ʻAʻole anei e ulu pono ana ka puʻuwai hema o kāu keiki liʻiliʻi? (Hypoplastic Left Heart Syndrome - HLHS) E kamaʻilio kākou e pili ana i kēia!

I kēia lā, e kamaʻilio mākou e pili ana i kahi maʻi puʻuwai paʻakikī iki, a kakaʻikahi loa, akā he mea nui loa. He ʻano kēia i kapa ʻia ʻo Hypoplastic Left Heart Syndrome , a i ʻole (HLHS) no ka pōkole. E makaʻu paha ʻoe ke lohe ʻoe i kēia, akā mai hopohopo. E kamaʻilio maʻalahi kākou e pili ana i kēia, ma ke ʻano hiki iā ʻoe ke hoʻomaopopo. No ka mea, he mea nui loa ka makaʻala i nā mea e like me kēia.

He aha ka hypoplastic left heart syndrome (HLHS)?

I ka ʻōlelo maʻalahi, (HLHS) he maʻi puʻuwai hānau i loaʻa i ka wā hānau . Ma kēia, ʻaʻole ulu pono kekahi mau ʻāpana o ka ʻaoʻao hema o ka puʻuwai o ka pēpē. E noʻonoʻo e like me ka pamu. ʻO ka ʻaoʻao hema o kēia pamu ka mea e hoʻouna i ke koko momona oxygen i ke kino holoʻokoʻa. No laila, ke ulu pono ʻole kēia ʻaoʻao hema, kū mai kahi pilikia. I kēia hihia, ʻo kēia mau ʻāpana o ka ʻaoʻao hema o ka puʻuwai ka mea nui e hoʻopilikia ʻia:

• Ventricle hema : ʻO kēia ke keʻena nui ma ka ʻaoʻao hema haʻahaʻa o ka puʻuwai. Hoʻopuka ia i ke koko oxygenated i loko o ka aorta . I ka HLHS, pinepine ia he liʻiliʻi loa e hana pono.
• Aorta : ʻO kēia ke kīʻaha koko nui loa i loko o ko mākou kino. Lawe ia i ke koko mai ka puʻuwai a i ke kino holoʻokoʻa. I loko o (HLHS), ʻaʻole paha e ulu pono kēia.
• ʻO nā kiwikā mitral a me aortic: Ua like kēia me nā puka. ʻAe wale lākou i ke koko e kahe i hoʻokahi ʻaoʻao. ʻAe ka kiwikā aortic i ke koko e kahe mai ka ventricle hema i loko o ka aorta. ʻAe ka kiwikā mitral i ke koko e kahe mai ke keʻena luna (atrium) ma ka ʻaoʻao hema o ka puʻuwai i ke keʻena haʻahaʻa (kiwikā mitral ). I ka HLHS, ʻaʻole paha e hana pono kēia kiwikā a i ʻole he liʻiliʻi loa paha.

I kekahi manawa, loaʻa paha i nā pēpē me HLHS kahi lua liʻiliʻi ma ka paia ma waena o nā keʻena ʻelua o luna o ka puʻuwai (atrial septal defect) . ʻO ka maʻamau, pono kēia he paia mānoanoa.

He aha ka ʻokoʻa ma waena o ka puʻuwai maʻamau a me ka puʻuwai me (HLHS)?

ʻElua ʻaoʻao o ko kākou puʻuwai maʻamau. Hoʻopuka ka ʻaoʻao ʻākau i ke koko nele i ka oxygen mai ke kino a i nā māmā e hōʻiliʻili i ka oxygen. A laila, hele ke koko i loaʻa ka oxygen i ka ʻaoʻao hema a hoʻopuka ʻia i ke koena o ke kino. Eia nō naʻe, i loko o kahi pēpē me HLHS, ua liʻiliʻi loa ka ʻaoʻao hema o ka puʻuwai ʻaʻole hiki iā ia ke hoʻopuka i ke koko lawa i ke koena o ke kino. A laila, lawe ka ventricle ʻākau. Hoʻāʻo ia e hoʻopuka i ke koko i nā māmā a me ke koena o ke kino. Hana ʻia kēia ma o ka ductus arteriosus.Ma o kahi kīʻaha koko kūikawā i kapa ʻia ʻo ductus arteriosus. ʻO kēia (ductus arteriosus) he kīʻaha koko i loaʻa i kēlā me kēia pēpē i loko o ka ʻōpū. Pani pinepine ia i kekahi mau lā ma hope o ka hānau ʻana. Akā i loko o kahi pēpē me (HLHS), inā pani ʻia kēia kīʻaha koko, inā waiho ʻole ʻia, hiki ke hoʻoweliweli i ke ola. No ka mea, ʻaʻole hana ka ʻaoʻao hema, ua ālai ʻia ke ala wale nō e hiki ai ke koko i ke kino.

