Tsandanyama dzemwana wenyu dziri kuita sedziri kuderera here? Ngatikurukurei nezvenyaya iyi (Becker Muscular Dystrophy)!

Wakamboona here kuti vamwe vana kana vechidiki vane dambudziko rekufamba, kumhanya, kana kukwira masitepisi kupfuura vamwe? Kana kuti wakambonzwa sekunge tsandanyama dzavo dziri kupera simba zvishoma nezvishoma? Pamwe chikonzero cheizvi chirwere chatichataura nezvacho nhasi, chinonzi `(Becker Muscular Dystrophy)`. Usazvidya moyo, ngatitsanangure zvese nemazwi akareruka.

Chii chinonzi `(Becker Muscular Dystrophy)`? Zvichitaurwa zviri nyore...

`(Becker Muscular Dystrophy)`, inozivikanwawo se`(BMD)` muchidimbu, chirwere chisingawanzoitiki chemajini. Chinoitika ndechekuti tsandanyama dziri mumuviri dzinopera simba zvishoma nezvishoma uye mashandiro adzo anoderera. Kutaura chokwadi, ichi chirwere chemajini. Chirwere ichi chinonyanya kubata vakomana nevarume. Chikonzero cheizvi `(X-linked inheritance)`, zvinoreva kuti chinotorwa kubva kuna amai (kana vari ivo vanotakura chirwere) kumwana wechirume.

Kushaya simba kwetsandanyama uku kunowanzotanga mumakumbo nemuchiuno, uye nekufamba kwenguva, kunogona kupararira kusvika mumaoko ako epamusoro, kureva muviri wako wepamusoro.

Pamhando dzemusana dzinozivikanwa pari zvino, BMD inogona kunge iri yechitatu inonyanya kuwanikwa pakati pevanhu vakuru, mushure me myotonic dystrophy uye facioscapulohumeral dystrophy.

Ndeupi musiyano uripo pakati pe `(Becker Muscular Dystrophy)` uye `(Duchenne Muscular Dystrophy)`?

Ungave wakambonzwa nezvechirwere chinonzi `(Duchenne Muscular Dystrophy)` kana `(DMD)`. Zvese `(BMD)` uye `(DMD)` zvinokonzerwa nekuchinja kwemajini akafanana, kureva jini rinopa puroteni inonzi `(dystrophin). Puroteni iyi inonzi `(dystrophin)` inokosha zvikuru pahutano hwemhasuru dzedu.

Asi heino musiyano:

• Munhu ane DMD anenge asina puroteni ye dystrophin mutsandanyama dzake.
• Munhu ane BMD ane dystrophin mutsandanyama dzake, asi hazvina kukwana.

Saka, chirwere chinonzi `(BMD)` hachina kunyanya kuoma pane `(DMD)`, uye zviratidzo zvinoonekwa uye zvinofambira mberi zvishoma nezvishoma pane `(DMD)`. Zvisinei, zviratidzo zvacho zvakafanana kune vese vari vaviri.

Ndiani anonyanya kubatwa nechirwere ichi (Becker Muscular Dystrophy)?

Sezvatakambotaura, BMD inonyanya kubata varume. Zvisinei, vakadzi vane BMD (kureva avo vane jini rinokonzera chirwere asi vasingaratidze zviratidzo) vanogona kuva nezviratidzo dzimwe nguva. Zvisinei, kazhinji hazvina kunyanya kuipa, uye hazvina kunyanya kuipa.

Kazhinji, zviratidzo zvinotanga pakati pemakore mashanu negumi nemashanu. Zvisinei, vamwe vanhu vanogona kuona zviratidzo gare gare.

`(Becker Muscular Dystrophy)` yakajairika zvakadii?

BMD chirwere chisingawanzoitiki.Chirwere ichi chinokanganisa vana vari pakati pe3 ne6 pavana 100,000 vanoberekwa. Uye sezvatataura, chinokanganisa vakomana zvakanyanya.

