Ke ʻeha nei kāu keiki liʻiliʻi i kahi ālai ʻia i loko o ke kīʻaha koko nui o ko lākou puʻuwai? E aʻo kākou e pili ana i ka Interrupted Aortic Arch (IAA) ma nā ʻōlelo maʻalahi.

ʻAʻole hiki ke wehewehe ʻia ka hauʻoli āu e ʻike ai ke nānā aku ʻoe i kāu pēpē hānau hou, ʻeā? I ka manawa like, aia kahi kuleana nui i loko o kou puʻuwai no kēlā ola liʻiliʻi. I kekahi manawa he mea maʻamau ka makaʻu nui a me ka hopohopo ke lohe ʻoe e pili ana i kekahi mau maʻi a nā keiki liʻiliʻi e loaʻa ai. I kēia lā, ke kamaʻilio nei mākou e pili ana i kahi maʻi puʻuwai koʻikoʻi e hiki ke loaʻa i nā pēpē hānau hou, akā he mea kakaikahi loa ia, a pono e mālama koke ʻia e ke kauka. Ua kapa ʻia kēia ʻano he Interrupted Aortic Arch, a i ʻole (IAA) no ka pōkole.

I ka ʻōlelo maʻalahi, he aha ka Interrupted Aortic Arch (IAA)?

I mea e hoʻomaopopo ai i kēia, e nānā mua kākou i ke ʻano o ka hana ʻana o ko kākou puʻuwai. E noʻonoʻo i ko kākou puʻuwai e like me ka pamu wai. ʻEhā mau keʻena nui o kēia pamu. ʻElua ma luna a ʻelua ma lalo. ʻO ke koko, ka mea i hoʻohana ʻia e ke kino a he haʻahaʻa ka nui o ka oxygen, he uliuli iki, e komo i loko o ke keʻena ʻākau o luna. Mai laila mai, hele ia i ke keʻena haʻahaʻa a hoʻouna ʻia i nā māmā. I loko o nā māmā, ua lako pono kēia koko i ka oxygen a loaʻa i kahi kala ʻulaʻula nani.

I kēia manawa, komo kēia koko ʻulaʻula i hoʻopiha ʻia me ka oxygen i loko o ke keʻena hema o luna o ka puʻuwai. Mai laila mai, hele ia i ke keʻena hema o lalo a hoʻokuʻu ʻia i loko o ke kino ma o ke kīʻaha koko nui loa a koʻikoʻi loa i loko o ko kākou kino, ʻo ia hoʻi ka aorta . Ua like kēia aorta me ka ʻōnaehana paipu nui e lawe ana i ka wai mai ko kākou pahu wai a i ka hale holoʻokoʻa. Ua like kona ʻano me ke kō kō.

No laila, ʻo ka mea e hana ʻia ana i loko o kahi pēpē me kahi maʻi i kapa ʻia ʻo Interrupted Aortic Arch (IAA) ʻo ia ka mea ʻaʻole i piha ke kīʻaha koko nui, ʻo ka aorta. Aia kahi mea e ālai ana i loko, e like me kahi hakahaka i haki. I ka ʻōlelo maʻalahi, ua haki ka paipu wai nui ma waenakonu. Ma muli o kēia, ʻaʻohe mea pehea ka ikaika o ka puʻuwai e pamu ai, ʻaʻole hele ke koko momona oxygen maikaʻi i nā ʻāpana haʻahaʻa o ke kino.

Pehea ʻoe e ʻike ai inā loaʻa i kāu pēpē kēia maʻi? He aha nā hōʻailona?

ʻO ka maʻamau, hoʻomaka nā pēpē me IAA e hōʻike i nā hōʻailona i loko o nā hola mua a i ʻole nā ​​​​lā ma hope o ka hānau ʻana. Ma ke ʻano he mau mākua, he mea nui loa iā ʻoe e makaʻala i kēia.

Inā ʻike ʻoe i hoʻokahi a ʻoi aku paha o kēia mau hōʻailona i kāu pēpē, pono ʻoe e ʻike koke i ke kauka a lawe paha i kāu pēpē i ka Emergency Treatment Unit (ETU) o ka haukapila me ka hoʻokaʻulua ʻole .

No ke aha e hana ai kēia i nā pēpē?

Ke lohe ʻoe i kahi mea e like me kēia, ʻo ka nīnau mua e hiki mai ana i kou manaʻo ma ke ʻano he makua, "No ke aha i hana ʻia ai kēia i kā mākou pēpē? Ua hana hewa anei mākou?"

I ka ʻōlelo maʻalahi, hiki mai kēia ʻano i ka wā e ulu ana kahi pēpē i loko o ka ʻōpū, ma muli o ka nalowale ʻana o kahi ʻāpana liʻiliʻi loa o kahi chromosome e hoʻopilikia ai i ka hoʻokumu ʻia ʻana o ka puʻuwai a me nā kīʻaha koko nui.

Loaʻa pinepine i nā pēpē me IAA ka maʻi DiGeorge.Hiki ke hana me kekahi ʻano maʻi ʻē aʻe i kapa ʻia ʻo DiGeorge syndrome. Hoʻokumu ʻia kēia e ka nalowale ʻana o kahi ʻāpana o ka chromosome like e like me ka mea i ʻōlelo ʻia ma luna. Loaʻa pinepine paha i nā keiki me DiGeorge syndrome nā maʻi a loaʻa paha kekahi mau loli o ka helehelena (e like me ka lehelehe māwae).

ʻO nā kīnā puʻuwai ʻē aʻe e hele pū me IAA

Loaʻa pinepine i nā pēpē me IAA hoʻokahi a ʻoi aku paha nā kīnā puʻuwai ʻē aʻe. ʻO kekahi o nā mea maʻamau ka Ventricular Septal Defect (VSD).

