He aha ʻo Porphyria? Mai makaʻu, e kamaʻilio maʻalahi kākou e pili ana.

Ua loaʻa anei iā ʻoe kahi ʻano wela a ʻā paha o kou ʻili ke hele ʻoe i waho i ka lā? A i ʻole ua loaʻa paha iā ʻoe kahi ʻeha nui o ka ʻōpū me ka nausea a me ka luaʻi ʻana me ke kumu ʻole? He ʻokoʻa kēia mau hōʻailona mai nā maʻi maʻamau. Malia paha ma hope o kēia he maʻi laha ʻole loa i lohe ʻole ʻia e ka poʻe he nui. ʻO ia kekahi maʻi i kapa ʻia ʻo Porphyria . Mai makaʻu i kou lohe ʻana i kēia inoa. I kēia lā, e kamaʻilio mākou e pili ana i kēia me ka maʻalahi a ma ke ʻano hiki iā ʻoe ke hoʻomaopopo.

I ka ʻōlelo maʻalahi, he aha ka Porphyria?

No ka hoʻomaopopo ʻana i kēia, e noʻonoʻo mua kākou i kahi hale hana liʻiliʻi i loko o ko kākou kino. Aia kahi protein koʻikoʻi loa e hāʻawi ana i ko kākou mau ʻulaʻula koko i ko lākou kala ʻulaʻula a lawe i ka oxygen mai nā māmā a i ke koena o ke kino. Ua kapa ʻia ʻo 'heme'. Hana ʻia kēia heme i loko o ko kākou kino ma kekahi mau ʻanuʻu, e like me kahi laina hōʻuluʻulu. E like me ke hōʻuluʻulu ʻana i kahi kaʻa. Ma kēlā me kēia ʻanuʻu, hana nā protein kūikawā i kapa ʻia ʻo nā enzymes .

I kēia manawa, e noʻonoʻo, he aha ka mea e hana ʻia inā paʻa kekahi ʻanuʻu i kēia kaulahao hana, a inā ʻaʻole hana pono kekahi mea hana (enzyme)? ʻAʻole hiki ke hana ʻia ka ʻanuʻu aʻe, a puʻu nā mea maka, ʻeā? Pēlā nō, ke loaʻa kahi hemahema i kahi ʻanuʻu i ka hana ʻana o ka heme, hoʻomaka nā kemika i hana ʻia ma ka ʻanuʻu ma mua, i kapa ʻia ʻo 'porphyrins' a me 'porphyrin precursors', e hōʻiliʻili i loko o ke kino. ʻO ka hōʻiliʻili ʻana o kēia mau kemika i loko o ke kino e hoʻoulu ai i nā hōʻailona o ka porphyria. Hiki i kēia mau mea ke hōʻino i ka ʻōnaehana nerve a i ʻole ka ʻili.

He aha nā ʻano nui o ka porphyria?

Hiki ke hoʻokaʻawale ʻia ʻo Porphyria i ʻelua mau māhele nui, ma muli o ke ʻano o ka ʻike ʻia ʻana o nā hōʻailona. ʻO ka hoʻomaopopo ʻana i kēia hoʻokaʻawale ʻana e kōkua iā ʻoe e hoʻomaopopo maikaʻi i ka maʻi.

Aia kekahi mau ʻano ʻē aʻe ma lalo o kēia mau ʻano nui ʻelua. No ka laʻana, ʻo ke ʻano i kapa ʻia ʻo 'Acute Intermittent Porphyria (AIP)', ka mea i hāʻule ma lalo o ka porphyria acute, a me ke ʻano i kapa ʻia ʻo 'Porphyria Cutanea Tarda (PCT)', ka mea i hāʻule ma lalo o ka cutaneous porphyria, ʻaʻole i maʻamau.

He aha nā mea hoʻoulu e hōʻike ai i nā hōʻailona?

ʻOiai he genetic ka porphyria, ʻaʻole i ʻike mau ʻia nā hōʻailona. Aia kekahi mau mea e hiki ai ke hoʻoulu i nā hōʻailona, ​​a i ʻole kahi "hoʻouka". Ua kapa ʻia kēia mau mea ʻo 'nā mea hoʻoulu'. ʻO ka pale ʻana i kēia mau mea ke ala maikaʻi loa e mālama ai i ka maʻi.

• Kekahi mau lāʻau lapaʻau: Hiki i kekahi mau lāʻau ʻeha , nā pila hoʻomalu hānau, nā anesthetics, a me kekahi mau lāʻau hopu ke hana i kēia. Inā loaʻa iā ʻoe ka porphyria, pono ʻoe e kamaʻilio me kāu kauka ma mua o ka lawe ʻana i kekahi lāʻau lapaʻau.
• ʻO ka waiʻona a me ka puhipaka : ʻO ka inu nui ʻana a me ka puhipaka, ʻoiai i ka manawa like, nā mea hoʻonāukiuki nui.
• ʻAi a me ka hoʻokē ʻai: Hiki i nā mea e like me nā ʻai haʻahaʻa-carb , nā ʻai pohō kaumaha paʻa, a me ka hoʻokē ʻai ke hoʻoulu i nā hōʻailona.
• Ke kaumaha noʻonoʻo a me ke kino: Hiki i nā mea e like me ka hana nui, ke kaumaha noʻonoʻo, a me ke ʻoki kino ke hoʻopilikia iā ia.
• Nā Maʻi: Hiki ke ʻike ʻia nā hōʻailona ma muli o ke kaumaha i kau ʻia ma ke kino e kekahi maʻi a maʻi ʻē aʻe paha.
• Lā: ʻO ka lā ke kumu nui no ka poʻe me Cutaneous Porphyria.

