ʻAʻole hiki ke wehewehe ʻia ka hauʻoli o ka lawe ʻana mai i kahi pēpē hānau hou i ka home, ʻeā? Akā i kekahi manawa, me kēlā hauʻoli, aia kekahi mau manawa e hiki mai ai kahi makaʻu iki i ka noʻonoʻo. E hopohopo kekahi makuahine a makuakāne paha inā ʻike lākou he uliuli iki nā lehelehe a me nā manamana lima o kā lākou pēpē. Inā ʻike lākou i kahi mea e like me kēia, hiki ke kumu o kahi maʻi puʻuwai hānau. I kēia lā ke kamaʻilio nei mākou e pili ana i ka Pulmonary Atresia , kahi maʻi puʻuwai koʻikoʻi akā hiki ke mālama ʻia.
I ka ʻōlelo maʻalahi, he aha ka Pulmonary Atresia?
He mea paʻakikī iki paha kēia, akā e maʻalahi kākou. Ua like ko kākou puʻuwai me kahi hale liʻiliʻi me ʻehā lumi. ʻO ka hana nui o nā lumi ʻelua ma ka ʻaoʻao ʻākau o kēia hale (ka atrium ʻākau a me ka ventricle) ʻo ia ka hōʻiliʻili ʻana i ke koko "lepo" i pau ka oxygen i kona hele ʻana ma ke kino holoʻokoʻa, a hoʻouna aku i nā māmā e hoʻomaʻemaʻe ʻia.
Hoʻouna ʻia ke koko i nā māmā ma o kahi kīʻaha koko nui i kapa ʻia ʻo Pulmonary Artery . Ma ka hoʻomaka ʻana o kēia kīʻaha koko, aia kahi valve e hana ana e like me ka puka. Kapa mākou iā ia ʻo Pulmonary Valve . Ke wehe ʻia kēia puka, hele ke koko i loko o nā māmā.
E noʻonoʻo, he aha ka mea e hana ʻia inā ʻaʻole i hoʻokumu ʻia kēia puka, ʻo ka valve pulmonary, i ka wā hānau, a i ʻole inā ua pani loa ʻia? ʻO ia ke kūlana a mākou e kapa nei he Pulmonary Atresia .
I ka ʻōlelo maʻalahi, ma kēia kūlana, ua ālai ʻia ka puka nui e hoʻouna ana i ke koko i piha i ka oxygen mai ka puʻuwai a i nā māmā mai ka wā hānau mai. ʻO ka hopena, ke kaʻapuni nei ke koko polū a nele i ka oxygen ma ke kino holoʻokoʻa.
He maʻi laha ʻole kēia. ʻOiai ma kahi ʻāina e like me ʻAmelika, hoʻokahi wale nō i loko o 6,700 mau pēpē i hānau ʻia me kēia maʻi.
ʻO nā kīnā puʻuwai ʻē aʻe e ʻike ʻia me kēia ʻano
Hiki i kekahi pēpē me ka pulmonary atresia ke loaʻa i kekahi mau pilikia puʻuwai ʻē aʻe ma waho aʻe o kēia kīnā nui.
- ʻAʻole e ulu pono ka ʻaoʻao ʻākau o ka puʻuwai: ʻO ka ventricle ʻākau, ka mea e pamu i ke koko i nā māmā, a me ka valve tricuspid ma luna ona, ʻaʻole paha e ulu pono a liʻiliʻi.
- He lua i loko o ka puʻuwai: ʻO ka maʻamau, he lua liʻiliʻi (Foramen Ovale) ko ka pēpē ma waena o nā keʻena ʻelua o luna (atria) o ka puʻuwai i kona wā i loko o ka ʻōpū. Pani ʻia kēia ma hope o ka hānau ʻana. Eia nō naʻe, i kekahi mau pēpē, hiki ke waiho hāmama ka lua. Eia kekahi, aia paha he lua ma ka paia ma waena o nā keʻena ʻelua o lalo (ventricles) o ka puʻuwai (Ventricular Septal Defect - VSD). ʻAe kēia lua i ke koko me nā pae oxygen haʻahaʻa e hui pū me ke koko me nā pae oxygen kiʻekiʻe.
