Ese n'agasebe gato kawe ntigahagarika kuva amaraso? Ese twavuga ku ndwara ya hemophilia?

Uribuka ukiri umwana, igihe wagwaga ukavunika ivi cyangwa inkokora, kuva amaraso byarahagararaga nyuma y'igihe runaka? Ntabwo ari ibintu bitangaje? Iyo tuvuye amaraso, imibiri yacu ifite uburyo bwo kuyahagarika, ari na bwo buryo butuma amaraso agwa. Ariko hari abantu bamwe na bamwe, kandi ubu buryo bwo kuvura amaraso ntibukora neza mu mibiri yabo. Niho tuvuga ku ndwara yitwa hemophilia . Ibi bishobora kuba bikomeye gato, ariko iyo bivuwe neza , ni indwara ishobora kuvurwa neza.

Hemophilia ni iki mu by'ukuri?

Muri make, hemophilia ni indwara aho amaraso yacu adafata neza, bivuze ko iyo tuvuye amaraso, adahagarara byoroshye . Ni indwara iterwa ahanini n'uturemangingo , bivuze ko ishobora kwanduzwa binyuze mu turemangingo kuva ku gisekuru kugeza ku kindi.

Tekereza ufite agasebe gato ku kuboko kwawe. Ubusanzwe, poroteyine zimwe na zimwe zo mu maraso yacu, zitwa ibintu bituma amaraso agwa , zikorana kugira ngo zifunge igisebe kandi zihagarike kuva amaraso. Ni nko gupfuka umwobo ku rukuta.

Ariko ku muntu urwaye hemophilia, kimwe muri ibi bintu bituma amaraso adakora neza kiba kibuze mu rugero ruhagije, cyangwa iyo mpamvu ntikora neza. Icyo ni cyo kibazo. Niyo mpamvu bashobora kuva amaraso igihe kirekire ndetse no mu gikomere gito, cyangwa bakaba bashobora gukomereka byoroshye.

Uburyo amaraso afungana mu buryo bworoshye

Iyo dutangiye kuva amaraso, hari intambwe nyinshi zo kubihagarika.

1. Ikintu cya mbere kibaho ni uko imiyoboro y'amaraso ifungana. Hanyuma ingano y'amaraso asohoka igabanuka gato.

2. Hanyuma, uturemangingo duto twitwa platelets mu maraso yacu tuza aho twakomeretse tugafatana, tugakora ubwoko bw'umuyoboro muto.

3. Hanyuma, ibintu byo kuvura amaraso navuze mbere birakora, bigakora kimwe kimwe, bigakora urusobe rukomeye rwitwa fibrin , bigakomeza umugozi wa platelets no guhagarika burundu kuva amaraso.

Ikibaho muri hemophilia ni uko muri iyi ntambwe ya gatatu, kimwe mu bintu bituma amaraso agwa nta ruhu kibura, bityo bigatuma amaraso agwa cyane adakora.

Ese hari ubwoko bwa hemophilia?

Yego, hari ubwoko bubiri bw'ingenzi bwa hemophilia, bitewe n'ikintu kidakora neza.

Hemophilia A

Ubu ni bwo bwoko bwa hemophilia bukunze kugaragara cyane. Buterwa no kutagira factor VIII ihagije yo kuvura amaraso cyangwa kudakora neza.

Hemophilia B

Ubwo bwoko bwitwa kandi indwara ya Noheli . Iterwa no kubura kwa factor ya cyenda yo kuvura amaraso (Factor IX).Nta kibazo niba nta gihagije gihari, nta kibazo niba kidakora neza.

Ibimenyetso by'ubwo bwoko bwombi birasa cyane. Ariko, ni ngombwa cyane kumenya neza ubwo bwoko bwombi mu gihe bwo kubuvura. Kuko ubuvuzi bugomba gutangwa hakurikijwe ikintu cyagabanutse.

Uburemere bw'iki kibazo

Uburemere bwa hemophilia bushobora gutandukana bitewe n'umuntu ku wundi, bitewe n'ingano y'ibintu bigabanya amaraso mu mubiri.