Ndezvipi zviratidzo zve `(Becker Muscular Dystrophy)`?

Zviratidzo zveBMD zvinowanzotanga pakati pemakore mashanu negumi nemashanu, asi zvinogona kuitika gare gare. Chinoitika ndechekuti kushaya simba kwetsandanyama kunowedzera zvishoma nezvishoma nekufamba kwenguva. Saka, zviratidzo zvinonyanya kuzivikanwa ndeizvi:

• Kuomerwa nekukwira masitepisi.
• Kuoma kwekufamba, uye kuoma kuri kuwedzera nekufamba kwenguva.
• Kusakwanisa kuita maekisesaizi (kunzwa kuneta kunyangwe uchiita kurovedza muviri zvishoma).
• Kurwadziwa kwetsandanyama uye/kana kugwinha kwetsandanyama (sekunge chinyamunhari).
• Kudonha kakawanda.
• Kufamba netsoka.
• Kunzwa kuneta nguva dzese (Kuneta).

Fungidzira, kana mwana wako asiri kumhanya nekutamba sezvaaimboita, obva ati "Amai, ndaneta" kunyangwe mushure mekufamba kwenguva diki, kana kuti akaneta nekukurumidza kupfuura vamwe vana paanenge achitamba kuchikoro, zvakanaka kuti uzvidye mwoyo nazvo.

Kuwedzera kune izvi, BMD inogona kukonzera zvimwe zviratidzo:

• Cardiomyopathy : Ichi chinhu chekungwarira.
• Kutadza kufema zvakanaka.
• Misiyano pakudzidza (sekutora nguva yakareba kunzwisisa zvimwe zvinhu kupfuura zvimwe).
• Kurasikirwa nekuenzana kwemuviri uye kushanda pamwe chete.

Vakadzi vane chirwere cheBMD vanogona kunge vaine chirwere chemwoyo kana kusasimba kwetsandanyama zvishoma. Zvinofungidzirwa kuti vanhu vanosvika 22% vane chirwere ichi vanozotanga kuva nezviratidzo, asi izvi zvinosiyana zvikuru kubva pamunhu kusvika pamunhu.

Chii chinokonzera `(Becker Muscular Dystrophy)`?

BMD chirwere chemajini chinogara nhaka. Chinokonzerwa nekuchinja kwemajini kunobva puroteni inonzi dystrophin. Dystrophin inokosha pakuchengetedza masero emhasuru mumuviri wedu akasimba uye akagadzikana.

Saka, kana paine shanduko mujini iri rekuti `(dystrophin)`, puroteni ye`(dystrophin)` haigadzirwe, kana kuti huwandu hunogadzirwa hunoderera zvikuru. Nekuda kweizvozvo, nekufamba kwenguva, tsandanyama dzinopera simba dzotanga kukuvara.

Becker Muscular Dystrophy inogarwa nhaka sei? Ichi chinhu chaunofanira kunzwisisa zvishoma!

`(BMD)` inogarwa nhaka nenzira inonzi `(X-linked recessive inheritance)`. Zvino ngatinzwisise izvi zviri nyore.

• X-linked zvinoreva kuti jini rinokonzera BMD riri paX chromosome. Sezvamunoziva, tine machromosome maviri ebonde, X naY.
• Kudzoreredza zvinoreva kuti kuti chirwere ichi chiitike, makopi ese ari maviri ejini rinoenderana (tine makopi maviri ejini rega rega) anofanira kunge aine pathogenic variant kana mutation inokonzera chirwere ichi.

