Loaʻa iā ʻoe a i ʻole kekahi o kou ʻohana kahi maʻi i kapa ʻia ʻo Phenylketonuria? Inā pēlā, ua kuhikuhi paha kāu kauka iā ʻoe i kahi lāʻau lapaʻau i kapa ʻia ʻo Pegvaliase. ʻOiai he lāʻau lapaʻau kūikawā kēia, he mea maʻamau ka loaʻa ʻana o kekahi mau nīnau a me nā hopohopo e pili ana i ia mea. No laila i kēia lā, e kamaʻilio kākou e pili ana i kēia lāʻau lapaʻau a me nā mea āu e makaʻala ai i ka wā e hoʻohana ai.
He aha kēia lāʻau pale Pegvaliase?
I ka ʻōlelo maʻalahi, ʻo Phenylketonuria (PKU) kahi kūlana i hiki ʻole ai i ko mākou kino ke hoʻoheheʻe pono i kahi waikawa amino i kapa ʻia ʻo phenylalanine. He mea kēia i loaʻa pinepine i nā meaʻai waiwai i ka protein a mākou e ʻai ai. Ke hōʻiliʻili kēia phenylalanine i loko o ke kino o ke kanaka me PKU, hōʻiliʻili ia i loko o ke koko a hiki ke hōʻino, ʻoi aku hoʻi i ka lolo, i ka hala ʻana o ka manawa.
No laila, ʻo ka hana a kēia inikini Pegvaliase, ʻo ia ka hoʻemi ʻana i nā pae o kēlā phenylalanine makemake ʻole ʻia, keu i loko o ke koko. Loaʻa pinepine ia ma lalo o ka inoa inoa ʻo Palynziq.
Akā he mea nui e hoʻomanaʻo i kēia: ʻAʻole kēia he lāʻau lapaʻau no nā mea āpau. Eia nō naʻe, hiki iā ia ke kōkua i ka kaohi ʻana i nā pae ʻino i loko o ke koko a hoʻēmi i ka pōʻino i hana ʻia e ka maʻi.
Nā mea e pono ai ʻoe e haʻi i kāu kauka ma mua o ka lawe ʻana i ka lāʻau lapaʻau
Ma mua o ka hoʻomaka ʻana i kēia lāʻau lapaʻau, he mea nui loa ia e kamaʻilio me kāu kauka e pili ana i kēia mau mea me ka maopopo a me ka ʻoiaʻiʻo. Mai hūnā i kekahi mea.
- E haʻi mai iā mākou inā he mau maʻi ʻaleka kou iā Pegvaliase a i ʻole kekahi lāʻau lapaʻau ʻē aʻe, meaʻai, a mea hoʻoluʻu paha.
- Inā hāpai ʻoe a i ʻole ke manaʻolana nei e hāpai, e ʻōlelo pono.
- Inā he makuahine hānai ʻoe, e hoʻomaopopo i kāu kauka e pili ana i kēlā.
Ma muli o kēia ʻike, e maʻalahi i ke kauka ke hoʻoholo inā kūpono kēia lāʻau lapaʻau iā ʻoe a ʻaʻole paha.
Pehea e hoʻohana pono ai i ka lāʻau pale?
He inikini subcutaneous kēia. ʻO ka maʻamau, e hāʻawi kāu kauka a kahu maʻi paha iā ʻoe i nā kuhikuhi maopopo e pili ana i ka hāʻawi ʻana iā ʻoe iho ma ka home. He mea nui loa ia e hahai pololei i kēia mau kuhikuhi.
- E lawe i ka lāʻau lapaʻau kūpono, i ka manawa kūpono: E lawe wale i ka lāʻau lapaʻau i ka wā a ma ka nui kūpono e like me ke kauoha a kāu kauka. Mai lawe ʻoi aku ma mua o ka nui i kauoha ʻia.
- E hoʻolei pono i nā nila a me nā syringe: Mai hoʻolei i nā nila a me nā syringe i hoʻohana ʻia i loko o ka ʻōpala. Akā, e waiho iā lākou i loko o kahi pahu ʻoi kūikawā. Inā ʻaʻohe āu, e noi i kāu kauka no hoʻokahi.
- Palapala ʻike kūikawā (MedGuide):Ma mua o kēlā me kēia lāʻau lapaʻau, e hāʻawi ʻia ʻoe i kahi 'MedGuide' kūikawā, kahi pepa liʻiliʻi me nā kikoʻī e pili ana i ka lāʻau lapaʻau. E heluhelu pono mau.
