X-Linked Agammaglobulinemia Chii? Zviratidzo, Zvinokonzera, & Kurapwa

X-linked agammaglobulinemia (XLA) chirwere chisingawanzo chiri chose chemageneti umo muviri wako usingakwanisi kugadzira maantibody akawanda ekurwisa hutachiona anonzi immunoglobulins. Izvi zvinoitika nekuti gene chaiyo inobatsira kugadzira masero anogadzira maantibody haishande zvakanaka, zvichikusiiya uri panjodzi yehutachiona hwakati.

Funga nezvemaantibody sechikwata chekuchengetedza chemuviri wako chinoyeuka uye chinorwisa hutachiona hwausangana nahwo kare. Kana uine XLA, chikwata ichi chekuchengetedza chine vanhu vashoma kwazvo, zvichiita kuti muviri wako usakwanise kuzvidzivirira kubva kubhakitiriya nemavhairasi akati.

Zviratidzo zveX-linked agammaglobulinemia ndeapi?

Chiratidzo chinonyanya kuzivikanwa cheXLA ndechekuwana hutachiona hwakakomba hwebhakitiriya kakawanda, kazhinji kutanga mumasvondo mashomanana ekutanga kana makore ehupenyu. Izvi hazvisi kungoita fodya kana zvirwere zvisina kukomba, asi hutachiona hunoratidzika kuva hwakakomba kwazvo kana kuramba huchidzoka kunyange mushure mekurapwa.

Aya ndiwo zviratidzo zvikuru zvaunogona kuona, uchifunga kuti ruzivo rwemunhu mumwe nemumwe runogona kusiyana:

• Kunetseka kwezvirwere zvekufema: Pneumonia, bronchitis, kana kuzvimba kwemhino kunowanzoitika mazuva mashoma kana mwedzi mishoma yega yega
• Kunetseka kwezvirwere zve nzeve: Kunetseka kwezvirwere zve nzeve dzepakati izvo zvinogona kukonzera matambudziko ekunzwa kana zvisina kurapwa zvakanaka
• Kunetseka kwezvirwere zve ganda ne tishu dzakapfava: Mabhuru, mabhuru, kana cellulitis anowanzooneka
• Kunetseka kwezvirwere zveura: Manyoka asingaperi kana kunetseka kwemudumbu izvo zvisingapindure zvakanaka kurapwa kwakajairwa
• Kunetseka kwezvirwere zve majoini: Kuzvimba, kurwadziwa, nekukomba kwe majoini, kunyanya mazvikokota, makwati, kana mawoko
• Kuvhira kwekuporesa kwemaronda: Kucheka, kukwenya, kana maronda ekubatanidza anotorwa nguva yakareba kupfuura zvaitarisirwa kuporesa
• Kusakwanisa kukura: Muvana vaduku nevana vaduku, kuwedzera uremu kushoma kana kukura kwakaderera

Chinonyanya kuoma nezve XLA ndechekuti aya marapirwo kazhinji haapindure nekukurumidza kune maantibayotiki sezvaizoita mumunhu ane hutano hwakanaka hwemuviri. Ungacherechedza kuti marapirwo anoramba aripo kwenguva yakareba kana kuti anoda mishonga ine simba kupfuura zvakajairwa.

Zvinokosha kucherechedza kuti vanhu vane XLA vanowanzoita zvakanaka nezvirwere zvehutachiona senge chickenpox kana measles, sezvo T-cells dzavo (chimwe chikamu che immune system) inoshanda zvakanaka. Izvi zvinogona kuva chiratidzo chinobatsira kune vanachiremba pakuita chirwere.

Chii chinokonzera X-linked agammaglobulinemia?

XLA inokonzerwa nekushanduka (mutations) muchiitiko chinonzi BTK, icho chinomiririra Bruton's tyrosine kinase. Ichi chiitiko chine mirairo yekugadzira protein inokosha kuti B-cells ikure zvakanaka.

B-cells ndidzi sero chena dzakasarudzika dzinoibva kuva plasma cells, idzo dziri fekitori dzema antibodies emuviri wako. Kana BTK gene isingashande zvakanaka, B-cells haigone kupera kukura kwadzo, saka unopera uine B-cells shoma kana kuti hapana B-cells ne plasma cells dzakakura.

