He aha ka Hypoplastic Left Heart Syndrome? Nā Hōʻailona, Nā Kumu, a me ka Lapaʻau

ʻO ka Hypoplastic Left Heart Syndrome (HLHS) he maʻi naʻau liʻiliʻi akā koʻikoʻi loa i hānau ʻia nā pēpē me ia. I kēia maʻi, ʻaʻole i ulu pono ka ʻaoʻao hema o ka naʻau i ka wā hāpai, no laila ʻaʻole hiki ke pūpū pono i ke koko i ke kino.

ʻO ia hoʻi, ʻaʻole hiki i ka naʻau o kāu pēpē ke hana i kāna hana nui loa o ka hoʻouna ʻana i ke koko piha i ka oxygen i kona kino holoʻokoʻa. ʻOiai ke kani nui kēia, makemake wau e ʻike ʻoe ua hoʻomohala nā kime lapaʻau i nā lapaʻau holomua e kōkua ana i nā keiki he nui me HLHS e ola i nā ola piha a hana nui.

He aha ka Hypoplastic Left Heart Syndrome?

Hana ʻia ka Hypoplastic Left Heart Syndrome i ka wā i hoʻomohala ʻole ʻia ai ka ʻaoʻao hema o ka naʻau o kahi pēpē. ʻO ka huaʻōlelo “hypoplastic” he “hoʻomohala ʻole ʻia” a i ʻole “liʻiliʻi loa.”

I ka naʻau olakino, ʻo ka ʻaoʻao hema e pūpū ana i ke koko piha i ka oxygen i ke kino holoʻokoʻa. I ka wā e loaʻa ai ka HLHS, ua nele, liʻiliʻi loa, a i ʻole ʻaʻole hana pono kekahi mau ʻāpana o ka naʻau hema. Hoʻopili kēia i ka ventricle hema (ka lumi pūpū nui o ka naʻau), ka mitral valve, ka aortic valve, a i kekahi manawa ʻo ka aorta ponoʻī.

Me ka ʻole o kahi ʻaoʻao hema e hana ana, hilinaʻi loa ka naʻau o kāu pēpē i ka ʻaoʻao ʻākau e hana i ka hana pālua. Hana kēia i kahi pilikia koke e pono ai ka nānā lapaʻau koke ma hope o ka hānau ʻana.

He aha nā hōʻailona o ka Hypoplastic Left Heart Syndrome?

ʻIke pinepine ʻia nā hōʻailona HLHS i loko o nā hola mua a i ʻole nā lā ma hope o ka hānau ʻana. Hana ʻia kēia mau hōʻailona no ka mea ʻaʻole lawa ke koko piha i ka oxygen i ke kino o kāu pēpē.

Eia nā hōʻailona maʻamau āu e ʻike ai:

• ʻIli uliuli a hina hina paha, ʻoi aku ka nui ma ka lehelehe, nā kui lima, a i ʻole nā kui wāwae
• Paʻakikī ka hanu a i ʻole ka wikiwiki o ka hanu
• ʻAʻole maikaʻi ka ʻai a i ʻole ke hōʻole e ʻai
• Luhi loa a i ʻole ka palaualelo
• Nāwaliwali ka puʻuwai a i ʻole ka wikiwiki o ka naʻau
• Anuanu nā lima a me nā wāwae
• Ke ʻauʻau nui i ka wā e ʻai ana

Hiki i kēia mau hōʻailona ke ulu koke a lilo paha i ʻoi aku ka nui i ka wā e hoʻomaka ai nā pilina naʻau maoli e kōkua i nā pēpē e ola i ka ʻōpū e pani.

He aha nā kumu o ka Hypoplastic Left Heart Syndrome?

Hoʻomohala ʻia ka HLHS i loko o nā hebedoma ʻewalu mua o ka hāpai ʻana i ka wā e hoʻomohala ana ka naʻau o kāu pēpē. ʻAʻole maopopo loa ke kumu pololei, akā ʻaʻole ia he mea āu i hana ai a i ʻole ʻaʻole i hana i ka wā hāpai.

Hana pinepine ʻia ka hapa nui o nā hihia HLHS me ka ʻole o ka mōʻaukala ʻohana o nā pilikia naʻau. Eia naʻe, hiki i ka genetics ke pāʻani i kahi hana liʻiliʻi i kekahi mau ʻohana. Inā loaʻa iā ʻoe kekahi keiki me HLHS, ʻoi aku ka nui o ka manawa (ma kahi o 2-3%) e loaʻa paha i nā keiki e hiki mai ana kēia a i ʻole nā ​​maʻi naʻau ʻē aʻe.

