Aia nā maʻi i ʻike nui ʻia i waena o kākou, a me nā maʻi i laha ʻole loa, ʻo ia hoʻi, he mea laha ʻole loa. ʻAʻole paha ʻoe i lohe i ia ʻano maʻi. I kēia lā, e kamaʻilio mākou e pili ana i ia ʻano maʻi laha ʻole, akā he mea nui e pili ana i ke koko, he mea nui e mālama pono ʻia. ʻO kona inoa ʻo Paroxysmal Nocturnal Hemoglobinuria, a mākou e kapa nei he PNH no ka pōkole.
I ka ʻōlelo maʻalahi, he aha ka PNH?
ʻAʻole he maʻi hoʻoilina ka PNH, akā he maʻi i hoʻoilina ʻia a i hoʻoili ʻia mai nā mākua a i nā keiki. Hiki ke ulu i kēlā me kēia makahiki. I ka ʻōlelo maʻalahi, he maʻi ko ke kanaka me kēia maʻi kahi e hoʻouka hewa ai
ka ʻōnaehana pale , ka ʻōnaehana e pale iā mākou mai ka maʻi, a luku i kā lākou mau hunaola koko ʻulaʻula ponoʻī. E noʻonoʻo iā ia e like me ka pale i hana ʻia me nā protein kūikawā e pale iā mākou mai ka maʻi. ʻAʻohe pale pale o kekahi mau hunaola koko ʻulaʻula i loko o ke kino o nā maʻi PNH, ʻo ia hoʻi kēlā mau protein kūikawā. No laila, ʻike ka ʻōnaehana pale i kēia mau hunaola me ka ʻole o ka pale, manaʻo lākou he mea hoʻouka kaua ʻino lākou i ke kino a luku iā lākou. ʻOiai hiki i kēia kūlana ke hoʻoweliweli i ke ola,
me ka mālama kūpono, hiki iā ʻoe ke kāohi i nā hōʻailona, hoʻēmi i nā pilikia a ola i kahi ola maikaʻi. ʻAʻole pili kēia maʻi i nā mea āpau ma ke ʻano like. Loaʻa i kekahi poʻe ka ʻoluʻolu iki. Hiki i kekahi ke hoʻopilikia nui ʻia e ia.
ʻO ka pilikia nui ka ʻūlū koko. Ma kahi o 30% o nā maʻi PNH e pilikia ana i ka loaʻa ʻana o kahi ʻūlū koko ma ka liʻiliʻi hoʻokahi i ko lākou ola.
He aha ke kumu o kēia?
ʻO ke kumu o kēia maʻi he mutation i loko o kahi gene. Akā,
ʻaʻole kēia he mea i hoʻoilina ʻia. ʻO ia hoʻi, ʻaʻole ia he mea āu e loaʻa ai mai kou mau mākua mai, ʻaʻole hoʻi he mea āu e hoʻoili ai i kāu mau keiki. He loli genetic maʻamau kēia e hana ʻia i ka wā o ke ola. Ma muli o kēia mutation gene, hoʻomaka kou iwi iwi e hana i nā hunaola koko ʻulaʻula ʻē aʻe ʻaʻohe o kēlā mau protein pale. No laila, ʻoiai ʻaʻole i pale ʻia kēia mau hunaola e ka ʻōnaehana pale, ua haki maʻalahi lākou. I ka lāʻau lapaʻau, kapa mākou i kēia kaʻina hana o ka haki ʻana o nā hunaola koko ʻulaʻula
he hemolysis . Manaʻo kekahi mau kauka he pili ka PNH i ka nāwaliwali o ka iwi iwi. ʻOi aku ka nui o ka poʻe me ke ʻano i kapa ʻia ʻo aplastic anemia e loaʻa iā lākou ka PNH. Eia kekahi, hiki i ka poʻe me PNH ke loaʻa i ka anemia aplastic ma hope. I kēia ʻano i kapa ʻia ʻo aplastic anemia, hoʻōki ka iwi iwi i ka hana ʻana i nā hunaola koko hou.
He aha nā hōʻailona o ka PNH?
Ua kapa ʻia ka maʻi ma hope o kekahi o kona mau hōʻailona nui. ʻO Paroxysmal ke ʻano "hiki koke mai", ʻo Nocturnal ke ʻano "i ka pō", a ʻo Hemoglobinuria ke ʻano "hoʻokuʻu ʻia ʻana o ka hemoglobin i loko o ka mimi". ʻO Hemoglobin ka protein e hāʻawi i ke koko i kona kala ʻulaʻula. No laila, i ka wā e haki ai nā hunaola koko ʻulaʻula, hoʻokuʻu ʻia kēia hemoglobin i loko o ka mimi,
a hiki i ka mimi i hoʻokuʻu ʻia i ka pō a i ke kakahiaka paha ke ʻeleʻele (e like me ka cola) a ʻulaʻula paha . Ma kahi o 50% o ka poʻe me PNH e ʻike i kēia hōʻailona. ʻO nā hōʻailona ke kumu nui o ʻekolu mau kumu:
ʻO ka nui o nā hunaola koko kīnā ʻole i loko o kou kino, ʻo ka nui aku o nā hōʻailona āu e ʻike ai.
