He hoaaloha anei kou i lōʻihi loa, he mau lima a me nā wāwae lōʻihi, a he wīwī? Ke ʻike mākou i kekahi e like me ia, manaʻo mākou he mea maikaʻi loa ia no kahi haʻuki e like me ka basketball. Akā i kēia lā e kamaʻilio mākou e pili ana i kahi maʻi olakino e hele mai me kēia mau ʻano kino, kahi mea a mākou e kamaʻilio nui ʻole ai, akā hiki ke lilo i mea nui loa. ʻO ia ka Marfan Syndrome. Hiki iā ia ke hoʻopilikia nui i ka puʻuwai, no laila he mea nui loa ka makaʻala i kēia.
I ka ʻōlelo maʻalahi, he aha ka Marfan Syndrome?
ʻO ka maʻi Marfan kahi maʻi genetic e kū mai ana i loko o kā mākou mau genes. Hoʻokumu ʻia kēia e ka 'ʻiʻo hoʻopili' i loko o ko mākou kino i ulu pono ʻole. E noʻonoʻo i ko mākou kino e like me kahi hale. ʻO nā mea āpau i loko o kēia hale, e like me nā piliki, nā paia, a me ka hale, ua paʻa pū ʻia a mālama ʻia e ka sima. Pēlā nō, ʻo ka ʻiʻo hoʻopili ka 'pili' e kōkua ai e hoʻopaʻa i nā mea āpau i loko o ko mākou kino, e like me nā ʻōpū, nā iwi, a me nā kīʻaha koko, a mālama iā lākou i ikaika.
I loko o ke kanaka me ka Marfan syndrome, nāwaliwali ka 'gum', a i ʻole nā ʻiʻo hoʻohui, i loko o ke kino. Ua like ia me ke kūkulu ʻana i hale me ka sima makepono. Hiki i kēia ke hoʻopilikia i nā wahi he nui, me nā maka, nā māmā, a me ka ʻōnaehana iwi. Akā ʻo nā hopena koʻikoʻi loa hiki ke kau ma ko mākou puʻuwai a me nā kīʻaha koko nui.
No ke aha e hoʻopilikia nui ai kēia i ka puʻuwai? ʻElua mau pilikia nui!
Hiki i nā ʻiʻo pili nāwaliwali ke hōʻino i ʻelua mau ʻāpana nui o ka puʻuwai.
1. Aorta: ʻO kēia ke kīʻaha koko nui loa e lawe ana i ke koko mai ko mākou puʻuwai a i ke kino holoʻokoʻa. E like me ka ʻōnaehana paipu nui e lawe ana i ka wai mai ka pahu wai i loko o ko mākou hale i nā wahi āpau.
2. Nā kiwikā o ka puʻuwai: Ua like kēia me nā puka liʻiliʻi i loko o ka puʻuwai. Hōʻoia kēia mau kiwikā e holo ke koko i hoʻokahi ala wale nō.
I kēia manawa, e nānā kākou i nā hopena ma kēia mau ʻāpana ʻelua.
1. He aha ka mea e hana ʻia ana i ka aorta?
He palupalu ka ʻiʻo hoʻohui o ke kanaka me ka Marfan syndrome ma nā paia o ko lākou aorta. Hiki i kēia ke hana i ʻelua mau pilikia nui:
- Hoʻonui ʻia ke koko o ke aorta: ʻO ka maʻamau, he palupalu ke kīʻaha koko. Eia nō naʻe, ma muli o kēia nāwaliwali, hoʻomaka mālie ka aorta e ākea a hoʻonui, ʻaʻole hiki ke kū i ke kaomi i hana ʻia e kēlā me kēia puʻuwai.