Pehea ka maʻamau o (HLHS)?

He maʻi laha ʻole ka (HLHS). Wahi a nā helu helu ma ʻAmelika Hui Pū ʻIa, hoʻopilikia ia ma kahi o hoʻokahi i loko o 3,800 mau pēpē i hānau ʻia i kēlā me kēia makahiki. ʻO (HLHS) ka mea maʻamau ma waena o 2% a me 3% o nā maʻi puʻuwai hānau āpau (CHD). ʻOi aku ka maʻamau i nā keikikāne ma mua o nā kaikamahine.

He aha nā hōʻailona o kahi pēpē me (HLHS)?

ʻAʻole paha e ʻike koke ʻia nā hōʻailona o kahi pēpē me (HLHS). Hiki ke ʻike ʻia i loko o kekahi mau hola a i ʻole mau lā ma hope o ka hānau ʻana. ʻO nā hōʻailona nui:

• Cyanosis : He kala polū-hina o ka ʻili, nā lehelehe, a me nā kui. Hiki ke ʻike ʻia kēia ma ke ʻano he hina hina i nā pēpē me ka ʻili ʻeleʻele. Hiki ke ʻike ʻia he polū i nā pēpē me ka ʻili māmā. ʻO ke kumu o kēia ʻaʻole lawa ka oxygen i ke kino.
• Paʻakikī ka hanu ʻana : Manaʻo paha ka pēpē he paʻakikī ke hanu.
• Paʻakikī i ka inu waiū: ʻAʻole makemake paha ka pēpē e inu i ka waiū, a i ʻole e kuha paha i ka wā e inu ana i ka waiū.
• Lethargy : Hiki i ke pēpē ke ʻike ʻia he hiamoe a palaualelo.
• Ka wikiwiki o ka puʻuwai.
• Ka hou ʻana , ka ʻili paʻa, a i ʻole ke anu ʻana.
• Ka naʻau palupalu .

Inā ʻike ʻoe i kekahi o kēia mau hōʻailona, ​​​​he mea nui e ʻimi koke i ke kōkua lapaʻau. Mai hopohopo, akā e hana koke.

He aha nā kumu o (HLHS)?

I ka hapanui o nā hihia, ʻaʻohe kumu kikoʻī no ka HLHS. I kekahi manawa hiki ke kumu o nā kumu genetic . ʻO nā pēpē me kekahi mau hoʻololi gene, e like me GJA1 a i ʻole NKX2-5, aia i ka nui o ka pilikia o ka hoʻomohala ʻana i ka HLHS. Eia kekahi, hiki i nā pēpē me kekahi mau kūlana genetic, e like me Turner syndrome a i ʻole Trisomy 18, ke hoʻomohala i ka HLHS.

Pehea e ʻike ʻia ai ka HLHS?

Hiki i nā kauka ke ʻike i ka HLHS me nā hoʻokolohua kiʻi ʻaʻole invasive. Hiki ke hana i kēia i ka wā hāpai a i ʻole ma hope o ka hānau ʻana o ka pēpē. I ka wā hāpai:

• ʻO ka ultrasound prenatal: He scan kēia e hana pinepine ʻia i ka wā hāpai.
• ʻO ka echocardiogram fetal: He scan kūikawā kēia e hiki ke nānā i nā pilikia puʻuwai ʻoiai ke pēpē i loko o ka ʻōpū.

Ma hope o ka hānau ʻana o ka pēpē: Hoʻomaopopo nā kauka i ke kūlana ma ka nānā ʻana i nā hōʻailona a me ka nānā ʻana i nā hopena hoʻāʻo. I kekahi manawa, ke hoʻolohe nei i ka puʻuwai o ka pēpē me ka stethoscope, lohe paha ʻoe i kahi murmur o ka puʻuwai . ʻO ke ʻano kēia ʻaʻole ke kahe pono nei ke koko.

He aha nā hoʻokolohua e hoʻohana ʻia no ka ʻike ʻana i ka maʻi (HLHS)?

• X-ray o ka umauma: Hiki i kēia ke nānā i ka nui a me ke ʻano o ka puʻuwai a me nā māmā o ke pēpē.
• Echocardiogram: He hoʻāʻo ultrasound nō hoʻi kēia. Hiki ke ʻike maopopo ʻia nā ʻano kūloko o ka puʻuwai.
• Electrocardiogram (EKG/ ECG ): Hoʻohana ʻia kēia e ana i nā loli uila e hana ʻia ana i ka wā o ka puʻuwai.
• Nānā ʻana o ka pulse oximetry: Hiki i kēia ke hoʻoholo i ka nui o ka oxygen i loko o ke koko o ke pēpē.

