Me kōrero tātou mō te LAM, he mate e pā ana ki ngā wāhine? (Lymphangioleiomyomatosis)

He poto te manawa, he ngenge, he mamae rānei tō uma i ētahi wā? He maha ngā wā ka whakaarohia ēnei hei tohu o tētahi mate noa pēnei i te mate huango. Heoi, he mate pūkahukahu tino onge e puta ai ēnei tohu, inā koa i roto i ngā wāhine. Ko te ingoa ko Lymphangioleiomyomatosis. He roa te ingoa, nō reira ka tapaina e mātou ko "LAM" hei whakarāpopototanga. Ki te mea he uaua ki a koe te whakahua i tēnei ingoa, whakaarohia pēnei: ​​"Lymph-ang-ge-o-ly-o-myo-ma-to-sis". Ahakoa he uaua, he mea tino nui kia mārama koe ki tēnei mate i roto i ngā kupu māmā.

He aha te LAM?

He mate onge te LAM, e tipu ai ngā pūtau rerekē i roto i ōu pūkahukahu, ka hanga he pūkahukahu iti. Nā ngā whakarerekētanga ira tēnei e tupu ai ngā pūtau uaua maeneene o tō tinana. Ka kino pea ōu pūkahukahu. Ehara i te mea ko ngā pūkahukahu anake, engari i ētahi wā ko ngā whatukuhu me te pūnaha lymphatic ka puta mai ēnei tipu.

Ko te nuinga o te wā ka kitea tēnei mate i roto i ngā wāhine i waenga i te 20 me te 40 tau te pakeke. Arā, i muri i te pakeketanga me mua i te paheketanga. Nō reira, e whakapono ana ngā tākuta he wāhi nui tō te homoni wahine o te estrogen ki te whanaketanga o tēnei mate.

E rua ngā momo matua o te mate LAM.

E rua ngā momo matua o te mate LAM, engari he iti noa ngā rerekētanga i waenganui i a rāua.

He aha ngā tohu o te mate LAM?

He tino rite ngā tohu o te LAM ki ērā o ētahi atu mate pūkahukahu, pērā i te mate huango me te mate pūkahukahu. I ētahi wā ka roa pea te tautuhi i tēnei mate. He mea nui kia mōhio ki tēnei.

• Te uaua ki te manawa (dyspnea): Koinei te tohu matua, te tohu tino kitea. Ahakoa ka kitea pea i te tīmatanga mā te mahi ngāwari, ka kino haere pea tēnei āhua i roto i te wā.
• Te wheehe: He tangi whiowhio i te wā e manawa ana, he rite ki te tangi o te tangata whai huango.
• Mamae o te uma: Tērā pea ka pāngia tēnei mamae ina koa ka manawa hōhonu koe.
• Maremare: Tērā pea ka mau tonu te maremare maroke.
• Te mare toto, te 'chyle' rānei: He wai hūpē te chyle e puta mai ana i roto i tō tātou pūnaha nakunaku. He rite tōna tae ki te miraka. Mēnā ka puta he mea pēnei, kaua e whakaroa .

He aha i puta ai tēnei LAM? He aha te take?

Ki te kī māmā noa, ko te take matua mō tēnei ko ngā huringa (ngā whakarerekētanga) e puta ana i roto i ngā ira e rua o tō tātou tinana, ko TSC1 me TSC2 . He rite ēnei ira ki ngā kaitiaki o tō tātou tinana. Ko tā rāua mahi matua he aukati i ētahi pūtau kia kaua e wehewehe me te whakanui rawa me te kore e taea te whakahaere. Ka kiia ēnei he ira pehi pukupuku.

