He pūwero kei roto i ō whatukuhu? Me kōrero tātou mō te ADPKD (Autosomal Dominant Polycystic Kidney Disease)

Tērā pea kei te pāngia tētahi o tō whānau e te mate tākihi. Kua kitea rānei kei te pāngia koe e te toto toto tiketike i roto i ō tau 30, 40 rānei, ā, kei te rapu koe i tētahi take. I tēnei rā kei te kōrero tātou mō tētahi mate tuku iho rerekē, engari he tino noa, e pā ana ki ngā tākihi. E kiia ana tēnei e te rongoā ko te Mate Tākihi Polycystic Autosomal Dominant. Nā te mea he roa rawa tēnei mō tātou, ka tapaina e tātou ko te ADPKD hei whakarāpopototanga.

He aha te ADPKD?

He māmā noa iho, ka puta he nui o ngā whēkau kī tonu i te wai i roto i ō whatukuhu i tēnei mate. Whakaarohia ngā whatukuhu hei tātari i roto i ō tātou tinana. I te tipu haere o ēnei whēkau, kāore e taea e ngā whatukuhu te mahi tika. Ka arahi pea tēnei ki ngā raruraru hauora pērā i te toto toto tiketike, ngā mate mimi, me ngā kōhatu whatukuhu. Ahakoa kāore e pāngia te katoa e tēnei mate, ka taea e ētahi tāngata te tae ki te ngoikore o te whatukuhu.

Ko tētahi o ngā āhuatanga ahurei o tēnei mate ko te kore pea e kitea he tohu mō ngā tau maha ahakoa kei a koe. He maha ngā wā ka tīmata te puta o ngā tohu i waenga i te 30 me te 40 tau te pakeke . Koia i huaina ai hoki ko te "pakeke PKD".

He aha ngā tohu o te ADPKD?

Ehara i te mea ka puta ngā tohu ki te katoa e pāngia ana e te ADPKD. Mō ētahi, tera pea ka roa ngā tau ka puta mai ngā tohu. Me titiro tātou ki ētahi o ngā tohu e pā ana ki tēnei mate. Ka wehewehea e au tēnei kia rua ngā wāhanga kia mārama ai koe.

He aha i puta ai te ADPKD? He mea tuku iho?

Āe, he mate tuku iho tēnei. Ko te tino take o tēnei he hapa kei roto i ngā ira e rua o roto i tō tātou DNA, arā, ko te `PKD1` me te `PKD2`. Ko ēnei ira ngā mea e hoatu ana ki ngā pūtau whatukuhu te tohu "āhea me tipu". Ina he hapa kei roto i tētahi o ēnei ira, ka tipu haere ngā pūtau whatukuhu, ā, ka hanga i aua whēkau i whakahuatia ake nei e tātou.

Whakaarohia, mō te nuinga o ngā mate tuku iho, me whiwhi koe i te ira hapa mai i tō whaea me tō pāpā. Engari i roto i te ADPKD, me whiwhi noa koe i te ira hapa mai i te mātua kotahi . Koia i kiia ai ko te "autosomal dominant".

Ko te tikanga o tēnei, mēnā kei tētahi o ō mātua te mate, e 50% te tūponotanga ka pāngia koe e te mate . Heoi, he tino onge, ka puta anō hoki te mate nā te huringa matapōkere o te ira o te tangata, engari kāore ngā mātua e rua e pāngia e te mate.

Me pēhea koe e mōhio ai mēnā kei a koe tēnei mate?

Mena kei te whakapae tō tākuta he raruraru tākihi kei runga i ō tohu, ka tukuna pea koe e ia ki tētahi tohunga tākihi (nephrologist). Ka pātai atu te tākuta ki a koe i ngā pātai pēnei i:

• He aha ō tohumate? Nōnahea i tīmata ai?
• E mōhio ana koe ki tō toto toto?
• He mamae kei hea? Mena kei hea?
• Kua pāngia koe e ngā kōhatu whatukuhu i mua?
• He mate tākihi tō tetahi o te whānau?

