Ke kahe pinepine nei ke koko ihu iā ʻoe? A i ʻole ua ʻike ʻoe i nā kiko ʻulaʻula liʻiliʻi ma kekahi mau ʻāpana o kou kino, no ka laʻana, ma kou maka, lehelehe, a i ʻole nā manamana lima? I kekahi manawa ʻaʻole mākou e manaʻo he mau mea maʻamau kēia, akā ma hope o lākou aia paha kahi maʻi olakino e pono ai ka nānā ʻana. ʻO ia ka HHT, akā ʻaʻole nui ka poʻe i ʻike e pili ana i ia mea. I kēia lā ke kamaʻilio nei mākou e pili ana i kēia HHT, a i ʻole ma nā ʻōlelo lapaʻau, `(Hereditary Hemorrhagic Telangiectasia)`, he maʻi hoʻoilina. Ua kapa pū kekahi iā ia ʻo `(Osler-Weber-Rendu syndrome)`.
He aha maoli ka HHT?
I ka ʻōlelo maʻalahi, he maʻi hoʻoilina ʻo HHT e hoʻopilikia ana i nā kīʻaha koko i loko o ko kākou kino. ʻIke ʻoe, loaʻa iā kākou kēia mau ʻōmole liʻiliʻi e lawe ana i ke koko ma o ko kākou kino, a mākou e kapa nei he mau kīʻaha koko. Ma kēia kūlana, ʻo ka mea e hana ʻia nei, ʻaʻole e ulu pono kēia mau kīʻaha koko, ʻoiai nā mea liʻiliʻi loa i kapa ʻia he capillaries. Kāhea mākou i kēia mau capillaries i hana ʻia ma ke ʻano ʻē he "telangiectasias".
E noʻonoʻo e pili ana i ia mea, loaʻa iā kākou nā aʻa nui e lawe ana i ke koko mai ko kākou puʻuwai a i ke koena o ko kākou kino, a me nā aʻa koko e lawe ana i kēlā koko i ka puʻuwai. Hana ʻia ka pilina ma waena o kēia mau mea ʻelua e nā mea i kapa ʻia he capillaries. I loko o HHT, i kekahi manawa hiki i kēia mau aʻa koko a me nā aʻa koko ke loaʻa nā pilina kūpono ʻole, me ka ʻole o ka hele ʻana ma waena o nā capillaries. Kāhea mākou i kēia mau malformations arteriovenous, a i ʻole AVM no ka pōkole.
He nāwaliwali loa a palupalu kēia mau kīʻaha koko ʻē. Hiki iā lākou ke poha a haki paha, e hoʻoulu ai i ke kahe koko (hemorrhaging). Pili nā hōʻailona a me nā pilikia i kahi e puka ai ke kahe koko.
ʻO wai ka mea hiki ke hoʻomohala i ka HHT?
Hiki i kēia maʻi i kapa ʻia ʻo HHT ke hoʻopilikia i nā wahine, nā kāne, a me nā keiki ʻōpio . ʻAʻole pili ka lāhui, ka hoʻomana, a me ke kala i kēia. ʻO ke kumu nui no ka mea ua hoʻoili ʻia mai kēlā hanauna a i kēia hanauna ma o nā genes, inā loaʻa i kekahi o ka ʻohana, loaʻa i nā poʻe ʻē aʻe ka manawa e loaʻa ai.
Ua manaʻo ʻia kēia he maʻi kakaikahi . Eia naʻe, ʻaʻole i ʻike pinepine ʻia. ʻO ke ʻano kēia, ʻoiai ua manaʻo ʻia he hoʻokahi i loko o 5,000 mau kānaka ma ka honua holoʻokoʻa e loaʻa ana i kēia maʻi, ʻaʻole ʻike ka hapa nui o lākou ua loaʻa iā lākou.
He aha ke kumu o ka HHT?
E like me kaʻu i ʻōlelo ai ma mua, he maʻi genetic piha ʻo HHT. ʻO ia hoʻi, ua hoʻoilina ʻia mai nā mākua a i nā keiki. Ua manaʻo ʻia he "maʻi dominant". I ka ʻōlelo maʻalahi, hiki i kahi keiki ke loaʻa i ka maʻi ʻoiai inā hoʻokahi makua wale nō, ʻo ka makuahine a i ʻole ka makuakāne, ka mea i hoʻoilina i ka gene ʻano ʻē .
Ua ʻike nā kānaka ʻepekema i nā haneli o nā mutations like ʻole i loko o ʻeono mau genes e pili ana iā HHT. Eia nō naʻe, i kēia manawa, ʻo nā mutations maʻamau i loko o ʻelua mau genes i kapa ʻia ʻo ENG lāua ʻo ACVRL1. Ke noiʻi nei nā kānaka ʻepekema i kēia.
