Ke loaʻa mau nei ke kuni i kāu keiki liʻiliʻi? Ua hoʻomaikaʻi anei ʻo ia ma hope o ke kuni no kekahi mau lā, a laila loaʻa hou ke kuni i kekahi mau lā ma hope? Ke noʻonoʻo nei paha ʻoe no ke aha e hana ai kēia me ka ʻole o kahi maʻi virus a bacteria paha. Hiki paha kēia ma muli o kahi maʻi laha ʻole i kapa ʻia ʻo SAIDs (Systemic Autoinflammatory Diseases) , kahi i lohe ʻole ʻia e ka poʻe he nui. Mai hopohopo, e kamaʻilio kākou e pili ana i ia mea ma nā ʻōlelo maʻalahi a hoʻomaopopo i nā mea āpau.
He aha nā SAID? E hoʻomaopopo maʻalahi kākou.
I ka ʻōlelo maʻalahi, ʻo nā SAID kahi hui o nā maʻi e hoʻoulu ai i nā kuni pinepine a hoʻi hou me ka ʻole o ka maʻi (e like me nā virus a bacteria paha). ʻO ke kumu o kēia he hana hewa i loko o ka ʻōnaehana pale kūlohelohe o kā mākou keiki. Ua kapa ʻia kēia ma mua ʻo 'Periodic Fever Syndromes'.
I kēia manawa ke noʻonoʻo nei paha ʻoe, he maʻi 'autoimmune' anei kēia? Lohe pinepine mākou e pili ana i nā maʻi autoimmune e like me ka Rheumatoid Arthritis a i ʻole Lupus. ʻAʻole, he ʻokoʻa kēia.
E noʻonoʻo he pūʻali koa pale ko kākou i loko o ko kākou kino, ʻo ia hoʻi ko kākou ʻōnaehana pale. ʻElua ʻāpana nui o kēia pūʻali koa:
1. ʻŌnaehana Palekana Pilikino: ʻO kēia ka pūʻali koa i hānau ʻia ai kākou, a ʻo ia ka mea mua e hoʻouka i kekahi huakino.
2. ʻŌnaehana Palekana Hoʻololi: He pūʻali koa kēia i hoʻomaʻamaʻa kūikawā ʻia e hakakā i nā ʻano germs like ʻole i ko kākou ulu ʻana.
I loko o nā SAID, ʻo ka pilikia me ka ʻōnaehana pale kūlohelohe. Hoʻonāukiuki kēlā ʻōnaehana pale a hoʻomaka i kahi hakakā i loko o ke kino me ka ʻole o ka ʻenemi. ʻO ka hopena o kēia hakakā, he mumū i loko o ke kino a me ke kuni.
Akā i nā maʻi autoimmune a mākou i kamaʻilio ai, ʻo ka pilikia me ka pūʻali koa adaptive i hoʻomaʻamaʻa ʻia ma hope. ʻAʻole hiki i kēlā pūʻali koa ke ʻike i nā cell maikaʻi i loko o kona kino ponoʻī a hoʻomaka e hoʻouka iā lākou.
ʻOi aku ka kakaikahi o nā SAID ma mua o nā maʻi autoimmune. Hoʻokumu pinepine ʻia lākou e nā kumu genetic , ʻo ia hoʻi, ua hoʻoilina ʻia. Hoʻomaka pinepine kēia mau hōʻailona i ka wā ʻōpiopio loa o ke keiki, e like me ka wā kamaliʻi a i ʻole ka wā kamaliʻi. Loaʻa koke i ke keiki kahi hanana a i ʻole ka hoʻouka ʻana o ka maʻi me ke kuni a me nā hōʻailona ʻē aʻe, kahi e hoʻopau pinepine ʻia i loko o kekahi mau lā. Hiki i ke keiki ke olakino loa ke ʻole ka maʻi. ʻOiai ʻaʻohe lāʻau lapaʻau, aia nā lāʻau lapaʻau kūpono e hiki ke kaohi i nā hōʻailona.
He aha nā ʻano nui o nā SAID?
