Loaʻa i kāu keiki liʻiliʻi kēia mau pilikia puʻuwai paʻakikī ʻehā? (Tetralogy o Fallot) - E kamaʻilio kākou e pili ana i kēia!

ʻO Tetralogy of Fallot, a i ʻole ToF no ka pōkole, he ʻano ia e kū pū ai nā ʻano ʻē ʻehā i loko o ka puʻuwai o kāu pēpē i ka wā hānau. Hoʻopilikia kēia mau pilikia i ka puʻuwai o ka pēpē e pamu i ke koko oxygenated i ke koena o ke kino. Ua ʻike ʻoe i ke koʻikoʻi o ka oxygen no nā mea āpau i loko o ko mākou kino e hana pono ai. He mea maʻamau ka makaʻu ke lohe ʻoe e pili ana i kēia ʻano, akā e kamaʻilio kākou e pili ana i ia mea me ka kikoʻī i hiki iā ʻoe ke hoʻomaopopo maikaʻi iā ia.

No ke aha he mea kūikawā kēia `(Tetralogy of Fallot)`? He aha ka mea e hana ʻia ana i loko o ka puʻuwai?

I ka ʻōlelo maʻalahi, i loko o ka puʻuwai olakino, hele ke koko ma kekahi ala. ʻO ia hoʻi, hele ke koko nele i ka oxygen i nā māmā, lawe i ka oxygen, a laila hoʻi i ka puʻuwai e hoʻolaha ʻia ma ke kino holoʻokoʻa. Akā ma muli o kēia mau pilikia ʻehā i loko o ka puʻuwai o kahi pēpē me ka "Tetralogy of Fallot", hele pololei kekahi o ke koko nele i ka oxygen i ke kino ma kahi o ka hele ʻana i nā māmā. ʻO kēia ka pilikia nui.

I kēia manawa, e nānā kākou i kēia mau pilikia puʻuwai ʻehā:

1. Ventricular Septal Defect (VSD) : E noʻonoʻo he ʻelua keʻena nui o kou puʻuwai, hoʻokahi ma ka ʻākau a hoʻokahi ma ka hema. Ke loaʻa kahi VSD ma ka paia ma waena o nā mea ʻelua, hui pū ke koko deoxygenated (koko polū) ma ka ventricle ʻākau me ke koko oxygenated (koko ʻulaʻula) ma ka ventricle hema. ʻAʻole maikaʻi kēia, no ka mea, pono ke kino i ke koko maʻemaʻe a oxygenated.

2. Pulmonary Artery Stenosis : ʻO ke kīʻaha koko nui e lawe ana i ke koko nele i ka oxygen mai ka ventricle ʻākau o ka puʻuwai i nā māmā (ke kīʻaha pulmonary) a me kona valve (ka valve) e lilo i mea haiki. Ua like ia me kahi paipu wai e paʻa ana. Hoʻemi kēia i ka nui o ke koko e hiki ke hele i nā māmā me kēlā me kēia puʻuwai. Hoʻopaʻa kēia i ka nui o ka oxygen e hiki ke hoʻohui ʻia i ke koko.

3. Aia ke kīʻaha koko nui e lawe ana i ke koko i ke kino ma kahi kūpono ʻole (`Overriding Aorta`) : ʻO ka `(Aorta)` ke kīʻaha koko nui loa e lawe ana i ke koko oxygen ma o ko kākou kino. ʻO ka maʻamau, pili kēia i ka lumi hema haʻahaʻa o ka puʻuwai. Akā me ka `(VSD)`, aia kēia `(Aorta)` ma luna o nā lumi ʻelua, e wehe ana i nā mea ʻelua. Ma muli o kēia, hoʻomaka ke koko nele i ka oxygen a me ke koko waiwai i ka oxygen e kahe ana ma o kēia `(Aorta)` i ke kino. I ka ʻōlelo pololei ʻana, ʻo kekahi o ke koko e pono e hele i nā māmā e loaʻa ai ka oxygen e hele pololei i ke kino.

