Leli gama kunzima ukulifunda, akunjalo? "Lymphangioleiomyomatosis". Akunankinga, ake silibize ngokuthi i-LAM . Lesi yisifo samaphaphu esingavamile kakhulu esithinta kakhulu abesifazane. Kuyaqondakala ukwesaba uma uzwa igama elinjalo. Kodwa ungakhathazeki. Kulesi sihloko, sizoxoxa ngokuthi iyini i-LAM, ukuthi kungani yenzeka, nokuthi yiziphi izindlela zokwelapha ezitholakalayo zayo ngendlela elula kakhulu nenobungane.
Kusho ukuthini nje ukuthi i-LAM?
Kalula nje, i-LAM yilapho amangqamuzana emisipha abushelelezi angajwayelekile ngaphakathi kwamaphaphu akho eqala ukukhula ngaphandle kokulawulwa. Njengezimila ezincane. Lawa maseli ahlangana ndawonye futhi akhe izakhiwo ezincane ezifana nama-bubble ezibizwa ngokuthi ama-cysts emaphashini akho. Ngokuhamba kwesikhathi, njengoba lezi zimila zikhula, zingalimaza izicubu zamaphaphu ezinempilo futhi zenze kube nzima ukuphefumula.
Lesi simo asigcini emaphashini kuphela. Ngezinye izikhathi, izimila zingakhula nasezinso kanye nasesimisweni se-lymphatic. Imbangela eyinhloko yalokhu ukuguquka kwezakhi zofuzo ezilawula ukukhula kwamaseli emzimbeni wethu.
Kubaluleke kakhulu ukukhumbula ukuthi lesi sifo sivame kakhulu kwabesifazane abaneminyaka ephakathi kuka-20 no-40, okungukuthi, phakathi kokuthomba kanye nokunqamuka kokuya esikhathini. Ngakho-ke, odokotela bakholelwa ukuthi i-hormone i-estrogen ihlobene naso.
Kunezinhlobo ezimbili eziyinhloko zama-LAM.
Isifo se-LAM singahlukaniswa ngezinhlobo ezimbili eziyinhloko, ngomehluko omncane phakathi kwalezi ezimbili.
| Uhlobo lwe-LAM | Incazelo elula |
|---|---|
| I-TSC-LAM | Lolu uhlobo lwe-LAM oluvela kwabesifazane abanesimo sofuzo esibizwa ngokuthi i-tuberous sclerosis. Lokhu kusho ukuthi luvela njengengxenye yesinye isimo sezokwelapha. |
| I-LAM engavamile | Lolu hlobo lubangelwa ushintsho olungahleliwe lwezakhi zofuzo (ukuguqulwa kwezakhi zofuzo) olwenzeka ngesikhathi sokuphila. Aluzuzwa njengefa. Ngakho-ke uma unalolu hlobo, akudingeki ukhathazeke ngokuthi izingane zakho zizodlulisela lesi sifo. |
Ziyini izimpawu zesifo se-LAM?
Ngenxa yokuthi izimpawu ze-LAM zifana kakhulu nezinye izifo zamaphaphu ezivamile, njenge-asthma, ngezinye izikhathi kungathatha isikhathi ukuxilonga. Lezi yizimpawu eziyinhloko.
- Ubunzima bokuphefumula (ukuphelelwa umoya): Nakuba kungabonakali ekuqaleni, lokhu kungakhululeka kunganda ngokuhamba kwesikhathi. Kungafaka phakathi ukuzizwa ukhathele ngisho noma uhamba amabanga amafushane, noma ukuphefumula kanzima lapho ukhuphuka izitebhisi.
- Ukuphefumula: Umsindo othambile ovela ngaphakathi esifubeni.
- Ubuhlungu besifuba: Kungase kube nobuhlungu besifuba obungazelelwe nobunzima.
- Ukukhwehlela: Ukukhwehlela okomile okuqhubekayo.
- Ukukhwehlela igazi noma 'i-chyle': I-Chyle iwuketshezi oluvela ohlelweni lwethu lokugaya ukudla. Inombala ofana nobisi.
Kungani lokhu kwenzeka? Iyini imbangela?