• Hemophilia yoroheje: Abantu bafite hemophilia yoroheje baba bafite ibintu byo kuvura amaraso biri hasi gato ugereranyije n’ibisanzwe. Bashobora kuva amaraso igihe gito gusa iyo bakomeretse cyane, babazwe, cyangwa bakuwe iryinyo. Bashobora no kutamenya ko bafite hemophilia kugeza igihe habaye ikintu gikomeye.
• Hemophilia iringaniye: Aba bantu bafite ibintu bike byo kuvura amaraso. Bashobora kuva amaraso menshi cyane bitewe n'ibikomere bito. Hari igihe bashobora kuva amaraso nta mpamvu (kuva amaraso mu buryo bwihuse).
• Hemophilia ikomeye: Aba bantu bafite urwego ruto cyane rw'ibitera amaraso kuvura. Bashobora kuva amaraso mu ngingo no mu mitsi nta mpamvu igaragara. Ndetse n'igisebe gito cyane gishobora gutera ikibazo gikomeye.

Kuki ibi bibaho? Impamvu ni izihe?

Hemophilia akenshi ni indwara iterwa n'uturemangingo , bivuze ko yandurira abana iva ku babyeyi binyuze mu turemangingo.

Uburyo bikomoka ku iragi (iragi rifitanye isano na X)

Ingirabuzimafatizo za hemophilia ziri kuri chromosome ya X. Urabizi, abagore bafite chromosome ebyiri za X (XX), mu gihe abagabo bafite chromosome imwe ya X na chromosome imwe ya Y (XY).

• Umwana w'umukobwa: Ahabwa X imwe na nyina n'indi X imwe na se.
• Umwana w'umuhungu: Ahabwa X imwe na nyina n'indi Y imwe na se.

Noneho, niba gene ifite inenge itera hemophilia iri kuri chromosome X,

• Iyo umuhungu azunguye iyo chromosome ya X ifite inenge kuri nyina, azarwara hemophilia. Kuko afite chromosome ya X imwe gusa. Iyo hari inenge kuri yo, nta yindi X ihari yo kuyisubiza.
• Iyo umukobwa ahawe chromosome X ifite inenge kuri nyina cyangwa se, ariko akaba afite chromosome X nzima, nta bimenyetso azagaragaza. Ariko azaba ari we uyifite . Ibi bivuze ko nubwo adafite hemophilia, ashobora kwanduza abana be iyo gene ifite inenge. Ni gake cyane, abagore bayifite nabo bashobora kugaragaza ibimenyetso byoroheje.

Niyo mpamvu hemophilia ikunze kugaragara ku bagabo .

Tekereza, niba umubyeyi afite ikibazo cyo kwandura hemophilia, buri mwana w’umuhungu abyara afite amahirwe angana na 50% yo kwandura hemophilia. Mu buryo nk’ubwo, buri mwana w’umukobwa afite amahirwe angana na 50% yo kwandura hemophilia.

Ese ikorwa mu miryango? (Guhinduka kw'umubiri mu buryo bwikora)

Si ngombwa buri gihe ko umuntu wo mu muryango agira hemophilia. Hari igihe, hafi 30% by'abarwaye hemophilia ishobora kubaho nk'impinduka mu ngirabuzimafatizo. Muri icyo gihe, nta wundi muntu wo mu muryango ushobora kuba yarahuye n'iyi ndwara mbere.

Ni ibihe bimenyetso bya hemophilia? Ipimwa gute?

Ibimenyetso bya hemophilia biratandukanye bitewe n'uburemere bw'indwara. Ibimenyetso bisanzwe birimo:

• Gukunda kugaragara ibikomere binini kandi byimbitse: Ndetse n'igisebe gito gishobora gutera ibikomere binini.
• Kuva amaraso bidashira bitewe n'igikomere, gukurwa iryinyo, cyangwa nyuma yo kubagwa.
• Kuva amaraso mu mazuru nta mpamvu.
• Ishinya zirimo kuva amaraso.
• Kuva amaraso mu ngingo (hemarthrosis): Ibi birababaza cyane. Ingingo zirabyimba, zishyuha, kandi ntizishobore kugenda neza. Ingingo nk'amavi, inkokora, n'utuguru ni byo bikunze kwibasirwa cyane. Mu gihe kirekire, ibi bishobora no kwangiza ingingo.
• Kuva amaraso mu mitsi: Ibi binatera ububabare no kubyimba.
• Kuva amaraso mu nkari cyangwa mu mwanda.
• Ku bana bato: Hari igihe ikimenyetso cya mbere kiba ari ukuva amaraso igihe kirekire nyuma yo gukebwa. Cyangwa nyuma yo guterwa urushinge, ahantu hashobora kubyimba cyane no kuva amaraso.
• Indwara ikomeye cyane, ariko idakunze kugaragara, ni ukuva amaraso mu bwonko. Ibi bishobora gutera ibimenyetso nko kuribwa umutwe cyane, kuruka, gusinzira no gucika intege. Ibi ni ibintu byihutirwa.