He aha inā e hala iaʻu kahi mahele lāʻau lapaʻau?
Inā poina koke ʻoe i kahi lāʻau lapaʻau, mai hopohopo. Mai lawe i ka lāʻau lapaʻau i poina. E lawe i ka lāʻau lapaʻau aʻe i kou manawa maʻamau. Mai lawe pālua i ka lāʻau lapaʻau.
He aha inā wau e lawe i ka overdose?
Inā manaʻo ʻoe ua lawe nui ʻoe i ka lāʻau lapaʻau, e kāhea koke aku i ke Kikowaena ʻIke ʻAi ʻona Lahui ma ka Halemai Lahui ʻo Colombo, a i ʻole e hele i ka Emergency Treatment Unit (ETU) o ka haukapila kokoke loa. He pilikia kēia, mai hoʻokaʻulua.
Nā mea e pono e makaʻala nui ʻia i ka wā e hoʻohana ai i kēia lāʻau lapaʻau:
He mea nui loa kēia ʻāpana. Pono ʻoe e makaʻala e pili ana i kēia mau mea i ka wā holoʻokoʻa o ka hoʻohana ʻana i kēia lāʻau.
Ka hopena maʻi ʻaleka koʻikoʻi
ʻO ka pilikia nui a koʻikoʻi loa o kēia lāʻau lapaʻau, ʻo ia ka hiki ke loaʻa kahi hopena alerika koʻikoʻi, a i ʻole ma ke ʻano lapaʻau, anaphylaxis. He pilikia hoʻoweliweli kēia i ke ola.
No kēia kumu, e kuhikuhi pinepine kāu kauka i kahi epinephrine auto-injector, kahi inikini kūikawā hiki ke hoʻohana ʻia i kahi pilikia. Pono ʻoe e mālama mau me ʻoe.
E noʻonoʻo ʻoe ua loaʻa iā ʻoe kahi pana a i loko o kahi manawa pōkole, ua paʻakikī iā ʻoe ke hanu, pehu kou maka a me kou lehelehe, a manaʻo ʻoe ua poniuniu. ʻO ia hoʻi, he hopena maʻi koʻikoʻi paha kāu. Inā hana ʻia kēlā, pono ʻoe e kiʻi koke i kēlā pana epinephrine a hele i ka lumi pilikia o ka haukapila (ETU) i ka hikiwawe loa.
Ma muli o kēia pilikia, e haʻi pinepine kāu kauka iā ʻoe e lawe pū me ʻoe i kekahi i ka wā a no hoʻokahi hola ma hope o ka inikini. ʻO kēia i mea inā loaʻa iā ʻoe kahi hopena alerika koʻikoʻi, hiki i kēlā kanaka ke kōkua iā ʻoe a lawe koke iā ʻoe i ka haukapila.
Nā hoʻokolohua lapaʻau maʻamau a me ka kaohi ʻana i ka ʻai
- Nā hoʻokolohua lapaʻau: E nānā mau kāu kauka iā ʻoe i kou wā e lawe ana i kēia lāʻau. E kauoha pū ʻo ia i nā hoʻokolohua koko maʻamau (hana koko) e nānā i ka pae o ka phenylalanine i loko o kou koko. Mai hōʻalo i kēia mau mea.
- ʻAi: E hoʻomanaʻo ʻoe i ka wā a mākou i kamaʻilio ai e pili ana i ka ʻai kūikawā āu e pono ai e hahai no PKU? ʻOiai e lawe ana i kēia lāʻau lapaʻau, pono ʻoe e hahai i ka ʻai kūikawā i kuhikuhi ʻia nou. Mai hana i kekahi hoʻololi i kāu ʻai me ka ʻole o ke kūkākūkā ʻana me kāu kauka.
Nā hopena ʻaoʻao o ka lāʻau lapaʻau
E like me nā lāʻau lapaʻau āpau, hiki i kēia ke loaʻa kekahi mau hopena ʻaoʻao. Mai hopohopo, akā he mea nui e makaʻala i kēia mau mea.
