Ese nawe ntugira ikibazo cyo guhagarika kuva amaraso? Reka twige ku ndwara ya hemophilia!

Ese wigeze wumva indwara yitwa Hemophilia ? Ushobora kumva ufite ubwoba gato iyo wumvise iri zina. Ariko ntugahangayike, kuko iyi ni indwara idakunze kugaragara. Uyu munsi, turaganira ku ndwara ya hemophilia, uko ikura, ibimenyetso byayo, ndetse niba hari uburyo bwo kuyivura, byose mu buryo bworoshye ushobora gusobanukirwa.

Hemophilia ni iki?

Muri make, hemophilia ni indwara y’amaraso ivuka mu bana. Ibi bivuze ko amaraso yawe adafatana neza. Ibi bishobora gutera kuva amaraso menshi, cyangwa se gukomereka gato.

Tekereza nawe, hari poroteyine zidasanzwe mu mibiri yacu zifasha amaraso yacu kubumba. Ibyo tubyita 'ibintu bibumba' . Bameze nk'abasirikare bato bakora kugira ngo bahagarike kuva amaraso. Iyo urwaye hemophilia, umubiri ntukora kimwe cyangwa byinshi muri ibyo 'bintu bibumba' bihagije. Hanyuma amaraso abumba make kandi uva amaraso menshi.

Hari ubwoko bwinshi bw'ingenzi bwa hemophilia. Iyi ndwara ishobora kuba ikomeye, iri hagati, cyangwa yoroheje, bitewe n'ingano y'ibintu bituma amaraso agwa.

Inkuru nziza ni uko hari uburyo bwo kuvura ibi. Abaganga barimo kugerageza gusubiza ibintu bigabanya amaraso mu mubiri. Kuri ubu nta muti wa hemophilia uraboneka. Ariko, abantu bafite iyi ndwara basanzwe bamara igihe kirekire nk'abandi iyo bavuwe neza. Abahanga mu bya siyansi barimo kandi gukora ubushakashatsi ku buryo bushya, nko kuvura imiterere y'uturemangingo , kugira ngo barebe niba ishobora gukira.

Ese hemophilia ishobora kuvuka nyuma yo kuvuka?

Yego, birashoboka. Ariko ni gake cyane. Ibi byitwa 'hemophilia iboneka'. Ibyo bivuze ko atari umurage. Ikibaho hano ni uko poroteyine zo kwirwanaho z'umubiri wacu , zitwa antibodies , rimwe na rimwe zitera nabi kimwe mu bintu bituma amaraso yacu agwa. Izi antibodies zitera mu buryo butari bwo zitwa 'autoantibodies' .

Ese hemophilia ni indwara isanzwe?

Oya, oya rwose. Iyi ni indwara idakunze kugaragara cyane. Dukurikije imibare yo muri Leta Zunze Ubumwe za Amerika (CDC Kanama 2022), abantu bagera ku 33.000 baho bafite hemophilia. Ikunze kwibasira abagabo. Ni gake cyane abagore bashobora no kugira ibimenyetso nko kugabanuka kw'amaraso no kuva amaraso menshi mu gihe cy'imihango.

Ni ubuhe bwoko bwa hemophilia?

Hari ubwoko butatu bw'ingenzi bwa hemophilia:

• Hemofiliya A: Iyi ni yo ndwara ikunze kugaragara cyane. Iterwa n'ibura ry'umuti wo kuvura amaraso nimero ya 8, izwi kandi nka factor VIII., iyo umubiri udafite ibyo bihagije. Abantu bagera ku 10 kuri 100.000 bashobora kugira ibi.
• Hemophilia B: Ibi biterwa no kubura kwa factor 9 yo kuvura amaraso, cyangwa factor IX . Abantu bagera kuri 3 ku 100.000 bafite ubwo bwoko bw'indwara.
• Hemophilia C: Nanone yitwa ikibazo cyo kubura factor XI , ibi ni gake cyane, bikagira ingaruka ku bantu bagera kuri umwe muri miliyoni.

Ese hemophilia ni indwara ikomeye?

Yego, rimwe na rimwe ibi bishobora kuba bikomeye. Abantu bafite hemophilia ikomeye bashobora kuva amaraso mu buryo bushyira ubuzima bwabo mu kaga, ibyo twita 'kuva amaraso menshi'. Ariko akenshi bava amaraso mu mitsi cyangwa mu ngingo.

Ni ibihe bimenyetso by'indwara yo kubura amaraso mu mubiri (hemophilia)?

