Ke luhi koke nei kāu keiki liʻiliʻi i ka wā e pāʻani ana a i ʻole e holo ana a puni? Ke luhi nei ʻo ia a ʻaʻole hiki ke holo a puni e like me nā keiki ʻē aʻe? Ua ʻike anei ʻoe i kekahi manawa e polū ana kona mau lehelehe a me kona mau manamana lima? Hiki i kēia ke lilo i mau hōʻailona o kahi pilikia puʻuwai liʻiliʻi. No laila, mai hopohopo. I kēia lā, ke kamaʻilio nei mākou e pili ana i ka Pulmonary Artery Stenosis , kahi maʻi puʻuwai hiki ke loaʻa i nā keiki. E kamaʻilio kākou e pili ana i nā mea āpau ma nā ʻōlelo maʻalahi.
I ka ʻōlelo maʻalahi, he aha ka Pulmonary Artery Stenosis?
ʻAe, he mea paʻakikī paha kēia inoa. Akā, he maʻalahi loa ke ʻano. E hoʻomaopopo kākou penei.
ʻEhā keʻena nui o ko kākou puʻuwai. ʻO ke keʻena haʻahaʻa ma ka ʻākau (Ventricle ʻĀkau) e hoʻopuka i ke koko haumia, ʻo ia hoʻi, ke koko me ka oxygen haʻahaʻa, i nā māmā. Lawe ʻia kēia koko i nā māmā e kahi kīʻaha koko nui. Ua kapa ʻia kēia kīʻaha koko ʻo ka Pulmonary Artery . I loko o nā māmā, ua piha kēia koko i ka oxygen, a hoʻi ke koko maʻemaʻe i ka puʻuwai a hoʻopuka ʻia i ke kino holoʻokoʻa.
I kēia manawa, e noʻonoʻo ua ālai ʻia kahi paipu wai ma kekahi wahi. A laila paʻakikī ke kahe ʻana o ka wai, ʻeā? Pēlā nō, inā ua ālai ʻia a ua haiki paha ke kīʻaha koko e lawe ana i ke koko i nā māmā (Pulmonary Artery) ma kekahi a ʻoi aku paha mau wahi, ʻo ia ka mea a mākou e kapa nei he `(Pulmonary Artery Stenosis)`. ʻO ke ʻano o ka huaʻōlelo stenosis he "blockage" a i ʻole "narrowing".
Ke ālai ʻia kēia kīʻaha koko, pono ka ventricle ʻākau e hana ikaika loa a hoʻokau i ke kaomi nui e pamu i ke koko mai kahi i ālai ʻia i nā māmā. Ua like ia me kahi motika e pono ai e hoʻokau i ka ikaika nui e pamu i ka wai ma o kahi paipu i ālai ʻia. Ke hana ʻia kēia i ka hala ʻana o ka manawa, hiki ke hōʻino ʻia ka ʻiʻo o ka puʻuwai.
He aha nā pilikia e hiki ke hana ma muli o kēia ʻano?
Inā ʻaʻole mālama pono ʻia kēia, e hoʻomau ka ʻaoʻao ʻākau o ka puʻuwai i ka hana ikaika, a hiki i ka hopena ke alakaʻi i kahi kūlana i kapa ʻia ʻo ka hāʻule ʻana o ka puʻuwai ʻākau . ʻO ke ʻano kēia, ʻaʻole hiki i ka ʻaoʻao ʻākau o ka puʻuwai ke pamu i ke koko i ke kino e like me ka mea e pono ai. Eia nō naʻe, inā ʻike ʻia a mālama ʻia i ka wā mua, hiki ke pale ʻia kēia mau kūlana.
He aha kēia mau hōʻailona? Pehea ʻoe e ʻike ai iā lākou ma ka nānā ʻana i kāu keiki?
Inā he ʻoluʻolu loa ke ālai ʻana o kēia kīʻaha koko, ʻaʻole paha e hōʻike kāu keiki i kekahi mau hōʻailona. Akā i ka wā e ʻoi aku ai ka ʻino o ke ālai ʻana, e hoʻomaka paha nā hōʻailona e ʻike ʻia. E nānā e ʻike inā loaʻa i kāu keiki kēia mau hōʻailona.
| Hōʻailona | He aha ke ʻano o kēia? |
|---|---|
| Ka paʻakikī o ka hanu ʻana/pōkole o ka hanu ʻana | Ma muli o ka loaʻa ʻole o ka oxygen kūpono i ke kino, ua paʻakikī ke keiki i ka hanu ʻana a hiamoe koke. |
| Luhi | ʻOiai ma hope o ka pāʻani ʻana no kekahi manawa a i ʻole ka hele wāwae ʻana, luhi koke ke keiki. ʻAʻole hiki iā ia ke holo a lele e like me nā keiki ʻē aʻe. |
| Puʻuwai wikiwiki | Ma muli o ka pono o ka puʻuwai e hana ikaika aʻe, e wikiwiki ka puʻuwai. |
| Pehu kino | E ʻike paha ʻoe i ka pehu ʻana ma kou mau wāwae, nā kuʻekuʻe wāwae, ka maka, nā lihilihi, a i ʻole ka ʻōpū. |
| Polū (Cyanosis) | Ke ʻaʻole lawa ka oxygen i loko o ke koko, e polū nā lehelehe, nā manamana lima, a ma lalo o nā kui. He hōʻailona koʻikoʻi kēia. |
| Pōʻino a nalowale paha ka ʻike | I kekahi manawa hiki ke poniuniu a nāwaliwali paha ma muli o ka nele o ka oxygen i ka lolo. |
ʻO ka mea nui loa, inā loaʻa i kāu keiki kekahi o kēia mau hōʻailona, mai hopohopo a e ʻike koke i ke kauka no ka ʻōlelo aʻo.
