o tō ate. Ko ēnei ngongo te tikanga ka kawe i te wai-mate mai i tō ate ki tō whekau iti hei awhina i te whakahaere i ngā ngako, engari ka whakaraweke haere te PSC i a rātou i te wā.

Whakaarohia ō ngongo wai-mate hei whatunga o ngā paipa e tukuna ana te wai-mate mai i tō ate. Ka puta te PSC, ka mumura ēnei paipa, ka toka, ka whakaitihia, ka uaua ai te rere tika o te wai-mate. Ka taea e tēnei wai-mate kī te whakaraweke i tō ate ka arahi ki ngā raruraru nui mena kāore i te maimoatia.

He aha ngā tohu o te Primary Sclerosing Cholangitis?

He tokomaha ngā tāngata e pangia ana e te PSC kāore e kite i ngā tohu i ngā wā tuatahi, koia te take i ngaro ai te mate mō ngā tau. Ka puta mai ngā tohu, ka tupu haeretia te whakaraweke o ngā ngongo wai-mate.

Ko ngā tohu tino noa ka kite koe:

• Te ngenge tonu e kore e pai ake me te okioki
• Te kiri ngawari (pruritus) ka kaha me te whānuitanga
• Te kowhai o tō kiri me ō kanohi (jaundice)
• Te mamae o te kopu, ina koa i te taha matau o runga
• Te mimi pouri me ngā paru maama
• Te ngaro o te taumaha kāore i te whakamārama
• Te kirika me te wiri i ngā wā o te mate

Ka tino raruraru te ngawari, ka kaha ake i te pō. Ka whakaatu etahi i te ahua kaore e taea te miri kia tae atu ki te whakaora. Ka puta tenei nā te hanga o ngā tote wai-mate ki tō kiri ina kore e taea te rere tika o te wai-mate.

I te whakahaere o te PSC, ka kite pea koe i ngā tohu e pā ana ki ngā raruraru ate, pēnei i te hanga wai i roto i tō kopu, te whakapohehe, te ngawari o te whākū me te toto.

He aha ngā momo Primary Sclerosing Cholangitis?

Ko te PSC te tikanga ka wehea ki ētahi momo e rua e whakaatu ana i ngā ngongo wai-mate e pā ana. Ko te mārama ki ēnei momo ka awhina i tō tākuta ki te whakatau i te huarahi maimoatanga pai me te kī he aha te ahua o te mate.

Ko te PSC ngongo nui te pānga ki ngā ngongo wai-mate nui ka kitea i ngā whakamātautau whakaahua pēnei i te MRCP (magnetic resonance cholangiopancreatography). Koinei te momo tino noa, e 90% pea o ngā keehi. Ko ngā tāngata e pangia ana e te PSC ngongo nui te tikanga he ahua tino pai o ngā ngongo wai-mate kī me te whānui e rite ana ki ngā “piriti i runga i te aho” i runga i te whakaahua.

Ko te PSC ngongo iti te pānga ki ngā ngongo wai-mate iti i roto i te ate kaore e kitea i ngā whakamātautau whakaahua noa. Ka taea te whakatau i tēnei momo mā te tātaritanga ate, ka tupu haere noa atu i te PSC ngongo nui. Heoi, ko etahi o ngā tāngata e pangia ana e te PSC ngongo iti ka puta mai ngā huringa i ō rātou ngongo nui.

He momo onge ano te PSC me te autoimmune hepatitis overlap syndrome, kei reira ngā āhuatanga o ngā mate e rua. Ko tēnei whakakotahitanga e hiahia ana ki ngā huarahi maimoatanga motuhake e whakautu ana ki ngā mate e rua.

He aha te take o te Primary Sclerosing Cholangitis?

Ko te take tika o te PSC kāore ano kia mohiotia, engari e whakapono ana ngā kairangahau he hua mai i te whakakotahitanga o te whakawhānuitanga ira me ngā whakaoho taiao. E kite ana tō pūnaha mate i te whakaeke i ō ngongo wai-mate ake, e pā ana ki te mumura me te toka e tohu ana i tēnei mate.

