Ngaba unazo ezi ngxaki ngemiphunga yakho? Masithethe nge-LAM (Lymphangioleiomyomatosis)

Ngaba wakha weva ngesi sifo esinegama elide elibizwa ngokuba yi-LAM (lymphangioleiomyomatosis)? Usenokulifumana lingaqhelekanga eli gama. Kodwa sisifo esingaqhelekanga esichaphazela imiphunga. Namhlanje, siza kuthetha ngaso ngokulula, ngendlela onokuyiqonda. Ungakhathazeki, siza kuchaza yonke into.

Yintoni kanye kanye i-LAM (Lymphangioleiomyomatosis)?

Ngamafutshane, i-LAM sisifo esingaqhelekanga esibangela ukuba ii-cysts ezizaliswe lulwelo zakheke ngaphakathi kwemiphunga yakho. Oku kungonakalisa imiphunga yakho. Ngamanye amaxesha kunokubangela ii-cysts kwizintso zakho nakwinkqubo ye-lymphatic. Khawucinge ngento enokwenzeka ukuba iiseli zemisipha egudileyo emzimbeni wakho ziqala ukukhula ngokungalawulekiyo. Yiloo nto eyenzekayo apha. Ibangelwa kukuguquka kwezakhi zofuzo.

Le meko, ebizwa ngokuba yi-LAM (ebizwa ngokuba yi-“limf-AN-gee-oh-ly-oh-my-oh-muh-TOH-sis”) , ichaphazela kakhulu abasetyhini . Idla ngokuchaphazela abasetyhini abaphakathi kweminyaka engama-20 nama-40, okanye abasetyhini abaye badlula kwixesha lokufikisa kwaye sele besondele exesheni. Ngenxa yesi sizathu, oogqirha bacinga ukuba i-hormone i-estrogen okanye ubukho besibeleko bunokuba nefuthe ekuphuhlisweni kwezi thumba.

Ziziphi iintlobo eziphambili ze-LAM?

Kukho iintlobo ezimbini eziphambili ze-LAM:

1. I-TSC-LAM: Abanye abafazi banemeko yemfuza ebizwa ngokuba yi-``Tuberous Sclerosis Complex - TSC''. Ukuba baphuhlisa i-LAM, ibizwa ngokuba yi-TSC-LAM.

2. I-Sporadic LAM: Oku kwenzeka ngokungacwangciswanga, oko kuthetha ukuba utshintsho lwe-gene luyenzeka ngokungacwangciswanga ngexesha lobomi. Into ebalulekileyo kukuba olu hlobo lwe-Sporadic LAM lungadluliselwa kubantwana bakho.

Ixhaphake kangakanani le sifo ibizwa ngokuba yi-LAM?

I-LAM sisifo esingaqhelekanga . Kuqikelelwa ukuba malunga nomfazi omnye kwi-140,000 unokuba nesi sifo. Nangona kunjalo, phakathi kwama-30% nama-80% abasetyhini abane-"Tuberous Sclerosis" banokuba ne-LAM.

Zithini iimpawu zesifo se-LAM?

Umntu one-LAM unokufumana iimpawu ezifana nezi:

• Ubunzima bokuphefumla (ukuphelelwa ngamandla) : Ngamanye amaxesha oku kunokunyuka kancinci kancinci.
• Ukuphefumla sisandi somlozi esiphuma esifubeni .
• Iintlungu zesifuba .
• Ukukhwehlela .
• Ngamanye amaxesha kukho igazi okanye i-chyle (`(chyle)` - ulwelo lwe-lymphatic oluvela kwinkqubo yokugaya ukutya) oluphuma kunye ne-mucus .

Musa ukucinga ukuba une-LAM kuba nje unale mpawu, kuba zinokuba ziimpawu zezinye izifo zemiphunga. Ngoko ke, kubalulekile ukufuna ingcebiso kagqirha.

Kutheni le nto i-LAM isenzeka? Yintoni unobangela?

Eyona nto ibangela i-LAM kukuguquka kwezakhi zofuzo ezibini emzimbeni wethu ezibizwa ngokuba yi-`(TSC1)` kunye ne-`(TSC2)`. Ezi zakhi zofuzo zimbini zifana nezakhi zofuzo ezithintela ukukhula kwezinto ezifana neeseli zomhlaza ngokungeyomfuneko emzimbeni wethu. Ngoko ke, ukuba kukho isiphene kwenye yezi zakhi zofuzo zimbini, ezo seli zemisipha egudileyo endizikhankanyileyo ngaphambili ziqala ukukhula ngokungalawulekiyo. Ngenxa yoko, oku kulandelayo:

• Iithumba zemiphunga (i-pulmonary lymphangioleiomyomatosis) ziyavela.
• Ii-tumor (i-angiomyolipomas) zenzeka kwizintso.
• Ii-cysts zenzeka kwinkqubo ye-lymphatic.

