Ua ʻike anei ʻoe i kekahi o kou ʻohana a i ʻole kekahi āu i ʻike ai e palupalu koke a hiolo i ka wā e hoʻoikaika kino ana a i ʻole i ka wā e makaʻu loa ai lākou? I kekahi manawa, ma hope o ia mau mea aia kahi maʻi puʻuwai i lohe ʻole ʻia e mākou, akā hiki ke koʻikoʻi loa. ʻO kekahi o ia mau maʻi ʻo Long QT Syndrome (LQTS). ʻOiai paha he paʻakikī iki ka inoa, e hoʻomaopopo maʻalahi kākou.
He aha ka Long QT Syndrome (LQTS)?
I ka ʻōlelo maʻalahi, ʻo Long QT Syndrome (LQTS) kahi pilikia me ka ʻōnaehana uila o kou puʻuwai.
E noʻonoʻo i kou puʻuwai e like me ka uila ma ke kāmela. Ma hope o kona kuʻi ʻana i hoʻokahi manawa, pono e hoʻopiha iki ʻia ma mua o ka hiki ke kuʻi hou. Pēlā e pono ai ka ʻōnaehana uila o ka puʻuwai e hoʻouka hou ma waena o nā kuʻi. ʻO ka manawa e pono ai kēia e hana ʻia ʻo ia ka mea a mākou e ana ai ma kahi ECG (Electrocardiogram) ma ke ʻano he QT interval .
ʻOi aku ka lōʻihi o kēia manawa hoʻouka hou no kekahi me LQTS ma mua o kekahi mea maʻamau. ʻO ke ʻano kēia, ʻoi aku ka lōʻihi o ka hoʻomākaukau ʻana o ka ʻōnaehana uila o ka puʻuwai no ke kuʻi aʻe.
Pehea lā e hiki ai kēia?
Hoʻokele ʻia ka hana uila o ko kākou puʻuwai e nā ʻāpana liʻiliʻi i hoʻopiʻi ʻia i ka uila i kapa ʻia he ions (no ka laʻana, sodium, potassium, calcium). Neʻe kēia mau ions i loko a i waho o nā ʻiʻo puʻuwai ma o nā puka liʻiliʻi i kapa ʻia he ion channels .
I loko o ka Long QT Syndrome, ʻaʻole hana pono kēia mau kahawai ion a i ʻole ua nele lākou i ka lawa. ʻO kēia ke kumu o ka hoʻouka hou ʻana o ka puʻuwai i ka wā lohi.
Ke hoʻolōʻihi ʻia ka manawa QT ma kēia ʻano, hiki ke loaʻa kahi puʻuwai puʻuwai weliweli loa, hoʻoweliweli i ke ola, wikiwiki, a kūpono ʻole (arrhythmia). Ma nā ʻōlelo lapaʻau, kapa mākou i kēia ʻo Torsades de Pointes . He ʻano kēia o kahi kūlana weliweli i kapa ʻia ʻo Ventricular Tachycardia .
He aha nā ʻano nui o LQTS?
Hiki ke puʻunaue ʻia nā LQTS i ʻelua ʻano nui: hoʻoilina a loaʻa.
1. LQTS hoʻoilina/hānau
Hoʻokumu ʻia kēia e kahi kīnā hoʻoilina. ʻO ia hoʻi, ua hoʻoilina ʻia mai nā mākua a i nā keiki. Maanei pū kekahi, aia kekahi mau ʻano nui.
