Ua ʻike anei ʻoe he ʻeleʻele iki kou mimi, a he koko paha, i ke kakahiaka? Ke hakakā nei ʻoe me ka luhi mau, me ka ʻike ʻana i ka maeʻele? He mau hōʻailona paha kēia o kahi maʻi koko laha ʻole i lohe ʻole ʻia e ka poʻe he nui i kapa ʻia ʻo PNH, a i ʻole Paroxysmal Nocturnal Hemoglobinuria. Mai makaʻu ke lohe ʻoe i kēia inoa. E kamaʻilio kākou e pili ana i nā mea āpau me ka maʻalahi a me ka maopopo.
He aha maoli ka PNH?
I ka ʻōlelo maʻalahi, ʻo ka PNH ka wā e hoʻouka hewa ai ko kākou ʻōnaehana pale ponoʻī a luku i ko kākou mau hunaola koko ʻulaʻula olakino ponoʻī. E noʻonoʻo, ʻo ka mea e hana nei ma ʻaneʻi ua like ia me kahi pūʻali koa i manaʻo ʻia e pale i kahi ʻāina, e hoʻouka ana i nā kānaka o ia ʻāina.
ʻO kēia no ka mea aia kahi hoʻololi genetic i loko o kekahi o nā cell i loko o ko kākou iwi iwi. ʻO ka hopena, ua nāwaliwali iki nā cell koko ʻulaʻula i hana ʻia e kēlā iwi iwi. Hiki ke ʻike maʻalahi ʻia a luku ʻia kēia mau cell nāwaliwali e kā mākou ʻōnaehana pale.
- Ka luku ʻia ʻana o nā hunaola koko ʻulaʻula: Ma nā ʻōlelo lapaʻau, ua kapa ʻia kēia haki ʻana o nā hunaola koko ʻulaʻula he hemolysis .
- Nā loli o ke kala o ka mimi: ʻO ka Hemoglobin kahi protein i loaʻa i loko o nā hunaola koko ʻulaʻula e lawe ana i ka oxygen. Ke wāwahi nā hunaola, hoʻokuʻu ʻia kēia hemoglobin i loko o ke koko, kānana ʻia e nā puʻupaʻa, a hoʻokuʻu ʻia i loko o ka mimi. Ua kapa ʻia kēia he hemoglobinuria . ʻO ia ke kumu e ʻike ʻia ai ka mimi he ʻeleʻele, ʻeleʻele, a i ʻole ke kala koko. ʻIke pinepine ʻia kēia i ka mimi kakahiaka.
Akā e hoʻomanaʻo ʻaʻole like nā hōʻailona o ka PNH no kēlā me kēia kanaka. ʻAʻole paha e ʻike kekahi poʻe i kekahi loli o ke kala o ka mimi.
ʻO kekahi pilikia koʻikoʻi o kēia maʻi, ʻo ia ka hoʻonui ʻia o ka pilikia o nā ʻūpā koko . Hiki i kekahi o kēia mau ʻūpā ke hoʻoweliweli i ke ola. Eia kekahi, hiki i kēia maʻi ke hoʻopilikia ʻaʻole wale i nā hunaola koko ʻulaʻula, akā, i nā hunaola koko keʻokeʻo hoʻi e hakakā ana i ka maʻi a me nā platelets e hoʻōki ai i ke kahe ʻana o ke koko.
He aha nā lāʻau lapaʻau no ka maʻi PNH?
ʻO ka mālama ʻana no ka PNH e pili ana i kāu mau hōʻailona a me ke koʻikoʻi o kou kūlana. E kūkākūkā kāu kauka me ʻoe i ka lāʻau lapaʻau kūpono loa iā ʻoe. Aia ka papa ma lalo nei i kekahi o nā lāʻau lapaʻau nui i loaʻa i kēia manawa.
