Ese uzi indwara ya Hemophilia B? Reka tubisobanure!

Ese wigeze ubona ko hari abantu bakomeza kuva amaraso nubwo baba bakomeretse gato? Cyangwa se wabonye ko ibice by'umubiri wawe bihinduka ubururu kandi bifite imirongo y'amaraso? Impamvu imwe y'ibi bintu ishobora kuba indwara tugiye kuvugaho uyu munsi yitwa Hemophilia B. Ntugahangayike, tuzavuga kuri ibi mu buryo bworoshye ushobora gusobanukirwa.

Hemophilia B ni iki?

Mu magambo make, hemophilia B ni indwara yo kuva amaraso ivuka mu muryango. Ibaho iyo habayeho impinduka mu ngirabuzimafatizo ifasha amaraso yacu kuvura amaraso. Abantu bafite hemophilia B bafite urugero ruto rwa factor IX (izwi kandi nka factor 9, F9, FIX), poroteyine y'ingenzi ifasha kuvura amaraso kuvura. Iyo itavuwe, iyi ndwara ishobora gushyira ubuzima mu kaga. Inkuru nziza ni uko abaganga barimo kuyivura bakoresheje uburyo bwa factor 9. Uburyo bushya bwo kuvura, nko kuvura ngirabuzimafatizo, burimo gukorwaho ubushakashatsi.

Iyi ndwara igira ingaruka zite ku mubiri wawe?

Kimwe n'ubundi bwoko bwa hemophilia, abantu bafite hemophilia B bava amaraso menshi kandi bakamara igihe kirekire kuruta uko bisanzwe iyo bakomeretse, babazwe, cyangwa bakuwe iryinyo. Iyi ndwara ikunze kwibasira abagabo, ariko ishobora no kwibasira abagore.

Abaganga bashyira hemophilia mu byiciro byoroheje, biri hagati, kandi bikomeye . Abantu bafite hemophilia ikomeye B bashobora kuva amaraso mu ngingo zabo. Ibi birababaza cyane, kandi uko igihe kigenda gihita, bishobora gutera indwara nka rubagimpande. Hari abantu bakeneye kubagwa kugira ngo bakureho ingingo zangiritse kandi zisimbuzwe. Bashobora no kuva amaraso mu bwonko bwabo, ibyo bikaba byashyira ubuzima bwabo mu kaga.

Hemophilia B ikunze kugaragara gute?

Dukurikije imibare yo muri Leta Zunze Ubumwe za Amerika, abagabo bagera kuri 4 ku 100.000 bafite hemophilia B. Abagore nabo barayirwara, ariko biragoye kuvuga neza umubare wayo. Kuko nubwo bafite ibimenyetso nk'imihango myinshi, abantu benshi ntibatekereza ko ifitanye isano na hemophilia.

Ese uzi itandukaniro riri hagati ya hemophilia A na B?

Hemofiliya A na B byombi ni indwara zo kuva amaraso mu buryo bw’umubiri. Nubwo ibimenyetso bisa cyane, impinduka mu miterere y’uturemangingo zibitera ziratandukanye. Ubushakashatsi bwagaragaje ko ibimenyetso bya hemofiliya B bishobora kuba bike cyane ugereranyije n’ibya hemofiliya A. Ibi bivuze ko hemofiliya B nayo ari indwara ikomeye, ariko abantu bayirwaye bashobora kugira ibibazo bike byo kuva amaraso menshi. Dore izindi tandukaniro:

• Ubushakashatsi bwerekana ko abantu barwaye hemophilia B bava amaraso make mu ngingo (hemathroses) kandi batakaza amagufwa menshi.
• Kuva amaraso mu buryo butunguranye ni gake cyane ku bantu bafite hemophilia B.
• Hari igihe, mu gihe cyo kuvura hemophilia, umubiri utera ubudahangarwa bw’umubiri bubangamira ubuvuzi. Ariko, abantu bafite hemophilia B ntibakunze kugira ibi bibazo.

Kuki ibi byari byitwaga mbere 'indwara ya Noheli'?

