Ua hānau ʻia kāu keiki liʻiliʻi me kahi pilikia puʻuwai paʻakikī? I kekahi manawa ua haʻi aku paha nā kauka iā ʻoe he hoʻokahi wale nō pamu nui o ka puʻuwai o kāu pēpē, he ventricle. ʻO kahi ʻoki kūikawā a nā kauka e ʻōlelo ai i kēlā mau hihia ua kapa ʻia ʻo 'Fontan procedure'. He mea maʻamau loa ka makaʻu a me ke kānalua ke lohe ʻoe i kēia inoa. Akā inā ʻike piha mākou i kēia, e loaʻa iā ʻoe ka ikaika nui a me ka ikehu e hele ai i kēia huakaʻi. E kamaʻilio maʻalahi anei kākou e pili ana i kēia?
He aha ke ʻano o ke kaʻina hana Fontan ma ka ʻōlelo maʻalahi?
I mea e hoʻomaopopo ai i kēia, e nānā mua kākou i ke ʻano o ka hana ʻana o ka puʻuwai olakino. ʻEhā mau keʻena nui o ka puʻuwai olakino. ʻElua ma luna (atria) a ʻelua ma lalo (ventricles). ʻO kēia mau keʻena ʻelua nā pamu nui ʻelua o ka puʻuwai. Hoʻouna kekahi i ke koko haumia a nele i ka oxygen i nā māmā e lawe i ka oxygen. Hoʻopuka kekahi i ke koko maʻemaʻe a waiwai i ka oxygen i ke koena o ke kino.
Eia nō naʻe, hānau ʻia kekahi mau pēpē me kekahi o kēia mau ʻāpana ʻelua i hana pono ʻole ʻia. A laila pono ke ʻāpana hoʻokahi e hana i ka hana e hana i kēia mau hana ʻelua. E noʻonoʻo i ka luhi o ke kanaka hoʻokahi e hana i ʻelua mau hana. E like me kēlā, hiki i kēia ʻāpana hoʻokahi ke luhi koke no ka mea pono ia e pamu i ke koko i nā māmā a me ke kino.
ʻO ka hana a ke kaʻina hana Fontan, ʻo ia ka hoʻemi ʻana i ka ukana hana ma kēlā kelepona hoʻokahi. Ua like ia me ka hāʻawi ʻana i kekahi e kōkua iā ʻoe e hana ikaika.
Ma kēia kaʻina hana, hoʻopili nā kauka ʻoki i ke koko nele i ka oxygen mai ke kino haʻahaʻa a i ke aʻa pulmonary, e kāpae ana i ka puʻuwai. Ua like kēia me ka hana ʻana i kahi 'pōkole pōkole' hou. ʻO ka maʻamau, ma mua o kēia kaʻina hana, hana ʻia kekahi kaʻina hana i kapa ʻia ʻo ke kaʻina hana Glenn e kuhikuhi i ke koko nele i ka oxygen mai ke kino o luna a i nā māmā. ʻO ke kaʻina hana Fontan ka hana hope loa i kēia moʻo o nā kaʻina hana.
Ke hana ʻia nā ala hou ma kēia ʻano, ʻo ka hana wale nō a kēlā cell hoʻokahi, ʻo ia ke kaomi ʻana i ke koko maʻemaʻe a waiwai i ka oxygen ma ke kino holoʻokoʻa. He mea hōʻoluʻolu nui kēia no kēlā puʻuwai liʻiliʻi.
ʻO wai ka mea e pono ai kēia ʻoki kino a no ke aha?
Hana nui ʻia kēia ʻoki kino ma nā keiki i hānau ʻia me hoʻokahi ventricle. Hana pinepine ʻia ma waena o nā makahiki 2 a me 5 .
Akā, ʻaʻole no nā kānaka a pau kēia ʻoki kino. E nānā pono ke kauka i kāu keiki e ʻike inā kūpono lākou no kēia. Eia kekahi laʻana:
- Pono ka ʻōnaehana hana o ke keiki e ikaika loa.
