Pane mumwe munhu mumhuri menyu akamborwara nekenza yemuura here? Ngatikurukurei nezvechirwere ichi chemajini (Familial Adenomatous Polyposis - FAP)!

Pane mumwe munhu mumhuri yako, zvichida achiri mudiki, akabatwa nekenza yemuura here? Kana kuti chiremba akakuudza here kuti une matumbu madiki (polyps) muura wako pamwe nematambudziko emudumbu? Ungave wakanzwa nezvechinhu chakadaro kana kuti kwete, zvingave zvakakosha kuziva nezvechirwere ichi chinonzi 'FAP' chatichataura nezvacho nhasi. Nekuti kunyangwe chisingawanzoitiki, kana chikaonekwa nekukurumidza, chinogona kukuponesa matambudziko makuru akawanda.

Chii chinonzi FAP nemashoko akareruka?

Muchidimbu, Familyal Adenomatous Polyposis, kana kuti FAP muchidimbu, chirwere chemajini chinokonzera huwandu hukuru hwemapundu madiki (polyps) anonzi adenomas, ayo anogona kuita kenza, kuumbika muura hwako uye dzimwe nguva mukati me rectum yako.

Funga nezvazvo, vamwe vanhu vanokura rimwe kana maviri emabundu aya muura hwavo pavanenge vachikura. Izvozvo zvakajairika. Asi kune munhu ane `FAP`, vanowanzova nemabundu aya anopfuura zana, dzimwe nguva zviuru , asi anotanga kukura achiri mudiki, pamwe nemakore gumi. Nokudaro, mukana wekuti rimwe remabundu aya richashanduka kuita kenza (`kenza yemudumbu`), kureva kuti, njodzi muhupenyu hwavo hwese, yakakura kwazvo .

Kuti vadzore njodzi iyi, vanachiremba vanowanzo kurudzira kuvhiyiwa kubvisa ura hwese (total colectomy) . Dzimwe nguva, rectum inobviswawo (proctocolectomy). Izvi zvinodaro nekuti muFAP, mabundu aya anokura nekukurumidza zvekuti haagone kudzorwa nekuabvisa rimwe nerimwe. Kutaura zvazviri, kana kuvhiyiwa uku kukasaitwa, vanhu vazhinji vane FAP vanozobatwa nekenza pazera repakati .

Vanhu vane FAP vanogona kuita mamwe marudzi emamota, kwete muura chete, asiwo mune dzimwe nzvimbo, dzakadai seganda, nyama dzakapfava, mazino, nemapfupa. Kunyangwe mushure mekunge ura hwabviswa, ungangoda kuramba uchiongororwa kuti uone mamwe mamota aya, uye ungangoda kuvhiyiwawo.

Ndeipi njodzi yekubatwa negomarara kune vanhu vane FAP?

Kana munhu ane FAP akasarapwa, ane mukana wekuti awane kenza yemuura mukuru ne100% . Uyewo, kenza inogona kukura nekukurumidza uye achiri mudiki pane vamwe vanhu. Kana nhengo yemhuri ichizivikanwa kuti ine chirwere ichi, vana vari mumhuri idzodzo vanofanira kuongororwa colonoscopy gore rega rega kutanga pazera remakore gumi .

Kuwedzera kune kenza yemuura, vanhu vane FAP vari panjodzi yekubatwa nedzimwe mhando dzekenza:

• Kenza yechikamu chepamusoro cheura diki (`Duodenal cancer`) - inenge 8%
• Kenza yethyroid yePapillary - inenge 2%
• Kenza yepancreatic - inenge 2%
• Kenza yechiropa inonzi ``Hepatoblastoma`` (kunyanya kuvana) - inenge 1.5%
• Kenza yemudumbu - inenge 1%
• Matumbu europi nemusana - asingasviki 1%

Kune mhando dzakasiyana dzeFAP here?

Ehe, kune rudzi rukuru rwe 'FAP' nedzimwe mhando dzakasiyana-siyana pamusoro pazvo.