Iyi ni inkuru ishimishije cyane. Mu myaka irenga 100, abaganga batekerezaga ko hariho ubwoko bumwe gusa bwa hemophilia. Ariko mu 1952, umushakashatsi yakoresheje amaraso y’abantu barindwi bafite hemophilia (umwe witwa 'Noheli') kugira ngo avure abandi bafite hemophilia. Igitangaje ni uko ubuvuzi bwagize akamaro! Ni bwo abashakashatsi babonye ko Bwana Christmas yari afite ubwoko butandukanye bwa hemophilia, ni ukuvuga ko yaterwaga n’impinduka mu ngirabuzimafatizo zitandukanye. Bityo ubwo bwoko bwa kabiri bwa hemophilia bise 'Noheli factor' cyangwa 'indwara ya Noheli'. Nyuma yaje kwitwa hemophilia B.

Ni iki gitera hemophilia B?

Nkuko byavuzwe mbere, hemophilia B ni indwara yo kuva amaraso mu buryo bw’uturemangingo. Niba ufite hemophilia B, bivuze ko warazwe gene idasanzwe igira ingaruka ku ngano ya factor 9. Ubusanzwe, hari gene yitwa F9, ikubwira uburyo bwo gukora factor 9. Hemophilia B ibaho iyo iyi gene ya F9 ihindutse, ikarema gene idasanzwe igabanya urugero rwa factor 9, cyangwa ikavanaho burundu factor 9. Akenshi, abahungu baragwa hemophilia B iyo ba nyina ari bo banduye iyi ndwara.

Dore uko bigenda:

• Twese tubona chromosomes X ebyiri kuri mama, chromosome X imwe na chromosome Y imwe kuri data.
• Iyo umugore afite gene idasanzwe ya F9 kuri imwe muri chromosomes X ze, azaba afite hemophilia B, ariko ntazagaragaza ibimenyetso. Ibi biterwa nuko afite gene isanzwe ya F9 kuri chromosomes X ze.
• Uyu mugore ashobora guha umuhungu we chromosome X ifite gene idasanzwe ya F9. Kubera ko abahungu bafite chromosome X imwe gusa, bazarwara hemophilia B.

Hari igihe ihinduka ry’umubiri ritunguranye ritavanwa kuri nyina ku mwana we.Hemophilia B ishobora kandi guterwa n'inzira yitwa involution. Ibi bibaho iyo igi n'intanga bihurira hamwe bigakora urusoro, hanyuma bigatangira kwigabanyamo ibice bishya. Utu turemangingo dushya tuba dufite kopi z'uturemangingo twari mu karemangingo ka mbere. Rero, rimwe na rimwe amakosa, cyangwa impinduka, bishobora kubaho iyo utu turemangingo twiganwe. Iyo habayeho ikosa iyo gene ya F9 yiganwe, umwana wawe ashobora kurwara hemophilia B cyangwa akaba umutwaro wa hemophilia B. (Abahanga mu by'amateka n'abashakashatsi mu by'ubuvuzi bemeza ko ibi byabaye ku Mwamikazi Victoria w'Ubwongereza. Nubwo nta muntu n'umwe mu muryango we wari ufite hemophilia, yari afite hemophilia. Yanduje iyi ndwara ku bana be. Ubwo abo bana bashyingirwaga mu miryango y'abami bo muri Esipanye no mu Burusiya, abana babo nabo barwaye iyi ndwara. Nyuma yaje kumenyekana ko ari hemophilia B.)

Ni ibihe bimenyetso bya hemophilia B?

Twamaze kuvuga ko ibimenyetso bya hemophilia B biri mu byiciro byoroheje, biri hagati, n'ibikomeye. Abaganga babishyira mu byiciro bakurikije urugero rwa factor 9 mu maraso.

Hemophilia yoroshye B

Abantu bafite ingero za factor 9 hagati ya 6% na 49% bafite hemophilia yoroshye B. Ibimenyetso ni byoroshye. Hari igihe abantu bafite hemophilia yoroshye B basuzumwa bamaze gukura, iyo bitegura kubagwa, iyo bagiye kubyara, nyuma yo gukomereka bikomeye, cyangwa, ku bagore, iyo barimo kuvurwa kuva amaraso menshi mu mihango.

