Ke kuʻi koke nei kou puʻuwai me ke ʻano ʻē? He Brugada Syndrome paha ia?

Ua nalowale koke anei kou ʻike, ua manaʻo ʻoe ua puni ka maka? A i ʻole ua loaʻa iā ʻoe ka manaʻo o ka puʻuwai wikiwiki, a i ʻole ka paʻakikī o ka hanu ʻana? I kekahi manawa hiki ke hana ʻia kēia e kahi mea i emi iki ka koʻikoʻi. Eia nō naʻe, i nā hihia kakaikahi, hiki iā lākou ke lilo i mau hōʻailona o kahi maʻi puʻuwai i kapa ʻia ʻo Brugada Syndrome. E kamaʻilio iki kākou e pili ana i kēia i kēia lā, no ka mea, he mea nui e makaʻala i ia mea.

He aha ka Brugada Syndrome?

I ka ʻōlelo maʻalahi, ʻo Brugada Syndrome kahi kūlana e hoʻoulu ai i ka hana uila maʻamau ʻole i loko o nā keʻena haʻahaʻa o kou puʻuwai, i kapa ʻia ʻo ventricles. Hiki i kēia ke lilo koke i kou puʻuwai i kona rhythm. Ua kapa ʻia kēia he ventricular fibrillation a i ʻole v-fib . E noʻonoʻo i kou puʻuwai e lele ana ma kahi o ka pamu ʻana i ke koko pono. ʻO kēia ke kumu o ka loaʻa ʻole o ke koko i kou lolo. ʻO ia ka manawa e nalowale ai kou ʻike (syncope) .

Hiki i kēia ke lilo i kūlana weliweli, no ka mea, hiki i kēia 'v-fib' ke alakaʻi i ka make koke o ka puʻuwai . ʻO ka hapa nui o ka manawa, hana ʻia kēia i ka wā e hoʻomaha ai ʻoe a hiamoe paha. Wahi a nā kānaka noiʻi ʻo Brugada Syndrome ke kuleana no kahi 4% o nā make koke o ka puʻuwai ma ka honua holoʻokoʻa.

Akā, ʻaʻole kēia he maʻi maʻamau loa. Ma kahi o ʻekolu a ʻehā paha kānaka mai loko mai o nā tausani he ʻumi i loaʻa i kēia maʻi.

He aha nā hōʻailona e ʻike ʻia?

Hiki ke ʻike ʻia nā hōʻailona o ka Brugada Syndrome i kēlā me kēia makahiki. Eia nō naʻe, hoʻomaka pinepine lākou ma kahi o 40 mau makahiki. ʻAʻole paha e hōʻike iki kekahi poʻe i kekahi mau hōʻailona. Ua ʻōlelo ʻia ma mua o 70% o ka poʻe ʻaʻohe o lākou hōʻailona. Eia naʻe, inā ʻike ʻia nā hōʻailona, ​​​​hiki iā lākou ke komo pū me:

• Puʻuwai puʻuwai kūpono ʻole (`ventricular arrhythmia`) : ʻO ke ʻano kēia ua hoʻopilikia ʻia ke kuʻina o ka puʻuwai.
• Syncope: Ka nalowale koke o ka ʻike a me ka hiolo ʻana.
• Pōniuniu : He manaʻo o ka wili ʻana.
• Paʻakikī ka hanu ʻana .
• Nā palpitations o ka puʻuwai : He manaʻo o ka lele ʻana a i ʻole ka wili ʻana i loko o ka umauma.
• Hoʻopau ʻia ka puʻuwai : ʻO kēia kekahi o nā hōʻailona mua e ʻike ʻia. Make paha kekahi poʻe i ko lākou hiamoe ʻana ma muli o kēia.

Mea nui: Inā loaʻa pinepine iā ʻoe kekahi a ʻoi aku paha o kēia mau hōʻailona, ​​​​ʻoi aku ka maikaʻi o ke kipa ʻana i ke kauka no ka ʻōlelo aʻo.

He aha nā mea e hoʻoikaika ai i ka Brugada syndrome?

