Hmeichhia bik natna vei LAM chungchang hi kan sawi dawn em ni? (Lymphangioleiomyomatosis) tih hi a awm a.

A châng chuan thawk harsa i ti em, i chau em, i rilru a na em? Hengte hi natna tlanglawn tak, asthma ang chi chhinchhiahna angin kan ngai fo thin. Amaherawhchu, lung natna a awm lo hle a, chu chuan heng symptoms te hi a thlen a, a bik takin hmeichhiaah hian a awm a ni. Lymphangioleiomyomatosis an ti a. A hming hi a sei deuh a, a tawi zawngin "LAM" kan ti ang. He hming hi lam harsa i tih chuan hetiang hian han ngaihtuah teh: "Lymph-ang-ge-o-ly-o-myo-ma-to-sis". Tlem a buaithlak viau nachungin, he natna hi a awlsam zawnga hriatthiam a pawimawh hle thei a ni.

A awlsam zawngin LAM chu eng nge ni?

LAM hi thil awm lo tak a ni a, i lung chhungah cell pangngai lo a lo thang lian a, cyst te te a siam thin. Hei hi genetic mutations avanga lo awm a ni a, chu chuan i taksaa smooth muscle cells te chu thunun theih lohvin a tipung a ni. Hei hian i lung a tichhe thei a ni. Lung chauh ni lovin, a chang chuan kal leh lymphatic system pawhin heng thanna te hi a siam thei a ni.

He natna hi hmeichhia kum 20 leh 40 inkar ah hmuhchhuah a ni ber a, chu chu puitlin hnu leh menopause hmain a ni. Chuvangin Doctor te chuan hmeichhe hormone estrogen hian he natna lo piang tur hian hmun a chang niin an ngai.

LAM natna hi chi hnih a awm a.

LAM natna hi chi hnihah then theih a ni a, an pahnih inkarah hian danglamna tlem a awm.

LAM natna lan chhuah dan chu engte nge ni?

LAM natna lan chhuah dan hi lung natna dang, asthma leh bronchitis te nen a inang hle. Hei hi a chang chuan hriatchhuah nan hun rei tak a la ngai thei. Hei hi hriat chian a pawimawh hle.

• Thâwk harsa (dyspnea): Hei hi a lan chhuah dan ber leh langsar ber a ni. A tir lamah chuan thawhrimna tlemte chauh hmanga hriat theih ni mah se, hun kal zelah he dinhmun hi a zual thei hle.
• Wheezing: Thâwk chhuah laiin whistling ri a ni a, asthma vei nen a inang.
• Chest pain: He natna hi a bik takin thawk lak hian hriat theih a ni.
• Khuh: Khuh hring (persistent dry cough) a awm thei.
• Thisen khuh emaw ‘chyle’ emaw: Chyle hi kan ei leh in atanga lo chhuak mucous fluid a ni. A rawng chu milky a ni. Hetiang thil hi a thleng a nih chuan tihkhawtlai loh a tha zawk .

Engvangin nge he LAM hi a lo awm? Eng nge a chhan?

A awlsam zawngin sawi ila, hei hi a chhan ber chu kan taksaa gene pahnih TSC1 leh TSC2 -a inthlak danglamna (mutations) vang a ni. Heng gene pahnih hi kan taksa vengtu ang mai an ni. An hna ber chu cell thenkhat chu thunun theih lohvin a tul lovah an inthen darh leh an pung zel tur tihtawp hi a ni. Hengte hi tumor suppressor genes kan ti a.

Hêng vênhimtu gene pahnih zînga pakhatah hian chhiatna a awm a nih chuan han ngaihtuah teh. Thil thleng chu smooth muscle cells te hian "Okay, enough, stop dividing" tih signal an hmu lo. Chuvangin cell te chu thunun theih lohvin an inthen tan ta a ni. Chumi rah chhuah chu:

• Lung chhungah hian cyst a lo awm thin.
• Kidney-ah hian tumor (angiomyolipomas) a lo awm a.
• Lymphatic system ah hian cyst a lo awm thin.

He gene dik lo hi i nu leh pa (tuberous sclerosis thlentu) hnen atangin i rochun thei a, a nih loh leh i dam chhungin chhan hriat lohvin heng genes-ah hian random takin inthlak danglamna i nei thei bawk (chu chuan sporadic LAM a thlen thin).

LAM natna avanga harsatna leh hlauhawmna

LAM natna vei tam ber leh lian ber chu lung tlakchham hi a ni . Damdawi lam hawi chuan hei hi pneumothorax .LAM vei hmeichhia zinga a chanve aia tam chuan an dam chhungin vawi khat tal he natna hi an tawk ang. A châng chuan a lo lang leh thei bawk. Dik tak chuan hmeichhe tam tak chu lungphun (lung abscess) an neih hnuah LAM an vei tih hmuhchhuah hmasak ber an ni a, damdawi ina dah an ni.

Tin, harsatna dang pawh a awm thei bawk.

LAM natna hi engtin nge an hriat theih?

I natna lan chhuah dan a ngaihthlak hnuah i doctor-in he natna hi a rinhlelh chuan test eng emaw zat a ti ang a, chu chu finfiah turin a ti ang.

