Ua haʻi aku kāu kauka iā ʻoe a i ʻole kekahi o kou ʻohana i loaʻa i ka maʻi Pompe a pono ʻoe e hoʻomaka e lawe i kahi lāʻau pale i kapa ʻia ʻo Avalglucosidase Alfa ma ke ʻano he lāʻau lapaʻau? He mea maʻamau ke manaʻo iki i ka makaʻu a me ka hopohopo ke lohe ʻoe i kahi inoa e like me kēia. Akā, mai hopohopo. Ma kēia ʻatikala, e kamaʻilio mākou e pili ana i ke ʻano o kēia lāʻau lapaʻau, kāna hana, a me nā mea e pono ai mākou e makaʻala.
He aha maoli kēia lāʻau lapaʻau? (He aha ka Avalglucosidase Alfa?)
I ka ʻōlelo maʻalahi, ʻo Avalglucosidase Alfa he kope i hana ʻia e ke kanaka o kahi enzyme i hana maoli ʻia i loko o ko kākou kino. Kūʻai ʻia aku ia ma lalo o ka inoa ʻo Nexviazyme . No loko ia o kahi papa o nā lāʻau i kapa ʻia ʻo Enzyme Replacement Therapy .
E noʻonoʻo, ʻo ka maʻi Pompe kahi kūlana kahi e hōʻemi ai a hoʻopau loa paha kahi kīnā genetic i loko o ko mākou kino i ka hana ʻana o kahi enzyme kikoʻī. Ke nalowale kēia enzyme, hoʻomaka kahi ʻano kō i kapa ʻia ʻo glycogen e hōʻiliʻili i loko o kā mākou mau cell, ʻoiai nā cell muscle. Hoʻoulu kēia hōʻiliʻili i nā hōʻailona e like me ka nāwaliwali o nā ʻiʻo a me ka paʻakikī o ka hanu ʻana.
No laila, ʻo ka hana a kēia lāʻau pale Avalglucosidase Alfa , ʻo ia ke hāʻawi ʻana i ka enzyme i nalowale i ke kino mai waho. Ua like ia me ka hoʻopiha ʻana i kahi pahu me ka wai mai waho. Akā, ʻaʻole hiki i kēia ke ho'ōla piha i ka maʻi. Eia nō naʻe, kōkua nui kēia lāʻau i ka kaohi ʻana i ka pōʻino i hana ʻia e ka maʻi a me ka hōʻemi ʻana i nā hōʻailona a me ka hoʻomaʻalahi ʻana i ke ola.
Nā mea e pono ai ʻoe e haʻi i kāu kauka ma mua o ka lawe ʻana i ka lāʻau lapaʻau
He mea nui loa ia e kūkākūkā i kou mōʻaukala olakino piha me kāu kauka ma mua o ka hoʻomaka ʻana i kēia lāʻau lapaʻau. Pono ʻoe e haʻi kūikawā i kekahi o kēia mau kūlana:
| Kūlana olakino a i ʻole ka manawa kūpono | No ke aha ʻoe e hōʻike ai? |
|---|---|
| Maʻi puʻuwai a i ʻole ka hāʻule ʻana o ka puʻuwai | Ma muli o ka hiki i kēia lāʻau lapaʻau ke hoʻopilikia i ke kaulike wai o ke kino, he mea nui e makaʻala i nā kūlana puʻuwai. |
| Maʻi puʻupaʻa | Inā he pilikia o ka hanu ʻana, pono e makaʻala loa ka hui lapaʻau e pili ana i ia mea. |
| Nā maʻi ʻaleka i nā lāʻau lapaʻau, nā meaʻai, a i ʻole nā mea ʻē aʻe | He mea nui e ʻike inā he maʻi ʻoe i ka Avalglucosidase Alfa a i ʻole kekahi o kāna mau mea hoʻohui. |
| Inā hāpai ʻoe a i ʻole e hoʻāʻo nei e hāpai | Pono ʻoe e kūkākūkā i ka palekana o kēia lāʻau lapaʻau i ka wā hāpai me kāu kauka. |
| Inā hānai ʻia ka pēpē me ka waiū | He mea nui e ʻimi i ka ʻōlelo aʻo lapaʻau e pili ana i ka hele ʻana o ka lāʻau lapaʻau i ka pēpē ma o ka waiū umauma. |
Pehea e hāʻawi ʻia ai kēia lāʻau lapaʻau?
ʻAʻole ʻo Avalglucosidase Alfa kahi lāʻau lapaʻau hiki iā ʻoe ke lawe i ka home. He inikini ia i hāʻawi ʻia ma o kahi hopena saline i loko o kahi aʻa (infusion intravenous) . ʻO ke ʻano kēia, pono ʻoe e hele i kahi haukapila a i ʻole kahi keʻena lapaʻau kūikawā e loaʻa ai kēia lāʻau. Ma laila, e hāʻawi ke kauka a kahu maʻi paha iā ʻoe i ka inikini.
- No nā kamaliʻi ʻōpio: Hāʻawi ʻia kēia lāʻau lapaʻau i kekahi manawa i nā keiki ma luna o hoʻokahi makahiki. Eia nō naʻe, na ke kauka pediatric ka hoʻoholo. Ua hahai ʻia nā hana palekana kūikawā no kēia.
- He aha inā e hala wau i kahi lāʻau? He mea nui loa ia e lawe i kēia lāʻau lapaʻau i ka lā i hoʻonohonoho ʻia. Inā hala ʻoe i kahi lā i hoʻonohonoho ʻia, e kāhea koke i kāu kauka a e hoʻomaopopo iā lākou i hiki iā lākou ke hoʻonohonoho i kahi manawa ʻē aʻe.
