Chii chinonzi Familial Adenomatous Polyposis? Zviratidzo, Zvinokonzera, & Kurapa

Familial adenomatous polyposis (FAP) chirwere chisingawanzo chiri mumagene chinoita kuti mazana kusvika kuzviuru zvekurima madiki anonzi polyps akure mumatumbo ako makuru nemu rectum. Aya polyps anenge asina ngozi pakutanga, asi achachinja kuva kenza kana asingarapwi.

Chirwere ichi chinogara mumhuri chinotapukira vanhu vanenge 1 pavagumi ve10,000 pasi rose. Kunyange FAP inogona kunzwa yakanyanya kana wawanzwa nezvayo kekutanga, kunzwisisa mamiriro ako uye kushanda nesangano rako rezvekurapa kunogona kukubatsira kuti uikwanise zvakanaka uye urambe uine hupenyu hwakanaka.

Ndeapi zviratidzo zveFamilial Adenomatous Polyposis?

Zviratidzo zveFAP kazhinji hazviratidziki kusvika paudiki hwako kana makore makumi maviri ekutanga, apo polyps yave ne nguva yekukura uye kuwanda. Vanhu vazhinji vane FAP havaoni chero zviratidzo mumaitiro ekutanga, ndosaka kuongororwa nguva dzose kwakakosha kwemhuri dzine chirwere ichi.

Kana zviratidzo zvikaonekwa, unogona kuona zviratidzo zvakati wandei zvinoratidza kuti chimwe chinhu chinoda kurapwa:

• Ropa mumukaka wako kana kubuda ropa kubva mu rectum
• Kuchinja mumaitiro emukaka wako, senge manyoka kana kuvimbiswa
• Kurwadziwa mudumbu kana kuchema kunoramba kuripo
• Kurasikirwa uremu kusina tsananguro
• Kuneta kunoramba kuchikura
• Anemia nekuda kwekurasikirwa neropa kwenguva refu

Vamwe vanhu vane FAP vanokurawo kukura kusingakuvadzi muzvikamu zvimwe zvemuviri wavo. Izvi zvinogona kusanganisira mabhonzo madiki pasi peganda rako, mazino akawanda, kana kukura mudumbu rako.

Yeuka, kuva nezviratidzo izvi hazvirevi kuti une FAP. Matambudziko mazhinji emudumbu anogona kukonzera matambudziko akafanana, saka zvakakosha kutaura nachiremba wako nezvezvauri kuona.

Ndeapi marudzi eFamilial Adenomatous Polyposis?

FAP ine mafomu maviri makuru, uye kunzwisisa rudzi rwunogona kuva narwo kunobatsira chiremba wako kugadzira chirongwa chekurapa chakanakira mamiriro ako.

Classic FAP ndiyo fomu inowanzoitika, kwaunokura mazana kusvika kuzviuru zvepolyps mumatumbo ako makuru ne rectum. Vanhu vazhinji vane classic FAP vachakura kenza yematumbo makuru nemakore makumi mana kana matumbo avo asingabviswe.

Attenuated FAP (AFAP) ndiyo shanduro yakapfava kwaunokura polyps shoma, kazhinji pakati pe10 ne100. Polyps muAFAP inowanzoonekwa gare gare muhupenyu, kazhinji mumakore makumi mana kana makumi mashanu, uye njodzi yekenza inokura zvishoma nezvishoma.

Kunewo fomu yakanyanya kuoma inonzi Gardner syndrome, iyo inosanganisa polyps yematumbo makuru yeFAP ne kukura muzvikamu zvimwe zvemuviri wako. Vanhu vane Gardner syndrome vanogona kukura kukura kwemashizha, cysts yeganda, uye mazino akawanda pamwe chete ne polyps yematumbo avo makuru.

Chii chinokonzera Familial Adenomatous Polyposis?

FAP inoitika nekuda kwekuchinja mu gene chaiyo inonzi APC, iyo kazhinji inobatsira kudzora kuti masero anokura sei uye kugovaniswa mumatumbo ako makuru. Kana gene iyi isingashandi zvakanaka, masero anokura asingadzoreki uye anoita polyps.

