Kei te mōhio koe ki tēnei mate ira e pā ana ki ngā pukahukahu me te ate? (Alpha-1 Antitrypsin Deficiency)

Kei te pāngia hoki koe e te maremare me te uaua ki te manawa kua roa nei e pāngia ana? Tērā pea koe e whakaaro he mea noa tēnei. Engari i ētahi wā, tera pea he tino take nui kei muri i ēnei tohu kāore anō kia rangona whānuitia. I tēnei rā ka kōrero tātou mō tēnei āhuatanga. Ko te Alpha-1 Antitrypsin Deficiency, ko te `(Alpha-1 Antitrypsin Deficiency)` rānei. Ka karangahia hoki e ētahi ko "Alpha-1" te whakarapopototanga.

He aha te kupu māmā noa iho, arā, ko Alpha-1?

He mate ira tuku iho a Alpha-1 i riro mai i ō tātou mātua. Ko te mea ka tupu i roto i tēnei, kāore e taea e tō tātou tinana te whakaputa i te nui o te pūmua e kiia nei ko te alpha-one antitrypsin `(AAT)`, he pūmua e tiaki ana i ō tātou pukahukahu. Whakaarohia tēnei pūmua `AAT` hei kaitiaki mō ō tātou pukahukahu. Ina ngaro tēnei kaitiaki, ka nui ake te tūponotanga ka pakaru ō tātou pukahukahu.

Nō reira, ko te tangata whai mana Alpha-1 kei te nui ake te mōrearea ki te whakawhanake i ngā mate pūkahukahu, inā koa ko te emphysema (te pakaru o ngā pēke hau i roto i ngā pūkahukahu), te cirrhosis (te whara o te ate), me te panniculitis, he mate kiri onge. I ētahi wā, ka taea e ēnei mate te whakararu i te ora.

Nā konei, ka karangatia hoki tēnei mate e ētahi tāngata ko te "COPD ira".

Me pēhea te puta o tēnei āhuatanga? Ko wai te hunga tino mōrearea?

Hei whakawhanake i te Alpha-1, me riro mai i a tātou te ira rerekē mai i ō tātou mātua e rua. He māmā noa iho, ko ngā ira ngā tohutohu e whakatau ana i te mahi a ō tātou tinana. Mēnā he iti noa te panonitanga o ēnei tohutohu, ka taea te whakarerekē i te mahi a te tinana.

I roto i te Alpha-1, ka raru te hanga o te pūmua `AAT` nā te whakarerekētanga ira e kiia nei ko `SERPINA1`. Nō reira, kāore te pūmua `AAT` e hangaia i roto i te nui e ranea ana, kāore rānei te pūmua e hangaia ana e hanga ana e hanga ana e hanga ana i te āhua hē. Ina hanga he āhua hē, kāore e taea e taua pūmua te wehe atu i te ate me te haere mā roto i te toto ki ngā pukahukahu. Kātahi ka mau taua pūmua āhua hē ki roto i te ate, ka kino ai te ate. Waihoki, nā te mea kāore he pūmua hei haere ki ngā pukahukahu, ka ngoikore hoki ngā pukahukahu.

Heoi, ka riro mai i ētahi tāngata tēnei ira hapa i te mātua kotahi anake (tō rātou whaea, tō rātou pāpā rānei). Ka kiia rātou he "kaimau". Ahakoa he nui te pūmua 'AAT' e whakaputaina ana e ō rātou tinana hei tiaki i ō rātou pukahukahu, kei te tūpono tonu rātou ki te pakaru o ngā pukahukahu. He tino teitei tēnei tūponotanga mēnā ka momi hikareti rātou.

He aha te pānga o tēnei ki ngā pukahukahu me te ate?

Hei mārama ki tēnei, me tango he tauira iti.

Ka hangaia tō tātou pūmua 'AAT' i roto i te ate. Kātahi ka haere mā roto i te toto ki ngā pūkahukahu. He whākōkī kei roto i ō tātou pūkahukahu e kiia nei ko 'Neutrophil Elastase' e āwhina ana i a rātou ki te whawhai ki ngā mate. Whakaarohia tēnei whākōkī he hōia tino toa, he pūkenga. Ka whakangaro i ngā mate. Engari ina oti, me aukati tetahi. Ki te kore, ka tīmata te whakaeke i ō tātou pūtau pūkahukahu hauora.

