Ke loaʻa iā ʻoe kahi ʻoki liʻiliʻi, e pau ke kahe ʻana o ke koko ma hope o kekahi manawa, ʻeā? ʻO kēia ma muli o kahi ʻano hana kupaianaha i loko o ko mākou kino. Akā no kekahi poʻe, ʻaʻole hiki i ke kahe ʻana ke hoʻōki maʻalahi i kēia. ʻOiai kahi ʻoki liʻiliʻi ke hoʻomau i ke kahe ʻana o ke koko. I kēia lā, e kamaʻilio mākou e pili ana i kahi maʻi i kapa ʻia ʻo hemophilia, kahi i lohe ai ka poʻe he nui akā ʻaʻole maopopo maoli. Ma mua o ko mākou makaʻu ʻana e pili ana i ia mea, e hoʻomaopopo kākou i ke ʻano maoli.
Pehea ʻoe e ʻike ai inā he hemophilia kou?
I ka ʻōlelo maʻalahi, ma nā wahi a pau e kahe ana ke koko i loko o ko kākou kino, e hana ʻia kekahi mea e like me ka 'plug' e hoʻōki i ke kahe ʻana o ke koko. Kāhea mākou i kēia he fibrin clot. No ka hana ʻana i kēia clot, pono e hana pū nā protein kūikawā he nui i loko o ko kākou koko. ʻO kēia nā mea a mākou e kapa nei he "clotting factors."
ʻO ka Hemophilia kahi kūlana i nele ai kekahi o kēia mau mea hoʻopaʻa koko, ʻo ia hoʻi ka factor VIII a i ʻole ka factor IX, a i ʻole ua emi iho paha mai ka hānau ʻana. A laila, ʻaʻole i hana pono ʻia ke koko, a hoʻomau ke kahe ʻana me ka ʻole o ka pau ʻana.
Inā kānalua kāu kauka he maʻi kou, e hana lākou i kekahi mau hoʻokolohua ma kou koko.
- Ka hana mua: Lawe ʻia kahi hāpana o kou koko a hoʻāʻo ka lab i ka lōʻihi o ka manawa e hoʻopili ai.
- KaʻAnuʻu Hana 2: Inā hōʻike kēlā mau hoʻokolohua i kekahi mau mea ʻē, e hana ʻia nā hoʻokolohua kikoʻī hou aku e ʻike i ka nui o ka factor VIII a me ka factor IX i loko o kou koko.
Hiki i kēia mau hoʻokolohua ke kōkua i kāu kauka e hoʻoholo pono i ke ʻano o ka hemophilia āu (Hemophilia A a i ʻole B), a me kona koʻikoʻi.
He aha nā lāʻau lapaʻau no ka hemophilia?
ʻOkoʻa ka mālama ʻana no ka hemophilia ma muli o ke ʻano a me ke koʻikoʻi o ke kūlana āu e loaʻa nei. No ka laʻana, inā he hemophilia ʻoluʻolu kou, pono paha ʻoe i ka mālama ʻana ma hope o kahi ʻeha a ma mua paha o ke ʻoki ʻana.
Eia nō naʻe, inā loaʻa iā ʻoe ka hemophilia koʻikoʻi , kahi e kahe pinepine ai ke koko, pono ʻoe e hoʻomau i ka lawe ʻana i ka lāʻau lapaʻau mau e pale ai i ke kahe ʻana o ke koko a e pale i kou mau hono mai ka hōʻino ʻia a me ka nāwaliwali.
Aia kekahi mau ʻano lapaʻau nui:
- Ke pani nei i ka mea hoʻoheheʻe koko i hoʻopau ʻia (Replacement therapy)
- Nā lāʻau lapaʻau kūikawā ʻē aʻe
- Ka mālama ʻana no nā hopena ʻaoʻao e like me ke kahe ʻana o ke koko i loko o nā hono
Ke hāʻawi nei i ka mea hana clotting nele
ʻO kēia ka lāʻau lapaʻau nui. Pili kēia i ka hāʻawi ʻana i kou kino i kahi mahele o ka mea hana clotting i nele ma o kahi vein (intravenously a i ʻole IV).
