He uaua te pānui i te ingoa, kāore? "Lymphangioleiomyomatosis". Kāore he raruraru, me kī noa ko LAM . He mate pūkahukahu tino onge tēnei e pāngia ana e ngā wāhine. He mea mārama te mataku ina rongo koe i tētahi ingoa pēnei. Kaua e manukanuka. I roto i tēnei tuhinga, ka kōrerohia e tātou he aha te LAM, he aha i puta ai, me ngā maimoatanga e wātea ana mōna i roto i te huarahi tino māmā me te hoa.
He aha te tikanga o te kupu LAM?
Ki te kī ngāwari, ko te LAM te wā ka tīmata te tipu haere o ngā pūtau uaua maeneene rerekē i roto i ō pūkahukahu. Pērā i ngā pukupuku iti. Ka huihui ēnei pūtau, ka hanga he hanganga iti rite ki te mirumiru e kiia nei ko ngā cysts i roto i ō pūkahukahu. I roto i te wā, i te tipu haere o ēnei pukupuku, ka taea e rātou te kino i te kiko pūkahukahu hauora, ā, ka uaua ake te manawa.
Kāore tēnei āhuatanga e pā ana ki ngā pukahukahu anake. I ētahi wā, ka puta hoki ngā pukupuku i roto i ngā whatukuhu me te pūnaha lymphatic. Ko te tino take o tēnei he whakarerekētanga ira i roto i ngā ira e whakahaere ana i te tipu o ngā pūtau i roto i tō tātou tinana.
He mea nui rawa kia maumahara ko tēnei mate te mea tino kitea i waenga i ngā wāhine i waenga i te 20 me te 40 tau te pakeke, arā, i waenga i te pakeketanga me te wā menopause. Nō reira, e whakapono ana ngā tākuta he pānga tō te homoni estrogen ki tēnei mate.
E rua ngā momo matua o ngā LAM.
E rua ngā momo matua o te mate LAM, engari he iti noa ngā rerekētanga i waenganui i a rāua.
| Momo LAM | Whakaahuatanga māmā |
|---|---|
| TSC-LAM | He momo LAM tēnei e pā ana ki ngā wāhine he mate ira e kiia nei ko te tuberous sclerosis. Ko te tikanga ka pā mai tēnei mate hei wāhanga o tētahi atu mate hauora. |
| LAM Tūpono | Ko tēnei momo mate ka puta mai i tētahi huringa ira tūpono (whakarerekētanga ira) e puta ana i te wā e ora ana. Ehara i te mea tuku iho. Nō reira, ki te pāngia koe e tēnei momo mate, kāore koe e manukanuka kei horahia te mate e āu tamariki. |
He aha ngā tohu o te mate LAM?
Nā te mea he tino ōrite ngā tohu o te LAM ki ētahi atu mate pūkahukahu noa, pērā i te mate huango, ka roa pea te wā e tautuhia ai. Koinei ngā tohu matua.
- Te uaua ki te manawa (dyspnea): Ahakoa kāore e kitea i te tīmatanga, ka piki haere pea tēnei mamae i roto i te wā. Ka uru atu pea ki te ngenge ahakoa te hikoi poto, te haunga rānei i te wā e piki ana i ngā arawhata.
- Te wheehe: He oro ngawari e puta mai ana i roto i te uma.
- Mamae o te uma: Tērā pea ka puta he mamae ohorere, he mamae nui o te uma.
- Maremare: He maremare maroke pumau.
- Te mare toto, te 'chyle' rānei: He wai hūpē te chyle e puta mai ana i tā tātou pūnaha nakunaku. He rite ki te miraka te tae.
He aha i puta ai tēnei LAM? He aha te take?
E ai ki tā mātou i kōrero ai i mua ake nei, nā te huringa o ngā ira tēnei i puta ai. E rua ngā ira i roto i tō tātou tinana, ko TSC1 me TSC2 te ingoa. He rite ēnei ki ngā 'kaitiaki' e whakahaere ana i te tipu o ngā pūtau i roto i tō tātou tinana. Ka kiia rātou ko ngā ira 'kaitātari pukupuku'. Arā, ko te mahi a ēnei ira he ārai i ngā pūtau kei wehewehea me te hanga pukupuku.
