Kei a koe ēnei raruraru ki ō pukahukahu? Me kōrero tātou mō te LAM (Lymphangioleiomyomatosis)

Kua rongo kē koe i tēnei mate he roa nei tōna ingoa, ko te LAM (lymphangioleiomyomatosis)? Tērā pea ka kitea e koe he rerekē te ingoa. Engari he mate onge tēnei e pā ana ki ngā pukahukahu. I tēnei rā, ka kōrerohia e tātou he mea ngāwari noa iho, i roto i tētahi huarahi e mārama ai koe. Kaua e manukanuka, ka whakamāramahia e mātou ngā mea katoa.

He aha rawa te LAM (Lymphangioleiomyomatosis)?

He māmā noa iho, he mate onge te LAM e puta ai ngā pūkahukahu kī tonu i te wai. Ka taea e tēnei te kino i ō pūkahukahu. I ētahi wā ka puta hoki ngā pūkahukahu i roto i ō whatukuhu me te pūnaha lymphatic. Whakaarohia te mea ka tupu mēnā ka tīmata te tipu haere o ngā pūtau uaua maeneene o tō tinana. Koinei te mea ka tupu i konei. Nā ngā whakarerekētanga ira i puta mai.

Ko tēnei mate, e kiia nei ko LAM (e whakahuatia ana ko "limf-AN-gee-oh-ly-oh-my-oh-muh-TOH-sis") , ka pāngia te nuinga o ngā wāhine . Ko te nuinga o te wā ka pāngia ngā wāhine i waenga i te 20 me te 40 tau te pakeke, ngā wāhine rānei kua pakeke haere, ā, kua tata te paheketanga. Nā tēnei, e whakaaro ana ngā tākuta he pānga pea te homoni estrogen, te noho mai rānei o te kōpū ki te whanaketanga o ēnei pukupuku.

He aha ngā momo matua o te LAM?

E rua ngā momo matua o te LAM:

1. TSC-LAM: Kei ētahi wāhine tētahi mate ira e kiia nei ko te "Tuberous Sclerosis Complex - TSC". Mēnā ka pāngia rātou e te LAM, ka kiia ko te TSC-LAM.

2. LAM Tūpono: Ka puta noa tēnei i te matapōkere, arā, ka puta he whakarerekētanga ira i roto i tētahi ira i te wā e ora ana. Ko te mea nui, kia kaua tēnei momo LAM Tūpono e tukuna ki āu tamariki.

He pēhea te noa o te rongonui o tēnei mate e kiia nei ko te LAM?

He mate tino onge te LAM. E kiia ana kotahi i roto i te 140,000 ngā wāhine ka pāngia e tēnei mate. Heoi, i waenga i te 30% me te 80% o ngā wāhine e pāngia ana e te "Tuberous Sclerosis" ka pāngia e te LAM.

He aha ngā tohu o te mate LAM?

Tērā pea ka pāngia te tangata whai LAM e ngā tohu pēnei i:

• Te uaua ki te manawa (dyspnea) : I ētahi wā ka piki haere haere tēnei.
• Ko te wheezing he tangi whiowhio e puta mai ana i te uma .
• Te mamae o te uma .
• Maremare .
• I ētahi wā ka puta te toto, te wai lymphatic rānei mai i te pūnaha nakunaku) me te hūpē .

Kaua e whakaaro kei a koe te LAM nā te mea kei a koe ēnei tohu, nā te mea he tohu anō hoki ēnei o ētahi atu mate pūkahukahu. Nō reira, he mea nui kia rapu tohutohu hauora.

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

Ko te tino take o te LAM ko ngā whakarerekētanga ira i roto i ngā ira e rua o roto i tō tātou tinana e kiia nei ko `(TSC1)` me `(TSC2)`. He rite ēnei ira e rua ki ngā ira ārai pukupuku e aukati ana i ngā mea pēnei i ngā pūtau matepukupuku kei tipu koretake i roto i tō tātou tinana. Nō reira, ki te mea he hapa kei roto i tētahi o ēnei ira e rua, ka tīmata ngā pūtau uaua maeneene i kōrerotia e au i mua ake nei ki te tipu kore e taea te whakahaere. Nō reira,:

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

Me pēhea te pāngia o te tangata e te LAM?

