Ke loaʻa pinepine nei hoʻi iā ʻoe ke kuni me ke kumu ʻole? Loaʻa iā ʻoe nā pilikia ʻē aʻe me lākou? Hiki paha i kēia ke 'Familial Mediterranean Fever (FMF)'!

Loaʻa koke iā ʻoe ke kuni me ka ʻole o ka ʻōlelo aʻo? Loaʻa iā ʻoe ka ʻeha o ka ʻōpū, ka manaʻo o ka paʻa ʻana i kou umauma, a i ʻole ka ʻeha o nā hono? I kekahi manawa e nalowale ana kēia mau hōʻailona ma hope o kekahi mau lā, a laila e ʻike hou ʻia ma hope o kekahi manawa? He mea maʻamau ka makaʻu nui i ka wā e hana ʻia ai kēia. I kēia lā e kamaʻilio mākou e pili ana i kahi maʻi e hoʻoulu ai i kēia mau hōʻailona, ​​​​he mea ʻē iki ia, akā he mea nui loa ia e ʻike.

He aha ka 'Familial Mediterranean Fever (FMF)'?

I ka ʻōlelo maʻalahi, ʻo ka Familial Mediterranean Fever, a i ʻole FMF no ka pōkole, he maʻi ia no ke ola holoʻokoʻa e loaʻa mai ana mai ko kākou mau genes. ʻO ka mea e hana ʻia ana i loko o kēia, ʻo ia hoʻi, me ke kumu ʻole, loaʻa koke iā kākou ke kuni, a i ka manawa like, aia ka mumū ma nā wahi like ʻole o ke kino. I ka pololei, he mea kēia e hana ʻia ma muli o kahi pilikia liʻiliʻi i loko o kā kākou ʻōnaehana pale ponoʻī. ʻO ia ke kumu i kapa ʻia ai he maʻi autoinflammatory .

Hoʻopilikia nui ia i ka poʻe e noho ana ma nā ʻāina Mediterranean. Hoʻoulu ia i ka mumū o nā membrane lahilahi i kapa ʻia ʻo 'nā membrane serous' (ʻo kēia mau mea e uhi ana i nā wahi e like me ka lua umauma a me ka lua ʻōpū), i kapa ʻia ʻo 'serositis'. Hiki iā ia ke hoʻoulu i ka mumū o nā 'membranes synovial' e uhi ana i ko mākou mau hono, i kapa ʻia ʻo 'synovitis'. ʻO ka maʻamau, mau nā hōʻailona no kekahi mau lā a laila emi iho.

He aha nā hōʻailona o FMF?

Hoʻomaka pinepine nā hōʻailona FMF i ka wā kamaliʻi, ma mua o 20 mau makahiki. Hiki ke koʻikoʻi kēia mau hōʻailona i ka wā mua, akā hiki ke hoʻomaikaʻi mālie i kou wā e ʻelemakule ai. Ke loaʻa iā ʻoe ka maʻi, ʻaʻole paha ʻoe e loaʻa i kekahi mau hōʻailona no nā pule, nā mahina, a i ʻole nā ​​​​makahiki. Eia nō naʻe , hiki i ka ʻike mua a me ka mālama ʻana ke kōkua i ka hōʻemi ʻana i nā hōʻailona a pale i nā pilikia lōʻihi e like me ka amyloidosis.

I kēia manawa, e nānā kākou i kēia mau hōʻailona:

• He kuni kiʻekiʻe a hiki i ka 40.6 degere Celsius (105 Fahrenheit), i kekahi manawa hele pū me ke anu a me ka haʻalulu.
• Hiki ke hele mai me ka ʻeha poʻo a me ka ʻāʻī ʻoʻoleʻa.
• ʻEha ka ʻōpū a me ka pehu (bloating).
• Paʻa nā ʻiʻo o ka ʻōpū a hiki ke paʻa ka ʻōpū .
• ʻEha ka umauma , ka mea e hoʻonui ai i ka wā e hanu ai.
• ʻEha a me ka pehu ʻana o nā hono (pinepine i hoʻokahi hui).
• ʻO ka pehu a me ka ʻeha i loko o nā testicles i nā kāne.
• ʻEha nā ʻiʻo , ʻoi aku hoʻi ma ke kino haʻahaʻa.
• Hiki ke ulu aʻe kahi ʻeha ʻili ʻulaʻula a piʻi aʻe ma nā wāwae .