Asi hechino chinhu chinonyanya kukosha:

• Varume (XY) vane chromosome imwe chete yeX. Saka, kana paine chikanganiso mujini rinoenderana nechromosome imwe chete yeX, zvakakwana kukonzera `(BMD)`.
• Vakadzi vane ma chromosome maviri eX (XX) . Saka kuti chirwere cheX-linked recessive chiitike, kazhinji makopi ese ari maviri egene anofanira kunge asina kukwana. Zvisinei, vakadzi vane gene rakakanganisika pa chromosome imwe chete yeX vanonzi "vatakuri." Kazhinji, vatakuri ava havaratidzi zviratidzo. Zvisinei, kashoma, zviratidzo zvishoma kana zviri pakati nepakati zvinogona kuitika.

Zvino tarisa kuti izvi zvinotanga sei muzvizvarwa:

• Kune amai vane chirwere ichi (vane jini risina kunaka pane imwe X chromosome) :
• Kana mwanakomana aberekwa, pane mukana we50% wekuti mwanakomana wacho achava nechirwere ichi (BMD).
• Kana uine mwanasikana, pane mukana we50% wekuti achava nechirwere ichi.
• Kuna baba vane chirwere cheBMD :
• Haakwanise kutapurira chirwere ichi kuvanakomana vake (nekuti baba ndivo vanotapurira chromosome yeY kumwanakomana).
• Asi vanasikana vake vese vachatakura chirwere ichi (nekuti baba ndivo vanopa mwanasikana wacho X chromosome isina kunaka).

Unonzwisisa here? Izvi zvingaita sezvakaoma zvishoma, asi muchidimbu, mukomana anowanzo kubatwa naamai vake, kana amai vacho vari ivo vakatakura chirwere ichi.

`(Becker Muscular Dystrophy)` inoonekwa sei?

Kana iwe kana mwana wako muchifungirwa kuti une BMD, chiremba wenyu angangoita bvunzo yemuviri, bvunzo yetsinga, uye bvunzo yetsandanyama. Vachakubvunzaiwo nezvezviratidzo zvenyu uye nhoroondo yenyu yehutano, kusanganisira kana paine mumwe munhu mumhuri menyu akambova nematambudziko akafanana.

Munguva yekuongororwa uku, chiremba anogona kuona zvinhu zvakaita se:

• Tsandanyama dzemakumbo nechiuno dzadzikira.
• Kunyangwe tsandanyama dziri muhudyu dzichigona kutaridzika sedzakakura pakutanga (izvi zvinonzi "pseudohypertrophy" ), dzinenge dzisina simba. Zvinoita sekunge dzakangozvimba kubva mukati zvichienda kunze.
• Kukombama kwemusana (scoliosis) uye zvimwe zvirema zvechipfuva.
• Kusashanda zvakanaka kwetsandanyama, semuenzaniso, kusimba kwetsandanyama, marunda, uye ganda muzvitsitsinho nemakumbo (contractures) .

Ndezvipi zviedzo zvinoshandiswa kuongorora `(Becker Muscular Dystrophy)`?

Kana chiremba wako achifungidzira kuti iwe kana mwana wako ane BMD, vanogona kukurudzira bvunzo dzinotevera:

• Kuongororwa kweropa reCreatine kinase: Kana tsandanyama dzakuvara, dzinoburitsa enzyme inonzi creatine kinase muropa. Munhu ane BMD anogona kuva nechikamu checreatine kinase ichi chakapetwa kashanu kana kupfuura pane zvakajairwa.
• Kuongororwa kweropa remajini: Kuongororwa kwemajini uku, kunoongorora shanduko mujini reDystrophin, kunogona kunyatsoongorora BMD.

Kana iwe kana mwana wako zvikaonekwa kuti une BMD, chiremba wako anogonawo kukurudzira kuti upiwe Electrocardiogram (EKG) kana Echocardiogram kuti utarise matambudziko emhasuru dzemwoyo anogona kukonzerwa neBMD.

Becker Muscular Dystrophy inorapwa sei?

Zvinosuwisa kuti pari zvino hapana mushonga weBMD. Saka, chinangwa chikuru chekurapa ndechekudzora zviratidzo uye kuchengetedza hupenyu hwakanaka.

