Ese ni iki Familial Adenomatous Polyposis? Ibimenyetso, Impamvu, n’Ubuvuzi

Familial adenomatous polyposis (FAP) ni indwara idasanzwe iterwa na gène, itera imikurire myinshi mito yitwa polyps mu mara manini no mu kibuno. Aya ma polyps aba atari kanseri mbere, ariko azahinduka kanseri niba atabyazwe.

Iyi ndwara iherwa mu miryango igera kuri 1 kuri 10.000 ku isi hose. Nubwo FAP ishobora kuguha ikibazo iyo uyimenye bwa mbere, gusobanukirwa uko iyi ndwara imeze no gukorana n’abaganga bawe bishobora kugufasha kuyigenzura neza no kugira ubuzima bwiza.

Ni ibihe bimenyetso bya Familial Adenomatous Polyposis?

Ibimenyetso bya FAP ntibigaragara mbere y’imyaka y’ubwangavu cyangwa mu myaka ya mbere ya 20, igihe polyps imaze igihe ikura kandi ikwirakwira. Abantu benshi bafite FAP ntabimenyetso bagira mu ntangiriro, niyo mpamvu gusuzuma buri gihe ari ingenzi cyane ku miryango ifite iyi ndwara.

Iyo ibimenyetso bigaragaye, ushobora kubona ibimenyetso byinshi bigaragaza ko ukeneye ubuvuzi:

• Amaraso mu ntege
• Guhinduka mu mirire yawe, nko guhitwa cyangwa kubabara
• Kubabara mu nda cyangwa gucika intege bidashira
• Gutakaza ibiro bitasobanutse
• Urugwiro rugaragara rwiyongera uko iminsi igenda
• Anemia iterwa no gutakaza amaraso buri gihe

Bamwe mu bantu bafite FAP baragira kandi imikurire itari kanseri mu bice by’umubiri wabo. Ibi bishobora kuba harimo imikurire mito munsi y’uruhu, amenyo y’inyongera, cyangwa imikurire mu gifu.

Wibuke ko kugira ibi bimenyetso ntibisobanura ko ufite FAP. Ibibazo byinshi bisanzwe byo mu gifu bishobora gutera ibibazo nk’ibi, niyo mpamvu ari ingenzi kuvugana na muganga wawe ku byo urimo kunyuramo.

Ni iyihe mitype ya Familial Adenomatous Polyposis?

FAP ifite imyanya ibiri nyamukuru, kandi gusobanukirwa uburyo ushobora kuba ufite bigufasha muganga wawe gutegura gahunda y’ubuvuzi ibereye uko uhagaze.

Classic FAP ni yo myanya isanzwe, aho ugira ama polyps amagana cyangwa ibihumbi mu mara manini no mu kibuno. Abantu benshi bafite classic FAP bazagira kanseri y’amara manini mbere y’imyaka 40 niba amara yabo atakuweho.

Attenuated FAP (AFAP) ni uburyo buke aho ugira polyps nke, ubusanzwe hagati ya 10 na 100. Aya ma polyps muri AFAP asanzwe agaragara nyuma y’imyaka myinshi, akenshi mu myaka ya 40 cyangwa 50, kandi ibyago byo kurwara kanseri bikura gahoro gahoro.

Hariho kandi uburyo bukomeye cyane buke cyane bwitwa Gardner syndrome, buhuza ama polyps y’amara ya FAP hamwe n’imikurire mu bindi bice by’umubiri. Abantu bafite Gardner syndrome bashobora kugira imikurire mu magufwa, imikurire y’uruhu, n’amenyo y’inyongera hamwe na polyps zabo z’amara.

Ese ni iki gitera Familial Adenomatous Polyposis?

FAP iterwa n’impinduka mu gène runaka yitwa APC, isanzwe ifasha kugenzura uko uturemangingo dukura kandi tugakwirakwira mu mara. Iyo iyi gène idakora neza, uturemangingo turushaho gukura kandi tukagira polyps.

