Ke ʻike nei ʻoe i ka mumū i loko o kou kino? E aʻo kākou e pili ana i kēia maʻi hou i kapa ʻia ʻo VEXAS Syndrome.

Ke manaʻo nei ʻoe i kekahi manawa e puka mai ana kekahi mea ʻē mai loko mai o kou kino, e like me ka mumū? Loaʻa iā ʻoe i kekahi manawa ke kuni, ʻeha nā hono, nā pilikia ʻili, a pēlā aku? ʻOiai paha he mea pili ʻole kēia mau mea, malia paha ʻo ke kumu o kēia mau mea āpau ke ʻano like ʻole. ʻO kekahi o ia ʻano maʻi ua kapa ʻia ʻo VEXAS Syndrome . E kamaʻilio iki kākou e pili ana i kēia i kēia lā, no ka mea, he mea nui loa ka ʻike ʻana i kēia.

He aha ka VEXAS Syndrome? I ka pōkole...

I ka ʻōlelo maʻalahi, he maʻi autoimmune kakaikahi loa ka VEXAS Syndrome. I kēia manawa ke noʻonoʻo nei paha ʻoe i ke ʻano o kēia maʻi autoimmune. E noʻonoʻo, he ʻōnaehana pale ko ko mākou kino. Ua like ia me kahi pūʻali koa e pale ana i ko mākou ʻāina. ʻO ka hana a kēia ʻōnaehana ka pale ʻana iā mākou ma ke kaua ʻana i nā germs a me nā maʻi e hele mai ana mai waho. Eia naʻe, i kekahi manawa ʻo kēia mea pale ponoʻī, ka ʻōnaehana pale, e hoʻomaka hewa ana e hoʻouka i nā cell olakino a me nā ʻiʻo o ko mākou kino ponoʻī . Me he mea lā ʻaʻole hiki iā mākou ke ʻike ʻo wai ko mākou ponoʻī a ʻo wai ka ʻenemi. ʻO ia ka mea a mākou e kapa nei he maʻi autoimmune.

ʻO ia ka mea e hana ʻia i kahi kanaka me ka VEXAS Syndrome. Hoʻouka ka ʻōnaehana pale i nā ʻāpana like ʻole o ke kino, e hoʻoulu ai i ka mumū a me ka pehu . Me he mea lā he ahi liʻiliʻi mau e hele ana i loko o ke kino.

Hiki i ka VEXAS Syndrome ke hoʻopili i nā ʻāpana o ke kino:

• I kou koko
• I ka iwi ʻiʻo
• I nā kīʻaha koko
• No kou ʻili
• ʻO ke cartilage (ʻoi aku hoʻi ma nā pepeiao a me ka ihu)
• I nā hono
• I nā māmā
• No nā maka
• ʻO nā inoa o nā pua i loko o nā kāne

E noʻonoʻo i ka nui o ka pilikia e hiki mai ana i ka wā e hoʻopilikia ʻia ai nā wahi he nui i ka manawa hoʻokahi. ʻO ke kumu o kēia maʻi he mutation genetic . I mea e pololei ai, he loli i loko o kahi gene i kapa ʻia ʻo ka gene UBA1 . Hoʻopuka kēia gene UBA1 i ka enzyme E1 ubiquitin-activating, a i ʻole ka enzyme E1 no ka pōkole. ʻO ka hana a kēia enzyme ka hoʻomaʻemaʻe ʻana i nā huahana ʻōpala e like me nā protein pono ʻole e hōʻiliʻili ana i loko o kā mākou mau cell a kōkua i ka hoʻoponopono ʻana i nā pōʻino i nā cell. Ua like ia me kekahi e lawe ana i ka ʻōpala i loko o ko mākou hale. Akā i ka wā e loaʻa ai iā ʻoe ka VEXAS Syndrome, ʻaʻole hana pono ka enzyme E1 i hana ʻia e ka gene UBA1. He aha ka mea e hana ʻia a laila? Hōʻiliʻili ka ʻōpala i loko o nā cell.

