He aha te Hypoplastic Left Heart Syndrome? Tohutohu, Take, me te Maimoatanga

Ko te Hypoplastic Left Heart Syndrome (HLHS) tētahi mate ngakau onge engari he kino, e whānau ana ngā pēpi me te mate nei. I roto i tēnei āhuatanga, kāo te taha maui o te ngakau e whānau pai ana i te wā hapu, nāna i kore e taea te pupuhi i te toto ki te tinana.

Ko te tikanga, kāore e taea e te ngakau o tō pēpi te mahi nui rawa atu, arā, te tuku i te toto whai-hākari-waiora ki tōna tinana katoa. Ahakoa he āhua whakawehi tēnei, e hiahia ana au kia mōhio koe kua whakawhanakehia e ngā rōpū hauora ngā maimoatanga angitu e āwhina ana i te tini o ngā tamariki me te HLHS kia ora ai i ngā oranga tino whānuitia, mahi.

He aha te Hypoplastic Left Heart Syndrome?

Ka puta te Hypoplastic Left Heart Syndrome ina kore te taha maui o te ngakau o te pēpi e whānau pai ana. Ko te kupu "hypoplastic" he tikanga noa iho "kore e whānau pai" rānei "iti rawa" rānei.

I roto i te ngakau hauora, ko te taha maui te pupuhi i te toto whai-hākari-waiora ki te tinana katoa. Ina puta te HLHS, he maha ngā wāhanga o te ngakau maui kāore rānei i te noho, iti rawa rānei, kāore rānei e mahi pai ana. Hei tāpiri atu ki ēnei, ko te ruma pupuhi matua o te ngakau (te ventricle maui), te taumanu mitral, te taumanu aortic, ā, i ētahi wā, ko te aorta anō.

Me kore he taha maui e mahi ana, ka whakawhirinaki katoa te ngakau o tō pēpi ki te taha matau hei mahi rua. He wero tere tēnei e hiahia ana ki te tiaki hauora tere i muri i te whānau.

He aha ngā tohu o te Hypoplastic Left Heart Syndrome?

Ka puta ngā tohu HLHS i roto i ngā hāora tuatahi, rānei ngā rā i muri i te whānau. Ka puta ēnei tohu nā te kore o te tinana o tō pēpi e whiwhi i te toto whai-hākari-waiora.

Ko ēnei ngā tohu tino noa ka kitea e koe:

• Te tae kiri kikorangi, rānei hina, ina koa i ngā ngutu, ngā maihao, ngā manawa rānei
• Te uaua ki te manawa, te tere o te manawa rānei
• Te iti o te kai, te whakakahore rānei i te kai
• Te ngenge nui, te ngāwari rānei ki te moe
• Te ngoikore o te pupuhi, te tere o te ngakau rānei
• Te makariri o ngā ringa me ngā waewae
• Te wera nui i te wā kai

Ka tere te whānau o ēnei tohu, ā, ka kaha ake pea i te wā e tīmata ana ngā hononga ngakau taiao e āwhina ana i ngā pēpi kia ora i te kopu e kati ana. Mēnā ka kite koe i tētahi o ēnei tohu, rapuhia te tiaki hauora tere.

He aha ngā take o te Hypoplastic Left Heart Syndrome?

Ka whānau te HLHS i roto i ngā wiki waru tuatahi o te hapu, i te wā e hanga ana te ngakau o tō pēpi. Kāore i te tino mōhiotia te take tika, engari ehara i te mea i mahi koe, kāore rānei i mahi koe i te wā hapu.

Ko te nuinga o ngā take o te HLHS ka puta noa atu, kāore hoki he hītori whānau o ngā raruraru ngakau. Heoi, pea he iti te wāhi o te ira tangata i ētahi whānau. Mēnā he tamaiti tētahi o ō tamariki me te HLHS, he iti te tupono (tau 2-3%) pea ka pangia ō tamariki e tēnei, ētahi atu rānei mate ngakau.

Pea he wāhi ngā take taiao i te wā hapu, engari kei te ako tonu ngā kairangahau i ēnei hononga. Ko te mea nui rawa atu kia mōhio koe kāore e taea te aukati i te HLHS, ā, ehara i tō he.

Nōhea te haere ki te taote mō te Hypoplastic Left Heart Syndrome?

