He mea kākaʻikahi loa kekahi mau maʻi. A he pilikia nui ka noho ʻana me lākou. ʻO Mucopolysaccharidosis VI, a i ʻole MPS VI a i ʻole Maroteaux-Lamy syndrome, kekahi o ia mau maʻi laha ʻole. He lāʻau lapaʻau kūikawā ʻo Galsulfase e lawe mai i ka hōʻoluʻolu i ka poʻe e ʻeha nei i kēia maʻi. I kēia lā, ke kamaʻilio nei mākou e pili ana i kēia lāʻau lapaʻau koʻikoʻi loa. Inā ua kuhikuhi ʻia kēia lāʻau lapaʻau iā ʻoe, kāu keiki a i ʻole kekahi āu i ʻike ai, e lilo kēia ʻatikala i mea pono loa iā ʻoe.
He aha ka lāʻau ʻo Galsulfase?
I ka ʻōlelo maʻalahi, he lāʻau lapaʻau pani enzyme ʻo Galsulfase. I kēia manawa e nānā kākou i ke ʻano o ia mea.
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. ʻO kēia mau limahana ka mea a mākou e kapa nei he mau enzymes . Ma MPS VI, ʻo kahi enzyme kūikawā e pono ai e wāwahi i nā kō paʻakikī i loko o ko kākou kino ʻaʻole i hana ʻia e ke kino i ka wā hānau a i ʻole ua hana iki ʻia.
No laila, ke nalowale kēia enzyme, hoʻomaka kēlā mau kō e hōʻiliʻili i loko o nā hunaola o ke kino. ʻO ia ke kumu e hōʻino ʻia ai nā ʻano kino like ʻole e like me nā iwi, ka puʻuwai, nā māmā, a me nā maka a ʻike ʻia nā hōʻailona.
Hana kēia lāʻau lapaʻau, i kapa ʻia ʻo Galsulfase, ma ka hāʻawi ʻana i ke kino i ka enzyme i nele mai waho. Ke komo kēia lāʻau lapaʻau i loko o ke kino, wāwahi ia i nā kō i hōʻiliʻili ʻia a kōkua i ka kaohi ʻana i nā hōʻailona. ʻO Naglazyme ka inoa inoa no kēia lāʻau lapaʻau.
He mea nui: ʻAʻole kēia he lāʻau lapaʻau no ka maʻi. Eia nō naʻe, hāʻawi ia i ka hōʻoluʻolu nui ma ka kaohi ʻana i nā hōʻailona a me ka hoʻomaikaʻi ʻana i ke ʻano o ke ola.
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 hoʻomaopopo i kāu kauka e pili ana i kou mōʻaukala olakino piha. E hōʻoia e haʻi iā lākou e pili ana i kēia mau mea:
| ʻIke e pono ai ʻoe e hāʻawi | No ke aha he mea nui kēlā? |
|---|---|
| Nā maʻi ʻaleka | E haʻi i kāu kauka inā he mau maʻi ʻaleka kou i ka gliclazide, nā lāʻau lapaʻau ʻē aʻe, nā meaʻai, a i ʻole nā protein hamster. He mea nui kēia no ka mea hoʻohana ʻia kēia mau mea i ka hana ʻana i kekahi mau lāʻau lapaʻau. |
| Ka hanu ʻana a i ʻole ka apnea hiamoe | Hiki i kekahi poʻe me MPS VI ke loaʻa i ka apnea hiamoe. Hiki i kēia lāʻau lapaʻau ke hoʻololi i kēia kūlana, no laila e makaʻala kāu kauka i kēia. |
| Kuni a i ʻole ka maʻi | Inā loaʻa iā ʻoe kahi maʻi i kēia manawa, e like me ke anu a i ʻole ka maʻi palū, e ʻoluʻolu e haʻi mai iā mākou e pili ana i ia mea ma mua o ka loaʻa ʻana o ka lāʻau lapaʻau. |
| Ka hāpai ʻana a i ʻole ka hānai ʻana i ka waiū | Inā hāpai ʻoe, e hoʻāʻo ana e hāpai, a i ʻole e hānai waiū ana, e ʻoluʻolu e hoʻomaopopo i kāu kauka ma mua o ka mālama ʻana. |
Pehea e hāʻawi ʻia ai kēia lāʻau lapaʻau?
ʻAʻole kēia he lāʻau lapaʻau hiki iā ʻoe ke lawe i ka home.
- He inikini i loko o kahi aa: He lāʻau lapaʻau kēia i hāʻawi ʻia i loko o kahi aa, e like me ka saline. I ka lāʻau lapaʻau, kapa mākou i kēia he intravenous infusion .
- Ma ka haukapila: Hāʻawi ʻia kēia lāʻau lapaʻau ma ka haukapila a i ʻole ke keʻena lapaʻau kūikawā e ke kauka a kahu maʻi paha i aʻo ʻia.
- Ka Lōʻihi: ʻO ka maʻamau, he mau hola ka lōʻihi o ka lawelawe ʻana i kēia lāʻau lapaʻau, a ma ia manawa e mālama ʻia ai ʻoe ma lalo o ka mākaʻikaʻi a ke kauka.
He kūpono kēia no nā keiki?
ʻAe, hāʻawi ʻia kēia lāʻau lapaʻau i nā keiki ma luna o 5 mau makahiki. Eia nō naʻe, ua hoʻoholo ʻia ka nui me ke akahele kūikawā, e noʻonoʻo ana i nā mea e like me ke kūlana a me ke kaumaha o ke keiki. No laila, inā ʻoe e hāʻawi nei i kēia lāʻau lapaʻau i kāu keiki, e kamaʻilio me kāu kauka pediatric e pili ana i ia mea.
