Aloha! I kēia lā, e kamaʻilio mākou e pili ana i kahi lāʻau lapaʻau i ʻano ʻē iki, akā no kekahi poʻe he mea nui loa ia no ko lākou ola. ʻO ia ʻo Sapropterin. Inā ʻoe, kekahi āu i ʻike ai, a i ʻole kāu keiki ponoʻī he maʻi laha ʻole i kapa ʻia ʻo `Phenylketonuria`, i ʻike ʻia hoʻi ʻo `(PKU)` no ka pōkole, a laila ua lohe paha ʻoe i kēia lāʻau. No laila, e nānā kākou i ke ʻano o kēia lāʻau lapaʻau, pehea e kōkua ai i ko mākou kino, a me nā mea e pono ai mākou e makaʻala loa i ka wā e hoʻohana ai.
ʻO ka mea mua, e nānā kākou, he aha ka Phenylketonuria (PKU)?
I ka ʻōlelo maʻalahi, ʻo ka 'Phenylketonuria' he maʻi genetic i hoʻoilina ʻia. ʻAʻole hiki i ke kanaka me kēia maʻi ke wāwahi pono a hana i kahi waikawa amino i kapa ʻia ʻo 'Phenylalanine'. Ke noʻonoʻo nei paha ʻoe, he aha ka 'Phenylalanine'? Loaʻa ia i nā meaʻai waiwai i ka protein a mākou e ʻai ai, no ka laʻana, ka waiū, ka ʻiʻo, ka iʻa, nā hua manu, a me nā nati.
No laila, i loko o ke kanaka me ka `PKU`, hoʻomaka kēia `phenylalanine` (Phenylalanine) e hōʻiliʻili i loko o ke kino. Ua like ia me kahi ʻōpala liʻiliʻi e hōʻiliʻili ana. ʻAʻole maikaʻi kēia hōʻiliʻili ʻana, ʻoiai no ka lolo. Inā ʻaʻole i kāohi ʻia kēia i ka wā ʻōpiopio, hiki ke keakea ʻia ka ulu ʻana o ka lolo, a hiki ke loaʻa nā kūlana e like me nā pilikia ulu noʻonoʻo, nā loli ʻano, a me ka maʻi epilepsy. ʻO ia ke kumu e pono ai ka poʻe me ka `PKU` e hahai i kahi ʻai kūikawā haʻahaʻa i ka `phenylalanine` (Phenylalanine).
No laila pehea e kōkua ai ʻo Sapropterin i kēia?
ʻO kēia kahi e komo mai ai ka lāʻau lapaʻau ʻo `Sapropterin` (Sapropterin)`. ʻAʻole ia he lāʻau lapaʻau no `PKU`, akā kōkua ia i ka hoʻēmi ʻana i ka pae o `Phenylalanine` (Phenylalanine) i loko o ke kino o kekahi mau maʻi `PKU` (ʻaʻole nā mea a pau). ʻO ia hoʻi, kōkua kēia lāʻau lapaʻau i ke kino e wāwahi iki i `Phenylalanine` (Phenylalanine)` (Phenylalanine) i kahi ʻoi aku ka maikaʻi. Akā, pono e hoʻohana pū ʻia me kēlā ʻai kūikawā. Na ke kauka e hoʻoholo inā kūpono kēia lāʻau lapaʻau iā ʻoe a i ʻole kāu keiki, a me ka nui e hāʻawi ai.
Hiki ke loaʻa kēia lāʻau lapaʻau ma lalo o nā inoa lama e like me ``JAVYGTOR`` a i ʻole ``KUVAN``.
He aha nā mea e pono iaʻu e haʻi aku i ke kauka ma mua o ka hoʻomaka ʻana i kēia lāʻau lapaʻau?
Inā loaʻa iā ʻoe a i ʻole ka mea e lawe ana i kēia lāʻau lapaʻau kekahi o kēia mau kūlana, pono ʻoe e haʻi i kāu kauka ma mua o ka hoʻomaka ʻana i ka lāʻau lapaʻau. ʻOi aku ka maikaʻi o ka hūnā ʻole ʻana i kēia mau mea, no ka mea hiki ke hoʻopilikia i kou olakino ponoʻī.
- Loaʻa iā ʻoe kekahi mau maʻi ʻai ʻē aʻe? Eia kekahi laʻana, anorexia a i ʻole bulimia.
