Indwara ya Hirschsprung ni iki? Ibimenyetso, Impamvu, n'Ubuvuzi

Indwara ya Hirschsprung ni uburwayi umuntu avukana butera ikibazo mu ruhago rukuru (colon) bigatuma abana batabasha guhita bagira umwanya wo kujya ku musarani. Iyi ndwara ibaho iyo hari utunyangingo tw'imbere mu ruhago rudafasha mu gukuraho imyanda mu ruhago rukuru.

Umwana umwe kuri 5000 avukana iyi ndwara, kandi igaragara cyane mu bahungu kurusha abakobwa. Inkuru nziza ni uko iyo havuzwe neza, abana bafite indwara ya Hirschsprung bashobora kubaho ubuzima bwiza kandi busanzwe.

Indwara ya Hirschsprung ni iki?

Indwara ya Hirschsprung ibaho iyo utunyangingo tw'imbere mu ruhago rukuru twitwa ganglion cells tudakura neza mu ruhago rukuru. Utunyangingo duhari dufasha imitsi y'amara kumenya igihe igomba kuruhuka n'igihe igomba gukora kugira ngo imyanda igende.

Iyo utwo tunyangingo tudahari, igice cy'amara cyahindutse ntikibasha gukuraho imyanda uko bikwiye. Tekereza nk'umuyoboro w'amazi ufite ikibazo-ibintu byose bihagarara inyuma y'aho byabuze inzira.

Iyi ndwara ihera ku kibuno maze igakwirakwira mu ruhago rukuru. Mu bihe byinshi, igice gito cy'amara ni cyo kibwirwa, ariko rimwe na rimwe gishobora kugera no ku bice birebire by'amara.

Ni ibihe bimenyetso by'indwara ya Hirschsprung?

Ibimenyetso by'indwara ya Hirschsprung bigaragara mu nda y'ibyumweru bike bya mbere, nubwo rimwe na rimwe bitagaragara kugeza umwana akuruye. Buri mwana ni umwihariko, ariko hari ibimenyetso bisanzwe bikwiye kwitonderwa.

Mu bana bashya, ibimenyetso by'ingenzi birimo:

• Kudakora umwanya wa mbere (meconium) mu masaha 48 nyuma yo kuvuka
• Kuruka ibara ry'icyatsi cyangwa icyatukura
• Igifu kibyimba kandi kigoye
• Kuvuza umuyaga ariko nta mwanya wo kujya ku musarani
• Kubura ubushake bwo kunywa ibere no kudakura neza
• Gukora umwanya cyane nyuma yo gushyira urutoki cyangwa thermometer mu kibuno

Ibi bimenyetso bya mbere bibaho kuko imyanda itabasha kunyura mu gice cy'amara cyahindutse. Ibi bituma habaho igitutu kidakwiye kandi bikabuza kunywa ibere no gukura neza.

Mu bana bato n'abana bakuru, ushobora kubona:

• Impatwe zidakira n'ubwo wakoresheje uburyo busanzwe bwo kuvura
• Kudakura uko bikwiye
• Uburwayi n'ubushobozi buke
• Igifu kibyimba
• Gukora umwanya gake kandi ari mwinshi

Bamwe mu bana bafite iyi ndwara mu buryo buke bashobora kutayigaragaza kugeza igihe bageze mu kigero cy'abana bato cyangwa se bakuru. Ibi bihe bishobora kuba bigoye kubimenya kuko ibimenyetso bishobora kugaragara nk'impatwe zisanzwe.

Ni iki giteza indwara ya Hirschsprung?

Indwara ya Hirschsprung iba mu gihe cyo gutwita hakiri kare iyo umwana ari mu nda. Impamvu nyamukuru ntiyaramenyekana neza, ariko tuzi ko ijyana n'ibibazo by'uko utunyangingo tw'imbere mu mubiri dukura kandi tugenda.

Mu mezi make ya mbere yo gutwita, utunyangingo tw'imbere mu mubiri dutangira mu buryo bw'amara y'umwana maze buhoro buhoro tugana ku kibuno. Mu bana bafite indwara ya Hirschsprung, utwo tunyangingo duhagarara mbere y'uko tugera aho tugomba kugera.

Iyi ndwara isa n'ifite ibintu by'umurage, bisobanura ko ishobora kuba mu muryango. Niba umwana umwe afite indwara ya Hirschsprung, abavandimwe be bafite amahirwe ari hagati ya 3-12% yo kuyifata, bitewe n'ibintu bitandukanye.

