He aha te Schwannomatosis? Tohutohu, Take, me te Maimoatanga

Ko te Schwannomatosis he āhuatanga tuku iho onge e hua ai ngā pūngāwerewere pai e kiia nei ko ngā schwannomas ki te tipu i runga i ōu uaua. Ka tipu ēnei pūngāwerewere mai i te hipoki tiaki kei te taha o ngā muka uaua, ka hanga ai ngā tipu ka taea te whakaeke i te mamae me ētahi atu tohu puta noa i tō tinana.

Ahakoa ko te kupu “pūngāwerewere” pea ka rongo wehi, ehara ngā schwannomas i te mate pukupuku, kāore hoki e horapa ki ētahi atu wāhanga o tō tinana. Whakaarohia rātou ko ngā tipu kore e hiahiatia e pēhi ana i ō uaua, pēnei i te hu e pēhi ana i tō waewae. Ka pā tēnei āhuatanga ki te iti iho i te 1 i roto i ngā 40,000 o te tangata, ka meinga ai he mea onge rawa, engari he tino pono mō te hunga e pā ana ki a rātou.

He aha ngā tohu o te schwannomatosis?

Ko te tohu matua o te schwannomatosis ko te mamae roa e taea te rereke mai i te kore ngāwari iti ki ngā wāhanga kino, kaore e taea te ora. Ko tēnei mamae te nuinga o te wā ka rereke te ahua mai i ngā mamae me ngā mamae noa, na te mea i puta mai i ngā pūngāwerewere e pēhi ana i ō huarahi uaua.

Ko ēnei ngā tohu tino noa ka pā pea ki a koe:

• Te mamae roa pea he tahuna, he kōtiti, he koi rānei te ahua
• Te ngenge, te ngoikore rānei i ngā wāhi e pā ana
• Te ngoikore o ngā uaua, ina koa i ngā ringa me ngā waewae
• Ngā pūngāwerewere e kitea ana, e kitea ana rānei i raro i te kiri
• Te mamae e kino ake ana me te nekehanga, te pa rānei
• Ngā mamae o te upoko ina pā ngā pūngāwerewere ki ngā uaua o te anga

Kaore e tino noa, ka pā pea ki ētahi o te hunga ngā raruraru whakarongo mena ka tipu ngā pūngāwerewere tata ki ngā uaua whakarongo. Ka taea te kore whakarite o ngā tauira mamae, ka puta mai pea i te wa poto, ka kino ake rānei i te wa roa. Ko te mea e whakawehi ana i tēnei āhuatanga ko te mea pea kāore te mamae e rite tonu ki ngā pūngāwerewere e kitea ana, na te mea ka tipu etahi pūngāwerewere hohonu ake i roto i tō tinana.

He aha ngā momo o te schwannomatosis?

E rua ngā momo matua o te schwannomatosis e mohiotia ana e ngā tākuta, e whakaū ana i ō rātou take tuku iho. Ko te mārama ki te momo kei a koe ka āwhina i te aratohu i ngā whakatau maimoatanga me ngā whakaaro mo te whānau.

Ko te momo tuatahi e pā ana ki ngā rerekētanga i roto i te kaitiaki SMARCB1, e whakaatu ana i te 85% o ngā take katoa. Ko tēnei ahua te nuinga o te wā e hua ai ngā pūngāwerewere puta noa i te tinana, a, ka hua pinei i ngā tohu whānui ake. Ko te momo tuarua e pā ana ki ngā rerekētanga i roto i te kaitiaki LZTR1, a, ka hua pinei i te iti o ngā pūngāwerewere, ahakoa ka taea tonu te nui o ngā tohu.

He momo onge ano e kiia nei ko te schwannomatosis mosaic, kei reira te rerekētanga tuku iho e puta ana i te tīmatanga o te whanaketanga, engari kāore e pā ana ki ngā pūtau katoa i roto i tō tinana. Ko tēnei momo te nuinga o te wā e hua ai ngā pūngāwerewere i ngā rohe motuhake, kāore i puta noa i tō tinana katoa.

