E kamaʻilio kākou e pili ana i ka LAM, kahi maʻi e pili pono ana i nā wahine? (Lymphangioleiomyomatosis)

Ke manaʻo nei ʻoe i kekahi manawa ua pōkole ka hanu, luhi, a ʻeha paha ka umauma? Manaʻo pinepine mākou i kēia mau mea he mau hōʻailona o kahi maʻi maʻamau e like me ka hānō. Eia nō naʻe, aia kahi maʻi māmā kakaikahi loa e hoʻoulu ai i kēia mau hōʻailona, ​​​​ʻoi aku hoʻi i nā wahine. Ua kapa ʻia ʻo Lymphangioleiomyomatosis. He lōʻihi iki ka inoa, no laila e kapa mākou iā ia ʻo "LAM" no ka pōkole. Inā ʻike ʻoe he paʻakikī ke haʻi ʻana i kēia inoa, e noʻonoʻo penei: "Lymph-ang-ge-o-ly-o-myo-ma-to-sis". ʻOiai he mea paʻakikī iki, hiki ke lilo i mea nui loa iā ʻoe ke hoʻomaopopo i kēia maʻi ma nā ʻōlelo maʻalahi.

I ka ʻōlelo maʻalahi, he aha ka LAM?

He ʻano laha ʻole ka LAM kahi e ulu ai nā pūnaewele ʻē i loko o kou mau māmā, e hana ana i nā cysts liʻiliʻi. Hoʻokumu ʻia kēia e nā hoʻololi genetic e hoʻoulu ai i nā pūnaewele ʻiʻo laumania i loko o kou kino e ulu me ke kaohi ʻole. Hiki i kēia ke hōʻino i kou mau māmā. ʻAʻole wale nā ​​māmā, akā i kekahi manawa hiki i nā puʻupaʻa a me ka ʻōnaehana lymphatic ke hoʻomohala i kēia mau ulu.

ʻIke pinepine ʻia kēia maʻi i nā wahine ma waena o 20 a me 40 mau makahiki. ʻO ia hoʻi, ma hope o ka wā ʻōpio a ma mua o ka menopause. No laila, manaʻo nā kauka he kuleana ko ka hormone wahine estrogen i ka ulu ʻana o kēia maʻi.

ʻElua ʻano nui o ka maʻi LAM.

Hiki ke hoʻokaʻawale ʻia ka maʻi LAM i ʻelua ʻano nui, me nā ʻokoʻa iki ma waena o nā mea ʻelua.

He aha nā hōʻailona o ka maʻi LAM?

Ua like loa nā hōʻailona o ka LAM me nā maʻi māmā ʻē aʻe, e like me ka hānō a me ka bronchitis. Hiki i kēia ke lōʻihi ka manawa e ʻike ai. He mea nui e makaʻala i kēia.

• Paʻakikī ka hanu ʻana (dyspnea): ʻO kēia ka hōʻailona nui a maʻamau. ʻOiai hiki ke ʻike mua ʻia me ka hoʻoikaika iki wale nō, hiki i kēia ʻano ke ʻoi aku ka ʻino i ka hala ʻana o ka manawa.
• Wheezing: He leo hūʻū i ka wā e hanu ana, e like me ke kani ʻana o ka mea me ka hānō.
• ʻEha umauma: Hiki ke ʻike ʻia kēia ʻeha ke hanu hohonu.
• Kuke: Aia paha 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. Inā hana ʻia kekahi mea e like me kēia, ʻoi aku ka maikaʻi o ka hoʻokaʻulua ʻole .

No ke aha e hana ʻia ai kēia LAM? He aha ke kumu?

I ka ʻōlelo maʻalahi, ʻo ke kumu nui o kēia nā loli (nā mutations) e kū mai ana i nā genes ʻelua i loko o ko mākou kino i kapa ʻia ʻo TSC1 a me TSC2 . Ua like kēia mau genes ʻelua me nā kiaʻi o ko mākou kino. ʻO kā lākou hana nui ka hoʻōki ʻana i kekahi mau cell mai ka māhele ʻana a me ka hoʻonui ʻana me ke kumu ʻole me ka ʻole o ke kaohi. Kāhea mākou i kēia mau genes tumor suppressor.

