Created at:1/16/2025
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Ko te mate Ehlers-Danlos (EDS) tētahi rōpū o ngā āhuatanga kua oti te whakawhiwhi e pā ana ki ngā kiko hono o tō tinana – ko te ‘kāpia’ e pupuri ana i tō kiri, ngā hononga, ngā oko toto, me ngā whekau. Whakaarohia te kiko hono hei anga o tētahi whare; ki te kore e kaha penei i te mea e tika ana, ka taea e ngā wāhanga rerekē o tō tinana te ngoikore ake, te ngāwari rānei ki te pakaru atu i te mea noa.
He rerekē te pānga o tēnei āhuatanga ki te tangata katoa, ā, he tokomaha ngā tāngata e ora ana i te ora, i te mahi kaha me te tautoko tika me te whakahaere. Ahakoa he iti te maha, e pā ana ki te 1 i roto i ngā 5,000 o te tangata, ko te mārama ki ō tohu ka āhei te awhina i a koe ki te mahi tahi me ngā kaiwhakarato hauora kia kitea te huarahi pai ki mua.
Ka puta te mate Ehlers-Danlos ina kore tō tinana e hanga pai i te collagen. Ko te collagen te anga e homai ana te hanganga me te kaha ki tō kiri, ngā hononga, me ngā whekau. Ki te kore tenei anga i hanga tika, ka taea e ia te whakahohe i enei kiko kia ngāwari ake te piko, te ngoikore rānei ki te whara atu i te mea e tika ana.
E 13 ngā momo EDS rerekē, ia momo e pā ana ki te tinana i ngā huarahi iti te rerekē. Ko te momo tino noa, ko te hypermobile EDS, e pā ana ki ō hononga, ā, ka whakahohe i a rātou kia piko rawa.
Ko te nuinga o ngā momo EDS ka tukuna i roto i ngā whānau, ahakoa ka puta pea i te wa tuatahi i te tangata kahore he hītori whānau. Ko te āhuatanga e pā ana ki te iwi katoa, ā, ka rereke ngā tohu mai i te ngawari ki te wero ake.
Ko ngā tohu ka pā ki a koe e whakawhirinaki ana ki te momo EDS kei a koe, engari he tohu whānui kei te kitea e te tini o te iwi. Ka ngawari pea ō tohu, kāore rānei e kitea, ka nui ake rānei te kitea, ā, ka pā ki tō oranga o ia rā.
Ko ngā tohu tino noa ko:
He maha ngā tāngata e pā ana ki ngā raruraru o te pūnaha kai, te pupuhi o te ngakau, te māuiui rānei ina tū.
Ka taea e ētahi momo EDS onge te whakahohe i ngā tohu nui ake e pā ana ki tō ngakau, ngā oko toto, ngā whekau rānei. Ki te pāngia koe e te mamae nui o te uma, ngā huringa tirohanga ohorere, ngā tohu rānei e awangawanga ana, he mea nui kia rapu āwhina hauora tonu.
E 13 ngā momo EDS e mohiotia ana, ia momo he tauira ake o ngā tohu. Ko te mārama ki te momo kei a koe ka āhei te awhina i tō rōpū hauora ki te whakarato i te tiaki me te tirotiro tika.
Ko ngā momo tino noa ko:
He tohu tātai motuhake ia momo, ā, ka taea te whakau i ētahi mā te whakamātautau ira, engari ka taea te whakatau i ētahi atu i runga i ngā tohu hauora. Ka taea e tō tākuta te awhina ki te whakatau i te momo e pai ana ki ō tohu me tō hītori whānau.
Ko ngā momo EDS onge pea ka pā nui ake ki ngā pūnaha tinana motuhake, pēnei i tō whakarongo, ngā niho, te mahi ngakau rānei. Ko ēnei momo e hiahia ana ki te tiaki motuhake mai i ngā tohunga hauora maha.
Ko te EDS te take o ngā huringa (mutations) i roto i ō ira e whakahaere ana i te hanga collagen e tō tinana. Ko ēnei huringa ira e tuku ana i te nuinga mai i tētahi mātua, i ngā mātua rānei, ahakoa ka puta pea i te wa ohorere kahore he hītori whānau.
He ira rerekē te kawenga mō ngā momo EDS rerekē. Hei tauira, ko ngā huringa i roto i ngā ira COL5A1, COL5A2 e whakahohe ana i te classical EDS, ko ngā huringa i roto i te ira COL3A1 e whakahohe ana i te momo vascular tino kino.
