He aha te mate o Hirschsprung? Tohutohu, Take, me te Maimoatanga

Ko te mate o Hirschsprung he hapa whānau e pā ana ki te pūkahukahu nui (colon) ā, ka uaua ai te haere o te kakau ki ngā pēpi. Ka puta tēnei āhuatanga ina ngaro ētahi pūtau io e āwhina ana i te pūkahukahu ki te pehi i te paru i ngā wāhanga o te colon.

E pā ana ki te 1 i roto i ngā pēpi 5,000 e whānau ana me tēnei āhuatanga, ā, he maha atu i ngā tamaiti kārekau i ngā kōhine. Ko te rongo pai, mā te maimoatanga tika, ka taea e ngā tamariki me te mate o Hirschsprung te noho oranga hauora, noho mārama.

He aha te mate o Hirschsprung?

Ka puta te mate o Hirschsprung ina kore ngā pūtau io e kiia nei ko ngā pūtau ganglion e whānau pai ana i te pakitara o te colon. Ka korero ēnei pūtau motuhake ki ngā uaua o te pūkahukahu ina whakangāwari me te whakawhāiti kia neke te paru.

Kaore ēnei pūtau io, kaore te wāhanga o te colon e pā ana e pehi i te paru i te tikanga. Whakaarohia he paipa māra he kōtiti kei roto – ka mutu katoa i muri i te wāhanga kua ārai.

Ka tīmata tonu te āhuatanga i te tūturu ka horapa ki runga ki te colon. I te nuinga o ngā take, ko te wāhanga raro o te colon anake te pā, engari i etahi wā ka uru atu ki ngā wāhanga roa atu o te pūkahukahu.

He aha ngā tohu o te mate o Hirschsprung?

Ka puta ngā tohu o te mate o Hirschsprung i ngā wiki tuatahi o te ora, ahakoa i etahi wā kaore i kitea ra ano kia muri ake i te tamarikitanga. He rerekē ia pēpi, engari he tohu noa e titiro atu ana.

I ngā pēpi hou, ko ngā tohu tino whakamārama ko:

• Kāore te haere o te kakau tuatahi (meconium) i roto i ngā haora 48 i muri i te whānautanga
• Ruaki kikorangi, kākāriki rānei
• Puku pupuhi e ngākau pakari ana
• Hau, engari kāore he nekehanga o te kakau
• Uaua ki te whāngai me te iti o te pikinga taumaha
• Nekehanga kakau pakaru i muri i te whakauru i te matihao, rānei i te tūturu

Ka puta ēnei tohu tuatahi nā te kore e neke te paru i te wāhanga o te colon e pā ana i te tikanga. Ko te whakahoki mai ka puta he pēhanga kore pai ka ārai i te whāngai me te tipu noa.

I ngā pēpi tawhito me ngā tamariki, ka kite pea koe:

• Te mate pūkahukahu roa e kore e pai ake me ngā maimoatanga noa
• Kore te pikinga taumaha i te tere noa
• Te ngenge me te kore o te kaha
• Puku pupuhi
• Nekehanga kakau nui, iti hoki

I etahi tamariki me ngā momo ngawari o te āhuatanga, kaore pea e whakaatu tohu kia tae ra ano ki te tauira, ki te tawhito atu rānei. Ka uaua ake te whakatau i ēnei take, na te mea ka rite ngā tohu ki te mate pūkahukahu noa i te tīmatanga.

He aha ngā take o te mate o Hirschsprung?

Ka puta te mate o Hirschsprung i te wā o te hapu i te wā e whānau ana te pēpi i roto i te kopu. Kāore i tino mōhiotia te take tika, engari e mōhio ana tātou e pā ana ki ngā raruraru ki te tipu me te neke o ētahi pūtau io.

I ngā marama tuatahi o te hapu, ka tīmata ngā pūtau io motuhake i te pūnaha kai o te pēpi e whānau ana ka neke haere ki raro ki te tūturu. I ngā pēpi me te mate o Hirschsprung, ka mutu te neke o ēnei pūtau i mua i te tae atu ki tō rātou wāhi whakamutunga.