Ua ʻike nā kānaka noiʻi ma kahi o 60 mau ʻano SAID a hiki i kēia manawa, a ke ʻike mau ʻia nei nā ʻano hou. Eia kekahi o nā ʻano maʻamau.
| Inoa o ka maʻi (ʻAno o SAID) | He hoʻolauna pōkole |
|---|---|
| ʻO ka Fiva Kaiwaenahonua ʻOhana (FMF) | ʻO kēia ke ʻano maʻamau o ka SAID i ʻike ʻia ma ke ʻano genetically, e hoʻoulu ai i ka mumū ʻeha ma ka ʻōpū, ka umauma, a me nā hono. |
| Ka Fiva Kau, Aphthous-Stomatitis, Pharyngitis, Adenitis (PFAPA) | Hoʻomaka ia i nā keiki ʻōpio, ʻoi aku hoʻi ma mua o ka piha ʻana o 4 makahiki. ʻO nā hōʻailona e like me ka ʻeha o ka puʻu, nā ʻeha waha, a me nā ʻōpū pehu ma ka ʻāʻī e hele pū me ke kuni. Nalo pinepine ia ma hope o 10 makahiki. |
| ʻO ka Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS) | Hiki i kēia ke hoʻomaka i ka wā kamaliʻi a i ʻole i ka wā ʻōpio. |
| Ka hemahema o ka Mevalonate Kinase (MKD) | Hoʻomaka pinepine kēia ma mua o ka piha ʻana o hoʻokahi makahiki o ke keiki. |
| Nā maʻi Autoinflammatory e pili ana iā NLRP3 (CAPS) | He hui pū ʻana kēia o ʻekolu mau maʻi like ʻole. |
| ʻO ka maʻi o ke kanaka makua (AOSD) | E like me ka inoa e hōʻike nei, he maʻi kēia e hoʻomaka ana i ka wā makua. |
| ʻO ka maʻi Blau | ʻIke pinepine ʻia kēia i nā keiki ma lalo o 4 mau makahiki. Hoʻopilikia nui ia i ka ʻili, nā maka, a me nā hono. |
He aha nā hōʻailona maʻamau o kēia mau maʻi?
ʻO ka hōʻailona nui a maʻamau o nā SAIDs ka hoʻi hou ʻana o ke kuni . Eia nō naʻe, ma waho aʻe o ke kuni, hiki ke ʻike ʻia nā hōʻailona ʻē aʻe ma muli o ke ʻano o ka maʻi.
ʻO ka mea nui, ʻo ia ka ʻike wale ʻia ʻana o kēia mau hōʻailona i ka wā o ka maʻi. Ke pau kēlā wā, hiki i ke keiki ke olakino piha me ka ʻole o nā hōʻailona.
| Inoa o ka maʻi (ʻAno o SAID) | Nā hōʻailona e makaʻala ai |
|---|---|
| FMF | ʻEha nui ma ka ʻōpū, ka umauma, a me nā hono. Nā ʻeha ʻili ma nā wāwae lalo a i ʻole nā kuʻekuʻe wāwae. |
| PFAPA | ʻEha ka ʻāʻī, nā ʻōpū waha, nā ʻōpuʻu lymph pehu ma ka ʻāʻī. |
| Nā Pahele | Anu a haʻalulu ke kino. He ʻeha ma nā ʻiʻo o ke kino, ʻoi aku hoʻi ma ke kino a me nā lima. Hiki ke ʻike ʻia nā kiko ʻulaʻula ʻeha ma ke kino holoʻokoʻa. |
| MKD | Ke anu nei ke kino a me ka haʻalulu, me ke poʻo ʻeha, ka ʻōpū ʻeha, ka nalowale o ka makemake, a me nā hōʻailona like me ke anu maʻamau. |
| Nā Maʻi NLRP3 | ʻO ka ʻili e ʻānai ana, ka ʻeha poʻo, ka luhi, ka ʻeha o nā hono, a me nā maʻi maka (conjunctivitis). |
| ʻO ka maʻi Blau | Nā ʻeha ʻili ma nā lima, nā wāwae, a i ʻole ke kino o ke keiki. ʻEha nā hono a me ka ʻeha o nā maka. |
No ke aha e loaʻa ai kēia mau maʻi? He aha nā kumu?