Hiki i kekahi mau mea ke hoʻoulu i nā hōʻailona o ka maʻi Brugada e ʻike koke ʻia a i ʻole e ʻoi aku ka ʻino. Kāhea mākou i kēia mau mea he 'mea hoʻoulu'.

• Kuni : Hiki ke ʻike ʻia kēia mau hōʻailona ke loaʻa i kahi kanaka me Brugada syndrome ke kuni.He mea nui loa ka hoʻohaʻahaʻa koke ʻana i ke kuni . ʻOiai inā he implantable cardioverter defibrillator (ICD) kāu, pono nō ʻoe e hoʻohaʻahaʻa i ke kuni. Hiki ke hana ʻia kēia me nā lāʻau lapaʻau hoʻohaʻahaʻa kuni i loaʻa ma ka hale kūʻai lāʻau lapaʻau.
• Luhi loa i ka wela / Make wai : Paʻa ka wai i loko o ke kino, luhi ma muli o ka wela nui.
• ʻO kekahi mau lāʻau lapaʻau :
• Nā mea ālai i ke kahawai sodium.
• Litiuma.
• Nā lāʻau antidepressant Tricyclic.
• ʻOna : Inu ʻana i ka waiʻona nui loa.
• Nā lāʻau lapaʻau : Ke hoʻohana nei i nā lāʻau lapaʻau e like me ka cocaine a me ka marijuana.

He mea nui loa ka ʻike ʻana i kēia mau mea a pale aku iā lākou i ka hiki.

He aha nā kumu no kēia?

ʻAʻole ʻike ʻia ke kumu kikoʻī o Brugada Syndrome i kekahi manawa. A i ʻole he genetic paha. ʻAʻole maopopo loa i kēia manawa ka mutation genetic e hoʻoulu ai i kēia maʻi ma kahi o 70% o ka poʻe.

Eia nō naʻe, he 18 a ʻoi aku paha nā ʻano like ʻole o kekahi poʻe. ʻO ka mea maʻamau o kēia he ʻano like ʻole o kahi gene i kapa ʻia ʻo SCN5A . Hoʻopilikia kēia mau ʻano like ʻole i ke ala e hele ai nā hōʻailona uila i loko o ka puʻuwai.

He maʻi kēia e hiki ke hoʻoilina ʻia mai hoʻokahi makua wale nō . ʻO ia hoʻi, he 50% ka manawa e loaʻa ai i kahi keiki a kekahi me kahi hoʻololi gene e pili ana me ka maʻi Brugada.

ʻO wai ka mea i ʻoi aku ka paʻakikī o ka loaʻa ʻana o kēia? (Nā kumu pilikia)

ʻOi aku ka maʻamau o kēia maʻi i nā kāne . He 8 a 10 mau manawa ka nui o ka maʻamau i nā kāne ma mua o nā wahine.

Eia kekahi, inā ua make kekahi o kou ʻohana i ka hopu koke ʻana o ka puʻuwai, a i ʻole ua ʻike ʻia ʻo ia me Brugada syndrome , he mea naʻauao nō hoʻi iā ʻoe ke hoʻāʻo ʻia no kēia.

ʻO kekahi mea ʻē aʻe, ua ʻōlelo ʻia ua ʻoi aku ka maʻamau o kēia maʻi i waena o ka poʻe o ka hanauna ʻĀsia .

Pehea e ʻike ai ke kauka i kēia? (Diagnosis)

No ka hoʻoholo ʻana inā loaʻa iā ʻoe ʻo Brugada Syndrome, e hana ke kauka i kēia mau mea:

• E hāʻawi ʻia ʻoe i kahi hoʻokolohua kino .
• E nānā ʻia kāu mōʻaukala olakino .
• E nīnau lākou e pili ana i ka mōʻaukala olakino o kou ʻohana (mōʻaukala ʻohana biological) , ʻoiai e ʻike inā ua make koke kekahi o kou ʻohana i ka make puʻuwai.
• E noi lākou iā ʻoe e hana i kekahi mau hoʻokolohua kūikawā .

He aha nā hoʻokolohua e hana ʻia?

