He lāʻau lapaʻau no ka maʻi Gaucher: E kamaʻilio kākou e pili ana i ka lāʻau pale Velaglucerase alfa

I kekahi manawa lohe mākou e pili ana i nā maʻi laha ʻole loa, ʻaʻole mākou i lohe i ka inoa. ʻO ka maʻi Gaucher, a i ʻole `(Gaucher's Disease)`, kekahi o ia mau maʻi laha ʻole i hoʻokumu ʻia e nā kumu genetic. ʻOiai he paʻakikī ke ola me ia ʻano maʻi, me ka holomua o ka lāʻau lapaʻau hou, aia nā lāʻau lapaʻau maikaʻi e lawe mai i ka hōʻoluʻolu iā lākou. I kēia lā e kamaʻilio mākou e pili ana i kekahi o ia ʻano lāʻau lapaʻau, ʻo ka lāʻau pale `Velaglucerase alfa`.

I ka ʻōlelo maʻalahi, he aha ka maʻi ʻo Gaucher?

I mea e hoʻomaopopo ai i kēia, e noʻonoʻo kākou i ko kākou kino e like me kahi hale hana nui. Ma kēia hale hana, aia nā 'limahana' kūikawā e hana ana i nā hana like ʻole e hana pono ai nā mea āpau. ʻO kēia mau limahana ka mea a mākou e kapa nei he 'enzymes'. Loaʻa i kēlā me kēia enzyme kahi hana kikoʻī i hāʻawi ʻia iā ia.

I loko o ke kanaka me ka maʻi Gaucher, ʻo kekahi o kēia mau enzymes, a i ʻole kekahi o nā mea hana, ua nele maoli. ʻO ka hopena, ʻaʻole hiki ke wāwahi pono ʻia kekahi mau mea momona i loko o ke kino a hoʻopau ʻia ma ke ʻano he ʻōpala. A laila hoʻomaka mālie kēia mau mea e hōʻiliʻili i loko o nā ʻōpū e like me ke akepaʻa, ka spleen, a me ka iwi iwi. ʻO kēia ke kumu nui o nā hōʻailona.

No laila he aha ke kuleana o kēia lāʻau pale Velaglucerase alfa?

Ua like ʻo `Velaglucerase alfa` (inoa inoa `VPRIV`) me kahi `mea hana pani` a nā kānaka ʻepekema i hana ai ma ka hale hana e pani i ka mea hana i nalowale ma ka hale hana. I ka pololei, he enzyme kēia i hana ʻia ma ke ʻano hana e pani i ka `enzyme` i nele i nā maʻi Gaucher i loko o ko lākou kino.

Ke hāʻawi ʻia kēia lāʻau lapaʻau i ke kino, lawe ia i ka hana i kū i ka hana, wāwahi i nā waihona momona, a kōkua i ka kaohi ʻana i nā hōʻailona.

Pehea e hāʻawi ʻia ai kēia lāʻau lapaʻau?

ʻAʻole kēia he lāʻau lapaʻau hiki iā ʻoe ke hoʻohana ma ka home. ʻO Velaglucerase alfa kahi inikini i hāʻawi ʻia ma ke ʻano he wai saline i loko o kahi aʻa (infusion i loko o kahi aʻa). Hāʻawi ʻia kēia lāʻau lapaʻau ma kahi haukapila a i ʻole keʻena lapaʻau kūikawā, ma lalo o ka nānā ʻana o nā limahana lapaʻau. No laila, ʻaʻohe kumu e hopohopo ai no kou palekana.

Nā mea e haʻi aku i kāu kauka ma mua o ka hoʻomaka ʻana i ka lāʻau lapaʻau

He mea nui loa i kāu kauka ke ʻike i kou mōʻaukala olakino piha e hoʻoholo ai inā kūpono kēia lāʻau lapaʻau iā ʻoe a palekana hoʻi. E hōʻoia e haʻi iā lākou e pili ana i kēia mau mea.

