Ke haʻi aku ke kauka iā ʻoe ua loaʻa i kāu keiki kahi maʻi laha ʻole i kapa ʻia ʻo Mucopolysaccharidosis type VII, a i ʻole MPS VII (Sly Syndrome) no ka pōkole, ʻo ʻoe, ma ke ʻano he makuahine a makua paha, pono ʻoe e haʻalulu nui a hopohopo. I ka manawa like, i kou lohe ʻana e pili ana i ka lāʻau pale i kapa ʻia ʻo Vestronidase alfa (inoa inoa: MEPSEVII) ma ke ʻano he lāʻau lapaʻau no kēia, pono ʻoe e loaʻa nā nīnau he nui i loko o kou manaʻo. He mea maʻamau ka noʻonoʻo ʻana i nā mea e like me, "He aha kēia lāʻau lapaʻau? E kōkua anei ia i ke keiki? Aia kekahi mau hopena ʻaoʻao?" Mai makaʻu. I kēia lā, e kamaʻilio mākou e pili ana i kēia lāʻau lapaʻau ma ka ʻōlelo maʻalahi hiki iā ʻoe ke hoʻomaopopo, ma ke ʻano aloha.
I ka ʻōlelo maʻalahi, he aha ka Vestronidase alfa?
E noʻonoʻo i ko kākou kino e like me kahi hale hana nui. Loaʻa i kēia hale hana nā 'limahana' kūikawā e hana i nā hana like ʻole. Kāhea mākou i kēia mau limahana he 'enzymes'. I loko o kahi keiki me ke kūlana e like me MPS VII, ʻaʻole hana ke kino i kahi enzyme koʻikoʻi, a i ʻole hana iki ia.
ʻO Vestronidase alfa kahi lāʻau lapaʻau e hoʻoponopono ai i kēlā hemahema . I ka pololei, he lāʻau lapaʻau pani enzyme (ERT) . ʻO ka hana a kēia lāʻau pale, ʻo ia ke hāʻawi i ke keiki i kahi enzyme i hana ʻole ʻia ma ke kino. Ke komo kēia enzyme i loko o ke kino, wāwahi ia i nā mea ʻino e hōʻiliʻili ana i loko o ke kino a kōkua i ka kaohi ʻana i nā hōʻailona.
ʻO ka mea nui loa, ʻaʻole kēia he lāʻau lapaʻau no ka maʻi. Eia nō naʻe, hiki i kēia lāʻau lapaʻau ke kōkua i ka kaohi ʻana i nā hōʻailona, hoʻomaikaʻi i ke ʻano o ke ola o ke keiki, a hoʻolilo i ke keiki i kahi ola ʻoluʻolu.
Pehea e hāʻawi ai i kēia lāʻau lapaʻau i ke keiki?
ʻAʻole kēia lāʻau lapaʻau he pila hiki ke ale ʻia a hoʻokomo ʻia paha ma ka home. Hāʻawi ʻia ʻo Vestronidase alfa ma ke ʻano he infusion, e like me ka saline, i loko o kahi vein .
Hana mau ʻia kēia kaʻina hana ma kahi haukapila a i ʻole ke keʻena lapaʻau , ma lalo o ka nānā pono ʻana o nā kauka a me nā kahu maʻi. Hiki ke hala i kekahi mau hola. Nānā mau ke kime lapaʻau i ke kūlana o ke keiki (ka helu o ka puʻuwai, ke koko, a me nā mea ʻē aʻe) ʻoiai ke keiki e loaʻa ana kēia lāʻau. No laila, ʻaʻole pono ʻoe e hopohopo e pili ana i ka palekana o kāu keiki.
ʻOiai hiki ke hāʻawi ʻia kēia lāʻau lapaʻau i nā pēpē hānau hou, aia kekahi mau hana palekana kūikawā e pono e mālama ʻia ma muli o ka makahiki a me ke kūlana o ke keiki. Na ke kauka o kāu keiki e hoʻomaopopo iā ʻoe e pili ana i kēia.
Nā mea e pono ai ʻoe e haʻi i kāu kauka ma mua o ka hoʻomaka ʻana i ka lāʻau lapaʻau
Ma mua o ka hoʻomaka ʻana i kēia lāʻau lapaʻau, he mea nui loa ia e haʻi i ke kauka i kekahi ʻike e pili ana i ke olakino o kāu keiki. E hoʻoholo kēia ʻike pehea e mālama ʻia ai ke keiki a me nā hana palekana e hana ʻia.
| Mea e hoʻomaopopo ʻia | No ke aha he mea nui kēlā? |
|---|---|
| ʻO kēlā me kēia ʻano maʻi allergy | E hōʻoia e haʻi i kāu kauka inā he mau maʻi ʻaleka kou i ka vestronidase alfa, nā lāʻau lapaʻau ʻē aʻe, nā meaʻai, nā kala, a i ʻole nā mea mālama. E kōkua kēia i ka pale ʻana i nā hopena maʻi ʻaleka koʻikoʻi. |
| Hāpai a i ʻole e hoʻāʻo ana e hāpai | Inā he ʻōpio ka mea e loaʻa ana ka lāʻau lapaʻau, he mea nui loa ia e hoʻomaopopo iā lākou inā hāpai lākou a i ʻole e hoʻāʻo nei e hāpai. |
| Ka hānai ʻana i ka waiū umauma | Inā e loaʻa ana ka lāʻau lapaʻau i ka makuahine, pono ʻo ia e hoʻomaopopo i ke kauka inā e hānai waiū ana ʻo ia i ka pēpē. |
He aha nā mea e pono ai kākou e makaʻala i ka wā e hāʻawi ai i ka lāʻau lapaʻau?
E makaʻala i nā maʻi alerika koʻikoʻi.
