Kua pāngia tetahi o tō whānau e te mate pukupuku koroni? Haere mai, me kōrero tātou mō tēnei mate ira (Familial Adenomatous Polyposis - FAP)!

Kua pāngia tetahi o tō whānau e te mate pukupuku koroni, i te wā taitamarikitanga pea? Kua kōrerotia mai rānei e te tākuta he iti ngā tipu (polyps) kei roto i tō koroni me ngā raruraru puku? Ahakoa kua rongo koe i tēnei mea, he mea nui kia mōhio koe ki tēnei mate e kiia nei ko te FAP ka kōrerohia e tātou i tēnei rā. Ahakoa he onge, ki te kitea wawe, ka taea e ia te whakaora i ngā raruraru nui.

He aha te FAP i roto i ngā kupu māmā noa iho?

Ki te whakarapopototanga, ko te Familial Adenomatous Polyposis, ko te FAP rānei, he āhuatanga ira e puta ai te maha o ngā tipu iti (polyps) e kiia nei ko ngā adenomas, ka taea te huri hei mate pukupuku, ki roto i tō koroni, ā, i ētahi wā ki roto i tō rectum.

Whakaarohia, ka puta mai i ētahi tāngata tētahi, e rua rānei o ēnei pukupuku i roto i tō rātou koroni i a rātou e pakeke haere ana. He mea noa tēnā. Engari mō te tangata whai FAP, he maha ngā wā neke atu i te kotahi rau, i ētahi wā he mano o ēnei pukupuku, engari ka tīmata te tipu i te wā taitamariki tonu, tae noa ki te tekau tau te pakeke. Nō reira, he tino teitei te tūponotanga ka huri tētahi o ēnei pukupuku hei mate pukupuku (mate pukupuku colorectal), arā, ko te mōrearea mō te oranga katoa .

Hei whakahaere i tēnei mōrearea, ka tūtohu ngā tākuta kia pokangahia te tango i te koroni katoa (total colectomy) . I ētahi wā, ka tangohia hoki te rectum (proctocolectomy). Nā te mea i roto i te FAP, ka tere rawa te tipu o ēnei pukupuku, ā, kāore e taea te whakahaere mā te tango takitahi. Inaa, ki te kore tēnei pokanga e mahia, he maha ngā tāngata whai FAP ka pāngia e te mate pukupuku i te pakeketanga .

Ka taea e te hunga whai FAP te whakawhanake i ētahi atu momo pukupuku, ehara i te mea i roto i te koroni anake, engari i ētahi atu wāhi anō hoki, pērā i te kiri, te kiko ngohengohe, ngā niho, me ngā wheua. Ahakoa kua tangohia te koroni, me haere tonu pea ō tirotiro hei tirotiro i ēnei atu pukupuku, ā, me pokanga pea koe mō rātou.

He aha te mōrearea o te whakawhanake i te mate pukupuku mō te hunga whai FAP?

Ki te kore e rongoatia, tata ki te 100% te tūponotanga o te tangata whai FAP ki te pāngia e te mate pukupuku koroni. Waihoki, ka taea e te mate pukupuku te whanake wawe ake, ā, i te wā taitamariki ake i ētahi atu tāngata. Mena e mōhiotia ana kei te pāngia tētahi mema o te whānau e te mate, me whai colonoscopy ngā tamariki o aua whānau i ia tau, mai i te 10 tau te pakeke .

Haunga te mate pukupuku koroni, kei te tūpono te hunga whai FAP ki te whakawhanake i ētahi atu momo mate pukupuku:

• Te mate pukupuku o te taha whakarunga o te whekau iti ('Te mate pukupuku Duodenal') - tata ki te 8%
• Matepukupuku tairoi papillary - tata ki te 2%
• Matepukupuku repeure - tata ki te 2%
• Te mate pukupuku ate e kiia nei ko te "Hepatoblastoma" (ina koa i roto i ngā tamariki) - tata ki te 1.5%
• Matepukupuku puku - tata ki te 1%
• Ngā pukupuku o te roro me te taurakira - iti iho i te 1%

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He rerekē ngā momo FAP?

Āe, kei reira tētahi momo matua o te 'FAP' me ētahi atu momo anō hoki hei tāpiri atu ki tēnā.