Njengoba sishilo ngaphambili, lokhu kubangelwa ushintsho kumajini. Kunezinhlobo ezimbili zamajini emzimbeni wethu ezibizwa nge -TSC1 kanye ne -TSC2 . Lezi zifana 'nabalindi' abalawula ukukhula kwamaseli emzimbeni wethu. Zibizwa ngokuthi amajini 'okucindezela ithumba'. Okusho ukuthi, umsebenzi walezi zakhi zofuzo ukuvimbela amaseli ukuthi angahlukani ngokungadingekile futhi akhe amathumba.
Umuntu one-LAM unesici, noma ukuguquka kwezakhi zofuzo, kungaba yi-TSC1 noma i-TSC2. Khona-ke lawo 'ma-guard' awasebenzi kahle. Ngenxa yalokho, amangqamuzana emisipha abushelelezi aqala ukuhlukana ngokungalawuleki, akha izinhlobo zezimila esikhulume ngazo ngaphambili.
- Ama-cyst emaphashini
- Izimila zezinso (i-angiomyolipomas)
- Amathumba ohlelweni lwe-lymphatic
Yiziphi izinkinga ezingaba khona ngenxa yalesi sifo?
Inkinga evame kakhulu futhi eyingozi ye-LAM iphaphu eliwile (i-pneumothorax) . Cabanga nge-cyst efana ne-bubble ephashini eqhuma ngokuzumayo, ivumela umoya ukuthi uvuze esikhaleni esiphakathi kwamaphaphu nodonga lwesifuba. Lokhu kubangela ukuthi amaphaphu awile. Abesifazane abangaphezu kwengxenye abane-LAM bazobhekana nalesi simo okungenani kanye empilweni yabo. Ngokuvamile, i-LAM itholakala okokuqala lapho kuvela iphaphu eliwile.
Kungase kube nezinye izinkinga:
- Ukuqongelela koketshezi 'lwe-chylous' emaphashini (chylothorax)
- Okunye ukunqwabelana koketshezi emaphashini (i-pleural effusion)
- Ukuvinjelwa kohlelo lwe-lymphatic
Udokotela uyixilonga kanjani i-LAM?
Ngemva kokulalela izimpawu zakho nokukuhlola, uma udokotela esola lesi sifo, uzoyala ukuhlolwa okuningana ukuze akuqinisekise.
| Ukuhlolwa | Wenzani ngalokhu? |
|---|---|
| Ukuhlolwa kokusebenza kwamaphaphu | Kukala ukuthi amaphaphu akho asebenza kahle kangakanani nokuthi ungangenisa futhi ukhiphe umoya ongakanani. |
| I-Pulse Oximetry | Banamathisela into efana ne-clip emunweni wakho bese behlola izinga lomoya-mpilo egazini lakho. |
| Ukuthwebula izithombe (ukuskena) | I -CT scan , ikakhulukazi i-HRCT (High-Resolution CT scan) , ingabona ngokucacile lawo ma-cyst amancane emaphashini. |
| Ukuhlolwa kwegazi kwe-VEGF-D | I-VEGF-D iyiprotheni. Amazinga ale protheni avame ukuba phezulu egazini labantu abane-LAM. Lokhu kusiza kakhulu ekuxilongeni lesi sifo. |
| I-biopsy yamaphaphu | Uma ezinye izivivinyo zingakwazi ukuqinisekisa ukuxilongwa, ingxenye encane yezicubu ithathwa emaphashini bese ihlolwa ngaphansi kwe-microscope. Lokhu kungenziwa kusetshenziswa inqubo efana ne -bronchoscopy noma i-VATS . |
Yiziphi izindlela zokwelapha i-LAM?
Okokuqala, akukho ikhambi le-LAM okwamanje.Kodwa ungakhathazeki. Manje sekukhona izindlela zokwelapha ezisebenzayo ezingasiza ekulawuleni lesi sifo, zinciphise izimpawu, futhi zivimbele ukuthi singabi sibi kakhulu.
Ukwelashwa okuyinhloko umuthi obizwa ngokuthi i-sirolimus . Ubizwa nangokuthi i-rapamycin. Lo muthi usebenza ngokuvimbela ukukhula kwamaseli emisipha angajwayelekile. Lokhu kungagcina lesi sifo sizinzile futhi kunciphise amathumba ezinso kanye nohlelo lwe-lymphatic.