Hiki i nā kumu kūlohelohe i ka wā hāpai mua ke kōkua, akā ke aʻo nei nā mea noiʻi i kēia mau pilina. ʻO ka mea nui loa e hoʻomaopopo ai ʻaʻole hiki ke pale ʻia ka HLHS, a ʻaʻole ia he hewa kou.

I ka wā hea e ʻike ai i ke kauka no ka Hypoplastic Left Heart Syndrome?

ʻIke pinepine ʻia ka HLHS ma mua o ka hānau ʻana i nā ultrasound prenatal maʻamau, maʻamau ma waena o 18-24 mau wiki o ka hāpai ʻana. Inā ʻike kāu kauka i ka HLHS ma mua o ka hānau ʻana, e hana lākou i kahi hoʻolālā kikoʻī no ka hānau ʻana a me ka mālama koke.

Eia naʻe, inā ʻaʻole i ʻike ʻia ka HLHS ma mua o ka hānau ʻana, pono ʻoe e ʻimi i ka mālama lapaʻau pilikia koke inā hōʻike kāu pēpē i nā hōʻailona hopohopo. Hoʻopili kēia i ka ʻili uliuli a hina paha, ka paʻakikī o ka hanu, ka ʻai ʻole, a i ʻole ka hiamoe nui.

ʻOiai inā ʻaʻole ʻoe maopopo ua hewa kekahi mea, e hilinaʻi i kou manaʻo. ʻOi aku ka maikaʻi o ka loaʻa ʻana o nā limahana lapaʻau e loiloi i kāu pēpē inā hopohopo ʻoe e pili ana i ko lākou hanu, ʻai, a i ʻole nā ​​pae hana.

He aha nā pilikia o ka Hypoplastic Left Heart Syndrome?

Me ka ʻole o ka lapaʻau, he mea weliweli ka HLHS i loko o nā lā mua a i ʻole nā ​​wiki o ke ola. ʻOiai me ka lapaʻau, hiki ke loaʻa nā pilikia mau e kōkua kāu hui lapaʻau iā ʻoe e hoʻokele.

Hiki i nā pilikia ke hoʻopili i:

• Nā pilikia o ka naʻau (arrhythmias)
• Nā clots koko e hiki ke hele i nā ʻāpana ʻē aʻe o ke kino
• Hoʻemi ʻia ka hoʻomanawanui hoʻoikaika i ka wā e ulu ana kāu keiki
• Stroke, ʻoiai he mea maʻamau kēia me ka mālama lapaʻau kūpono
• Nā pilikia o ka puʻupaʻa a i ʻole ka ate mai ka emi ʻana o ke kahe koko
• Nā hoʻopaneʻe hoʻomohala, ʻoiai ke ulu mau nei nā keiki he nui
• Pono no nā hana naʻau hou aku ma hope i ke ola

ʻOiai ke kani nui paha kēia papa inoa, e hoʻomanaʻo he loea kāu hui lapaʻau keiki i ka pale ʻana a me ka hoʻokele ʻana i kēia mau pilikia. He nui nā keiki me HLHS e hele i ke kula, pāʻani pāʻani, a komo i nā hana maʻamau o ke kamaliʻi.

Pehea ka hōʻoia ʻana o ka Hypoplastic Left Heart Syndrome?

ʻIke pinepine ʻia ka HLHS ma mua o ka hānau ʻana i nā ultrasound prenatal maʻamau. Hiki i kāu kauka ke ʻike i ke ʻano o ka naʻau a ʻike i ka wā ʻaʻole e ulu pono ka ʻaoʻao hema.

Inā ʻaʻole i ʻike ʻia ka HLHS ma mua o ka hānau ʻana, hiki i nā kauka ke hōʻoia ma hope o ka hānau ʻana me kekahi mau hoʻokolohua. Hoʻopili kēia i ka echocardiogram (ultrasound o ka naʻau), kahi e hōʻike ana i nā kiʻi kikoʻī o ke ʻano a me ka hana o ka naʻau o kāu pēpē.

Hiki i nā hoʻokolohua hou ke hoʻopili i nā X-ray umauma, nā electrocardiograms (EKGs), a me ka pulse oximetry e ana i nā pae oxygen i ke koko o kāu pēpē. Kōkua kēia mau hoʻokolohua i nā kauka e hoʻomaopopo pono i ka hana o ka naʻau o kāu pēpē a hoʻolālā i ke ala lapaʻau maikaʻi loa.

He aha ka lapaʻau no ka Hypoplastic Left Heart Syndrome?