He hapa, he whakarerekētanga rānei kei roto i tētahi o ēnei ira, arā, ko te TSC1, ko te TSC2 rānei, o te tangata whai LAM. Kātahi ka kore e mahi tika aua 'kaitiaki'. Nō reira, ka tīmata te wehewehe korekore o ngā pūtau uaua maeneene, ka hanga i ngā momo pukupuku i kōrerohia e tātou i mua ake nei.
- Ngā pūkahukahu
- Ngā pukupuku whatukuhu (angiomyolipomas)
- Ngā pukupuku o te pūnaha lymphatic
He aha ngā raruraru pea ka pā mai i tēnei mate?
Ko te raruraru tino noa, tino morearea hoki o te LAM ko te hinga o te pūkahukahu (pneumothorax) . Whakaarohia he pūkahukahu rite ki te mirumiru i roto i te pūkahukahu ka pahū ohorere, ka taea ai te rere o te hau ki roto i te āputa i waenganui i te pūkahukahu me te pakitara o te uma. Ka hinga te pūkahukahu. 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. He maha ngā wā, ka kitea tuatahitia te LAM ina puta he hinga o te pūkahukahu.
Tērā pea ētahi atu raruraru:
- Te kohikohinga o te wai 'chylous' huri noa i ngā pukahukahu (chylothorax)
- Ko ētahi atu wai e kohia ana i te taha o ngā pukahukahu (te rerenga wai i roto i te pleura)
- Te aukati i te pūnaha lymphatic
Me pēhea te tātaritanga a te tākuta i te LAM?
I muri i te whakarongo ki ō tohu me te tirotiro i a koe, ki te whakapae te tākuta he mate tēnei, ka whakaritea e ia ētahi whakamātautau hei whakaū i tēnā.
| Whakamātautau | He aha tāu e mahi ai ki tēnei? |
|---|---|
| Ngā whakamātautau mahi a te pūkahukahu | Ka ine i te pai o te mahi a ō pūkahukahu me te nui o te hau ka taea e koe te ngongo me te tango. |
| Te Oximetry Pulse | Ka tāpirihia e rātou tētahi mea pērā i te topenga ki tō maihao, ā, ka tirohia te taumata hāora i roto i tō toto. |
| Whakaahua (matawai) | Ka taea e te matawai CT , inā koa te matawai HRCT (High-Resolution CT) , te kite mārama i aua pukupuku iti i roto i ngā pukahukahu. |
| Whakamātautau toto VEGF-D | He pūmua te VEGF-D. He nui te taumata o tēnei pūmua i roto i te toto o te hunga whai LAM. He tino āwhina tēnei ki te tautuhi i te mate. |
| Te koiora o ngā pūkahukahu | Ki te kore e taea e ētahi atu whakamātautau te whakaū i te mate, ka tangohia he wahi iti o te kiko mai i te pukahukahu ka tirohia i raro i te karuārai. Ka taea tēnei te mahi mā te whakamahi i tētahi tikanga pēnei i te bronchoscopy , te VATS rānei. |
He aha ngā maimoatanga mō te LAM?
Tuatahi, kāore anō kia kitea he rongoā mō te LAM.Kaua e manukanuka. He rongoā whai hua kei te wātea inaianei hei āwhina i te whakahaere i tēnei mate, hei whakaiti i ngā tohu, hei ārai hoki i tōna kino haere.
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 ngā pūtau uaua rerekē ka mahi tēnei rongoā. Ka taea e tēnei te pupuri i te pumau o te mate, me te whakaiti i ngā pukupuku i roto i ngā whatukuhu me te pūnaha lymphatic.
Hei tāpiritanga, i runga i tō āhua, ka tūtohu pea tō tākuta i ētahi atu maimoatanga pēnei i:
- Te whakaora hāora: Mena he iti te taumata hāora i roto i te toto.