E whakaatu ana tēnei āhuatanga he wāhanga ira, ko te tikanga ka taea te rere i roto i ngā whānau. Mena he tamaiti kotahi te mate o Hirschsprung, he 3-12% te tūponotanga o ngā teina ki te whiwhi ano hoki, kei runga i ngā take maha.

Kua honoa etahi ira ki tēnei āhuatanga, ko te ira RET te mea tino hono. Heoi, ko te whai i ēnei huringa ira kaore e whakamana he tamaiti ka whānau ki te mate – ka whakanui noa i te tūponotanga.

He aha ngā momo o te mate o Hirschsprung?

Ka whakarōpū ngā tākuta i te mate o Hirschsprung i runga i te nui o te colon e pā ana ki ngā pūtau io ngaro. Ko te mārama ki te momo ka āwhina ki te whakatau i te huarahi maimoatanga pai.

Ko te mate poto-wāhanga te momo tino noa, e pā ana ki te 80% o ngā take. I roto i tēnei ahua, ko te wāhanga raro o te colon (tūturu me te colon sigmoid) anake te kore o ngā pūtau io. He maha ngā tamariki me tēnei momo he tohu ngawari me te pai rawa atu i muri i te maimoatanga.

Ko te mate roa-wāhanga e pā ana ki te wāhanga nui ake o te colon ā, ka puta i te 20% o ngā take. Ka puta tēnei momo i ngā tohu nui ake ā, ka hiahia pea ki te maimoatanga uaua ake. I etahi tamariki pea ka hiahia ki ngā pokanga maha, ki ngā wero pūkahukahu tonu rānei.

I roto i ngā take onge, ka taea e te āhuatanga te pā ki te colon katoa, ki te pūkahukahu iti rānei. Ka hiahia ēnei take ki te tiaki motuhake ā, ka uru pea ki te mahi tahi me te rōpū o ngā tohunga tamariki.

Nōhea te kite i te tākuta mō te mate o Hirschsprung?

Me whakapiri atu koe ki te tākuta o tō tamaiti tonu mena kāore tō pēpi hou e haere i tōna kakau tuatahi i roto i ngā haora 48 i muri i te whānautanga. Koinei tetahi o ngā tohu whakatūpato tuatahi nui.

Ko ētahi atu take hāngai hei rapu awhina hauora ko te ruaki kikorangi, kākāriki rānei, he puku pupuhi, he pakari rānei, he tohu rānei kāore tō pēpi e whāngai pai ana, ā, e ahua kore pai ana. Ka taea e ēnei tohu te tohu i te ārai nui e hiahia ana ki te maimoatanga tonu.

Mō ngā pēpi tawhito me ngā tamariki, ko te mate pūkahukahu tonu e kore e whakautu ki ngā huringa kai, ki ngā maimoatanga ngawari rānei e tika ana kia haere ki te tākuta. Mena kei te iti iho i te toru ngā nekehanga kakau o tō tamaiti i te wiki, kei te ahua uaua rānei, he mea tika te kōrero ki tō tākuta.

Kaua e tatari mena ka kite koe kāore tō tamaiti e tipu ana, e piki ana rānei te taumaha i te mea e tumanakohia ana, ina koa mena ka puta tēnei me ngā tohu pūkahukahu. Ko te whakatau whakamutunga me te maimoatanga ka ārai i ngā raruraru ka āwhina i tō tamaiti ki te tipu.

He aha ngā take tūponotanga mō te mate o Hirschsprung?

He maha ngā take ka whakanui ake i te tūponotanga o te whānautanga o te pēpi me te mate o Hirschsprung. Ko te mārama ki ēnei take tūponotanga ka āwhina i ngā whānau me ngā tākuta kia tupato ki ngā tohu whakamutunga.

