Ingabe ukhona emndenini wakho, ikakhulukazi esemncane, oke waba nomdlavuza wamathumbu amakhulu? Noma ingabe udokotela ukutshelile ukuthi unama-polyp amaningi kumathumbu amakhulu? Mhlawumbe lokhu kungenxa yesimo sofuzo esitholakala emindenini. Namuhla sizokhuluma ngesinye isimo esingavamile kodwa esibaluleke kakhulu. Yi-FAP, noma i-Familial Adenomatous Polyposis. Nakuba igama liyinkimbinkimbi kancane, masiliqonde kalula.
Kalula nje, iyini i-FAP?
I-FAP yisimo sofuzo esingadluliselwa kusuka esizukulwaneni kuya kwesinye. Abantu abanalesi simo bahlakulela inani elikhulu lamathumba abizwa ngokuthi ama-adenoma , angakhula abe umdlavuza, emathunjini abo kanye nase-rectum.
Manje ungase ucabange, "Ingabe kuvamile ukuba ne-cyst emathunjini akho?" Yebo, kuvamile ukuthi abanye abantu bakhulele eyodwa noma ezimbili zalezi cyst njengoba bekhula. Kodwa umuntu one-FAP angakhula amakhulu, mhlawumbe ngisho nezinkulungwane, zalezi cyst. Futhi kuqala esemncane kakhulu, mhlawumbe eneminyaka engu-15 noma engu-16 ubudala.
La ma-polyp awabi nomdlavuza ekuqaleni. Kodwa abizwa ngokuthi 'angaphambi komdlavuza'. Lokhu kusho ukuthi uma engelashwa, kunengozi enkulu yokuthi elilodwa noma amaningi ala ma-polyp ekugcineni aphenduke umdlavuza we-colonectal .
Cabanga ukuthi kungaba nzima kangakanani ukususa amakhulu ala mathumba ngalinye ngalinye. Cishe akunakwenzeka. Yingakho, ukuze kulawulwe le ngozi enkulu, odokotela batusa ukususwa kwamathumbu amakhulu ngokuhlinzwa, i-colectomy ephelele . Ngezinye izikhathi, i-rectum nayo iyasuswa (proctocolectomy).
Ngaphandle kwalokhu kuhlinzwa, abantu abaningi abane-FAP cishe bazoba nomdlavuza lapho sebekhulile. Futhi, abantu abane-FAP basengozini yokuthola izimila hhayi emathunjini kuphela kodwa nakwezinye izingxenye zomzimba (isibonelo, esiswini, emathunjini amancane, esikhumbeni, nasemathanjeni). Ngakho-ke, ngisho nangemva kokususwa kwamathunjini, bazodinga ukuhlolwa njalo kwezokwelapha kukho konke ukuphila kwabo.
Iyini ingozi yokuba nomdlavuza nge-FAP?
Uma ingelashwa, i-FAP inethuba elicishe libe yi-100% lokuthola umdlavuza wamathumbu amakhulu. Lesi yisimo esibi kakhulu. Abantu abane-FAP bathola umdlavuza besebancane kakhulu futhi ngesivinini esisheshayo kunomuntu ojwayelekile. Ngakho-ke, uma wazi ukuthi kunomlando womndeni we-FAP, ingane yakho kufanele ihlolwe i-colonoscopy njalo ngonyaka kusukela eneminyaka eyi-10.
Ngaphezu komdlavuza wamathumbu amakhulu, abantu abane-FAP nabo basengozini yokuthola ezinye izinhlobo zomdlavuza.
| Uhlobo lomdlavuza | Ingozi yokwenzeka ( ngengozi ) |
|---|---|
| Umdlavuza we-Duodenal | ~8% |
| Umdlavuza we-thyroid we-papillary | ~2% |
| Umdlavuza we-pancreatic | ~2% |
| Umdlavuza wesibindi (i-Hepatoblastoma) - ikakhulukazi ezinganeni | ~1.5% |
| Umdlavuza wesisu | ~1% |
| Izimila zobuchopho nomgogodla | Ngaphansi kuka-1% |
Ingabe kunezinhlobo ezahlukene zama-FAP?
