Ngaba ngamanye amaxesha uziva uphelelwa ngumphefumlo, udiniwe, okanye unentlungu esifubeni? Sihlala sicinga ngezi mpawu njengesifo esiqhelekileyo njenge-asthma. Nangona kunjalo, kukho isifo esingaqhelekanga semiphunga esibangela ezi mpawu, ingakumbi kubasetyhini. Sibizwa ngokuba yiLymphangioleiomyomatosis. Eli gama lide kancinci, ngoko ke siza kulibiza ngokuthi "LAM" ngamafutshane. Ukuba ufumana kunzima ukulibiza eli gama, cinga ngalo ngolu hlobo: "Lymph-ang-ge-o-ly-o-myo-ma-to-sis". Nangona kunzima kancinci, kunokuba kubaluleke kakhulu ukuba usiqonde esi sifo ngokulula.
Kalula nje, yintoni i-LAM?
I-LAM yimeko engaqhelekanga apho iiseli ezingaqhelekanga zikhula ngaphakathi kwemiphunga yakho, zenze ii-cysts ezincinci. Oku kubangelwa kukuguquka kwezakhi zofuzo okubangela ukuba iiseli zemisipha egudileyo emzimbeni wakho zikhule ngokungalawulekiyo. Oku kungonakalisa imiphunga yakho. Akukuphela nje imiphunga, kodwa ngamanye amaxesha izintso kunye nenkqubo ye-lymphatic nazo zinokuphuhlisa ezi zihluma.
Esi sifo sidla ngokufunyanwa kubafazi abaphakathi kweminyaka engama-20 ukuya kwengama-40 ubudala. Oko kukuthi, emva kokufikisa nangaphambi kokuya exesheni. Ke ngoko, oogqirha bakholelwa ukuba i-estrogen yehomoni yabasetyhini idlala indima ekuphuhlisweni kwesi sifo.
Kukho iintlobo ezimbini eziphambili zesifo se-LAM.
Isifo se-LAM sinokwahlulwa sibe ziintlobo ezimbini eziphambili, umahluko omncinci phakathi kwezi zimbini.
| Uhlobo lwe-LAM | Ingcaciso elula |
|---|---|
| I-TSC-LAM | Oku kunxulumene ne-Tuberous Sclerosis, imeko yemfuza. Abanye abafazi abane-Tuberous Sclerosis nabo banesifo se-LAM. Oku kunokuba yimfuza. |
| I-LAM engaqhelekanga | Oku kubangelwa lutshintsho olungaqhelekanga lwemfuza (uguquko) olwenzeka ngexesha lobomi bakho. Aluzuzwa njengelifa. Ngoko ke ukuba unolu hlobo lwe-LAM, aluyi kudluliselwa kubantwana bakho. |
Eyona nto ibalulekileyo kukuba esi sifo sinqabile kakhulu. Ngokwezibalo, sichaphazela umfazi omnye kuphela kwi-140,000. Nangona kunjalo, phakathi kwe-30% kunye ne-80% yabasetyhini abane-tuberous sclerosis nabo banokuba ne-LAM.
Zithini iimpawu zesifo se-LAM?
Iimpawu ze-LAM zifana kakhulu nezezinye izifo zemiphunga, ezifana ne-asthma kunye ne-bronchitis. Oku ngamanye amaxesha kungathatha ixesha ukuxilonga. Kubalulekile ukukwazi oku.
- Ubunzima bokuphefumla (ukuphelelwa ngamandla): Olu lolona phawu luphambili noluqhelekileyo. Nangona ekuqaleni lunokuvakala kuphela ngomzamo omncinci, le meko inokuba mandundu ngokuhamba kwexesha.
- Ukuphefumla: Isandi sokukhala xa uphefumla, esifana nesomntu one-asthma.
- Intlungu yesifuba: Le ntlungu inokuvakala ngakumbi xa uphefumla nzulu.
- Ukukhwehlela: Kusenokubakho ukukhwehlela okomileyo okungapheliyo.