Ko te mea he tane te take tūponotanga tino kaha – e whā wā te tūponotanga o ngā tamaiti ki te whiwhi i tēnei āhuatanga i ngā kōhine. Kāore i tino mārama te take mō tēnei rerekētanga ira tangata, engari ka kitea tonu i ngā taupori rerekē.

He wāhanga nui te hītori whānau. Ko te whai i te mātua, te teina rānei me te mate o Hirschsprung ka whakanui ake i te tūponotanga. He nui ake te tūponotanga mena he wahine te mema whānau e pā ana, kei te momo roa-wāhanga rānei o te mate.

E hono ana ētahi āhuatanga ira ki te mate o Hirschsprung, tae atu ki te mate Down, e puta ana i te 2-10% o ngā tamariki me tēnei āhuatanga pūkahukahu. Ko ētahi atu mate ira penei i te mate Waardenburg me te mate hypoventilation pokapū whānau ka whakanui ake te tūponotanga.

Ka taea e ētahi whakarerekētanga ira onge te whakaputa i ngā whānau kia nui ake te tūponotanga, ahakoa ko ēnei anake te iti o ngā take. Ko te nuinga o ngā tamariki me te mate o Hirschsprung kāore he whakarerekētanga ira motuhake.

He aha ngā raruraru pea o te mate o Hirschsprung?

Ahakoa he tino maimoatia te mate o Hirschsprung, ka taea te arahi ki ngā raruraru nui mena kaore i whakatauhia, i whakahaeretia rānei tika. Ko te mōhio ki ēnei raruraru pea ka āwhina i te maimoatanga tere.

Ko te raruraru tino nui ko te āhuatanga e kiia nei ko te enterocolitis, ko te mumura o ngā pūkahukahu. Ka taea tēnei te puta i mua, i muri rānei i te pokanga ā, e hiahia ana ki te tiaki hauora tonu. Ko ngā tohu ko te kirika, te korere pakaru, te ruaki, me te puku pupuhi.

Ko te megacolon poino he raruraru nui atu e nui haere ana, e mumura ana te colon. Ka taea e tēnei āhuatanga morearea te pakaru i te pakitara pūkahukahu mena kaore i maimoatia tere me ngā rongoā, i etahi wā hoki te pokanga āwangawanga.

Ka taea te whānau i ngā raruraru tipu me te kai ina kore e taea e ngā tamariki te tango pai i ngā matūkai na te raru pūkahukahu tonu. I etahi tamariki pea ka hiahia ki te tautoko kai motuhake, ki ngā taapiri rānei hei āwhina i a rātou ki te tipu noa.

I muri i te pokanga, i etahi tamariki pea ka wheako tonu i ngā wero penei i te mate pūkahukahu roa, ngā aituā paru, te uaua rānei ki te whakangungu wharepaku. Heoi, mā te manawanui me te tiaki whai muri tika, ka pai ake te nuinga o ēnei take i te wā.

Ko te ki hei ārai i ngā raruraru ko te whakatau whakamutunga, te maimoatanga pokanga tika, me te whai muri tonu me te rōpū hauora o tō tamaiti. Ko te nuinga o ngā tamariki e whiwhi ana i te tiaki tika ka haere tonu ki te noho oranga noa, hauora.

Me pēhea te whakatau i te mate o Hirschsprung?

Ko te whakatau i te mate o Hirschsprung e whakauru ana i ngā whakamātautau maha e āwhina ana i ngā tākuta ki te kite i te pai o te mahi o te colon me te whai rānei i ngā pūtau io. Ko te tukanga he tino ngāwari, ahakoa pea ka hiahia ki ngā whakamātautau rerekē hei tiki i te pikitia katoa.

Ka tīmata tō tākuta me te tirotiro tinana me te hītori hauora taipitopito. Ka ui rātou mō ngā nekehanga kakau, ngā tikanga whāngai, me ngā tohu katoa kua kitea e koe. Ka kitea e rātou te puku o tō tamaiti hei tirotiro i te pupuhi, i ngā wāhanga ngawari rānei.

