Loaʻa i kāu pēpē ka HLHS? E aʻo kākou e pili ana i ke Kaʻina Hana Norwood ma nā ʻōlelo maʻalahi

Ma ke ʻano he makua, maopopo iaʻu ke kaumaha a me ka makaʻu āu e manaʻo ai ke haʻi aku kāu kauka iā ʻoe he kīnā puʻuwai ko kāu pēpē hānau hou i kapa ʻia ʻo Hypoplastic Left Heart Syndrome (HLHS). Me he mea lā ua hiolo koke kou honua holoʻokoʻa. Akā, mai hopohopo. I kēia lā, ua holomua nui ka ʻepekema lapaʻau. ʻO ke Kaʻina Hana Norwood kahi hana mua koʻikoʻi loa i ka hāʻawi ʻana i ke ola i kēlā mau pēpē. I kēia lā, e kamaʻilio mākou e pili ana i kēia ma ke ʻano maʻalahi i hiki iā ʻoe ke hoʻomaopopo.

He aha ke Kaʻina Hana Norwood?

I ka ʻōlelo maʻalahi, ʻo ke kaʻina hana ʻo Norwood kahi ʻoki puʻuwai kūikawā i hana ʻia ma nā pēpē me kahi maʻi puʻuwai hānau (hānau ʻia me) i kapa ʻia ʻo Hypoplastic Left Heart Syndrome (HLHS) .

E noʻonoʻo e like me kahi hale liʻiliʻi me ʻehā lumi. He ʻelua ona ʻāpana, ʻo ka ʻaoʻao ʻākau a me ka ʻaoʻao hema. ʻO ka maʻamau, ʻo ka ʻaoʻao hema o ka puʻuwai e pamu i ke koko maʻemaʻe a waiwai i ka oxygen i ke kino holoʻokoʻa. ʻO ka ʻaoʻao ʻākau e pamu i ke koko deoxygenated mai ke kino i hope i nā māmā e loaʻa ka oxygen.

Eia nō naʻe, ʻaʻole e ulu pono ka ʻaoʻao hema o ka puʻuwai o kahi pēpē me HLHS . ʻO ia hoʻi, he liʻiliʻi loa a nāwaliwali kēlā ʻaoʻao. No laila, ʻaʻole hiki i kēlā ʻaoʻao ke pamu i ke koko i ke kino. ʻO kēia ka pilikia nui loa.

Hana ke kaʻina hana ʻo Norwood i kahi mea kupaianaha. Hana nā kauka i nā ala hou no ke kahe ʻana o ke koko ma o ka puʻuwai. ʻO ia hoʻi, hoʻohuli lākou i ka hana a ka ʻaoʻao hema o ka puʻuwai e pono ai e hana i ka ʻaoʻao ʻākau, kahi e hana maikaʻi nei . Ma hope o ke ʻoki ʻana, hoʻomaka ka ventricle ʻākau o ka pēpē e pamu i ke koko oxygenated i ke kino holoʻokoʻa, a me ka hoʻouna ʻana i ke koko i nā māmā.

ʻO wai ka mea e pono ai kēia ʻoki kino?

ʻO ka mea nui e pono ai kēia ʻoki kino no nā pēpē me ka Hypoplastic Left Heart Syndrome (HLHS) . Pono lākou e hana i kēia ʻoki kino i loko o nā pule ʻelua mua o ka hānau ʻana.

I ka wā hānau, loaʻa i kēlā me kēia pēpē kahi pilina liʻiliʻi ma waena o nā kīʻaha koko nui ʻelua i loko o ka puʻuwai (aorta a me ke aʻa pulmonary). Ua kapa ʻia kēia he 'patent ductus arteriosus'. ʻAe kēia i kahi pēpē me HLHS e loaʻa ke koko i ko lākou kino no nā lā mua. Eia naʻe, e pani maoli kēia pilina ma hope o kekahi mau lā. Inā hana ʻia kēia, ʻaʻohe ala o ka pēpē e loaʻa ai ke koko i ko lākou kino, he mea weliweli loa ia.