- ʻAortic Aneurysm: I kekahi manawa, ʻaʻole e pau wale kēia hoʻonui ʻana. Hoʻomaka ka ʻāpana nāwaliwali loa o ke aʻa e puʻupuʻu e like me ka pōpō. ʻO kēia ka mea a mākou e kapa nei he "Aortic Aneurysm". ʻO kēia ka pilikia weliweli loa o ka Marfan syndrome. No ka mea, hiki i kēia puʻupuʻu e like me ka pōpō ke pahū (rupture) a haehae paha (dissection) i kēlā me kēia manawa. He pilikia hoʻoweliweli ia i ke ola.
2. He aha ka mea e hana ʻia i nā kiwikā o ka puʻuwai?
Hiki i nā ʻiʻo hoʻopili nāwaliwali ke hana i nā valves puʻuwai ʻaʻole e hana pono. Hiki iā lākou ke lilo i nāwaliwali a ʻaʻole e pani pono. ʻO kēia ke kumu:
- Ka Regurgitation Valve: Inā ʻaʻole pani pono kahi valve, hiki i ke koko ke hoʻi hou i loko o ka puʻuwai. ʻO kēia ke kumu o ka hana ikaika ʻana o ka puʻuwai e pamu i ke koko i ke kino. I ka hala ʻana o ka manawa, hiki i kēia ke alakaʻi i ka hāʻule ʻana o ka puʻuwai. ʻO nā valves maʻamau i hoʻopilikia ʻia ʻo ia ka Aortic Valve a me ka Mitral Valve.
- ʻO ka Mitral Valve Prolapse: Ma kēia kūlana, lilo ka mitral valve ma ka ʻaoʻao hema o ka puʻuwai i nāwaliwali a ʻaʻole pani pono, akā pelu i hope.
ʻO ka mea nui, ʻo ia ma kahi o ʻeiwa o loko o ʻumi mau kānaka me ka Marfan syndrome e loaʻa i nā pilikia puʻuwai a i ʻole aortic, no laila he mea nui e makaʻala i kēia.
He aha kēia mau hōʻailona o ka maʻi puʻuwai? Pehea mākou e ʻike ai iā lākou?
I ka hapanui o nā hihia, ʻaʻohe paha he mau hōʻailona i nā pae mua. Eia nō naʻe, hiki ke ʻike ʻia kēia mau hōʻailona i ka wā e ākea ai ka aorta a i ʻole e ʻoi aku ka nui o nā pilikia valve. Inā loaʻa iā ʻoe kekahi a ʻoi aku paha o kēia mau mea, he mea nui loa ia e ʻike i ke kauka.
| Hōʻailona | He wehewehe maʻalahi |
|---|---|
| ʻEha ma ka umauma a i ʻole ke kua i luna | He mea weliweli loa ia inā koke a koʻikoʻi ka ʻeha. |
| Ka pōkole o ka hanu a i ʻole ka paʻakikī o ka hanu ʻana | ʻO ka luhi e hiki mai ana me ka hele wāwae iki a i ʻole ka hana ʻana. |
| Nā Palpitations (manaʻo e like me ka wikiwiki o kou puʻuwai) | Ke manaʻo nei e loli ana ka helu o kou puʻuwai a i ʻole ke kuʻi nei kou umauma. |
| Manaʻo poniuniu a poʻo māmā paha | Manaʻo poniuniu i ke kū ʻana a i ʻole ke ala koke ʻana. |
| Ka luhi a me ka nāwaliwali maʻamau ʻole | Ke luhi mau nei me ke kumu ʻole. |
| Ka pehu ʻana o nā wāwae a me nā wāwae | He hōʻailona paha ia o ka emi ʻana o ka hana a ka puʻuwai. |
Pehea e ʻike pono ai ke kauka i kēia maʻi?
Hiki ke paʻakikī iki ka ʻike ʻana i ka maʻi Marfan no ka mea ʻaʻole like nā hōʻailona o kēlā me kēia kanaka, no laila e nānā ke kauka i kekahi mau kumu.
- Hoʻokolokolo kino: E nānā ke kauka i kou kiʻekiʻe, ka lōʻihi o ka lima a me ka wāwae, ka lōʻihi o ka manamana lima, ke ʻano o ka umauma, nā maka, a me ka iwi kuamoʻo.
