ʻAʻole anei e ulu pono ana ka ʻaoʻao ʻākau o ka puʻuwai o kāu keiki liʻiliʻi? (Hypoplastic Right Heart Syndrome)

He aha inā ua hānau ʻia kāu keiki liʻiliʻi me kahi pilikia puʻuwai? E noʻonoʻo pehea ka ʻeha o ia mea. Hānau ʻia kekahi mau pēpē me ka ʻaoʻao ʻākau o ko lākou puʻuwai ʻaʻole i ulu pono. Ua kapa ʻia kēia e nā kauka he Hypoplastic Right Heart Syndrome. Hoʻemi kēia i ka nui o ka oxygen i loaʻa i ka pēpē. E kamaʻilio kākou e pili ana i kēia me nā kikoʻī hou aku, ea?

He aha ka Hypoplastic Right Heart Syndrome?

I ka ʻōlelo maʻalahi, ʻo ka Hypoplastic Right Heart Syndrome kahi maʻi puʻuwai kakaikahi i hānau ʻia me kekahi mau pēpē . ʻO ka mea e hana ʻia ma kēia, ʻaʻole i ulu piha kekahi mau ʻāpana o ka ʻaoʻao ʻākau o ka puʻuwai o ke pēpē. E noʻonoʻo e like me kahi pamu liʻiliʻi. ʻO ka ʻaoʻao ʻākau o ka puʻuwai ka mea e lawe i ke koko nele i ka oxygen mai ke kino a hoʻouna iā ia i nā māmā e loaʻa ai ka oxygen. No laila, inā ʻaʻole e ulu pono kēia ʻaoʻao ʻākau, ʻaʻole ia e hana pono i kāna hana.

I kēia kūlana, hiki ke hoʻopilikia ʻia nā ʻāpana o ka puʻuwai:

• ʻO ka valve Tricuspid: Ua like kēia me ka puka ma waena o ka atrium ʻākau a me ka ventricle ʻākau o ka puʻuwai. Ke hana pono ʻole ia, ke ālai nei ia i ke kahe o ke koko.
• ʻO ka valve pulmonary: ʻO kēia ka valve kahi e hoʻomaka ai ke aʻa pulmonary, ka mea e lawe i ke koko mai ka ventricle ʻākau i nā māmā.
• ʻAna ʻākau: ʻO kēia ke keʻena nui e hoʻokahe koko ana ma ka ʻaoʻao ʻākau o ka puʻuwai. Inā liʻiliʻi ia, e emi ana ke koko e hoʻokahe ai.
• ʻAʻa pulmonary: ʻO kēia ke aʻa nui e lawe i ke koko mai ka ventricle ʻākau i nā māmā.

Ma muli o ke koʻikoʻi a i ʻole ke akahai o ke ʻano, hiki ke hoʻemi ʻia a hoʻonui ʻia paha ka hana o kēia mau ʻāpana o ka puʻuwai.

I kekahi manawa, hiki i nā pēpē me HRHS ke loaʻa i nā maʻi puʻuwai ʻē aʻe. Eia kekahi laʻana:

• ʻO ke kīnā septal atrial (ASD): He lua ma ka paia ma waena o nā keʻena ʻelua o luna (atria) o ka puʻuwai.
• ʻO ke kīnā septal ventricular (VSD): He lua ma ka paia ma waena o nā keʻena haʻahaʻa ʻelua (ventricles) o ka puʻuwai.
• ʻO ke kīnā o ke kahawai atrioventricular: He lua nui ma waenakonu o ka puʻuwai me kekahi mau pilikia ʻē aʻe.

Pehea ke koʻikoʻi o kēia kūlana?

Hiki i ka maʻi puʻuwai ʻākau Hypoplastic (HRHS) ke ʻoi aku ka koʻikoʻi no kekahi poʻe ma mua o kekahi . Pono ka poʻe me nā kūlana koʻikoʻi, e like me nā pae oxygen haʻahaʻa loa, i ka mālama koke ʻana . Eia nō naʻe, ʻaʻole paha e loaʻa i kekahi poʻe ka koʻikoʻi, me nā pilikia liʻiliʻi wale nō. ʻAʻole paha e ʻike ʻia kēlā poʻe a hiki i ko lākou wā ʻelemakule.

Pehea ka maʻamau o kēia ʻano?

He kūlana kākaʻikahi loa kēia.Ua manaʻo nā kānaka noiʻi he emi ma mua o 50,000 mau kānaka ma ʻAmelika Hui Pū ʻIa ka HRHS. He mea kakaikahi ia ma mua o ka Hypoplastic Left Heart Syndrome, kahi kūlana i ulu pono ʻole ai ka ʻaoʻao hema o ka puʻuwai.

He aha nā hōʻailona o ka hypoplastic right heart syndrome (HRHS)?

