Eli gama kunzima ukulifunda, akunjalo? "Lymphangioleiomyomatosis". Akukho ngxaki, masilibize ngokuba yi-LAM . Esi sisifo semiphunga esingaqhelekanga esichaphazela kakhulu abafazi. Kuyaqondakala ukuba woyike xa usiva igama elinjalo. Kodwa ungakhathazeki. Kweli nqaku, siza kuthetha ngokuba yintoni i-LAM, kutheni isenzeka, kunye nonyango olufumanekayo ngayo ngendlela elula neyobuhlobo.
Ithetha ukuthini i-LAM nje?
Ngamafutshane, i-LAM kuxa iiseli zemisipha ezigudileyo ngaphakathi emiphungeni yakho ziqala ukukhula zingalawuleki. Njengeethumba ezincinci. Ezi seli ziyahlangana kwaye zenze izakhiwo ezincinci ezifana namaqamza ezibizwa ngokuba zii-cysts emiphungeni yakho. Ekuhambeni kwexesha, njengoko ezi thumba zikhula, zingonakalisa izicubu zemiphunga ezisempilweni kwaye zenze kube nzima ukuphefumla.
Le meko ayipheleli nje kwimiphunga. Ngamanye amaxesha, iithumba zinokukhula nakwizintso nakwinkqubo ye-lymphatic. Isizathu esiphambili soku kukuguquka kwezakhi zofuzo ezilawula ukukhula kweeseli emzimbeni wethu.
Kubaluleke kakhulu ukukhumbula ukuba esi sifo sixhaphake kakhulu kubasetyhini abaphakathi kweminyaka engama-20 ukuya kwengama-40, oko kukuthi, phakathi kokufikisa nokuya exesheni. Ke ngoko, oogqirha bakholelwa ukuba i-hormone ye-estrogen inento yokwenza naso.
Kukho iintlobo ezimbini eziphambili ze-LAMs.
Isifo se-LAM sinokwahlulwa sibe ziintlobo ezimbini eziphambili, umahluko omncinci phakathi kwezi zimbini.
| Uhlobo lwe-LAM | Inkcazo elula |
|---|---|
| I-TSC-LAM | Olu luhlobo lwe-LAM oluvela kubasetyhini abanemeko yemfuza ebizwa ngokuba yi-tuberous sclerosis. Oku kuthetha ukuba luvela njengenxalenye yesinye isifo. |
| I-LAM engaqhelekanga | Olu hlobo lubangelwa lutshintsho olungaqhelekanga lwemfuza (uguquko) olwenzeka ngexesha lobomi. Aluzuzwa njengelifa. Ngoko ke ukuba unalo olu hlobo, akufuneki ukhathazeke ngokuba abantwana bakho baza kudlulisela esi sifo. |
Zithini iimpawu zesifo se-LAM?
Ngenxa yokuba iimpawu ze-LAM zifana kakhulu nezinye izifo zemiphunga eziqhelekileyo, ezifana ne-asthma, ngamanye amaxesha kungathatha ixesha ukuxilonga. Ezi ziimpawu eziphambili.
- Ubunzima bokuphefumla (ukuphelelwa ngamandla): Nangona kungabonakali ekuqaleni, oku kungakhululeki kunokwanda ngokuhamba kwexesha. Kungabandakanya ukuziva udiniwe nokuba uhamba umgama omfutshane, okanye ukuphefumla kancinci xa unyuka izinyuko.
- Ukuphefumla: Isandi esithambileyo esiphuma ngaphakathi esifubeni.
- Intlungu yesifuba: Kunokubakho iintlungu zesifuba ezingalindelekanga neziqatha.
- Ukukhwehlela: Ukukhwehlela okomileyo okungapheliyo.
- Ukukhohlela igazi okanye 'chyle': I-Chyle lulwelo oluphuma kwinkqubo yethu yokugaya ukutya. Inombala ofana nobisi.
Kutheni le nto i-LAM isenzeka? Yintoni unobangela?
Njengoko besitshilo ngaphambili, oku kubangelwa kukutshintsha kwezakhi zofuzo. Kukho iintlobo ezimbini zezakhi zofuzo emzimbeni wethu ezibizwa ngokuba yi-TSC1 kunye ne -TSC2 . Ezi zifana 'nabalindi' abalawula ukukhula kweeseli emizimbeni yethu. Zibizwa ngokuba ziizakhi zofuzo 'zokuthintela ithumba'. Oko kukuthi, umsebenzi wezi zakhi zofuzo kukuthintela iiseli ukuba zingahlukani ngokungeyomfuneko kwaye zenze iithumba.
