He mea hauʻoli ka lawe ʻana mai i kahi pēpē hānau hou i ka home no ka ʻohana. Akā i ka manawa like, hiki iā ia ke lawe mai i kahi makaʻu iki i nā mākua. "Ke ola maikaʻi nei ke pēpē?", "Ke hanu maikaʻi nei ke pēpē?", "No ke aha ʻo ia e uē nui nei?" Ua loaʻa paha iā ʻoe nā nīnau e like me kēia. ʻOiai ʻo ka hapa nui o ka manawa he mau mea maʻamau kēia, i kekahi manawa aia nā kūlana kakaikahi e pono ai mākou e akahele iki. I kēia lā e kamaʻilio mākou e pili ana i kahi maʻi puʻuwai kakaikahi loa e hiki ke loaʻa i nā pēpē hānau hou, akā pono ka mālama koke ʻana. ʻO ia ka 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 ʻoe he pamu wai nui ʻehā keʻena kou puʻuwai.
- ʻO nā keʻena luna ʻelua (Atria): ʻO ke koko "polū" i nele i ka oxygen mai ke kino e komo i loko o kekahi keʻena, a ʻo ke koko "ʻulaʻula" i loaʻa ka oxygen mai nā māmā e komo i loko o kekahi.
- ʻO nā keʻena haʻahaʻa ʻelua (ventricles): ʻO kēia nā keʻena pamu nui. Ma kekahi ʻaoʻao, hoʻouna lākou i ke koko nele i ka oxygen i nā māmā e kiʻi iā ia. Ma kekahi ʻaoʻao, pamu lākou i ke koko ʻulaʻula waiwai i ka oxygen e hoʻolaha ʻia ma ke kino holoʻokoʻa.
ʻO ke kīʻaha koko nui e lawe ana i ke koko i piha i ka oxygen ma ke kino holoʻokoʻa, ua kapa ʻia ʻo Aorta . ʻO ke ʻano maʻamau, he koʻokoʻo a he ʻāʻī paha.
I kēia manawa, ma ke ʻano o ka Interrupted Aortic Arch (IAA) , ʻaʻole i piha ke kiko o ka aorta. Ua like ia me ka paipu nui e lawe ana i ka wai mai ka pahu wai a i ka hale holoʻokoʻa ua haki i ʻelua ʻāpana . Ma muli o kēia, ʻaʻohe mea pehea ka ikaika o ka puʻuwai e pamu ai, ʻaʻole hiki iā ia ke hoʻouna i ke koko momona i ka oxygen i nā ʻāpana haʻahaʻa o ke kino. He kūlana koʻikoʻi loa kēia a hoʻoweliweli i ke ola . No ka mea, inā ʻaʻole loaʻa ke koko i nā ʻāpana o ke kino, hiki i kēlā mau ʻōkana ke hāʻule a hoʻoweliweli nui i ka pēpē.
He mea kākaʻikahi loa kēia maʻi. Hoʻopilikia ia i ʻelua o kēlā me kēia 100,000 pēpē i hānau ʻia ma Sri Lanka. No laila, ʻoiai he mea nui e makaʻala i kēia, mai hopohopo pono ʻole.
Pehea ʻoe e ʻike ai inā loaʻa i kāu pēpē kēia maʻi? He aha nā hōʻailona?
ʻO ka maʻamau, hōʻike ka pēpē me ka IAA i nā hōʻailona i loko o nā lā mua ma hope o ka hānau ʻana. ʻO kēia no ka mea ma mua o ka hānau ʻana, ke loaʻa nei ka puʻuwai o ka pēpē i ka oxygen mai ke kino o ka makuahine, ʻaʻole ia he pilikia, akā ma hope o ka hānau ʻana, pono ka puʻuwai o ka pēpē e hana ma kāna iho. ʻO ia ka manawa e kū mai ai kēia pilikia.
Pono ʻoe e makaʻala nui e pili ana i nā hōʻailona ma lalo nei.
| Hōʻailona | Ua wehewehe maʻalahi ʻia |
|---|---|
| Ka paʻakikī i ka hānai waiū a me ka luhi nui | ʻOiai ke omo iki ka pēpē i ka waiū, me he mea lā e hakakā ana ʻo ia, e hou ana, a e hana nui ana. No ka mea, ʻoiai kēlā hana liʻiliʻi e pono ai ka nui o ka hoʻoikaika mai ka puʻuwai mai. |
| Hanu wikiwiki | No ka mea, ʻaʻole lawa ka oxygen i ke kino, hoʻomaka ka pēpē e hanu wikiwiki e hoʻoponopono. Me he mea lā e ʻū ana ka umauma. |
| Puʻuwai wikiwiki | Ma muli o ka hana ikaika ʻana o ka puʻuwai e pamu i ke koko ma ke kino holoʻokoʻa, hoʻomaka ka puʻuwai e kuʻi wikiwiki. |
| Ke huli nei ka ʻili i ka hina a i ʻole ka uliuli | ʻOiai ʻaʻole kahe pono ke koko waiwai i ka oxygen i nā ʻāpana haʻahaʻa o ke kino o ke pēpē, e like me nā wāwae a me nā wāwae, hiki i ka ʻili ma ia mau wahi ke lilo i hina hina a kaumaha paha. |
| Ka hiamoe a me ka nāwaliwali maʻamau ʻole | Inā hiamoe mau kāu pēpē a palaualelo, malia paha no ka mea ʻaʻole i loaʻa i kona kino ka ikehu e pono ai. |
Inā ʻike ʻoe i kekahi o kēia mau hōʻailona, e ʻike koke i ke kauka me ka hoʻokaʻulua ʻole. Inā pono, e lawe i kāu pēpē i ka Emergency Treatment Unit (ETU) o ka haukapila kokoke loa.