Abantu benshi bafite FAP baraherwa iyi gène mbi n’umwe mu babyeyi babo. Niba umwe mu babyeyi bawe afite FAP, ufite amahirwe 50% yo guherwa iyi ndwara. Ibi bita autosomal dominant inheritance, bisobanura ko ukeneye kopi imwe gusa ya gène yahindutse kugira ngo ugire FAP.

Ariko kandi, abantu bagera kuri 25% bafite FAP nta mateka y’iyi ndwara mu miryango yabo. Muri iyi mimerere, impinduka ya gène yabaye mu buryo butunguranye, ikaba ari ibyo abaganga bita “de novo” mutation.

Gène ya APC ikora nk’igifunga cyo kugabanya ubukura bw’uturemangingo mu mara yawe. Iyo ikora neza, ibwira uturemangingo igihe cyo guhagarika gukura no kwaguka. Iyo gène ihindutse, uturemangingo atakaza icyo kimenyetso cy’ingenzi cyo guhagarara kandi akomeza kwiyongera, amaherezo akagira polyps.

Ni ryari ukwiye kujya kwa muganga kubera Familial Adenomatous Polyposis?

Ukwiye kujya kwa muganga ako kanya niba ubona amaraso mu ntege, cyane cyane niba bibaye kenshi cyangwa bikazana ibindi bimenyetso nko kubabara mu nda cyangwa guhinduka mu mirire yawe. Nubwo ibi bimenyetso bishobora kuba bifite impamvu nyinshi, bihora bikwiye kuvuzwa.

Niba ufite amateka y’iyi ndwara mu muryango wawe cyangwa kanseri y’amara manini, ni ingenzi kuvugana na muganga wawe ku bipimo bya gène no gusuzuma, nubwo udafite ibimenyetso. Gutangira gusuzuma hakiri kare bishobora gufata polyps mbere y’uko zihinduka kanseri.

Tegura gahunda yo kujya kwa muganga niba ufite ibibazo byo mu gifu bikomeza ibyumweru birenga bike. Ibi birimo guhitwa, kubabara, kubabara mu nda, cyangwa gutakaza ibiro bitasobanutse.

Ntugatege amatwi gusaba ubufasha niba urimo guhangayikishwa n’amateka y’iyi ndwara mu muryango wawe. Abajyanama b’indwara ziterwa na gène bashobora kugufasha gusobanukirwa ibyago byawe no gufata ibyemezo byiza ku bijyanye no gusuzuma no gukora ibizamini.

Ni ibihe bintu byongera ibyago bya Familial Adenomatous Polyposis?

Ikintu gikomeye cyongera ibyago bya FAP ni ukugira umubyeyi ufite iyi ndwara, kuko FAP ari indwara iherwa mu miryango.

Kugira amateka y’iyi ndwara mu muryango wawe, cyane cyane niba yabaye mu myaka mike cyangwa niba abantu benshi bo mu muryango barayirwaye, bishobora kugaragaza FAP mu muryango wawe. Rimwe na rimwe FAP iba idasobanuwe imyaka myinshi niba abantu bo mu muryango bapfuye bakiri bato cyangwa batabonye ubuvuzi bukwiye.

Imyaka igira uruhare mu gihe ibimenyetso bya FAP bigaragara, ariko si mu gihe uzayirwara. Niba ufite gène ya FAP, polyps isanzwe itangira gukura mu myaka y’ubwangavu, nubwo ushobora kutamenya ibimenyetso kugeza mu myaka ya 20 cyangwa 30.

Bitandukanye n’izindi ndwara nyinshi, imibereho nko kurya, imyitozo ngororamubiri, cyangwa kunywa itabi ntibitera FAP. Iyi ndwara iterwa na gène itera uburwayi utitaye ku mibereho yawe, nubwo kugira ubuzima bwiza muri rusange bishobora kugufasha kuyigenzura neza.

Ni ibihe bibazo bishobora guterwa na Familial Adenomatous Polyposis?

Ikibazo gikomeye cyane cya FAP ni kanseri y’amara manini, itera hafi 100% y’abantu bafite classic FAP idavuwe, akenshi mbere y’imyaka 40. Niyo mpamvu kubagwa kwirinda akenshi bisabwa mbere y’uko kanseri igira amahirwe yo gukura.