Hari imigeni myinshi ihujwe n'iyi ndwara, gene RET ikaba ari yo ikunze kugaragara. Ariko, kugira izi mpinduka z'imigeni ntibihamya ko umwana azagira iyo ndwara-byongera gusa amahirwe.

Ni iyihe mitype y'indwara ya Hirschsprung?

Abaganga basobanura indwara ya Hirschsprung bashingiye ku bice by'amara byabuze utunyangingo tw'imbere mu mubiri. Gusobanukirwa ubwoko bufasha mu gupima uburyo bwiza bwo kuvura.

Indwara ya short-segment ni yo ikunze kugaragara, igize hafi 80% by'ababirwayi. Muri ubwo bwoko, igice gito cy'amara (kibuno na sigmoid colon) ni cyo kibwirwa. Abana bafite ubwo bwoko bakunze kugira ibimenyetso bidakomeye kandi bakira neza nyuma yo kuvurwa.

Indwara ya long-segment igira ingaruka ku gice kinini cy'amara kandi iba mu kigero cya 20% by'ababirwayi. Ubwo bwoko bushobora gutera ibimenyetso bikomeye kandi bishobora gusaba uburyo bugoye bwo kuvura. Bamwe mu bana bashobora gukenera kubagwa inshuro nyinshi cyangwa bagakomeza kugira ibibazo by'amara.

Mu bihe bitoroshye, iyi ndwara ishobora kugira ingaruka ku ruhago rwose cyangwa ikagera no mu mara mato. Ibi bihe bisaba ubuvuzi bwihariye kandi bikunze gukorwa n'itsinda ry'abaganga b'abana.

Ni ryari ukwiye kujya kwa muganga kubera indwara ya Hirschsprung?

Ukwiye guhamagara muganga w'umwana wawe ako kanya niba umwana wawe mushya adakora umwanya wa mbere mu masaha 48 nyuma yo kuvuka. Iki ni kimwe mu bimenyetso by'ingenzi byo kuburira.

Izindi mpamvu zihutirwa zo gushaka ubuvuzi harimo kuruka ibara ry'icyatsi cyangwa icyatukura, igifu kibyimba kandi kigoye, cyangwa ibimenyetso byerekana ko umwana adashobora kunywa ibere neza kandi asa n'utabyishimiye. Ibi bimenyetso bishobora kugaragaza ikibazo gikomeye gisaba ubuvuzi bwihuse.

Ku bana bato n'abana bakuru, impatwe zidakira n'ubwo wakoresheje impinduka mu mirire cyangwa uburyo bworoshye bwo kuvura bigomba gusuzumwa na muganga. Niba umwana wawe adakora umwanya inshuro eshatu mu cyumweru cyangwa asa n'uhangayitse cyane, ni byiza kubiganiraho na muganga.

Ntugatege amatwi niba ubona umwana wawe adakura cyangwa adakura neza, cyane cyane iyo bibaye hamwe n'ibimenyetso by'amara. Kumenya hakiri kare no kuvura birashobora gukumira ingaruka mbi kandi bigafasha umwana wawe gukura neza.

Ni ibihe bintu byongera ibyago byo kugira indwara ya Hirschsprung?

Hari ibintu byinshi bishobora kongera amahirwe y'uko umwana avukana indwara ya Hirschsprung. Gusobanukirwa ibyo bintu bishobora gufasha imiryango n'abaganga kuba maso ku bimenyetso bya mbere.

Kuba umuhungu ni yo ngingo ikomeye y'ibyago-abahungu bafite amahirwe ane yo kugira iyi ndwara kurusha abakobwa. Impamvu y'itandukaniro ry'igitsina ntiyaramenyekana neza, ariko igaragara cyane mu bantu batandukanye.

Amateka y'umuryango agira uruhare runini. Kugira umubyeyi cyangwa umuvandimwe ufite indwara ya Hirschsprung byongera ibyago cyane. Ibyago biri hejuru niba umuntu ufite iyo ndwara ari umukobwa cyangwa afite ubwoko bwa long-segment bw'indwara.

Hari uburwayi bumwe na bumwe bw'imigeni bujyana n'indwara ya Hirschsprung, harimo na Down syndrome, iba mu kigero cya 2-10% cy'abana bafite iyi ndwara y'amara. Ubundi burwayi bw'imigeni nka Waardenburg syndrome na congenital central hypoventilation syndrome na byo byongera ibyago.

Hari impinduka nke z'imigeni zishobora gutuma imiryango iba mu kaga, nubwo izi mpamvu zigize igice gito cy'ababirwayi. Abana benshi bafite indwara ya Hirschsprung ntabwo bafite izi mpinduka z'imigeni.