Hemophilia B iringaniye

Abantu bafite ingero za factor 9 hagati ya 1% na 5% bafite hemophilia B yoroheje. Ibimenyetso nabyo ni byoroheje. Abana bafite iyi ndwara akenshi batangira kugaragaza ibimenyetso bafite amezi 18.

Aba bana bashobora kugira ibimenyetso nk'ibi:

• Ibikomere: Bikomeretsa kandi bigakomeretsa byoroshye cyane.
• Kuva amaraso kudasanzwe: Niba ubaye, urimo kuva amaraso, cyangwa urimo gukura iryinyo, ushobora kuva amaraso menshi kandi mu gihe kirekire kuruta uko bisanzwe.
• Kuva amaraso mu buryo butunguranye: Ni gake cyane, kuva amaraso bishobora gutangira nta mpamvu igaragara.

Hemophilia ikomeye B

Abantu bafite ingero za factor 9 ziri munsi ya 1% bafite hemophilia B ikomeye. Ibimenyetso birakomeye. Bamwe mu bana bashobora gutangira kuva amaraso igihe cyo kuvuka, nyuma gato yo kuvuka, cyangwa nyuma yo gukebwa. Abandi bana bashobora kutagaragaza ibimenyetso kugeza amezi make nyuma yo kuvuka. Ibimenyetso bisanzwe birimo:

• Kuva amaraso: Impinja nto n'abana bato bashobora kuva amaraso mu kanwa kabo iyo ikintu gito, nk'igikinisho, kibageze mu kanwa.
• Ibibyimba ku mitwe yabo: Iyo umwana muto akubise umutwe ahantu runaka, ashobora kumera ibibyimba bisa n'amagi y'inkoko.
• Kurira kenshi, kubura amahoro, cyangwa kwanga gukurura cyangwa kugenda:Ibi bishobora kubaho iyo umwana afite amaraso mu mitsi cyangwa ingingo imbere mu mubiri we. Ashobora kugaragara nk'umukara kandi akabyimba ahantu runaka. Ashobora kumva ashyushye iyo umukozeho kandi umwana akaba afite ububabare.
• Hematoma: Hematoma ni amaraso apfundika munsi y'uruhu. Umwana ashobora kandi kugira hematoma nyuma yo guterwa urushinge.
• Ingorane zo guhumeka: Hari igihe ururimi rw'umwana rushobora kubyimba bitewe no kuva amaraso no kuziba inzira z'ubuhumekero.

Abaganga babimenya bate?

Abaganga bapima hemophilia B bakora isuzuma ry’umubiri, bakareba ibimenyetso nko gukomereka no kubyimba ingingo, kandi bakabaza niba hari umuntu wo mu muryango ufite hemophilia cyangwa izindi ndwara z’amaraso.

Byongeye kandi, ibizamini bikurikira nabyo birakorwa:

• Umubare wuzuye w'amaraso (CBC): Ibi bigenzura ubwoko bw'uturemangingo mu maraso n'umubare watwo.
• Ikizamini cya Prothrombin Time (PT): Iki kizasuzuma uburyo amaraso yawe yihuta cyane.
• Ikizamini cya Activated Partial Thromboplastin Time: Iki kandi ni ikizamini gipima igihe bifata kugira ngo amaraso agire icyo apfa.
• Ikizamini cya Fibrinogen: Ikizamini gipima ingano ya poroteyine yitwa fibrinogen, ifasha amaraso kubumba.
• Ikizamini cy'ibintu bigabanya amaraso: Iki nicyo gifasha kumenya ubwoko nyabwo bwa hemophilia n'uburemere bw'indwara.

Ni ubuhe buryo bwo kuvura ibi?

Abaganga bakunze kuvura hemophilia B bakoresheje uburyo bwo gusimbuza factor . Ibi bikubiyemo gutera inshinge za factor 9 mu mitsi. Iyi factor 9 ikora kugira ngo isimbuze factor ibura, ifasha mu gukumira kuva amaraso menshi cyangwa kugenzura kuva amaraso iyo bibaye.