Abantu bafite FAP bahura n’ibindi bibazo byinshi by’ubuzima bishobora kugira ingaruka ku bice bitandukanye by’umubiri wabo:

• Kanseri y’amara mato, cyane cyane mu duodenum (igice cya mbere cy’amara mato)
• Polyps z’igifu na kanseri ishobora kuba mu gifu
• Kanseri ya thyroid, cyane cyane mu bagore bafite FAP
• Imikurire mu mwijima yitwa hepatoblastomas, idasanzwe ariko ikunze kugaragara mu bana bafite FAP
• Imikurire mu bwonko, nubwo idasanzwe

Bamwe mu bantu bafite FAP bagira desmoid tumors, ari zo mikurire itari kanseri ishobora gukura mu nda, mu gituza, cyangwa mu maboko. Nubwo atari kanseri, iyi mikurire ishobora gukura cyane ikanatera igitutu ku zindi ngingo, rimwe na rimwe ikaba ikeneye ubuvuzi.

Inkuru nziza ni uko, hakoreshejwe ubugenzuzi n’ubuvuzi bikwiye, ibyinshi muri ibi bibazo bishobora kwirindwa cyangwa gufatwa hakiri kare igihe bishobora kuvurwa neza. Gusuzuma buri gihe hamwe n’itsinda ryawe ry’ubuvuzi ni ingenzi kugira ngo ube imbere y’ibibazo bishoboka.

Familial Adenomatous Polyposis imenyekanwa gute?

Kumenya FAP bisanzwe bikubiyemo gusuzuma gène, colonoscopy, no kureba amateka y’ubuzima bw’umuryango. Muganga wawe azatangira akubaza ibibazo birambuye ku bimenyetso byawe niba hari umuntu wo mu muryango wawe warwaye kanseri y’amara manini cyangwa FAP.

Colonoscopy ni ikizamini nyamukuru gikoreshwa mu gushaka polyps mu mara manini no mu kibuno. Muri ubu buryo, muganga wawe akoresha umuyoboro woroshye ufite camera kugira ngo asuzume imbere y’amara yawe. Niba basanze polyps amagana, cyane cyane ku muntu uri munsi y’imyaka 50, FAP iba ishobora kubaho.

Gusuzuma gène bishobora kwemeza uburwayi bwa FAP binyuze mu gushaka impinduka muri gène ya APC. Iki kizamini gikoreshwa mu gupima amaraso kandi gishobora kukubwira neza niba ufite gène ya FAP. Ariko kandi, iki kizamini ntikibona impinduka ya gène mu bantu bagera kuri 10-15% bafite FAP neza hashingiwe ku bimenyetso byabo.

Muganga wawe ashobora kandi kugusaba ibindi bipimo kugira ngo asuzume polyps cyangwa imikurire mu bindi bice by’umubiri wawe. Ibi bishobora kuba harimo endoscopy yo hejuru kugira ngo asuzume igifu na amara mato, cyangwa ibizamini byo kureba thyroid n’izindi ngingo.

Ubuvuzi bwa Familial Adenomatous Polyposis ni buhe?

Ubuvuzi nyamukuru bwa FAP ni kubagwa kwirinda kugira ngo hakuweho amara manini n’ikibuno mbere y’uko kanseri itera. Ibi bishobora kuguha ikibazo, ariko aya mabagiro ashobora gukiza ubuzima kandi akagufasha kubaho ubuzima busanzwe kandi bwiza nyuma yabyo.

Umuganga wawe azakugira inama yo gukora imwe muri iyi mibagiro ibiri nyamukuru. Total proctocolectomy hamwe na ileostomy ikuraho amara manini n’ikibuno byose, kandi ikora umwanya mu nda aho imyanda ishobora kuva mu gipfunsi. Ubundi, total proctocolectomy hamwe na ileal pouch-anal anastomosis ikuraho amara manini n’ikibuno ariko ikora umupfunsi wo mu mutima ukomoka mu mara mato, bikagufasha kugira imyanda nk’uko bisanzwe.