Nā hōʻailona maʻamau i hoʻokumu ʻia e ka haki ʻana o nā sela koko ʻulaʻula a me ka anemia
| Manaʻo luhi loa a nāwaliwali hoʻi | Manaʻo luhi ʻoiai ma hope o ka hana ʻana i kahi hana liʻiliʻi. |
| ʻEha poʻo | ʻEha pinepine ka ʻeha. |
| Paʻakikī ka hanu ʻana | Ke kani ʻana o ka hanu ʻoiai ke hele wāwae nei i kahi mamao pōkole. |
| Nā palpitations o ka puʻuwai | Manaʻo e kuʻi wikiwiki ana kou puʻuwai. |
| ʻeha ka ʻōpū | ʻEha ʻōpū me ke kumu ʻole. |
| Paʻakikī ke ale ʻana | Ka paʻakikī i ka ale ʻana i ka meaʻai a i ʻole nā wai. |
| ʻIli keʻokeʻo a melemele paha | Melemele o ka ʻili a me nā keʻokeʻo o nā maka. |
| ʻEha maʻalahi | ʻOiai kahi ʻeha liʻiliʻi hiki ke hana i nā palapū nui. |
| Ka hana pono ʻole o ka moekolohe i nā kāne | ʻO ka paʻakikī i ka hoʻokō ʻana a i ʻole ka mālama ʻana i ka hoʻonāukiuki i ka wā o ka moekolohe. |
He mea nui loa: He kūlana weliweli ka ʻūpā koko e pono ai ke kōkua lapaʻau koke. E makaʻala pono i nā hōʻailona ma lalo nei.
| Kahi o ke koko koko | Nā hiʻohiʻona e nānā ai |
|---|
| ʻIli | He ʻāpana ʻulaʻula, ʻeha, a pehu paha ma ka ʻili. |
| Lima a i ʻole ka wāwae | ʻEha ka lima/wāwae, mehana, a me ka pehu. |
| ʻōpū | ʻEha nui o ka ʻōpū, nā ʻōpū palapū, a me ke kahe ʻana o ke koko. |
| lolo | ʻEha nui me ka luaʻi ʻole a i ʻole ka luaʻi ʻana, nā ʻūlū, ka paʻakikī i ka neʻe ʻana, ka ʻōlelo ʻana, a i ʻole ka ʻike ʻana. |
| Nā māmā | Paʻakikī ka hanu ʻana, ʻeha ʻoi o ka umauma e like me ka pahi, ke kuʻi ʻana i ke koko, ka hou ʻana. |
Inā he kānalua iki kou he ʻūpā koko kou e like me kēia,
E hoʻomaopopo koke i kāu kauka. E hele koke i ka Halemai Pilikia (ETU) kokoke loa, ʻoiai inā pilikia ʻoe i ka hanu ʻana, pilikia i ka ʻōlelo ʻana/ʻike ʻana, ʻeha poʻo koke, ʻeha umauma, a i ʻole nā hopu ʻana. Pehea e ʻike pono ai i ka maʻi?
Ke hele ʻoe e ʻike i kāu kauka, e nīnau ʻo ia e pili ana i kāu mau hōʻailona a me ka mōʻaukala olakino. A laila e nānā ʻo ia iā ʻoe a kauoha i kekahi mau hoʻāʻo koko. ʻO ka hoʻāʻo nui e hana ʻia no kēia he hoʻāʻo koko kūikawā i kapa ʻia ʻo flow cytometry . ʻO kēia ka mea e hōʻoia ai aia i loko o kāu mau hunaola koko ʻulaʻula kēia mau protein pale. Eia kekahi, e nānā ʻia kāu helu hunaola koko (helu koko piha) a me nā pae hao. I kekahi manawa pono paha kahi hāpana iwi. He aha nā lāʻau lapaʻau no ka PNH?
ʻElua mau pahuhopu nui o ka mālama ʻana i ka PNH. ʻO kekahi e hōʻoluʻolu i nā hōʻailona, a ʻo kekahi e pale i nā pilikia weliweli (ʻoi aku hoʻi nā ʻūpā koko). ʻO ka lāʻau āu e loaʻa ai e pili ana i ke koʻikoʻi o kou kūlana.- Nā meaolaola hou aʻe: Hāʻawi ʻia ka waikawa Folic a me nā mea hoʻohui hao e kāohi i ka anemia.