E noʻonoʻo inā he kīnā i loko o kekahi o kēia mau ʻano pale ʻelua. ʻO ka mea e hana ʻia, ʻaʻole loaʻa i nā ʻiʻo laumania ka hōʻailona e ʻōlelo ai, "ʻAe, ua lawa, e hoʻōki i ka māhelehele ʻana." No laila, hoʻomaka nā ʻiʻo e māhelehele me ka ʻole o ke kāohi. ʻO ka hopena:

• Hoʻokumu ʻia nā cysts i loko o nā māmā.
• Hoʻokumu ʻia nā puʻupaʻa (angiomyolipomas) i loko o nā puʻupaʻa.
• Hoʻokumu ʻia nā cysts i loko o ka ʻōnaehana lymphatic.

Hiki iā ʻoe ke hoʻoilina i kēia gene hewa mai kou mau mākua (ʻo ia ke kumu o ka tuberous sclerosis), a i ʻole hiki iā ʻoe ke loaʻa kahi loli i kēia mau genes i ka wā o kou ola me ke kumu ʻole (ʻo ia ke kumu o ka sporadic LAM).

Nā pilikia a me nā pilikia o ka maʻi LAM

ʻO ka pilikia maʻamau o ka LAM ka hiolo ʻana o ka māmā , i ʻike ʻia ma ke ʻano lapaʻau he pneumothorax .ʻOi aku ma mua o ka hapalua o nā wahine me LAM e ʻike i kēia maʻi ma ka liʻiliʻi hoʻokahi i ko lākou ola. I kekahi manawa hiki ke hoʻi hou. ʻO ka ʻoiaʻiʻo, ua ʻike mua ʻia ka nui o nā wahine me LAM ma hope o ko lākou loaʻa ʻana o kahi abscess māmā a ua hoʻokomo ʻia i ka haukapila.

Eia kekahi, hiki ke loaʻa nā pilikia ʻē aʻe.

Pehea e ʻike ʻia ai ka maʻi LAM?

Ma hope o ka hoʻolohe ʻana i kāu mau hōʻailona, ​​​​inā kānalua kāu kauka i kēia maʻi, e kauoha ʻo ia i kekahi mau hoʻokolohua e hōʻoia iā ia.

Nā hoʻokolohua a ke kauka e hana ai

• Nā Hoʻāʻo Hana Mama: Pili kēia iā ʻoe e hanu i loko a i waho ma o kahi ʻōmole i hoʻopili ʻia i kahi mīkini. Hiki i kēia ke ana i ka maikaʻi o ka hana ʻana o kou mau mama a me ka nui o ka ea e hiki iā lākou ke paʻa.
• Pulse Oximetry: Hoʻopili ʻia kahi mea liʻiliʻi, e like me kahi clip, i kou manamana lima a ana i ka nui o ka oxygen i loko o kou koko. He hoʻāʻo maʻalahi loa kēia a ʻaʻohe ʻeha.
• Nā Kiʻi Kiʻi:
• CT Scan (Computed Tomography):Hiki i kēia ke lawe i nā kiʻi 3D kikoʻī o kou mau māmā. Hiki i kēia ke kōkua i ka ʻike maopopo inā he mau cysts e pili pono ana iā LAM.
• ʻO ke kiʻi HRCT (High-Resolution Computed Tomography): He mana holomua kēia o ke kiʻi CT. Hiki iā ia ke kiʻi i nā kiʻi maopopo loa o kahi ʻāpana liʻiliʻi loa o nā māmā. He mea nui loa kēia no ka ʻike ʻana i ka LAM.
• Hoʻāʻo koko VEGF-D: He protein ka VEGF-D i loaʻa i loko o ko kākou koko. He nui ka poʻe me LAM i loaʻa nā pae kiʻekiʻe o kēia protein i loko o ko lākou koko ma mua o ka maʻamau. No laila, he mea kōkua nui kēia hoʻāʻo koko i ka ʻike ʻana i ka maʻi.
• Biopsy Mama: I kekahi manawa, inā ʻaʻole maopopo 100% nā hoʻokolohua ʻē aʻe, hiki i kāu kauka ke hoʻoholo e lawe i kahi ʻāpana liʻiliʻi loa o ka ʻiʻo mai kou mama no ka hoʻāʻo ʻana. Hiki ke hana ʻia kēia me ka hoʻohana ʻana i kahi kaʻina hana i kapa ʻia ʻo bronchoscopy (he ʻōmole lahilahi me kahi kāmela i hoʻokomo ʻia i loko o nā alaloa ea) a i ʻole VATS (ʻokiʻoki thoracic kōkua wikiō) .

He aha nā lāʻau lapaʻau no ka maʻi LAM?

