Pono anei kāu pēpē e hana i ke Kaʻina Hana Norwood? E kamaʻilio maʻalahi kākou e pili ana.

Pehea kou makaʻu a me kou haʻalulu i ka wā i haʻi aku ai nā kauka iā ʻoe he maʻi puʻuwai hānau paʻakikī ko kāu keiki liʻiliʻi? I ka wā i haʻi aku ai lākou iā ʻoe e pono ʻoe e hana i kahi 'Norwood Procedure', ʻaʻole paha ʻoe i maopopo i ka huaʻōlelo. He mea maʻamau loa ia. I ka manawa e like me kēia, he tausani mau nīnau e hiki mai ana i ka noʻonoʻo. Mai hopohopo, e kamaʻilio maʻalahi mākou e pili ana i kēia, ma ke ʻano hiki iā ʻoe ke hoʻomaopopo.

He aha ke Kaʻina Hana Norwood?

I ka ʻōlelo maʻalahi, ʻo ke kaʻina hana Norwood ka hana mua a koʻikoʻi loa i hana ʻia e hoʻopakele i ke ola o nā pēpē i hānau ʻia me kahi kīnā puʻuwai paʻakikī loa. Kāhea mākou i kēia maʻi puʻuwai ʻo Hypoplastic Left Heart Syndrome (HLHS) .

E nānā, he ʻehā keʻena nui o ka puʻuwai olakino. ʻO ka ʻaoʻao ʻākau ke kuleana no ka hoʻouna ʻana i ke koko nele i ka oxygen o ke kino i nā māmā. ʻO ke koko waiwai i ka oxygen mai nā māmā e hele mai i ka ʻaoʻao hema o ka puʻuwai. ʻO ka ʻaoʻao hema ke kuleana no ka pamu ʻana i kēia koko maʻemaʻe waiwai i ka oxygen i ke kino holoʻokoʻa.

Eia nō naʻe, ʻaʻole i ulu piha ka ʻaoʻao hema o ka puʻuwai o kahi pēpē me HLHS. ʻO ke ʻano kēia, ʻaʻole hana ka ʻāpana nui e pamu ana i ke koko maʻemaʻe i ke kino. No laila he paʻakikī no ia pēpē ke ola.

ʻO kēia ka manawa e hana ai nā kauka i ke kaʻina hana Norwood. ʻAe kēia kaʻina hana i ka ʻaoʻao ʻākau o ka puʻuwai o ka pēpē, ka mea e hana maikaʻi nei, e hana i ka hana a ka ʻaoʻao hema e hana ai. ʻO ia hoʻi, ua hoʻololi ʻia ke kahe koko o ka puʻuwai i hiki ai i ka ventricle ʻākau ke pamu i ke koko i nā māmā, ʻoiai i ka manawa like e pamu ana i ke koko oxygenated i ke kino holoʻokoʻa.

ʻO wai ka mea e pono ai kēia ʻoki kino?

ʻO ka mea nui e pono ai kēia ʻoki kino no nā pēpē me ke kūlana HLHS a mākou i kamaʻilio ai ma mua. Pono kēia mau pēpē i kēia ʻoki kino i loko o nā pule ʻelua mua o ka hānau ʻana.

E noʻonoʻo, i ka wā e noho ana ke pēpē i loko o ka ʻōpū, ʻaʻole pilikia kēia nāwaliwali o ka puʻuwai no ka mea loaʻa iā ia ka oxygen mai ke kaula umbilical. ʻOiai ma hope o ka hānau ʻana, no nā lā mua, ua wehe ʻia kahi ʻāpana liʻiliʻi e like me ka paipu o ka puʻuwai ma waena o nā kīʻaha koko nui (aorta a me ka pulmonary artery) (patent ductus arteriosus). ʻAe kēia i kekahi koko e kahe i ke kino.

Eia nō naʻe, pani maoli kēia ʻōmole i loko o kekahi mau lā. ʻO ia ka manawa e paʻakikī ai ka pēpē i ka hanu ʻana, a ua pilikia nui kona ola. No laila, hāʻawi nā kauka iā ia i kahi lāʻau lapaʻau kūikawā e pale aku ai i ka pani ʻana o ka ʻōmole. I kēia manawa, ua mākaukau ka pēpē no kēia hana Norwood.

