Mwana wako ane zviratidzo izvi here? Zvingave zviri Hunter Syndrome!

Wakaona here kunonoka mukukura kwemwana wako, kana shanduko muchimiro chake kana majoini ake nguva pfupi yadarika? Dzimwe nguva, chiri kukonzera zvinhu izvozvo chingava chirwere chisingawanzoitika chatisati tanzwa zvakawanda nezvacho. Nhasi tichataura nezvechirwere chemajini chinonzi Hunter Syndrome. Usatye paunonzwa izvi, nekuti chinhu chakakosha ndechekuzviziva.

Chii chinonzi Hunter Syndrome? Ngatinzwisise zviri nyore!

Zvichitaurwa zviri nyore, Hunter Syndrome chirwere chisingawanzoitika mumajini. Ichi chirwere apo mamwe mamorekuru akaomarara mumuviri wemwana wako (anonzi ``Glycosaminoglycans'' kana ``GAGs'') haaparadzwe zvakanaka uye haagayiwe. Kungofanana nemarara mudzimba dzedu anoungana kana tikasaarasa zvakanaka, mamorekuru aya eshuga anoungana mukati memasero emuviri, kunyanya muzvikamu zvinonzi ``Lysosomes''. Nekufamba kwenguva, kuungana uku kunotanga kukuvadza nhengo dzakasiyana-siyana nematishu mumuviri. Kukuvadzwa uku kunogona kukanganisa kukura kwemuviri nepfungwa kwemwana.

Vanachiremba vanoparadzanisa Hunter Syndrome mumhando mbiri huru:

1. Rudzi rwakanyanya: Urwu rudzi rwakanyanya kuoma runokurumidza kukura. Panyaya iyi, hunyanzvi hwemwana hunokanganiswawo. Kazhinji, pakati pemakore matanhatu nemasere, mwana anotanga kunetseka kuita mabasa ezuva nezuva. Vanhu vanosvika 60% vane Hunter Syndrome vane rudzi urwu rwakanyanya kuoma.

2. Rudzi rusina kunyanya kuoma: Zviratidzo zvinouya zvishoma nezvishoma. Hunhu hwepfungwa hungasakanganiswa zvakanyanya.

Hunter Syndrome iboka guru rezvirwere zvinonzi ``Mucopolysaccharidoses``. Saka, inonziwo ``Mucopolysaccharidosis type II`` kana ``MPS II``.

Hunter Syndrome yakajairika zvakadii?

Ichi chirwere chisingawanzoitiki. Zvakare, chinonyanya kubata vakomana. Maererano nenhamba, munhu mumwe chete pavakomana 100,000 kusvika 170,000 ndiye anobatwa nechirwere ichi. Zvisinei, vasikana vanogona kutakura majini anochinja anokonzera chirwere ichi. Izvi zvinoreva kuti kunyangwe vasina zviratidzo, vanogona kupa majini kuvana vavo.

Ndezvipi zviratidzo zvemwana ane Hunter Syndrome?

Zviratidzo izvi zvinowanzotanga kuonekwa mumwana ari pakati pemakore maviri nemana. Zviratidzo zvinogona kusiyana kubva pamunhu kuenda kune mumwe, uye zvinogonawo kusiyana muhukuru. Ngatitarisei zviratidzo zvikuru zvinogona kuonekwa:

• Kuomarara kwemajoini, kuoma kukotama: Zvinogona kuita sekunge majoini "akabatwa".
• Kukora kwechiso: Nzvimbo dzakadai semhino, miromo, nerurimi dzinogona kukobvu uye kuita sedzakaomarara.
• Kunonoka kubuda kwemazino kana kuva nemikana mikuru pakati pemazino.
• Musoro wakakura kupfuura zvakajairika, chipfuva chakafara, uye mutsipa mupfupi.
• Kurasikirwa nekunzwa (kurasikirwa nekunzwa) kunowedzera zvishoma nezvishoma nekufamba kwenguva.
• Kunonoka kukura: Kureba kunogona kudzikira, kunyanya mushure memakore mashanu.
• Chiropa nechiropa zvakakura.
• Kuonekwa kwemapundu machena paganda.

Zvakanakisa kusatya kana ukaona chimwe kana zviviri zvezviratidzo izvi, asi kana mwana wako akaramba aine zviratidzo zvinopfuura chimwe chete, kuchenjera kutsvaga rubatsiro rwechiremba.

Sei Hunter Syndrome ichiitika? Chii chinokonzera?

Chikonzero chikuru cheizvi ikushanduka kwemajini mujini re'IDS'. Iri jini re'IDS' rinodzora kugadzirwa kweenzyme inonzi `(Iduronate 2-sulfatase)` kana `(I2S)` mumuviri wedu. Basa reiyi enzyme ye`(I2S)` nderekuputsa mamorekuru eshuga akaomarara anonzi `(Glycosaminoglycans)` kana `(GAGs)` atakataura nezvawo kare.

