Mwana wenyu mudiki ane zviratidzo izvi here? Ngatikurukurei nezveWiskott-Aldrich Syndrome.

Mwana wako mudiki anogara achirwara here? Dzimwe nguva ane matambudziko eganda akadai se eczema, anobuda ropa rakawanda here kunyangwe nekukuvara kudiki, kana kuti anogara achikuvara kwese kwese? Kunyangwe izvi zvingaita sezvakajairika, dzimwe nguva panogona kunge paine chirwere chemajini chisingawanzoitika mushure meizvi chatisati tanzwa zvakawanda nezvacho. Chimwe chezvirwere zvakadaro iWiskott-Aldrich Syndrome. Ngatitaurei nezvazvo zvakadzama nhasi.

Chii chinonzi chirwere ichi cheWiskott-Aldrich?

Zvichitaurwa zviri nyore, ichi chirwere chisingawanzoitiki chemajini . Chinonyanya kukanganisa masoja emuviri wemwana wako uye mashandiro emamwe masero muropa. Izvi zvinogona kukonzera zviratidzo zvakawanda panguva imwe chete. Izvi zvinosanganisira:

• Eczema: Chirwere cheganda chinokonzera ganda rakaoma uye rinokwenya.
• Kushaikwa kwedziviriro yemuviri: Masero machena eropa anorwisa zvirwere mumuviri haashande nemazvo.
• Kubuda ropa uye mavanga: Kunyangwe kubatwa kudiki kunogona kukonzera kubuda ropa, uye mavanga anogona kuitika munzvimbo dzakasiyana nekuda kwekuoma kwekugwamba kweropa.

Dambudziko iri dzimwe nguva rinogona kukonzera matambudziko anotyisidzira hupenyu hwako. Rinogonawo kupfupisa hupenyu hwako. Zvisinei, nekurapwa kuri kuitwa, njodzi idzi dzinogona kuderedzwa zvakanyanya .

Dambudziko iri rakajairika zvakadii?

Izvi hazviwanzoitiki . Maererano nehuwandu, zvinofungidzirwa kuti vatatu chete pamamiriyoni evakomana vanobatwa neWiskott-Aldrich Syndrome. Kunyangwe munyika yakaita seAmerica, kune vanhu vasingasviki zviuru zvishanu vane chirwere ichi. Chinonyanya kubata vakomana , uye kashoma kuti vasikana vazvibate.

Chii chinonzi chirwere chinokonzerwa neWAS?

Chiremba wako anogonawo kutaura nezveWiskott-Aldrich Syndrome sechirwere chine chekuita neWAS. Izvi zvinoreva mamiriro ezvinhu anokanganisa masoja emuviri nekuda kwekuchinja kwemajini eWAS. Semuenzaniso, X-linked thrombocytopenia chirwere chine chekuita neWAS.

Zvisinei, chirwere chinonzi ``congenital neutropenia'' chinokonzerwa nekuchinja kwemajini kwakasiyana, kusina chekuita nejini rekuti ``WAS''. Vanachiremba vanongoziva chirwere ichi chete kana mwana awana hutachiona hwakakomba hwebhakitiriya.

Ndezvipi zviratidzo zveWiskott-Aldrich Syndrome?

Tatotaura kuti pane zviratidzo zvikuru zvitatu zvechirwere ichi. Ngatizvitarisei zvakadzama .

• Eczema: Izvi zvinoita kuti ganda riome zvakanyanya uye rikwenye., dzimwe nguva inogona kutsvuka uye kupwanyika. Zvakafanana ne eczema inosangana nevamwe vadiki, asi izvi zvinogona kunge zvakanyanya kuipa.

• Kushaikwa kwedziviriro yemuviri: Dambudziko iri rinoitika kana masero machena eropa anobatsira kuchengetedza miviri yedu ine hutano asingashande zvakanaka. Izvi zvinoderedza kugona kwemuviri kurwisa zvirwere . Dzimwe nguva masoja emuviri anogona kutotanga kurwisa muviri wawo. Izvi zvinogona kukonzera kutapukirwa kakawanda, uye zvirwere zvakaita serheumatoid arthritis, vasculitis (kuzvimba kwetsinga dzeropa), anemia (kuderera kweropa), leukemia (rudzi rwekenza yeropa), kana lymphoma (rudzi rwekenza yema lymph nodes).