ʻO nā kīnā puʻuwai ʻē aʻe e hiki ke hana me kēia ʻano
Hiki i nā pēpē me IAA ke loaʻa kekahi mau kīnā puʻuwai ʻē aʻe i ka manawa like.
- ʻO ke kīnā o ka Ventricular Septal (VSD): He lua kēia ma ka paia ma waena o nā keʻena pumping haʻahaʻa ʻelua (ventricles) o ka puʻuwai. ʻO ka mea kupanaha, ʻo ka loaʻa ʻana o ka VSD i kekahi manawa hiki ke lilo i kōkua manawa pōkole i nā hihia o IAA, no ka mea, e ʻae ana i kekahi koko oxygenated e hele ma waena o ka lua i ke kino.
- ʻO ke kīnā o ka Atrial Septal (ASD): He lua kēia ma ka paia ma waena o nā keʻena ʻelua o luna o ka puʻuwai.
- ʻO Truncus Arteriosus: ʻO kēia kahi e hui pū ai nā kīʻaha koko nui ʻelua e puka mai ana mai ka puʻuwai (ka aorta a me ke aʻa pulmonary) e hana i hoʻokahi.
No ke aha e hiki ai kēia i nā pēpē? He aha ke kumu?
Ma ke ʻano he mau mākua, ʻo ka nīnau mua e hiki mai ana i kou manaʻo ke lohe ʻoe i kēia, "ʻO kākou anei kēia hewa?" ʻAʻole loa.
ʻO ka Interrupted Aortic Arch (IAA) kahi maʻi i hoʻokumu ʻia e kahi kīnā genetic . I ka ʻōlelo maʻalahi, ʻo kā mākou chromosomes ka 'puke' e loaʻa ana nā kuhikuhi āpau no ke ʻano o ke kūkulu ʻia ʻana o ko mākou kino. Ma kēia kūlana, ua nalowale kahi ʻāpana liʻiliʻi loa o kahi chromosome e loaʻa ana nā kuhikuhi no ke ʻano o ke kūkulu ʻia ʻana o ka puʻuwai a me nā kīʻaha koko. ʻO kēia ke kumu o ka hana pono ʻole ʻana o ka aorta.
I ka manawa pinepine, loaʻa i nā pēpē me IAA kekahi ʻano genetic ʻē aʻe i kapa ʻia ʻo DiGeorge syndrome . Hoʻokumu ʻia kēia e kahi ʻāpana nalowale o ka chromosome like. Loaʻa paha i nā keiki me DiGeorge syndrome nā maʻi pinepine a me kekahi mau loli o ka helehelena (e like me ka lehelehe māwae).
Pehea e ʻike pono ai nā kauka i kēia?
Ke lawe ʻoe i kāu pēpē i ka haukapila, inā nānā nā kauka i ka pēpē a manaʻo he maʻi puʻuwai e like me kēia, ʻo ka hoʻāʻo nui e hōʻoia i ia he echocardiogram .
Ua like ka echocardiogram me ka nānā ʻana i kahi wikiō o ka puʻuwai o kahi pēpē. ʻAʻohe ʻeha. Hoʻohana ia i nā nalu ultrasound e ʻike maopopo i nā keʻena o ka puʻuwai, nā valves, a me nā kīʻaha koko. I ke ʻano o IAA, hiki iā ia ke hōʻike maopopo i ka paʻa ʻana i loko o ka aorta.
I kekahi manawa, hiki ke ʻike ʻia kahi kīnā puʻuwai e like me kēia i ka wā o kahi scan anomaly (ultrasound scan) i hana ʻia ʻoiai ke pēpē i loko o ka ʻōpū.
He aha nā lāʻau lapaʻau no kēia? Hiki ke ho'ōla ʻia ke pēpē?
ʻAe, ʻoiaʻiʻo nō. ʻOiai he maʻi koʻikoʻi kēia, hiki ke ho'ōla loa ʻia me ke ʻoki kino . Hiki ke hoʻokaʻawale ʻia ke kaʻina hana lapaʻau i ʻelua ʻāpana nui.
