Ese umwana wawe afite ibi bimenyetso? Reka tuvuge kuri Wiskott-Aldrich Syndrome.

Ese umwana wawe ahora arwara? Ese rimwe na rimwe agira ibibazo by'uruhu nka eczema, ava amaraso menshi ndetse no ku gisebe gito, cyangwa ahora ababara ahantu hose? Nubwo ibi bishobora gusa n'ibisanzwe, rimwe na rimwe hashobora kubaho indwara idakunze kugaragara inyuma y'iyi ndwara tutarayumva cyane. Imwe muri izo ndwara ni Wiskott-Aldrich Syndrome. Reka tubiganireho birambuye uyu munsi.

Iyi ndwara ya Wiskott-Aldrich ni iyihe?

Muri make, iyi ni indwara idakunze kugaragara mu maraso . Igira ingaruka cyane ku budahangarwa bw'umubiri bw'umwana wawe ndetse n'imikorere y'uturemangingo tumwe na tumwe mu maraso. Ibi bishobora gutera ibimenyetso byinshi icyarimwe. Harimo:

• Eczema: Indwara y'uruhu itera uduce twumye kandi turyana ku ruhu.
• Kubura ubudahangarwa bw'umubiri: Uturemangingo tw'amaraso tureza turwanya indwara mu mubiri ntidukora neza.
• Kuva amaraso no gukomereka: Ndetse no gukoraho gato bishobora gutera kuva amaraso, kandi gukomereka bishobora kubaho ahantu runaka bitewe n'ingorane zo kuvura amaraso.

Iyi ndwara rimwe na rimwe ishobora gutera ingorane zishobora gushyira ubuzima bwawe mu kaga. Ishobora kandi kugabanya igihe cyo kubaho. Ariko, hamwe n'ubuvuzi buriho ubu, izi ngaruka zishobora kugabanuka cyane .

Iyi ndwara ikunze kugaragara gute?

Ibi mu by’ukuri ni gake cyane . Mu mibare, bivugwa ko abahungu batatu muri miliyoni imwe ari bo barwara indwara ya Wiskott-Aldrich. Ndetse no mu gihugu nka Amerika, hari abantu bari munsi ya 5.000 bafite iyi ndwara. Ikunze kwibasira abahungu , kandi ni gake cyane ku bakobwa kuyirwara.

Indwara ifitanye isano na WAS ni iki?

Muganga wawe ashobora kandi kuvuga Wiskott-Aldrich Syndrome nk'indwara iterwa na WAS. Ibi bivuga indwara zigira ingaruka ku budahangarwa bw'umubiri bitewe n'impinduka mu ngirabuzimafatizo ya WAS. Urugero, thrombocytopenia ifitanye isano na X nayo ni indwara iterwa na WAS.

Ariko, indwara yitwa "congenital neutropenia" iterwa n'impinduka zitandukanye mu ngirabuzimafatizo, zidafitanye isano na gene ya "WAS". Abaganga bamenya iyi ndwara gusa iyo umwana arwaye indwara ikomeye ya bagiteri.

Ni ibihe bimenyetso by'indwara ya Wiskott-Aldrich?

Twamaze kuvuga ko hari ibimenyetso bitatu by'ingenzi by'iyi ndwara. Reka tubirebe mu buryo burambuye .

• Eczema: Ibi bituma uruhu rwumuka cyane kandi rukaryana., rimwe na rimwe ishobora gutukura no gucika uduheri. Ni nk'indwara y'iseru bamwe mu bana bato barwara, ariko ibi bishobora kuba bibi kurushaho.

• Kubura ubudahangarwa bw'umubiri: Iyi ndwara ibaho iyo uturemangingo tw'amaraso dufasha kugira ubuzima bwiza tudakora neza. Ibi bigabanya ubushobozi bw'umubiri bwo kurwanya indwara . Hari igihe ubudahangarwa bw'umubiri bushobora no gutangira kwibasira umubiri wabwo. Ibi bishobora gutera indwara nyinshi, n'indwara nka rubagimpande, vasculitis (kubyimba kw'imitsi y'amaraso), kubura amaraso (umubare muto w'amaraso), leukemia (ubwoko bwa kanseri y'amaraso), cyangwa lymphoma (ubwoko bwa kanseri y'uturemangingo tw'amaraso).

• Ibibazo byo kuva amaraso (Microthrombocytopenia): Iki ni cyo gihe umubare wa platelets, zifasha amaraso kuziba, ugabanuka cyangwa ukaba muto cyane . Mu by'ukuri, izi ni zo selile zifasha guhagarika kuva amaraso. Rero iyo ari nke, ndetse n'agasebe gato gashobora gutera kuva amaraso bidahagarara. Ibi bishobora gutera gukomereka , kuva amaraso mu mazuru, rimwe na rimwe kuva amaraso, kuva amaraso munsi y'uruhu (purpura), n'utudomo duto dutukura (petechiae) ku ruhu.