| ʻAno hānau o LQTS | He wehewehe maʻalahi |
|---|---|
| ʻO ka maʻi Romano-Ward | ʻO kēia ke ʻano maʻamau. I kēia hihia, loaʻa paha i kekahi o nā mākua ka LQTS. Aia he 50% ka manawa e hoʻoilina ai ke keiki i ke kūlana. He mea maʻamau ka lohe ʻana (lohe) i kēia poʻe. |
| ʻO Jervell a me Lange-Nielsen Syndrome | He mea kākaʻikahi loa kēia. I kēia hihia, ʻo nā mākua ʻelua he mau mea lawe o ka gene LQTS (ʻaʻole paha lākou e loaʻa nā hōʻailona). Aia he 25% ka manawa e hoʻoilina ai ke keiki i ke kūlana. ʻO nā keiki me kēia kūlana, hānau kuli pinepine lākou. |
| ʻO ka maʻi Timoteo | He ʻano laha ʻole nō hoʻi kēia. Hiki iā ia ke hoʻopilikia ʻaʻole wale i ka puʻuwai, akā, i nā ʻāpana ʻē aʻe o ke kino. |
2. Loaʻa nā LQTS
ʻAʻole kēia i hana ʻia e nā kumu hoʻoilina. Hiki i kekahi poʻe ke hānau ʻia me kahi nāwaliwali iki i kā lākou mau kahawai ion. Akā, ʻaʻole ia he pilikia. Eia nō naʻe, hiki ke kū mai kēia kūlana ma muli o kekahi kumu o waho.
ʻO nā kumu nui:
- ʻO kekahi mau lāʻau lapaʻau: Kekahi mau antibiotics, antidepressants, antiarrhythmics, a me diuretics.
- Hoʻemi ʻia ka paʻakai o ke kino: Hoʻemi ʻia ka nui o ka potassium, magnesium, a i ʻole calcium i loko o ke koko.
- Nā kumu ʻē aʻe: Nā maʻi ʻai e like me ka anorexia nervosa, ka hōʻino ʻana i ka ʻōnaehana nerve, a me kekahi mau mea ʻawahia.
He aha kēia mau hōʻailona?
ʻO ka mea weliweli loa e pili ana i ka LQTS ʻo ia ka ʻike ʻole ʻia o kekahi mau hōʻailona ma kahi o 50% o ka poʻe . Eia nō naʻe, ke kū mai nā hōʻailona, he koʻikoʻi pinepine lākou. Hiki mai kēia mau hōʻailona ke kū mai ke ʻano o ka puʻuwai weliweli i ʻōlelo ʻia ma mua i kapa ʻia ʻo Torsades de Pointes.
| ʻO ka hōʻailona nui | He aha ka mea e hana ʻia ai? |
|---|---|
| ʻO ka syncope | Ma muli o ka puʻuwai kūpono ʻole, ʻaʻole lawa ke koko i ka lolo. Hiki i kēia ke kumu o ka nalowale koke ʻana o ka ʻike a me ka hiolo ʻana. |
| Nā hopu ʻana | Hiki ke hana ʻia kekahi kūlana i kapa ʻia he hopu ʻana ma muli o ka emi ʻana o ke kahe o ke koko i ka lolo i kekahi manawa. |
| Hoʻopaʻa ʻia ka puʻuwai | Inā hoʻomau kēlā ʻano puʻuwai weliweli, e oki loa paha ka puʻuwai i ke kuʻi ʻana. |
| Make koke | Hiki ke make ka hopu ʻana o ka puʻuwai inā ʻaʻole mālama koke ʻia. I kekahi manawa, ʻo ia ka hōʻailona mua o kekahi me LQTS. |
Nā kūlana kahi e ʻike nui ʻia ai nā hōʻailona:
- ʻOiai ʻoe e hoʻoikaika kino ana a ma hope paha o kou pau ʻana.
- I ka wā e hoʻoulu koke ʻia ai ka noʻonoʻo ikaika (e.g., i ka wā makaʻu loa, pūʻiwa).
- I ka wā hiamoe a i ʻole i ke ala koke ʻana mai ka hiamoe.
ʻO wai ka mea i ʻoi aku ka pilikia o ka loaʻa ʻana o kēia maʻi?
ʻOi aku ka nui o ka pilikia o ka poʻe ma lalo nei i ka hoʻomohala ʻana i ka LQTS:
- Nā hoahānau pili koko (nā mākua, nā kaikaina, nā keiki) o kekahi me LQTS.
- ʻO nā kānaka me ka moʻolelo ʻohana o ka nalowale o ka hoʻomanaʻo i wehewehe ʻole ʻia, nā hopu ʻana, a i ʻole ka make koke ʻana i ka wā ʻōpiopio.
