Hemofiliya Ni Iki? Ibimenyetso, Impamvu, n'Ubuvuzi

Hemofiliya ni indwara y'umuzuko iterwa n'ikibazo cy'imyororokere aho amaraso yawe adakama neza iyo wakomerekeye. Ibi bibaho kuko umubiri wawe utabasha gukora imwe mu mprotin uzwi nka facteur de coagulation zifasha guhagarika amaraso. Nubwo byumvikana biteye ubwoba, abantu babarirwa muri za miriyoni bafite hemofiliya babayeho ubuzima buzuye kandi buhamye bafashwe neza n'abaganga kandi bakurikiza amabwiriza.

Hemofiliya Ni Iki?

Hemofiliya ni indwara ikomoka ku miryango ifite ingaruka ku bushobozi bw'amaraso yawe bwo gukama. Iyo wikomerekeye cyangwa ukagira ikibazo, amaraso yawe agomba gukama akaba nk'igitambaro kugira ngo ahagarare. Abantu bafite hemofiliya bafite ibyiciro bike bya protin zikama amaraso, bityo amaraso akamara igihe kinini kugira ngo ahagarare.

Tekereza ko gukama kw'amaraso ari nk'urukurikirane rw'ibikorwa aho buri cyiciro gishingiye ku cyabanje. Muri hemofiliya, ikintu kimwe cy'ingenzi muri urwo rukurikirane kibura cyangwa kigabanuka. Ibi ntibivuze ko uzapfa amaraso kubera urupapuro rwagukomerekeje, ariko bivuze ko ibikomere bisaba kwitabwaho cyane no kuvurwa.

Iyi ndwara ikunda kwibasira abagabo, nubwo abagore bashobora kuba abatwaye iyi ndwara kandi rimwe na rimwe bagahura n'ibimenyetso bike. Ibaho kuva umuntu avutse, ariko ibimenyetso bishobora kutaboneka kugeza mu bwana iyo abana batangiye kugenda cyane.

Ni Izihe Nkoresho za Hemofiliya?

Hari ubwoko bubiri nyamukuru bwa hemofiliya, buri bwoko buterwa no kubura facteur de coagulation zitandukanye. Hemofiliya A ni yo yiganje, ikaba ikubiyemo abantu bagera kuri 80% bafite iyi ndwara. Ibi bibaho iyo umubiri wawe utabasha gukora protin ihagije ya facteur VIII.

Hemofiliya B, izwi kandi nka Christmas disease, ibaho iyo ubuze cyangwa ugira facteur IX nke. Ubu bwoko bwombi butera ibimenyetso bisa, ariko bukeneye uburyo butandukanye bwo kuvura kuko burimo facteur de coagulation zitandukanye.

Buri bwoko bushobora kuba buke, buciriritse, cyangwa bukabije bitewe n'ingano ya facteur de coagulation umubiri wawe utanga. Imikorere ikabije ifite munsi ya 1% y'ibipimo bisanzwe bya facteur de coagulation, mu gihe imikorere mike ishobora kugira 5-40% y'ibipimo bisanzwe.

Ni Izihe Nimenyetso bya Hemofiliya?

Ikimenyetso nyamukuru ni ukura amaraso igihe kirekire kurusha igihe gisanzwe nyuma yo gukomereka cyangwa ibikorwa by'abaganga. Ushobora kubona ko ibikomere bito bikamara igihe kinini kugira ngo amaraso ahagarare, cyangwa ko ukomeretswa vuba cyane kubera ibintu bito.

Dore ibimenyetso by'ingenzi byo kwitondera, uhereye ku bimenyetso bisanzwe:

• Ibisigo binini kandi byimbitse bigaragara vuba kubera ibintu bito
• Amaraso akomeza igihe kirekire nyuma yo gukomeretsa cyangwa kuvura amenyo
• Kuzana amaraso mu mazuru kenshi bitoroshye guhagarika
• Kubabara no kubyimba mu ngingo, cyane cyane mu mavi, amaboko, n'ibirenge
• Amaraso mu mpiswi cyangwa mu ntege
• Igihe cy'ukwezi kiremereye mu bagore batwaye iyi gene

Amaraso yo imbere mu ngingo n'imikaya ashobora gutera ububabare bukomeye no kubyimba. Ubu bwoko bw'amaraso bushobora kubaho nta gukomereka bigaragara, cyane cyane mu bihe bikomeye. Amaraso mu ngingo ni ikibazo cyane kuko ibintu byinshi bishobora kwangiza ingingo uko bwije n'uko bukeye.