Ko te mahi "whakamutu", arā, te mahi "whakaweto", ka mahia e tā tātou pūmua `AAT`. He rite ki te rangatira e kī ana ki te hōia, "Āe, kua mutu, kati."

Ināianei, i roto i te tangata whai Alpha-1, kāore he āpiha `AAT` i roto i te tinana. Kātahi ka kore he tangata hei aukati i taua hōia toa (`Neutrophil Elastase`). Ka haere tonu tana whakaeke i ngā pukahukahu. Mā tēnei ka whakangaromia te pūmua `Elastin` i roto i ngā pukahukahu. Ko tēnei elastin te mea e homai ana ki ngā pēke hau (alveoli) i roto i ngā pukahukahu te ngāwari me te kaha pērā i te here rapa. Ina ngaro, ka ngoikore ngā pēke hau, ka piko pērā i te poihau kua memeha. Koinei te mea e kiia nei e tātou ko te `Emphysema` . Mā tēnei ka uaua te manawa me te whiwhi hāora.

He rerekē te āhua o te ate. Nā ētahi hapa ira, ka hē te āhua o te pūmua 'AAT'. Pērā i tētahi pepa kua hē te whakapiko. Nā tēnei āhua hē, kāore e taea e te pūmua te wehe atu i te ate, ā, ka piri ki roto i te ate. Ina nui rawa te pūmua e piri pēnei ana, ka pakaru te ate, ā, ka tīmata te hanga i ngā koha. Ko te cirrhosis tēnā .

He aha ngā tohu o te mate Alpha-1?

He rerekē ngā tohu e puta mai ana i te Alpha-1 i runga i te okana o te tinana e pāngia ana. He rite tonu ēnei ki ngā tohu o te COPD (Mate Pūkahukahu Ārai Mau).

Me pēhea te tautuhi i tēnei mate?

Ko te huarahi matua ki te tautuhi i te mate Alpha-1 ko te whakamātautau toto . Nā te mea he rite ngā tohu ki ētahi atu mate, ka roa pea te wā ki te whiwhi i te tautuhi tika. Mena kei a koe ngā tohu ate, kua tautuhia rānei koe he COPD, ā, kāore ō tohu e pai ake ahakoa te maimoatanga, ka whakatau pea tō tākuta ki te whakamātautau i a koe mō te mate Alpha-1.

Ko ngā whakamātautau e mahia ana i te nuinga o te wā ko:

• Ngā whakamātautau toto: Ka whakamātauria tō toto mō te taumata o te pūmua `AAT`. Mena he iti te taumata, ka mahia he whakamātautau ira hei tirotiro mena he hapa ira tōu e pā ana ki te Alpha-1.
• Ngā whakamātautau whakaahua: Mā te hihi-X o te uma, mā te karapa CT rānei ka taea te whakatau i te whānuitanga me te wāhi o te pakaru o te pukahukahu.
• Ngā whakamātautau mahi a ō pūkahukahu: Ahakoa kāore e taea e ēnei te kite tika i te Alpha-1, ka taea e ēnei te hoatu he whakaaro ki tō tākuta mō te pai o te mahi a ō pūkahukahu.
• Te Ultrasound o te Ate, te FibroScan® rānei: Mēnā kei te whakapaetia he pakaru o te ate, ka mahia ēnei matawai hei tirotiro mō ngā kōhatu i roto i te ate.
• Kohikohi ira o te ate: Mena he kino te kino o te ate, ka tangohia he wahi iti o te kiko o te ate, ka tirotirohia hei whakatau i te nui o te kino.

He aha ngā maimoatanga mō te Alpha-1?

Ahakoa kāore he rongoā motuhake mō te mate Alpha-1, he maha ngā maimoatanga hei whakahaere i ngā tohu, hei whakaiti i te kino o ngā okana, hei whakapai ake hoki i te kounga o te oranga.