Ko te barium enema te whakamātautau whakaahua tuatahi e mahihia ana. Ka inu tō tamaiti, ka whiwhi rānei i te wai motuhake e whakaatu ana i ngā X-rays, e āwhina ana i ngā tākuta ki te kite i te ahua me te mahi o te colon. I te mate o Hirschsprung, ka whakaatu tonu tēnei whakamātautau i te wāhanga paku i whai ake i te wāhanga whānui ake i runga.

Ko te whakatau whakamutunga ka puta mai i te biopsy tūturu, kei hea ngā tākuta e tango ana i te wahi iti o te kiko mai i te pakitara tūturu hei tirotiro i raro i te microscope. Ka taea e tēnei whakamātautau te whakaatu tika mena kei te whai, kei te ngaro rānei ngā pūtau io.

I etahi wā ka whakamahi ngā tākuta i te manometry anorectal, he whakamātautau e ine ana i te pēhanga me te mahi uaua i te tūturu me te tūturu. Ka taea tēnei te āwhina ki te whakatau i ngā mahi uaua kore noa e tohu ana i te mate o Hirschsprung.

He aha te maimoatanga mō te mate o Hirschsprung?

Ko te maimoatanga matua mō te mate o Hirschsprung ko te pokanga hei tango i te wāhanga o te colon kāore he pūtau io, ka hono anō ngā wāhanga hauora. Ahakoa ka ahua nui tēnei, he tino angitu ēnei mahi pokanga ka āwhina i ngā tamariki ki te hoki ki te mahi kakau noa.

Ko te nuinga o ngā tamariki he tukanga "pull-through", kei hea ngā tākuta pokanga e tango ana i te wāhanga o te colon e pā ana ka toia te wāhanga hauora ki raro ki te hono ki te tūturu. Ka taea tonu tēnei te mahi i roto i tetahi mahi pokanga kotahi, ina koa mō te mate poto-wāhanga.

I etahi tamariki, ina koa ētahi me te mate whānui ake, ki ngā raruraru rānei, pea ka hiahia ki te colostomy wā poto tuatahi. Ka hanga tēnei i te tuwhera i te pakitara puku kei hea ka taea te puta te paru ki te putea kohikohi, e hoatu ana ki te pūkahukahu iti te wā ki te okioki me te ora i mua i te pokanga matua.

Ko te pokanga e mahihia ana e ngā tākuta pokanga tamariki e tohunga ana ki ēnei momo mahi. Ka taea te mahi i te nuinga o ngā tukanga mā ngā tikanga iti-whakauru, ko te tikanga he iti ake ngā tapahi me ngā wā ora tere ake.

I muri i te pokanga, ka hoki te nuinga o ngā tamariki ki te mahi kakau noa, ahakoa pea ka torutoru marama te wā kia noho katoa ki te tauira noa. Ka hoatu e tō rōpū pokanga ngā tohutohu taipitopito mō te tiaki muri-pokanga me te whai muri.

Me pēhea te whakahaere i te tiaki whare i te wā ora?

Ko te tiaki i tō tamaiti i te kāinga i muri i te pokanga e whakauru ana i te whai i ngā tohutohu o tō rōpū hauora me te titiro ki ngā tohu o te ora, ki ngā raruraru pea rānei. Ko te nuinga o ngā whānau e kite ana mā te whakarite tika, ka pai te ora o te kāinga.

Kia ma, kia maroke te wāhi pokanga kia rite ki ngā tohutohu a tō tākuta pokanga. Pea ka hiahia koe ki te whakarereke i ngā kakahu i ia wā ka titiro ki ngā tohu o te mate penei i te whero nui ake, te pupuhi, te tukunga rānei. Kaua e whakaroa ki te karanga atu ki tō tākuta mena he awangawanga tōu.

Pea ka hiahia ki te whakarereke i te whāngai i te tīmatanga, ina koa mena he colostomy tō tamaiti. Ka hoatu e tō rōpū hauora ngā aratohu motuhake mō ngā kai hei tuku me te wā hei whakanui i te kai. Tīmata haere haere, titiro ki te pehea te manawanui o tō tamaiti ki ngā kai rerekē.