He aha te take o te schwannomatosis?

Ko te hua o te schwannomatosis ko ngā rerekētanga tuku iho e whakakore ana i te tikanga o ō pūtau ki te whakahaere i te tipu o te pūngāwerewere. Ka pā ēnei rerekētanga ki ngā kaitiaki e mahi ana hei “pūkaha” hei aukati i te tipu o te pūtau kore e hiahiatia i runga i ō uaua.

Ko te nuinga o ngā take e puta mai ana i ngā huringa tuku iho ngāwari, ko te tikanga ka puta ngāwari mai kāore i te tuku iho mai i ngā mātua. Ko te 15-20% o ngā take ka tuku iho mai i te mātua e mau ana i te rerekētanga tuku iho. Ina tuku iho, he 50% te tūponotanga o te tuku i te āhuatanga ki ia tamaiti.

Ko ngā kaitiaki pūngāwerewere te pito o ngā rerekētanga, e pupuri ana i ngā pūtau mai i te tipu kore e whakahaeretia. Ina kore ēnei kaitiaki e mahi tika, ka taea e ngā pūtau schwanna (ngā pūtau e takai ana i ngā uaua) te maha nui, ka hanga ai ngā pūngāwerewere tohu. Kāore ngā take taiao e kitea ana he mahi nui ki te whanaketanga o tēnei āhuatanga.

Nōhea te haere ki te tākuta mō te schwannomatosis?

Me rapu awhina hauora koe mena ka pā ki a koe te mamae roa, kaore i te whakamārama, kāore hoki e whakautu ki ngā tikanga whakaora mamae noa. He mea nui tēnei, ina koa ki te ahua rereke te mamae, ki te puta mai rānei me ētahi atu tohu neurological.

Whakapa atu ki tō kaiwhakarato hauora i te wa poto mena ka kite koe i ngā pūngāwerewere maha i raro i tō kiri, ina koa ki te mamae rānei, ki te tipu rānei. Ko tetahi whakakotahitanga o te mamae roa, te ngenge, te ngoikore o ngā uaua, te tipu rānei e kitea ana e hiahiatia ana te aromatawai ngaio. Kaua e tatari mena kei te pāngia koe e te mamae kino e āwangawanga ana i ō mahi o ia rā, i tō moe rānei.

Mena he hītori whānau kei a koe o te schwannomatosis, o ngā āhuatanga e rite ana pēnei i te neurofibromatosis, kōrerohia te tohutohu tuku iho ki tō tākuta ahakoa i mua i te puta mai o ngā tohu. Ka taea e te kite wawe me te aroturuki te whakahaere i ngā tohu kia pai ake, me te aukati i ngā raruraru.

He aha ngā take tūponotanga mō te schwannomatosis?

Ko te take tūponotanga matua mō te schwannomatosis ko te whai hītori whānau o te āhuatanga. Mena he schwannomatosis tētahi mātua, he 50% te tūponotanga o ia tamaiti ki te whiwhi i te rerekētanga tuku iho, ahakoa kāore te katoa e whiwhi ana i te rerekētanga ka whanake i ngā tohu.

Ka taea e te tau te awe i te wa e puta mai ai ngā tohu, me te nuinga o te hunga e whanake ana i ngā tohu i waenga i ngā tau 25 me te 30. Heoi, ka taea te puta mai o ngā tohu i tetahi tau, mai i te tamarikitanga tae noa ki te pakeke. Kāore i rite ki ētahi āhuatanga tuku iho, ka pā te schwannomatosis ki ngā tāne me ngā wāhine rite tonu.

