Ua ʻike anei ʻoe i kahi hōʻailona hānau nui, ʻulaʻula ʻeleʻele, e like me ka waina ma ke kino o kāu pēpē i kona wā i hānau ʻia ai? A i ʻole, i ka ulu ʻana o kāu pēpē, ʻike ʻia kekahi wāwae he lōʻihi a mānoanoa ma mua o kekahi? E ʻike paha ʻoe he mau aa koko i wili ʻia. Inā loaʻa i kāu pēpē kekahi a ʻoi aku paha o kēia mau hōʻailona, he mea nui iā ʻoe e makaʻala i ke ʻano kakaikahi a mākou e kamaʻilio nei i kēia lā i kapa ʻia ʻo Klippel-Trenaunay Syndrome (KTS). ʻOiai he mea paʻakikī paha ke kani ʻana, e mālama kākou i ka maʻalahi.
I ka ʻōlelo maʻalahi, he aha ka Klippel-Trenaunay Syndrome (KTS)?
He maʻi hānau laha ʻole ʻo KTS i loaʻa i ka wā hānau. Hoʻopilikia wale ia i hoʻokahi i loko o hoʻokahi miliona mau kānaka ma ka honua holoʻokoʻa. No laila, ʻaʻole ia he mea maʻamau. ʻEkolu mau pilikia nui e ʻike ʻia i kekahi me kēia maʻi.
1. He hōʻailona hānau ʻulaʻula ma ka ʻili: Ua kapa ʻia kēia he "Port-wine stain" no ka mea ua like ia me ke kala o kahi waina liʻiliʻi.
2. Nā aʻa Varicose: ʻO kēia ke kūlana a mākou e kapa nei he "Varicose veins".
3. Ke ulu nei kekahi lima a wāwae paha ma mua o kekahi: Hiki i kekahi wāwae ke ulu a lōʻihi a mānoanoa paha ma mua o kekahi.
Ma waho aʻe o kēia mau hōʻailona nui ʻekolu, hiki i kekahi poʻe ke loaʻa i nā pilikia me ka ʻōnaehana lymphatic, kahi e kōkua ai i ka mālama ʻana i ke kaulike wai i loko o ko mākou kino.
I kekahi manawa kapa ʻia kēia e nā kauka ʻo "CLVM." ʻO ke ʻano kēia, hoʻopilikia kēia ʻano maʻi i kekahi mau ʻāpana o ko mākou kino.
- C (Capillaries): ʻO kēia nā kīʻaha koko maikaʻi loa e hoʻopili ai i ko kākou mau aa koko a me nā aʻa. Hoʻokumu ʻia kēlā hōʻailona hānau ʻulaʻula e nā loli i loko o kēia mau mea.
- L (ʻŌnaehana Lymphatic): ʻO ia hoʻi ka ʻōnaehana lymphatic. ʻĀpana o kā mākou ʻōnaehana pale kino.
- V (Veins): ʻO ia hoʻi nā aʻa koko. ʻO nā kīʻaha koko e lawe i ke koko i ka puʻuwai.
- M (Malformation): ʻO ke ʻano kēia, ʻaʻole i ulu maʻamau kahi ʻāpana.
Ua ʻike mua ʻia kēia maʻi e ʻelua mau kauka Farani i ka makahiki 1900. ʻO ia ke kumu i kapa ʻia ai kēia maʻi ma muli o lāua.
He aha nā hōʻailona o kekahi me KTS?
E nānā pono kākou i ke ʻano o ka hopena o kēia maʻi i nā kīʻaha koko, nā ʻiʻo palupalu, nā iwi, a me nā kīʻaha lymphatic o ke kanaka me KTS. No ka hoʻomaopopo pono ʻana i kēia mau hōʻailona, e nānā i ka papa ma lalo.