Ko te nuinga o ngā momo EDS e whai ana i te tauira e kiia nei he autosomal dominant. Ko te tikanga tenei me whiwhi koe i tētahi ira kua huri mai i tētahi mātua kia whiwhi ai i te āhuatanga. Heoi, ko ētahi momo onge e hiahia ana ki te whiwhi i ngā ira kua huri mai i ngā mātua e rua.
Ki te pāngia koe e te EDS, he 50% pea te tupono o te tuku atu ki ō tamariki, ahakoa ka rerekē tenei e whakawhirinaki ana ki te momo motuhake. Ka taea e te tohutohu ira te awhina i a koe ki te mārama ki tō āhuatanga motuhake me ō kōwhiringa whakatōpū whānau.
Me whakaaro koe ki te kōrero ki tētahi kaiwhakarato hauora ki te kite koe i ngā tohu maha e āhei ana te tohu i te EDS, ina koa ki te pā ana ki tō kounga o te ora. Ko te mohiotanga wawe ka taea te awhina i a koe ki te whiwhi i te tiaki tika me te aukati i ngā raruraru.
Whakahaere he whakatau ki te pāngia koe e:
Rapua te awhina hauora tonu ki te pāngia koe e ngā tohu e āhei ana te tohu i te vascular EDS, pēnei i te mamae ohorere nui o te uma, te kopu rānei, ngā huringa tirohanga ohorere, ngā mamae o te mahunga nui rānei. Ahakoa he onge te vascular EDS, e hiahia ana ki te aromatawai tonu.
Kaua e manukanuka ki te ngawari o ō tohu – he tokomaha ngā tāngata e ora ana i te ora noa me te whakahaere tika. Ko te whiwhi i te whakatau ka taea te whakamarie me te awhina i a koe ki te whiwhi i te tautoko me te maimoatanga tika.
Ko te take morearea matua mō te EDS ko te whai i te hītori whānau o te āhuatanga. Mai i te nuinga o ngā momo e tukuna ana, ko te whai i tētahi mātua me te EDS e nui ake ai ō tupono ki te whiwhi i te āhuatanga.
Heoi, he maha ngā take ka pā ki te pānga o te EDS ki a koe:
He mea nui te mahara kahore te whai i ngā take morearea e tikanga ana ka whanake koe i ngā raruraru. He tokomaha ngā tāngata me te EDS e ora ana i te ora kaha, i te whakaoti pai rānei mā te ako ki te whakahaere i ō rātou tohu.
I etahi wā ka puta te EDS i roto i ngā whānau kahore he tangata atu e pā ana. Ka puta pea tenei nā te mea he ngawari ake ngā tohu o ngā whakatupuranga o mua kahore i kitea, i ngā huringa ira hou rānei.
Ahakoa he tokomaha ngā tāngata me te EDS e whakahaere pai ana i tō rātou āhuatanga, he mea whai hua te mārama ki ngā raruraru pea kia taea ai te mahi tahi me tō rōpū hauora ki te aukati i a rātou. Ko te nuinga o ngā raruraru ka taea te whakahaere ina kitea wawe.
Ko ngā raruraru whānui ka pā atu ki a koe ko:
He raruraru nui ake pea, engari he iti rawa te maha. Ka uru pea ēnei ki ngā raruraru o te waahanga ngakau, te whānui o te aorta, te pakaru whekau rānei i roto i ētahi momo EDS, ina koa te vascular EDS.
Ko te rongo pai ko te tirotiro auau me te tiaki aukati ka taea te awhina i te kite i ngā raruraru pea wawe. Ko te mahi tata atu ki tō rōpū hauora ka taea te awhina i a koe ki te whakarite i ngā raruraru me te pupuri i tō kounga o te ora.
Mai i te mea he āhuatanga ira te EDS, kaore e taea te aukati i te puta. Heoi, ka taea e koe te mahi i ngā mahi maha ki te aukati i ngā raruraru me te whakahaere i ō tohu, ka taea te whakarereke nui i te pānga o te āhuatanga ki tō ora.