He mea nui te whakahaere mamae mō te whakamarie me te ora. Hoatu ngā rongoā kia rite ki te whakahau me te kaua e ngaro ngā rāihana ahakoa e ahua whakamarie ana tō tamaiti. Ko te whai i te mahinga whakahaere mamae pumau ka āwhina i ngā tamariki ki te ora tere ake.

Tirohia ngā tohu o ngā raruraru penei i te kirika, te ruaki tonu, ngā huringa rānei i ngā nekehanga kakau e awangawanga ana koe. Ka hoatu e tō rōpū pokanga he rārangi o ngā tohu whakatūpato me ngā korero whakapā āwangawanga.

Mahara ko ia tamaiti e ora ana i tōna tere ake. I etahi pea ka hoki ki ngā mahi noa i roto i ngā wiki torutoru, i etahi atu ka hiahia ki te wā roa atu. Whai i te ara o tō tamaiti, kaua e tere te tukanga ora.

Me pēhea te whakarite mō tō hui tākuta?

Ko te whakarite mō tō hui ka āwhina i te whakarite ka whiwhi koe i ngā korero tino whai hua ka mahi i ngā whakatau pai mō te tiaki o tō tamaiti. Ka taea e te whakahaere iti i mua te whakanui i te hua o tō haerenga.

Kia mau he rekoata taipitopito o ngā nekehanga kakau o tō tamaiti, tae atu ki te auau, te āhua, me ngā tauira katoa e kitea ana e koe. Arapā hoki ngā tikanga whāngai, ngā huringa taumaha, me ngā tohu penei i te ruaki, i te mamae puku rānei. Ka āwhina ēnei korero i ngā tākuta ki te mārama ki te mea kei te puta.

Tuhia katoa ō patai i mua i te hui kia kaua e wareware i tetahi mea nui. Whakauruhia ngā patai mō te whakatau, ngā whiringa maimoatanga, te mea e tumanakohia ana i te wā ora, me ngā awangawanga katoa mō ngā hua roa.

Kawe mai he rārangi katoa o ngā rongoā, ngā taapiri rānei e tangohia ana e tō tamaiti, tae atu ki ngā rāpoi me te auau e hoatu ana. Whakahua hoki ngā mate pāwera, ngā whakautu o mua rānei ki ngā rongoā.

Mena e taea ana, kawe mai tētahi mema whānau, tētahi hoa rānei mō te tautoko, ina koa mena kei te kōrero koe mō te pokanga, mō ngā mahere maimoatanga uaua rānei. Ko te whai i tētahi atu e whakarongo ana ka āwhina i a koe ki te mahara ki ngā korero ka whakarato i te tautoko ngākau.

Kaua e wehi ki te tono mō ngā korero tuhituhi, ngā rauemi rānei mō te āhuatanga o tō tamaiti. He maha ngā whānau e kite ana he mea whai hua te whai i ngā rauemi ka taea te arotake i te kāinga ina kore e ngākau nui.

He aha te tango matua mō te mate o Hirschsprung?

Ko te mate o Hirschsprung he hapa whānau e taea te maimoatanga e pā ana ki te kaha o te colon ki te neke i te paru i te tikanga. Ahakoa ka ahua nui te whakatau i te tīmatanga, ko te nuinga o ngā tamariki e whiwhi ana i te maimoatanga tika ka haere tonu ki te noho oranga noa, hauora.

He mea nui te mohiotanga whakamutunga me te maimoatanga mō ngā hua pai. Mena ka kite koe i ngā tohu penei i ngā nekehanga kakau whakamutunga i ngā pēpi hou, i te mate pūkahukahu tonu rānei i ngā tamariki tawhito, kaua e whakaroa ki te kōrero i ēnei awangawanga ki tō tākuta.

