Loaʻa iā ʻoe nā hōʻailona e like me ka luhi mau, ka pōkole o ka hanu i ka wā e hele iki ana, a i ʻole ka mimi ʻeleʻele i ke kakahiaka? I kekahi manawa hiki i kēia ke lilo i mau hōʻailona o kahi maʻi koko laha ʻole i kapa ʻia ʻo Paroxysmal Nocturnal Hemoglobinuria, a i ʻole PNH e like me kā mākou kauka e kapa nei. Mai makaʻu i kou lohe ʻana i kēia inoa. ʻOiai he mea paʻakikī iki, e kamaʻilio kākou e pili ana i kēia maʻi a me kahi lāʻau hou no ia mea i kapa ʻia ʻo Fabhalta ma nā ʻōlelo maʻalahi a kikoʻī.
He aha maoli ka PNH?
I ka ʻōlelo maʻalahi, he maʻi koko kakaikahi loa ka PNH i hoʻokumu ʻia e kahi kīnā genetic. Loaʻa i ko mākou mau hunaola koko ʻulaʻula (RBC) kahi uhi protein pale a puni lākou. Eia nō naʻe, i loko o ka PNH, ʻaʻole i hoʻokumu ʻia kēia uhi pale.
E noʻonoʻo i ko kākou mau hunaola koko ʻulaʻula e like me nā koa me ka ʻole o ke kapa kila. Me ka ʻole o kēia pale, huikau ka ʻōnaehana pale ponoʻī o ko kākou kino a hoʻomaka e hoʻouka a luku i kēia mau hunaola koko ʻulaʻula olakino, me ka manaʻo he ʻenemi lākou. ʻO kēia haki mua ʻana o nā hunaola koko ʻulaʻula ka mea a mākou e kapa nei ma ke ʻano lapaʻau he hemolysis . Hiki i kēia luku ke hana ʻia ma loko o nā kīʻaha koko (intravascular) a ma waho o nā kīʻaha koko (extravascular).
Ke luku ʻia nā hunaola koko ʻulaʻula ma kēia ʻano, ʻo nā hōʻailona nui hiki ke lilo i anemia a me nā ʻūpā koko. Hiki i kēia mau ʻūpā koko ke hoʻoweliweli i ke ola. Hiki i ke kanaka me PNH ke ʻike i nā hōʻailona he nui, a i ʻole he kakaikahi wale nō paha kā lākou.
| Nā hōʻailona maʻamau o ka maʻi PNH | |
|---|---|
| Luhi mau a nāwaliwali paha | Paʻakikī ka hanu ʻana |
| ʻEha poʻo | Puʻuwai wikiwiki a ʻano ʻole paha |
| ʻEha ʻōpū | ʻIli palapū maʻalahi |
| ʻO ka mimi ʻeleʻele (ʻeleʻele), ʻoi aku hoʻi i ka pō a i ke kakahiaka paha | ʻUlaʻula, ʻeha, a me ka pehu ʻana ma kahi kikoʻī o ka ʻili (he hōʻailona paha ia o ke koko koko) |
ʻO ka mālama ʻana no ka PNH e komo pū ana me nā hoʻoili koko, ka waikawa folic a i ʻole nā papa hao no ka anemia, nā anticoagulants, a me nā lāʻau lapaʻau e pale ai i nā hunaola koko ʻulaʻula mai ka hoʻouka ʻana e ka ʻōnaehana pale. ʻO ka mālama ʻana āu e loaʻa ai e pili ana i ka nui o nā hōʻailona āu e loaʻa ai a me ke koʻikoʻi o lākou.
Pehea e hana ai ka lāʻau lapaʻau ʻo Fabhalta?
ʻO nā lāʻau lapaʻau e like me ka eculizumab (Soliris) a i ʻole ravulizumab (Ultomiris) i hāʻawi pinepine ʻia ma ke ʻano he inikini no ka PNH e hana ana ma ke ālai ʻana i kahi ʻāpana o kā mākou ʻōnaehana pale i kapa ʻia ʻo ka ʻōnaehana complement, i kapa ʻia ʻo C5. ʻOiai ke hoʻōki nei kēia mau mea i ka hemolysis intravascular, hiki i kekahi poʻe ke hoʻomau i ka loaʻa ʻana o ka anemia ma muli o ka hemolysis extravascular.