Vanhu vazhinji vane FAP vanogara vane gene yakakanganisika kubva kune mumwe wevabereki vavo. Kana mumwe wevabereki vako ane FAP, une mukana we50% wekuwana chirwere ichi. Izvi zvinonzi autosomal dominant inheritance, zvinoreva kuti unongoda kopi imwe chete yegene yakachinja kuti uve neFAP.

Nekudaro, vanenge 25% vevanhu vane FAP havafaniri kuva ne nhoroondo yemhuri yeiyi mamiriro. Mumamiriro ezvinhu aya, kuchinja kwegene kwakaitika zvisingatarisirwi, kuchigadzira izvo vanachiremba vanoti "de novo" mutation.

Gene yeAPC inoshanda senge pedal yekubata kukura kwemasero mumatumbo ako makuru. Kana ichishanda zvakanaka, inoudza masero kuti arege kukura uye kugovaniswa. Kana gene ikakanganisika, masero anorasikirwa nechiratidzo ichi chakakosha chekumisa uye anoramba achizviwanza, pakupedzisira achigadzira polyps.

Rini unofanira kuona chiremba nekuda kweFamilial Adenomatous Polyposis?

Unofanira kuona chiremba nekukurumidza kana ukaona ropa mumukaka wako, kunyanya kana zvikaitika kakawanda kana zvichiuya nezvimwe zviratidzo senge kurwadziwa mudumbu kana kuchinja mumaitiro emukaka wako. Kunyange zvazvo zviratidzo izvi zvingave nezvikonzero zvakawanda, zvinoda nguva dzose kurapwa nachiremba.

Kana uine nhoroondo yemhuri yeFAP kana kenza yematumbo makuru, zvakakosha kutaura nachiremba wako nezvekuongororwa kwemagene nekuongororwa, kunyange usati uine chero zviratidzo. Kutanga kuongororwa pakutanga kunogona kubata polyps usati yava kenza.

Rongedza musangano kana uine zviratidzo zvemudumbu zvinoramba zvichiitika kwemavhiki anopfuura mashoma. Izvi zvinosanganisira manyoka anoramba achiitika, kuvimbiswa, kurwadziwa mudumbu, kana kurasikirwa uremu kusina tsananguro.

Usamirire kutsvaga rubatsiro kana uchizvinzwa wakaneta nenhoroondo yemhuri yeFAP. Varapi vemagene vanogona kukubatsira kunzwisisa njodzi dzako uye kuita sarudzo dzakachenjera nezvekuongororwa nekuongororwa.

Ndeapi njodzi dzeFamilial Adenomatous Polyposis?

Njodzi yako huru yeFAP ndeyekuve nemubereki ane chirwere ichi, sezvo FAP iri chirwere chinogara mumhuri. Kana mumwe wevabereki vako ane FAP, une mukana we50% wekuwana gene yakakanganisika.

Kuva nenhoroondo yemhuri yekenza yematumbo makuru, kunyanya kana yakaita pakare kare kana vanhu vakawanda vemhuri vakabatwa, zvinogona kuratidza FAP mumutsara wemhuri yako. Dzimwe nguva FAP haizivikanwi kwemazuva mazhinji kana nhengo dzemhuri dzakafa dzichiri vaduku kana dzisina kuwana kurapwa kwakanaka.

Zera rinotamba basa pakutanga kwezviratidzo zveFAP, asi kwete kana uchakura chirwere ichi. Kana uine gene yeFAP, polyps inowanzo tanga kukura mumakore ako ekukura, kunyange zvazvo ungasazvioni zviratidzo kusvika kumakore ako makumi maviri kana makumi matatu.

Kusiyana nezvimwe zvirwere zvakawanda, zvinhu zvehupenyu senge kudya, kurovedza muviri, kana kusvuta hazvikonzeri FAP. Chirwere ichi chinogara mumhuri chinoitika pasinei nokuti hupenyu hwako hwakanaka sei, kunyange zvazvo kuchengetedza hutano hwakanaka kunogona kukubatsira kutonga chirwere ichi nenzira iri nani.

Ndeapi matambudziko anogona kuitika eFamilial Adenomatous Polyposis?

