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ʻO ka mea mua, e nānā kākou, no ke aha e pau ʻole ai ke kahe koko i ka hemophilia?
I ka ʻōlelo maʻalahi, aia nā protein kūikawā e kōkua i ko mākou kāpili ʻana o ke koko. Kāhea mākou i kēia mau mea he " nā kumu kāpili ." E like me ka mea e pono ai ka mortar e hoʻopaʻa i nā piliki i ke kūkulu ʻana i kahi pā, he mea nui kēia mau mea kāpili e hoʻōki i ke kahe ʻana o ke koko. ʻO ke kanaka me ka hemophilia, ua nele i kekahi o kēia mau mea kāpili.- ʻAʻohe mea i kapa ʻia ʻo Factor VIII i ka poʻe me ka hemophilia A.
- ʻAʻohe mea i kapa ʻia ʻo Factor IX i ka poʻe me ka hemophilia B.
No laila he aha kēia lāʻau lapaʻau hou i kapa ʻia ʻo Qfitlia (fitusiran)?
He lāʻau lapaʻau kēia e hana ana ma ke ʻano ʻē loa. Ua kamaʻilio mākou ma mua e pili ana i ka ʻoiaʻiʻo e pono ke koko i kahi mea i kapa ʻia ʻo thrombin e hoʻopaʻa. I loko o ko mākou kino ponoʻī, aia kekahi protein ʻē aʻe e kāohi ana i ka hana ʻana o kēia thrombin, ʻo ia hoʻi, ke hoʻōki nei i ka hana ʻana o nā thrombin hou aʻe. Ua kapa ʻia ʻo ia he antithrombin .E noʻonoʻo i kēia ʻano. Loaʻa iā ʻoe kahi mea hoʻopaʻa koko (thrombin) i loko o kou kino e kōkua ana i kou hoʻopaʻa koko. Akā aia kekahi mea ʻē aʻe (antithrombin) e kāohi a pale iā ia mai ka hana ʻana. I ka hemophilia, haʻahaʻa mua ka hana ʻana o ka mea kōkua (thrombin). No laila, ke hana maʻamau ka mea e pale ai (antithrombin), emi hou aku ka hoʻopaʻa koko. ʻO ka mea a Qfitlia e hana ai, ʻo ia ka hoʻemi ʻana i ka nui o ka antithrombin e pale ai iā ia. A laila, ʻae ʻia ka nui o ka thrombin a kou kino e hana maoli ai e hana pono me ka ʻole o ke keakea ʻia. ʻO ia hoʻi, hoʻohana ia i ka ʻōnaehana ponoʻī o ke kino e paipai i ke kāpili koko, ma mua o ka hāʻawi ʻana iā ʻoe i ka mea hoʻopaʻa koko āu i nalowale ai. ʻO kēia ka lāʻau hoʻemi antithrombin mua e hoʻohana ʻia i ka mālama ʻana i ka hemophilia.E wehewehe hou aku kākou i ka ʻokoʻa ma waena o kēia mau ʻano lapaʻau ʻelua.
Hiki iā ʻoe ke hoʻomaopopo maʻalahi i kēia ʻokoʻa mai ka papa ma lalo nei.| ʻAno | Lapaʻau kuʻuna (Factor Replacement) | ʻO ka lāʻau lapaʻau ʻo Qfitlia (fitusiran) |
|---|---|---|
| Hana | Hāʻawi ʻia ma waho ka mea hoʻopaʻa koko i nele i ke kino. | Hoʻemi ia i ka antithrombin, ka mea e pale ai i ke kāpili ʻana o ke koko, a hiki i ke kino ke hoʻohana hou aku i kāna thrombin ponoʻī. |
| Pehea e hāʻawi ai | I ka manawa pinepine, hoʻokomo ʻia i loko o ka intravenous. | Ma lalo o ka ʻili (subcutaneous). |
| Alapine (frequency) | He mea pono paha ia i nā manawa he nui i ka pule. | Hoʻokahi manawa i ka mahina a i ʻole i kēlā me kēia ʻelua mahina (6-12 manawa i ka makahiki). |
| No ka poʻe me nā mea hoʻopaʻa | ʻAʻole holo pono ka lāʻau lapaʻau kumu maʻamau, a pono nā lāʻau lapaʻau kūikawā ʻē aʻe (nā mea hana bypassing). | Hiki ke hoʻohana pono ʻia kēia lāʻau lapaʻau i nā poʻe me nā mea hoʻopaʻa (nā antibody e kūʻē i nā kumu). |
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Check Availability →He kūpono anei kēia lāʻau lapaʻau iaʻu a pehea e hoʻohana ai?