Aia kekahi lāʻau lapaʻau no (HLHS)?

ʻAe, aia nā lāʻau lapaʻau. ʻO ka mea mua, pono e hāʻawi ʻia i ka pēpē kahi lāʻau lapaʻau i kapa ʻia ʻo prostaglandin . Mālama kēia i ka ductus arteriosus e wehe. Hana kēia i kahi ala no ke kahe ʻana o ke koko ma ke kino. Eia kekahi, hiki ke hāʻawi ʻia nā lāʻau lapaʻau ʻē aʻe e kōkua i ka puʻuwai o ka pēpē e hana maikaʻi. Pono paha e kōkua ʻia ka pēpē e hanu. A laila, hiki ke hana ʻia kekahi mau ʻoki kino . Hoʻohana ʻia kēia mau ʻoki kino e hoʻohuli i ke kahe koko i nā māmā a me ke kino. Ma hope o kēia mau ʻoki kino, ua aneane hoʻoili ʻia ka hana o ka puʻuwai i ka ventricle ʻākau. ʻO ia ka mea e pamu i ke koko i ke kino holoʻokoʻa.

He aha nā ʻoki kino e hana ʻia no (HLHS)?

Hana nā kauka lapaʻau i ʻekolu mau kaʻina hana nui: ke kaʻina hana Norwood , ke kaʻina hana Glenn , a me ke kaʻina hana Fontan . Hana ʻia kēia mau kaʻina hana ma ke kaʻina hana. 1. Kaʻina hana Norwood: Hana ʻia kēia kaʻina hana i loko o nā pule ʻelua mua o ke ola no nā pēpē me HLHS. Ma kēia kaʻina hana, nā kauka lapaʻau:

• Hoʻololi hou ʻia ka aorta i ulu ʻole o ke pēpē i hiki ai i ke koko ke kahe i ke kino.
• Hoʻokomo ʻia kahi shunt , kahi ʻōmole liʻiliʻi, e hoʻohuli i ke koko mai ka ventricle ʻākau, mai ka aorta paha, a i nā aʻa pulmonary e alakaʻi ai i nā māmā.
• Hoʻokumu ia i kahi pilina ma waena o nā keʻena o luna (atria) o ka puʻuwai. Kōkua kēia i ka hoʻolako ʻana i ke kino me ke koko momona i ka oxygen mai nā māmā.

2. Hana ʻoki ʻelua ʻaoʻao o Glenn shunt: No ka pēpēPono e hana ʻia ke ʻoki lua ma kahi o 4 a 6 mahina. I ka wā o ke ʻoki ʻana o Glenn:

• Ke wehe ʻia nei ka shunt kahiko.
• Hoʻokomo ʻia kahi shunt hou a hoʻopili i ka superior vena cava (SVC) o ke pēpē (nāna e lawe i ke koko nele i ka oxygen mai ke kino o luna a i ka puʻuwai) i ke aʻa pulmonary.
• Hoʻemi kēia shunt i ke kaumaha ma ka ventricle ʻākau, no ka mea, hiki i ke koko ke hele pololei i nā māmā.

3. Kaʻina hana Fontan: Hana ʻia ke kaʻina hana hope loa ma waena o nā makahiki he 18 mahina a me 4 mau makahiki . Hoʻohuli kēia kaʻina hana i ke koko āpau e hoʻi ana mai ke kino a i nā māmā, e kāpae ana i ka puʻuwai. I ka wā o ke kaʻina hana Fontan:

• Hoʻopili ʻia ka inferior vena cava (IVC) o ke pēpē (ka mea e lawe ana i ke koko nele i ka oxygen mai ke kino haʻahaʻa a i ka puʻuwai) i ke aʻa pulmonary.

Aia kekahi mau pilikia i ka mālama ʻana?

ʻAe, hiki i kekahi mau pēpē ke nalowale ko lākou ola i ka wā e hele ana mai kekahi ʻoki kino i kekahi. Eia kekahi, hiki ke loaʻa kekahi mau pilikia ma hope o kēlā me kēia ʻoki kino. Eia kekahi laʻana:

• ʻO nā pilikia me ka ventricle ʻākau e hana pono ʻole ana.
• Ka liki ʻana o ka valve aortic.
• Maʻi akepaʻa.
• Nā ʻano puʻuwai ʻē.
• Nā ʻūpā koko.
• Ka maʻi ʻana.
• Paʻakikī i ka ʻai ʻana.
• Nā ʻōʻili.
• Nā pilikia puʻupaʻa.
• Paʻa ka puʻuwai.

