ʻO ka Marfan Syndrome a me kou puʻuwai: E kamaʻilio kākou e pili ana i kēia!

Ua ʻike paha ʻoe i nā kānaka i ʻoi aku ke kiʻekiʻe, he lōʻihi ko lākou mau lālā, a ʻoi aku ka lahilahi ma mua o nā poʻe ʻē aʻe. Loaʻa i nā kānaka a pau kēia maʻi, akā ʻaʻole ia he maʻi. Akā i kekahi manawa, hiki i kēia ʻano kino ke lilo i hōʻailona o kahi maʻi genetic i kapa ʻia ʻo Marfan Syndrome . He ʻano kēia e kū mai ana i ka wā ʻaʻole i ulu pono nā ʻiʻo pili i loko o ko mākou kino. I ka ʻōlelo maʻalahi, ʻo kēia ʻiʻo pili ka mea e hoʻopaʻa ai i nā ʻāpana like ʻole o ko mākou kino a hāʻawi iā lākou i ka ikaika. Ke hoʻonāwaliwali ʻia kēia mau mea, hiki ke hoʻopilikia ʻia nā ʻāpana he nui o ke kino, ʻoiai ka puʻuwai, nā maka, nā kīʻaha koko, a me ka ʻōnaehana iwi . I kēia lā, e kamaʻilio mākou e pili ana i ke ʻano o kēia ʻano i kapa ʻia ʻo Marfan Syndrome i ka puʻuwai.

Pehea ka hopena o ka Marfan Syndrome i ka puʻuwai?

Ke loaʻa iā ʻoe ka Marfan syndrome, ʻelua mau ʻāpana nui o ka puʻuwai e hoʻopilikia nui ʻia.

1. Aorta: ʻO kēia ke kīʻaha koko nui loa e lawe ana i ke koko momona i ka oxygen mai ko kākou puʻuwai a i ke kino holoʻokoʻa. Ua like ia me ka paipu nui e lawe ana i ka wai mai kahi pahu wai i kou hale.

2. Nā kiwikā puʻuwai: ʻO kēia nā ʻāpana e hana ana e like me nā puka ma waena o nā keʻena i loko o ka puʻuwai a me nā aa koko nui e lawe ana i ke koko i waho o ka puʻuwai. Hōʻoia lākou e kahe ke koko i hoʻokahi ala wale nō.

E nānā kākou i kēia mau mea a pau no ka mea e hana ʻia ana.

He aha ka mea e hana ʻia i ka aorta?

ʻO ka maʻi Marfan ke kumu o ka nāwaliwali o nā ʻiʻo pili i loko o nā paia o ka puʻuwai. E like me ka paipu laholio kahiko, nalowale kona ikaika a me kona elasticity. Hiki i kēia ke alakaʻi i ʻelua mau pilikia nui:

• Dilation Aortic: Hoʻomaka mālie ka aorta e hoʻonui a hoʻonui.
• ʻAneurysm Aortic: Ma kekahi mau wahi, lilo ka paia o ka puʻuwai i nāwaliwali a hiki ke puʻupuʻu i waho e like me ka pōpō.

E noʻonoʻo, ua like kēia aneurysm me kahi huila paikikala e puʻupuʻu ana mai kekahi wahi ke nāwaliwali ka ʻōmole.

ʻO ka mea nui loa, ʻo ka ʻāpana o ka puʻuwai kokoke loa i ka puʻuwai, i kapa ʻia ʻo 'aortic root', ʻo ia ka ʻāpana i hoʻonui pinepine ʻia a hoʻonui ʻia paha ma kēia ʻano i nā poʻe me ka Marfan syndrome. He kūlana weliweli loa kēia, no ka mea, inā ulu nui kēia 'aneurysm' a pahū ('aortic dissection' a haki paha), hiki ke hoʻoweliweli i ke ola.

ʻO nā kūlana ʻē aʻe e like me ka maʻi Ehlers-Danlos, ka maʻi Loeys-Dietz, ka valve aortic bicuspid, a me ka maʻi Turner hiki ke hoʻoulu i ka hoʻonui ʻana o ka puʻuwai, akā hiki ke hana ʻia ma nā wahi ʻē aʻe o ka puʻuwai.

He aha ka mea e hana ʻia i nā kiwikā o ka puʻuwai?

