Aia anei kou moʻolelo ʻohana i ka cholesterol kiʻekiʻe? E kamaʻilio kākou e pili ana i kēia (Heterozygous Familial Hypercholesterolemia - HeFH)

Ua lohe paha ʻoe e pono ʻoe e mālama i kāu cholesterol e pale iā ʻoe iho mai nā maʻi puʻuwai. Akā i kekahi manawa, ʻaʻohe mea nui i kou kaohi ʻana i kāu ʻai a me ka hoʻoikaika kino, ʻaʻole i emi iho kou pae cholesterol. Malia paha ua loaʻa i kekahi o kou ʻohana ka hōʻeha puʻuwai i ka wā ʻōpiopio. Hiki paha kēia ma muli o kahi maʻi genetic e holo ana i loko o nā ʻohana. ʻO ia ke ʻano a mākou e kamaʻilio nei i kēia lā, ʻo Heterozygous Familial Hypercholesterolemia, a i ʻole HeFH no ka pōkole. Inā haʻi aku ke kauka iā ʻoe ua loaʻa iā ʻoe kēia, he mea nui e loaʻa koke ka lāʻau lapaʻau.

He aha maoli ka HeFH?

ʻAʻole ʻo HeFH kahi maʻi i loaʻa iā kākou mai waho mai , e like me ke anu. He kūlana ia i hoʻokumu ʻia e kahi hoʻololi genetic i hoʻoilina ʻia e kākou mai ko kākou makuahine a makuakāne paha. I ka ʻōlelo maʻalahi, hoʻemi kēia hoʻololi genetic i ka hiki o ke kino ke wehe i ka cholesterol ʻino mai ke koko.

E noʻonoʻo e pili ana i ia mea, hele ka cholesterol ma o nā kīʻaha koko i loko o ko kākou kino. ʻElua ʻano nui o kēia.

I ke ʻano o HeFH, hoʻoilina ʻoe i kahi gene mai kekahi o kou mau mākua e hoʻopilikia ana i ke kaʻina hana o ka wehe ʻana i ka cholesterol maikaʻi ʻole (LDL) . Inā loaʻa iā ʻoe kēia gene, he 50% ka manawa e loaʻa ai i kāu keiki.

Ua lohe paha ʻoe i kekahi maʻi ʻē aʻe me ka inoa like, ʻo Homozygous Familial Hypercholesterolemia (HoFH). Hoʻokumu ʻia kēia e ka gene kīnā i hoʻoilina ʻia mai nā mākua ʻelua. ʻOi aku ka ʻino o ke ʻano o HoFH ma mua o HeFH, akā he mea kakaikahi loa ia.

He aha nā hōʻailona o kēia?

Inā ʻaʻole mālama ʻia, hiki i ka HeFH ke hoʻonui i kou pae kolesterol LDL a me ka cholesterol holoʻokoʻa. Hiki i kēia kolesterol LDL keu ke kūkulu ʻia ma nā paia o ko kākou mau kīʻaha koko, e like me ka lepo e pani ana i ka paipu, e hoʻohaiki ana i ke ala no ke kahe ʻana o ke koko. Kāhea mākou i kēia "atherosclerosis" a i ʻole "paʻakikī o nā aʻa."

Ke hana ʻia kēia, pono ka puʻuwai e hana ikaika ma mua o ka maʻamau e pamu i ke koko a puni ke kino. I ka hala ʻana o ka manawa, hiki i ka puʻuwai ke hōʻino ʻia a loaʻa i nā maʻi puʻuwai. ʻO kekahi o nā hōʻailona nui o kēia ʻano, ʻo ia ka ʻeha o ka umauma, i ʻike ʻia ʻo angina. Inā ʻaʻole mālama ʻia, hiki i kēia ke alakaʻi i ka hōʻeha puʻuwai a i ʻole ka hahau.

Nā hōʻailona e pono ai ka nānā koke ʻana

Inā loaʻa iā ʻoe nā hōʻailona ma lalo nei, e hele koke i ka ʻOihana Pilikia (ETU) o ka haukapila.

• Nā hōʻailona o ka hōʻeha puʻuwai:
• Paʻa ka umauma, ka ʻūlū, a i ʻole ka ʻeha
• ʻEha kua luna a ʻāʻī paha
• ʻO ka ʻeha e pālahalaha ana i lalo o ka lima
• Ka luaʻi a i ʻole ka luaʻi
• Paʻakikī ka hanu ʻana
• Nā hōʻailona o ka hahau ʻana:
• Paʻakikī i ka ʻōlelo ʻana
• ʻO ka numbness i ka lima a i ʻole ka wāwae
• Ke kūlou ʻana o kekahi ʻaoʻao o ka maka
• Ka nalowale o ke kaulike o ke kino

Nā hiʻohiʻona o ke kino ma luna

Hiki i ke cholesterol kiʻekiʻe i kekahi manawa ke hana i nā hōʻailona ma ka ʻili o ke kino.

Pehea ʻoe e ʻike ai inā loaʻa iā ʻoe kēia maʻi?