I te nuinga o ngā wā ka kitea te HLHS i ngā whakamātautau ultrasound i mua i te whānau, i te nuinga i waenganui i ngā wiki 18-24 o te hapu. Mēnā ka kite tō taote i te HLHS i mua i te whānau, ka hanga e rātou tētahi mahere taipitopito mō te whānau me te tiaki tere.

Heoi, mēnā kāore i kitea te HLHS i mua i te whānau, me rapu e koe te tiaki hauora tere mena ka whakaatu tō pēpi hou i ētahi tohu āwangawanga. Hei tāpiri atu ki ēnei, ko te tae kiri kikorangi, hina rānei, te uaua ki te manawa, te iti o te kai, te moe nui rānei.

Ahakoa kāore koe e tino mohio kei te he tetahi mea, whakawhirinaki ki ō tūmanako. He pai tonu te whakamātautau i tō pēpi e ngā tohunga hauora mēnā kei te manukanuka koe mō tōna manawa, kai, rānei taumata kaha.

He aha ngā take morearea mō te Hypoplastic Left Heart Syndrome?

Kāore i rite ki te tini o ngā āhuatanga hauora, kāore he take morearea mārama e whakapiki ana i ō tūponotanga ki te whānau i tētahi pēpi pangia. Ko te nuinga o ngā take ka puta noa atu i ngā whānau kāore he hītori o ngā raruraru ngakau.

Heoi, he torutoru ngā take pea ka whakapiki haere i te tūponotanga:

• He hītori whānau o te HLHS, ētahi atu rānei mate ngakau whānau
• Ētahi āhuatanga ira tangata, ahakoa he onge ēnei
• Te hapu me te tama (ka pangia ngā tama e te HLHS i te nuinga atu i ngā kōhine)

He mea nui kia mahara kāore ēnei take e tikanga ka puta te HLHS. Ko te nuinga o ngā pēpi me ēnei take morearea ka whānau me ngā ngakau hauora, ā, ko te nuinga o ngā pēpi me te HLHS kāore he take morearea.

He aha ngā raruraru pea o te Hypoplastic Left Heart Syndrome?

Me kore te maimoatanga, he mate kino te HLHS i roto i ngā rā, wiki rānei tuatahi o te ora. Ahakoa he maimoatanga, ka taea te wero tonu ka āwhina tō rōpū hauora ki a koe ki te arahi.

Ko ngā raruraru pea e whai ake nei:

• Ngā raruraru o te tere o te ngakau (arrhythmias)
• Ngā toto kōtiti e taea ana te haere ki ētahi atu wāhanga o te tinana
• Te iti o te manawanui ki te whakakori i te wā e tipu ana tō tamaiti
• Te pōkaikaha, ahakoa he onge tēnei me te tiaki hauora tika
• Ngā raruraru whatukuhu, ate rānei mai i te iti o te toto
• Ngā whakaroa whanaketanga, ahakoa he maha ngā tamariki e whānau pai ana
• Te hiahia mō ngā pokanga ngakau atu i muri mai i te ora

Ahakoa pea ka āhua whakawehi tēnei rārangi, kia mahara kei te mōhio tō rōpū hauora ki te aukati me te whakahaere i ēnei raruraru. He maha ngā tamariki me te HLHS e haere ana ki te kura, e takaro ana i ngā hākinakina, ā, e whai wāhi ana ki ngā mahi tamariki noa.

Me pēhea te whakatau i te Hypoplastic Left Heart Syndrome?

I te nuinga o ngā wā ka whakatauhia te HLHS i mua i te whānau i ngā whakamātautau ultrasound i mua i te whānau. Ka taea e tō taote te kite i te hanganga o te ngakau me te tohu ina kore te taha maui e whānau pai ana.

Mēnā kāore i whakatauhia te HLHS i mua i te whānau, ka taea e ngā taote te whakatau i muri i te whānau mā ngā whakamātautau maha. Hei tāpiri atu ki ēnei, he echocardiogram (ultrasound o te ngakau), e whakaatu ana i ngā pikitia taipitopito o te hanganga me te mahi o te ngakau o tō pēpi.

Pea he whakamātautau atu, penei i ngā X-ray uma, electrocardiograms (EKGs), me te pulse oximetry hei ine i ngā taumata hākari-waiora i roto i te toto o tō pēpi. Ka āwhina ēnei whakamātautau i ngā taote ki te mōhio tika ki te mahi o te ngakau o tō pēpi me te whakarite i te huarahi maimoatanga pai.