No laila, ʻo ka mea mua a nā kauka e hana ai, ʻo ia ke hāʻawi ʻana i kahi lāʻau lapaʻau kūikawā (i kapa ʻia ʻo `Prostaglandin`) e pale aku i ke kīʻaha koko mai ka pani ʻana. Akā, he manawa pōkole wale nō ia. Hana ʻia ke ʻoki ʻana ʻo Norwood ma ke ʻano he hopena mau loa.

I kekahi manawa, ʻaʻole hiki ke hana ʻia kēia ʻoki kino no nā pēpē i hānau mua ʻia, he haʻahaʻa ke kaumaha hānau, a i ʻole he mau pilikia ʻē aʻe. I ia mau hihia, hoʻohana nā kauka i nā ʻano hana ʻē aʻe, e like me nā kāʻei aʻa pulmonary.

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

Ma mua a i ka wā o ke ʻoki kino

Ma mua o ke ʻoki ʻana, aia ke pēpē i loko o ka ʻāpana mālama koʻikoʻi (ICU). E like me ka mea i ʻōlelo ʻia ma mua, e hoʻomau lākou i ka hāʻawi ʻana i ka lāʻau lapaʻau e mālama i ke kīʻaha koko kūikawā e wehe. Pono paha e hoʻonui i ka lua ma waena o nā keʻena ʻelua o luna o ka puʻuwai (kahi `septostomy`).

He hana paʻakikī loa ke kaʻina hana Norwood, he nui nā hola (ʻo ka maʻamau he 8-10 hola). Hana pono ke kime o nā kauka i kēia mau mea:

• Hana ʻia kahi lua ma waena o nā keʻena ʻelua o luna (atria) o ka puʻuwai e ʻae i ke koko e kahe.
• Hoʻopili ʻia ka aorta liʻiliʻi i ke aʻa pulmonary nui, a ua hoʻokomo ʻia kahi ʻāpana kūikawā e hana i kahi aorta hou a nui aʻe.
• Hoʻopili ʻia kēia aorta hou i ka ventricle ʻākau e hana ana.
• ʻO ka mea aʻe, ua hoʻokomo ʻia kahi 'shunt' , kahi ʻōmole hana liʻiliʻi, e hoʻouna i ke koko i nā māmā. Hiki i kēia ke hele mai ka aorta a i nā māmā (Blalock-Taussig shunt) a i ʻole mai ka ventricle ʻākau a i nā māmā (Sano shunt).

Ma loko o kēia ʻoki kino, ua hoʻopili ʻia ka pēpē i kahi mīkini bypass cardiopulmonary . ʻO ke ʻano kēia, ke hana ʻia nei ka puʻuwai a me nā māmā o ka pēpē e kēia mīkini no kekahi manawa.

Ma hope o ke ʻoki kino

Ma hope o ke ʻoki ʻana, hoʻihoʻi ʻia ke pēpē i ka ʻāpana mālama koʻikoʻi (ICU). Ke hele ʻoe e ʻike i ke pēpē, e ʻike ʻoe i ka nui o nā paipu, nā uea, a me nā mīkini. Mai makaʻu ke ʻike ʻoe iā lākou. Aia lākou a pau ma laila no ka pono o ke pēpē, e kōkua i ke pēpē . E ʻike ʻoe i kahi kāʻei e uhi ana i ka ʻeha ʻoki ma waenakonu o ka umauma.

ʻOiai ma hope o kēia ʻoki kino, hiki i ka ʻili o ke pēpē ke loaʻa kahi ʻulaʻula iki (cyanosis) . ʻO kēia no ka mea ke hui pū nei kekahi koko deoxygenated a me ke koko oxygenated i loko o ka puʻuwai. He mea maʻamau kēia i kēia pae.