- Mōʻaukala lapaʻau ʻohana: ʻOiai he maʻi hoʻoilina kēia, e nīnau ʻia ʻoe inā ua loaʻa i kekahi o kou ʻohana kēia mau hōʻailona a i ʻole ua make paha i ka hopu koke ʻana o ka puʻuwai.
- Nā hoʻokolohua kūikawā: Hana ʻia kekahi mau hoʻokolohua e hoʻoholo ai i ke kūlana kikoʻī o ka puʻuwai.
- Echocardiogram: Ua like kēia me ke scan o ka puʻuwai. Hiki iā ia ke ʻike maopopo i nā mea e like me ka nui o ka aorta a me ka hana o nā valves.
- Electrocardiogram (EKG): Kōkua kēia i ka nānā ʻana i ka hana uila o ka puʻuwai, ʻo ia hoʻi, e ʻike inā he mau pilikia me ke kuʻina o ka puʻuwai.
- Ka hoʻāʻo ʻana i ka Genetic: He hoʻāʻo koko e hoʻoholo ai inā loaʻa iā ʻoe ka hoʻololi genetic e hoʻoulu ai i ka Marfan syndrome.
- Ke pale ʻana i nā hana e hoʻokaumaha nui ai i ka puʻuwai. No ka laʻana, ʻaʻole kūpono nā haʻuki koʻikoʻi e like me ka hāpai kaumaha, ka rugby, a me ka pôpeku.
- Inā lilo ka aorta i mea ākea loa, hana ʻia ke ʻoki kino e pale aku ai i ka haehae ʻana (dissection). ʻOi aku ka maikaʻi o ka hoʻolālā ʻana no kēia ma mua o ka lilo ʻana i mea pilikia.
- Hiki i ke kauka ke kuhikuhi i nā lāʻau lapaʻau e like me "Beta-blockers" a i ʻole "ARBs". Hana kēia ma ka hoʻomalu ʻana i ke koko a me ka hoʻēmi ʻana i ke kaomi ma ka aorta. Hoʻolohi kēia i ka wikiwiki o ka hoʻonui ʻana o ke aʻa.
- Ke wehe nei i ka ʻāpana i hōʻino ʻia o ka aorta a me ka hoʻoponopono ʻana iā ia me kahi ʻōmole hana (graft).
- Ke hoʻoponopono ʻana a i ʻole ke pani ʻana i nā kiwikā puʻuwai hana hewa me kahi kiwikā hana ʻia.
- He mea nui e hana i kahi scan e like me ka echocardiogram ma ka liʻiliʻi hoʻokahi manawa i ka makahiki e nānā i ka nui o ka aorta. Hiki i kēia ke kōkua i ka ʻike ʻana i nā pilikia i ka wā mua inā ulu lākou.
- Ke ʻoi aku ke anawaena o ka aorta ma mua o 5 kenimika.
- Inā e ākea koke loa ke aʻa i loko o hoʻokahi makahiki.
- Inā loaʻa i kekahi o kou ʻohana ka mōʻaukala o ka dissection aortic.
Inā loaʻa i kekahi o kou ʻohana kēia maʻi a i ʻole he mau hōʻailona like, ʻo ka haʻi ʻana i kāu kauka e pili ana i ia mea he mea kōkua nui ia i ka ʻike ʻana i ka maʻi.
ʻAe, he aha nā lāʻau lapaʻau no kēia?