Ni gute umuganga ashobora kumenya ko iyi ari hemophilia? (Isuzuma)

Niba wowe cyangwa umwana wawe mufite kimwe muri ibi bimenyetso, ikintu cyiza cyo gukora ni ukujya kwa muganga. Ubusanzwe muganga azasuzuma indwara muri ubu buryo bukurikira:

1. Kubaza amateka y'umuryango: Kureba niba hari umuntu wo mu muryango ufite ikibazo cyo kuva amaraso mu maraso cyangwa ibindi bibazo byo kuva amaraso.

2. Hakorwa isuzuma ry'umubiri: Kureba niba hari ibikomere, ingingo zabyimbye, n'ibindi.

3. Gupima amaraso bikorwa:

• Ibizamini byo gusuzuma: Ibi bipima igihe bifata kugira ngo amaraso yivunde. Urugero, ibizamini nka PTT (Igihe cya Thromboplastin) na PT (Igihe cya Prothrombin) . Agaciro ka PTT gashobora gutinda mu maraso ya hemophilia.
• Isuzuma ry’ibintu bigabanya amaraso: Ibi bikoreshwa mu kumenya niba Factor VIII na Factor IX bifite ikibazo, kandi ku rugero rungana iki. Ibi bikoreshwa mu kumenya ubwoko n’uburemere bwa hemophilia.

Ndetse no mu gihe cyo gutwita, iyo hari amateka y'umuryango wagize ikibazo cyo kubura amaraso mu nda, ibizamini bishobora gukorwa kugira ngo harebwe niba umwana afite ikibazo cyo kubura amaraso mu nda.

Ni ubuhe buryo bwo kuvura ibi? (Ubuvuzi)

Nubwo hemophilia ari indwara idakira, hari uburyo bwiza bwo kuvura.Ubu buryo bwo kuvura bushobora gufasha mu kugenzura no gukumira kuva amaraso, kandi bukagufasha kubaho neza.

Uburyo bwo kuvura busimbura

Ubu ni bwo buryo bw'ingenzi bwo kuvura. Bukubiyemo guha umubiri ikintu kigabanya amaraso (Factor VIII cyangwa Factor IX) kidahagije mu mubiri binyuze mu mitsi (iterwa mu mitsi cyangwa IV). Ibi bintu bikorwa mu maraso y'umuntu cyangwa binyuze mu ikoranabuhanga rya genetiki (ibicuruzwa bihuza).

Ubu buvuzi bukorwa mu buryo bubiri:

1. Ubuvuzi bwo kwirinda indwara: Muri ibi, icyo kintu gihabwa umubiri inshuro nyinshi mu cyumweru, mbere yuko amaraso atangira kuva. Ibi bishobora gukumira kuva amaraso mu buryo butunguranye, cyane cyane mu ngingo. Ibi bikunze gukorwa ku bantu bafite indwara yo kuva amaraso mu buryo bukabije.

2. Ubuvuzi butangwa igihe cyose umuntu asabye: Muri ibi, icyo gikorwa gitangwa gusa nyuma yuko amaraso atangiye kuva. Ubu buvuzi bukoreshwa ku bantu bafite hemophilia yoroheje n'abatari ku buvuzi bwo kwirinda.

Indi miti

• Desmopressin (DDAVP): Uyu ni umuti ushobora gukoreshwa ku bantu bafite hemophilia yoroheje A. Ukora mu kurekura Factor VIII yabitswe mu mubiri. Ushobora gutangwa nk'umuti wo mu mazuru cyangwa nk'urushinge.
• Imiti igabanya ubukana bw'amaraso: Urugero , aside tranexamic . Iyi miti ikora mu kugabanya gushonga kw'amaraso. Igira akamaro mu bihe nko gukurwa amenyo no kuva amaraso mu mazuru.
• Imiti igabanya ububabare: Ushobora gufata ibintu nka parasetamol kugira ngo ugabanye ububabare buterwa no kuva amaraso mu ngingo. Ariko, imiti nka aspirine na NSAIDs (urugero: ibuprofen, diclofenac) ntiyemerwa ku bantu bafite hemophilia, kuko ishobora kongera ibyago byo kuva amaraso.