Mu bihe bitoroshye ariko bikomeye, amaraso ashobora kuba mu bwonko cyangwa mu zindi ngingo z'ingenzi. Ibi bishoboka cyane nyuma yo gukomereka mu mutwe kandi bisaba ubufasha bw'abaganga vuba. Ibimenyetso birimo kubabara umutwe cyane, kuruka, gucika intekerezo, cyangwa intege ku ruhande rumwe rw'umubiri.

Ni Iki Gitera Hemofiliya?

Hemofiliya iterwa n'impinduka z'imyororokere zigira ingaruka ku mikorere ya facteur de coagulation. Izi mpinduka ziraturuka mu miryango, bisobanura ko zikomoka ku babyeyi ku bana binyuze muri gene ziri kuri chromosome X.

Kubera ko abagabo bafite chromosome X imwe gusa, bakeneye kopi imwe gusa ya gene yahindutse kugira ngo bagire hemofiliya. Abagore bafite chromosome X ebyiri, bityo bakeneye impinduka kuri zombi kugira ngo bagire iyi ndwara yose, ibyo bikaba bitoroshye.

Hagati ya bibiri bya gatatu by'ibintu byaturutse ku babyeyi batwaye iyi gene. Ariko kandi, hafi kimwe cya gatatu cy'ibintu bibaho kubera impinduka z'imyororokere zidasanzwe, bisobanura ko nta mateka y'iyi ndwara mu muryango.

Ihinduka ry'imyororokere rigira ingaruka ku mabwiriza umubiri wawe ukoresha mu gukora facteur VIII cyangwa IX. Utabonye izi protin zihagije, uburyo busanzwe bwo gukama kw'amaraso burahagarara, bigatera amaraso gukomeza igihe kirekire.

Ni Ryari Ukwiye Kubona Muganga kubera Hemofiliya?

Ugomba gushaka ubufasha bw'abaganga vuba niba ugize ibimenyetso by'amaraso akomeye. Ibi birimo kubabara umutwe cyane nyuma yo gukomereka mu mutwe, amaraso menshi adahagarara, cyangwa ibimenyetso by'amaraso yo imbere nk'amaraso mu mpiswi cyangwa mu ntege.

Kubibazo bikomeza, reba muganga wawe niba ubona ibimenyetso bidasanzwe byo gukomeretswa, amaraso mu mazuru kenshi, cyangwa ububabare n'ibyimba mu ngingo nta mpamvu isobanutse.

Niba ufite amateka y'umuryango wa hemofiliya kandi uteganya kubyara, inama y'abaganga ku bijyanye n'imyororokere irashobora kugufasha kumva ibyago. Abagore batwaye iyi ndwara bashobora kungukirwa no gupimwa kugira ngo bamenye ibipimo byabo bya facteur de coagulation.

Ubufasha bw'ibitaro ni ingenzi kuri buri gukomereka mu mutwe, ibikomere bikomeye, cyangwa amaraso adasubiza uburyo busanzwe bwo gutabara. Ntugatege amatwi ngo urebe niba amaraso ahagarara ubwabyo muri ibi bihe.

Ni Izihe Impamvu zishobora Guteza Hemofiliya?

Impamvu nyamukuru ni ukugira amateka y'umuryango wa hemofiliya, kuko ari indwara ikomoka ku miryango. Niba nyoko ari umutwayi cyangwa se wawe afite hemofiliya, ufite amahirwe menshi yo kuzaragwa iyi ndwara cyangwa kuba umutwayi.

Kuba umugabo byongera cyane ibyago byawe kuko impinduka z'imyororokere ziri kuri chromosome X. Abagabo bakeneye kopi imwe gusa ya gene yahindutse kugira ngo bagire hemofiliya, mu gihe abagore bakeneye kopi ebyiri.

Ndetse nta mateka y'umuryango, impinduka z'imyororokere zidasanzwe zishobora kubaho, nubwo ibyo bidasobanuka. Amwe mu moko ashobora kugira ibipimo bike byiyongereye, ariko hemofiliya ibasira abantu b'ingeri zose n'amoko yose ku isi.

Ni Izihe Ngaruka zishoboka za Hemofiliya?

Kwibasira ingingo ni imwe mu ngaruka zikomeye z'igihe kirekire iyo amaraso akomeza kuba mu ngingo imwe. Uko bwije n'uko bukeye, ibi bishobora gutera arthrite, ububabare buhoraho, no kugabanuka kw'ubushobozi bwo kugenda niba bitavuwe neza.

Dore ingaruka nyamukuru zishobora kubaho:

• Kwibasira ingingo burundu kubera amaraso akomeza kubaho
• Ububabare buhoraho no kugabanuka kw'ubushobozi bwo kugenda
• Amaraso mu mikaya ashobora gukanda imiyoboro y'imbere
• Dukurikira amaraso (bitoroshye hamwe no gupima ibinyabuzima bya none)
• Iterambere ry'ibintu bigira ingaruka ku buvuzi

Ibintu ni antikorora sisitemu y'umubiri rimwe na rimwe itera kuvura facteur de coagulation. Ibi bibaho ku bantu bagera kuri 20-30% bafite hemofiliya A ikomeye kandi bigatuma kuvura bigorana.

