Singakhuluma nge-LAM, isifo esithinta ngqo abesifazane? (Lymphangioleiomyomatosis)

Ingabe ngezinye izikhathi uzizwa uphelelwa umoya, ukhathele, noma uzwa ubuhlungu esifubeni? Sivame ukucabanga ngalokhu njengezimpawu zesifo esivamile njenge-asthma. Kodwa-ke, kunesifo samaphaphu esingavamile kakhulu esibangela lezi zimpawu, ikakhulukazi kwabesifazane. Sibizwa ngokuthi i-Lymphangioleiomyomatosis. Igama lide kancane, ngakho-ke sizolibiza ngokuthi "LAM" ngamafuphi. Uma uthola leli gama linzima ukulibiza, cabanga ngalo kanje: "Lymph-ang-ge-o-ly-o-myo-ma-to-sis". Nakuba kuyinkimbinkimbi kancane, kungaba kubaluleke kakhulu ukuthi uqonde lesi sifo ngamagama alula.

Kalula nje, iyini i-LAM?

I-LAM yisimo esingavamile lapho amangqamuzana angajwayelekile ekhula khona ngaphakathi kwamaphaphu akho, akha ama-cyst amancane. Lokhu kubangelwa ukuguquka kwezakhi zofuzo okubangela ukuba amangqamuzana emisipha ebushelelezi emzimbeni wakho akhule ngokungalawuleki. Lokhu kungalimaza amaphaphu akho. Akuyona nje amaphaphu kuphela, kodwa ngezinye izikhathi izinso kanye nohlelo lwe-lymphatic nakho kungathuthukisa lokhu kukhula.

Lesi sifo sivame ukutholakala kwabesifazane abaneminyaka ephakathi kuka-20 no-40. Okusho ukuthi, ngemva kokuthomba nangaphambi kokunqamuka kokuya esikhathini. Ngakho-ke, odokotela bakholelwa ukuthi i-hormone yesifazane i-estrogen idlala indima ekuthuthukisweni kwalesi sifo.

Kunezinhlobo ezimbili eziyinhloko zesifo se-LAM.

Isifo se-LAM singahlukaniswa ngezinhlobo ezimbili eziyinhloko, ngomehluko omncane phakathi kwalezi ezimbili.

Ziyini izimpawu zesifo se-LAM?

Izimpawu ze-LAM zifana kakhulu nezezinye izifo zamaphaphu, njenge-asthma kanye ne-bronchitis. Lokhu kungathatha isikhathi ngezinye izikhathi ukuxilonga. Kubalulekile ukuqaphela lokhu.

• Ubunzima bokuphefumula (ukuphelelwa umoya): Lolu uphawu oluyinhloko noluvame kakhulu. Nakuba ekuqaleni lungase luzwakale kuphela ngokuzikhandla okuncane, lesi simo singaba sibi kakhulu ngokuhamba kwesikhathi.
• Ukuphefumula: Umsindo wokukhala komoya lapho uphefumula, ofana nowomuntu one-asthma.
• Ubuhlungu besifuba: Lobuhlungu bungase buzwakale ikakhulukazi uma uphefumula kakhulu.
• Ukukhwehlela: Kungase kube nokukhwehlela okomile okuqhubekayo.
• Ukukhwehlela igazi noma 'i-chyle': I-Chyle iwuketshezi oluvela ohlelweni lwethu lokugaya ukudla. Inombala ofana nobisi. Uma kwenzeka into efana nale, kungcono ukungalibali nhlobo .

Kungani lokhu kwenzeka? Iyini imbangela?

Kalula nje, isizathu esiyinhloko salokhu izinguquko (ukuguqulwa kwezakhi zofuzo) ezenzeka ezizakhi zofuzo ezimbili emzimbeni wethu ezibizwa ngokuthi i-TSC1 kanye ne-TSC2 . Lezi zakhi zofuzo ezimbili zifana nabaqaphi bomzimba wethu. Umsebenzi wazo oyinhloko ukuvimba amanye amangqamuzana ukuthi angahlukani futhi azalane ngokungadingekile ngaphandle kokulawula. Sibiza lezi zakhi zofuzo ezicindezela isimila.

