Loaʻa iā ʻoe kēia mau pilikia me kou mau māmā? E kamaʻilio kākou e pili ana i ka LAM (Lymphangioleiomyomatosis)

Ua lohe paha ʻoe i kēia maʻi me ka inoa lōʻihi i kapa ʻia ʻo LAM (lymphangioleiomyomatosis)? E ʻike paha ʻoe he mea ʻē ka inoa. Akā, he maʻi laha ʻole ia e hoʻopilikia ana i nā māmā. I kēia lā, e kamaʻilio maʻalahi mākou e pili ana i ia mea, ma ke ʻano hiki iā ʻoe ke hoʻomaopopo. Mai hopohopo, e wehewehe mākou i nā mea āpau.

He aha maoli ka LAM (Lymphangioleiomyomatosis)?

I ka ʻōlelo maʻalahi, he maʻi laha ʻole ka LAM e hoʻoulu ai i nā cysts piha i ka wai e hana i loko o kou mau māmā. Hiki i kēia ke hōʻino i kou mau māmā. I kekahi manawa hiki iā ia ke hana i nā cysts i loko o kou mau puʻupaʻa a me ka ʻōnaehana lymphatic. E noʻonoʻo i ka mea e hana ʻia inā hoʻomaka koke nā pūnaewele ʻiʻo laumania i loko o kou kino e ulu ma waho o ke kaohi. ʻO ia ka mea e hana ʻia ma ʻaneʻi. Hoʻokumu ʻia ia e nā mutations genetic.

ʻO kēia kūlana, i kapa ʻia ʻo LAM (puana ʻia ʻo "limf-AN-gee-oh-ly-oh-my-oh-muh-TOH-sis") , hoʻopilikia nui ia i nā wahine . Hoʻopilikia pinepine ia i nā wahine ma waena o 20 a me 40 mau makahiki, a i ʻole nā ​​​​wahine i hele i ka wā ʻōpio a kokoke i ka menopause. Ma muli o kēia, manaʻo nā kauka he pili paha ka hormone estrogen a i ʻole ke alo o kahi uterus me ka ulu ʻana o kēia mau puʻupuʻu.

He aha nā ʻano nui o LAM?

ʻElua ʻano nui o LAM:

1. TSC-LAM: Loaʻa i kekahi mau wahine kahi maʻi genetic i kapa ʻia ʻo "Tuberous Sclerosis Complex - TSC". Inā loaʻa iā lākou ka LAM, ua kapa ʻia ʻo TSC-LAM.

2. Sporadic LAM: Hana ʻia kēia ma ke ʻano hūpō, ʻo ia hoʻi, hana ʻia kahi mutation i loko o kahi gene ma ke ʻano hūpō i ka wā o ke ola. ʻO ka mea nui, ʻaʻole e hāʻawi ʻia kēia ʻano Sporadic LAM i kāu mau keiki.

Pehea ka maʻamau o kēia maʻi i kapa ʻia ʻo LAM?

He maʻi laha ʻole ka LAM. Ua manaʻo ʻia aia ma kahi o hoʻokahi i loko o 140,000 mau wahine e loaʻa i kēia maʻi. Eia nō naʻe, ma waena o 30% a me 80% o nā wahine me ka "Tuberous Sclerosis" e loaʻa paha iā LAM.

He aha nā hōʻailona o ka maʻi LAM?

Hiki i kekahi kanaka me LAM ke ʻike i nā hōʻailona e like me:

• Paʻakikī ka hanu ʻana (dyspnea) : I kekahi manawa hiki ke hoʻonui mālie ʻia kēia.
• He leo hūʻū ka wheezing e puka mai ana mai ka umauma mai .
• ʻEha ka umauma .
• Kuke .
• I kekahi manawa aia ke koko a i ʻole chyle (`(chyle)` - wai lymphatic mai ka ʻōnaehana hoʻoheheʻe) e puka mai ana me ka mucus .

Mai manaʻo he LAM kou no ka mea he mau hōʻailona kēia, no ka mea, hiki iā lākou ke lilo i mau hōʻailona o nā maʻi māmā ʻē aʻe. No laila, he mea nui e ʻimi i ke kōkua lapaʻau.

