Te pupuhi pinepine o te uma, te kore e piki te taumaha... He Cystic Fibrosis (CF) tēnei? Kia mōhio tātou ki tēnei.

He ihu pūmau tonu tō tamaiti? Ahakoa te nui o te kai e hoatu ana e rātou, ka hiroki haere rātou, kāore e mōhiotia? He ahua uaua ki te manawa i ētahi wā? He mea noa ki ngā mātua te mataku ina kite rātou i ēnei tohu. He tohu pea ēnei o tētahi mate e kiia nei ko te Cystic Fibrosis (CF), kāore pea tātou i te tino rongo i tēnei, engari he mea tino nui kia mōhiotia. Me kōrero tātou mō tēnei mea me te kore wehi, me te ngāwari noa iho i tēnei rā.

He aha rawa te cystic fibrosis (CF)?

He māmā noa iho, he mate ira tēnei e tukuna iho ana mai i ngā mātua . He ira motuhake kei reira e whakahaere ana i te nekehanga o te tote (chloride) me ngā wai i roto i ngā pūtau o tō tātou tinana. Ka kiia tēnei ko te ira CFTR (cystic fibrosis transmembrane conductance regulator) . Nō reira, ka puta te mate CF ina he hapa, he whakarerekētanga rānei kei roto i tēnei ira.

He aha te mea ka puta mai i tēnei hapa? I te nuinga o te wā, he angiangi, he pahekeheke ngā wai i roto i ō tātou tinana, pērā i te hūpē me te pūhā .  Engari i roto i te tinana o te tangata whai CF, ka tino matotoru, ka matotoru, ka piri hoki ēnei wai.

Whakaarohia te mea ka tupu ina puea ake tēnei hūpē matotoru ki roto i ngā pukahukahu. Ka uaua te manawa, ka ngāwari te mau o ngā iroriki , ā, ka nui haere ngā mate . I roto i te wā, ka pā kino tēnei ki ngā pukahukahu, ka ngoikore te manawa, tae noa ki te mate.

Waihoki, ka āraihia e ēnei hinu matotoru ngā awaawa o te repeure. Kātahi ka kore e tukuna ngā whākōkī nakunaku e āwhina ana ki te nakunaku i te kai e kainga ana e tātou . Nō reira, ka puta te kore kai pai me te kore tipu. Hei tāpiri, ka puta pea ngā mate ate, te mate huka ('Cystic Fibrosis-Related Diabetes' - CFRD) me ngā raruraru momona.

Kaua e manukanuka. I ēnei rā, nā ngā maimoatanga matatau, kua piki ake te roanga o te oranga o ngā tūroro CF mō ngā tekau tau.

He aha ngā tohu o tēnei mate?

Ka rerekē ngā tohu o te CF i ia tangata, i ia tangata. Ka rerekē anō hoki i runga i te kaha o te mate. Me titiro tātou ki ngā tohu matua.

Te mate pukupuku cystic kāore i te rite ki te tikanga

He momo CF ngāwari ake tēnei. Ka puta ngā tohu i muri mai i te oranga. I ētahi wā kotahi anake te okana ka pāngia.

He aha ngā take o te CF?

E ai ki tā tātou i kōrero ai i mua ake nei, nā te hapa o te ira e kiia nei ko te CFTR tēnei i hua ai. Kia pāngia ai te tamaiti e tēnei mate, me whiwhi te tamaiti i tētahi kape o te ira hapa mai i te whaea me te pāpā .

Me pēhea te tautuhi i te mate?

He mea tino nui te tautuhi wawe i tēnei mate, nā te mea ka taea te tīmata wawe i te maimoatanga, ā, ka taea hoki te whakaiti i ngā raruraru ka pā mai ā muri ake nei. I Sri Lanka, kei te whakamātautauhia ngā tamariki mō tēnei i te whānautanga mai i ētahi hōhipera.

Ko ngā whakamātautau matua e whakamahia ana hei tautuhi i te mate ko:

• Whakamātautau werawera: Koinei te whakamātautau pono rawa atu hei tautuhi i te CF.Ka inehia e tēnei whakamātautau kore mamae te nui o te tote (hauora) i roto i tō werawera. Mēnā he nui ake te nui i te mea noa, he tohu tēnei nō te CF.
• Whakamātautau toto: Ka whakamātauhia te taumata o te matū e kiia nei ko te 'Immunoreactive Trypsinogen (IRT)' i roto i te toto. He teitei tēnei taumata i roto i ngā tūroro CF.
• Whakamātautau ira (whakamātautau DNA): Ka tirohia tika tēnei mō ngā hapa o te ira `CFTR`.