Hiki i ka maʻi Marfan ke hoʻopilikia i nā valves o ka puʻuwai. Inā ʻaʻole hana pono kēia mau valves, pono ka puʻuwai e hana ikaika e pamu i ke koko. I ka hala ʻana o ka manawa, hiki i kēia ke alakaʻi i ka hāʻule ʻana o ka puʻuwai.Hiki iā ia ke hele i kēlā me kēia ʻaoʻao. ʻElua mau maʻi valve nui i ʻike ʻia e pili ana me ka Marfan syndrome:

• Ka luaʻi ʻana o ka valve aortic: Ke pani pono ʻole ʻia ka valve ma waena o ka aorta a me ke keʻena hema haʻahaʻa o ka puʻuwai (ka ventricle hema), e kahe hou ana kekahi o ke koko i hoʻopaʻa ʻia i loko o ka puʻuwai. Ua like ia me ka faucet e leaka ana.
• ʻO ka mitral valve prolapse: ʻO ka mitral valve, aia ma waena o ke keʻena luna (atrium hema) a me ke keʻena haʻahaʻa (ventricle hema) ma ka ʻaoʻao hema o ka puʻuwai, ʻaʻole pani pono, a puka mai ke keʻena luna i waho. I kekahi manawa hiki i kēia ke hoʻoheheʻe i ke koko i hope (mitral regurgitation).

Pehea ka maʻamau o ka maʻi puʻuwai i nā poʻe me ka Marfan syndrome?

ʻO kaʻoiaʻiʻo, ʻoi aku ka nui o ka pilikia o ka poʻe me ka Marfan syndrome i ka loaʻa ʻana o nā pilikia puʻuwai. Ua hōʻike ʻia e ka noiʻi ʻeiwa o ka ʻumi o ka poʻe me ka Marfan syndrome e loaʻa i kekahi ʻano pilikia me ko lākou valve puʻuwai a i ʻole aorta. No laila he mea nui e makaʻala i kēia.

He aha nā hōʻailona o ka Marfan syndrome e hoʻopilikia ai i ka puʻuwai?

Inā loaʻa iā ʻoe ka Marfan syndrome, e like me ka aneurysm aortic a i ʻole ka maʻi valve, hiki iā ʻoe ke ʻike i kekahi mau hōʻailona. Eia nō naʻe, loaʻa paha i kekahi poʻe kēia mau kūlana me ka ʻole o nā hōʻailona. ʻO ia ke kumu he mea nui nā hoʻokolohua lapaʻau.

Eia nā hōʻailona i ʻike ʻia ma ke ʻano laulā:

• ʻEha umauma a ʻeha paha ke kua i luna
• Ke ʻū ʻana i ke koko (he hōʻailona ʻoi aku ka weliweli o kēia)
• Ka paʻakikī i ka ale ʻana i ka meaʻai (inā e kaomi ana ka puʻuwai i hoʻonui ʻia ma ka esophagus )
• Manaʻo poniuniu a poʻo māmā paha
• Manaʻo luhi loa a nāwaliwali paha
• Manaʻo o ka puʻuwai puʻuwai kūpono ʻole (palpitations puʻuwai)
• Ka leo haʻalulu o ka leo (inā kaomi ka puʻuwai ma luna o kahi aʻalolo e pili ana i nā kaula leo)
• Ka paʻakikī o ka hanu ʻana , ʻoiai ke luhi ʻoe a moe paha
• Ka pehu ʻana , ʻoiai ma nā wāwae a me nā kuʻekuʻe wāwae
• ʻŌhūhū (wheezing) i ka wā e hanu ana

E noʻonoʻo he hoaaloha kou i lōʻihi loa a wiwi hoʻi. Ua ʻōlelo ʻo ia he pinepine kona ʻeha umauma a he paʻakikī ke hele. Inā pēlā, he manaʻo maikaʻi e ʻike i ke kauka e ʻike inā pili ia i ka Marfan syndrome.

He aha ke kumu o ka Marfan syndrome e hoʻopilikia ai i ka puʻuwai?

E like me ka mea i ʻōlelo ʻia ma mua, ʻo ka Marfan syndrome kahi maʻi pili i nā ʻiʻo pili.ʻAʻole i hoʻokumu pono ʻia. Loaʻa nā ʻiʻo hoʻopili olakino ma nā wahi a pau o ko kākou kino. Hāʻawi ia i ka ikaika, ke ʻano, a me ka maʻalahi i nā ʻōkana a me nā kīʻaha koko. Loaʻa kēia ʻiʻo hoʻopili ma nā paia o ka puʻuwai a me nā kīʻaha koko, ʻoiai ka aorta, a ma nā valves o ka puʻuwai.