Zvirwere izvi zvinonzi "X-linked" nekuti gene reBTK rinowanikwa pachromosome ye-X. Sezvo varume vane chromosome imwe chete ye-X (XY), vanoda kopi imwe chete yakanganisika yegene kuti vawane XLA. Vakadzi vane ma chromosomes maviri e-X (XX), saka vaizoda ma kopi akanganisika pama chromosomes ese maviri kuti akanganiswe, izvo zvisingawanzoitiki.

Nzira iyi yekuwedzera inoreva kuti XLA inokanganisa varume chete uye inopfuudzwa kubva kunaamai vanotakura shanduko yemajini. Amai vanotakura kazhinji vane masisitimu avo ekuzvidzivirira akajairika asi vane mukana we50% wekupfuudza chirwere ichi kumwanakomana wese.

Marudzi eX-linked agammaglobulinemia nde api?

XLA haina marudzi akasiyana-siyana seamamwe mamiriro ezvinhu, asi vanachiremba vanoziva kuti kuomarara kunogona kusiyana zvikuru kubva kumunhu kuenda kumunhu. Vamwe vanhu vanosangana nezvirwere zvakawanda kana zvakakomba, nepo vamwe vangave nechirwere chinenge chisinganyanyi kuoma.

Kupokana kazhinji kunotsamira pane kuti gene reBTK rakakanganiswa sei. Shanduko dzakawanda dzemajini dzinovhara puroteni kubva kushanda, nepo dzimwe dzinoita kuti ishande zvishoma. Zvisinei, kunyangwe nekusiyana uku, vanhu vose vane XLA vanogovana dambudziko rimwe chete rekusakwanisa kugadzira maantibodies akakwana.

Chiremba wenyu angasiyanisawo pakati pemamiriro ekutanga uye akaonekwa gare gare. Vana vazhinji vane XLA vanotanga kuratidza zviratidzo mukati megore maviri ekutanga ehupenyu, asi dzimwe nguva, zviitiko zvisinganyanyi kuoma hazviziveki kusvika pazera rekukura kana kunyange mukukura.

Nguva yekuona chiremba weX-linked agammaglobulinemia?

Unofanira kutsvaka rubatsiro rwechiremba kana iwe kana mwana wako ane zvirwere zvakawanda, zvakakomba, kana zvisingawanzoitiki izvo zvisingatarisiri maitiro akajairika. Izvi zvinokosha zvikuru kana zvirwere zvisingapindure zvakanaka pakurapa kwakajairika kana zvichienderera mberi nekukurumidza mushure mekupedza maantibiotic.

Funga kuona chiremba nekukurumidza kana ukaona chero chezviratidzo izvi zvechinyevero:

• Zvirwere zvekunzwa zvinopfuura zvina mumwedzi gumi nemambiri
• Zvirwere zviviri kana kupfuura zve sinus zvakakomba mukati memwedzi gumi nemambiri
• Pneumonia inowanzoitika kupfuura kamwe kamwe
• Zvirwere zvinotoda kurapwa muchipatara kana mishonga inoiswa mutsinga
• Zvirwere zvinoramba zvichiitika kunyange mushure mekurapwa kwakakodzera nemishonga inorwisa mabhakitiriya
• Zvirwere zvisingawanzoitika munzvimbo dzakadai semajoini kana matishu akadzika
• Kukura kwakashata kana kuvanduka kwevana pamwe chete nezvirwere zvinowanzoitika

Kana paine nhoroondo yemhuri yehutachiona hwakaderera kana uri mukadzi ane hama dzevarume dzakanga dzine zvirwere zvakakomba zvakawanda muhudiki, zvinokosha kutaura izvi nachiremba wako. Kuziva nekurapa nekukurumidza kunogona kuita musiyano mukuru mukudzivirira matambudziko.

Usanetseka kukurudzira iwe pachako kana mwana wako kana chimwe chinhu chisinganzwisisiki, kunyangwe vamwe vachiti zvirwere izvo ndezve“zvakajairika.” Tenda pfungwa dzako kana zvirwere zvichiita sekunge zvakawanda kana zvakakomba zvakanyanya.

Zvinhu zvinokonzera ngozi ye X-linked agammaglobulinemia ndeapi?

Chinhu chikuru chinokonzera ngozi yeXLA ndechekuva nekuchinja kwemajini kunokonzera mamiriro acho. Sezvo iri chirwere chinogona kuwanikwa kubva kumhuri, nhoroondo yemhuri ndiyo inokosha zvikuru mukuona ngozi.