Mu bihe bitoroshye, amaraso akomeye ashobora guhitana ubuzima, cyane cyane iyo aba mu bwonko, mu muhogo, cyangwa mu zindi ngingo z'ingenzi. Ariko kandi, hamwe no kuvurwa neza no kwitabwaho, ingaruka nyinshi zishobora kwirindwa cyangwa kuvurwa neza.

Hemofiliya Imenyekanwa Gute?

Kumenya iyi ndwara bisanzwe bikubiyemo ibizamini by'amaraso bipima ibipimo bya facteur de coagulation yawe n'uburyo amaraso yawe akamara. Muganga wawe azatangira akuzuza amaraso n'ibizamini bipima igihe cyo gukama.

Ibipimo byihariye bya facteur bishobora kugaragaza neza facteur de coagulation ibura n'uburemere bw'icyo kibazo. Ibi bizamini bipima urwego rw'imikorere ya facteur VIII na IX mu maraso yawe.

Ibizamini by'imyororokere bishobora kugaragaza impinduka yihariye itera hemofiliya kandi bigafasha mu gupanga umuryango. Ibi bizamini bishobora kandi kugaragaza niba abagore bo mu muryango ari abatwayi ba gene.

Niba hari amateka y'umuryango, gupima bishobora gukorwa mu gihe cyo gutwita binyuze muri amniocentesis cyangwa chorionic villus sampling. Gupima abana bavutse bishya ntibikorwa buri gihe, ariko gupima birakenewe niba hari amateka y'umuryango azwi.

Ni Ubuhe Bvuzi bwa Hemofiliya?

Ubuvuzi nyamukuru burimo gusimbuza facteur de coagulation ibura binyuze mu gukingira. Izi facteur concentrates zishobora gutangwa buri gihe kugira ngo zirinde amaraso cyangwa iyo amaraso abaho.

Ubuvuzi bwo kwirinda bisobanura guhabwa inkingo za facteur buri gihe kugira ngo habeho ibipimo bihagije byo gukama. Ubu buryo bufasha kwirinda amaraso adasanzwe no kwangiza ingingo, cyane cyane mu bantu bafite hemofiliya ikomeye.

Ubuvuzi bujyanye n'igihe bisobanura gutanga facteur concentrates gusa iyo amaraso abaho. Ubu buryo bushobora gukwiriye abantu bafite hemofiliya mike cyangwa iciriritse badafite amaraso akenshi.

Ubuvuzi bushya burimo facteur zikora igihe kirekire zisaba inkingo nke kandi ubuvuzi budakoresha facteur bukora mu buryo butandukanye kugira ngo bufashe gukama. Bamwe mu bantu bagira akamaro mu miti ifasha kwirinda kwangirika kw'amaraso.

Uburyo bwo Kwita ku Buzima bw'Imurire muri Hemofiliya?

Abantu benshi bafite hemofiliya bigisha gutanga inkingo za facteur mu rugo. Ibi bifasha kuvura vuba iyo amaraso abaho kandi bigatuma kuvura kwirinda birushaho koroherwa.

Gabanya ibikoresho byo gutabara byuzuye hamwe na facteur concentrates, seringues, n'ibindi bikoresho. Menya neza ko abagize umuryango bazi uburyo bwo kumenya ibimenyetso by'amaraso no igihe cyo gushaka ubufasha bw'ibitaro.

Shyiraho igikombe n'umuvuduko ku bice by'amaraso mugihe utegura kuvura facteur. Shyira hejuru igice cyakomerekeye niba bishoboka kandi wirinde imiti nk'aspirin ishobora kongera ibyago by'amaraso.

Komeza ibitabo by'amaraso kugira ngo ukureho ibintu, kuvura, n'ibisubizo. Aya makuru afasha itsinda ryawe ry'ubuvuzi kugira ngo rihindure gahunda yawe yo kuvura kandi rimenye imiterere.

Hemofiliya Ishobora Kwibasirwa Gute?

Kubera ko hemofiliya ari indwara y'imyororokere, ntishobora kwirindwa mu buryo busanzwe. Ariko kandi, inama y'abaganga ku bijyanye n'imyororokere no gupima bishobora gufasha imiryango gufata ibyemezo byiza ku bijyanye no kubyara.

Abatwayi bashobora gupimwa kugira ngo bamenye ibyago byabo byo guha abana babo iyi ndwara. Gupima abana batwite birahari ku miryango ifite amateka ya hemofiliya azwi.