• Matambudziko ekubuda ropa (Microthrombocytopenia): Apa ndipo apo huwandu hwemaplatelet, anobatsira ropa kuti rigwambe, hunoderera kana kuti huve hudiki kwazvo . Kutaura zvazviri, aya ndiwo masero anobatsira kumisa kubuda ropa. Saka kana aya ari mashoma, kunyangwe kucheka kudiki kunogona kukonzera kubuda ropa kusingamire. Izvi zvinogona kukonzera mavanga , kubuda ropa mumhino, dzimwe nguva manyoka ane ropa, kubuda ropa pasi peganda (purpura), uye madonhwe madiki matsvuku (petechiae) paganda.

Usatya chirwere ichi nekuti une chimwe kana zviviri zvezviratidzo izvi. Asi kana zvinhu izvi zvikaramba zviripo, zvakanaka kuti uone chiremba.

Vacheche vane chirwere cheWiskott-Aldrich chakanyanya kuoma (kusashanda zvakanaka kwemuviri) vanogonawo kuratidza zviratidzo zvakaita se:

• Eczema
• Kushomeka kwedziviriro yemuviri
• Thrush yakanyanya
• Chirwere chemabayo

Dzimwe nguva, kana paine neutropenia yekuzvarwa nayo inokonzerwa negene reWAS rinowedzera kushanda, hutachiona hwakakomba hwebhakitiriya kana myelodysplastic syndrome (chirwere chemubofu) hunogona kuitika.

Chikonzero cheizvi chii?

Chikonzero chikuru cheWiskott-Aldrich Syndrome kuchinja kwemajini, kana kuti mutation, muWAS gene . Iri gene reWAS riri paruoko rupfupi rwe X chromosome yedu. Iri gene rinogadzira protein yeWiskott-Aldrich syndrome. Iri puroteni riri mumasero edu ese eropa.

Iyi puroteni yeWiskott-Aldrich syndrome inobatsira masero edu kunamira kune mamwe masero nematishu kuburikidza ne "kunamatira." Kunamatira uku kwakakosha zvikuru nekuti kunobatsira masoja edu emuviri kukunda vavengi vakaita semabhakitiriya nemavhairasi anopinda mumuviri.

Saka, kana uine gene rakachinja mujini rako re'WAS', masero ako eropa haazokwanisi kunamira kune mamwe masero nematishu zvakanaka. Izvi zvinodzivirira masoja emuviri kuti asaite basa rawo nemazvo. Ndizvo zvinokonzera zviratidzo zveWiskott-Aldrich Syndrome.

Mukuzvarwa neutropenia, kushanduka kwemajini kunomisa kufamba kwemarudzi maviri emasero, ma neutrophils nema monocytes, zvichidzivirira masoja emuviri kuti asaburitse masero aya kuti arwe nehutachiona.

Izvi zvinobva kuzvizvarwa nezvizvarwa here?

Ehe, ichi chirwere chinogona kugarwa nhaka. Chinogarwa nhaka nenzira ye "X-linked recessive". Izvi zvinoreva kuti mwana anofanira kugamuchira shanduko yemajini kubva kuvabereki vese.

Tinogara nhaka machromosome edu ebonde kubva kuvabereki vedu. Vanhurume vane chromosome imwe chete ye'X' uye imwe ye'Y'. Vanhukadzi vane machromosome maviri e'X'. Chirwere ichi chinokanganisa vakomana zvakanyanya, nekuti kuchinja kwemajini uku kunokanganisa mashandiro echromosome yavo yega ye'X'.

Kunyangwe chirwere ichi chiine maitiro emhuri, vanopfuura 30% yevarwere vese vanochiwana ``de novo'' , zvichireva kuti chinokonzerwa nekuchinja kwemajini kunoitika panguva yekubata pamuviri.

Unozviziva sei izvi?

Chiremba anowanzo ongorora chirwere cheWiskott-Aldrich Syndrome achiri mudiki kana kuti achiri mudiki . Mwana anoongororwa uye bvunzo dzinodiwa dzinoitwa. Zviratidzo zvekutanga zvechirwere ichi zvinogona kunge ropa mutsvina, kubuda ropa kusingawanzoitiki, kana mavanga. Chiremba anogona kuita bvunzo dzinotevera kuti asimbise chirwere ichi:

• Kuverengwa kweropa rose (CBC)
• Kuongororwa kweropa remajini
• Kuongororwa kweropa rekunze

Kana izvi zvikasaonekwa muhucheche, zviratidzo zvinova pachena zvakanyanya muhudiki.