Heano maitiro makuru anoita kuti uve neXLA:

• Kuva murume: Sezvo XLA iri X-linked, varume ndivo chete vanowanzobatwa
• Kuva naamai vanotakura: Vakadzi vanotakura kopi imwe chete yakashata yeBTK gene vane mukana we50% wekuipasa kumwanakomana wavo mumwe nemumwe
• Nhoroondo yemhuri: Kuva nehama dzevarume (kunyanya kurutivi rwaamai) vane nhoroondo yezvirwere zvakakomba zvakawanda kana hutachiona hwakaderera hwakazivikanwa
• Muenzaniso wemhuri yamai: Muenzaniso wekufa kwevana vevarume kana zvirwere zvakakomba zvinokanganisa vakomana mumutsara wemhuri yamai

Zvakakosha kunzwisisa kuti XLA haikonzerwi nezvinhu vabereki vakaita kana vasina kuita panguva yekuzvitakura. Haisi yakabatana nezvinhu zvehupenyu, zvinhu zvakapoteredza, kana kurapwa panguva yekuzvitakura. Shanduko yemajini inokonzera XLA inogona kuwanikwa kubva kumakore apfuura kana kuti inogona kuitika sechitsva chitsva.

Muimwe nguva, shanduko yemajini inoitika kekutanga mumhuri, zvinoreva kuti hapana nhoroondo yemhuri yapfuura. Izvi zvinoitika mune anenge 15-20% yezviitiko zveXLA uye zvinonzi "de novo" kana kuti mutation itsva.

Ndezvipi zvinogona kuitika zvinetso zveX-linked agammaglobulinemia?

Pasina kurapwa kwakakodzera, XLA inogona kukonzera matambudziko akakomba akawanda, asi zvakakosha kuziva kuti mazhinji emaizvi anogona kudzivirirwa kana kutarisirwa zvakanaka nekurapwa kwakakodzera. Kunzwisisa matambudziko aya kunokubatsira iwe nechikwata chako chehutano kuti murambe muri vakangwarira uye mutora matanho ekudzivirira.

Matambudziko akajairika anosanganisira:

• Kukuvadzwa kwenguva refu kwemapapu: Kuwanda kwezvirwere zvekufema kunogona kukuvadza tishu remapapu nekufamba kwenguva, zvichiita kuti pave nebronchiectasis (nzira dzemhepo dzakafara zvachose)
• Kurasikirwa kunzwa: Kuwanda kwezvirwere zvekunzwa kunogona kukuvadza zvikamu zvidiki zvekunzwa, zvichikonzera kurasikirwa kunzwa kwese kana kwenguva pfupi
• Kukuvadzwa kwemapfupa: Kuwanda kwehutachiona mumapfupa kunogona kukonzera kukuvara kwenguva refu, kurwadziwa, uye kuderedzwa kwekufamba kana kusarapwa nekukurumidza
• Kukura uye kunetseka kwekuvandudzika: Hutachiona hwakawanda uye kuzvimba kunogona kukanganisa maitiro ekukura evana
• Kusagadzikana: Hutachiona hwakawanda hwekutambudzika kunogona kukuvadza ganda remukati, zvichiita kuti zvive zvakaoma kunwa zvinovaka muviri zvakakodzera
• Sepsis: Hutachiona hwakakomba hunogona dzimwe nguva kupararira muropa, zvichiita kuti pave nematambudziko anotyisa hupenyu

Matambudziko asingawanzoitika asi ane ngozi zvakanyanya anogona kusanganisira:

• Meningitis: Zvirwere zvebhakitiriya zvinogona kusvika kuuropi nemutadzi wechigunwe dzimwe nguva
• Osteomyelitis: Zvirwere zvemapfupa zvinogona kuoma kurapa uye zvinogona kukonzera matambudziko kwenguva refu
• Matambudziko ane chekuita nejekiseni: Jekiseni mhenyu dzinogona kukonzera zvirwere zvakakomba kune vanhu vane XLA

Mashoko akanaka ndeakuti nerurapwo rwakakodzera, kusanganisira kurapwa kwenguva dzose kwe immunoglobulin uye kushandiswa kwakakodzera kweantibiotics, vanhu vakawanda vane XLA vanogona kurarama hupenyu hunowanzoita hwakajairika nekuwedzera kuderedzwa kwe njodzi ye aya matambudziko. Kuongororwa pakutanga nekutarisirwa kwemishonga kunogadzira musiyano mukuru mukudzivirira aya migumisiro yakakomba.