Āhea ʻoe e ʻike ai i ke kauka? Pehea e hana ʻia ai ka hōʻailona?

No ka mea, he mea kākaʻikahi kēia maʻi a ua like nā hōʻailona me nā maʻi maʻamau ʻē aʻe, hiki i kekahi manawa ke lōʻihi ka manawa e ʻike ai. He mea nui e ʻike i ke kauka inā loaʻa iā ʻoe kekahi o kēia mau hōʻailona, ​​ʻoiai inā pinepine lākou.

I ka porphyria koʻikoʻi

Inā loaʻa iā ʻoe ka ʻeha nui o ka ʻōpū me nā hōʻailona e like me ka nausea, ka luaʻi ʻana, ka paʻa ʻana o ka ʻōpū, ka piʻi ʻana o ka puʻuwai, ke koko kiʻekiʻe, ka nāwaliwali o nā ʻiʻo, ka huikau, a i ʻole nā ​​​​​​hōʻailona, ​​​​he mea nui loa e hele koke i ka haukapila, ʻoiai kahi Emergency Treatment Unit (ETU), no ka mea, hiki i kēia ʻano ke hoʻoweliweli i ke ola i kekahi manawa.

I loko o ka Porphyria ʻili

Inā loaʻa i kou ʻili nā pehu, nā ʻeha, ka mumū, ka ʻulaʻula, a i ʻole nā ​​​​​​kalai maʻalahi ma hope o ka lā, e ʻike i ke kauka dermatologist.

Kaʻina hana hōʻoia:

1. Hoʻokolokolo lapaʻau: ʻO ka mea mua, e nānā ke kauka iā ʻoe, e nīnau pono iā ʻoe e pili ana i kāu mau hōʻailona a me ka mōʻaukala lapaʻau ʻohana.

2. Nā hoʻokolohua keʻena hoʻokolohua: Inā kānalua ʻia ka porphyria, e hoʻāʻo ʻia kāu mimi, koko, a me nā hāpana ʻōpala paha no nā pae o nā porphyrins. I nā hihia koʻikoʻi, hiki i ka mimi ke lilo i ʻulaʻula ʻeleʻele a poni paha.

3. Nā Hoʻāʻo Hoʻoilina: Inā hōʻoia ʻia ka maʻi e nā hoʻokolohua hoʻokolohua, hiki ke hana ʻia nā hoʻāʻo hoʻoilina e ʻike ai i ka hoʻololi hoʻoilina kikoʻī i hoʻokumu ai.

He aha nā koho lapaʻau?

ʻOkoʻa nā lāʻau lapaʻau no ka porphyria ma muli o ke ʻano o ka maʻi a me ke koʻikoʻi o nā hōʻailona. ʻOiai ʻaʻohe lāʻau lapaʻau, hiki ke hoʻomalu ʻia nā hōʻailona a hiki ke ola i kahi ola maʻamau.

Lapaʻau no ka Porphyria ʻĀkiu

Ke koʻikoʻi nā hōʻailona (hoʻouka koʻikoʻi), pono ka mālama ʻana i ka haukapila a me ka mālama ʻia.

• Ke hāʻawi ʻana i ka saline (nā wai IV): E kaulike i ka paʻakai a me ka wai o ke kino.
• Hoʻokomo Heme: Kāohi i ka hemahema heme i loko o ke kino a hoʻemi i ka nui o ka porphyrin e hōʻiliʻili ana.
• Ka hāʻawi ʻana i ka glucose: ʻO ka hāʻawi ʻana i nā kō e like me ka dextrose ma o ka intravenous a i ʻole ma ka waha e hōʻike i ke akepaʻa e hōʻemi i ka hana ʻana o ka porphyrin.
• Nā lāʻau lapaʻau ʻē aʻe: Nā lāʻau hoʻopau ʻeha no ka ʻeha nui, a me nā lāʻau lapaʻau no ka nausea a me ka luaʻi.
• Nā lāʻau lapaʻau hou e like me Givosiran (Givlaari): No ka poʻe i loaʻa nā hōʻailona pinepine, hāʻawi ʻia kēia lāʻau lapaʻau i kēlā me kēia mahina e hōʻemi i ko lākou alapine (pinepine) a me ke koʻikoʻi.

Lapaʻau no ka Porphyria ʻili

• Ka palekana o ka lā:ʻO kēia ka mea nui loa. He mea nui ka ʻaʻahu ʻana i nā lima lōʻihi, nā pāpale, a me ka hoʻopili ʻana i ka sunscreen me kahi pae palekana kiʻekiʻe, e like me SPF 50+.
• Phlebotomy: Pili kēia i ka wehe ʻana i kahi koko liʻiliʻi (ma kahi o hoʻokahi ʻāpana) mai ke kino i kēlā me kēia mau pule. Kōkua kēia i ka hōʻemi ʻana i ka nui o ka hao keu i loko o ke kino a kōkua i ka wehe ʻana i nā porphyrins i hōʻiliʻili ʻia i loko o ke akepaʻa.
• Nā lāʻau lapaʻau: Hiki i nā lāʻau lapaʻau haʻahaʻa e like me chloroquine a hydroxychloroquine paha ke wikiwiki i ka wehe ʻana o nā porphyrins mai ke akepaʻa.