He aha kāu e hana ai inā e poina ʻoe i kahi lāʻau?
He mea nui loa ka loaʻa ʻana o kēia lāʻau lapaʻau i ka lā a me ka manawa i hoʻonohonoho ʻia no ka holomua o kēia lāʻau lapaʻau. Inā no kekahi kumu ʻaʻole hiki iā ʻoe ke hele mai no ka lāʻau lapaʻau i ka lā i hoʻonohonoho ʻia, mai hōʻole iā ia. E kelepona i kāu kauka a i ʻole ka haukapila i ka hikiwawe loa a hoʻonohonoho i kahi lā ʻē aʻe.
He aha inā piʻi ka nui o ka lāʻau lapaʻau (overdose)?
ʻOiai ua hāʻawi ʻia kēia ma kahi haukapila, ma lalo o ka nānā ʻana o ke kauka, ua haʻahaʻa loa ka manawa kūpono o ka overdose. Eia nō naʻe, inā kānalua ʻoe ua hana ʻia kēia, e hoʻomaopopo koke i nā limahana lapaʻau. Inā pono, e hoʻokaʻaʻike aku lākou i ke Kikowaena ʻIke Poisons National ma ka Halemai Lahui ʻo Colombo no ka ʻōlelo aʻo, a i ʻole e kuhikuhi iā ʻoe i ka Emergency Treatment Unit (ETU) .
He aha nā hopena ʻaoʻao e hiki ke loaʻa mai ka lāʻau lapaʻau?
E like me nā lāʻau lapaʻau āpau, hiki ke loaʻa kekahi mau hopena ʻaoʻao me ka galsulfase. Hiki i kekahi o kēia mau mea ke koʻikoʻi, no laila he mea nui e makaʻala iā lākou.
| 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. |
| 🔴 Nā pilikia hanu | Paʻakikī ka hanu ʻana, ka wheezing. |
| 🔴 ʻEha umauma | Manaʻo o ka ʻūmiʻi a i ʻole ka ʻeha ma ka umauma. |
| 🔴 Nā hōʻailona koʻikoʻi ʻē aʻe | Nā loli o ka ʻike, ʻeha pepeiao a i ʻole ka maʻi, poniuniu, kuni a i ʻole ke anu, koko kiʻekiʻe. |
| ʻAʻole maʻamau ka koʻikoʻi (akā e haʻi i kāu kauka inā hoʻomau ia) | |
| 🟢 ʻEha kino (Nā ʻeha a me nā ʻāʻī) | ʻEha i nā ʻiʻo a me nā hono. |
| 🟢 Nā pilikia o ka ʻōnaehana hoʻoheheʻe | ʻEha ʻōpū, nausea, luaʻi, diarrhea. |
| 🟢 Nā hiʻohiʻona liʻiliʻi ʻē aʻe | ʻEha poʻo, luhi. |
ʻAʻole i loko o kēia papa inoa nā hopena ʻaoʻao āpau. Inā loaʻa iā ʻoe kekahi mau hōʻailona ʻē aʻe, e ʻoluʻolu e hoʻomaopopo i kāu kauka.
Nā lāʻau lapaʻau ʻē aʻe e launa pū me kēia lāʻau lapaʻau
Hiki i kekahi mau lāʻau lapaʻau ke launa pū me ka gliclazide. Eia kekahi laʻana:
- Kolorokuina
- Gentamicin
- ʻO Hydroxychloroquine
ʻAʻole kēia he papa inoa piha. No laila, e haʻi i kāu kauka e pili ana i nā lāʻau lapaʻau āpau āu e lawe nei (nā lāʻau lapaʻau i kauoha ʻia, nā lāʻau lapaʻau ma luna o ke kūʻai aku, nā huaora, nā lāʻau lapaʻau kuʻuna, nā lāʻau lapaʻau Ayurvedic). Eia kekahi, e haʻi iā ia inā ʻoe e puhipaka a inu waiʻona paha.
Ua ʻike mākou ʻaʻole maʻalahi ka noho ʻana me ia ʻano maʻi laha ʻole. Akā, hiki i nā lāʻau lapaʻau hou e like me kēia ke kōkua iā ʻoe e hoʻokele i kāu mau hōʻailona a ola i kahi ola maikaʻi aʻe. ʻO ka mea nui loa, ʻo ia ka mālama ʻana i kahi pilina mau me kāu kauka a hahai pololei i kā lākou ʻōlelo aʻo.
Leka Lawe-Home
- He lāʻau lapaʻau pani enzyme ʻo Galsulfase e kāohi ana i nā hōʻailona o kahi maʻi laha ʻole i kapa ʻia ʻo MPS VI. ʻAʻole ia e ho'ōla piha i ka maʻi.
- Hāʻawi ʻia kēia lāʻau lapaʻau ma kahi haukapila a i ʻole ke keʻena lapaʻau, ma o kahi aa koko. ʻAʻole hiki ke lawe ʻia i ka home a hoʻohana ʻia.
- Ma mua o ka hoʻomaka ʻana i ka lāʻau lapaʻau, he mea nui e hoʻomaopopo i kāu kauka e pili ana i nā maʻi allergies āpau āu e loaʻa nei, nā kūlana olakino ʻē aʻe, a me nā lāʻau lapaʻau āpau āu e lawe nei.
- Inā loaʻa iā ʻoe nā hōʻailona maʻi koʻikoʻi e like me ka paʻakikī o ka hanu ʻana a me ka pehu ʻana o ka maka, e ʻimi koke i ke kōkua lapaʻau.
- Mai poina i kāu lā lapaʻau i hoʻonohonoho ʻia. Inā hana ʻoe pēlā, e kāhea koke aku i kāu kauka.
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