Sapropterin

Anter

• Ma mua o ka lawe ʻana i ka sapropterin,
• Hiki i ka Sapropterin ke kumu i nā hopena ʻaoʻao. E haʻi i kāu kauka inā koʻikoʻi kekahi o kēia mau ʻōuli a mai hele aku paha:
• Hiki i kekahi o nā hopena ʻaoʻao ke koʻikoʻi. Inā ʻike ʻoe i kekahi o kēia mau ʻōuli e kāhea koke i kāu kauka:
• Hiki i nā ʻōuli o ka overdose ke hoʻopili i kēia mau mea:

Hoʻohana ʻia ʻo Sapropterin me kahi papaʻai i kaupalena ʻia e kaohi ai i nā pae phenylalanine o ke koko i nā mākua a me nā keiki ʻo 1 mau makahiki a ʻoi paha o ka makahiki i loaʻa ka phenylketonuria (PKU; kālele, hoʻomanaʻo, a hoʻonohonoho i ka ʻike). E hana wale ʻo Sapropterin no kekahi poʻe i loaʻa ka PKU, a ʻo ke ala wale nō e haʻi aku inā kōkua ke sapropterin i kekahi mea maʻi e hāʻawi i ka lāʻau no kekahi manawa a ʻike inā paha e emi ana kāna pae phenylalanine. Aia ʻo Sapropterin i kahi papa o nā lāʻau i kapa ʻia he cofactors. Hana ia ma ke kōkua ʻana i ke kino e wāwahi i ka phenylalanine no laila ʻaʻole ia e kūkulu i ke koko.

Hele mai ka Sapropterin ma ke ʻano he papaha a ma ke ʻano he pauka e kāwili ʻia me nā wai a mea ʻai palupalu a lawe ʻia e ka waha. Lawe ʻia ia i hoʻokahi manawa i ka lā me ka meaʻai. Lawe i ka sapropterin ma kahi o ka manawa like i kēlā me kēia lā. E hāhai pono i nā ʻōkuhi i kāu lepili kuhikuhi, a nīnau i kāu kauka a kauka paha e wehewehe i kahi ʻāpana āu i maopopo ʻole ai. Lawe pono i ka sapropterin e like me ke kuhikuhi. Mai lawe i ka mea ʻoi aku a liʻiliʻi paha o ia mea a lawe pinepine iā ia ma mua o ka mea i kauoha ʻia e kāu kauka.

Inā ʻaʻole hiki iā ʻoe ke ale i nā papa, e kau i ka helu o nā papa sapropterin i haʻi ʻia iā ʻoe e lawe i loko o ke kīʻaha i piha ka 4 a 8 mau auneke (1/2 a 1 kīʻaha a i ʻole 120 a 240 mau mililiter) o ka wai a i ʻole ka wai ʻāpala. E hoʻoulu i ka hui a haki paha i nā papa me ka puna e hoʻonā ai i nā papa. ʻAʻole hiki i nā papa ke hoʻopau loa; aia paha he mau ʻāpana o ka papa e lana ana ma ka piko o ka wai. Ke hoʻoheheʻe ʻia ka nui o nā papa, inu i ka hui āpau. Inā noho nā ʻāpana o ka papa i loko o ke kīʻaha ma hope o kou inu ʻana i ka hui, ninini hou i ka wai a i ʻole ka wai ʻāpala i loko o ke kīʻaha a inu ia e ʻike pono ʻoe e ale i nā lāʻau āpau. E inu pono i ka hui holoʻokoʻa ma waena o 15 mau minuke ma hope o kou hoʻomākaukau ʻana. Hiki ke hoʻopili ʻia a hoʻopili ʻia me nā papa Sapropterin me nā meaʻai palupalu e like me ka ʻōpala a me ka pudding.