Ubusanzwe, abantu bafite hemophilia yoroshye kugeza ku iringaniye B bakeneye ubu buvuzi gusa iyo bagomba kubagwa. Ariko, abantu bafite hemophilia yoroshye B bashobora gukenera kuvurwa buri gihe hakoreshejwe factor replacement therapy.

Ese hari ingorane zishobora kubaho mu gihe cyo kuvurwa?

Hari ingorane zimwe na zimwe zishobora kubaho ku bantu bafata ubu buvuzi. Ingorane nyamukuru ni ugutera imiti igabanya ubukana n'ubwandu bwa virusi .

Ibikoresho bibuza

Inhibitors zibaho iyo umubiri uhagaritse kwakira factor replacement nk'igice cy'amaraso asanzwe. Izi antipressants zibuza factor replacement therapy gukora neza. Ibi bituma bigorana cyane guhagarika cyangwa kugabanya kuva amaraso. Abantu bafite indwara zikomeye zo kuva amaraso kandi bahabwa factor therapy nyinshi bashobora kugira iyi ngorane. Abaganga bavura ibi bakoresheje ibindi bintu bishobora kurangiza amaraso mu gihe adaciye ku gice cya 9.

Indwara za virusi

Ni gake cyane, kwandura virusi nka hepatite C bishobora kubaho iyo hakoreshejwe imiti isimbura factor, ikozwe mu maraso yatanzwe. Ariko, ukurikije uburyo bwo gupima buriho ubu, iyi ngaruka ni nto cyane.

Ese ibyago byo kuba ibi bishobora kugabanuka?

Kubera ko hemophilia B ari indwara umuntu avukana, ntushobora kuyibuza kuyikuramo cyangwa kuyitera abana bawe. Reka twige byinshi ku bijyanye n'imikorere y'uturemangingo ituma umuntu avukana:

• Niba uri umugore kandi ufite gene idasanzwe ya F9 kuri imwe muri chromosomes X yawe (bivuze ko uri umutwaro wayo), abana bawe b'abahungu bafite amahirwe 50% yo kurwara hemophilia B, naho abana bawe b'abakobwa bafite amahirwe 50% yo kuba abatwaza iyo gene.
• Abakobwa bawe, bakura iyi gene, bashobora kwanduza abahungu babo hemophilia B. Abakobwa babo bashobora kuba abanduza.
• Abahungu bawe barwaye hemophilia B bashobora guha abakobwa babo iyi chromosome.
• Niba uri umugabo kandi ukaba ufite hemophilia B, abahungu bawe ntibazarwara iyi ndwara. Ariko abakobwa bawe bose bazaba batwara chromosome ifite gene ya F9 idasanzwe.

Ubujyanama ku bijyanye n'imiterere y'umubiri ni ingenzi cyane muri iki gihe. Niba wowe cyangwa umuntu wo mu muryango wawe afite ikibazo cyo kubura amaraso, ni byiza guhura n'umujyanama mu bijyanye n'imiterere y'umubiri mbere yo kubyara umwana.

Ese niba ntwite, nshobora kumenya mbere y'igihe niba umwana wanjye afite iyi ndwara?

Yego, urashobora. Abaganga bashobora gufata icyitegererezo cy'amaraso yo mu mugongo wawe bakayapima kugira ngo barebe niba hari ibintu bituma amaraso agwa. Muri ubwo buryo, wowe na bo mushobora kumenya mbere y'igihe icyo mugomba kwitega igihe mubyara umwana wanyu n'icyo mugomba gukora kugira ngo hirindwe ingorane zo kuva amaraso.

Niba mfite hemophilia B, ni iki nkwiye kwitega?

Hemophilia B ni indwara idakira. Abaganga ntibashobora kuyivura burundu. Ariko hari uburyo bwo kuvura bushobora gukumira cyangwa kugabanya ububabare bukomeye bw'ingingo n'ibindi bibazo by'uburwayi. Abantu benshi barwaye hemophilia B bashobora kugira ubuzima bwiza iyo bavuwe.

Abantu bafite hemophilia B ikomeye bakeneye guhabwa inshinge zisimbura izindi (infusions cyangwa inshinge) buri gihe kugira ngo bavure ibibazo byo kuva amaraso. Bashobora kujya kwa muganga kugira ngo bavurwe, umuntu wo mu muryango cyangwa ubitaho akabaha inshinge, cyangwa bashobora kuba babasha kwitera inshinge ubwabo. Ariko, abantu bafite hemophilia B ikomeye bakeneye kuvurwa buri gihe kugira ngo birinde kuva amaraso menshi.

