Sodium Phenylbutyrate: ʻO ka mea e pono ai ʻoe e ʻike e pili ana i kēia lāʻau lapaʻau

Ua kuhikuhi anei kāu kauka i kahi lāʻau lapaʻau i kapa ʻia ʻo Sodium Phenylbutyrate iā ʻoe a i ʻole kekahi o kou ʻohana, malia paha kāu keiki? He paʻakikī iki ka inoa, no laila e huikau iki paha ʻoe a hoihoi i ke ʻano o kēia lāʻau lapaʻau. Mai makaʻu i kekahi mea. Ma kēia ʻatikala, e kamaʻilio maʻalahi a aloha mākou e pili ana i ke kumu o kēia lāʻau lapaʻau, pehea e hoʻohana ai, a me nā mea e pono ai mākou e makaʻala.

He aha ka mea e hana maoli me kēia lāʻau lapaʻau?

I ka ʻōlelo maʻalahi, hoʻohana ʻia kēia lāʻau lapaʻau, i kapa ʻia ʻo sodium phenylbutyrate, e mālama i nā kūlana i kapa ʻia ʻo urea cycle disorders (UCDs) .

E noʻonoʻo, ma hope o ka ʻeli ʻana i nā meaʻai waiwai i ka protein a mākou e ʻai ai, ua hana ʻia kahi huahana ʻōpala i kapa ʻia ʻo ammonia i loko o ko mākou kino. I loko o ke kanaka olakino, aia ke kaʻina hana i ke kino e hoʻololi i kēia ammonia i mea ʻino ʻole i kapa ʻia ʻo urea a hoʻokuʻu iā ia i loko o ka mimi. ʻO kēia ka mea a mākou e kapa nei ʻo ke kaʻina hana urea. Eia naʻe, i loko o ke kanaka me ka maʻi kaʻina hana urea (UCD), ʻaʻole i hoʻokō pono ʻia kēia kaʻina hana. ʻO ka mea e hana ʻia, ʻo ia ka hōʻiliʻili ʻana o ka ammonia weliweli i loko o ke kino, ʻoi aku hoʻi i loko o ke koko. Hiki i kēia ke lilo i mea ʻino loa i ka lolo a me ka ʻōnaehana nerve.

ʻO kēia kahi e komo mai ai kahi lāʻau i kapa ʻia ʻo sodium phenylbutyrate. Hana kēia lāʻau ma ka hōʻemi ʻana i ka nui o ka ammonia ʻino e hōʻiliʻili ana i loko o ke kino, e hōʻemi ana i ka pilikia o nā pilikia olakino koʻikoʻi.

Nā mea e haʻi aku i kāu kauka ma mua o ka lawe ʻana i ka lāʻau lapaʻau

He mea nui i kāu kauka ke ʻike i kou kūlana olakino piha ma mua o ke kau ʻana i kēia lāʻau lapaʻau. Inā loaʻa iā ʻoe kekahi o nā kūlana i helu ʻia ma lalo nei, e hōʻoia e haʻi i kāu kauka e pili ana i ia mea.

• Inā he maʻi diabetes kou.
• Inā ʻoe e hahai ana i ka papaʻai haʻahaʻa paʻakai.
• Inā loaʻa iā ʻoe ka puʻuwai palupalu.
• Inā he maʻi puʻupaʻa kou.
• Inā he haʻahaʻa kou pae potassium i loko o kou koko.
• Inā he maʻi kou akepaʻa.
• Inā pehu kou mau wāwae, kuʻekuʻe wāwae, a lima paha.
• Inā ua loaʻa iā ʻoe kahi maʻi allergy ma mua i kēia lāʻau lapaʻau a i ʻole kekahi lāʻau lapaʻau ʻē aʻe, meaʻai, a mea hoʻoluʻu paha.
• Inā hāpai ʻoe a i ʻole ke manaʻolana nei e hāpai.
• Inā he makuahine hānai waiū ʻoe.

Ma muli o kēia ʻike, e hana ke kauka i ka hoʻolālā lapaʻau kūpono loa iā ʻoe.

Pehea e pono ai iaʻu ke hoʻohana i kēia lāʻau lapaʻau?

He lāʻau lapaʻau kēia i loaʻa ma ke ʻano o nā granules. No laila, he mea nui loa ka loaʻa ʻana o ka ʻike maikaʻi i ke ʻano o ka hoʻohana ʻana.

