Ua haʻi aku kāu kauka iā ʻoe a i ʻole kekahi o kou ʻohana he maʻi laha ʻole i kapa ʻia ʻo ka maʻi Pompe? Inā pēlā, e kamaʻilio mākou e pili ana i kahi lāʻau lapaʻau no ia i kēia lā. ʻO ka inoa o kēia lāʻau lapaʻau ʻo Avalglucosidase Alfa. Ua ʻike paha ʻoe iā ia ma ka inoa inoa ʻo Nexviazyme. He paʻakikī iki kēia inoa, no laila he nui paha kāu mau nīnau e pili ana i ia mea. Mai hopohopo, e kamaʻilio mākou e pili ana i kēia mau mea a pau ma ke ʻano maʻalahi loa e hiki ai iā ʻoe ke hoʻomaopopo.
I ka ʻōlelo maʻalahi, he aha ke ʻano o kēia lāʻau lapaʻau?
E noʻonoʻo e like ko kākou kino me kahi hale hana nui. No ka hana pono ʻana o nā mea āpau ma kēia hale hana, pono nā limahana e hana ana i nā hana like ʻole. Kāhea mākou i kēia mau limahana i loko o ko mākou kino he mau enzymes .
Ua hānau ʻia nā kānaka me ka maʻi Pompe me ka nele o kahi enzyme kikoʻī. ʻO ka hana a kēia enzyme, ʻo ia ke wāwahi a kāohi i ke kō i kapa ʻia ʻo glycogen i loko o ko kākou mau ʻiʻo a me nā cell. Ke nalowale kēia enzyme, hoʻomaka ke kō i kapa ʻia ʻo glycogen e hōʻiliʻili i loko o ko kākou mau ʻiʻo. Ua like ia me ka ʻōpala i loko o kahi hale hana. Ke hōʻiliʻili kēia, nāwaliwali nā ʻiʻo, paʻakikī iā lākou ke hanu, a pilikia ka puʻuwai.
No laila, ʻo ka mea a kēia lāʻau i kapa ʻia ʻo Avalglucosidase Alfa e hana ai, ʻo ia ke pani i ka hana o kēlā enzyme i nalowale a hoʻemi ʻia. ʻO ia hoʻi, ua like ia me ka lawe ʻana mai i kekahi mai waho e hana i ka hana like ma kahi o ka limahana i hoʻemi ʻia. Ke komo kēia lāʻau i loko o ke kino ma o kahi aʻa, hoʻomaka ia e wāwahi i kēlā glycogen i hōʻiliʻili ʻia a wehe iā ia. A laila e emi mālie nā hōʻailona, a hoʻomaka ʻoe e manaʻo i ka hōʻoluʻolu.
Nā mea e pono ai ʻoe e haʻi i kāu kauka ma mua o ka lawe ʻana i kēia lāʻau lapaʻau
Ma mua o ka hoʻomaka ʻana i kēia lāʻau lapaʻau, he mea nui loa ia e ʻike kāu kauka i kou kūlana olakino piha. ʻO kēia i hiki ai iā ʻoe ke mālama ʻia me ka palekana. E hōʻoia e haʻi iā lākou e pili ana i kēia mau mea.
- Inā he maʻi puʻuwai kou .
- Inā he maʻi puʻupaʻa kou.
- Inā ua loaʻa iā ʻoe kahi hopena maʻi ma mua i kēia lāʻau lapaʻau Avalglucosidase Alfa, kekahi lāʻau lapaʻau ʻē aʻe, meaʻai, kala, a mea hoʻomalu paha.
- Inā ʻoe e hāpai ana a i ʻole e hoʻāʻo ana e hāpai.
- Inā he makuahine hānai waiū ʻoe.
He mea nui loa ia no kou palekana ponoʻī e haʻi i kāu kauka i kēia ʻike me ka hūnā ʻole.
Pehea e hoʻohana ai i ka lāʻau lapaʻau?
ʻAʻole kēia he lāʻau lapaʻau hiki iā ʻoe ke hoʻohana ma ka home. Hāʻawi ʻia ia ma lalo o ka nānā ʻana a ke kauka ma kahi haukapila a i ʻole ke keʻena lapaʻau.
- He lāʻau lapaʻau i hāʻawi ʻia ma o kahi aʻa:Hāʻawi ʻia kēia lāʻau lapaʻau i loko o kahi aʻa, e like me ka saline. Kāhea mākou i kēia kaʻina hana he infusion .
- Hāʻawi ʻia e kahi hui lapaʻau: Hāʻawi ʻia kēia lāʻau lapaʻau iā ʻoe e ke kauka i aʻo ʻia a i ʻole nā limahana kahu maʻi, no laila ʻaʻohe mea e hopohopo ai.
Hiki ke hāʻawi ʻia kēia lāʻau lapaʻau i nā keiki liʻiliʻi?
ʻAe, i kekahi mau hihia, hāʻawi ʻia kēia lāʻau lapaʻau i nā keiki e like me hoʻokahi makahiki. Akā hana ʻia ia ma lalo o nā ʻōlelo aʻoaʻo lapaʻau kūikawā a me nā hana palekana. Inā ʻoe e hāʻawi ana i kēia lāʻau lapaʻau i kāu keiki, e kamaʻilio pono me kāu hui lapaʻau a wehewehe i nā mea āpau.
He aha kāu e hana ai inā piʻi ka nui o ka lāʻau?