X-linked agammaglobulinemia inogona kudziviswa sei?

Sezvo XLA iri mamiriro ezvemagariro, haugone kudzivirira mamiriro acho pachawo kubva kuitika. Zvisinei, kune matanho akakosha mhuri dzinogona kutora kuti dzizive njodzi pakutanga uye kudzivirira akawanda ematambudziko akakomba ane chekuita neXLA.

Kune mhuri dzine nhoroondo inozivikanwa yeXLA, mazano ezvemagariro anogona kuva anokosha kwazvo. Munyori wemagariro anogona kukubatsira kunzwisisa nzira yekuwedzera, kukurukura sarudzo dzekuyedza, uye kuongorora sarudzo dzekurapa mhuri. Kuongororwa kwepamuviri kuripo kune mhuri dzinoziva kuti dzinakura BTK gene mutation.

Kana XLA yaongororwa, kudzivirira kunotarisa pakudzivirira zvirwere nematambudziko azvo:

• Kurapa kwenguva dzose ne immunoglobulin: Iri ndiro danho guru rekudzivirira, richinopfumisa muviri nema antibodies aunokwanisa kuita.
• Kudzivisa mishonga inorarama: Mishonga inorarama inogona kukonzera zvirwere zvakakomba kune vanhu vane XLA
• Maitiro akanaka ekutsvaira: Kuwachisa maoko nguva dzose uye kudzivisa nzvimbo dzakazara vanhu panguva yekupararira kwezvirwere.
• Kurapwa nekukurumidza kwezvirwere: Kurapwa nekukurumidza neantibiotic kunogona kudzivirira zvirwere zvidiki kuti zvisaite zvakakomba.
• Kuongororwa kwenguva dzose nevanachiremba: Kuongororwa nguva dzose kunobatsira kubata matambudziko pakutanga.

Kudzivirira kunorevawo kuva nechido cheutano hwako. Rambai muchiita misangano yenguva dzose, chengetai kutaurirana kwakanaka nechikwata chenyu chekurapa, uye musanetseka kutsvaka kurapwa kana chimwe chinhu chisingaiti.

X-linked agammaglobulinemia inoongororwa sei?

Kuongorora XLA kazhinji kunosanganisira matanho akawanda, kutanga nekutanga kuziva maitiro ezvirwere zvakakomba zvebhakitiriya. Chiremba wenyu angangotanga ne nhoroondo yakazara yezvokurapa nekuongororwa kwemuviri, achinyanya kutarisa nhoroondo yenyu yezvirwere nemhuri yenyu.

Maitiro ekuongorora kazhinji anosanganisira bvunzo idzi dzakakosha:

• mazinga eImmunoglobulin: Bvunzo yeropa inoyerera mazinga ako eIgG, IgA, uye IgM antibodies, izvo zvinowanzova zvishoma kana kusavapo muXLA
• Kuverenga kweB-cell: Bvunzo ye flow cytometry inotarisa huwandu hweB-cells dziri muropa rako, kazhinji inowana shoma kana kusavapo.
• bvunzo yekupindura kwe antibody: Chiremba wenyu angangotarisa kana iwe unogona kuita antibodies kumishonga yawakagamuchira.
• bvunzo yemajini: Kuongorora jini reBTK kunosimbisa chirwere uye kunoratidza shanduko chaiyo yemajini.

Dzimwe nguva bvunzo dzimwe dzinopa rubatsiro mukudzivisa mamiriro ezvinhu kana kuongorora matambudziko:

• Kuongorora ropa rose kuti uone nhamba yemasero emuviri anorwisa zvirwere
• Kuongorora zvirwere zvakati wandei zvingave zviripo
• Kuongorora mifananidzo senge X-ray yemapapu kana CT scan kuti uone kukuvara kwemapapu
• Kuongorora kunzwa kana paine nhoroondo yekutapukirwa kwemichina kakawanda

Maitiro ekurapa anogona kutora nguva yakareba, kunyanya kana XLA isingafungidzirwi pakarepo. Vanhu vazhinji vanowana chirwere chavo mushure mekuona nyanzvi dzakawanda kana mushure mekupinda muchipatara kakawanda nekuda kwezvirwere. Izvi zvakajairika, sezvo XLA iri shoma uye pakutanga inogona kufungidzirwa kunge imimwe mamiriro ezvinhu.