Bashobora kandi kurwara izindi ndwara zigira ingaruka ku buzima bwabo muri rusange no ku buzima bwabo bwose. Urugero, abantu benshi barwaye hemophilia B bafite ibibazo bikomeye mu mavi cyangwa mu kibuno cyabo, bikabasaba kubagwa kugira ngo bakureho ingingo zangiritse hanyuma bakazisimbuza izindi nshya. Niba ufite hemophilia B, baza muganga wawe icyo wakwitega. Ni we muntu mwiza wo kuguha amakuru meza kuko azi uko umeze n'ubuzima bwawe muri rusange.

Ni iki nkwiye kumenya niba umwana wanjye afite iyi ndwara?

Niba umwana wawe afite hemophilia B yoroheje cyangwa iri hagati, ni ngombwa kumenyesha muganga wawe iby’iyi ndwara kugira ngo abashe kwirinda kuva amaraso menshi mu gihe umwana wawe yabazwe cyangwa agakurwa iryinyo. Dore izindi nama. Muganga wawe ashobora kugira izindi nama:

• Iyo abana bawe bakiri bato cyane, genzura neza ko intebe zabo ndende n'intebe zabo zo mu modoka bifite imikandara ikwiye yo kwirinda.
• Iyo bamaze gukura gato bagakina n'abandi bana, abarera n'abarimu babo bagomba kumenya icyo bakora mu gihe umwana yakomeretse ku bw'impanuka agatangira kuva amaraso.
• Umwana wawe azakenera kwirinda ibikorwa bimwe na bimwe, nko gukina imikino isaba gukoranaho n'abandi cyangwa ishobora gutuma agwa.

Bite se niba umwana wanjye afite hemophilia B ikomeye?

Abana bafite hemophilia B ikomeye bazakenera kuvurwa ubuzima bwabo bwose - kugira ngo birinde cyangwa bigabanye kuva amaraso, cyangwa kugira ngo barebe ibimenyetso. Dore zimwe mu mbogamizi ushobora guhura nazo, hamwe n'inama zimwe na zimwe zishobora kugufasha:

• Abana barwaye hemophilia B, iyo biga kugenda, bashobora gutangira kuva amaraso iyo bagonze cyangwa baguye. Ntibishoboka kwirinda kugwa kose, ariko ni byiza gushyira ibipfukisho byo kwirinda ku mfuruka zityaye z'ibikoresho byo mu nzu.
• Uko umwana wawe agenda akura agatangira kwiruka no gusimbuka, ganira na muganga wawe ku bijyanye n'ibikoresho byo kwirinda nk'ingofero n'amavi. Mu by'ukuri, abana bose bagomba kwambara ingofero iyo bagendera ku igare. Umwana wawe ashobora gukenera uburinzi bw'inyongera kugira ngo wirinde impanuka zishobora gutera kuva amaraso.
• Umwana wawe azakenera kwivuza buri gihe kugira ngo amaraso ahagarike kuva. Ashobora kumva arakaye kandi ababaye bitewe n'uko atabasha kumarana umwanya n'inshuti ze no kubura akazi k'ishuri mu gihe agomba kujya kwivuza.
• Umwana urwaye hemophilia aba azi ko abarimu be n'abandi bazi iby'uburwayi bwe. Ashobora kumva amerewe nabi iyo abarimu n'abandi ku ishuri bagerageje kumufasha ariko bakamufata mu buryo butandukanye.
• Abana bakuru n'urubyiruko bashobora gukenera ubufasha mu guhangana n'ibyiyumvo byabo no guhangana n'uko abandi babyitwaramo. Niba umwana wawe ari mu mimerere nk'iyo, ganira na muganga wawe ku byerekeye gahunda cyangwa amatsinda yo gufasha urubyiruko.

Mfite hemophilia B. Nitwara nte?

