Malia paha ua haʻi ʻia ʻoe a i ʻole kekahi o kou ʻohana e kāu kauka e pili ana i kahi lāʻau hou a maʻa ʻole. Hiki ke kapa ʻia ʻo Olipudase Alfa (Xenpozyme). ʻAʻole kēia he lāʻau maʻamau a hoʻohana pinepine ʻia a mākou e lohe pinepine ai. He lāʻau kēia i hāʻawi ʻia no kahi maʻi kūikawā loa. ʻO ia ke kumu he mea maʻamau iā ʻoe ke loaʻa nā nīnau he nui e pili ana i ia mea. Loaʻa paha iā ʻoe nā nīnau e like me "He aha ka mea e hana ʻia me kēia?", "Palekana anei kēia?", "He aha nā mea e makaʻala ai?" Mai hopohopo, e kamaʻilio maopopo a maʻalahi mākou e pili ana i kēia lāʻau, kāna hana, a me nā mea e makaʻala ai.
He aha ka mea e hana maoli me kēia lāʻau lapaʻau?
Hoʻohana ʻia kēia lāʻau lapaʻau e mālama i kahi maʻi hoʻoilina laha ʻole loa. I ka ʻōlelo lapaʻau, ua kapa ʻia ʻo ia ʻo Acid Sphingomyelinase Deficiency , a i ʻole (ASMD) no ka pōkole.
I ka ʻōlelo maʻalahi, ʻaʻole hana ka poʻe me ASMD i kahi enzyme i hana maoli ʻia i loko o ko lākou kino. ʻO ka enzyme kahi ʻano protein e kōkua ana i nā kaʻina hana kemika like ʻole i loko o ko mākou kino. Ke nele kēia enzyme kikoʻī, hoʻomaka kahi ʻano lipid kūikawā i kapa ʻia ʻo sphingomyelin e hōʻiliʻili i loko o nā ʻōpū koʻikoʻi e like me ko mākou ake, spleen, a me nā māmā. E noʻonoʻo iā ia e like me kahi paipu wai e pani ʻia ana e ka lepo a i ka hopena e pani loa ʻia.
ʻO kēia kahi e hele mai ai kahi lāʻau lapaʻau i kapa ʻia ʻo Olipudase Alfa i ke kōkua. ʻO ka hana a kēia lāʻau lapaʻau, ʻo ia ka hāʻawi ʻana i ka enzyme i nele i ke kino mai waho. Ua kapa ʻia kēia he enzyme replacement therapy . A laila e emi mālie ana ka nui o ka momona i hōʻiliʻili pono ʻole ʻia, e kāohi ʻia ka pōʻino i nā ʻōpū, a e hoʻomaha ʻia nā hōʻailona.
Nā mea e pono ai ʻoe e haʻi i kāu kauka ma mua o ka hoʻohana ʻana i kēia lāʻau lapaʻau
Ma mua o kou hoʻomaka ʻana e lawe i kēia lāʻau lapaʻau, he mea nui e hoʻomaopopo i kāu kauka e pili ana i kēia mau mea. Mai hūnā i kēia mau mea, no ka mea, he mea nui loa ia no kou palekana.
- Inā he maʻi ʻaleka kou i kēia lāʻau lapaʻau, ʻo Olipudase alfa, kekahi lāʻau lapaʻau ʻē aʻe, meaʻai, nā kala a i ʻole nā mea hoʻomalu i hoʻohui ʻia i ka meaʻai.
- Inā hāpai ʻoe, a i ʻole ke kali nei i kahi pēpē i ka wā e hiki mai ana , he mea nui loa kēia. E kamaʻilio hou aku kākou e pili ana i ia mea.
- Inā he makuahine hānai ʻoe.