Ntugatinye iyi ndwara kuko ufite ikimenyetso kimwe cyangwa bibiri muri ibi. Ariko niba ibi bintu bikomeje, ni byiza kujya kwa muganga.

Abana bato bafite indwara ya Wiskott-Aldrich ikabije cyane (kubura ubushobozi bwo gukora) bashobora no kugaragaza ibimenyetso nk'ibi:

• Eczema
• Kubura ubudahangarwa bw'umubiri
• Indwara y'ibisebe ikabije
• Umusonga

Hari igihe, mu gihe cy’indwara yo mu bwoko bwa neutropenia iterwa no kwiyongera k’imikorere ya gene ya WAS, indwara zikomeye za bagiteri cyangwa myelodysplastic syndrome (indwara y’umusemburo w’amagufwa) zishobora kubaho.

Impamvu ni iyihe?

Impamvu nyamukuru itera indwara ya Wiskott-Aldrich Syndrome ni impinduka mu miterere y’uturemangingo, cyangwa impinduka mu miterere y’uturemangingo, muri gene ya WAS . Iyi gene ya WAS iherereye ku kaboko gato ka chromosome yacu ya X. Iyi gene ikora poroteyine ya Wiskott-Aldrich syndrome. Iyi poroteyine iboneka mu turemangingo tw’amaraso yacu twose.

Iyi poroteyine ya Wiskott-Aldrich syndrome ifasha uturemangingo twacu gufatana n'utundi turemangingo n'uturemangingo binyuze mu "gufatana." Uku gufatana ni ingenzi cyane kuko bifasha ubudahangarwa bwacu bw'umubiri gutsinda abanzi nka bagiteri na virusi byinjira mu mubiri.

Rero, niba ufite ihinduka ry’uturemangingo mu ngirabuzimafatizo yawe ya 'WAS', uturemangingo tw’amaraso yawe ntituzashobora gufatana n’utundi turemangingo n’uturemangingo neza. Ibi bizabuza ubudahangarwa bw’umubiri gukora akazi kabwo neza. Ibyo nibyo bitera ibimenyetso bya Wiskott-Aldrich Syndrome.

Mu ndwara ya neutropenia ivukanwa, impinduka mu miterere y’uturemangingo ihagarika urujya n’uruza rw’ubwoko bubiri bw’uturemangingo, utunyangingo tw’amaraso n’utunyangingo tw’amaraso, bikabuza ubudahangarwa bw’umubiri kurekura utwo turemangingo ngo turwanye indwara.

Ese ibi ni ibintu bikomoka ku bisekuruza?

Yego, iyi ni indwara ishobora kuragwa. Iragwa mu buryo bwa "X-linked recessive". Ibi bivuze ko umwana agomba kubona impinduka mu ngirabuzimafatizo ziturutse ku babyeyi bombi.

Imiterere y'imibonano mpuzabitsina tuyikura ku babyeyi bacu. Abagabo bafite chromosome imwe ya `X` na chromosome imwe ya `Y`. Abagore bafite chromosome ebyiri za `X`. Iyi ndwara yibasira abahungu cyane, kuko iyi mihindagurikire y'uturemangingo igira ingaruka ku mikorere ya chromosome yabo yonyine ya `X`.

Nubwo iyi ndwara ifite imiterere y’umuryango, abarwayi barenga 30% bayirwara "de novo" , bivuze ko iterwa n’impinduka nshya mu ngirabuzimafatizo ibaho igihe cyo gusama.

Ubimenya ute?

Ubusanzwe umuganga asuzuma indwara ya Wiskott-Aldrich Syndrome akiri umwana muto cyangwa mu bwana . Umwana arasuzumwa kandi hagakorwa ibizamini bikenewe. Ibimenyetso bya mbere by'iyi ndwara bishobora kuba amaraso mu mwanda, kuva amaraso ku buryo budasanzwe, cyangwa gukomereka. Umuganga ashobora gukora ibizamini bikurikira kugira ngo yemeze iyo ndwara:

• Umubare w'amaraso yuzuye (CBC)
• Isuzuma ry'amaraso mu buryo bw'uturemangingo
• Gupima amaraso yo mu mpande

Iyo ibi bitagaragaye mu bwana, ibimenyetso birushaho kugaragara mu bwana.