Ni iyihe ngaruka mbi zishoboka z'indwara ya Hirschsprung?

Nubwo indwara ya Hirschsprung ivurwa neza, ishobora gutera ingaruka mbi zikomeye niba itabonywe kandi itabayeho neza. Kumenya ibyo bibazo bishobora gufasha mu kuvura vuba.

Ingaruka mbi ikomeye ni uburwayi bwitwa enterocolitis, ari bwo kubyimba kw'amara. Ibi bishobora kuba mbere cyangwa nyuma yo kubagwa kandi bisaba ubuvuzi bwihuse. Ibimenyetso birimo umuriro, guhitamo, kuruka, n'igifu kibyimba.

Toxic megacolon ni indi ngaruka mbi ikomeye aho amara aba akomeye cyane kandi ababara. Ubwo burwayi buhitana umuntu bushobora gutera uruhu rw'amara gusenyuka niba budakize vuba hakoreshejwe imiti igabanya ubukana n'ubuganga bwihuse.

Ibibazo byo gukura no kurya bishobora kubaho iyo abana badashobora kubona intungamubiri neza kubera ibibazo by'amara. Bamwe mu bana bashobora gukenera ubufasha bwihariye mu mirire cyangwa imiti ifasha mu gukura.

Nyuma yo kubagwa, bamwe mu bana bashobora kugira ibibazo bikomeza nk'impatwe zidakira, guhita bagira umwanya, cyangwa kugira ikibazo cyo kujya ku musarani. Ariko, bihanganye kandi bakurikiranwe neza, ibyinshi muri ibyo bibazo birakira neza uko iminsi igenda.

Ikintu nyamukuru cyo gukumira ingaruka mbi ni ukumenya hakiri kare, kuvura neza, no gukurikiranwa n'abaganga. Abana benshi bavurwa neza bakura neza.

Indwara ya Hirschsprung imenyekanwa ite?

Kumenya indwara ya Hirschsprung bisaba ibizamini byinshi bifasha abaganga kureba uko amara akora neza niba utunyangingo tw'imbere mu mubiri duhari. Uburyo busanzwe burasobanutse, nubwo bishobora gusaba ibizamini bitandukanye kugira ngo hamenyekane neza.

Muganga wawe azatangira akugenzura umubiri kandi akamenya amateka yawe y'ubuzima. Azakubaza ibyerekeye umwanya, uburyo bwo kurya, nibimenyetso byose wabonye. Azanagenzura igifu cy'umwana wawe kugira ngo arebe niba kibyimba cyangwa hari aho kibabaza.

Barium enema ni ikizamini cyo kubona ishusho cyakunze gukorwa bwa mbere. Umwana wawe anywa cyangwa ahabwa umuti wihariye ugaragara kuri X-rays, bituma abaganga babona imiterere n'imikorere y'amara. Mu ndwara ya Hirschsprung, iki kizamini kigaragaza igice gito gikurikirwa n'igice kinini hejuru.

Kumenya neza biba binyuze mu gukuramo igice gito cy'umubiri mu kibuno kugira ngo gisuzuzwe kuri microscope. Iki kizamini gishobora kugaragaza neza niba utunyangingo tw'imbere mu mubiri duhari cyangwa tudahari.

Rimwe na rimwe abaganga bakoresha anorectal manometry, ikizamini kipima igitutu n'imikorere y'imitsi mu kibuno. Ibi bishobora gufasha kumenya imiterere idasanzwe y'imitsi iranga indwara ya Hirschsprung.

Ni iki kivura indwara ya Hirschsprung?

Uburyo nyamukuru bwo kuvura indwara ya Hirschsprung ni ukubaga kugira ngo bakureho igice cy'amara cyabuze utunyangingo tw'imbere mu mubiri maze bakahuze ibice byiza. Nubwo ibi bishobora kugaragara nk'ibidasanzwe, ibi bibaga bikunda kugira icyo bikora kandi bifasha abana gusubira mu buzima busanzwe.

Abana benshi bagira icyo twita "pull-through" procedure, aho ababagisha bakuraho igice cy'amara cyahindutse maze bakazana igice cyiza kugira ngo kihuze n'ikibuno. Ibi bikunze gukorwa mu gihe kimwe, cyane cyane ku ndwara ya short-segment.

Bamwe mu bana, cyane cyane abafite indwara ikomeye cyangwa ingaruka mbi, bashobora gukenera colostomy by'agateganyo. Ibi bituma habaho umwanya mu gifu aho imyanda ishobora kuva, bigatuma amara mato aruhuka kandi agakira mbere y'ubuganga nyamukuru.

