He mate kei roto i a tātou e tino kitea ana, me ētahi atu mate he tino onge, arā, he tino onge. Kāore pea koe i rongo mō tēnei momo mate. I tēnei rā ka kōrero tātou mō tētahi mate onge, engari he mea nui e pā ana ki te toto, he mea nui kia tika te whakahaere. Ko tōna ingoa ko Paroxysmal Nocturnal Hemoglobinuria, e kīia nei ko PNH.
He aha te PNH?
Ehara te PNH i te mate ira, engari he mate tuku iho, he mate tuku iho rānei mai i ngā mātua ki ngā tamariki. Ka taea te whanake i ngā tau katoa. Ki te kī ngāwari, he mate tō te tangata whai mate nei, ā, ka whakaekea hētia e
te pūnaha ārai mate , arā, te pūnaha e tiaki ana i a tātou i te mate, ā, ka whakangaromia e ia ōna ake toto whero. Whakaarohia he rite ki te whakangungu rakau i hangaia ki ngā pūmua motuhake hei tiaki i a tātou i te mate. Kāore ētahi toto whero i roto i te tinana o ngā tūroro PNH i tēnei whakangungu rakau tiaki, arā, aua pūmua motuhake. Nō reira, ka mōhio te pūnaha ārai mate ki ēnei pūtau me te kore he whakangungu rakau, ka whakaaro he whakaeke kino rātou ki te tinana, ā, ka whakangaromia e ia. Ahakoa he mate mōrearea tēnei āhuatanga,
mā te maimoatanga tika, ka taea e koe te whakahaere i ngā tohu, te whakaiti i ngā raruraru, me te noho ora pai. Kāore tēnei mate e pā ki te katoa i te ara kotahi. He iti noa te mamae o ētahi tāngata. Ko ētahi atu ka pāngia e te kino rawa atu.
Ko te mōrearea matua ko ngā toto toto. Tata ki te 30% o ngā tūroro PNH kei te mōrearea ki te whakawhanake i te toto toto toto i te iti rawa i te kotahi i roto i ō rātou oranga.
He aha te take mō tēnei?
Ko te take o tēnei mate he whakarerekētanga ira i roto i te ira. Engari
ehara tēnei i te mea tuku iho. Arā, ehara i te mea ka riro mai i ō mātua, ehara hoki i te mea ka tukuna ki ō tamariki. He whakarerekētanga ira matapōkere tēnei e puta ana i te wā o te ora. Nā tēnei whakarerekētanga ira, ka tīmata tō hinu wheua ki te whakaputa i ngā toto whero rerekē kāore i a rātou aua pūmua tiaki. Nō reira, nā te mea kāore ēnei pūtau i tiakina e te pūnaha ārai mate, ka ngawari te pakaru. I roto i te rongoā, ka kiia tēnei tukanga o te pakaru o ngā toto toto whero
ko te hemolysis . E whakapono ana ētahi tākuta he hononga te PNH ki te ngoikore o te hinu wheua. Ina koa, ko te hunga whai mate e kiia nei ko te anemia aplastic ka nui ake te tūponotanga ki te whakawhanake i te PNH. Waihoki, ko te hunga whai PNH ka taea te whakawhanake i te anemia aplastic i muri mai. I roto i tēnei āhuatanga e kiia nei ko te anemia aplastic, ka mutu te hanga o ngā toto hou e te hinu wheua.
He aha ngā tohu o te PNH?
Ko te ingoa o te mate nei i ahu mai i tētahi o ōna tohu matua. Ko te tikanga o te kupu Paroxysmal ko te "puta ohorere", ko te tikanga o te kupu Nocturnal ko te "pō", ā, ko te tikanga o te kupu Hemoglobinuria ko te "tukunga o te hemoglobin i roto i te mimi". Ko te hemoglobin te pūmua e homai ana i te tae whero ki te toto. Nō reira, ina pakaru ngā pūtau toto whero, ka tukuna tēnei hemoglobin ki roto i te mimi,
ā, ko te mimi e tukuna ana i te pō, i te ata rānei, ka pōuri (pērā i te kora) ka whero rānei . Tata ki te 50% o te hunga whai PNH ka pāngia e tēnei tohu. E toru ngā take matua e puta ai ngā tohu:
Ka nui ake ngā tohu ka pā ki a koe, ka nui ake ngā pūtau toto he o roto i tō tinana.
