Ke kuhikuhi kāu kauka i kahi lāʻau lapaʻau i kapa ʻia ʻo Nitisinone nou a i ʻole kāu keiki, he mea maʻamau ke manaʻo iki i ka makaʻu a me ka hopohopo. ʻO kēia no ka mea ʻaʻole kēia he lāʻau lapaʻau i hoʻohana nui ʻia. Akā, mai hopohopo. Ma kēia ʻatikala, e kamaʻilio mākou e pili ana i kēia lāʻau lapaʻau ma ke ʻano maʻalahi loa e hiki ai iā ʻoe ke hoʻomaopopo. E like me ka wehewehe ʻana a kahi hoaaloha iā ʻoe.
He aha kēia lāʻau lapaʻau i kapa ʻia ʻo Nitisinone?
I ka ʻōlelo maʻalahi, ʻo Nitisinone kahi lāʻau lapaʻau e hoʻolohi i ka wāwahi ʻana o ka amino acid tyrosine i loko o ko kākou kino. Hoʻohana nui ʻia ia e mālama i kahi maʻi genetic kakaikahi loa i kapa ʻia ʻo hereditary tyrosinemia type I.
E noʻonoʻo, ʻaʻole hiki i ke kanaka me kēia maʻi ke hoʻoheheʻe pono a wāwahi paha i ka tyrosine. A laila hiki ke hōʻiliʻili i loko o ke kino a hōʻino i nā ʻōpū e like me ke akepaʻa a me nā puʻupaʻa. Hoʻōki ka lāʻau lapaʻau ʻo Nitisinone i kēlā hōʻiliʻili ʻino. Loaʻa pū kēia lāʻau lapaʻau ma lalo o ka inoa inoa ʻo Orfadin.
He aha kaʻu e haʻi aku ai i ke kauka ma mua o ka hoʻohana ʻana i kēia lāʻau lapaʻau?
Ma mua o ke kau ʻana o kāu kauka i kēia lāʻau lapaʻau, he mea nui loa ia e ʻike i kou mōʻaukala olakino piha. No laila, e hōʻoia e haʻi iā ia e pili ana i kēia mau mea:
- E haʻi mai iā mākou inā he hopena maʻi kou i ka Nitisinone a i ʻole kekahi lāʻau lapaʻau ʻē aʻe, meaʻai, a mea hoʻoluʻu paha .
- Inā hāpai ʻoe, a i ʻole e kali koke ana i kahi pēpē, e ʻōlelo pēlā.
- Inā he makuahine hānai waiū ʻoe, e haʻi mai iā mākou e pili ana i ia mea.
Ma muli o kēia ʻike, e hoʻoholo kāu kauka inā kūpono kēia lāʻau lapaʻau iā ʻoe a i ʻole pono e hoʻololi i ka dosage.
Pehea pololei ʻoe e hoʻohana ai i kēia lāʻau lapaʻau?
No ka loaʻa ʻana o ka hopena maikaʻi loa mai kahi lāʻau lapaʻau, he mea nui e hoʻohana pono.
- E lawe i kēia lāʻau lapaʻau me ke aniani wai.
- ʻO ka mea nui loa, ʻo ia ka lawe ʻana i kēia lāʻau lapaʻau ma ka liʻiliʻi he hoʻokahi hola ma mua o ka ʻai ʻana. Mai lawe pū me ka meaʻai. ʻO kēia no ka mea hiki i ka lawe ʻana me ka meaʻai ke hōʻemi i ka omo ʻana o ka lāʻau lapaʻau i loko o ke kino.
- Inā ʻoe e hāʻawi ana i nā keiki ʻōpio, hiki iā ʻoe ke wehe pono i ke capsule, hoʻoheheʻe i ka pauka lāʻau lapaʻau i loko o kahi wai liʻiliʻi, formula, a i ʻole applesauce, a hāʻawi iā lākou me ka puna. Eia nō naʻe, pono ʻoe e hāʻawi koke iā lākou ma hope o ka hoʻoheheʻe ʻana, a ʻaʻole maikaʻi ke mālama ʻana.
- E lawe i ka nui kikoʻī e like me ke kauoha a kāu kauka. Mai lawe ʻoi aku ma mua o ka nui i kauoha ʻia no kekahi kumu.
- Mai ho'ōki koke i ka lawe ʻana i ka lāʻau me ka ʻole o ke aʻo ʻana a kāu kauka.
Nou wale nō kēia lāʻau lapaʻau. Mai kaʻana like me kekahi no kekahi kumu.
Ke hāʻawi ʻia i nā keiki liʻiliʻi
Hāʻawi ʻia kēia lāʻau lapaʻau i nā pēpē hānau hou ma lalo o kekahi mau kūlana i koho ʻia. Eia nō naʻe, e ʻoluʻolu e kūkākūkā i kēia me kāu kauka pediatric a e hahai pono i kāna mau kuhikuhi.
He aha inā lawe nui wau i ka lāʻau lapaʻau?
Inā manaʻo ʻoe ua lawe hewa ʻoe ma mua o ka nui i kuhikuhi ʻia, e kelepona koke i ke Kikowaena ʻIke Poisons National ma ka Halemai Lahui ʻo Colombo, a i ʻole e hele i ka Emergency Treatment Unit (ETU) o ka haukapila kokoke loa.
He aha kāu e hana ai inā e hala ʻoe i kahi manawa hoʻopaʻa lāʻau lapaʻau?
