Me ako noa tātou mō te Pulmonary Alveolar Proteinosis (PAP), he mate e ārai ai ngā pēke hau i roto i ngā pukahukahu.

Kei te ahua uaua koe ki te manawa i ētahi wā, me te mea kei te kikī tō uma? Kei te ngenge koe ahakoa he poto noa te hikoi? He maha ngā wā ka wareware tātou ki ēnei mea, me te whakaaro he ngenge noa iho. Heoi, i tēnei rā ka kōrero tātou mō tētahi mate hauora nui kei muri i ēnei tohu, ahakoa he tino onge. Ko te Pulmonary Alveolar Proteinosis, ko te PAP rānei te whakarapopototanga.

He aha te Pūmua Alveolar Pūkahukahu (PAP)?

Whakaarohia kua kī tonu ō tātou pūkahukahu i ngā miriona o ngā pēke hau ririki. Ka kiia ēnei pēke hau ririki he alveoli . Ina manawa tātou, mā roto i ēnei pēke hau ka ngongohia e tātou te hāora.

I roto i te PAP, ko te mea ka tupu ko te kohikohinga o ngā pūmua, ngā ngako, me ētahi atu mea ki roto i ēnei pēke hau ririki, pērā i te para. Ko te take o tēnei, kāore ngā pūtau e horoi ana i te matū rite ki te hinu e kiia nei ko te surfactant, e kitea ana i roto i ngā pakitara o ēnei pēke hau, e mahi tika ana. Kātahi ka kohikohia tēnei surfactant, ka ārai i ngā pēke hau. Nō reira, kāore e taea e te hau te uru tika, nō reira kāore te toto e whiwhi i te nui o te hāora e hiahiatia ana. Ka uaua te manawa.

He momo matua o te PAP?

Ae, e toru ngā momo matua o te PAP. He rerekē te pūtake o ia momo.

Ko wai kei te nui ake te mōrearea ki te whakawhanake i tēnei mate?

Ko te mate pūnaha ārai mate me te mate PAP tuarua ka pā ki te hunga i waenga i te 30 me te 60 tau te pakeke. He nui ake te kitea o ēnei momo e rua i roto i ngā tāne . Ko te hunga momi hikareti me te hunga e pāngia ana e te puehu, ngā matū rānei, he nui ake te mōrearea.

Ko te PAP whānau te mea e tino kitea ana i roto i ngā tamariki kei raro iho i te 10 tau te pakeke, engari ka taea te puta i ngā tau katoa.

He aha ngā tohu o te mate PAP?

Ko te tohu matua, ko te tohu tino kitea o tēnei mate ko te poto o te manawa (dyspnea) . Ka pāngia te nuinga o te tangata e te poto o te manawa i te wā e whakakorikori tinana ana, e mahi ana rānei. Heoi, ka pāngia pea ētahi e tēnei uauatanga ahakoa kei te tū noa rātou.

Hei tāpiri, ka taea hoki e koe te kite i ēnei tohu:

• Mamae o te uma
• Maremare (tērā pea he huware, he toto rānei)
• Te tae kikorangi o te kiri me ngā maikuku (cyanosis) - Ka puta tēnei ina kore e nui te hāora i roto i te toto.
• Ngenge tonu
• Kirika
• Ngā mate pūkahukahu auau
• Ngā maihao e takoto ana i te karapu
• Te ngaronga taumaha me te kore take

Me pēhea te tautuhi a te tākuta i tēnei mate?

Ki te whakapae koe kei a koe te mate PAP, ka tirohia tuatahitia koe e tō tākuta, ka whakarongo ki ō pukahukahu me te whakamahi i te stethoscope. Kātahi ka pātai atu rātou ki a koe mō tō hītori hauora whānau me tō āhua noho. Ki te kaipaipa koe, ki te pāngia koe e te puehu, e ngā matū rānei, me kōrero koe ki tō tākuta .