E hoʻomākaukau i ka pauka sapropterin, e hoʻohui i nā ʻike o ka pauka (s) me 4 a 8 auneke (1/2 a 1 kīʻaha a i ʻole 120 a 240 mau mililiter) o ka wai a i ʻole ka wai ʻāpala, a i ʻole kekahi mau meaʻai palupalu e like me ka ʻoma āpau a i ʻole ka puding. E kāwili maikaʻi loa i ka pauka i loko o ka wai a i ʻole nā ​​meaʻai palupalu a hiki i ka wā e pau ai ka pauda. E inu a ʻai paha i ka hui āpau i loaʻa iā ʻoe ka hopena piha. ʻAi a inu paha i ka hui i loko o 30 mau minuke o ka hoʻomākaukau ʻana.

Inā he makua a kahu paha ʻoe e hāʻawi ana i ka pauka i kahi keiki he 22 paona (10 kg) a ʻoi iki paha, pono ʻoe e loaʻa i nā ʻōlelo kikoʻī mai ke kauka e pili ana i ka nui o ka wai a wai wai ʻāpala paha e hoʻohana ai, a pehea ka nui o ka mea i hoʻomākaukau ʻia. hui e hāʻawi i kāu keiki. E ana i ka nui o ka wai a i ʻole ka wai ʻāpala āu e hoʻohana nei me kahi kīʻaha lāʻau a hoʻohana i kahi syringe dosing waha e ana a hāʻawi i ka lāʻau i ke keiki. Kiola i nā hui i koe ma hope o ka hāʻawi ʻia ʻana o ka lāʻau.

E hoʻomaka kāu kauka iā ʻoe ma ke koʻo o ka sapropterin a nānā pinepine i kāu pae phenylalanine pae. Inā ʻaʻole e emi kāu pae phenylalanine, hiki i kāu kauka ke hoʻonui i kāu lāʻau o sapropterin. Inā ʻaʻole e emi kāu pae phenylalanine ma hope o 1 mau mahina o ka mālama ʻana me kahi mahele kiʻekiʻe o ka sapropterin, e ʻike ʻoe a me kāu kauka ʻaʻole pane kāu ʻano i ka sapropterin. E haʻi aku kāu kauka iā ʻoe e hōʻoki i ka lawe ʻana i ka lāʻau.

Hiki i ke Sapropterin ke kōkua i ka kaohi ʻana i nā pae phenylalanine o ke koko, akā ʻaʻole ia e hoʻōla iā PKU. E hoʻomau i ka lawe sapropterin ʻoiai ʻoe e manaʻo maikaʻi. Mai hōʻoki i ka lawe ʻana i ka sapropterin me ke kamaʻilio ʻole ʻana me kāu kauka.

E noi i kāu kauka lāʻau a kauka paha no kahi kope o ka ʻike a ka mea hana no ka mea maʻi.

Kau ʻia kēia lāʻau no nā hoʻohana ʻē aʻe; e noi i kāu kauka a kauka paha no ka ʻike hou aku.