Arotakehia ēnei rautaki aukati:
Ki te whakatōpū koe i tētahi whānau, ā, kei a koe te EDS, ka taea e te tohutohu ira te awhina i a koe ki te mārama ki ngā morearea me ngā kōwhiringa e wātea ana. Kāore tenei e tikanga me kore koe e whānau – he tokomaha ngā tāngata me te EDS he mātua whakamiharo e whakahaere pai ana i tō rātou āhuatanga me te ora whānau.
Ko te mohiotanga wawe me te whakauru i te nuinga ka āhei te whakaputa i ngā hua pai ake. Ki te rere te EDS i roto i tō whānau, ko te noho mataara ki ngā tohu i roto i ngā tamariki ka taea te awhina i a rātou ki te whiwhi tautoko i mua atu i te muri.
Ko te whakatau i te EDS e hiahia ana ki te aromatawai tika i ō tohu, tō hītori whānau, me te whakamātautau tinana. Kāhore he whakamātautau kotahi e taea te whakatau i ngā momo EDS katoa, no reira ka whakamahi tō tākuta i ngā tohu motuhake i whakawhanakehia e ngā tohunga i roto i te mara.
Ko tō kaiwhakarato hauora ka tīmata i te nuinga mā te:
Mō ētahi momo EDS, ka taea e te whakamātautau ira te whakau i te whakatau. Heoi, mō te hypermobile EDS (te momo tino noa), ko te whakatau i runga i ngā tohu hauora no te mea kāore ano kia mohiotia te take ira.
Ka tuku pea tō tākuta ki ngā tohunga pēnei i ngā tohunga ira, ngā tohunga rheumatologists, ngā tohunga ngakau rānei mō te aromatawai atu. Ko tēnei huarahi whānui e whakarite ana ka whiwhi koe i te whakatau tika me te tirotiro tika mō tō momo EDS motuhake.
Ahakoa kāhore he rongoā mō te EDS, he maha ngā maimoatanga whai hua e taea te awhina i a koe ki te whakahaere i ngā tohu me te aukati i ngā raruraru. Ko te matua ko te kimi i te huinga tika o ngā huarahi e mahi ana mō tō āhuatanga motuhake.
Ko te maimoatanga e arotahi ana ki te nuinga ki te:
Ka uru pea tō rōpū maimoatanga ki ngā tohunga rongoā tinana, ngā tohunga rheumatologists, ngā tohunga ngakau, me ētahi atu tohunga e whakawhirinaki ana ki ō tohu. Ko tēnei huarahi rōpū e whakarite ana ka whakautuhia ngā āhuatanga katoa o tō āhuatanga.
He tokomaha ngā tāngata e kite ana ko te whakakotahi i ngā maimoatanga hauora me ngā whakarereketanga oranga e homai ana te hua pai rawa. Ko te mea e mahi ana ka rerekē mai i te tangata ki te tangata, no reira kaua e pouri ki te roa te kimi i tō mahere maimoatanga pai.
Ko te whakahaere kāinga he wāhanga nui o te ora pai me te EDS. Ko ngā whiringa o ia rā e mahi ana koe ka taea te pānga nui ki te āhua o tō ngakau me tō mahi, ā, he tokomaha ngā tāngata e kite ana i te angitu nui me ngā rautaki tiaki whaiaro auau.
Ko te whakahaere kāinga whai hua e whakauru ana ki te:
Ko te ako ki te whakarongo ki tō tinana he mea nui. I ētahi rā ka pai pea koe, ka hiahia rānei koe ki te mahi atu, i ētahi atu rā pea ka hiahia koe ki te okioki atu. E rua ngā mea noa me te EDS.
Whakaarohia te pupuri i tētahi tātai tohu hei kimi i ngā tauira i roto i te mea e awhina ana, e whakaiti ana rānei i ō tohu. Ka taea te whakamahi i tēnei mōhiohio ina mahi tahi me tō rōpū hauora hei whakarereke i tō mahere maimoatanga.
Ko te whakarite mō tō whakatau ka taea te awhina i a koe ki te whakamahi pai i tō wā me tō kaiwhakarato hauora. Ko te whakarite pai i te nuinga ka āhei te whakaputa i ngā kōrero hua ake me te tiaki pai ake.
I mua i tō whakatau:
Kaua e manukanuka ki te whai i ngā whakautu katoa, ki te whakamārama pai rānei i ngā mea katoa. Kei reira tō tākuta hei awhina i te whakakotahi i te mīharo me te arataki i a koe ki te whakahaere pai ake i ō tohu.