He maha ngā take ka āwhina ki te whanaketanga o te PSC:

• Ngā take ira - etahi ira ka nui ake te pānga ki a koe
• Te mahi kore o te autoimmune - ka whakaeke tō pūnaha mate i ngā kiko hauora
• Ngā mate huaketo i roto i ngā ngongo wai-mate
• Ngā mea pokanoa e whakaraweke ana i ngā pūtau ngongo wai-mate
• Te hanganga wai-mate koretake
• Ngā raruraru oko toto e pā ana ki te whānuitanga toto o te ngongo wai-mate

Ko te hononga kaha rawa atu ki te mate puku mumura, ina koa te ulcerative colitis. E 70-80% pea o ngā tāngata e pangia ana e te PSC he IBD ano, ahakoa kāore i te tino mārama te hononga i waenga i ēnei mate. Ko te whai IBD kāore e tikanga ka whānau koe i te PSC, engari ka nui ake tō tūponotanga.

Ko etahi take onge o te sclerosing cholangitis tuarua ka rite ki te PSC, tae atu ki ētahi rongoā, ngā mate huaketo, te whara ngongo wai-mate mai i te pokanga. Heoi, ko te primary sclerosing cholangitis pono ka whānau mai kāore he take o waho e mohiotia ana.

Nōhea te haere ki te tākuta mō te Primary Sclerosing Cholangitis?

Me whakapiri atu koe ki tō kaiwhakarato hauora mena ka kite koe i ngā tohu tonu e taea ai te tohu i ngā raruraru ate. Ko te kite wawe me te maimoatanga ka awhina i te whakaheke i te whanaketanga o te mate me te aukati i ngā raruraru.

Rapua te tiaki hauora tere mena ka kite koe:

• Te kowhai o tō kiri, te ma o ō kanohi
• Te ngawari nui, tonu, kāore he take kiri mārama
• Te mimi pouri me ngā paru maama
• Te ngenge tonu e āwangawanga ana i ngā mahi o ia rā
• Te mamae o te kopu tonu, ina koa i te taha matau o runga
• Te ngaro o te taumaha kāore i te whakamārama

Mena kei a koe te mate puku mumura, he mea nui te aroturuki tonu mō te PSC nā te mea ka puta tahi ngā mate e rua. Ka tūtohu pea tō gastroenterologist i ngā whakamātautau mahi ate me ngā rangahau whakaahua.

Rapua te tiaki mate tere mena ka whānau koe i te kirika, te wiri, me te mamae o te kopu, nā te mea ka taea e tēnei te tohu i te mate ngongo wai-mate nui e kiia nei ko te cholangitis e hiahia ana ki te maimoatanga tere.

He aha ngā take tūponotanga mō te Primary Sclerosing Cholangitis?

He maha ngā take ka nui ake ai tō tūponotanga ki te whānau i te PSC, ahakoa ko te whai i ēnei take tūponotanga kāore e whakamana ka whiwhi koe i te mate. Ko te mārama ki tō tūponotanga ka awhina ki te kite wawe me te aroturuki.

Ko ngā take tūponotanga tino nui:

• Te whai mate puku mumura, ina koa te ulcerative colitis
• Ko te tane - e rua te maha o ngā tāne e pā ana i te wā i ngā wāhine
• Te tau i waenga i te 30-50 tau, ahakoa ka puta i tetahi tau
• Te tīpuna o te raki o Uropi, Scandinavia rānei
• Te hītori whānau o te PSC, ngā mate autoimmune rānei
• Te whai i ētahi tohu ira pēnei i te HLA-B8 me te HLA-DR3

Ko etahi take tūponotanga iti ake ko te whai i ētahi atu mate autoimmune pēnei i te autoimmune hepatitis, te mate thyroid, te mate celiac rānei. Ka taea pea te whakaoho taiao kāore ano kia tino mārama.

