Ese umwana wawe ahora arwaye? Bishobora kuba ari XLA (X-Linked Agammaglobulinemia). Reka tuganire!

Nubwo rimwe na rimwe dutekereza ko ari ibisanzwe ko abana bacu barwara kenshi, kuri bamwe iki kibazo gishobora kuba ikibazo gikomeye. Cyane cyane niba umuhungu ahora arwaye indwara zandurira mu mikorobe, bishobora guterwa n’indwara idakunze kugaragara mu buryo bw’uturemangingo. Uyu munsi tugiye kuvuga kuri iyo ndwara.

XLA (X-Linked Agammaglobulinemia) ni iki?

None se XLA (agammaglobulinemia ifitanye isano na X) ni iki? Mu magambo make, ni indwara iterwa n'uturemangingo . Umubiri wacu ufite ingabo z'ingenzi cyane zo kurwanya indwara, kandi ni bwo budahangarwa bwacu bw'umubiri . Ubwoko bwihariye bw'uturemangingo muri uru rwego bwitwa B-cells . Utu turemangingo twa B ni two dukora poroteyine zitwa antibodies zo kurwanya indwara iyo imibiri yacu irwaye. Utu turemangingo dukora nk'abasirikare barinda igihugu cyacu.

Umuntu urwaye XLA (X-Linked Agammaglobulinemia) ntakora utwo turemangingo twa B neza. Cyangwa se tugakora duke cyane muri two. None se, bigenda bite iyo udashobora gukora uturemangingo tw’umubiri? Urwara byoroshye kandi urwara kenshi. Nanone, ingingo zo mu mubiri wacu zifitanye isano n’ubudahangarwa bw’umubiri, nka limfu , tonsils, na adenoids, ntizikura neza, kandi rimwe na rimwe ntizibaho na gato. Iyi ndwara ikunze kugaragara ku bahungu . Tuzavuga ku mpamvu y’ibi nyuma.

Iyi ndwara, yitwa XLA (X-Linked Agammaglobulinemia), izwi kandi ku yandi mazina:

• Agammaglobulinemia ya Bruton
• Agammaglobulinemiya yo mu gihe cy'amavuko
• Hypogammaglobulinemie

Ariko, izina Hypogammaglobulinemia rikoreshwa no ku yindi ndwara isa nayo. Iyo ni CVID (Common Variable Immunodeficiency) . CVID (Common Variable Immunodeficiency) ubusanzwe ntabwo ikabije nka XLA (X-Linked Agammaglobulinemia), kandi ikunze kugaragara mu gihe cy'ubukure. Ariko, abana bafite XLA (X-Linked Agammaglobulinemia) basuzumwa mbere y'umwaka umwe cyangwa bakiri bato cyane.

Ni irihe tandukaniro riri hagati ya XLA (X-Linked Agammaglobulinemia) na SCID (Severe Combined Immunodeficiency)?

Noneho ushobora kuba wibaza niba iyi ari XLA (X-Linked Agammaglobulinemia) cyangwa indwara ikomeye y’ubudahangarwa bw’umubiri ushobora kuba warumvise yitwa SCID (Severe Combined Immunodeficiency) . Oya, hari itandukaniro rito hagati y’ibyo byombi. Muri XLA (X-Linked Agammaglobulinemia) , uturemangingo twavuze mbere twibasirwa cyane cyane. Muri SCID (Severe Combined Immunodeficiency) , uturemangingo twa T turabasirwa.Ubundi bwoko bw'ingenzi bw'uturemangingo tw'umubiri twitwa T-cells (rimwe na rimwe B-cells nazo zishobora kwibasirwa). Zombi ni indwara zituma ubudahangarwa bw'umubiri bugabanuka kandi akenshi zigatera indwara. Ariko itandukaniro rikomeye hagati y'izo zombi ni ubwoko bw'uturemangingo twagizweho ingaruka.

XLA (X-Linked Agammaglobulinemia) ikunze kugaragara gute?

Iyi ndwara yitwa XLA (X-Linked Agammaglobulinemia) mu by'ukuri ni gake cyane . Ibyo bivuze ko atari indwara abantu benshi barwara. Ariko, nk'uko twabivuze mbere, ikunze kugaragara ku bahungu . Mu mibare, abahungu bagera ku 1 mu 200.000 bavukana XLA (X-Linked Agammaglobulinemia).

Ni ibihe bimenyetso bya XLA (X-Linked Agammaglobulinemia)?