I kekahi manawa, no nā pēpē i hānau mua ʻole ʻia, he kaumaha haʻahaʻa i ka wā hānau, a i ʻole he mau pilikia olakino koʻikoʻi ʻē aʻe, ʻaʻole hiki ke hana koke ʻia kēia ʻoki kino. I ia mau hihia, hiki i nā kauka ke hoʻohana i nā ʻano hana manawa pōkole ʻē aʻe.

He aha ka mea e hana ʻia i ka wā o ke ʻoki kino?

He hana ʻoki kino paʻakikī loa kēia e lawe ana i kekahi mau hola, i kekahi manawa hoʻokahi lā. Eia ka mea a ke kauka e hana ai ma nā ʻōlelo maʻalahi:

1. Ke hoʻonui nei i kahi lua ma waena o nā keʻena o luna (atria) o ka puʻuwai: Kōkua kēia i ke koko i piha i ka oxygen mai nā māmā e kahe maʻalahi i ka ʻaoʻao ʻākau o ka puʻuwai.

2. Ke kūkulu hou ʻana o ke aʻa nui (aorta): Hoʻopili ʻia ka aorta liʻiliʻi a ulu ʻole o ka pēpē i ke aʻa pulmonary, ke aʻa e lawe ana i ke koko i nā māmā, a ua hoʻokomo ʻia kahi ʻāpana kūikawā e hana i kahi kīʻaha koko hoʻokahi, nui a ikaika.

3. Hoʻohui i ke aʻa hou i ka ventricle ʻākau: I kēia manawa ua pili kēia aorta nui i hana hou ʻia i ka ventricle ʻākau, ʻo ia wale nō ka ʻāpana hana o ka puʻuwai. I kēia manawa hiki i ka ʻaoʻao ʻākau ke pamu i ke koko i ke kino holoʻokoʻa.

4. Ke kau ʻana i kahi shunt e hoʻouna i ke koko i nā māmā: ʻOiai ke hele nei ke koko āpau ma o ka aorta hou i ke kino, pono e hana ʻia kahi ala kaʻawale e hoʻouna i ke koko i nā māmā. Hana ʻia kēia me ka hoʻohana ʻana i kahi ʻōmole liʻiliʻi i kapa ʻia he shunt. Hoʻopili ʻia kēia mai ka aorta hou i nā aʻa pulmonary (Blalock-Taussig a i ʻole BT shunt) a i ʻole mai ka ventricle ʻākau i nā aʻa pulmonary (Sano shunt).

I loko o kēia ʻoki kino holoʻokoʻa, hoʻokele ʻia ka puʻuwai a me nā māmā o ka pēpē e kahi mīkini kūikawā i kapa ʻia ʻo cardiopulmonary bypass .

He aha ka mea e hana ʻia ma hope o ke ʻoki kino?

Ma hope o ke ʻoki kino, e hoʻokomo ʻia kāu pēpē i ka Intensive Care Unit (ICU) , kahi e mālama ai nā kauka a me nā kahu maʻi i hoʻomaʻamaʻa kūikawā ʻia i kāu pēpē 24 mau hola o ka lā.

E ʻike ʻoe he nui nā paipu a me nā uea i hoʻopili ʻia i ke kino o ka pēpē. Hoʻohana ʻia lākou e hāʻawi i ka pēpē i ka lāʻau lapaʻau e pono ai, ka oxygen, ka meaʻai, a me ke ana mau ʻana i nā hana o ke kino. Mai makaʻu e ʻike iā lākou , aia lākou a pau no ka pono o ka pēpē.

ʻOiai ma hope o ke ʻoki ʻana, e like me kā mākou i kūkākūkā ai ma mua, hiki i ka ʻili a me nā lehelehe o ke pēpē ke loaʻa kahi ʻano polū iki ma muli o ka hui ʻana o ke koko oxygen kiʻekiʻe a me ke koko oxygen haʻahaʻa. He mea maʻamau kēia i kēia manawa.

Ma hope o hoʻokahi pule ma ke keʻena mālama koʻikoʻi, ke hoʻomaikaʻi ke kūlana o ka pēpē, e hoʻoneʻe ʻia ʻoe i kahi keʻena maʻamau. Ma mua o kou hoʻi ʻana i ka home, e hāʻawi nā kauka a me nā kahu maʻi iā ʻoe i ka wehewehe piha ʻana i ke ʻano o ka mālama ʻana i kāu pēpē, pehea e hāʻawi ai iā ia i ka lāʻau lapaʻau, a me nā hōʻailona e makaʻala ai.

