Ingabe unalezi zinkinga ngamaphaphu akho? Ake sixoxe nge-LAM (Lymphangioleiomyomatosis)

Wake wezwa ngalesi sifo esinegama elide elibizwa ngokuthi i-LAM (lymphangioleiomyomatosis)? Ungase uthole igama lingavamile. Kodwa yisifo esingavamile esithinta amaphaphu. Namuhla, sizokhuluma ngaso kalula, ngendlela ongayiqonda. Ungakhathazeki, sizochaza konke.

Iyini ngempela i-LAM (Lymphangioleiomyomatosis)?

Kalula nje, i-LAM yisifo esingavamile esibangela ukwakheka kwama-cyst agcwele uketshezi emaphashini akho. Lokhu kungalimaza amaphaphu akho. Ngezinye izikhathi kungabangela nama-cyst ezinso zakho kanye nohlelo lwe-lymphatic. Cabanga ukuthi bekuzokwenzekani uma amangqamuzana emisipha ebushelelezi emzimbeni wakho eqala ukukhula ngokungazelelwe engalawuleki. Yilokho okwenzekayo lapha. Kubangelwa ukuguquka kwezakhi zofuzo.

Lesi simo, esibizwa ngokuthi i-LAM (esibizwa ngokuthi “limf-AN-gee-oh-ly-oh-my-oh-muh-TOH-sis”) , sithinta kakhulu abesifazane . Ngokuvamile sithinta abesifazane abaneminyaka ephakathi kuka-20 no-40, noma abesifazane asebedlule esikhathini sokuthomba futhi abasondela esikhathini sokunqamuka kokuya esikhathini. Ngenxa yalokhu, odokotela bacabanga ukuthi i-hormone i-estrogen noma ukuba khona kwesibeletho kungase kube nomthelela ekukhuleni kwalezi zimila.

Yiziphi izinhlobo eziyinhloko ze-LAM?

Kunezinhlobo ezimbili eziyinhloko ze-LAM:

1. I-TSC-LAM: Abanye besifazane banesifo sofuzo esibizwa ngokuthi ``Tuberous Sclerosis Complex - TSC''. Uma bethola i-LAM, ibizwa ngokuthi i-TSC-LAM.

2. I-Sporadic LAM: Lokhu kwenzeka ngokungahleliwe, okusho ukuthi ukuguquka kwezakhi zofuzo kwenzeka ngokungahleliwe ngesikhathi sokuphila. Into ebalulekile ukuthi lolu hlobo lwe-Sporadic LAM lungadluliselwa ezinganeni zakho.

Sivame kangakanani lesi sifo esibizwa ngokuthi i-LAM?

I-LAM empeleni iyisifo esingavamile kakhulu . Kulinganiselwa ukuthi cishe owesifazane oyedwa kwabayi-140,000 angaba nalesi sifo. Kodwa-ke, phakathi kuka-30% no-80% wabesifazane abane-"Tuberous Sclerosis" bangaba ne-LAM.

Ziyini izimpawu zesifo se-LAM?

Umuntu one-LAM angase abe nezimpawu ezifana nalezi:

• Ubunzima bokuphefumula (ukuphelelwa umoya) : Ngezinye izikhathi lokhu kunganda kancane kancane.
• Ukukhala komoya umsindo wokukhala komoya ovela esifubeni .
• Ubuhlungu besifuba .
• Ukukhwehlela .
• Ngezinye izikhathi kuba khona igazi noma i-chyle (`(chyle)` - uketshezi lwe-lymphatic oluvela ohlelweni lokugaya ukudla) oluphuma ne-mucus .

Ungacabangi ukuthi une-LAM ngoba nje unalezi zimpawu, njengoba zingaba yizimpawu zezinye izifo zamaphaphu. Ngakho-ke, kubalulekile ukufuna iseluleko sezokwelapha.

Kungani lokhu kwenzeka? Iyini imbangela?

Imbangela eyinhloko ye-LAM ukuguqulwa kwezakhi zofuzo ezimbili emzimbeni wethu ezibizwa ngokuthi `(TSC1)` kanye `(TSC2)`. Lezi zakhi zofuzo ezimbili zifana nezakhi zofuzo ezivimbela ukukhula kwezinto ezifana namangqamuzana omdlavuza emzimbeni wethu ngokungadingekile. Ngakho-ke, uma kukhona iphutha kunoma yiliphi lala ma-gene amabili, lawo maseli emisipha ebushelelezi engikhulume ngawo ekuqaleni aqala ukukhula ngokungalawuleki. Ngenxa yalokho, yilokhu:

• Izimila zamaphaphu (i-pulmonary lymphangioleiomyomatosis) ziyavela.
• Ama-tumor (angiomyolipomas) akheka ezinso.
• Ama-cyst akheka ohlelweni lwe-lymphatic.