Doctor-in test a tih te

• Lung Function Tests: Hei hi machine nena inzawm tube hmanga thawk chhuah leh chhuah a ni. Hei hian i lung a hnathawh that dan leh boruak a chelh theih zat a teh thei a ni.
• Pulse Oximetry: Thil te tak te, clip ang chi hi i kut zungtang-ah bun a ni a, i thisena oxygen awm zat a teh thin. Hei hi test awlsam tak leh hrehawm lo tak a ni.
• Imaging Scan te hi:
• CT Scan (Computed Tomography) hmanga tihchhuah a ni.Hei hian i lungphu 3D image chipchiar tak a la thei a ni. Hei hian LAM tana bik cyst a awm leh awm loh chiang taka hriat theih nan a pui thei a ni.
• HRCT scan (High-Resolution Computed Tomography): Hei hi CT scan hmasawn tak a ni. Lung hmun tlemte tak tak thlalak chiang tak tak a la thei. Hei hi LAM diagnose atan a pawimawh hle.
• VEGF-D thisen test: VEGF-D hi kan thisena protein awm a ni. LAM vei tam tak chuan an thisenah he protein hi a tlangpui aiin a sang zawk hle. Chuvangin he thisen test hian natna hriatchhuahna kawngah a pui hle a ni.
• Lung Biopsy: A chang chuan test dang 100% a chiang lo a nih chuan i doctor chuan i lung atanga tissue te tak te tak te chu test nan lak chhuah a tum mai thei. Hei hi bronchoscopy (tube te tak te, thawkna kawngah camera dah) emaw VATS (video-assisted thoracic surgery) emaw hmanga tih theih a ni.

LAM natna enkawl dan tur engte nge ni?

A hmasa berin LAM damdawi a la awm lo. Mahse, lungngai suh. Tunah chuan natna thunun thei, natna lanchhuahna tihziaawm thei, natna zual zel tur tikhawtlai thei damdawi tha tak tak a awm ta.

Enkawlna ber chu sirolimus tih damdawi a ni . Rapamycin an ti bawk. He damdawi hian control theih loha cell lo thang lian chu a titawp a ni. Hei hian natna a zual zelna tur chu nasa takin a thunun thei a ni. Tin, kal leh lymphatic system-a tumor awmte a tihtlem bakah symptoms a tihziaawm thei bawk.

Chu bâkah, i doctor chuan i dinhmun a zirin enkawlna dang a rawt thei bawk.

• Oxygen Therapy: Thisen a oxygen level a hniam chuan in lama hman theih turin oxygen tank siam a ni.
• Inhaled bronchodilators: Heng damdawi, inhaler hmanga pek hian thawk harsatna a tiziaawm thei a ni.
• Pulmonary Rehabilitation: Hei hi lung tan exercise program bik ang mai a ni. Thâwk lakna exercise leh taksa tana ṭha exercise hmangin i nunphung tihchangtlun a ṭanpui a ni.
• Lung Transplant tih hi: 1.1.Natna hi a zual hle a, enkawlna dang hmanga thunun theih a nih loh chuan lung thlak hi duhthlan tur hnuhnung ber a ni thei.

Engtiang chiahin nge natna hi a lo zual zel?

Hei hi mi tin a inang lo. LAM hmasawnna rate hi thil engemaw zatah a innghat a ni.

• LAM i neih chi hrang hrang: Sporadic LAM nei te hian TSC-LAM nei te aiin lung function an nei tlem zawk thei.
• I kum: Hmeichhe thenkhat tan chuan menopause hnuah taksaa estrogen level a tlahniam chuan natna zual zel chu a tawp emaw, a tlahniam emaw a ni.
• Enkawlna i dawn dan: Mi tam tak chuan Sirolimus ang damdawi hmangin natna hi an control tha thei hle.

Tun hma aiin tunah chuan dinhmun a tha zawk hle. Tunlai enkawlna hmang hian LAM vei zinga 90% aia tam chu an hmuhchhuah atanga kum 10 hnuah an dam leh thin. Mi tam tak (64% vel) chu lung transplant ngai lovin kum 20 emaw a aia rei emaw an dam thei a ni.

Engtikah nge i doctor hmuh a, ETU-a kal tur

LAM nena kan khawsak hian engtikah nge damdawi lam thurawn lak tur tih leh engtikah nge Emergency Department (ETU) pan tur tih hriat a pawimawh hle.

Mi thenkhat chuan LAM hi cancer a ni em tih an zawt thin. LAM hi cancer anga ngaih a ni tlangpui lo. Mahse, kawng engemaw takah chuan, hmun danga cell-te chu lungah an lo kal dan ang chiah hian cancer ang chi mizia an nei a ni. Mahse, microscope hmanga en chuan heng cell te hi cancer cell angin an lang lo.

Hetiang natna nunna atana hlauhawm i nei tih i hriat hian mangang leh hlauhna i neih hi thil pangngai a ni. Mahse, nangmah chauh i ni lo tih hre reng ang che. I doctor-te, i chhungte leh i ṭhiante ṭanpuinain, he harsatna hmachhawn thei tur hian chakna i hmu ang.