Whakaarohia te kawe mai i tētahi hoa whakawhirinaki, tētahi mema whānau rānei ki te whakaaro koe he mea whai hua. Ka mahara pea rātou ki ngā mōhiohio, ka whakarato rānei i te tautoko ngakau i te whakatau.
Ko te mate Ehlers-Danlos he āhuatanga e taea te whakahaere e pā ana ki te tangata katoa i ngā huarahi rerekē. Ahakoa ka whakaputa pea i ngā wero, he tokomaha ngā tāngata me te EDS e ora ana i te ora, i te mahi kaha rānei me te tautoko tika me te huarahi maimoatanga.
Ko ngā mea tino nui hei mahara ko te mohiotanga wawe e āhei ana te whakaputa i ngā hua pai ake, ā, he maha ngā maimoatanga whai hua e wātea ana. Ko te mahi tahi me ngā kaiwhakarato hauora mohio me te ako ki te whakahaere i ō tohu ka taea te whakarereke nui ki tō kounga o te ora.
Kāore koe i te kotahi anake i te whakahaere i te EDS. He hapori tautoko, ngā rauemi whai hua, me ngā tohunga hauora e mārama ana ki te āhuatanga. Me te manawanui, te whakatairanga i a koe, me te pūnaha tautoko tika, ka taea e koe te whakahaere pai i te ora me te EDS.
Ko te nuinga o ngā momo EDS ehara i te mate kino, ā, e ora ana te iwi i ngā oranga noa. Heoi, ko te vascular EDS (te momo onge rawa) ka nui ake te kino nā te mea e pā ana ki ngā oko toto me ngā whekau. Ahakoa me te vascular EDS, he tokomaha ngā tāngata e ora ana me te tirotiro tika me te tiaki. Ka awhina tō rōpū hauora i a koe ki te mārama ki tō āhuatanga motuhake me ētahi atu whakatūpato pea ka hiahia koe ki te tango.
Ae, he tino whai hua te whakakori mō te nuinga o ngā tāngata me te EDS. Ko te matua ko te whiriwhiri i ngā momo whakakori tika me te mahi haumaru. Ko ngā mahi iti te pānga pēnei i te kauhoe, te yoga ngawari, te whakakori kaha rānei ka taea te awhina i te pumau i ō hononga me te whakaiti i te mamae. Ka taea e tō tohunga rongoā tinana te awhina i a koe ki te whakawhanake i tētahi kaupapa whakakori e haumaru ana, e whai hua ana hoki mō ō hiahia motuhake.
E pā ana te EDS ki te iwi i ngā huarahi rerekē puta noa i ō rātou oranga. He tokomaha ngā tāngata e kite ana i ō rātou tohu e pai ake ana i te ako i ngā rautaki whakahaere pai ake, i ētahi atu pea ka kite i te nui ake o te ngoikore o ngā hononga, te mate rānei i te wā. Ko te rongo pai ko te whakahaere tika, he tokomaha ngā tāngata e pupuri ana i tō rātou kounga o te ora tae noa ki ō rātou tau maha. Ko te tiaki hauora auau me te noho kaha ka taea te awhina i te aukati i ngā raruraru.
He tokomaha ngā tāngata me te EDS e whai ana i ngā haputanga angitu, ahakoa he mea nui te mahi tata atu ki ngā kaiwhakarato hauora e mārama ana ki te āhuatanga. Ka taea e ētahi tohu te kino ake i te wā hapu nā ngā huringa homoni, ā, pea ka hiahiatia ētahi atu whakatūpato e whakawhirinaki ana ki tō momo EDS. Ko te kōrero ki tō tākuta me tētahi tohunga hauora whaea-pēpi ka taea te awhina i te whakarite i te haputanga haumaru rawa.
Ka taea e ngā tamariki me te EDS te whakaatu i ngā tohu pēnei i te piko o ngā hononga, ngā whara maha, te kiri ngawari, te whara ngāwari rānei. Ka amuamu pea rātou ki te mamae o ngā hononga, ka ngenge rānei. Heoi, he maha ngā tamariki e piko ana, no reira he mea nui te titiro ki ngā tauira me te kōrero i ngā awangawanga ki tō tākuta tamariki. Ko te mohiotanga wawe ka taea te awhina i ngā tamariki ki te whiwhi tautoko tika me te ako ki te whakahaere i ō rātou tohu mai i te tamarikitanga.