Ese nawe ntugira agasebe gato gatuma amaraso ahagarara? Reka tuvuge kuri hemophilia!

Iyo ugize igikomere gito cyangwa ukubiswe ahantu runaka, kuva amaraso kurahagarara nyuma y'igihe runaka, si byo se? Ibyo biterwa n'uburyo busanzwe bwo kuvura amaraso mu mubiri wacu. Ariko tekereza kuri ibi, hari abantu badahagarika kuva amaraso byoroshye. Nubwo hari igikomere gito, bava amaraso igihe kirekire. Umubiri uhinduka ubururu n'ibisebe. Impamvu y'iki kibazo ishobora kuba indwara yitwa hemophilia. Nubwo abantu benshi batazi neza ibi, iki ni ikintu cy'ingenzi tugomba kumenya. Reka tubiganireho mu buryo bworoshye, mu buryo ushobora gusobanukirwa.

Hemophilia ni iki mu by'ukuri?

Muri make, hemophilia ni indwara idakunze kugaragara aho amaraso yacu adafunga neza. Amaraso yacu arimo poroteyine zihariye zifasha guhagarika kuva amaraso iyo atangiye. Ibyo tubyita "ibintu bifunga." Nk'uko sima ikenewe kugira ngo amatafari afatanye mu kubaka urukuta, ibyo bintu bifunga ni ingenzi kugira ngo amaraso ahagarike.

Umuntu urwaye hemophilia ntakora kimwe cyangwa byinshi muri ibi bintu bituma amaraso adakomera bihagije. Ibi bivuze ko no gukomereka gato bishobora gutera kuva amaraso igihe kirekire. Hari igihe kuva amaraso bishobora kubaho mu mubiri, cyane cyane mu ngingo no mu mitsi. Ibi bishobora gushyira ubuzima mu kaga.

Abaganga bagabanya ubukana bw'iyi ndwara mu byiciro bitatu, bitewe n'ingano y'ibintu bituma amaraso agwa.

• Hemophilia yoroheje: Kuva amaraso bikunze kubaho nyuma y'igikomere gikomeye cyangwa kubagwa.
• Hemophilia iringaniye: Ndetse n'ibikomere bito bishobora gutera kuva amaraso menshi. Umubiri ushobora no guhinduka ubururu.
• Hemophilia ikomeye: Kuva amaraso bishobora kubaho imbere no hanze, ndetse nta mpamvu igaragara.

Nubwo nta muti wuzuye uhari, hamwe n’uburyo bushya bwo kuvura buhari, birashoboka kugenzura kuva amaraso no kubaho ubuzima busanzwe.

Ese hari ubwoko bw'ingenzi bwa hemophilia?

Yego, hari ubwoko butatu bw'ingenzi bw'iyi ndwara. Bushingiye ku bwoko bw'ibintu bituma amaraso agwa mu mubiri bubura. Kugira ngo ubyumve neza, reba imbonerahamwe iri hepfo.

Ni ibihe bimenyetso by'iyi ndwara?

Ibimenyetso by'ingenzi bya hemophilia ni ukuva amaraso adasanzwe cyangwa kuva amaraso menshi no gukomereka. Uburemere bw'ibi bimenyetso buterwa n'uko ufite hemophilia yoroheje, iringaniye, cyangwa ikomeye.

Kuva amaraso menshi cyane

Tekereza, uhita uva amaraso mu mazuru nta mpamvu. Cyangwa se ukomeretse gato ku rutoki rwawe cyangwa nyuma yo kubagwa, kuva amaraso ntibihagarara igihe kirekire. Iyo umwana muto afite iyi ndwara, ashobora no gushyira igikinisho mu kanwa ke akava amaraso.

Gukomereka

Uruhu rwacu ruhinduka ubururu iyo amaraso atemba munsi yarwo. Abantu barwaye hemophilia bashobora guhinduka ubururu no gukomereka byoroshye, kabone n'iyo bakubitwa n'ikintu gito. Iyo umwana muto akubise umutwe ahantu runaka, ashobora kumera utubyimba tunini twitwa "goose eggs".