- Ukukhwehlela igazi okanye 'chyle': I-Chyle lulwelo oluvela kwinkqubo yethu yokugaya ukutya. Inombala ofana nobisi. Ukuba kwenzeka into efana nale, kungcono ungalibazisi konke konke .
Kutheni le nto i-LAM isenzeka? Yintoni unobangela?
Ngamafutshane, isizathu esiphambili soku kukutshintsha (ukuguqulwa kwezakhi zofuzo) okwenzeka kwiijini ezimbini emzimbeni wethu ezibizwa ngokuba yi-TSC1 kunye ne-TSC2 . Ezi jini zimbini zifana nabalindi bomzimba wethu. Umsebenzi wazo ophambili kukuthintela ezinye iiseli ukuba zingahlukani kwaye zingaphindaphindani ngokungeyomfuneko ngaphandle kolawulo. Ezi jini sizibiza ngokuba zi-tumor suppressor genes.
Khawucinge ukuba bekukho isiphene kwenye yezi jini zimbini zokukhusela. Okwenzekayo kukuba iiseli zemisipha ezigudileyo azifumani mqondiso wokuba, "Kulungile, kwanele, yeka ukwahlukana." Ngoko ke iiseli ziqala ukwahlukana ngokungalawulekiyo. Isiphumo sesi:
- Ii-cysts zenzeka emiphungeni.
- Ii-tumor (i-angiomyolipomas) zenzeka kwizintso.
- Ii-cysts zenzeka kwinkqubo ye-lymphatic.
Ungayifumana njengelifa le gene engalunganga kubazali bakho (ebangela i-tuberous sclerosis), okanye ungatshintsha ezi gene ngokungacwangciswanga ngexesha lobomi bakho ngaphandle kwesizathu esicacileyo (esibangela i-LAM engaqhelekanga).
Iingxaki kunye neengozi zesifo se-LAM
Eyona ngxaki ixhaphakileyo neyona inkulu ye-LAM kukuwohloka komphunga . Ngokwezonyango, oku kubizwa ngokuba yi-pneumothorax .Ngaphezulu kwesiqingatha sabasetyhini abane-LAM baya kuba nale meko ubuncinane kube kanye ebomini babo. Ngamanye amaxesha inokuphinda ivele. Enyanisweni, abafazi abaninzi baqala ukuxilongwa nge-LAM emva kokuba benesifo semiphunga baze bangeniswe esibhedlele.
Ukongeza, ezinye iingxaki zinokwenzeka.
| Ingxaki | Ingcaciso elula |
|---|---|
| Ukuwa kwemiphunga (iPneumothorax) | Ama-cysts emaphaphu anokuqhuma, nto leyo ebangela ukuba umoya uqokeleleke phakathi kwamaphaphu nodonga lwesifuba. Oku kunokubangela ubunzima ngesiquphe bokuphefumla kunye nentlungu eqatha esifubeni. |
| Ukuqokelelana kolwelo lwe-chylous olujikeleze imiphunga (i-Chylothorax) | Ulwelo olufana nobisi (chyle) oluvela kwinkqubo ye-lymphatic luqokelelana lujikeleze imiphunga. Oku kubangela ubunzima bokuphefumla. |
| Ezinye izinto eziqokelelana ulwelo ezijikeleze imiphunga (iPleural Effusion) | Ukuqokelelwa kolwelo ngaphandle kolwelo lwe-pleural phakathi kwee-membrane ezigubungela imiphunga. |
Isifo se-LAM sifunyanwa njani?
Emva kokumamela iimpawu zakho, ukuba ugqirha wakho urhanela ukuba esi sifo sikhona, uya kuyalela iimvavanyo ezininzi ukuze aqinisekise ukuba sikhona.
Uvavanyo oluqhutywa ngugqirha
- Uvavanyo Lokusebenza Kwemiphunga: Oku kubandakanya ukuphefumla ngaphakathi nangaphandle ngetyhubhu eqhagamshelwe kumatshini. Oku kunokulinganisa ukuba imiphunga yakho isebenza kakuhle kangakanani kunye nokuba ingakanani na umoya enokuyibamba.