He mea whakamīharo, ko te whakakī te ahua he pānga tiaki ki te PSC i ngā tāngata e pangia ana e te ulcerative colitis, ahakoa kāore ngā tākuta e tūtohu ana i te whakakī nā ōna maha atu raruraru hauora.

He aha ngā raruraru pea o te Primary Sclerosing Cholangitis?

Ka taea e te PSC te arahi ki ngā raruraru nui i te whakahaere o te mate me te kino o te toka ngongo wai-mate. Ko te mohio ki ēnei tūponotanga ka awhina i a koe me tō rōpū hauora ki te aroturuki mō ngā tohu whakatūpato me te whakauru wawe ina hiahiatia.

Ko ngā raruraru tino noa:

• Te cirrhosis ate me te whakaraweke ate
• Te toto nui o te portal e arahi ana ki te ate nui me te toto variceal
• Ngā mate huaketo o ngā ngongo wai-mate (cholangitis)
• Ngā kohatu ngongo wai-mate e hanga ana nā te rere wai-mate kino
• Ngā ngoikore o te huaora ngako-whakauru (A, D, E, K)
• Te osteoporosis mai i te ngoikore o te huaora D me te mate ate

Ko tetahi o ngā āwangawanga tino nui ko te cholangiocarcinoma, he momo mate pukupuku ngongo wai-mate e whānau ana i te 10-15% pea o ngā tāngata e pangia ana e te PSC. Ko tēnei mate pukupuku te tikanga he uaua ki te kite wawe, koia te take i nui ai te aroturuki tonu me te whakaahua me ngā whakamātautau toto.

Ko ngā tāngata e pangia ana e te PSC me te mate puku mumura he nui ake te tūponotanga o te mate pukupuku colorectal, e hiahia ana ki te tirotiro colorectal colonoscopy auau. I tua atu, he nui ake te tūponotanga o te mate pukupuku gallbladder, ahakoa he iti rawa tēnei raruraru.

Ko te rongo pai ko te maha o ngā raruraru ka taea te aukati, te whakahaere pai rānei me te tiaki hauora tika me te aroturuki. Ko ngā hui aroturuki auau ka awhina i te hopu i ngā raruraru wawe ina tino taea te maimoatanga.

Me pēhea te whakatau i te Primary Sclerosing Cholangitis i te kāinga?

Ahakoa he mea nui te maimoatanga hauora, he maha ngā mea ka taea e koe te mahi i te kāinga hei tautoko i tō hauora me te whakapai ake i tō kounga ora me te PSC. Ko ēnei rautaki tiaki-whaiaro te mahi pai ina whakakotahitia ki te tiaki hauora auau.

Arotake ki te pupuri i te kai pai nā te mea ka taea e te PSC te whakaraweke i te tango ngako me te huaora. Kai i te kai tōtika e whai hua ana i ngā hua, ngā huawhenua, me ngā pūmua ngawari. Ka hiahia pea koe ki te whakaiti i te kai ngako mena he raruraru tō te whakahaere, ka tūtohu pea tō tākuta i ngā tāpiringa triglyceride-waenganui.

Ko te tango i ō tāpiringa huaora i tohua tonu he mea nui, ina koa ngā huaora ngako-whakauru. He maha ngā tāngata e pangia ana e te PSC e whānau ana i ngā ngoikore ka arahi ki ngā raruraru wheua, ngā raruraru tirohanga, me te whakaora kino o ngā whara mena kāore i te maimoatia.

Mō te whakahaere i te ngawari i te kāinga, ngana ki te pupuri i tō kiri makuku me ngā hinu kāore he kakara, te tango i ngā kaukau makariri me te oatmeal, te paura tunutunu rānei, me te kakahu huruhuru, manawa pai. Pupuri i ō ngā kuku poto hei whakaiti i te kino o te kiri mai i te miri.