Pehea ʻoe e ʻike ai inā loaʻa i kāu pēpē kēia maʻi?
ʻO ka maʻamau, hoʻomaka nā hōʻailona o ka Pulmonary Atresia e ʻike ʻia i loko o nā hola mua a i ʻole nā lā ma hope o ka hānau ʻana. ʻO ka hōʻailona nui a maʻalahi hoʻi e ʻike ʻia:He kūlana i kapa ʻia ʻo cyanosis .
ʻO ke ʻano kēia, ʻike ʻia nā lehelehe, nā manamana lima, a me nā manamana wāwae o ka pēpē i polū ma muli o ka nele o ka oxygen i loko o ke koko. ʻO kēia kala polū ke kumu i kapa ai kekahi poʻe i kēia maʻi he "blue baby syndrome."
| Hōʻailona | Wehewehena |
|---|---|
| ʻIli polū (Cyanosis) | Ka loli ʻana o ke ʻano polū a poni paha o nā lehelehe, nā manamana lima, a ma lalo o nā kui. |
| Paʻakikī ka hanu ʻana | Ka hanu wikiwiki ʻana, ka hanu paʻakikī (Dyspnea). |
| Luhi maʻamau ʻole | ʻAʻohe ikaika o ka pēpē e omo ai, a ua hiamoe mau. |
| Ka paʻakikī o ka inu ʻana i ka waiū | Ka hōʻole ʻana e inu i ka waiū no ka mea e hoʻokano ai iā ʻoe ʻoiai ma hope o ka inu ʻana i kahi mea liʻiliʻi. |
| ʻIli anu a palupalu | Ke hoʻopā ʻoe i nā lālā o ke pēpē, ua anu a pipili lākou, e like me ka hou. |
| Uē pinepine/hoʻomaha ʻole | Ua pilikia ke keiki a uē mau me ke kumu ʻole. |
No ke aha e hana ai kēia? Nā kumu a me nā kumu pilikia
ʻAʻole ʻike ʻia ke kumu kikoʻī o kēia ʻano . Hiki mai kēia kīnā puʻuwai i loko o nā pule 8 mua o ka ulu ʻana o kahi pēpē i loko o ka ʻōpū. Eia nō naʻe, ua ʻike ʻia kekahi mau mea e hoʻonui ai i ka pilikia.
- Nā kumu hoʻoilina: ʻOi aku ke kiʻekiʻe o ka pilikia inā loaʻa i kekahi o ka ʻohana ka maʻi puʻuwai hānau a i ʻole kekahi mau maʻi hoʻoilina (e like me ka maʻi DiGeorge).
- Ke olakino o ka makuahine i ka wā hāpai:
- Ka puhipaka i ka wā hāpai.
- ʻAʻole ka mālama pono ʻana i ka maʻi diabetes .
- Ka hoʻohana ʻana i kekahi mau lāʻau lapaʻau (nā lāʻau teratogenic) i kūpono ʻole no ka hoʻohana ʻana i ka wā hāpai.
- Nā kumu makua:
- Loaʻa kahi maʻi puʻuwai hānau i kekahi o nā mākua.
- Ka makahiki makuahine kiʻekiʻe.
Pehea e ʻike pololei ai nā kauka i kēia maʻi?
I ka hapanui o nā hihia, hiki ke ʻike ʻia kēia mau kūlana ma mua o ka hānau ʻana o ke pēpē, ʻo ia hoʻi, ma o nā scan i hana ʻia i ka wā hāpai.
- I ka wā hāpai: Inā kānalua ke kauka e pili ana i ka puʻuwai o ka pēpē i ka wā o ka scan maʻamau (ultrasound), e kuhikuhi ʻo ia iā ʻoe no kahi scan kūikawā i kapa ʻia he fetal echocardiogram . ʻAe kēia i ka hana a me ke ʻano o ka puʻuwai o ka pēpē e ʻike maopopo ʻia.