ʻO ka mea nui loa, inā ʻaʻole mālama pono ʻia kēia maʻi, hiki i kekahi manawa ke hoʻoweliweli i ke ola.No laila, he mea nui e ʻimi i ke kōkua lapaʻau inā loaʻa iā ʻoe nā hōʻailona. E hāʻawi nā kauka i ka lāʻau lapaʻau e pono ai e kāohi i kāu mau hōʻailona.

He aha ka manaʻo o ka inoa ʻo VEXAS?

ʻO ka inoa ʻo VEXAS ka hui pū ʻana o nā leka mua o kekahi mau huaʻōlelo e kōkua i ka ʻike ʻana i kēia maʻi. E nānā kākou i ke ʻano o lākou:

• V - Vacuoles: He mau hakahaka poepoe kēia e hana ʻia ana i loko o nā pūnaewele ʻē. Hiki ke ʻike ʻia kēia mau vacuoles i loko o nā pūnaewele iwi o ka poʻe me VEXAS Syndrome.
• ʻEzyme E - E1: E like me kā mākou i ʻōlelo ai ma mua, ʻo kēia ka enzyme E1 i hana pono ʻole ma muli o kahi mutation i loko o ka gene UBA1.
• X - X-linked: Ua ʻike ʻoe ua hoʻoholo ʻia ko kākou wahine e ʻelua chromosomes. Loaʻa i nā wahine nā chromosomes XX a loaʻa i nā kāne nā chromosomes XY. Aia ka gene UBA1 i hoʻololi ʻia e hoʻoulu ai i ka VEXAS Syndrome ma ka chromosome X.
• A - Autoinflammation: ʻO kēia ka inoa lapaʻau no ka mumū e kū mai ana i ka wā e hoʻouka ai ka ʻōnaehana pale i kona kino ponoʻī.
• S - Somatic: ʻO ka hoʻololi ʻana o ka genetic e hoʻoulu ai i ka VEXAS Syndrome he ʻano hoʻololi i kapa ʻia ʻo "somatic." ʻO ke ʻano kēia, hiki wale mai ia a ʻaʻole i hoʻoilina ʻia mai nā mākua mai. ʻO ke ʻano kēia, ʻaʻole pono ʻoe e hopohopo e pili ana i kāu mau keiki e loaʻa ana ia mea no ka mea ua loaʻa iā ʻoe.

Maopopo iā ʻoe pehea i loaʻa ai ka inoa ʻo VEXAS? Hōʻike kēlā me kēia leka i kahi ʻāpana koʻikoʻi e pili ana i ka maʻi.

Pehea ka maʻamau o ka VEXAS Syndrome?

He ʻano maʻi laha ʻole kēia. Wahi a nā loea, ʻoiai ma kahi ʻāina e like me ʻAmelika, hoʻopilikia kēia maʻi i hoʻokahi i loko o 13,000 mau kānaka. ʻOiai ʻaʻole pololei nā helu helu ma Sri Lanka, ua maopopo he ʻano maʻi maʻamau ʻole kēia.

He aha nā hōʻailona o ka VEXAS Syndrome?

ʻO ka hōʻailona nui o kēia maʻi ka mumū . No laila, pili nā hōʻailona ʻē aʻe i kahi o ke kino e loaʻa ai ka mumū. E nānā inā loaʻa iā ʻoe kekahi o kēia mau hōʻailona:

• Loaʻa pinepine iaʻu ke kuni.
• Haʻahaʻa ka pae oxygen i loko o ke koko (hypoxemia) .
• Nā ʻano ʻili like ʻole a me ka eczema.
• Pehu kino.
• ʻEha hui.
• Kuke.
• Ka paʻakikī o ka hanu ʻana (dyspnea).
• ʻUlaʻula o nā maka.
• ʻEha poʻo.
• ʻO ka pehu ʻana o nā testicles i nā kāne (orchitis).