- I-Pulse Oximetry: Isixhobo esincinci, esifana ne-clip, sincamathiselwa emnweni wakho kwaye silinganisa ubungakanani beoksijini egazini lakho. Olu luvavanyo olulula kakhulu nolungenabuhlungu.
- Iiskeni zeMifanekiso:
- I-CT Scan (i-Computed Tomography):Oku kungathatha imifanekiso eneenkcukacha ze-3D yemiphunga yakho. Oku kunokunceda ukubona ngokucacileyo ukuba kukho naziphi na ii-cysts ezikhethekileyo kwi-LAM.
- I-HRCT scan (i-High-Resolution Computed Tomography): Le yinguqulelo ephucukileyo ye-CT scan. Ingathatha imifanekiso ecacileyo yenxalenye encinci kakhulu yemiphunga. Oku kubaluleke kakhulu ekuxilongeni i-LAM.
- Uvavanyo lwegazi lwe-VEGF-D: I-VEGF-D yiproteni efumaneka egazini lethu. Abantu abaninzi abane-LAM banamanqanaba aphezulu kakhulu ale proteni egazini labo kunesiqhelo. Ngoko ke olu vavanyo lwegazi luluncedo kakhulu ekufumaniseni isifo.
- I-Lung Biopsy: Ngamanye amaxesha, ukuba ezinye iimvavanyo aziqinisekanga ngokupheleleyo, ugqirha wakho unokugqiba ekubeni athathe iqhekeza elincinci kakhulu lesicwili esiphuma emphungeni wakho ukuze silivavanye. Oku kungenziwa ngokusebenzisa inkqubo ebizwa ngokuba yi-bronchoscopy (ityhubhu encinci enekhamera efakwe kwimigudu yomoya) okanye i-VATS (utyando lwesifuba oluncediswa yividiyo) .
Ziziphi iindlela zonyango zesifo se-LAM?
Okokuqala, akukho nyango lwe-LAM okwangoku. Kodwa ungakhathazeki. Ngoku kukho unyango olusebenzayo kakhulu olunokulawula isifo, lunciphise iimpawu, kwaye lucothise ukuqhubela phambili kwaso.
Unyango oluphambili liyeza elibizwa ngokuba yi -sirolimus . Likwabizwa ngokuba yi-rapamycin. Eli yeza lisebenza ngokuthintela ukukhula kweeseli ezikhula zingalawulekiyo. Oku kunokunceda ukulawula ukonakala ngakumbi kwesi sifo ngomlinganiselo omkhulu. Linokunceda ekunciphiseni iithumba kwizintso nakwinkqubo ye-lymphatic kwaye linciphise iimpawu.
Kubalulekile: I-Sirolimus inokubangela ezinye iziphumo ebezingalindelekanga, ingakumbi ukonakala kwezintso kunye nokwanda kokuchaphazeleka zizifo. Ke ngoko, kubalulekile ukuxoxa ngezibonelelo kunye neengozi nogqirha wakho ngaphambi kokuba uqale eli yeza.
Ukongeza, ugqirha wakho unokucebisa ezinye iindlela zonyango ngokuxhomekeke kwimeko yakho.
- Unyango lwe-oksijini: Ukuba inqanaba le-oksijini egazini liphantsi, iitanki ze-oksijini zibonelelwa ukuze zisetyenziswe ekhaya.
- Iibronchodilators ezifuthwayo: La mayeza, anikwa nge-inhaler, anokunciphisa ubunzima bokuphefumla.
- Ukuvuselelwa Kwemiphunga: Oku kufana nenkqubo ekhethekileyo yokuzilolonga yemiphunga. Inceda ukuphucula umgangatho wobomi bakho ngokuzilolonga ukuphefumla kunye nokuzilolonga okulungele umzimba.
