ʻAʻole hiki ke wehewehe ʻia ka hauʻoli āu e ʻike ai ke nānā aku ʻoe i kāu pēpē hānau hou, ʻeā? Akā, a laila, ke lohe ʻoe e pili ana i kekahi o nā hoʻokolohua i hana ʻia ma ka haukapila ma hope o ka hānau ʻana o ka pēpē, manaʻo iki ʻoe i ka makaʻu a me ka hoihoi. ʻO kekahi o ia mau hoʻokolohua ʻo ia ka hoʻokolohua PKU. ʻAʻole paha ʻoe i lohe i kēia inoa. Akā, he hoʻokolohua koʻikoʻi loa kēia. I kēia lā, ke kamaʻilio nei mākou e pili ana i kēia maʻi laha ʻole i kapa ʻia ʻo PKU, akā inā ʻike mua ʻia, hiki ke hoʻomalu piha ʻia a hiki i ka pēpē ke ola i ke ola olakino.
I ka ʻōlelo maʻalahi, he aha ka PKU (Phenylketonuria)?
He maʻi hoʻoilina laha ʻole ka PKU, a i ʻole Phenylketonuria. Ua hoʻoilina ʻia mai nā mākua mai. ʻAʻole hiki i kahi pēpē me kēia maʻi ke hoʻoheheʻe pono, a wāwahi paha, i ka waikawa amino phenylalanine, ka mea i loaʻa i nā meaʻai protein a mākou e ʻai ai.
E noʻonoʻo i ko kākou kino e like me kahi hale hana nui. ʻO ka meaʻai a kākou e ʻai ai, ʻo ia nā mea maka a kākou e hoʻokomo ai i loko. Hoʻohana ʻia kēia mau mea maka e nā limahana liʻiliʻi i kapa ʻia he enzymes e hana i nā mea e pono ai ko kākou kino. ʻO kahi pēpē me PKU, he liʻiliʻi a ʻaʻohe paha hana o kahi enzyme kūikawā i kapa ʻia he phenylalanine hydroxylase (PAH), ka mea e wāwahi ai i ka phenylalanine.
No laila he aha ka mea e hana ʻia? ʻO ka Phenylalanine, ka mea i loaʻa mai ka waiū umauma a ma hope mai nā meaʻai a ka pēpē e ʻai ai, hoʻomaka e hōʻiliʻili i loko o ke koko ma kahi o ka hoʻopau ʻia ʻana mai ke kino. Ke piʻi ka nui o ka phenylalanine e hōʻiliʻili ana ma kēia ʻano, lilo ia i mea ʻawahia i ka lolo o ka pēpē . Inā ʻaʻole mālama ʻia, hiki i kēia ke hōʻino i ka lolo a hoʻoulu i nā pilikia koʻikoʻi e like me ke kīnā noʻonoʻo a me nā lohi o ka ulu ʻana.
Akā ʻo ka mea maikaʻi loa ma ʻaneʻi, ma nā ʻāina he nui, me Sri Lanka, hana nā haukapila i kēia hoʻāʻo PKU ma kēlā me kēia pēpē i hānau ʻia. No laila, hiki ke ʻike mua ʻia ka maʻi. ʻOiai hiki ke hoʻomaka ʻia ka lāʻau lapaʻau i ka wā e ʻike ʻia ai, loaʻa i ka pēpē ka manawa kūpono e ulu maʻamau a olakino hoʻi, e pale ana i nā hōʻailona koʻikoʻi mai ka ulu ʻana.
He aha nā hōʻailona o ka PKU?
ʻIke ʻia ke olakino maikaʻi o kahi pēpē me PKU i ka wā hānau. No laila, i ka wā mua, ʻaʻohe paha he mau loli i ʻike ʻia. Inā ʻaʻole mālama ʻia, e hoʻomaka nā hōʻailona e ʻike ʻia ma waena o ʻekolu a me ʻeono mahina. I loko o kēia manawa, e ʻike paha ʻoe i nā lohi iki i ka ulu ʻana o kāu pēpē.
I ka hiki ʻana o kahi pēpē i hoʻokahi makahiki, inā ʻaʻole mālama ʻia, e ʻike ʻia paha nā hōʻailona ma lalo nei.
| Hōʻailona | Wehewehena |
|---|---|
| He ʻala ʻē | He ʻala ʻē a pilau hoʻi mai ka hanu ʻana o ke pēpē, ka hou, a i ʻole ka mimi. |
| Nā loli kala | ʻOi aku ka māmā o ke kala o ka ʻili, ka lauoho, a me nā maka o ke pēpē i hoʻohālikelike ʻia me ke koena o ka ʻohana. |
| Nā maʻi ʻili | Nā maʻi ʻili e like me ka eczema. |
| Ka lohi o ka ulu ʻana | ʻAʻole e piʻi ana ke kaumaha a me ke kiʻekiʻe e like me ka makahiki (Loaʻa ka ulu ʻana). |
| Nā hiʻohiʻona ʻē aʻe | Nā hōʻailona e like me ka luaʻi ʻana, ka nausea, a me ka haʻalulu. |
| Nā hōʻailona koʻikoʻi e hiki ke kū mai inā ʻaʻole mālama ʻia | |
| Nā pilikia o ka hana | Hoʻomaulia pinepine, hana hoʻowahāwahā, a me ka hoʻāʻo e hōʻeha iā lākou iho. |
| Hana nui | Ka hana nui ʻana, ʻaʻole hiki ke noho ma kahi hoʻokahi. |
| Nā hopu ʻana | Nā kūlana e like me ka hopu ʻana . |
Pehea ka hopena o ka hāpai ʻana i kahi makuahine me PKU?