Kuwana kurapwa kwakakwana kwakakosha nekuti kuchinja maitiro ekudzivirira uye kurapa zvirwere. Kana wawanikwa uine chirwere ichi, timu yako yehutano inogona kugadzira chirongwa chekurapa chakakwana chakagadzirirwa zvaunoda.

Kurapwa kweX-linked agammaglobulinemia ndechii?

Kurapwa kukuru kweXLA ndiko kushandiswa kwe immunoglobulin replacement therapy, iyo inopa muviri wako maantibody ausingakwanisi kuzvigadzira. Kurapwa uku kwachinja maonero evanhu vane XLA uye kunobvumira vanhu vazhinji kurarama hupenyu hunowanzova hwakanaka, hune hutano.

Immunoglobulin replacement therapy inosanganisira kuiswa kwenguva dzose kwemaantibody akaunganidzwa kubva kune vanopa ropa vane hutano. Unogona kugamuchira kurapwa uku nenzira mbiri:

• Intravenous immunoglobulin (IVIG): Inopihwa kuburikidza neIV mazuva ese 3-4, kazhinji muchipatara kana pakati pekuiswa kwemishonga
• Subcutaneous immunoglobulin (SCIG): Inoiswa pasi peganda vhiki nevhiki kana mazuva maviri pavhiki, kazhinji inoitirwa kumba mushure mekudzidziswa kwakakwana

Chiremba wako acashanda newe kuti aone kuti nzira ipi inoshanda zvakanaka kune mararamiro ako nezvinodiwa nehutano hwako. Zvose zvinobudirira, asi vamwe vanhu vanofarira nyore yekurapa kumba neSCIG.

Kunze kwe immunoglobulin therapy, kurapwa kunosanganisirawo:

• Kurapa neantibayotiki: Kurapa nokukurumidza kwehutachiona hwemabhakitiriya, dzimwe nguva nemakore akafamba kupfuura kazhinji
• Antibayotiki dzekuvimba: Vamwe vanhu vanobatsirwa neantibayotiki dzezuva nezuva kudzivirira hutachiona
• Kudzivisa majekiseni ehupenyu: Aya anogona kukonzera hutachiona hwakakomba kune vanhu vane XLA
• Kutarisa nguva dzose: Kuongororwa kweropa nguva dzose uye kuongororwa kuti uone kushanda kwemishonga
• Kutsigira kurapa: Kurapa matambudziko chaiwo senge kurasikirwa kunzwa kana matambudziko emuviri

Chinangwa chekurapa ndechekudzivirira hutachiona uye kuchengetedza mazinga eimmunoglobulin akajairika mumuviri wako. Vanhu vazhinji vanoona kudzikira kwakanyanya muhuwandu nehukomba hwehutachiona kana vangotanga kurapwa kwenguva dzose.

Kurapa kazhinji kwehupenyu hwese, asi vanhu vazhinji vanoshanduka zvakanaka kune zvirongwa uye vanoona kuti zvinova chikamu chinogona kutarisirwa chechirongwa chavo chehutano. Timu yako yezvokurapa ichagadzirisa zvirongwa zvako zvekurapa zvichienderana nekupindura kwako uye chero shanduko muhutano hwako.

Maitiro ekutarisira X-linked agammaglobulinemia pamba?

Kutarisira XLA pamba kunosanganisira kugadzira chirongwa chinotsigira sisitimu yako yekudzivirira uye chinobatsira kudzivirira hutachiona. Chinhu chakakosha chaunogona kuita ndechekuchengetedza kuenderera mberi nekurapa kwako kwakanyorwa uye kuramba uine hukama hwepedyo nechikwata chako chehutano.