Haunga ēnei whakamātautau, ka tūtohu pea tō tākuta i ētahi atu whakamātautau hei whakaū i te mate me te rapu i ngā raruraru. Hei tauira, he hihi-X o te uma, he whakamātautau mahi pūkahukahu (PFT), me ngā whakamātautau huware me te tūtae.

He aha ngā maimoatanga?

Nā te mea he mate uaua te CF, ka whakaratohia te maimoatanga e tētahi rōpū tohunga maha-mātauranga, tae atu ki tētahi tohunga manawa, tētahi kaiwhakaora tinana, me tētahi tohunga kai. Ko ngā whāinga matua o te maimoatanga he horoi i te hūpē o ngā pukahukahu, te ārai i ngā mate, me te whakarato i te kai e tika ana.

Ngā rongoā

Ka tohua pea e tō tākuta ngā rongoā pēnei i:

• Ngā rongoā paturopi: Ārai me te rongoā i ngā mate pūkahukahu.
• Ngā rongoā whakaiti i te hūpē: Ka hoatu ēnei mā te whakamahi i tētahi mea manawa, mā te nebulizer rānei. Ka āwhina ēnei ki te whakaiti i te hūpē matotoru, ā, ka māmā ake te maremare.
• Ngā rongoā whakawhanui i te manawa: Ka whakawhanui ake i ngā ara manawa, ka māmā ake hoki te manawa.
• Ngā tāpiringa whākōkī repeure: Me tango ēnei i ia kai me ia paramanawa. Ka āwhina i te nakunaku me te mimiti matūkai.
• Ngā kaiwhakatikatika CFTR: Koinei ngā momo rongoā hou, ā, koia te mea tino whai hua. Ka āwhina ēnei rongoā ki te whakaora i te mahi a te pūmua `CFTR` hapa e tino puta ai te mate. Ka taea e tēnei te whakangawari i te hūpē, te whakapai ake i te mahi a ngā pukahukahu, te whakaiti i te maremare, tae atu ki te whakaiti i te pikinga o te taumaha. Ko `(Trikafta, Kalydeco, Orkambi)` ētahi o ēnei momo rongoā.

Ngā Tikanga Whakawātea Huarahi Manawa (ACT)

He mea ēnei e tika ana kia mahia i ia rā, kia rua pea i te rā. Ka āwhina ēnei ki te wetewete me te tango i te hūpē matotoru e piri ana i roto i ngā pukahukahu.

• Te whakaora tinana o te uma: Me patopato te tangata i te tuara me te uma kia ngawari ai te hūpē.
• Te Wēti: He wēti tēnei e hono ana ki tētahi mīhini motuhake. Ina mau, ka pā te wiri auau teitei ki te uma, ka puta he mahi, ka wetekina te hupe.
• Ngā taputapu PEP: Mā te whakaputa manawa mā roto i ngā taputapu iti pēnei i te `(Flutter, Acapella)` ka puta he pēhanga ki roto i ngā pukahukahu, ā, ka āwhina i te tango i te hūpē.

Ngā pokanga

Tērā pea ka hiahiatia he pokanga mō ētahi raruraru.

• Te whakawhitinga pukahukahu: He huarahi whakamutunga tēnei ina tino kino te mate, ā, kua tata kore rawa he mahi a ngā pukahukahu.
• Te whakawhitinga ate: Mena kua tino pakaru te ate.
• Ētahi atu: Ngā mahi pēnei i te tango i ngā polyp ihu, te whakauru i te ngongo whāngai ki roto i te puku hei whakarato kai.

Ngā raruraru pea o te CF

Ki te kore e rongoatia tika te CF, ka puta pea ngā momo raruraru.

• Ngā pūkahukahu: Te whānui haere tonu o ngā ara manawa ('Bronchiectasis'), te hinganga o ngā pūkahukahu ('Pneumothorax'), ngā mate pāwera mau tonu.
• Pancreatitis: Te mate huka e pā ana ki te CF (`CFRD`).
• Ate: He whara o te ate (Cirrhosis).
• Whēkau: Te aukati i te whēkau, te nui ake o te mōrearea o te mate pukupuku koroni.
• Pūnaha whakaputa uri: Te kore tamariki a te tāne me te whakaroa i te whānautanga tamariki a ngā wāhine.
• Ngā wheua: Te angiangi o ngā wheua (Osteoporosis).

Ngā Pātai Auau (FAQs)