Ke nāwaliwali kēia mau ʻiʻo pili ma muli o ka maʻi Marfan, nalowale ko lākou ikaika a me ka elasticity. ʻO kēia ke kumu o ka puʻuwai e hoʻonui a pehu, ʻaʻole hiki ke kū i ke kaomi o ke koko. ʻO kēia ke kumu o ka maʻi o nā valves o ka puʻuwai a hiki ʻole ke wehe a pani pono paha.

He aha nā hoʻokolohua puʻuwai e kōkua i ka ʻike ʻana i ka Marfan syndrome?

Hiki i ka maʻi Marfan ke paʻakikī ke ʻike i kekahi manawa no ka mea ʻokoʻa nā hōʻailona mai kēlā kanaka a kēia kanaka a hiki ke hoʻohālike i nā maʻi ʻē aʻe. ʻIke ka hapa nui o ka poʻe he loaʻa iā lākou i ka wā ʻōpiopio a ʻelemakule paha.

Inā manaʻo kekahi kauka he Marfan syndrome kou, e hana lākou i nā mea e like me:

• E hana ʻia kahi hoʻokolokolo kino piha . E nānā ʻia kou kiʻekiʻe, kaumaha, ka lōʻihi o kou lima a me kou wāwae, ke ʻano o ka umauma, nā maka, a me ke kala o ka ʻili.
• E nīnau lākou e pili ana i ka mōʻaukala olakino o kou ʻohana . E nānā lākou e ʻike inā ua loaʻa i kekahi o kou ʻohana ka Marfan syndrome a i ʻole nā ​​​​hōʻailona like.
• Ua kauoha ʻia kekahi mau scan kūikawā ('nā hoʻāʻo kiʻi') e nānā i ka puʻuwai . ʻO nā mea nui:
• Echocardiogram (Echo): ʻAe kēia iā ʻoe e ʻike maopopo i ka nui, ke ʻano, a me ka hana o ka puʻuwai a me nā kīʻaha koko, e like me ka ultrasound scan o ka puʻuwai.
• Electrocardiogram (EKG): Ke ana kēia i ka hana uila o ka puʻuwai, ʻo ia hoʻi, ke ʻano o ka puʻuwai.
• Ka hoʻāʻo ʻana i ka genetic: Hiki ke hana i kēia hoʻāʻo e hōʻoia inā he hoʻololi genetic kou (i loko o ka gene FBN1) e hoʻoulu ai i ka maʻi Marfan.

He aha nā lāʻau lapaʻau no ka mālama ʻana i nā pilikia puʻuwai i hoʻokumu ʻia e ka Marfan syndrome?

ʻOiai ʻaʻole hiki ke ho'ōla piha ʻia ka maʻi Marfan, aia nā lāʻau lapaʻau e kāohi i nā hopena ma ka puʻuwai a pale i nā pilikia koʻikoʻi. Loaʻa kēia i ʻelua ʻano: nā lāʻau lapaʻau ʻoki ʻole a me nā lāʻau lapaʻau ʻoki.

Hiki i ke kauka ke ʻōlelo i ke ʻoki kino i nā hihia aʻe:

• Inā he 5 kenimika (ma kahi o 1.97 ʻīniha) a ʻoi aku paha ke anawaena o kou kīʻaha puʻuwai (aorta).
• Inā piʻi ka helu o ka puʻuwai ma 0.5 kenimika (ma kahi o 0.197 ʻīniha) a ʻoi aku paha i loko o hoʻokahi makahiki (ua kapa ʻia kēia ʻo 'ka hoʻonui wikiwiki').
• Inā ua hana ʻia kēia ʻano ʻoki kino i kou mau hoahānau koko (nā lālā o ka ʻohana pili kino) (ma muli paha o nā mana hoʻoilina, pono paha e hana ʻia ke ʻoki kino ʻoiai ma kahi anawaena liʻiliʻi).

ʻO ka poʻe me nā kino liʻiliʻi, ʻoi aku ka liʻiliʻi o nā kīʻaha koko, no laila pono paha lākou i ke ʻoki ʻana ʻoiai inā ʻoi aku ka liʻiliʻi o ke anawaena.

Nā lāʻau lapaʻau ʻoki ʻole

ʻO kēia nā hana mua i ka mālama ʻana i ka Marfan syndrome.