He mea whakamīharo, ahakoa ngā tāngata e whiwhi ana i te rerekētanga tuku iho kāore e whanake tonu i te āhuatanga. Ko tēnei ahuatanga, e kiia nei ko te kore-whakauru, ko te tikanga ko te whai i te kaitiaki kāore e whakamana ka whai koe i ngā tohu. E whakaaro ana ngā kairangi ko te 90% o ngā tāngata e whai ana i te rerekētanga ka whanake i etahi tohu, engari ka rereke te kaha me te wā.

He aha ngā raruraru pea o te schwannomatosis?

Ko te raruraru tino nui o te schwannomatosis ko te mamae roa e taea te āwangawanga nui i tō kounga ora. Ko tēnei mamae te nuinga o te wā ka uaua ki te whakahaere me ngā maimoatanga noa, ā, pea ka hiahiatia ngā huarahi whakahaere mamae motuhake.

Ko ēnei ngā raruraru pea ka whanake i te wa roa:

• Te mamae roa kino e āwangawanga ana i ngā mahi o ia rā
• Te ngoikore o ngā uaua e ahu whakamua ana, te pararaihi rānei i ngā wāhi e pā ana
• Te kino o ngā uaua mai i te pēhanga pūngāwerewere
• Te ngaro o te whakarongo mena ka pā ngā pūngāwerewere ki ngā uaua whakarongo
• Te pēhi i te uaua o te tuara i ngā take onge
• Te pouri me te manukanuka e pā ana ki te mamae roa
• Ngā raruraru moe na te kore ngāwari tonu

Ahakoa ko te nuinga o ngā schwannomas e noho ana hei pai, he iti te tūponotanga (iti iho i te 5%) ka taea te kino. He mea onge tēnei huringa, engari e hiahiatia ana te aroturuki mā ngā tirotiro hauora noa. Ka taea hoki te nui o te pānga hinengaro o te noho me te mamae roa me te kore mōhio, ka hiahiatia pea te tautoko mai i ngā tohunga hauora hinengaro.

Me pēhea te whakatau i te schwannomatosis?

Ko te whakatau i te schwannomatosis te nuinga o te wā e tīmata ana mā tō tākuta e tango i te hītori hauora taipitopito, me te mahi i te whakamātautau tinana tino pai. Ka ui rātou ki ō tauira mamae, ki tō hītori whānau, ka tirotiro i ngā pūngāwerewere e kitea ana, i ngā wāhi rānei e awangawanga ana.

He mahi nui ngā rangahau whakaahua ki te whakatau. Ka taea e ngā tātaritanga MRI te whakaatu i ngā schwannomas puta noa i tō tinana, ahakoa ngā mea iti rawa ki te kite. Pea ka tono tō tākuta i ngā tātaritanga MRI maha o ngā rohe tinana rerekē hei tiki i te whakaahua tino pai. Ka taea hoki te whakamahi i ngā tātaritanga CT hei tirotiro i ngā wāhi motuhake kia tino marama ake.

Ka taea e te whakamātautau tuku iho te whakau i te whakatau mā te kite i ngā rerekētanga i roto i ngā kaitiaki SMARCB1, LZTR1 rānei. Ko tēnei whakamātautau e pā ana ki te tauira toto ngāwari, ā, ka taea hoki te āwhina ki te whakatau mena pea ka tuku iho te āhuatanga ki ō tamariki. I etahi wā, pea ka tūtohu ngā tākuta i te tātaritanga o te pūngāwerewere hei whakau he schwannoma, hei whakakore i ētahi atu āhuatanga.

He aha te maimoatanga mō te schwannomatosis?

Ko te maimoatanga mō te schwannomatosis e aro nui ana ki te whakahaere i te mamae me ngā tohu, na te mea kāore he rongoā mō te āhuatanga tuku iho i teie nei. Ka mahi tahi tō rōpū hauora ki a koe hei whakawhanake i te mahere maimoatanga whaiaro e whakaū ana i ō tohu motuhake me ō hiahia.