Inā loaʻa iā ʻoe ka hemophilia A a i ʻole B a ʻoi aku ma mua o 12 mau makahiki, e noʻonoʻo paha kāu kauka inā kūpono kēia lāʻau lapaʻau iā ʻoe. ʻO ka mea nui loa, hiki ke hoʻohana ʻia i nā poʻe i loaʻa nā mea pale (kūʻē) i ka lāʻau lapaʻau kumu mua. Eia naʻe, ma mua o ka hoʻomaka ʻana i kēia lāʻau lapaʻau, e hana kāu kauka i kekahi mau hoʻāʻo koko . 1. Pae Antithrombin: Ana kēia i ka nui o ka antithrombin i loko o kou koko. Hoʻoholo ʻia ka nui o Qfitlia ma muli o kēia waiwai. 2. Hana o ke akepaʻa: Nānā ʻia kou akepaʻa ma mua a i ka wā o ka lāʻau lapaʻau. Hāʻawi ʻia kēia lāʻau lapaʻau ma ke ʻano he injection ma lalo o ka ʻili (subcutaneously). ʻO ia hoʻi, ʻaʻole ia e hāʻawi ʻia i loko o kahi vein. Hiki iā ʻoe a i ʻole kahi mea mālama ke hāʻawi iā ʻoe ma ka home ma hope o ke aʻo ʻana. Hāʻawi ʻia ia ma ka ʻili o ka ʻōpū, ka ʻūhā, a i ʻole ke kua o ka lima luna. Pono ʻoe e pale i nā wahi me nā palapū a i ʻole nā ʻoki. Inā ʻoe e lawe nei i nā lāʻau lapaʻau hemophilia ʻē aʻe, pono paha ʻoe e hoʻōki a hoʻololi paha i kēlā mau lāʻau lapaʻau ma hope o ka hoʻomaka ʻana iā Qfitlia. E wehewehe pono kāu kauka i kēia iā ʻoe. Mai ho'ōki i ka lawe ʻana i kāu mau lāʻau lapaʻau o kēia manawa a i ʻole e ho'ōki i ka lawe ʻana iā Qfitlia me ka ʻole o ke aʻo ʻana a kāu kauka. ʻO ka hana ʻana pēlā e hoʻonui ai i kou pilikia o ke kahe ʻana o ke koko.Pono anei kākou e makaʻala i nā hopena ʻaoʻao? Pehea e hiki ai iā kākou ke pale aku iā lākou?
E like me nā lāʻau lapaʻau āpau, hiki iā Qfitlia ke loaʻa kekahi mau hopena ʻaoʻao. He mea nui e makaʻala iā lākou a hana i nā hana e pale aku ai iā lākou.- Nā ʻAʻa Koko : No ka mea, hoʻolaha kēia lāʻau lapaʻau i ke kāpili ʻana o ke koko, he mea kakaikahi loa ka hana ʻana o nā ʻaʻa koko i makemake ʻole ʻia i loko o nā aʻa koko. Na kāu kauka e hoʻoholo i ka nui kūpono no kou pae antithrombin e hōʻemi i kēia pilikia.
- Nā enzymes akepaʻa kiʻekiʻe: Loaʻa paha i kekahi poʻe nā enzymes akepaʻa kiʻekiʻe. ʻO ia ke kumu e nānā mau ai nā kauka i ke kūlana o ke akepaʻa me nā hoʻāʻo koko .
- Nā Pōhaku ʻAi a me ka Uha ʻAi: Inā ʻike koke ʻoe i nā hōʻailona e like me ka ʻeha nui o ka ʻōpū, ka nausea, a me ka luaʻi ʻana , e hoʻomaopopo koke i kāu kauka.
- Nā maʻi: Aia kahi pilikia liʻiliʻi o ka maʻi ma kahi i hoʻokomo ʻia ai. No laila, he mea nui e hoʻokomo mau me ka maʻemaʻe a e like me ke kuhikuhi a kāu kauka. He mea nui nō hoʻi nā hana hoʻomaʻemaʻe maikaʻi, e like me ka holoi lima pinepine.
Leka Lawe-Home
- He lāʻau lapaʻau hou ʻo Qfitlia (fitusiran) no ka hemophilia A a me B e hana ana ma ke ʻano ʻokoʻa.
- Hana kēia ma ka hoʻemi ʻana i ka nui o ka antithrombin, ka mea e pale ai i ke kāpili ʻana o ke koko, ma kahi o ka hāʻawi ʻana i ka mea hana kāpili haʻahaʻa o ke kino ma waho.
- He inikini kēia lāʻau lapaʻau ma lalo o ka ʻili, ʻaʻole i loko o kahi aʻa, a hāʻawi pinepine ʻia, e like me hoʻokahi manawa i ka mahina a i ʻole i kēlā me kēia ʻelua mahina.
- E hoʻoholo kāu kauka inā kūpono kēia lāʻau lapaʻau iā ʻoe ma hope o kekahi mau hoʻāʻo koko.
- I mea e pale aku ai i nā hopena ʻaoʻao, he mea nui e hana i nā hoʻokolohua koko (ʻoiai ka hana antithrombin a me ke akepaʻa) i nā manawa maʻamau e like me ke kauoha a kāu kauka.
- Mai ho'ōki i ka mālama ʻana iā Qfitlia me ka ʻole o ke aʻo lapaʻau no kekahi kumu.
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