A hming hi chhiar a harsa deuh a ni lawm ni? "Lymphangioleiomyomatosis" tih a ni. Harsatna a awm lo, LAM ti mai ila. Hei hi lung natna hmuh tur awm lo tak a ni a, hmeichhia a vei tam ber. Chutiang hming i hriat chuan hlauthawng chu hriatthiam theih a ni. Mahse, lungngai suh. He thuziak ah hian LAM chu eng nge a nih, engvangin nge a lo awm tih leh a enkawlna tur engte nge awm tih te chu awlsam tak leh friendly takin kan sawi dawn a ni.
LAM tih awmzia chu eng nge ni?
A awlsam zawngin, LAM chu i lung chhunga smooth muscle cell pangngai lo tak takte chu thunun theih loha an lo thang lian tan hian a ni. Tumor te te ang maiin. Heng cell te hi an inzawm khawm a, i lung chhungah bubble ang chi cyst an tih te te an siam thin. Hun a kal zel a, heng tumor te hi an lo thang lian zel chuan lung tissue hrisel tak tak a tichhia thei a, thawk pawh a ti harsa thei hle.
Hetiang dinhmun hi lung chhungah chauh a awm lo. A chang chuan kal leh lymphatic system-ah pawh tumor a lo awm thei bawk. Hei hi a chhan ber chu kan taksaa cell thang chakna control tu genes-a mutation a ni.
A bik takin he natna hi hmeichhia kum 20 leh 40 inkar, chu chu puitling leh menopause inkar a ni ber tih hriat reng a pawimawh hle. Chuvangin doctor-te chuan hormone estrogen hian inzawmna a nei niin an ngai.
LAM hi chi hnih lian tak a awm a.
LAM natna hi chi hnihah then theih a ni a, an pahnih inkarah hian danglamna tlem a awm.
| LAM chi hrang hrang | A sawifiahna awlsam tak |
|---|---|
| TSC-LAM a ni | Hei hi LAM chi khat a ni a, hmeichhia, tuberous sclerosis an tih, genetic condition nei te zingah a awm thin. Hei hian damdawi lam dinhmun dang pakhatah a lo awm tihna a ni. |
| Sporadic LAM a awm thin | Hetiang chi hi dam chhunga random genetic change (mutation) avanga lo awm a ni. Rochun a ni lo. Chuvangin he chi hi i neih chuan i fate’n natna an kai theih tur chungchangah i buai a ngai lo. |
LAM natna lan chhuah dan chu engte nge ni?
LAM natna lan chhuah dan hi lung natna tlanglawn dang, Asthma ang chi nen a inang em em a, a chang chuan a hriat theih nan hun rei tak a la ngai thei bawk. Hengte hi a lan chhuah dan berte an ni.
- Thâwk harsa (dyspnea): A tir lamah chuan hriat theih loh ni mah se, hun kal zelah he natna hi a pung thei a ni. Chutah chuan hla vak lo kal lai pawha chau tih te, stairs chhoh lai pawha thawk lak chhuah te pawh a tel thei.
- Wheezing: A rilru chhungril atanga thawm nem tak lo chhuak.
- Chhung na: A rilru na tak, na tak a awm thei.
- Khuh: Khuh hring (dry cough) awm reng.
- Thisen khuh emaw ‘chyle’ emaw: Chyle hi kan ei leh in atanga lo chhuak mucous fluid a ni. A rawng chu milky a ni.
Engvangin nge he LAM hi a lo awm? A chhan chu eng nge ni?
Kan sawi tawh ang khan hei hi genes inthlak danglam vang a ni. Kan taksaah hian gene chi hnih a awm a , chungte chu TSC1 leh TSC2 an ni . Chungte chu kan taksaa cell lo thang zel tur thununtu ‘guardian’ ang an ni. ‘Tumor suppressor’ gene an ti a. Chu chu heng genes te hna chu a tul lova cell te inthen darh a, tumor siam lo tura ven hi a ni.
LAM vei chuan heng gene zinga pakhat, TSC1 emaw TSC2 emaw-ah hian defect, a nih loh leh mutation a nei a. Tichuan chu ‘guard’ te chu an thawk tha lo. Chumi avang chuan smooth muscle cell te chu thunun theih lohvin an inthen tan a, a hmaa kan sawi tawh ang khan tumor chi hrang hrang an siam ta a ni.
- Lung cyst a awm thin
- Kidney tumor (angiomyolipomas) te hi a awm thei lo.
- Lymphatic system atanga natna hrik awm
He natna hian eng harsatna nge a thlen theih?
LAM natna hlauhawm ber leh hlauhawm ber chu lung tlak (pneumothorax) a ni. Lung chhunga bubble ang chi cyst pakhat chu a puak nghal a, chu chuan lung leh rilru bang inkar hmunah boruak a luang lut thei tih han mitthla teh. Hei hian lung a tikehsawm a ni. LAM vei hmeichhia zinga a chanve aia tam chuan an dam chhungin vawi khat tal he natna hi an tawk ang. Vawi tam tak chu LAM hi lung tlakchhiat a awm hian hriat hmasak ber a ni.
Harsatna dang pawh a awm thei:
- Lung vel (chylothorax) vel a tui ‘chylous’ a awm khawm .
- Lung chhehvela tui dang awmkhawm (pleural effusion) .
- Lymphatic system tihkhawtlai a ni
Engtin nge doctor hian LAM hi a hriat theih ang?