• "Classic" FAP: Izvi zvinoonekwa nekuvapo kweadenomas dzinopfuura 100 muura.
• "Attenuated" FAP (AFAP): Iyi mhando isina kunyanya kuoma. Kune ma cysts ari pakati pe20 ne100 muura.
• Gardner syndrome: Kufanana ne 'FAP' yekare, kune mabundu anopfuura zana muura. Pamusoro pezvo, mamwe marudzi emabundu anokura kune dzimwe nzvimbo mumuviri.
• Turcot syndrome: Bundu rekenza rinokura muuropi pamwe chete nemabundu akawanda muura.

Dambudziko iri rinonzi FAP rakajairika zvakadii?

FAP chirwere chisingawanzoitiki . Chinobata munhu mumwe chete pavanhu 8,000. FAP inoumba 0.5% yekenza yemuura wese. Mhando dzekenza dzakadai seAFAP, Gardner syndrome, uye Turcotte syndrome dzinotowedzera kuwanda.

Chii chakasiyana pakati peFAP neLynch syndrome?

Lynch syndrome chimwe chirwere chinogara nhaka chinowedzera njodzi yekubatwa nekenza yemuura mukuru nedzimwe kenza. Lynch syndrome inonziwo HNPCC (hereditary nonpolyposis colorectal cancer syndrome). Sezvinoreva zita racho, izvi hazvirevi hazvo kuti huwandu hwakawanda hwemabundu emuura mukuru hunokura .

Vanhu vane chirwere cheLynch syndrome vanogona kuwana kenza kunyangwe vaine bundu rimwe chete kana akawanda muura. Uyewo, bundu nekenza zvinokura zvishoma gare gare pane muFAP, uye njodzi yacho yakaderera zvishoma. Matambudziko maviri aya anokonzerwa nekuchinja kwemajini kwakasiyana .

Ndezvipi zviratidzo zveFAP? Tinozviziva sei?

MuFAP, mapolyps emudumbu anotanga kuumbwa kare kupfuura muvanhuwo zvavo, kazhinji mumakore ekuyaruka . Mapolyps aya anogona kusakonzera zviratidzo kusvika akura zvine njodzi. Asi kana ukaitwa colonoscopy, chiremba wako anogona kuawana.

Vanhu vane `FAP` vanogona kunge vaine mazana kana zviuru zvema polyps muura hwavo . Vanhu vane `AFAP` vane kanenge makumi maviri. Nekuti ma polyps akawanda uye anokura nekukurumidza mu 'FAP', ane mukana wekuti atange zviratidzo kupfuura ma polyps. Zviratidzo zvinosanganisira:

• Kubuda ropa mutsvina
• Manyoka
• Marwadzo emudumbu asingaperi

Vanhu vane FAP dzimwe nguva vanogona kuva nezviratidzo zvinogona kuonekwa navanachiremba kunze:

• Dermatofibromas: Mapundu ane fibrous, akafanana nemavanga ari pasi peganda.
• Mapundu eganda: Mapundu akatenderera, azere nekeratin ari pasi peganda.
• Maosteomas: Mabundu asiri ekenza anoumbwa mumapfupa. Anowanzo kuwanikwa mushaya kana mudehenya.
• Mazino ekuwedzera kana mazino akakuvara:Izvi zvinoonekwa pa X-ray yemazino.
• Congenital hypertrophy of the retinal pigment epithelium (CHRPE): Izvi zvinogona kuonekwa panguva yekuongororwa kwemaziso. Zvisinei, kazhinji hazvikonzeri matambudziko ekuona.

Zviratidzo zveFAP zvinowanzotanga pazera ripi?

MuFAP, kukura kwemudumbu kunowanzotanga pazera remakore gumi nematanhatu. MuAFAP, kunogona kutanga gare gare. Kana vabereki vako navanachiremba vachiziva kuti uri panjodzi yechirwere ichi, vanogona kutanga kukuyedza mushure memakore gumi. Kana ukasadaro, unogona kuonekwa uine chirwere ichi nekuda kwezviratidzo zvako.

Chii chinonyanya kukonzera FAP?

