Une matambudziko aya here nemapapu ako? Ngatikurukurei nezve LAM (Lymphangioleiomyomatosis)

Wakambonzwa nezvechirwere ichi chine zita refu rinonzi LAM (lymphangioleiomyomatosis) here? Ungawana zita iri risinganzwisisike. Asi chirwere chisingawanzoitiki chinokanganisa mapapu. Nhasi, tichataura nezvazvo zviri nyore, nenzira yaunogona kunzwisisa. Usazvidya mwoyo, tichatsanangura zvese.

Chii chaizvo chinonzi LAM (Lymphangioleiomyomatosis)?

Zvichitaurwa zviri nyore, LAM chirwere chisingawanzoitiki chinoita kuti ma cysts azere nemvura aumbe mumapapu ako. Izvi zvinogona kukuvadza mapapu ako. Dzimwe nguva zvinogona kukonzera ma cysts muitsvo dzako uye lymphatic system. Fungidzira zvaizoitika kana masero emhasuru akatsetseka mumuviri wako akatanga kukura asingadzoreki. Ndizvo zvinoitika pano. Zvinokonzerwa nekuchinja kwemajini.

Dambudziko iri, rinonzi LAM (rinodudzwa kuti “limf-AN-gee-oh-ly-oh-my-oh-muh-TOH-sis”) , rinonyanya kubata vakadzi . Rinowanzo bata vakadzi vari pakati pemakore makumi maviri nemakumi mana, kana vakadzi vakapfuura makore ekuyaruka uye vava pedyo nekuguma kuenda kumwedzi. Nekuda kweizvi, vanachiremba vanofunga kuti hormone inonzi estrogen kana kuvapo kwechibereko zvinogona kunge zvine chekuita nekukura kwemabundu aya.

Ndedzipi mhando huru dzeLAM?

Kune mhando mbiri huru dzeLAM:

1. TSC-LAM: Vamwe vakadzi vane chirwere chemajini chinonzi ``Tuberous Sclerosis Complex - TSC''. Kana vakakura vachirapwa, chinonzi TSC-LAM.

2. Sporadic LAM: Izvi zvinoitika zvisina tsarukano, zvichireva kuti shanduko inoitika mujini chero nguva muhupenyu. Chinhu chakakosha ndechekuti rudzi urwu rweSporadic LAM harupfuurirwe kuvana vako.

Chirwere ichi chinonzi LAM chakajairika zvakadii?

Kurapwa kwemimba (LAM) chirwere chisingawanzoitiki . Zvinofungidzirwa kuti mukadzi mumwe chete pavakadzi 140,000 anogona kunge aine chirwere ichi. Zvisinei, pakati pe30% ne80% yevakadzi vane ``Tuberous Sclerosis`` vanogona kunge vaine chirwere ichi.

Ndezvipi zviratidzo zvechirwere cheLAM?

Munhu ane LAM anogona kuona zviratidzo zvakaita se:

• Kuoma kufema (dyspnea) : Dzimwe nguva izvi zvinogona kuwedzera zvishoma nezvishoma.
• Kufemereka kurira kwemuridzo kunobva pachipfuva .
• Kurwadziwa kwechipfuva .
• Kukosora .
• Dzimwe nguva ropa kana kuti chyle (`(chyle)` - lymph fluid inobva mudumbu) inobuda pamwe chete nemadzihwa .

Usafunge kuti une chirwere cheLam nekuda kwekuti une zviratidzo izvi, sezvo zvichigonawo kuva zviratidzo zvezvimwe zvirwere zvemapapu. Saka, zvakakosha kutsvaga rubatsiro rwechiremba.

Sei chirwere ichi chichiitika? Chii chinokonzera izvi?

Chikonzero chikuru cheLAM kuchinja kwemajini maviri mumuviri wedu anonzi `(TSC1)` uye `(TSC2)`. Majini maviri aya akafanana nemajini anodzvinyirira bundu anomisa zvinhu zvakaita semasero ekenza kukura zvisina kufanira mumuviri wedu. Saka, kana paine chikanganiso mune chero yemajini maviri aya, masero emhasuru akatsetseka andambotaura pakutanga anotanga kukura zvisingadzoreki. Nekuda kweizvozvo, izvi ndizvo:

• Mabundu emumapapu (pulmonary lymphangioleiomyomatosis) anokura.
• Mabundu (angiomyolipomas) anobuda muitsvo.
• Ma cysts anoumbwa mu lymphatic system.

