Ua loli ke kala o kou mimi? Ke luhi nei ʻoe? E aʻo kākou e pili ana i kēia maʻi koko laha ʻole (PNH)

ʻO ke koko e kahe ana ma o ko kākou mau aa koko ua like ia me ka muliwai e hāʻawi ana i ke ola, ʻeā? ʻO nā hunaola koko ʻulaʻula i loko o kēia koko e lawe i ka oxygen e pono ai no kēlā me kēia hunaola i loko o ko kākou kino. No laila e noʻonoʻo i ka mea e hiki mai ana inā haki koke kēia mau hunaola koko ʻulaʻula koʻikoʻi a luku ʻia me ke kumu ʻole? He mea weliweli iki ia. I kēia lā, e kamaʻilio kākou e pili ana i kahi maʻi koko kakaʻikahi loa, akā he mea nui loa, e pono ai kākou a pau e makaʻala. Ua kapa ʻia kēia ʻo Paroxysmal Nocturnal Hemoglobinuria, a i ʻole PNH no ka pōkole.

I ka ʻōlelo maʻalahi, he aha ka PNH?

He maʻi koko laha ʻole ka PNH. I kēia hihia , ua luku ʻia nā hunaola koko ʻulaʻula i loko o ko kākou koko me ke kumu ʻole. ʻO ka pololei, hoʻopau kā kākou ʻōnaehana pale ponoʻī i kēia mau hunaola koko ʻulaʻula. ʻO kēia no ka mea, ʻaʻohe ʻano protein kūikawā o nā hunaola koko ʻulaʻula o ka poʻe me kēia maʻi e pale aku iā lākou. No laila, ʻaʻole hiki i ka ʻōnaehana pale o ko kākou kino ke ʻike iā lākou he 'nā hunaola ponoʻī'. Ke luku koke ʻia nā hunaola koko ʻulaʻula ma kēia ʻano, ʻaʻole hiki i ko kākou iwi iwi ke hana i nā hunaola koko ʻulaʻula hou i ka wikiwiki like. A laila emi ke koko i loko o ke kino. Kāhea mākou i kēia maʻi he anemia . Eia kekahi, ʻo kekahi ʻano weliweli o kēia maʻi, ʻo ia ka hoʻonui ʻana i ka pilikia o nā ʻōpū koko i loko o nā kīʻaha koko. ʻAʻole pili ka maʻi PNH i nā kānaka a pau ma ke ʻano like. Loaʻa i kekahi poʻe nā hōʻailona palupalu loa. Hiki iā lākou ke ola maʻamau. Akā no kekahi poʻe, hiki ke ʻoi aku ka koʻikoʻi o ka maʻi. Pono paha lākou e hoʻomau i ka lawe ʻana i nā lāʻau lapaʻau a me nā hoʻoili koko.

He aha ke kumu o ka maʻi PNH?

ʻO ka mea nui ma ʻaneʻi, ʻaʻole he maʻi hānau ʻia ka PNH. ʻO ia hoʻi, ʻaʻole ia e hoʻoili ʻia mai ka makuahine a i ke keiki. ʻAʻole hoʻi he maʻi lele. He maʻi laha ʻole ia. E noʻonoʻo, ma kahi ʻāina e like me ʻAmelika, i ka wā e ʻike ʻia ai he 1.8 miliona mau maʻi kanesa hou i kēlā me kēia makahiki, he 400 a 500 wale nō mau maʻi PNH i ʻike ʻia. Hoʻokumu ʻia kēia maʻi e kahi mutation i loko o kahi gene i kapa ʻia ʻo `PIGA` i loko o nā cell kumu i loko o ko kākou iwi iwi. I ka ʻōlelo maʻalahi, aia kahi hewa liʻiliʻi i nā 'kuhikuhi' i loaʻa i ka iwi iwi, ʻo ia ka hale hana e hana ana i nā cell koko ʻulaʻula. Ma muli o kēia hewa, ʻaʻole loaʻa i nā cell koko ʻulaʻula e hana ʻia ana kēlā uhi protein pale.

Loaʻa iā ʻoe kēia mau hōʻailona?

Ke wāwahi ʻia nā hunaola koko ʻulaʻula, hoʻokuʻu ʻia ka pigment ʻulaʻula i kapa ʻia ʻo hemoglobin i loko o lākou i loko o ke koko. Hoʻoneʻe ʻia kēia mau huahana ʻōpala mai ke kino ma o nā puʻupaʻa i loko o ka mimi. No laila, ʻike ʻia ke kala o ka mimi he ʻulaʻula, ʻeleʻele, a ʻeleʻele paha ma mua o ka maʻamau . Eia naʻe, ʻaʻole like ke kala o ka mimi o nā kānaka a pau me PNH. ʻO ka hapalua wale nō o nā maʻi e ʻike i kēia hōʻailona. A he ʻeleʻele loa ia i loko o ka mimi i hoʻokuʻu ʻia i ka pō a i ʻole ke ala mua ʻana i ke kakahiaka. Ma waho aʻe o kēia hōʻailona nui, aia paha kekahi mau hōʻailona ʻē aʻe. E nānā kākou i ke ʻano o lākou.