Ma hope o hoʻokahi pule ma ke keʻena mālama koʻikoʻi, e hoʻoneʻe ʻia ke pēpē i kahi keʻena maʻamau. Pono ʻoe e noho ma ka haukapila no 2-3 mau pule e hoʻōla piha ai. Ma hope o kou hoʻi ʻana i ka home, e hāʻawi nā kauka a me nā kahu maʻi iā ʻoe i nā ʻōlelo kikoʻī e pili ana i ka mālama ʻana i ke pēpē, pehea e hāʻawi ai iā ia i ka lāʻau lapaʻau, a pehea e nānā ai i kona pae oxygen.

Nā ʻoki kino no HLHS

ʻAʻole pau ka HLHS me hoʻokahi wale nō ʻoki kino. He ʻekolu kahua ia o nā ʻoki kino . ʻO Norwood wale nō ke kahua mua.

He aha nā pono a me nā pilikia?

Nā Pōmaikaʻi

ʻO ka pōmaikaʻi nui loa a hoʻokahi wale nō o kēia, ʻo ia ka loaʻa ʻana o ka manawa kūpono i ka pēpē e ola ai . Ma mua o nā makahiki 1980, ʻaʻohe lāʻau lapaʻau no nā pēpē i hānau ʻia me HLHS. Akā mai ka wā i hana ʻia ai ke kaʻina hana Norwood, ua loaʻa i kēia mau pēpē ka manawa kūpono e ola ai i ka wā kamaliʻi a me ka wā ʻōpio.

Nā Pōʻino a me nā Pilikia

E like me nā hana ʻoki kino nui a pau, aia nā pilikia e pili ana. He mea nui e makaʻala i kēia mau mea.

• Ka pale ʻana o ka shunt a i ʻole ka hana ʻole: Hiki ke ālai ʻia ka ʻōmole liʻiliʻi e lawe ana i ke koko i nā māmā.
• Ka hāiki ʻana o nā kīʻaha koko (Stenosis): Hiki ke hāiki ka aorta i wehe ʻia e ke ʻoki ʻana a i ʻole nā ​​kīʻaha koko e alakaʻi ana i nā māmā.
• Nā ʻano ʻino o ka puʻuwai (Arrhythmia): Hiki ke lilo ke ʻano o ka puʻuwai i mea kūpono ʻole.
• Ka nāwaliwali o ka hana a ka puʻuwai: Ma muli o ke kaumaha o ka ventricle ʻākau, hiki ke nāwaliwali kāna hana.
• Nā lohi o ka neurodevelopmental: Hiki ke lohi kekahi mau lohi i ka ulu ʻana o ka lolo a me ka ʻōnaehana nerve. ʻO ka hopena, pono paha kekahi mau pēpē i ke kōkua kūikawā me nā mea e like me ke kamaʻilio ʻana a me ka hele wāwae ʻana.

ʻOi aku ka kiʻekiʻe o ka nui o ke ola ʻana ma ka haukapila ma hope o ke kaʻina hana Norwood ma kahi o 90%. Eia nō naʻe, aia kekahi mau hihia kahi i hiki ʻole ai kekahi mau pēpē i ke ʻoki lua.

Āhea ʻoe e ʻike ai i kāu kauka?

Pono ʻoe e makaʻala pono i kāu pēpē ma hope o kou hoʻi ʻana i ka home. Inā he mau hopohopo kāu, e kāhea koke aku i kāu kauka.E makaʻala loa i kēia mau mea:

• Inā he mau nīnau kāu e pili ana i ka mālama ʻana i kāu pēpē ma ka home.
• Inā ʻaʻole e piʻi pono ke kaumaha o ka pēpē.
• Inā haʻahaʻa ka pae oxygen o ka pēpē ma mua o ka mea a ke kauka i ʻōlelo ai.
• Inā ʻaʻole pane maikaʻi ka pēpē i ka lāʻau lapaʻau i kuhikuhi ʻia.
• Inā ʻoi aku ka hiamoe o ka pēpē ma mua o ka maʻamau a i ʻole ua emi ka nui o ka waiū i lawe ʻia.