He aha nā hōʻailona o ka HHT?
Hiki ke ʻokoʻa loa nā hōʻailona o HHT mai kēlā kanaka a kēia kanaka. Aia ia i kahi o ke kino e loaʻa ai nā kīʻaha koko ʻē aʻe aʻu i ʻōlelo ai. ʻAʻole paha i loaʻa i kekahi poʻe nā hōʻailona nui. Akā, hiki i kekahi poʻe ke loaʻa nā hōʻailona koʻikoʻi loa.
Eia nō naʻe, ʻo ka hōʻailona maʻamau i waena o ka poʻe me HHT, ʻo ia ke kahe pinepine ʻana o ka ihu (epistaxis) . Loaʻa paha i kekahi poʻe ke kahe o ka ihu i nā manawa he nui i ka lā. Ua loaʻa paha kēia pilikia mai ka wā kamaliʻi mai.
Eia kekahi, ʻike paha kekahi poʻe me HHT i nā kiko ʻulaʻula liʻiliʻi (telangiectasias) ma ko lākou kino. Hiki ke ʻike ʻia kēia mau mea he ʻano ʻē iki iā ʻoe. ʻIke pinepine ʻia kēia mau mea ma:
- Ma ka maka
- Ma nā manamana lima a i ʻole nā manamana lima
- I loko o nā lima
- I loko o ka waha (ma luna o ka membrane mucous)
- Ma nā lehelehe
- A puni ka ihu
Ma waho aʻe o kēia mau mea, hiki i kekahi poʻe me HHT ke ʻike pū kekahi:
- Anemia : ʻO ke ʻano kēia o ka nele o nā hunaola koko ʻulaʻula i loko o ke kino. Hiki ke hana kēia ma muli o ke kahe pinepine ʻana o ke koko.
- Ke kahe ʻana o ke koko mai ka ʻōpū a i ʻole nā naʻau : ʻAʻole paha e ʻike ʻia kēia, akā aia paha ke koko i loko o ka ʻōpala a i ʻole he ʻeleʻele paha ka ʻōpala.
Nā maʻi Arteriovenous (AVM) a me ko lākou hopena
Hiki i nā kānaka me HHT ke loaʻa i nā aneurysms (AVMs) i kekahi manawa i loko o nā kīʻaha koko nui. Hiki i kēia mau AVM ke ulu nui i loko o nā ʻōpū e like me nā māmā, ka lolo, ke kaulahao kuamoʻo, a me ke akepaʻa. A laila hiki iā lākou ke hana i nā hōʻailona like ʻole e pili ana i kēlā me kēia ʻōpū.
- Inā he mau AVM kou i loko o kou mau māmā:
- ʻO ke kala ʻulaʻula o ka ʻili (ʻoiai nā lehelehe, nā kui)
- Ke ʻū ʻana i ke koko (hemoptysis)
- Manaʻo koke i ka luhi
- Ka paʻakikī o ka hanu ʻana (dyspnea)
- Inā loaʻa iā ʻoe nā `(AVM)` i loko o ka lolo: ʻO kēia nā mea i ʻoi aku ka weliweli. No ka mea, inā pahū kekahi o kēia mau `(AVM)` i loko o ka lolo, he mea koʻikoʻi loa ia.
- Pōʻino
- ʻIke pālua
- Nā hopu ʻana
- Hiki ke loaʻa nā kūlana e like me ka hahau ʻana.
- Inā loaʻa iā ʻoe nā AVM akepaʻa:
- Hiki i ka puʻuwai ke komo i loko o ke kūlana o ka "Heart failure" no ka mea, i ka wā e holo wikiwiki ai ke koko ma o ka "(AVM)" i loko o ke akepaʻa, pono ka puʻuwai e hana ikaika e hoʻolako i ke koko i ke kino holoʻokoʻa.
- Inā loaʻa iā ʻoe nā AVM spinal:
- ʻEha hope
- Hiki ke ʻike ʻia ka maneʻo a i ʻole ka nalowale o ka ʻike ma nā lima a i ʻole nā wāwae.
He mea nui: Inā poha kēia mau "(AVMs)" a kahe ke koko, he pilikia nui loa ia. No laila, inā loaʻa iā ʻoe kekahi o nā hōʻailona i ʻōlelo ʻia ma luna, he mea nui loa ia e ʻimi koke i ke kōkua lapaʻau.
Hiki i kaʻu mau keiki ke loaʻa iā lākou ke HHT?
ʻAe, inā loaʻa iā ʻoe ʻo HHT, he 50% ka manawa e loaʻa ai i kēlā me kēia o kāu mau keiki ka maʻi.ʻAe. ʻO ia hoʻi, i kēlā me kēia manawa e hānau ʻia ai ke keiki, aia he 50% ka manawa e loaʻa ai i ke keiki ka HHT a he 50% ka manawa e loaʻa ʻole ai. Ua like ia me ka hoʻolei ʻana i kahi ʻāpana kālā a loaʻa nā poʻo a i ʻole nā huelo.