Saka, mumunhu ane chirwere cheHunter syndrome `(MPS II)`, iyi enzyme `(I2S)` haigadzirwe mumuviri, kana kuti inogadzirwa zvishoma. Nekuti iyi enzyme haipo, mamorekuru eshuga iwayo `(GAGs)` anoungana muzvikamu zvinonzi `(Lysosomes)` mukati memasero. `(Lysosomes)` inzvimbo dziri mukati memasero dzinopwanya uye dzinodzokorodza mamorekuru asina basa. Nekuti `(GAGs)` anoungana nenzira iyi, chirwere che`(MPS II)` ndecheboka rezvirwere zvinonzi `(Lysosomal storage disorder)`. Imhaka yekuunganidzwa uku kunoita kuti nhengo dzakasiyana-siyana dzemuviri dzikuvadzwe.

Ndiani ari panjodzi huru yekuzviwana?

Kana mumwe munhu mumhuri, kureva kuti, ane hukama hwemuviri, aine chirwere ichi, njodzi yekuti vamwe vatambure nacho yakakura.

Sezvatakambotaura, vakomana vanowanzogara nhaka chirwere ichi. Izvi zvinokonzerwa nekuti chirwere ichi chakabatana nechromosome X. Unoziva, vasikana vanogara nhaka machromosome maviri eX, vakomana vanogara nhaka imwe yeX chromosome uye imwe yeY chromosome. Saka, kunyangwe musikana akagara nhaka X chromosome negene iri risina kunaka, imwe X chromosome ine hutano inogona kupa enzyme inodiwa. Saka vanogona kusaratidza zviratidzo uye kuva mutakuri. Asi kana mukomana akagara nhaka X chromosome negene iroro risina kunaka, acharwara nechirwere ichi nekuti haana imwe X chromosome.

Ndezvipi zvinetso zvinogona kuitika zveHunter Syndrome?

Zvichienderana nekuoma kwechirwere ichi, matambudziko akasiyana-siyana anogona kuitika. Vanachiremba vanoshandisa mishonga uye dzimwe nguva kunyange kuvhiyiwa kudzora matambudziko aya. Ngationei kuti matambudziko aya ndeapi:

• Kuoma kwekufema: Kuoma kwekufema kunogona kuitika nekuda kwekukora kwenyama uye kuvharika kwenzira dzekufema.
• Chirwere chemwoyo (`(Chirwere chemwoyo)`).
• Kusarongeka kwemajoini nemapfupa.
• Kudzikira zvishoma nezvishoma kwekushanda kwehuropi.
• Carpal tunnel syndrome (`(Carpal tunnel syndrome)`): Chirwere chinokonzerwa nekumanikidzwa kwetsinga munzvimbo yeruoko.
• Hernias (`(Hernias)`).
• Zvirwere zvakaita sepfari (`(Kubatwa nepfari)`).
• Matambudziko emaitiro.

Matambudziko aya haaitike nenzira imwe chete kune wese munhu. Anogona kusiyana zvichienderana nemamiriro emwana. Saka, zvakakosha kutaura nachiremba nguva dzose uye kuziva mamiriro emwana.

Ungaziva sei kana uine Hunter Syndrome?

Chiremba wemwana wenyu achaita bvunzo dzinoverengeka kuti asimbise chirwere ichi.

• Kuongororwa kweweti: Izvi zvinoongorora huwandu hweshuga hwakanyanya muweti (inonzi maGAG).
• Kuongororwa kweropa: Izvi zvinogona kuona kana basa re enzyme `(I2S)` muropa riri shoma kana kuti risiripo. Ichi chiratidzo chikuru chechirwere.
• Kuongororwa kwemajini: Izvi ndizvo zvinosimbisa kana paine shanduko mune gene chaiyo yeIDS.

Ndeipi mishonga yekurapa Hunter Syndrome?

Hunter Syndrome inorapwa zvichienderana nezviratidzo zvemwana. Izvi zvinoda rutsigiro rwechikwata chenyanzvi. Vanhu vane ruzivo mumabasa akasiyana-siyana vanoshanda pamwe chete kugadzirisa mamiriro emwana. Zvinangwa zvikuru zvekurapa ndezvekunonotsa kufambira mberi kwechirwere, kuona nekurapa matambudziko angangomuka nekuda kwechirwere ichi nekukurumidza, uye kuvandudza hupenyu hwemwana.