• FAP "Tawhito": Ko te āhua o tēnei mate ko te neke atu i te 100 adenomas i roto i te koroni.
• "Attenuated" FAP (AFAP): He momo iti ake te kino o tēnei. E 20 ki te 100 ngā whē i roto i te koroni.
• Te mate Gardner: Pērā i te FAP matarohia, neke atu i te 100 ngā pukupuku i roto i te koroni. Hei tāpiri, ka puta mai ētahi atu momo pukupuku i ētahi atu wāhi o te tinana.
• Te mate Turcot: Ka puta he pukupuku matepukupuku i roto i te roro me ngā pukupuku maha i roto i te whekau.

He pēhea te noa o te ingoa o tēnei āhuatanga ko te FAP?

He tino onge te mate FAP. Tata ki te kotahi i roto i te 8,000 tāngata ka pāngia. Tata ki te 0.5% o ngā mate pukupuku koroni katoa te FAP. He onge ake ngā momo iti pēnei i te AFAP, te mate Gardner, me te mate Turcotte.

He aha te rerekētanga i waenga i te FAP me te mate Lynch?

Ko te mate Lynch tētahi atu mate tuku iho e whakanui ana i te mōrearea o te mate pukupuku koroni me ētahi atu mate pukupuku. Ko te mate Lynch e kiia ana ko te HNPCC (hereditary nonpolyposis colorectal cancer syndrome). E ai ki te ingoa, ehara i te mea ka puta he maha o ngā pukupuku koroni .

Ahakoa kotahi, neke atu rānei ngā pukupuku kei roto i te koroni, ka pāngia pea te hunga whai mate pukupuku e te mate Lynch. Waihoki, ka roa ake te whanaketanga o ngā pukupuku me te mate pukupuku i te FAP, ā, he iti ake te mōrearea. Nā ngā whakarerekētanga ira rerekē ēnei āhuatanga e rua.

He aha ngā tohu o te FAP? Me pēhea tātou e mōhio ai?

I roto i te FAP, ka tīmata te hanga o ngā polyp o te koroni i mua atu i te taupori whānui, i te nuinga o te wā i ngā tau taiohi . Tērā pea kāore ēnei polyp e puta he tohu tae noa ki te nui rawa atu. Engari ki te whai koe i tētahi colonoscopy, ka taea e tō tākuta te kimi i a rātou.

Tērā pea he rau, he mano rānei ngā polyp kei roto i te koroni o te hunga whai FAP. E 20 pea te iti rawa o ngā polyp kei roto i te FAP. Nā te mea he maha ake ngā polyp, ā, he tere ake te tipu i roto i te FAP, he nui ake te tūponotanga ka puta ngā tohu i ngā polyp. Ko ngā tohu ko:

• Te toto o te rectum
• Korere
• Mamae puku mau tonu

Tērā pea ka kitea e te hunga whai FAP ngā tohu e kitea ana e ngā tākuta i waho:

• Dermatofibromas: He puku muka, he rite ki te koha, kei raro i te kiri.
• Ngā pūwero kiriuhi: He puku porowhita, kī tonu i te keratin, kei raro i te kiri.
• Osteomas: Ngā pukupuku kore-matepukupuku e puta mai ana i roto i ngā wheua. Ko te nuinga o te wā ka kitea i roto i te kauae, i te angaanga rānei.
• Niho tāpiri, niho pakaru rānei:Ka kitea ēnei i runga i te hihi-X o ngā niho.
• Te nui haere o te epithelium pigment retinal i roto i te whānautanga (CHRPE): Ka kitea ēnei i te wā e tirotirohia ana te kanohi. Heoi, kāore e puta he raruraru tirohanga i te nuinga o te wā.

I tehea tau ka tīmata ngā tohumate FAP?

I roto i te FAP, ka tīmata te tipu o te koroni i te 16 tau te pakeke. I roto i te AFAP, ka tīmata pea i muri tata mai. Mena ka mōhio ō mātua me ō tākuta kei te mōrearea koe ki te mate, ka tīmata pea rātou ki te whakamātautau i a koe i muri i te 10 tau te pakeke . Ki te kore, ka kitea pea koe kei te mate koe nā ō tohumate.

He aha te tino take o te FAP?