Pehea e ʻike ʻia ai ka HHT?
Hiki ke hana i nā hoʻāʻo genetic e hōʻoia i ka HHT. Akā ʻo ka hapa nui, ʻike nā kauka iā ia ma muli o ka ʻike lapaʻau, e like me nā hōʻailona a me ka mōʻaukala ʻohana. Ke ʻike ʻoe i ke kauka, e hana pinepine lākou i kēia mau mea:
- E nīnau lākou iā ʻoe i nā nīnau kikoʻī e pili ana i kou mōʻaukala olakino pilikino (e.g. pehea ka lōʻihi o ke kahe ʻana o ke koko i kou ihu, pehea ka pinepine, a me nā pilikia ʻē aʻe āu i loaʻa ai).
- E nīnau e pili ana i ka mōʻaukala olakino o kou ʻohana pili (nā mākua, nā kaikaina, nā keiki) no ka mea, he holo kēia ma nā ʻohana.
- E nānā ʻia kou kino (e ʻike inā he mau kiko ʻulaʻula a i ʻole nā mea like).
- Inā pono, e nānā i nā hoʻokolohua (e laʻa me nā ʻano `(scan)`, `(Endoscopy)`) e nānā i ke kūlana o nā ʻōpū o loko .
Hiki i ke kauka ke kānalua a hoʻoholo paha he HHT kou inā loaʻa iā ʻoe ma ka liʻiliʻi he ʻekolu o nā hōʻailona penei:
- Ka kahe pinepine ʻana o ke koko ihu.
- ʻO ke alo o kekahi mau telangiectasias ma nā wahi i ʻike pinepine ʻia ai lākou ma ka ʻili (e like me ka maka, nā lehelehe, nā manamana lima).
- ʻO ke alo o nā "telangiectasias" a i ʻole nā "AVM" i loko o nā ʻōpū o loko (e.g. māmā, lolo, ʻōpū, naʻau liʻiliʻi) (loaʻa kēia mau mea ma o nā hoʻokolohua wale nō).
- He lālā pili o ka ʻohana me HHT.
He aha nā lāʻau lapaʻau no HHT?
ʻO ka mea pōʻino, ʻaʻohe lāʻau lapaʻau no HHT. Eia nō naʻe, aia nā lāʻau lapaʻau e hiki ke kōkua i ka kaohi ʻana i nā hōʻailona, hōʻemi i nā hōʻailona, a hoʻemi i ka pilikia o nā pilikia koʻikoʻi. Ke hana nei nā kānaka ʻepekema e ʻimi i nā lāʻau lapaʻau no HHT, ʻoiai me ka hoʻomohala ʻana o nā lāʻau lapaʻau anti-angiogenic.
ʻOiai ke mālama nei kāu kauka i nā hōʻailona o kēia manawa, e nānā pū ʻo ia no nā pilikia e pili ana i ka HHT i loaʻa ʻole paha nā hōʻailona.
Hiki ke komo pū me kēia mau mea i ka lāʻau lapaʻau:
- Lapaʻau Laser (`(Ablation)`): He ʻoki kino liʻiliʻi e hoʻohana ana i nā kukuna laser e hoʻōki i nā wahi kahe koko (telangiectasias) i maʻalahi i ke kahe koko.
- Embolization: He hana ʻoki liʻiliʻi e ālai ai i kahi kīʻaha koko e alakaʻi ana i kahi kahe koko a i ʻole kahi AVM i pilikia i ke kahe koko.
- Lapaʻau no ka anemia: Hāʻawi ʻana i nā papa hao, a hāʻawi i ke koko (hoʻoili koko) inā pono.
- No ke kāohi ʻana i ke kahe ʻana o ka ihu: E hoʻohana i nā huahana e like me nā poni a me ka saline spray e mālama i ka pulu o loko o ka ihu.
- No ka wehe ʻana i nā AVM: Hiki ke wehe ʻia kekahi mau AVM me ka radiation therapy a i ʻole ke ʻoki kino.
- Nā lāʻau lapaʻau anti-angiogenic: Hiki ke hāʻawi ʻia kēia mau mea ma ke ʻano he infusions a i ʻole he mau pila.
Hiki paha iā ʻoe ke ʻike i nā loea no ka mālama ʻana i nā ʻōnaehana kino kikoʻī, e like me ke akepaʻa, nā māmā, ka ʻōnaehana gastrointestinal, a me ka lolo.
Hiki ke pale ʻia ka HHT?