Mushonga wakanakisa uripo parizvino wekuzadzisa zvinangwa izvi ndeye enzyme replacement therapy (`(Enzyme replacement therapy)`). Muizvi, enzyme `(I2S)` isipo inotsiviwa ne enzyme yakagadzirwa nevanhu (`(Idursulfase (Elaprase®))`). Kurapa uku kunowanzoitwa netsinga kamwe chete pasvondo.

Pamusoro pezvo, tsvakiridzo pamusoro pekurapa majini (kana kugadzirisa majini) iri kuenderera mberi parizvino. Izvi zvinogona kuunza tariro huru kuvarwere vane Hunter Syndrome mune ramangwana. Zvisinei, mhedzisiro ichiri kumirira.

Pane nzira yekudzivirira izvi here?

Sezvo ichi chiri chirwere chemajini, zvinosuwisa kuti hachigone kudzivirirwa. Zvisinei, zvakakosha kuti vabereki vemwana ane Hunter Syndrome vataure nenyanzvi yezvemajini vasati vaita mumwe mwana. Nyanzvi iyi inogona kubatsira vabereki kunzwisisa njodzi yekupfuudza chirwere ichi kune mumwe mwana.

Ramangwana remunhu ane Hunter Syndrome rakaita sei?

Mushonga wakakwana weizvi hausati wawanikwa.Zvirwere zvakakomba zvechirwere ichi zvinogona kuisa upenyu panjodzi. Avhareji yehupenyu hwevana vakadaro iri pakati pemakore gumi kusvika makumi maviri. Zvisinei, avo vane zvirwere zvishoma vanogona kurarama kwenguva yakareba, kusvika vava vanhu vakuru.

Kune vanhu vazhinji, kurapwa kwakadai semishonga, physiotherapy, uye kuvhiyiwa kunogona kubatsira kugadzirisa matambudziko echirwere uye kuvandudza hupenyu hwavo.

Mwana wangu achakwanisa kushanda zvakanaka zvakare here?

Vana vane Hunter Syndrome vanogona kunetseka nemabasa ezuva nezuva uye kufamba-famba sezvo zviratidzo zvavo zvichiwedzera zvishoma nezvishoma. Mamwe mabasa angada kugadziriswa. Chiremba wemwana wako achataura newe nezvemabasa uye marapirwo anogona kukubatsira kutsungirira zviratidzo.

Inguvai yaunofanira kuona chiremba?

Kana mwana wako akatanga kuratidza zviratidzo zveHunter Syndrome, kana ukaona kunonoka kukura kwemwana, taura nachiremba wemwana wako nekukasika. Kutanga kurapwa nekukurumidza kunogona kubatsira kudzivirira kukuvara kwenhengo dzemuviri nenyama zvachose.

Chii chaunofanira kubvunza chiremba?

Kana wangoziva kuti mwana wako ane Hunter Syndrome, unogona kubvunza chiremba mibvunzo yakaita seiyi:

• Hunter Syndrome yakakomba sei?
• Chii chichaitika kumwana wangu munguva pfupi uye kwenguva refu?
• Chirwere ichi chichakanganisa sei hupenyu hwemwana wangu?
• Ndezvipi zvingasarudzwa pakurapa?

Bvunza mibvunzo iyi uye bvisa kusava nechokwadi kwaungave nako. Nekuti kana uchiziva zvakawanda, unokwanisa kubatsira mwana wako zviri nani.

Musiyano uripo pakati peHunter neHurler syndrome ndeupi?

Hunter Syndrome neHurler Syndrome zvirwere zviviri zviri muboka rezvirwere zvinonzi ``Lysosomal storage disorders'', ``Mucopolysaccharidoses''.

Hurler syndrome ndiyo mhando yakanyanya kuipa yechirwere cheMucopolysaccharidosis type I (MPS I). MuMPS I, enzyme alpha-L-iduronidase haipo. Hurler syndrome yakanyanya kuipa kupfuura Hunter syndrome.

Mharidzo yekuenda nayo kumba

Ndinonzwisisa kuti zvakaoma sei kuziva kuti mwana wako ane chirwere chakadai seHunter Syndrome. Zvinogona kurwadza moyo, kunyanya kana ukanzwa nezvenguva yekurarama kwemwana wako. Munguva ino yakaoma, yeuka kuti hausi wega.

Chinhu chinonyanya kukosha ndechekushanda navanachiremba vemwana wako kuti udzidze zvakawanda nezvechirwere ichi uye marapirwo acho. Zvakare, gara nevanhu vanotsigira, vakaita seshamwari dzako nemhuri yako. Rutsigiro rwavo nenyaradzo zvichave sosi huru yesimba munguva ino. Yeuka, nematambudziko ese, pane tariro.

Hunter Syndrome, Hunter Syndrome, MPS II, Zvirwere zveMajini, MaEnzymes, Zvirwere zveVana, Zviratidzo, Kurapwa