ʻAʻole hiki ke ho'ōla loa ʻia ka maʻi Marfan. No ka mea, he mea ia i loko o ko kākou mau genes. Eia nō naʻe, aia nā lāʻau lapaʻau maikaʻi loa e kāohi i ka pōʻino i hana ʻia i ka puʻuwai, pale i nā kūlana koʻikoʻi, a ʻae i nā kānaka e ola i kahi ola maʻamau. Hiki ke hoʻokaʻawale ʻia ka lāʻau lapaʻau i ʻelua mau ʻāpana nui.
| Nā Lapaʻau ʻAʻole ʻoki kino | Lapaʻau ʻoki kino |
|---|---|
Nā loli o ke ʻano o ka nohona: | No ke aha he mea pono ke ʻoki kino?: |
Nā lāʻau lapaʻau: | Nā ʻano hana ʻoki kino: |
Nā hoʻokolohua lapaʻau maʻamau: | Ua ʻōlelo ʻia ke ʻoki kino no: |
Nā kūlana pilikia e pono ai ke komo koke ʻana i ka haukapila! (Nā Hōʻailona Hoʻolaha)
ʻO kēia mau hōʻailona paha he mau hōʻailona o ka haki ʻana o ka aortic a i ʻole ka dissection. Inā loaʻa kekahi o kēia mau mea, e hele koke i ka Emergency Department (ETU) kokoke loa.
| Inā loaʻa iā ʻoe kēia mau hōʻailona, e hele koke i ka ETU! | |
|---|---|
| - ʻEha koke a hiki ʻole ke hoʻomanawanui ma ka umauma, ke kua, a i ʻole ka ʻōpū . | - Paʻakikī nui ka hanu ʻana. |
| - Ka nalowale o ka ʻike. | - Ka mae a ʻūlū paha ma kekahi ʻāpana o ke kino. |
| - Ka hou nui ʻana, ka ʻili anuanu. | - Ka luaʻi a me ka luaʻi. |
Hiki paha ke ola maikaʻi me ka Marfan syndrome?
ʻAe loa! I nā makahiki i hala aku nei, ua pōkole ke ola o ka poʻe me kēia maʻi. Akā i kēia lā, mahalo i nā lāʻau lapaʻau holomua, nā nānā mau ʻana, a me nā ʻoki kino, hiki i kahi kanaka me ka maʻi Marfan ke ola i kahi ola olakino, a hiki i ko lākou mau makahiki 70 a 80 paha, e like me ke kanaka maʻamau.
ʻO ka mea nui loa, ʻo ia ka mālama mau ʻana i ka launa pū ʻana me kāu kauka, e hahai pololei i kāna mau ʻōlelo aʻoaʻo, e hoʻāʻo ʻia i ka manawa kūpono, a e hoʻohana pono i kāu mau lāʻau lapaʻau.
ʻOi aku hoʻi, inā e hoʻolālā ana kahi wahine me ka maʻi Marfan e hāpai, pono ʻo ia e kūkākūkā me kahi kauka puʻuwai a me kahi kauka wahine ma mua o ka hāpai ʻana. No ka mea, kiʻekiʻe ke kaumaha ma ka puʻuwai i ka wā hāpai, pono e mālama pono ʻia.
Ma ka hoʻomaopopo ʻana i kēia kūlana, ka lawe ʻana i nā hana e pono ai, a me ka noho ʻana me ka noʻonoʻo pono, hiki iā ʻoe ke pale i kou puʻuwai a ola i ke ola olakino a lōʻihi.
Leka Lawe-Home
- ʻO ka maʻi Marfan kahi maʻi hoʻoilina e hoʻonāwaliwali ai i nā ʻiʻo pili o ke kino.
- Hiki i kēia ke loaʻa nā hopena koʻikoʻi ma ka puʻuwai a me ke kīʻaha koko nui, ʻo ia hoʻi ka aorta.
- ʻO ka hoʻolaha ʻana o ka aortic a me ka aneurysm nā pilikia weliweli loa.
- He mea nui e ʻike i ke kauka i ka manawa i hoʻonohonoho ʻia a hana i nā scan e like me ka echocardiogram.
- E hōʻalo i nā hana e hoʻokaumaha ai i ka puʻuwai, e like me ka hāpai kaumaha.
- E makaʻala i nā hōʻailona hōʻike, e like me ka ʻeha nui a koke o ka umauma.
- Me ka mālama pono a me ka lāʻau lapaʻau, hiki iā ʻoe ke ola i kahi ola maʻamau a lōʻihi loa.
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