ʻO kēia kahi e lilo ai ka lāʻau lapaʻau waha mua i kapa ʻia ʻo Fabhalta i mea nui.
E noʻonoʻo i ko kākou ʻōnaehana pale ma ke ʻano he pūʻali koa e pale aku ana i ko kākou ʻāina. Ma PNH, huikau nā ʻāpana o kēia pūʻali koa a hoʻouka i ko kākou mau hunaola koko ʻulaʻula olakino ponoʻī. Hoʻopaʻa ʻo Fabhalta i kahi protein i kapa ʻia ʻo factor B e hoʻoulu ai i kēia huikau. Hoʻōki kēia i ka ʻōnaehana pale mai ka hoʻouka ʻana i nā hunaola koko ʻulaʻula. Hoʻonui kēia i ka helu o nā hunaola koko ʻulaʻula olakino, hōʻemi i nā hōʻailona, a hōʻemi i ka pono no ke kahe koko.
Pehea e pono ai iaʻu ke hoʻohana i kēia lāʻau lapaʻau?
ʻO Fabhalta kahi capsule i ale ʻia i ʻelua manawa i ka lā, me ka ʻai a me ka ʻole o ka meaʻai. Mai uhaʻi, kuʻi, a nau paha i ka capsule. Inā poina ʻoe i kahi mahele lāʻau, e lawe koke iā ia i kou hoʻomanaʻo ʻana a laila e lawe i nā mahele lāʻau i koe e like me ka maʻamau. Inā ʻoe e lawe nei i kahi lāʻau PNH ʻē aʻe (e like me eculizumab a i ʻole ravulizumab), e wehewehe kāu kauka i ke ala kūpono e hoʻololi ai i Fabhalta.
He mea nui loa: He mea nui e loaʻa iā ʻoe kāu mau lāʻau pale āpau e pono ai ma ka liʻiliʻi he ʻelua pule ma mua o ka hoʻomaka ʻana i kēia lāʻau lapaʻau. Hoʻokomo pū kēia me ka lāʻau pale pneumococcal a me ka lāʻau pale meningitis. Inā ʻaʻole hiki iā ʻoe ke loaʻa i kāu mau lāʻau pale a ʻaʻole hiki ke hoʻomaka iā Fabhalta, e hāʻawi kāu kauka iā ʻoe i nā lāʻau pale a hoʻomaka iā ʻoe i kahi papa o nā antibiotics i ka manawa like.
ʻOiai inā e hoʻomaikaʻi ana kāu mau hōʻailona, mai hoʻōki i ka lawe ʻana iā Fabhalta me ke aʻo ʻole ʻia e ke kauka . Inā hana ʻoe pēlā, e hoʻomaka hou paha kāu mau hunaola koko ʻulaʻula e haki wikiwiki hou.
Pehea i aʻo ʻia ai nā hopena o kēia lāʻau lapaʻau?
Ua mālama ʻia ʻelua mau haʻawina nui e hōʻoia i ka pono a me ka palekana o Fabhalta. Ua komo pū nā mea ʻelua i nā maʻi me PNH a me ka anemia i loaʻa mai ia mea.
1. Haʻawina APPLY-PNH: Ua pili kēia i ka poʻe i loaʻa i ka anemia ʻoiai ua lawe lākou i nā lāʻau PNH ʻē aʻe (eculizumab a i ʻole ravulizumab) no ka liʻiliʻi he 6 mau mahina. Ua māhele ʻia lākou i ʻelua mau pūʻulu, ua hāʻawi ʻia kekahi hui iā Fabhalta, a ua hoʻomau ka hui ʻē aʻe i ka lawe ʻana i ka lāʻau kahiko.
2. Haʻawina APPOINT-PNH: Ua pili kēia i nā poʻe maʻi i loaʻa ʻole i kekahi lāʻau lapaʻau hoʻohui ʻē aʻe no PNH. Ua hāʻawi ʻia iā lākou a pau ʻo Fabhalta.