• Nā hoʻololi ʻana i ke ʻano o ka nohona: Pono ʻoe e pale i nā hana e hoʻokaumaha hou aku i ka puʻuwai.
• ʻAʻole maikaʻi ke hana i nā mea e like me ka hāpai ʻana i nā mea kaumaha a i ʻole ke kuʻi ʻana.
• ʻAʻole kūpono nā haʻuki hopena kiʻekiʻe e like me ka pôpeku, ka rugby, a me ka mokomoko.
• E kamaʻilio me kāu kauka e ʻike i nā hoʻoikaika kino palekana a kūpono hoʻi iā ʻoe.
• Nā lāʻau lapaʻau:
• Hoʻomaopopo nā kauka i nā lāʻau lapaʻau i kapa ʻia ʻo 'Angiotensin II receptor blockers (ARBs)' a i ʻole 'beta-blockers '. Kōkua kēia i ka hoʻolohi ʻana i ka wikiwiki o ka hoʻonui ʻana o nā kīʻaha puʻuwai a kaohi i ke koko.
• Nā hoʻokolohua kiʻi puʻuwai maʻamau :
• Pono e nānā pinepine ʻia ka nui o ka puʻuwai a me ke kūlana o nā valves e ke kauka. Hiki ke hana ʻia kēia me ka hoʻohana ʻana i nā scans e like me ka `transthoracic echocardiography (Echo)`, `computerized tomographic angiography (CTA)`, a i ʻole `magnetic resonance angiography (MRA)`.
• Hiki i kēia mau hoʻokolohua ke ʻike i nā loli i loko o ka puʻuwai ma mua o ka haki ʻana (dissection).

Nā lāʻau lapaʻau

I kekahi manawa, ʻaʻole hiki ke kāohi ʻia nā pilikia cardiovascular me nā lāʻau lapaʻau a me nā hoʻololi ʻana i ke ʻano o ka nohona wale nō. ʻO ia ka manawa e pono ai ke ʻoki kino.

ʻO ka pahuhopu nui o ke ʻoki puʻuwai no ka maʻi Marfan ka hoʻoponopono ʻana i kahi ʻāpana nāwaliwali o ka valve puʻuwai, wehe iā ia a hoʻokomo i kahi valve hana, a hoʻoponopono paha i kahi valve puʻuwai i hōʻino ʻia a hoʻololi paha me kahi mea hou.

ʻO ka pahuhopu nui o kēia mau ʻoki ʻana, ʻo ia ka pale ʻana i nā pilikia hoʻoweliweli i ke ola e like me ka aortic dissection.

ʻO ka hapa nui o ka manawa, ua hoʻolālā ʻia a hana ʻia kēia mau ʻoki kino i kahi lā i hoʻoholo mua ʻia (ʻoki kino koho). Eia nō naʻe, inā haki koke a haki paha kahi aʻa coronary, pono e hana ʻia ma ke ʻano he kaʻina hana ulia pōpilikia.

Aia kekahi mau ʻano nui o ke ʻoki puʻuwai i hana ʻia no ka maʻi Marfan:

• Hoʻololi aʻa aortic: ʻO kēia ke ʻoki maʻamau no ka mea e pehu pinepine a hoʻonui paha kēia ʻāpana o ka puʻuwai.
• Ka hoʻoponopono a i ʻole ke pani ʻana o ka valve aortic.
• Ka hoʻoponopono ʻana i kahi puʻupuʻu ma ke aʻa kokoke i ka puʻuwai ('Hoʻoponopono ʻana i ka aneurysm aortic piʻi').
• Ka hoʻoponopono a i ʻole ke pani ʻana o ka valve mitral.

E kūkākūkā ke kauka lapaʻau puʻuwai me ʻoe a hoʻoholo i ke ʻano o ke ʻoki kino kūpono loa iā ʻoe.

Hiki ke pale ʻia ka maʻi puʻuwai inā loaʻa iā ʻoe ka Marfan syndrome?

ʻO ka mea pōʻino, ʻaʻole hiki ke pale ʻia ka maʻi Marfan, no ka mea, he mea hoʻoilina ia. Pēlā nō, ʻaʻole hiki ke pale loa ʻia ka maʻi puʻuwai i hoʻokumu ʻia e ia.

Eia nō naʻe, aia kekahi mau mea hiki iā ʻoe ke hana e hōʻemi i kou pilikia o nā pilikia koʻikoʻi, e like me ka dissection aortic:

• Ke pale ʻana i nā hana e hoʻokau ai i ke kaumaha ma ka puʻuwai (e like me kā mākou i kūkākūkā ai ma mua, ke pale ʻana i ka hāpai kaumaha a me nā haʻuki kaumaha nui).
• E hana i nā hoʻokolohua kiʻi puʻuwai i ka manawa e like me ke kauoha a ke kauka.
• ʻO ka lawe ʻana i nā lāʻau lapaʻau a pau i kuhikuhi ʻia me ke pololei a me ka manawa kūpono.