Eia nā hōʻailona o kēia maʻi:

• ʻO ke kala polū o ka ʻili o nā lima a me nā wāwae. Ua kapa ʻia kēia he cyanosis . ʻO ia hoʻi, ʻaʻole lawa ka oxygen i ke kino.
• Paʻakikī ka hanu ʻana. Ua kapa ʻia kēia he dyspnea .
• Ka hanu wikiwiki. Ua kapa ʻia kēia he tachypnea .
• ʻAʻole inu pono ke pēpē i ka waiū.
• Luhi koke, hiamoe mau.

Inā loaʻa i kāu pēpē kekahi a ʻoi aku paha o kēia mau hōʻailona, ​​​​ʻoi aku ka maikaʻi o ka ʻike koke ʻana i ke kauka.

He aha ke kumu o kēia?

ʻO ka ʻoiaʻiʻo, ʻaʻole i ʻike ʻia ke kumu kikoʻī o ka hypoplastic right heart syndrome (HRHS) .

Eia nō naʻe, manaʻoʻiʻo nā kānaka noiʻi he pili paha ka genetics , ʻoiai ua hōʻike ʻia he nui nā lālā o ka ʻohana hoʻokahi i loaʻa iā HRHS i ka wā hānau. No laila, pono ka noiʻi hou aku.

He aha nā pilikia ʻē aʻe e hiki ai i kēia ke hana?

Hiki i ka Hypoplastic Right Heart Syndrome (HRHS) ke hana i nā pilikia e like me:

• Haʻahaʻa nā pae oxygen i loko o ke kino. Ua kapa ʻia kēia he hypoxemia .
• Ka hāʻule ʻana o ka puʻuwai ʻākau.
• Puʻuwai kūpono ʻole. Ua kapa ʻia kēia he arrhythmia .

I ka wā ʻōpiopio, ʻoiai no ka poʻe i hoʻopilikia ʻole ʻia e HRHS, hiki ke hana ʻia nā hāʻule keiki ma muli o ka haʻahaʻa o ka oxygen i loko o ke kino.

Pehea e ʻike ʻia ai kēia maʻi?

I kekahi manawa, hiki i kahi mea mālama ola ke ʻike i ka HRHS ʻoiai ke pēpē i loko o ka ʻōpū (ka ulu ʻana o ka fetal) . Hoʻohana lākou i kahi scan ultrasound e ʻike i ka puʻuwai o ke pēpē. Inā makemake ʻoe e nānā pono, hiki iā ʻoe ke hana i kahi echocardiogram fetal . ʻAʻole invasive kēia mau hoʻāʻo ʻelua.

He aha nā hoʻokolohua e hana ʻia ma hope o ka hānau ʻana o ke pēpē?

Ma hope o ka hānau ʻana o ka pēpē, hiki ke hana ʻia kēia mau hoʻokolohua non-invasive e ʻike ai inā loaʻa i ka pēpē ka HRHS:

• ʻO ka pulse oximeter (pulse ox): He mea hana liʻiliʻi e like me ke clip e ana i nā pae oxygen ma ke kau ʻana i kou manamana lima a pepeiao paha.
• Electrocardiogram (EKG/ECG): He hoʻāʻo e ana i ka hana uila o ka puʻuwai.
• Echocardiogram - echo): He hoʻāʻo kēia e hoʻohana ana i nā nalu kani, e like me ka ultrasound, e kiʻi i nā kiʻi o ka puʻuwai. Hiki iā ia ke hōʻike i ke ʻano o ka puʻuwai, pehea e hana ai nā valves, a pehea e kahe ai ke koko.
• MRI o ka puʻuwai (Magnetic Resonance Imaging): Hoʻohana i nā kahua magnetic a me nā nalu lekiō e hana i nā kiʻi kikoʻī o ka puʻuwai.

He aha nā lāʻau lapaʻau no kēia?

ʻO ka lāʻau lapaʻau no ka hypoplastic right heart syndrome (HRHS) hiki ke komo pū me nā lāʻau lapaʻau, nā kaʻina hana minimally invasive, a i ʻole ke ʻoki kino . ʻO ka pahuhopu nui o kēia mau lāʻau lapaʻau a pau, ʻo ia ke kōkua i nā māmā o ka pēpē e loaʻa ke koko a me ka oxygen lawa .

Lāʻau lapaʻau

• Hoʻohana ʻia ka Prostaglandin e kōkua i ka mālama ʻana i ka ductus arteriosus o ka pēpē e wehe. ʻO kēia ductus arteriosus kahi ʻōmole a ka pēpē e hoʻohana ai e loaʻa ke koko oxygenated mai ka makuahine i loko o ka ʻōpū. I ka hoʻomaka ʻana o ka pēpē e hanu ma o nā māmā ma hope o ka hānau ʻana, pani pinepine kēia ʻōmole i loko o kekahi mau lā. Eia nō naʻe, i loko o kahi pēpē me HRHS, no ka mea ʻaʻole hana pono ka ʻaoʻao ʻākau o ka puʻuwai, ʻo ka mālama ʻana i kēia ʻōmole e wehe e kōkua i ke kahe ʻana o ke koko i nā māmā.

