Te mamae nui o te puku me te ngaro o te māramatanga mō te kore take... Me ako tātou mō tēnei mate onge? (Acute Hepatic Porphyria)

Whakaarohia, kāore he take mārama, ka pāngia koe e te mamae puku e kore e taea te manawanui. I tua atu i tēnā, ka ngoikore ō waewae, ka rite koe ki te hemo, ā, ka whakapairuaki koe. Ahakoa i muri i te kitenga i ētahi tākuta me te mahi i ngā whakamātautau maha, kāore anō kia kitea te tino take o te mate. Kua pāngia koe, tētahi atu rānei e mōhio ana koe, kua pāngia rānei koe e tēnei wheako? Tērā pea ko te take he mate ira tino onge kāore anō kia rongohia e te tini o te tangata o tō tātou whenua. I tēnei rā ka kōrero tātou mō tētahi o aua mate, ko te Acute Hepatic Porphyria , ko te AHP rānei.

He aha te Acute Hepatic Porphyria (AHP)?

He mate ira onge te AHP e pā ana ki tō tātou pūnaha io , ā, i ētahi wā ki tō tātou kiri. He tino onge, e pā ana ki te 5 i roto i te 100,000 tāngata. Ka taea e ia te whakaputa i ngā tohu ohorere, kino, mamae hoki. I ētahi wā, ka mōrearea te ora.

Hei mārama ki tēnei, me kōrero tātou mō te toto i roto i tō tātou tinana. Kei roto i ō tātou toto whero he pūmua e kiia nei ko te " Hemoglobin ". Ko tana mahi matua he kohi i te hāora e uru ana ki ngā pukahukahu ina manawa tātou, ka tukuna atu ki ngā okana me ngā kiko katoa o te tinana. He rite ki te 'ratonga tuku' e kawe ana i te hāora.

Tērā tētahi atu wāhanga he mea nui hei hanga i tēnei pūmua e kiia nei ko te "Hemoglobin", ā, e kiia ana e mātou ko te "Hēme" .

Ko te mea e tupu ana i roto i te tinana o te tangata whai mate AHP he hekenga, he ngaronga rānei o te whākōkī e hiahiatia ana i tētahi wā o te tukanga whakaputa i tēnei wāhanga e kiia nei ko te "Heme". He rite ki te tunu kai, ki te ngaro tētahi o ngā kai, kāore e taea te whakarite tika i te kai.

Ko te ngoikore o tēnei whākōkī ka puta i roto i tō tātou ate . Ka karangatia hoki te ate ko '(Hepatic)'. Koia te take i tapaina ai tēnei mate ko 'Acute Hepatic Porphyria '.

Nā, he aha te mea ka tupu ina kore e taea e tātou te hanga i te `(Heme)`? Ko ngā rauemi mata i whakamahia mōna, arā, ko te `(Porphobilinogen – PBG)` me te `(Aminolevulinic acid – ALA)`, ka tīmata ki te kohikohi i roto i te ate. Ka kohikohi ēnei paitini i roto i te toto, ka haere puta noa i te tinana, ka kino rawa atu ki tō tātou pūnaha io. Nā taua kino o te io ka puta ake ngā tohu pēnei i te mamae nui , te ngoikore , me te whakapairuaki .

He aha ngā momo matua o te mate AHP?

E whā ngā momo matua o te AHP. Ko ia momo e puta mai ana i te korenga o tētahi whākōkī rerekē i roto i te tukanga hanga "(Heme)". Engari ko ēnei momo e whā ka pā nui ki te ate me te pūnaha io.

Te Porphyria Wawe Koretake (AIP)

Koinei te momo e pā ana ki te 80% o ngā tūroro AHP, arā, te nuinga. I roto i tēnei, he ngoikore te whākōkī `(Hydroxymethylbilane synthase – HMBS)`. Ko te take o tēnei he panoni i roto i te ira `(HMBS)`. I roto i te rongoā, ka kiia tēnei he ira `(mutation)` . I ētahi wā ka taea tēnei te tuku iho mai i ngā mātua ki ngā tamariki. I ētahi wā rānei ka puta noa i roto i te tangata kāore he hītori whānau.

He aha te āhua o ngā tohumate o te AHP?