1. He aha e hana ai ma mua koke o ke ʻoki kino
Ma hope o ka hōʻoia ʻana, ua hoʻokomo koke ʻia ka pēpē i kahi keʻena mālama koʻikoʻi neonatal (NICU). ʻO ka pahuhopu nui ka mālama ʻana i ke kahe koko i ke kino a hiki i ka wā e mākaukau ai ka pēpē no ke ʻoki ʻana.
Hoʻohana nā kauka i kahi hana akamai e hana ai i kēia. Ma mua o ka hānau ʻana o kēlā me kēia pēpē, aia kahi pilina manawa pōkole (ductus arteriosus) ma waena o ka aorta a me ke aʻa e lawe ana i ke koko i nā māmā. Pani kēia ma kāna iho i loko o kekahi mau hola a i ʻole nā lā ma hope o ka hānau ʻana.
Akā ma ke kūlana IAA, ʻaʻole e ʻae nā kauka i kēia pilina manawa pōkole e pani. Ua like ia me kahi 'bypass' i hoʻohana ʻia i ka wā e pani ʻia ai ke alanui nui. E mālama i kēia bypass e wehe.Hāʻawi ʻia kahi lāʻau i kapa ʻia ʻo Prostaglandin E1 i ka pēpē i loko o ka saline. ʻAe kēia i ke kino haʻahaʻa o ka pēpē e loaʻa i kekahi koko a hiki i ke ʻoki ʻana.
2. ʻO ke ʻoki ola e hoʻōla ai
Ke paʻa ke kūlana o ke pēpē, hana ʻia ke ʻoki ʻana e nā kauka lapaʻau puʻuwai o nā keiki.
- ʻO ka mea nui: Ua hoʻopili hou ʻia nā ʻāpana ʻelua o ka aorta i ʻoki ʻia a hoʻokaʻawale ʻia, e hana ana i kahi ʻāʻī piha.
- Nā mea hou aʻe: I ka manawa like, inā loaʻa i ka pēpē kahi VSD (kahi lua i loko o ka puʻuwai), e pani ʻia nō hoʻi ia i ka wā o kēia ʻoki kino. E pani ʻia nō hoʻi ka bypass (PDA) i wehe ʻia me ka lāʻau lapaʻau ma mua, no ka mea, i kēia manawa ua pau ke ala nui, ʻaʻohe pono no ia.
He ʻoki kino paʻakikī loa kēia, akā ua hoʻokō pono nā kauka akamai ma Sri Lanka i ia mau ʻoki kino.
Pehea lā ka wā e hiki mai ana o ke pēpē ma hope o ke ʻoki kino?
ʻO kēia ka mea nui loa. Ma hope o kahi ʻoki holomua, hiki i ka hapa nui o nā keiki ke ola i kahi ola maʻamau a olakino . Wahi a nā helu helu, ʻoiai he 10 mau makahiki ma hope o ke ʻoki ʻana, ʻoi aku ma mua o 81% o ka poʻe e ola nei.
Akā ʻo ka mea nui e hoʻomanaʻo ai, ma hope o ke ʻoki ʻana, pono ʻoe e noho ma lalo o ka nānā ʻana o kahi kauka puʻuwai no ke koena o kou ola . Pono ʻoe e hele i nā keʻena lapaʻau i ka manawa i hoʻonohonoho ʻia a loaʻa nā hoʻokolohua e like me ka echocardiogram e ʻike ai inā e hana pono ana kou puʻuwai. I kekahi manawa, ke ʻelemakule ʻoe, pono paha ʻoe i kekahi lāʻau lapaʻau hou. No laila, he mea nui e hahai i nā ʻōlelo a kāu kauka.
He mea maʻamau ka manaʻo e like me ka hiolo ʻana o kou honua holoʻokoʻa ke ʻike ʻoe ua loaʻa i kāu pēpē kēia maʻi. Akā e hoʻomanaʻo, ua holomua loa ka ʻepekema lapaʻau i kēia lā. Inā ʻike mua ʻia ka maʻi a mālama pono ʻia, loaʻa i kāu pēpē kahi manawa kūpono e hauʻoli ai e like me nā keiki ʻē aʻe.
Leka Lawe-Home
- ʻO ka Interrupted Aortic Arch (IAA) ʻo ia ka wā i piha ʻole ai ke kīʻaha koko nui e lawe ana i ke koko mai ka puʻuwai o ke pēpē i ke kino.
- He kūlana kakaikahi loa kēia, akā he kūlana koʻikoʻi e pono ai ke kōkua koke ʻia e ke kauka .
- E makaʻala loa i nā hōʻailona e like me ka paʻakikī i ka hānai waiū, ka hanu wikiwiki, a me ka ʻili hina.
- ʻO ke kīnā genetic ke kumu o kēia, ʻaʻole ka hewa o nā mākua.
- He ʻoki kino ke ʻano o ka mālama ʻana, a ma hope o ke ʻoki kino holomua, hiki i ka hapa nui o nā keiki ke ola maʻamau .
- He mea nui e noho ma lalo o ka mākaʻikaʻi a ke kauka cardiologist no ke koena o kou ola, ʻoiai ma hope o ke ʻoki ʻana.
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