- ʻO Factor VIII no ka poʻe me ka hemophilia AHāʻawi.
- Hāʻawi ʻia ʻo Factor IX i nā poʻe me ka hemophilia B.
Hana ʻia kēia mau mea ma nā ʻano ʻelua. ʻO kekahi mai ka plasma koko i hāʻawi ʻia . ʻO kekahi he mau mea DNA synthetic a recombinant paha i hana ʻia ma ka hale hana . Ua hoʻolālā ʻia nā ʻano mea hou i loaʻa i kēia lā e mau no ka manawa lōʻihi i loko o ke kino. No laila, ʻaʻole pono lākou e hoʻokomo pinepine ʻia e like me ka wā ma mua.
Nā lāʻau lapaʻau kūikawā ʻē aʻe
Ma waho aʻe o ka lāʻau lapaʻau clotting factor, aia kekahi mau lāʻau lapaʻau hou e hiki ke kōkua i ka kaohi ʻana i ke kahe ʻana o ke koko. E wehewehe kāu kauka i kēia mau mea iā ʻoe.
| Lāʻau lapaʻau (inoa hōʻailona) | No wai? | Nā mea nui |
|---|---|---|
| ʻO Emicizumab-kxwh (HEMLIBRA) | No nā kānaka me ka hemophilia A | Kōkua ia i ka pale ʻana i ke kahe ʻana o ke koko. ʻAʻole hāʻawi ʻia kēia ma ke ʻano intravenous, akā ma ke ʻano he inikini subcutaneous i hoʻokahi manawa i ka pule. |
| Nā lāʻau lapaʻau e like me Adynovate, Idelvion | No nā kānaka me ka hemophilia A a i ʻole B | He mau mea hoʻohuihui i hoʻomaikaʻi ʻia kēia i hāʻawi ʻia ma kahi o Factor VIII a i ʻole IX. Noho lōʻihi kēia i loko o ke kino. |
| ʻO Desmopressin (DDAVP) | No nā poʻe me ka hemophilia A ʻoluʻolu | Hoʻonui kēia lāʻau lapaʻau i nā pae o ke kino o ka factor VIII no kekahi manawa. Hiki ke hāʻawi ʻia ma ke ʻano intravenous, ma ke ʻano he injection, a i ʻole ma ke ʻano he ihu spray. |
| ʻAkika Tranexamic, ʻakika Aminocaproic | Ma nā hihia kūikawā | He mau lāʻau lapaʻau kēia i lawe ʻia ma ke ʻano pila. Hana lākou ma ka pale ʻana i ka heheʻe maʻalahi o kahi ʻūpā koko. |
| ʻObizur | No nā kānaka me ka hemophilia A i loaʻa | He kūlana kūikawā kēia. ʻAʻole ia he mea i hānau ʻia me ʻoe. Hiki iā ia ke ulu aʻe ma hope o ke ola ma muli o nā kumu e like me ka hāpai ʻana a me ke kanesa. Hoʻōla ʻia me kahi ʻano factor VIII i loaʻa mai nā puaʻa. |
E makaʻala i ke kahe ʻana o ke koko i loko o nā hono a me nā pilikia ʻē aʻe.
ʻO kahi hōʻailona maʻamau o ka hemophilia ka kahe ʻana o ke koko i loko o nā hono . Hiki ke hana kēia ma nā wahi e like me nā kuli, nā kuʻekuʻe lima, a me nā kuʻekuʻe wāwae. Ke hana ʻia kēia, pehu a ʻeha ka hono.
- Lapaʻau: He mea nui e loaʻa koke kāu lāʻau lapaʻau kumu. A i ʻole, hiki ke hōʻino mau loa i ke hono. Eia kekahi, e aʻo aku kāu kauka iā ʻoe e hoʻomaha i ke hono a kau i ka hau . E hōʻemi kēia i ka ʻeha a me ka pehu.