Ehara i te mea he rite tonu ngā tohu o te katoa e pāngia ana e te AHP. Tērā pea kāore he tohu o ētahi tāngata i roto i ō rātou oranga katoa. Engari, tērā pea ka pāngia ētahi e ngā whakaekenga maha, kino hoki. He mea nui kia mōhio ki ēnei tohu.

He aha ngā mea e whakaoho ana i te kino haere o te mate?

Ka taea e te tangata whai AHP te noho noa i te ao. Heoi, tera pea ētahi mea ka whakaoho i te mate. He mea tino nui kia mōhio ki ēnei.

• Ngā rongoā motuhake: Tērā pea ētahi rongoā pēnei i ngā paturopi kei roto ko ngā `(Barbiturates)` me ngā `(Sulfa)`. Mena kei a koe te AHP, he mea nui kia kōrero koe ki tō tākuta i mua i te tango i tētahi rongoā.
• Te inu waipiro: Ko te waipiro te tino take o te kaha haere o tēnei mate.
• Te kai tino pakari, te nohopuku rānei: Mā te kai i ngā kai iti te kaora me te warowaihā (māngaro) ka kino ake te mate.
• Ngā huringa homoni: Ngā huringa o ngā taumata homoni, inā koa i roto i ngā wāhine, e pā ana ki te huringa paheketanga.
• Te ahotea me ngā mate urutā: Ka taea e tetahi momo ahotea ki te tinana, tae atu ki tētahi mate urutā pēnei i te kirika, te makariri rānei, te whakararuraru i te mate.

Āhea koe me toro atu ki te tākuta?

Ki te pā mai ēnei tohu ki a koe, kaua e whakahawea he mea noa iho. He mea nui kia rapu tohutohu hauora, inā koa i ngā wā e whai ake nei:

• Mena kei te pāngia koe e te mamae puku nui kāore i te whakamāramatia, ā, ka hokihoki mai anō hoki.
• Mena ka puta ngā tohumate io pērā i te ngoikore me te māuiui i te wā kotahi.
• Ki te mōhio koe kei te pāngia tētahi o tō whānau e te mate `(Porphyria)`.
• Ki te kite koe kei te huri te tae o tō mimi ki te whero pouri/parauri.

Ki te pāngia koe e ngā tohu kino, pērā i te mamae kore e taea te manawanui, te uaua ki te manawa, te pōhēhē hinengaro, te ngaronga rānei o ngā waewae me ngā waewae, haere tonu ki te Wāhanga Maimoatanga Ohotata (ETU) o te hōhipera.

He rongoā kei te wātea mō te AHP?

Nā te mea he mate ira tēnei, kāore anō kia kitea he rongoā. Heoi anō, he tino whai hua ngā maimoatanga hei whakahaere me te ārai i te kaha haere o te mate.

I ngā wā tino kino, me kawe ki te hōhipera me te maimoatanga.

• Ko te mea nui ko te tautuhi i te pūtake o te horapa o te mate, me te whakamutu i tōna whanaketanga.
• Ka hoatu ngā rongoā whakamamae mamae kaha hei whakahaere i te mamae nui.
• Ka hoatu ngā rongoā hei whakahaere i te whakapairuaki me te ruaki.
• Mā te hoatu i te huka (glucose) ki te tinana, arā, te huka me te tote mā roto i tētahi uaua, ka taea te whakaiti i te hanga o ngā paitini i roto i te ate.
• Hei maimoatanga motuhake , ka mahia ngā werohanga `(Heme)` (ngā werohanga Hemin)`. Ka mahia tēnei mā te hoatu `(Heme)` ki waho, ka tukuna he tohu ki te ate e mea ana, "Kei a koe `(Heme)`, kāore koe e hiahia ki te hanga i tētahi atu," me te whakamutu i te hanga o ngā paitini.
• Haunga tēnei, kei reira ngā rongoā hou e hangaia ana mā te hangarau ira hei ārai i te hokinga mai o te mate mō te hunga e kaha haere ana te whakaekenga.

Mā te tākuta e tirotiro i a koe koe e whakatau te maimoatanga tino tika māu.

AHP, Porphyria Hepatic Koi, Porphyria, Mamae puku, Ngā mate roro, Ngā mate ira, Heme, Hemoglobin, Porphyrin, Ngā mate onge