Umntu one-LAM unesiphako, okanye utshintsho, kwenye yezi jini, nokuba yi-TSC1 okanye i-TSC2. Emva koko ezo 'zikhuselo' azisebenzi kakuhle. Ngenxa yoko, iiseli zemisipha ezigudileyo ziqala ukwahlukana ngokungalawulekiyo, zenze iintlobo zeethumba esithethe ngazo ngaphambili.
- Ii-cysts zemiphunga
- Iithumba zezintso (i-angiomyolipomas)
- Iithumba zenkqubo ye-lymphatic
Ziziphi iingxaki ezinokubakho zesi sifo?
Eyona ngxaki ixhaphakileyo neyingozi ye-LAM kukuwohloka komphunga (pneumothorax) . Khawuthelekelele i-cyst efana ne-bubble emphungeni eqhuma ngequbuliso, ivumela umoya ukuba uvuza ungene kwindawo ephakathi komphunga nodonga lwesifuba. Oku kubangela ukuba umphunga uwohloke. Ngaphezulu kwesiqingatha sabasetyhini abane-LAM baya kuba nale meko ubuncinane kanye ebomini babo. Rhoqo, i-LAM ifunyanwa okokuqala xa umphunga uwohlokile.
Kusenokubakho nezinye iingxaki:
- Ukuqokelelwa kolwelo 'oluyi-chylous' olujikeleze imiphunga (chylothorax)
- Ezinye izinto eziqokelelana ulwelo ezijikeleze imiphunga (i-pleural effusion)
- Ukuvalwa kwenkqubo ye-lymphatic
Ugqirha uyixilonga njani i-LAM?
Emva kokumamela iimpawu zakho nokukuxilonga, ukuba ugqirha urhanela ukuba esi sifo sikhona, uya kuyalela iimvavanyo ezininzi ukuze aqinisekise ukuba sikhona.
| Uvavanyo | Wenza ntoni ngale nto? |
|---|---|
| Uvavanyo lokusebenza kwemiphunga | Ilinganisa indlela imiphunga yakho esebenza ngayo kunye nobungakanani bomoya onokuwuthatha nowukhuphayo. |
| I-Pulse Oximetry | Bancamathisela into efana nekliphu emnweni wakho baze bajonge inqanaba leoksijini egazini lakho. |
| Ukufota (ukuskena) | I -CT scan , ingakumbi i-HRCT (High-Resolution CT scan) , inokuzibona ngokucacileyo ezo cysts zincinci emiphungeni. |
| Uvavanyo lwegazi lwe-VEGF-D | I-VEGF-D yiproteni. Amanqanaba ale proteni adla ngokuba phezulu egazini labantu abane-LAM. Oku kunceda kakhulu ekuxilongeni esi sifo. |
| i-biopsy yemiphunga | Ukuba ezinye iimvavanyo azikwazi ukuqinisekisa ukuxilongwa, iqhekeza elincinci lesicubu lithathwa emphungeni lize lihlolwe phantsi kwe-microscope. Oku kungenziwa kusetyenziswa indlela efana ne -bronchoscopy okanye i-VATS . |
Ziziphi iindlela zonyango lwe-LAM?
Okokuqala, akukho nyango lwe-LAM okwangoku.Kodwa ungakhathazeki. Ngoku kukho unyango olusebenzayo olunokunceda ukulawula esi sifo, ukunciphisa iimpawu, kunye nokuthintela ukuba singabi mandundu.
Unyango oluphambili liyeza elibizwa ngokuba yi -sirolimus . Likwabizwa ngokuba yi-rapamycin. Eli yeza lisebenza ngokuthintela ukukhula kweeseli zemisipha ezingaqhelekanga. Oku kunokugcina esi sifo sizinzile kwaye kunciphise iithumba kwizintso nakwinkqubo ye-lymphatic.
Ukongeza, kuxhomekeke kwimeko yakho, ugqirha wakho unokucebisa ezinye iindlela zonyango ezifana nezi:
- Unyango lweoksijini: Ukuba inqanaba leoksijini egazini liphantsi.