4. Ka hoʻonui ʻia ʻana o ka paia o ke keʻena haʻahaʻa ʻākau o ka puʻuwai (`Ventricular Hypertrophy`) : Ma muli o nā pilikia a pau i ʻōlelo ʻia ma luna, ʻoiai ka haiki ʻana o ke kīʻaha koko e hoʻouna ana i ke koko i nā māmā, pono ke keʻena haʻahaʻa o ka puʻuwai (`(Right Ventricle)`) e hana ikaika e pamu i ke koko. E like me ka nui ʻana o ka ʻiʻo ke hāpai ʻoe i kahi kaumaha, e nui pū ka papa ʻiʻo ma ka paia o kēia keʻena i ka hala ʻana o ka manawa. Hiki i kēia ke hoʻopilikia hou aku i ka hana o ka puʻuwai.

Ke hui pū ʻia kēia mau pilikia ʻehā, e kū mai ke kūlana i ʻike ʻia ʻo "Tetralogy of Fallot".

Pehea ka maʻamau o kēia maʻi? ʻO wai ka mea i ʻoi aku ka paʻakikī o ka loaʻa ʻana?

ʻO ka Tetralogy o Fallot he maʻi laha ʻole ia.Wahi a nā helu helu ʻAmelika, loaʻa kēia ma kahi o hoʻokahi i loko o 2,000 mau pēpē i hānau ʻia. Eia nō naʻe, ma waena o nā maʻi puʻuwai hānau (`(Congenital Heart Disorders)`), he ʻano maʻamau kēia. ʻOi aku ka maʻamau o kēia ʻano ma waena o nā pēpē kāne. Eia kekahi, ua ʻike nā kauka hiki ke ʻike ʻia ʻo `(ToF)` i waena o nā pēpē me nā pilikia chromosomal ʻē aʻe e like me `(Down Syndrome)`.

He mea kākaʻikahi loa, hiki i kekahi poʻe ke loaʻa nā pilikia i ko lākou wā makua inā ʻaʻole lākou e mālama ʻia i ka wā kamaliʻi.

Pehea e ʻike ai nā kauka i ka Tetralogy o Fallot?

Hiki i kāu kauka ke ʻike i kēia maʻi i ka wā hāpai a ma hope paha o ka hānau ʻana o ka pēpē . ʻO ka hapa pinepine, ʻike ʻia i loko o nā pule mua a i ʻole nā ​​​​mahina ma hope o ka hānau ʻana o ka pēpē.

Nā hoʻokolohua prenatal

Inā hōʻike kahi scan ultrasound maʻamau i ka wā hāpai i kekahi mau ʻano ʻē i loko o ka puʻuwai, hiki i ke kauka ke hana i kahi scan kūikawā i kapa ʻia he echocardiogram fetal . Hiki ke hana ʻia kēia ma waena o 18 a me 22 mau pule o ka hāpai ʻana e ʻike maopopo i ke ʻano o ka puʻuwai o ka pēpē a hoʻoholo inā he mau pilikia.

Nā hoʻokolohua ma hope o ka hānau ʻana o ke pēpē

Ma hope o ka hānau ʻana o ka pēpē, ke nānā ke kauka i ka pēpē a hoʻolohe i ka puʻuwai, hiki ke lohe ʻia kahi leo ʻē (`Heart Murmur`) . He hōʻailona maʻamau kēia i ʻike ʻia i nā pēpē me `(ToF)`.

Eia kekahi, ke hana ʻia nei kekahi mau hoʻokolohua ʻē aʻe e hōʻoia i kēia kūlana:

• ʻO ka Pulse Oximetry : He hoʻāʻo maʻalahi loa kēia a ʻeha ʻole. Ma mua o kou lawe ʻana i kāu pēpē i ka home, kau ʻia kahi sensor liʻiliʻi ma ka lima a i ʻole ka wāwae o kāu pēpē e ana i ka pae oxygen i loko o ko lākou koko. ʻO nā pae oxygen haʻahaʻa hiki ke lilo i hōʻailona o kahi maʻi i kapa ʻia ʻo (ToF).
• ʻEchocardiogram (ʻike ʻia hoʻi he echo) : Ua like kēia me kahi scan. Hoʻohana ia i nā nalu kani e ʻike maopopo i ke ʻano o ka puʻuwai o ke pēpē, me kona mau valves a me ke kahe koko.
• ʻO ke kiʻi X-ray o ka umauma a i ʻole ka Cardiac CT Scan : Hiki i kēia ke hōʻike i kekahi manawa i kahi ʻano "ʻano kāmaʻa" ʻē aʻe o ka puʻuwai. He ʻano kēia o ToF.
• Electrocardiogram (EKG a i ʻole ECG) : Hoʻāʻo kēia i ka hana uila o ka puʻuwai. Hiki iā ia ke ʻike i nā ʻano ʻē i ka ʻōʻō o ka puʻuwai a i ʻole inā e hana ikaika loa ana nā keʻena o ka puʻuwai.
• Ka Hoʻokomo ʻana i ka Catheter o ka Puʻuwai : He hoʻāʻo paʻakikī iki kēia. Hana ʻia inā pono wale nō. Hoʻokomo ʻia kahi ʻōmole liʻiliʻi (catheter) ma o kahi kīʻaha koko ma ka wāwae a lima paha i loko o ka puʻuwai e ana i ke kaomi i loko o ka puʻuwai, lawe i nā hāpana koko, a ʻike maopopo i ke ʻano.

I ka wā e ʻike ai i kēia maʻi i kahi kanaka makua a i ʻole kahi keiki ʻelemakule iki aʻe, hiki ke hoʻohana ʻia nā hoʻokolohua i ʻōlelo ʻia ma luna, e like me `(EKG)` a me `(Cardiac Catheterization),`.

Pehea e mālama ʻia ai kēia maʻi `(Tetralogy of Fallot)`?

ʻO nā lāʻau lapaʻau nui no ka pēpē me `(ToF)` he mau lāʻau lapaʻau a me ke ʻoki kino . Ma ke ʻano he hana mua, hāʻawi paha nā kauka i ka pēpē i nā lāʻau lapaʻau e like me `(Beta-blockers)` e kāohi i nā hōʻailona o ka pēpē a hoʻomaikaʻi i ke kahe koko. Hiki nō hoʻi iā lākou ke hāʻawi i ka oxygen keu.

ʻO ke ʻoki ʻana e hoʻoponopono ai i kēia mau pilikia puʻuwai ʻehā e hana pinepine ʻia ma waena o ʻekolu a me ʻeono mahina ma hope o ka hānau ʻana o ke pēpē. Ma hope o kēia ʻoki ʻana, hoʻi ke ʻano o ke kahe ʻana o ke koko ma o ka puʻuwai i ka maʻamau.

He aha nā koho ʻoki kino?

Hiki ke ʻokoʻa nā koho lapaʻau ma muli o ke kūlana o ka pēpē. I kekahi manawa, inā liʻiliʻi loa ka pēpē a nāwaliwali loa paha, he mea paʻakikī paha ke hana i kahi ʻoki nui i ka manawa hoʻokahi. I ia mau hihia, hiki ke hana ʻia kahi ʻoki maʻalahi (`(Palliative Surgery)`) e kāohi i nā hōʻailona no kekahi manawa a hiki i ka wā e ʻoi iki aʻe ai ka pēpē a ikaika aʻe. A laila wale nō e hiki ai ke hana ʻia ka `(Complete Repair)`.

Aia kekahi mau kaʻina hana ʻoki kino nui:

• Hoʻoponopono Piha : Ma kēia kaʻina hana, hoʻonui ke kauka lapaʻau i ka valve i haiki a me ke ala e lawe ai i ke koko i nā māmā. Eia kekahi, ua pani ʻia ka lua ma waena o nā keʻena haʻahaʻa ʻelua o ka puʻuwai (VSD) me kahi ʻāpana.
• Ke Kaʻina Hana BTT Shunt : ʻO kēia ke ʻoki kino manawa pōkole i ʻōlelo ʻia ma mua. Hoʻokomo ʻia kahi ʻōmole liʻiliʻi (Shunt) ma waena o ke aʻa lima o ke pēpē (Subclavian Artery) a me ke aʻa pulmonary (Pulmonary Artery). ʻAe kēia i ke koko hou aku e kahe i nā māmā.
• Ke Kaʻina Hana Catheter : I kekahi manawa, hiki ke hoʻokomo ʻia kahi stent i loko o ka puʻuwai ma o kahi catheter me ka ʻole o ke ʻoki ʻana e wehe i ka ventricle ʻākau. Hiki ke hoʻohana ʻia hoʻi e pale i ka patent ductus arteriosus o kahi pēpē (kahi paipu e hoʻopili ai i ke aʻa pulmonary a me ka aorta) mai ka pani ʻana.