Umntu ufumana njani i-LAM?

Kukho iindlela ezimbini umntu anokuphuhlisa ngazo i-LAM. Enye kukufumana i-gene enesiphene kumzali onesiphako kwi-gene ethi `(TSC1)` okanye `(TSC2)`. Olu lifa lubangela imeko ethi `(Tuberous Sclerosis)`, kwaye kunye nayo kuza nomngcipheko wokufumana i-LAM. Ngaphezulu kwama-30% abasetyhini abane-`(Tuberous Sclerosis)` bane-LAM.

Enye indlela kukuba utshintsho kwi-gene `(TSC2)` lwenzeka ngokungacwangciswanga, ngaphandle konxibelelwano oluvela kwilifa. Isizathu esichanekileyo soku asaziwa. I-LAM ekhula ngale ndlela ibizwa ngokuba yi-Sporadic LAM. Kule meko, ezinye iimpawu ze-`(Tuberous Sclerosis)` azibonakali.

Ngubani osengozini enkulu yokufumana i-LAM?

Nangona oku kuyamangalisa, i-LAM idla ngokwenzeka kubafazi . Bambalwa amadoda afunyaniswe ene-LAM ukuza kuthi ga ngoku. Inye kuphela indoda exeliweyo ukuba ine-LAM engaqhelekanga.

Kwakhona, i-LAM inokuba yingozi kakhulu ngexesha lokukhulelwa naxa kusetyenziswa amayeza ane-estrogen .

Ziziphi iingxaki ezinokubakho ngenxa ye-LAM?

Eyona ngxaki ixhaphakileyo ye-LAM yi-pneumothorax . Njengokuqhuma kwebhaluni, umoya uyavuza uphuma emiphungeni uze ubangele ukuba imiphunga iwe. Ngaphezulu kwesiqingatha sabasetyhini abane-LAM baya kuba nesinye isiqendu se-pneumothorax ebomini babo. Ngamanye amaxesha oku kunokwenzeka kwakhona. I-LAM idla ngokuchongwa emva kwesi siqendu.

Ezinye iingxaki ziquka:

• Ukuqokelelana kolwelo olubizwa ngokuba yi-chyle olujikeleze imiphunga (chylothorax).
• Ukuqokelelana kolwelo olujikeleze imiphunga (i-pleural effusion).
• Ukuvalwa kwenkqubo ye-lymphatic.

Isifo se-LAM sifunyanwa njani?

Ukuze axilonge i-LAM, ugqirha uza kuqala akuhlole aze akubuze ngeempawu zakho. Emva koko uza kuyalela iimvavanyo kunye novavanyo lomfanekiso ukuze abone oku kulandelayo:

• Jonga indlela esebenza kakuhle ngayo imiphunga yakho.
• Jonga inqanaba leoksijini egazini.
• Jonga naluphi na utshintsho oluvela kwimiphunga.
• Jonga ukuba kukho na inani elingaphezulu kwesiqhelo le "proteni" ezithile egazini.

Ngenxa yokuba iimpawu ze-LAM zifana nezezinye izifo zemiphunga eziqhelekileyo, ngamanye amaxesha kunokuba nzima koogqirha ukuzixilonga.

Zeziphi iimvavanyo ezisetyenziselwa ukufumanisa i-LAM?

Kusenokufuneka wenze uvavanyo olufana nolu:

• Uvavanyo lokusebenza kwemiphunga : Olu vavanyo lujonga ukuba imiphunga yakho ikunceda njani ukuphefumla ngaphakathi nangaphandle.
• Ukujonga inqanaba leoksijini egazini (`(pulse oximetry)`) : Uvavanyo olulula olubandakanya ukuncamathisela into efana nekliphu emnweni wakho.
• Uvavanyo lwemifanekiso : Olu luquka ii-CT scans (ii-Computed Tomography scans), i-MRI (i-Magnetic Resonance Imaging), okanye ii-HRCT (i-High-Resolution Computed Tomography). I-HRCT scan inokuvelisa umfanekiso ocacileyo wendawo encinci kakhulu yemiphunga, nto leyo eyenza kube lula ukufumanisa ezinye izifo zemiphunga.
• Uvavanyo lwe-VEGF-D : Ugqirha wakho unokwenza uvavanyo lwegazi ukujonga usulelo, ukuba amanye amalungu akho asebenza kakuhle kangakanani, kunye namanqanaba akho eproteni ebizwa ngokuba yi-VEGF-D (Vascular Endothelial Growth Factor-D). Abantu abaninzi abane-LAM banamanqanaba aphezulu ale proteni, kodwa ayinguye wonke umntu owenza njalo.
• I-biopsy yemiphunga : Oku kuquka ukuthatha iqhekeza elincinci lesicubu semiphunga size silivavanye kwilebhu. Le sampulu ingathathwa nge-bronchoscopy (inkqubo apho ityhubhu enekhamera idluliselwa emiphungeni) okanye ngotyando lwesifuba oluncediswa yividiyo (VATS).