Ukora iki iyo uvuye amaraso?

Hari ibintu bike ugomba gukora iyo uvuye amaraso. Wibuke uburyo bwa RICE .

• R (Kuruhuka): Humura ingingo cyangwa imitsi yakomeretse. Irinde ko igenda.
• I (Urubura): Shyira agapaki k'urubura ahantu hakomeretse mu gihe cy'iminota 15-20, inshuro nyinshi ku munsi. Ibi bizagabanya kubyimba no kubabara.
• C (Gukanda): Zingira agace kakomeretse n'igitambaro cya elastic kugira ngo gafashe gato.
• E (Uburebure): Gumisha ukuboko cyangwa ukuguru kwakomeretse hejuru y'uburebure bw'umutima.

Mu gihe ukora ibi bintu, ugomba kuvugana na muganga wawe hanyuma ukabona Factor treatment nibiba ngombwa.

Uburyo bwo kubaho n'indwara ya hemophilia

Kubana n'indwara ya hemophilia bishobora kugorana, ariko iyo uyobowe neza , ubizi neza, kandi ugaterwa inkunga, ushobora kubaho ubuzima busanzwe kandi bukora.

• Ubufasha butangwa n'itsinda ry'abaganga b'inzobere: Mu gihe cyo kuvura hemophilia,Ni ingenzi cyane kubona ubufasha bw'itsinda rigizwe n'inzobere mu by'amaraso, abaforomo, abaganga b'imitsi n'abakozi b'imibereho myiza. Bazakugira inama ku kuvura, imyitozo ngororamubiri, n'uburyo bwo kwirinda indwara.
• Gukora ibikorwa bitekanye: Abantu barwaye hemophilia bagomba kwirinda siporo zishobora gutera imvune nyinshi (urugero: rugby, boxing). Ahubwo, kora imyitozo ngororamubiri itekanye nko koga, kugenda n'amaguru, no kugendera ku igare (ufite ingofero). Vugana n'umuganga w'imitsi kugira ngo aguhitiremo imyitozo ikubereye.
• Gukomeza kugira ubuzima bwiza bw'amenyo: Ni ingenzi cyane kwirinda kwangirika kw'amenyo no kurwara ishinya, kuko hari ibyago byo kuva amaraso mu gihe ukora ibikorwa nko gukuramo amenyo. Komeza urebe muganga w'amenyo.
• Ibimenyetso by'ubwishingizi bw'indwara: Buri gihe gendana agapapuro k'imiringa cyangwa ikarita ivuga ko urwaye hemophilia. Bishobora kuba ingirakamaro cyane mu gihe cy'impanuka.
• Ubufasha mu mibereho myiza: Kuganira n'abandi bantu bafite hemophilia n'imiryango yabo no gusangira ubunararibonye ni imbaraga zikomeye. Niba hari imiryango nk'iyo, yifatanye nayo.
• Icyizere cy'ejo hazaza: Uburyo bushya bwo kuvura hemophilia buri kuvumburwa buri gihe. Ubushakashatsi burimo gukorwa ku bintu nk'ubuvuzi bw'uturemangingo . Bityo dushobora kugira icyizere cy'ejo hazaza.

Ubutumwa bwo kujyana mu rugo

Hemophilia ni ikibazo cyo kuvura amaraso, ariko si ikintu cyo gutinya kandi gishobora gukemurwa neza .

Igikuru ni ukujya kwa muganga vuba niba ufite ibimenyetso, ugahabwa isuzuma ry’ukuri, kandi ugakurikiza ubuvuzi butangwa.

Niba wowe cyangwa undi muntu uzi afite hemophilia, ibuka ko utari wenyine. Ubifashijwemo n'ubumenyi, inkunga n'ubuvuzi ukeneye, nawe ushobora kubaho ubuzima bwiza kandi bukora. Ntugacike intege!

Hemofiliya , kuva amaraso, kuvura amaraso, indwara z'uturemangingo, Factor VIII, Factor IX