ʻO ka moʻo ʻokiʻoki piha no ke kūlana HLHS

ʻO ke kaʻina hana ʻo Norwood ka hana mua wale nō ia o kēia huakaʻi. I mea e loaʻa ai i kahi pēpē me HLHS kahi ola maʻamau, pono lākou e hana i kekahi mau ʻoki ʻana ʻekolu.

He aha nā pilikia a me nā pilikia e hiki mai ana?

E like me nā ʻoki kino nui a pau, loaʻa i ke kaʻina hana Norwood kekahi mau pilikia. E wehewehe kāu kauka i kēia mau mea iā ʻoe. ʻO nā pilikia nui e hiki ke hana ʻia penei:

• Ka hoʻohaiki ʻana o nā kīʻaha koko: Ma hope o ke ʻoki ʻana, hiki ke hoʻohaiki ʻia ka aorta i hana hou ʻia a i ʻole ke aʻa e lawe ana i ke koko i nā māmā (stenosis).
• Nā pilikia shunt: ʻO ka shunt e hoʻouna ana i ke koko i nā māmā e ālai ʻia a hana pono ʻole paha.
• Puʻuwai puʻuwai ʻē aʻe: Hiki ke loli ke ʻano o ka puʻuwai (arrhythmia).
• Ka nāwaliwali o ka hana a ka puʻuwai: Hiki ke hoʻopilikia ʻia ka hiki ke pamu o ka puʻuwai a i ʻole ka hana o kona mau kiwikā.
• Nā lohi o ka neurodevelopmental: Aia kahi manawa liʻiliʻi e hoʻopilikia ʻia ka ulu ʻana o ka lolo, e alakaʻi ana i nā pilikia aʻo a i ʻole ka hana (Neurodevelopmental delay).

Pehea nā hopena a me ke ola?

ʻO kēia ka nīnau nui loa a kēlā me kēia mākua. ʻO ka ʻoiaʻiʻo, ma mua o ka ʻike ʻia ʻana o kēia ʻoki kino i nā makahiki 1980, ʻaʻohe lāʻau lapaʻau no nā pēpē i hānau ʻia me HLHS. ʻO ke kaʻina hana Norwood kahi manawa kūpono no kēia mau pēpē e ola.

Wahi a nā helu helu, ʻo 90% ka manawa kūpono e ola ai ma ka haukapila ma hope o kēia ʻoki kino.ʻO ka nui o ke ola ʻana no ʻelima mau makahiki ma hope o ke ʻoki ʻana ma waena o 60% a me 75%. Minamina, make koke kekahi mau pēpē ma mua o ka hiki ʻana i ke kahua o ke ʻoki ʻana ʻelua.

Eia nō naʻe, inā e holomua nā ʻoki ʻekolu, hiki i kēia mau keiki ke ulu a lilo i poʻe mākua ʻōpio. E loaʻa iā lākou ka manawa kūpono e hele i ke kula, pāʻani, a ola i kahi ola maʻamau.

Āhea wau e ʻike ai i ke kauka?

Ma hope o ka lawe ʻana mai i kāu pēpē i ka home, he kuleana nui kou. Pono ʻoe e makaʻala loa i nā mea aʻe. Inā ʻike ʻoe i kekahi mea e like me kēia, e kāhea koke aku i kāu kauka.

• Inā ʻaʻole e piʻi pono ke kaumaha o ka pēpē.
• Inā hāʻule ka pae oxygen o kāu pēpē ma lalo o ka pae i haʻi ʻia e kāu kauka (e hāʻawi ʻia ʻoe i kahi mea hana e ana i ka oxygen ma ka home).
• Inā ʻaʻole pane maikaʻi ka pēpē i ka lāʻau lapaʻau i kuhikuhi ʻia.
• Inā he mau nīnau a kānalua paha kāu e pili ana i ke ʻano e mālama ai i kāu pēpē.

No ka mea, he maʻi laha ʻole ka HLHS, he mea nui loa ke koho ʻana i ke kauka akamai a me ka haukapila e hana pinepine ana i kēia ʻano hana ʻoki kino. Mai makaʻu a hilahila paha e nīnau pinepine i kāu kauka e pili ana i nā mea āu e maopopo ʻole ai.