Isifo se-LAM sinyangwa njani?

Ngelishwa, okwangoku akukho nyango lwe-LAM. Nangona kunjalo, iyeza i -sirolimus (ekwaziwa ngokuba yi-rapamycin, igama lebrendi i-Rapamune®) linokunceda ukuzinzisa isifo kwaye lithintele ukuba singabi mandundu. I-Sirolimus inokunceda ukunciphisa iithumba zezintso (i-angiomyolipomas), ukunciphisa iithumba kunye nokuqokelelana kolwelo kwinkqubo ye-lymphatic, kwaye incede ekunciphiseni iimpawu zakho.

Njengezinye iindlela zonyango:

• Unyango lweoksijini : Kwabo banengxaki yokuphefumla.
• Iibronchodilators ezifuthwayo : Ezi zifana nee-inhaler. Zivula indlela yomoya emiphungeni kwaye zenze kube lula ukuphefumla.
• Ukuvuselelwa kweMiphunga : Le yinkqubo edibanisa umthambo, imfundo, kunye neengcebiso.
• Ukufakelwa imiphunga : Njengendlela yokugqibela kwabo banesifo esiye safikelela kwinqanaba eliphezulu kakhulu.

Masifunde nangemiphumo emibi kunye neengxaki zonyango.

I-Sirolimus ngamanye amaxesha inokubangela umonakalo omkhulu kwizintso kwaye yonyuse umngcipheko wosulelo olukhulu . Ke ngoko, kubaluleke kakhulu ukuxoxa ngezinto ezilungileyo nezingalunganga zokusebenzisa eli yeza nogqirha wakho ngaphambi kokuba wenze isigqibo.

Luhlobo luni lwekamva umntu one-LAM anokulilindela?

I-LAM inokuba mandundu ngokuhamba kwexesha. Isicwangciso sakho sonyango sinokutshintsha kuxhomekeke ekubeni loluphi unyango olukufaneleyo. Uza kusebenza nogqirha wakho wemiphunga (i-pulmonologist) ukuze ubone eyona ndlela ilungileyo yokujonga imeko yakho. Uza kujonga rhoqo ukusebenza kwemiphunga yakho. Isicwangciso sakho sonyango siza kusekelwa koku:

• iminyaka yakho.
• Imeko yakho yokuya exesheni (nokuba ukhe wafikelela kwixesha lokuya exesheni okanye akunjalo).
• Indlela ekhawuleza ngayo ukusebenza kwemiphunga.
• Nokuba izintso zakho okanye inkqubo ye-lymphatic zichaphazelekile.

Isasazeka ngokukhawuleza kangakanani i-LAM?

Indlela ekhawuleza ngayo ukusasazeka kwe-LAM inokwahluka ukusuka kumntu ukuya komnye . Kukho izinto ezininzi ezinokuchaphazela oku:

• Uhlobo lwe-LAM onayo : Nangona abantu abane-`(TSC-LAM)` benomsebenzi ongcono kakhulu wemiphunga kunabo bane-`(Sporadic LAM)`, izinga lokuncipha komsebenzi wemiphunga liyafana kakhulu kuzo zombini.
• Ubudala bakho : Kwabanye abantu, i-LAM ayisasazeki emva kokuya exesheni, xa amanqanaba e-estrogen emzimbeni ehla.
• Indlela osabela ngayo kunyango : Iyeza iSirolimus linokuzinzisa esi sifo kubantu abaninzi.

Kunini apho kufuneka khona ukufakelwa imiphunga?

Abantu abaninzi abane-LAM (malunga nama-64%) banokuphila iminyaka engama-20 nangaphezulu emva kokufunyaniswa ukuba bane-LAM ngaphandle kokufuna ukufakelwa imiphunga. Nangona kunjalo, ngenxa yokuba iiseli ze-LAM aziqali emiphungeni, ii-cysts zisenokukhula nasemva kokufakelwa imiphunga .