ʻO ka mea mua, ʻaʻohe lāʻau lapaʻau no ka LAM. Akā, mai hopohopo. I kēia manawa, aia nā lāʻau lapaʻau kūpono loa e hiki ke kāohi i ka maʻi, hōʻemi i nā hōʻailona, ​​a hoʻolohi i ka holomua o ka maʻi.

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 cell e ulu ana ma waho o ke kaohi. Hiki i kēia ke kōkua i ka kaohi ʻana i ka ʻino hou aʻe o ka maʻi i kahi nui. Hiki iā ia ke kōkua i ka hoʻēmi ʻana i nā puʻupaʻa i loko o nā puʻupaʻa a me ka ʻōnaehana lymphatic a hōʻemi i nā hōʻailona.

Eia kekahi, hiki i kāu kauka ke kuhikuhi i nā lāʻau lapaʻau ʻē aʻe ma muli o kou kūlana.

• Lapaʻau Oxygen: Inā haʻahaʻa ka pae oxygen i loko o ke koko, hoʻolako ʻia nā pahu oxygen no ka hoʻohana ʻana ma ka home.
• Nā bronchodilators i hanu ʻia: Hiki i kēia mau lāʻau lapaʻau, i hāʻawi ʻia ma o kahi inhaler, ke hōʻemi i ka pilikia hanu.
• Hoʻōla Hou ʻana i ka Puʻuwai: Ua like kēia me kahi papahana hoʻoikaika kino kūikawā no nā māmā. Kōkua ia i ka hoʻomaikaʻi ʻana i kou ola ma o nā hoʻomaʻamaʻa hanu a me nā hoʻomaʻamaʻa kino.
• Hoʻoili ʻana i ka māmā:Ke holomua loa ka maʻi a ʻaʻole hiki ke kaohi ʻia e kekahi lāʻau ʻē aʻe, ʻo ka hoʻoili ʻana o ka māmā paha ke koho hope loa.

Pehea ka wikiwiki o ka holomua ʻana o ka maʻi?

ʻOkoʻa kēia mai kēlā kanaka a kēia kanaka. ʻO ka wikiwiki o ka holomua ʻana o ka LAM e pili ana i kekahi mau kumu.

• ʻO ke ʻano o ka LAM āu: ʻO ka poʻe me ka sporadic LAM e emi iki paha ka hana o ka māmā ma mua o ka poʻe me TSC-LAM.
• Kou makahiki: No kekahi mau wahine, ma hope o ka menopause, pau a lohi paha ka holomua o ka maʻi i ka wā e emi ana ka nui o ka estrogen i loko o ke kino.
• ʻO kāu pane i ka lāʻau lapaʻau: Hiki i nā poʻe he nui ke kaohi maikaʻi i ka maʻi me nā lāʻau lapaʻau e like me Sirolimus.

ʻOi aku ka maikaʻi o ke kūlana i kēia manawa ma mua o ka wā ma mua. Me nā lāʻau lapaʻau hou, ʻoi aku ma mua o 90% o ka poʻe me LAM e ola ana he 10 mau makahiki ma hope o ka hōʻoia ʻana. Hiki i nā kānaka he nui (ma kahi o 64%) ke ola no 20 mau makahiki a ʻoi aku paha me ka ʻole o ka pono o ka hoʻoili ʻana i ka māmā.

I ka manawa hea e ʻike ai i kāu kauka a hele i ka ETU

I ka wā e noho pū ana me LAM, he mea nui loa ia e ʻike i ka wā e ʻimi ai i ke aʻo lapaʻau a me ka wā e hele ai i ka ʻOihana Pōpilikia (ETU).

Nīnau kekahi poʻe inā he maʻi kanesa ʻo LAM. ʻAʻole i manaʻo pinepine ʻia ʻo LAM he maʻi kanesa. Eia nō naʻe, ma kekahi mau ʻano, e like me ke ʻano o ka hele ʻana o nā cell mai kahi ʻē aʻe a i nā māmā, he ʻano like ko lākou me ke kanesa. Eia nō naʻe, i ka nānā ʻana ma lalo o kahi microscope, ʻaʻole like kēia mau cell me nā cell kanesa.

He mea maʻamau ke hopohopo a me ka makaʻu i ka wā e ʻike ai ʻoe he maʻi hoʻoweliweli kou e like me kēia. Akā, e hoʻomanaʻo, ʻaʻole ʻoe hoʻokahi. Me ke kākoʻo o kāu mau kauka, kou ʻohana, a me kou mau hoaaloha, e loaʻa iā ʻoe ka ikaika e kū ai i kēia pilikia.