Ibimenyetso by'ingenzi ni ukuva amaraso menshi cyangwa kuva amaraso menshi no gukomereka.

Ibimenyetso bikunze kugaragara

Abantu barwaye hemophilia bashobora kugira ibikomere binini ndetse n'impanuka nto. Ibi bivuze ko amaraso aba arimo kuva munsi y'uruhu.

• Wenda nyuma yo kubagwa, nyuma yo gukura iryinyo, cyangwa se gukomereka ku rutoki gusa , kuva amaraso bishobora kumara igihe kirekire kidasanzwe.
• Kuva amaraso mu mazuru bishobora kubaho mu buryo butunguranye nta mpamvu.

Uko amaraso yawe agabanuka cyangwa agabanuka biterwa n'uko ufite hemophilia ikomeye, iringaniye cyangwa yoroheje.

• Abantu bafite hemophilia ikomeye bashobora kuva amaraso kenshi nta mpamvu igaragara.
• Abantu bafite hemophilia iringaniye bashobora kuva amaraso igihe kirekire kidasanzwe iyo bagize impanuka ikomeye.
• Abantu bafite hemophilia yoroheje bava amaraso adasanzwe nyuma yo kubagwa bikomeye cyangwa impanuka ikomeye.

Hashobora kandi kuba hari ibindi bimenyetso:

• Kubabara ingingo bitewe no kwishyira hamwe kw'amaraso mu mubiri. Ahantu nko ku karenge, mu mavi, mu matako no mu bitugu hashobora kubabara, kubyimba, cyangwa kumva bishyushye iyo ubikozeho.
• Kuva amaraso mu bwonko. Ibi ni ibintu bidasanzwe ku bantu bafite hemophilia ikomeye. Niba ufite amaraso mu bwonko, ushobora kugira umutwe udashira, kutabona neza, cyangwa gusinzira cyane . Niba ufite hemophilia, ugomba guhita ubonana na muganga niba ubonye kimwe muri ibi bimenyetso.

Ibimenyetso ku bana bato n'impinja

Hari igihe hemofiliya iboneka iyo umwana w'umuhungu akebwe kandi akava amaraso menshi kurusha uko bisanzwe. Ubundi, abana bagaragaza ibimenyetso nyuma y'amezi make bavutse. Ibimenyetso bikunze kugaragara ni ibi bikurikira:

• Kuva amaraso:Impinja zishobora kuva amaraso mu kanwa iyo zirumaga agakinisho gato.
• Ibibyimba ku mutwe: Impinja n'abana bato bashobora kugira ibibyimba binini kandi byuzuye (amagi y'ingwe) iyo bikubise ku mutwe.
• Kurira kenshi, kubura amahoro, no kudashaka kugenda: Ibi bishobora kubaho iyo amaraso yinjiye mu mitsi cyangwa mu ngingo. Ahantu hashobora kumva hakomeretse, habyimbye, hashyushye iyo ukozeho, cyangwa umwana ashobora kumva ububabare nubwo yaba akozweho gato.
• Hematoma: 'Hematoma' ni uruhurirane rw'amaraso apfundika munsi y'uruhu mu bana bato n'impinja. Ubwo bwoko bwa hematoma bushobora no kubaho nyuma yo guterwa urushinge.

Ni izihe mpamvu zitera hemophilia?

Izi 'bintu bigabanya amaraso' zikomoka ku turemangingo tumwe na tumwe two mu mubiri wacu. Muri hemophilia y’urubyaro, uturemangingo twigisha ikorwa ry’izi 'bintu bigabanya amaraso' turahinduka, bivuze ko duhinduka. Izi jeni zahindutse zishobora gutuma 'ibintu bigabanya amaraso' bitari byo cyangwa ntizikore 'ibintu bigabanya amaraso' bihagije. Ariko, hafi 20% by'abarwayi ba hemophilia bahita barwara iyi ndwara, bivuze ko bashobora kuyirwara nubwo nta muntu n'umwe mu muryango wigeze ayirwara mbere.

Ni gute hemophilia iragwa?