Ko te tino take o te FAP he whakarerekētanga ira . Ka kiia tēnei ira ko te ira adenomatous polyposis coli (APC) . Ka kiia hoki ko te ira pehi pukupuku. Ko te tikanga o tēnei ka ārai tēnei i ngā pūtau kei tipu haere i waho o te mana whakahaere me te hanga pukupuku. Ina puta he whakarerekētanga ira, ka whakararuraruhia te mahi a tēnei ira.

Ko te whakarerekētanga ira e puta ai te FAP he whakarerekētanga ira-ira. Ko te tikanga tēnei ehara i te mea e puta ana i te wā o te ora, engari he mea e puta ana i te wā haputanga . He mea tēnei e tukuna iho ana i ia whakatipuranga ki ia whakatipuranga. Mena kei tētahi o ō mātua tēnei whakarerekētanga ira, e 50% te tūponotanga ka riro mai i a koe. Heoi, tata ki te 30% o ēnei whakarerekētanga ira he mea hou (ngā whakarerekētanga taketake), arā, ehara i te mea tuku iho . Nō reira, tata ki te 30% o ngā tūroro kua kitea he FAP kāore pea he hītori whānau mō te mate.

He aha ngā raruraru ka pā mai i te FAP?

Ko te raruraru nui rawa atu hei whakahaere i roto i te FAP ko te mate pukupuku koroni . Mā te pokanga hei tango i tō koroni (colectomy) ka tino whakaiti i tēnei mōrearea. Heoi, ko te noho me te kore koroni ka pā ki tō oranga nā te mea ka rerekē te āhua o tō tuku paru. Me whakamahi pea koe i te putea ileostomy, i te putea ileal rānei hei utu mō te koroni.

Nā te mea kei roto i ngā pūtau katoa o tō tinana te whakarerekētanga ira e puta ai te FAP, ka taea e ngā pukupuku te whanake i ngā wāhi katoa o tō tinana . Ka taea hoki e ētahi atu pukupuku te whanake i waho o te koroni, ā, i ētahi wā ka taea te huri hei matepukupuku. Ka tūtohu pea tō tākuta i tētahi wā tirotiro hei rapu i ēnei momo pukupuku:

• Ngā polyp o te duodenum/periampullary:Ka puta ēnei i te wāhanga o runga o tō whekau iti (duodenum), i te wāhi rānei e hono ai te awa au, te awa pancreatic rānei ki te whekau iti. Tata ki te katoa e pāngia ana e te FAP ka pāngia e ēnei. Ka taea e te tokoiti o te hunga te pāngia e te mate pukupuku duodenal, te mate pukupuku ampullary, te mate pukupuku pancreatic i roto i te awa, te mate pukupuku awa au rānei.
• Ngā polyp o te puku: Tata ki te 90% o te hunga whai FAP ka pāngia e ngā polyp o te puku, engari 1% noa iho o ēnei ka huri hei matepukupuku.
• Ngā pukupuku Desmoid: He pukupuku kore-matepukupuku ēnei e puta ana i roto i te kiko honohono. Ka puta i roto i te 15% o te hunga whai FAP. Ahakoa ehara i te matepukupuku, i ētahi wā (tata ki te 5%) ka pāngia e ngā raruraru hauora nui, tae atu ki te mate. Ka tino kaha, ka horapa ki ngā hanganga tata, ka pēhi, ka ārai rānei i ngā oko toto, ngā whekau, me ngā io. He uaua ki te tango, ā, ka taea te hoki mai anō.
• Matepukupuku tairoi papillary: Ka pā tēnei momo matepukupuku tairoi ki te 2% o te hunga whai FAP. He iti ake te mōrearea, ā, he maha ngā wā ka taea te rongoā.
• Matepukupuku ate: Ko ngā tamariki whai FAP, inā koa, he iti noa te mōrearea ki te whakawhanake i tētahi matepukupuku ate onge e kiia nei ko te hepatoblastoma.
• Medulloblastoma: He mate pukupuku roro tēnei. Ka pā mai pea me te mate Turcot, e pā ana ki te FAP. Ahakoa te FAP, he tino onge.
• Ngā polyp rectal: Ki te kore tō rectum e tangohia me tō colon, kei te tūpono koe ki te whakawhanake i ngā polyp rectal, nō reira me whai whakamātautau proctoscopy koe puta noa i tō oranga.

