Tingataure nezve LAM, chirwere chinonyanya kubata vakadzi here? (Lymphangioleiomyomatosis)

Dzimwe nguva unonzwa kushaya mweya, kuneta, kana kurwadziwa pachipfuva here? Tinowanzo funga nezveizvi sezviratidzo zvechirwere chinowanzoitika senge asthma. Zvisinei, pane chirwere chemapapu chisingawanzoitiki chinokonzera zviratidzo izvi, kunyanya kuvakadzi. Chinonzi Lymphangioleiomyomatosis. Zita racho rakareba zvishoma, saka ticharidaidza kuti "LAM" muchidimbu. Kana ukaona zita iri rakaoma kutaura, funga nezvaro seizvi: "Lymph-ang-ge-o-ly-o-myo-ma-to-sis". Kunyangwe zvakaoma zvishoma, zvinogona kuve zvakakosha kuti unzwisise chirwere ichi nenzira iri nyore.

Zvichitaurwa zviri nyore, chii chinonzi LAM?

LAM chirwere chisingawanzoitiki apo masero asina kujairika anokura mumapapu ako, achigadzira ma cysts madiki. Izvi zvinokonzerwa nekuchinja kwemajini kunoita kuti masero emhasuru dzakatsetseka mumuviri wako akure asingadzoreki. Izvi zvinogona kukuvadza mapapu ako. Kwete mapapu chete, asi dzimwe nguva itsvo ne lymphatic system zvinogonawo kukura izvi.

Chirwere ichi chinowanzoonekwa mumadzimai ari pakati pemakore makumi maviri nemakumi mana. Kureva kuti, mushure mekuyaruka uye vasati vasvika pakuguma kuenda kumwedzi. Saka, vanachiremba vanotenda kuti hormone yechikadzi inonzi estrogen ine basa guru mukukura kwechirwere ichi.

Kune mhando mbiri huru dzechirwere cheLAM.

Chirwere cheLAM chinogona kukamurwa kuita mhando mbiri huru, nemusiyano mudiki pakati padzo.

Ndezvipi zviratidzo zvechirwere cheLAM?

Zviratidzo zveLAM zvakafanana zvikuru nezvimwe zvirwere zvemapapu, zvakaita seasthma nebronchitis. Izvi zvinogona kutora nguva kuti zviongororwe. Zvakakosha kuziva izvi.

• Kuoma kufema (dyspnea): Ichi ndicho chiratidzo chikuru uye chinowanzoonekwa. Kunyangwe pakutanga chingangonzwika nekushanda nesimba zvishoma, chirwere ichi chinogona kuipa nekufamba kwenguva.
• Kufema: Kurira kwemuridzo kana uchifema, kwakafanana nemunhu ane asthma.
• Kurwadziwa pachipfuva: Kurwadziwa uku kunogona kunzwika kunyanya kana uchifema zvakadzika.
• Kukosora: Panogona kunge paine kukosora kwakaoma kunogara kwenguva refu.
• Kukosora ropa kana kuti 'chyle': Chyle imvura inobuda mudumbu inobva mudumbu redu. Ine ruvara rwakaita semukaka. Kana chimwe chinhu chakadai chikaitika, zviri nani kusanonoka zvachose .

Sei chirwere ichi chichiitika? Chii chinokonzera izvi?

Zvichitaurwa zviri nyore, chikonzero chikuru cheizvi kuchinja (kuchinja) kunoitika mumajini maviri mumuviri wedu anonzi TSC1 naTSC2 . Majini maviri aya akafanana nevarindi vemuviri wedu. Basa rawo guru nderekumisa mamwe masero kuti asapatsanuke uye awedzere zvisina kufanira pasina kudzora. Tinodaidza majini aya kuti adzivise bundu.

Fungidzira kana paine dambudziko mune rimwe remajini maviri aya anodzivirira. Chinoitika ndechekuti masero emhasuru akatsetseka haawane chiratidzo chekuti, "Zvakanaka, zvakwana, rega kupatsanurana." Saka masero anotanga kupatsanurana zvisingadzoreki. Mhedzisiro yacho ndeiyi:

• Matumbu anoumbwa mumapapu.
• Mabundu (angiomyolipomas) anobuda muitsvo.
• Ma cysts anoumbwa mu lymphatic system.

Unogona kugara nhaka jini iri risina kunaka kubva kuvabereki vako (rinokonzera tuberous sclerosis), kana kuti unogona kuchinja majini aya zvisina kurongeka muhupenyu hwako pasina chikonzero chakajeka (rinokonzera LAM isingawanzoitiki).

Matambudziko nenjodzi dzechirwere cheLAM

Dambudziko rinowanzoitika reLAM mapapu akadonha , anozivikanwa sepneumothorax .Vakadzi vanopfuura hafu vane LAM vachasangana nedambudziko iri kanenge kamwe chete muhupenyu hwavo. Dzimwe nguva rinogona kudzoka. Kutaura zvazviri, vakadzi vazhinji vanotanga kuonekwa vaine LAM mushure mekunge vabatwa nechirwere chemapapu voendeswa kuchipatara.

Pamusoro pezvo, mamwe matambudziko anogona kuitika.

Chirwere cheLAM chinoonekwa sei?

Mushure mekuteerera zviratidzo zvako, kana chiremba wako achifungidzira kuti une chirwere ichi, achakuraira bvunzo dzinoverengeka kuti asimbise.