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

ʻO ke kumu nui o ka LAM, ʻo ia nā mutations i loko o ʻelua mau genes i loko o ko kākou kino i kapa ʻia ʻo `(TSC1)` a me `(TSC2)`. Ua like kēia mau genes ʻelua me nā genes tumor suppressor e kāohi ana i nā mea e like me nā cell cancer mai ka ulu pono ʻole ʻana i loko o ko kākou kino. No laila, inā he kīnā i loko o kekahi o kēia mau genes ʻelua, hoʻomaka kēlā mau cell muscle laumania aʻu i ʻōlelo ai ma mua e ulu me ka hiki ʻole ke kāohi ʻia. ʻO ka hopena, eia kēia:

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

Pehea e loaʻa ai i kekahi ka LAM?

ʻElua ala e hiki ai i ke kanaka ke loaʻa ka LAM. ʻO kekahi ma ka hoʻoilina ʻana i ka gene kīnā mai kahi makua nona ka hemahema i ka gene `(TSC1)` a i ʻole `(TSC2)`. ʻO kēia hoʻoilina ka hopena i ke kūlana `(Tuberous Sclerosis)`, a me ia ka pilikia o ka loaʻa ʻana o ka LAM. ʻOi aku ma mua o 30% o nā wahine me `(Tuberous Sclerosis)` he LAM.

ʻO ke ala ʻē aʻe, ʻo ia ka loli ʻana o ka gene `(TSC2)` e hana kūʻokoʻa ana, me ka ʻole o ka pilina hoʻoilina. ʻAʻole ʻike ʻia ke kumu pololei o kēia. ʻO ka LAM e ulu ana ma kēia ʻano ua kapa ʻia ʻo Sporadic LAM. I kēia hihia, ʻaʻole ʻike ʻia nā hōʻailona ʻē aʻe o `(Tuberous Sclerosis)`.

ʻO wai ka mea i ʻoi aku ka nui o ka pilikia o ka hoʻomohala ʻana i ka LAM?

ʻOiai he mea kupanaha kēia, ʻike pinepine ʻia ka LAM i nā wahine . He kakaikahi wale nō nā kāne i ʻike ʻia me ka LAM a hiki i kēia lā. Hoʻokahi wale nō kāne i hōʻike ʻia he sporadic LAM.

Eia kekahi, hiki i ka LAM ke ʻoi aku ka koʻikoʻi i ka wā hāpai a me ka hoʻohana ʻana i nā lāʻau lapaʻau i loaʻa ka estrogen .

He aha nā pilikia e hiki mai ana o ka LAM?

ʻO ka pilikia maʻamau o ka LAM ʻo ia ka pneumothorax . E like me ka pahū ʻana o ka baluna, puka ka ea mai loko mai o nā māmā a hiolo nā māmā. ʻOi aku ma mua o ka hapalua o nā wahine me LAM e loaʻa i hoʻokahi manawa o ka pneumothorax i ko lākou ola. I kekahi manawa hiki ke hana hou ʻia kēia. Hoʻomaopopo pinepine ʻia ka LAM ma hope o kēia hanana.

ʻO nā pilikia ʻē aʻe:

• Ka hōʻiliʻili ʻana o ka wai i kapa ʻia he chyle a puni nā māmā (chylothorax).
• Ka hōʻiliʻili ʻana o ka wai a puni nā māmā (pleural effusion).
• Ka ālai ʻana o ka ʻōnaehana lymphatic.

Pehea e ʻike ʻia ai ka maʻi LAM?

No ka ʻike ʻana i ka LAM, e nānā mua ke kauka iā ʻoe a nīnau e pili ana i kāu mau hōʻailona. A laila e kauoha lākou i nā hoʻokolohua a me nā hoʻokolohua kiʻi e hoʻoholo i kēia mau mea:

• E nānā i ka hana maikaʻi o kou mau māmā.
• E nānā i ka pae o ka oxygen i loko o ke koko.
• E nānā no nā loli i loko o nā māmā.
• E nānā e ʻike inā he nui aku ka nui o kekahi mau "protein" i loko o ke koko ma mua o ka maʻamau.

Ma muli o ka like o nā hōʻailona o LAM me nā maʻi maʻi māmā maʻamau, hiki i kekahi manawa ke paʻakikī i nā kauka ke ʻike.