• ʻO ke kīnā o ka ventricular Septal (VSD): I ka ʻōlelo maʻalahi, he lua kēia ma ka paia (septum) ma waena o nā keʻena pumping nui ʻelua (ventricles) o ka puʻuwai. ʻOiai he kīnā maʻamau kēia, hiki i ka VSD ke lilo i mea hoʻopakele ola no kahi pēpē me IAA. E noʻonoʻo i ka wā hiki ʻole i ke koko ke kahe i lalo o ka aorta, hiki i kekahi o ke koko oxygenated ma ka ʻaoʻao hema o ka aorta ke kahe ma o kēia lua i ka ʻaoʻao ʻākau, kahi e hiki ai ke kahe i nā māmā e hāʻawi i kekahi koko i nā ʻāpana haʻahaʻa o ke kino. Ua like ia me ka loaʻa ʻana o kahi bypass liʻiliʻi i ka wā e ālai ʻia ai ke alanui nui. Akā he hopena manawa pōkole wale nō kēia.

He aha nā ʻano hōʻailona a me nā ʻano lapaʻau?

He mea nui e ʻike a mālama i kēia maʻi i ka wā hikiwawe loa.

ʻIkepili

ʻIke pinepine ʻia kēia maʻi me ka Echocardiogram . Kāhea wale mākou iā ia he 'Echo test'. Ua like kēia me ke scan o ka puʻuwai. Hiki i kēia hoʻāʻo ʻeha ʻole ke ʻike maopopo i ke ʻano a me ka hana o nā keʻena o ka puʻuwai, nā valves, a me nā kīʻaha koko. I kekahi manawa, hiki ke ʻike mua ʻia kēia maʻi me nā scan i hana ʻia ʻoiai ke pēpē i loko o ka ʻōpū.

Lapaʻau

ʻO ke ʻoki kino ke ʻano o ka lāʻau no IAA. Eia nō naʻe, a hiki i ka wā e mākaukau ai ka pēpē no ke ʻoki kino, e hana mua nā kauka i kahi mea ʻē aʻe e hoʻopakele i ke ola o ka pēpē.

1. Ka Hana ʻEkahi - E Mālama Ana i ka ʻAuwai Kupanaha:

Loaʻa i kēlā me kēia pēpē hānau hou kahi pilina manawa pōkole ma waena o ka puʻuwai, ka aorta, a me ka ʻōmole e lawe ana i ke koko i nā māmā i kapa ʻia ʻo Ductus Arteriosus . He mea nui loa kēia i ka wā e noho ana ke pēpē i loko o ka ʻōpū. Pani kēia ʻōmole iā ia iho i loko o kekahi mau hola o ka hānau ʻana.

Akā i loko o kahi pēpē me IAA, no ka mea ua haehae ʻia ka aorta, ʻo ke ala ʻē aʻe wale nō no ke koko oxygenated e kahe i lalo o ke kino ma o ka Ductus Arteriosus. No laila, ʻo ka mea mua a nā kauka e hana ai, ʻo ia ka hāʻawi ʻana i ka pēpē i kahi lāʻau i kapa ʻia ʻo Prostaglandin E1 , ka mea e mālama ai i kēlā ʻōmole manawa pōkole me ka ʻole o ka pani ʻana. Kōkua kēia i ka mālama ʻana i ka lako koko i ke kino o ka pēpē a hiki i ke ʻoki ʻana.

2. Ka lua o ka hana - ʻoki kino:

Ke kūpaʻa ke kūlana o ke pēpē, hana nā kauka lapaʻau kūikawā i ke ʻoki kino. ʻO kēia ka mea e pili ana i:

• ʻO ka lawe ʻana i nā wēlau haʻihaʻi ʻelua o ka aorta a hoʻohui iā lāua e hana i kahi kīʻaha koko piha a me ke ālai ʻole.
• Ke pani ʻana i ka Ductus Arteriosus manawa pōkole i wehe ʻia ma ka hoʻokomo ʻana i ka prostaglandin.
• Inā he VSD (he puka ma ka paia o ka puʻuwai), e pani ʻia nō hoʻi ia i ka wā o kēia ʻoki kino.

Pehea ka manaʻo o ke pēpē ma hope o ke ʻoki kino?

ʻAʻohe mea ʻoi aku ka paʻakikī no nā mākua ke hoʻomanawanui ma mua o ka lohe ʻana ua loaʻa i kā lākou keiki hānau hou kahi maʻi e hoʻoweliweli ana i ke ola. Akā mai nā mea a mākou i kūkākūkā ai, hiki iā ʻoe ke ʻike e hiki ke mālama pono ʻia kēia ʻano me ka nānā koke ʻana i ke kauka.

Hiki ke maikaʻi loa ke olakino o nā keiki ma hope o ke ʻoki kino IAA. Wahi a nā helu helu, ʻoiai he 10 mau makahiki ma hope o ke ʻoki kino, 81% o nā keiki e ola nei. ʻO ia hoʻi, kiʻekiʻe loa ka helu holomua.

Akā he pilikia kēia. I ka wā e ʻike ʻia ai nā hōʻailona, ​​​​pono e hoʻokomo ʻia ka pēpē i ka ʻāpana mālama koʻikoʻi (ICU) o ka haukapila a pono e hoʻomaka koke ka mālama ʻana. ʻOiai ma hope o ke ʻoki ʻana, he mea nui e hoʻomau i ka lawe ʻana i ka pēpē i ke keʻena lapaʻau a hana i nā hoʻokolohua e pono ai, e like me ka ʻōlelo a nā kauka.