E aʻo kākou e pili ana i ka Esophageal Atresia, kahi maʻi laha ʻole kahi i pili ʻole ai ka esophagus o kahi pēpē hānau hou i ka ʻōpū.

Ke hoʻomaka nei kāu pēpē hānau hou e ʻū a e ʻūlū i ka wā e inu ai ʻo ia i ka waiū? Ke huʻa nei kona waha? A i ʻole ke ʻano uliuli o kona ʻili? Ma ke ʻano he makua, he mea maʻamau ka makaʻu a me ka hopohopo ke ʻike ʻoe i kēia mau mea. ʻO nā hōʻailona e like me kēia i kekahi manawa he hōʻailona ia o kahi kīnā hānau kakaikahi akā koʻikoʻi i kapa ʻia ʻo Esophageal Atresia (EA) . Mai makaʻu ke lohe ʻoe i kēia inoa. I kēia lā, e kamaʻilio mākou e pili ana i nā mea āpau ma ke ʻano maʻalahi loa hiki iā ʻoe ke hoʻomaopopo.

He aha ka Esophageal Atresia (EA)?

I ka ʻōlelo maʻalahi, he kīnā hānau ka Esophageal Atresia. Hana ʻia ia i ka wā ʻaʻole i hana piha ʻia ka esophagus o kāu pēpē, ʻo ia hoʻi ka ʻōmole e lawe ana i ka meaʻai mai ka waha a i ka ʻōpū. ʻO ke ʻano o ka huaʻōlelo "atresia" ua ālai ʻia a i ʻole ua ālai ʻia kekahi alahele i loko o ke kino. I kēia kūlana, ua ālai ʻia ka esophagus kahi e pili ai i ka ʻōpū. ʻO ka hopena, ʻaʻohe ala o ka pēpē e ʻai ai a inu paha ma ke ʻano maʻamau.

E noʻonoʻo i kēia, ua like ia me kahi paipu wai e haki ana i waenakonu a hoʻokaʻawale i ʻelua ʻāpana.

Hiki i kēia kūlana ke lilo i mea paʻakikī iki. Ma kahi o 90% o nā pēpē me EA he pilikia ʻē aʻe kekahi. Ua kapa ʻia ʻo ia he tracheoesophageal fistula (TEF) . ʻO kēia ka manawa e pili ai ka esophagus i hana maikaʻi ʻole ʻia i ka windpipe, ka windpipe. He mea weliweli loa kēia no ka mea, hiki i nā mea a ka pēpē e moni ai, ʻoiai ka lawai, ke komo i loko o nā māmā ma kahi o ka ʻōpū. Inā hana ʻia kēia, e paʻakikī i ka pēpē ke hanu, e ʻūlū, a hiki paha ke loaʻa i nā maʻi māmā.

He aha nā ʻano nui o kēia maʻi?

Ua māhele ʻia ka atresia esophageal i kekahi mau ʻano nui. Ua hoʻokaʻawale ʻia kēia mau mea ma muli o kahi i ālai ʻia ai ka esophagus a inā a pehea e pili ai i ka windpipe. Hiki ke paʻakikī iki kēia, akā i mea e maʻalahi ai ke hoʻomaopopo, e nānā i ka papa ma lalo.

E hana nā kauka i nā hoʻokolohua like ʻole e hoʻoholo ai i ke ʻano o kēia mau ʻano o kāu pēpē. Hoʻolālā ʻia ka lāʻau lapaʻau e like me ia.

He aha nā hōʻailona e hōʻike ana ua loaʻa i ka pēpē kēia maʻi?

ʻEkolu mau hōʻailona nui a nā kauka a me nā kahu maʻi e hoʻohana ai e ʻike koke i kēia maʻi. Ua kapa ʻia hoʻi lākou ʻo "ʻEkolu C."