He aha nā hoʻokolohua i hoʻohana ʻia e ʻike ai i ka LAM?

Pono paha ʻoe e hana i nā hoʻokolohua e like me kēia:

• Nā hoʻāʻo hana māmā : Nānā kēia i ka maikaʻi o ke kōkua ʻana o kou mau māmā iā ʻoe e hanu i loko a i waho.
• Ka hoʻāʻo ʻana i ka pae oxygen koko (`(pulse oximetry)`) : He hoʻāʻo maʻalahi e pili ana i ka hoʻopili ʻana i kahi mea e like me ke clip i kou manamana lima.
• Nā hoʻāʻo kiʻi : ʻO kēia mau mea e komo pū me nā scan CT (Computed Tomography scans), MRI (Magnetic Resonance Imaging), a i ʻole HRCT (High-Resolution Computed Tomography) scans. Hiki i kahi scan HRCT ke hana i kahi kiʻi maopopo o kahi wahi liʻiliʻi loa o nā māmā, e maʻalahi ai ka ʻike ʻana i kekahi mau maʻi māmā.
• Hoʻāʻo VEGF-D : Hiki i kāu kauka ke hana i kahi hoʻāʻo koko e nānā i nā maʻi, pehea ka maikaʻi o ka hana ʻana o kāu mau kino ʻē aʻe, a me kou mau pae o kahi protein i kapa ʻia ʻo VEGF-D (Vascular Endothelial Growth Factor-D). He nui ka poʻe me LAM i loaʻa nā pae kiʻekiʻe o kēia protein, akā ʻaʻole nā ​​​​mea āpau.
• ʻO ka biopsy māmā : Pili kēia i ka lawe ʻana i kahi ʻāpana liʻiliʻi o ka ʻiʻo mai ka māmā a hoʻāʻo iā ia ma kahi keʻena hoʻokolohua. Hiki ke lawe ʻia kēia hāpana ma o ka bronchoscopy (kahi hana e hoʻokomo ʻia ai kahi paipu me kahi kāmela i loko o nā māmā) a i ʻole ma ke ʻoki ʻana i ka thoracic i kōkua ʻia e ke wikiō (VATS).

Pehea e mālama ʻia ai ka maʻi LAM?

ʻO ka mea pōʻino, ʻaʻohe lāʻau lapaʻau no LAM i kēia manawa . Eia nō naʻe, hiki i ka lāʻau sirolimus (ʻike ʻia hoʻi ʻo rapamycin, inoa inoa Rapamune®) ke kōkua i ka hoʻopaʻa ʻana i ka maʻi a pale i kona ʻino ʻana. Hiki iā Sirolimus ke kōkua i ka hoʻēmi ʻana i nā puʻupaʻa puʻupaʻa (angiomyolipomas), hoʻemi i nā puʻupuʻu a me ka hōʻiliʻili ʻana o ka wai i loko o ka ʻōnaehana lymphatic, a kōkua i ka hoʻēmi ʻana i kāu mau hōʻailona.

E like me nā lāʻau lapaʻau ʻē aʻe:

• ʻO ka lāʻau lapaʻau oxygen : No ka poʻe pilikia i ka hanu ʻana.
• Nā bronchodilator hanu ʻia : Ua like kēia me nā inhalers. Wehe lākou i nā ala ea i loko o nā māmā a hoʻomaʻalahi i ka hanu ʻana.
• Hoʻoponopono hou ʻana i ka pulmonary : He papahana kēia e hoʻohui pū ana i ka hoʻoikaika kino, ka hoʻonaʻauao, a me ke aʻo ʻana.
• Hoʻoili māmā : Ma ke ʻano he koho hope loa no ka poʻe i holomua ko lākou maʻi i kahi pae holomua loa.

E aʻo pū kākou e pili ana i nā hopena ʻaoʻao a me nā pilikia o ka lāʻau lapaʻau.

Hiki i ka Sirolimus ke hōʻeha nui i nā puʻupaʻa a hoʻonui i ka pilikia o nā maʻi koʻikoʻi . No laila, he mea nui loa e kūkākūkā i nā pono a me nā pōʻino o ka hoʻohana ʻana i kēia lāʻau lapaʻau me kāu kauka ma mua o ka hoʻoholo ʻana.