ʻO wai ka mea i loaʻa pinepine i kēia maʻi?

Wahi a nā kauka, ʻoi aku ka kiʻekiʻe o ka pilikia o ka hoʻomohala ʻana i ka PNH no kekahi poʻe.

• No ka poʻe me ka iwi iwi nāwaliwali: Inā no kekahi kumu ua nāwaliwali ka hana o ka iwi iwi.
• No ka poʻe me ka maʻi i kapa ʻia ʻo Aplastic Anemia : He maʻi nō hoʻi kēia e hoʻopilikia ai i ka iwi iwi. Loaʻa i ka poʻe me kēia maʻi ka pilikia o ka loaʻa ʻana o ka PNH ma hope.
• No nā poʻe o ka hanauna ʻĀsia: ʻOi aku ka maʻamau o kēia maʻi i nā poʻe ʻĀsia ma mua o nā hui lāhui ʻē aʻe.

ʻIke pinepine ʻia ka PNH i nā pākeke i ko lākou mau makahiki 30 , akā hiki ke loaʻa i ka poʻe ʻōpio, me nā kamaliʻi ʻōpio a me nā pēpē.

Pehea e ʻike ai i ka maʻi?

No ka mea, he maʻi laha ʻole ka PNH a ua like kona mau hōʻailona me nā maʻi ʻē aʻe, hiki i kekahi manawa ke lōʻihi ka manawa e ʻike ai i ka maʻi. ʻOi loa aku hoʻi i kahi kanaka nona ke kala mimi ʻaʻole e loli, inā ʻaʻole kānalua ke kauka i kēia, hiki ke hoʻopaneʻe ʻia ka ʻike. No ka ʻike ʻana i ka maʻi, e nīnau mua kāu kauka iā ʻoe e pili ana i kāu mau hōʻailona a me ka mōʻaukala olakino. A laila, e kauoha ʻo ia i nā hoʻokolohua koko like ʻole. No kēia mau mea, ʻo ka hoʻāʻo maikaʻi loa a pololei loa e hōʻoia i ka PNH he hoʻāʻo koko kūikawā i kapa ʻia ʻo 'Flow Cytometry'. Hiki i kēia hoʻāʻo ke ana pololei i ka nui o nā hunaola koko ʻulaʻula i nele i kēlā protein pale i loko o kou koko.

He aha nā lāʻau lapaʻau no ka PNH?

ʻO ka mea pōmaikaʻi, aia kekahi mau lāʻau lapaʻau kūpono no ka mālama ʻana i ka PNH. E loiloi kāu kauka i kou kūlana a hoʻoholo i ka lāʻau lapaʻau kūpono loa iā ʻoe.

• Nā mea hoʻopau i ka ʻōnaehana pale: Hana nā lāʻau lapaʻau e like me eculizumab (Soliris) a me ravulizumab ( Ultomiris ) ma ke kāohi ʻana i kā mākou ʻōnaehana pale mai ka luku ʻana i nā hunaola koko ʻulaʻula. Hoʻemi pū kēia i ka pilikia o nā ʻūpā koko.
• Nā Steroid: Hoʻohana ʻia kēia mau mea i kekahi manawa e kāohi i ka luku ʻia ʻana o nā hunaola koko ʻulaʻula.
• Nā mea hoʻokahe koko:Hāʻawi ʻia kēia mau mea e hōʻemi i ka pilikia o nā ʻūpā koko.
• Nā hoʻoili koko: Ke haʻahaʻa loa ka helu koko o ke kino, hāʻawi ʻia ke koko i waho e hoʻonui i ka helu o nā hunaola koko ʻulaʻula.

Eia kekahi, aia nā lāʻau lapaʻau e kōkua i ke kino e hana i nā hunaola koko ʻulaʻula hou aʻe i loko.

• Nā huaora e like me ka waikawa folic
• Kumu ulu kanaka
• ʻO Androgen, ka hormone kāne

Hiki i ka hoʻoili iwi iwi ke ho'ōla loa i ka PNH. Eia nō naʻe, no ka mea he hana ʻoki kino pilikia loa a paʻakikī hoʻi, paipai wale nā ​​kauka inā ʻaʻole i holo pono nā lāʻau lapaʻau ʻē aʻe a pau. PNH Sinhala, Paroxysmal nocturnal hemoglobinuria, nā maʻi koko, ka haki ʻana o nā hunaola koko ʻulaʻula, ka mimi ʻeleʻele, nā maʻi iwi ʻiʻo, anemia