Niba umwana wawe agaragaza ibimenyetso by'uko ubudahangarwa bw'umubiri budafite imbaraga, nko kwandura indwara kenshi, hashobora kuba ngombwa ko akorerwa ibizamini by'inyongera mu bwana bwe. Ibi biterwa nuko umubiri w'umwana ushobora kudashobora kurwanya bagiteri na virusi neza, kandi ushobora kudashobora kwakira neza inkingo zimwe na zimwe.

Muganga ashobora gukora isuzuma ry'amaraso kugira ngo arebe niba umubiri w'umwana wawe urimo gukora ubudahangarwa nyuma yo guhabwa urukingo. Utu dusimba ni two dutuma ubudahangarwa bw'umubiri butera imbaraga zo kwirinda indwara zikomeye. Byongeye kandi, hazakorwa irindi suzuma ry'amaraso kugira ngo hagenzurwe uturemangingo tw'amaraso tw'umwana wawe, cyane cyane uturemangingo twa T na immunoglobulines (nabyo bifasha mu gukora ubudahangarwa bw'umubiri).

Ni ubuhe buryo bwo kuvura ibi?

Indwara ya Wiskott-Aldrich ishobora kuvurwa muri ubu buryo bukurikira:

• Kuvura indwara zandurira mu mubiriAntibiyotike cyangwa imiti igabanya ubukana bw'indwara.
• Hatangwa imiti igabanya ubukana bw'umubiri (immunoglobulin) kugira ngo hagarurwe ubudahangarwa bw'umubiri bwagabanutse.
• Guterwa amaraso hakoreshejwe uburyo bwo gutera amaraso mu gihe cyo kuva amaraso.
• Eczema ishobora kuvurwa n'imiti yo mu bwoko bwa "topics" hamwe n'imiti yo kwisiga itangwa na muganga (OTC) .

Iyo umwana wawe arwaye indwara cyangwa indwara, bishobora kuba bikomeye ndetse bikanashyira ubuzima bwe mu kaga . Kugira ngo urinde ubuzima bw'umwana wawe, ushobora no kugerageza ubu buryo bwo kuvura:

• Gutera uturemangingo tw’inyuma : Iki gishobora kuba igisubizo cyiza cyane gihari muri iki gihe.
• Kuvura imiterere y’uturemangingo (ibi biracyari mu cyiciro cy’ubushakashatsi).

Muganga w'umwana wawe azaganira nawe kuri ubu buryo bwo kuvura kandi ateganye uburyo bwo kuvura kugira ngo agere ku musaruro mwiza ku mwana wawe no kunoza ubuzima bwe.

Niba umwana wanjye afite iyi ndwara, ni iki nakwitega?

Niba umwana wawe afite indwara ya Wiskott-Aldrich Syndrome, muganga wawe azategura gahunda yo kumufasha kwirinda ingorane zishobora guterwa n’ubuzima bwe bitewe n’uko ubudahangarwa bw’umubiri bwe budakora neza. Nyuma yo kumenyekana, ushobora koherezwa mu bujyanama ku bijyanye n’imiterere y’umubiri . Ibi bishobora kugufasha wowe n’umuryango wawe kumenya byinshi ku bijyanye n’iyi ndwara n’uburyo bwo kuyivura buhari.

Ndetse n'indwara zisanzwe zo mu bwana nka chickenpox zishobora guteza ibibazo bikomeye ku buzima bw'umwana wawe. Kubera ko ubudahangarwa bwe bw'umubiri budakomeye nk'ubw'abandi bana bo mu kigero cye, ashobora gukenera kujya kwa muganga ako kanya. Kuvura indwara n'indwara hakiri kare bishobora kugufasha kubona ibisubizo byiza.

Muganga wawe ashobora kukubwira kudaha umwana wawe inkingo za virusi nzima (nk'inkingo y'ibicurane) kuko ubwoko bw'icyorezo bushobora kurwara umwana wawe. Nubwo umwana wawe yahabwa inkingo zimwe na zimwe, zishobora kutagira akamaro. Iyo umwana wawe arwaye virusi, kuvurwa hakiri kare n'imiti igabanya ubukana bw'indwara ziterwa na virusi akenshi bikora neza. Ariko n'indwara zisanzwe zishobora gutera ingorane.

Umwana wawe ashobora gukenera gupimwa kanseri buri gihe mu buzima bwe bwose kuko aba afite ibyago byinshi byo kurwara kanseri zimwe na zimwe, cyane cyane kanseri y’amaraso cyangwa kanseri y’amaraso .

Ese hari umuti wuzuye w'ibi?