- Nā lāʻau lapaʻau kikoʻī: Aia nā lāʻau lapaʻau e like me Eculizumab (Soliris) a me Ravulizumab (Ultomiris) e pale ana i ka haki ʻana o nā hunaola koko ʻulaʻula. Hāʻawi ʻia kēia mau mea ma ke ʻano intravenous. Loaʻa iā lākou ka pono o ka hoʻomalu ʻana i ka anemia, ka hōʻemi ʻana i ka pono no nā hoʻoili koko waho, a me ka hōʻemi ʻana i ka pilikia o nā ʻūpā koko. ʻO Pegcetacoplan (Empaveli) kekahi lāʻau lapaʻau hou i hoʻohana ʻia no kēia kumu.
- Nā Hoʻoili Koko: Inā koʻikoʻi ka anemia, hiki ke loaʻa ka hōʻoluʻolu manawa pōkole ma ka hāʻawi ʻana i ke koko mai kahi kanaka olakino.
- Nā lāʻau hoʻokahe koko: Hāʻawi ʻia nā lāʻau hoʻokahe koko i ka poʻe i pilikia nui i ke koko.
- Hoʻoili Kelepona Kumu ʻIwi: ʻO kēia wale nō ke ala e ho'ōla loa ai i ka PNH. Pili kēia i ka wehe ʻana i ka iwi maʻi a me ka hoʻoili ʻana i nā cell kumu mai kahi kanaka olakino (pinepine he kaikaina). Eia naʻe, no ka mea he hana pilikia loa kēia a paʻakikī hoʻi, ʻōlelo pinepine ʻia no ka poʻe ʻōpio me ka maʻi koʻikoʻi loa.
Na kāu kauka e hoʻoholo i ke ʻano lapaʻau e kūpono loa iā ʻoe. He mea nui loa hoʻi ka mālama ʻana iā ʻoe iho.
I ka wā e noho pū ana me PNH, ʻoi aku ka nui o ka mālama ʻana iā ʻoe iho.- ʻAi maikaʻi: E ʻai i nā meaʻai momona. ʻO ka ʻai ʻana i nā meaʻai waiwai i ka hao (spinach, ʻiʻo) me kahi mea waiwai i ka wikamina C (ʻalani, tangerine) e kōkua i ke kino e omo maikaʻi i ka hao.
- Hoʻoikaika Kino: Hiki i ka luhi ke hoʻolilo i mea paʻakikī ke hoʻoikaika kino. E nīnau i kāu kauka i nā hoʻoikaika kino kūpono iā ʻoe.
- E pale iā ʻoe iho mai nā maʻi : E holoi pinepine i kou mau lima. E pale i nā wahi i piha ai ka poʻe a me nā wahi i noho ai ka poʻe maʻi. Inā loaʻa iā ʻoe ke kuni, e haʻi koke i kāu kauka.
- Ola kino: He mea maʻamau ke kaumaha, huhū, a hopohopo ke lohe ʻoe he maʻi laha ʻole kou e like me kēia. ʻO ke kamaʻilio ʻana e pili ana i kēia mau manaʻo me kahi kanaka hilinaʻi a i ʻole ke kākāʻōlelo hiki ke lilo i mea hōʻoluʻolu nui.
- Hāpai: Inā he wahine ʻoe e hoʻolālā ana e hāpai, e ʻoluʻolu e nīnau i kāu kauka ma mua o ka hāpai ʻana. Hiki i ka PNH ke hoʻopilikia iā ʻoe a me kāu pēpē i ka wā hāpai.
Leka Lawe-Home
- He maʻi koko laha ʻole ka PNH i hoʻokumu ʻia e kahi loli hoʻoilina, akā ʻaʻole i hoʻoilina ʻia.
- ʻO ka pilikia nui a weliweli loa o kēia maʻi, ʻo ia ka ʻūlū koko. E makaʻala mau i kāna mau hōʻailona hōʻike (ʻeha poʻo nui, ʻeha umauma, paʻakikī ka hanu ʻana).
- ʻOiai hiki ke hoʻoweliweli i ke ola, aia nā lāʻau lapaʻau kūpono loa i loaʻa i kēia lā e kaohi i nā hōʻailona a hoʻomaikaʻi i ka maikaʻi o ke ola.
- ʻOiai ʻo ka hoʻoili ʻana o ka iwi iwi wale nō ke ʻano lapaʻau piha no ka PNH, he kiʻekiʻe ka pilikia a kūpono wale nō no nā poʻe maʻi i koho ʻia.
- He mea nui loa e mālama mau i ka launa pū ʻana me kāu kauka a hahai i kāna mau kuhikuhi.
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