Hiki ke ʻike koke ʻia kēia mau hōʻailona i loko o kekahi mau hola. Eia naʻe, ʻaʻole e ʻike nā kānaka a pau i kēia mau hōʻailona a pau.ʻO ke kuni ka hōʻailona maʻamau. I ka wā kamaliʻi, ʻo ke kuni wale nō paha ka hōʻailona. ʻO ka maʻamau, mau ka maʻi no kekahi mau lā.

Nā Hōʻailona Prodromal o FMF

Hoʻomaka kekahi poʻe e ʻike i nā hōʻailona liʻiliʻi i kekahi mau lā ma mua o ka hoʻomaka ʻana o kēia kuni. E like me ka manaʻo o kekahi poʻe ma mua o ka "migraine" a i ʻole ka manaʻo o nā wahine ma mua o ko lākou "menstruation". Kāhea mākou i kēia mau " hōʻailona prodromal". Ke manaʻo ʻoe i kēia mau mea, loaʻa iā ʻoe kahi hōʻailona "ʻĀ... i kēia manawa ke hiki mai nei ka pilikia".

He aha kēia mau hōʻailona hoʻohālikelike?

• Hopohopo, hoʻomaha ʻole
• Hoʻonāukiuki
• ʻEha poʻo
• Ka luaʻi
• ʻEha kino
• Malaise ( nele i ka hoihoi, make ʻole o ke ola )

No ke aha lā e hana ʻia ai kēia 'FMF'?

ʻO kēia ma muli o nā loli i loko o ke gene `MEFV` i loko o ko kākou kino. Kōkua kēia gene `MEFV` i ka hana ʻana i kahi protein koʻikoʻi loa, ʻo ia hoʻi kahi protein i kapa ʻia ʻo `pyrin`. ʻO kā mākou ʻōnaehana pale kūlohelohe - ʻo ia hoʻi, ka ʻāpana o ka ʻōnaehana pale e hana kūʻē i nā hoʻoweliweli e like me nā maʻi koke - hilinaʻi i kēia protein `pyrin`. No laila, i ka wā e hiki ʻole ai i ka gene `MEFV` ke hana pono i ka `pyrin`, ʻaʻole hiki i kēlā protein ke hana pono i loko o ka ʻōnaehana pale. ʻO ia ka manawa e kū mai ai kēia pilikia.

Ma kahi o 30 mau hoʻololi genetic (`variations`) i ʻike ʻia e hoʻoulu ai i ka `FMF`. ʻO kēia mau mea, ʻo ka mea maʻamau ʻo `V726A`, `M680I`, `E148Q`, `M694V` a me `M694I`. ʻO kēia mau mea ka mea maʻamau i nā heluna kanaka o ke kumu Mediterranean a me ka Hikina Waena. Eia kekahi laʻana:

• Nā ʻArabia
• Nā ʻAmenia
• Helene
• Nā Iranians
• Nā Italia
• poʻe Iudaio
• Nā Kurds
• Nā Tureke

Nā mea hoʻonāukiuki no FMF

ʻOiai ke ʻano pinepine nei ka FMF e hiki koke mai me ke kumu ʻole, no kekahi poʻe ua hoʻoulu ʻia e kekahi mau mea. No kekahi poʻe, hiki ke hoʻoulu ʻia ke kūlana e ke kaumaha kino a noʻonoʻo paha. Wahi a ka poʻe hiki ke hoʻoulu ʻia ka FMF e nā mea e like me:

• Ka hōʻike ʻana i ke anu
• Hoʻoikaika kino a luhi nui paha
• He maʻi i hala koke aku nei
• He ʻeha a ʻoki kino paha i hala koke aku nei
• Ke kaumaha nui (e like me kahi pilikia ola nui)
• ʻO ka manawa e loaʻa ai i nā wahine ko lākou wā mahina a i ʻole ka ovulation

Nā pilikia kūpono o FMF

ʻOiai ke emi nei ke kuni o FMF ma hope o kekahi mau lā, hiki i ka mumū mau i loko o ke kino ke hoʻopilikia i ko mākou kino i ka hala ʻana o ka manawa. ʻO ia ke kumu e ʻōlelo ai nā kauka i ka mālama mau ʻana no kēia. Ke loaʻa kahi kaʻina hana mumū mau e like me kēia, ua kau ʻia ko mākou ʻōnaehana pale ma lalo o ke kaomi nui, a hiki ke loaʻa nā maʻi ʻē aʻe.

ʻO ka pilikia koʻikoʻi loa o kēia, ʻo ia ke ʻano i kapa ʻia ʻo 'secondary amyloidosis' . Hiki i kēia ke alakaʻi i ka hāʻule ʻana o nā puʻupaʻa. No laila he mea nui loa ia e makaʻala i kēia.

Hiki i nā pilikia ʻē aʻe ke komo pū me:

• ʻO ka splenomegaly (splenomegaly)
• Ka hānau ʻole ʻana
• Nā maʻi autoimmune ʻē aʻe ('Maʻi autoimmune lua')

Pehea e ʻike ʻia ai ka FMF?

Pehea e ʻike ai ke kauka i ka FMF? Hana lākou i nā mea e like me:

• E hoʻolohe pono lākou i kāu mau hōʻailona, ​​​​​​i ka pinepine o ka loaʻa ʻana, a me ka lōʻihi o ko lākou loaʻa ʻana .
• E nīnau lākou inā ua loaʻa i kekahi o kou ʻohana kēia maʻi a pehea ke ʻano o kou hoʻoilina genetic .
• Ke maʻi ʻoe, e nānā lākou iā ʻoe e ʻike inā he mumū a ʻeha paha i loko o kou kino .
• I ka wā maʻi , hana ʻia nā hoʻāʻo koko e ʻike inā he mau hōʻailona o ka mumū .
• Paipai ʻia ka hoʻāʻo ʻana i ka genetic e nānā i nā mutations i loko o ka gene MEFV, ka mea i hoʻokumu pinepine i ka FMF.
• E nānā pū lākou i ke ʻano o kāu pane ʻana i nā lāʻau lapaʻau no FMF.

Lapaʻau no ka `FMF`

ʻO ka lāʻau lapaʻau nui no FMF ʻo ia ka lāʻau anti-inflammatory colchicine. Hana kēia ma ka hoʻemi ʻana i ka hana o kahi ʻano o ke koko keʻokeʻo i kapa ʻia ʻo neutrophils, e pili ana i ka mumū. Hiki i ka Colchicine ke kōkua i ka pale ʻana a i ʻole ka hoʻēmi ʻana i ke alapine o nā lapalapa FMF, a hoʻemi i ka pilikia o nā pilikia. Ua ho'ōla ʻia ma mua o 90% o ka poʻe me FMF . Pono ka hapa nui o ka poʻe e lawe i kēia lāʻau i kēlā me kēia lā no ke koena o ko lākou ola.

Eia nō naʻe, ʻaʻole i loaʻa i kahi helu liʻiliʻi loa o ka poʻe nā hopena i makemake ʻia mai ka colchicine, a i ʻole ʻaʻole hiki iā lākou ke lawe i kēia lāʻau lapaʻau ma muli o nā kumu olakino ʻē aʻe. No kēlā poʻe, kuhikuhi nā kauka i nā ʻano lāʻau lapaʻau ʻē aʻe. ʻO kekahi o lākou:

• ʻAnakinra (ʻAnakinra)
• ʻO Rilonacept
• ʻO Canakinumab

He aha ka mea e hana inā loaʻa iā ʻoe ka 'FMF'?

