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

He aha ka mea e hana ʻia inā e hana ʻia nā ʻūpā koko i loko o nā kīʻaha koko liʻiliʻi i loko o kou mau puʻupaʻa, e ālai ana i ke kahe koko i kou mau puʻupaʻa? He mea weliweli ke noʻonoʻo ʻia, ʻeā? ʻO ia ka mea a mākou e kamaʻilio nei i kēia lā. Ua kapa ʻia kēia ʻo Atypical Hemolytic Uremic Syndrome, a i ʻole aHUS no ka pōkole. ʻOiai ʻaʻole hiki ke ho'ōla ʻia, hiki ke mālama maikaʻi ʻia me ka mālama kūpono . No laila e aʻo hou kākou e pili ana i ia mea.

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

Ua lohe paha ʻoe i kahi maʻi i kapa ʻia ʻo HUS. Kāhea mākou iā ia he "HUS maʻamau." Hoʻokumu pinepine ʻia ia e kahi maʻi bacteria e like me E. coli, ka mea e hoʻoulu ai i ka maʻi diarrhea. Akā ʻo ke ʻano a mākou e kamaʻilio nei i kēia lā, ʻo aHUS, he ʻokoʻa ia.

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 ka maʻi i nā wahine. ʻO kēia no ka mea hiki i ka hāpai ʻana ke lilo i mea hoʻoulu nui no ka maʻi. He mea kakaikahi loa ia e hōʻike ana kekahi mau noiʻi he ʻelua wale nō i loko o ka miliona o ka poʻe i loaʻa i ka maʻi.

No ke aha e ulu ai ʻo aHUS?

I ka ʻōlelo maʻalahi, he nui ka poʻe i loaʻa i ka aHUS he loli genetic, a i ʻole ka mutation gene, i loko o ko lākou kino. E noʻonoʻo e like me he 'pihi' i loko o kou kino e hiki ai ke hoʻoulu i kēia maʻi. Akā, ʻo ka loaʻa ʻana o kēlā pihi ʻaʻole ia e hoʻoulu koke i ka maʻi. Pono ia i kahi mea ma waho ona e 'hoʻā ʻia', a i ʻole e hoʻāla ʻia. Kāhea mākou i kēia mau 'mea hoʻoulu'.

Hiki i nā mea hoʻonāukiuki nui ke:

• Hāpai keiki
• Kekahi mau kūlana maʻi
• Ka maʻi ʻaʻai
• Kekahi mau lāʻau lapaʻau
• He hoʻoili kino
• ʻO kekahi mau maʻi olakino lōʻihi (e like me ka sclerosis systemic a i ʻole ka hypertension malignant)

He aha nā hōʻailona?

ʻIke pinepine ʻia nā hōʻailona aHUS ma hope koke o ka loaʻa ʻana o kahi mea hoʻonāukiuki. Hiki i nā flare-up ke akahai a koʻikoʻi paha. I nā hihia akahai, ʻaʻole paha e hoʻopilikia nui ʻia nā puʻupaʻa, akā i nā hihia koʻikoʻi, hiki iā lākou ke alakaʻi i ka hāʻule ʻana o nā puʻupaʻa .

Nā hōʻailona hiki iā ʻoe ke ʻike i nā pae mua:

• ʻEha kino a me ka luhi nui
• 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. Hoʻopilikia nui ka aHUS i kou mau puʻupaʻa. Eia nō naʻe, 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 ai i ka maʻi?

Inā ʻaʻohe mea o kou ʻohana i loaʻa i ka maʻi ma mua, hiki ke paʻakikī iki ka ʻike ʻana i ka aHUS. ʻO ka maʻamau, ʻo kahi nephrologist a hematologist paha ka mea maikaʻi loa e ʻike e pili ana i kēia. E nānā pono lākou i nā mea i ʻōlelo ʻia ma ka papa ma luna a kuhikuhi iā ʻoe no nā hoʻokolohua e pono ai.

He aha nā koho lapaʻau?

ʻO ka mea pōmaikaʻi, aia ʻelua mau lāʻau lapaʻau i ʻāpono ʻia no aHUS.