Nā ʻoki kino no nā pākeke

He mea kākaʻikahi loa, hiki i kekahi poʻe ke ola i ka wā kamaliʻi me ke ʻoki ʻole ʻana a hiki i ka wā makua. Paipai nā loea i kēlā poʻe e hana i ke ʻoki piha e pale aku i nā pilikia i ka wā e hiki mai ana a hōʻemi i ka pilikia o ka make koke.

Ma kēia kaʻina hana, pani ke kauka lapaʻau loea i ka maʻi puʻuwai hānau makua (VSD) i ka hemahema me kahi ʻāpana, wehe i ka atrium ʻākau, a hoʻoponopono a pani paha i ka valve pulmonary. Hoʻonui ʻia ke aʻa pulmonary, ka mea e hoʻolako ana i ke koko i nā māmā ʻelua. I kekahi manawa kau ʻia kahi paipu ma waena o ka atrium ʻākau a me ke aʻa pulmonary e hoʻomaikaʻi i ke kahe koko.

ʻAʻole pono ka hapa nui o nā pākeke e hana ʻia ana no ka hoʻoponopono ʻana no kahi kīnā puʻuwai (ToF) i kahi ʻoki kino hou aʻe, akā pono lākou e hoʻomau i ka ʻike ʻana i kahi kauka cardiologist hānau makua no ka nānā mau ʻana.

He aha nā pilikia e hiki ke hana ma hope o ke ʻoki ʻana?

Ma hope o ke ʻoki ʻana o Tetralogy of Fallot, i kekahi manawa (a i kekahi manawa ma hope o kekahi manawa), hiki ke kū mai kekahi mau pilikia. Akā, mai hopohopo, ua makaʻala mau nā kauka i kēia.

• Nā Haʻalulu Uila: Hiki i ka ʻāpana e pani ana i ka VSD ke hoʻopilikia i nā hōʻailona uila mai nā keʻena o luna o ka puʻuwai (Atria) a i nā keʻena o lalo (Ventricles). Hiki ke hoʻoponopono ʻia kēia me kahi pacemaker. Me kahi ToF i hoʻoponopono ʻia, aia kahi pilikia o nā arrhythmias (nā puʻuwai puʻuwai kūpono ʻole) ma nā keʻena o luna a me lalo o ka puʻuwai. Inā loaʻa iā ʻoe kahi hanana syncopal, pono ʻoe e nānā no kēia.
• Ke Koena o ka Ventricular Septal Defect : I kekahi manawa aia paha kahi leaka liʻiliʻi a puni ka ʻāpana i hoʻohana ʻia e pani i ka VSD.
• Aneurysm : ʻO ka ʻāpana i hoʻohana ʻia e hoʻoponopono i kahi valve hiki ke hoʻoulu i nā wahi nāwaliwali i loko o ka valve e pehu i waho a hana i kahi aneurysm (kahi pehu e like me ka balloon i loko o kahi kīʻaha koko). Aia kekahi pilikia kiʻekiʻe o ka hoʻomohala ʻana i kahi aneurysm i loko o ka aorta piʻi.
• Nā Pilikia o ka Shunt : Ma muli o ke kau ʻia ʻana o ka shunt i ka wā o ke ʻoki kino mua, hiki ke loaʻa nā pilikia e like me ka hāiki ʻana (stenosis), ke koko kiʻekiʻe i loko o ke aʻa pulmonary, a me ke kaumaha nui ma ka ʻaoʻao hema o ka puʻuwai.
• Nā Valve Leaky : Hiki i ke koko ke kahe i hope ma o nā valves i loko o kou mau māmā, aorta, a i ʻole ka ʻaoʻao ʻākau o kou puʻuwai (Tricuspid). I kekahi manawa, pono paha kahi valve pulmonary (Valve Replacement).