| Ka hopena ʻaoʻao/hōʻailona | Wehewehena |
|---|---|
| Inā ʻike ʻoe i kēia mau hōʻailona, e haʻi koke aku i kāu kauka. | |
| Nā maʻi ʻaleʻale koʻikoʻi | ʻŌhūhū ʻili, maneʻo nui, pehu o nā lehelehe, ke alelo, ka maka, paʻakikī ka hanu ʻana. |
| ʻEha a ʻeha paha ka umauma | Ke manaʻo nei e like me ke kuʻikuʻi nei kou umauma i kou hanu ʻana. |
| Nā palpitations o ka puʻuwai | Ke manaʻo nei e kuʻi wikiwiki ana kou puʻuwai a i ʻole ke loli nei ka helu o kou puʻuwai. |
| Nā hōʻailona o ke koko haʻahaʻa | Manaʻo puni ke poʻo, māmā ke poʻo, nāwaliwali, a me ka luhi ʻē. |
| Nā hōʻailona koʻikoʻi ʻē aʻe | Ka luaʻi ʻana, ʻeha nā hono, ka pehu ʻana a i ʻole ka ʻili ʻana o ka ʻili, a me ka huikau. |
| Inā hoʻomau kēia mau hōʻailona a hoʻopilikia paha, e haʻi i kāu kauka. | |
| Nā hopena ma ka wahi inikini | ʻUlaʻula, maneʻo, ʻeha, a pehu paha ma kahi i hoʻokomo ʻia ai. |
| Nā hopena ʻaoʻao maʻamau | ʻEha ke poʻo, luli, ʻeha ka ʻōpū, kahe, kū, ʻeha ka ʻāʻī, hāʻule ka lauoho, luhi. |
Hiki paha nā pilikia me nā lāʻau lapaʻau ʻē aʻe?
ʻAe, hiki i kekahi mau lāʻau lapaʻau ke launa pū me kēia lāʻau pale. No ka laʻana, nā mea e like me ka lāʻau pale medroxyprogesterone acetate, kahi i hoʻohana ʻia no ka kaohi hānau a i ʻole ka lāʻau lapaʻau hormone.
ʻAʻole kēia he papa inoa piha. No laila, e haʻi i kāu kauka e pili ana i nā lāʻau lapaʻau āpau āu e lawe nei (me nā huaora, nā lāʻau lapaʻau lāʻau, a me nā mea hoʻohui). Eia kekahi, e haʻi i kāu kauka inā ʻoe e puhipaka a inu waiʻona paha.
Pehea e mālama pono ai i ka lāʻau lapaʻau?
He mea nui loa kēia. No ka mālama ʻana i ka maikaʻi o ka lāʻau lapaʻau, pono e mālama pono ʻia.
- E hoʻokomo i loko o ka pahu hau: E mālama i ka lāʻau lapaʻau i loko o ka pahu hau ma kahi mahana ma waena o 2 a me 8 kekelē Celsius. Mai waiho i loko o ka pahu hau.
- Mahana o ka lumi: Inā pono, hiki iā ʻoe ke mālama i kēia lāʻau lapaʻau ma ka mahana o ka lumi (20-25 kekelē Celsius) a hiki i 30 mau lā.
- ʻO ka mea nui loa: He mea nui loa ʻaʻole ʻoe e hoʻihoʻi i ka lāʻau lapaʻau i loko o ka pahu hau ma hope o ka lawe ʻia ʻana mai loko mai o ka pahu hau. Inā mālama ʻia ma ka mahana o ka lumi, pono e hoʻohana ʻia i loko o 30 mau lā. Ma hope o 30 mau lā, e hoʻolei maluhia i nā lāʻau lapaʻau i koe.
- Mai hoʻohana i ka lāʻau lapaʻau i pau ka lā.
Leka Lawe-Home
- Hana ka lāʻau pale Pegvaliase ma ka hoʻemi ʻana i ka pae o ka phenylalanine i loko o ke koko o ka poʻe me PKU. ʻAʻole ia e ho'ōla piha i ka maʻi.
- ʻO ka pilikia nui o kēia lāʻau lapaʻau he hopena alerika koʻikoʻi (anaphylaxis). E mālama mau i ka inikini epinephrine i kuhikuhi ʻia.
- E hahai i ka papaʻai kūikawā i kauoha ʻia e kāu kauka i ka wā e lawe ana i kēia lāʻau.
- E haʻi i kāu kauka e pili ana i nā lāʻau lapaʻau ʻē aʻe, nā huaora, a me nā lāʻau lapaʻau kuʻuna āu e lawe nei.
- E mālama pono i ka lāʻau lapaʻau i loko o ka pahu hau. Mai hoʻihoʻi i ka lāʻau lapaʻau i loko o ka pahu hau ke lawe ʻia i waho.
- Inā he mau nīnau a hopohopo paha kāu, mai hopohopo a e kamaʻilio mau me kāu kauka.
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