Whakaarohia mēnā he hapa kei roto i tētahi o ēnei ira tiaki e rua. Ko te mea ka tupu, kāore ngā pūtau uaua maeneene e whiwhi i te tohu hei mea, "Āe, kati te wehewehe." Nō reira ka tīmata te wehewehe o ngā pūtau me te kore e taea te whakahaere. Ko te mutunga iho:

• Ka puta ngā pūkahukahu i roto i ngā pūkahukahu.
• Ka puta ngā pukupuku (angiomyolipomas) i roto i ngā whatukuhu.
• Ka puta ngā pūkahukahu i roto i te pūnaha lymphatic.

Ka taea e koe te tuku iho i tēnei ira hapa mai i ō mātua (nāna te mate tuberous sclerosis), ka taea rānei e koe te huri noa i ēnei ira i roto i tō oranga, kāore he take mārama (nāna te mate LAM karekau e kitea).

Ngā raruraru me ngā mōrearea o te mate LAM

Ko te raruraru tino noa o te LAM ko te hinganga o te pukahukahu , e mōhiotia ana i te taha hauora ko te pneumothorax .Neke atu i te haurua o ngā wāhine whai LAM ka pāngia e tēnei mate i te iti rawa kotahi i roto i ō rātou oranga. I ētahi wā ka hoki anō. Inaa, he maha ngā wāhine ka kitea tuatahitia he LAM i muri i te pāngia e te whewhe pūkahukahu, ā, ka haria ki te hōhipera.

Hei tāpiritanga, ka puta pea ētahi atu raruraru.

Me pēhea te tautuhi i te mate LAM?

I muri i te whakarongo ki ō tohumate, ki te whakapae tō tākuta he mate tēnei, ka whakaritea e ia ētahi whakamātautau hei whakaū i tēnā.

Ngā whakamātautau e mahia ana e te tākuta

• Ngā Whakamātautau Mahi a ō Pūkahukahu: Kei roto i tēnei ko te manawa ki roto, ki waho hoki mā roto i tētahi ngongo e hono ana ki tētahi mīhini. Ka taea e tēnei te ine i te pai o te mahi a ō pūkahukahu me te nui o te hau ka taea e rātou te pupuri.
• Ine Pūwero: Ka tāpirihia he taputapu iti, he rite ki te topenga, ki tō maihao, ā, ka ine i te nui o te hāora i roto i tō toto. He whakamātautau tino māmā, kāore hoki he mamae.
• Ngā Matawai Whakaahua:
• Matawai CT (Tomography Rorohiko):Ka taea e tēnei te tango whakaahua 3D taipitopito o ō pukahukahu. Ka āwhina tēnei ki te kite mārama mēnā he pukupuku e pā ana ki te LAM.
• Matawai HRCT (High-Resolution Computed Tomography): He putanga matatau tēnei o te matawai CT. Ka taea e ia te tango whakaahua tino mārama o tētahi wāhanga iti rawa o ngā pukahukahu. He mea tino nui tēnei mō te tautuhi i te LAM.
• Whakamātautau toto VEGF-D: He pūmua kei roto i ō tātou toto te VEGF-D. He nui ake ngā taumata o tēnei pūmua i roto i ō rātou toto i te mea noa. Nō reira, he tino āwhina tēnei whakamātautau toto ki te tautuhi i te mate.
• Kohikohi Pūkahukahu: I ētahi wā, ki te kore e tino 100% te tino mārama o ētahi atu whakamātautau, ka whakatau pea tō tākuta ki te tango i tētahi wahi kiko iti rawa mai i tō pūkahukahu hei whakamātautau. Ka taea tēnei te mahi mā te whakamahi i tētahi tikanga e kiia nei ko te bronchoscopy (he ngongo angiangi me te kāmera e whakaurua ana ki roto i ngā ara manawa) me te VATS (tapahi uma āwhina-ataata) rānei.

He aha ngā maimoatanga mō te mate LAM?

Tuatahi, kāore anō kia kitea he rongoā mō te LAM. Kaua e manukanuka. He tino whai hua ngā maimoatanga inaianei hei whakahaere i te mate, hei whakaiti i ngā tohu, hei whakaroa hoki i te haere whakamua o te mate.