Umuntu uthola kanjani i-LAM?

Kunezindlela ezimbili umuntu angathuthukisa ngazo i-LAM. Enye iwukuthola ifa lesakhi sofuzo esingalungile kumzali onesici ku-`(TSC1)` noma `(TSC2)`. Leli fa liphumela esimweni `(Tuberous Sclerosis)`, futhi lihambisana nengozi yokuthola i-LAM. Abesifazane abangaphezu kuka-30% abane-`(Tuberous Sclerosis)` bane-LAM.

Enye indlela ukuthi ukuguquka kwezakhi zofuzo ze-`(TSC2)` kwenzeka ngokuzenzakalelayo, ngaphandle kokuxhumana okuzuzwe njengefa. Imbangela eqondile yalokhu ayaziwa. I-LAM ekhula ngale ndlela ibizwa ngokuthi i-Sporadic LAM. Kulokhu, ezinye izimpawu ze-`(Tuberous Sclerosis)` azibonakali.

Ubani osengozini enkulu yokuthola i-LAM?

Nakuba lokhu kumangalisa, i-LAM ivame ukwenzeka kwabesifazane . Bambalwa kakhulu abesilisa asebetholakale benesifo se-LAM kuze kube manje. Kubikwe ukuthi indoda eyodwa kuphela ine-LAM engavamile.

Futhi, i-LAM ingaba nzima kakhulu ngesikhathi sokukhulelwa nalapho kusetshenziswa imithi equkethe i-estrogen .

Yiziphi izinkinga ezingaba khona ngenxa ye-LAM?

Inkinga evame kakhulu ye-LAM yi-pneumothorax . Njengokuqhuma kwebhaluni, umoya uyavuza emaphashini bese ubangela ukuba amaphaphu awe. Abesifazane abangaphezu kwengxenye abane-LAM bazoba nesiqephu esisodwa se-pneumothorax empilweni yabo. Ngezinye izikhathi lokhu kungenzeka kaningi. I-LAM ivame ukutholakala ngemva kwalesi siqephu.

Ezinye izinkinga zifaka:

• Ukuqongelela koketshezi olubizwa ngokuthi i-chyle emaphashini (chylothorax).
• Ukuqongelela koketshezi emaphashini (i-pleural effusion).
• Ukuvinjelwa kohlelo lwe-lymphatic.

Isifo se-LAM sitholakala kanjani?

Ukuze kuhlolwe i-LAM, udokotela uzoqala akuhlole bese ebuza ngezimpawu zakho. Bazobe sebeyala ukuhlolwa nokuhlolwa kwezithombe ukuze kutholakale okulandelayo:

• Bona ukuthi amaphaphu akho asebenza kahle kangakanani.
• Hlola izinga lomoya-mpilo egazini.
• Hlola noma yiziphi izinguquko emaphashini.
• Hlola ukuthi kukhona yini inani elingaphezu kwejwayelekile "lamaprotheni" athile egazini.

Ngenxa yokuthi izimpawu ze-LAM zifana nezezinye izifo zamaphaphu ezivamile, ngezinye izikhathi kungaba nzima ngodokotela ukuzixilonga.

Yiziphi izivivinyo ezisetshenziswa ukuxilonga i-LAM?

Kungadingeka wenze izivivinyo ezinjengalezi:

• Ukuhlolwa kokusebenza kwamaphaphu : Lokhu kuhlola ukuthi amaphaphu akho akusiza kanjani ukuthi uphefumulele ngaphakathi nangaphandle.
• Ukuhlola izinga lomoya-mpilo egazini (`(pulse oximetry)`) : Ukuhlolwa okulula okuhilela ukunamathisela into efana ne-clip emunweni wakho.
• Ukuhlolwa kokuthatha izithombe : Lokhu kufaka phakathi ukuskena kwe-CT (i-Computed Tomography scans), i-MRI (i-Magnetic Resonance Imaging), noma i-HRCT (i-High-Resolution Computed Tomography). Ukuskena kwe-HRCT kungaveza isithombe esicacile ngisho nendawo encane kakhulu yamaphaphu, okwenza kube lula ukuthola ezinye izifo zamaphaphu.
• Ukuhlolwa kwe-VEGF-D : Udokotela wakho angase ahlole igazi ukuze ahlole ukutheleleka, ukuthi ezinye izitho zakho zisebenza kahle kangakanani, kanye namazinga akho ephrotheni ebizwa ngokuthi i-VEGF-D (Vascular Endothelial Growth Factor-D). Abantu abaningi abane-LAM banamazinga aphezulu ale phrotheni, kodwa akuwona wonke umuntu onawo.
• I-biopsy yamaphaphu : Lokhu kuhilela ukuthatha ingxenye encane yezicubu emaphashini bese uyihlola elabhorethri. Le sampula ingathathwa nge-bronchoscopy (inqubo lapho ipayipi elinekhamera lidluliselwa khona emaphashini) noma ngokuhlinzwa kwesifuba okusizwa ngevidiyo (i-VATS).