I natna lan chhuah dan a ngaithla a, a enfiah hnuah doctor-in he natna hi a rinhlelh chuan test eng emaw zat a ti ang a, a finfiah ang.
| Fiahna | Hei hi engtin nge i tih? |
|---|---|
| Lung hnathawh dan test a ni | I lung hnathawh that dan leh boruak i luh leh chhuah theih zat a teh a ni. |
| Pulse Oximetry hmanga tih a ni | I kut zungtang-ah clip ang chi thil an dah a, i thisena oxygen level an enfiah thin. |
| Imaging (scan) te chu . | CT scan , a bik takin HRCT (High-Resolution CT) scan chuan chu lung chhunga cyst te te chu chiang takin a hmu thei a ni. |
| VEGF-D thisen test a ni | VEGF-D hi protein a ni. He protein level hi LAM vei thisenah a sang tlangpui. Hei hian natna hriatchhuahna kawngah a \angkai hle. |
| Lung biopsy a ni | Test dangin a natna hi a finfiah theih loh chuan lung atanga tissue te tak te la chhuakin microscope hmangin an enfiah thin. Hei hi bronchoscopy emaw VATS emaw ang chi technique hmangin tih theih a ni . |
LAM enkawlna tur engte nge ni?
A hmasa berin LAM damdawi a la awm lo.Mahse, lungngai suh. Tunah chuan he natna hi thunun thei tur, a lan chhuah dan tihtlem thei tur leh a zual zel tur venna tur damdawi tha tak tak a awm tawh a ni.
Enkawlna ber chu sirolimus tih damdawi a ni . Rapamycin an ti bawk. He damdawi hian taksa peng hrang hrang (abnormal muscle cells) te tihpunna a titawp a, hna a thawk a ni. Hei hian natna chu a ti nghet thei a, kal leh lymphatic system-a tumor awmte a ti tlem thei bawk.
Chu bakah, i dinhmun a zirin i doctor chuan enkawlna dang a rawt thei bawk a, chungte chu:
- Oxygen therapy: Thisen a oxygen level a hniam chuan.
- Inhaled bronchodilators: Thâwk harsa tihziaawmna.
- Pulmonary rehabilitation: Lung chakna tichaktu exercise leh nunphung lama thurawn pek.
- Lung transplant: Natna a nasat em em a, enkawlna dang hmanga control theih loh a nih chuan a hnuhnung ber a ni.
Pawimawh: Sirolimus hian side effect a nei thei a, chu chu kal a tichhia a, natna kai theihna a tipung thei bawk. Chuvangin, he damdawi hi i tan hmain i doctor nen a thatna leh that lohna te sawi hmasa phawt ang che.
LAM nena kan awm dun huna hriat tur thil
LAM hi hun rei tak chhunga awm thei a ni. Chuvangin, i pulmonologist nen in thawhhona tha tak i neih a ngai dawn a ni. Natna kal zel dan, chu chu natna a kal chak dan hi mi hrang hrangah a inang lo. Thil engemaw zatah a innghat a ni:
- Eng chi LAM nge i neih tih ah a innghat.
- I kum (mi thenkhat tan chuan menopause hnuah natna hi a na zual tawh lo).
- I taksain enkawlna a dawn dan.
Lung transplant hi hlau suh. Mi tam zawk (64% vel) chuan an natna an hmuhchhuah atanga kum 20 emaw a aia rei emaw thlengin lung transplant an mamawh dawn lo. Enkawlna thar avang hian tunah chuan LAM vei te dam rei zawng a sang tawh hle. Mi 90% aia tam chu natna an hmuhchhuah atanga kum 10 hnuah pawh an la dam.
Damdawi lam thurawn lak hun tur
Hetiang natna nena kan khawsak hian i taksa enkawl hi a pawimawh hle.
| Heng symptoms te hi i neih chuan doctor rawn la: | |
| |
| Heng symptoms te hi i neih chuan ETU (Emergency Treatment Unit) ah kal nghal rawh: | |
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LAM hi cancer a ni em?
Hei hi mi tam tak tan chuan harsatna a ni. LAM hi cancer anga ngaih a ni tlangpui lo . Amaherawhchu, cancer ang chi mizia thenkhat a nei a. Entirnan, cell te hi taksa peng dang atanga lung leh kal thlengin a darh tawh niin a lang thei. Mahse, microscope hmanga en chuan heng cell te hi cancer cell angin an lang lo va, cell pangngai ang an ni zawk. Tin, thin emaw thluak ang chi taksa peng hrang hrangah an darh lo bawk.
Hetiang nun chhung zawnga natna i nei tih i hriat hian i hnuai atanga lei a hrual ang maia inhriat hi thil pangngai tak a ni. Mahse, nangmah chauh i ni lo. I doctor, chhungte, leh i ṭhiante ṭanpuinain he dinhmun hi i tuar chhuak thei a ni.
Take-Home Thuchah a ni
- LAM hi lung natna awm lo tak a ni a, hmeichhiaah a tam zawk.
- A lan chhuah dan ber chu thawk harsa, khuh, leh rilru na a ni.
- Hei hi i sual vang ni lovin, genetic mutation vang a ni.
- Damdawi famkim awm lo mah se, sirolimus ang chi damdawi hmangin natna hi a control tha hle.
- I thawkna lama mithiam nen inbiak reng tur a ni.
- Emergency-ah chuan rilru natna nasa tak emaw, thawk harsatna nasa tak emaw a nih chuan ETU-ah kal nghal tur a ni.
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