Cabanga nje ukube bekukhona iphutha kwelinye lala ma-protective genes amabili. Okwenzekayo ukuthi amaseli emisipha ebushelelezi awatholi isignali yokuthi, "Kulungile, sekwanele, yeka ukwahlukana." Ngakho amaseli aqala ukwahlukana ngokungalawuleki. Umphumela uba:

• Ama-cyst akheka emaphashini.
• Ama-tumor (angiomyolipomas) akheka ezinso.
• Ama-cyst akheka ohlelweni lwe-lymphatic.

Ungazuza lesi sakhi sofuzo esingalungile kubazali bakho (esibangela i-tuberous sclerosis), noma ungaba noshintsho kulezi zakhi zofuzo ngokungahleliwe ngesikhathi sokuphila kwakho ngaphandle kwesizathu esibonakalayo (esibangela i-LAM engavamile).

Izinkinga kanye nezingozi zesifo se-LAM

Inkinga evame kakhulu nenkulu ye-LAM ukuwohloka kwamaphaphu . Ngokwezokwelapha, lokhu kubizwa ngokuthi i-pneumothorax .Abesifazane abangaphezu kwengxenye abane-LAM bazobhekana nalesi simo okungenani kanye empilweni yabo. Ngezinye izikhathi singaphinde sibuye. Eqinisweni, abesifazane abaningi baqala ukutholakala bene-LAM ngemva kokuba benomkhuhlane wamaphaphu bese belaliswa esibhedlela.

Ngaphezu kwalokho, kungase kube nezinye izinkinga.

Isifo se-LAM sitholakala kanjani?

Ngemva kokulalela izimpawu zakho, uma udokotela wakho esola lesi sifo, uzoyala ukuhlolwa okuningana ukuze asiqinisekise.

Ukuhlolwa okwenziwa udokotela

• Ukuhlolwa Kokusebenza Kwamaphaphu: Lokhu kuhilela ukuphefumula ngaphakathi nangaphandle ngepayipi elixhunywe emshinini. Lokhu kungalinganisa ukuthi amaphaphu akho asebenza kahle kangakanani nokuthi angabamba umoya ongakanani.
• I-Pulse Oximetry: Idivayisi encane, efana ne-clip, inamathiselwe emunweni wakho futhi ilinganisa inani lomoya-mpilo egazini lakho. Lokhu ukuhlolwa okulula kakhulu futhi okungenabuhlungu.
• Ukuskena Izithombe:
• I-CT Scan (i-Computed Tomography):Lokhu kungathatha izithombe ezinemininingwane ze-3D zamaphaphu akho. Lokhu kungasiza ekuboneni ngokucacile ukuthi kukhona yini ama-cyst aqondene ngqo ne-LAM.
• Ukuskena kwe-HRCT (i-High-Resolution Computed Tomography): Lena inguqulo ethuthukisiwe yokuskena kwe-CT. Kungathatha izithombe ezicacile zengxenye encane kakhulu yamaphaphu. Lokhu kubaluleke kakhulu ekuxilongweni kwe-LAM.
• Ukuhlolwa kwegazi kwe-VEGF-D: I-VEGF-D iyiprotheni etholakala egazini lethu. Abantu abaningi abane-LAM banamazinga aphezulu kakhulu ale protheni egazini labo kunokuvamile. Ngakho-ke lokhu kuhlolwa kwegazi kuyasiza kakhulu ekuxilongeni lesi sifo.
• Ukuhlolwa Kwamaphaphu: Ngezinye izikhathi, uma ezinye izivivinyo zingaqinisekile ngokuphelele, udokotela wakho angase anqume ukuthatha ingxenye encane kakhulu yezicubu emaphashini akho ukuze ihlolwe. Lokhu kungenziwa kusetshenziswa inqubo ebizwa ngokuthi i-bronchoscopy (ithubhu elincane elinekhamera efakwe emigudwini yomoya) noma i-VATS (ukuhlinzwa kwesifuba okusizwa ngevidiyo) .

Yiziphi izindlela zokwelapha isifo se-LAM?