Ma mua o ka lawe ʻana i ka sapropterin,

• e haʻi i kāu kauka a me kāu kauka lāʻau inā he maʻi ʻoe i ka sapropterin a i ʻole nā ​​lāʻau ʻē aʻe.
• e haʻi i kāu kauka a me ka mea kauka lāʻau he aha nā lāʻau lapaʻau a me nā lāʻau ʻole i kuhikuhi ʻia, nā wikamina, nā mea hoʻopihaʻai, a me nā huahana lāʻau āu e lawe nei a hoʻolālā paha e lawe E ʻike pono i kekahi o kēia mau mea: levodopa (ma Sinemet, ma Stalevo); methotrexate (Otrexup, Rasuvo, Trexall, nā mea ʻē aʻe); ʻO nā mea kāohi PDE5 e like me sildenafil (Revatio, Viagra), tadalafil (Cialis), a me vardenafil (Levitra); proguanil (i Malarone), pyrimethamine (Daraprim), a me trimethoprim (Primsol, ma Bactrim, Septra). Pono paha kāu kauka e hoʻololi i nā kaha o kāu mau lāʻau a nānā pono iā ʻoe no nā hopena ʻaoʻao.
• e haʻi i kāu kauka inā he anorexia paha kāu (i kahi maʻi ʻai kahi e ʻai liʻiliʻi ai ke kanaka a / a i ʻole nā ​​hoʻoikaika nui ʻana e mālama i ke kaupaona o ke kino i manaʻo ʻia he maʻamau no kāna makahiki a me kona kiʻekiʻe) a i ʻole kekahi ʻano ʻano ʻē aʻe. kumu e mālama maikaʻi ʻole ʻia ʻoe, ai ʻole ke ake a me nā maʻi puʻupaʻa.
• e haʻi i kāu kauka inā he kuni kou a i ʻole inā maʻi ʻoe i kēlā me kēia manawa i kāu wā e lapaʻau ai. Hiki i ka fiva a me ka maʻi ke hoʻopili i kāu pae phenylalanine, no laila pono i kāu kauka e hoʻoponopono i kāu mahele lāʻau sapropterin.
• E haʻi i kāu kauka inā hāpai ʻoe, e hoʻolālā e hāpai, a i ʻole e hānai umauma ana. Inā hāpai ʻoe i ka lawe ʻana i ka sapropterin, e kāhea i kāu kauka.

Pono ʻoe e hoʻomau i ka hahai i ka papaʻai phenylalanine haʻahaʻa ʻoiai ʻoe e lawe nei i ka sapropterin. E mālama pono i nā kuhikuhi a kāu kauka a me ka mea mālama pono. Mai hoʻololi i kāu papaʻai i kēlā me kēia ʻano me ka ʻole o ke kamaʻilio ʻana me kāu kauka a i ʻole nutristist.

Inā hoʻomanaʻo ʻoe i ka lāʻau i hala ma hope ma ka lā like, e lawe i ka lāʻau i hala i kou wā e hoʻomanaʻo ai. Eia nō naʻe, inā ʻaʻole ʻoe e hoʻomanaʻo a hiki i ka lā aʻe, kāpae i ka lāʻau i hala a hoʻomau i kāu papa hana maʻamau. Mai lawe ma mua o hoʻokahi mahele lāʻau i hoʻokahi lā a i ʻole lawe i ka lāʻau pālua e pani no kahi mea i nalo.

Hiki i ka Sapropterin ke kumu i nā hopena ʻaoʻao. E haʻi i kāu kauka inā koʻikoʻi kekahi o kēia mau ʻōuli a mai hele aku paha:

• pāhoehoe
• nausea
• ka luaʻi
• ʻeha ka ʻōpū
• ʻeha poʻo
• ka ʻeha, ʻeha puʻu, a me nā ʻōuli anu
• fidgeting, hoʻoneʻe, a kamaʻilio nui paha

Hiki i kekahi o nā hopena ʻaoʻao ke koʻikoʻi. Inā ʻike ʻoe i kekahi o kēia mau ʻōuli e kāhea koke i kāu kauka:

• wheezing, shortness of breath, batuk, flushing, nausea, ʻōhū
• ʻeha ma ka ʻōpū o luna, nausea, luaʻi, ʻeleʻele, lohi a i ʻole kahi noho koko, luaʻi i ke koko

Hiki i ka Sapropterin ke kumu i nā hopena ʻē aʻe. Kāhea i kāu kauka inā he pilikia ʻokoʻa kāu i ka lawe ʻana i kēia lāʻau.

Inā ʻoe e ʻike i kahi hopena koʻikoʻi, e hoʻouna ʻoe a i ʻole kāu kauka i kahi hōʻike i ka papahana hōʻike ʻino a MedWatch Adverse Event Food (FDA) ma ka pūnaewele (http://www.fda.gov/Safety/MedWatch) a i ʻole kelepona. 1-800-332-1088).