Yego, guterwa uturemangingo tw’ingirabuzimafatizo (byitwa kandi guterwa umwongo w’amagufwa) bishobora kuvura indwara ya Wiskott-Aldrich Syndrome.Ubwo bwoko bw'impinduka zikuraho uturemangingo tw'umubiri dukora amaraso hanyuma zikabisimbuza uturemangingo tw'umubiri dufite ubuzima bwiza. Kubera ko indwara ya Wiskott-Aldrich ituma uturemangingo tw'amaraso tudasanzwe dukora, impyiko zishobora gusimbuza utwo turemangingo uturemangingo dufite ubuzima bwiza kandi dukora.

Ese indwara ya Wiskott-Aldrich ishobora kwica?

Indwara ya Wiskott-Aldrich Syndrome ishobora kwica . Byavuzwe mbere ko abana badafite uturemangingo tw’umubiri bafite igihe cyo kubaho cy’imyaka 15. Ibimenyetso biterwa n’indwara zandura, kuva amaraso mu bwonko bw’umwana, indwara zikomeye cyangwa kanseri bishobora gushyira ubuzima mu kaga, kandi urupfu rushobora kubaho vuba.

Ariko, hamwe n’iterambere ry’ubumenyi bw’ubuvuzi, uburyo bwo kuvura bugezweho bwatumye bishoboka kongera igihe cyo kubaho cy’umwana muri rusange.

Ese ibi bishobora kwirindwa?

Iyi ni indwara iterwa n'uturemangingo kandi ntishobora kwirindwa . Niba uteganya kubyara kandi ukaba ushaka kumenya ibyago byo kubyara umwana ufite ikibazo cy'uturemangingo, vugana na muganga wawe ku bijyanye no gupima uturemangingo .

Ni ryari nkwiye kubonana na muganga w'umwana wanjye?

Niba umwana wawe arwaye kandi akaba yarasanzwe afite indwara ya Wiskott-Aldrich Syndrome, hamagara muganga we ako kanya . Kubera ko ubudahangarwa bw'umubiri bwe budakora neza, ashobora kwandura indwara kenshi. Muganga wawe ashobora kuvura indwara cyangwa ubwandu, cyangwa akarinda ingorane zishobora kumuviramo.

Reba muganga wawe niba umwana wawe afite kimwe muri ibi bikurikira:

• Amaraso mu muyoboro w'amacandwe (Impiswi iva amaraso)
• Kuva amaraso mu mazuru kenshi
• Ahantu hanini hagaragara ibikomere
• Impinduka ku ruhu rwabo
• Kwandura indwara zisubira (urugero: inkoko cyangwa thrush ikomeye, indwara za bagiteri)

Ni ibihe bibazo ngomba kubaza muganga?

Ushobora kubaza muganga ibibazo nk'ibi:

• Imyaka y'ubuzima bw'umwana wanjye ni iyihe?
• Ni ibihe bicuruzwa nakoresha mu kuvura eczema y'umwana wanjye?
• Ese hari ingaruka mbi ku miti utanga?
• Ese umwana wanjye akwiriye guterwa uturemangingo tw’umubiri?

Ni irihe tandukaniro riri hagati ya Wiskott-Aldrich Syndrome na Ataxia-Telangiectasia?

Indwara ya Wiskott-Aldrich Syndrome na Ataxia-Telangiectasia byombi ni indwara ziterwa n’uturemangingo zigira ingaruka ku mikorere y’ubudahangarwa bw’umubiri.Ariko, ataxia-telangiectasia iterwa n'impinduka mu ngirabuzimafatizo ya ATM. Igira ingaruka ku mikorere n'imikorere y'umubiri wawe. Inatuma imiyoboro y'amaraso isa n'aho imeze nk'izunguruka ku ruhu rwawe.

Ibintu by'ingenzi ugomba kwibuka

Kumenya ko umwana wawe afite indwara idakunze kugaragara izamugiraho ingaruka ubuzima bwe bwose bishobora kugutera ubwoba. Bishobora gutera ubwoba. Ariko ntugahangayike. Muganga w'umwana wawe azakubwira byinshi kuri iyo ndwara n'uko wafasha umwana wawe mu rugo. Ubudahangarwa bw'umubiri bw'umwana wawe bushobora kuba budakora neza, bityo ashobora kurwara kenshi.

Ni ngombwa kwiyitaho mu gihe wita ku mwana wawe. Ababyeyi benshi n'imiryango babona ihumure rikomeye n'ubufasha mu kuganira n'umujyanama mu by'ubuzima bwo mu mutwe .

Bitewe n'iterambere mu buvuzi buriho ubu, abana bafite iyi ndwara ubu bashobora kubaho ubuzima bwuzuye kandi bushimishije. Bityo rero komeza gukomera.

Indwara ya Wiskott -Aldrich, indwara ziterwa n'uturemangingo, ubudahangarwa bw'umubiri, eczema, kuva amaraso, gutera uturemangingo tw'umubiri, ubuzima bw'umwana