- Nā keiki i hānau kuli (ma muli o ka pilikia o Jervell a me Lange-Nielsen Syndrome).
- ʻO nā kānaka me nā maʻi puʻuwai ʻē aʻe e like me ka cardiomyopathy.
- ʻO ka poʻe e hoʻohana ana i nā lāʻau lapaʻau e hoʻolōʻihi i ka wā QT.
Inā ua ʻike ʻia kekahi o kou ʻohana me LQTS, he manaʻo maikaʻi e hoʻāʻo ʻia no ia mea. ʻO ka mea mua, e haʻi i kāu kauka e pili ana i ia mea. E kuhikuhi ʻo ia iā ʻoe no kahi hoʻāʻo ECG.
Pehea ʻoe e ʻike ai inā loaʻa iā ʻoe ʻo LQTS?
ʻO ke ala nui e ʻike ai i kēia ma o ka hoʻohana ʻana i ka ECG (electrocardiogram).Hoʻāʻo. Ana ke kauka i ka wā QT ma ka ECG. ʻO ka maʻamau, inā ʻoi aku ka lōʻihi o kēia manawa ma mua o 450 milliseconds, hiki ke kānalua ʻia ʻo LQTS.
Eia kekahi, hiki i ke kauka ke ʻōlelo i kekahi mau hoʻokolohua ʻē aʻe:
- Nā hoʻāʻo koko: E nānā i nā pae o nā paʻakai i loko o ke kino, e like me ka potassium a me ka magnesium.
- Hoʻāʻo Koʻikoʻi Hoʻoikaika Kino: E ʻike pehea e loli ai ka hana o kou puʻuwai i ka wā o ka hoʻoikaika kino.
- Nānā Ambulatory (Holter Monitor): Kau ʻia kahi mea liʻiliʻi ma kou umauma e hoʻopaʻa mau i ka puʻuwai o kou puʻuwai no 24 mau hola a ʻoi aku paha.
- Ka hoʻāʻo ʻana i ka genetic: E nānā i nā hoʻololi genetic e pili ana me LQTS.
He aha nā lāʻau lapaʻau no kēia?
ʻOiai ʻaʻole hiki ke ho'ōla loa ʻia ʻo LQTS, aia nā lāʻau lapaʻau e hiki ke kōkua i ka kaohi ʻana i nā hōʻailona, pale i ka pilikia o ka make koke, a alakaʻi i kahi ola maʻamau.
| ʻAno lapaʻau | Wehewehena |
|---|---|
| Nā lāʻau lapaʻau | Ua hāʻawi ʻia nā lāʻau lapaʻau beta-blocker i nā kānaka he nui (ʻoiai ka poʻe me ka ʻole o nā hōʻailona). Kōkua kēia i ka hoʻomalu ʻana i ka helu o ka puʻuwai a hōʻemi i ka pane ʻana o ka puʻuwai i nā mea hoʻonāukiuki koke. |
| Nā Hāmeʻa |
|
| ʻoki kino | ʻO ka Left Cardiac Sympathetic Denervation (LCSD) no nā poʻe maʻi kiʻekiʻe i paʻakikī ke kaohi me nā lāʻau lapaʻau a me nā mea hanaʻO kēia ʻoki kino e pili ana i ka wehe ʻana i kekahi o nā aʻalolo e hele ana i ka puʻuwai, e hōʻemi ana i ka pane a ka puʻuwai i nā hoʻonāukiuki koke. |
Nā mea e noʻonoʻo ai i ka wā e noho ana me LQTS
Hiki ke ola i kahi ola maʻamau a piha me LQTS. Eia nō naʻe, aia kekahi mau mea e pono ai ʻoe e makaʻala i kou ola o kēlā me kēia lā.
1. Nā walaʻau koke a me ke kaumaha o ka noʻonoʻo
No nā poʻe me kekahi ʻano LQTS, hiki i nā leo koke a leo nui (e like me ke kani ʻana o ka alarm, ke kani ʻana o ke kelepona) a i ʻole ka haʻalulu koke a makaʻu paha ke lilo i mea weliweli.