Hoʻopili ka lapaʻau no ka HLHS i kahi moʻo o ʻekolu mau hana ʻoki kino i hoʻolālā ʻia, i kapa pinepine ʻia ʻo ke ala Norwood. Kōkua kēia mau hana ʻoki kino e hoʻohuli i ke kahe koko i hiki ai i ka naʻau o kāu pēpē ke hana me hoʻokahi ventricle wale nō e hana ana.

Hana pinepine ʻia ka hana ʻoki mua (hana Norwood) i loko o ka wiki mua o ke ola. Hana ʻia ka hana ʻoki lua (hana Glenn) ma kahi o 4-6 mau mahina, a hana pinepine ʻia ka hana ʻoki ʻekolu (hana Fontan) ma waena o nā makahiki 2-4.

Ma waena o nā hana ʻoki kino, pono kāu pēpē i ka nānā pono a pono paha i nā lāʻau lapaʻau e kōkua i ka hana ʻana o kā lākou naʻau. Manaʻo kekahi mau ʻohana i ka hoʻololi naʻau ma ke ʻano he koho lapaʻau ʻē aʻe, ʻoiai he mau pilikia a me nā manaʻo ponoʻī kēia ala.

E hana pū kāu hui lapaʻau me ʻoe e hoʻoholo i ka hoʻolālā lapaʻau maikaʻi loa no kāu kūlana kikoʻī. ʻOkoʻa kēlā me kēia keiki, a hiki ke loli ke manawa ma muli o ka maikaʻi o ka ulu ʻana o kāu pēpē a me ka pane ʻana i ka lapaʻau.

Pehea e mālama ai i ka home no ke keiki me Hypoplastic Left Heart Syndrome?

ʻO ka mālama ʻana i ke keiki me HLHS ma ka home e pono ai ka nānā ʻana i kā lākou mau pono kūikawā, akā loaʻa i nā ʻohana he nui ka hoʻomaʻamaʻa ʻana o kā lākou mau hana me ka manawa a me ka hoʻomaʻamaʻa.

Pono paha kāu keiki i nā lāʻau lapaʻau maʻamau e kōkua i ka hana pono ʻana o kā lākou naʻau. Hiki i kēia ke hoʻopili i nā diuretics e wehe i ka wai nui, nā lāʻau lapaʻau e kōkua i ka pūpū ʻana o ka naʻau, a i ʻole nā ​​mea hoʻoheheʻe koko e pale aku i nā clots.

Hiki ke paʻakikī ka hānai ʻana no ka mea luhi pinepine nā keiki me HLHS. Pono paha ʻoe e hāʻawi i nā ʻai liʻiliʻi a pinepine a hana pū me kahi mea ʻai e hōʻoia i ka loaʻa ʻana o kāu keiki i nā calorie lawa no ka ulu olakino.

E nānā i nā hōʻailona e hōʻike ana paha i ka pono o kāu keiki i ka mālama lapaʻau, e like me ka paʻakikī o ka hanu, ka ʻai ʻole, ka huhū nui, a i ʻole nā ​​loli i kā lākou pae hana maʻamau. E aʻo aku kāu hui lapaʻau iā ʻoe i nā mea e nānā ai a me ka wā e kāhea ai.

Pehea ʻoe e hoʻomākaukau ai no kāu mau hālāwai kauka?

Hiki i ka hoʻomākaukau ʻana no nā hālāwai ke kōkua iā ʻoe e hana i ka nui o kāu manawa me ka hui lapaʻau o kāu keiki. E mālama i kahi papa inoa o nā nīnau e kū mai ma waena o nā kipa, a mai kānalua e nīnau e pili ana i kekahi mea e hopohopo ai ʻoe.

E lawe mai i kahi moʻolelo o nā lāʻau lapaʻau o kāu keiki, me ka wā e lawe ai lākou a me nā hopena ʻaoʻao āu i ʻike ai. Eia kekahi, e mālama i ka ʻike o nā ʻano ʻai o kāu keiki, nā pae hana, a me nā hōʻailona āu i ʻike ai.

Inā hiki, e lawe mai i kekahi lālā ʻohana a hoaaloha paha i nā hālāwai. Hiki i ke kākoʻo ke kōkua iā ʻoe e hoʻomanaʻo i ka ʻike nui a manaʻo ʻole i ka nui. Loaʻa i nā ʻohana he nui ka mea kōkua e lawe i nā memo i nā hālāwai a i ʻole e nīnau inā hiki iā lākou ke hoʻopaʻa i nā ʻōlelo aʻo nui.

He aha ka mea nui e pili ana i ka Hypoplastic Left Heart Syndrome?

ʻO ka HLHS he maʻi naʻau koʻikoʻi loa e pono ai ka mālama lapaʻau ola lōʻihi, akā he mea nui e ʻike he nui nā keiki me HLHS e ulu a ola i nā ola piha. Ua hoʻomaikaʻi nui nā holomua i nā ʻenehana hana ʻoki kino a me ka mālama lapaʻau i nā hopena i nā makahiki i hala.