• Nā maʻi ʻaleka āu e loaʻa nei:Inā ua loaʻa iā ʻoe kekahi mau maʻi ʻaleka ma mua i nā lāʻau lapaʻau ʻē aʻe, ʻoiai ka imiglucerase (kahi lāʻau lapaʻau like), nā meaʻai, nā mea hoʻoluʻu, a i ʻole nā ​​mea mālama, e haʻi pono i kāu kauka e pili ana iā lākou.
• Hāpai keiki: Inā hāpai ʻoe, a i ʻole e kali ana i kahi pēpē i ka wā e hiki mai ana, pono ʻoe e haʻi i kāu kauka.
• Ka hānai waiū ʻana: Inā he makuahine ʻoe e hānai waiū ana i kahi keiki, e hoʻomaopopo i kāu kauka e pili ana i kēia.
• Nā maʻi a me nā lāʻau lapaʻau ʻē aʻe: E hāʻawi i kāu kauka i kahi papa inoa piha o nā lāʻau lapaʻau ʻē aʻe (i kauoha ʻia a kūʻai ʻia paha ma luna o ke kauka), nā huahana lāʻau, a me nā huaora āu e lawe nei. He mea nui hoʻi e haʻi i kāu kauka inā ʻoe e puhipaka a inu waiʻona paha.

E hoʻohana no nā keiki

Hāʻawi ʻia kēia lāʻau lapaʻau i kekahi manawa i nā keiki ʻōpio e like me 4 mau makahiki, akā ma lalo wale nō o ke aʻo kūikawā a me ka nānā ʻana a ke kauka. Inā ʻoe e noʻonoʻo ana e hāʻawi i kēia lāʻau i kāu keiki, e kamaʻilio me kāu kauka pediatric e pili ana i ia mea.

He aha nā hopena ʻaoʻao e hiki ke loaʻa mai kēia lāʻau lapaʻau?

E like me nā lāʻau lapaʻau āpau, hiki i ka lāʻau pale ʻo `Velaglucerase alfa` ke hana i nā hopena ʻaoʻao i kekahi poʻe. Eia naʻe, ʻaʻole loaʻa i nā kānaka a pau. Hiki ke hoʻokaʻawale ʻia kēia mau mea i ʻelua mau ʻano nui. He koʻikoʻi kekahi a pono e hōʻike koke ʻia. He mea maʻamau kekahi a e nalo i loko o kekahi mau lā.

Nā mea ʻē aʻe e noʻonoʻo ai i ka wā o ka mālama ʻana

• Ka loaʻa ʻana o ka lāʻau lapaʻau i ka manawa kūpono: I mea e loaʻa ai ka pono nui mai kēia lāʻau lapaʻau, he mea nui loa ia e loaʻa ka lāʻau lapaʻau i ka manawa kūpono a me ka manawa kūpono. Inā ʻaʻole hiki iā ʻoe ke hele i ka haukapila i ka lā i hoʻonohonoho ʻia, e hoʻomaopopo i ke kauka a i ʻole ka haukapila ma mua a hoʻonohonoho i kahi manawa no ka lā aʻe.
• Nā hoʻokolohua lapaʻau:E nānā pinepine ke kauka iā ʻoe e ʻike i ka maikaʻi o ka hana a ka lāʻau lapaʻau a pehea ia e pili ai i kou kino. No laila, mai poina i nā kipa ʻana i ke keʻena lapaʻau.
• Overdose: He lāʻau lapaʻau kēia i hāʻawi ʻia e ka 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 kelepona koke i ke Kikowaena ʻIke Poisons National ma ka Halemai Lahui ʻo Colombo , a i ʻole e hele i ka Emergency Treatment Unit (ETU) o ka haukapila kokoke loa.
• Nou wale nō kēia: Ua kuhikuhi ʻia kēia lāʻau lapaʻau no kou kūlana a me ke ʻano o kou kino. No laila, mai kaʻana like i kēia lāʻau lapaʻau me kekahi no kekahi kumu.