He tāngata kei roto i tō whānau kua pāngia e te mate manawa i mua i tō rātou koroheketanga, arā, i te 40-50 tau te pakeke? He mea noa rānei kia kī tonu te hunga o tō whānau, "He nui te cholesterol, he nui te cholesterol"? I te nuinga o te wā, e whakaaro ana tātou ko te take o tēnei ko ngā mea pēnei i tā tātou e kai ana, e inu ana, me te kore e whakakorikori tinana. He pono tēnā, ā, he pānga anō hoki tēnā. Engari i ētahi wā, ko te tino take o tēnei cholesterol teitei he raruraru kei roto i tō tātou tinana, i roto i ō tātou ira. Koinei te mea e kiia nei e tātou ko te 'Familial Hypercholesterolemia', ko te 'FH' rānei i roto i te rongoā. I tēnei rā, ka kōrerohia e tātou tēnei mea mā te ngāwari, i roto i te huarahi e mārama ai koe.
He aha te tikanga o te Familial Hypercholesterolemia (FH)?
Ko te Familial Hypercholesterolemia he mate ira e tukuna iho ana mai i te whaea, i te pāpā, i te tokorua rānei. Koinei te wā e tino teitei ai te cholesterol "kino", te cholesterol LDL rānei, i roto i ō tātou tinana mai i te whānautanga.
Whakaarohia ō tātou uaua toto he uaua. He rite tēnei cholesterol 'LDL' ki tētahi paparanga kōwhai, he rite ki te ware pi e piri ana ki ngā pakitara o aua uaua. I te nuinga o te wā, ka tupu haere ēnei. Engari i roto i te tangata he 'FH', he tino teitei te taumata cholesterol 'LDL', ā, ka tīmata te hanga tere me te matotoru o tēnei paparanga ware pi i roto i ngā uaua toto. Ka kiia tēnei ko te 'atherosclerosis'. I roto i te wā, ka tipu tēnei paparanga, ka whakawhāiti i ngā uaua toto, i ētahi wā ka ārai katoa i a rātou. Koinā te wā ka puta ai ngā mea morearea ki te ora pērā i ngā mate manawa me ngā whiu.
Me iti iho i te 100 mg/dL te taumata o te cholesterol `LDL` o te tangata hauora noa. Heoi, mō te tangata whai `FH`, ka nui ake pea tēnei uara, pērā i te 160 mg/dL, 190 mg/dL . I ētahi wā kino, ka neke atu pea i te 400 mg/dL. Ko te mea tino morearea ko te mea kua noho tēnei mate mai i te tamarikitanga, ki te kore e rongoatia, ka tino teitei te mōrearea o te mate ngakau i te wā taitamariki rawa atu i te tangata noa.
Ahakoa te āhua whakamataku o tēnei kōrero, ko te rongo pai, ki te kitea wawetia e koe, ki te tango i te rongoā tika, ā, ki te huri i tō momo noho, ka tata taea e koe te whakahaere katoa i tēnei āhuatanga me te noho hauora.
E rua ngā momo matua o te FH.
E rua ngā momo matua o tēnei āhuatanga, i runga i te āhua o tōna tukunga iho. He tino māmā noa iho tēnei ki te mārama.
Whakaarohia e rua ngā 'tohutohu' a ō tātou tinana mō te whakahaere i te cholesterol. Ko tētahi e whiwhi ana tātou mai i tō tātou whaea, ko tētahi mai i tō tātou pāpā.
1. Heterozygous FH: Koinei te momo tino kitea. Ko te mea e tupu ana i konei ko te hapa kei roto i te 'pukapuka tohutohu' (ira) e tukuna iho ana i te matua kotahi , te whaea, te matua rānei. Ko te tikanga he pai tetahi pukapuka, ā, he iti te hapa o tetahi atu pukapuka. Nā tēnei ka paku hē te whakahaere i te cholesterol.
2. FH Homozygous: He tino onge tēnei, ā, he tino kino hoki.Momo. Ko te mea e tupu ana i konei ko ngā 'tohutohu' e rua i riro mai i a māmā me pāpā he hapa. Kātahi ka ngoikore rawa te tikanga e whakahaere ana i te cholesterol. He tino teitei ngā taumata cholesterol 'LDL' o ēnei tāngata.
He mate ira te 'FH' kei roto i te wāhanga 'autosomal dominant'. Ko te tikanga o tēnei, kāore e hiahiatia kia riro i te tamaiti te ira hapa mai i ngā mātua e rua kia riro mai ai te mate, engari me riro mai i te mātua kotahi anake .
Ko te tikanga tēnei, ki te mea he 'FH' tōu, e 50% te tūponotanga ka riro i tāu tamaiti te mate. Waihoki, e 50% te tūponotanga ka pāngia hoki ō tuākana, tuākana rānei e te mate.
He pēhea te noa o tēnei mate? He aha ngā tohu?