E mālama i kēia lāʻau i loko o ka pahu i komo ai, pani paʻa ʻia, a ʻaʻole hiki i nā kamaliʻi. E mālama iā ia i kahi, kahi maloʻo, kahi maloʻo, mai kahi wela a me ka momona (ʻaʻole i loko o ka lua a me ke kaʻa). Mai wehe i ka desiccant (ʻāpana liʻiliʻi i hoʻopili ʻia me ka lāʻau e omo ai i ka waiū).

Pono e hoʻolei ʻia nā lāʻau i koi ʻole ʻia i nā ala kūikawā e hōʻoia i ka hiki ʻole i nā holoholona, ​​nā keiki, a me nā poʻe ʻē aʻe ke hoʻopau iā lākou. Eia nō naʻe, ʻaʻole pono ʻoe e kaomi i kēia lāʻau i ka lua. Akā, ʻo ke ala ʻoi loa e hoʻolilo i kāu lāʻau i loko o kahi papahana hoʻihoʻi lāʻau. E kamaʻilio me kāu kauka lāʻau a kāhea paha i kāu ʻōpala a me nā hana hoʻopōʻaiapuni kūloko e aʻo e pili ana i nā papahana hoʻihoʻi i kou kaiāulu. E ʻike i ka pūnaewele palekana o kā FDA's Disposal of Medicines (http://goo.gl/c4Rm4p) no ka ʻike hou aku inā ʻaʻohe ou komo i kahi papahana hoʻihoʻi.

He mea nui e mālama i nā lāʻau āpau mai ka maka a hiki i nā keiki e like me nā ipu (e like me nā minder pill i kēlā me kēia pule a me nā mea no nā kulu maka, nā kalima, nā pākuʻi, a me nā inhalers) ʻaʻole pale keiki a hiki i nā keiki ʻōpio ke wehe maʻalahi iā lākou. No ka pale ʻana i nā keiki ʻōpio mai ka lāʻau make, laka mau i nā pāpale palekana a kau koke i ka lāʻau i kahi palekana - kahi i luna a mamao a mai ko lākou alo a hiki. http://www.upandaway.org

I ke kumu o ka overdose, e kāhea i ka helpline kaohi lāʻau make ma 1-800-222-1222. Loaʻa ka ʻike ma ka pūnaewele ma https://www.poisonhelp.org/help. Inā ua hina ka mea i hoʻomāinoino ʻia, loaʻa ka hopu ʻana, pilikia i ka hanu ʻana, a i ʻole hiki ʻole ke ala aʻe, e kāhea koke i nā lawelawe kūikawā ma 911.

Hiki i nā ʻōuli o ka overdose ke hoʻopili i kēia mau mea:

• ʻeha poʻo
• ʻūpā

E mālama i nā manawa a pau me kāu kauka a me ka hale hana. E kauoha kāu kauka i kekahi mau hoʻokolohua lab e nānā i ka pane o kou kino i ka sapropterin.

Mai ʻae i kahi mea ʻē aʻe e lawe i kāu lāʻau. E nīnau i kāu kauka lāʻau i nā nīnau āu e pili ana i ka hoʻopiha hou ʻana i kāu kuhikuhi.

He mea nui iā ʻoe e mālama i kahi papa inoa i kākau ʻia a me nā lāʻau lapaʻau ʻole (over-the-counter) lāʻau āu e lawe nei, a me nā huahana e like me nā huaora, nā minelala, a i ʻole nā ​​mea hoʻopiliʻai aʻe. Pono ʻoe e lawe i kēia papa inoa me ʻoe i kēlā me kēia manawa ke kipa ʻoe i ke kauka a i ʻole inā ʻae ʻia ʻoe i kahi haukapila. He ʻike nui nō hoʻi ia e lawe pū me ʻoe inā pilikia ʻo ka pilikia.

• Kuvan^®