Kātahi ka whakaritea e te tākuta ētahi whakamātautau hei tirotiro i ō whatukuhu.

• Matawai Ultrasound: Ka whakamahia ngā ngaru oro hei tango pikitia o roto o te tinana. Koinei te whakamātautau tuatahi e mahia ana i te nuinga o te wā.
• Matawai MRI: He matawai kaha e whakamahi ana i ngā aukume me ngā ngaru reo irirangi hei kite i ngā pukupuku he iti rawa e kore e kitea ki te ultrasound.
• Matawai CT: Ko tētahi atu tikanga matawai kaha e whakamahi ana i ngā hihi-X hei tiki whakaahua taipitopito o te tinana.

Hei tāpiritanga, ka taea te whakamātautau i tō DNA kia kitea ai mēnā kei a koe te ira hapa `PKD1`, `PKD2` rānei. Heoi, ahakoa ka taea e tēnei whakamātautau ira te whakaatu mēnā kei a koe te ira hapa, kāore e taea te matapae i te wā ka whanake te mate, te kaha rānei o tōna kino .

Te maimoatanga me ngā mea ka taea e koe te mahi i te kāinga

Kāore anō kia kitea he rongoā mō te ADPKD. Engari he maha ngā mea ka taea e tātou te mahi hei whakahaere i ngā raruraru hauora e puta mai ana i te mate, hei ārai hoki i te ngoikore o te whatukuhu.

Te maimoatanga hauora

• Ngā rongoā hei whakaroa i te ngoikore o te whatukuhu: Mō ngā pakeke e mōrearea ana ki te tere haere o te mate, ka hoatu ngā rongoā pēnei i te `Tolvaptan (Jynarque)` hei whakaroa i te hekenga o te mahi a te whatukuhu.
• Ngā rongoā e whakahaere ana i te toto toto tiketike.
• Ngā rongoā paturopi mō ngā mate mimi.
• Ngā rongoā whakamamae.

Ki te kore e pai ō whatukuhu, me whai pea koe i tētahi maimoatanga e kiia nei ko te dialysis . Ko te tikanga o tēnei ko te whakamahi i tētahi mīhini hei tātari i ō toto, te tango i ngā para, te tote, me te wai nui mai i tō tinana. Ko tētahi atu kōwhiringa ko te whakawhitinga whatukuhu. Kōrero ki tō tākuta mō tēnei mea kia mōhio ai koe ki ētahi atu kōrero.

Ngā mea ka taea e koe te mahi i te kāinga

Hei tiaki i ō whatukuhu, hei pupuri hoki i tā rātou mahi pai mō te wā roa rawa atu, he mea nui kia noho hauora tonu koe.

• Kai tika: Kai i te kai taurite, iti te tote. Ka taea e te tote te whakanui ake i te toto toto.
• Kia kaha tonu: Ka āwhina te whakakori tinana ki te whakahaere i te taumaha me te toto totoka, engari karohia ngā hākinakina pā, ka kino pea ō whatukuhu.
• Āta karohia te kai hikareti: Ka kino te kai hikareti i ngā oko toto i roto i ngā whatukuhu, ā, ka piki ake hoki te tūponotanga o te hanganga o ngā pūmua.
• Inumia he nui te wai: Ka taea hoki e te matewai te hanga pinepine i ngā kiore.

Ētahi atu raruraru ka puta mai i te ADPKD

Ka taea hoki e te ADPKD te whakanui ake i te mōrearea o ētahi atu raruraru hauora, ā, he mea nui kia mōhio ki ēnei hoki.

Ko te tere o te whanaketanga o te mate e whakawhirinaki ana ki te mea he hapa tōu ira `PKD1`, `PKD2` rānei. I te nuinga o te wā, ko te hunga he hapa tō rātou ira `PKD1` ka tere ake te pāngia e te ngoikore o te whatukuhu i ērā he hapa `PKD2`. Nō reira, he pai ake te kōrero ki tō tākuta kia mōhio ai koe he aha tō āhua.