- Ma hope o ka hānau ʻana o ka pēpē: Inā lohe ke kauka i kahi leo ʻē (heart murmur) i ka wā e hoʻolohe ana i ka puʻuwai o ka pēpē, a inā he uliuli ka pēpē, ua kānalua ʻia kēia maʻi. A laila, hana ʻia kekahi mau hoʻāʻo e hōʻoia i ka maʻi.
| Hoʻāʻo | He aha ka hana a kēia? |
|---|---|
| ʻO ka Pulse Oximetry | Hoʻopili ʻia kahi mea liʻiliʻi e like me ke ʻūmiʻi i ka manamana wāwae nui o ke pēpē a ana i ka nui o ka oxygen i loko o ke koko. |
| X-ray umauma | Hāʻawi ia i kahi manaʻo maʻamau o ka nui, ke ʻano, a pehea e kahe ai ke koko i nā māmā. |
| ʻElectrocardiogram (ECG) | Ana ʻia ka hana uila o ka puʻuwai e ʻike ai inā he kaomi ma luna o ka ʻiʻo o ka puʻuwai a i ʻole he ʻano mele kūpono ʻole. |
| ʻEchocardiogram (Echo) | ʻO kēia ka hoʻāʻo koʻikoʻi loa . Ua like ia me ke scan puʻuwai. Hiki ke ʻike maopopo ʻia ke ʻano o ka puʻuwai, ka hana o nā valves, ke ʻano o ke kahe ʻana o ke koko, a inā paha he mau lua. |
He aha nā lāʻau lapaʻau no kēia?
Pono e mālama ʻia ka Pulmonary Atresia i ka wā e ʻike ʻia ai. ʻAʻole hiki ke ho'ōla piha ʻia me ka lāʻau lapaʻau. Eia nō naʻe, hoʻohana ʻia nā lāʻau lapaʻau a me nā ʻano hana ʻē aʻe e hoʻopakele i ke ola o ka pēpē a hoʻopaʻa i ke kūlana a hiki i ka wā e mākaukau ai no ke ʻoki ʻana. ʻO ka hopena hope loa, ʻo ia ka hana ʻana i kekahi mau ʻoki ʻana .
1. Lapaʻau koke (nā lāʻau lapaʻau a me nā kaʻina hana)
- Lapaʻau: Ma hope o ka hānau ʻana o kahi pēpē, aia kekahi kīʻaha koko kūikawā (Ductus Arteriosus) e pani maʻamau. Hāʻawi ʻia kahi lāʻau lapaʻau i kapa ʻia ʻo Alprostadil ma o kahi aʻa koko (IV) e mālama i kēia kīʻaha koko e wehe. Ke wehe ʻia kēia kīʻaha koko, hiki i kekahi o ke koko nele i ka oxygen ke hele i nā māmā e loaʻa ai ka oxygen. He hopena manawa pōkole kēia.
- Nā Kaʻina Hana: I kekahi manawa, kau ʻia kahi stent e mālama i ke kīʻaha koko e wehe. A i ʻole hana ʻia kahi septostomy e hoʻonui i ka lua ma waena o nā keʻena ʻelua o luna o ka puʻuwai. Hana ʻia kēia mau mea e hoʻomaikaʻi iki i ke kahe ʻana o ke koko.
2. ʻoki kino (ʻoki kino)
ʻO ke ʻano o ke ʻoki kino e pono ai ka pēpē, ma muli ia o ka ulu ʻana o ka ʻaoʻao ʻākau o kona puʻuwai a inā paha he mau lua i loko o ka puʻuwai.
ʻO ka mea nui, ʻaʻole e "ho'ōla" loa kēia mau ʻoki kino i ka maʻi. Akā, hoʻihoʻi lākou i ka hana o ka puʻuwai i ka hiki, e ʻae ana i ka pēpē e ola i ke ola maʻamau.
He nui nā keiki e hana ʻia i ʻekolu mau pae o ke ʻoki puʻuwai hāmama , i hana ʻia i loko o nā makahiki mua o ke ola o kahi pēpē.
1. ʻO ke ʻoki kino mua (BTT Shunt): Hana pinepine ʻia kēia i loko o nā lā mua ma hope o ka hānau ʻana o ke pēpē. Maanei, ua hoʻopili ʻia kahi ʻōmole hana liʻiliʻi (shunt) i kahi lālā o kahi aʻa nui e hoʻolako ana i ke koko i ke kino, ʻo ia ke aʻa e lawe ana i ke koko i nā māmā. Hoʻokumu kēia i kahi ala hou no ke koko e kahe i nā māmā.