E rua ngā huarahi ka pāngia ai te tangata e te LAM. Ko tētahi ko te tuku iho i te ira hapa mai i tētahi mātua he hapa tōna ira `(TSC1)`, `(TSC2)` rānei. Nā tēnei tuku iho ka hua ake te mate `(Tuberous Sclerosis)`, ā, ka haere tahi mai me tēnei te mōrearea o te pāngia o te LAM. Neke atu i te 30% o ngā wāhine whai `(Tuberous Sclerosis)` he LAM.

Ko tētahi atu huarahi ko te puta ohorere o te whakarerekētanga ira i roto i te ira `(TSC2)`, kāore he hononga tuku iho. Kāore i te mōhiotia te tino take o tēnei. Ko te LAM e whanake ana i tēnei ara ka kiia ko te Sporadic LAM. I tēnei take, kāore e puta ngā tohu kē atu o te `(Tuberous Sclerosis)`.

Ko wai kei te nui ake te mōrearea ki te whakawhanake i te LAM?

Ahakoa he mea miharo tēnei, ko te nuinga o te wā ka pā te LAM ki ngā wāhine . He torutoru noa iho ngā tāne kua kitea he LAM kei a rātou tae noa mai ki tēnei wā. Kotahi noa iho te tāne kua pūrongohia he LAM tūpono noa iho.

Waihoki, ka kaha ake te kino o te LAM i te wā e hapu ana , me te wā e whakamahi ana i ngā rongoā kei roto ko te estrogen .

He aha ngā raruraru pea ka pā mai i te LAM?

Ko te raruraru tino noa o te LAM ko te pneumothorax . Pērā i te pahūtanga o te poihau, ka turuturu te hau i roto i ngā pukahukahu, ā, ka hinga ngā pukahukahu. Neke atu i te haurua o ngā wāhine whai LAM ka pāngia e te pneumothorax i te iti rawa kotahi te wā i roto i ō rātou oranga. I ētahi wā ka puta anō tēnei. He maha ngā wā ka kitea te LAM i muri i tēnei wā.

Ētahi atu raruraru:

• He pupūnga wai e kiia nei ko te chyle (`(chyle)`) e karapoti ana i ngā pukahukahu (`(chylothorax)`).
• Te kohi wai i te taha o ngā pukahukahu (te rerenga wai i roto i te pleura).
• Te aukati i te pūnaha lymphatic.

Me pēhea te tautuhi i te mate LAM?

Hei tautuhi i te LAM, ka tirohia koe e te tākuta i te tuatahi, ka pātai atu mō ō tohu. Kātahi ka whakaritea he whakamātautau me ngā whakamātautau whakaahua hei whakatau i ngā mea e whai ake nei:

• Tirohia te pai o te mahi a ōu pūkahukahu.
• Tirohia te taumata o te hāora i roto i te toto.
• Tirohia mēnā he huringa kei roto i ngā pukahukahu.
• Tirohia mēnā he nui ake i te tikanga te nui o ētahi "pūmua" i roto i te toto.

Nā te mea he rite ngā tohu o te LAM ki ērā o ētahi atu mate pūkahukahu noa, he uaua pea i ētahi wā ki ngā tākuta te tautuhi i te mate.

He aha ngā whakamātautau e whakamahia ana hei tautuhi i te LAM?

Me mahi pea koe i ngā whakamātautau penei:

• Ngā whakamātautau mahi a ōu pūkahukahu : Ka tirohia e ēnei te pai o te āwhina a ōu pūkahukahu i a koe ki te manawa ki roto, ki waho hoki.
• Te tirotiro i te taumata hāora i roto i te toto (`(pulse oximetry)`) : He whakamātautau māmā noa iho e uru ana ki te whakapiri i tētahi mea pēnei i te topenga ki tō maihao.
• Ngā whakamātautau whakaahua : Kei roto i ēnei ko ngā matawai CT (matawai Tomography Rorohiko), MRI (Whakaahua Resonance Āhua), HRCT (Tomography Rorohiko Taumira Teitei rānei). Ka taea e te matawai HRCT te whakaputa pikitia mārama o tētahi wāhi iti rawa o ngā pukahukahu, ā, ka māmā ake te tautuhi i ētahi mate pukahukahu.
• Whakamātautau VEGF-D : Tērā pea ka whakahaerehia e tō tākuta he whakamātautau toto hei tirotiro mō ngā mate pāwera, te pai o te mahi a ō whekau kē atu, me ō taumata o te pūmua e kiia nei ko te VEGF-D (Vascular Endothelial Growth Factor-D). He maha ngā tāngata whai LAM he nui ā rātou taumata o tēnei pūmua, engari kāore i te katoa.
• Ko te koiora pūkahukahu : Ko te tango i tētahi wahi iti o te kiko mai i te pūkahukahu ka whakamātautau i roto i te taiwhanga. Ka taea te tango i tēnei tauira mā te bronchoscopy (he tikanga e tukuna ai he ngongo me te kāmera ki roto i ngā pūkahukahu) mā te pokanga uma āwhina-ataata rānei (VATS).