Ububabare bw'ingingo n'ibibyimba

Iyi ni yo ngorane ikomeye cyane ya hemophilia. Kuva amaraso bishobora kugaragara mu mubiri, cyane cyane mu ngingo nk'amavi, inkokora, n'utuguru. Hanyuma ingingo zirabyimba, zirababara, kandi zigashyuha iyo uzikozeho. Iyo ibi bibaye ku bana bato, bashobora kurira buri gihe, kwanga gupfukama, cyangwa kugenda.

Ni iki gitera hemophilia?

Hemophilia ikunze kuba indwara iterwa n'uturemangingo ihererekanywa kuva ku gisekuru kugeza ku kindi.Ibyo bivuze ko biterwa n'impinduka mu ngirabuzimafatizo dukomoka kuri ba mama cyangwa ba data. Reka tubisobanukirwe mu buryo bworoshye.

Imibiri yacu itegetswe gukora ibintu bituma amaraso agwa binyuze mu turemangingo. Iyo habayeho impinduka cyangwa inenge muri izi ngirabuzimafatizo, ayo mabwiriza ntakora neza. Ingaruka ni uko umubiri udakora ingano y'ibintu bituma amaraso agwa ukeneye.

Iyi nenge y’uturemangingo iboneka kuri chromosome ya X.

• Umukobwa afite chromosomes ebyiri za X (XX), imwe ikomoka kuri nyina n'indi ikomoka kuri se.
• Umuhungu afite chromosome imwe ya X na chromosome imwe ya Y (XY). X ikomoka kuri nyina naho Y ikomoka kuri se.

Noneho reba uko ibi bigenda bihita mu bisekuruza:

• Iyo umubyeyi afite chromosome X ifite iyi gene ifite inenge (tuyita 'umutwaro'), ubusanzwe ntagaragaza ibimenyetso kuko afite indi chromosome X nzima.
• Niba umuhungu w'uyu mubyeyi araga chromosome X ifite inenge, azarwara hemophilia kuko nta yindi chromosome X nzima afite yo kuyigenzura.
• Iyo umukobwa w'uyu mubyeyi azunguye chromosome X ifite inenge, nawe azaba ari umuntu uyirwaye. Ashobora kugaragaza ibimenyetso byoroheje, nko kuva amaraso menshi mu gihe cy'imihango.

Hari igihe hemofiliya ishobora kwigaragaza mu buryo butunguranye, idaturutse ku nkomoko, bitewe n'impinduka mu ngirabuzimafatizo mu gihe cy'ubuzima bw'umwana uri mu nda. Nanone, ni gake cyane, ishobora kubaho iyo ubudahangarwa bw'umubiri bw'umuntu bwibasiye ibintu bye bwite byo kuvura amaraso. Ibi tubyita 'hemofiliya isanzwe'.

Ni gute umuganga asuzuma ibi?

Niba wowe cyangwa umwana wawe mufite ibi bimenyetso, muganga wawe azabanza akubaze niba hari umuntu wo mu muryango wawe wigeze kugira ibi bibazo. Hanyuma, azagutegeka gupima amaraso kugira ngo byemezwe.

• Umubare wuzuye w'amaraso (CBC): Ipima ubwoko n'ingano y'uturemangingo mu maraso yawe.
• Ikizamini cya Prothrombin Time (PT): Kigenzura igihe bifata kugira ngo amaraso yawe yivunge.
• Ikizamini cya Thromboplastin Time (PTT): Iki ni ikindi kizamini gipima igihe amaraso avura.
• Ibipimo by'ibipimo byihariye byo kuvura amaraso: Ibi nibyo bigaragaza neza ibipimo byo kuvura amaraso udafite n'uko biri hasi.

Ni ubuhe buryo bwo kuvura ibi?