He tino angitu te pokanga ki te maimoatanga i tēnei āhuatanga, ā, ka kite te nuinga o ngā tamariki i te pai nui o ō rātou tohu i muri mai. Ahakoa ka roa te wā ora, te manawanui rānei, ka kite te nuinga o ngā whānau kua hoki te ora ki te noa i roto i ngā marama torutoru o te maimoatanga.

Mahara ko te whai i te mate o Hirschsprung kaore e ārai i te mea ka taea e tō tamaiti te whakatutuki i te ora. Mā te tiaki hauora tika me tō tautoko aroha, ka uru ngā tamariki me tēnei āhuatanga ki te kura, ki ngā hākinakina, ki ngā mahi katoa e whakapai ana i te tamarikitanga.

Ngā Pātai Nui e Pā ana ki te Mate o Hirschsprung

Ka taea e taku tamaiti te whakahaere i ōna nekehanga kakau i te tikanga i muri i te pokanga?

Ka whānau te nuinga o ngā tamariki i te whakahaere kakau noa i muri i te pokanga, ahakoa pea ka torutoru marama ki te tau te wā kia noho katoa. I etahi tamariki pea ka hiahia ki te āwhina atu ki te whakangungu wharepaku, ki te whakahaere rānei i ngā aituā i ia wā, engari ko te nuinga o te nuinga ka taea te whakahaere pai i te wā. Ka mahi tahi tō rōpū pokanga ki a koe hei whakautu i ngā awangawanga tonu ka whakarato i ngā rautaki hei āwhina i tō tamaiti ki te angitu.

Ka taea te ārai i te mate o Hirschsprung?

Kāore he huarahi hei ārai i te mate o Hirschsprung inaianei, na te mea he āhuatanga whanaketanga e puta ana i te wā hapu. Heoi, mena he hītori whānau tōu mō te āhuatanga, ka taea e te tohutohu ira i mua i te hapu te āwhina i a koe ki te mārama ki ngā take tūponotanga o tō whānau. Ko te mea nui ko te mohiotanga whakamutunga me te maimoatanga ina whānau te tamaiti.

E hia te roa o te pokanga me te pēhea te ora?

Ko te pokanga pull-through e torutoru haora ki te 4 haora te roa, kei runga i te nui o te colon e pā ana. Ko te nuinga o ngā tamariki e noho ana i te whare hauora mō ngā rā 3-7 i muri i te pokanga. Ko te ora katoa e torutoru wiki ki ngā marama torutoru te roa, kei hea ka hoki haere haere tō tamaiti ki ngā mahi noa me te kai. Ka hoatu e tō rōpū pokanga he waikōrero taipitopito me te mahere ora.

Ka hiahia rānei taku tamaiti ki ngā herenga kai motuhake i muri i te maimoatanga?

Ka taea e te nuinga o ngā tamariki te hoki ki te kai noa katoa i muri i te pokanga me te ora. I te tīmatanga, pea ka tūtohutia e tō tākuta te tīmatanga me ngā kai ngāwari-ki-te-teitei ka whakanui haere haere i te maha. I etahi tamariki ka whai hua mai te kai iti ake, te maha atu rānei o ngā kai, te whakauru rānei i te nui atu o te muka ki ō rātou kai hei tautoko i ngā nekehanga kakau hauora, engari kāore ēnei he herenga uaua.

He aha ngā tūponotanga ka whai ano ngā tamariki a muri ake nei i te mate o Hirschsprung?

Mena he tamaiti kotahi tōu me te mate o Hirschsprung, he nui ake te tūponotanga mō ngā tamariki a muri ake nei i te taupori whānui, engari he iti tonu. Ko te tūponotanga tika e whakawhirinaki ana ki ngā take penei i te ira tangata o te tamaiti e pā ana, te nui o tōna mate rānei, engari ko te tikanga kei waenga i te 3-12%. Ka taea e tō tākuta te whakarato i ngā korero tūponotanga motuhake ake i runga i te āhuatanga o tō whānau ā, pea ka tūtohutia te tohutohu ira.