He mau mea weliweli kēia ke lohe ʻia, akā e kamaʻilio nā kauka me ʻoe e pili ana i kēia mau pilikia a hoʻāʻo e hāʻawi iā ʻoe i ka lāʻau maikaʻi loa.

He lāʻau maikaʻi anei ka hoʻoili puʻuwai?

I kekahi manawa, ʻōlelo paha nā kauka lapaʻau i kahi hoʻoili puʻuwai ma kahi o ka hana ʻana i kēlā mau ʻoki ʻekolu. Eia nō naʻe, pono nā pēpē i loaʻa i kahi hoʻoili puʻuwai e lawe i nā lāʻau lapaʻau (nā lāʻau hoʻopau pale ʻana i ke kino) no ke koena o ko lākou ola.

Hiki ke mālama ʻia ka HLHS ma mua o ka hānau ʻana?

ʻAʻole hiki ke ho'ōla loa i ka HLHS i kēia manawa ma o ke ʻoki ʻana i ka wā hāpai. Eia nō naʻe, hiki i nā kauka ʻokiʻoki fetal ke hana i kekahi mau hana e pale aku ai i kekahi o nā hopena maikaʻi ʻole e hiki mai ana i kahi pēpē e ulu ana me HLHS (e.g., kahi valve aortic liʻiliʻi). Akā, he mau hihia kūikawā wale nō kēia.

Hiki ke hoʻemi ʻia ka pilikia o (HLHS)?

ʻOiai ʻaʻole ʻike ʻia ke kumu o ka hapa nui o nā hihia o HLHS, he paʻakikī ke ʻōlelo pehea e pale loa ai. Eia nō naʻe, he mea nui mau ka hahai ʻana i nā ʻano olakino i ka wā hāpai:

• Ke pale ʻana i ka waiʻona a me ka puhipaka.
• Ke kaohi ʻana i nā maʻi ʻē aʻe e like me ka Diabetes Mellitus .
• Ka ʻai ʻana i kahi meaʻai olakino.
• ʻO ka lawe ʻana i kahi huaora prenatal i loaʻa ma ka liʻiliʻi he 400 micrograms (400 mcg) o ka folic acid i kēlā me kēia lā .

Inā he moʻolelo ʻohana ko ʻoe a i ʻole kāu hoa no HLHS, he manaʻo maikaʻi e kamaʻilio me ke kākāʻōlelo genetic ma mua o ka hāpai ʻana.

Inā loaʻa i kaʻu keiki (HLHS), he aha kaʻu e manaʻo ai?

Ma hope o ka mālama ʻana no HLHS, pono kāu keiki e ʻike i kahi kauka puʻuwai ma ka liʻiliʻi hoʻokahi manawa i ka makahiki no ke koena o kona ola. E hōʻoia kēia e hana pono ana kona puʻuwai, nā māmā, a me nā ʻōpū ʻē aʻe. I ka wā e ulu ai kāu keiki, pono ʻo ia e ʻike i kahi loea i ka maʻi puʻuwai hānau makua . Pono nā keiki he nui me HLHS e lawe i nā lāʻau lapaʻau puʻuwai no ke koena o ko lākou ola. Pono nō hoʻi lākou e lawe i nā antibiotics ma mua o kekahi ʻoki kino, me ke ʻoki niho. Hiki i kēia ke hōʻemi i ka pilikia o ka endocarditis (kahi maʻi o loko o ka puʻuwai).

He aha ka nānā ʻana no ke kūlana (HLHS)?

Inā ʻaʻole mālama ʻia, he ʻano maʻi ʻo HLHS e hiki ai ke make i loko o nā lā a i ʻole nā ​​pule o ka hānau ʻana. Me ka mālama ʻana, pili ka nānā ʻana i ka paʻakikī o ke kīnā o ka puʻuwai i loko o ka pēpē. E nīnau i ke kauka o kāu pēpē e pili ana i nā pilikia o kēlā me kēia ʻoki kino. Hiki i kekahi mau keiki ke emi iho ka hiki ke hoʻoikaika kino no ke ola holoʻokoʻa. Manaʻo pinepine nā kauka e kaupalena i ka hana kino ikaika, e like me nā haʻuki hoʻokūkū.

He aha ka nui o ke ola ʻana o nā pēpē me (HLHS)?