Nā kaʻina hana invasive liʻiliʻi

• Hiki i ke kauka ke hoʻohana i kahi ʻōmole lahilahi i kapa ʻia he catheter e hoʻokomo i kahi stent (kahi ʻōmole metala liʻiliʻi e like me ka ʻupena) i loko o ka ductus arteriosus o ke pēpē. Mālama kēia i ka ʻōmole e hāmama a ʻae i ke koko e kahe maikaʻi i nā māmā.
• ʻO kekahi, ʻo ia ka perforation o ka valve pulmonary . Ma kēia, ua hoʻokomo ʻia kahi catheter ma o kahi kīʻaha koko i loko o ka valve pulmonary i wehe pono ʻole ʻia, e hana ana i kahi lua i loko o ka valve. ʻAe kēia i ke koko e kahe i loko o ke aʻa pulmonary. Hoʻohana pū kekahi mau kauka i kahi kaʻina hana i kapa ʻia ʻo ablation no kēia.

ʻoki kino

Pono ke ʻoki kino no nā hihia koʻikoʻi o HRHS e mālama ai. Hana pinepine ʻia kēia mau mea ma nā pae he nui.

• ʻO ka shunt Blalock-Taussig-Thomas (BTT): Hoʻohana ʻia kēia e hoʻihoʻi i ke kahe koko i nā māmā o kahi pēpē me HRHS. Hoʻohana ia i kahi lole synthetic e hana i kahi pilina ma waena o ke aʻa subclavian (kahi aʻa i loko o ka iwi collar) a me ke aʻa pulmonary.
• Ma hope o kekahi mau mahina a i ʻole mau makahiki, hiki i ke kauka lapaʻau ke hana i kahi kaʻina hana Glenn a/a i ʻole kahi kaʻina hana Fontan . Hoʻokumu kēlā me kēia o kēia mau kaʻina hana i kahi ala hou no ke koko e kahe a puni ka ventricle ʻākau i hana ʻole ʻia. ʻO ka maʻamau, hoʻopuka ka ventricle ʻākau i ke koko i loko o ke aʻa pulmonary.

ʻO nā kaʻina hana ʻo Glenn lāua ʻo Fontan e ʻae i ke koko e kahe pololei mai kahi aa koko nui i loko o ke aʻa pulmonary, mai kahi e hele ai i nā māmā e kiʻi i ka oxygen.

Aia kekahi mau pilikia i ka mālama ʻana?

E like me nā ʻoki kino āpau, aia kekahi mau pilikia me kēia mau lāʻau lapaʻau. Hiki ke ʻokoʻa nā pilikia ma muli o ka lāʻau lapaʻau. Eia kekahi laʻana:

• Hiki ke neʻe ka stent mai kona kūlana mua.
• Hiki i ke ʻoki ʻana o ka BTT shunt ke hoʻopilikia a hōʻino paha i nā kīʻaha koko i kahi aʻalolo.
• Hiki i ka perforation valve pulmonary ke hana i kahi puʻuwai kūpono ʻole (arrhythmia) a hōʻino paha i kahi kīʻaha koko.
• Hiki i nā ʻoki kino nui ke hoʻoulu i ke koko kiʻekiʻe, ka pleural effusions, ka hopu ʻana o ka puʻuwai, nā maʻi puʻupaʻa a i ʻole ke akepaʻa, a i ʻole ka puʻuwai kūpono ʻole.

Akā, mai hopohopo. E hoʻomaopopo nā kauka iā ʻoe e pili ana i kēia mau mea a pau a e hana i nā hana e pono ai.

ʻEhia ka lōʻihi e ho'ōla ai?

ʻOkoʻa ka manawa hoʻōla ma muli o ka lāʻau lapaʻau. ʻO nā ʻoki kino nui ka lōʻihi loa. Hiki i kāu keiki ke noho ma ka haukapila no kekahi mau lā, ʻoi aku paha ma mua o hoʻokahi pule. Pono lākou e nānā pono ʻia ma hope o ko lākou hoʻi ʻana i ka home.

He aha kaʻu e manaʻo ai inā ma kēia kūlana kaʻu pēpē?