- He kuni kou? A i ʻole he ʻano maʻi paha kāu?
- He maʻi puʻupaʻa kou?
- He maʻi akepaʻa kou?
- Ua loaʻa anei iā ʻoe nā maʻi ʻūlū, e like me ka maʻi epilepsy?
- He maʻi ʻaleʻale paha ʻoe i ka Sapropterin, nā lāʻau lapaʻau ʻē aʻe, nā meaʻai, nā kala, a i ʻole nā mea hoʻomalu?
- Ua hāpai ʻoe? A i ʻole ke manaʻolana nei ʻoe e hāpai?
- He makuahine hānai waiū ʻoe?
He mea nui loa e kūkākūkā ākea i kēia mau mea a pau me ke kauka.
ʻAe, pehea lā wau e hoʻohana ai i kēia lāʻau lapaʻau?
He mea kēia e pono ai ʻoe e ʻike pono. Pono ʻoe e hahai pololei i nā mea a ke kauka e ʻōlelo ai.
- Pono e lawe waha ʻia kēia mau pila me ka ʻai .
- E lawe i ka lāʻau lapaʻau a kāu kauka i hāʻawi ai iā ʻoe, e like me ka mea i ʻōlelo ʻia ma ka lepili kauoha, i ka nui i kauoha ʻia, i ka manawa i kauoha ʻia.
- Hiki ke ale ʻia nā papa me ka wai, a i ʻole hiki ke hoʻoheheʻe ʻia i loko o 4 a 8 auneke (ma kahi o 120 a 240 milliliters) o ka wai a i ʻole ka wai ʻāpala.
- ʻO ka mea nui loa: Pono ʻoe e inu i loko o 15 mau minuke o ka hoʻoheheʻe ʻia ʻana. Mai waiho iā ia ma waho no ka lōʻihi ma mua o kēlā.
- Inā makemake ʻoe e hoʻoheheʻe koke nā papa, hiki iā ʻoe ke kuʻi iā lākou a hoʻoulu maikaʻi paha me kahi puna i ka wā e hoʻoheheʻe ana.
- I kekahi manawa, ʻike paha ʻoe i nā ʻāpana liʻiliʻi e lana ana i loko o ka wai me ka ʻole o ka heheʻe loa ʻana o nā pila. He mea maʻamau kēia. Inā he mau ʻāpana liʻiliʻi i koe i loko o ke aniani ma hope o ka lawe ʻana i ka lāʻau lapaʻau, e hoʻohui i kahi wai hou aʻe a i ʻole ka wai ʻāpala, e hoʻowali, a inu pū i kēlā. Ma kēlā ʻano, e loaʻa iā ʻoe ka nui piha o ka lāʻau lapaʻau.
- ʻO kekahi koho ʻē aʻe, ʻo ia ke kuʻi i nā papa a hoʻohui iā lākou me kahi meaʻai liʻiliʻi, e like me ka applesauce a i ʻole ka pudding.
- He mea nui loa e lawe i kāu lāʻau lapaʻau i ka manawa like i kēlā me kēia lā, i ka manawa like i kēlā me kēia lā. Mai lawe ʻoe ma mua o ka mea a kāu kauka e ʻōlelo ai iā ʻoe, a i ʻole e hoʻōki i ka lawe ʻana i kāu lāʻau lapaʻau me ke noi ʻole aku iā ia.
He mea kūikawā inā hāʻawi ʻia i nā keiki liʻiliʻi:
Inā ʻoe e hāʻawi ana i kēia lāʻau lapaʻau i kāu keiki liʻiliʻi, pono ʻoe e kamaʻilio me kāu kauka keiki e pili ana i ia mea. ʻOiai hiki ke hāʻawi ʻia kēia lāʻau lapaʻau i nā pēpē e like me hoʻokahi mahina i kekahi mau hihia kūikawā, aia kekahi mau mea e makaʻala ai. No laila, e hahai pono i ka mea a ke kauka e ʻōlelo ai.
He aha kāu e hana ai inā lawe nui ʻoe i ka lāʻau lapaʻau?
Inā hana ʻia kēlā, ʻo ia hoʻi, inā manaʻo ʻoe ua lawe ʻoe i ka lāʻau lapaʻau ma mua o kou manaʻo, e kāhea koke i ke kikowaena hoʻomalu lāʻau make kokoke loa a i ʻole ka lumi pilikia o ka haukapila. Mai hoʻonele i ka manawa.
