Ua ʻike anei ʻoe i kēia maʻi puʻupaʻa laha ʻole? (Atypical Hemolytic Uremic Syndrome - aHUS)

E noʻonoʻo i nā ʻōpū koko e hana ana i loko o nā kīʻaha koko liʻiliʻi i loko o kou kino. ʻOi loa aku i loko o nā kīʻaha koko i loko o kou mau puʻupaʻa. Ke ālai kēia mau ʻōpū koko i ke kahe o ke koko i kou mau puʻupaʻa, hiki ke loaʻa nā pilikia koʻikoʻi e like me ka hāʻule ʻana o ka puʻupaʻa. ʻO ia ka mea e hana ʻia ma ka Atypical Hemolytic Uremic Syndrome, a i ʻole aHUS. ʻOiai ʻaʻohe lāʻau lapaʻau no kēia ʻano, hiki ke mālama maikaʻi ʻia me ka lāʻau kūpono .

He aha ka ʻokoʻa ma waena o ka HUS "Maʻamau" a me ka "Atypical"?

ʻO kēia kūlana, i kapa ʻia ʻo aHUS, he ʻokoʻa iki ia mai ka "HUS maʻamau" a mākou e ʻike mau ai. ʻO ke kumu nui o ka HUS maʻamau kekahi mau ʻano bacteria e hoʻoulu ai i ka maʻi diarrhea, no ka laʻana, nā maʻi bacteria e like me `(E. coli)` a me `(Shigella)`. Eia nō naʻe, ʻoi aku ka paʻakikī o ke kumu o aHUS.

Hiki ke loaʻa kēia maʻi, i kapa ʻia ʻo aHUS, i kēlā me kēia makahiki. I nā keiki ʻōpio, hoʻopilikia like ia i nā keikikāne a me nā kaikamahine. Eia nō naʻe, i ko lākou ʻelemakule ʻana, ʻoi aku ka nui o ka loaʻa ʻana o kēia maʻi i nā wahine. ʻO kēia no ka mea hiki i ka hāpai ʻana ke lilo i mea hoʻoulu no ka ʻike ʻia ʻana o ka maʻi. He maʻi kakaikahi loa ia. ʻOiai ʻaʻole maopopo ka nui o ka poʻe i loaʻa i kēia maʻi, ua hōʻike kekahi mau noiʻi he ʻelua wale nō i loko o ka miliona o ka poʻe ma nā ʻāina e like me ʻAmelika Hui Pū ʻIa i loaʻa i kēia maʻi.

He aha ke kumu o ka aHUS?

ʻO ka hapa nui o nā hihia aHUS i hoʻokumu ʻia e kahi loli i loko o nā genes, i kapa ʻia he mutation gene . Eia naʻe, ʻaʻole nā ​​​​mea āpau i loaʻa ka mutation gene e loaʻa i ka maʻi. I mea e ʻike ʻia ai nā hōʻailona, ​​​​pono kahi mea hoʻoulu.

I ka ʻōlelo maʻalahi, hiki i ke kanaka i loaʻa i kēia maʻi ke loaʻa i ka maʻi inā hana ʻia kekahi o kēia mau mea:

• Hāpai keiki
• Kekahi mau kūlana maʻi
• Ka maʻi ʻaʻai
• Kekahi mau lāʻau lapaʻau
• He hoʻoili kino
• Nā maʻi mau ʻē aʻe, no ka laʻana "systemic sclerosis" a i ʻole "malignant hypertension"

Akā i kekahi manawa, ma kahi o ka hapalua o nā maʻi aHUS, ʻaʻohe loli genetic i loaʻa i nā kauka. Kāhea mākou i kēia mau hihia he "idiopathic." ʻO ia hoʻi, ʻaʻole hiki ke loaʻa ke kumu. Manaʻo nā kānaka noiʻi ua hoʻokumu ʻia ke kūlana e kahi loli genetic i ʻike ʻole ʻia.

He aha nā hōʻailona o ka aHUS?

ʻIke pinepine ʻia nā hōʻailona o ka aHUS ma hope o kahi mea hoʻonāukiuki. Hiki i kēia mau lapalapa ke ʻano akahai a koʻikoʻi paha. I nā hihia akahai, ʻaʻole paha e hoʻopilikia nui ʻia nā puʻupaʻa. I nā hihia koʻikoʻi, hiki iā lākou ke alakaʻi i ka hāʻule ʻana o nā puʻupaʻa.

I ka wā mua o ka maʻi, hiki iā ʻoe ke ʻike i nā hōʻailona e like me kēia:

• Ka nāwaliwali loa a me ke ola ʻole
• Manaʻo maʻi ʻole

He maʻi holomua kēia. ʻO ke ʻano kēia e ʻoi aku ka ʻino o nā hōʻailona i ka hala ʻana o ka manawa, ʻoiai inā ʻaʻole i ʻike mua ʻia ka maʻi. ʻOiai ʻo nā puʻupaʻa ke kumu nui o ka aHUS, hiki ke hoʻopilikia ʻia nā ʻōpū ʻē aʻe e like me ka lolo, ke akepaʻa, nā māmā, a me ka puʻuwai e nā poloka o nā kīʻaha koko.