Isifo se-LAM selashwa kanjani?

Ngeshwa, okwamanje ayikho ikhambi le-LAM. Kodwa-ke, umuthi i-sirolimus (owaziwa nangokuthi i-rapamycin, igama lomkhiqizo i-Rapamune®) ungasiza ekuqiniseni lesi sifo futhi uvimbele ukuthi singabi sibi kakhulu. I-Sirolimus ingasiza ekunciphiseni izimila zezinso (i-angiomyolipomas), ukunciphisa izimila kanye nokuqongelela koketshezi ohlelweni lwe-lymphatic, futhi kusize ekunciphiseni izimpawu zakho.

Njengezinye izindlela zokwelapha:

• Ukwelashwa nge-oxygen : Kulabo abanenkinga yokuphefumula.
• Ama-bronchodilator aphefumulayo : Lawa afana nama-inhaler. Avula imigudu yokuphefumula emaphashini futhi enze kube lula ukuphefumula.
• Ukuvuselelwa kwamaphaphu : Lolu uhlelo oluhlanganisa ukuzivocavoca, imfundo, kanye nokwelulekwa.
• Ukufakelwa amaphaphu : Njengendlela yokugcina kulabo abanesifo esiye safinyelela esigabeni esiphambili kakhulu.

Ake sifunde nangemiphumela emibi kanye nezinkinga zokwelashwa.

I-Sirolimus ngezinye izikhathi ingabangela ukulimala okukhulu kwezinso futhi yandise ingozi yokutheleleka okukhulu . Ngakho-ke, kubaluleke kakhulu ukuxoxa ngezinzuzo kanye nokubi kokusebenzisa lo muthi nodokotela wakho ngaphambi kokwenza isinqumo.

Hlobo luni lwekusasa umuntu one-LAM angalulindela?

I-LAM ingaba yimbi kakhulu ngokuhamba kwesikhathi. Uhlelo lwakho lokwelashwa lungashintsha kuye ngokuthi yikuphi ukwelashwa okufanele wena. Uzosebenzisana nodokotela wakho wamaphaphu (udokotela wamaphaphu) ukuze uthole indlela engcono kakhulu yokuqapha isimo sakho. Uzohlola njalo ukusebenza kwamaphaphu akho. Uhlelo lwakho lokwelashwa luzosekelwe ku:

• iminyaka yakho.
• Isimo sakho sokunqamuka kokuya esikhathini (kungakhathaliseki ukuthi usudlule esikhathini sokunqamuka kokuya esikhathini noma cha).
• Indlela ukusebenza kwamaphaphu okwehla ngayo ngokushesha.
• Kungakhathaliseki ukuthi izinso zakho noma uhlelo lwakho lwe-lymphatic luthintekile.

I-LAM isakazeka ngokushesha kangakanani?

Ukuthi ukusabalala kwe-LAM kungashesha kangakanani kumuntu nomuntu . Kunezici eziningana ezingathinta lokhu:

• Uhlobo lwe-LAM onalo : Nakuba abantu abane-`(TSC-LAM)` benomsebenzi wamaphaphu ongcono kakhulu kunalabo abane-`(Sporadic LAM)`, izinga lokwehla komsebenzi wamaphaphu lifana kakhulu kuzo zombili.
• Ubudala bakho : Kwabanye abantu, i-LAM ayisabalali ngemva kokunqamuka kokuya esikhathini, lapho amazinga e-estrogen emzimbeni ehla.
• Indlela osabela ngayo ekwelashweni : Umuthi i-sirolimus ungasiqinisa lesi sifo kubantu abaningi.

Kunini lapho kudingeka khona ukufakelwa amaphaphu?

Abantu abaningi abane-LAM (cishe ama-64%) bangaphila iminyaka engama-20 noma ngaphezulu ngemva kokuxilongwa ngaphandle kokudinga ukufakelwa amaphaphu. Kodwa-ke, ngenxa yokuthi amaseli e-LAM awaqali emaphashini, ama-cyst asengakhula ngisho nangemva kokufakelwa amaphaphu .

