Ke kuhikuhi kāu kauka iā Nitisinone nou a i ʻole kāu keiki aloha, he mea maʻamau ka loaʻa ʻana o nā nīnau he nui. ʻO kēia no ka mea ʻaʻole ia he lāʻau lapaʻau no kahi maʻi maʻamau i loaʻa i nā kānaka a pau. No laila i kēia lā, e kamaʻilio kākou e pili ana i kēia lāʻau lapaʻau i kapa ʻia ʻo Nitisinone, kāna hana maoli, pehea e hoʻohana ai, a me nā hana palekana āu e pono ai e lawe, ma ke ʻano maʻalahi loa e hiki ai iā ʻoe ke hoʻomaopopo.
He aha ke ʻano o kēia lāʻau lapaʻau? He aha ka hopena me Nitisinone?
I ka ʻōlelo maʻalahi, ʻo Nitisinone kahi lāʻau lapaʻau e hoʻolohi a kāohi i ka wikiwiki o ka wāwahi ʻana o ko mākou kino i kahi waikawa amino i kapa ʻia ʻo tyrosine. E noʻonoʻo i ka tyrosine ma ke ʻano he poloka hale i hoʻohana ʻia e hana i nā protein i loko o ko mākou kino.
Hoʻohana kūikawā ʻia kēia lāʻau lapaʻau e mālama i kahi maʻi hoʻoilina laha ʻole i kapa ʻia ʻo Hereditary Tyrosinemia Type I. I ka poʻe me kēia maʻi, ʻaʻole hiki i ke kino ke wāwahi pono (metabolize) i ka tyrosine. A laila hōʻiliʻili ia i loko o ke kino a lilo i mau mea ʻawahia e hiki ke hōʻino i ke ake, nā puʻupaʻa, a me ka ʻōnaehana nerve. No laila, ʻo ka mea a Nitisinone e hana ai, ʻo ia ke kāohi i ka hana ʻia ʻana o kēlā mau mea ʻino.
Nā mea e pono ai ʻoe e haʻi i kāu kauka ma mua o ka hoʻomaka ʻana i ka lāʻau lapaʻau
Ma mua o ka hoʻomaka ʻana i kēia lāʻau lapaʻau, pono ʻoe e wehewehe pono i kēia mau mea i kāu kauka. He mea nui loa kēia no kou palekana.
- Inā loaʻa iā ʻoe kahi hopena alerika i ka Nitisinone a i ʻole kekahi lāʻau lapaʻau ʻē aʻe, meaʻai, a mea hoʻoluʻu paha.
- Inā ʻoe e hāpai ana a i ʻole e hoʻāʻo ana e hāpai.
- Inā he makuahine hānai waiū ʻoe.
Pehea e pono ai iaʻu ke hoʻohana i kēia lāʻau lapaʻau?
He mea nui loa e lawe i ka lāʻau lapaʻau e like me ke kauoha a ke kauka, ma ke ʻano kikoʻī.
- Pehea e lawe ai: I kekahi manawa hiki iā ʻoe ke hoʻoheheʻe i ka pila i loko o kahi wai liʻiliʻi a hāʻawi i kāu keiki me ka hoʻohana ʻana i kahi syringe waha. A i ʻole, hiki iā ʻoe ke kuʻi i ka pila a kāwili i kahi liʻiliʻi me kahi mea e like me ka applesauce a hāʻawi i kāu keiki. Eia naʻe, ʻaʻohe mea āu e hana ai, pono ʻoe e hoʻohana koke i ka lāʻau lapaʻau ma hope o ka hana ʻana.
- I ka manawa: E hoʻomaʻamaʻa i ka lawe ʻana i kāu lāʻau lapaʻau i ka manawa like i kēlā me kēia lā. Mai lawe pinepine iā ia ma mua o ka mea a kāu kauka e ʻōlelo ai.
- Mai ho'ōki koke i ka lawe ʻana i kēia lāʻau lapaʻau: Mai ho'ōki i ka lawe ʻana i kēia lāʻau lapaʻau me ka ʻole o ke aʻo ʻana a kāu kauka. ʻO ka hana ʻana pēlā e hoʻonui paha i kou kūlana.
Hiki ke hāʻawi ʻia kēia lāʻau lapaʻau i nā keiki liʻiliʻi?
ʻAe, hiki ke kuhikuhi ʻia kēia lāʻau lapaʻau i kahi keiki hānau hou me kēia maʻi. Akā, pono e hana ʻia ma lalo o ka nānā pono ʻana a ke kauka pediatric. Aia kekahi mau mea kūikawā e noʻonoʻo ai i ka hāʻawi ʻana i nā keiki.
Nā mea nui: Ua kuhikuhi ʻia kēia lāʻau lapaʻau nou wale nō. Mai kaʻana like me kekahi no kekahi kumu.
Nā mea kūikawā e noʻonoʻo ai i ka wā e lawe ai i ka lāʻau lapaʻau
ʻElua mau mea nui loa e pono ai ʻoe e makaʻala i ka wā e lawe ana i kēia lāʻau.
1. Nā hoʻokolohua i hoʻonohonoho ʻia a me nā nānā ʻana
He mea nui e hele i ke keʻena lapaʻau no nā nānā ʻana e like me ke kauoha a kāu kauka. ʻOiai e lawe ana i kēia lāʻau lapaʻau, pono ʻoe e hana i kekahi mau hoʻokolohua koko koʻikoʻi e hana mau ʻia e nānā i kou helu koko a me ka hana o ke akepaʻa. Mai haʻalele i kēia mau hoʻokolohua.
