He maʻi laha ʻole ka maʻi Fabry, he maʻi hoʻoilina i loaʻa ma muli o ka nele o kahi enzyme koʻikoʻi loa i loko o ko kākou kino. Hiki i kēia ke hoʻopilikia i nā ʻāpana like ʻole o ke kino, e like me ka puʻuwai, nā puʻupaʻa, a me ka ʻōnaehana nerve, i ka hala ʻana o ka manawa. No laila, inā loaʻa iā ʻoe ka maʻi Fabry, ua kuhikuhi paha kāu kauka iā ʻoe i kahi lāʻau lapaʻau i kapa ʻia ʻo Agalsidase Beta. I kēia lā, e kamaʻilio mākou e pili ana i kēia lāʻau lapaʻau ma ke ʻano maʻalahi a maopopo.
He aha ke ʻano lāʻau lapaʻau kēia?
I ka ʻōlelo maʻalahi, ʻo Agalsidase Beta (inoa inoa ʻo Fabrazyme) kahi lāʻau lapaʻau e pani ana i ka enzyme hemahema i loko o ke kino o ke kanaka me ka maʻi Fabry. Ma nā ʻōlelo lapaʻau, ua kapa ʻia kēia ʻo 'Enzyme Replacement Therapy' (ERT).
ʻO ka mea nui loa, ʻaʻole kēia he lāʻau lapaʻau no ka maʻi. Eia nō naʻe, hiki i kēia lāʻau lapaʻau ke kōkua nui i ka kaohi ʻana i ka pōʻino i hana ʻia e ka maʻi i ke kino a hōʻemi i nā hōʻailona.
Nā mea nui loa e haʻi aku i kāu kauka
Ma mua o ka hoʻomaka ʻana i kēia lāʻau lapaʻau, he mea nui e ʻike ke kauka i kekahi mau mea e pili ana i kou kūlana olakino. No laila, inā loaʻa iā ʻoe kekahi o kēia mau mea, e haʻi pono i ke kauka e pili ana i ia mea.
- Inā he maʻi puʻuwai kou .
- Inā ua loaʻa iā ʻoe kahi hopena maʻi i ka Agalsidase Beta, mannitol, a i ʻole kekahi lāʻau lapaʻau ʻē aʻe, meaʻai, a mea hoʻoluʻu paha.
- Inā hāpai ʻoe, a i ʻole ke manaʻo nei e hāpai koke.
- Inā he makuahine ʻoe e hānai waiū ana i kāu keiki .
Pehea ʻoe e hoʻohana ai i kēia lāʻau lapaʻau?
Hāʻawi ʻia kēia lāʻau lapaʻau ma ke ʻano he infusion intravenous (infusion) i loko o kahi aa. ʻAʻole kēia he mea hiki iā ʻoe ke hana ma ka home. Hāʻawi wale ʻia kēia lāʻau lapaʻau e kahi mea mālama ola kino i aʻo ʻia ma kahi haukapila a i ʻole ke keʻena lapaʻau, ma lalo o ka nānā ʻana a ke kauka.
E hoʻohana no nā keiki liʻiliʻi
Pono e akahele loa i ka hāʻawi ʻana i kēia lāʻau lapaʻau i nā keiki liʻiliʻi. I kekahi manawa hāʻawi ʻia i nā keiki a hiki i 2 mau makahiki, akā ʻo ke kauka wale nō nāna e nānā i ke keiki e hoʻoholo.
He aha inā lawe nui wau i ka lāʻau lapaʻau?
ʻOiai ua hāʻawi ʻia kēia lāʻau lapaʻau ma kahi haukapila, ma lalo o ka nānā ʻana a kahi loea, haʻahaʻa loa ka manawa kūpono o ka overdose. Eia nō naʻe, inā manaʻo ʻoe ua hana ʻia kekahi mea, 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) kokoke loa.
He aha kāu e hana ai inā e poina ʻoe i kahi mahele o ka lāʻau lapaʻau?
He mea nui loa ka lawe ʻana i ka lāʻau lapaʻau i ka lā i hoʻonohonoho ʻia a i ka manawa i hoʻonohonoho ʻia no kēia lāʻau lapaʻau. Inā no kekahi kumu ʻaʻole hiki iā ʻoe ke hele mai i ka haukapila i ka lā i hoʻonohonoho ʻia, e hoʻomaopopo i kāu kauka a i ʻole ke keʻena lapaʻau ma mua a hoʻonohonoho i kahi lā ʻē aʻe.
Ma hea ʻoe e mālama ai i ka lāʻau lapaʻau?
ʻOiai ua hāʻawi ʻia kēia lāʻau lapaʻau ma ka haukapila a i ʻole ke keʻena lapaʻau, ʻaʻole ʻoe e ʻae ʻia e lawe i ka home a mālama paha.