Me pēhea koe e tino mōhio ai mēnā kei a koe te FAP?

Ki te hiahia koe ki te mōhio mēnā kua riro i a koe te whakarerekētanga ira APC, ka taea e koe te kimi mā te whakamātautau ira . Ka tangohia e te whakamātautau ira he tauira o tō DNA (pēnei i te toto, i te huware rānei) ka rapu i ngā whakarerekētanga ira motuhake. Mēnā kei a koe te whakarerekētanga ira, ko te mahi e whai ake nei ko te whai i tētahi colonoscopy hei tirotiro mō ngā adenomas.

Ka kitea te FAP ina neke atu i te 100 ngā polyp (20 mēnā he AFAP) me te kitea o te whakarerekētanga ira APC . Ehara i te mea ko te FAP anake te mate tuku iho e puta ai ngā polyp adenomatous. He rite tonu te mate polyposis e pā ana ki te MUTYH, engari nā te whakarerekētanga ira i roto i tētahi atu ira e kiia nei ko te MUTYH i puta ai.

He aha te mahere maimoatanga mō te tangata whai FAP?

Kei roto i te mahere maimoatanga mō te FAP te aroturukitanga mō te wā katoa me te pokanga ā-ture.I ngā wāhanga tōmua, ki te mea he iti noa iho ō polyp (AFAP rānei), ka tangohia takitahitia pea e tō tākuta i te wā o te colonoscopy (polypectomy). Ina nui rawa ngā polyp, ina matepukupuku rānei, me mahi pokanga pea.

Pokanga

Ko te nuinga o te hunga whai FAP matarohia me tango katoa te colectomy i te mutunga o ngā tau rua tekau, i te tīmatanga rānei o ngā tau toru tekau . Mā tō tākuta e whakatau te wā e tika ai te pokanga i runga i ō āhuatanga mōrearea. Ka kōrero anō hoki ia ki a koe mō ngā momo colectomy rerekē ka taea e koe te mahi.

Ina tangohia tō koroni, ka hangaia he āputa i waenganui i tō whekau iti me te pūrua (te nono rānei). I ētahi wā, ka taea e te taote pokanga te hono anō i ēnei pito e rua. Kātahi ka taea e koe te neke i te puku mā roto i tō pūrua pērā i te tikanga. Ki te kore e taea, ka hangaia e rātou he 'ostomy', arā, he whakatuwheratanga hou i roto i tō puku hei putanga mai o tō paru.

Te tirotiro

Mā tō tīma hauora koe e tohutohu mō te auau o ngā whakamātautau tirotiro mō ngā momo pukupuku rerekē, i runga i ō āhuatanga mōrearea whaiaro. Mō te FAP matarohia, ka tūtohutia kia mahia he colonoscopy i ia tau mai i te 10 tau te pakeke tae noa ki te tango colectomy . Mō te AFAP, me tīmata te tirotiro i ia tau, ahakoa i te 20 tau te pakeke.

I muri i tō tangohanga o te kōpū, me haere tonu ngā whakamātautau sigmoidoscopy, e tirotiro ana i te wāhanga o raro o tō pūnaha nakunaku (GI - gastrointestinal tract). Mena kei te toe tonu tētahi wāhanga o tō kōpū, me tirotirohia i te iti rawa i ia 6 ki te 12 marama. Mena i tangohia tō kōpū, ā, i whakakapia ki tētahi putea ileum, me tirotirohia i te iti rawa i ia 1 ki te 4 tau.

Ko ētahi atu whakamātautau kua whakaritea ko:

• Endoscopy o runga: He rite tēnei ki te colonoscopy, engari ka mahia ki te taha o runga o tō pūnaha nakunaku. Ka tukuna he endoscope ki raro i tō korokoro, ka uru ki roto i tō puku me te taha o runga o tō whekau iti (duodenum) hei tirotiro mō ngā polyps. Mena he polyps kei roto, ka taea e tō tākuta te tango i aua polyps i te wā kotahi e whakahaerehia ai te tikanga.
• Ngā whakamātautau ultrasound mō te mate pukupuku tairoi, te ate rānei.
• Ngā whakamātautau CT scan (computered tomography scan) MRI rānei (magnetic resonance imaging) mō ngā pukupuku desmoid.

Ka taea te ārai i te FAP?