• Ka nui kūpono: E lawe wale i ka nui i kuhikuhi ʻia e kāu kauka. Aia kahi puna kūikawā i hana ʻia no ke ana ʻana i kēia lāʻau. E hoʻohana. Mai ana me nā tīpune o ka hale, no ka mea, ʻaʻole pololei lākou.
• Ka Manawa o ka lawe ʻana: E hoʻomaʻamaʻa i ka lawe ʻana i ka lāʻau i ka manawa like i kēlā me kēia lā.
• Pehea e lawe ai: Hiki iā ʻoe ke inu i kēia pauka i kāwili pololei ʻia me ka wai, ka wai huaʻai, a i ʻole ka waiū pēpē ʻole protein. A i ʻole, hiki iā ʻoe ke kāwili i kahi teaspoon o ia mea i loko o kahi meaʻai palupalu e like me ka applesauce.
• He mea nui loa: E ale i ka lāʻau lapaʻau i ka wā e hui pū ʻia ai. Mai nau i ka pauka lāʻau lapaʻau a i ʻole ka meaʻai i hui pū ʻia me ka lāʻau lapaʻau.
• ʻAi: Hāʻawi pinepine ʻia kēia lāʻau lapaʻau me kahi ʻai haʻahaʻa protein kūikawā . Hiki i kāu kauka ke haʻi aku iā ʻoe e lawe i nā huaora a i ʻole nā ​​​​mea hoʻohui meaʻai ʻē aʻe. E hahai pololei i kēlā mau kuhikuhi.
• No nā keiki: Inā ʻoe e hāʻawi ana i kēia lāʻau lapaʻau i kahi keiki, e hahai pono i nā ʻōlelo a kāu kauka. Aia paha he mau hana palekana kūikawā.

He aha kāu e hana ai inā ʻoe e poina e lawe i kahi lāʻau lapaʻau a overdose paha?

Ka nui o ka lāʻau

Inā manaʻo ʻoe ua lawe nui ʻoe i ka lāʻau lapaʻau, mai hoʻokaʻulua. E hele koke i ka Halemai Pilikia (ETU) kokoke loa. A i ʻole e kelepona i ke Kikowaena ʻIke ʻAi ʻona Lahui ma ka Halemai Lahui ʻo Colombo no ka ʻōlelo aʻoaʻo.

Inā ʻoe e poina i kahi mahele o ka lāʻau lapaʻau (Missed Dose)

Inā poina iā ʻoe ke lawe i kahi lāʻau, e lawe koke 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 ʻole a lawe i kāu lāʻau aʻe i ka manawa i hoʻonohonoho ʻia. Mai lawe pālua i ka lāʻau.

Nā hopena ʻaoʻao hiki ke loaʻa o kēia lāʻau lapaʻau

E like me nā lāʻau lapaʻau āpau, hiki i kēia ke loaʻa kekahi mau hopena ʻaoʻao. Mai hopohopo, ʻaʻole e loaʻa i nā kānaka a pau. Akā, he mea nui e makaʻala iā lākou.

Nā mea hou aku e ʻike ai

Nā lāʻau lapaʻau ʻē aʻe e launa pū me kēia lāʻau lapaʻau

Hiki i nā lāʻau lapaʻau ʻē aʻe āu e lawe nei ke launa pū me kēia lāʻau lapaʻau, ʻoiai:

• Haloperidol
• ʻO Probenecid
• Nā lāʻau steroid e like me Prednisone a i ʻole Cortisone
• ʻAkika Valproic

Ka mālama ʻana a me ka hoʻolei ʻana i nā lāʻau lapaʻau

• Mālama: E mālama ʻia mai ka hiki ʻana aku o nā keiki a me nā holoholona. E mālama ma ka mahana o ka lumi (20-25 Celsius) mai ka makū, a e pani pono i ka ʻōmole. E hoʻolei i ke koena o ka lāʻau lapaʻau 45 mau lā ma hope o ka wehe ʻana i ka ʻōmole.
• Ka Hoʻolei ʻana: Pono e hoʻolei palekana ʻia nā lāʻau lapaʻau i hoʻohana ʻole ʻia a i ʻole nā ​​lāʻau lapaʻau i pau ka lā. ʻOi aku ka maikaʻi o ka nīnau ʻana i kāu kauka e pili ana i kēia. Inā ʻoe e hoʻolei ana iā lākou i loko o ka ʻōpala, e kāwili i ka lāʻau lapaʻau me kahi mea e like me ke kope kope a i ʻole ka lepo a waiho i loko o kahi ʻeke i hoʻopaʻa pono ʻia.

ʻOiai ʻoe e lawe ana i kēia lāʻau lapaʻau, e kauoha kāu kauka i nā hoʻokolohua koko e nānā i kou mau pae ammonia koko a me nā mea ʻē aʻe. He mea nui loa ka hana pololei ʻana i kēia mau hoʻokolohua.