| ʻAno lapaʻau | I ka ʻōlelo maʻalahi, he aha ka mea e hana ʻia? |
|---|---|
| Hoʻōla lāʻau Androgen | He ʻano hormone kāne kēia. Hoʻoulu lākou i ka iwi iwi a hoʻonui i ka hana ʻana o nā hunaola koko ʻulaʻula. Kōkua lākou i ka hoʻēmi ʻana i ka anemia. |
| Nā lāʻau anticoagulant | Pale lākou i ke kūkulu ʻia ʻana o nā ʻūpā koko i loko o nā kīʻaha koko. Loaʻa kēia mau mea ma ke ʻano he inikini a i ʻole he mau pila. |
| Nā hoʻoili koko | Ke haʻahaʻa loa ka pae o nā hunaola koko ʻulaʻula a i ʻole nā platelet, ua uku ʻia ka hemahema e nā hoʻoili koko o waho. |
| Hoʻoili iwi iwi | ʻO kēia wale nō ke ala e ho'ōla loa ai i ka PNH. Akā, he mea weliweli loa ia. No laila, hoʻohana wale ʻia no nā hihia koʻikoʻi loa. |
| Nā mea hoʻopaʻa hoʻohui | Hoʻopaʻa lākou i ka ʻāpana o ka ʻōnaehana pale e luku ana i nā hunaola koko ʻulaʻula a hoʻōki i kēlā kaʻina hana. ʻO Soliris lāua ʻo Ultomiris kēia mau ʻano lāʻau. |
| Nā kumu ulu | Kōkua ia i ka hoʻonui ʻana i ka hana ʻana o nā hunaola koko ʻulaʻula a keʻokeʻo paha ma ka hoʻoulu ʻana i ka iwi iwi. |
| Nā lāʻau lapaʻau ʻē aʻe | Hoʻonohonoho pū ke kauka i ka waikawa folic, nā mea hoʻohui hao, nā steroid, a me nā immunosuppressants e like me ka mea e pono ai. |
No laila he aha kēia ʻo Empaveli?
He lāʻau hoʻopau hoʻopihapiha hou a ikaika loa ʻo Empaveli i hoʻohana ʻia e mālama i ka PNH.
E noʻonoʻo i kā mākou ʻōnaehana pale ma ke ʻano he kaulahao o nā protein i hoʻopili ʻia. ʻO kekahi ʻāpana o kēia kaulahao i kapa ʻia ʻo 'Complement system'. I loko o ka PNH, lilo kēia ʻōnaehana i mea hana nui a luku i nā hunaola koko ʻulaʻula.
Ua kuhikuhi nā lāʻau lapaʻau kahiko (e.g. eculizumab) i kahi wahi i kapa ʻia ʻo C5 ma kēia kaulahao. Ua hoʻōki wale lākou i ka luku ʻia ʻana o loko o nā kīʻaha koko (intravascular hemolysis).
Akā, ʻo kēia lāʻau lapaʻau hou, i kapa ʻia ʻo Empaveli, ke kuhikuhi nei i kahi wahi mua, i kapa ʻia ʻo C3. ʻO ka pōmaikaʻi o kēia, ʻo ia ka mea e kāohi ai i ka pōʻino i loko o nā kīʻaha koko, a me ka pōʻino i loko o nā ʻōpū e like me ke akepaʻa a me ka spleen (extravascular hemolysis).
No laila, he mea pono loa paha ʻo Empaveli no nā poʻe maʻi i hoʻomau i ka anemia a ʻaʻole i hoʻōla piha ʻia mai nā lāʻau lapaʻau mua.
Pehea e hoʻohana ai iā Empaveli?
He lāʻau lapaʻau ʻo Empaveli hiki ke hoʻohana ʻia ma ka home.
- ʻAno Hana: He inikini subcutaneous kēia.
- Ka pinepine: Hāʻawi pinepine ʻia i ʻelua manawa i ka pule .
- Ka nui o ka lāʻau: Hiki i kāu kauka ke hoʻoponopono i kāu nui ma ka nānā ʻana i ka pae o kahi enzyme i kapa ʻia ʻo 'Lactate Dehydrogenase (LDH)' i loko o kou koko. Ana nā pae LDH i ka nui o nā hunaola koko ʻulaʻula e wāwahi ʻia ana.
- Pehea e hoʻohana ai: ʻElua ala e hoʻohana ai i kēia. ʻO kekahi, ʻo ia ka hoʻohana ʻana i kahi pamu liʻiliʻi e hoʻouna i ka lāʻau lapaʻau i loko o ke kino ma luna o 30-60 mau minuke. ʻO kekahi, he ʻōnaehana inikini hoʻokahi manawa i hoʻopili ʻia i ka ʻōpū.
Mai hopohopo, na kāu kauka a i ʻole kekahi kahu maʻi i aʻo mua ʻia e aʻo iā ʻoe pehea e hoʻohana ai i kēia.