Mazano ekutarisira zuva nezuva anosanganisira:

• Kuchenesa kwakanaka: Geza maoko ako kazhinji neshamwari nemvura, kunyanya usati wadya uye pashure pekunge uri munzvimbo dzevanhu vakawanda
• Kuvhiya maronda: Chenesa zvikakuvadza uye zvikwambo nekukurumidza uye tarisa zviratidzo zvehutachiona senge kuwedzera kutsvukira, kupisa, kana puru
• Kutevedzera mishonga: Tora mishonga yese yakanyorwa sezvakarayirwa, kusanganisirawo mamwe marapirwo ekudzivirira hutachiona
• Kutarisa tembiricha: Tarisa tembiricha yako kana uchinzwa kusagadzikana, sezvo fivha kazhinji ichiratidza kudiwa kwekuchengetedzwa kwehutano
• Kunwa mvura zvakakwana nekudya kwakanaka: Chengeta hutano hwakanaka hwakazara nekudya kwakanaka nekudya kwakakwana kwemvura

Zvakakoshawo kuziva nguva yekutsvaka kurapwa kwehutano. Taura nachiremba wako kana ukawana fivha, chikosoro chinoramba chichiitika, kuneta kwakanyanya, kana chero zviratidzo zvinoita sekunetsa. Usamirire kuona kana zviratidzo zvichavandudza pachazvo, sezvo kurapwa kwekukurumidza kazhinji kunonyanya kushanda.

Kana uri kugamuchira immunoglobulin subcutaneous pamba, chengeta rekodhi yakadzama yezvinwiwa zvako, kusanganisira mazuva, mazai, uye chero zvinokonzeresa. Ruzivo urwu runobatsira timu yako yehutano kuti iite zvirinani kurapwa kwako.

Funga kupfeka bhandi rechiratidzo chekurapa kana kutakura kadhi rinoratidza mamiriro ako. Izvi zvinogona kukosha kana uchida kuchengetedzwa kwehutano hwekukurumidza uye haugone kutaura nhoroondo yako yehutano.

Ungagadzirira sei kuenda kunachiremba?

Kuagadzirira misangano yako yekurapa kunogona kubatsira kuve nechokwadi chekuti unowana zvakanyanya kubva panguva yako nechikwata chako chehutano. Kuunza ruzivo rwakarongeka nezviratidzo zvako nemibvunzo kunobatsira chiremba wako kupa hanya yakanakisa inobvira.

Usati wasangana nachiremba,unganidza ruzivo urwu rwakakosha:

• Nhoroondo yehutachiona: Nyora mashoko akakwana pamusoro pehutachiona hwapera nguva pfupi yadarika, kusanganisira mazuva, zviratidzo, kurapwa, uye kwenguva yakareba sei kwakagara.
• Ruzivo rwemishonga: Uyai neruzivo rwakakwana rwemishonga yose, zvinowedzerwa, nekurapwa kuri kushandiswa iye zvino.
• Zvinyorwa zve kurapwa: Kana muri kugamuchira kurapwa kwe immunoglobulin, uyai nezvinyorwa zve kurapwa kwapfuura uye migumisiro ipi neipi.
• Nhoroondo yemhuri: Gadzirirai ruzivo pamusoro pe nhengo dzemhuri dzine matambudziko ekudzivirira muviri kana hutachiona hwakawanda.
• Mibvunzo nezvinetso: Nyora mibvunzo chaiyo yaunoda kubvunza panguva yekushanya kwako.

Funga kuuya nemumwe wemhuri kana shamwari pakuchengeta kwako, kunyanya pakushanya kukuru sekutanga kuonana nachiremba kana mapurogiramu ekurapa. Vanogona kukubatsira kurangarira ruzivo rwakakurukurwa panguva yekushanya uye kupa rutsigiro rwepfungwa.

Usanetseka kubvunza timu yako yekutarisira utano kuti ijekese chero chinhu chisinganzwisisiki. Mutauro wekurapa unogona kuva unonetsa, uye zvakakosha kuti unzwe wakasununguka nerongwa rako rekurapa. Bvunza nezvemigumisiro inogona kuitika, zvaunofanira kutarisira kubva pakurapwa, uye nguva yekubata timu yekurapa nezvinetso.

Kana muri kuona chiremba mutsva, bvunza nezve ruzivo rwavo pakurapa XLA kana mamwe matambudziko ekudzivirira muviri. Kunyange XLA iri shoma, unofanirwa kutarisirwa nevanopa utano vanonzwisisa mamiriro ako uye vanogona kubatana zvakanaka nevanachiremba vezvikamu.

Chii chinonyanya kukosha pamusoro pe X-linked agammaglobulinemia?