Aia kekahi mau hoʻāʻo maʻalahi e hiki ke kōkua iā ʻoe a i kāu keiki e ʻike inā loaʻa iā lākou ka HeFH. ʻO ka mea nui loa, ʻo ia ka loaʻa ʻana o ka maʻi a me ka mālama ʻana i ka wā hikiwawe loa. ʻOiai ʻaʻole pilikia nā keiki ʻōpio i ka maʻi puʻuwai, ʻo kēia mau pae cholesterol kiʻekiʻe e hoʻonui iā lākou i ka pilikia o ka loaʻa ʻana o ka maʻi puʻuwai i ka wā e hiki mai ana.

Inā loaʻa iā ʻoe a i ʻole kekahi o kou ʻohana kekahi o kēia mau mea, e ʻike pono i ke kauka a kamaʻilio e pili ana.

• ʻO ke kiʻekiʻe o ka cholesterol LDL i emi ʻole me ka kaohi ʻana i ka ʻai (ma luna o 190 mg/dL i kahi pākeke, ma luna o 160 mg/dL i kahi keiki ma lalo o 16 mau makahiki).
• He kiʻekiʻe ka cholesterol i loko o ka ʻohana.
• Loaʻa i ka hāʻule puʻuwai a i ʻole ka maʻi puʻuwai ma mua o 60 makahiki i nā hoahānau kāne a i ʻole ma mua o 70 makahiki i nā hoahānau wahine.
• ʻO ke alo o nā puʻupuʻu ʻili e like me nā xanthomas i ʻōlelo ʻia ma luna.
• ʻEha ka umauma.

E hana kāu kauka i kahi hoʻokolohua kino e nānā i kēia mau hōʻailona. Eia kekahi, e ana kahi hoʻāʻo koko i kāu mau pae cholesterol. I ka HeFH, ʻoi aku ka nui o nā pae cholesterol LDL ma mua o 200 mg/dL a ʻoi aku ka nui o nā pae cholesterol āpau ma mua o 300 mg/dL.

I kekahi mau hihia, hiki ke ʻōlelo ʻia kahi hoʻāʻo koʻikoʻi e nānā i ka hana o ka puʻuwai a i ʻole kahi hoʻāʻo genetic kūikawā e nānā i nā loli genetic.

Pehea e mālama ʻia ai?

ʻO ka pahuhopu nui o ka mālama ʻana iā HeFH, ʻo ia ka hoʻohaʻahaʻa ʻana i kāu mau pae kolesterol "maikaʻi ʻole" LDL a pale iā ʻoe mai nā maʻi puʻuwai. Pono kēia i ka hui pū ʻana o nā loli o ke ʻano o ka nohona a me nā lāʻau lapaʻau.

Nā loli o ke ʻano o ka nohona

• ʻAi olakino: E hōʻalo i nā meaʻai kiʻekiʻe i nā momona saturated a me nā trans (pipi, puaʻa, ʻaila niu, yolks hua manu, waiū momona). Akā, e hoʻokomo i nā mea kanu, nā huaʻai, nā legumes, nā nati, ka iʻa, a me ka moa wīwī i kāu papaʻai.
• Hoʻoikaika Kino: E hana i kahi mea e hoʻoulu ai i kou puʻuwai, e like me ka hele wāwae, ʻauʻau, a paikikala paha, ma ka liʻiliʻi he 4 mau lā o ka pule no ka liʻiliʻi he 30 mau minuke.
• Kaohi kaumaha: Inā ʻoe he momona loa, e hoʻēmi i ke kaumaha ma o ka ʻai a me ka hoʻoikaika kino.
• E pale i ka puhipaka: He mea pilikia ka puhipaka no ka maʻi puʻuwai, no laila inā loaʻa iā ʻoe kēia ʻano maʻa, e hoʻōki koke.

Lāʻau lapaʻau

E kuhikuhi pinepine kāu kauka i kahi papa o nā lāʻau i kapa ʻia ʻo statins . Hana kēia mau mea ma ke kāohi ʻana i ke kino mai ka hana ʻana i ka cholesterol. No ka mea, kiʻekiʻe loa nā pae cholesterol ma HeFH, pono paha ʻoe e lawe i kēia mau lāʻau i nā nui kiʻekiʻe.

Inā ʻaʻole lawa nā statins wale nō e kāohi i nā pae, e kuhikuhi kāu kauka i nā lāʻau lapaʻau ʻē aʻe.

• Nā mea pale PCSK9: He mau lāʻau lapaʻau kēia i hāʻawi ʻia ma ke ʻano he inikini. Kōkua lākou i ke akepaʻa e wehe i ka cholesterol LDL.
• ʻEzetimibe: Hana kēia lāʻau lapaʻau ma ka hōʻemi ʻana i ka omo ʻana o ke kino i ka cholesterol mai nā meaʻai a mākou e ʻai ai.

I kekahi manawa pono paha e hoʻohana pū i ʻelua a ʻekolu paha o kēia mau lāʻau lapaʻau.

Nā lāʻau lapaʻau ʻē aʻe

Ke kiʻekiʻe loa nā pae cholesterol no ka kaohi ʻana me nā lāʻau lapaʻau a me nā hoʻololi ʻana i ke ʻano o ka nohona, hiki ke hāʻawi ʻia iā ʻoe kahi lāʻau i kapa ʻia ʻo LDL apheresis . Ua like kēia me ka dialysis, kahi e hoʻouna ʻia ai kou koko ma o kahi mīkini e kānana ana i ka cholesterol LDL maikaʻi ʻole a laila hoʻihoʻi hou iā ia i loko o kou kino.