Munhu anowana sei LAM?

Pane nzira mbiri dzinoita kuti munhu awane LAM. Imwe ndeyekugara nhaka gene risina kunaka kubva kumubereki ane dambudziko mu `(TSC1)` kana `(TSC2)` gene. Kugara nhaka uku kunoguma nechirwere che`(Tuberous Sclerosis)`, uye pamwe chete nacho kunouya nenjodzi yekubatwa neLAM. Vakadzi vanopfuura 30% vane `(Tuberous Sclerosis)` vane LAM.

Imwe nzira ndeyekuti kuchinja kwejini rekuti `(TSC2)` kunoitika kwega, pasina hukama hwenhaka. Chikonzero chaicho cheizvi hachizivikanwe. LAM inokura nenzira iyi inonzi Sporadic LAM. Muchiitiko ichi, zvimwe zviratidzo zve`(Tuberous Sclerosis)` hazvionekwe.

Ndiani ari panjodzi huru yekubatwa nechirwere cheLAM?

Kunyangwe izvi zvichishamisa, LAM inowanzoonekwa kuvakadzi . Varume vashoma chete ndivo vakaonekwa vaine LAM kusvika pari zvino. Murume mumwe chete ndiye anonzi ane LAM inokonzereswa neapo neapo.

Uyewo, LAM inogona kurwadza zvakanyanya panguva yekuzvitakura uye kana uchishandisa mishonga ine estrogen .

Ndezvipi zvinetso zvinogona kuitika nekuda kweLAM?

Dambudziko rinowanzoitika reLAM ipneumothorax . Sekunge bharumu rinoputika, mweya unobuda mumapapu woita kuti mapapu aputsike. Vakadzi vanopfuura hafu vane LAM vanozove nechirwere chepneumothorax kamwe chete muhupenyu hwavo. Dzimwe nguva izvi zvinogona kuitika kakawanda. LAM inowanzoonekwa mushure mechiitiko ichi.

Mamwe matambudziko anosanganisira:

• Kuunganidzwa kwemvura inonzi chyle yakakomberedza mapapu (chylothorax).
• Kuungana kwemvura yakakomberedza mapapu (pleural effusion).
• Kuvharirwa kwehurongwa hwe lymphatic.

Chirwere cheLAM chinoonekwa sei?

Kuti chiremba aongorore chirwere cheLAM, anotanga akuongorora uye obvunza nezvezviratidzo zvako. Vachabva varaira bvunzo nemifananidzo kuti vaone zvinotevera:

• Ona kuti mapapu ako ari kushanda zvakanaka sei.
• Tarisa huwandu hweokisijeni muropa.
• Tarisa kana paine shanduko mumapapu.
• Tarisa kana muropa muine huwandu hunopfuura hwakajairika hwe "mapuroteni" mamwe.

Nekuti zviratidzo zveLAM zvakafanana nezvezvimwe zvirwere zvemapapu zvakajairika, dzimwe nguva zvinogona kuomera vanachiremba kuzviongorora.

Ndezvipi zviedzo zvinoshandiswa kuongorora LAM?

Ungangoda kuita bvunzo dzakadai sedzinotevera:

• Kuongororwa kwebasa remapapu : Izvi zvinotarisa kuti mapapu ako anokubatsira sei kufema nekubuda.
• Kuongorora mwero weokisijeni muropa (`(pulse oximetry)`) : Muedzo uri nyore unosanganisira kubatanidza chimwe chinhu chakaita sechipikiri pamunwe wako.
• Miedzo yekufungidzira : Izvi zvinosanganisira CT scans (Computed Tomography scans), MRI (Magnetic Resonance Imaging), kana HRCT (High-Resolution Computed Tomography). HRCT scan inogona kuburitsa mufananidzo wakajeka wenzvimbo diki yemapapu, zvichiita kuti zvive nyore kuona zvimwe zvirwere zvemapapu.
• Kuongororwa kweVEGF-D : Chiremba wako anogona kukuongororwa ropa kuti aone kana paine hutachiona, kuti mimwe mitezo yako iri kushanda zvakanaka sei, uye huwandu hwepuroteni yako inonzi VEGF-D (Vascular Endothelial Growth Factor-D). Vanhu vazhinji vane LAM vane huwandu hwakawanda hwepuroteni iyi, asi havasi vese vanozviita.
• Kuongororwa kwemapapu : Izvi zvinosanganisira kutora chidimbu chidiki chenyama kubva mumapapu wochiyedza murabhoritari. Muenzaniso uyu unogona kutorwa ne bronchoscopy (nzira iyo chubhu ine kamera inopinzwa mumapapu) kana nekuvhiyiwa kwechipfuva nevhidhiyo (VATS).