E ai ki te whakatau tata, kotahi i roto i te 250 tāngata o te ao e pāngia ana e te mate noa 'Heterozygous FH'. Engari ko te mea tino pouri ko te 9 i roto i te 10 o ēnei tāngata kāore i te mōhio kei a rātou te mate .
He maha ngā wā, kāore e whakaatuhia he tohu motuhake o te FH i ngā wāhanga tōmua. Tērā pea kāore koe e mōhio kei a koe te FH tae noa ki te wā e pāngia ai koe e te mate manawa, pērā i te mamae o te uma. Engari, tērā pea kua roa rawa. Nā te mea ka tīmata te hanga o ngā putunga cholesterol i roto i ngā oko toto o te tangata whai FH mai i te rā i whānau ai rātou.
I roto i te wā, ka puta pea ngā tohu e whai ake nei i te nui haere o te cholesterol.
| Tohu | He whakamārama māmā |
|---|---|
| Te mate ngakau i te taitamarikitanga | Te mamae o te uma (angina) e puta ana i te wā e whakakorikori tinana ana, i te wā okioki rānei, i te whakaeke ngakau, i te whiu rānei i te wā taitamariki (tāne - kei raro iho i te 55 tau te pakeke, wāhine - kei raro iho i te 65 tau te pakeke). |
| Ngā Xanthomas | He puku kōwhai nā te putunga o te cholesterol i raro i te kiri. Ka kitea whānuitia ēnei i ngā tuke, i ngā turi, i ngā hononga o ngā maihao, me te taha o te uaua Achilles (i runga ake i te rekereke). |
| Ngā Xanthelasmas | Ngā putunga cholesterol kōwhai e hanga ana i raro i te kiri huri noa i ngā kamo. |
| Ākau o te Kornea | He mowhiti mā, hina, kikorangi rānei e karapoti ana i te kanohi pango. He mea noa ake tēnei i roto i te hunga kaumātua, engari ki te kitea i roto i te tangata kei raro iho i te 45 tau te pakeke , he tohu pea tēnei nō te FH. |
| Te huri i te moni | Te mamae o ngā waewae o raro, inā koa i te wā e hikoi ana. Tērā pea nā te whaiti o ngā uaua e tuku toto ana ki ngā waewae te take. |
Me pēhea te tātaritanga o te FH?
Mena kei te whakapae tō tākuta kei a koe te FH, ka tirohia e rātou ētahi mea hei whakaū i te mate.
- Whakamātautau toto: Koinei te mea tino nui. Mā te whakamātautau toto e kiia nei ko te 'Lipid Profile', e tirotiro ana i ō taumata cholesterol, ka kitea ngā taumata tiketike rawa o te cholesterol 'LDL'. Mena he nui ake i te 190 mg/dL i roto i te pakeke, he nui ake rānei i te 160 mg/dL i roto i te tamaiti, e whakapaetia ana kei a koe te 'FH'.
- Hītori hauora whānau: He mea tino nui tēnei. Ka pātai atu tō tākuta ki a koe i ngā pātai pēnei i te, "Kua pāngia tō pāpā, tō whaea, tō koroua, tō tuākana, teina rānei e ngā mate manawa i te wā e tamariki ana?" "He nui te cholesterol o tētahi o tō whānau?"
- Whakamātautau tinana: Ka tirohia tō tinana e te tākuta mō ngā tohu o te xanthomas (ngā puku kiri) te corneal arcus rānei (te mowhiti kanohi).
- Whakamātautau ira (whakamātautau DNA): I ētahi wā, ka taea te whakahaere whakamātautau ira hei whakaū i te mate. Ka taea e tēnei te tirotiro i ngā hapa o ngā ira matua e puta ai te FH (pēnei i te APOB, LDLR, PCSK9 rānei).
He aha ngā maimoatanga?
Ehara te mate 'FH' i te mate e taea te rongoā. Nā te mea he mea nō roto mai i ō tātou ira. Engari ka taea te whakahaere pai . Ko te whāinga matua o te maimoatanga he whakaiti i ngā taumata cholesterol 'LDL' i te mea ka taea, me te whakaiti i te mōrearea o te mate ngakau.
1. Ngā rongoā (ngā momo rongoā)
Kāore e ranea ngā whakarerekētanga āhua noho anake hei whakahaere i te cholesterol mō te tangata whai 'FH'. Nō reira , me tango tētahi, neke atu rānei o ngā rongoā mō te toenga o ō rātou oranga .
- Ngā Statin:Koinei ngā rongoā tino noa, ā, ko ngā rongoā raina tuatahi ēnei. Mā te whakaiti i te hanga o te cholesterol e te ate ka mahi.
- Ngā aukati PCSK9: He momo rongoā werohanga hou ēnei. He tino whai hua mō te hunga kāore e taea te whakahaere i ō rātou taumata cholesterol mā te whakamahi i ngā statin anake.
