He mea paʻakikī iki ka heluhelu ʻana i ka inoa, ʻeā? "Lymphangioleiomyomatosis". ʻAʻohe pilikia, e kapa wale kākou iā ia he LAM . He maʻi māmā laha ʻole kēia e pili nui ana i nā wahine. He mea maopopo ke makaʻu ke lohe ʻoe i kahi inoa e like me ia. Akā, mai hopohopo. Ma kēia ʻatikala, e kamaʻilio mākou e pili ana i ke ʻano o ka LAM, no ke aha e hana ʻia ai, a me nā lāʻau lapaʻau i loaʻa no ia ma ke ʻano maʻalahi a aloha hoʻi.
He aha ka manaʻo maʻalahi o ka LAM?
I ka ʻōlelo maʻalahi, ʻo LAM ka wā e hoʻomaka ai nā ʻiʻo laumania maʻamau ʻole i loko o kou mau māmā e ulu me ke kaohi ʻole. E like me nā puʻupuʻu liʻiliʻi. Hoʻopili kēia mau ʻāpana a hana i nā ʻano pehu liʻiliʻi i kapa ʻia he cysts i loko o kou mau māmā. I ka hala ʻana o ka manawa, i ka ulu ʻana o kēia mau puʻupuʻu, hiki iā lākou ke hōʻino i nā ʻiʻo māmā olakino a hoʻolilo iā ia i mea paʻakikī ke hanu.
ʻAʻole i kaupalena ʻia kēia maʻi i nā māmā. I kekahi manawa, hiki ke ulu aʻe nā puʻupaʻa a me ka ʻōnaehana lymphatic. ʻO ke kumu nui o kēia he mutation i loko o nā genes e kaohi ana i ka ulu ʻana o nā cell i loko o ko kākou kino.
He mea nui loa ia e hoʻomanaʻo he maʻamau kēia maʻi i nā wahine ma waena o 20 a me 40 mau makahiki, ʻo ia hoʻi, ma waena o ka wā ʻōpio a me ka menopause. No laila, manaʻo nā kauka he pili ka hormone estrogen me ia.
ʻElua ʻano nui o nā LAM.
Hiki ke hoʻokaʻawale ʻia ka maʻi LAM i ʻelua ʻano nui, me nā ʻokoʻa iki ma waena o nā mea ʻelua.
| ʻAno LAM | Ka wehewehe maʻalahi |
|---|---|
| TSC-LAM | He ʻano LAM kēia e kū mai ana i nā wahine me kahi maʻi genetic i kapa ʻia ʻo tuberous sclerosis. ʻO ke ʻano kēia e kū mai ana ma ke ʻano he ʻāpana o kekahi maʻi olakino ʻē aʻe. |
| LAM Sporadic | Hoʻokumu ʻia kēia ʻano e kahi loli genetic (mutation) i hana ʻia i ka wā o ke ola. ʻAʻole ia he hoʻoilina. No laila, inā loaʻa iā ʻoe kēia ʻano, ʻaʻole pono ʻoe e hopohopo e pili ana i kāu mau keiki e hoʻoili i ka maʻi. |
He aha nā hōʻailona o ka maʻi LAM?
Ma muli o ka like loa o nā hōʻailona o ka LAM me nā maʻi māmā maʻamau ʻē aʻe, e like me ka hānō, hiki i kekahi manawa ke lōʻihi ka manawa e ʻike ai. ʻO kēia nā hōʻailona nui.
- Paʻakikī ka hanu ʻana (dyspnea): ʻOiai ʻaʻole ʻike ʻia i ka wā mua, hiki i kēia ʻeha ke hoʻonui i ka hala ʻana o ka manawa. Hiki ke komo pū me ka luhi ʻoiai ke hele wāwae i nā wahi pōkole, a i ʻole ka hanu ʻana i ka wā e piʻi ai i nā alapiʻi.
- ʻŪhū: He leo palupalu e puka mai ana mai loko mai o ka umauma.
- ʻEha umauma: Hiki ke loaʻa koke ka ʻeha umauma koʻikoʻi.
- Kuke: He kuke maloʻo mau.
- ʻUmi ʻana i ke koko a i ʻole 'chyle': He wai mucous ka Chyle e puka mai ana mai kā mākou ʻōnaehana hoʻoheheʻe. He waiū kona kala.
No ke aha e hana ʻia ai kēia LAM? He aha ke kumu?
E like me kā mākou i ʻōlelo ai ma mua, ua hoʻokumu ʻia kēia e ka loli o nā genes. ʻElua ʻano genes i loko o ko mākou kino i kapa ʻia ʻo TSC1 a me TSC2 . Ua like kēia me nā 'kiaʻi' e kāohi ana i ka ulu ʻana o nā cell i loko o ko mākou kino. Ua kapa ʻia lākou he mau genes 'tumor suppressor'. ʻO ia hoʻi, ʻo ka hana a kēia mau genes, ʻo ia ke pale aku i nā cell mai ka māhele ʻana me ke kumu ʻole a me ka hoʻokumu ʻana i nā tumors.