No ka mea he maʻi hoʻoilina ia, ʻaʻohe ala e pale aku ai i ka HHT a i ʻole e hōʻemi i kou pilikia o ka loaʻa ʻana. Eia nō naʻe, inā loaʻa i kou mau mākua, nā kaikaina, a i ʻole nā keiki ka HHT, he mea nui e haʻi i kāu kauka. Hiki i kēia ke kōkua iā ʻoe e ʻike inā loaʻa iā ʻoe ke ʻano i ka wā mua, e hoʻomaka i ka lāʻau lapaʻau i ka wā mua, a pale i nā pilikia.
He aha ka manaʻolana no ka poʻe me HHT?
Hiki i ka poʻe me HHT ke ola i kahi ola maʻamau. Eia nō naʻe, he koʻikoʻi nā AVM a me ke kahe koko mau ʻana i loko o nā māmā a me ka lolo a pono e mālama ʻia. Me ka mālama pono ʻana, hiki i ka hapa nui o ka poʻe ke ola i kahi ola maʻamau.
He aha e hana ai no ke kahe ʻana o ke koko ihu ma muli o HHT?
He mea maʻamau ka kahe ʻana o ke koko ihu ma HHT, no laila aia kekahi mau mea hiki iā ʻoe ke hana e kōkua i ka pale ʻana iā lākou:
- E hōʻalo i ka hoʻohana ʻana i kekahi mau lāʻau lapaʻau, ʻoiai nā lāʻau ʻeha e like me ka aspirin a me nā NSAID (e.g. ibuprofen, diclofenac). Hiki i kēia ke hōʻemi i ke kāpili ʻana o ke koko a hoʻonui i ke kahe ʻana o ke koko. E nīnau i kāu kauka ma mua o ka lawe ʻana i kekahi lāʻau lapaʻau.
- E mālama i kahi puke moʻolelo. E kākau i lalo i nā meaʻai āu e ʻai ai a me nā hana āu e hana ai e hoʻoulu ai i ke kahe koko o kou ihu. A laila hiki iā ʻoe ke pale aku i kēlā mau mea.
- E mālama i ka pulu a me ka poni ʻia o loko o kou ihu. Hiki ke hana ʻia kēia ma ka hoʻohana ʻana i kahi humidifier, ka hoʻopili ʻana i nā poni i ʻōlelo ʻia e kāu kauka, a i ʻole ka hoʻohana ʻana i ka saline nasal spray. ʻOi aku ka maʻalahi o ke kahe ʻana o ke koko i ka ihu maloʻo.
He aha hou aʻe kaʻu e hiki ai ke nīnau aku i kaʻu kauka e pili ana i ka HHT?
Inā ua ʻike ʻia ʻoe me HHT, he mea maikaʻi ke nīnau i kāu kauka i nā nīnau e like me kēia:
- Pono anei e hoʻāʻo ʻia ke koena o koʻu ʻohana no kēia?
- Hiki iaʻu ke pāʻani i nā haʻuki? He aha nā ʻano haʻuki maikaʻi a he aha nā mea ʻaʻole?
- Aia kekahi mau hana kikoʻī e pono iaʻu ke pale aku?
- Aia kekahi waiʻona, nā meaʻai kūikawā, a i ʻole nā lāʻau lapaʻau ʻē aʻe e pono iaʻu e pale aku?
- He aha kaʻu e hana ai e pale aku ai i ke kahe ʻana o ke koko ihu a i ʻole e hoʻōki koke iā lākou?
- He palekana anei iaʻu ke hāpai? Aia kekahi mau mea kūikawā e pono iaʻu e makaʻala?
- Inā loaʻa iaʻu ke kahe koko, i ka manawa hea e pono ai iaʻu e ʻimi koke i ke kōkua lapaʻau?
ʻO ka mea hope loa, he aha e hoʻomanaʻo ai
He maʻi hoʻoilina ka HHT i ʻike pinepine ʻole ʻia. Hiki i nā hōʻailona ke ʻano like ʻole mai ke kahe koko pinepine ʻana o ka ihu a hiki i nā pilikia koʻikoʻi e pili ana i nā ʻōnaehana kino he nui. Inā manaʻo ʻoe he HHT kou a i ʻole kekahi o kou ʻohana, e ʻoluʻolu e kamaʻilio me ke kauka.Hiki i ka mālama mua ʻana ke kōkua i ka pale ʻana i nā pilikia. Hiki i ka hoʻāʻo ʻana i ka genetic ke kōkua i ka hoʻoholo ʻana inā ua pilikia nā lālā ʻē aʻe o ka ʻohana i ke kūlana. ʻO ka noho ʻana me ka ʻike ka mea nui loa i kahi kūlana e like me kēia.
HHT , Telangiectasia Hemorrhagic Hereditary, maʻi Osler-Weber-Rendu, kahe koko ihu, nā kīʻaha koko, nā maʻi genetic, AVM, telangiectasia
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