He mea noa te mataku me te ohorere ina kī mai te tākuta kei te pāngia tetahi o tō whānau, inā koa he tamaiti nohinohi, e te mate Pompe. He mate onge, he mate ira, nō reira he mea noa te manukanuka. Engari me kī atu ahau ki a koe, kaua e manukanuka. Ahakoa kāore anō kia kitea he rongoā mō tēnei mate i tēnei wā, he maha ngā maimoatanga whai hua hei āwhina i ngā tūroro kia roa ake te ora me te iti ake o ngā raruraru.
Ko te rongoā matua mō te mate Pompe ko te ERT (Whakaora Whakakapinga Whākōkī).
Ki te kī ngāwari, ko te mate Pompe te take o te korenga o tētahi whākōkī e tika ana mō ngā pūtau uaua o roto i ō tātou tinana. Ina ngaro tēnei whākōkī, ka kohia he momo huka e kiia nei ko te glycogen ki roto i ngā pūtau, ka kino ai ngā uaua. Whakaarohia he iti noa ngā kaimahi e mahi ana i roto i ō tātou tinana, ā, ka kiia rātou he whākōkī. I roto i te mate Pompe, kāore tēnei kaimahi e mahi tika.
Nō reira, ko te Whakaora Whakakapinga Whākōkī (ERT) te wā e hoatu ai e tātou te whākōkī ngaro, te kaimahi rānei, mai i waho o te tinana. Mō tēnei, ka tukuna he rongoā kei roto he whākōkī hangai e kiia nei ko te `alglucosidase alfa` ki te tinana mā roto i te uaua (infus IV). Koinei anake te maimoatanga kua whakaaetia mō te mate Pompe i tēnei wā.
Nā tēnei maimoatanga ERT kua tino whakapiki ake i te roanga o te oranga o ngā tūroro Pompe, inā koa ngā pēpi. I ngā wā o mua, he mea onge te ora o tētahi pēpi me tēnei mate neke atu i te tau.
Ki te kore he maimoatanga ERT, ka mātotoru haere ngā uaua o te ngākau o ngā pēpi e pangia ana e te mate Pompe, ka ngoikore ngā uaua o te tinana, ā, ka mate i roto i te tau tuatahi o te oranga. Ka ngaro hoki i a rātou ngā uaua e hiahiatia ana hei manawa. I muri i te tīmatanga o te maimoatanga ERT, ka tino rerekē tēnei āhuatanga. Ko ngā pēpi i whiwhi tuatahi i te maimoatanga ERT kua tata ki te 20 tau te pakeke inaianei.
He aha te mea nui ai te tīmata wawe i te maimoatanga?
Koinei te mea tino nui. Ka taea e te maimoatanga ERT te aukati i te kino haere o te mate, engari kāore e taea te whakakore i te kino kua pā ki ngā uaua. Ko te tikanga, ki te whakaroahia te maimoatanga, ka ngaro pea tō kaha ki te manawa, ki te hikoi rānei.
Ka ngana ngā tākuta ki te tīmata i te maimoatanga ERT i roto i ngā rā tuatahi o te oranga , inā koa mō ngā pēpi, i muri i te tautuhi i te mate. Ahakoa he iti noa te whakaroa, ka taea e te pēpi te aukati i te hikoi mō ake tonu atu.
Mō te hunga e pāngia ana e te mate Pompe kua tīmata wawe, ka tirotirohia, ka aroturukitia hoki ngā tohu e te tākuta kia mōhio ai ko tēhea te wā pai hei tīmata i te ERT.
Ehara tēnei i te haerenga takitahi - he tautoko mai i tētahi rōpū tohunga
Ahakoa he whakaora ora pea te maimoatanga ERT, kāore e ranea. Hei whakarato i te kounga pai o te oranga mō te tangata e pangia ana e te mate Pompe, me whai rōpū tohunga maha-mātauranga. Ko te tohunga ira haumanu te tangata matua e whakahaere ana i te maimoatanga.