Dambudziko rakakura reFAP ndiro kenza yematumbo makuru, iyo inokura muvanhu vanenge 100% vane FAP isina kurapwa, kazhinji nemakore makumi mana. Ndosaka kuvhiyiwa kwekuvimba kazhinji kunokurudzirwa usati kenza yave mukana wekukura.

Vanhu vane FAP vanosangana nematambudziko akawanda ehutano anogona kukanganisa zvikamu zvakasiyana zvemuviri wavo:

• Kenza yematumbo madiki, kunyanya mu duodenum (chikamu chekutanga chematumbo ako madiki)
• Polyps dzemudumbu nekuda kwekukura kwenzara
• Kenza ye thyroid, kunyanya muvakadzi vane FAP
• Tumors yechiropa inonzi hepatoblastomas, iyo isingawanzoitika asi inowanzoitika kuvana vane FAP
• Tumors dzehuropi, kunyange zvazvo izvi zvisingawanzoitiki

Vamwe vanhu vane FAP vanokura desmoid tumors, iyo isina kenza inokura inogona kuumbwa mudumbu rako, chipfuva, kana maoko. Kunyange zvazvo isina kenza, tumors idzi dzinogona kukura uye kudzvanya pamativi ari pedyo, dzimwe nguva dzichida kurapwa.

Mashoko akanaka ndeawo kuti nekutarisa kwakakodzera nekurapa, akawanda ematambudziko aya anogona kudzivirirwa kana kubatwa pakutanga kana ari kurapika zvakanyanya. Kuongororwa nguva dzose nesangano rako rezvekurapa ndiro kiyi yekurarama pamberi pematambudziko anogona kuitika.

Familial Adenomatous Polyposis inoziiwa sei?

Kuziva FAP kazhinji kunosanganisira kusanganiswa kwekuongororwa kwemagene, colonoscopy, uye kuongororwa kwenhoroondo yemhuri. Chiremba wako anotanga kubvunza mibvunzo yakadzama nezvezviratidzo zvako uye kana mumwe munhu wemhuri yako ane kenza yematumbo makuru kana FAP.

Colonoscopy ndiro bvunzo huru inoshandiswa kutsvaga polyps mumatumbo ako makuru ne rectum. Panguva iyi, chiremba wako anoshandisa chubhu yakapfava ine kamera kuongorora mukati memukaka wako. Kana vakawana mazana e polyps, kunyanya mumunhu ane makore asingasviki makumi mashanu, FAP inova mukana wakakura.

Kuongororwa kwemagene kunogona kusimbisa FAP diagnosis nekutsvaga kuchinja mu gene yeAPC. Bvunzo iyi inoshandisa sampuli yeropa iri nyore uye inogona kukuzivisa zvakajeka kana une gene yeFAP. Nekudaro, bvunzo iyi haina kuwana kuchinja kwegene muvanhu vanenge 10-15% vane FAP zvakajeka zvichienderana nezviratidzo zvavo.

Chiremba wako anogona kukurudzirawo bvunzo dzakawedzerwa kuti aongorore polyps kana tumors muzvikamu zvimwe zvemuviri wako. Izvi zvinogona kusanganisira upper endoscopy kuongorora mudumbu rako nematumbo madiki, kana bvunzo dzekuona kuti uone thyroid yako nezvimwe zvikamu zvemuviri wako.

Kurapa kweFamilial Adenomatous Polyposis ndekupi?

Kurapa kukuru kweFAP ndekuvhiyiwa kwekuvimba kubvisa matumbo ako makuru ne rectum usati kenza yakakura. Izvi zvinogona kunzwa zvinotyisa, asi kuvhiyiwa uku kunogona kuponesa hupenyu uye kunokubvumira kurarama hupenyu hwakanaka, hunogara uchifara.

Chiremba wako wekuvhiya anowanzo kukurudzira imwe yezviitiko zviviri zvikuru. Total proctocolectomy ne ileostomy inobvisa matumbo ako makuru ne rectum, uye inogadzira mukana mudumbu rako kwaunobuda marara mu bhegi rekutora. Kana zvakadaro, total proctocolectomy ne ileal pouch-anal anastomosis inobvisa matumbo ako makuru ne rectum asi inogadzira bhegi riri mukati kubva mumatumbo ako madiki, ichikubvumira kuti ubude marara zvakajairika.