- Ezetimibe: Ko te mahi a tēnei rongoā mā te whakaiti i te mimiti o te cholesterol mai i ngā kai e kainga ana e tātou. He maha ngā wā ka hoatu ngātahitia me ngā statin.
- Ētahi atu rongoā: Hei tāpiritanga, kei reira ētahi atu rongoā pērā i ngā `Bile acid sequestrants` me ngā `Fibrates`. Mā tō tākuta e whakatau ko tēhea rongoā te mea tino pai māu.
2. Ngā huringa āhua noho
Haunga te tango rongoā, he mea nui anō hoki te whai i tētahi momo noho hauora. Ehara i te mea ka whakapiki ake i te whai huatanga o te rongoā anake tēnei, engari ka taea hoki te whakaiti i te mōrearea o te mate ngakau.
| Me aha? | Ngā mea hei karo |
|---|---|
| Ngā kai hauora mō te ngākau: Huawhenua, hua rākau, pīni, witi katoa muka (raihi parauri, ōti), ika (tāmana, makarera, hereni), miraka ngako iti me te mīti. | Ngako kukū me ngā ngako whakawhiti: Ngā kai parai, ngā kai tere, ngā hua tunu (keke, pātī), ngā mīti momona, te whakamahi nui i te hinu kokonati me te hinu nikau. |
| Whakakorikori tinana auau: Whakakorikori tinana pēnei i te hikoi tere, te oma, te eke pahikara rānei mō te 30 meneti pea ia rā, mō te iti rawa i te 5 ngā rā ia wiki. | Te hikareti me te waipiro: Me mutu rawa te hikareti. Ka tino kino te kino o ngā uaua toto. Me whakawhāiti te inu waipiro. |
| Te pupuri i te taumaha hauora: Mā te whakahaere i te taumaha tinana ka taea te whakaiti i te taumahatanga ki te ngākau. | Ngā inu reka me ngā kai he nui te huka: Ka piki ake te taumaha me ētahi atu raruraru hauora i ēnei. |
3. Ētahi atu maimoatanga motuhake
Mō te hunga he tino kino te FH homozygous, kāore pea e taea e te rongoā anake te whakahaere i ō rātou cholesterol. Mō ēnei tāngata, ka mahia he maimoatanga e kiia nei ko te LDL apheresis. He rite tēnei ki te dialysis. Ka tangohia he toto iti, ka tukuna mā roto i tētahi mīhini, ka tangohia te cholesterol LDL kino, kātahi ka werohia anō ki roto i te tinana. Ka mahia tēnei kotahi, e rua rānei i te wiki.
Mena kei a koe te FH, me aha koe?
Ki te mea kua tāpaetia koe kei te pāngia e te `FH`, ehara i te mea mōu anake. He karere nui tēnei mō tō whānau katoa.
Mena kei a koe te FH, ko tō kawenga te whakamātau i ō mātua, ō tuākana, me ō tamariki mō te cholesterol. Ka kiia tēnei he 'tātaritanga cascade'. Ka āwhina tēnei ki te tautuhi i ētahi atu mema o te whānau i mua i te puta mai o ngā tohu, ā, ka tīmata hoki te maimoatanga mō rātou.
Mena he hītori whānau tō te tamaiti mō te 'FH', ka taea te whakamātau i te cholesterol mai i te rua tau te pakeke. Nā te mea ko te wawe o te tīmatanga o te maimoatanga mō tēnei mate, ka taea te whakaiti i ngā raruraru ā muri ake nei.
Ki te mea kei a koe te 'FH', kaua e mataku. Me whakapā atu ki tō tākuta. Tangohia ō rongoā i te wā tika. Me whakarerekē i tō momo noho. Kātahi ka taea e koe te ora roa, te ora hauora pērā i te katoa.
Karere Tangohia ki te Kāinga
- Ko te Familial Hypercholesterolemia (FH) he mate ira e tukuna iho ana i tetahi whakatipuranga ki tetahi whakatipuranga, ā, ka piki ake ngā taumata o te cholesterol kino (LDL) mai i te whānautanga.
- Mena kua pāngia tetahi o tō whānau e te mate ngakau i te wā taitamariki, he nui rānei tōna cholesterol, he mea tino nui kia whakamātauria ia mō te FH.
- He tokomaha te hunga e pāngia ana e tēnei mate kāore i te mōhio kei a rātou te mate. Mā te kitenga wawe ka taea te whakaora i ngā oranga.
- He mea nui te rongoā mō te wā roa me te oranga hauora hei whakahaere i te FH.
- Ki te kitea tō mate 'FH', me whakamātau hoki ō mātua, ō tuākana, tuākana, me ō tamariki. Mā tēnei ka āwhina i a rātou ki te whakaora i ō rātou oranga.
- Mā te maimoatanga tika, ka taea e koe te ora hauora me te roa o te oranga. Nō reira kaua e mataku, me toro atu ki tō tākuta kia whiwhi maimoatanga.











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