2. ʻAi Kūikawā
ʻO kēia ka mea nui loa . ʻO ka hahai ʻana i kahi papaʻai kūikawā he mea nui ia e like me ka lawe ʻana i kēia lāʻau lapaʻau, a ʻoi aku paha ma mua o ka lawe ʻana i kēia lāʻau.
Pono ʻoe e hahai i kahi papaʻai kūikawā i ka wā e lawe ana i kēia lāʻau lapaʻau. Pono ʻoe e kaupalena i ka nui o nā waikawa amino i kapa ʻia ʻo Tyrosine a me Phenylalanine i kāu papaʻai. E kamaʻilio me kāu kauka e pili ana i kēia a, inā pono, he meaʻai meaʻai ma kahi haukapila aupuni, a e hahai pono i kā lākou mau kuhikuhi. Inā ʻaʻole ʻoe e hahai pono i kēia papaʻai, ʻaʻole ʻoe e loaʻa i nā pono piha o ka lāʻau lapaʻau.
He aha nā hopena ʻaoʻao e hiki ke loaʻa mai kēia lāʻau lapaʻau?
E like me nā lāʻau lapaʻau āpau, hiki i ka Nitisinone ke hana i kekahi mau hopena ʻaoʻao. E hoʻokaʻawale kākou i kēia i ʻelua mau ʻano.
| Nā hopena ʻaoʻao e pono e hōʻike koke ʻia i ke kauka | |
|---|---|
| Nā hōʻailona alerika | ʻŌhūhū ʻili, maneʻo, pehu o ka maka, nā lehelehe, a i ʻole ke alelo. |
| Nā loli i nā maka | Nā loli o ka ʻike, ka ʻulaʻula o nā maka, ka ʻeha o ka maka, ka ʻike i ka mālamalama. |
| Nā pilikia o ke akepaʻa | Melemele o nā maka a i ʻole ka ʻili, ka mimi ʻeleʻele, ka ʻōpala palalulu, ka ʻeha ma ka ʻaoʻao ʻākau o luna o ka ʻōpū. |
| Nā hōʻailona koʻikoʻi ʻē aʻe | ʻO ka ʻeha a i ʻole ke kahe ʻana o ke koko ma ke ʻano ʻē, ka luhi nui, ka manaʻo wela, ka nalowale o ka makemake e ʻai, ka nausea, a me nā puʻupuʻu ʻeha ma nā poho lima a me nā kapuwaʻi o kou mau wāwae. |
| Nā hopena ʻaoʻao ʻaʻole i koʻikoʻi pinepine, akā pono e hōʻike ʻia i ke kauka inā hoʻomau lākou | |
|---|---|
| Ka maʻi pākī | Ke hele nei ma luna o kahi noho palupalu |
| ʻIli maloʻo | ʻIli maloʻo |
| Pohō lauoho | Pohō lauoho |
ʻAʻole i loko o kēia papa inoa nā hopena ʻaoʻao āpau. Inā ʻike ʻoe i kekahi mea ʻē aʻe i ʻike ʻole ʻia, e kamaʻilio me kāu kauka.
He aha kāu e hana ai inā e poina ʻoe i kahi mahele a inu nui?
He aha inā e hala iaʻu kahi mahele o ka lāʻau lapaʻau?
Inā poina iā ʻoe ke lawe i kahi lāʻau, e lawe koke iā ia i kou hoʻomanaʻo ʻana. Akā inā kokoke ka manawa no kāu lāʻau aʻe, e kāpae i ka lāʻau i poina a lawe i kāu lāʻau aʻe i ka manawa i hoʻonohonoho ʻia. Mai lawe i kahi lāʻau pālua e hoʻopiha ai i kahi lāʻau i poina.
He aha inā lawe nui wau i ka lāʻau lapaʻau?
Inā manaʻo ʻoe ua lawe nui ʻoe i kēia lāʻau lapaʻau, e kelepona koke i ke Kikowaena ʻIke ʻAi Poisons ma ka Halemai Lahui ʻo Colombo . A i ʻole, e hele i ka Halemai Pilikia (ETU) kokoke loa i ka hikiwawe loa.
Pehea e mālama ai i ka lāʻau lapaʻau?
- E mālama i kahi hiki ʻole i nā keiki .
- E mālama ma ka mahana o ka lumi (ma waena o 20 a me 25 kekelē Celsius).
- E hoʻolei aku i ka lāʻau lapaʻau i pau ka lā.
Leka Lawe-Home
- He lāʻau lapaʻau kūikawā ʻo Nitisinone e pono e lawe ʻia e like me ke kauoha a ke kauka a ma ka nui i kauoha ʻia.
- Ma waho aʻe o ka lawe ʻana i kēia lāʻau lapaʻau, he mea nui hoʻi e hahai i kahi papaʻai kūikawā (haʻahaʻa i ka tyrosine a me ka phenylalanine) e pili ana me ia.
- Mai ho'ōki i ka lawe ʻana i ka lāʻau lapaʻau me ke aʻo ʻole ʻia e ke kauka.
- E hele i nā keʻena lapaʻau i nā lā i hoʻonohonoho ʻia a e hana i nā hoʻāʻo koko a pau e pono ai.
- Inā ʻike ʻoe i kekahi mau hopena koʻikoʻi, e like me ka melemele o nā maka a i ʻole ka ʻeha ʻē, e hoʻomaopopo koke i kāu kauka.
- E mālama i nā lāʻau lapaʻau ma kahi palekana mai ka hiki ʻana aku o nā keiki.
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