• ʻŪhū
• Paʻa (ʻūʻū)
• Cyanosis (ʻuliuli ke kala ʻana o ka ʻili)

ʻO ka cyanosis ka wā e polū ai ka ʻili, ʻoiai nā lehelehe a me nā manamana lima, ke loaʻa ʻole i ke kino ka oxygen lawa.

Ma waho aʻe o kēia mau mea nui, hiki iā ʻoe ke ʻike i nā mea ʻē aʻe:

• Ua uhi ʻia ka waha o ke pēpē i ka nui o ka huʻa.
• Ke kahe ʻana o ka wai ma mua o ka maʻamau a i ʻole ka luaʻi ʻana i ka waiū.
• Ke hoʻāʻo nei au e hāʻawi iā ia i ka waiū, ua luaʻi ʻo ia a ʻūʻū.
• Ke paʻakikī nei ka hanu ʻana, a aia kekahi leo ʻē i kou hanu ʻana.

No ke aha e hana ai kēia i nā pēpē? He aha ke kumu nui?

Ua kapa mākou i kēia he kīnā hānau. Hoʻokumu ʻia ia e kahi loli e hana ʻia ana i ka wā e ulu ana ka pēpē i loko o ka ʻōpū. ʻO ka maʻamau, i nā pae mua o ka fetus, ʻo ka esophagus a me ka trachea he ʻōmole hoʻokahi. Ma hope mai, māhele ʻia i ʻelua ʻāpana a ulu i ʻelua mau ʻōmole kaʻawale. ʻO ke kumu nui o ka ulu ʻana o ka Esophageal Atresia ʻo ia ka hoʻokaʻawale ʻana o ke ʻano o hoʻokahi ʻōmole a hoʻōki i ka ulu ʻana.

Eia nō naʻe, ʻaʻole i loaʻa i nā kānaka ʻepekema kahi pane paʻa i ka nīnau, "No ke aha e pau ai kēlā ulu ʻana i ka hapalua?" Manaʻo lākou he hopena paha nā kumu hoʻoilina a me nā kumu kaiapuni i kēia. ʻO ia hoʻi, ʻo kekahi mau loli e kū mai ana i ka DNA o ka pēpē a me kekahi mau mana kaiapuni e kū nei i ka makuahine i ka wā hāpai paha ke kumu.

Aia kekahi mau mea pilikia?

ʻOiai ʻaʻohe kumu pololei i hōʻoia ʻia o ke ʻano, ua ʻike nā kānaka noiʻi i kekahi mau ʻano maʻamau o nā pēpē i hānau ʻia me ka EA. Hiki i kēia ke hoʻonui iki i ka pilikia:

• ʻOi aku ka nui o nā makahiki o ka makuahine ma mua o 35 a/a i ʻole ʻoi aku ka nui o nā makahiki o ka makuakāne ma mua o 40.
• ʻO ka hānau ʻana o nā keiki ma o nā hana insemination artificial (e like me IVF, IUI).
• He mau māhoe a he mau māhoe ʻekolu paha.

Eia kekahi, ʻike pinepine ʻia ka EA me nā kīnā hānau ʻē aʻe a me nā maʻi genetic. Eia kekahi laʻana:

• Trisomy (13, 18 a i ʻole 21)
• ʻAhahui VACTERL
• ʻO ka maʻi CHARGE
• Maʻi puʻuwai hānau
• Nā mea keakea ʻē aʻe o ka ʻōnaehana hoʻoheheʻe (GI atresias)
• Nā hemahema o ka ʻōnaehana puʻupaʻa a me ka genitourinary

Pehea e ʻike ai nā kauka i kēia maʻi? (Diagnosis)

ʻO ka hapa nui o ka manawa, ʻike ʻia kēia maʻi ma hope o ka hānau ʻana o ka pēpē. Eia nō naʻe, i kekahi manawa, hiki i nā scan prenatal ke hāʻawi i nā hōʻailona.