ʻOiai he lāʻau lapaʻau kēia i hāʻawi ʻia e ka haukapila, haʻahaʻa loa ka manawa kūpono o ka overdose. Akā inā manaʻo ʻoe ua overdose paha ʻoe, mai hopohopo. ʻElua mau mea āu e hana koke ai.
1. E hele koke i ka Emergency Treatment Unit (ETU) o ka haukapila.
2. A i ʻole, e kelepona i ke Kikowaena ʻIke Lāʻau Make Lahui ma ka Halemai Lahui ʻo Colombo .
He kūlana wikiwiki loa kēia, no laila he mea nui e hana koke.
He aha inā e hala iaʻu kahi mahele lāʻau lapaʻau?
He mea nui ka lawe ʻana i kēia lāʻau lapaʻau i ka manawa kūpono a ma ka lā i kuhikuhi ʻia no ka holomua o ka lāʻau. Inā ʻaʻole hiki iā ʻoe ke hele i ka haukapila i ka lā i kuhikuhi ʻia, mai poina. E kelepona koke aku i kāu hui lapaʻau a e haʻi iā lākou. E haʻi aku lākou iā ʻoe i ka mea e hana ai ma hope.
Nā mea kūikawā e pono e ʻike
- Nā hoʻokolohua lapaʻau: Mai poina i nā hoʻokolohua maʻamau ma nā keʻena lapaʻau. E kamaʻilio me kāu kauka e pili ana inā e hoʻomaikaʻi ana kāu mau hōʻailona, e ʻino ana, a inā he mau pilikia hou kāu.
- Nā hopena aleri: Loaʻa paha i kekahi poʻe kahi hopena aleri koʻikoʻi i kēia lāʻau lapaʻau a i ʻole ma hope koke iho o ka hāʻawi ʻia ʻana iā lākou. No ka hōʻemi ʻana i kēia pilikia, hāʻawi paha kāu hui mālama ola iā ʻoe i nā lāʻau lapaʻau ʻē aʻe (e like me ka piritan) ma mua o kou hoʻomaka ʻana e lawe. No laila e hahai pono i kā lākou mau kuhikuhi.
He aha nā hopena ʻaoʻao e hiki ke loaʻa mai kēia lāʻau lapaʻau?
E like me nā lāʻau lapaʻau āpau, hiki i kēia ke hana i nā hopena ʻaoʻao i kekahi poʻe. Akā, ʻaʻole i loaʻa i nā kānaka a pau. ʻO ka mea nui, ʻo ia ka ʻike i kēlā mau hopena ʻaoʻao a me ka mea e hana ai inā loaʻa.
| ʻAno o ka hopena ʻaoʻao | Nā hōʻailona |
|---|---|
| 🚨 Nā hopena ʻaoʻao e pono e hōʻike koke ʻia i kāu hui lapaʻau | |
| Nā hopena alerika | ʻŌhūhū ʻili, maneʻo, ʻulaʻula, pehu o ka maka, nā lehelehe, ke alelo a i ʻole ka puʻu. |
| Nā hopena hoʻokomo | ʻEha ka umauma, paʻakikī ka hanu ʻana, nāwaliwali, manaʻo poniuniu. |
| 🙂 Nā hopena ʻaoʻao e pono e hōʻike ʻia i ke kauka inā hoʻomau lākou a hoʻopilikia paha | |
| Nā hopena ʻaoʻao maʻamau |
|
ʻO ka mea nui loa, inā manaʻo ʻoe i kekahi mea ʻē, ʻoiai inā he mea liʻiliʻi ia, mai makaʻu e haʻi aku i ka hui lapaʻau.
Hiki paha nā pilikia me nā lāʻau lapaʻau ʻē aʻe?
Wahi a ka ʻike o kēia manawa, ʻaʻole i hōʻike ʻia kēia lāʻau lapaʻau e hoʻoulu i nā pilikia nui me nā lāʻau lapaʻau ʻē aʻe. Eia nō naʻe, e hāʻawi i kāu kauka i kahi papa inoa piha o 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. Hiki i kēia ke kōkua i ka pale ʻana i nā pilikia pono ʻole.
Leka Lawe-Home
- ʻO ka Avalglucosidase Alfa kahi lāʻau lapaʻau e pani ai i kahi enzyme nele i loko o ke kino o ka poʻe me ka maʻi Pompe.
- Hāʻawi ʻia kēia lāʻau lapaʻau ma ka haukapila, ma o kahi aʻa (infusion). No laila, ʻaʻole e hāʻawi ʻia iā ʻoe ka lāʻau lapaʻau e lawe i ka home.
- Ma mua o ka hoʻomaka ʻana i ka lāʻau lapaʻau, e haʻi piha i kāu kauka e pili ana i kou mau maʻi āpau a me nā maʻi allergies.
- Inā loaʻa iā ʻoe kekahi mau hōʻailona koʻikoʻi e like me ka paʻakikī o ka hanu ʻana, ka ʻeha o ka umauma, a i ʻole ka ʻulaʻula o ka ʻili i ka wā a ma hope paha o ka lāʻau lapaʻau, e hoʻomaopopo koke i ka hui lapaʻau.
- E hōʻoia e lawe i kāu lāʻau lapaʻau a hele i nā keʻena lapaʻau i nā lā i hoʻonohonoho ʻia. Mai poina i nā manawa i hoʻonohonoho ʻia.
- Inā he mau nīnau hou aku kāu e pili ana i kēia lāʻau lapaʻau, e ʻoluʻolu e kūkākūkā me kāu kauka.
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