Kubera ko abana bafite XLA (X-Linked Agammaglobulinemia) bafite ubudahangarwa bw'umubiri budakomeye, utunyangingo twa lymph, tonsils, na adenoids akenshi biba ari duto cyangwa ntibibeho . Ibi bituma bakunze kwandura bagiteri kenshi kuva bakiri bato. Urugero:

• Bronchite : Iyi ni indwara ya bronchi, imiyoboro ijyana mu bihaha.
• Indwara zo mu matwi (Otitis media) : Indwara zo mu gutwi ko hagati.
• Sinusiti : Kwandura kwandura mu ruhu hafi y'izuru.
• Umusonga : Indwara ikomeye ifata ibihaha.
• Indwara zo mu gifu : Ibintu nko kubabara mu gifu no gucibwamo.

Ariko ni ngombwa kwibuka ko abana bafite XLA (X-Linked Agammaglobulinemia) akenshi badakunze kwandura indwara za virusi (urugero: Cytomegalovirus (CMV) , RSV (Respiratory Syncytial Virus) , cyangwa indwara z'ibihumyo . Baterwa ahanini n'indwara za bagiteri.

Ni iki gitera XLA (X-Linked Agammaglobulinemia)?

Nkuko twabivuze mbere, XLA (X-Linked Agammaglobulinemia) ni indwara iterwa n’uturemangingo . Ibi bivuze ko umwana ayikura kuri nyina, se, cyangwa bombi. Dufite gene yitwa BTK mu mubiri wacu. Iyi gene itegeka umubiri wacu gukora uturemangingo twa B. Tuzi ko uturemangingo twa B dukora uturemangingo kandi tukarwanya indwara.

Rero, iyo habayeho impinduka cyangwa impinduka muri iyi gene ya BTK, uturemangingo twa B ntidushobora kuremwa neza. Icyo gihe, ikibaho ni uko umuntu udafite iyo gene mutation adashobora kurwanya indwara nk'uko abikora. Niyo mpamvu ahora arwara, ndetse rimwe na rimwe akarwara indwara zikomeye zishobora gushyira ubuzima mu kaga.

'X-linked' ni iki?

Noneho reka turebe icyo 'X-linked' bivuze. Twese tubona gene zacu ku babyeyi bombi. Izi gene ziba ku bintu byitwa chromosomes . Izi gene zibwira imibiri yacu uburyo bwo gukora poroteyine bakeneye kugira ngo ikore. Akenshi, nubwo haba hari impinduka muri gene imwe, indi kopi (iy'undi mubyeyi) iba ikiriho, bityo umubiri ushobora gukora uko bikwiye.

Ariko, chromosomes z'abagabo ku gitsina si zimwe. Zifite chromosome imwe ya X na chromosome imwe ya Y. Rero, niba hari impinduka muri gene kuri iyi chromosome ya X, nta yindi kopi ihari yo kuyikemura. Gene ya BTK twavuze iherereye kuri iyi chromosome ya X. Niyo mpamvu yitwa 'X-linked'. Rero niba umuhungu afite impinduka muri gene ya BTK kuri chromosome ye ya X, azarwara XLA (X-Linked Agammaglobulinemia).

Abakobwa bafite chromosomes ebyiri za X. Nubwo bafite impinduka zitera XLA (X-Linked Agammaglobulinemia) kuri imwe muri chromosomes zabo za X, gene ya BTK iri kuri chromosome ya X iracyakora neza, bityo bashobora gukora umubare ukenewe wa B-cells. Bityo ntibarwara iyi ndwara, ariko bashobora kuyitwara . Ibyo bivuze ko bashobora gutwara gene batagaragaje ibimenyetso.

Ni ibihe bintu bishobora gutera XLA (X-Linked Agammaglobulinemia)?

Kugeza ubu, ikintu cyonyine kizwi gishobora gutera indwara ya XLA (X-Linked Agammaglobulinemia) ni amateka y’umuryango w’iyi ndwara . Ibi bivuze ko irazwe.

Ese abakobwa bashobora kugira XLA (X-Linked Agammaglobulinemia)?

Yego, ni gake cyane , umwana w’umukobwa ashobora no kurwara XLA (X-Linked Agammaglobulinemia). Ariko kugira ngo ibyo bishoboke, ababyeyi bombi bagomba kuba bafite chromosome X ifite gene ya BTK yahinduwe. Ni ukuvuga ko nyina ari we uyitwara naho se akaba afite XLA (X-Linked Agammaglobulinemia). Ubusanzwe, abana b’abakobwa bashobora kuba ari bo batwara iyi mihindagurikire y’uturemangingo. Hanyuma, nubwo badafite iyi ndwara, bashobora kwanduza abana babo. Niba abo bana ari abahungu, bashobora kurwara XLA (X-Linked Agammaglobulinemia).

Ni izihe ngorane zishobora guterwa na XLA (X-Linked Agammaglobulinemia)?