Ko te whakahaere mamae te nuinga o te wā e hiahia ana ki te huarahi maha-āhuatanga e whakakotahi ana i ngā rautaki rerekē. Ko ngā rongoā pea ko ngā rongoā anti-seizure pēnei i te gabapentin, ngā rongoā pouri e āwhina ana ki te mamae o ngā uaua, me ētahi wā ngā rongoā mamae kaha ake mō ngā wāhanga kino. Ka taea e te rongoā tinana te āwhina ki te pupuri i te nekehanga me te kaha me te whakaiti i te mamae.

Pea ka whakaarohia te pokanga mō ngā pūngāwerewere e whakaeke ana i ngā tohu kino, e pēhi ana rānei i ngā hanganga nui. Heoi, he tūponotanga te pokanga, kāore hoki i te hiahiatia i te wa katoa na te mea he pai ngā schwannomas. Ka whakatau tika tō taote pokanga i ngā painga pea ki ngā tūponotanga pēnei i te kino o ngā uaua. Ka kite ētahi i te whakaora mā ngā huarahi ke pēnei i te acupuncture, te rongoā mirimiri, te whakaaroaro rānei mō te whakahaere mamae.

Me pēhea te whakahaere i te schwannomatosis i te kāinga?

Ko te whakahaere i te schwannomatosis i te kāinga e pā ana ki te whakawhanake i ngā rautaki hei whakahaere i te mamae roa me te pupuri i tō kounga ora. Ka taea e te whakatū i ngā mahi o ia rā pumau te āwhina i a koe ki te whakahaere pai i ngā tohu me te whakaiti i te ahotea ki tō tinana.

Ka taea e te rongoā wera me te makariri te hoatu i te whakaora mamae mo te wa poto mō te tini o te iwi. Whakamātauhia ngā kaukau wera, ngā papa wera, ngā peihana huka rānei hei kite i te mea pai rawa mō ō tauira mamae motuhake. Ka taea e te whakakori ngawari pēnei i te kauhoe, te hikoi, te yoga rānei te āwhina ki te pupuri i te ngawari me te kaha me te kore e whakatū te ahotea nui ki ō uaua.

He mea nui rawa ngā tikanga whakahaere ahotea, na te mea ka taea e te ahotea te whakaiti i te kite mamae. Whakaarohia ngā mahi pēnei i ngā mahi manawa hohonu, te whakaaroaro, te whakamahinga uaua whakamua rānei. Ka taea e te pupuri i te puka mamae te āwhina i a koe ki te kite i ngā mea whakaeke me te aroturuki i ngā maimoatanga e pai ana mōu.

Me pēhea te whakarite mō tō hui tākuta?

I mua i tō hui, hangaia he rārangi taipitopito o ō tohu, tae atu ki te wā i tīmata ai rātou, he aha te mea e pai ake ai, te kino ake rānei, me pehea te pānga ki ō mahi o ia rā. Kia tino marama ki te momo, te wāhi, te kaha rānei o tetahi mamae e pāngia ana koe.

Kohikohia tō hītori hauora tino pai, tae atu ki ngā rangahau whakaahua o mua, ngā hua whakamātautau, me ngā maimoatanga kua whakamātauhia e koe. Mena he hītori whānau kei a koe o te schwannomatosis, o ngā āhuatanga e rite ana, kohikohia ēnei korero. Maua he rārangi o ngā rongoā me ngā taapiri katoa e inu ana koe i tēnei wā.

Whakaritea ngā pātai mō tō āhuatanga, ngā kōwhiringa maimoatanga, me te mea e tatari ana koe ki te haere whakamua. Whakaarohia te kawe mai i tētahi hoa whakawhirinaki, i tētahi mema whānau rānei hei āwhina i a koe ki te mahara ki ngā korero nui i kōrerotia i te hui. Kaua e mangere ki te tono ki tō tākuta kia whakamārama i tetahi mea kaore koe e mārama ki ngā kupu ngāwari ake.

He aha te mea nui mō te schwannomatosis?