He kīnā ko ke kanaka me LAM, a he mutation paha, i loko o kekahi o kēia mau genes, ʻo TSC1 a i ʻole TSC2 paha. A laila, ʻaʻole hana pono kēlā mau 'pale'. ʻO ka hopena, hoʻomaka nā pūnaewele ʻiʻo laumania e māhelehele me ka ʻole o ke kāohi ʻia, e hana ana i nā ʻano puʻupuʻu a mākou i kamaʻilio ai ma mua.
- Nā cysts māmā
- Nā puʻupaʻa puʻupaʻa (angiomyolipomas)
- Nā puʻupuʻu ʻōnaehana lymphatic
He aha nā pilikia e hiki mai ana o kēia maʻi?
ʻO ka pilikia maʻamau a weliweli hoʻi o ka LAM, ʻo ia ka hiolo ʻana o ka māmā (pneumothorax) . E noʻonoʻo i kahi cyst e like me ka pehu i loko o ka māmā e pohā koke ana, e ʻae ana i ka ea e komo i loko o ka hakahaka ma waena o ka māmā a me ka paia umauma. ʻO kēia ke kumu o ka hiolo ʻana o ka māmā. ʻOi aku ma mua o ka hapalua o nā wahine me LAM e ʻike i kēia ʻano ma ka liʻiliʻi hoʻokahi i ko lākou ola. I ka manawa pinepine, ʻike mua ʻia ka LAM ke hiki mai kahi hiolo ʻana o ka māmā.
Aia paha he mau pilikia ʻē aʻe:
- Ka hōʻiliʻili ʻana o ka wai 'chylous' a puni nā māmā (chylothorax)
- ʻO ka hōʻiliʻili ʻana o ka wai a puni nā māmā (pleural effusion)
- Ka ālai ʻana o ka ʻōnaehana lymphatic
Pehea e ʻike ai ke kauka i ka LAM?
Ma hope o ka hoʻolohe ʻana i kāu mau hōʻailona a me ka nānā ʻana iā ʻoe, inā kānalua ke kauka i kēia maʻi, e kauoha ʻo ia i kekahi mau hoʻokolohua e hōʻoia i ia mea.
| Hoʻāʻo | He aha kāu e hana ai me kēia? |
|---|---|
| Nā hoʻokolohua hana māmā | Ana ia i ka hana maikaʻi o kou mau māmā a me ka nui o ka ea hiki iā ʻoe ke lawe i loko a i waho. |
| ʻO ka Pulse Oximetry | Hoʻopili lākou i kahi mea e like me ke kinipi i kou manamana lima a nānā i ka pae oxygen i loko o kou koko. |
| Nā kiʻi (nā scan) | Hiki i ka CT scan , ʻoiai ka HRCT (High-Resolution CT scan) , ke ʻike maopopo i kēlā mau cysts liʻiliʻi i loko o nā māmā. |
| Hoʻāʻo koko VEGF-D | He protein ʻo VEGF-D. ʻO ka maʻamau, kiʻekiʻe nā pae o kēia protein i loko o ke koko o ka poʻe me LAM. He mea kōkua nui kēia i ka ʻike ʻana i ka maʻi. |
| ʻO ka biopsy māmā | Inā ʻaʻole hiki i nā hoʻokolohua ʻē aʻe ke hōʻoia i ka maʻi, lawe ʻia kahi ʻāpana liʻiliʻi o ka ʻiʻo mai ka māmā a nānā ʻia ma lalo o kahi microscope. Hiki ke hana ʻia kēia me ka hoʻohana ʻana i kahi ʻano hana e like me ka bronchoscopy a i ʻole VATS . |
He aha nā lāʻau lapaʻau no ka LAM?
ʻO ka mea mua, ʻaʻohe lāʻau lapaʻau no LAM.Akā, mai hopohopo. Aia nā lāʻau lapaʻau kūpono i kēia manawa e hiki ke kōkua i ka kaohi ʻana i kēia maʻi, hōʻemi i nā hōʻailona, a pale i kona ʻino ʻana.
ʻO ka lāʻau lapaʻau nui he lāʻau i kapa ʻia ʻo sirolimus . Ua kapa ʻia hoʻi ʻo ia he rapamycin. Hana kēia lāʻau lapaʻau ma ka hoʻōki ʻana i ka ulu ʻana o nā hunaola ʻiʻo ʻē. Hiki i kēia ke mālama i ka maʻi i ka paʻa a hoʻēmi i nā puʻupaʻa i loko o nā puʻupaʻa a me ka ʻōnaehana lymphatic.