Ngā tohu noa e puta mai ana i te pakaru o ngā pūtau toto whero me te anemia
| Tino ngenge, ngoikore hoki te tinana | Te ngenge tonu ahakoa i muri i te mahi i tētahi mahi iti. |
| Ānini | Ngā mamae o te upoko i ngā wā katoa. |
| Te uaua ki te manawa | Te whewhe ahakoa he poto noa te tawhiti e hikoi ana. |
| Ngā tere o te ngakau | Te āhua nei kei te tere te pao o tō ngākau. |
| Mamae puku | Te mamae o te puku, kāore he take. |
| Te uaua ki te horomi | Te uaua ki te horomi i te kai, i te wai rānei. |
| Kiri mā, kiri kōwhai rānei | Te kōwhai o te kiri me te mā o ngā kanohi. |
| Ngāwari te marō | Ahakoa he whara iti noa iho, ka taea e ia te whakaputa i ngā marō nui. |
| Te ngoikore o te moepuku i roto i ngā tāne | Te uaua ki te whakatutuki, ki te pupuri rānei i te hihiri i te wā o te moepuku. |
He mea tino nui: He mate morearea te toto totoka e hiahia ana kia arohia wawetia e te hauora. Kia tūpato ki ngā tohu e whai ake nei.
| Te wāhi o te toto totoka | Ngā āhuatanga hei tirotiro māu |
|---|
| Kiri | He wāhi whero, mamae, pupuhi rānei o te kiri. |
| Ringa, Waewae rānei | Mamae te ringa/waewae, te mahana, me te pupuhi. |
| Puku | Te mamae nui o te puku, te mate pukupuku, me te toto. |
| Roro | Te ānini nui, me te ruaki, kāore rānei, ngā haurangi, te uaua ki te neke, ki te kōrero, ki te kite rānei. |
| Ngā pūkahukahu | Te uaua ki te manawa, te mamae nui o te uma pērā i te maripi, te maremare toto, te werawera. |
Ki te mea he iti noa iho tō whakapae he toto totoka pēnei tōu,
Me whakamōhio tonu atu ki tō tākuta. Haere tonu ki te Tari Whawhati Tata (ETU) o te hōhipera tata, ina koa mēnā ka pāngia koe e te uaua ki te manawa, te uaua ki te kōrero/kite, te ānini ohorere, te mamae o te uma, te haurangi rānei. Me pēhea te tautuhi tika i te mate?
Ina haere koe ki te tākuta, ka pātai ia mō ō tohu me tō hītori hauora. Kātahi ka tirohia koe e ia, ka whakahau i ētahi whakamātautau toto. Ko te whakamātautau matua e mahia ana mō tēnei he whakamātautau toto motuhake e kiia nei ko te flow cytometry . Mā tēnei ka whakarite kei roto i ō toto whero ēnei pūmua tiaki. Hei tāpiri, ka tirohia hoki te maha o ngā toto toto (te maha o ngā toto toto) me ngā taumata rino. I ētahi wā, ka hiahiatia pea he tauira hinu wheua. He aha ngā maimoatanga mō te PNH?
E rua ngā whāinga matua o te rongoā i te PNH. Ko tētahi he whakaora i ngā tohu, ko tētahi he ārai i ngā raruraru kino (ina koa ko ngā toto totoka). Ka whakawhirinaki te maimoatanga ka whiwhi koe ki te kaha o tō mate.- Ngā matūkai tāpiri: Ka hoatu he waikawa folic me ngā tāpiringa rino hei whakahaere i te anemia.