Icyo ushobora kwirinda ni ingaruka z'amaraso binyuze mu kuyagenzura neza. Ubuvuzi bwo kwirinda buhoraho, kwirinda ibikorwa by'ibyago, no kugira isuku nziza y'amenyo byose bifasha kugabanya amaraso.

Kuguma uzi inkingo no kugira ubuzima bwiza muri rusange bifasha kwirinda indwara zishobora kugorana kuvura hemofiliya.

Ugomba Gutegura Gute Urugendo rwawe kwa Muganga?

Mbere y'urugendo rwawe, kora amakuru ku mateka y'umuryango w'indwara z'amaraso. Andika ibibazo byihariye ku bimenyetso, uburyo bwo kuvura, n'impinduka mu mibereho.

Zana urutonde rw'imiti yose n'ibindi bikoresho ukoresha, harimo n'ibicuruzwa byo mu maduka. Komeza umubare w'amaraso yabayeho vuba, harimo igihe byabayeho n'igihe byamaze.

Tegura kuganira ku rwego rwawe rw'imikorere n'ibindi bikorwa byose wabonye. Muganga wawe akeneye kumva uko hemofiliya igira ingaruka ku buzima bwawe bwa buri munsi kugira ngo agufashe kuvura neza.

Tekereza kuzana umuntu wo mu muryango cyangwa inshuti kugira ngo aguhe inkunga kandi agufashe kwibuka amakuru y'ingenzi yavuzwe mu gihe cy'urugendo.

Ni Iki K'ingenzi cyo Kumenya kuri Hemofiliya?

Hemofiliya ni indwara y'imyororokere ishobora kuvurwa igira ingaruka ku gukama kw'amaraso, ariko ntigomba kugabanya ubuzima bwawe cyane. Hamwe no kuvurwa neza, kuvurwa, no guhindura imibereho, abantu benshi bafite hemofiliya bashobora kubaho ubuzima buzuye kandi buhamye.

Kumenya iyi ndwara hakiri kare no kuvurwa neza ni ingenzi mu kwirinda ingaruka nk'ukwangirika kw'ingingo. Gukorana n'itsinda ry'abaganga b'inzobere bifasha guhamya ko uhabwa ubuvuzi bujyanye n'igihe.

Ikintu nyamukuru ni ukumva ubwoko bwawe bw'ihariye n'uburemere bwa hemofiliya, hanyuma ukora gahunda yuzuye yo kuvura ihuye n'imibereho yawe. Gukurikirana buri gihe no guhindura ibintu bifasha kunoza ubuvuzi bwawe uko bwije n'uko bukeye.

Ibibazo Bikunze Kubahwa kuri Hemofiliya

Abantu bafite hemofiliya bashobora gukina siporo?

Yego, abantu benshi bafite hemofiliya bashobora gukina siporo bafite ubwirinzi bukwiye. Ibikorwa bitakora cyane nk'koga, gusiganwa, na tennis ni amahitamo meza. Siporo zikora cyane zisaba kwitondera no kuganira n'itsinda ryawe ry'ubuvuzi ku bijyanye n'ingamba z'ubwirinzi n'impinduka mu buvuzi.

Hemofiliya yandura?

Oya, hemofiliya ntiyandura. Ni indwara y'imyororokere uvuka ufite, si indwara ishobora gukwirakwira kuva ku muntu umwe ku wundi. Ntushobora kwandura hemofiliya uba hafi y'umuntu uyifite cyangwa amaraso akakugeraho.

Abagore bashobora kugira hemofiliya?

Nubwo bitoroshye, abagore bashobora kugira hemofiliya niba baragwa gene yahindutse ku babyeyi bombi. Akenshi, abagore ni abatwayi bashobora kugira ibimenyetso bike by'amaraso. Abatwayi b'abagore bagifite ibibazo by'amaraso, cyane cyane mu gihe cy'ukwezi, kubyara, cyangwa kubagwa.

Abantu bafite hemofiliya babayeho igihe kingana iki?

Hamwe no kuvurwa kwa none, abantu bafite hemofiliya bashobora kugira igihe cyo kubaho gisanzwe cyangwa hafi gisanzwe. Ikintu nyamukuru ni ugukira ubufasha bw'abaganga bukwiye, gukurikiza gahunda yo kuvura, no gucunga iyi ndwara mu buryo bwo kwirinda ingaruka.

Hari ubuvuzi bwa hemofiliya?

Ubu, nta buvuzi bwa hemofiliya, ariko ubuvuzi burahamye mu gucunga iyi ndwara. Ubushakashatsi ku bijyanye no kuvura gene bwerekana ibyiringiro by'ejo hazaza, kandi igeragezwa rimwe ryagaragaje ibyiza mu gutanga ibisubizo byo kuvura igihe kirekire.