- Ma hope: Ma hope o ka pau ʻana o ka ʻeha, hiki ke ʻōlelo ʻia no ka hoʻōla kino e kōkua i ka hoʻihoʻi ʻana i ka neʻe ʻana a me ka ikaika o nā hono.
Eia kekahi, pono ʻoe e makaʻala i nā hana kino āu e hana ai. ʻAʻole kūpono nā haʻuki āpau no ka mea me ka hemophilia. Hiki i nā haʻuki pili weliweli ke hoʻoulu i ke kahe koko i loko. E kamaʻilio me kāu kauka e hoʻoholo i nā hoʻoikaika kino a me nā haʻuki kūpono a kūpono ʻole iā ʻoe.
Hiki i nā lāʻau lapaʻau ke hoʻoulu i nā pilikia?
ʻOiai ua palekana loa nā lāʻau lapaʻau i kēia lā, aia ʻelua mau mea āu e ʻike ai.
1. Nā maʻi i lawe ʻia e ke koko
I ka wā ma mua, ma kahi o nā makahiki 1980, he kiʻekiʻe ka pilikia o ka loaʻa ʻana o nā maʻi e like me ka HIV a me ka hepatitis mai nā kumu koko. Akā i kēia manawa, ʻaʻole pono ʻoe e hopohopo no kēlā. I kēia mau lā, ua nānā pono ʻia nā mea hāʻawi koko, a ua hoʻāʻo ʻia nā koko i hāʻawi ʻia no nā maʻi a hoʻomaʻemaʻe ʻia me ka hoʻohana ʻana i nā ʻano kūikawā. No laila, i kēia manawa ua haʻahaʻa loa ka pilikia o ka loaʻa ʻana o kahi maʻi mai ka lāʻau lapaʻau. Eia nō naʻe, ʻōlelo nā kauka e loaʻa ka lāʻau pale no ka hepatitis A a me B.
2. Nā loli i loko o ka ʻōnaehana pale (Nā mea hoʻopale)
He mea paʻakikī iki kēia, akā he mea nui e hoʻomaopopo. I kekahi manawa, ʻike ka ʻōnaehana pale o ko mākou kino, ʻo ia hoʻi ka ʻōnaehana pale o ko mākou kino, i kēia mea hoʻopaʻa koko a mākou e hāʻawi ai iā ia ma ke ʻano he 'ʻenemi'. A laila, hoʻomaka ia e luku i kēlā mea. Kāhea mākou i kēia ʻo ke kūkulu ʻia ʻana o nā "inhibitors" . Inā hana kēia, ʻaʻole paha e holo pono ka lāʻau āu e lawe nei. No laila, e hoʻāʻo mau kāu kauka i kou koko e ʻike inā hana kēia.
Leka Lawe-Home
- ʻO ka Hemophilia kahi maʻi hānau e hoʻopilikia ai i ke kahe koko.
- ʻO ka lāʻau lapaʻau nui, ʻo ia ka hoʻokele ʻana i ka factor exogenous VIII a i ʻole IX, kahi mea i nele i ke kino.
- Na kāu kauka e hoʻoholo i ka lāʻau lapaʻau maikaʻi loa nāu, ma muli o ke ʻano a me ke koʻikoʻi o kou kūlana.
- He mea maʻamau ke kahe ʻana o ke koko i loko o nā hono, a inā loaʻa, he mea nui loa e ʻimi koke i ka lāʻau lapaʻau.
- He palekana loa nā lāʻau lapaʻau no ka hemophilia i hāʻawi ʻia i kēia lā. Mai makaʻu pono ʻole iā lākou.
- Inā loaʻa iā ʻoe a i ʻole kāu keiki kēia maʻi, e hana mau me kāu kauka a hahai i kāna ʻōlelo aʻo.











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