Ubwoko bwombi bwa hemophilia A na B ni indwara zifitanye isano n'ibitsina. Ziranduranwa n'abantu mu buryo bwa X-linked recessive. Reka turebe uko bigenda:

• Twese tubona seti imwe ya chromosomes kuri mama wacu n'iseti imwe ya chromosomes kuri data. Iyo ubonye chromosome X kuri mama wawe na chromosome X kuri so, uba uri umukobwa. Iyo ubonye chromosome X kuri mama wawe na chromosome Y kuri so, uba uri umuhungu. Muri make, mama ahora aha umwana we chromosome X. Igitsina cyawe kigenwa niba se aguha chromosome X cyangwa Y.
• Iyo umugore afite inenge muri gene ikora iyi 'clotting factor' kuri imwe muri chromosomes ze ebyiri za X gusa, ashobora kuba afite hemophilia, ariko ashobora kutagira ibimenyetso kuko afite gene nzima kuri chromosomes ze za X.
• Iyo umubyeyi ufite chromosome ya X ifite inenge nk'iyi afite umwana w'umuhungu, hari amahirwe angana na 50% ko umwana azaragwa chromosome ya X ifite inenge. Iyo bibaye, umwana w'umuhungu azarwara hemophilia.
• Iyo uwo mubyeyi afite umukobwa, hari amahirwe angana na 50% ko umwana azagira chromosome X ifite inenge. Ariko, ashobora kutagaragaza ibimenyetso kuko avukana chromosome X nzima iva kuri se. Icyo gihe uwo mukobwa nawe azaba ari we uyitwara.

Ibyo bivuze ko umugore uvukana chromosome ya X ifite inenge aba ari we utwara hemophilia. Ashobora kuba adafite ibimenyetso, ariko ashobora kwanduza abana be. Buri mwana afite afite amahirwe ya 50% yo kurwara hemophilia.Abana b'abahungu bashobora kugaragaza ibimenyetso byinshi iyo barazwe hemophilia, kuko batabona chromosome X nzima iva kuri se.

Ese abakobwa nabo bagira ibimenyetso bya hemophilia?

Yego, birashoboka. Ariko ibimenyetso akenshi biba byoroheje. Urugero, umugore ufite gene ya hemophilia ashobora kuba adafite ubushobozi buhagije bwo kuvura amaraso. Ibi bibaye, ashobora kuva amaraso menshi mu gihe cy'imihango ye, gukomereka byoroshye, kuva amaraso menshi nyuma yo kubyara, cyangwa kuva amaraso mu ngingo ze, bigatera ibibazo mu ngingo.

Ni gute abaganga bapima hemophilia?

Muganga azabanza kukubaza amateka yuzuye y'ubuzima bwawe hanyuma agukorere isuzuma ry'umubiri. Niba ufite ibimenyetso bya hemophilia, azababaza n'amateka y'umuryango wawe. Bashobora gukora ibizamini nka:

• Umubare wuzuye w'amaraso (CBC): Ibi bikorwa kugira ngo harebwe ubwoko bw'uturemangingo mu maraso.
• Ikizamini cya Prothrombin Time (PT): Ibi bishobora kugenzura uburyo amaraso yawe yihuta cyane.
• Ikizamini cya Thromboplastin Time (PTT): Iki ni ikindi kizamini gipima igihe bifata kugira ngo amaraso agire ubwoya.
• Ikizamini cy’ibintu byihariye byo kuvura amaraso: Iki kizamini cy’amaraso gipima urwego rw’ibintu byihariye byo kuvura amaraso, nka factor VIII na factor IX .

Ni izihe ngero z'ibitera kuvura amaraso?

Ibintu bigabanya amaraso ni ibintu bifasha mu kugenzura kuva amaraso. Abaganga bashyira hemophilia mu byiciro byoroheje, biri hagati, cyangwa bikomeye bitewe n'ingano y'ibi bintu bigabanya amaraso mu maraso yawe:

• Abantu bafite ubushobozi bwo kuvura amaraso hagati ya 5% na 30% by'urugero rusanzwe bafite hemophilia yoroheje.
• Abantu bafite ubushobozi bwo kuvura amaraso hagati ya 1% na 5% by'urugero rusanzwe bafite hemophilia iri hagati.
• Abantu bafite munsi ya 1% by'ibintu bisanzwe byo kuvura amaraso bafite hemophilia ikomeye.

Ni ubuhe buryo bwo kuvura hemophilia?

Abaganga bavura hemophilia bongera urugero rw'ibintu bituma amaraso adakora neza cyangwa bagasimbuza ibintu bibura bituma amaraso adakora neza. Ibi byitwa uburyo bwo kuvura indwara zisimbura izindi .