Eia kekahi, ma muli o kou kūlana, hiki i kāu kauka ke ʻōlelo i nā lāʻau lapaʻau ʻē aʻe e like me:
- ʻO ka lāʻau lapaʻau oxygen: Inā haʻahaʻa ka pae oxygen i loko o ke koko.
- Nā lāʻau bronchodilators hanu ʻia: E hōʻemi i ka pilikia hanu ʻana.
- Hoʻoponopono hou ʻana i ka pulmonary: Nā hoʻomaʻamaʻa a me nā ʻōlelo aʻoaʻo no ke ʻano o ka nohona e hoʻoikaika ai i nā māmā.
- Hoʻoili māmā: He koho hope loa ke koʻikoʻi loa ka maʻi a ʻaʻole hiki ke kāohi ʻia me nā lāʻau ʻē aʻe.
He mea nui: Hiki i ka Sirolimus ke hana i nā hopena ʻaoʻao, e like me ka hōʻino ʻana o nā puʻupaʻa a me ka hoʻonui ʻia o ka pilikia o nā maʻi. No laila, e kamaʻilio me kāu kauka e pili ana i nā pono a me nā pōʻino o kēia lāʻau lapaʻau ma mua o ka hoʻomaka ʻana.
Nā mea e ʻike ai i ka wā e noho pū ana me LAM
He maʻi lōʻihi ka LAM. No laila, pono ʻoe e hana pū me kāu kauka pulmonologist. ʻOkoʻa ka holomua o ka maʻi, ʻo ia hoʻi, ka wikiwiki o ka holomua ʻana o ka maʻi, mai kēlā kanaka a i kēia kanaka. Aia ia i kekahi mau kumu:
- Aia nō ia i ke ʻano o ka LAM āu.
- Kou makahiki (no kekahi poʻe, ua pau ka maʻi i ka ʻino ma hope o ka menopause).
- Pehea e pane ai kou kino i ka lāʻau lapaʻau.
Mai makaʻu i ka hoʻoili ʻana o ka māmā. ʻAʻole pono ka hapa nui o ka poʻe (ma kahi o 64%) i ka hoʻoili ʻana o ka māmā a hiki i 20 mau makahiki a ʻoi aʻe paha ma hope o ka hōʻoia ʻana. Mahalo i nā lāʻau lapaʻau hou, ʻoi aku ke kiʻekiʻe o ke ola o ka poʻe e noho nei me LAM. ʻOi aku ma mua o 90% o ka poʻe e ola nei he 10 mau makahiki ma hope o ka hōʻoia ʻana.
I ka manawa hea e ʻimi ai i ke aʻo lapaʻau
I ke ola ʻana me kahi maʻi e like me kēia, he mea nui loa ka mālama ʻana i kou kino.
| Inā loaʻa iā ʻoe kēia mau hōʻailona, e ʻike i kāu kauka: | |
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| E hele koke i ka ETU (Emergency Treatment Unit) inā loaʻa iā ʻoe kēia mau hōʻailona: | |
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He maʻi kanesa anei ʻo LAM?
He pilikia kēia no nā kānaka he nui. ʻAʻole i manaʻo pinepine ʻia ʻo LAM he maʻi kanesa . Eia nō naʻe, he mau ʻano like kona me ke kanesa. No ka laʻana, hiki i nā pūnaewele ke ʻike ʻia ua laha mai nā ʻāpana ʻē aʻe o ke kino a i nā māmā a me nā puʻupaʻa. Eia naʻe, i ka nānā ʻana ma lalo o kahi microscope, ʻaʻole like kēia mau pūnaewele me nā pūnaewele kanesa, akā e like me nā pūnaewele maʻamau. Eia kekahi, ʻaʻole lākou e laha i nā ʻōpū e like me ke akepaʻa a i ʻole ka lolo.
He mea maʻamau ka manaʻo ua haehae ʻia ka honua mai lalo ou ke ʻike ʻoe he maʻi kou e like me kēia no ke ola holoʻokoʻa. Akā, ʻaʻole ʻoe hoʻokahi. Me ke kākoʻo o kāu kauka, ʻohana, a me nā hoaaloha, hiki iā ʻoe ke kū i kēia kūlana.
Leka Lawe-Home
- He maʻi maʻi māmā kakaikahi loa ka LAM i ʻike pinepine ʻia i nā wahine.
- ʻO nā hōʻailona nui ka paʻakikī o ka hanu ʻana, ke kuʻi, a me ka ʻeha o ka umauma.
- ʻO kēia ma muli o kahi mutation genetic, ʻaʻole naʻe kou hewa.
- ʻOiai ʻaʻohe lāʻau lapaʻau piha, hiki ke kaohi maikaʻi ʻia ka maʻi me nā lāʻau lapaʻau e like me sirolimus.
- E mālama mau i ka launa pū ʻana me kāu loea hanu.
- I nā pilikia e like me ka ʻeha nui o ka umauma a i ʻole ka paʻakikī nui o ka hanu ʻana, e hele koke i ka ETU.
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