| Hōʻailona | He wehewehe maʻalahi |
|---|---|
| ʻO ka waihoʻoluʻu waina awa | ʻO kēia paha ka hōʻailona mua o KTS. Hoʻokumu ʻia ia e ka pehu ʻana o nā kīʻaha koko liʻiliʻi (capillaries) ma lalo o ka ʻili. Hiki ke ʻokoʻa ke kala mai ka poni māmā a i ka ʻulaʻula waina ʻeleʻele. I kou wā ʻelemakule, e lilo paha kēia wahi i mea ʻeleʻele a māmā paha. I kekahi manawa, ʻike ʻia nā pehu liʻiliʻi ma kahi, hiki ke pahū a kahe ke koko. |
| Nā aa koko varicose | Hiki i kēia kūlana ke hoʻopilikia i ka aneane o nā kānaka a pau me KTS. Ke loaʻa kēia mau pilikia i nā aʻa koko ma luna o ka ʻili, lilo nā aʻa koko i polū i nā wāwae, ʻoiai ma ka ʻūhā a me nā ʻūhā. I kekahi manawa, hiki nō hoʻi ke hoʻopilikia i nā aʻa koko e holo hohonu ana i loko o ke kino. Inā hana ʻia kēia, aia kahi pilikia o nā ʻūpā koko (Deep Vein Thrombosis - DVT). |
| Ka ulu ʻana o ka lima a i ʻole ka wāwae ma ke ʻano ʻē | I ka manawa pinepine, hoʻokahi wale nō lima a wāwae paha e ulu nui aʻe ma mua o kekahi. Hiki ke hoʻomaka kēia i ka wā kamaliʻi. Hoʻopilikia pinepine ia i hoʻokahi wāwae. Hiki i kekahi wāwae ke lōʻihi a mānoanoa paha ma mua o kekahi. Hiki i kēia ke pilikia i ka hele wāwae a me ka neʻe ʻana. |
| Nā pilikia o ka ʻōnaehana lymphatic | Loaʻa paha i kekahi poʻe nā kīʻaha lymph keu a ʻano ʻē paha. Ke hana pono ʻole kēia mau mea, hiki iā lākou ke hoʻokahe i ka wai lymphatic, hoʻoulu i ka pehu ʻana i nā wāwae, a i ʻole ke kumu o nā pilikia pelvic, bladder, a i ʻole nā pilikia ʻōpū. |
| ʻEha | Hiki i nā aa koko ke ʻā a ʻeha paha. Hiki i nā pilikia me ke kahe koko ke hoʻoulu i nā wāwae e pehu a lilo i ʻeha. Eia kekahi, hiki i nā wāwae i hoʻonui ʻia ke kaumaha a ʻeha paha. |
No ke aha lā e hana maoli ai kēia kūlana?
ʻO ka hapa nui o ka manawa, hoʻokumu ʻia ʻo KTS e kahi loli i kekahi o kā mākou mau genes. I ka pololei, hoʻokumu ʻia ia e kahi mutation i loko o ka gene i kapa ʻia ʻo PIK3CA.
ʻO ka mea nui, ʻaʻole kēia he mea i hoʻoilina ʻia mai ka makuahine a i ke keiki. Hiki pinepine kēia hoʻololi ʻana o ka genetic, me ke kumu ʻole. No laila, ma ke ʻano he mau mākua, ʻaʻohe kumu e kaumaha ai a hoʻāhewa paha iā ʻoe iho no kēia.
I kekahi manawa, loaʻa iā KTS ka poʻe i loaʻa ʻole kahi loli i ka gene PIK3CA, no laila manaʻoʻiʻo nā kānaka noiʻi he kuleana paha nā loli genetic ʻē aʻe.
He aha nā pilikia ʻē aʻe e hiki ke hana ma muli o KTS?
Inā ʻaʻole mālama pono ʻia ʻo KTS, hiki ke loaʻa kekahi mau pilikia, a he mea nui e ʻike iā lākou.
- Nā ʻūpā koko: Hiki i nā ʻūpā koko (DVT) ke hana ʻia i loko o nā aʻa koko hohonu o nā wāwae. ʻO ka pilikia nui loa, inā haki kēia ʻūpā koko a paʻa i loko o kahi aʻa koko i loko o nā māmā, hiki ke lilo i mea hoʻoweliweli i ke ola (Pulmonary Embolism).
- Nā maʻi ʻili: Hiki ke loaʻa nā maʻi bacteria e kū mai ana ma lalo o ka ʻili (cellulitis).
- Pehu: Hiki i nā pilikia me ka ʻōnaehana lymphatic ke hoʻoulu i ka hōʻiliʻili ʻana o ka wai i loko o ke kino a me ke kumu o ka pehu ʻana (Lymphedema).
- Ke kahe koko kūloko: Hiki ke kahe koko mai ka ʻōpū, nā ʻōpū, ka ʻōpū mimi, a i nā wahine paha, ka ʻōnaehana hānau.
I ka mea pinepine ʻole, hiki i kekahi poʻe me KTS ke loaʻa nā kīnā hānau e pili ana i nā manamana lima a me nā manamana wāwae paha.
- Loaʻa nā manamana lima keu (Polydactyly)
- Syndactyly (nā manamana lima i hui pū ʻia)
Pehea e ʻike pololei ai ke kauka i ka KTS?