Inā hoʻomau kekahi a ʻoi aku paha o kēia mau hōʻailona, ​​​​he mea naʻauao ke ʻimi i ka ʻōlelo aʻo lapaʻau ma mua o ka hoʻokuke ʻana iā lākou me he maʻi maʻamau lā.

No ke aha e kū mai ai ka VEXAS Syndrome? He aha ke kumu?

E like me kā mākou i kamaʻilio ai ma mua, ʻo ke kumu nui o kēia he hoʻololi ʻana o ka genetic i loko o ka gene UBA1 . ʻO nā hoʻololi genetic nā loli i loko o kā mākou DNA. Kaʻina DNA . Ke māhele ʻia nā cell, ʻo ia hoʻi, ke hana nā cell i nā kope o lākou iho, i kekahi manawa hiki i kahi hapa o kēia kaʻina DNA ke kū i kahi kūpono ʻole, piha ʻole, a hōʻino ʻia paha. ʻO ia ka manawa e ʻike ʻia ai nā hōʻailona o nā kūlana genetic.

ʻO ka mea e hana ʻia i kekahi me ka VEXAS Syndrome, ʻaʻole hana pono ka gene UBA1, a ʻaʻole hana ʻia ka enzyme E1 e like me ka mea e pono ai. ʻO ka maʻamau, ua like ka enzyme E1 me ka "mea hoʻomaʻemaʻe" i loko o kā mākou mau cell. ʻO kāna hana ka hoʻomaʻemaʻe ʻana i nā ʻōpala, e like me nā protein kahiko a hōʻino ʻia, i loko o nā cell. Akā i ka wā e loaʻa ai iā ʻoe ka VEXAS Syndrome, ʻaʻole hana pono kēia hui "mea hoʻomaʻemaʻe". He aha ka mea e hana ʻia a laila? Hōʻiliʻili nā protein i hōʻino ʻia a me nā ʻōpala i loko o nā cell. Ke ʻike kā mākou ʻōnaehana pale i kēia ʻōpala i hōʻiliʻili ʻia, manaʻo ia he maʻi a he hoʻoweliweli paha ma laila. Akā no ka mea ʻaʻohe maʻi ma laila, hoʻouka ka ʻōnaehana pale i nā ʻiʻo olakino. ʻO ia ke kumu o ka mumū. E noʻonoʻo e like me ke kaʻa ʻōpala ʻaʻole e hoʻomaʻemaʻe i ka hale a hōʻiliʻili ka ʻōpala.

ʻO wai ka mea i ʻoi aku ka pilikia o ka hoʻomohala ʻana i ka VEXAS Syndrome?

Ua ʻike ʻia nā noiʻi he ʻoi aku ka nui o ka loaʻa ʻana o kēia maʻi i nā kāne . ʻOi aku ka maʻamau i nā poʻe ma luna o 50 mau makahiki. ʻO ke ʻano kēia, hiki i nā loli genetic e hele mai me ka ʻelemakule ke pāʻani i kahi kuleana.

He aha nā pilikia e hiki ke loaʻa mai ka VEXAS Syndrome?

Inā hoʻopilikia ka mumū i hoʻokumu ʻia e ka VEXAS Syndrome i kou iwi iwi , hiki iā ia ke alakaʻi i kahi kūlana i kapa ʻia ʻo ka hāʻule ʻana o ka iwi iwi. Hiki i kēia ke hoʻoweliweli i ke ola.