- He aha inā e hoʻonui ʻia ka nui o ka lāʻau? ʻOiai ua hāʻawi ʻia kēia ma kahi haukapila, ua haʻahaʻa loa ka manawa e hiki ai kēia. Eia nō naʻe, inā manaʻo ʻoe ua lawe nui ʻoe i ka lāʻau lapaʻau, pono ʻoe e hoʻomaopopo koke i ka hui lapaʻau. A i ʻole, hiki iā ʻoe ke kelepona i ke Kikowaena ʻIke Poisons National ma ka Halemai Lahui ʻo Colombo a i ʻole e hele i ka Emergency Treatment Unit (ETU) .
Nā mea e makaʻala ai i ka wā e lawe ana i ka lāʻau lapaʻau
Ma hope o kou hoʻomaka ʻana i ka lāʻau lapaʻau, e nānā mau kāu kauka iā ʻoe e ʻike i kou ʻano. E hōʻoia e hele no kēia mau nānā ʻana. Inā ʻaʻole e hoʻomaikaʻi kāu mau hōʻailona a i ʻole e ʻoi aku ka ʻino, e haʻi i kāu kauka.
He mea nui: Hiki i kēia lāʻau lapaʻau ke hoʻoulu i nā hopena alerika koʻikoʻi i kekahi poʻe. 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 lāʻau i kapa ʻia ʻo piritan) ma mua o ka hāʻawi ʻana iā ʻoe i kēia lāʻau pale. No laila e hahai pono i nā ʻōlelo a kāu kauka.
E launa pū anei me nā lāʻau lapaʻau ʻē aʻe?
ʻAʻohe hōʻike i kēia manawa no nā pilina koʻikoʻi ma waena o Avalglucosidase Alfa a me nā lāʻau ʻē aʻe. Eia nō naʻe, e hōʻoia e haʻi i kāu kauka e pili ana i nā lāʻau āpau āu e lawe ai (me nā lāʻau lapaʻau no nā kūlana ʻē aʻe, nā huaora, nā lāʻau lapaʻau lāʻau, a me nā mea hoʻohui) . E haʻi pū i kāu kauka inā ʻoe e puhipaka a inu waiʻona paha, no ka mea, hiki i kekahi o kēia mau mea ke hoʻopilikia i kāu mālama ʻana.
He aha nā hopena ʻaoʻao e hiki ke loaʻa mai ka lāʻau lapaʻau?
E like me nā lāʻau lapaʻau āpau, hiki i kēia ke loaʻa kekahi mau hopena ʻaoʻao. Akā, ʻaʻole i loaʻa i nā kānaka a pau. E hoʻokaʻawale kākou i kēia i ʻelua mau ʻano.
| Nā hopena ʻaoʻao e pono e hōʻike koke ʻia i ke kauka | |
|---|---|
| Nā hopena alerika | ʻŌhūhū ʻili, maneʻo, pehu o ka maka, nā lehelehe a i ʻole ke alelo. |
| Ka pilikia o ka hanu ʻana | Pōkole o ka hanu, hanu wikiwiki. |
| ʻEha umauma | Ka manaʻo o ka ʻūmiʻi a i ʻole ka ʻūmiʻi ʻana i loko o ka umauma. |
| Ke koko haʻahaʻa | Pōʻino, ʻike maka pōʻeleʻele, manaʻo e hāʻule paha ʻoe i ke kū ʻana, luhi nui. |
| Ka holoi ʻana | ʻUlaʻula koke a me ka manaʻo o ka wela ma ka maka a me ka ʻāʻī. |
| ʻAʻole maʻamau ka koʻikoʻi (akā e haʻi i kāu kauka inā hoʻomau ia) | |
| ʻEha poʻo | ʻEha poʻo maʻamau. |
| Ka luaʻi | Manaʻo e luaʻi ana. |
| Ka maʻi pākī | ʻŌpū. |
| ʻEha ʻiʻo a hui paha | ʻEha i nā ʻiʻo a i ʻole nā hono. |
ʻAʻole i hoʻokomo ʻia nā hopena ʻaoʻao āpau i kēia papa inoa. Inā loaʻa iā ʻoe kekahi mea ʻē, mai makaʻu e kamaʻilio me kāu kauka e pili ana i ia mea.
Leka Lawe-Home
- ʻO ka Avalglucosidase (Nexviazyme) kahi lāʻau lapaʻau pani enzyme i hoʻohana ʻia e mālama i ka maʻi Pompe. ʻAʻole ia e ho'ōla i ka maʻi, akā ke kāohi nei ia i nā hōʻailona.
- Hāʻawi ʻia kēia lāʻau lapaʻau ma ka haukapila ma ke ʻano he inikini i loko o kahi aʻa.
- Ma mua o ka hoʻomaka ʻana i ka lāʻau lapaʻau, e haʻi i kāu kauka e pili ana i kou mau kūlana olakino a me nā lāʻau lapaʻau ʻē aʻe āu e lawe nei.
- E makaʻala i nā hopena alerika koʻikoʻi i ka wā e loaʻa ai ka lāʻau pale. Inā ʻike ʻoe i nā hōʻailona e like me ka paʻakikī o ka hanu ʻana a i ʻole ka pehu ʻana o ka maka, e hoʻomaopopo koke i ka hui lapaʻau.
- He mea nui loa ka loaʻa ʻana o ka lāʻau lapaʻau i ka manawa kūpono. Mai hoʻokuʻu i ka lāʻau lapaʻau no kekahi kumu. E kamaʻilio me kāu kauka inā he mau hopohopo kāu.
- Mai kānalua e nīnau i kāu kauka e pili ana i kekahi mau nīnau a hopohopo paha āu e loaʻa ai.
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