Tirohia te ripanga i raro nei mō ngā momo tohunga e hiahiatia ana mō tēnei me te momo tautoko ka whakaratohia e rātou.
| Tākuta/kaiwhakaora tohunga | He aha te āwhina e whiwhi ana rātou? |
|---|---|
| Tākuta ngakau | Ka aro turukihia ngā pānga ki te ngākau, ā, ka tohua te maimoatanga e tika ana. |
| Tākuta manawa | Ka aroturukihia e rātou ngā uauatanga o te manawa, ā, ka whakaratohia he taputapu whakangāwari manawa mēnā e tika ana. |
| Tohunga kai | He rongoā mō ngā raruraru o te horomitanga me te pūnaha nakunaku. |
| Kaimātai Kai | Whakaratohia te matūkai e tika ana mō te tipu pai, ā, ki te tika, me whāngai motuhake, me whāngai rānei mā te ngongo whāngai. |
| Kaiwhakaora Tinana | Ka whakaratohia ngā mahi whakakori tinana me ngā maimoatanga hei whakapakari i ngā uaua me te whakaiti i te ngoikore. |
| Kaiwhakaora Mahi | Te āwhina i a rātou ki te mahi i ngā mahi o ia rā (pēnei i te kakahu, te kai, me ērā atu) me te takitahi. Mēnā e tika ana, ka akona rātou ki te whakamahi i ngā taputapu āwhina pēnei i te tokotoko. |
| Kaiwhakaora Kōrero | Āwhina ki te wikitoria i ngā uauatanga kōrero e puta mai ana i te ngoikore o ngā uaua o te kanohi me te arero. |
Ētahi atu maimoatanga i tua atu i te ERT
Ngā rongoā e pā ana ki te pūnaha ārai mate
Ka mōhiotia e te tinana o ētahi pēpi tēnei rongoā whākōkī (ERT) e tukuna ana ki waho he 'tauhou', ā, ka tīmata ki te hanga paturopi hei ārai i tēnei. Ka kiia tēnei he āhuatanga 'CRIM-negative'. Ki te tupu tēnei, kāore e pai te mahi a te rongoā ERT.
Hei ārai i tēnei, ka tīmata ngā tākuta i tētahi maimoatanga whakaoho ātete ārai mate (ITI) . I roto i tēnei, me te maimoatanga ERT, ka hoatuhia ētahi rongoā hei whakahaere i te pūnaha ārai mate.
- Rituximab
- Methotrexate
- Immunoglobulin ā-roto-uaua (IVIG)
Ko tā ēnei rongoā he "tinihanga" i te pūnaha ārai mate kia whakaaro ai rātou nō te tinana te ERT. Kātahi ka kore te tinana e whawhai ki te ERT.
Te whakaora tinana me te whakaora mahi
Nā te mea he āhuatanga nui te ngoikore o te uaua o te mate Pompe, he mea nui te whakaora tinana . Ka taea e ia te whakapakari i ngā uaua me te pupuri i te mahi a te tinana. Ka taea hoki e te whakaora mahi te āwhina i te whakawhanake i ngā pūkenga e hiahiatia ana hei mahi i ngā mahi o ia rā me te takitahi.
Te rongoā kōrero me te kai
Nā te ngoikore pea o ngā uaua e hiahiatia ana hei ngaungau me te horomi kai, ka taea e te kaiwhakaora kōrero te āwhina i ngā mahi horomi. Waihoki, i ētahi wā, ka hiahiatia he ngongo whāngai hei whāngai i te pēpi ki te kai e tika ana. Ka taea e te tohunga kai te whakarato i ngā aratohu e tika ana mō tēnei.
He mea tino nui hoki tō tūranga.
I te wā e noho tahi ana koe me te mate Pompe, he mea tino nui tō takoha hei tūroro, hei mātua rānei.
"Ko te mea nui i mahia e au ko te ako mō te mate Pompe. Nā tēnei i homai he nui te māia me te whakamarie i ahau ki te noho tahi me ia rā," te kī a Ryan Colburn, e pāngia ana e te mate.
Nō reira, me mahi tahi koe me tō tākuta kia mārama ai koe ki ō hiahia, ā, kia ārahi hoki koe i te whiwhi i te maimoatanga pai rawa atu. Kaua rawa e wehi ki te pātai atu ki tō tākuta i ō pātai mō tēnei mate, mō tēnei maimoatanga rānei.
Karere Tangohia ki te Kāinga
- Ahakoa kāore e taea te whakaora katoa i te mate Pompe, mā ngā maimoatanga o ēnei rā ka taea e ngā tūroro te ora roa ake, te ora pai ake hoki.
- Ko te rongoā matua ko te rongoā whakakapinga whākōkī (ERT). He rongoā whakaora ora tēnei.
- He mea tino nui kia tīmata wawe tonu te maimoatanga, nā te mea kāore e taea te whakakore i te kino o te uaua.
- Haunga te ERT, he mea nui te tautoko mai i ngā tohunga ngaio pēnei i te whakaora tinana, te kai totika, me te whakaora kōrero.
- Hei tūroro, hei mātua rānei, ka taea e koe te whakatutuki i ngā hua pai rawa atu mā te mōhio ki te mate me te mahi tahi me tō tākuta.
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