E aʻo kākou e pili ana i ka lāʻau pale Alglucosidase alfa no ka maʻi Pompe?

ʻO ko mākou kino, ʻo ia hoʻi, he mīkini paʻakikī a kupaianaha. No nā mea āpau i loko o kēia mīkini e hana pono ai, he mea nui kahi ʻano protein kūikawā i kapa ʻia ʻo 'enzymes'. E noʻonoʻo, i kekahi manawa ma muli o nā kumu genetic, ʻaʻole paha e hana ʻia kekahi enzyme i loko o ke kino o ke kanaka a i ʻole e hana ʻia i kahi liʻiliʻi. ʻO ka maʻi Pompe kahi maʻi kakaikahi akā koʻikoʻi e kū mai ana ma kēia ʻano. I kēia lā e kamaʻilio mākou e pili ana i kahi lāʻau lapaʻau kūikawā i hāʻawi ʻia e kāohi i kēia maʻi, ʻo ia hoʻi, kahi lāʻau pale i kapa ʻia ʻo Alglucosidase alfa.

He aha ka Alglucosidase alfa?

I ka ʻōlelo maʻalahi, ʻo Alglucosidase alfa kahi inikini kūwaho o ka enzyme i nele i ke kino o ke kanaka me ka maʻi Pompe. Ua like kēia me ka lawe ʻana mai i kahi kanaka o waho e hana i kahi hana i ka wā e hele ʻole ai kahi limahana. Ua kapa ʻia kēia ʻano lapaʻau ʻo Enzyme Replacement Therapy (ERT) i ka ʻepekema lapaʻau.

ʻO ka mea nui e hoʻomanaʻo ai ma ʻaneʻi, ʻaʻole kēia he lāʻau lapaʻau no ka maʻi. Eia nō naʻe, hiki i kēia lāʻau lapaʻau ke kāohi i nā hōʻailona, ​​​​hōʻemi i ka nāwaliwali o nā ʻiʻo, a hoʻomaikaʻi nui i ke ʻano o ke ola o ka mea maʻi. Hiki ke loaʻa kēia lāʻau lapaʻau ma ka mākeke ma lalo o nā inoa inoa e like me `Lumizyme` a i ʻole `Myozyme`.

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 kekahi lāʻau lapaʻau kikoʻī, he mea nui loa ia e hoʻomaopopo i kāu kauka e pili ana i kou mōʻaukala olakino piha. ʻO ka ʻoi aku ka piha o kāu kauka i kou olakino, ʻoi aku ka palekana a me ka holomua o ka lāʻau lapaʻau. No laila, inā pili kekahi o kēia mau mea iā ʻoe, e hōʻoia e haʻi aku.

• Maʻi puʻuwai: Inā loaʻa iā ʻoe kekahi maʻi puʻuwai a i ʻole nā ​​pilikia e pili ana i ka puʻuwai.
• Ka pilikia hanu: Inā loaʻa iā ʻoe kahi maʻi māmā a i ʻole kekahi pilikia hanu.
• Nā Allergies: Inā he mau allergies kou i kekahi mea, pono ʻoe e haʻi mai iā mākou. ʻOi loa aku:
• Inā ua loaʻa iā ʻoe kahi hopena maʻi ma mua i kēia lāʻau lapaʻau a i ʻole kekahi o kāna mau mea hoʻohui, e like me ka mannitol.
• Inā loaʻa iā ʻoe kahi maʻi allergy i nā protein hamster a i ʻole rat (he mea nui kēia no ka mea hoʻohana ʻia kēia mau mea i ka hana ʻana o kekahi mau lāʻau lapaʻau).
• Inā he maʻi allergy kou i kekahi lāʻau lapaʻau ʻē aʻe, meaʻai, mea hoʻomalu a mea hoʻoluʻu paha.
• Hāpai keiki: Inā ʻoe e hāpai nei, a i ʻole e hoʻāʻo nei e hāpai i ka wā e hiki mai ana.
• Inā he makuahine hānai waiū ʻoe: Inā he makuahine ʻoe e hānai waiū ana i kahi keiki.

Pehea e hāʻawi ʻia ai kēia lāʻau lapaʻau?

ʻAʻole kēia lāʻau lapaʻau he pila e ale ʻia, ʻaʻole hoʻi he inikini hiki ke hāʻawi ʻia ma ka home.

Hāʻawi wale ʻia kēia lāʻau lapaʻau ma kahi haukapila a i ʻole ke keʻena lapaʻau kūikawā, ma lalo o ka nānā pono ʻana o nā kauka a me nā limahana kahu maʻi . E nānā mau lākou i kou puʻuwai, ke koko, a me ka hanu ʻana i ka wā o ka lāʻau lapaʻau.

Ke hāʻawi nei ʻoe i kēia i nā keiki liʻiliʻi?

ʻAe, hiki ke kuhikuhi ʻia kēia lāʻau lapaʻau no nā pēpē e like me hoʻokahi mahina. Eia nō naʻe, hiki i kahi kauka keiki ke hoʻoholo. Aia nā hana palekana kūikawā e pono e mālama ʻia i ka wā e hāʻawi ai i kēia lāʻau lapaʻau i kahi keiki.

He aha ka mea e hana ai inā ʻoe e lawe i ka overdose?

Hāʻawi ʻia kēia ma kahi haukapila, no laila ua haʻahaʻa loa ka manawa e hiki ai kēia. Eia nō naʻe, inā manaʻo ʻoe ua lawe nui ʻoe i kēia lāʻau lapaʻau, e hoʻomaopopo koke i nā limahana lapaʻau. I kahi pilikia, hiki iā ʻoe ke kelepona i ke Kikowaena ʻIke Poisons National ma ka Halemai Lahui ʻo Colombo . A i ʻole e hele ʻoe i ka Emergency Treatment Unit (ETU) kokoke loa.

He aha inā e hala wau i ka lāʻau lapaʻau?

He mea nui loa ka hele ʻana i kāu manawa i hoʻonohonoho ʻia. Inā no kekahi kumu ʻaʻole hiki iā ʻoe ke hele, mai hoʻokuʻu iā ia, akā e kāhea koke aku i kāu kauka a i ʻole ka haukapila e hoʻonohonoho i kahi lā a me kahi manawa ʻē aʻe.

He aha nā hopena ʻaoʻao i manaʻo ʻia?

E like me nā lāʻau lapaʻau āpau, hiki i kēia lāʻau lapaʻau ke loaʻa kekahi mau hopena ʻaoʻao. ʻOiai ʻo ka hapa nui o kēia mau mea he mea liʻiliʻi, aia kekahi mau mea e pono ai mākou e makaʻala nui. E nānā kākou i kēia ma kahi papa e maʻalahi ai ke hoʻomaopopo.

Aia kekahi pilikia me nā lāʻau lapaʻau ʻē aʻe?

ʻAʻole nui ka launa pū ʻana o kēia lāʻau lapaʻau me nā lāʻau lapaʻau ʻē aʻe. Eia nō naʻe, he mea nui e haʻi i kāu kauka e pili ana i nā lāʻau lapaʻau āpau āu e lawe nei (me nā huaora a me nā huahana lāʻau lapaʻau), a inā ʻoe e puhipaka a inu waiʻona paha.