Kana mwana wako akaratidza zviratidzo zvekushaya simba kwemuviri wake, zvakaita sekutapukirwa nezvirwere nguva nenguva, bvunzo dzekuwedzera dzinogona kudiwa pahudiki. Izvi zvinokonzerwa nekuti muviri wemwana ungasakwanisa kurwisa mabhakitiriya nemavhairasi zvakanaka, uye ungasagamuchira zvakanaka mamwe majekiseni.

Chiremba anogona kuita bvunzo yeropa kuti aone kana muviri wemwana wako uri kugadzira masoja emuviri mushure mekubayiwa jekiseni. Masoja aya ndiwo anogadzira mhinduro yemasoja emuviri kudzivirira zvirwere zvakakomba. Pamusoro pezvo, kumwe kuongororwa kweropa kuchaitwa kuti aongorore masero machena eropa remwana wako, kunyanya masero eT ne immunoglobulins (anobatsirawo kugadzira masoja emuviri).

Ndezvipi zvinorapwa izvi?

Wiskott-Aldrich Syndrome inogona kurapwa nenzira dzinotevera:

• Rapa hutachionaMishonga inorwisa mabhakitiriya kana mishonga inorwisa mavhairasi.
• Mishonga yekudzivirira muviri (immunoglobulin) inopihwa kuti idzorere ma antibodies anenge apera mumuviri.
• Kuwedzerwa ropa-platelet yematambudziko ekubuda ropa.
• Eczema inogona kurapwa nemishonga inoiswa pamusoro uye mafuta anogadzirwa nemaoko (OTC) anonyorovesa .

Kana mwana wako akabatwa nechirwere kana hutachiona, zvinogona kuva zvakakomba uye zvinotoisa upenyu panjodzi . Kuti udzivirire hupenyu hwemwana wako, unogona zvakare kuedza mishonga iyi:

• Kuisirwa masero emuviri : Iyi ingave ndiyo mhinduro yakanakisisa iripo parizvino.
• Kurapa majini (izvi zvichiri muchikamu chekutsvagisa).

Chiremba wemwana wenyu achakurukura nemi nzira idzi dzekurapa uye acharonga kurapwa kuti awane mhedzisiro yakanakisa kumwana wenyu uye kuvandudza hupenyu hwake.

Kana mwana wangu aine chirwere ichi, chii chandinofanira kutarisira?

Kana mwana wako aine Wiskott-Aldrich Syndrome, chiremba wako achagadzira hurongwa hwekurapa hunobatsira kudzivirira matambudziko anogona kutyisidzira hupenyu hwake nekuti masoja emuviri wake haashande zvakanaka. Mushure mekuongororwa, unogona kutumirwa kunorapwa nemajini . Izvi zvinogona kukubatsira iwe nemhuri yako kudzidza zvakawanda nezvechirwere ichi uye nzira dzekurapa dziripo.

Kunyangwe zvirwere zvinowanzoitika kuvana zvakaita sechickenpox zvinogona kukonzera matambudziko makuru kuhutano hwemwana wako. Nekuti masoja ake ekudzivirira chirwere haana kusimba sevamwe vana vezera rake, angangoda kuona chiremba nekukasika. Kurapa zvirwere nehutachiona nekukurumidza kunogona kukubatsira kuwana mhedzisiro iri nani.

Chiremba wako anogona kukuudza kuti usape mwana wako majekiseni ehutachiona mhenyu (akadai sejekiseni refuruu) nekuti rudzi rwehutachiona mhenyu runogona kurwarisa mwana wako. Kunyangwe mwana wako akawana mamwe majekiseni, anogona kusashanda zvakanaka. Kana mwana wako akarwara nehutachiona, kurapwa nekukurumidza nemishonga inorwisa mavhairasi kunowanzo shanda zvakanaka. Asi kunyange zvirwere zvakajairika zvinogona kukonzera matambudziko.

Mwana wenyu angangoda kuongororwa kenza nguva nenguva muhupenyu hwake hwese nekuti ari panjodzi yakakura yekuva nerudzi rwekenza, kunyanya leukemia kana lymphoma .

Pane mushonga wakakwana weizvi here?