Nā mea nui: Nou wale nō kēia lāʻau lapaʻau. Mai hāʻawi i kāu lāʻau lapaʻau i kekahi, ʻoiai inā loaʻa iā lākou nā hōʻailona like me ʻoe.
He aha inā e hala iaʻu kahi mahele o ka lāʻau lapaʻau?
Inā hana kēia, e lawe i ka lāʻau i hala i ka wā e hoʻomanaʻo ai ʻoe. Eia nō naʻe, inā kokoke ka manawa no kāu lāʻau aʻe, e kāpae i ka lāʻau i hala a lawe i kāu lāʻau aʻe i ka manawa i hoʻonohonoho ʻia. Mai lawe i ʻelua mau lāʻau i ka manawa hoʻokahi.
Aia kekahi mau lāʻau ʻē aʻe e hiki ke launa pū me kēia lāʻau?
ʻAe, aia paha. Aia kekahi mau lāʻau lapaʻau e hiki ke launa pū me Sapropterin. Eia kekahi laʻana:
- ʻAvanafil `(avanafil)`
- ʻO Levodopa `(levodopa)`
- ʻO Methotrexate
- ʻO Pemetrexed
- ʻO Pyrimethamine
- ʻO Sildenafil `(sildenafil)`
- ʻO Tadalafil `(tadalafil)`
- ʻO Vardenafil `(vardenafil)`
ʻAʻole i piha kēia papa inoa. No laila, e haʻi i kāu kauka e pili ana i nā lāʻau lapaʻau āpau āu e lawe ai (i kauoha ʻia a kūʻai ʻia paha ma luna o ke kūʻai), nā mea kanu, nā huaora, a me nā mea hoʻohui meaʻai āu e hoʻohana ai. Eia kekahi, e haʻi i kāu kauka inā ʻoe e puhipaka, inu waiʻona, a hoʻohana paha i nā lāʻau lapaʻau kū ʻole i ke kānāwai. Hiki i kekahi mau mea ke launa pū me kēia lāʻau.
He aha kaʻu e makaʻala nui ai i ka wā e lawe ai i kēia lāʻau?
He ʻāpana koʻikoʻi loa nō hoʻi kēia.
- E nānā mau ana kāu kauka i kou kūlana. No laila, mai poina e hele i nā keʻena lapaʻau i nā lā i hoʻonohonoho ʻia a hana i kāu mau hoʻāʻo koko . ʻOi loa aku, pono ʻoe e nānā mau i kou pae phenylalanine.
- E hala paha ma kahi o 4 mau pule no ka hopena o kēia lāʻau lapaʻau ma ke kino. No laila, mai hopohopo.
- Inā ʻaʻole hoʻomaikaʻi kāu mau hōʻailona, a inā paha e ʻoi aku ka ʻino, e haʻi i kāu kauka.
- He mea nui loa: Pono ʻoe e hahai i kahi papaʻai kūikawā i ka wā e lawe ana i kēia lāʻau lapaʻau. ʻO ia hoʻi ka ʻai ʻana i nā meaʻai haʻahaʻa i ka phenylalanine. E wehewehe pono kāu kauka a meaʻai meaʻai paha i kēia iā ʻoe. Mai hoʻololi i kāu papaʻai me ka ʻole o ka ʻōlelo aʻoaʻo a kāu kauka.
He aha nā hopena ʻaoʻao e hiki ke loaʻa mai kēia lāʻau lapaʻau?
E like me nā lāʻau lapaʻau āpau, hiki i kēia ke loaʻa nā hopena ʻaoʻao liʻiliʻi. He koʻikoʻi kekahi, a ʻo kekahi ʻaʻole koʻikoʻi loa.
Inā hana ʻia kēia mau mea, pono ʻoe e haʻi koke aku i ke kauka!
- Inā he hopena alerika: ʻO ia hoʻi ka ʻulaʻula o ka ʻili, ka maneʻo, ka hives, ka pehu ʻana o ka maka, nā lehelehe, a me ke alelo.
- Inā ʻeleʻele a koko paha kou mau ʻōpala, a inā ʻoe e luaʻi i ke koko.
- Paʻakikī ka hanu ʻana.
- Inā manaʻo ʻoe ua poniuniu, ua māmā ke poʻo, a i ʻole e hāʻule ana ʻoe.