- Pono nā māmā e olakino pono e loaʻa ai ke koko e hele pololei mai ana i nā māmā.
Aia ma ka papa ma lalo nei kekahi o nā maʻi puʻuwai nui i mālama ʻia me kēia ʻoki kino.
| Kūlana | He wehewehe maʻalahi |
|---|---|
| ʻO ka Hypoplastic Left Heart Syndrome (HLHS) | ʻAʻole e ulu pono ka ʻaoʻao hema o ka puʻuwai (me ka ventricle hema). (ʻO kēia ke kumu maʻamau no ke ʻoki ʻana iā Fontan) |
| ʻO ka Atresia Tricuspid | ʻAʻole i ulu pono ka valve tricuspid ma ka ʻaoʻao ʻākau o ka puʻuwai. |
| ʻO ka Atresia Pulmonary | Ka pale ʻana o ka kiwikā pulmonary e lawe ana i ke koko i nā māmā. |
| Ventricle hema komo pālua | Hoʻopili ʻia nā kīʻaha koko nui ʻelua e komo ana i ka puʻuwai i ka ventricle hema. |
He aha ka mea e hana ʻia ma mua a i ka wā o ke ʻoki kino?
Ma hope o ka hoʻoholo ʻana ua kūpono kāu keiki no ke ʻoki kino, e hana ke kauka i kekahi mau hoʻokolohua.
- ʻO ka Transthoracic Echocardiogram (TTE): He scan ultrasound kēia o ka puʻuwai. Hāʻawi ia i kahi kiʻi maikaʻi o ke ʻano o ka puʻuwai, nā valves, a me ka hana.
- Electrocardiogram (EKG): Ana i ka hana uila o ka puʻuwai.
- Ka Hoʻokomo ʻana i ka Catheter o ka Puʻuwai: Hoʻokomo ʻia kahi ʻōmole liʻiliʻi (catheter) ma o kahi kīʻaha koko i loko o ka puʻuwai e ana i ke kaomi a me nā pae oxygen i loko o ka puʻuwai.
- CT a i ʻole MRI Scan: I kekahi mau hihia, pono kēia e kiʻi i nā kiʻi ʻekolu-dimensional (3D) o ka puʻuwai a me nā kīʻaha koko e hoʻopuni ana.
I ka lā o ke ʻoki kino, hāʻawi ʻia ke keiki i ka anesthesia e hoʻohiamoe iā ia. ʻO ke ʻano kēia, ʻaʻole e ʻeha ke keiki. I ka wā o ke ʻoki kino, e hoʻopili ʻia ke keiki i kahi mīkini kūikawā e hana manawa pōkole i ka hana o ka puʻuwai a me nā māmā (mīkini puʻuwai-māmā) . E hoʻolako kēia mīkini i ke koko i ke kino a hiki i ka wā e hana ai ke kauka ʻoki kino i ka pilina hou (mai ka vena cava haʻahaʻa a i ke aʻa pulmonary).
I kekahi manawa hana ke kauka lapaʻau i kahi lua liʻiliʻi (fenestration) ma kēia alahele hou.Hana ʻia kēia e hōʻemi i ke kaomi ma ka puʻuwai ma hope o ke ʻoki ʻana. Hiki i ke ʻoki holoʻokoʻa ke hala ma kahi o 4-5 mau hola .
He aha ka mea e hana ʻia ma hope o ke ʻoki kino? Aia kekahi mau pilikia?
Ma hope o ke ʻoki kino, ua hoʻokomo ʻia ke keiki i ka ʻāpana mālama koʻikoʻi (ICU) no ka nānā pono ʻana. Ma hope o kekahi mau lā, ua hoʻoneʻe ʻia lākou i kahi keʻena maʻamau. ʻO ka awelika o ka noho ʻana ma ka haukapila ma waena o 7 a me 13 mau lā .