He mea nui loa kēia. Inā hāpai kahi wahine me PKU a ʻaʻole i kāohi ʻia kona kūlana PKU i loko o ia manawa, hiki iā ia ke hoʻopilikia i ka pēpē i loko o ka ʻōpū. ʻO ka piʻi ʻana o ka phenylalanine i loko o ke koko o ka makuahine hiki ke hōʻeha i ka pēpē.
Hoʻonui kēia i ka pilikia o ka hāʻule ʻana o ka naʻau a hiki ke hana i nā pilikia like ʻole no ka pēpē i hānau ʻole ʻia.
- Maʻi puʻuwai hānau
- Kekahi mau loli i ke ʻano o ka helehelena
- Kaumaha hānau haʻahaʻa
- Poʻo liʻiliʻi (microcephaly)
Akā, mai makaʻu. Inā loaʻa iā ʻoe ka PKU, e kamaʻilio me kāu kauka a e hahai i kahi papaʻai kūikawā i kou wā hāpai, hiki iā ʻoe ke loaʻa kahi pēpē olakino piha .
No ke aha e ulu ai ka PKU? He aha ke kumu?
E like me kā mākou i kūkākūkā ai ma mua, he maʻi hoʻoilina ka PKU. Hoʻokumu ʻia ia e kahi mutation i loko o kahi gene i kapa ʻia ʻo `PAH` i loko o ko mākou kino. Ke kuhikuhi nei kēia gene `PAH` i ko mākou kino e hana i ka enzyme e wāwahi i ka phenylalanine. Ke loaʻa kahi mutation i loko o ka gene, ʻaʻole i hana ʻia kēlā enzyme, a i ʻole he hemahema i kāna hana ʻana.
Hoʻoilina ʻia kēia maʻi ma ke ʻano autosomal recessive. I ka ʻōlelo maʻalahi, penei:
I mea e loaʻa ai i kahi pēpē ka maʻi, pono lākou e loaʻa i hoʻokahi kope o ka gene i hoʻololi ʻia mai ko lākou makuahine a me ko lākou makuakāne . Inā loaʻa iā lākou ka gene mai hoʻokahi makua wale nō, ʻaʻole e loaʻa i ka pēpē ka maʻi. Eia naʻe, he mea lawe paha kēlā makua. ʻO ke ʻano kēia, ʻaʻole paha lākou e loaʻa i nā hōʻailona, akā hiki iā lākou ke hoʻoili i ka gene i kā lākou mau keiki i ka wā e hiki mai ana.
Pehea e ʻike ai nā kauka i ka PKU?
Ua maʻalahi loa ka ʻike ʻana i ka PKU i kēia manawa mahalo i ka nānā ʻana i nā keiki hānau hou.
Ma waena o 24 a me 72 mau hola ma hope o ka hānau ʻana o kāu pēpē, e hou ke kauka a kahu maʻi paha ma ka haukapila i ke kuʻekuʻe wāwae o kāu pēpē me kahi nila liʻiliʻi a hōʻiliʻili i kekahi mau kulu koko ma kahi kāleka kūikawā. Ua kapa ʻia kēia he 'heel prick test.' Hoʻohana ʻia kēia hāpana koko e nānā i ka pae o ka phenylalanine i loko o ke koko o kāu pēpē.
Inā kiʻekiʻe ke kiʻekiʻe o ka phenylalanine i ke koko, hana ʻia nā hoʻāʻo koko a me ka mimi hou aʻe e hōʻoia i ka hōʻailona. I kekahi manawa, hiki ke hana ʻia kahi hoʻāʻo genetic e ʻike i ka loli genetic kikoʻī e hoʻoulu ai i ka maʻi.
He aha nā lāʻau lapaʻau no ka PKU?
ʻAʻohe lāʻau lapaʻau no ka PKU. Eia nō naʻe, hiki ke mālama maikaʻi ʻia ke ʻano i ke ola holoʻokoʻa . Hiki ke hoʻi hou mai nā hōʻailona inā hoʻōki ʻia ka lāʻau lapaʻau. ʻO ka lāʻau lapaʻau nui he meaʻai kūikawā a i kekahi manawa he lāʻau lapaʻau.
1. He ʻai kūikawā haʻahaʻa i ka phenylalanine
ʻO kēia ka ʻāpana koʻikoʻi a nui loa o ka hoʻokele PKU.Pono ke kanaka me PKU e hahai i ka ʻai haʻahaʻa i ka phenylalanine no ke koena o ko lākou ola.