He aha ke kumu o kēia?
Aia kekahi mau ʻano nui o nā kumu e kū ai kēia kūlana.
1. Nā kumu hānau
He maʻi puʻuwai hānau pinepine kēia.ʻO ke ʻano kēia, aia kahi kīnā i loko o ka puʻuwai a i ʻole nā kīʻaha koko e ulu ana i ka wā e noho ana ke pēpē i loko o ka ʻōpū. Loaʻa paha i kekahi mau keiki kēia kūlana (Pulmonary Artery Stenosis) me ka ʻole o nā pilikia puʻuwai ʻē aʻe. Akā, loaʻa paha i ka hapa nui o ka poʻe nā pilikia puʻuwai ʻē aʻe me kēia. Eia kekahi laʻana:
- Tetralogy o Fallot: He ʻano paʻakikī kēia kahi e kū pū ai nā kīnā puʻuwai ʻehā.
- Atresia Pulmonary: Ka nele ʻana o ka valve e lawe ana i ke koko i nā māmā mai ka hānau ʻana.
- ʻO ke kīnā Septal Atrial a i ʻole Ventricular: He puka ma waena o nā keʻena luna ʻelua a i ʻole ma waena o nā keʻena lalo ʻelua o ka puʻuwai.
- Hiki ke ʻike ʻia kēia ʻano me kekahi mau kīnā puʻuwai hānau paʻakikī ʻē aʻe.
2. Nā kūlana olakino ʻē aʻe
Hiki i kēia ke hana me kekahi mau kūlana genetic.
- ʻO Williams Syndrome: He ʻano genetic kēia e hoʻopilikia ai i ka puʻuwai o ke keiki a me nā ʻōpū ʻē aʻe.
- ʻO Alagille Syndrome: He kūlana e hoʻoulu ai i nā pilikia o ke akepaʻa a me ka puʻuwai.
- ʻO ka maʻi Rubella: Inā loaʻa ka makuahine i ka rubella i ka wā hāpai, hiki i ka pēpē ke loaʻa nā kīnā puʻuwai e like me kēia.
3. Nā kumu hana ʻoki kino
I ka liʻiliʻi loa, hiki ke ālai ʻia kēia kīʻaha koko ma hope o ke ʻoki puʻuwai ʻē aʻe a i ʻole kahi hoʻoili māmā.
Pehea e ʻike ai ke kauka i kēia? (Diagnosis)
Inā ʻoe e lawe i kāu keiki i ke kauka a lohe lākou i kahi leo ʻē (murmur) ke kau lākou i kahi stethoscope ma ka umauma o kāu keiki, hiki iā ʻoe ke kānalua i kēia. A laila e paipai paha lākou i kekahi mau hoʻokolohua e hōʻoia i ke kūlana.
| Hoʻāʻo | I ka ʻōlelo maʻalahi, he aha kāu e hana ai me kēia? |
|---|---|
| ECG (Electrocardiogram) | Hoʻopaʻa ʻia ka hana uila o ka puʻuwai e ʻike ai inā he keu ke kaomi ma ka puʻuwai. |
| X-ray umauma | Hiki ke nānā ʻia ka nui a me ke ʻano o ka puʻuwai a me nā māmā, a me ke kūlana o nā kīʻaha koko. |
| ʻEchocardiogram (Echo Scan) | ʻO kēia ka hoʻāʻo koʻikoʻi loa. Hoʻohana ia i nā nalu kani e lawe i kahi wikiō o ka puʻuwai a me nā kīʻaha koko, e hōʻike ana i kahi o ka paʻa ʻana a me kona koʻikoʻi. |
| MRI a i ʻole CT Scan no ka puʻuwai | Ma ka lawe ʻana i nā kiʻi ʻekolu-dimensional (3D) o ka puʻuwai a me nā kīʻaha koko, hiki iā mākou ke aʻo pehea e kahe ai ke koko me ka maopopo loa. |
| Ka hoʻokomo ʻana o ka catheter cardiac | Hoʻokomo ʻia kahi ʻōmole lahilahi (catheter) ma o kahi kīʻaha koko ma ka wāwae a lima paha i ka puʻuwai, e ana ana i ke kaomi i loko o ke kīʻaha koko a loaʻa ka ʻike hou aku e pili ana i ka paʻa ʻana. |
Ma hope o kēia mau hoʻokolohua, e kuhikuhi aku kāu kauka iā ʻoe i kahi cardiologist pediatric.