Pehea e hāʻawi ai i ka lāʻau lapaʻau a me nā mea e pono ai ke ʻike
Hāʻawi ʻia kēia lāʻau lapaʻau ma ke ʻano he inikini i loko o kahi aʻa. I ka pololei, ua inikini ʻia i loko o ke kino ma o kahi aʻa, e like me ka wai saline. Hana pinepine ʻia kēia kaʻina hana ma kahi haukapila a i ʻole ke keʻena lapaʻau, ma lalo o ka nānā ʻana a ke kauka a i ʻole nā limahana kahu maʻi i aʻo ʻia.
Eia nō naʻe, i kekahi mau hihia, hiki paha iā ʻoe ke kiʻi ma ka home. Inā pēlā, e hāʻawi ʻia ʻoe i ke aʻo piha ʻana i ka hoʻomākaukau pono ʻana a me ka hoʻokomo ʻana i ka inikini. Pono ʻoe e hahai i nā kuhikuhi e like me ka mea a ke kauka e ʻōlelo ai iā ʻoe.
Ke hoʻolei ʻana i nā nila a me nā syringe
Mai hoʻolei i nā nila a me nā syringe āu e hoʻohana ai ma ka home i loko o ka ʻōpala maʻamau . Aia kahi pahu ʻoi kūikawā no lākou. Inā ʻaʻohe āu, e nīnau i kāu kauka a i ʻole ka haukapila e pili ana i hoʻokahi.
Ke hāʻawi nei ʻoe i kēia i nā keiki liʻiliʻi?
ʻAe, hiki ke kuhikuhi ʻia kēia lāʻau lapaʻau no nā pēpē hānau hou a me nā keiki no nā kūlana olakino kikoʻī. Eia nō naʻe, aia kekahi mau mea e makaʻala ai. No laila, inā ʻoe e hāʻawi ana i kēia lāʻau lapaʻau i kahi keiki, e kamaʻilio pono me ke kauka e pili ana i ia mea a wehewehe i nā mea āpau.
He aha ka mea e hana ai inā hoʻonui ʻia ka nui o ka lāʻau lapaʻau?
Inā manaʻo ʻoe ua lawe nui ʻoe i ka lāʻau lapaʻau, mai hopohopo a kāhea koke aku i ke Kikowaena ʻIke ʻAi Poisons Lahui ma ka Halemai Lahui , a i ʻole e hele i ka Emergency Treatment Unit (ETU) o ka haukapila kokoke loa.
Nā mea kūikawā e makaʻala ai a me nā hopena ʻaoʻao
ʻOiai ʻoe e lawe ana i kēia lāʻau lapaʻau, e noi aku kāu kauka iā ʻoe e hele mai no nā nānā maʻamau e nānā i kou kūlana. Pono nō hoʻi lākou e hana i nā mea e like me ke koko. Hiki ke lōʻihi ka manawa no ʻoe e ʻike ai i nā pono piha o kēia lāʻau lapaʻau, no laila he mea nui e hoʻomanawanui i kāu lāʻau lapaʻau.
Hiki i kekahi poʻe ke loaʻa i nā hopena alerika koʻikoʻi a i ʻole nā hopena infusion ke hāʻawi ʻia kēia lāʻau. No ka hōʻemi ʻana i kēia pilikia, hiki i kāu kauka ke hāʻawi iā ʻoe i nā lāʻau lapaʻau ʻē aʻe (e like me ka piritan) ma mua o ka hāʻawi ʻana iā ʻoe i kēia lāʻau. E hahai pono i nā kuhikuhi.
ʻO ka hāpai ʻana a me ka kaohi hānau: He mea nui loa kēia. ʻO ka lawe ʻana i kēia lāʻau lapaʻau i ka wā hāpai hiki ke hana i nā kīnā hānau koʻikoʻi i ka pēpē. No laila, he mea nui e hoʻohana i kahi ʻano kaohi hānau kūpono i ka wā e lawe ana i kēia lāʻau lapaʻau a no 14 mau lā ma hope o ka hoʻōki ʻana i ka lāʻau lapaʻau. E kamaʻilio me kāu kauka e pili ana i kēia a koho i ke ʻano hana kūpono loa iā ʻoe.