- Ngā rongoā whakawhanui i ngā pūkahukahu manawa: Whakaitihia te uauatanga o te manawa.
- Te whakaora i ngā pūkahukahu: Ngā mahi whakakori tinana me ngā tohutohu mō te āhua noho hei whakapakari i ngā pūkahukahu.
- Te whakawhitinga pūkahukahu: He kōwhiringa whakamutunga tēnei ina tino kino te mate, ā, kāore e taea te whakahaere mā ētahi atu maimoatanga.
He mea nui: Ka pā mai pea ngā pānga taha o te Sirolimus, pērā i te kino o te whatukuhu me te pikinga ake o te mōrearea o ngā mate. Nō reira, kōrero ki tō tākuta mō ngā painga me ngā huakore o tēnei rongoā i mua i te tīmatanga.
Ngā mea hei mōhio māu ina noho tahi me te LAM
He mate roa te LAM. Nō reira, me mahi tahi koe me tō tākuta manawa. Ka rerekē te tere o te whanaketanga o te mate i ia tangata, i ia tangata. He maha ngā āhuatanga e pā ana ki tēnei:
- Kei te momo LAM kei a koe.
- Tō tau (mō ētahi tāngata, ka mutu te kino haere o te mate i muri i te paheketanga).
- Te urupare a tō tinana ki te maimoatanga.
Kaua e wehi i te whakawhitinga pūkahukahu. Ko te nuinga o te tangata (tata ki te 64%) kāore e hiahia ki te whakawhitinga pūkahukahu tae noa ki te 20 tau neke atu rānei i muri i te tātaritanga. Nā ngā maimoatanga hou, kua teitei ake te roanga o te oranga o te hunga e ora ana me te LAM. Neke atu i te 90% o te tangata kei te ora tonu i te 10 tau i muri i te tātaritanga.
Āhea te rapu tohutohu hauora
I te wā e noho tahi ana koe me tēnei mate, he mea tino nui te tiaki i tō tinana.
| Mena kei a koe ēnei tohu, tirohia tō tākuta: | |
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| Haere tonu ki te ETU (Emergency Treatment Unit) mēnā ka pāngia koe e ēnei tohu: | |
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He mate pukupuku te LAM?
He raruraru tēnei mō te tini o te tangata. Kāore te LAM e kiia he mate pukupuku i te nuinga o te wā . Heoi, he āhuatanga ōna e rite ana ki te mate pukupuku. Hei tauira, ka puta te āhua o ngā pūtau kua horapa mai i ētahi atu wāhanga o te tinana ki ngā pukahukahu me ngā whatukuhu. Heoi, ina tirohia i raro i te karuārai, kāore ēnei pūtau e rite ana ki ngā pūtau mate pukupuku, engari he rite ki ngā pūtau noa. Waihoki, kāore e horapa ki ngā whekau pērā i te ate, i te roro rānei.
He mea noa te whakaaro kua haehaea te whenua i raro i a koe ina rongo koe kei te pāngia koe e tēnei mate mō te wā roa. Engari kāore koe i te mokemoke. Mā te tautoko a tō tākuta, tō whānau, me ō hoa, ka taea e koe te whakatau i tēnei āhuatanga.
Karere Tangohia ki te Kāinga
- He mate pūkahukahu tino onge te LAM, ā, he maha ake ngā wā ka pā ki ngā wāhine.
- Ko ngā tohu matua ko te uaua ki te manawa, te mare, me te mamae o te uma.
- Nā te whakarerekētanga ira tēnei i puta ai, ehara i te mea nāu.
- Ahakoa kāore he rongoā katoa, ka taea te whakahaere pai i te mate mā te whakamahi i ngā rongoā pēnei i te sirolimus.
- Kia mau tonu te whakapā atu ki tō tohunga manawa.
- Mēnā he āhuatanga ohorere pērā i te mamae nui o te uma, te uaua rānei ki te manawa, me haere tonu ki te ETU.
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