Nguva yekuvhiyiwa inotsamira pazvinhu zvakati wandei, kusanganisira kuti une polyps dzakawanda sei, kana chero ipi zvayo ichiratidza zviratidzo zvekuchinja kuva kenza, uye zera rako nehutano hwese. Vanhu vazhinji vane classic FAP vanovhiyiwa mumakore avo ekupedzisira ekukura kana makore makumi maviri.

Kana usati wagadzirira kuvhiyiwa kana une attenuated FAP ine polyps shoma, chiremba wako anogona kukurudzira mishonga senge sulindac kana celecoxib. Mishonga iyi inogona kubatsira kudzikisira kukura kwe polyps, kunyange zvazvo isingakwanisi kudzivirira kenza zvachose uye haisi chinotsiva kuvhiyiwa mumamiriro ezvinhu ane njodzi yakakura.

Kuongororwa nguva dzose kwakakosha kunyange mushure mekurapa. Uchada colonoscopies inoramba ichienzaniswa ye chero tishu yematumbo yakasara, pamwe nekuongororwa kwekenza muzvikamu zvimwe zvemuviri wako senge thyroid yako, mudumbu, nematumbo madiki.

Nzira yekutonga sei Familial Adenomatous Polyposis pamba?

Kunyangwe usingakwanisi kudzivirira kana kurapa FAP kuburikidza nekuchinja mararamiro, kuchengetedza hutano hwako hwese kunogona kukubatsira kunzwa zvakanaka uye kunogona kuderedza mamwe matambudziko.

Rambai makabatana nesangano renyu rezvekurapa uye chengetai misangano yose yakarongwa, kunyange kana muchinzwa zvakanaka. FAP inoda kuongororwa kweupenyu hwese, uye kuongororwa nguva dzose ndiro dziviriro yako yakanaka pamberi pematambudziko.

Funga kubatana negroup rekutsigira vanhu vane FAP kana hereditary cancer syndromes. Kubatana nevamwe vanonzwisisa zvauri kuona kunogona kupa rutsigiro rwepfungwa rwakakosha uye mazano anoshanda.

Chengeta rekodhi yakadzama yenhoroondo yako yezvekurapa, mhinduro dzebvunzo, uye ruzivo rwehutano hwemhuri. Ruzivo urwu runova rwakakosha kana ukaendesa kana kuchinja vanachiremba, uye runogona kuva rwakakosha kuvana vako nevamwe vemhuri yako.

Usazengurire kubvunza mibvunzo kana kutsvaga maonero ekurapa kwako. FAP chirwere chakaoma, uye unofanira kunzwa wakavimbika uye wakaziva nezvesarudzo dzako dzekurapa.

Ungagadzirira sei musangano wako nachiremba?

Usati wasangana nachiremba,unganidza ruzivo rwakawanda sezvinobvira nezvenhoroondo yemhuri yako yehutano, kunyanya chero nyaya dzekensa yematumbo makuru, polyps, kana dzimwe kenza. Kunyange ruzivo nezvehama dzakafa dzichiri vaduku kana dzichibva pazvikonzero zvisingazivikanwe zvinogona kubatsira.

Nyora zviratidzo zvako zvose, kusanganisira apo zvakatanga, kazhinji zvinoitika, uye chii chinoita kuti zvive nani kana zvakanyanya kuipa. Usakanganwa kutaura nezvezviratidzo zvingaita sekusina hukama, senge mabhonzo eganda kana matambudziko emazino.

Gadzira runyorwa remishonga yose, mavhitamini, uye zvinowedzerwa zvauri kutora. Uyewo, nyora mibvunzo yose yaunoda kubvunza chiremba wako, kutanga neyakakosha kwazvo kana usati wava nenguva.

Funga kuuya nemumwe wemhuri kana shamwari kumusangano wako, kunyanya kana uri kukurukura kuongororwa kwemagene kana sarudzo dzekurapa. Kuva nemunhu mumwe chete ipapo kunogona kukubatsira kuyeuka ruzivo rwakakosha uye kupa rutsigiro rwepfungwa.