E ʻike mua ke kauka i ke kūlana ma ka nānā ʻana i ke ʻano kino o ka pēpē. Inā loaʻa ma ka liʻiliʻi ʻelua o nā hōʻailona nui ʻekolu a mākou i kūkākūkā ai ma mua (nā māka hānau, nā aʻa kolo, a me nā lālā i hoʻonui ʻia), ua manaʻo ʻia he KTS paha ia. ʻOiai ʻike pinepine ʻia kēia mau hōʻailona i ka wā hānau, aia kekahi mau manawa hiki ke ʻike ʻia ma mua o ka hoʻi ʻana o ka pēpē mai ka haukapila.
Hiki i ke kauka ke kauoha i nā hoʻokolohua like ʻole e hōʻoia i kēia kūlana a ʻike pono i nā hopena i ke kino.
| Hoʻāʻo | He aha kāu e ʻike ai ma kēia? |
|---|---|
| ʻO ke kiʻi CT a i ʻole ka kiʻi MRI | E nānā i ke kūlana o nā ʻiʻo palupalu a me nā iwi o ke kino, a me ka nui o ka ulu ʻana ma ke ʻano ʻē. |
| MRA (Angiogram Resonance Magnetic) | He ʻano kūikawā kēia o ke kiʻi MRI. Hiki iā ia ke ʻike maopopo i ke kūlana o nā kīʻaha koko a me nā aa koko. |
| ʻO ke kani ʻana o ka Doppler Ultrasound | E nānā i ke ʻano o ke kahe ʻana o ke koko ma o nā aʻa koko a me nā ʻaʻa, a ʻike inā he ālai ʻia a kahe hou paha ma nā wahi. |
He aha nā lāʻau lapaʻau no KTS?
ʻAʻohe lāʻau lapaʻau no KTS. Eia nō naʻe, he nui nā lāʻau lapaʻau e hiki ke kōkua i nā kānaka e hoʻokele i kā lākou mau hōʻailona a me nā pilikia a ola i kahi ola maʻamau. Aia ka lāʻau lapaʻau i nā hōʻailona o kēlā me kēia kanaka.
| ʻAno lapaʻau | He aha ka mea e hana ʻia ai? |
|---|---|
| Nā lāʻau hoʻokahe koko (Anticoagulants) | Kōkua kēia lāʻau lapaʻau i ka hōʻemi ʻana i ka pilikia o nā ʻōpū koko. |
| ʻO ka lāʻau lapaʻau Sirolimus | Hoʻolohi a hoʻōki paha kēia lāʻau lapaʻau i ka ulu ʻana o nā kīʻaha koko ʻē, a laila e hōʻemi ana i nā hōʻailona a me nā pilikia. |
| Nā Kāʻei Hoʻopaʻa | Kōkua kēia i ka hōʻemi ʻana i ka pehu a me ka ʻeha ma nā wāwae, a hōʻemi i ka pilikia o nā ʻūpā koko. Kōkua lākou i ke kahe ʻana o ke koko mai nā wāwae a i ka puʻuwai. |
| Nā hāpai kāmaʻa | Inā ʻaʻole like ka lōʻihi o kou mau wāwae, ʻo ka hoʻohui ʻana i kahi ʻāpana kiʻekiʻe i hoʻokahi kāmaʻa hiki ke maʻalahi ka hele wāwae. Kōkua nō hoʻi kēia i ka hōʻemi ʻana i ka pilikia o ka scoliosis. |
| Hoʻōla laser | Hoʻohana ʻia kēia lāʻau lapaʻau e hoʻomāmā i ke kala o kahi māka hānau "port-wine stain" ma ka ʻili. |
| ʻO ke ʻano hana sclerotherapy | Hoʻokomo ʻia kahi wai kūikawā i loko o nā aʻa pilikia, kahi e hoʻopau ai a hoʻopaʻa iā lākou. He lāʻau lapaʻau kūpono kēia no nā aʻa varicose. |
| ʻoki kino | Hiki ke hana ʻia ke ʻoki ʻana e hoʻoponopono i nā pilikia nui o nā aʻa koko, hoʻohālikelike i ka lōʻihi o ka wāwae, a hoʻemi paha i ka nui o ka lima/wāwae i hoʻonui ʻia ma ka wehe ʻana i nā ʻiʻo keu. |
He aha kekahi mau mea e makaʻala nui ai i ka wā e noho ai me KTS?
I ka wā e noho ana me KTS, he mea nui loa ka hoʻomaopopo maikaʻi ʻana i kou kino a me ka mālama ʻana i kekahi mau mea.
- E mālama pono i kou ʻili: ʻO ka mālama ʻana i kou ʻili i ka maʻemaʻe a me ka ʻole o nā ʻeha e hiki ke hōʻemi i ka pilikia o nā maʻi ʻili.