He aha te maimoatanga mō te Hypoplastic Left Heart Syndrome?

Ko te maimoatanga mō te HLHS he maha ngā pokanga e toru, e kiia ana i te nuinga ko te huarahi pokanga Norwood. Ka āwhina ēnei pokanga ki te whakarereke i te rere o te toto kia taea ai e te ngakau o tō pēpi te mahi me te ventricle kotahi anake e mahi ana.

Ko te pokanga tuatahi (pokanga Norwood) i te nuinga o ngā wā ka puta i roto i te wiki tuatahi o te ora. Ko te pokanga tuarua (pokanga Glenn) ka puta i te 4-6 marama te pakeke, ā, ko te pokanga tuatoru (pokanga Fontan) i te nuinga o ngā wā ka puta i waenganui i ngā tau 2-4.

I waenganui i ngā pokanga, ka hiahia tō pēpi ki te tirotiro ata, ā, pea ka hiahia ki ngā rongoā hei āwhina i tōna ngakau kia mahi pai ake. Ka whakaaro ētahi whānau ki te whakawhiti ngakau hei whiringa maimoatanga atu, ahakoa he wero, he whakaaro rānei tēnei ara.

Ka mahi ngātahi tō rōpū hauora ki a koe hei whakatau i te mahere maimoatanga pai mō tō āhuatanga motuhake. He rerekē ia tamaiti, ā, ka rerekē pea te wā e ai ki te pai o te tipu o tō pēpi me te whakautu ki te maimoatanga.

Me pēhea te tiaki i te kāinga mō tētahi tamaiti me te Hypoplastic Left Heart Syndrome?

Ko te tiaki i tētahi tamaiti me te HLHS i te kāinga e hiahia ana ki te aro ki ōna hiahia motuhake, engari he maha ngā whānau e kite ana ka pai ake ō rātou mahi i te wā me te mahi.

Pea ka hiahia tō tamaiti ki ngā rongoā auau hei āwhina i tōna ngakau kia mahi pai. Pea ko ēnei ngā diuretics hei tango i te wai nui, ngā rongoā hei āwhina i te ngakau kia pupuhi pai ake, ngā kaiwhakangawari toto rānei hei aukati i ngā kōtiti.

Ka uaua pea te whāngai nā te ngāwari o ngā tamariki me te HLHS ki te ngenge. Pea ka hiahia koe ki te tuku i ngā kai iti ake, maha atu, ā, mahi tahi me te kaiwhakaako kai hei whakarite i te whiwhi o tō tamaiti i ngā kalori tika mō te tipu hauora.

Tirohia ngā tohu e whakaatu ana pea kei te hiahia tō tamaiti ki te tiaki hauora, penei i te uaua ake ki te manawa, te iti o te kai, te nui o te ngangau, ngā huringa rānei i tōna taumata mahi noa. Ka whakaako tō rōpū hauora ki a koe te mea hei titiro me te wā hei karanga.

Me pēhea te whakarite mō ō hui taote?

Ko te whakarite mō ngā hui ka āwhina i a koe ki te whakamahi pai i tō wā me te rōpū hauora o tō tamaiti. Kia mau ki te rārangi o ngā pātai e puta mai ana i waenganui i ngā haerenga, ā, kaua e whakaroa ki te ui atu mō tetahi mea e awangawanga ana koe.

Kawe mai i te rekoata o ngā rongoā o tō tamaiti, tae atu ki te wā e inu ana rātou me ētahi pānga taha kua kitea e koe. Hei tāpiri atu ki ēnei, kia mau ki te ara o ngā tauira kai o tō tamaiti, ngā taumata mahi, me ētahi tohu kua kitea e koe.

Mēnā ka taea, kawe mai tētahi mema whānau, hoa rānei ki ngā hui. Ko te tautoko ka āwhina i a koe ki te mahara ki ngā mōhiohio nui me te iti ake te whakawehi. He maha ngā whānau e kite ana he āwhina te tuhi i ngā korero i ngā hui, te ui rānei mēnā ka taea te tuhi i ngā tohutohu nui.

He aha te mea nui mō te Hypoplastic Left Heart Syndrome?