Chikonzero chikuru cheFAP ikuchinja kwemajini . Jini iri rinonzi adenomatous polyposis coli (APC) gene . Rinonziwo tumor suppressor gene. Izvi zvinoreva kuti jini iri rinodzivirira masero kukura asingadzoreki uye kugadzira mabundu. Kana kushanduka kwemajini kukaitika, basa rejini iri rinokanganiswa.

Kuchinja kwemajini kunokonzera FAP ikushanduka kwemajini. Izvi zvinoreva kuti hachisi chinhu chinoitika muhupenyu, asi chimwe chinhu chinoitika panguva yekubata pamuviri . Ichi chinhu chinopfuudzwa kubva kuchizvarwa kuenda kune chimwe. Kana mumwe wevabereki vako aine kuchinja uku, une mukana we50% wekukugara nhaka . Zvisinei, inenge 30% yekuchinja uku kutsva (kuchinja kwekutanga), kureva kuti, hakuna kugara nhaka . Saka, inenge 30% yevarwere vanoonekwa vaine FAP vanogona kunge vasina nhoroondo yemhuri yechirwere ichi.

Ndezvipi zvinetso zvinogona kuitika nekuda kweFAP?

Dambudziko guru rekugadzirisa muFAP igomarara remuura . Kuvhiyiwa kubvisa muura wako (colectomy) kunogona kuderedza njodzi iyi zvikuru. Zvisinei, kurarama usina muura kunogona kukanganisa hupenyu hwako nekuti nzira yaunobvisa tsvina inochinja. Ungangoda kushandisa ileostomy pouch kana ileal pouch panzvimbo pemuura.

Nekuti kushanduka kwemajini kunokonzera FAP kuripo musero rega rega mumuviri wako, mabundu anogona kukura chero kupi zvako mumuviri wako . Mamwe mabundu anogona kukurawo kunze kweura, uye dzimwe nguva anogona kuita kenza. Chiremba wako anogona kukurudzira purogiramu yekuongorora kuti utarise mhando idzi dzemabundu:

• Mapolyps eDuodenal/periampullary:Izvi zvinokura muchikamu chepamusoro cheura hwako hudiki (duodenum), kana kuti apo duct ye bile kana pancreatic duct inosangana neura hudiki. Vanhu vanenge vese vane FAP vanokura izvi. Vanhu vashoma vanogona kukura nekenza ye duodenal, kenza ye ampullary, kenza ye pancreatic mu duct, kana kenza ye bile duct.
• Mapolyps emudumbu: Vanhu vanosvika 90% vane FAP vanowana mapolyps emudumbu, asi 1% chete yeaya anozopedzisira ava kenza.
• Matumbu eDesmoid: Aya matumbu asiri ekenza anoumbwa munyama dzinobatana. Anowanikwa muvanhu vanosvika 15% vane FAP. Kunyangwe asiri ekenza, dzimwe nguva anogona (anenge 5%) kukonzera matambudziko makuru ehutano uye kunyange rufu. Anogona kuva nehasha, kupararira kune zvivako zviri pedyo, uye kudzvanya kana kuvhara mitsipa yeropa, nhengo dzemuviri, uye tsinga. Anonetsa kubvisa uye anogona kudzoka.
• Kenza yethyroid yePapillary: Rudzi urwu rwekenza yethyroid runowanikwa muvanhu vanosvika 2% vane FAP. Haruna njodzi yakawanda uye runowanzo rapa.
• Kenza yechiropa: Vana vane FAP, kunyanya, vane mukana mudiki wekubatwa nekenza yechiropa isingawanzoitiki inonzi hepatoblastoma.
• Medulloblastoma: Iyi ikenza yeuropi. Inogona kuitika neTurcot syndrome, iyo inobatanidzwa neFAP. Kunyangwe neFAP, hazviwanzoitiki.
• Mapombi emudumbu: Kana ukasabviswa rectum yako pamwe chete neura hwako, uri panjodzi yekubatwa nemapombi emudumbu, saka uchafanirwa kuongororwa proctoscopy muhupenyu hwako hwese.

Ungaziva sei kuti une FAP?

