Ua ʻike anei ʻoe i kēia maʻi laha ʻole i loko o ka puʻuwai o kāu keiki liʻiliʻi? (Pulmonary Atresia)

ʻAʻohe mea ʻoi aku ka hauʻoli ma mua o ka nānā ʻana i kahi pēpē hānau hou. Akā i kekahi manawa, ke hoʻomaka kēlā mea liʻiliʻi e uē i ka wā e omo ana i ka waiū, a i ka wā e lilo koke ai ʻo ia i polū, makaʻu nā makuahine a me nā makuakāne a pau. I kēia lā e kamaʻilio mākou e pili ana i kahi maʻi kakaikahi akā koʻikoʻi o ka puʻuwai i loaʻa i ka wā hānau, kahi e hōʻike ana i kēlā mau hōʻailona. I ka lāʻau lapaʻau, kapa mākou i kēia ʻo Pulmonary Atresia . Mai hopohopo ke lohe ʻoe i kēia inoa. Me nā lāʻau lapaʻau holomua i loaʻa i kēia lā, hiki ke mālama pono ʻia kēia ʻano. E hoʻomaopopo pono kākou i kēia mai ka hoʻomaka ʻana.

I ka ʻōlelo maʻalahi, he aha ka Pulmonary Atresia?

I mea e hoʻomaopopo ai i kēia, e hoʻomanaʻo mua kākou pehea e hana ai ko kākou puʻuwai. ʻEhā mau keʻena nui o ko kākou puʻuwai. ʻO ke keʻena haʻahaʻa ma ka ʻākau (ventricle ʻākau) e hoʻopuka i ke koko haumia a nele i ka oxygen i nā māmā e lawe i ka oxygen. ʻO ke aʻa koko nui e lawe ana i kēia koko i nā māmā, ʻo ia ke aʻa pulmonary.

E noʻonoʻo, aia kahi ʻāpana e like me ka puka ma waena o ka ventricle ʻākau o ka puʻuwai a me kēia aʻa pulmonary. Kāhea mākou i kēia ʻo ka valve pulmonary. Wehe a pani kēia valve e hoʻouna i ke koko i nā māmā.

ʻO ka atresia pulmonary kahi kūlana i ulu pono ʻole ai ka valve pulmonary i ka wā e ulu ana ka pēpē i loko o ka ʻōpū. I kekahi manawa, hiki ke uhi piha ʻia kēia valve e kahi membrane mānoanoa. ʻO ke ʻano kēia ua ālai loa ʻia ke ala nui no ke kahe ʻana o ke koko mai ka ventricle ʻākau a i nā māmā.

"Nā ala ʻē aʻe" o ka puʻuwai a me nā ʻano like ʻole o kēia maʻi

Ke ālai ʻia kēia alanui nui, pono i nā māmā ke loaʻa i ke koko e ola ai. ʻO ka mea pōmaikaʻi, ʻo kekahi mau alanui "manawa pōkole" i loko o ka puʻuwai i ka wā hāpai e hana ma ke ʻano he mea hoʻopakele i kēia manawa.

• ʻO Foramen Ovale: He lua liʻiliʻi ma waena o nā keʻena ʻelua o luna o ka puʻuwai. ʻAe kēia i ke koko nele i ka oxygen e kahe mai ka ʻaoʻao ʻākau a i ka hema.
• ʻO Ductus Arteriosus: He pilina liʻiliʻi ma waena o ke aʻa nui e lawe ana i ke koko i ke kino (aorta) a me ke aʻa e lawe ana i ke koko i nā māmā (pulmonary artery). Ma o kēia, e kahe ana kahi koko liʻiliʻi mai ke kino i nā māmā.

He mea maʻamau ka pani ʻana o kēia mau alahele no kekahi mau lā ma hope o ka hānau ʻana. Eia nō naʻe, hiki i kahi pēpē me ka Pulmonary Atresia ke ola wale nō inā e wehe mau ana kēia mau alahele. No laila, i ka wā e hānau ai ke pēpē, e hāʻawi nā kauka i ka pēpē i ka lāʻau lapaʻau e mālama i kēia mau alahele (ʻoi aku ka Ductus Arteriosus).