E like me kā mākou i ʻōlelo ai ma mua, he nui nā SAID he mau maʻi genetic . ʻO ke ʻano kēia ua hoʻokumu ʻia kēia mau maʻi e kahi mutation (variant) i loko o kahi gene kikoʻī.
| Inoa o ka maʻi (ʻAno o SAID) | Gene pili |
|---|---|
| FMF | ʻO ka gene MEFV |
| PFAPA | ʻAʻole i loaʻa ke kumu kūpono. |
| Nā Pahele | ʻO ka gene TNFRSF1A |
| MKD | ʻO ke kine MVK |
| Nā Maʻi NLRP3 | ʻO ka gene NLRP3 |
| AOSD | ʻAʻole i loaʻa ke kumu kūpono. |
| ʻO ka maʻi Blau | ʻO ka gene NOD2 |
He aha ka mea e hiki ke hana inā ʻaʻole loaʻa ka lāʻau lapaʻau kūpono?
He mea nui loa kēia. He mea nui e kāohi i kēia kūlana ma ka lawe ʻana i ka lāʻau kūpono. No ka mea, inā he mumū mau i loko o ke kino e like me kēia, hiki iā ia ke alakaʻi i kahi kūlana i kapa ʻia ʻo Amyloidosis . I ka ʻōlelo maʻalahi, hiki ke waiho ʻia kekahi ʻano protein i loko o ko kākou mau puʻupaʻa, e hōʻino mau ana i nā puʻupaʻa. No laila, he mea nui loa e ʻike koke i ka maʻi a mālama koke iā ia.
Pehea e ʻike ai ke kauka i kēia maʻi?
I ka ʻoiaʻiʻo, hiki ke paʻakikī iki ka ʻike ʻana i nā SAID no ka mea hiki ke like nā hōʻailona me nā maʻi ʻē aʻe, e like me ka lupus.
No laila, ʻo ka mea nui loa, ʻo ia ke ʻike ʻana i ke kauka loea i kēia ʻano maʻi. ʻO ke kauka loea i nā maʻi e pili ana i nā hono a me ka ʻōnaehana pale ʻana ua kapa ʻia ʻo ia he Rheumatologist .
E noʻonoʻo kāu kauka i kekahi mau kumu e hana ai i kahi hōʻailona. E nīnau paha ʻo ia e pili ana i nā hōʻailona o kāu keiki a inā he moʻolelo ʻohana o ke ʻano. Manaʻo paha kāu kauka he mau SAID, ʻoiai inā:
- Inā loaʻa i ke keiki ke kuni pinepine.
- Inā ua loaʻa i kekahi o ka ʻohana nā maʻi e like me kēia.
- ʻOiai he mea maʻamau kēia mau maʻi i waena o kekahi mau lāhui kanaka, ke noʻonoʻo ʻia nei hoʻi kēlā.
He aha nā hoʻokolohua e hana ʻia?
Hiki i ke kauka ke paipai i kekahi mau hoʻokolohua e hōʻoia i ka maʻi.
- Nā hoʻāʻo koko (Nā hoʻāʻo Lab): Hiki i nā hoʻāʻo e like me ka C-reactive protein (CRP) a me CBC (Full Blood Count) ke nānā i ka mumū i loko o ke kino. Piʻi kēia mau pae i ka wā maʻi a hoʻi i nā pae maʻamau ke olakino ke kino.
- Hoʻāʻo mimi: Nānā i nā pae protein kiʻekiʻe i loko o ka mimi. I kekahi mau maʻi, e like me MKD, hiki i nā hoʻāʻo mimi ke ʻike i nā waikawa kikoʻī.