He loea kāu hui lapaʻau keiki i ka mālama ʻana i nā keiki me nā maʻi naʻau paʻakikī, a e lilo lākou i hoa i kēia huakaʻi. ʻOiai e loaʻa ana nā pilikia, loaʻa i nā ʻohana he nui ka manaʻo he mau keiki ikaika a hana nui kā lākou mau keiki me HLHS e komo i ke kula, nā pāʻani, a me nā hana pilikanaka.

E hoʻomanaʻo ʻaʻole ʻoe wale nō i kēia huakaʻi. Loaʻa nā hui kākoʻo, nā kaiāulu pūnaewele, a me nā kumuwaiwai no nā ʻohana i hoʻopilikia ʻia e HLHS. Hiki i ka pilina ʻana me nā ʻohana ʻē aʻe ke hāʻawi i nā ʻōlelo aʻo maikaʻi, ke kākoʻo manaʻo, a me ka manaʻolana no ka wā e hiki mai ana.

Nā nīnau pinepine e pili ana i ka Hypoplastic Left Heart Syndrome

Hiki i kaʻu keiki me HLHS ke ola i kahi ola maʻamau?

ʻO ka nui o nā keiki me HLHS e ulu a alakaʻi i nā ola hana a piha, ʻoiai e pono lākou i ka mālama naʻau ola lōʻihi. ʻOiai paha he mau palena hana, komo nā keiki he nui i ke kula, nā pāʻani, a me nā hana pilikanaka. ʻOkoʻa kēlā me kēia keiki, a e kōkua kāu hui lapaʻau e hoʻoholo i ka mea kūpono no kāu kūlana kikoʻī.

Pono anei kaʻu keiki i kahi hoʻololi naʻau ma hope?

ʻAʻole pono. Hoʻolālā ʻia ke ala hana ʻoki kino ʻekolu-paepae (ala Norwood) e kōkua i ka hana ʻana o ka naʻau o kāu keiki no ka lōʻihi me hoʻokahi ventricle. ʻOiai paha kekahi mau keiki e pono ai kahi hoʻololi ma hope, nui ka poʻe e ola no nā makahiki he nui me kā lākou naʻau i hana hou ʻia. E nānā kāu hui lapaʻau i ka hana o ka naʻau o kāu keiki a e kūkākūkā i nā koho inā pono.

Hiki iaʻu ke loaʻa hou nā keiki ma hope o ka loaʻa ʻana o kahi pēpē me HLHS?

ʻAe, nui nā ʻohana e hele i ka loaʻa ʻana o nā keiki hou aku. ʻOiai he nui iki ka pilikia (ma kahi o 2-3%) o ka loaʻa ʻana o kekahi keiki me ka hemahema naʻau, ʻo ka hapa nui o nā keiki e hiki mai ana e hānau ʻia me nā naʻau olakino. Hiki i kāu kauka ke kūkākūkā i ka ʻōlelo aʻoaʻo genetic inā makemake ʻoe i ka ʻike kikoʻī e pili ana i nā pilikia.

Pehea ka lōʻihi o nā hana ʻoki kino a pehea ka hoʻōla ʻana?

Lawe pinepine ʻia kēlā me kēia hana ʻoki kino i nā hola he nui, a ʻokoʻa ka manawa hoʻōla ma muli o ka hana ʻoki kino a me ka pane kikoʻī o kāu keiki. Pono pinepine ka hana ʻoki mua i ka manawa lōʻihi loa ma ka haukapila, pinepine he mau wiki. Hiki i nā hana ʻoki kino ma hope ke loaʻa nā manawa hoʻōla pōkole. E hāʻawi kāu hui hana ʻoki kino iā ʻoe i nā manawa kikoʻī a me nā manaʻo no kēlā me kēia kaʻina hana.

He aha nā hana e hiki i kaʻu keiki ke komo i ka wā e ulu ana?

ʻokoʻa nā ʻōlelo aʻoaʻo hana no kēlā me kēia keiki ma muli o kā lākou hana naʻau a me ka olakino holoʻokoʻa. Hiki i nā keiki he nui me HLHS ke komo i nā hana leʻaleʻa, nā pāʻani kula, a me nā hanana pilikanaka. E hana pū kāu cardiologist me ʻoe e hoʻoholo i nā pae hana kūpono a hiki ke ʻōlelo aku e pale aku i kekahi mau pāʻani hoʻokūkū kiʻekiʻe ʻoiai e paipai ana i nā ʻano hana kino ʻē aʻe.