- Ngā rongoā motuhake: Kei reira ngā rongoā pēnei i a Eculizumab (Soliris) me Ravulizumab (Ultomiris) e ārai ana i te pakaru o ngā toto whero. Ka hoatu ēnei mā roto i te uaua. He painga tō rātou ki te whakahaere i te anemia, te whakaiti i te hiahia mō ngā whakawhiti toto o waho, me te whakaiti i te tūponotanga o te toto totoka. Ko Pegcetacoplan (Empaveli) tētahi atu rongoā hou e whakamahia ana mō tēnei kaupapa.
- Te Whakawhiti Toto: Mena he kino te mate toto iti, ka taea te whakaora i te wā poto mā te koha toto mai i tētahi tangata hauora.
- Ngā rongoā whakangawari toto: Ka hoatu ngā rongoā whakangawari toto ki te hunga he nui te mōrearea o te toto totoka.
- Te Whakatō Pūtau Taketake o te Hinu Wheua: Koinei anake te huarahi hei whakaora katoa i te PNH. Ko te tango i te hinu wheua mate me te whakato i ngā pūtau take mai i tētahi tangata hauora (he maha ngā wā he tuākana, he teina rānei). Heoi, nā te mea he tino mōrearea, he uaua hoki tēnei tikanga, he tikanga noa iho tēnei mō ngā taiohi he tino kino te mate.
Mā tō tākuta e whakatau ko tēhea tikanga maimoatanga te mea tino tika māu. He mea tino nui hoki te tiaki i a koe anō.
I te wā e noho tahi ana koe me te PNH, he mea nui ake i ngā wā katoa te tiaki i a koe anō.- Kai pai: Kai i ngā kai whai kiko. Mā te kai i ngā kai he nui te rino (pīnihi, mīti) me te kai i ngā kai he nui te huaora C (karaka, tāne) ka āwhina i te tinana kia pai ake te mimiti i te rino.
- Whakakorikori Tinana: Ka uaua pea te whakakorikori tinana i te ngenge. Pātai atu ki tō tākuta he aha ngā whakakorikori tinana e tika ana māu.
- Tiakina koe i ngā mate urutā : Horoia ō ringaringa i ngā wā katoa. Āta karohia ngā wāhi e kī ana te tangata me ngā wāhi e noho ana te hunga tūroro. Mēnā ka pāngia koe e te kirika, me kōrero tonu ki tō tākuta.
- Hauora hinengaro: He mea noa te pōuri, te riri, me te manukanuka ina rongo koe kei a koe tētahi mate onge pēnei i tēnei. He tino whakamarie te kōrero mō ēnei kare ā-roto ki tētahi tangata pono, ki tētahi kaiwhakatakoto whakaaro rānei.
- Te Hapūtanga: Mena he wahine koe e whakaaro ana kia hapu, me toro atu ki tō tākuta i mua i te haputanga. Ka taea e te PNH te whakararu i a koe me tō pēpi i te wā e hapu ana.
Karere Tangohia ki te Kāinga
- He mate toto onge te PNH e puta mai ana i te whakarerekētanga ira, engari ehara i te mea tuku iho.
- Ko te raruraru matua me te tino morearea o tēnei mate ko te toto totoka. Kia tūpato tonu ki ōna tohu whakatūpato (te ānini nui, te mamae o te uma, te uaua ki te manawa).
- Ahakoa he mea mōrearea tēnei ki te ora, he tino whai hua ngā maimoatanga e wātea ana i ēnei rā hei whakahaere i ngā tohu me te whakapai ake i te kounga o te oranga.
- Ahakoa ko te whakawhitinga wheua wheua anake te maimoatanga tino whakaora mō te PNH, he nui te mōrearea, ā, e tika ana mō ētahi tūroro anake.
- He mea tino nui kia mau tonu tō whakapā atu ki tō tākuta, me te whai i ana tohutohu.
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