Kamaʻilio mau kākou a pau e pili ana i ka cholesterol, a hopohopo kākou no ia mea, ʻeā? Akā, e noʻonoʻo, ua lohe anei ʻoe i kahi maʻi hoʻoilina kahi i kiʻekiʻe loa ai nā pae cholesterol mai ka wā kamaliʻi, malia paha i ka wā kamaliʻi? I kēia lā, e kamaʻilio kākou e pili ana i kekahi maʻi kakaʻikahi akā koʻikoʻi loa. Ua kapa ʻia kēia ʻo Homozygous Familial Hypercholesterolemia (HoFH). ʻOiai he paʻakikī iki ka inoa, e hoʻomaopopo maʻalahi kākou.
He aha ka Homozygous Familial Hypercholesterolemia?
I ka ʻōlelo maʻalahi, he maʻi hoʻoilina kēia e paʻakikī ai i ko mākou kino ke wehe i ka cholesterol "maikaʻi ʻole", ʻo ia hoʻi, ka LDL (Low-Density Lipoprotein) cholesterol, mai ko mākou koko. Ua ʻike mākou a pau he mea waxy ka cholesterol e pono ai ko mākou mau cell. ʻO ka hana a ka LDL cholesterol, ʻo ia ke lawe i kēia cholesterol ma ke kino holoʻokoʻa ma o ke koko.
Akā i ka wā e kiʻekiʻe loa ai nā pae LDL, hoʻomaka kēia cholesterol keu e hōʻiliʻili i loko o nā kīʻaha koko e hoʻolako ana i ke koko i ko mākou puʻuwai, ʻo ia hoʻi nā aʻa. I ka hala ʻana o ka manawa, hiki i kēia mau waihona ke ālai i nā aʻa, e ʻoki ana i ke koko a me ka oxygen i ka puʻuwai, a e hoʻonui ana i ka pilikia o ka hōʻeha puʻuwai .
He maʻi hānau ʻia kēia. ʻO ke ʻano kēia, hiki iā ʻoe ke loaʻa i ka cholesterol kiʻekiʻe mai kou wā kamaliʻi. He maʻi koʻikoʻi kēia ʻaʻole hiki ke ho'ōla ʻia a pono e mālama ʻia i ke ola holoʻokoʻa.
Inā ʻaʻole mālama ʻia, hiki i nā kāne me kēia maʻi ke loaʻa i ka maʻi puʻuwai i ko lākou mau makahiki 40 a me nā wahine i ko lākou mau makahiki 50. No laila, he mea nui loa ia e makaʻala i kēia a e ʻimi i ka lāʻau kūpono.
He aha ke kumu o kēia?
Kāhea mākou i kēia he maʻi hoʻoilina. ʻO ka ʻōlelo pololei, loaʻa iā ʻoe kēia maʻi inā loaʻa iā ʻoe ʻelua kope o kahi gene i hana pono ʻole, hoʻokahi mai kou makuahine a hoʻokahi mai kou makuakāne .
ʻO ka maʻamau, hoʻopau ko kākou ake i ka cholesterol LDL keu mai ke koko. Loaʻa i ke ake nā 'mea loaʻa LDL' kūikawā no kēia. Ua like kēia me nā magnet e hopu i ka cholesterol a wehe iā ia mai ke koko. Akā i loko o kekahi me kēia maʻi, ma muli o ka gene kīnā i hoʻoilina ʻia, ʻaʻole hana pono kēia mau mea loaʻa. ʻO ka hopena, piʻi nui ka pae cholesterol me ke kāohi ʻole ʻia.
Ua lohe paha ʻoe i ka 'Heterozygous' Familial Hypercholesterolemia. ʻO kēia ka wā e hoʻoilina ʻia ai ka gene kīnā mai hoʻokahi makua wale nō. ʻAʻole koʻikoʻi kēia kūlana e like me 'Homozygous'.
He aha nā hōʻailona o kēia maʻi?
Aia kekahi mau hōʻailona nui e hiki ke hoʻohana ʻia e ʻike ai i kēia maʻi. E nānā kākou iā lākou ma kahi papa.
| ʻAno | Wehewehena |
|---|---|
| Kiʻekiʻe loa ka pae kolesterol | Hiki i nā pae cholesterol holoʻokoʻa ke 600 mg/dL a ʻoi aku paha (ʻoi aku ka liʻiliʻi o ka pae olakino ma mua o 200). |
| Nā waihona ʻili (Xanthomas) | Nā puʻupuʻu melemele a palupalu ma lalo o ka ʻili ma nā kuʻekuʻe lima, nā kuli, a me nā puʻupuʻu. He mau waihona kolesterol kēia. |
| Nā waiho ʻana ma nā lihilihi (Xanthelasmas) | Nā waihona momona melemele ma nā lihilihi. |
| Nā loli o ka maka (Arcus cornealis) | He apo hina a keʻokeʻo paha a puni ka maka ʻeleʻele. |
| Nā hōʻailona o ka puʻuwai | Hiki ke ʻike ʻia nā hōʻailona e like me ka ʻeha o ka umauma (angina), ka pōkole o ka hanu, a me ka wikiwiki o ka puʻuwai i ka wā ʻōpiopio. |
Pehea e ʻike ai i ka maʻi?