Yebo, kukhona uhlobo oluyinhloko lwe-FAP kanye nezinhlobo eziningana ezincane ezihlukile kancane futhi ezingabi nzima kangako. Ake sibone ukuthi ziyini.
| Uhlobo lwe-FAP | Incazelo |
|---|---|
| I-FAP Yakudala (i-FAP Yakudala) | Lolu uhlobo oluvame kakhulu. Ama-polyp angaphezu kwe-100, mhlawumbe izinkulungwane, akhula emathunjini amakhulu. |
| I-Attenuated FAP (AFAP) | Lokhu akukubi kangako. Inani lama-cyst akhula emathunjini liphakathi kuka-20 no-100. |
| Isifo sikaGardner | Lokhu kufana ne-Classic FAP, okusho ukuthi kuvela izimila ezingaphezu kwe-100. Ngaphezu kwalokho, ezinye izinhlobo zezimila nazo ziyavela kwezinye izingxenye zomzimba, isibonelo, esikhumbeni, ezicutshini ezithambile, nasemathanjeni. |
| I-Turcot syndrome | Lokhu kubangela ukwakheka kwezimila eziningi emathunjini, kanye nokwakheka kwesimila somdlavuza ebuchosheni. |
Uyini umehluko phakathi kwe-FAP ne-Lynch Syndrome?
I-Lynch syndrome ingenye inkinga yezakhi zofuzo engadluliselwa ezizukulwaneni ngezizukulwane futhi yandise ingozi yomdlavuza wamathumbu amakhulu. Kodwa kukhona umehluko omkhulu phakathi kwalezi ezimbili. Abantu abane-Lynch syndrome abakhi amakhulu ama-polyp, njengoba benza ku-FAP. Ngezinye izikhathi, ama-polyp elilodwa noma amabili ayakhula, futhi angaba umdlavuza ngokushesha. Yingakho i-Lynch syndrome ngezinye izikhathi ibizwa ngokuthi isimo "esingeyona i-polyposis". Zombili lezi zimo zibangelwa ukukhubazeka kwezakhi zofuzo ezahlukene.
Ziyini izimpawu ze-FAP?
Ku-FAP, izimila zamathumbu amakhulu ziqala ukukhula kusenesikhathi kakhulu kunakubantu abaningi, ngokuvamile zisencane. Lezi zimila ngokuvamile azibangeli zimpawu kuze kube yilapho sezinkulu ngendlela eyingozi. Yingakho ukuhlolwa kubaluleke kangaka.
Kodwa-ke, ngenxa yokuthi inani lama-cyst likhulu kakhulu futhi akhula ngokushesha, ngezinye izikhathi izimpawu zingavela. Izimpawu ezinjalo zifaka:
- Ukopha kwegazi endulo
- Uhudo oluqhubekayo
- Ubuhlungu besisu obungapheli
Ngaphezu kwalokhu, abantu abane-FAP bangase babe nezimpawu zangaphandle udokotela angazibona kalula.
- Ama-Dermatofibromas: Amaqhubu aqinile, afana nezibazi akha ngaphansi kwesikhumba.
- Ama-cyst e-Epidermal: Amaqhubu ayisiyingi agcwele i-keratin eyakheka ngaphansi kwesikhumba.
- Ama-Osteoma:Izimila ezingezona umdlavuza ezakheka emathanjeni. Lezi zivame ukubonakala ekhanda noma ethangeni.
- Amazinyo engeziwe noma amazinyo athintekile: Lokhu kungatholakala nge-X-ray yamazinyo.
- Amabala e-retinal anombala (CHRPE): Lawa angabonakala ngesikhathi sokuhlolwa kwamehlo. Kodwa-ke, ngokuvamile awathinti umbono.