Muri ubu buryo bwo kuvura indwara zisimbura izindi, uhabwa ibintu bigabanya amaraso bikozwe mu maraso y’umuntu (ibice bigabanya amaraso mu maraso) cyangwa ibintu bigabanya amaraso bivanze . Ubusanzwe, abantu bafite amaraso menshi ni bo bonyine bakeneye ubwo buryo bwo kuvura indwara zisimbura izindi buri gihe. Abantu bafite amaraso make kugeza ku rugero rwo hagati bahabwa ubwo buryo iyo bagiye kubagwa. Bahabwa imiti igabanya ubukana bw’amaraso.Imiti ibuza amaraso gushonga ishobora no gutangwa.

Ibi bintu bikomoka ku maraso bikozwe mu maraso y'abantu. Bisukurwa kandi bigapimwa kugira ngo bigabanye ibyago byo kwandura indwara zandura nka hepatite na virusi itera SIDA . Ibi bintu bisimbura ibi bitangwa mu buryo bwo guterwa mu mitsi (IV) .

Niba ufite ikibazo gikomeye cyo kuva amaraso mu maraso kandi ukava amaraso kenshi, muganga wawe ashobora kukwandikira uburyo bwo kuvura buzwi nka 'prophylactic factor infusions' kugira ngo wirinde kuva amaraso.

Ese hari ingorane ziri mu kuvura?

Bamwe mu bantu bahabwa ubu buryo bwo gusimbuza imiti bashyiraho antibodies , zitwa inhibitors , zirwanya ibintu bituma amaraso abagwa. Abaganga bakoresha uburyo bwitwa Immune Tolerance Induction (ITI) kugira ngo babuvure. ITI ikubiyemo kubaha inshinge za buri munsi z’ibintu bituma amaraso abagwa kugira ngo bagabanye urugero rw’ibintu bituma amaraso abagwa. Ubu buryo bushobora kuba ubuvuzi bw’igihe kirekire, kandi bamwe bashobora gukenera ubu buryo mu gihe cy’amezi cyangwa imyaka.

Ese hemophilia ishobora kwirindwa?

Oya, ntibishoboka. Niba ufite hemophilia kandi ufite abana, muganga wawe ashobora kukugira inama yo gupima imiterere y'uturemangingo . Muri ubwo buryo, wowe n'abana bawe mushobora kumenya niba bashobora kwanduza hemophilia mu gisekuru gitaha.

Ni ubuhe bwizere umuntu urwaye hemophilia agomba kugira?

Niba ufite hemophilia, ushobora gukenera kuvurwa ubuzima bwawe bwose. Uko ubuvuzi ukeneye bungana biterwa n'ubwoko bwa hemophilia ufite, ubukana bwayo, ndetse niba ufite 'ibibuza' cyangwa utabyifitemo.

Imyaka yo kubaho k'umuntu urwaye hemophilia ni iyihe?

Nk’uko bigaragazwa n’Ishyirahamwe Mpuzamahanga ry’Abarwaye Hemophilia, imyaka y’ubuzima bw’umugabo urwaye hemophilia ni mike ugereranyije n’imyaka 10 y’umuntu udafite hemophilia, nk’uko bigaragazwa n’amakuru yo mu 2012 n’Ishyirahamwe Mpuzamahanga ry’Abarwaye Hemophilia. Icyakora, bavuga ko abana barwaye hemophilia basuzumwe kandi bakavurwa bakiri bato bafite igihe cyo kubaho gisanzwe .

Ariko si bose bameze kimwe. Iby’ukuri ku muntu umwe bishobora kuba atari ukuri ku wundi. Niba wowe cyangwa umwana wawe afite hemophilia, baza muganga wawe icyo wakwitega. Azi neza uko umwana wawe/umwana wawe amerewe n’ubuzima bwe muri rusange.

Nitwara nte?

Abantu bafite hemophilia bashobora gukenera ubuvuzi buhoraho kugira ngo birinde kuva amaraso. Bashobora kandi kureka imirimo imwe n'imwe n'imiti. Ariko, hari byinshi ushobora gukora kugira ngo ucunge ingaruka hemophilia igira ku buzima bwawe.

Ibintu bidakwiye gukorwa

Ibintu byoroshye ku bandi gusimbuka, kugwa, cyangwa kugwa bishobora guteza ibibazo bikomeye ku bantu bafite hemophilia. Bagomba kwirinda ibikorwa bibashyira mu kaga gakomeye ko kugwa cyangwa gukubitwa bikomeye. Ingero:

• Gukina imikino nk'umupira w'amaguru, hockey, na rugby.
• Kwitabira ibintu nk'iteramakofe no gukina imikino yo gusiganwa ku maguru.
• Gutwara moto.
• Gukina umupira ku rubura.