- Iibronchodilators ezifuthwayo: Zinciphisa ubunzima bokuphefumla.
- Ukuvuselelwa kweMiphunga: Ukuzilolonga kunye neengcebiso zendlela yokuphila eziqinisa imiphunga.
- Ukufakelwa imiphunga: Indlela yokugqibela xa isifo sinzima kakhulu kwaye singenakulawulwa ngezinye iindlela zonyango.
Kubalulekile: I-Sirolimus inokubangela iziphumo ebezingalindelekanga, ezinje ngokonakala kwezintso kunye nomngcipheko ophezulu wosulelo. Ngoko ke, thetha nogqirha wakho malunga neengenelo kunye neengxaki zale mithi ngaphambi kokuba uyiqalise.
Izinto ekufuneka uzazi xa uhlala ne-LAM
I-LAM yimeko yexesha elide. Ngoko ke, kuya kufuneka usebenzisane ngokusondeleyo nogqirha wakho wemiphunga. Ukuqhubela phambili kwesi sifo, oko kukuthi, isantya esiqhubekela phambili ngaso isifo, kuyahluka ukusuka kumntu ukuya komnye. Kuxhomekeke kwizinto ezininzi:
- Kuxhomekeke ekubeni loluphi uhlobo lwe-LAM onayo.
- Ubudala bakho (kwabanye abantu, esi sifo siyayeka ukuba mandundu emva kokuya exesheni).
- Indlela umzimba wakho osabela ngayo kunyango.
Musa ukoyika ukufakelwa imiphunga. Uninzi lwabantu (malunga nama-64%) aluyi kufuna ukufakelwa imiphunga de kube yiminyaka engama-20 okanye ngaphezulu emva kokuxilongwa. Ngenxa yonyango olutsha, ixesha lokuphila kwabantu abaphila ne-LAM liphezulu kakhulu ngoku. Abantu abangaphezu kwama-90% basaphila emva kweminyaka eli-10 emva kokuxilongwa.
Ixesha lokufuna ingcebiso kwezonyango
Xa uphila nesifo esifana nesi, kubaluleke kakhulu ukunyamekela umzimba wakho.
| Ukuba unale mpawu, bona ugqirha wakho: | |
| |
| Yiya kwi-ETU (iYunithi yoNyango oluNgxamisekileyo) ngoko nangoko ukuba unezi mpawu: | |
|
Ngaba i-LAM ngumhlaza?
Le yingxaki kubantu abaninzi. I-LAM ayisoloko ithathwa njengomhlaza . Nangona kunjalo, ineempawu ezifana nomhlaza. Umzekelo, iiseli zinokubonakala ngathi zisasazeke kwezinye iindawo zomzimba ukuya emiphungeni nasezintsoni. Nangona kunjalo, xa zijongwa ngemakroskopu, ezi seli azibonakali ngathi ziiseli zomhlaza, kodwa zifana neeseli eziqhelekileyo. Kwakhona, azisasazeki kwizitho ezifana nesibindi okanye ubuchopho.
Kuqhelekile ukuziva ngathi umhlaba ukrazukile phantsi kwakho xa ufumanisa ukuba unesifo esinje ubomi bakho bonke. Kodwa awuwedwa. Ngoncedo lukagqirha wakho, usapho kunye nabahlobo, ungamelana nale meko.
Umyalezo Wokuya Ekhaya
- I-LAM sisifo semiphunga esingaqhelekanga kakhulu esixhaphakileyo kwabasetyhini.
- Iimpawu eziphambili kubunzima bokuphefumla, ukukhohlela, kunye nentlungu esifubeni.
- Oku kungenxa yotshintsho lwemfuza, asikuko ukuba unetyala.
- Nangona kungekho nyango lupheleleyo, esi sifo sinokulawulwa kakuhle ngamayeza anjengesirolimus.
- Hlala unxibelelana rhoqo nengcali yakho yokuphefumla.
- Kwiimeko ezingxamisekileyo ezifana neentlungu eziqatha esifubeni okanye ubunzima obukhulu bokuphefumla, yiya kwi-ETU ngoko nangoko.
💬 Comments (0)
Akukho zimvo zithunyelweyo okwangoku. Faka uluvo lwakho apha okokuqala.
Faka uluvo lwakho