Kana uchida kuziva kana wakagara nhaka APC gene mutation, unogona kuziva nekuita genetic testing . Kuongororwa kwemajini kunotora sampuro yeDNA yako (yakadai seropa kana mate) uye kunotsvaga magene chaiwo ekuchinja. Kana uine mutation, danho rinotevera nderekuita colonoscopy kuti utarise adenomas.

FAP inoonekwa kana paine ma polyps anosvika zana (20 kana ari AFAP) uye kuvapo kweAPC gene mutation . FAP haisiriyo yega mamiriro ekugara nhaka anokonzera adenomatous polyps. MUTYH-associated polyposis chirwere chakafanana, asi chinokonzerwa nekuchinja kwejini rakasiyana rinonzi MUTYH.

Ndeipi hurongwa hwekurapa munhu ane FAP?

Chirongwa chekurapa FAP chinosanganisira kutarisirwa kwehupenyu hwese uye kuvhiyiwa kwemutemo.Pakutanga, kana uine nhamba shoma yema polyps (kana AFAP), chiremba wako anogona kuabvisa ega ega panguva yekuvhiyiwa kwecolonoscopy (polypectomy). Kana ma polyps acho akura zvakanyanya kana kuita kenza, kuvhiyiwa kunogona kudiwa.

Kuvhiya

Vanhu vazhinji vane FAP yekare vachada kuvhiyiwa colectomy zvachose vava nemakore ekuma20 kana kutanga kwemakore ekuma30 . Chiremba wako ndiye achasarudza kuti unofanira kuvhiyiwa riini zvichienderana nezvinhu zvako zvine njodzi. Iye achataurawo newe nezvemhando dzakasiyana dze colectomy dzaunogona kuita.

Kana ura hwako hwabviswa, panobva pava nemukana uripo pakati peura hwako hudiki nerectum (kana anus). Dzimwe nguva, chiremba anogona kubatanidza mativi maviri aya. Zvadaro unogona kuita kuti ura hubude kuburikidza nerectum yako semazuva ese. Kana izvozvo zvisingakwanisike, vanogadzira 'ostomy', inova buri idzva mudumbu mako kuti tsvina yako ibude.

Kuongorora

Chikwata chenyu chezvekurapa chichakupai mazano ekuti munofanira kuongororwa kangani mhando dzakasiyana dzemamota, zvichienderana nezvikonzero zvenyu zvengozi. Kune classic FAP, colonoscopy inokurudzirwa gore rega rega kubva pazera remakore gumi kusvika pakuvhiyiwa kwecolectomy . Kune AFAP, kuongororwa kunofanira kutanga gore rega rega, kunyangwe pazera remakore makumi maviri.

Mushure mekuvhiyiwa kwecolectomy, unofanira kuramba uchiongororwa sigmoidoscopy, iyo inoongorora chikamu chezasi chedumbu rako (GI - gastrointestinal tract). Kana uine chikamu cherectum yako chasara, chinofanira kuongororwa kanenge mwedzi 6 kusvika 12 yega yega. Kana rectum yako yakabviswa ikatsiviwa neleal pouch, inofanira kuongororwa kanenge makore 1 kusvika 4 ega ega.

Mimwe miedzo yakarongwa inogona kusanganisira:

• Endoscopy yepamusoro: Izvi zvakafanana ne colonoscopy, asi zvinoitwa pachikamu chepamusoro chedumbu rako. Endoscope inopfuudzwa nepahuro pako uye mudumbu mako nechikamu chepamusoro cheura hwako hudiki (duodenum) kuti utarise kana paine ma polyps. Kana aripo, chiremba wako anogona kuabvisa panguva imwe chete nemaitiro acho.
• Kuongororwa kweUltrasound kwegomarara rethyroid kana rechiropa.
• CT scan (computed tomography scan) kana MRI (magnetic resonance imaging) bvunzo dze desmoid bundu.

FAP inogona kudzivirirwa here?

Kupa mazano ezvemajini kuti unzwisise njodzi yekupa chirwere cheFAP kuvana vakoZvinogona kubatsira. Kutaura nezvenjodzi idzi kunogona kukubatsira kuronga mhuri yako. Kazhinji, hazvigoneke kudzivirira shanduko yemajini kuti isaitike panguva yekubata pamuviri, asi kune nzira dzakasiyana dzekuronga mhuri dzaunogona kufunga nezvadzo.