He aha ke ʻano o ka wā e hiki mai ana e hiki i kekahi me LAM ke manaʻo?

Hiki i ka LAM ke lilo i mea ʻino aʻe i ka hala ʻana o ka manawa. Hiki ke loli kāu hoʻolālā lapaʻau ma muli o ke ʻano o ka lāʻau kūpono iā ʻoe. E hana pū ʻoe me kāu pulmonologist (pulmonologist) e hoʻoholo i ke ala maikaʻi loa e nānā ai i kou kūlana. E nānā mau ʻo ia i ka hana o kou māmā. E hoʻokumu ʻia kāu hoʻolālā lapaʻau ma luna o:

• kou mau makahiki.
• ʻO kou kūlana menopause (inā paha ua hala ʻoe i ka menopause a ʻaʻole paha).
• Pehea ka wikiwiki o ka emi ʻana o ka hana o ka māmā.
• Inā ua hoʻopilikia ʻia kou mau puʻupaʻa a i ʻole ka ʻōnaehana lymphatic.

Pehea ka wikiwiki o ka hoʻolaha ʻana o ka LAM?

Pehea ka wikiwiki o ka laha ʻana o ka LAM e ʻokoʻa ai mai kēlā kanaka a kēia kanaka . Aia kekahi mau kumu e hiki ke hoʻopilikia i kēia:

• Ke ʻano o ka LAM āu e loaʻa ai : ʻOiai ʻoi aku ka maikaʻi o ka hana māmā o ka poʻe me ka `(TSC-LAM)` ma mua o ka poʻe me ka `(Sporadic LAM)`, ʻo ka nui o ka emi ʻana o ka hana māmā ua like like ia ma nā mea ʻelua.
• Kou makahiki : No kekahi poʻe, oki ka laha ʻana o ka LAM ma hope o ka menopause, ke emi iho ka pae o ka estrogen i loko o ke kino.
• Pehea ʻoe e pane ai i ka lāʻau lapaʻau : Hiki i ka lāʻau Sirolimus ke hoʻopaʻa i ka maʻi i nā poʻe he nui.

I ka manawa hea e pono ai ke kāpili ʻana o ka māmā?

Hiki i nā poʻe he nui me LAM (ma kahi o 64%) ke ola i 20 mau makahiki a ʻoi aku paha ma hope o ka hōʻoia ʻana me ka ʻole o ka pono o ka hoʻoili ʻana i ka māmā. Eia nō naʻe, no ka mea, ʻaʻole hoʻomaka nā pūnaewele LAM i loko o nā māmā, hiki nō i nā cysts ke ulu aʻe ma hope o ka hoʻoili ʻana i ka māmā .

He aha ka manaʻolana ola me LAM? (Manaʻolana Ola)

ʻO ka lōʻihi o kou ola ʻana me LAM e pili ana i ke koʻikoʻi o ka maʻi a me ka wikiwiki o kona laha ʻana. Akā, ma ke ʻano holoʻokoʻa, ʻoi aku ka maikaʻi o ke kūlana i kēia manawa ma mua o ka wā ma mua. ʻOi aku ma mua o 90% o ka poʻe me LAM e ola ana he 10 mau makahiki ma hope o ka ʻike ʻia ʻana.

Pehea wau e mālama ai iaʻu iho?

E kamaʻilio me kāu kauka pulmonologist (pulmonologist) e pili ana i ka mea e manaʻo ai a pehea e hana ai i kahi hoʻolālā no ka noho ʻana me LAM. E ʻike i kāu kauka i ka manawa kūpono, e lawe i kāu mau lāʻau lapaʻau e like me ke kauoha, a e haʻi koke i kāu kauka inā loaʻa iā ʻoe kekahi mau hōʻailona hou, inā e ʻoi aku ka ʻino o kāu mau hōʻailona, ​​a inā he mau hopena ʻaoʻao kāu mai kāu lāʻau lapaʻau.

Āhea ʻoe e ʻike ai i ke kauka? Āhea ʻoe e hele ai i ka mālama ulia pōpilikia?

Inā pilikia ʻoe i ka hanu ʻana, ka ʻū, a i ʻole nā ​​​​hōʻailona hopohopo ʻē aʻe, inā hoʻomau a ʻoi aʻe paha ka ʻino, e ʻike pono i ke kauka.