2. ʻO ke ʻoki kino ʻelua (Glenn Procedure): Hana ʻia kēia ʻoki kino ke piha ka pēpē i 4 a 8 mau mahina . Maanei, wehe ʻia ka ʻōmole i hoʻokomo mua ʻia a hoʻopili pololei ʻia ke koko nele i ka oxygen mai ke kino o luna i nā māmā.
3. ʻO ke kolu o ke ʻoki kino (Fontan Procedure): ʻO kēia ka pae hope loa o kēia moʻo o nā ʻoki kino. Aia ke pēpē ma waena o 2 a me 4 mau makahiki.Hana ʻia kēia ma waena. Maanei, ʻo ke koko nele i ka oxygen e hele mai ana mai ka ʻaoʻao haʻahaʻa o ke kino e kuhikuhi pololei ʻia ana i nā māmā.
Ke pau kēia mau ʻoki ʻekolu, e hele pololei ke koko deoxygenated a pau i loko o ke kino i nā māmā e loaʻa ka oxygen, ma kahi o ka hele ʻana i ka ʻaoʻao ʻākau o ka puʻuwai.
He aha ka mea e hana ʻia ma hope o ke ʻoki kino?
No ka mea he mau ʻoki kino nui kēia, pono ke pēpē e noho ma ka haukapila, ma ka ʻāpana mālama koʻikoʻi (ICU), no hoʻokahi a ʻelua paha pule. Pono lākou i nā mea e like me ka ventilator e kōkua iā lākou e hanu, nā mea nānā e nānā i ka hana o ko lākou puʻuwai, a me nā lāʻau ʻeha.
ʻAʻole hiki i kekahi mau pēpē ke omo ma hope o ke ʻoki ʻana. I kēlā mau hihia, hāʻawi ʻia ka waiū ma o kahi ʻōmole nasogastric. ʻOiai ma hope o kou hoʻi ʻana i ka home, pono ʻoe e makaʻala pono i ke kaumaha a me ka ulu ʻana o kāu pēpē.
ʻO ka mea nui loa, pono e mālama ʻia kēia keiki e ke kauka puʻuwai no ke koena o kona ola . He mea nui e hele i nā keʻena lapaʻau no nā nānā ʻana i nā manawa i hoʻonohonoho ʻia.
I kou wā ʻelemakule, pono paha ʻoe i nā ʻoki kino hou aʻe a i ʻole nā lāʻau lapaʻau ʻē aʻe. Eia kekahi, he mea nui loa nā nānā lapaʻau maʻamau no ka mea aia kahi pilikia o nā pilikia e like me ka arrhythmia a me ka hāʻule ʻana o ka puʻuwai.
Leka Lawe-Home
- He maʻi puʻuwai hānau koʻikoʻi ka atresia pulmonary kahi e ālai ʻia ai ka valve e lawe ana i ke koko mai ka puʻuwai i nā māmā.
- ʻO ka hōʻailona nui, ʻo ia ka polū o ke kala o nā lehelehe a me nā manamana lima o ke pēpē (cyanosis). Hiki ke ʻike ʻia ka paʻakikī o ka hanu ʻana a me ka makemake ʻole e inu i ka waiū.
- Hiki ke ʻike ʻia kēia maʻi ma o nā scan i ka wā hāpai a i ʻole ma hope koke iho o ka hānau ʻana o ke pēpē.
- He ʻano hana ʻoki puʻuwai hāmama ka lāʻau lapaʻau i hana ʻia i nā pae. ʻOiai ʻaʻole kēia e ho'ōla i ka maʻi, hāʻawi lākou i ke keiki i ka manawa kūpono e ola ai i kahi ola maikaʻi.
- Pono kēia mau keiki i ka mālama ʻia a me ka nānā ʻana e ke kauka cardiologist no ko lākou ola a pau.
- Inā loaʻa i kāu keiki kēia mau hōʻailona, mai hopohopo a e ʻike koke i ke kauka. He mea nui ka ʻike koke ʻana a me ka mālama ʻana no ka hoʻopakele ʻana i ke ola o ke keiki.
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