Aia kekahi mau hoʻokolohua nui i hoʻohana ʻia e ʻike i kēia maʻi:

• Hoʻāʻo ʻana i ka geneticsHiki i kēia hoʻāʻo ke hoʻoholo inā he ʻano hoʻololi kikoʻī kou e pili ana me ka maʻi Brugada ma ka lawe ʻana i kahi hāpana o kou lawai a i ʻole ke koko. He manaʻo maikaʻi nō hoʻi e hana i kēia hoʻāʻo e ʻike inā loaʻa i nā poʻe ʻē aʻe o kou ʻohana kēia ʻano hoʻololi. ʻOi loa aku inā he ʻohana pili kou ('ʻohana mua') me ka maʻi Brugada, e like me kou mau mākua, nā kaikaina, a i ʻole nā ​​keiki, pono ʻoe e nānā ʻia no kēia .
• Electrocardiogram (EKG a i ʻole ECG) : Hoʻohana ʻia kēia e hoʻopaʻa i ka hana uila o kou puʻuwai i kona wā e kuʻi ai. Ke nānā nei nā kauka i kahi ʻano kikoʻī (ke ʻano Brugada syndrome type 1 EKG) ma ka EKG. I kekahi manawa hiki ke ʻike ʻia kahi ʻano nāwaliwali i kapa ʻia ʻo type 2 a i ʻole type 3, akā ʻaʻole hiki i kēlā wale nō ke hōʻoia i ka maʻi. Eia naʻe, ma muli o nā mea hoʻoulu i ʻōlelo ʻia ma luna (e.g. kuni), hiki ke loli kēia ʻano 2 a i ʻole type 3 i kahi ʻano type 1. I kekahi manawa ʻaʻole ʻike koke ʻia kēia ʻano ma ka EKG, no laila hiki i ke kauka ke hana hou i ka EKG ʻelua a ʻekolu paha manawa, e hoʻololi ana i nā alakaʻi. I kekahi manawa, hana ʻia kahi EKG ma hope o ka ʻai piha.
• EKG me ka lāʻau lapaʻau (hoʻāʻo hoʻokūkū lāʻau lapaʻau) : Ma mua o ka EKG, hiki i kāu kauka ke hāʻawi iā ʻoe i kahi lāʻau lapaʻau e kōkua i kāu EKG e ʻike maopopo inā loaʻa iā ʻoe ka maʻi Brugada. Hoʻohana ʻia kēia hoʻāʻo e ʻike inā he ʻano 2 a ʻano 3 paha kāu EKG, hiki ke loli i kahi ʻano 1.

Ma muli o kāu mau hopena EKG, hiki ke hana ʻia nā hoʻokolohua hou aʻe:

• Hoʻāʻo Electrophysiology (EP) : Pili kēia i ka hoʻokomo ʻana i kahi ʻōmole lahilahi (catheter) ma o kahi aʻa nui i kou wāwae (aʻa femoral) i loko o kou puʻuwai e ana i ka hana uila i loko o kou puʻuwai. Hiki ke hana ʻia kēia inā ʻaʻole maopopo ka hōʻailona ma hope o nā hoʻokolohua ʻē aʻe.
• Nā hoʻāʻo lab: Hana ʻia nā hoʻāʻo koko e nānā i nā pae o nā electrolytes e like me ka potassium, calcium, a me ka magnesium i loko o ke koko, a e nānā i nā kumu ʻē aʻe o nā rhythms puʻuwai kūpono ʻole.

Pehea e mālama ai? (Lapaʻau)

ʻO ka pahuhopu nui o ka mālama ʻana i ka Brugada Syndrome ʻo ia ka pale ʻana i ka hanana ʻana o nā arrhythmias ventricular a me ka hoʻomalu koke ʻana iā lākou inā loaʻa.