Hiki iā mākou ke ʻike i ke kūlana o ka Pulmonary Atresia ma nā ʻano nui ʻelua.

1. Atresia Pulmonary me ka Intact Ventricular Septum (IVS): Maanei, ua piha ka paia ma waena o nā keʻena haʻahaʻa ʻelua o ka puʻuwai (septum). ʻAʻohe lua. Eia naʻe, ma muli o kēia, ʻaʻohe wahi o ka ventricle ʻākau e pamu ai i ke koko, a pinepine ʻaʻole ia e ulu pono a he liʻiliʻi.

2. ʻO ka Atresia Pulmonary me ka Ventricular Septal Defect (VSD): Maanei, me ka pani ʻia ʻana o ka valve pulmonary, aia kahi lua ma ka paia ma waena o nā keʻena haʻahaʻa ʻelua o ka puʻuwai (Ventricular Septal Defect - VSD). ʻAe kēia lua i ke koko e kahe mai ke keʻena ʻākau a i ka hema, no laila hiki ke ʻoi aku ka nui o ke keʻena ʻākau ma mua o ka maʻamau.

Hoʻoholo ʻia nā ʻano lapaʻau ma muli o ke ʻano o kēia mau ʻano ʻelua i loaʻa i ka pēpē.

He aha nā hōʻailona e hiki i nā mākua ke nānā?

ʻIke pinepine ʻia kēia mau hōʻailona i loko o nā hola mua a i ʻole nā ​​lā ma hope o ka hānau ʻana o ke pēpē. ʻOiai ua makaʻala nui nā kauka i kēia ma ka haukapila, he mea nui iā ʻoe e makaʻala i kēia ma hope o kou hoʻi ʻana i ka home.

No ke aha i hana ʻia ai kēia i kaʻu pēpē? He aha nā kumu pilikia?

Hiki mai kēia nīnau i ka noʻonoʻo o kēlā me kēia mākua. "Ua hana hewa anei au?" E noʻonoʻo paha ʻoe. ʻO ka mea mua, ʻaʻole i ʻike ʻia ke kumu pololei o kēia. ʻAʻole na ʻoe ia hewa. Hoʻokumu ʻia ia e kahi hana hewa i loko o kahi kaʻina hana paʻakikī loa e hana ʻia i ka wā e hoʻokumu ʻia ai ka puʻuwai o ka pēpē i nā pule 8 mua o ka hāpai ʻana.

Eia nō naʻe, ua ʻike ʻia kekahi mau kumu e hoʻonui iki i ka likelika, a i ʻole ka pilikia, o kēia ʻano hana.

• Nā kumu hoʻoilina: ʻOi aku ka kiʻekiʻe o ka pilikia inā loaʻa i kekahi o ka ʻohana (makuahine, makuakāne) kahi maʻi puʻuwai hānau. Hiki ke pili pū me kekahi mau kūlana hoʻoilina, e like me ka maʻi DiGeorge.
• Ola kino o ka makuahine i ka wā hāpai: Ua loaʻa i ka makuahine ka maʻi diabetes i kāohi ʻole ʻia i ka wā hāpai.
• Kekahi mau lāʻau lapaʻau i lawe ʻia i ka wā hāpai: ʻO ka hoʻohana ʻana i kekahi mau lāʻau lapaʻau hiki ke loaʻa nā hopena maikaʻi ʻole i ka ʻōpū (teratogenic). ʻO ia ke kumu i ʻōlelo ʻia ai ʻaʻole e lawe i kekahi lāʻau lapaʻau i ka wā hāpai me ka ʻole o ke aʻo ʻana a ke kauka.
• Puhipaka: Ka puhipaka ʻana o ka makuahine i ka wā hāpai a ma mua paha.
• Makahiki makuahine: Hiki ke piʻi iki ka pilikia o kēia ʻano kīnā hānau i ka piʻi ʻana o ka makahiki o ka makuahine.

Pehea e ʻike ai nā kauka i kēia?

Me ka ʻenehana o kēia wā, hiki ke ʻike ʻia kēia maʻi ma mua o ka hānau ʻana o ka pēpē, ʻo ia hoʻi, i ka wā hāpai .