Kune nzira mbiri huru dzekurapa BMD:

1. Corticosteroids: Semuenzaniso, mishonga yakaita seprednisolone. Iyi inobatsira kuvandudza mashandiro emapapu, kunonoka kukura kwe scoliosis, kunonoka kukura kwe cardiomyopathy, uye kuwedzera makore ekurarama.

2. Kugadziriswa: Izvi zvinobatsira murwere kuchengetedza kugona kwake kushanda kwenguva yakareba uye kuvandudza hupenyu hwake.

• Kurapa kwemuviri kunobatsira kusimbisa tsandanyama.
• Kurapa nekutaura, kurapwa nebasa, uye kurapwa kwekuzorora zvinogona kubatsira pamabasa ezuva nezuva.

Pamusoro peizvi, kune mamwe marapirwo anogona kubatsira neBMD:

• Zvinobatsira pakufamba-famba - semuenzaniso tsvimbo, mawiricheya, mabraces.
• Mishonga ye `(Cardiomyopathy)` - semuenzaniso `(ACE inhibitors)` uye `(beta-blockers)` .
• Kuvhiyiwa kwekubatsira ne scoliosis uye contractures.
• Kana matambudziko ekufema akanyanya (kutadza kufema) , tracheostomy (nzira yekuvhiya yekuvhura trachea) uye kufema kwekugadzira zvinogona kudiwa.

Mashoko akanaka ndeekuti mishonga mitsva yakati wandei yekurapa `(BMD)` iri kuongororwa mukiriniki parizvino uye inogona kuratidza kuti ine tariro mune ramangwana. (Chikanganiso chekushandura chiri muchinyorwa, chinofanira kunge chiri Sinhala: Mashoko akanaka ndeekuti mishonga mitsva yakati wandei yekurapa `(BMD)` iri kuongororwa mukiriniki parizvino. Inogona kuratidza kuti ine tariro mune ramangwana.)

Nerombo rakanaka, mishonga mitsva yakawanda inogona kurapa BMD iri mukuyedzwa kwekiriniki parizvino, uye tinogona kutarisira mhedzisiro yakanaka kubva mairi mune ramangwana.

Becker Muscular Dystrophy inogona kudzivirirwa here?

Sezvo BMD iri chirwere chinogara nhaka, hapana chatingaita kuti tidzivise chirwere ichi.

Zvisinei, kana uine BMD, kana kuti uchinetseka kuti ungave uine BMD kana chimwe chirwere chemajini, taura nachiremba wako nezvazvo usati wava nevana.Zvakanaka kwazvo kutsvaga mazano ezvemajini.

Chii chinonzi Becker Muscular Dystrophy?

Maonero emunhu ane BMD anogona kusiyana kubva pamunhu kuenda kune mumwe. Uku kufambira mberi zvishoma nezvishoma kwehurema. Zvisinei, kuoma kwehurema kunosiyana. Vamwe vanhu vangada wiricheya, nepo vamwe vangangoda kushandisa zvinhu zvekufambisa (tsvimbo, madondoro).

Zvisinei, kana munhu ane ``(BMD)`` aine chirwere chemwoyo kana matambudziko ekufema, makore ekurarama anogona kupfupikiswa.

Matambudziko anogona kuitika nekuda kweBMD ndeaya:

• Matambudziko emwoyo, kunyanya `(Cardiomyopathy)`.
• Kutadza kufema zvakanaka kunokonzerwa nekushaya simba kwetsandanyama dzekufema.
• Pneumonia kana zvimwe zvirwere zvekufema.
• Nekufamba kwenguva, kuremara kunowedzera uye munhu wacho anotadza kuita basa rake ega.
• Kutyoka kwemapfupa.

Munhu ane `(Becker Muscular Dystrophy)` anotarisirwa kurarama kwenguva yakareba sei?