Nā mea e pono ai ʻoe e ʻike e pili ana i ka pono a me ka palekana o kēia lāʻau lapaʻau
Ua hōʻoia ʻia ka holomua o Empaveli ma ʻelua mau haʻawina lapaʻau nui, ʻo PEGASUS lāua ʻo PRINCE.
I ka ʻōlelo maʻalahi, ma nā haʻawina ʻelua, ʻoi aku ka kiʻekiʻe o ka hemoglobin o nā maʻi i lawe iā Empaveli ma mua o ka poʻe i lawe i nā lāʻau ʻē aʻe. Ua emi iki hoʻi ko lākou kahe koko.
Nā hopena ʻaoʻao a me ka palekana
ʻO nā hopena ʻaoʻao nui i ʻike ʻia me Empaveli:
- ʻEha, ʻulaʻula, a pehu paha ma kahi i inikini ʻia: He mea maʻamau kēia. Hiki iā ʻoe ke loaʻa ka hōʻoluʻolu ma ke kau ʻana i kahi ʻeke hau a i ʻole ka hoʻomohu ʻana i loko o ka wai mehana.
- Hoʻonui ʻia ka pilikia o nā maʻi: Ma muli o ke kāohi ʻana o kēia lāʻau lapaʻau i kahi ʻāpana o ka ʻōnaehana pale, aia kahi pilikia o ka lilo ʻana i mea maʻalahi i kekahi mau maʻi bacteria (ʻoi aku ka bacteria i hoʻopuni ʻia).
No ka hōʻemi ʻana i kēia pilikia, e hāʻawi kāu kauka iā ʻoe i nā lāʻau pale āpau āu e pono ai ma mua o kou hoʻomaka ʻana e lawe iā Empaveli. Pono ʻoe e mālama i kahi Kāleka Palekana Maʻi me ʻoe i kou wā e lawe ana i ka lāʻau lapaʻau a no 2 mau mahina ma hope o kou hoʻōki ʻana i ka lawe ʻana. E kōkua kēia i ka poʻe e mālama nei iā ʻoe e ʻike e lawe ana ʻoe i kēia lāʻau lapaʻau inā he pilikia.
He aha ka papahana Empaveli REMS?
He ʻano paʻakikī kēia huaʻōlelo, akā i ka ʻōlelo maʻalahi, ʻo ia hoʻi he papahana palekana kūikawā . Ma muli o ka pilikia o ka maʻi ʻana me nā lāʻau lapaʻau e like me Empaveli, hoʻokō ʻia kēlā mau papahana e nā keʻena aupuni.
Ma lalo o kēia papahana `REMS (Risk Evaluation and Mitigation Strategy)`:
1. Pono ke kauka nāna e kuhikuhi i kāu lāʻau lapaʻau e kākau inoa ʻia ma kēia papahana.
2. Pono hoʻi e hōʻoia ʻia ka hale kūʻai lāʻau lapaʻau e hāʻawi ana iā ʻoe i ka lāʻau lapaʻau no kēia.
3. E ke kauka, pono ʻoe e ʻike pono e pili ana i nā pilikia o ka lāʻau lapaʻau a me nā hōʻailona o kahi maʻi (kuni, ʻeha kino, ʻeha poʻo nui).
ʻO kēia ka mea e hōʻoia ai ua ʻike piha ʻoe i nā pono a me nā pilikia o kēia lāʻau lapaʻau a ke hoʻohana nei me ka palekana.
Leka Lawe-Home
- He maʻi laha ʻole ka PNH akā hiki ke hoʻokele ʻia kahi e hoʻopau ai kāu ʻōnaehana pale ponoʻī i nā hunaola koko ʻulaʻula.
- He lāʻau lapaʻau hou a maikaʻi loa ʻo Empaveli no ka PNH e hoʻōki ana i ka luku ʻia ʻana o nā hunaola koko.
- Hiki i kēia lāʻau lapaʻau ke hoʻoulu i ka maʻi, no laila e hōʻoia e loaʻa iā ʻoe nā lāʻau lapaʻau a kāu kauka e hāʻawi ai iā ʻoe. E makaʻala i nā hōʻailona o ka maʻi.
- Hāʻawi ʻia ʻo Empaveli ma lalo o kahi ʻano hana kūikawā (REMS). E mālama mau i ke Kāleka Palekana Maʻi āu e loaʻa ai me ʻoe.
- Inā he mau nīnau kāu e pili ana i kou kūlana a i ʻole ka lāʻau lapaʻau, e ʻoluʻolu e kamaʻilio me kāu kauka me ke kānalua ʻole.
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