Kua pāngia koe e te mamae puku kino kāore i taea e koe te whakaaro? Kāore rānei koe e rongo i te mamae e kore e taea te whakamanawanui pērā i te pupuhi me te wera ina haere koe i te rā? Kei muri i ēnei mea, tera pea he mate onge e kiia nei ko 'Porphyria', kāore anō kia rongohia e te tini o te tangata o tō tātou whenua. Tērā pea he ahua rerekē te ingoa, engari he āhuatanga tēnei i puta mai i tētahi tukanga matū i roto i tō tātou tinana e hē ana. Nō reira, me kōrero noa tātou mō tēnei, me te mea kei te kōrero tātou ki tētahi hoa.

He aha te tikanga o te Porphyria?

Hei mārama ki tēnei, me titiro tuatahi tātou ki ō tātou toto. He whero te tae o ō tātou toto, ā, ka haria te hāora puta noa i te tinana nā te mea kei roto he matū motuhake e kiia nei ko te 'heme' . Ko te whakaputanga o tēnei 'heme' i roto i ō tātou tinana he rite ki te mahi a tētahi wheketere nui. He tukanga uaua tēnei e waru (8) ngā taahiraa e mahia ana.

Whakaarohia te tunu kai. Me tāpiri e koe ngā mea rerekē i ngā taumata rerekē, taahiraa i te taahiraa. Waihoki, he pūmua motuhake e kiia nei ko ngā whākōkī hei āwhina i ia taahiraa o te hanga i tēnei 'heme'. He whākōkī rerekē mō ia taahiraa.

Ka puta te porphyria ina pakaru tetahi o ēnei taahiraa e waru, ā, kāore te whākōkī e kawenga ana mōna e mahi tika ana. Ko te tikanga o tēnei, ko ngā matū e kiia nei ko ngā porphyrin me ngā matū whakataki porphyrin, e puta ana i te wā o te tukanga hanga heme, kāore e tangohia mai i te tinana, engari ka tīmata te kohikohi ki roto i ngā pūtau. He rite ki te pakaru o te mīhini i runga i te raina whakaputa wheketere, ka puranga ngā taonga. Ko te kohikohinga o ēnei matū ki roto i te tinana te take o ngā tohu maha.

I te nuinga o te wā, ko te take o ēnei ngoikoretanga whākōkī he whakarerekētanga ira e ahu mai ana i roto i ngā whānau. Ko te tikanga tēnei he tūponotanga ka ahu mai tēnei mate i roto i ngā whānau. Engari kaua e manukanuka, ka taea e ngā tākuta mohio te āwhina i te tautuhi i te mate, te whakahaere i ngā tohu, me te whakaiti i te pānga ki tō oranga.

He aha ngā momo matua o te porphyria?

E waru pea ngā momo matua o te porphyria. Engari ka taea te wehewehe katoa i ēnei ki ngā rōpū matua e rua i runga i ō rātou tohu. He tino māmā tēnei ki te maumahara.

Nā, kia titiro tātou ki ngā momo āhua o ēnei kāwai e rua.

Ngā momo Porphyria Whakapeka

Koinei ngā momo e puta ai ngā "whakaeke" ohorere.

• Te Porphyria Wawe Koretake (AIP): Koinei te momo tino noa o ngā momo koretake. Ka puta he mamae puku nui, engari kāore he raruraru kiri.
• Te Coproporphyria Tuku Iho (HCP): Ka pā mai te mamae o te puku me te pupuhi o te kiri ina pāngia e te rā.
• Te Porphyria Momo Rerekē (VP): Ka taea hoki e tēnei te mamae o te puku me te pupuhi kiri.
• ALAD-Deficiency Porphyria: He tino onge tēnei. Ko te tikanga ka puta ngā tohu i te tamarikitanga.

Ngā momo Porphyria Kiri

Koinei ngā momo e pā ana ki te kiri. E rua ngā wāhanga o ēnei: ​​ko ngā mea e puta ai ngā mirumiru me ngā mea e puta ai te mamae kāore he mirumiru.

Te porphyria kiri pupuhi:

• Te Tarda Kutikuti Porphyria (PCT): Koinei te momo porphyria tino noa, ā, ka puta i te nuinga o te wā i te pakeketanga.
• Te Porphyria Erythropoietic Congenital (CEP): He tino onge tēnei mate, he kino hoki. Ka kitea ngā tohu i te whānautanga mai.

Te porphyria kiri kore-pūwero:

• Ko te Protoporphyria Erythropoietic (EPP) me te Porphyria X-linked (XLP): I roto i ēnei momo e rua, kaua e pupuhi, ka tino mamae te kiri, ka mumura, ka pupuhi, ka whero ina pāngia e te rā.

He aha ngā tohu o tēnei mate?

He rerekē ngā tohu i runga i te momo porphyria kei a koe. He tino ngawari ngā tohu o ētahi tāngata, ko ētahi he kino pea ngā tohu ka mōrearea ki te ora. Tērā pea kei a ētahi tāngata te ira engari kāore rawa e pāngia e tetahi tohu.

Me whakarōpūtia ēnei āhuatanga kia māmā ake ai te mārama.

He aha ngā take me ngā mea e whakaoho ai te porphyria?