Ubuganga busanzwe bukorwa n'abaganga babagisha abana babishoboye. Ibi bibaga bikunze gukorwa hifashishijwe uburyo buto, bisobanura ko habaga ibikomere bito kandi umwana agakira vuba.

Nyuma yo kubagwa, abana benshi bagaruka mu buzima busanzwe, nubwo bishobora kumara amezi kugira ngo byose bigende neza. Itsinda ryanyu ry'abaganga rizatanga amabwiriza arambuye yo kwita ku mwana nyuma yo kubagwa no gukurikirana.

Uko wakwita ku mwana wawe mu rugo mu gihe ari gukira

Kwita ku mwana wawe mu rugo nyuma yo kubagwa bisaba gukurikiza amabwiriza y'abaganga bawe neza no kureba ibimenyetso byo gukira cyangwa ibibazo bishoboka. Imiryango myinshi isanga iyo yiteguye neza, gukira mu rugo bigenda neza.

Komereza ahantu habagwe hakeye kandi humye hakurikijwe amabwiriza y'umuganga wawe. Ushobora gukenera guhindura imyenda buri gihe kandi ukareba ibimenyetso by'indwara nko kubyimba, kubyimba, cyangwa ibintu bivuye mu kibomere. Ntukabe ikibazo guhamagara muganga wawe niba ufite ikibazo.

Kurya bishobora gukenera guhinduka, cyane cyane niba umwana wawe yarakorewe colostomy. Itsinda ryanyu ry'abaganga rizatanga amabwiriza yihariye ku biribwa byo gutanga n'igihe cyo kongera ibiryo. Tangira buhoro buhoro kandi urebe uko umwana wawe yihanganira ibiryo bitandukanye.

Gufasha umwana wawe kudafata ububabare ni ingenzi kugira ngo akire neza. Muhe imiti uko yategetswe kandi ntuyireke nubwo umwana wawe asa n'aho ameze neza. Kugira gahunda yo kugabanya ububabare bifasha abana gukira vuba.

Kureba ibimenyetso by'ingaruka mbi nko kugira umuriro, kuruka bikomeye, cyangwa impinduka mu mwanya bikubabaza. Itsinda ryanyu ry'abaganga rizatanga urutonde rw'ibimenyetso byo kuburira n'amakuru yo guhamagara mu gihe cy'akaga.

Wibuke ko buri mwana akura mu buryo bwe. Bamwe bashobora gusubira mu bikorwa bisanzwe mu byumweru bike, abandi bakenera igihe kinini. Kurikiza umwana wawe kandi ntukihute mu nzira yo gukira.

Uko wakwitegura kujya kwa muganga

Kwita ku mwana wawe mu rugo nyuma yo kubagwa bisaba gukurikiza amabwiriza y'abaganga bawe neza no kureba ibimenyetso byo gukira cyangwa ibibazo bishoboka. Imiryango myinshi isanga iyo yiteguye neza, gukira mu rugo bigenda neza.

Komeza inyandiko irambuye y'imyanya y'umwana wawe, harimo kenshi, imiterere, nibintu byose ubona. Kandi komeza inyandiko y'imirire, impinduka z'uburemere, nibimenyetso byose nko kuruka cyangwa kubabara mu gifu. Aya makuru afasha abaganga gusobanukirwa neza icyabaye.

Andika ibibazo byawe byose mbere yo kujya kwa muganga kugira ngo utabyibagirwa. Harimo ibibazo ku bijyanye no kumenya indwara, uburyo bwo kuvura, icyo witeze mu gihe cyo gukira, nibibazo byose ku bijyanye n'ingaruka z'igihe kirekire.

Zana urutonde rwuzuye rw'imiti cyangwa ibindi bintu umwana wawe afata, harimo n'umwanya wo kuyifata. Vuga kandi ibyo umwana wawe agira allergie cyangwa uko yigeze agira allergie.

Niba bishoboka, zana undi muntu wo mu muryango cyangwa inshuti kugira ngo aguhe inkunga, cyane cyane niba muganira ku bijyanye no kubagwa cyangwa gahunda zikomeye zo kuvura. Kugira undi muntu wumva bishobora kugufasha kwibuka amakuru no kugufasha mu byiyumvo.

Ntugatinye gusaba amakuru yanditse cyangwa ibikoresho ku bijyanye n'uburwayi bw'umwana wawe. Imiryango myinshi isanga ari byiza kugira ibikoresho bashobora gusuzuma mu rugo igihe batari bahangayitse.