Igihe cyo kubagwa gishingiye ku bintu byinshi, birimo umubare wa polyps ufite, niba hari izirimo kugaragaza ibimenyetso byo kuba kanseri, n’imyaka yawe n’ubuzima bwawe muri rusange. Abantu benshi bafite classic FAP babagwa mu myaka yabo ya nyuma ya 10 cyangwa 20.

Niba utariteguye kubagwa cyangwa ufite attenuated FAP ifite polyps nke, muganga wawe ashobora kugusaba imiti nka sulindac cyangwa celecoxib. Aya miti ashobora kugufasha kugabanya ubukura bwa polyps, nubwo atabuza kanseri burundu kandi atari ikintu gishobora gusimbura kubagwa mu bihe bifite ibyago byinshi.

Ubugenzuzi buhoraho ni ingenzi na nyuma y’ubuvuzi. Uzakenera colonoscopies zihoraho ku buhoro bwose bwasigaye, ndetse no gusuzuma kanseri mu bindi bice by’umubiri wawe nka thyroid, igifu, na amara mato.

Uko wakwitwara muri iyi ndwara

Nubwo utazi gukumira cyangwa gukiza FAP binyuze mu guhindura imibereho, kwita ku buzima bwawe muri rusange bishobora kugufasha kumva neza kandi bikagabanya ibibazo bimwe na bimwe.

Komereza gukorana n’abaganga bawe kandi ube witabira gahunda yawe yose, nubwo wumva umeze neza. FAP isaba ubugenzuzi ubuzima bwose, kandi gusuzuma buri gihe ni byo birinda ibibazo.

Tekereza kwinjira mu itsinda ry’abantu bafite FAP cyangwa indwara ziterwa na gène. Guhuza n’abandi basobanukirwa ibyo unyuramo bishobora gutanga inkunga ikomeye yo mu mutima n’inama zifatika.

Komeza kwandika amateka yawe y’ubuvuzi, ibisubizo by’ibizamini, n’amakuru y’ubuzima bw’umuryango wawe. Aya makuru aba akomeye cyane niba wimukiye ahandi cyangwa uhindura abaganga, kandi ashobora kuba afatika ku bana bawe n’abandi bo mu muryango wawe.

Ntukabe ikibazo cyo kubaza ibibazo cyangwa gusaba ubundi buvuzi ku gahunda yawe y’ubuvuzi. FAP ni indwara igoranye, kandi ukwiye kumva ufite icyizere kandi uzi ibyemezo byawe by’ubuvuzi.

Uko wakwitegura kujya kwa muganga

Mbere yo kujya kwa muganga, kora uko ushoboye kose kugira ngo ubone amakuru menshi ku mateka y’ubuzima bw’umuryango wawe, cyane cyane ibyabayeho bya kanseri y’amara manini, polyps, cyangwa izindi kanseri. Nubwo amakuru ku bantu bo mu muryango bapfuye bakiri bato cyangwa bapfuye batamenyekanye impamvu, ashobora kuba afatika.

Andika ibimenyetso byawe byose, harimo igihe byatangiye, uko kenshi biba, n’icyo bibafasha cyangwa kibitera. Ntukwibagirwe kuvuga ibimenyetso bishobora kugaragara bitari byo, nko kwishima kw’uruhu cyangwa ibibazo by’amenyo.

Tegura urutonde rw’imiti yose, amavitamini, n’ibindi byongera ubuzima ukoresha. Nanone, andika ibibazo byose ushaka kubaza muganga wawe, utangire n’ibikomeye mu gihe udashobora kubona igihe gihagije.

Tekereza kuzana umuntu wo mu muryango wawe cyangwa inshuti yawe mu gahunda yawe, cyane cyane niba urimo kuvugana ku bijyanye no gusuzuma gène cyangwa uburyo bwo kuvura. Kugira undi muntu uri aho bishobora kugufasha kwibuka amakuru akomeye no gutanga inkunga yo mu mutima.