• Ma mua o ka hānau ʻana o ka pēpē: Inā i ka wā o kāu scan anatomy ma kahi o 20 mau pule, ʻoi aku ka nui o ka wai amniotic a puni ka pēpē ma mua o ka maʻamau (ua kapa ʻia kēia he polyhydramnios ), kānalua paha ke kauka. ʻO kēia no ka mea maʻamau ka ale ʻana o ka pēpē i kēia nui o ka wai. Inā ʻaʻole hiki i ka pēpē ke ale, hōʻiliʻili ka wai. Eia kekahi, inā ʻike ʻia ka ʻōpū o ka pēpē he hakahaka (me ka liʻiliʻi a ʻaʻohe pehu o ka ʻōpū) i ka wā o ke scan, he hōʻailona ʻē aʻe kēlā.
• Ma hope o ka hānau ʻana o ka pēpē: Inā hōʻike ka pēpē i nā hōʻailona a mākou i kūkākūkā ai ma mua (ʻū, ʻūlū, polū) ma hope koke o ka hānau ʻana, kānalua koke nā kauka i kēia. ʻO ka hoʻāʻo maʻalahi loa e hōʻoia i kēia, ʻo ia ke hoʻokomo i kahi ʻōmole lahilahi (ʻōmole nasogastric) ma o ka ihu a i ʻole ka waha o ka pēpē a hoʻāʻo e hoʻokomo i loko o ka ʻōpū. Inā paʻa ka esophagus, ʻaʻole hiki i ka ʻōmole ke komo i loko o ka ʻōpū. Hoʻōki ia i ka hapalua.

A laila, hana ʻia kahi X-ray e hōʻoia i kēia a e ʻike pono i ke ʻano o ka hemahema o ka esophagus. Hiki i ka X-ray ke ʻike inā ua komo ka wai i loko o nā māmā a i ʻole ua komo ka ea i loko o ka ʻōpū. Ma hope o kēia hōʻoia ʻana, e nānā pū ke kauka i ka pēpē no nā hemahema hānau ʻē aʻe e pili ana.

Pehea e mālama ʻia ai? Hiki ke ho'ōla ʻia kēia?

ʻAe! He maʻi hiki ke ho'ōla ʻia kēia. ʻO ke ʻoki kino wale nō ka lāʻau lapaʻau no kēia.ʻO ka hapa nui o ka manawa, hana ʻia kēia ʻoki ma hope koke iho o ka hānau ʻana o ke pēpē.

Ka hoʻokele ma mua o ke ʻoki ʻana

Ma mua o ka hana ʻana i ke ʻoki kino, hana nā kauka i kekahi mau hana e hoʻopaʻa ai i ke kūlana o ka pēpē.

• Ke hoʻopaʻa ʻana i ka hanu ʻana: I mea e pale ai i ka hōʻiliʻili ʻana o ka mucus a me ka phlegm i loko o ka puʻu mai ka hiki ʻana i nā māmā, wehe pinepine ʻia lākou me kahi ʻōmole (suction). Inā paʻakikī ka pēpē i ka hanu ʻana, ua hoʻopili ʻia lākou i kahi ventilator.
• Ka hāʻawi ʻana i ka meaʻai palekana: ʻOiai ʻaʻole hiki ke hānai ʻia ka pēpē ma ka waha, hāʻawi ʻia ka meaʻai e pono ai ma o kahi aʻa ( ʻai parenteral ) a i ʻole ma o kahi paipu i kau pololei ʻia i loko o ka ʻōpū ( ʻai enteral ).
• Ke pale ʻana i nā maʻi: Hāʻawi ʻia nā lāʻau antibiotic IV e pale aku i nā maʻi maʻi māmā (pneumonia).