He aha kāu e manaʻo ai i ka wā e noho ai me kēia maʻi?

ʻOiai ke hoʻi nei ke kahe o ke koko i loko o ka puʻuwai i ka maʻamau ma hope o ke ʻoki ʻana no Tetralogy of Fallot, aia nō kekahi mau mea āu a kāu keiki e pono ai e makaʻala:

• Ka Pōpilikia o ka Maʻi Puʻuwai : ʻOi aku ka kiʻekiʻe o ka pilikia o ka endocarditis. No laila, pono ʻoe e makaʻala loa i ke olakino o kou niho. Hiki i kāu kauka ke aʻo aku iā ʻoe e lawe i nā antibiotics e pale aku i ka maʻi i ka wā o ka mālama ʻana i nā niho.
• Ka Hoʻomohala Lohi : Hiki i nā pēpē me Tetralogy o Fallot ke lohi ka ulu ʻana a me ka piʻi ʻana o ke kaumaha. Pono paha lākou i ka hoʻōla kino, ka ʻōlelo, a i ʻole ka hana.
• Nā Pōʻino o ka Hāpai Keiki : Pono nā wāhine ʻelemakule e hana i kahi nānā puʻuwai kūikawā ma mua o ka hāpai ʻana. Hiki ke kiʻekiʻe iki aʻe ka pilikia o ka hāʻule ʻana o ka pēpē ma mua o ka maʻamau.
• Nā Kapu Lāʻau Lapaʻau : Ma ke ʻano he kanaka makua, pono paha ʻoe e hoʻohana i kekahi mau lāʻau lapaʻau a me nā ʻano kaohi hānau me kou maʻi puʻuwai.

He aha ka wā e hiki mai ana no ke kūlana `(ToF)`?

Me ka holomua o ka ʻepekema lapaʻau, ʻoi aku ka maikaʻi o ka wā e hiki mai ana o nā pēpē me ka `(Tetralogy of Fallot)` ma mua o ka wā ma mua. He kūleʻa loa ka mālama ʻoki kino i ka hoʻoponopono ʻana i nā hemahema i ke ʻano o ka puʻuwai a me ke kahe koko. He kiʻekiʻe loa hoʻi ka holomua o ke ʻoki kino i nā pākeke e kahi kauka puʻuwai hānau ʻike.

I kēia lā, ʻo ka hapa nui o nā pēpē i hana ʻia i ke ʻoki ʻana e hoʻoponopono i ka Tetralogy o Fallot e ola ikaika ana. Eia nō naʻe, aia paha kekahi mau kapu i nā hana paʻakikī, e like me nā haʻuki hoʻokūkū. He maikaʻi loa ka nui o ke ola lōʻihi ma hope o ke ʻoki ʻana o ToF. ʻO ka hapa nui o ka poʻe i hana ʻia i ke ʻoki ʻana e ola a hiki i ko lākou mau makahiki 30. Eia naʻe, inā ʻaʻole i hana ʻia ke ʻoki ʻana, aia wale nō ma kahi o 1 i loko o 3 mau kānaka me Tetralogy o Fallot e ola no 10 mau makahiki.

Hiki i kekahi mau pākeke i hana ʻia ke ʻoki kino no Tetralogy of Fallot i ko lākou wā kamaliʻi ke loaʻa i nā pilikia i ka wā makua e pono ai ka lāʻau lapaʻau, ka mālama ʻana i ke kino, a i ʻole ke ʻoki kino hou aʻe. No laila, he mea nui i nā pākeke ke kūkākūkā me kā lākou kauka puʻuwai ma mua o ke ʻoki kino ʻana ma kekahi ʻāpana o ke kino.

Aia kekahi mea hiki iaʻu ke hana e hoʻomaikaʻi ai i kaʻu pēpē?