Noho tonu ki ngā tirotiro katoa i tūtohutia, tae atu ki ngā colonoscopies mena kei a koe te IBD me ngā rangahau whakaahua auau hei aroturuki mō ngā raruraru. Aukati i te waipiro katoa, nā te mea ka taea e ia te whakaraweke i te kino o te ate, kia tupato ki ngā rongoā-kore-tohutohu ka taea te pā ki tō ate.

Me pēhea te whakarite mō tō hui tākuta?

Ko te whakarite pai mō ō hui ka awhina i te whakarite ka whiwhi koe i te uara nui mai i tō wā me tō rōpū hauora. Ko te whakarite pai ka awhina ano i tō tākuta ki te whakatau i ngā whakatau e pā ana ki tō tiaki.

I mua i tō hui, hanga he rārangi o ō tohu katoa, tae atu ki te wā i tīmata ai rātou me te āhua o te huringa i te wā. Tātaihia ngā tauira kua kite koe, pēnei i te ahua o te ngawari i te wā o te rā, te pai ake rānei o te ngenge me te okioki.

Kawe mai he rārangi katoa o ngā rongoā, ngā tāpiringa, me ngā hua-kore-tohutohu katoa e tangohia ana e koe, tae atu ki ngā horopeta. Kohikohia hoki ngā hua whakamātautau hou, ngā ripoata whakaahua, ngā rēkōta rānei mai i ētahi atu kaiwhakarato hauora kua uru mai ki tō tiaki.

Tuhia ngā pātai e hiahia ana koe ki te ui ki tō tākuta. Ka taea e ēnei te whakauru i ngā āwangawanga e pā ana ki te whakahaere tohu, ngā whiringa maimoatanga, ngā whakarerekētanga noho, te aha rānei e tatari ana koe i te whakahaere o tō mate. Kaua e manukanuka ki te maha o ngā pātai - e hiahia ana tō rōpū hauora ki te awhina i a koe ki te mārama ki tō mate.

Whakaarohia te kawe mai i te mema whānau, te hoa whakawhirinaki rānei hei awhina i a koe ki te mahara ki ngā korero nui i kōrerotia i te hui. Ka taea hoki e rātou te whakarato i te tautoko ngākau me te awhina i te tautoko i ō hiahia mena ka hiahiatia.

He aha te tango matua e pā ana ki te Primary Sclerosing Cholangitis?

Ko te PSC he mate nui engari ka taea te whakahaere e hiahia ana ki te tiaki hauora tonu me ngā whakarerekētanga noho. Ahakoa kāore he rongoā i tēnei wā, he maha ngā tāngata e pangia ana e te PSC e ora ana i ngā oranga tino pai, whai tikanga me te maimoatanga tika me te aroturuki.

Ko te whakatau me te maimoatanga wawe ka taea te whakapai ake i tō tirohanga me tō kounga ora. Ko te matua ko te mahi tata ki te rōpū hauora e whai wheako ana ki te whakahaere i te PSC, te noho tonu ki ngā maimoatanga me te aroturuki, me te tango i te mahi kaha ki tō tiaki ake.

Kia mahara ko te PSC te pānga ki te katoa. Ko etahi he mate tupu haere noa e noho pumau ana mō ngā tau, ko ētahi atu ka hiahia pea ki ngā whakauru nui ake. Ko tō haerenga takitahi ka motuhake, me whakaatu tō mahere maimoatanga i ō hiahia me ō āhuatanga motuhake.

Noho whakaaro nui me te whakamārama. Ko te rangahau ki ngā maimoatanga PSC kei te haere tonu te whakapai ake, kei te whānau ngā maimoatanga hou ka taea te tuku i ngā whiringa pai ake i te wā kei te heke mai. Arotake ki te mea ka taea e koe te whakahaere i tēnei rā i te wā e mahi tahi ana koe me tō rōpū hauora ki te whakarite mō āpōpō.