He mea kaumaha iki kēia, akā he mea nui e ʻike. Ma waena o 20% a me 60% o nā pēpē me (HLHS) e ola ana i ka makahiki mua o ke ola. Ma hope o kēlā, ʻo ka nui o ke ola no nā makahiki ʻelima, ʻumi, a me ʻumikūmālima e hiki mai ana ma kahi o 40%. ʻOi aku ka maikaʻi o nā hopena o nā pēpē i hānau ʻia me ke kaumaha hānau maʻamau, me ka ʻole o ka hānau mua ʻana, ma mua o nā pēpē i hānau ʻia me ke kaumaha hānau haʻahaʻa. Ua ʻike ʻia kekahi noiʻi he nui nā pēpē i ola i ka makahiki mua e ola ana i ka makahiki 18.

He mea paʻakikī ke ʻike i kēia mau helu. Akā e hoʻomanaʻo, ke holomua nei ka ʻepekema lapaʻau i kēlā me kēia lā. Ke hiki mai nei nā lāʻau lapaʻau hou, nā ʻenehana hou. No laila he mea nui e hoʻomau i ka manaʻolana.

Pehea lā wau e mālama ai i kaʻu keiki?

Hiki i nā keiki i hānau ʻia me kēia maʻi ke ola olakino me ka mālama lōʻihi mai kahi kauka puʻuwai. Hiki iā ʻoe ke mālama i kāu keiki ma o:

• E hāʻawi i kāu keiki i ka lāʻau pale maʻi palū (flu) a me ka lāʻau pale COVID-19 i kēlā me kēia makahiki.
• E lawe i kāu keiki e ʻike i ke kauka cardiologist i kēlā me kēia ʻeono mahina a i ʻole hoʻokahi manawa i ka makahiki .
• E hāʻawi i kāu keiki i ka lāʻau lapaʻau i kuhikuhi ʻia i ka manawa kūpono .
• E kaupalena i ka hana kino ikaika a kāu keiki , e like me ka ʻōlelo a kāu kauka.
• Inā he pilikia ko kāu keiki i ke aʻo ʻana , e kōkua iā lākou.

He aha nā nīnau e pono iaʻu e nīnau aku i kaʻu kauka?

Inā loaʻa i kāu pēpē ka HLHS, hiki iā ʻoe ke nīnau i ke kauka i nā nīnau e like me:

• He aha nā pilikia o ke ʻoki kino?
• Aia kekahi mau pilikia e makaʻala ai ma hope o ke ʻoki ʻana?
• He aha nā lāʻau lapaʻau e pono ai kaʻu keiki? Aia kekahi mau hopena ʻaoʻao i kēlā mau lāʻau lapaʻau?
• Pehea e pili ai ke kūlana (HLHS) i ke ola o kaʻu keiki?
• He aha ka mālama hoʻomau e pono ai i kaʻu keiki ma hope o ke ʻoki kino?
• Inā loaʻa iaʻu kahi pēpē ʻē aʻe, aia kekahi pilikia e loaʻa pū ana i kēlā pēpē (HLHS)?

Hiki i ka loaʻa ʻana o kahi keiki me HLHS ke lilo i mea hoʻoluhi manaʻo a mehameha hoʻi . I ka wā e hana ai me ke kaumaha a me ka maopopo ʻole o ka maʻi puʻuwai o kāu keiki, he mea nui e ʻimi i kahi hui kākoʻo a i ʻole kahi ʻē aʻe e loaʻa ai ke kākoʻo naʻau. ʻO ka noho ikaika ʻana i ka noʻonoʻo e kōkua iā ʻoe e hoʻoponopono i nā piʻi a me nā iho o ke olakino o kāu keiki.

Leka Lawe-Home

He maʻi puʻuwai koʻikoʻi ʻo (HLHS) i hānau ʻia. Eia nō naʻe, inā ʻike mua ʻia a mālama pono ʻia, loaʻa i nā keiki ka manawa kūpono e ola ai i kahi ola maikaʻi. Pono kēia i kekahi mau ʻoki kino paʻakikī a me ka nānā ʻana o ke kauka no ka manawa lōʻihi. ʻO ka mea nui loa, ʻo ia ka ʻike ʻaʻole ʻoe hoʻokahi. Aia nā kauka, nā kahu maʻi, a me nā limahana olakino ʻē aʻe e kōkua iā ʻoe a me kāu keiki. He mea nui hoʻi ke loaʻa ke kākoʻo mai nā mākua ʻē aʻe i loaʻa nā ʻike like. Mai hoʻopau i ka wiwo ʻole. ʻO nā mōhai āu e hana ai no kāu keiki he mea waiwai ʻole ia.