- Ukufakelwa kweMiphunga:Xa isifo sele sihambele phambili kakhulu kwaye singenakulawulwa lolunye unyango, ukufakelwa imiphunga kungaba lolona khetho lokugqibela.
Isifo siqhubeka ngokukhawuleza kangakanani?
Oku kuyahluka kumntu nomntu. Izinga lokukhula kwe-LAM lixhomekeke kwizinto ezahlukeneyo.
- Uhlobo lwe-LAM onayo: Abantu abane-LAM engaqhelekanga banokuba nomsebenzi wemiphunga ophantsi kancinci kunabo bane-TSC-LAM.
- Ubudala bakho: Kwabanye abafazi, emva kokuya exesheni, ukuqhubela phambili kwesi sifo kuyayeka okanye kuyeke njengoko amanqanaba e-estrogen emzimbeni ehla.
- Impendulo yakho kunyango: Abantu abaninzi banokulawula esi sifo kakuhle ngamayeza afana neSirolimus.
Imeko ingcono kakhulu ngoku kunangaphambili. Ngonyango lwanamhlanje, abantu abangaphezu kwama-90% abane-LAM bayaphila emva kweminyaka eli-10 bexilongiwe. Abantu abaninzi (malunga nama-64%) banokuphila iminyaka engama-20 nangaphezulu ngaphandle kokufuna ukufakelwa imiphunga.
Ixesha lokubona ugqirha wakho nokuya kwi-ETU
Xa uhlala ne-LAM, kubaluleke kakhulu ukwazi ukuba kufuneka ufumane nini ingcebiso yezonyango kwaye kufuneka uye nini kwiSebe leeNgxamiseko (ETU).
| Amathuba | Kwenziwe ntoni |
|---|---|
| Bona ugqirha wakho... | |
| Iimpawu eziqhelekileyo | Ukuba iimpawu ezinje ngobunzima bokuphefumla nokukhohlela ziyaqhubeka okanye zibonakala zisiba mandundu. |
| Imibuzo malunga nonyango | Ukuba unemibuzo malunga namayeza owasebenzisayo okanye ukhathazekile ngemiphumo emibi. |
| Yiya kwiYunithi yoNyango oluNgxamisekileyo (ETU) ngoko nangoko... | |
| Iimpawu zokuqhuma kwemiphunga |
|
Abanye abantu bayabuza ukuba i-LAM ngumhlaza na. I-LAM ayisoloko ithathwa njengomhlaza. Nangona kunjalo, ngeendlela ezithile, njengendlela iiseli ezivela ngayo kwenye indawo ukuya emiphungeni, zineempawu ezifana nomhlaza. Nangona kunjalo, xa zijongwa nge-microscope, ezi seli azibonakali njengeeseli zomhlaza.
Kuqhelekile ukuziva wothukile kwaye usoyika xa ufumanisa ukuba unesifo esisongela ubomi esifana nesi. Kodwa khumbula, awuwedwa. Ngenkxaso yoogqirha bakho, usapho lwakho, kunye nabahlobo bakho, uya kufumana amandla okujongana nalo mceli mngeni.
Umyalezo Wokuya Ekhaya
- I-LAM (Lymphangioleiomyomatosis) sisifo esingaqhelekanga semiphunga esichaphazela kakhulu abasetyhini.
- Ubunzima bokuphefumla, iintlungu zesifuba, kunye nokukhwehlela okungapheliyo zezona mpawu ziphambili.
- I-pneumothorax yingxaki eqhelekileyo . Ukuba ufumana iintlungu zesifuba eziqatha ngequbuliso kwaye kunzima ukuphefumla, yiya kwigumbi longxamiseko ngokukhawuleza.
- Nangona esi sifo singenakunyangwa ngokupheleleyo, amayeza afana ne-sirolimus anokunceda ukulawula esi sifo kakuhle kwaye akuvumele ukuba uphile ubomi obuqhelekileyo.
- Ungaze woyike ukuthetha ngokukhululekileyo nogqirha wakho malunga neempawu zakho, unyango, okanye naziphi na izinto ezikuxhalabisayo.
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