Iyini isikhathi sokuphila nge-LAM? (Ukulindeleke Kokuphila)

Ukuthi ungaphila isikhathi esingakanani ne-LAM kuncike ebunzimeni besifo nokuthi sisakazeka ngokushesha kangakanani. Kodwa, ngokuvamile, isimo singcono kakhulu manje kunangaphambili. Abantu abangaphezu kuka-90% abane-LAM baphila iminyaka eyi-10 ngemva kokuxilongwa.

Ngizinakekela kanjani?

Khuluma nodokotela wakho wezifo zamaphaphu (i-pulmonologist) ngalokho ongakulindela nokuthi ungalwenza kanjani uhlelo lokuphila ne-LAM. Bonana nodokotela wakho ngesikhathi, thatha imithi yakho njengoba uyalelwe, bese utshela udokotela wakho ngokushesha uma unezimpawu ezintsha, uma izimpawu zakho ziba zimbi kakhulu, noma uma unemiphumela emibi evela ekwelashweni kwakho.

Kufanele uye nini kudokotela? Kufanele uye nini esimeni esiphuthumayo?

Uma unenkinga yokuphefumula, ukukhwehlela, noma ezinye izimpawu ezikhathazayo, kungakhathaliseki ukuthi ziyaqhubeka noma ziya ziba zimbi kakhulu, qiniseka ukuthi ubona udokotela.

Nini ukuya e-Emergency Treatment Unit (ETU) :

Uma unezimpawu zokuwa kwamaphaphu (i-pneumothorax) noma ezinye izimpawu ezimbi, iya esibhedlela ngokushesha:

• Uma unenkinga yokuphefumula.
• Uma unezinhlungu zesifuba ezinzima .
• Uma ukhwehlela igazi .
• Uma isikhumba, izindebe, nezinzipho ziphenduka zibe luhlaza okwesibhakabhaka (`(cyanosis)`) .

Yimiphi imibuzo ebalulekile okufanele uyibuze udokotela?

Kungaba usizo ukubuza udokotela wakho imibuzo efana nale:

• Ingabe ngine-`(I-Sporadic LAM)` noma `(TSC-LAM)`?
• Yiziphi izindlela zokwelapha enginazo?
• Kufanele ngiyisebenzise kanjani imithi yami?
• Yiziphi izimpawu ezintsha noma eziya ziba zimbi okufanele ngiziqaphele?
• Ingabe ngizodinga ukufakelwa amaphaphu?

Ingabe i-LAM ingumdlavuza?

I-LAM ngokuvamile ayibhekwa njengomdlavuza . Kodwa-ke, ngandlela-thile iziphatha njengomdlavuza osakazeka kusuka endaweni eyodwa emzimbeni uye kwenye (umdlavuza we-metastatic). Lokhu kusho ukuthi amaseli aqala endaweni eyodwa futhi ahamba ngemithambo yegazi kanye nohlelo lwe-lymphatic aye emaphashini nasezinso. Lolu hlobo lokukhula ngezinye izikhathi lubizwa ngokuthi i-benign cell metastasis.

Kodwa ososayensi abakaqiniseki ukuthi la maseli aqala kuphi. Futhi, la mathumba abonakala engasabalali kabanzi kwezinye izitho zomzimba, njengesibindi noma ubuchopho, njengomdlavuza. Uma ebhekwa nge-microscope, la maseli abukeka njengamaseli ajwayelekile kunamaseli omdlavuza.

Ngenxa yokwelashwa okusha kanye nokuqonda okwengeziwe nge-LAM, abantu manje sebephila isikhathi eside ngaphandle kokufakelwa amaphaphu. Kodwa kuthatha isikhathi ukujwayela le ndlela entsha yokuphila. Ukufunda ngesifo sokuphila konke kungase kuzwakale sengathi umhlabathi usususiwe ngaphansi kwezinyawo zakho. Kodwa ithimba lakho lezokwelapha - kanye nethimba lakho lokusekela - lingakusiza ukuthi ubuyele esimweni sakho.

Okokugcina, okufanele ukukhumbule

I-LAM ingaba yisifo esinzima, kodwa awuwedwa. Ngokuthuthuka kwesayensi yezokwelapha, kunezindlela eziningi zokuphatha lesi sifo. Into ebaluleke kakhulu ukuhlala unethemba, ulandele iseluleko sikadokotela wakho, futhi ungalilahli ithemba. Khuluma nodokotela wakho kanye nabathandekayo bakho ngezinto ezikukhathazayo kanye nokwesaba kwakho. Khumbula, baningi abantu abangakusiza kulolu hambo.

` I-LAM, isifo samaphaphu, isifo esingavamile, ubunzima bokuphefumula, i-sirolimus, ukufakelwa kwamaphaphu, ukuguqulwa kwezakhi zofuzo