He rerekē ngā tikanga maimoatanga i runga i te okana e pāngia ana:

Te maimoatanga mō ngā pukahukahu

• Whakaora Whakanui: He maimoatanga motuhake tēnei mō te Alpha-1. Ko te tikanga he pure i te pūmua 'AAT' i tangohia mai i ngā kaituku toto hauora, ka hoatu ki te tinana mā roto i te uaua, he rite ki te tote. Ahakoa kāore e taea e tēnei te whakakore i te kino kua puta kē, ka taea e ia te aukati, te whakahaere rānei i ngā kino o muri mai ki ngā pukahukahu.
• Rongoā: Ka hoatu ngā rongoā pēnei i ngā mea whakangā , pērā i ngā rongoā whakaroa ihu, ki ngā tūroro e pāngia ana e te COPD hei whakangāwari i te manawa.
• Whakaora hāora: Mena he iti te taumata hāora i roto i te toto, ka hoatu he hāora tāpiri mā roto i tētahi ngongo iti e whakanohoia ana ki roto i te ihu, i tētahi kopare waha rānei.
• Ngā kaupapa whakaora manawa: Ka whakaratohia he whakangungu hei whakahaere i te manawa mā roto i ngā mahi manawa me ētahi atu mahi ā-tinana.
• Te whakawhitinga pūkahukahu: Mena kua tino pakaru ngā pūkahukahu, me whakamahi pea he whakawhitinga pūkahukahu hauora hei huarahi whakamutunga.

Te maimoatanga mō te ate

Ka rongoatia ngā tohu o te kino o te ate. Heoi, ko te whakawhitinga ate anake te huarahi hei whakaora katoa i te mate ate Alpha-1. Ina whakawhitingahia he ate hauora, ka tīmata te ate ki te whakaputa i te pūmua 'AAT' tika.

He aha taku e taea ai kia ora hauora ai ahau me te Alpha-1?

Ahakoa kua kitea koe kei te mate koe i te Alpha-1, ehara i te mutunga o tō oranga. He maha ngā mea ka taea e koe te mahi hei whakaiti i te kino ki ō whekau.

Haunga tēnā, kia mōhio hoki ki ēnei mea:

• Me noho tawhiti atu i ngā wāhi e momi hikareti ana te tangata. (Kaua e whakamahi i te auahi tuarua)
• Kaua e hongi i ngā mea kino ki ō pukahukahu, pērā i te puehu me ngā matū. Mēnā ka pāngia koe e ēnei mea i te mahi, mauria he kopare tiaki.
• Kati rawa te inu waipiro, whakawhāitihia rānei te nui o te waipiro. Ka taea e te waipiro te whakanui ake i te kino o te ate.
• Kaua e whakamahi i ngā rongoā whakamamae mamae (hei tauira, te nui rawa o te paracetamol) me ētahi atu otaota rānei ka pā ki te ate, me te kore e kōrero ki tō tākuta.
• Whiwhihia ngā werohanga katoa e tika ana. He mea nui rawa te whiwhi werohanga mō ngā mate pēnei i te niumōnia, te rewharewha (rewharewha), te COVID-19, me te hepatitis A me te B, e pā ana ki te ate.
• Horoia ō ringaringa i ngā wā katoa, kia noho mā, ā, kia tiakina koe i ngā mate urutā.
• Mena kei te pāngia tētahi o tō whānau e te mate Alpha-1, he mea pai kia whakamātauria koe anō. Kōrero ki tō tākuta mō tēnei.
• Mena kei a koe te Alpha-1, he kaikawe rānei koe i te mate, he mea tino nui kia kōrero koe ki tētahi kaitohutohu ira mēnā kei te whakaaro koe ki te whānau tamariki.

Āhea koe me toro atu ki te tākuta?

• Mena kei a koe ngā tohu o te pukahukahu, o te ate rānei i kōrerotia i mua ake nei.
• Mena kua tākutatia tetahi o tō whānau ki te mate Alpha-1.
• Mena kua tāpaetia tō mate COPD, te mate huango rānei, ā, kua rongoatia hoki, engari kāore ō tohu e pai ake ana, kōrero ki tō tākuta mō te whakamātautau Alpha-1.
• Mena kei a koe te Alpha-1, me toro atu ki tō tākuta tonu mēnā ka puta he tohu hou, ka kino rānei ngā tohu o nāianei.

He uaua te noho tahi me te Alpha-1. Engari mā te mātauranga tika, te maimoatanga, me te momo noho tika, ka taea hoki e koe te noho ora hauora, ora kaha. Ko te huarahi pai ki te mahi i tēnā ko te kōrero tuwhera ki tō tākuta, me te whakatakoto i tētahi mahere e pai ana māu.