Chinhu chinonyanya kukosha kunzwisisa pamusoro peXLA ndechekuti kunyange iri mamiriro akakomba anoda kutarisirwa kwenguva refu, vanhu vane XLA vanogona kurarama hupenyu hwazvo hwese, hwakanangana nekurapwa kwakakodzera. Kuongororwa pakutanga nekutarisirwa kwenguva dzose kwechiremba kunoita musiyano mukuru mukudzivirira matambudziko uye kuchengetedza hutano hwakanaka.

Kurapa kwenguva dzose kwemasero e immunoglobulin kunobudirira kwazvo mukudzivirira hutachiona hwakawanda, hwakakomba hunoratidza XLA isina kurapwa. Vanhu vakawanda vanoona kuvandudzika kukuru muhuwandu hwehutachiona hwavo uye hutano hwavo hwese pavano tanga kurapwa kwakakodzera.

Yeuka kuti XLA inokanganisa munhu wese zvakasiyana, uye chirongwa chako chekurapa chinofanira kugadzirirwa zvinodiwa zvako uye mararamiro ako. Shanda pedyo nechikwata chako chehutano kuti uwane nzira yekurapa inokushandira zvakanaka, ingave kurapwa kwe-IV muhospital kana kurapwa kwe-subcutaneous kumba.

Ramba uchibatana nehutano hwako nekutevera zviratidzo zvako, kuchengetedza kutaurirana kwakanaka nechikwata chako chekurapa, uye usamborega kutsvaka rubatsiro kana uchida. Nekutonga kwakakodzera, XLA haifanire kudzora kugona kwako kushanda, kufamba, kurovedza muviri, kana kunakidzwa nezviitiko zveupenyu.

Mibvunzo inobvunzwa kazhinji nezve X-linked agammaglobulinemia

Vanhu vane XLA vanogona kurarama hupenyu hwakajairwa here?

Hungu, nekurapa kwakakodzera, vanhu vane XLA vanogona kuva nehuwandu hweupenyu hwakaenzana nehwakajairwa. Kurapa kwenguva dzose kwemasero e immunoglobulin nekutarisirwa kwakakodzera kwechiremba kwakavandudza zvikuru mhedzisiro. Kunyange XLA ichida kutarisirwa kwenguva dzose, haina kukurumidza kupfupisa hupenyu kana yakarapwa zvakanaka.

XLA inotapuriranwa here?

Kwete, XLA haitapuriranwi zvachose. Iyo mamiriro ezvekurapa aunazvo kubva pakuzvarwa, kwete chimwe chinhu chaunogona kubata kana kuparadzira kune vamwe vanhu. Zvisinei, vanhu vane XLA vanogona kuva vanokurumidza kubata hutachiona kubva kune vamwe nekuda kwemamiriro avo ekudzivirira muviri asina simba.

Vakadzi vanogona kuva vatakuri veXLA vasingazvizivi here?

Hungu, vakadzi vanogona kuva vatakuri veBTK gene mutation pasina kuva nezviratidzo ivo pachavo. Vakadzi vatakuri vane kopi imwe yakajairwa nekopi imwe yakanganisika yegene, asi kopi yavo yakajairwa kazhinji inopa basa rakakwana rekudzivirira muviri kwakanaka. Kuongororwa kwemajini kunogona kuona mamiriro evatakuri.

Chii chinoitika kana munhu ane XLA akawana COVID-19 kana mamwe mahutachiona ehutachiona?

Vanhu vane XLA vanowanzogona kurwisa hutachiona hwehutachiona zvakanaka nekuti masero avo e-T nemamwe mativi emuviri wavo anoshanda zvakanaka. Zvisinei, vanofanira kutora matanho ekudzivirira panguva yekupararira kwehutachiona uye kukurukura nezvekudzivirirwa nechikwata chavo chehutano, sezvo mamwe majekiseni angave asina kukwana.

Vana vane XLA vanogona kupinda chikoro zvakajairika here?

Vana vakawanda vane XLA vanogona kupinda chikoro nguva dzose kana kurapwa kwavo kwakaringana. Zvisinei, vangada kudzivisa zvimwe zviitiko senge kusangana nevaunodzidza navo vane hutachiona, uye havafaniri kupihwa majekiseni ehupenyu anowanzodiwa kuti vapinde chikoro. Kubatana nevanamukoti vechikoro nevatungamiriri kunobatsira kuve nechivanze chakachengeteka.