Nā lāʻau lapaʻau ʻē aʻe e launa pū me kēia lāʻau lapaʻau
Hiki i kekahi o nā lāʻau lapaʻau āu e lawe nei ke launa pū me Agalsidase Beta. No laila, he mea nui loa e haʻi i kāu kauka e pili ana i nā lāʻau lapaʻau āpau āu e lawe nei (nā lāʻau lapaʻau i kauoha ʻia, nā mea kūʻai aku ma luna o ke kauka, nā huahana lāʻau lapaʻau, nā huaora). ʻOi loa aku inā ʻoe e lawe ana i kekahi o kēia mau lāʻau lapaʻau:
- amiodarone
- klorokuina
- gentamicin
- ʻO Hydroxychloroquine
- monobenzone
ʻAʻole i loko o kēia papa inoa nā pilina lāʻau āpau, no laila ʻoi aku ka palekana o ka hoʻomaopopo ʻana i kāu kauka e pili ana i nā mea āpau āu e lawe nei.
He aha nā hopena ʻaoʻao e hiki mai ana?
E like me nā lāʻau lapaʻau āpau, hiki i kēia ke loaʻa kekahi mau hopena ʻaoʻao. He koʻikoʻi kekahi, he mea maʻamau kekahi. He mea nui e makaʻala i kēia mau mea.
| Ka hopena ʻaoʻao | Wehewehena |
|---|---|
| Nā hopena ʻaoʻao e pono e hōʻike koke ʻia i ke kauka a i ʻole ke kahu maʻi | |
| Nā hopena maʻi koʻikoʻi | ʻŌhūhū ʻili, maneʻo, pehu o ka maka, nā lehelehe a i ʻole ke alelo. |
| Paʻakikī ka hanu ʻana | Pōkole o ka hanu, hanu wikiwiki. |
| ʻEha a ʻeha paha ka umauma | Me he mea lā e ʻūmi ʻia ana kou puʻuwai. |
| Nā palpitations o ka puʻuwai | Ke manaʻo nei e kuʻi wikiwiki ana kou puʻuwai a i ʻole ke loli nei ka helu o kou puʻuwai. |
| ʻĒ aʻe | ʻO ka pōʻino nui, ka pehu ʻana o nā lima a i ʻole nā wāwae, ke kaumaha. |
| Nā hopena ʻaoʻao ʻaʻole i koʻikoʻi pinepine (akā pono e hōʻike i ke kauka inā hoʻomau lākou) | |
| ʻO kou manaʻo ke hoʻokomo ʻoe i ka lāʻau lapaʻau | Kuni a i ʻole ke anu. |
| ʻEha nui | ʻEha kino, ʻeha poʻo. |
| Nā pilikia o ka ʻōnaehana hoʻoheheʻe | Ka luaʻi, ka ʻeha o ka ʻōpū a i ʻole ka ʻoluʻolu ʻole. |
| Kūlana noʻonoʻo | Manaʻo hopohopo, hopohopo. |
ʻAʻole i loko o kēia papa inoa nā hopena ʻaoʻao āpau. Inā loaʻa iā ʻoe kekahi mea ʻē aʻe, e kamaʻilio me kāu kauka e pili ana i ia mea.
Leka Lawe-Home
- He lāʻau lapaʻau pani enzyme ʻo Agalsidase Beta (Fabrazyme) no ka maʻi Fabry. ʻOiai ʻaʻole ia e ho'ōla loa i ka maʻi, hiki iā ia ke kōkua nui i ka kaohi ʻana i nā hōʻailona a me nā pilikia.
- Hāʻawi ʻia kēia lāʻau lapaʻau ma o ka intravenous ma ka haukapila, no laila he mea nui loa ka loaʻa ʻana o ka lāʻau lapaʻau i ka manawa kūpono.
- Pono ʻoe e haʻi i kāu kauka e pili ana i nā lāʻau lapaʻau ʻē aʻe āu e lawe nei, nā maʻi allergies āu e loaʻa ai, a inā ʻoe e hāpai ana a hānai waiū paha.
- Inā ʻike ʻoe i kekahi mau hōʻailona e like me ka paʻakikī o ka hanu ʻana, ka ʻeha o ka umauma, a i ʻole ka ʻulaʻula o ka ʻili i ka wā a ma hope koke iho paha o ke kau ʻana i ka lāʻau lapaʻau, e haʻi koke aku i kāu kauka a kahu maʻi paha.
- Inā he mau nīnau kāu e pili ana i kēia lāʻau lapaʻau, mai makaʻu a kānalua paha e nīnau i kāu kauka.
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