Inā loaʻa iā ʻoe kēia mau hōʻailona, he mea nui e ʻike i kāu kauka. E hana mua ke kauka i kahi hoʻokolokolo kino a nīnau iā ʻoe i kekahi mau nīnau.
- Ua ʻike anei ʻoe i kekahi mea e like me nā kiko melemele ma kou ʻili?
- Ua ʻeha kou umauma?
- Ke manaʻo nei ʻoe ua nele ʻoe i ka hanu?
- Loaʻa i kou mau mākua ke cholesterol kiʻekiʻe?
A laila, e hana kāu kauka i kahi hoʻāʻo koko e nānā i kāu mau pae cholesterol. Hiki iā lākou ke hana i kahi hoʻāʻo genetic kūikawā e ʻike inā loaʻa iā ʻoe ka gene hewa e hoʻoulu ai i ka maʻi. Hiki i kāu kauka ke hoʻāʻo i kou ʻohana pili.
Nā ʻano lapaʻau a me ka hoʻokele
ʻO ka pahuhopu nui o ka mālama ʻana, ʻo ia ka hoʻohaʻahaʻa i kāu mau pae cholesterol LDL a me ka hoʻemi ʻana i kou pilikia o ka maʻi puʻuwai.E kuhikuhi pinepine ʻia ʻoe i ke kauka loea i nā maʻi cholesterol e like me kēia (Lipidologist).
Nā loli i ke ʻano o ka nohona a me ka ʻai
ʻO ka hana mua, ʻo ia ka lawe ʻana i kahi papaʻai olakino no ka puʻuwai e haʻahaʻa ana i ka momona momona, ka cholesterol, a me ke kō. He mea nui hoʻi e haʻalele i ka puhipaka, e kaupalena i ka waiʻona, a e hoʻoikaika kino i kēlā me kēia lā.
Nā lāʻau lapaʻau
Ma muli o ke kiʻekiʻe loa o kou pae cholesterol, hiki ke hoʻomaka ʻia ʻoe ma nā dosis kiʻekiʻe o nā statins , kahi e hana ai ma ka hoʻōki ʻana i ke akepaʻa mai ka hana ʻana i ka cholesterol.
Me kēlā, hiki ke hoʻohui ʻia nā lāʻau lapaʻau ʻē aʻe e hōʻemi ana i ka nui o ka cholesterol i omo ʻia mai ka meaʻai (e like me Ezetimibe , nā mea hoʻopaʻa PCSK9 , Lomitapide ).
Nā lāʻau lapaʻau kūikawā
Inā ʻaʻole hiki ke hoʻemi ʻia ka cholesterol me nā lāʻau lapaʻau wale nō, pono e noʻonoʻo ʻia nā ʻano lapaʻau ʻē aʻe.
- Apheresis: He hana like kēia me ka dialysis no nā maʻi puʻupaʻa. Hoʻopili ʻia ʻoe i kahi mīkini ma ka haukapila, lawe ʻia kou koko mai kou kino aku, kānana ʻia ka cholesterol LDL, a hoʻihoʻi ʻia ke koko i hoʻomaʻemaʻe ʻia i kou kino. He hana kēia e lōʻihi ai kekahi mau hola a pono e hana mau ʻia.
- Hoʻoili ʻAke: Inā ʻaʻole holomua kekahi o nā lāʻau lapaʻau i luna, ʻo ka hoʻoili ʻake paha ke ala hope loa. Loaʻa i ke ake olakino hou nā mea loaʻa LDL, hiki ke kōkua i ka kaohi ʻana i ka cholesterol. He ʻoki kino koʻikoʻi loa kēia, a hiki ke hala ka hoʻōla ʻana i 6 mau mahina a i hoʻokahi makahiki.
I ka wā e hana ʻia ai ia ʻano lapaʻau koʻikoʻi, he mea nui loa ia e ʻimi i ke kākoʻo naʻau mai ka ʻohana a me nā hoaaloha. E kamaʻilio ākea e pili ana i kēia me kāu kauka.
Leka Lawe-Home
- He maʻi koʻikoʻi ka Homozygous Familial Hypercholesterolemia, akā he mea kakaikahi, kahi maʻi genetic e hoʻoulu ai i nā pae cholesterol kiʻekiʻe loa.
- ʻO kēia no ka mea ua hoʻoilina lākou i ʻelua mau genes kīnā mai ko lākou makuahine a me ko lākou makuakāne.
- Ma ka ʻike ʻana a me ka hoʻomaka ʻana i ka lāʻau lapaʻau i ka wā ʻōpiopio, hiki ke hoʻemi ʻia ka pilikia o ka maʻi puʻuwai i ka wā ʻōpiopio.
- ʻO ke ola o ka lāʻau lapaʻau he no ke ola a hiki ke komo pū me ka lāʻau lapaʻau, ka ʻai, ka hoʻoikaika kino, a me ka mālama kūikawā paha (apheresis).
- Inā ua loaʻa i kekahi o kou ʻohana ka maʻi puʻuwai a i ʻole ke cholesterol kiʻekiʻe i kona wā ʻōpiopio, he mea naʻauao iā ʻoe ke nānā ʻia kāu cholesterol e like me ka ʻōlelo a kāu kauka.
- ʻAʻole ʻoe hoʻokahi i kēia huakaʻi. E hana pū me kāu kauka, ʻohana, a me nā hui kākoʻo e hoʻokele pono i kēia kūlana.
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