Uburyo bwo kuvura buzaterwa n'ubwoko bwa hemophilia ufite n'uburemere bw'indwara yawe. Uburyo bw'ingenzi bwo kuvura ni ugusimbuza.

Muri make, ibi bikubiyemo guha umubiri wawe inshinge y’ikintu kivura amaraso kiri hasi mu maraso yawe. Ibi bintu bivura amaraso bishobora gukorwa mu maraso y’umuntu cyangwa bigakorerwa muri laboratwari. Ubu buryo bwo kuvura bushobora gufasha gukumira kuva amaraso no kuyahagarika vuba niba bibaye.

Byongeye kandi, ubu hari uburyo bushya butandukanye bwo kuvura:

• Imiti nka Marstacimab-hncq (Hympavzi®) na Concizumab (Alhemo®) : Iyi miti ibuza poroteyine ibuza amaraso kuvura kandi ikangurira enzyme ifasha amaraso kuvura.
• Fitusiran (Qfitlia®): Uyu muti ubuza umwijima wacu gukora ikintu (antithrombin) kibuza amaraso kuzura.
• Ubuvuzi bw'uturemangingo: Ubu ni bwo buryo bugezweho bwo kuvura. Muri ubu buryo, gene nzima ishyirwa mu mubiri kugira ngo isimbuze gene ifite inenge, bigatuma umubiri ukora ibintu bikenewe byo kuvura.

Ni ryari ugomba gushaka inama z'abaganga byihutirwa?

Niba ubonye impinduka mu mubiri wawe, nko kuva amaraso menshi cyangwa gukomereka, hita ubibwira muganga wawe. Nanone, niba ubonye kimwe muri ibi bimenyetso bikomeye bikurikira, jya kwa muganga wita ku barwayi b’inkeragutabara (ETU) vuba.

Ibintu ugomba kumenya mu gihe ubana n'indwara ya hemophilia

Hemophilia ni indwara ibaho ubuzima bwose, cyangwa indwara idakira. Bityo uzakenera ubuvuzi no gukurikiranwa mu buzima bwawe bwose. Ariko ntugahangayike. Hamwe n'ubuvuzi bushya, umuntu urwaye hemophilia ashobora kubaho ubuzima bwegereye ubwo umuntu udafite iyo ndwara.

Dore bimwe mu bintu bishobora kugufasha kumva umerewe neza mu gihe ubana n'iyi ndwara:

• Genzura ibiro byawe: Uko ugenda wiyongera ibiro, umuvuduko ku ngingo zawe urushaho kwiyongera. Kugumana ibiro byiza ni ingenzi mu kurinda ingingo zamaze kwangirika bitewe no kuva amaraso mu ngingo.
• Wite ku menyo yawe neza: Ni ngombwa koza amenyo yawe neza kandi ukita ku buzima bw'amenyo yawe. Kubera ko uburyo nko gukuramo amenyo n'imiyoboro y'amazi bishobora gutera kuva amaraso, ni byiza kubyirinda.
• Tekereza ku buzima bwo mu mutwe: Kubana n'indwara idakira bishobora gutera guhangayika no kwiheba. Vugana na muganga wawe kuri ibi kandi usabe inama ku buzima bwo mu mutwe nibiba ngombwa.
• Gira imyitozo ngororamubiri mu mutekano: Gukora ibikorwa bigabanya ibyago byo gukomereka ni byiza ku mubiri wawe no mu bwonko bwawe. Baza muganga wawe imyitozo ikubereye.

Iyo ufite hemophilia, ndetse n'igikomere gito abandi bashobora kutabona gishobora kukubera ikibazo gikomeye. Gukomereka mu ivi ku kaguru ko ku meza bishobora gutuma amaraso yinjira mu ngingo. Kuva amaraso mu mazuru ntibishobora guhagarara nubwo wakoroye cyane. Kubana n'iki kibazo bishobora kugorana. Ariko ibuka ko utari wenyine. Itsinda ry'abaganga rihora rihari kugira ngo rigufashe.