Zimwe mu ngorane zishobora kubaho kuri XLA (X-Linked Agammaglobulinemia) ni izi:

• Indwara y'ibihaha idakira : Kwandura ibihaha kenshi bishobora kwangiza ibihaha uko igihe kigenda gihita.
• Kwandura gukwirakwira mu bindi bice by'umubiri : Urugero, kwandura bishobora gukwirakwira mu maraso (sepsis) cyangwa mu bwonko (meningitis).
• Hari kandi gukeka ko hashobora kuba hari ibyago byinshi byo kurwara ubwoko bumwe na bumwe bwa kanseri , ariko ubushakashatsi bukomeje gukorwa kuri ibi.

Ni gute XLA (X-Linked Agammaglobulinemia) ipimwa?

Muganga ashobora gukora ibizamini byinshi by'amaraso kugira ngo amenye niba wowe cyangwa umwana wawe afite XLA (X-Linked Agammaglobulinemia). Niba ibi bizamini by'amaraso bigaragaza ko uturemangingo twa B cyangwa uturemangingo tw'umubiri turi hasi, muganga wawe azakora ibizamini bya genetiki . Ibi bireba impinduka muri gene ya BTK muri ADN yawe .

Ni gute XLA (X-Linked Agammaglobulinemia) ivurwa?

Ikibabaje ni uko nta muti wa XLA (X-Linked Agammaglobulinemia). Ariko, hari uburyo bwo kuvura bushobora gufasha gukumira ingorane zikomeye. Izingenzi ni izi:

• Ubuvuzi bwo Gusimbuza Immunoglobulin (RIgG) : Ibi bikubiyemo gutanga uturemangingo tw’umubiri duturutse ku batanga imiti bazima mu mitsi (IV). Ubu buvuzi butangwa nibura rimwe mu kwezi . Ibi bifasha kugenzura urugero ruto rw’uturemangingo mu mubiri ku rugero runaka.
• Kuvura indwara hakiri kare : Mu gihe wowe cyangwa umwana wawe mukekwaho kuba mufite indwara, muganga azatangira kuvura indwara za bagiteri akoresheje imiti yica udukoko . Ni ngombwa gutangira kuvura hakiri kare.
• Kwirinda inkingo nzima : Abantu bafite XLA (X-Linked Agammaglobulinemia) ntibagomba guhabwa inkingo nzima . Izi nkingo zishobora gutera indwara zikomeye kandi zishobora gushyira ubuzima mu kaga. Urugero, harimo urukingo rwa MMR (iseru, mumps, rubella) , urukingo rwa chickenpox - varicella, n'urukingo rwa polio rukoreshwa mu kanwa . Kubwibyo, ni ngombwa kuvugana na muganga wawe kuri ibi kandi ukabimenyeshwa neza.

Ni iki umuntu urwaye XLA (X-Linked Agammaglobulinemia) yakwitega?

Abantu barwaye XLA (X-Linked Agammaglobulinemia) bazakenera gufata imiti ubuzima bwabo bwose . Ibi ni ukugira ngo bagabanye ibyago byo kurwara indwara. Bagomba gukomeza kugirana umubano wa hafi na muganga wabo no gushaka ubuvuzi vuba igihe cyose habayeho indwara iyo ari yo yose. Wowe cyangwa umwana wawe ufite XLA (X-Linked Agammaglobulinemia) mushobora gusiba iminsi myinshi y'ishuri n'akazi kubera uburwayi kurusha abaturage muri rusange.

Abantu barwaye XLA (X-Linked Agammaglobulinemia) bamara igihe kingana iki?

Ni ikintu cyiza cyane ko, hamwe n’iterambere ry’uburyo bwo kuvura , abantu bafite XLA (X-Linked Agammaglobulinemia) mu bihugu byateye imbere nka Amerika babaho kugeza bakuze . Ariko, mu bihugu bikiri mu nzira y’amajyambere, biracyagoye cyane gusuzuma no kwakira ubuvuzi. Kubwibyo, muri rusange, igihe cyo kubaho cy’abana bafite XLA (X-Linked Agammaglobulinemia) muri bene ibyo bihugu gishobora kugabanuka. Ariko muri Sri Lanka, ubu hari uburyo bwiza bwo kuvura bene ibyo bibazo.

Ese XLA (X-Linked Agammaglobulinemia) ishobora kwirindwa?