He mau pono a me nā pilikia kēia hana ʻoki kino, a he mea nui loa ia e ʻike iā lākou.
| ʻAno pilikia | Nā pilikia hiki ke loaʻa |
|---|---|
| Nā pilikia pōkole (Ma hope o ke ʻoki kino) | ʻO ka effusions pleural, nā pilikia puʻupaʻa, ka hāʻule ʻana o ka puʻuwai, nā puʻuwai kūpono ʻole, nā maʻi akepaʻa. |
| Nā pilikia lōʻihi (me ka manawa) | ʻO ka enteropathy nalowale protein, nā ʻūpā koko (thromboembolism), ka maʻi akepaʻa e pili ana iā Fontan (FALD), a me ka bronchitis plastic. |
I ka hala ʻana o ka manawa, hiki i kekahi mau maʻi ke ʻike i ka emi ʻana o ka pono o kēia ʻoki kino. Ua kapa ʻia kēia he 'Fontan i nele'. I kēlā manawa, pono paha kahi hoʻoili puʻuwai . Manaʻo nā kauka i kēia ʻāpana o ka huakaʻi.
He aha ke ʻano o ka wā e hiki mai ana e hiki i ke keiki ke manaʻolana?
I ka wā ma mua, ʻaʻole maopopo ka wā e hiki mai ana o kahi keiki i hānau ʻia me kahi cell hoʻokahi. Akā i kēia lā, me ka holomua o ka ʻepekema lapaʻau, ua hāʻawi ka hana Fontan i kēia mau keiki i ka manawa kūpono e neʻe ai ma mua o ka wā kamaliʻi, i ka wā ʻōpio, a i ka wā makua.
Wahi a nā helu helu, o ka poʻe i hana ʻia ke ʻoki Fontan:
- Ma kahi o 90% e ola ana ma hope o 10 mau makahiki.
- Ma kahi o 85% e ola ana ma hope o 30 mau makahiki.
He nui ka poʻe e ola nei i nā ola olakino ma mua o 50 mau makahiki. Akā ʻo ka mea nui loa, ʻo kēia mau keikiHe mea nui e noho ma lalo o ka nānā ʻana a ke kauka puʻuwai i kou ola a pau. He mea koi ʻia e hana i nā hoʻokolohua a me nā nānā kino maʻamau.
Āhea ʻoe e pono ai e ʻike i ke kauka?
Ke hoʻi ʻoe i ka home ma hope o ke ʻoki ʻana, e hoʻomaopopo koke i kāu kauka inā loaʻa iā ʻoe kekahi o kēia mau hōʻailona:
- Inā e kahe ana ke koko a i ʻole kekahi wai ʻē aʻe mai ka ʻeha ʻoki.
- Inā he kuni a he mau hōʻailona ʻē aʻe paha o ka maʻi (kuke, anu).
ʻOiai he paʻakikī ke kūlana o kāu keiki, mai makaʻu e nīnau i ke kauka no kekahi mea āu e maopopo ʻole ai. ʻO ka loaʻa ʻana o kahi hoʻomaopopo maikaʻi i ke kūlana o kāu keiki e kōkua iā ʻoe e mālama pono i kāu keiki.
Leka Lawe-Home
- ʻAʻole he lāʻau lapaʻau piha ke kaʻina hana Fontan no ka maʻi puʻuwai hoʻokahi-cell. He kaʻina hana koʻikoʻi loa ia e hoʻololi ai i ke kahe o ke koko, e hōʻemi ana i ke kaomi ma ka puʻuwai, a e maʻalahi ai ke ola.
- Hoʻemi kēia ʻoki kino i ka ukana hana ma ke kelepona hoʻokahi o ke keiki a hoʻonui nui i nā pae oxygen o ke kino.
- He mea nui e makaʻala i nā pilikia lōʻihi, e like me ka maʻi ate, hiki ke ulu aʻe i ka hala ʻana o ka manawa.
- ʻO ka mea nui loa: He mea nui e ʻike i kahi kauka cardiologist a hana i nā hoʻokolohua mau ʻana i kou ola a pau ma hope o ke ʻoki ʻana.
- E kamaʻilio ākea me kāu kauka. E kūkākūkā i nā nīnau a hopohopo paha āu e loaʻa ai me lākou. He mea nui loa kou ʻike no ka pono o kāu keiki.
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