Hōʻike ka papa ma lalo nei i nā ʻano hopena ʻaoʻao nui e hiki ke hana me kēia lāʻau lapaʻau.
| Nā hopena ʻaoʻao o ka lāʻau pale ʻo Olipudase Alfa | |
|---|---|
| Nā hopena ʻaoʻao koʻikoʻi e pono ʻoe e hōʻike koke i kāu kauka | |
| Nā hopena maʻi a i ʻole Angioedema | ʻŌhūhū ʻili, maneʻo, pehu o ka maka, nā maka, nā lehelehe, ke alelo, nā lima a i ʻole nā wāwae, paʻakikī ke ale a i ʻole ka hanu ʻana. |
| Nā hopena hoʻokomo | ʻEha ka umauma, paʻakikī ka hanu ʻana, nāwaliwali, manaʻo poniuniu. |
| Nā hōʻailona o ka hōʻeha o ke akepaʻa | ʻEha ma ka ʻaoʻao ʻākau o luna o ka ʻōpū, nalowale ka makemake e ʻai, nausea, ʻōpala māmā, mimi melemele ʻeleʻele a ʻeleʻele paha, melemele o nā maka a i ʻole ka ʻili, luhi ʻē. |
| Nā hopena ʻaoʻao ʻaʻole i koʻikoʻi pinepine, akā pono e hōʻike ʻia i ke kauka inā hoʻomau lākou | |
| Nā hiʻohiʻona ʻē aʻe | Kuke, luaʻi (diarrhea), kuni, ʻeha poʻo, ʻeha hui, nausea, luaʻi. |
Aia kekahi pilikia me nā lāʻau lapaʻau ʻē aʻe?
E like me kā mākou ʻike, ʻaʻole i hōʻike ʻia kēia lāʻau lapaʻau e hoʻopili nui me nā lāʻau lapaʻau ʻē aʻe. Eia naʻe, mai manaʻo ʻoe ʻo ia ke ʻano. E hāʻawi i kāu kauka i kahi papa inoa piha o nā lāʻau lapaʻau āpau āu e lawe ai (me nā huaora, nā lāʻau lapaʻau lāʻau, a me nā mea hoʻohui). Eia kekahi, e hōʻoia e haʻi i kāu kauka inā ʻoe e puhipaka a inu waiʻona paha.
Leka Lawe-Home
- ʻO Olipudase Alfa kahi lāʻau lapaʻau pani enzyme kūikawā no kahi maʻi genetic laha ʻole i kapa ʻia ʻo ASMD.
- Hāʻawi ʻia kēia ma ke ʻano he injection i loko o kahi aa koko, maʻamau i loko o kahi haukapila.
- Ma mua o ka hoʻomaka ʻana i ka lāʻau lapaʻau, e haʻi i kāu kauka e pili ana i kāu mau maʻi allergies āpau, nā hoʻolālā hāpai, a me nā lāʻau lapaʻau ʻē aʻe āu e lawe nei.
- ʻOiai e lawe ana i kēia lāʻau lapaʻau, e makaʻala i nā hōʻailona o nā hopena alerika koʻikoʻi, nā hopena injection, a i ʻole ka hōʻino ʻana o ke akepaʻa (e laʻa, ka melemele o ka ʻili, ka mimi ʻeleʻele). E haʻi koke i kāu kauka inā ʻike ʻoe i kekahi o kēia mau hōʻailona.
- Mai hāpai i ka wā e lawe ana i kēia lāʻau lapaʻau. E kamaʻilio me kāu kauka e pili ana i ka kaohi hānau kūpono.
- E hahai pono i nā ʻōlelo a kāu kauka a hele i nā manawa hoʻonohonoho lapaʻau a pau i hoʻonohonoho ʻia.
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