Ma muli o kahi i loaʻa ai ka mumū, ʻoi aku ka nui o ka loaʻa ʻana o kekahi me VEXAS Syndrome i nā pilikia olakino ʻē aʻe, e like me:

• Leukemia ( kahi ʻano maʻi ʻaʻai koko)
• ʻO Myocarditis (ka mumū o ka ʻiʻo puʻuwai)
• Anemia
• Dermatitis ( mumu ʻili)
• Chondritis (ka mumū o ke cartilage)
• ʻO ka Vasculitis (ka mumū o nā kīʻaha koko)
• ʻO ka mumū o nā hono (Arthritis )
• ʻO ke koko i loko o ke aʻa hohonu (Deep Vein Thrombosis - DVT)
• ʻO Colitis (ka mumū o ka ʻōpū nui)

E nānā i ke koʻikoʻi o nā kūlana hiki i kēia ke hana. ʻO ia ke kumu he mea nui ka ʻike mua ʻana a me ka mālama ʻana.

Pehea e ʻike ai nā kauka i ka VEXAS Syndrome?

E ʻike ke kauka i ka VEXAS Syndrome ma ka nānā kino ʻana iā ʻoe a me ka hana ʻana i nā hoʻāʻo genetic . E nānā pono ʻo ia i kāu mau hōʻailona a nīnau iā ʻoe e pili ana i ka lōʻihi o kou loaʻa ʻana o kēia mau hōʻailona.

Eia nō naʻe, ʻo ke ala wale nō e ʻike pono ai inā loaʻa iā ʻoe ka VEXAS Syndrome ma o ka hoʻāʻo ʻana i ka genetics. Ma kēia, e lawe kāu kauka i kahi hāpana o kou koko, ʻili, lauoho, a i ʻole nā ​​​​ʻiʻo ʻē aʻe a hoʻouna aku i kahi lab. E hoʻāʻo nā loea ma laila i kāu DNA e ʻike inā loaʻa iā ʻoe ka mutation i loko o ka gene UBA1 e hoʻoulu ai i ka VEXAS Syndrome.

He aha nā lāʻau lapaʻau no ka VEXAS Syndrome?

ʻO nā lāʻau lapaʻau nui no kēia maʻi i kēia manawa:

• Hoʻemi nā Corticosteroids i ka mumū.
• Hoʻolohi nā Immunosuppressants i kāu ʻōnaehana pale.
• Inā hōʻike kou iwi iwi i nā hōʻailona o ka hāʻule ʻana, pono paha ʻoe i kahi hoʻoili iwi iwi . Hiki i kahi hoʻoili iwi iwi ke hōʻemi i ke koʻikoʻi o kekahi mau maʻi autoimmune.

Hiki paha iā ʻoe ke kuhikuhi ʻia i kahi rheumatologist , he kauka i loea i nā maʻi hui a me nā maʻi autoimmune, hiki ke hāʻawi i ke alakaʻi pololei loa i ka mālama ʻana i kēia mau kūlana.

Hiki ke pale ʻia ka VEXAS Syndrome?

ʻO ka mea pōʻino, ʻaʻohe ala e pale aku ai i kēia maʻi i kēia manawa . Hiki ke hana ʻia ka mutation i loko o ka gene UBA1 me ka ʻike ʻole ʻia, me ke kumu ʻole i ʻike ʻia. No laila, ʻaʻole hiki iā mākou ke hoʻōki iā ia ma mua.

He aha ke kūlana o ke ola o kekahi me ka VEXAS Syndrome?

He mea koʻikoʻi loa kēia. ʻO ka ʻoiaʻiʻo, he ʻokoʻa nā kānaka a pau, a ʻokoʻa ke ʻano o ka hopena o ka VEXAS Syndrome i kou kino mai kēlā kanaka a kēia kanaka. Eia nō naʻe, e like me kā mākou i ʻōlelo ai ma mua, hiki i kēia ʻano ke hoʻoweliweli i ke ola inā ʻaʻole mālama ʻia. No laila, e kamaʻilio ākea me kāu kauka e pili ana i nā mea āu e manaʻo ai a me nā koho lapaʻau maikaʻi loa iā ʻoe. Hiki iā ia ke kōkua iā ʻoe e hoʻokele i kāu mau hōʻailona a kuhikuhi iā ʻoe i nā loea olakino noʻonoʻo a me nā lawelawe kākoʻo ʻē aʻe inā pono.