Chirwere cheLAM chinorapwa sei?

Zvinosuwisa kuti pari zvino hapana mushonga weLAM. Zvisinei, mushonga we sirolimus (unozivikanwawo se rapamycin, zita rekuti Rapamune®) unogona kubatsira kugadzirisa chirwere ichi nekudzivirira kuti chisanyanya kuipa. Sirolimus inogona kubatsira kuderedza bundu reitsvo (angiomyolipomas), kuderedza bundu uye kuungana kwemvura mu lymphatic system, uye kubatsira kuderedza zviratidzo zvako.

Semamwe marapirwo:

• Kurapa neokisijeni : Kune avo vanonetseka nekufema.
• Zvinofema bronchodilators : Izvi zvakafanana nezvifema zvinofema. Zvinovhura nzira dzekufema mumapapu uye zvinoita kuti zvive nyore kufema.
• Kurapwa kwemapapu : Ichi chirongwa chinosanganisa kurovedza muviri, dzidzo, uye mazano.
• Kuisirwa mapapu : Senzira yekupedzisira kune avo vane chirwere chasvika padanho repamusoro.

Ngatidzidzeiwo nezvemigumisiro yekurapwa uye matambudziko anokonzerwa nekurapwa.

Sirolimus dzimwe nguva inogona kukonzera kukuvara kwakanyanya kweitsvo uye kuwedzera njodzi yezvirwere zvakakomba . Saka, zvakakosha zvikuru kukurukura zvakanakira nezvakaipa zvekushandisa mushonga uyu nachiremba wako usati wasarudza.

Munhu ane chirwere che LAM angatarisira remangwana rerudzii?

Kurapwa kweLam kunogona kuipa nekufamba kwenguva. Hurongwa hwenyu hwekurapa hunogona kuchinja zvichienderana nekuti ndeipi nzira yekurapa yakakunakirai. Muchashanda nachiremba wemapapu enyu (pulmonologist) kuti vaone nzira yakanakisisa yekutarisa mamiriro enyu ezvinhu. Iye achagara achiongorora mashandiro emapapu enyu. Hurongwa hwenyu hwekurapa huchabva pane zvinotevera:

• zera rako.
• Mamiriro ako ekusvika panguva yekuenda kumwedzi (ingave wakambosvika panguva yekuenda kumwedzi kana kuti kwete).
• Kukurumidza kudzikira kwebasa remapapu.
• Ingave itsvo dzako kana lymphatic system zvakakanganiswa.

Kupararira kwechirwere cheLam kunokurumidza sei?

Kukurumidza kupararira kweLAM kunosiyana kubva pamunhu kuenda kumunhu . Pane zvinhu zvakawanda zvinogona kukanganisa izvi:

• Rudzi rweLAM yauinayo : Kunyange hazvo vanhu vane `(TSC-LAM)` vaine mashandiro akanaka emapapu pane avo vane `(Sporadic LAM)`, mwero wekuderera kwebasa remapapu wakafanana mune ese ari maviri.
• Zera rako : Kune vamwe vanhu, LAM inomira kupararira mushure mekuguma kuenda kumwedzi, apo huwandu hweestrogen mumuviri hunoderera.
• Maitiro aunoita kana warapwa : Mushonga weSirolimus unogona kugadzirisa chirwere ichi muvanhu vazhinji.

Kuisirwa mapapu kunodiwa rini?

Vanhu vazhinji vane LAM (inenge 64%) vanogona kurarama makore makumi maviri kana kupfuura mushure mekuonekwa kuti vane chirwere ichi vasingade kuisirwa mapapu. Zvisinei, nekuda kwekuti masero eLAM haatangi mumapapu, ma cysts anogona kukura kunyangwe mushure mekuisirwa mapapu .