Niba uhangayikishijwe na XLA (X-Linked Agammaglobulinemia), bivuze ko hari umuntu wo mu muryango wawe ufite iyi ndwara, ushobora kubonana na muganga agasuzuma imiterere y’uturemangingo . Ibi bishobora kugufasha kumenya indwara z’uturemangingo ushobora kwanduza umwana wawe. Niba uri umuntu utwara imiterere y’uturemangingo itera XLA (X-Linked Agammaglobulinemia), iyo ufite umwana, hari amahirwe 50% ko uwo mwana azaragwa iyo miterere. Niba uwo mwana ari umuhungu, ashobora kurwara XLA (X-Linked Agammaglobulinemia). Niba ufite XLA (X-Linked Agammaglobulinemia), abakobwa bawe bazaba ari bo bayirwara. Umujyanama mu by’uturemangingo cyangwa muganga wategetse ko ikizamini kizaguha inama nyinshi kuri ibi.

Nitwara nte?

Uburyo bwiza bwo kwita ku buzima bwawe hamwe na XLA (X-Linked Agammaglobulinemia) ni ugushyira imbere ubuzima bwawe . Komeza gahunda za muganga. Menya kumenya ibimenyetso by'ubwandu. Baza muganga icyo wakora niba ugaragaje ibimenyetso by'ubwandu.

Ni ryari ngomba kujya kwa muganga?

Ni byiza kuvugana na muganga mu bihe nk'ibi:

• Niba umwana wawe arwaye nyuma yo guhabwa urukingo ruzima (urugero: urukingo rwa MMR, urukingo rw'inkoko).
• Niba umwana wawe akunze kwandura indwara za bagiteri .
• Niba kandi ukunze kwandura bagiteri (kuko iyi ishobora kuba indwara nka CVID, ifata n'abantu bakuru).

Muganga azashobora kukubwira niba ibi bigomba gukorwaho ubushakashatsi bwimbitse cyangwa bitagomba gukorwaho ubushakashatsi.

Ni ibihe bibazo ngomba kubaza muganga wanjye?

Bishobora kugufasha kubaza ibibazo nk'ibi mugihe ugiye kwa muganga wawe:

• Ni ibihe bimenyetso by'ubwandu nkwiye kwitondera?
• Nkwiye gukora iki mu gihe ntekereza ko njye cyangwa umwana wanjye afite ubwandu?
• Ni ubuhe buryo bwo kuvura?
• Ni kangahe njye cyangwa umwana wanjye dukeneye kuvurwa?

Ni ibisanzwe ko abana bato barwara kenshi. Ariko niba umwana wawe akunda kwandura indwara za bagiteri, hashobora kuba hari ikindi kintu kibiteye. Vugana na muganga wawe ku mpungenge ufite. Gusuzuma no kuvurwa hakiri kare ni bwo buryo bwiza bwo kubona ibisubizo byiza.

Niba ubana na XLA (X-Linked Agammaglobulinemia) - indwara y’uturemangingo aho umubiri wawe udakora neza uturemangingo twa B - kurikiza amabwiriza ya muganga wawe neza. Ni ngombwa cyane kumenya ibimenyetso by’ubwandu kugira ngo ubashe kuvurwa vuba bishoboka.

Ibintu by'ingenzi cyane ugomba kwibuka muri iyi nkuru

Noneho rero, dore ibintu bike ugomba kwibuka mu byo twavuze kuri XLA (X-Linked Agammaglobulinemia):

• XLA (X-Linked Agammaglobulinemia) ni iki?Indwara idakunze kugaragara mu ngirabuzimafatizo yibasira cyane cyane abahungu .
• Ibi bibaho iyo uturemangingo twa B tw’umubiri tudakura neza , bigatuma uturemangingo tw’umubiri tugabanuka ndetse n’indwara zandura kenshi ziterwa na bagiteri .
• Ibintu nk'imitsi yo mu bwonko na adenoid bishobora kugabanuka cyangwa bigashira.
• Nubwo nta muti wihariye uhari, ushobora kugenzurwa neza hakoreshejwe uburyo bwo kuvura indwara z’ubuzima bwose (RIgG) no kuvurwa vuba hifashishijwe imiti igabanya ubukana bw’indwara.
• Si byiza guha aba bantu inkingo nzima.
• Niba umwana wawe w'umuhungu akunze kurwara bikomeye, ni ngombwa cyane gushaka inama kwa muganga vuba. Gusuzuma umwana hakiri kare no kumuvura bizamufasha cyane kubaho ubuzima busanzwe.

Ndizera ko aya makuru azagufasha. Niba ufite ikibazo icyo ari cyo cyose, ntutindiganye kuvugana na muganga w'umuryango wawe!

Agammaglobulinemia ifitanye isano na X , XLA, B-seli, ubudahangarwa bw'umubiri, indwara ziterwa n'uturemangingo, indwara zikunze kugaragara, abahungu, ubudahangarwa bw'umubiri, gene ya BTK, ubuvuzi bwa RIgG