He mea hoʻoikaika nui nā hopena o kēia mau haʻawina, ma hope o 24 mau pule.
| Pōmaikaʻi i loaʻa | Hopena |
|---|---|
| Hoʻonui ʻia ka pae hemoglobin | ʻOi aku ma mua o 82% o nā maʻi i lawe iā Fabhalta i loaʻa ka piʻi ʻana o ka hemoglobin o 2 g/dL, ke hoʻohālikelike ʻia me ka poʻe ʻole i lawe i nā lāʻau ʻē aʻe. |
| Ke pale ʻana i nā hoʻoili koko | ʻAʻole pono ka 95% o nā maʻi i lawe iā Fabhalta i nā hoʻoili koko o waho i ka wā o ke aʻo ʻana, hoʻohālikelike ʻia me 50% wale nō o ka poʻe i lawe i nā lāʻau ʻē aʻe. |
E hoʻomanaʻo, ʻoiai nā hopena o kēia mau haʻawina, ʻokoʻa paha kāu mau hopena mai kēlā kanaka a kēia kanaka. ʻOi aku ka maikaʻi o ke kūkākūkā ʻana i nā pono a me nā pilikia o ka lāʻau lapaʻau me kāu kauka ma mua o ka hoʻoholo ʻana.
Pehea ʻoe e ʻike ai inā e hana ana ka lāʻau lapaʻau?
Inā holomua ka lāʻau Fabhalta, e hoʻomaka ana kāu mau cell koko ʻulaʻula a me ka hemoglobin e piʻi aʻe. Hiki iā ʻoe ke ʻike i nā mea e like me:
- Hoʻonui ʻia ka ikehu a me ka luhi i emi iho
- Ua emi ka waihoʻoluʻu ʻeleʻele o ka mimi
- Hoʻonui ʻia ka makemake a me ka emi ʻana o ka ʻeha o ka ʻōpū
- Ka hōʻemi ʻana i ka pono no ke kahe koko o waho
- ʻO ka hiki ke hana maʻalahi i nā hana o kēlā me kēia lā
ʻOiai ʻokoʻa nā hopena o ka lāʻau lapaʻau mai kēlā kanaka a kēia kanaka, e mālama i kahi moʻolelo o kāu mau hōʻailona a kūkākūkā me kāu kauka i kēlā me kēia manawa āu e ʻike ai iā ia.
Nā hopena ʻaoʻao a pehea e mālama ai iā lākou?
E like me nā lāʻau lapaʻau āpau, hiki iā Fabhalta ke hana i nā hopena ʻaoʻao. ʻO ka mea maʻamau ka ʻeha poʻo, ka maʻi diarrhea, ka nausea, ka ʻeha ʻōpū, a me ka ʻulaʻula o ka ʻili. Hiki ke ʻike ʻia nā hōʻailona o ke anu maʻamau a me nā maʻi bacteria a viral paha.
Nā maʻi koʻikoʻi e pono ai ka nānā ʻana!
Ma muli o ke kāohi ʻana o kēia lāʻau lapaʻau i kekahi ʻāpana o ka ʻōnaehana pale, aia kahi pilikia o nā maʻi koʻikoʻi mai kekahi mau ʻano bacteria. ʻOi loa aku hoʻi ʻo *Streptococcus pneumoniae*, *Neisseria meningitidis*, a me *Haemophilus influenzae type B*. Hiki i kēia mau maʻi ke hoʻoweliweli i ke ola inā ʻaʻole i ʻike ʻia a mālama koke ʻia.