Munhu ane FAP anotarisirwa kurarama kwenguva yakareba sei?

Kana ikasarapwa, avhareji yenguva yekurarama inenge makore makumi mana nemaviri . Zvisinei, nekurapwa kwakakodzera uye kurapwa, unogona kurarama hupenyu hwakajairika . Mushure mekubviswa kweura hwako, njodzi huru inobva kune dzimwe kenza dzemudumbu uye mabundu ane matambudziko akawanda. Aya haawanzoitiki sekenza yemudumbu.

Kana ndine chirwere ichi, chii chandinofanira kutarisira?

Kana ukaonekwa uine FAP, unogona kutarisira kuve nebvunzo dzehutano kwenguva refu uye pamwe nekuvhiyiwa kakawanda kubvisa bundu . Bundu rinokura kunze kweura rako harina mukana wekuva nekenza kupfuura ma polyps muura rako. Kunyange zvazvo bundu re desmoid risiri kenza, dzimwe nguva rinogona kuva dambudziko diki kana dambudziko guru.

Unogona kutarisira kuvhiyiwa colectomy zvachose usati watanga hupenyu hwako . Mushure meizvozvo, ura hwako hunogona kubatanidzwazve, kana kuti ungave ne leal pouch kana ostomy. Imwe neimwe yesarudzo idzi ine njodzi yematambudziko chaiwo. Zvisinei, unogona kurarama hupenyu hurefu, hune hutano mushure mekuvhiyiwa colectomy.

Zvakajairika kunzwa kusuruvara uye kuzvidya mwoyo paunoziva kuti une chirwere chemajini chinoda kurapwa kwehupenyu hwako hwese. Zvisinei, kana wangozviziva, unogona kutora matanho ekugadzirisa njodzi yako yekenza . Kunyange zvazvo kuvhiyiwa kuri mune ramangwana rako, vanachiremba vachaedza kuzviita zviri nyore sezvinobvira.

Vanhu vazhinji vanogona kuvhiyiwa ne laparoscopic kubvisa ura, izvo zvinoitwa nekucheka kudiki uye zvinopora nekukurumidza . Vanhu vane chikwama che leal vanogona kuvhiyiwa kakawanda, asi vanozokwanisa kushandisa chimbuzi sezvavaiita kare.

Zvinhu zvakakosha zvekurangarira kubva pane zvatakurukura (Mharidzo Yekuenda Kumba)

Zvakanaka, saka, kubva pane zvatataura nezve `FAP`, hezvino zvinhu zvakakosha zvaunofanira kuyeuka:

• FAP chirwere chemajini chinogona kupfuudzwa nezvizvarwa.
• Izvi zvinokonzera mazana kana zviuru zvema polyps muura, izvo zvinogona kuita kenza.
• Kana ikasarapwa, njodzi yekubatwa nekenza yemuura yakakura kwazvo .
• Zvakakosha zvikuru kutanga kuongororwa colonoscopy kubva pazera diki (makore 10-12) .
• Kurapa kukuru kunoitwa nekuvhiyiwa kubvisa ura (colectomy) .
• Kunyangwe mushure mekunge ura hwabviswa, kuongororwa kwehupenyu hwese kunodiwa kuti utarise bundu riri kukura kune imwe nzvimbo mumuviri.
• Kunyangwe ichi chiri chirwere chehupenyu hwese chinofanira kugadziriswa, nekurapwa kwakakodzera uye kuongororwa, zvinokwanisika kurarama hupenyu hwakanaka uye hwakajairika .

Kana iwe kana mumwe munhu mumhuri yako aine chimwe chezviratidzo izvi, kana kuti uchida kuziva zvakawanda nezvazvo, tsvaga rubatsiro rwechiremba . Kuziva nekukurumidza uye kutora matanho ekutanga ndizvo zvinhu zvinonyanya kukosha.

` FAP, Familyal Adenomatous Polyposis, Zvirwere zveMajini, Colon Polyps, Kenza yeMatumbu, APC Gene, Colonoscopy, Colectomy, Kuvhiya