I muri i tēnei, ka taea te whakahaere i ētahi whakamātautau hei whakaū i te mate:

• Ngā whakamātautau toto: Tirohia te taumata hāora i roto i te toto. Waihoki, ka taea te whakahaere i ngā whakamātautau motuhake pēnei i te GM-CSF autoantibody hei kimi i te pūtake o te mate PAP.
• Whakamātautau mahi a ō pukahukahu: Ka ine i te pai o te mahi a ō pukahukahu.
• Ko te hihi-X o te uma, ko te matawai CT rānei: Hei tiro mārama i te āhua o ngā pukahukahu.
• Te tirotiro i te pūkahukahu:He tikanga e whakaurua ai he ngongo angiangi me te kāmera e piri ana mā te waha i raro i te mate whakamoe hei tirotiro i te roto o te ara manawa me ngā pukahukahu.
• Kohikohi pūkahukahu: Ka tangohia he wahi iti o te kiko mai i te pūkahukahu i te wā o te bronchoscopy, i te wā rānei o te pokanga hei tirotiro.

He aha ngā maimoatanga mō te PAP?

Kāore he rongoā mō te PAP i tēnei wā, engari he maha ngā maimoatanga tino whai hua hei āwhina i a koe ki te whakahaere i ō tohumate me te ora i tētahi oranga pai ake.

Ko te maimoatanga matua, ko te maimoatanga e whakamahia whānuitia ana mō te PAP ko te "Whole-Lung Lavage" (WLL) .

He aha tēnei horoinga pūkahukahu?

Ko te mea e tupu ana i tēnei, ka whakamahia e te tākuta he bronchoscope (he ngongo kāmera) hei horoi i ngā pukahukahu takitahi ki te tote (wai tote kua horoia) hei tango i ngā pūmua me ngā ngako kua mau. I muri i tēnei, ka māmā ake te manawa.

I te wā e mahia ana tēnei maimoatanga:

• Ka whakamoea rawatia koe, nō reira kāore koe e rongo i tetahi mea.
• Ka whakaurua e te tākuta te pūkahukahu mā te waha, ka tohu ki tetahi o ngā pukahukahu.
• Ka tukuna te hāora ki tētahi atu pukahukahu e tētahi mīhini.
• I ētahi wā ka mau koe i tētahi koti wiri i tō uma. Mā tēnei wiri ka wetekina ngā mea e mau ana i roto i ō pēke hau.
• Kātahi ka werohia te wai tote ki roto i ngā pukahukahu, ā, ina tangohia, ka puta mai me te mea i piri.
• Tērā pea ka horoia anō hoki te pukahukahu i taua wā anō, i muri rānei i ētahi rā.

Ētahi atu maimoatanga

• Ngā rongoā whakangāwari i te manawa: Ka whakangāwari ēnei rongoā i ngā uaua huri noa i ngā ara manawa, kia māmā ake ai te manawa.
• Te rongoā whakakapinga GM-CSF: Ka hongia tēnei mā te nebulizer, ka hoatu rānei hei werohanga. Ka whakapai ake i te mahi a ngā pūtau e horoi ana i ngā ara manawa.
• Te whakawhitinga pūkahukahu: Ko te hunga kua tino kino te mate, ā, kua pakaru ō rātou pūkahukahu, me whakawhitinga pūkahukahu hauora hei huarahi whakamutunga.
• Te Plasmapheresis me te whakawhiti plasma: Ko te tango i te wāhanga wai o te toto (plasma) ka whakakapi ki te plasma mai i te tangata hauora. I roto i te PAP autoimmune, ka āwhina tēnei ki te aukati i te pūnaha ārai mate ki te whakaeke i ngā pūtau hauora.
• Hāora tāpiri: Ka tukuna te hāora mā te kopare, mā te ngongo iti rānei e whakanohoia ana ki te ihu hei whakangāwari i te manawa.

He aha tāku e taea ai hei tangata whai PAP?

Mena kei a koe te PAP, he mea tino nui kia whai koe i te maimoatanga e tohua ana e tō tākuta, me te tiaki hoki i tō momo noho.

• Āta karohia te momi hikareti, me ngā wāhi hoki e momi hikareti ana ētahi atu.
• Tikina ngā kano ārai mate rewharewha me te niumōnia e ai ki ngā taunakitanga a tō tākuta.
• Me noho tawhiti atu i te hunga e mate ana.
• Kai i ngā kai totika , ā, whakahaere i tō taumaha.
• Horoia pai ō ringaringa i muri i te whakamahinga o te wharepaku, i mua atu hoki, ā, i muri hoki i te kai.
• Kōrero ki tō tākuta, ā, whakawhanakehia he mahere whakakori tinana haumaru e pai ana ki a koe.