Me pēhea te rongoā i te mate LAM?

Engari, kāore anō kia kitea he rongoā mō te LAM i tēnei wā . Heoi, ka taea e te rongoā sirolimus (e mōhiotia ana ko te rapamycin, te ingoa waitohu Rapamune®) te āwhina i te whakatau i te mate me te ārai i tōna kino haere. Ka taea e te Sirolimus te āwhina i te whakaiti i ngā pukupuku whatukuhu (angiomyolipomas), te whakaiti i ngā pukupuku me te hanga wai i roto i te pūnaha lymphatic, me te āwhina i te whakaiti i ō tohu.

Ka rite ki ētahi atu maimoatanga:

• Whakaora hāora : Mō te hunga e uaua ana ki te manawa.
• Ngā rongoā whakawhanui i te manawa : He rite ēnei ki ngā rongoā whakawhanui. Ka whakatuwherahia e rātou ngā ara manawa o roto i ngā pukahukahu, ā, ka māmā ake te manawa.
• Te whakaora i ngā pūkahukahu : He kaupapa tēnei e whakakotahi ana i te whakakori tinana, te mātauranga, me te tohutohu.
• Te whakawhitinga pukahukahu : Hei huarahi whakamutunga mō te hunga kua neke haere te mate ki te wāhanga tino kino.

Me ako anō hoki tātou mō ngā pānga taha me ngā raruraru o te maimoatanga.

I ētahi wā ka pāngia kinotia ngā whatukuhu e te Sirolimus, ā, ka piki ake te mōrearea o ngā mate urutā kino . Nō reira, he mea nui kia kōrerohia ngā painga me ngā huakore o te whakamahi i tēnei rongoā me tō tākuta i mua i te whakatau.

He aha te momo āpōpō ka taea e te tangata whai LAM te tumanako?

Ka kino haere pea te LAM i roto i te wā. Tērā pea ka rerekē tō mahere maimoatanga i runga i te maimoatanga e tika ana māu. Ka mahi tahi koe me tō tākuta manawa (takuta manawa) ki te whakatau i te huarahi pai hei aroturuki i tō āhua. Ka tirotirohia e ia te mahi a ō pukahukahu i ia wā. Ka ahu mai tō mahere maimoatanga i:

• tō tau.
• Tō āhua paheketanga (ahakoa kua pahure koe i te paheketanga, kāore rānei).
• Te tere o te hekenga o te mahi a ngā pukahukahu.
• Ahakoa kua pāngia ō whatukuhu, tō pūnaha lymphatic rānei.

Kia pēhea te tere o te horapa o te LAM?

Ka rerekē te tere o te horapa o te LAM i ia tangata, i ia tangata . He maha ngā āhuatanga ka pā ki tēnei:

• Te momo LAM kei a koe : Ahakoa he pai ake te mahi a ngā pukahukahu o te hunga whai `(TSC-LAM)` i ērā he `(Sporadic LAM)`, he rite tonu te tere o te hekenga o te mahi a ngā pukahukahu i ngā mea e rua.
• Tō tau : Mō ētahi tāngata, ka mutu te horapa o te LAM i muri i te paheketanga, ina heke te taumata o te estrogen i roto i te tinana.
• Te urupare ki te maimoatanga : Ka taea e te rongoā sirolimus te whakatau i te mate i roto i te tini o te tangata.

Āhea te wā e hiahiatia ai te whakawhitinga pukahukahu?

He tokomaha ngā tāngata whai LAM (tata ki te 64%) ka ora mō ngā tau 20, neke atu rānei i muri i te tātaritanga me te kore e hiahiatia he whakawhitinga pūkahukahu. Heoi, nā te mea kāore ngā pūtau LAM e tīmata i roto i ngā pūkahukahu, ka taea tonu e ngā kiore te whanake ahakoa kua whakawhitinga pūkahukahu .