E ai ki tā tātou i kōrero ai i mua ake nei, ko te take matua mō tēnei he whakarerekētanga ira. Engari ko te mea nui i konei , ehara i te mea ko te hunga katoa kei a rātou te ira ka pāngia e ngā tohu. He tokomaha te hunga e noho ana hei kawe i tēnei ira, kāore he raruraru.

Kia puta ai he "whakaeke" me ngā tohu o te porphyria whakapeka, me whai "whakaoho" motuhake. Mā ēnei whakaoho ka nui ake te "heme" e hiahiatia ana e te tinana, ā, ka puta te ngoikore o taua tukanga, ka kohia hoki ngā matū kino ki roto i te tinana.

Anei ētahi o ngā mea matua e whakaoho ana i te riri:

• Te pikinga o ngā taumata homoni wahine: Ina koa i ētahi wā o te huringa paheketanga o te wahine ia marama.
• Ētahi rongoā: Hei tauira, ētahi rongoā whakamamae mamae, rongoā whakangote, me ngā pire ārai whānau.
• Te inu nui rawa o te waipiro.
• Te momi hikareti.
• Te whakawhāiti i te kai: Te nohopuku, te kai rānei i tētahi kai tino iti te warowaihā.

Me pēhea te kitenga a te tākuta i tēnei?

Ina kōrero koe ki tō tākuta mō ō tohumate, ka tirotirohia koe e ia, ka pātai atu mēnā kua pāngia tētahi o tō whānau e ngā tohumate rite. Nā te mea he mate onge te porphyria, ka roa pea te wā ki te tautuhi tika i te mate.

Ko ngā whakamātautau taiwhanga te nuinga o te wā hei whakaū i te mate.

• Ngā whakamātautau mimi: I te wā o te "whakaekenga" whakapeka o te porphyria, he tino teitei ngā taumata o ngā matū e kiia nei ko te ALA me te PBG i roto i te mimi. Koinei te whakamātautau matua hei tautuhi i te mate.
• Ngā whakamātautau toto
• Ngā whakamātautau tūtae

Mā ēnei whakamātautau ka āwhina koe ki te whakatau ko tēhea momo porphyria tōu. Kātahi, ki te tika, ka taea te whakahaere i ngā whakamātautau ira.Mā te mahi pēnā, ka taea hoki e tātou te whakaū i te huringa ira i puta ai tēnei.

Me pēhea te rongoā?

Ka rerekē ngā kōwhiringa maimoatanga i runga i te momo porphyria kei a koe me te kaha o ō tohu. Ka whakawhanakehia e tō tākuta he mahere maimoatanga e tika ana māu.

Te Maimoatanga mō ngā Porphyria Whakapeka

Ki te pā mai he "whakaeke" ki a koe, me kawe koe ki te hōhipera, ā, me rongoatia hoki .

• Ka tukuna he rongoā e kīia ana ko 'Hemin' mā roto i te uaua hei whakaiti i ngā taumata o ngā matū kino (ALA me PBG) kua kohia ki roto i te tinana.
• Ka hoatu ētahi atu rongoā hei whakahaere i ngā tohu pēnei i te mamae nui, te whakapairuaki, me te haurangi.
• Ka hoatu he wai tote (wai IV) hei whakatika i te matewai me te taumata tote i roto i te tinana.
• Hei ārai i te hokinga mai o te "whakaekenga", me werohanga marama i te kano kano Givosiran .
• Ko te mea nui ko te karo i ngā mea whakaoho i kōrerotia e tātou i mua ake nei.

Te Maimoatanga mō ngā Porphyria Kiri

I roto i ēnei momo mate, ko te maimoatanga matua, ko te tiaki i te kiri.

Hei tāpiri, kei reira ētahi atu maimoatanga i runga i te momo:

• Mō te EPP/XLP: He mea tāpiri iti kei raro i te kiri, ko 'Afamelanotide' te ingoa. Mā te rongoā e tukuna mai i tēnei ka āwhina i te whakaiti i te mamae e puta mai ana i te pānga o te rā.
• Mō te PCT:
• Whakapūpū toto: Hei tango i te rino nui mai i te tinana, ka tangohia he iti o te toto i ngā wā motuhake .
• Hydroxychloroquine: Ko tēnei rongoā, e whakamahia ana i ngā horopeta iti, ka āwhina i te tinana ki te whakakore i ngā porphyrin kua kohia i roto i te tinana mā te mimi.
• Mō te CEP: He mea nui te tiaki i te rā. Mena he kino te toto toto iti, me whakawhiti toto pea. Ko te huarahi anake hei whakaora i tēnei mate ko te whakawhitinga wheua .

Me pēhea te noho me tēnei āhuatanga?

Kia kitea tō mate porphyria, ka rerekē te āhua o tō oranga. Ka noho hei wāhanga o tō oranga ngā tirotiro auau me tō tākuta. He mea nui tēnei hei aroturuki i te āhua me te kite mēnā ka puta he raruraru.

Ka taea e te noho me tētahi mate onge pēnei te tino taumaha o te hinengaro. I ētahi wā ka whakaaro pea koe he aha tēnei e pā ana ki a koe anake. Kōrero ki tō tākuta, ki tō whānau, ki tētahi hoa pono rānei mō ō kare ā-roto. He āwhina nui anō hoki te whakauru atu ki tētahi rōpū tautoko mō te hunga whai mate pēnei. Kia mahara kāore koe i te mokemoke.