ʻOiai ʻo ka phenylalanine kahi ʻāpana o ka protein, pono e kaupalena ʻia nā meaʻai waiwai i ka protein .
- ʻIʻo, iʻa, moa
- Nā hua manu
- Waiū a me nā huahana waiū (yogurt, tīhi)
- ʻO nā palaoa e like me nā lentil a me nā chickpeas
- Nā huahana soya
- Nā nati
Eia nō naʻe, ʻoiai ke kino e pono ai i kahi nui o ka protein no ka ulu ʻana, pono ʻoe e kamaʻilio me kahi meaʻai meaʻai e hoʻoholo ai i ka nui o ka protein hiki iā ʻoe ke ʻai. E hana lākou i kahi papaʻai momona e kūpono i ke keiki/kanaka me PKU.
He mea nui loa: Pono e pale loa ʻia ka mea ʻono hana ʻia ʻo 'aspartame'. Hoʻopau ʻia ʻo Aspartame i loko o ke kino e hana i ka phenylalanine. He nui nā mea inu, nā meaʻai, nā gums nau, kekahi mau huaora, a me nā syrup cough i kapa ʻia ʻo 'diet' a i ʻole 'sugar-free'. No laila, he mea nui loa e heluhelu pono i ka lepili ma mua o ka ʻai ʻana a inu ʻana paha i kekahi mea.
Pono e hoʻomaka e hāʻawi i nā pēpē hānau hou me PKU i kahi waiū kūikawā ʻaʻole i loaʻa koke ka phenylalanine.
2. Nā lāʻau lapaʻau
No kekahi poʻe maʻi PKU, he mea kōkua paha nā lāʻau lapaʻau ma waho aʻe o ka meaʻai. Pono e hoʻohana ʻia kēia mau mea ma ke ʻōlelo a ke kauka wale nō.
- ʻO Sapropterin dihydrochloride (Kuvan®): Kōkua kēia lāʻau lapaʻau i kekahi mau maʻi e wāwahi i ka phenylalanine i loko o ke kino. Eia nō naʻe, pono ʻoe e hoʻomau i ka hahai ʻana i kahi papaʻai i ka wā e lawe ana i kēia lāʻau lapaʻau.
- ʻO Pegvaliase (Palynziq®): He lāʻau pale kēia i ʻōlelo ʻia no nā poʻe mākua. Hāʻawi ia i ke kino me kahi enzyme e wāwahi i ka phenylalanine.
Hiki paha ke ola i kahi ola maʻamau me ka PKU?
ʻAe, ʻoiaʻiʻo nō! ʻOiai he maʻi ola ka PKU, inā ʻike mua ʻia a mālama pono ʻia, hiki ke hoʻēmi ʻia kona hopena i ke olakino.
Hiki ke paʻakikī i kekahi manawa ka hahai ʻana i ka ʻai haʻahaʻa-protein no ke koena o kou ola. Akā, ʻaʻole ʻoe hoʻokahi. Aia mau kāu kauka a me ka meaʻai meaʻai e kōkua iā ʻoe. Eia kekahi, ʻo ka hui pū ʻana me nā hui kākoʻo me nā poʻe ʻē aʻe me PKU hiki ke kōkua iā ʻoe e kaʻana like i nā ʻike a loaʻa ka ikaika.
Ua ʻike paha ʻoe i kekahi mau ʻōmole inu 'ʻai' a me nā ʻeke gum nau me kahi ʻōlelo aʻo e ʻōlelo ana "Phenylketonurics: Loaʻa ka phenylalanine." ʻO kēia ke hoʻomaopopo i ka poʻe me PKU aia ka aspartame i loko o ka huahana, nona ka phenylalanine.
Inā ʻoe a i ʻole kou ʻohana e hopohopo nei e pili ana i kou kūlana PKU, mai kānalua e kamaʻilio me ke kākāʻōlelo olakino noʻonoʻo . He mea nui like ke olakino noʻonoʻo me ke olakino kino.
Leka Lawe-Home
- He maʻi hoʻoilina hiki ke mālama ʻia a hiki ke kāohi ʻia ka PKU. Mai makaʻu pono ʻole iā ia.
- ʻO ka ʻike mua ʻana i ka maʻi ma o ka nānā ʻana i nā keiki hānau hou ke kī i ka holomua o ka mālama ʻana.
- ʻO ka lāʻau lapaʻau nui, ʻo ia ka hahai ʻana i kahi papaʻai kūikawā haʻahaʻa i ka phenylalanine no ke ola.
- I ke kūʻai ʻana i nā meaʻai a me nā mea inu i kapa ʻia ʻo 'Diet' a i ʻole 'Sugar-free', e nānā mau inā loaʻa ka aspartame i loko.
- E hahai pono i nā kuhikuhi a kāu kauka a me ka meaʻai meaʻai. E hoʻomau i ka launa pū me lākou i nā manawa a pau.
- Me ka mālama pono ʻana, hiki i kahi keiki a i ʻole ke kanaka me PKU ke ola i kahi ola olakino, maʻamau, a hauʻoli loa.
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