Hiki iā ʻoe ke hāʻawi iā ʻoe iho i nā lāʻau lapaʻau e like me kēia:

• Nā lāʻau lapaʻau e like me Isoproterenol .
• Nā lāʻau lapaʻau e like me ka quinidine .
• He mea liʻiliʻi i hoʻokomo ʻia i loko o ka puʻuwai (Implantable Cardioverter Defibrillator - ICD)Inā ua loaʻa iā ʻoe kahi hōʻeha puʻuwai (ventricular tachycardia), maule, a i ʻole ka hopu ʻana o ka puʻuwai, hiki ke hoʻokomo ʻia ʻoe me kahi ICD. ʻIke kēia mea hana i kahi ʻano puʻuwai weliweli a hāʻawi i kahi haʻalulu uila liʻiliʻi i ka puʻuwai e hoʻihoʻi iā ia i ka maʻamau.
• ʻO ka Ablation ( i kekahi mau hihia): Pili kēia i ka ʻike ʻana a me ka luku ʻana i nā wahi o ka puʻuwai e hoʻoulu ai i nā hōʻailona uila maʻamau ʻole.

ʻOiai inā ʻaʻohe ou hōʻailona, ​​​​hiki i kāu kauka ke hoʻoholo e hoʻokomo i kahi ICD ma muli o kou mōʻaukala ʻohana a me nā hopena hoʻāʻo. Hiki i kekahi mau kauka ke hoʻoholo e nānā mau iā ʻoe a mālama wale iā ʻoe inā loaʻa iā ʻoe nā hōʻailona. Eia nō naʻe, ʻaʻole paha e ʻae nā kauka ʻē aʻe i kēia ʻano hana, no ka mea, ʻo kāu hōʻailona mua paha ka make koke ʻana o ka puʻuwai.

Aia kekahi mau pilikia i ka mālama ʻana?

ʻAʻole hiki ke palekana piha nā ICD. I kekahi manawa hiki ke hana ʻia nā mea e like me kēia:

• Hiki iā ʻoe ke hāʻawi i kahi hoʻohoka i ka manawa kūpono ʻole .
• Aia paha he hewa ma kekahi o nā kaula o ka mīkini.
• Hiki ke loaʻa kahi maʻi a puni ka mīkini.

No laila, ma hope o ke kau ʻana i kahi ICD, he mea nui loa e hele i nā hoʻokolohua e like me ka ʻōlelo a ke kauka.

ʻEhia ka lōʻihi e ho'ōla ai?

Ma hope o ke kau ʻia ʻana o kahi ICD, hiki iā ʻoe ke hana i ka hapa nui o kāu mau hana maʻamau i loko o kekahi mau lā. Eia nō naʻe, pono ʻoe e hoʻōki i ka hoʻokele kaʻa no hoʻokahi pule. Hiki iā ʻoe ke hana i kekahi hana ikaika i kēlā me kēia lā, akā mai hana a hiki i ka luhi. Mai hāpai i nā mea kaumaha a hana paha i ka hoʻoikaika kino a hiki i ka wā e ʻōlelo ai kāu kauka iā ʻoe.

Hiki ke pale ʻia kēia? (Kāohi ʻana)

Inā hoʻoilina ʻoe i ka Brugada Syndrome mai kou mau mākua mai, ʻaʻole hiki ke hoʻololi ʻia. Eia nō naʻe, inā ʻike ʻoe he kanaka o kou ʻohana ka maʻi Brugada Syndrome, hiki iā ʻoe a me kou ʻohana ke hana i kahi hoʻāʻo genetic e ʻike ai inā loaʻa iā ʻoe.

Eia kekahi, inā ʻoe e manaʻolana ana e loaʻa kahi keiki, hiki iā ʻoe ke hālāwai me kahi kākāʻōlelo genetic a kamaʻilio me lākou e pili ana i ka pilikia o ka hoʻoili ʻana i kēia i kāu mau keiki.

He aha kāu e manaʻo ai i kou wā e noho ana me ka Brugada syndrome?

ʻAʻohe lāʻau lapaʻau no Brugada Syndrome. Eia nō naʻe, hiki i ka lāʻau lapaʻau ke hōʻemi nui i ka pilikia o ka make koke ʻana o ka puʻuwai.

ʻO ka poʻe me ka maʻi Brugada i loaʻa nā hōʻailona akā ʻaʻole i loaʻa ka lāʻau lapaʻau, ʻoi aku ka nui o ka pilikia o ka make koke ʻana o ka puʻuwai. ʻOi aku ka haʻahaʻa o kēia pilikia i ka poʻe i loaʻa nā hōʻailona a he EKG maʻamau ko lākou.