- Hoʻāʻo hoʻoilina: Hiki i kēia hoʻāʻo ke ʻike i nā hoʻololi hoʻoilina. Eia nō naʻe, i kekahi manawa, loaʻa paha i ke keiki ka SAID akā ʻaʻole paha e ʻike ka hoʻāʻo hoʻoilina. No laila, inā maikaʻi ʻole ka hopena o ka hoʻāʻo, ʻaʻole hiki ke ʻōlelo ʻia he 100% ʻaʻole i loaʻa ka maʻi.
He aha nā lāʻau lapaʻau no kēia?
ʻO ke ʻano a me ke koʻikoʻi o ka maʻi ke ʻano o ka mālama ʻana no nā SAID. ʻOiai ʻaʻole hiki ke ho'ōla loa ʻia kēia mau maʻi, hiki i nā lāʻau lapaʻau ke kāohi nui i nā hōʻailona.
- FMF: Hoʻohana ʻia kahi lāʻau i kapa ʻia ʻo Colchicine no kēia. Hoʻemi ia i ka mumū i loko o ke kino. Inā ʻaʻole hana kēlā lāʻau no ke keiki, hiki iā lākou ke huli i nā lāʻau e like me Canakinumab .
- PFAPA: Hiki ke hāʻawi ʻia nā steroid e like me Prednisone no kahi manawa pōkole e hōʻemi koke i ka lōʻihi o ka maʻi.
- Nā Pahele: CanakinumabHe maikaʻi loa ka lāʻau. Hoʻohana pū ʻia nā lāʻau anti-inflammatory e like me glucocorticoids .
- MKD: He kūpono ka lāʻau lapaʻau ʻo Canakinumab no kēia. Hiki i nā NSAID (painkillers) a i ʻole nā steroids ke kōkua i ka wā maʻi.
- Nā Maʻi NLRP3: Hoʻohana pono ʻia nā lāʻau lapaʻau hoʻololi i ka pale ʻana e like me Canakinumab , Rilonacept , a me Anakinra no kēia.
- ʻO Blau Syndrome: Ma muli o nā hōʻailona, hiki ke hoʻohana ʻia nā immunosuppressants, nā mea hoʻopaneʻe TNF, a me nā lāʻau maka.
E ho'ōla loa ʻia anei kēia maʻi?
No ke ola holoʻokoʻa kekahi mau SAID. Eia nō naʻe, hiki i kekahi, e like me PFAPA, ke nalo wale aku i ka wā e ulu ai ke keiki. ʻOiai he mau maʻi ia no ke ola holoʻokoʻa, hiki ke emi ka pinepine o ka maʻi a me ke koʻikoʻi o nā hōʻailona i ka hala ʻana o ka manawa. E hāʻawi kāu kauka iā ʻoe i nā kikoʻī kikoʻī e pili ana i ke kūlana o kāu keiki.
Hiki ke paʻakikī ka mālama ʻana i kahi keiki me kēia maʻi. Akā me ke kōkua o nā loea kūpono, hiki iā ʻoe ke hoʻokele i nā hōʻailona o kāu keiki a hāʻawi iā lākou i ka wā kamaliʻi maikaʻi loa.
Leka Lawe-Home
- Inā loaʻa pinepine ke kuni o kāu keiki me ke kumu ʻole, mai hoʻowahāwahā wale iā lākou a e ʻike koke i ke kauka.
- ʻO nā SAID kahi hui o nā maʻi laha ʻole i hoʻokumu ʻia e kahi pilikia ma ka ʻāpana kūlohelohe o ka ʻōnaehana pale.
- ʻAʻole kēia he mau maʻi lele; hoʻokumu pinepine ʻia lākou e nā kumu genetic.
- He mea nui loa ka ʻike ʻana i kahi rheumatologist (he kauka loea i nā hono a me ka ʻōnaehana pale) e ʻike pololei i ka maʻi.
- ʻOiai ʻaʻole hiki ke ho'ōla loa ʻia kēia mau mea, hiki i nā lāʻau lapaʻau ke kōkua i ka kaohi ʻana i nā hōʻailona a ʻae iā ʻoe e ola i kahi ola maʻamau.
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