Ehe, kuisirwa stem cell (inonziwo bone marrow transplant) kunogona kurapa Wiskott-Aldrich Syndrome.Mhando idzi dzema "stem cells" dzinobvisa masero emuviri anogadzira ropa uye dzinoatsiva nemasero e "stem cells" ane hutano. Nekuti Wiskott-Aldrich Syndrome inokonzera kuti masero eropa asawanzogadzirwa, "stem cell transplant" inogona kutsiva masero iwayo nemasero ane hutano, anoshanda.

Wiskott-Aldrich Syndrome inouraya here?

Chirwere cheWiskott-Aldrich chinogona kuuraya . Zvakambotaurwa kuti vana vasina kuisirwa stem cell vane makore angangoita gumi nemashanu ekurarama. Zviratidzo zvinokonzerwa nehutachiona, kubuda ropa muuropi hwemwana, hutachiona hwakanyanya kana kenza zvinogona kutyisidzira hupenyu, uye rufu runogona kuitika nekukurumidza.

Zvisinei, nekufambira mberi kwesainzi yezvekurapa, nzira dziri kushandiswa pari zvino dzekurapa dzakaita kuti zvikwanisike kuvandudza hupenyu hwemwana.

Izvi zvinogona kudzivirirwa here?

Ichi chirwere chemajini uye hachigone kudzivirirwa . Kana uchironga kuva nevana uye uchida kuziva njodzi yako yekuva nemwana ane chirwere chemajini, taura nachiremba wako nezvekuongororwa kwemajini .

Ndinofanira kuona chiremba wemwana wangu nguvai?

Kana mwana wako achirwara uye akaonekwa kuti ane Wiskott-Aldrich Syndrome, taura nachiremba wake nekukasika . Nekuti masoja avo ekudzivirira chirwere haasi kushanda zvakanaka, vanogona kuwana hutachiona kakawanda. Chiremba wako anogona kurapa chirwere kana hutachiona, kana kudzivirira matambudziko anotyisidzira hupenyu.

Ona chiremba wako kana mwana wako aine chimwe chezvinotevera:

• Ropa mutsvina (Manyoka ane ropa)
• Kubuda ropa mumhino kakawanda
• Nzvimbo huru dzemavanga
• Kuchinja kweganda ravo
• Kutapurirana kunoitika nguva nenguva (semuenzaniso chickenpox kana thrush, kutapurirana kwemabhakitiriya)

Mibvunzo ipi yandinofanira kubvunza chiremba?

Unogona kubvunza chiremba mibvunzo yakaita seiyi:

• Mwana wangu anotarisirwa kurarama kwenguva yakareba sei?
• Ndezvipi zvigadzirwa zvandinogona kushandisa kurapa eczema yemwana wangu?
• Pane migumisiro yakaipa pakurapa kwamunokurudzira here?
• Mwana wangu akakodzera here kuisirwa stem cell?

Ndeupi musiyano uripo pakati peWiskott-Aldrich Syndrome neAtaxia-Telangiectasia?

Wiskott-Aldrich Syndrome uye Ataxia-Telangiectasia zvirwere zvemajini zvinokanganisa mashandiro emuviri wemuviri.Zvisinei, ataxia-telangiectasia inokonzerwa nekuchinja kwemajini eATM. Inokanganisa kufamba kwako uye kubatana kwemasero. Inoitawo kuti mitsipa yeropa iite kunge yakatsveyama paganda rako.

Zvinhu zvinonyanya kukosha zvaunofanira kuyeuka

Kuziva kuti mwana wako ane chirwere chisingawanzoitika chichavakanganisa kwehupenyu hwake hwese kunogona kukuomera. Zvinogona kushamisa. Asi musazvidya mwoyo. Chiremba wemwana wenyu achakuudzai zvakawanda nezvechirwere ichi uye kuti mungabatsira sei mwana wenyu kumba. Masoja emuviri wemwana wenyu anogona kunge asiri kushanda zvakanaka, saka anogona kurwara kakawanda.

Zvakakosha kuzvitarisira iwe pachako paunenge uchitarisira mwana wako. Vabereki vazhinji nemhuri vanowana nyaradzo huru nerutsigiro mukutaura nenyanzvi yezveutano hwepfungwa .

Nekufambira mberi kuri kuitwa pakurapa, vana vane chirwere ichi vava kukwanisa kurarama hupenyu hwakazara uye hunofadza. Saka ramba wakasimba.

Wiskott -Aldrich syndrome, zvirwere zvemajini, masoja ekudzivirira muviri, eczema, kubuda ropa, kuisirwa masero ekutanga, hutano hwemwana