Pehea e mālama ai iaʻu iho? (Mālama ponoʻī)

Inā haʻi aku ke kauka iā ʻoe he Brugada Syndrome kou, ʻo ka mea nui loa, ʻo ia ka pale ʻana i nā mea hoʻonāukiuki he nui e hiki ke hoʻopilikia i kēlā mele.

• E pale i ke kuni. Inā loaʻa iā ʻoe ke kuni, e hoʻohaʻahaʻa koke.
• E noho mamao aku mai kekahi mau lāʻau lapaʻau a me nā mea ʻino . E noi i kāu kauka no kahi papa inoa o nā lāʻau lapaʻau āu e maʻi ai.
• E hōʻalo loa i ka waiʻona nui a me nā lāʻau lapaʻau .
• E inu i ka wai he nui e pale aku ai i ka dehydration .
• E akahele i ka wā e hana ai i nā hana hoʻoikaika kino .

Āhea ʻoe e pono ai e ʻike i ke kauka? / Pono ʻoe e hele i ka lumi pilikia?

Inā loaʻa iā ʻoe ka maʻi Brugada, pono ʻoe e ʻike i kāu kauka ma ka liʻiliʻi hoʻokahi manawa i ka makahiki no ka nānā ʻana . Inā loaʻa iā ʻoe kahi ICD, pono ʻoe e nānā ʻia ma ka liʻiliʻi ʻelua i ka makahiki . Inā manaʻo ʻoe i kekahi mea ʻē, e haʻi i kāu kauka .

Pilikia! Inā loaʻa iā ʻoe kahi hopu puʻuwai, ʻaʻole hiki iā ʻoe ke kōkua iā ʻoe iho. No laila, pono ʻoe i kekahi e kōkua iā ʻoe. Inā ʻoe i ka pilikia o ka hopu ʻana o ka puʻuwai, e haʻi i kou ʻohana e aʻo i ka CPR (Cardiopulmonary Resuscitation) , a kāhea iā 1990 (ka lawelawe ambulance ulia pōpilikia o Sri Lanka) a i ʻole ka helu ulia pōpilikia kokoke loa. E hoʻomaopopo pū i kāu wahi hana e pili ana i kēia pilikia.

Nā nīnau koʻikoʻi e nīnau aku i kāu kauka

Ke hele ʻoe e ʻike i ke kauka, mai poina e nīnau i nā nīnau e like me kēia:

• Pono anei iaʻu e loaʻa kahi ICD?
• Pehea e pono ai ke mālama ʻia kahi ICD?
• Hiki iā ʻoe ke haʻi mai iaʻu e pili ana i kahi hui kākoʻo kahi e hiki ai i nā poʻe ʻē aʻe me kēia maʻi ke hui pū?
• He aha nā lāʻau lapaʻau e pono iaʻu e pale aku? Hiki iā ʻoe ke hāʻawi mai iaʻu i kahi papa inoa?
• Pono anei e hoʻāʻo ʻia ke koena o koʻu ʻohana no kēia?

Leka Lawe-Home

He mea maʻamau ke kaumaha ke ʻike ʻoe he maʻi kou e hiki ai ke alakaʻi i ka hāʻule puʻuwai koke. Eia nō naʻe, ʻo ka ʻike ʻana ua pilikia ʻoe e hāʻawi iā ʻoe i ka manawa kūpono e hana i nā hana e hōʻemi ai i kēlā pilikia . E ʻimi kāu kauka i ke koho lapaʻau maikaʻi loa nāu.

Inā hiki, he mea hōʻoluʻolu nui ia inā loaʻa i kou home a me ke kula o kāu keiki kahi Automatic External Defibrillator (AED) . Eia kekahi, e noi i kou mau hoalauna a me nā hoa hana e aʻo i ka CPR. Hiki i kēia mau mea ke kōkua i ka hoʻopakele ʻana i nā ola. E hahai mau i nā ʻōlelo a kāu kauka, e hana i nā nānā maʻamau, a pale i nā mea hoʻoweliweli ola .

ʻO ka maʻi Brugada, nā arrhythmias puʻuwai, ka hopu koke ʻana o ka puʻuwai, nā maʻi genetic, EKG, ICD, maʻi puʻuwai