He aha nā lāʻau lapaʻau no kēia?
ʻO ka mea maikaʻi loa, aia nā lāʻau lapaʻau kūpono loa no kēia. Hoʻoholo ʻia ka lāʻau lapaʻau e nā hōʻailona o ke keiki a me ka nui o ke ālai ʻana i loko o ke kīʻaha koko.
1. Hoʻonui ʻana i ka Balloon / Angioplasty
ʻAʻole kēia he ʻoki kino. E noʻonoʻo penei.
- Hoʻokomo nā kauka i kahi ʻōmole lahilahi ma o kahi kīʻaha koko ma ka wāwae a i ka puʻuwai.
- Aia kahi lua liʻiliʻi ma ka hopena o kēlā paipu.
- Hoʻokomo ʻia ka ʻōmole me ka piko o ka pōpō ma loko o ke kīʻaha koko i paʻa a hoʻopiha ʻia ka pōpō ma kahi o ka paʻa.
- I ka wā e pehu ai ka pōpō, e kīkoʻo a hoʻopālahalaha ana ke kīʻaha koko i paʻa.
- A laila e hoʻopiha ʻia ka pōpō a wehe ʻia ka ʻōmole.
Hiki i kēia ke hōʻemi nui i ke kūpikipikiʻana.
2. Ke kau ʻana o ka Stent
ʻO kēia ke ʻano hana kaulana loa a kūleʻa hoʻi. He ʻōmole metala liʻiliʻi ka stent i ke ʻano e like me ka ʻupena.
- Hana ʻia kēia ma ke ʻano like me ka mālama ʻana i ka pōpō pehu i ʻōlelo ʻia ma luna.
- Akā ma ʻaneʻi, ua kau ʻia kēia stent ma luna o ka baluna a hoʻouna ʻia i kahi i ālai ʻia.
- Ke hoʻopiha ʻia ka pōpō ma kahi o ka paʻa ʻana, hoʻonui ka stent a kaomi i ka paia o ke kīʻaha koko.
- ʻOiai ma hope o ka wehe ʻia ʻana o ka pōpō, noho mau ka stent ma kona wahi, e kōkua ana i ka mālama ʻana i ke kīʻaha koko i ka laulā a mai ka pani hou ʻia. Ua like ia me ke kākoʻo ʻana i kahi tunnel e kokoke ana e hiolo.
Makemake nā kauka e kau i nā stents no ka mea he 96% ka holomua koke o lākou, mālama i ke kīʻaha koko e hāmama no ka manawa lōʻihi, a emi ke kumukūʻai a emi ka pilikia ma mua o ke ʻoki ʻana.
3. ʻoki kino
ʻO kekahi mau keiki, ʻoiai inā he paʻakikī loa ka paʻa ʻana a i ʻole hele pū ʻia me nā kīnā puʻuwai ʻē aʻe, pono paha e hana i ke ʻoki puʻuwai hāmama e hoʻoponopono ai i ka paʻa.
He aha ka mea e hana ʻia ma hope o ka lāʻau lapaʻau?
Inā kau ʻia kahi stent, pono ke keiki e lawe i nā lāʻau hoʻokahe koko a me nā lāʻau antibiotic no kahi manawa pōkole e pale aku ai i ka maʻi. Ma hope o kēia mau lāʻau lapaʻau, e ʻike koke ke keiki i ka hōʻoluʻolu. I ka hoʻomaikaʻi ʻana o ke kahe koko i nā māmā, e emi koke ka pōkole o ka hanu a me ka luhi.
Pono ke keiki i ka nānā mau ʻana o ke kauka. Pono ʻoe e ʻike i ke kauka i ka manawa i hoʻonohonoho ʻia a loaʻa nā hoʻokolohua e like me ka echo scan e nānā i ke kūlana o ka puʻuwai.
Leka Lawe-Home
- ʻO ka Pulmonary Artery Stenosis ka haiki ʻana o ke kīʻaha koko nui e lawe ana i ke koko i nā māmā. ʻO ia ke kīnā puʻuwai hānau maʻamau i nā keiki.
- E makaʻala inā luhi maʻalahi kāu keiki, luhi i ka wā e pāʻani ana, a i ʻole he mau lehelehe polū kona.
- Inā loaʻa iā ʻoe kēia mau hōʻailona, mai hopohopo a e ʻike i ke kauka kokoke. ʻO ka wikiwiki o kou ʻike ʻana, ʻo ka maʻalahi ka mālama ʻana.
- I kēia manawa, aia nā lāʻau lapaʻau holomua loa, ʻaʻole ʻokiʻoki no kēia, e like me ka balloon therapy a me ke kau ʻana o stent.
- Me ka mālama kūpono a me ka nānā ʻana o ke kauka no ka manawa lōʻihi, hiki i ka hapa nui o nā keiki me kēia maʻi ke ola olakino a maʻamau. No laila, mai hopohopo.
- Mai kānalua e nīnau i ke kauka i kekahi mau nīnau āu e pili ana i ke kūlana o kāu keiki a i ʻole ka lāʻau lapaʻau.
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