Miedzo inoitwa nachiremba

• Kuongororwa Kwekushanda Kwemapapu: Izvi zvinosanganisira kufema nekubuda nepachubhu yakabatana nemuchina. Izvi zvinogona kuyera kuti mapapu ako ari kushanda zvakanaka sei uye kuti anogona kuchengeta mweya wakawanda sei.
• Pulse Oximetry: Chishandiso chidiki, chakafanana neclip, chinosungirirwa pamunwe wako uye chinoyera huwandu hweoxygen muropa rako. Iyi bvunzo iri nyore kwazvo uye isingarwadzi.
• Kudhirowa Mifananidzo:
• Kuongororwa kweCT (Computed Tomography):Izvi zvinogona kutora mifananidzo yakadzama yemapapu ako mu3D. Izvi zvinogona kubatsira kuona zvakajeka kana paine ma cysts anoenderana neLAM.
• HRCT scan (High-Resolution Computed Tomography): Iyi imhando yepamusoro yeCT scan. Inogona kutora mifananidzo yakajeka yechikamu chidiki chemapapu. Izvi zvakakosha zvikuru pakuongorora LAM.
• Kuongororwa kweropa kweVEGF-D: VEGF-D ipuroteni inowanikwa muropa redu. Vanhu vazhinji vane LAM vane huwandu hwakanyanya hwepuroteni iyi muropa ravo kupfuura zvakajairwa. Saka kuongororwa kweropa uku kunobatsira zvikuru pakuongorora chirwere ichi.
• Kuongororwa kweMapapu: Dzimwe nguva, kana mimwe miedzo isina chokwadi chakazara, chiremba wako anogona kusarudza kutora kanyama kadiki kubva mumapapu ako kuti kaongororwe. Izvi zvinogona kuitwa uchishandisa nzira inonzi bronchoscopy (chubhu diki ine kamera yakaiswa mumhepo) kana VATS (video-assisted thoracic surgery) .

Ndeipi mishonga yekurapa chirwere cheLAM?

Chekutanga, hapana mushonga weLAM parizvino. Asi musazvidya moyo. Iye zvino kune mishonga inoshanda zvikuru inogona kudzora chirwere, kuderedza zviratidzo, uye kunonotsa kufambira mberi kwechirwere.

Mushonga mukuru unonzi sirolimus . Unonziwo rapamycin. Mushonga uyu unoshanda nekumisa kukura kwemasero anokura asingadzoreki. Izvi zvinogona kubatsira kudzora kuwedzera kwechirwere zvakanyanya. Unogonawo kubatsira kuderedza bundu muitsvo nemu lymphatic system uye kuderedza zviratidzo.

Pamusoro pezvo, chiremba wako anogona kukurudzira mamwe marapirwo zvichienderana nemamiriro ako ezvinhu.

• Kurapa Okisijeni: Kana huwandu hweokisijeni muropa hwakaderera, matangi eokisijeni anopihwa kuti ashandiswe kumba.
• Mishonga inofema ine bronchodilators: Mishonga iyi, inopihwa kuburikidza neinhaler, inogona kuderedza kuoma kwekufema.
• Kugadziriswa kweMapupa: Izvi zvakafanana nechirongwa chekurovedza muviri chemapapu. Zvinobatsira kuvandudza hupenyu hwako kuburikidza nekurovedza muviri kwekufema uye kurovedza muviri kwakanaka.
• Kuisirwa Mapapu:Kana chirwere chacho chakura zvakanyanya uye chisingagoni kudzorwa neimwe nzira yekurapa, kuisirwa mapapu kunogona kuva sarudzo yekupedzisira.

Chirwere ichi chinokurumidza kukura zvakadii?

Izvi zvinosiyana kubva pamunhu nemunhu. Kufambira mberi kweLAM kunoenderana nezvinhu zvakati wandei.

• Rudzi rweLAM yauinayo: Vanhu vane LAM isingawanzoitiki vanogona kunge vaine mashandiro emapapu asina kusimba zvishoma pane avo vane TSC-LAM.
• Zera rako: Kune vamwe vakadzi, mushure mekuguma kuenda kumwedzi, kufambira mberi kwechirwere kunomira kana kunonoka sezvo huwandu hweestrogen mumuviri huchidzikira.
• Mhinduro yako pakurapwa: Vanhu vazhinji vanogona kudzora chirwere ichi zvakanaka nemishonga yakaita seSirolimus.

Mamiriro ezvinhu ari nani zvikuru iye zvino kupfuura kare. Nekurapa kwemazuva ano, vanhu vanopfuura 90% vane LAM vanorarama makore gumi mushure mekuonekwa vaine chirwere ichi. Vanhu vazhinji (vanenge 64%) vanogona kurarama makore makumi maviri kana kupfuura vasingadi kuisirwa mapapu.

Nguva yekuona chiremba wako uye kuenda kuETU

Kana uchigara nechirwere cheLAM, zvakakosha kuziva nguva yekutsvaga mazano ekurapa uye nguva yekuenda kuEmergency Department (ETU).

Vamwe vanhu vanobvunza kana LAM iri kenza. LAM haiwanzoonekwa sekenza. Zvisinei, mune dzimwe nzira, dzakadai sekuti masero anobva kune imwe nzvimbo kuenda kumapapu, ane hunhu hwakafanana nekenza. Zvisinei, kana akatariswa nemaikorosikopu, masero aya haaratidzike semasero ekenza.

Zvakajairika kunzwa kushamisika uye kutya paunoziva kuti une chirwere chinouraya chakadai. Asi yeuka, hausi wega. Nerutsigiro rwevanachiremba vako, mhuri yako, neshamwari dzako, uchawana simba rekutarisana nedambudziko iri.