Āhea wau e ʻike ai i ke kauka?

Inā ʻike ʻoe i kekahi mau hōʻailona o ka VEXAS Syndrome, e like me ke kuni, ka ʻili ʻulaʻula, a i ʻole ka paʻakikī o ka hanu ʻana, e ʻike pono i ke kauka. Ma muli o ka hoʻoulu ʻana o ka VEXAS Syndrome i nā ʻano hōʻailona like ʻole e like me ka pili ʻole, hiki i kekahi manawa ke paʻakikī ke ʻike mua. Eia nō naʻe, e hoʻolohe i kou kino, a inā he mau hōʻailona kāu i maopopo ʻole ai a i ʻole pili ʻole i kāu mau kūlana olakino ʻē aʻe, e kamaʻilio me ke kauka e pili ana iā lākou.

Inā ua ʻike mua ʻia ʻoe me ka VEXAS Syndrome, a manaʻo ʻoe e hoʻomohala nei ʻoe i nā hōʻailona hou a i ʻole e ʻoi aku ka ʻino o kāu mau hōʻailona, ​​e ʻike koke i ke kauka.

Pilikia! Inā loaʻa iā ʻoe ke kuni ma mua o 103°F (39.5°C) no nā hola ʻelua a ʻoi aku ma mua o ka mālama ʻia ʻana ma ka home, e hele i kahi lumi pilikia. Inā pilikia ʻoe i ka hanu ʻana, e hele koke i ka haukapila a kāhea aku paha i 911 (a i ʻole kāu helu pilikia kūloko).

He aha nā nīnau e pono iaʻu e nīnau aku i kaʻu kauka?

Ke hele ʻoe e ʻike i ke kauka, e mākaukau e nīnau i nā nīnau e like me kēia:

• Loaʻa iaʻu ka VEXAS Syndrome, a i ʻole he maʻi ʻē aʻe paha?
• Pono anei iaʻu e hana i nā hoʻokolohua genetic?
• He aha ke ʻano o ka lāʻau lapaʻau e pono ai iaʻu?
• He aha nā hōʻailona a i ʻole nā ​​​​loli e pono iaʻu e makaʻala?
• He mea hoʻoweliweli anei ka VEXAS Syndrome i koʻu ola?

E lilo kēia mau nīnau i wahi hoʻomaka maikaʻi no ʻoe e hoʻomaka i kahi kūkākūkā me kāu kauka.

ʻO ka mea hope loa, e hoʻomanaʻo i kēia (Leka Lawe-Home)

He maʻi autoimmune kakaikahi ka VEXAS Syndrome i hoʻokumu ʻia e kahi hoʻololi gene kikoʻī. Hiki iā ia ke hoʻoulu i ka mumū ma loko o kou kino a hiki ke hoʻoweliweli i ke ola inā waiho ʻole ʻia. Akā, mai hoʻokuʻu i ka manaʻolana . Hiki i kāu kauka ke kōkua iā ʻoe e loaʻa i ka lāʻau kūpono e kāohi i kāu mau hōʻailona.

Inā ʻike ʻoe i kekahi mau hōʻailona o ka VEXAS Syndrome, e ʻike i ke kauka. E hilinaʻi iā ʻoe iho, e hoʻolohe i kou kino. Mai hoʻowahāwahā i nā hōʻailona e like me ke kuni, ka ʻulaʻula, a me ka paʻakikī o ka hanu ʻana. Me ka ʻike mua ʻana a me ka mālama kūpono, hiki iā ʻoe ke ola i kahi ola olakino.

ʻO ka maʻi VEXAS, nā maʻi Autoimmune, ka mumū, nā hoʻololi genetic, ka gene UBA1, ka iwi iwi