He aha te roanga o te oranga mō te LAM? (Te roanga o te oranga)

Ko te roa o te oranga me te LAM e whakawhirinaki ana ki te kaha o te mate me te tere o tōna horapa. Engari, i te nuinga o te wā, kua pai ake te āhua i nāianei i tō mua. Neke atu i te 90% o te hunga whai LAM e ora ana i roto i ngā tau 10 i muri i te tautuhia.

Me pēhea taku tiaki i ahau anō?

Kōrero ki tō tākuta pūkahukahu (pūkahukahu) mō ngā mea hei tumanako atu, me pēhea hoki te whakarite mahere mō te noho tahi me te LAM. Tirohia tō tākuta i te wā tika, tangohia ō rongoā e ai ki te whakahaunga, ā, me kōrero tonu atu ki tō tākuta mēnā he tohu hou kei a koe, mēnā ka kino haere ō tohu, mēnā rānei he pānga taha kei a koe mai i tō maimoatanga.

Āhea koe me toro atu ki te tākuta? Āhea koe me haere ki te tiaki ohorere?

Mena he uaua ki te manawa, he maremare, he tohu rānei e whakararuraru ana i a koe, ahakoa ka roa, ka kino rānei, me toro atu ki te tākuta.

Āhea te wā e haere ai ki te Wāhanga Maimoatanga Ohotata (ETU) :

Mena kei a koe ngā tohu o te hinganga o te pukahukahu (pneumothorax) me ētahi atu tohu kino rānei, haere tonu ki te hōhipera:

• Mena he uaua ki te manawa.
• Mena he mamae nui tōu uma .
• Ki te maremare koe i te toto .
• Ki te kikorangi te kiri, ngā ngutu, me ngā maikuku (`(cyanosis)`) .

He aha ngā pātai nui hei pātai atu ki te tākuta?

He pai pea te pātai atu ki tō tākuta i ēnei pātai:

• Kei a au te `(Sporadic LAM)` me te `(TSC-LAM)` rānei?
• He aha ngā kōwhiringa maimoatanga kei a au?
• Me pēhea taku whakamahi i taku rongoā?
• He aha ngā tohu hou, ngā tohu kino rānei me tirotiro e au?
• Me whai whakawhitinga pukahukahu ahau?

He mate pukupuku te LAM?

Kāore te LAM i te kiia he mate pukupuku . Heoi, i ētahi wā ka rite tana whanonga ki te mate pukupuku e horapa ana mai i tētahi wāhi o te tinana ki tētahi atu (mate pukupuku metastatic). Ko te tikanga ka tīmata ngā pūtau i tētahi wāhi ka haere mā roto i ngā oko toto me te pūnaha lymphatic ki ngā pukahukahu me ngā whatukuhu. I ētahi wā ka kiia tēnei momo tipu ko te benign cell metastasis.

Engari kāore anō kia tino mārama ngā kaipūtaiao ki te tīmatanga o ēnei pūtau. Waihoki, te āhua nei kāore ēnei pukupuku e horapa whānuitia ki ētahi atu okana, pērā i te ate, te roro rānei, pērā i te mate pukupuku. Ina tirohia i raro i te karuārai, he rite ake te āhua o ēnei pūtau ki ngā pūtau noa i ngā pūtau mate pukupuku.

Nā ngā maimoatanga hou me te māramatanga ake ki te LAM, kua roa ake te ora o te tangata me te kore he whakawhitinga pūkahukahu. Engari he roa te wā e hiahiatia ana hei urutau ki tēnei momo noho hou. Tērā pea ka rite te ako mō tētahi mate mō te wā roa ki te mea kua tangohia te whenua i raro i ō waewae. Engari ka taea e tō rōpū hauora – me tō rōpū tautoko whaiaro – te āwhina i a koe kia tū pakari anō.

Hei whakamutunga, he aha ngā mea hei maumahara

He mate uaua pea te LAM, engari kāore koe i te mokemoke. Nā te whanaketanga o te pūtaiao hauora, he maha ngā huarahi hei whakahaere i tēnei mate. Ko te mea nui ko te noho pai, te whai i ngā tohutohu a tō tākuta, kaua hoki e mutu te tumanako. Kōrero ki tō tākuta me ō whanaunga mō ō āwangawanga me ō mataku. Kia maumahara, he maha ngā tāngata ka taea te āwhina i a koe i tēnei haerenga.

` LAM, mate pūkahukahu, mate onge, uauatanga manawa, sirolimus, whakawhitinga pūkahukahu, whakarerekētanga ira