Kana uri kuona nyanzvi kekutanga, bvunza chiremba wako wekutanga kuti atumire rekodhi yako yezvekurapa usati wasvika. Izvi zvinobatsira kuve nechokwadi chekuti chiremba wako mutsva ane ruzivo rwese rwavanoda kupa kurapwa kwakanaka.

Chii chinonyanya kukosha nezveFamilial Adenomatous Polyposis?

FAP chirwere chakakomba chinogara mumhuri, asi nekurapa kwakakodzera nekuongororwa, vanhu vane FAP vanogona kurarama hupenyu hwazvo hwese, hunofara. Kiyi ndeyekuona pakutanga nekurapa kwakakurumidza, kazhinji kusanganisira kuvhiyiwa kwekuvimba usati kenza yakakura.

Kana uine nhoroondo yemhuri yeFAP kana kenza yematumbo makuru isingazivikanwi, kurairwa kwemagene nekuongororwa kunogona kukubatsira kunzwisisa njodzi dzako uye kuita sarudzo dzakachenjera nezvehutano hwako. Kuita zvinhu pakutanga kunonyanya kukosha nechirwere ichi.

Yeuka kuti kuva neFAP hakusi kukutsanangurira, uye kufambira mberi mukurapa kuri kuramba kuchivandudza mhinduro dzevanhu vane chirwere ichi. Shanda pedyo nesangano rako rezvekurapa, gara uchiziva nezvemamiriro ako, uye usazengurire kutsvaga rubatsiro kana uchida.

Mibvunzo inowanzo bvunzwa nezveFamilial Adenomatous Polyposis

Mubvunzo 1: Kana ndine FAP, vana vangu vachava nayowo here?

Kwete, mumwe nemumwe wevana vako ane mukana we50% wekuwana FAP kana uine gene. Izvi zvinoreva kuti vamwe vana vako vangave neFAP nepo vamwe vasingave navo. Kuongororwa kwemagene kunogona kuona kana vana vako vakawana chirwere ichi, kazhinji kutanga pakati pemakore gumi nemakore gumi nemaviri.

Mubvunzo 2: Ndingadzivirira sei FAP kuburikidza nekudya kana kuchinja mararamiro?

Nekudaro, haugone kudzivirira FAP kuburikidza nekuchinja mararamiro sezvo yakakonzerwa nekuchinja kwemagene. Nekudaro, kuchengetedza kudya kwakanaka nemararamiro kunogona kutsigira hutano hwako hwese uye kunogona kukubatsira kupora zviri nani kubva pakurapa senge kuvhiyiwa.

Mubvunzo 3: Kazhinji ndinoda colonoscopies kangani kana ndine FAP?

Kazhinji kunoenderana nemamiriro ako chaiwo, asi vanhu vazhinji vane FAP vanoda colonoscopies gore rega rega kana maviri kutanga mumakore avo ekukura. Mushure mekuvhiyiwa kwekuvimba, ucharamba uchida kuongororwa nguva dzose ye chero tishu yematumbo yakasara, kazhinji gore rega rega kana matatu.

Mubvunzo 4: Kuvhiyiwa kweFAP kukuru here, uye kupora kwacho kuri sei?

Hongu, kuvhiyiwa kweFAP ndekuvhiyiwa kukuru kwemudumbu, asi matekinoroji akavandudzwa zvikuru mumakore achangopfuura. Vanhu vazhinji vanopedza vhiki imwe muchipatara uye vanoda mavhiki matanhatu kusvika masere kuti vapore zvizere. Vanhu vazhinji vanodzoka kuzviitiko zvakajairika, kusanganisira basa nekurovedza muviri, mushure mekupora.

Mubvunzo 5: Ndingava nevana here mushure mekuvhiyiwa kweFAP?

Hongu, vanhu vazhinji vanogona kuva nevana mushure mekuvhiyiwa kweFAP, kunyange zvazvo mamwe maitiro ekurapa anogona kukanganisa kubereka zvishoma. Zvakakosha kukurukura kuronga mhuri nachiremba wako usati wavhiyiwa kuitira kuti vagone kufunga izvi muchirongwa chako chekurapa. Vakadzi vane FAP vanogona kazhinji kuva nepamuviri dzakajairika nekuzvarwa.