• I ka wā hāpai: Inā hōʻike kahi scan ultrasound maʻamau i kekahi mau ʻano ʻē i loko o ka puʻuwai o kāu pēpē, hiki i kāu kauka ke kuhikuhi iā ʻoe i kahi scan kūikawā i kapa ʻia he echocardiogram fetal . Hiki iā ʻoe ke ʻike maopopo i ke ʻano a me ka hana o ka puʻuwai o kāu pēpē.

Ma hope o ka hānau ʻana o ka pēpē, inā lohe ke kauka i kahi ʻōhumu puʻuwai kūpono ʻole a ʻike paha i ka hoʻololi ʻana o ka polū (cyanosis) i ka nānā ʻana i ka pēpē, e hana lākou i kekahi mau hoʻokolohua e hōʻoia i kēia kūlana.

• ʻO ka Pulse Oximetry: He hoʻāʻo maʻalahi loa kēia, ʻaʻohe ʻeha. Hoʻopili ʻia kahi mea liʻiliʻi e like me ke clip i ka manamana wāwae nui o ke pēpē a ana i ka pae o ka oxygen i loko o ke koko. Inā haʻahaʻa ka pae oxygen, he hōʻailona ia o ka pilikia o ka puʻuwai.
• X-ray o ka umauma: Hiki i kahi X-ray o ka umauma ke hāʻawi i kahi manaʻo pakahi o ka nui, ke ʻano, a me ke kahe o ke koko i nā māmā.
• Electrocardiogram (ECG/EKG): He hoʻāʻo e hoʻopaʻa i ka hana uila o ka puʻuwai. Hiki i kēia ke nānā i kekahi kaumaha ma ka ʻiʻo puʻuwai a me nā loli i ke ʻano o ka puʻuwai.
• ʻEchocardiogram ('Echo' scan): ʻO kēia ka hoʻāʻo koʻikoʻi a kikoʻī loa. Ua like ia me ka ultrasound scan. Hiki iā ia ke ʻike maopopo i nā mea āpau i loko o ka puʻuwai, me nā keʻena, nā valves, nā kīʻaha koko, a me ke ʻano o ke kahe ʻana o ke koko. ʻO ka Pulmonary Atresia ka mea i ʻike pololei ʻia me kēia hoʻāʻo.

He aha nā lāʻau lapaʻau no ka pēpē?

ʻOiai ʻaʻole hiki ke ho'ōla piha ʻia kēia maʻi, hiki i ke ʻoki kino a me nā lāʻau ʻē aʻe ke hoʻomaikaʻi nui i ke kahe koko i loko o ka puʻuwai o ka pēpē, e ʻae ana iā lākou e ola i kahi ola maʻamau. ʻOkoʻa ka hoʻolālā lapaʻau mai kēlā pēpē a i kēia pēpē.

Ka Papa Hana ʻEkahi: Ke Hoʻopaʻa ʻana i ka Pēpē

I ka hānau ʻana o ke pēpē, ʻo ka pahuhopu mua ka mālama ʻana i ke kīʻaha koko e hoʻōla ola ai i kapa ʻia ʻo Ductus Arteriosus e hāmama ana a pale iā ia mai ka pani ʻana.

• Lāʻau lapaʻau: Hāʻawi mau ʻia kahi lāʻau lapaʻau i kapa ʻia ʻo alprostadil ma o kahi aʻa koko (IV). Mālama kēia i ke kīʻaha koko manawa pōkole mai ka pani ʻana. Hoʻokumu kēia i kahi ala hou no ke kahe ʻana o ke koko i nā māmā.
• Nā ʻano hana ʻē aʻe: I kekahi manawa, hoʻohana nā kauka cardiologists i kahi ʻōmole lahilahi loa (catheter) e hele ana ma o kahi kīʻaha koko ma ka wāwae i ka puʻuwai a kau i kahi ʻāpana liʻiliʻi e like me ka mesh (stent) i loko o ka Ductus Arteriosus e mālama iā ia.

KaʻAnuʻu Hana ʻElua: Kaʻina Hana ʻoki kino

I ka manawa pinepine, pono i kahi pēpē me ka Pulmonary Atresia ke hana i kekahi mau ʻoki kino i loko o nā makahiki mua o ke ola. ʻAʻole hiki ke hana ʻia kēia mau ʻoki kino i ka manawa hoʻokahi, akā hana ʻia i nā pae i ka wā e ulu ana ka pēpē.