ʻOiai e kali ana kāu pēpē no ke ʻoki ʻana, hiki iā ʻoe ke kōkua iā ia ke loaʻa iā ia nā "tet spell" (ke polū ʻo ia a paʻakikī i ka hanu ʻana). Ke paʻakikī iā ia ke hanu, e hoʻokomo iā ia i kahi kūlana squat me kona mau kuli e kū pono ana i kona umauma . Hoʻonui kēia kūlana i ke kahe koko i nā māmā, hiki ke kōkua i kāu pēpē e ʻoluʻolu.

Eia kekahi, he mea nui loa ka hāʻawi ʻana i ka pēpē i nā wai e lawa ai e inu ai a e pale aku iā ia mai ka hoʻoikaika nui ʻana.

Āhea wau e ʻike ai i kaʻu kauka?

Inā loaʻa i kāu pēpē kahi "tet spell" koʻikoʻi, ʻo ia hoʻi ke polū a pilikia ka hanu ʻana, e lawe koke iā lākou i ka lumi pilikia o ka haukapila kokoke loa . Inā he kanaka makua ʻoe, inā manaʻo ʻoe he maule, poniuniu, pilikia ka hanu ʻana, a i ʻole ka ʻeha o ka umauma, pono ʻoe e hele i ka lumi pilikia.

Pono nā mākua i hana i kā lākou ʻoki mua ʻana no ka valve puʻuwai (ToF) i ka wā kamaliʻi e hana i kahi hoʻokolokolo puʻuwai piha i kēlā me kēia makahiki a ʻelua paha . Hiki ke hana ʻia nā hoʻokolohua like ʻole e nānā i ka hana o ka puʻuwai, no ka laʻana:

• `(Holter Monitor)` (He mea hana e hoʻopaʻa i kahi `ECG` i ka lā a pau)
• ʻElectrocardiogram (EKG)
• Hoʻāʻo Hoʻoikaika Kino
• Echocardiogram (scan puʻuwai)
• MRI o ka puʻuwai

Eia kekahi, e nānā mau kāu kauka i ka nui a me ka hana o ka ʻaoʻao ʻākau o kou puʻuwai.

Ma hope o ka hoʻoponopono ʻana o Tetralogy o Fallot, pono kāu keiki e hana i nā nānā maʻamau me kahi kauka puʻuwai pediatric. E hoʻomau ʻia kēia mālama maʻamau a hiki i ka wā makua.

ʻO kekahi mau nīnau hiki iā ʻoe ke nīnau i kāu kauka:

• Pono anei kaʻu keiki e kaupalena i kā lākou mau hana haʻuki ma hope o ke ʻoki ʻana?
• E pono anei i kaʻu keiki nā ʻoki kino hou aku?
• He aha nā lāʻau lapaʻau ʻē aʻe (inā he) e pono ai kaʻu keiki e lawe?

ʻO ka mea hope loa, pono wau e ʻōlelo... (Leka Lawe-Home)

He mea maʻamau ka manaʻo kaumaha a me ke kaumaha ke ʻike ʻoe he maʻi puʻuwai ko kāu keiki hānau hou. Akā e hoʻomanaʻo, ua kōkua kāu hui lapaʻau i nā mākua ʻē aʻe he nui i kū i nā kūlana like, a hiki iā lākou ke kōkua iā ʻoe. Inā ʻoe e hopohopo nei no ka mālama ʻana i kahi keiki me Tetralogy of Fallot, mai makaʻu e kamaʻilio me kahi mea lapaʻau. Eia kekahi, hiki i kahi mea lapaʻau ke kōkua i nā keiki a me nā ʻōpio e manaʻo nei ua ʻoi aku lākou ma mua o ko lākou mau hoa i ka ulu ʻana. E hilinaʻi me ka mālama lapaʻau kūpono a me ka mālama aloha, hiki i kēia mau keiki ke loaʻa kahi ola maikaʻi.

ʻO Tetralogy o Fallot, Maʻi Puʻuwai Pediatric, Nā Kīnā Puʻuwai Hānau, Nā Lua i loko o ka Puʻuwai, Blue Baby Syndrome, ʻOki Puʻuwai, ʻAno o ka Puʻuwai