Niba uri kubona umuganga w’inzobere bwa mbere, saba muganga wawe usanzwe kohereza dosiye yawe y’ubuvuzi mbere y’igihe. Ibi bifasha kwemeza ko muganga wawe mushya afite amakuru yose akeneye kugira ngo aguhe ubuvuzi bwiza.

Icyo ugomba kumenya kuri Familial Adenomatous Polyposis

FAP ni indwara ikomeye iterwa na gène, ariko hakoreshejwe ubuvuzi n’ubugenzuzi bikwiye, abantu bafite FAP bashobora kubaho ubuzima buhagije kandi bwiza. Ikintu nyamukuru ni ugutahura hakiri kare no kuvura hakiri kare, ubusanzwe harimo kubagwa kwirinda mbere y’uko kanseri itera.

Niba ufite amateka y’iyi ndwara mu muryango wawe cyangwa kanseri y’amara manini itasobanuwe, inama z’abaganga b’indwara ziterwa na gène no gusuzuma bishobora kugufasha gusobanukirwa ibyago byawe no gufata ibyemezo byiza ku buzima bwawe. Gutangira hakiri kare bigira uruhare rukomeye muri iyi ndwara.

Wibuke ko kugira FAP ntibikumenya, kandi iterambere mu buvuzi rikomeza kunoza ibyavuye ku bantu bafite iyi ndwara. Korana n’abaganga bawe, umenye neza uko iyi ndwara imeze, kandi ntutinye gusaba ubufasha igihe ubukeneye.

Ibibazo bikunze kubaho kuri Familial Adenomatous Polyposis

Q1: Niba mfite FAP, abana banjye bazayirwara?

Oya, buri mwana wawe afite amahirwe 50% yo guherwa FAP niba ufite iyi gène. Ibi bivuze ko bamwe mu bana bawe bashobora kugira FAP abandi ntibayigire. Gusuzuma gène bishobora kugaragaza niba abana bawe baherwe iyi ndwara, ubusanzwe bitangira hagati y’imyaka 10-12.

Q2: Nshobora kwirinda FAP binyuze mu mirire cyangwa guhindura imibereho?

Ikibabaje ni uko ntushobora kwirinda FAP binyuze mu guhindura imibereho kuko iterwa n’impinduka za gène. Ariko kandi, kugira imirire myiza n’imibereho myiza bishobora kugufasha kugira ubuzima bwiza muri rusange kandi bishobora kugufasha gukira neza ubuvuzi nko kubagwa.

Q3: Ni kangahe nkeneye colonoscopies niba mfite FAP?

Ubwinshi bwabyo biterwa n’uko uhagaze, ariko abantu benshi bafite FAP bakenera colonoscopies buri mwaka 1-2 bahereye mu myaka yabo ya mbere ya 10. Nyuma yo kubagwa kwirinda, uzakenera ubugenzuzi buhoraho ku buhoro bwose bwasigaye, ubusanzwe buri mwaka 1-3.

Q4: Kubagwa kwa FAP ni ukubagwa gukomeye, kandi gukira ni gute?

Yego, kubagwa kwa FAP ni ukubagwa gukomeye mu nda, ariko ubuhanga bwateye imbere cyane mu myaka yashize. Abantu benshi bamara ibyumweru hafi icyumweru mu bitaro kandi bakenera ibyumweru 6-8 kugira ngo bakire neza. Abantu benshi basubira mu mirimo isanzwe, harimo akazi n’imyitozo ngororamubiri, nyuma yo gukira.

Q5: Nshobora kubyara abana nyuma yo kubagwa kwa FAP?

Yego, abantu benshi bashobora kubyara abana nyuma yo kubagwa kwa FAP, nubwo imibagiro imwe ishobora kugira ingaruka gato ku kubyara. Ni ingenzi kuvugana n’abaganga bawe mbere yo kubagwa kugira ngo bashobore kubitekereza mu gahunda yawe y’ubuvuzi. Abagore bafite FAP bashobora kubyara no kubyara abana nk’uko bisanzwe.