Pehea e ʻike ʻia ai ka aHUS?

Inā ʻaʻohe mea o kou ʻohana i loaʻa i ka maʻi ma mua, hiki ke paʻakikī iki ka ʻike ʻana i ka aHUS. ʻO ke kauka loea i nā maʻi puʻupaʻa (nephrologist) a i ʻole ke kauka loea i nā maʻi koko (hematologist) ka mea kūpono loa e ʻike iā ia. E ʻimi nui lākou i kēia mau hōʻailona ʻekolu:

1. Anemia hemolytic

2. Ua emi ka helu platelet

3. Nā pilikia hana puʻupaʻa

Hiki i kāu kauka ke kauoha i nā hoʻokolohua like ʻole e hōʻoia i kēia kūlana.

He aha nā koho lapaʻau?

ʻElua ʻano lāʻau lapaʻau i ʻāpono ʻia e ka FDA e mālama i ka aHUS.

• ʻEculizumab (Soliris)
• ʻO Ravulizumab-cwcz (Ultomiris)

No kēia mau lāʻau ʻelua kahi hui i kapa ʻia ʻo "monoclonal antibodies." I ka ʻōlelo maʻalahi, he mau protein kūikawā kēia i hana ʻia i loko o ka hale hana e hana like me nā antibodies i hana maoli ʻia i loko o ko mākou kino. Hiki i kēia mau lāʻau ke hoʻonui i ka helu o nā platelets a me nā hunaola koko ʻulaʻula i loko o ke koko. Inā hoʻomaka ʻia kēia lāʻau i ka wā mua o ka maʻi, hiki iā ia ke hoʻohuli i ka hōʻino ʻana o nā puʻupaʻa.

Hāʻawi ʻia kēia mau lāʻau lapaʻau ma ke ʻano he inikini. Eia nō naʻe, hiki iā lākou ke hana i nā hopena ʻaoʻao e like me ke poʻo ʻeha, ke kuni, ka luaʻi, a me nā ʻeha kino. E hoʻomaopopo kāu kauka iā ʻoe e pili ana i kēia mau mea.

Eia kekahi, hoʻohana ʻia ka lāʻau lapaʻau plasma e mālama i nā hōʻailona. Ma kēia, hāʻawi ʻia ka mea maʻi i ka plasma i lawe ʻia mai kahi kanaka olakino a i ʻole e wehe ʻia ka plasma mai ke koko o ka mea maʻi a hoʻololi ʻia me ka plasma olakino.

Inā ʻaʻole pane nā puʻupaʻa i kēia mau lāʻau lapaʻau, pono paha ka dialysis puʻupaʻa a i ʻole ka hoʻoili puʻupaʻa .

Nā kūlana i ka wā e pono ai ʻoe e hele koke i ka haukapila

Inā loaʻa nā ʻōuli koʻikoʻi ma lalo nei, e hele koke i ka ʻOihana Pōpilikia (ETU) o ka haukapila kokoke loa.

• Haʻahaʻa loa ka hoʻopuka ʻana o ka mimi.
• Ka huikau a i ʻole ka nalowale o ka maopopo o ka noʻonoʻo.
• Ka pehu nui o ke kino.
• Paʻakikī ka hanu ʻana.

Pehea e mālama ai i ke olakino o ka noʻonoʻo i ka wā e noho pū ana me ka maʻi?

He mea maʻamau ka manaʻo kaumaha a me ka hopohopo i ka wā e noho ana me kahi maʻi mau e like me aHUS. ʻO ka ʻoi aku o ke koʻikoʻi o ka maʻi a me ka nui o nā hōʻailona āu e loaʻa ai, ʻoi aku ka nui o ka hopena i kou olakino noʻonoʻo. Akā, ʻaʻole pono ʻoe e kū hoʻokahi i kēia.

• E kamaʻilio me kou ʻohana a me kou mau hoaaloha: ʻO ka haʻi ʻana i kou mau manaʻo a me kou mau manaʻo me kahi mea āu e hilinaʻi ai hiki ke lilo i mea hōʻoluʻolu nui.
• E kamaʻilio me nā kauka a me nā kākāʻōlelo: E kamaʻilio me kāu kauka e pili ana i kēia. Hiki iā lākou ke kuhikuhi iā ʻoe i kahi kākāʻōlelo olakino noʻonoʻo inā pono.
• E noʻonoʻo i kou olakino holoʻokoʻa: E hiamoe nui. E ʻimi i nā ala e hōʻemi ai i ke kaumaha. E ʻai i kahi meaʻai maikaʻi no kou mau puʻupaʻa. E hoʻoikaika kino inā hiki. ʻO ke olakino kino e kōkua pū iā ʻoe e noho olakino noʻonoʻo.

ʻOiai he maʻi kēia no ke ola holoʻokoʻa, me ka ʻike mua ʻana, ka mālama kūpono , a me ka mālama ʻana i nā ʻōlelo aʻoaʻo lapaʻau, hiki iā ʻoe ke hoʻokele i nā hopena o kēia maʻi a ola i kahi ola maʻamau. No laila, e noho mau me kāu kauka a hahai i kāu hoʻolālā lapaʻau.