Chii chinotarisirwa kurarama neLAM? (Hupenyu Hunotarisirwa)

Nguva yaunogona kurarama nayo nechirwere cheLAM inoenderana nekuoma kwechirwere ichi uye kuti chinokurumidza kupararira sei. Asi, zvakazara, mamiriro acho ari nani zvikuru ikozvino kupfuura kare. Vanhu vanopfuura 90% vane chirwere cheLAM vanorarama makore gumi mushure mekuonekwa kuti vane chirwere ichi.

Ndinozvitarisira sei?

Taura nenyanzvi yemapapu nezvezvaunotarisira uye maitiro ekugadzira hurongwa hwekurarama neLAM. Ona chiremba wako nenguva, tora mishonga yako sezvawakarairwa, uye taurira chiremba wako nekukasika kana uine zviratidzo zvitsva, kana zviratidzo zvako zvikawedzera, kana kana uine migumisiro yakaipa kubva mukurapwa kwako.

Unofanira kuona chiremba riini? Unofanira kuenda kuchipatara riini?

Kana uchinetseka kufema, kukosora, kana zvimwe zviratidzo zvinonetsa, zvingave zvichiripo kana kuti zvakanyanya kuipa, iva nechokwadi chekuona chiremba.

Nguva yekuenda kuEmergency Treatment Unit (ETU) :

Kana uine zviratidzo zvekudonha kwemapapu (pneumothorax) kana zvimwe zviratidzo zvakakomba, enda kuchipatara nekukasika:

• Kana uchinetseka kufema.
• Kana uchirwadziwa zvakanyanya pachipfuva .
• Kana ukakosora ropa .
• Kana ganda, miromo, nenzara zvikashanduka kuita bhuruu (`(cyanosis)`) .

Ndeipi mibvunzo inokosha yekubvunza chiremba?

Zvingabatsira kubvunza chiremba wako mibvunzo yakaita seiyi:

• Ndine `(Sporadic LAM)` kana `(TSC-LAM)` here?
• Ndedzipi nzira dzekurapa dzandinadzo?
• Ndinofanira kushandisa sei mushonga wangu?
• Ndezvipi zviratidzo zvitsva kana kuti zvinotowedzera kuipa zvandinofanira kutarisa?
• Ndichada kuisirwa mapapu here?

Kurapwa kwegomarara (LAM) igomarara here?

LAM haiwanzoonekwa sekenza . Zvisinei, mune dzimwe nzira inoita sekenza inopararira kubva kune imwe nzvimbo mumuviri kuenda kune imwe (kenza inopararira). Izvi zvinoreva kuti masero anotangira panzvimbo imwe uye anofamba nemutsinga dzeropa ne lymphatic system kuenda kumapapu neitsvo. Rudzi urwu rwekukura dzimwe nguva runonzi benign cell metastasis.

Asi masayendisiti haasati aziva kuti masero aya anotangira kupi. Uyewo, bundu iri hariratidzike serinopararira zvakanyanya kune dzimwe nhengo, dzakadai sechiropa kana uropi, senge kenza. Kana richitariswa nemaikorosikopu, masero aya anotaridzika semasero akajairwa kupfuura masero ekenza.

Nekuda kwekurapwa kutsva uye kunzwisisa kwakawanda kweLAM, vanhu vava kurarama kwenguva yakareba vasina kuisirwa mapapu. Asi zvinotora nguva kuti vajaire mararamiro matsva aya. Kudzidza nezvechirwere chehupenyu hwese kunogona kunzwa sekunge ivhu rabviswa pasi petsoka dzako. Asi timu yako yezvekurapa - nechikwata chako chekutsigira - vanogona kukubatsira kuti umire zvakare.

Pakupedzisira, zvekurangarira

LAM inogona kuva chirwere chinonetsa, asi hausi wega. Nekufambira mberi mune zvesainzi yekurapa, kune nzira dzakawanda dzekugadzirisa chirwere ichi. Chinhu chakakosha ndechekugara uine tariro, kutevedzera zano rachiremba wako, uye kusarasa tariro. Taura nachiremba wako nevadikanwi vako nezvezvinokunetsa nezvaunotya. Yeuka, kune vanhu vazhinji vanogona kukubatsira parwendo urwu.

` LAM, chirwere chemapapu, chirwere chisingawanzoitiki, kuoma kufema, sirolimus, kuisirwa mapapu, kushanduka kwemajini