Iyini imbangela eyinhloko ye-FAP?
Njengoba sishilo ngaphambili, i-FAP ibangelwa ukuguquka kwezakhi zofuzo. Izakhi zofuzo ezithinta lokhu zibizwa ngokuthi i-APC (Adenomatous Polyposis Coli) .
Konke emzimbeni wethu kulawulwa izakhi zofuzo, njengohlelo lwekhompyutha. Le gene ye-APC 'iyizakhi zofuzo ezicindezela ithumba'. Okusho ukuthi, umsebenzi oyinhloko walesi sakhi sofuzo ukuvimba amangqamuzana ukuthi angahlukani ngokungalawuleki futhi akhe izimila. Kufana namabhuleki emotweni.
Umuntu one-FAP une-gene ye-APC eguqukile, okusho ukuthi inephutha. Bese kuthi, njengemoto enamabhuleki aphukile, amangqamuzana ahlukane ngokungalawuleki futhi akhe amakhulu amathumba.
Lesi sici sofuzo sizuzwe njengefa. Uma omunye wabazali enalesi sici sofuzo se-APC, ingane inethuba elingu-50% lokusizuza njengefa . Lokhu kusho ukuthi ingane ngayinye ingase ibe naso noma ingasitholi. Kodwa-ke, cishe abantu abangu-30% abatholakale bene-FAP abanawo umlando womndeni. Lokhu kusho ukuthi lesi sici sofuzo sivele emizimbeni yabo muva nje (ukuguqulwa kokuqala).
Yiziphi izinkinga ezingaba khona ze-FAP?
Inkinga ebaluleke kakhulu neyingozi okufanele uyiphathe nge-FAP umdlavuza wamathumbu amakhulu . Le ngozi incipha kakhulu uma i-colon isuswa ngokuhlinzwa. Kodwa-ke, kunezinkinga ezithile zokuphila ngaphandle kwe-colon. Inqubo yokuhamba kwamathumbu iyashintshwa. Kulokhu, kungasetshenziswa isikhwama se-ileostomy noma isikhwama se-ileal .
Ngenxa yokuthi isici sezakhi zofuzo ze-APC sikhona kuwo wonke amaseli emzimbeni, izimila zingakheka nangaphandle kwamathumbu. Ezinye zazo zinobungozi bokuba nomdlavuza.
- Ama-polyp e-Duodenal: Lawa akhula cishe kuwo wonke umuntu one-FAP. Inani elincane lawo lingakhula libe umdlavuza we-duodenum, i-bile duct, noma i-pancreatic duct.
- Ama-polyp esisu: Atholakala kubantu abangaba ngu-90%, kodwa amaphesenti amancane kuphela, cishe angu-1%, aphenduka umdlavuza.
- Izimila ezifile: Lezi aziwona umdlavuza. Kodwa zingaba nolaka kakhulu futhi zingasakazekela ezithweni nasemithanjeni yegazi eseduze, zilimaze. Kunzima ukuzisusa futhi zingabuya.
- Umdlavuza we-thyroid: Lokhu kungenzeka kubantu abangaba ngu-2% abane-FAP. Lokhu kuvame ukuphulukiswa ngokuphelele.
- Umdlavuza wesibindi: i-hepatoblastoma, ikakhulukazi ezinganeni ezine-FAPKukhona ingozi encane yokuba nohlobo olungavamile lomdlavuza wesibindi olubizwa ngokuthi.
Iphathwa futhi iphathwa kanjani?
Uhlelo lokwelapha i-FAP lungahlukaniswa izingxenye ezimbili: ukuhlinzwa kanye nokuhlolwa kokuphila konke.
1. Ukuhlinzwa
Iningi labantu abane-FAP yakudala kuzodingeka bahlinzwe i -colectomy ephelele, okuhilela ukususa ikholoni lonke, lapho beneminyaka engu-20 noma ekuqaleni kweminyaka engu-30. Udokotela wakho uzonquma ukuthi uzokwenze nini futhi kanjani lokhu kuhlinzwa, ngokusekelwe esimweni sakho.