ʻO kahi mea e hoʻomanaʻo ai, inā he wahine ʻoe me ka Marfan syndrome a ke noʻonoʻo nei e hāpai, pono ʻoe e kamaʻilio me kāu kauka e pili ana i ia mea. Ua hōʻike ʻia nā noiʻi a hiki i ka 40% o nā wahine me ka Marfan syndrome e loaʻa paha nā pilikia i ka wā hāpai. No laila, he mea nui loa e ʻimi i ka ʻōlelo aʻoaʻo lapaʻau ma mua o ka hāpai ʻana.

He aha ka nānā ʻana i ka wā e hiki mai ana no kekahi me ka maʻi puʻuwai ma muli o ka Marfan syndrome?

Me ka hoʻokele maikaʻi, ʻo ia hoʻi, ma hope o ka ʻōlelo aʻo lapaʻau a me ka loaʻa ʻana o ka lāʻau lapaʻau e pono ai, hiki i ke kanaka me Marfan syndrome ke ola i kahi ola maʻamau, malia paha he 70 a 80 paha mau makahiki.

Eia nō naʻe, ʻo nā pilikia o ka puʻuwai ke kumu nui o ka make ma waena o ka poʻe me ka Marfan syndrome, no laila he mea nui e noho mau me kāu cardiologist e nānā i kou kūlana.

Āhea wau e ʻike ai i ke kauka? He aha nā pilikia?

Inā ʻike ʻoe i kekahi o kēia mau hōʻailona, ​​​​hiki ke lilo i hōʻailona o kahi aneurysm aortic i haki. Pono ʻoe e ʻimi koke i ka lāʻau lapaʻau ulia pōpilikia:

• Ka nalowale koke ʻana o ka ʻike.
• Ka luaʻi a me ka luaʻi.
• ʻO ka ʻeha ma nā wahi a pau o ke kino.
• ʻO ka maʻi lolo (hiki ʻole ke hoʻoneʻe i kekahi mau ʻāpana o ke kino).
• Paʻakikī nui ka hanu ʻana.
• ʻEha koke, ʻeha hiki ʻole ke hoʻomanawanui - ma ka umauma, ke kua o luna, a i ʻole ka ʻōpū.
• Ka hou nui ʻana.
• Ke lilo nei ka ʻili i keʻokeʻo a anuanu paha.
• Ka naʻau palupalu loa.

Inā ʻike ʻoe i kahi hōʻailona e like me kēia, mai hoʻokaʻulua i hoʻokahi minute.

ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai (Leka Lawe-Home)

He maʻi hānau ʻia ka maʻi Marfan e hoʻopilikia ana i nā ʻiʻo pili o ke kino. Hiki iā ia ke hoʻopilikia nui i ka puʻuwai. Akā, mai hopohopo. Inā ʻike mua ʻia kēia maʻi a mālama pono ʻia, hiki iā ʻoe ke ola i kahi ola maʻamau.

ʻO ka mea nui loa:

• E ʻike i kāu kauka i nā manawa a pau.A laila hiki iā ia ke nānā i kou kūlana a hāʻawi i nā hoʻokolohua a me ka lāʻau lapaʻau e pono ai i ka manawa kūpono.
• E hana i nā hoʻololi e pono ai ke ʻano o ka nohona. E noho mamao aku mai nā mea e kaumaha ai kou puʻuwai.
• E lawe i ka lāʻau lapaʻau e like me ke kauoha.
• Inā ʻōlelo ke kauka i ke ʻoki kino, e kamaʻilio pono a koho i ka mea kūpono loa iā ʻoe.

ʻO ka nui o kou ʻike e pili ana i kou kūlana, ʻo ka maʻalahi hoʻi ia iā ʻoe ke mālama iā ia. No laila, ke manaʻolana nei mākou e pono kēia ʻike iā ʻoe. Inā he mau nīnau kāu, mai kānalua e nīnau i kāu kauka.

ʻO Marfan Syndrome, maʻi puʻuwai, ʻōnaehana cardiovascular, nā valves puʻuwai, nā ʻiʻo pili, nā maʻi genetic, ke ʻoki puʻuwai