ʻO ke ala maikaʻi loa e pale aku ai i ka maʻi, ʻo ia ka loaʻa ʻana o nā lāʻau pale āpau i ʻōlelo ʻia , holoi maikaʻi i kou mau lima, a noho mamao aku mai ka poʻe maʻi. Inā ʻike ʻoe i kekahi o kēia mau hōʻailona, e haʻi koke aku i kāu kauka a i ʻole e hele i ka lumi pilikia o ka haukapila kokoke loa (ETU).
| Nā hōʻailona hōʻike o kahi maʻi koʻikoʻi | |
|---|---|
| Kuni me ka phlegm a i ʻole me ka ʻole, ʻeha umauma a me ke kuni, ka puʻuwai wikiwiki | Nā hōʻailona e like me ke kuni a me ka ʻeha o ke kino |
| ʻIli palai | ʻEha poʻo nui me ke kuni, ka nausea, a me ka luaʻi ʻana, a i ʻole ka ʻoʻoleʻa o ka ʻāʻī/kua |
| Ka huikau | Nā loli o ka ʻike |
Inā ʻoe e hopohopo nei e pili ana i kekahi hopena ʻaoʻao, e kamaʻilio koke me kāu kauka. Inā manaʻo ʻoe ua make ʻoe mai kahi lāʻau lapaʻau, hiki iā ʻoe ke kelepona i ke Kikowaena ʻIke Poisons National ma ka Halemai Lahui ʻo Colombo .
He aha nā hoʻokolohua koko e pono ai i ka wā o ka mālama ʻana?
E hana kāu kauka i kekahi mau hoʻāʻo koko e nānā i kou olakino a me kāu pane ʻana i ka lāʻau lapaʻau.
- Nā pae o ke koko ʻulaʻula (RBC) a me ka hemoglobin:Nānā mau ʻia kēia mau mea ma ka PNH. Kōkua kēia i ka ʻike ʻana i ke ʻano o kāu pane ʻana i ka lāʻau lapaʻau a e hoʻoholo inā pono ʻoe i ka hoʻoili koko.
- Nā pae momona koko: Hiki iā Fabhalta ke hoʻonui i kāu mau pae cholesterol a me triglyceride. No laila, e paipai kāu kauka i kahi hoʻāʻo lipid profile hoʻokē ʻai ma mua a i ka wā o ka mālama ʻana.
Hiki paha nā pilikia me nā lāʻau lapaʻau ʻē aʻe?
ʻAe, hiki i kekahi mau lāʻau lapaʻau ke launa pū me Fabhalta. Hiki i kekahi mau lāʻau lapaʻau, e like me Rifampin, ke hōʻemi i ka pono o Fabhalta. ʻAʻole ʻōlelo ʻia ka lawe ʻana iā Fabhalta me kekahi mau lāʻau lapaʻau, e like me Gemfibrozil.
No laila, he mea nui loa e hoʻomaopopo i kāu kauka e pili ana i nā lāʻau lapaʻau āpau āu e lawe nei - me nā lāʻau lapaʻau i kauoha ʻia, nā lāʻau lapaʻau ma waho o ke kauka, nā wikamina, nā lāʻau lapaʻau kuʻuna, a me nā mea hoʻopihapiha.
Leka Lawe-Home
- He kūlana laha ʻole ka PNH kahi e hoʻopau ai ka ʻōnaehana pale ponoʻī o ke kino i nā hunaola koko ʻulaʻula no ka mea ʻaʻohe o lākou papa protein e pale aku iā lākou.
- He lāʻau lapaʻau waha hou ʻo Fabhalta no ka PNH e hana ana ma ka hoʻōki ʻana i kahi hana hewa i loko o ka ʻōnaehana pale a me ka pale ʻana i nā hunaola koko ʻulaʻula.
- Ma mua o ka hoʻomaka ʻana e hoʻohana i kēia lāʻau lapaʻau, pono ʻoe e pale aku i nā maʻi e like me ka meningitis a me ka pneumonia.
- Inā loaʻa iā ʻoe nā hōʻailona o kahi maʻi koʻikoʻi i ka wā o ka mālama ʻana, e like me ke kuni, ka ʻeha poʻo nui, a i ʻole ka huikau, e ʻimi koke i ke kōkua lapaʻau.
- ʻOiai inā e hoʻomaikaʻi ana kāu mau hōʻailona, mai hoʻōki i ka lawe ʻana i ka lāʻau me ka ʻole o ke aʻo ʻana a kāu kauka.
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