- E haʻi aku i ka ʻohana a me nā hoaaloha ʻaʻole palekana ke hoʻohenehene iā ʻoe.
- Ma kahi o nā uaki hoʻāla leo nui, e hoʻohana i nā mea e hoʻokani ana i nā mele mālie.
2. Hoʻoikaika kino a me nā haʻuki
No nā poʻe me kekahi ʻano LQTS, hiki i nā haʻuki hoʻokūkū a me ka hoʻoikaika kino ke lilo i mea weliweli loa.
- E nīnau pono i kāu kauka i nā hoʻoikaika kino kūpono a kūpono ʻole iā ʻoe.
- He mea weliweli loa ka ʻauʻau ʻana no kekahi poʻe. Aia ka pilikia o ka piholo ʻana inā nalowale kou ʻike i loko o ka wai. Mai ʻauʻau hoʻokahi.
3. E akahele loa me nā lāʻau lapaʻau!
ʻO kēia ka mea nui loa. Hiki i nā lāʻau lapaʻau maʻamau he nui ke hoʻonui i ka LQTS.
- E haʻi i nā kauka a pau e mālama iā ʻoe he LQTS kou.
- Ma mua o ke kau ʻana i ka lāʻau lapaʻau no kekahi maʻi ʻē aʻe, e kamaʻilio me kāu cardiologist.
- Mai kūʻai i kekahi lāʻau lapaʻau ma waho o ke kūʻai aku ma kahi hale kūʻai lāʻau a i ʻole ma kahi ʻē aʻe me ka ʻole o ke kūkākūkā ʻana me ke kauka (koe wale nō nā mea e like me ka paracetamol maʻemaʻe a me ka aspirin).
Āhea wau e ʻike ai i ke kauka?
Ke ʻike ʻia ʻoe me LQTS, pono ʻoe e ʻike i kāu kauka ma ka liʻiliʻi hoʻokahi manawa i ka makahiki no ka nānā ʻana. ʻOi aku ka maikaʻi o ka ʻike ʻana i kahi electrophysiologist, he kauka loea i nā maʻi puʻuwai.
Inā loaʻa kēia maʻi i kāu keiki, pono ʻoe e ʻike pinepine i ke kauka no ka mea pono e hoʻoponopono ʻia ka nui o ka lāʻau lapaʻau e like me ko lākou kaumaha.
Āhea wau e hele ai i ka ETU (Emergency Treatment Unit)?
Inā ua poina kekahi a hāʻule, e kāhea koke aku i kahi kaʻa kaʻa. I kēia manawa, inā he kanaka i aʻo ʻia e hana i ka CPR (cardiopulmonary resuscitation), e hāʻawi. I ke kūlana e like me kēia, he mea nui kēlā me kēia kekona.
Leka Lawe-Home
- ʻO ka Long QT Syndrome (LQTS) kahi maʻi genetic a i ʻole ke ʻano i loaʻa kahi e lōʻihi ai ka hoʻouka hou ʻana o ka ʻōnaehana uila o ka puʻuwai.
- ʻO ka nalowale koke ʻana o ka ʻike, nā hopu ʻana, a me ka hāʻule ʻana o ka puʻuwai i wehewehe ʻole ʻia nā hōʻailona nui. Eia nō naʻe, ʻaʻole paha e ʻike ka nui o ka poʻe i kekahi mau hōʻailona.
- Inā loaʻa i kekahi o kou ʻohana kēia maʻi a i ʻole he moʻolelo o ka make koke i ka wā ʻōpiopio, he mea nui loa e loaʻa iā ʻoe kahi hoʻokolohua ECG.
- Ma mua o ka lawe ʻana i kekahi lāʻau lapaʻau (ʻoiai no nā kūlana ʻē aʻe), e kamaʻilio me kāu kauka e hōʻoia i ka palekana no ʻoe.
- Me ka mālama kūpono (lāʻau lapaʻau, hāmeʻa ICD) a me nā hoʻololi ʻana i ke ʻano o ka nohona, hiki i kekahi me LQTS ke ola i kahi ola piha a olakino. ʻAʻohe pono e makaʻu, akā he mea nui e makaʻala.
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