Hiki i kēia lāʻau lapaʻau ke hoʻoulu i nā hopena alerika koʻikoʻi i kekahi mau keiki. No ka hōʻemi ʻana i kēia pilikia, hiki i kāu kauka ke hoʻoholo e hāʻawi iā ʻoe i nā lāʻau lapaʻau ʻē aʻe (no ka laʻana, nā antihistamines e like me piritan, nā mea hoʻohaʻahaʻa kuni) ma kahi o hoʻokahi hola ma mua o ka hāʻawi ʻana i ka lāʻau pale Vestronidase alfa. No laila e hahai pono i nā ʻōlelo a kāu kauka.
Ka lāʻau i hala ʻole
He mea nui loa ka loaʻa ʻana o kēia lāʻau pale i ka manawa kūpono. Inā no kekahi kumu ʻaʻole hiki iā ʻoe ke hele i ka haukapila i ka lā i hoʻonohonoho ʻia, e kāhea koke aku i kāu kauka a e hoʻomaopopo iā lākou, i hiki iā lākou ke aʻo iā ʻoe i ka lā aʻe e hiki mai ana.
Overdose
ʻOiai ua hāʻawi ʻia kēia lāʻau lapaʻau ma kahi haukapila, ua haʻahaʻa loa ka manawa kūpono o ka overdose. Eia nō naʻe, inā hana ʻia kēia a i ʻole ʻoe e kānalua nei, e hoʻomaopopo koke i ka hui lapaʻau. Inā loaʻa iā ʻoe kekahi pilikia ma ka home, e kelepona i ke Kikowaena ʻIke Poisons National ma ka Halemai Lahui ʻo Colombo . A i ʻole e lawe i ke keiki i ka Emergency Treatment Unit (ETU) o ka haukapila kokoke loa.
Nā pilikia me nā lāʻau lapaʻau ʻē aʻe
ʻAʻole i aʻo pono ʻia pehea e launa pū ai kēia lāʻau me nā lāʻau lapaʻau ʻē aʻe. No laila, he mea nui loa ia e hoʻomaopopo i kāu kauka e pili ana i nā lāʻau lapaʻau ʻē aʻe, nā huaora, nā lāʻau lapaʻau lāʻau, a i ʻole nā mea hoʻohui e lawe nei kāu keiki. Eia kekahi, mai hoʻomaka i kekahi mau lāʻau lapaʻau hou me ka ʻole o ka nānā mua ʻana me ke kauka o kāu keiki.
He aha nā hopena ʻaoʻao e hiki ke loaʻa mai kēia lāʻau lapaʻau?
E like me nā lāʻau lapaʻau āpau, hiki i ka lāʻau lapaʻau Vestronidase alfa ke hana i kekahi mau hopena ʻaoʻao. ʻOiai he liʻiliʻi ka hapa nui o kēia mau mea, aia kekahi mau mea e pono ai mākou e makaʻala nui.
| Nā hopena ʻaoʻao koʻikoʻi e pono ʻoe e hōʻike koke i kāu kauka a kahu maʻi paha | |
|---|---|
| Nā hōʻailona allergies koʻikoʻi | ʻUʻumi ʻili, eczema, ʻūhū ʻili, maneʻo, pehu o nā lehelehe, ke alelo a i ʻole ka maka. |
| Nā pilikia hanu | Paʻakikī ka hanu ʻana, ʻūmiʻi ka umauma, nā leo ʻē i ka wā e hanu ana. |
| Ke koko haʻahaʻa | Pōniuniu, maka polū, kokoke e nalowale ka ʻike. |
| Nā hopena ʻaoʻao ʻaʻole ia he mea weliweli, akā pono e hōʻike ʻia i ke kauka inā hoʻomau lākou | |
| Ka maʻi pākī | ʻŌpū. |
| ʻO ka ʻoluʻolu ʻole ma kahi i hoʻokomo ʻia | ʻEha, ʻulaʻula, a pehu paha a puni ka wahi i inikini ʻia. |
ʻAʻole i hoʻokomo ʻia kēia papa inoa i nā hopena ʻaoʻao āpau. Eia kekahi, inā ʻike ʻoe i kekahi mea ʻē, mai kānalua e kamaʻilio me kāu kauka e pili ana i ia mea.
Leka Lawe-Home
- He lāʻau lapaʻau pani enzyme ʻo Vestronidase alfa e kaohi ana i nā hōʻailona o kahi maʻi laha ʻole i kapa ʻia ʻo MPS VII (Sly Syndrome). ʻAʻole ia he lāʻau lapaʻau no ka maʻi.
- Hāʻawi ʻia kēia lāʻau lapaʻau ma ke ʻano he infusion i loko o kahi aʻa koko ma ka haukapila, ma lalo o ka mākaʻikaʻi a ke kauka.
- Ma mua o ka hoʻomaka ʻana i ka lāʻau lapaʻau, he mea nui e hoʻomaopopo i ke kauka e pili ana i nā maʻi allergies a pau o kāu keiki a me nā kūlana olakino ʻē aʻe.
- Inā loaʻa iā ʻoe nā hōʻailona maʻi ʻaleka koʻikoʻi e like me ka pehu ʻana o ka maka, ka paʻakikī o ka hanu ʻana, a i ʻole ka ʻulaʻula o ka ʻili i ka wā o ka mālama ʻana, e hoʻomaopopo koke i ka hui lapaʻau.
- Mai poina i kāu lā lapaʻau i hoʻonohonoho ʻia no kekahi kumu. Inā poina iā ʻoe, e kāhea koke aku i kāu kauka.
- Inā he mau nīnau a hopohopo paha kāu e pili ana i kēia lāʻau lapaʻau, e kūkākūkā me kāu kauka.
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