- Nā hōʻailona e like me ka hoʻoluliluli ʻana, ka hoʻomaha ʻole, ke kamaʻilio nui ʻana, a me ka holo ʻana a puni (hyperactivity). (He ʻoiaʻiʻo loa kēia no nā keiki ʻōpio)
- Inā loaʻa iā ʻoe ka ʻeha nui o ka ʻōpū.
ʻAʻole kēia mau mea he mea weliweli, akā inā hoʻomau lākou, e haʻi i kāu kauka :
- Kuke
- Nā ʻōpala wehe (diarrhea)
- ʻEha poʻo
- Ka luaʻi, ka luaʻi
- ʻeha ka ʻāʻī
- Ihu wai, ihu paʻa
ʻAʻole i hoʻokomo ʻia nā hopena ʻaoʻao āpau i kēia papa inoa. Inā ʻike ʻoe i kekahi mea ʻē, e nīnau i kāu kauka. Hiki iā ʻoe ke hōʻike i nā hopena ʻaoʻao i ka FDA (Food and Drug Administration) ma 1-800-FDA-1088.
Pehea ʻoe e mālama ai i kāu lāʻau lapaʻau?
- E mālama iā ia mai ka hiki ʻana aku o nā keiki . He mea nui loa kēia.
- E mālama ma ka mahana o ka lumi (ma waena o 20 a me 25 kekelē Celsius / 68 a me 77 kekelē Fahrenheit).
- E mālama i ka lāʻau lapaʻau i loko o ka pahu kumu i hoʻokomo ʻia ai. E pani pono i ka ʻōmole.
- Mai hoʻohana i nā lāʻau lapaʻau i pau ka lā. E hoʻolei aku iā lākou.
ʻO ka mea hope loa, he mea hoʻomanaʻo iki
He hōʻuluʻulu manaʻo wale nō kēia o nā mea a mākou i kūkākūkā ai e pili ana i ka lāʻau lapaʻau ʻo 'Sapropterin'. Inā he mau nīnau hou aku kāu e pili ana i kēia lāʻau lapaʻau, e ʻoluʻolu e nīnau i kāu kauka, kauka lāʻau, a i ʻole kekahi mea mālama ola kino ʻē aʻe. Mai makaʻu i kekahi mea a mālama iā ʻoe iho.
Nā mea nui e hoʻomanaʻo (Leka Lawe-Home)
ʻAe, no laila, e hoʻomanaʻo hou mākou iā ʻoe i nā manaʻo āu e pono ai e hoʻomanaʻo mai nā mea a mākou i kamaʻilio ai?
- ʻO Sapropterin kahi lāʻau lapaʻau e kōkua ai i ka mālama ʻana i nā pae phenylalanine i kekahi poʻe me ka phenylketonuria (PKU).
- ʻAʻole kēia he lāʻau lapaʻau , a pono e hoʻohana wale ʻia me kahi papaʻai kūikawā .
- Ma mua o ka lawe ʻana i ka lāʻau lapaʻau, e haʻi i kāu kauka e pili ana i kou mau kūlana olakino a me nā lāʻau lapaʻau āpau āu e lawe ai.
- Inā ʻoe e hoʻoheheʻe ana i ka lāʻau lapaʻau, e inu i loko o 15 mau minuke.
- He mea nui e hana i nā noiʻi kūpono i ka manawa kūpono.
- Inā loaʻa iā ʻoe kekahi mau hopena ʻaoʻao ʻē aʻe, e haʻi koke i kāu kauka .
- Mai hoʻololi i kāu meaʻai me ka ʻole o ke aʻo ʻana a kāu kauka.
Inā ua kuhikuhi ʻia kēia lāʻau lapaʻau iā ʻoe a i ʻole kāu keiki, e hahai pono i nā kuhikuhi a ke kauka e like me kāna i ʻōlelo ai iā ʻoe. A laila wale nō e hiki ai iā ʻoe ke loaʻa ka mea nui loa mai kēia lāʻau lapaʻau, e kāohi pono i kou maʻi 'PKU', a e ola i ke ola olakino. Makemake wau iā ʻoe i ka maikaʻi loa!
ʻO Sapropterin, Phenylketonuria, Phenylketonuria, PKU, Phenylalanine, Phenylalanine, Lāʻau lapaʻau, Nā hopena ʻaoʻao
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