Ko te rongoā matua he rongoā e kiia nei ko te sirolimus . E kiia ana hoki ko te rapamycin. Mā te aukati i te tipu o ēnei pūtau kāore i te whakahaeretia te mahi a tēnei rongoā. Ka āwhina tēnei ki te whakahaere i te whanaketanga o te mate ki te whānuitanga nui. Ka āwhina hoki ki te whakaiti i ngā pukupuku i roto i ngā whatukuhu me te pūnaha lymphatic me te whakaiti i ngā tohu.

Hei tāpiritanga, ka tūtohu pea tō tākuta i ētahi atu maimoatanga i runga i tō āhua.

• Whakaora Hāora: Mena he iti te taumata hāora i roto i te toto, ka whakaratohia he tāke hāora hei whakamahi ki te kāinga.
• Ngā rongoā whakawātea manawa: Ka taea e ēnei rongoā, e hoatu ana mā te inhaler, te whakaiti i te uauatanga o te manawa.
• Te Whakaora i ngā Pūkahukahu: He rite tēnei ki tētahi kaupapa whakakori tinana motuhake mō ngā pūkahukahu. Ka āwhina i te whakapai ake i tō kounga o te oranga mā roto i ngā mahi manawa me ngā mahi whakapakari tinana.
• Te Whakatō Pūkahukahu:Ina kua tino horapa te mate, ā, kāore e taea te whakahaere e tētahi atu maimoatanga, ko te whakawhitinga pukahukahu pea te kōwhiringa whakamutunga.

Kia pēhea te tere o te whanaketanga o te mate?

He rerekē tēnei i ia tangata, i ia tangata. Ko te tere o te whanaketanga o te LAM e whakawhirinaki ana ki ētahi āhuatanga.

• Te momo LAM kei a koe: Ko te hunga whai LAM tūpono ka iti ake pea te mahi a ō rātou pukahukahu i ērā he TSC-LAM.
• Tō tau: Mō ētahi wāhine, i muri i te paheketanga, ka mutu, ka puhoi rānei te haere whakamua o te mate i te mea ka heke te nui o te estrogen i roto i te tinana.
• Tō urupare ki te maimoatanga: He maha ngā tāngata ka taea te whakahaere pai i te mate mā te whakamahi i ngā rongoā pēnei i a Sirolimus.

He pai ake te āhua o te wā ināianei i tō mua. Mā ngā maimoatanga hou, neke atu i te 90% o te hunga whai LAM e ora tonu ana i roto i ngā tau 10 i muri i te tātaritanga. He tokomaha te hunga (tata ki te 64%) ka taea te ora mō ngā tau 20, neke atu rānei, me te kore e hiahiatia he whakawhitinga pukahukahu.

Āhea te wā e kite ai koe i tō tākuta me te haere ki te ETU

I te wā e noho tahi ana koe me te LAM, he mea tino nui kia mōhio koe ki te wā e rapu tohutohu hauora ai, me te wā e haere ai ki te Tari Whawhati Tata (ETU).

Ka pātai ētahi mēnā he mate pukupuku te LAM. Kāore te LAM i te kiia he mate pukupuku. Heoi, i ētahi āhuatanga, pērā i te huarahi e puta mai ai ngā pūtau i tētahi atu wāhi ki ngā pukahukahu, he rite tonu ō rātou āhuatanga ki te mate pukupuku. Heoi, ina tirohia i raro i te karuārai, kāore ēnei pūtau e rite ki ngā pūtau mate pukupuku.

He mea noa te ohorere me te mataku ina rongo koe kei te pāngia koe e tētahi mate morearea pēnei. Engari kia maumahara, ehara i te mea ko koe anake. Mā te tautoko a ō tākuta, tō whānau, me ō hoa, ka kitea e koe te kaha ki te aro atu ki tēnei wero.