Ngaphezu kwalokho, kuye ngesimo sakho, udokotela wakho angase ancome ezinye izindlela zokwelapha ezifana nalezi:
- Ukwelashwa nge-oxygen: Uma izinga le-oxygen egazini liphansi.
- Ama-bronchodilator aphefumulayo: Yehlisa ubunzima bokuphefumula.
- Ukuvuselelwa kwamaphaphu: Ukuzivocavoca kanye nezeluleko zendlela yokuphila eziqinisa amaphaphu.
- Ukufakelwa amaphaphu: Indlela yokugcina lapho lesi sifo sibi kakhulu futhi singenakulawulwa ngezinye izindlela zokwelapha.
Okubalulekile: I-Sirolimus ingabangela imiphumela emibi, njengokulimala kwezinso kanye nengozi eyengeziwe yokutheleleka. Ngakho-ke, khuluma nodokotela wakho ngezinzuzo kanye nokungalungi kwalo muthi ngaphambi kokuwuqala.
Izinto okufanele uzazi uma uhlala ne-LAM
I-LAM yisimo sesikhathi eside. Ngakho-ke, kuzodingeka usebenzisane eduze nodokotela wakho wamaphaphu. Ukuqhubeka kwesifo, okungukuthi, isivinini esiqhubeka ngaso lesi sifo, kuyahlukahluka kumuntu nomuntu. Kuncike ezintweni eziningana:
- Kuya ngokuthi uluhlobo luni lwe-LAM onayo.
- Iminyaka yakho (kwabanye abantu, lesi sifo siyayeka ukuba sibi kakhulu ngemva kokunqamuka kokuya esikhathini).
- Indlela umzimba wakho osabela ngayo ekwelashweni.
Ungesabi ukufakelwa amaphaphu. Iningi labantu (cishe ama-64%) ngeke lidinge ukufakelwa amaphaphu kuze kube yiminyaka engama-20 noma ngaphezulu ngemva kokuxilongwa. Ngenxa yezindlela zokwelapha ezintsha, isikhathi sokuphila sabantu abaphila ne-LAM sesiphezulu kakhulu manje. Abantu abangaphezu kwama-90% basaphila eminyakeni eyi-10 ngemva kokuxilongwa.
Nini lapho kufanele uthole iseluleko sezokwelapha
Uma uphila nesifo esifana nalesi, kubaluleke kakhulu ukunakekela umzimba wakho.
| Uma unalezi zimpawu, bheka udokotela wakho: | |
| |
| Yiya e-ETU (i-Emergency Treatment Unit) ngokushesha uma unalezi zimpawu: | |
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Ingabe i-LAM ingumdlavuza?
Lokhu kuyinkinga kubantu abaningi. I-LAM ngokuvamile ayibhekwa njengomdlavuza . Kodwa-ke, inezici ezithile ezifana nomdlavuza. Isibonelo, amaseli angabonakala sengathi asakazeke esuka kwezinye izingxenye zomzimba aya emaphashini nasezinso. Kodwa-ke, uma ebhekwa nge-microscope, lawa maseli awabonakali njengamaseli omdlavuza, kodwa afana namaseli avamile. Futhi, awasakazeki ezithweni ezifana nesibindi noma ubuchopho.
Kuvamile ukuzizwa sengathi umhlabathi ususiwe ngaphansi kwakho uma uthola ukuthi unesifo esinje impilo yakho yonke. Kodwa awuwedwa. Ngosizo lukadokotela wakho, umndeni nabangane, ungabhekana nalesi simo.
Umlayezo Wokuya Nawe Ekhaya
- I-LAM yisifo samaphaphu esingavamile kakhulu esivame kakhulu kwabesifazane.
- Izimpawu eziyinhloko ubunzima bokuphefumula, ukukhwehlela, kanye nobuhlungu besifuba.
- Lokhu kungenxa yokuguquka kwezakhi zofuzo, hhayi iphutha lakho.
- Nakuba kungekho ikhambi eliphelele, lesi sifo singalawulwa kahle ngemithi efana ne-sirolimus.
- Hlala uthintana njalo nochwepheshe wakho wokuphefumula.
- Ezimweni eziphuthumayo ezifana nobuhlungu obukhulu besifuba noma ubunzima obukhulu bokuphefumula, iya ku-ETU ngokushesha.
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