Te tohutohu ira hei mārama ki te mōrearea o te tuku i te mate FAP ki āu tamarikiHe āwhina pea. Mā te kōrero mō ēnei mōrearea ka āwhina i a koe ki te whakamahere i tō whānau. I te nuinga o te wā, kāore e taea te ārai i te puta o ngā whakarerekētanga ira i te wā e hapu ana, engari he maha ngā kōwhiringa whakamahere whānau ka taea e koe te whakaaro.

He aha te roanga o te oranga o te tangata whai FAP?

Ki te kore e rongoatia, ko te roanga o te oranga toharite he 42 tau . Heoi, mā te tiaki hauora me te maimoatanga tika, ka taea e koe te noho noa . I muri i te tango i tō koroni, ko tō mōrearea nui rawa atu ka puta mai i ētahi atu mate pukupuku o te pūnaha nakunaku me ngā pukupuku 'desmoid' e raru ana. He iti ake te kitea o ēnei i te mate pukupuku koroni.

Mena kei a au tēnei āhuatanga, he aha taku e tumanako ai?

Mena kua tāpaetia koe kei te pāngia e te FAP, ka taea e koe te tumanako kia whai whakamātautau hauora mō te wa katoa, me ētahi pokanga pea hei tango i ngā pukupuku . Ko ngā pukupuku e tupu ana i waho o tō koroni he iti ake te tūponotanga ka noho hei matepukupuku i ngā polyp i roto i tō koroni. Ahakoa ehara ngā pukupuku desmoid i te matepukupuku, i ētahi wā he iti noa iho te raruraru, he nui rānei te raruraru.

Ka taea e koe te tumanako ka tangohia katoatia tō puku i te tīmatanga o te oranga . Whai muri i tēnā, ka honoa anō pea ō whekau, ka pāngia rānei koe e te putea ileum, e te ostomy rānei. He mōrearea kei roto i ia o ēnei kōwhiringa ngā raruraru motuhake. Heoi, ka taea tonu e koe te ora roa, te ora hauora i muri i te tango i te puku.

He mea noa te pōuri me te manukanuka ina rongo koe he mate ira tōu e hiahia ana kia tiakina tō tinana mō te wā katoa. Heoi, ina mōhio koe, ka taea e koe te mahi i ngā mahi hei whakahaere i tō mōrearea mate pukupuku . Ahakoa kei te heke mai te pokanga, ka ngana ngā tākuta ki te whakahaere kia ngāwari te mahi.

He maha ngā tāngata ka taea te whakamahi i te tikanga laparoscopic hei tango i te koroni, ka mahia mā roto i ngā tapahi iti, ā, ka tere te ora . Tērā pea me mahi ētahi pokanga ki te hunga he putea ileum tō rātou, engari ā tōna wā ka taea e rātou te whakamahi i te wharepaku pērā i mua.

Ngā mea tino nui hei maumahara mai i ngā mea kua kōrerotia e tātou (Karere hei tango ki te kāinga)

Āe, mai i ngā kōrero kua kōrerotia e tātou mō te FAP, anei ngā mea tino nui hei maumahara māu:

• He mate ira te FAP ka taea te tuku iho i roto i ngā whakatipuranga.
• Ka puta ngā rau, ngā mano rānei o ngā polyp i roto i te koroni, ka huri pea hei mate pukupuku.
• Ki te kore e rongoatia, he tino teitei te mōrearea o te mate pukupuku koroni .
• He mea tino nui kia tīmata te whakamātautau colonoscopy mai i te tamarikitanga (10-12 tau te pakeke) .
• Ko te maimoatanga matua ko te pokanga hei tango i te koroni (colectomy) .
• Ahakoa kua tangohia te koroni, me tirotiro tonu mō te wa katoa kia kitea ai ngā pukupuku e tipu ana i ētahi atu wāhi o te tinana.
• Ahakoa he mate tēnei mō te wā katoa me whakahaere, mā te maimoatanga hauora me ngā whakamātautau tika, ka taea te ora i te oranga hauora, noa iho .

Mena kei a koe, kei tētahi rānei o tō whānau tētahi o ēnei tohu, ki te hiahia rānei koe ki te mōhio atu mō tēnei, me rapu tohutohu hauora . Ko te mōhio wawe me te mahi wawe ngā mea tino nui.