Ingakanani ixesha lokuphila nge-LAM? (Ulindelo Lobomi)

Ixesha onokuphila ngalo ne-LAM lixhomekeke kubukhulu besifo kunye nokuba sisasazeka ngokukhawuleza kangakanani. Kodwa, ngokubanzi, imeko ingcono kakhulu ngoku kunangaphambili. Abantu abangaphezu kwama-90% abane-LAM baphila iminyaka eli-10 emva kokuba befunyaniswe benesifo.

Ndingazinyamekela njani?

Thetha nogqirha wakho wezifo zamaphaphu (i-pulmonologist) malunga nento omawuyilindele kunye nendlela yokwenza isicwangciso sokuphila ne-LAM. Bona ugqirha wakho ngexesha, uthathe amayeza akho njengoko kuyalelwe, kwaye uxelele ugqirha wakho ngoko nangoko ukuba uneempawu ezintsha, ukuba iimpawu zakho ziya zisiba mandundu, okanye ukuba uneziphumo ebezingalindelekanga kunyango lwakho.

Ufanele uye nini kugqirha? Ufanele uye nini kunyango olungxamisekileyo?

Ukuba unengxaki yokuphefumla, ukukhwehlela, okanye ezinye iimpawu ezikukhathazayo, nokuba ziyaqhubeka okanye ziyanda, qiniseka ukuba ubona ugqirha.

Ixesha lokuya kwiYunithi yoNyango oluNgxamisekileyo (i-ETU) :

Ukuba uneempawu zokuwa kwemiphunga (pneumothorax) okanye ezinye iimpawu ezinzulu, yiya esibhedlele ngokukhawuleza:

• Ukuba unengxaki yokuphefumla.
• Ukuba unentlungu eqatha esifubeni .
• Ukuba ukhwehlela igazi .
• Ukuba ulusu, imilebe, kunye neenzipho zijika zibe luhlaza okwesibhakabhaka (`(cyanosis)`) .

Yeyiphi imibuzo ebalulekileyo ekufuneka uyibuze ugqirha?

Kunganceda ukubuza ugqirha wakho imibuzo efana nale:

• Ngaba ndine `(I-Sporadic LAM)` okanye `(TSC-LAM)`?
• Ziziphi iindlela zonyango endinazo?
• Ndingayisebenzisa njani amayeza am?
• Zeziphi iimpawu ezintsha okanye eziya ziba mandundu ekufuneka ndizijonge?
• Ngaba ndiza kuyidinga into yokufakelwa imiphunga?

Ngaba i-LAM ngumhlaza?

I-LAM ayisoloko ithathwa njengomhlaza . Nangona kunjalo, ngandlela thile iziphatha njengomhlaza osasazeka ukusuka kwenye indawo emzimbeni ukuya kwenye (umhlaza osasazekayo). Oku kuthetha ukuba iiseli ziqala kwenye indawo kwaye zihamba ngemithambo yegazi kunye nenkqubo ye-lymphatic ukuya emiphungeni nasezintso. Olu hlobo lokukhula ngamanye amaxesha lubizwa ngokuba yi-benign cell metastasis.

Kodwa izazinzulu azikaqiniseki ukuba ezi seli ziqala phi. Kwakhona, ezi thumba azibonakali ngathi zisasazeka kakhulu kwezinye izitho zomzimba, ezifana nesibindi okanye ubuchopho, njengomhlaza. Xa zijongwa ngemakroskopu, ezi seli zibukeka ngathi ziiseli eziqhelekileyo kuneeseli zomhlaza.

Ngenxa yonyango olutsha kunye nokuqonda okwandisiweyo kwe-LAM, abantu ngoku baphila ixesha elide ngaphandle kokufakelwa imiphunga. Kodwa kuthatha ixesha ukuqhelana nale ndlela intsha yokuphila. Ukufunda ngesifo sobomi bonke kunokuvakala ngathi umhlaba ususiwe phantsi kweenyawo zakho. Kodwa iqela lakho lezonyango - kunye neqela lakho lenkxaso - linokukunceda ukuba uphinde ume.

Okokugqibela, yintoni ekufuneka uyikhumbule

I-LAM inokuba sisifo esinzima, kodwa awuwedwa. Ngenxa yenkqubela phambili kwisayensi yezonyango, zininzi iindlela zokulawula esi sifo. Eyona nto ibalulekileyo kukuhlala unethemba, ulandele iingcebiso zikagqirha wakho, kwaye ungalahli ithemba. Thetha nogqirha wakho kunye nabantu obathandayo malunga neenkxalabo zakho kunye noloyiko lwakho. Khumbula, baninzi abantu abanokukunceda kolu hambo.

I- LAM, isifo semiphunga, isifo esingaqhelekanga, ubunzima bokuphefumla, i-sirolimus, ukufakelwa kwemiphunga, utshintsho lwemfuza