Ngā pātai auau e pā ana ki te Primary Sclerosing Cholangitis

P1. He mate whakatūpato te Primary Sclerosing Cholangitis?

Kāore te PSC i te whakatūpato tika pēnei i ētahi mate ira, engari he āhuatanga ira te ahua e nui ake ai te tūponotanga. Ko te whai i te mema whānau e pangia ana e te PSC, ngā mate autoimmune rānei ka nui ake pea tō tūponotanga, engari ko te nuinga o ngā tāngata e pangia ana e te PSC kāore he mema whānau e pā ana. Ko ētahi tohu ira he tino noa i ngā tāngata e pangia ana e te PSC, engari ko te whai i ēnei tohu kāore e whakamana ka whānau koe i te mate.

P2. Ka taea e ngā whakarerekētanga kai te awhina i te Primary Sclerosing Cholangitis?

Ahakoa kāore ngā whakarerekētanga kai e taea te whakaora, te aukati rānei i te whanaketanga o te PSC, he mea nui te kai pai mō te whakahaere i ngā tohu me te aukati i ngā raruraru. Ka hiahia pea koe ki te whakaiti i te kai ngako mena he raruraru tō te whakahaere, a he mea nui te tango i ngā tāpiringa huaora ngako-whakauru. Ka kite etahi i te aukati i te waipiro katoa me te kai i ngā kai iti ake, auau ake ka awhina ki te whakahaere tohu. Mahi tahi me tō rōpū hauora, te kaiwhakaako kai rānei e mohio ana ki ngā mate ate hei whakawhanake i te mahere kai e mahi ana mōu.

P3. Kia tere te whanaketanga o te Primary Sclerosing Cholangitis?

Ko te whanaketanga o te PSC he rerekē rawa atu i te tangata ki te tangata. Ko etahi he mate tupu haere noa e noho pumau ana mō ngā tau maha, ko ētahi atu ka tere ake te whanaketanga ki ngā raruraru pēnei i te cirrhosis. Ko ngā take ka āwhina ki te whanaketanga ko tō tau i te whakatau, te whai rānei i te mate puku mumura, me te āhua o tō whakautu ki ngā maimoatanga. Ko te aroturuki auau ka awhina i tō rōpū hauora ki te aroturuki i tō whanaketanga takitahi me te whakarite i ngā maimoatanga.

P4. Ka taea e te Primary Sclerosing Cholangitis te haere ki te remission?

Kāore te PSC e haere noa ki te remission katoa pēnei i ētahi atu mate autoimmune. Heoi, ka taea te noho pumau o te mate mō ngā wā roa, ka pai ake pea ngā tohu me te maimoatanga. Ka kite etahi i ngā wā e ahua ana te mate ki te tū, te pai ake rānei, engari ko ngā huringa ngongo wai-mate e noho tonu ana. Ko te whāinga o te maimoatanga ko te whakaheke i te whanaketanga, te whakahaere i ngā tohu, me te aukati i ngā raruraru, kāore i te whiwhi i te remission.

P5. He aha te roa o te ora me te Primary Sclerosing Cholangitis?

Ko te roa o te ora me te PSC he rerekē rawa atu i runga i ngā take pēnei i te tau i te whakatau, te kaha o te mate, te whakautu ki te maimoatanga, me te whānau rānei o ngā raruraru. He maha ngā tāngata e pangia ana e te PSC e ora ana mō ngā tekau tau i muri i te whakatau, ina koa ka hopukina wawe te mate me te whakahaere pai. Ko te wā toharite mai i te whakatau ki te whakawhiti ate, ngā raruraru nui rānei he 10-20 tau, engari kāore etahi e tae atu ki tenei wā. Arotake ki te mahi tahi me tō rōpū hauora hei whakapai ake i tō tiaki takitahi, kāore i te manukanuka ki ngā tatauranga kāore pea e tono ki tō āhuatanga motuhake.