- E noho ikaika e like me ka hiki: Mai noho ma ke kūlana hoʻokahi no ka lōʻihi loa. Hiki i ka hoʻoikaika kino mau a me ka hele wāwae ke hōʻemi i ka pilikia o nā ʻūpā koko.
- E ʻaʻahu i nā lole hoʻopaʻa e like me ke kuhikuhi a kāu kauka: Inā ua kauoha kāu kauka, e ʻaʻahu mau i nā socks kūikawā a i ʻole nā lole e hōʻemi ana i ka pehu.
- E hōʻalo i kekahi mau ʻano kaohi hānau: E kamaʻilio me kāu kauka e pili ana i ka pale ʻana i nā mea e like me nā lāʻau kaohi hānau i loaʻa ka hormone estrogen, ʻoiai lākou e hoʻonui ai i ka pilikia o nā ʻōpū koko.
- I ka wā hāpai a ma mua o ke ʻoki kino: I loko o kēia mau manawa, pono paha ʻoe e lawe i nā lāʻau hoʻomāmā koko e pale aku i nā ʻūpā koko, no ka mea, ʻoi aku ka nui o ka pilikia o nā ʻūpā koko. E kamaʻilio me kāu kauka e pili ana i kēia a e loaʻa ka ʻōlelo aʻo.
Āhea wau e ʻike ai i ke kauka? He aha nā manawa e hele ai i ka ETU?
He mea nui i kekahi me KTS ke hana i nā nānā kino maʻamau i ko lākou ola a pau. ʻO kēia ka manawa e hiki ai ke nānā ʻia ke kūlana, hiki ke ʻike ʻia ka hana ʻana o ka lāʻau lapaʻau, a hiki ke hana i nā hoʻololi e pono ai.
Eia nō naʻe, i kekahi manawa pono paha ʻoe e hele i ka haukapila i kahi pilikia. E makaʻala i kēia mau hōʻailona.
Eia nā kūlana e pono ai ʻoe e hele koke i ka ETU (Emergency Treatment Unit):
* Inā ʻike ʻoe i nā hōʻailona o ke koko ʻūlū: Inā ʻeha koke kekahi wāwae, pehu, ʻulaʻula, a mehana ke pā, hiki ke lilo i deep vein thrombosis (DVT).
* Inā loaʻa iā ʻoe nā hōʻailona o ke koko i loko o nā māmā: ʻEha koke o ka umauma, ka paʻakikī o ka hanu ʻana, ka hanu wikiwiki ʻana, a i ʻole ke kuʻi ʻana i ke koko, he pulmonary embolism paha ia. He pilikia kēia.
* Inā he kahe koko nui: Inā he kahe koko hiki ʻole ke kaohi ʻia mai nā wahi a pau o ke kino.
Inā ʻike ʻoe i kekahi o kēia mau hōʻailona, mai hoʻonele i ka manawa a e hele koke i ka ʻOihana Pōpilikia (ETU) o ka haukapila kokoke loa.
He maʻi paʻakikī ʻo KTS, no laila he nui paha kāu mau nīnau. I kēlā me kēia manawa āu e ʻike ai i kāu kauka, e hoʻolohe i nā mea āpau ma kou manaʻo. Mai noʻonoʻo, "He pilikia wale nō kēia ʻaʻole pono iaʻu e hoʻoponopono." ʻOi aku ka maikaʻi o ka wehe a me ka ʻoiaʻiʻo me kāu kauka.
Leka Lawe-Home
- ʻO ka Klippel-Trenaunay Syndrome (KTS) kahi kīnā hānau kakaikahi ʻaʻole i hana ʻia e kekahi hewa o nā mākua.
- ʻO nā hōʻailona nui ʻekolu: he hōʻailona hānau ʻulaʻula ʻeleʻele (ʻili waina awa), nā aʻa koko varicose, a me kekahi lima a wāwae paha i ʻoi aku ka nui ma mua o kekahi.
- ʻOiai ʻaʻole hiki ke ho'ōla loa ʻia kēia maʻi, nui nā lāʻau lapaʻau e hiki ke kōkua iā ʻoe e hoʻokele i kāu mau hōʻailona a ola i kahi ola maikaʻi loa.
- ʻO nā pilikia koʻikoʻi e makaʻala ai, ʻo ia nā ʻōpū koko (DVT, Pulmonary Embolism) a me ke kahe koko nui. E makaʻala mau i nā hōʻailona hōʻike o kēia mau mea.
- He mea nui loa e noho mau me kāu kauka i kou ola a hele i nā hoʻokolohua maʻamau.
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