I ka manawa hea e hele ai i ka Emergency Treatment Unit (ETU) :

Inā loaʻa iā ʻoe nā hōʻailona o ka hiolo ʻana o ka māmā (pneumothorax) a i ʻole nā ​​​​hōʻailona koʻikoʻi ʻē aʻe, e hele koke i ka haukapila:

• Inā pilikia ʻoe i ka hanu ʻana.
• Inā loaʻa iā ʻoe ka ʻeha nui o ka umauma .
• Inā ʻoe e ʻū ana i ke koko .
• Inā polū ka ʻili, nā lehelehe, a me nā kui (`(cyanosis)`) .

He aha nā nīnau koʻikoʻi e nīnau aku ai i ke kauka?

He mea kōkua paha ka nīnau ʻana i kāu kauka i nā nīnau e like me kēia:

• Loaʻa iaʻu ka `(Sporadic LAM)` a i ʻole `(TSC-LAM)`?
• He aha nā koho lapaʻau e loaʻa iaʻu?
• Pehea e pono ai iaʻu ke hoʻohana i kaʻu lāʻau lapaʻau?
• He aha nā hōʻailona hou a i ʻole e ʻoi aʻe ana ka ʻino e pono iaʻu e makaʻala?
• E pono anei iaʻu kahi hoʻoili māmā?

He maʻi kanesa anei ʻo LAM?

ʻAʻole i manaʻo pinepine ʻia ʻo LAM he kanesa . Eia nō naʻe, ma kekahi mau ʻano, hana ia e like me ke kanesa e laha ana mai kekahi wahi o ke kino a i kekahi (kane metastatic). ʻO ke ʻano kēia, hoʻomaka nā cell ma kahi hoʻokahi a hele ma o nā kīʻaha koko a me ka ʻōnaehana lymphatic i nā māmā a me nā puʻupaʻa. I kekahi manawa ua kapa ʻia kēia ʻano ulu ʻana he benign cell metastasis.

Akā, ʻaʻole maopopo i nā kānaka ʻepekema kahi i hoʻomaka ai kēia mau pūnaewele. Eia kekahi, ʻaʻole like kēia mau puʻupuʻu me he mea lā e pālahalaha nui ana i nā ʻōpū ʻē aʻe, e like me ke akepaʻa a i ʻole ka lolo, e like me ke kanesa. Ke nānā ʻia ma lalo o kahi microscope, ʻoi aku ka like o kēia mau pūnaewele me nā pūnaewele maʻamau ma mua o nā pūnaewele kanesa.

Mahalo i nā lāʻau lapaʻau hou a me ka hoʻomaopopo ʻana i ka LAM, ke ola nei ka poʻe i kēia manawa no ka lōʻihi me ka ʻole o ka hoʻoili ʻana i ka māmā. Akā, he manawa ka lōʻihi e hoʻomaʻamaʻa ai i kēia ʻano nohona hou. ʻO ke aʻo ʻana e pili ana i kahi maʻi no ke ola holoʻokoʻa, hiki ke manaʻo ʻia ua haehae ʻia ka lepo mai lalo o kou mau wāwae. Akā, hiki i kāu hui lapaʻau - a me kāu hui kākoʻo pilikino - ke kōkua iā ʻoe e kū hou i kou mau wāwae.

ʻO ka mea hope loa, he aha e hoʻomanaʻo ai

Hiki i ka LAM ke lilo i maʻi paʻakikī, akā ʻaʻole ʻoe hoʻokahi. Me nā holomua i ka ʻepekema lapaʻau, nui nā ala e mālama ai i kēia maʻi. ʻO ka mea nui loa, ʻo ia ke noho maikaʻi, e hahai i ka ʻōlelo aʻo a kāu kauka, a mai haʻalele i ka manaʻolana. E kamaʻilio me kāu kauka a me kāu poʻe aloha e pili ana i kou hopohopo a me kou mau makaʻu. E hoʻomanaʻo, he nui ka poʻe e hiki ke kōkua iā ʻoe ma kēia huakaʻi.

LAM , maʻi māmā, maʻi laha ʻole, pilikia hanu, sirolimus, hoʻoili māmā, nā hoʻololi hoʻoilina