Nguva yekurarama kwemunhu ane "BMD" inowanzopfupikiswa. Kureva kuti, pakati pemakore makumi mana nemakumi mashanu. "Dilated cardiomyopathy" (chirwere chinoita kuti tsandanyama dzemwoyo dzishaye simba uye dzikure) ndicho chinokonzera rufu.

Ndingatarisira sei munhu ane `(BMD)`? Kana kuti ndingazvitarisira sei?

Kana uine BMD, zvakakosha kuti uwane rubatsiro rwakanaka rwekurapa kudzivirira kana kurapa matambudziko eBMD, akadai sechirwere chemwoyo nematambudziko ekufema. Zvinogonawo kubatsira kubatana neboka rekutsigirana kwaunogona kugovana zvawakasangana nazvo uye kusangana nevamwe vanokunzwisisa.

Kana uri kutarisira munhu ane BMD, zvakakosha kuve nechokwadi chekuti ari kuwana rubatsiro rwekurapa rwakanakisisa, zvinhu zvinobatsira pakufamba, uye marapirwo anomubatsira kushanda ega. Ndiwe unofanira kuva mutsigiri wavo.

Ndirini pandinofanira kuona chiremba nezve `(Becker Muscular Dystrophy)`?

Kana iwe (kana mwana wako) wakamboonekwa uine Becker Muscular Dystrophy, zvakakosha kuti ugare uchishanyira timu yako yezvekurapa kuti uwane kurapwa uye kuti utarise zviratidzo zvako.

Tinoziva kuti chirwere chakadai seBecker Muscular Dystrophy hachisi nyore kunzwisisa uye kugadzirisa. Chinogona kukuremera. Chikwata chako chekurapa chichakupa hurongwa hwakasimba hwekutarisira hwakagadzirirwa zviratidzo zvako. Zvakakosha kuve nechokwadi chekuti uri kuwana rutsigiro rwaunoda uye uri kutarisira hutano hwako.

Muchidimbu, zvinhu zvatinofanira kurangarira (Mharidzo Yekuenda Kumba)

Zvakanaka, saka hezvino zvimwe zvinhu zviri nyore zvekurangarira nezve `(Becker Muscular Dystrophy)` kana `(BMD)` zvatakataura nezvazvo:

• "(BMD)`` chirwere chemajini chinopfuudzwa nezvizvarwa. Mukuita izvi, tsandanyama dzinopera simba zvishoma nezvishoma.
• IzviZvinonyanya kukanganisa varume.
• Chikonzero chacho idambudziko riri mujini rinogadzira puroteni inonzi "dystrophin".
• Zviratidzo zvinowanzotanga paudiki (pakati pemakore mashanu negumi nemashanu). Zviratidzo zvinosanganisira kunetseka kufamba, kuneta, uye kudonha kakawanda.
• Cardiomyopathy (chirwere chetsandanyama dzemwoyo) uye kutadza kufema zvinogona kuva matambudziko makuru echirwere ichi.
• Parizvino hapana mushonga wechirwere ichi. Zvisinei, kune nzira dzakasiyana-siyana dzekurapa dzinowanikwa kudzora zviratidzo uye kuvandudza hupenyu (semuenzaniso, corticosteroids, physiotherapy).
• Kana mumwe munhu mumhuri aine chirwere ichi, kuchenjera kutsvaga mazano ezvemajini usati wava nemwana.
• Zvakakoshawo kutsvaga mazano ekurapwa nguva dzose, pamwe nekugara wakasimba mupfungwa.

Usakanganwa kuti hausi wega. Paunenge uchisangana nedambudziko iri, tsvaga rubatsiro kubva kuna vanachiremba, mhuri, shamwari, nemapoka ekutsigira. Izvi zvichakupa simba guru!

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Becker muscular dystrophy, BMD, kusasimba kwetsandanyama, zvirwere zvemajini, dystrophin, X-linked, majini mutations, hutano hwevana, chirwere chemwoyo, physiotherapy