He āhuatanga taea te whakahaere te schwannomatosis, ahakoa he roa, ka taea te āwangawanga nui i tō ora. Ahakoa kāore he rongoā, he tini ngā tāngata e kite ana i ngā huarahi whai hua ki te whakahaere i ō rātou tohu me te pupuri i te kounga ora pai me te tiaki hauora tika me te tautoko.

Ko te mea nui rawa hei mahara ko te kore koe e noho kotahi i tēnei haerenga. Ko te mahi tata atu ki tētahi rōpū hauora e mārama ana ki tō āhuatanga ka taea te whakarereke nui ki te whakahaere i ngā tohu. Me te whakakotahitanga tika o ngā maimoatanga, ngā whakarerekētanga noho, me te tautoko, he tini ngā tāngata e whai ana i te schwannomatosis e ora ana i te ora whai hua, kaha.

Noho whakaaro pai me te mahi whakamua mō tō tiaki. Kei te haere tonu te rangahau ki ngā maimoatanga pai ake, ā, kei te whanake tonu ngā rautaki whakahaere mamae hou. Aro ki te mea ka taea e koe te whakahaere, ā, kaua e mangere ki te rapu tautoko ina hiahia koe.

Ngā pātai e ui pinei ana mō te schwannomatosis

He rite te schwannomatosis ki te neurofibromatosis?

Kāo, he āhuatanga motuhake te schwannomatosis mai i te neurofibromatosis, ahakoa he mate tuku iho e rua e pā ana ki te pūnaha io. Ko te schwannomatosis te nuinga o te wā e hua ai te mamae nui me ngā tohu iti ake i te neurofibromatosis. He rerekē hoki ngā rerekētanga tuku iho me ngā tauira tuku iho i waenga i ēnei āhuatanga.

Ka taea te aukati i te schwannomatosis?

Na te mea he āhuatanga tuku iho te schwannomatosis, kāore e taea te aukati i te tikanga noa. Heoi, mena he hītori whānau kei a koe o te āhuatanga, ka taea e te tohutohu tuku iho te āwhina i a koe ki te mārama ki ō tūponotanga me te mahi i ngā whakatau whakamārama mō te whakatū whānau. Ka taea e te kite wawe me te aroturuki te whakahaere i ngā tohu kia pai ake.

Ka kino ake rānei ō tohu schwannomatosis i te wa roa?

Ko te ahu whakamua o te schwannomatosis e rereke ana i te tangata ki te tangata. Ka pāngia ētahi i te kino haere o ngā tohu, ā, ka noho ētahi atu i te pumau mo ngā tau. Ka taea e te aroturuki noa me tō rōpū hauora te āwhina ki te aroturuki i ngā huringa, me te whakarite i ngā maimoatanga kia rite.

Ka taea e au te whānau tamariki mena he schwannomatosis kei a au?

Ae, ko te whai schwannomatosis kāore e aukati i a koe ki te whānau tamariki, engari he 50% te tūponotanga o te tuku i te rerekētanga tuku iho ki ia tamaiti. Ka taea e te tohutohu tuku iho te āwhina i a koe ki te mārama ki ngā tūponotanga me te tirotiro i ngā kōwhiringa pēnei i te whakamātautau i mua i te whānautanga. He tini ngā tāngata e whai ana i te schwannomatosis e whai ana i ngā whānau hauora me ngā tamariki.

He aha ngā huringa kai e taea te āwhina ki ngā tohu schwannomatosis?

Ahakoa kāore he kai motuhake e whakamāramahia ana hei whakaora i te schwannomatosis, ka taea e te pupuri i te kai pai te tautoko i tō hauora whānui, ā, pea ka āwhina ki te whakahaere mamae. Ka kite ētahi ko ngā kai anti-inflammatory pea ka āwhina ki te whakaiti i te mumura whānui i roto i te tinana. Kōrerohia tonu ngā huringa kai ki tō kaiwhakarato hauora hei whakarite kāore e āwangawanga ki ō rongoā.