Ma muli o ka haʻahaʻa o ka oxygen i ka wā ʻōpiopio o ke ola, hiki i nā poʻe me ka hypoplastic right heart syndrome (HRHS) ke kū i nā pilikia e like me:

• Nā pilikia ʻōlelo.
• Nā pilikia hoʻomanaʻo.
• ʻO ka nui o ka pilikia o ka puʻuwai, ke akepaʻa, a i ʻole nā ​​​​maʻi puʻupaʻa i ka wā ʻelemakule.
• Nā maʻi naʻau a i ʻole nā ​​maʻi hopohopo.
• Ka paʻakikī i ka hoʻolālā ʻana i kahi mea a i ʻole ka hoʻoponopono ʻana i kahi pilikia.

Akā, aia nā loea e kōkua me kēia mau mea a pau. No laila, mai hopohopo.

He aha ka wānana o kēia kūlana?

He mea kākaʻikahi loa ka poʻe me ka maʻi puʻuwai ʻākau hypoplastic koʻikoʻi (HRHS) e ola a hiki i ka wā makua me ke ʻoki ʻole ʻana . Eia nō naʻe, ʻaʻole paha e ʻike ka poʻe me ka HRHS ʻoi aku ka koʻikoʻi he maʻi ko lākou a hiki i ko lākou wā makua.

Pehea ʻoe e mālama ai i kahi keiki me HRHS?

ʻO ka mālama e pono ai kāu pēpē e pili ana i ke koʻikoʻi o kona kūlana . Inā ua hana ʻia ʻo ia i ke ʻoki ma hope koke iho o ka hānau ʻana, pono e nānā pono ʻia ʻo ia ma hope o kona hoʻi ʻana i ka home. E hāʻawi kāu kauka a kahu hānai paha iā ʻoe i nā ʻōlelo aʻoaʻo maikaʻi no ka mālama ʻana i kāu pēpē.

Āhea ʻoe e pono ai e ʻike i ke kauka?

Pono kahi pēpē i hana ʻia i ke ʻoki kino e ʻike i ke kauka no nā manawa hoʻopaʻa . E haʻi lākou iā ʻoe i ka mea e makaʻala ai a me ka mea e haʻi koke aku ai i ke kauka inā ʻike ʻoe i kekahi mea. Inā he mau nīnau a hopohopo paha kāu e pili ana i ke kūlana o kāu pēpē, e kāhea koke aku i ke kauka.

Inā pilikia kāu keiki i ke aʻo ʻana a i ʻole ka hoʻoponopono ʻana i ko lākou mau manaʻo, hiki iā ʻoe ke ʻimi i nā loea ʻē aʻe e hiki ke kōkua ma ia mau wahi. E hāʻawi kāu kauka i ke alakaʻi e pono ai.

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

Eia kekahi mau nīnau hiki iā ʻoe ke nīnau aku i ke kauka o kāu keiki:

• Pono anei kaʻu keiki i ke ʻoki kino?
• ʻEhia ka manawa e pono ai ke hana ʻoki kino?
• ʻEhia mau ʻoki kino e pono ai, a i ka manawa hea e hana ʻia ai?
• He aha ka manaʻolana no kaʻu keiki me kēia kūlana kūikawā?

Maopopo iaʻu ke paʻakikī o ka ʻike ʻana he maʻi puʻuwai ko kāu keiki. Eia nō naʻe, loaʻa i nā keiki i hānau ʻia i kēia lā nā lāʻau lapaʻau i loaʻa ʻole i nā hanauna i hala. ʻO ʻoe ke kanaka ikaika loa hiki iā ʻoe ke lilo no kāu keiki i kēia manawa. E aʻo e pili ana iā HRHS. E nīnau i kāu kauka e pili ana i nā mea i maopopo ʻole. E kamaʻilio me kāu kauka e hoʻoholo i ka mea maikaʻi loa no kāu keiki.

ʻO ka mea hope loa, e hoʻomanaʻo i kēia (Leka Lawe-Home)

ʻO ka Hypoplastic Right Heart Syndrome (HRHS) kahi maʻi laha ʻole i hānau ʻia ai nā pēpē, kahi i ulu pono ʻole ai ka ʻaoʻao ʻākau o ka puʻuwai. Hoʻemi kēia i ka nui o ka oxygen i loaʻa i ke kino. ʻO ka ʻike mua ʻana a me ka mālama kūpono (lāʻau lapaʻau, ʻoki liʻiliʻi, a i ʻole ke ʻoki nui) hiki ke hoʻomaikaʻi i ke ola o ke keiki . I loko o ia kūlana, he mea nui loa iā ʻoe ma ke ʻano he makua e hoʻomaopopo a hana pū me kāu mau kauka. E hoʻomanaʻo, ʻaʻole ʻoe hoʻokahi. Hiki iā ʻoe ke loaʻa ke kōkua āu e pono ai.

ʻO ka Hypoplastic Right Heart Syndrome, HRHS, Maʻi Puʻuwai Pēpē, Nā Kīnā Puʻuwai Hānau, Cyanosis, Paʻakikī Hanu, ʻOki Puʻuwai