Indi mikino, nka umupira w'amaguru, basketball na baseball, nayo ifite ibyago byinshi byo gukomereka. Niba ushaka gukina iyi mikino, ganira na muganga wawe ku bintu nko gukoresha ibikoresho byo kwirinda.

Imiti itari myiza gufata

Imiti igabanya ububabare nka aspirine, ibuprofen, na naproxen irinda kuvura amaraso. Nanone, gufata imiti igabanya amaraso nka heparin cyangwa warfarin si byiza.

Ibintu ushobora gukora kugira ngo ubeho neza

Hari ibintu byinshi ushobora gukora kugira ngo ugabanye ingaruka za hemophilia ku buzima bwawe:

• Injira mu myitozo ngororamubiri: Ushobora kuba ufite impungenge zo gukomereka mu gihe ukora imyitozo ngororamubiri. Ariko, ganira na muganga wawe ku buryo bwo gukomeza gukora siporo mu gihe ugabanya ibyago byo kuva amaraso.
• Gucunga stress : Hemophilia ni indwara ibaho ubuzima bwose, kandi ushobora gukenera gushyiraho imihati myinshi kugira ngo uyihuze n'inshingano z'umuryango wawe n'iz'akazi.
• Guma uhorana ubuzima bwiza bw'amenyo: Koza amenyo, kuyasukura, no kujya kwa muganga w'amenyo buri gihe bishobora kugabanya ibyago byo gukenera kuvurwa amenyo bishobora gutuma ava amaraso. Ntukibagirwe kubwira muganga wawe uko umerewe.
• Komeza kugira ibiro byiza: Niba ufite ingorane zo kugendagenda bitewe no kuva amaraso imbere no kwangirika kw'ingingo, kugenzura ibiro byawe bizagufasha.
• Menyesha abagukikije: Niba ufite hemophilia ikomeye, ushobora kugira ibihe bitunguranye byo kuva amaraso mu buryo butunguranye, nubwo waba ufata imiti. Menyesha umuryango wawe icyo wakora nibikubaho. Niba umwana wawe afite hemophilia, menyesha abamurera n'abakozi b'ishuri icyo wakora nibigera aho umwana wawe ava amaraso.

Ni ryari ngomba kujya kwa muganga?

Niba ubonye impinduka mu mubiri wawe, nko kuva amaraso menshi cyangwa gukomereka, gana muganga.

Igihe cyo kujya mu cyumba cy'ubutabazi bw'ibanze

Ugomba guhita ujya mu cyumba cy’ubutabazi bw’ibanze muri ibi bihe:

• Niba ufite umutwe ukomeye cyangwa urimo uzungera, ibi bimenyetso bishobora kugaragaza ko urimo kuva amaraso mu bwonko.
• Niba ufite ingingo zibyimbye cyangwa zibabaza, kandi udafite imiti yo kuzisimbura.

Ni ibihe bibazo ngomba kubaza muganga?

Niba wowe cyangwa umwana wawe musanzwe murwaye hemophilia, mushobora kubaza muganga wawe ibibazo nk'ibi:

• Ni ubuhe bwoko bwa hemophilia mfite/umwana wanjye?
• Ni ubuhe buryo bwo kuvura usaba?
• Ingaruka mbi z'ubuvuzi ni izihe?
• Ese njye/umwana wanjye nzakenera kuvurwa buri munsi?
• Ni ibihe bikorwa bitabereye njye/umwana wanjye gukora?
• Ni iyihe miti idashobora gufata njye/umwana wanjye?
• Nakora iki kugira ngo ngire ingaruka iyi ndwara igira ku buzima bwanjye/ubw'umwana wanjye?

Ubutumwa bwo kujyana mu rugo

Hemophilia ni indwara idakunze kugaragara, iterwa n'amaraso. Ishobora kugira ingaruka zikomeye ku buzima bwawe. Abantu bafite hemophilia bashobora gukenera ubuvuzi bw'ubuzima bwabo bwose. Ababyeyi b'abana bafite hemophilia bashobora guhangayikishwa no kurinda abana babo ingaruka mbi, ndetse no kubigisha kubana na hemophilia.

Muri iki gihe, abaganga ntibashobora gukiza hemophilia burundu. Ariko, bashobora gutanga imiti yo gukumira cyangwa kugabanya ibimenyetso bya hemophilia. Bashobora kandi gutanga inama ku ntambwe ushobora gufata kugira ngo ugabanye ingaruka za hemophilia ku buzima bwawe bwa buri munsi. Icy'ingenzi ni ugutuza no gukurikiza amabwiriza ya muganga wawe witonze.