E lawe i ka lāʻau i poina i ka wā e hoʻomanaʻo ai ʻoe. Eia nō naʻe, inā kokoke ka manawa no kāu lāʻau aʻe, e kāpae i ka lāʻau i poina a lawe i ka lāʻau aʻe i ka manawa i hoʻonohonoho ʻia. Mai lawe pālua i ka lāʻau e hoʻopiha ai i kahi lāʻau i poina.
Nā mea e makaʻala ai i ka wā e hoʻohana ai i kēia lāʻau lapaʻau
Aia kekahi mau mea nui e pono ai ʻoe e makaʻala i ka wā e lawe ana i kēia lāʻau.
1. Nā hoʻokolohua lapaʻau maʻamau: E hele i nā manawa i koho ʻia e kāu kauka i ka manawa kūpono. Pono paha ʻoe e hana i kahi haʻawina koko koʻikoʻi e ʻike ai pehea e hana ai ka lāʻau lapaʻau i loko o kou kino. Mai hōʻalo iā lākou.
2. ʻAi Kūikawā: ʻO kēia ka mea nui loa a me ka mea e pono ai. I ka wā e lawe ana i kēia lāʻau lapaʻau, pono ʻoe e kaupalena pono i nā meaʻai i loaʻa nā waikawa amino tyrosine a me phenylalanine . E hāʻawi kāu kauka a me kahi meaʻai meaʻai iā ʻoe i kahi papa inoa maopopo o nā meaʻai āu e pono ai a ʻaʻole pono e ʻai. He mea nui ka mālama pono ʻana i kēia ʻai no ka holomua o ka lāʻau.
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 nā hopena ʻaoʻao. ʻOiai ʻaʻole loaʻa i nā kānaka a pau, he mea nui e makaʻala iā lākou.
| Ka hoʻokaʻawale ʻana o nā hopena ʻaoʻao | |
|---|---|
| Nā hopena ʻaoʻao e pono ʻoe e hōʻike koke i kāu kauka | |
| Nā maʻi ʻaleka | ʻŌhūhū ʻili, maneʻo, pehu o ka maka, nā lehelehe a i ʻole ke alelo. |
| Nā pilikia maka | Nā loli o ka ʻike, ʻeha o ka maka, ʻulaʻula, ʻike i ka mālamalama. |
| Nā pilikia o ke akepaʻa | Mimi ʻeleʻele, ʻōpala keʻokeʻo, ʻeha ma ka ʻaoʻao ʻākau o luna o ka ʻōpū, melemele o nā maka a i ʻole ka ʻili (e like me ka jaundice). |
| Nā hōʻailona maʻamau | Ka nalowale o ka makemake e ʻai, ka nausea, ke kuni, a me ka malaise. |
| Nā hōʻailona koʻikoʻi ʻē aʻe | Ke kahe koko ʻana a i ʻole ka palapū maʻamau ʻole, nā puʻupuʻu ʻeha ma nā poho lima a me nā kapuwaʻi o nā wāwae. |
| Nā hopena ʻaoʻao ʻaʻole i koʻikoʻi pinepine, akā pono e hōʻike ʻia i ke kauka inā hoʻomau lākou | |
| ʻO ka ʻeha o ka ʻōpū | Ka maʻi kahe (pehu ʻana). |
| Nā loli o ka ʻili a me ka lauoho | ʻIli maloʻo, hāʻule ka lauoho. |
Inā ʻike ʻoe i kahi mea ʻē aʻe ʻaʻole ma kēia papa inoa, e kamaʻilio pū me kāu kauka e pili ana i ia mea.
Pehea ʻoe e mālama pono ai i ka lāʻau lapaʻau?
No ka mālama ʻana i ka maikaʻi o kēia lāʻau lapaʻau, he mea nui loa hoʻi e mālama pono iā ia.
- E mālama pono iā ia i loko o ka pahu hau. Pono e mālama ʻia ma kahi mahana ma waena o 2 a me 8 kekelē Celsius (36 a me 46 kekelē Fahrenheit).
- Mai mālama i loko o ka pahu hau no kekahi kumu. Mai hoʻokuʻu i ka lāʻau lapaʻau e hau.
- Mai waiho i kahi e hiki ai i nā keiki ke hopu aku.
- E hoʻolei aku i ka lāʻau lapaʻau i pau ka lā.
He hōʻuluʻulu manaʻo wale nō kēia pepa ʻike. Inā he mau nīnau hou aku kāu, mai kānalua e nīnau i kāu kauka.
Leka Lawe-Home
- He lāʻau lapaʻau kūikawā ʻo Nitisinone i hāʻawi ʻia e mālama i kahi maʻi genetic laha ʻole i kapa ʻia ʻo hereditary tyrosinemia type I.
- Pono e lawe ʻia kēia lāʻau lapaʻau i hoʻokahi hola ma mua o ka ʻai ʻana . Mai lawe pū me ka meaʻai.
- Mai ho'ōki koke i ka lawe ʻana i ka lāʻau me ka ʻole o ke aʻo ʻana a ke kauka.
- He mea pono ka ʻai kūikawā i ka wā o kēia mālama ʻana. E hahai pono i nā ʻōlelo aʻoaʻo a kāu kauka a me ka meaʻai meaʻai.
- Inā loaʻa iā ʻoe kekahi mau hopena koʻikoʻi e like me ka ʻeha o ka maka, ka melemele o ka ʻili, a i ʻole ke kahe koko ʻē , e hoʻomaopopo koke i kāu kauka.
- E mālama pono i ka lāʻau lapaʻau i loko o ka friji , kahi hiki ʻole i nā keiki ke loaʻa.
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