Okokuqala, alikho ikhambi le-LAM okwamanje. Kodwa ungakhathazeki. Manje kunezindlela zokwelapha eziphumelela kakhulu ezingalawula lesi sifo, zinciphise izimpawu, futhi zinciphise ukuqhubeka kwalesi sifo.

Ukwelashwa okuyinhloko umuthi obizwa ngokuthi i-sirolimus . Ubizwa nangokuthi i-rapamycin. Lo muthi usebenza ngokuvimbela ukukhula kwamaseli akhula ngokungalawuleki. Lokhu kungasiza ekulawuleni ukuwohloka okuqhubekayo kwesifo ngezinga elikhulu. Kungasiza futhi ekunciphiseni izimila ezinso kanye nohlelo lwe-lymphatic futhi kunciphise izimpawu.

Ngaphezu kwalokho, udokotela wakho angase ancome ezinye izindlela zokwelapha kuye ngesimo sakho.

• Ukwelashwa Nge-Oxygen: Uma izinga le-oxygen egazini liphansi, kuhlinzekwa amathangi e-oxygen ukuze asetshenziswe ekhaya.
• Ama-bronchodilator aphefumulayo: Le mithi, enikezwa nge-inhaler, inganciphisa ubunzima bokuphefumula.
• Ukuvuselelwa Kwemiphunga: Lokhu kufana nohlelo olukhethekile lokuzivocavoca lwamaphaphu. Kusiza ekuthuthukiseni ikhwalithi yempilo yakho ngokuzivocavoca kokuphefumula kanye nokuzivocavoca okulungele umzimba.
• Ukufakelwa Kwamaphaphu:Uma lesi sifo sesiqhubeke kakhulu futhi singenakukwazi ukulawulwa nganoma yiluphi olunye ukwelashwa, ukufakelwa amaphaphu kungaba yindlela yokugcina.

Lesi sifo sithuthuka ngokushesha kangakanani?

Lokhu kuyahlukahluka kumuntu nomuntu. Izinga lokuthuthuka kwe-LAM lincike ezintweni eziningana.

• Uhlobo lwe-LAM onalo: Abantu abane-LAM engavamile bangase babe nokusebenza kwamaphaphu okuphansi kancane kunalabo abane-TSC-LAM.
• Ubudala bakho: Kwabanye besifazane, ngemva kokunqamuka kokuya esikhathini, ukuqhubeka kwalesi sifo kuyayeka noma kuyehla njengoba amazinga e-estrogen emzimbeni ehla.
• Impendulo yakho ekwelashweni: Abantu abaningi bangasilawula kahle lesi sifo ngemithi efana neSirolimus.

Isimo sesingcono kakhulu manje kunangesikhathi esidlule. Ngezindlela zokwelapha zesimanje, abantu abangaphezu kuka-90% abane-LAM bayaphila eminyakeni eyi-10 ngemva kokuxilongwa. Abantu abaningi (cishe abangu-64%) bangaphila iminyaka engama-20 noma ngaphezulu ngaphandle kokudinga ukufakelwa amaphaphu.

Isikhathi sokubona udokotela wakho bese uya ku-ETU

Uma uhlala ne-LAM, kubaluleke kakhulu ukwazi ukuthi kufanele ufune nini iseluleko sezokwelapha nokuthi kufanele uye nini eMnyangweni Wezimo Eziphuthumayo (i-ETU).

Abanye abantu bayabuza ukuthi i-LAM ingumdlavuza yini. I-LAM ngokuvamile ayibhekwa njengomdlavuza. Kodwa-ke, ngezindlela ezithile, njengendlela amangqamuzana avela ngayo kwenye indawo aya emaphashini, anezici ezifana nomdlavuza. Kodwa-ke, uma ebhekwa nge-microscope, lawa maseli awabonakali njengamaseli omdlavuza.

Kuvamile ukuzizwa ushaqekile futhi wesaba lapho uthola ukuthi unesifo esisongela ukuphila njengalesi. Kodwa khumbula, awuwedwa. Ngokusekelwa odokotela bakho, umndeni wakho, nabangane bakho, uzothola amandla okubhekana nale nselele.