He mate ngakau kino te HLHS e hiahia ana ki te tiaki hauora ora tonu, engari he mea nui kia mōhio koe he maha ngā tamariki me te HLHS e tipu ana kia ora ai i ngā oranga whai hua. Kua pai ake ngā hua i ngā tekau tau kua pahure nei nā ngā whakapainga i ngā tikanga pokanga me te tiaki hauora.

Ko te rōpū hauora o tō tamaiti kei te mōhio ki te tiaki i ngā tamariki me ngā āhuatanga ngakau uaua, ā, ko rātou hei hoa i tēnei haerenga. Ahakoa ka puta mai ngā wero, he maha ngā whānau e kite ana he kaha, he mahi ngā tamariki me te HLHS e whai wāhi ana ki te kura, ngā hākinakina, me ngā mahi pāpori.

Kia mahara kāore koe i te kotahi i tēnei haerenga. He rōpū tautoko, ngā hapori ipurangi, me ngā rauemi motuhake mō ngā whānau e pā ana ki te HLHS. Ko te hono atu ki ētahi atu whānau ka whakawhiwhia ngā tohutohu whai hua, te tautoko hinengaro, me te tumanako mō te heke mai.

Ngā pātai e ui pinepinetia ana mō te Hypoplastic Left Heart Syndrome

Ka taea e taku tamaiti me te HLHS te ora i te ora noa?

He maha ngā tamariki me te HLHS e tipu ana kia arahina ai ngā oranga mahi, whai hua, ahakoa ka hiahia rātou ki te tiaki ngakau ora tonu. Ahakoa pea he iti ngā herenga mahi, he maha ngā tamariki e whai wāhi ana ki te kura, ngā hākinakina, me ngā mahi pāpori. He rerekē ia tamaiti, ā, ka āwhina tō rōpū hauora ki te whakatau i te mea e tika ana mō tō āhuatanga motuhake.

Ka hiahia rānei taku tamaiti ki te whakawhiti ngakau i te mutunga?

Kāore rawa. Ko te huarahi pokanga toru-wāhanga (huarahi Norwood) i hangaia hei āwhina i te mahi o te ngakau o tō tamaiti mo te roa me te ventricle kotahi. Ahakoa pea ka hiahia ētahi tamariki ki te whakawhiti i te mutunga, he maha e ora ana mo ngā tekau tau me tō rātou ngakau i hangaia. Ka tirotirohia e tō rōpū hauora te mahi o te ngakau o tō tamaiti me te kōrero mō ngā whiringa mēnā e hiahiatia ana.

Ka taea e au te whānau atu i muri i te whānau i tētahi pēpi me te HLHS?

Ae, he maha ngā whānau e haere tonu ana ki te whānau atu i ngā tamariki. Ahakoa he iti te whakapiki ake o te tūponotanga (tau 2-3%) o te whānau i tētahi atu tamaiti me te mate ngakau, ko te nuinga o ngā tamariki e whai ake nei ka whānau me ngā ngakau hauora. Ka taea e tō taote te kōrero mō te tohutohu ira tangata mēnā e hiahia ana koe ki ngā mōhiohio taipitopito atu mō ngā tūponotanga.

E hia te roa o ngā pokanga me te aha te whakaora?

Ko ia pokanga i te nuinga o ngā wā e torutoru haora te roa, ā, ka rerekē te wā whakaora e ai ki te pokanga me te whakautu motuhake o tō tamaiti. Ko te pokanga tuatahi i te nuinga o ngā wā e hiahia ana ki te noho roa i te whare hauora, i te nuinga o ngā wiki. Pea he poto ake ngā wā whakaora o ngā pokanga e whai ake nei. Ka hoatu e tō rōpū pokanga ngā wā me ngā tumanakohanga motuhake mō ia tukanga.

He aha ngā mahi ka taea e taku tamaiti te whai wāhi i te wā e tipu ana?

Ka rerekē ngā tohutohu mahi mō ia tamaiti e ai ki tōna mahi ngakau me tōna hauora whānui. He maha ngā tamariki me te HLHS e taea ana te whai wāhi ki ngā mahi whakangahau, ngā hākinakina kura, me ngā hui pāpori. Ka mahi tahi tō kaitohutohu ngakau ki a koe hei whakatau i ngā taumata mahi e tika ana, ā, pea ka tūtohu kia kaua e whai wāhi ki ētahi hākinakina whakataetae kaha, i te wā e whakatenatena ana i ētahi atu momo mahi tinana.