Inā hānau mua ʻia ke pēpē, kaumaha ʻole, a i ʻole he mau kīnā koʻikoʻi ʻē aʻe, e like me ke kīnā puʻuwai, pono ke pēpē e noho i loko o ka lumi mālama koʻikoʻi neonatal (NICU) a hiki i ka paʻa ʻana o kēlā mau kūlana ma mua o ke ʻoki ʻana.

ʻoki kino (Hoʻoponopono ʻoki kino)

Ke hoʻoholo ʻia ua mākaukau ka pēpē no ke ʻoki ʻana, hana ke kauka ʻoki i ke ʻoki ʻana. ʻElua mau pahuhopu nui o ke ʻoki ʻana:

1. Hoʻohui i ʻelua mau ʻāpana i hoʻokaʻawale ʻia o ka esophagus (ua kapa ʻia kēia he anastomosis ).

2. Ke pani nei i ka pilina pono ʻole (fistula) ma waena o ka pharynx a me ka trachea.

Me ka ʻenehana holomua o kēia wā, hana pinepine ʻia kēia ʻoki kino me ka wehe ʻole ʻana i ka umauma, akā ma ke komo ʻana i kahi kāmela a me nā mea kani ma o kekahi mau ʻoki liʻiliʻi loa (ʻoki kino thoracoscopic). Hoʻemi kēia i ka ʻeha no ka pēpē a hopena i ka hoʻōla wikiwiki ʻana.

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

Ma hope o ke ʻoki ʻana, lawe ʻia ke pēpē i ka NICU no ka nānā pono ʻana. I kekahi mau lā ma hope mai, hana ʻia kahi X-ray kūikawā i kapa ʻia he esophagram e nānā inā ua hoʻōla pono ʻia ka ʻoki a inā he mau leaka. Ke hōʻoia ʻia nā mea āpau, hoʻolauna mālie ʻia ke pēpē i ka ʻōnaehana hānai waha. He manawa iki ka manawa e maʻa ai ke pēpē i ke kaʻina hana ale.

He aha nā pilikia lōʻihi e hiki ke kū mai ma hope o ke ʻoki kino?

Hoʻōla piha ka hapa nui o nā pēpē a ola i ke ola maʻamau. Eia nō naʻe, hiki i kekahi mau keiki ke loaʻa i kekahi mau pilikia no kekahi manawa ma hope o ke ʻoki ʻana. He mea nui e makaʻala i kēia mau mea.

• Nā pilikia ale: ʻAʻole paha e hana pono nā ʻiʻo o ko lākou puʻu i kekahi mau keiki. Hiki i kēia ke hoʻoulu iā lākou e ʻūlū ke hoʻomaka lākou e ʻai i nā meaʻai paʻa. Pono e kuʻi maikaʻi ʻia ka meaʻai a hāʻawi ʻia me nā wai.
• ʻO ka maʻi Reflux Gastroesophageal (GERD): ʻO kēia ka manawa e holo hou ai ka waikawa o ka ʻōpū i luna o ka esophagus. Hiki i kēia ke hana i ka ʻeha puʻuwai a me ka indigestion. Inā ʻaʻole mālama ʻia, hiki iā ia ke hōʻino i ka esophagus. Loaʻa kēia ʻano ma kahi o ka hapalua o nā keiki i hana ʻia ke ʻoki EA.
• ʻO ke kaʻina hana o ka tracheomalacia:He hoʻonāwaliwali kēia o ke cartilage i loko o nā alaea. Hiki i kēia ke alakaʻi i ka wheezing, ka ʻū pinepine, a me nā maʻi māmā pinepine (bronchitis, pneumonia).

Inā loaʻa iā ʻoe kekahi o kēia mau pilikia, mai hopohopo. Aia nā lāʻau lapaʻau no kēia mau mea a pau. ʻO ka mea nui loa, ʻo ia ke kamaʻilio mau me kāu kauka e pili ana i ia mea a hahai i kāna mau kuhikuhi. Pono paha ʻoe e ʻimi i ke kōkua mai kahi Speech-Language Pathologist (SLP).