ʻOiai ʻaʻohe lāʻau lapaʻau no FMF, hiki ke mālama maikaʻi ʻia me ka lāʻau lapaʻau . No nā poʻe he nui, aneane nalowale loa ke kuni a me nā ʻeha o ke kino ke hoʻomaka lākou e lawe i ka lāʻau lapaʻau. No kekahi poʻe, ua emi ka pinepine a me ke alapine o ka flare-ups. Eia nō naʻe, i mea e hana pono ai kēia mau lāʻau lapaʻau, pono e lawe mau ʻia, ʻoiai inā ʻaʻole lākou he mau hōʻailona.ʻAʻole e hoʻōki koke ka lawe ʻana i kēia lāʻau i kou wā maʻi. Pale wale kēia lāʻau i ka maʻi mai ka hiki ʻana mai.

Hiki ke pale ʻia ka FMF?

ʻAʻole ʻike ka nui o ka poʻe he ʻano gene ko lākou e hoʻoulu ai i ka FMF. No ka loaʻa ʻana o ka FMF, pono ʻoe e kiʻi i ʻelua kope o ka ʻano gene mai kou makuahine a me kou makuakāne. Inā hoʻokahi kope wale nō kāu, ʻaʻole ʻoe e loaʻa i nā hōʻailona o FMF. Akā hiki iā ʻoe ke hoʻoili i kēlā ʻano gene i kāu mau keiki. Inā loaʻa i kāu hoa kēlā ʻano gene, loaʻa paha i kāu mau keiki ka FMF. Hiki i ka hoʻāʻo ʻana i ka genetic ke kōkua iā ʻoe e ʻike inā he mea lawe ʻoe i ka ʻano gene.

Ua loaʻa nā hōʻike e pili ana i kēlā mau kuni pinepine mai ka wā kahiko mai. Malia paha ua like ka wā kahiko o kēia maʻi i kapa ʻia ʻo 'FMF'. Manaʻo kekahi poʻe noiʻi ua kū mai paha kēia ʻano ma ke ʻano he ala e kōkua ai i ke kaua ʻana i nā maʻi nui e like me ka 'tuberculosis' i laha nui i kēlā manawa. ʻO ka ʻoiaʻiʻo, aia kekahi mau pono o ka loaʻa ʻana o ka mumū i loko o ke kino, akā i ka wā e nui loa ai ia e lilo ai i mea pilikia. ʻO ka mea pōmaikaʻi, aia nā lāʻau lapaʻau maikaʻi i kēia manawa e kāohi i kēia ʻano. No laila, inā loaʻa iā ʻoe ka 'FMF', hiki iā ʻoe ke ola i ke ola maʻamau.

ʻO ka mea nui loa āu e hoʻomanaʻo ai! (Leka Lawe-Home)

ʻAe, i kēia manawa ua ʻoi aku kou maopopo i ka mea a mākou i kamaʻilio ai i kēia lā, ʻo 'FMF'. ʻO ka mea nui loa, inā loaʻa iā ʻoe a i ʻole kekahi o kou ʻohana ke kuni me ke kumu ʻole, ʻeha ʻōpū, ʻeha hui, a pēlā aku, mai hoʻokuʻu wale iā ia he anu wale nō, akā e ʻike i ke kauka e ʻike pono i ka mea e hana nei. Inā ʻike ʻoe i ka maʻi a hoʻomaka i ka mālama ʻana i ka wā mua, hiki iā ʻoe ke pale i nā pilikia he nui a ola i ke ola maʻamau. E hoʻomanaʻo, he mea nui loa hoʻi e hoʻohana i ka lāʻau lapaʻau a kāu kauka i kuhikuhi ai, e like me ke kuhikuhi ʻana. Mai poina e kamaʻilio me kāu kauka e pili ana i nā nīnau a hopohopo paha āu e loaʻa ai.

ʻO FMF, ka maʻi kuni ʻohana, nā kuni pinepine, ʻeha ʻōpū, ʻeha hui, nā maʻi hoʻoilina, colchicine, amyloidosis