2. Ukuhlolwa njalo
Kuzodingeka uhlolwe ngezindlela ezahlukene ngaphambi nangemva kokuhlinzwa, kanye nempilo yakho yonke. Lena yindlela engcono kakhulu yokuvikela impilo yakho.
| Uhlobo lokuhlola | Inhloso | Isikhathi esinconywayo ngokuvamile |
|---|---|---|
| I-Colonoscopy | Ukuhlola izimila emathunjini amakhulu kanye nasemqaleni. | Kulabo abasengozini, njalo ngonyaka kusukela eminyakeni eyi-10 kuze kube yilapho behlinzwa. |
| I-Sigmoidoscopy | Ukuhlola isikhwama esisele se-rectum noma se-ileal ngemuva kokuhlinzwa. | Ngemva kokuhlinzwa, njalo ezinyangeni ezingu-6-12 noma njalo eminyakeni engu-1-4. |
| I-endoscopy ephezulu | Ukuhlola izimila esiswini nasemathunjini amancane (ikholoni). | Ngezikhathi ezivamile njengoba kunconywe udokotela. |
| Ukuskena nge-ultrasound | Ngomdlavuza we-thyroid noma wesibindi. | Ngeseluleko sezokwelapha. |
| Iskeni se-CT noma se-MRI | Ukuhlola izimila ezibangelwa yi-desmoid. | Ngeseluleko sezokwelapha. |
Impilo injani nge-FAP?
Kuvamile ukuzizwa udabuke futhi ushaqekile uma uthola ukuthi unesifo sofuzo esinjengalesi. Kodwa-ke, ukwazi ngalesi simo kusenesikhathi kuyindlela engcono kakhulu yokulawula ingozi yakho yomdlavuza.
Ngokunakekelwa okufanele kwezokwelapha nokuhlolwa njalo, umuntu one-FAP angaphila impilo evamile, ende, nenempilo . Ngemva kokususwa kwekholoni, ingozi enkulu ivela kwezinye izinhlobo zomdlavuza wendlela yokugaya ukudla noma izimila ezibuhlungu. Kodwa lezi azivamile kakhulu kunomdlavuza wekholoni.
Nakuba ukuhlinzwa kuyinguquko enkulu empilweni, ngobuchwepheshe obuthuthukisiwe banamuhla, ukuhlinzwa kwe-laparoscopic kungakusiza ukuthi ululame ngokushesha.
Into ebaluleke kakhulu ukwazi ukuthi awuwedwa. Kukhona odokotela, umndeni, nabangani abangakuqondisa futhi bakusekele kulolu hambo. Ngakho-ke, uma unemibuzo noma ukukhathazeka, khuluma nodokotela wakho ngakho ngokukhululekile.
Umlayezo Wokuya Nawe Ekhaya
- I-FAP yisifo esibi kakhulu esidluliselwa ngezizukulwane futhi sibangela ukwakheka kwamakhulu ezimila emathunjini.
- Uma ingelashwa, ingozi yokuba nomdlavuza wamathumbu icishe ibe yi-100%.
- Uma othile emndenini wakho eke waba nomdlavuza wamathumbu amakhulu noma izimila eziningi esemncane, khuluma nodokotela wakho mayelana nokuhlolwa kwezakhi zofuzo.
- Ukutholwa kusenesikhathi kanye nokuhlolwa njalo kubalulekile ukuze kusindiswe izimpilo. Ukuhlolwa kufanele kuqale lapho izingane ezisengozini zineminyaka eyi-10.
- Ukwelashwa okuyinhloko kokuvimbela ingozi yomdlavuza ukususwa kwamathumbu amakhulu ngokuhlinzwa (colectomy).
- Ngokwelashwa okufanele kanye nokuqashwa, ungaphila impilo egcwele nenempilo ngisho noma une-FAP.
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