Kubana na hemophilia B bivuze gufata ingamba z'inyongera zo kurinda ubuzima bwawe muri rusange, mu gihe wita ku miti yawe. Dore bimwe mu bitekerezo:

• Irinde indwara zandura: Baza muganga wawe inkingo zikubereye.
• Komeza kugira ibiro byiza: Niba ufite ingorane zo kugendagenda bitewe no kuva amaraso imbere no kwangirika kw'ingingo, kugenzura ibiro byawe bishobora kugufasha.
• Injira mu myitozo ngororamubiri: Ushobora gutinya gukomereka mu gihe ukora imyitozo ngororamubiri. Vugana na muganga wawe uburyo bwo kugabanya ibyago byo kuva amaraso mu gihe ukora imyitozo ngororamubiri.
• Gucunga imihangayiko yawe: Hemophilia B ni indwara ibaho mu buzima bwawe bwose. Bishobora gusaba imbaraga zidasanzwe kugira ngo uyihuze n'inshingano z'umuryango wawe n'iz'akazi.
• Irinde imiti igabanya ububabare imwe n'imwe: Niba ufite hemophilia B, ntugomba gufata aspirine cyangwa ibuprofen. Iyi miti igabanya ububabare ibangamira amaraso kuzura.

Ugomba kujya kwa muganga wawe kugira ngo agufashe kuvurwa igihe cyose ugiye kuvurwa, ndetse n'igihe cyose ugize ibindi bimenyetso, nko kuva amaraso bidasanzwe cyangwa ububabare bukomeye bw'ingingo.

Ni ryari ugomba kujya kwivuza byihutirwa?

Niba ufite igikomere ku mutwe , ugomba kwihutira gushaka abaganga. Ibi bimenyetso bishobora kugaragaza ko ufite amaraso mu bwonko (kuva amaraso mu bwonko):

• Umutwe.
• Intege nke.
• Isesemi no kuruka.
• Kubura ibinya cyangwa ubumuga.

Niba udashobora guhagarika kuva amaraso bitewe n'ubwoko ubwo aribwo bwose bw'igikomere, cyangwa niba utangiye kuva amaraso nta mpamvu, reba muganga wawe cyangwa ujye mu cyumba cy'ubutabazi bwihuse.

Amaherezo, ibintu byo kwibuka (Ubutumwa bwo kujyana mu rugo)

Hemophilia B ni ubundi bwoko bw'indwara idakunze kugaragara. Kimwe na hemophilia A, hemophilia B ni indwara yo kuva amaraso ivuka mu muryango. Nubwo ubushakashatsi bwerekana ko abantu bafite hemophilia B bashobora kugira ibimenyetso bike cyane ugereranyije n'abafite hemophilia A, hemophilia B iracyari indwara itera ibibazo byinshi by'ubuvuzi, imibereho, ndetse n'imitekerereze.

Abagore bafite iyi ndwara bashobora kugira impungenge z'uko abana babo bazayiragwa. Ababyeyi b'umwana urwaye hemophilia B bashobora kugira impungenge zo kurinda umwana wabo imvune zitunguranye. Nyuma, bashobora kuzakenera gufasha umwana wabo urimo gukura kwiga kubana na hemophilia B. Abantu bafite hemophilia B ikomeye bashobora gukenera kuvurwa ubuzima bwabo bwose kugira ngo hirindwe kuva amaraso menshi.

Ariko hari icyizere! Abashakashatsi barimo gukora ubushakashatsi ku buryo bushya bwo kuvura, nko gusimbuza utunyangingo tw’umubiri no kuvura utunyangingo tw’umubiri. Ibi bishobora kugira itandukaniro rikomeye mu buryo indwara ya hemophilia B ivurwa. Niba wowe cyangwa umwana wawe afite iyi ndwara, baza muganga wawe ku birebana n’ibizamini byo kwa muganga bishobora kukubera byiza. Wibuke ko, hamwe n’inama n’ubuvuzi bikwiye, ushobora kubaho ubuzima bwiza kandi bwiza ufite hemophilia B.

Hemofiliya B, kuva amaraso, indwara zikomoka ku ngirabuzimafatizo, kuvura amaraso, Factor IX, indwara z'uruhererekane, indwara ya Noheli