ʻO nā kaʻina hana ʻokiʻoki ʻekolu i hana pinepine ʻia:

1. BTT Shunt (ʻO ke ʻoki kino mua - i nā pule mua): Pili kēia i ka hoʻopili ʻana i kahi ʻōmole hana liʻiliʻi (shunt) ma waena o kahi lālā o kahi kīʻaha koko nui e lawe ana i ke koko i ke kino a me ke kīʻaha e lawe ana i ke koko i nā māmā. Hāʻawi kēia i kahi lako koko paʻa i nā māmā.

2.Ke Kaʻina Hana Glenn (ʻoki kino ʻelua - ma kahi o 4-8 mahina): Ke ʻoi iki aʻe ka pēpē, wehe ʻia ka shunt i kau mua ʻia. A laila, hoʻopili pololei ʻia ke koko nele i ka oxygen e hele mai ana mai ke kino o luna i ke aʻa pulmonary. Hoʻemi nui kēia i ka ukana hana ma ka puʻuwai.

3. Ke Kaʻina Hana Fontan (ʻoki kino ʻekolu a me ka hope loa - ma kahi o 2-4 mau makahiki): Ma kēia, ua hoʻopili pololei ʻia ke koko deoxygenated mai ke kino haʻahaʻa i ke aʻa pulmonary. Ma hope o kēia ʻoki kino, ua hoʻoponopono ʻia ke kahe o ke koko i hiki i ke koko deoxygenated āpau mai ke kino ke hele pololei i nā māmā, ma kahi o ka hele ʻana i ka ʻaoʻao ʻākau o ka puʻuwai.

Ma hope o kēia mau ʻoki kino, pono ke pēpē e noho ma ka haukapila, ma ke keʻena mālama koʻikoʻi (ICU), no hoʻokahi a ʻelua paha pule.

Pehea ke ola ma hope o ke ʻoki kino?

Ma hope o kēia mau ʻoki kino, hiki i ke keiki ke hele i ke kula a pāʻani ma ke ʻano maʻamau. Eia nō naʻe, he kūlana kēia e pono ai ka nānā ʻana o ke kauka no ke ola holoʻokoʻa.

• Nā hoʻokolohua lapaʻau maʻamau: Pono ʻoe e hele no nā manawa hoʻopaʻa maʻamau me kahi kauka puʻuwai. Pono ʻoe e loaʻa nā hoʻokolohua e like me ka echo scan a me ka ECG ma ka liʻiliʻi i hoʻokahi manawa i ka makahiki.
• Nā pilikia: I ka hala ʻana o ka manawa, aia kahi pilikia o nā pilikia e like me ka arrhythmia, nā pilikia ate, a me ka hāʻule ʻana o ka puʻuwai. ʻO ia ke kumu he mea nui nā nānā mau ʻana.
• Ke pale ʻana i nā maʻi: Aia kēia mau keiki i ka nui o ka pilikia o ka hoʻomohala ʻana i ka endocarditis, kahi maʻi o ka uhi o ka puʻuwai. No laila, pono ʻoe e nīnau i kāu kauka inā pono ʻoe e lawe i nā antibiotics e pale aku i ka maʻi ma mua o kekahi mau kaʻina hana lapaʻau, e like me ka unuhi ʻana i nā niho.
• Nā Hana: Pono ʻoe e kamaʻilio me kāu kauka a hoʻoholo i nā hoʻoikaika kino a me nā haʻuki hiki i kāu keiki ke hana a ʻaʻole hiki.

ʻAʻole hiki i nā huaʻōlelo ke hōʻike i ka ʻeha, ka makaʻu, a me ke kahaha āu e manaʻo ai ke ʻike ʻoe ua loaʻa i kāu keiki kēia maʻi. Akā e hoʻomanaʻo, ʻaʻole ʻoe hoʻokahi. He hui nui o nā kauka akamai a me nā kahu maʻi i hoʻolaʻa ʻia no ka hāʻawi ʻana i ka mālama maikaʻi loa no kāu keiki. E kamaʻilio me kāu kauka e pili ana i nā nīnau a hopohopo paha āu e loaʻa ai.