Icyingenzi ku bijyanye n'indwara ya Hirschsprung

Indwara ya Hirschsprung ni uburwayi buvurwa bujyana n'ikibazo cyo gukuraho imyanda mu ruhago rukuru. Nubwo kumenya iyi ndwara bishobora kugaragara nk'ibidasanzwe, abana benshi bavurwa neza bakura neza.

Kumenya hakiri kare no kuvura ni ingenzi kugira ngo hamenyekane neza. Niba ubona ibimenyetso nko gutinda gukora umwanya mu bana bashya cyangwa impatwe zidakira mu bana bakuru, ntutinye kubiganiraho na muganga.

Kubagwa bifasha cyane mu kuvura iyi ndwara, kandi abana benshi bagaragaza impinduka nyinshi mu bimenyetso byabo nyuma yabyo. Nubwo gukira bisaba igihe no kwihangana, imiryango ikunze kubona ko ubuzima busubira mu buryo mu mezi make nyuma yo kuvurwa.

Wibuke ko kugira indwara ya Hirschsprung ntibibuza umwana wawe kugera ku byo ashaka mu buzima. Hamwe no kwitabwaho neza n'inkunga yawe yuje urukundo, abana bafite iyi ndwara bagira uruhare mu ishuri, imikino, n'ibikorwa byose bigira uruhare mu buzima bw'abana.

Ibibazo bikunze kubaho ku bijyanye n'indwara ya Hirschsprung

Ese umwana wanjye azashobora kugenzura imyanya ye neza nyuma yo kubagwa?

Abana benshi bagira ubushobozi bwo kugenzura imyanya yabo nyuma yo kubagwa, nubwo bishobora kumara amezi cyangwa umwaka kugira ngo byose bigende neza. Bamwe mu bana bashobora gukenera ubufasha bundi mu kujya ku musarani cyangwa gucunga impanuka, ariko abenshi bagira ubushobozi bwo kugenzura imyanya yabo uko iminsi igenda. Itsinda ryanyu ry'abaganga rizakorana namwe kugira ngo mukemure ibibazo bikomeza kandi mufashe umwana wawe kugira icyo ageraho.

Ese indwara ya Hirschsprung irashobora gukumirwa?

Nta buryo bwo gukumira indwara ya Hirschsprung ubu kuko ari uburwayi buva mu iterambere ry'umubiri mu gihe cyo gutwita. Ariko, niba ufite amateka y'iyi ndwara mu muryango, kugisha inama ku bijyanye n'imigeni mbere yo gutwita bishobora kugufasha gusobanukirwa ibyago by'umuryango wawe. Ikintu cy'ingenzi ni ukumenya hakiri kare no kuvura igihe umwana avutse.

Kubagwa bimamara igihe kingana iki kandi gukira bigenda gute?

Kubagwa kwa pull-through bisanzwe bimamara amasaha 2-4, bitewe n'igice cy'amara cyahindutse. Abana benshi baba mu bitaro iminsi 3-7 nyuma yo kubagwa. Gukira burundu bisanzwe bimamara ibyumweru bike kugeza ku mezi make, aho umwana wawe azasubira mu bikorwa bisanzwe no kurya. Itsinda ryanyu ry'abaganga rizatanga gahunda irambuye y'igihe n'uburyo bwo gukira.

Ese umwana wanjye azakenera imihati yihariye mu mirire nyuma yo kuvurwa?

Abana benshi bashobora gusubira mu mirire isanzwe nyuma yo kubagwa no gukira. Mu ntangiro, muganga wawe ashobora kugutegeka gutangira ibiryo byoroshye kandi buhoro buhoro ukongeraho ibindi. Bamwe mu bana bagira inyungu mu kurya ibiryo bike, byinshi cyangwa bongera fibre mu mirire yabo kugira ngo bafashe amara gukora neza, ariko ibi si imihati ikomeye.

Ni ayahe mahirwe y'uko abana bazakurikiraho bazagira indwara ya Hirschsprung?

Niba ufite umwana umwe ufite indwara ya Hirschsprung, ibyago by'abana bazakurikiraho biri hejuru kurusha abandi ariko bikiri bike. Ibyago nyabyo biterwa n'ibintu nko gukura kw'umwana n'uburemere bw'indwara, ariko bisanzwe biri hagati ya 3-12%. Muganga wawe ashobora gutanga amakuru arambuye ku byago hakurikijwe imimerere y'umuryango wawe kandi ashobora kugutegeka kugisha inama ku bijyanye n'imigeni.