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

No kēia mau mea ʻelua i kahi papa o nā lāʻau i kapa ʻia ʻo 'monoclonal antibodies.' I ka ʻōlelo maʻalahi, he mau protein i hana ʻia i hana ʻia e like me nā antibodies kūlohelohe o ko mākou kino. Hoʻomalu lākou i kahi ʻāpana o ka ʻōnaehana pale o ke kino a hoʻōki i ke kāpili ʻana o ke koko.

Hiki i kahi lāʻau i kapa ʻia ʻo "Eculizumab" ke hoʻonui i ka helu o nā platelets a me nā hunaola koko ʻulaʻula i loko o kou koko. Inā hoʻomaka koke ʻia ka lāʻau lapaʻau , hiki i kēia lāʻau ke hoʻohuli i kekahi o nā pōʻino i nā puʻupaʻa.

Hāʻawi ʻia kēia mau lāʻau ʻelua ma ke ʻano inikini. Hiki iā lākou ke hana i nā hopena ʻaoʻao liʻiliʻi, e like me ke poʻo ʻeha, nā hōʻailona like me ka maʻi flu, ka ʻeha o ka ʻōpū, a me nā ʻeha kino. ʻO ka mea nui loa, aia kahi pilikia o nā maʻi koʻikoʻi, e like me ka meningitis (maʻi meningococcal), i ka wā e lawe ai i kēia mau lāʻau. No laila e haʻi aku kāu kauka iā ʻoe e kiʻi i kahi pana no kēlā, a hāʻawi paha iā ʻoe i nā antibiotics e pale aku i ka maʻi.

Ma waho aʻe o kēia mau lāʻau lapaʻau, hiki ke hoʻohana ʻia ka plasma therapy . Pili kēia i ka hāʻawi ʻana i ka plasma maʻi (ka ʻāpana wai o ke koko) i lawe ʻia mai kahi kanaka olakino (plasma infusion) a i ʻole ka wehe ʻana i ka plasma o ka mea maʻi a hoʻololi iā ia me ka plasma olakino (plasma exchange).

Inā ʻaʻole pane nā puʻupaʻa i kekahi o kēia mau lāʻau lapaʻau, ʻo ke koho hope loa he dialysis puʻupaʻa a i ʻole kahi hoʻoili puʻupaʻa .

Nā pilikia a me nā pilikia hiki ke loaʻa

Ma waho aʻe o ka hāʻule ʻana o ka puʻupaʻa, hiki i ka aHUS ke hana i nā pilikia ʻē aʻe.

Ke ola nei me kēia maʻi a me ke olakino noʻonoʻo

He mea maʻamau ke kaumaha a me ka hopohopo i ka wā e noho ana me kahi maʻi lōʻihi e like me kēia. ʻAʻole na ʻoe ia hewa. ʻO ia ke kumu he mea nui e mālama i kou olakino noʻonoʻo a me kou olakino kino .

• E noi i kāu hui lapaʻau no ke kōkua: Hiki i nā kauka, nā kahu maʻi, a me nā limahana hana kaiaulu ke alakaʻi iā ʻoe i nā wahi e hiki ai iā ʻoe ke loaʻa ke kākoʻo noʻonoʻo āu e pono ai.
• E ʻike i kahi kākāʻōlelo: ʻO ke kamaʻilio ʻana me kahi kākāʻōlelo laikini e hiki ke kōkua i ka hoʻomāmā ʻana i kou kaumaha a kūkulu i ka ikaika noʻonoʻo e pono ai ʻoe e hoʻoponopono i kēia kūlana.
• E mālama iā ʻoe iho: E hiamoe pono. E ʻimi i nā ala e hōʻemi ai i ke kaumaha. E ʻai i ka papaʻai kūpono no nā puʻupaʻa. E hoʻoikaika kino i ka nui o ka hiki. Hiki i kēia mau mea ke hoʻopilikia nui i kou olakino holoʻokoʻa.
• E kamaʻilio me kāu poʻe aloha: ʻO ke kamaʻilio ʻana e pili ana i kēia me ka ʻohana a me nā hoaaloha e hāʻawi iā ʻoe i ka hōʻoluʻolu nui.