Une tsandanyama dzisina simba here mumapfudzi ako nemuchiuno? Ngatikurukurei nezve Limb-Girdle Muscular Dystrophy (LGMD)!

Dzimwe nguva unonzwa sekuti mapfudzi ako, maoko, kana chiuno chako hazvina simba here? Unoona zvichikuomera here kusimuka pachigaro kana kukwira masitepisi? Dzimwe nguva unonzwa sekuti maoko ako haana simba here paunosimudza chimwe chinhu chinorema? Izvi zvingasava kuneta kwemuviri chete. Nhasi tichataura nezvechirwere chisingawanzoitiki asi chakakosha zvikuru. Ichi chinonzi Limb-Girdle Muscular Dystrophy , kana kuti LGMD muchidimbu.

Chii chinonzi Limb-Girdle Muscular Dystrophy (LGMD)?

Zvichitaurwa zviri nyore, Limb-Girdle Muscular Dystrophy izwi rekuti chirwere chinogara nhaka chinoderedza simba remhasuru dzimwe mumiviri yedu zvishoma nezvishoma. Ichirwere chisingaperi , zvichireva kuti chinogona kugara kweupenyu hwese. Chinogona kukanganisa chero ani zvake wezera ripi zvaro.

"Mimba dzemakumbo" mapfupa akakomberedza mapendekete edu nemahudyu. Kungofanana nemajoini anobatanidza maoko edu nemakumbo kumuviri wedu. Saka mune iyi mamiriro ekuti "(LGMD)``, tsandanyama dzine chekuita nenzvimbo idzi dzemapendekete nechiuno dzinonyanya kukanganiswa.

Ungave wakambonzwawo izwi rekuti "muscular dystrophy." Rinoreva boka rezvirwere zvemajini zvinokanganisa mashandiro emhasuru. Mumhando zhinji dze "muscular dystrophy," zviratidzo zvinowedzera zvishoma nezvishoma nekufamba kwenguva.

Chirwere ichi chinonzi LGMD chakajairika zvakadii?

Kutaura zvazviri, `(Limb-Girdle Muscular Dystrophy)` chirwere chisingawanzoitiki . Ingofungai, munyika yakaita seAmerica, kana mukawedzera marudzi ese aya `(LGMD)`, inongobata vanhu vaviri chete pazviuru zana. Kana mukaenzanisa ne`(Duchenne muscular dystrophy)` yatinoziva uye yatinotaura nezvayo zvishoma (iyi imhandowo ye`muscular dystrophy`), inobata anenge mumwe chete pavakomana 3600 muAmerica. Saka `(LGMD)` haina kuwanda zvakanyanya kupfuura izvozvo.

Pakati pemhando idzi dze `(LGMD)`, rudzi rwakajairika muUnited States ndi `(LGMD R1 calpain3-related)`. Izvi zvinonziwo `(calpainopathy)`. Pakati pe12% ne30% yevarwere vese ve`(LGMD)` ndeverudzi urwu.

Ndezvipi zviratidzo zvatinoona?

Zviratidzo zvikuru zveLimb-Girdle Muscular Dystrophy kushaya simba kwetsandanyama uye kupera simba kwetsandanyama kana kupera simba . Izvi zvinonyanya kukanganisa:

• Kumafudzi
• Kune maoko ekumusoro (chikamu cheruoko rwedu kubva pafudzi kusvika kugokora)
• Kunzvimbo yehudyu
• Zvezvidya (chikamu chemakumbo edu kubva muchiuno kusvika pabvi)

Zera rinotanga zviratidzo, pamwe nekukurumidzira kwezviratidzo izvi, zvinogona kusiyana kubva kune rumwe rudzi rweLMD kuenda kune rumwe.

Zviratidzo zvekutanga zvechirwere ichi kuoma kufamba . Semuenzaniso:

• Kufamba-famba kwedhadha kunogona kutanga, kufanana nekufamba-famba kwedhadha .
• Kubva panzvimbo yekugara, senge chigaro kana chigaro chechimbuziZvakaoma kumuka .
• Kuomerwa nekukwira masitepisi .

Uyewo, kana tsandanyama dziri mumapfudzi nemaoko ekumusoro dzikashaya simba, zvinhu zvakaita seizvi zvinogona kuitika:

• Kuoma kutambanudza musoro . Funga nezvazvo sekubvisa chimwe chinhu pasherufu.
• Zvakaoma kutambanudza maoko ako pamberi pako .
• Kusakwanisa kusimudza zvinhu zvinorema .
• Vamwe vanhu vangaomerwa nekushandisa maoko avo kunyangwe pakudya .

Mamwe marudzi eLGMD anogona kunge aine mamwe maitiro pamusoro peizvi. Mamwe acho anosanganisira:

• Matambudziko emwoyo : Semuenzaniso, zvinhu zvakaita sekushaya simba kwetsandanyama dzemwoyo (cardiomyopathy), kusashanda zvakanaka kwemuchina wekufambisa mweya, kana kuti kusagadzikana kwemoyo.
• Kutadza kufema zvakanaka .
• Kuomerwa nekumedza chikafu (dysphagia) .
• Kukotama , zvinoreva kuoma kwekukotama nekusunungura majoini.
• Kurwadziwa kwetsandanyama .
• Kuwanda kwetsandanyama : Dzimwe nguva, kunyangwe tsandanyama dzisina simba, dzinogona kutaridzika dzakakura kubva kunze.
• Kushaya simba mutsandanyama dziri kure, dzakadai semaoko nemakumbo .

Izvi zvinogona kukonzera matambudziko akakomba here?

Ehe, dzimwe nguva LGMD inogona kukonzera mamwe matambudziko. Asi hazvina kufanana kune wese munhu. Pane zvinhu zvakawanda zvinogona kuikanganisa:

• Une rudzii rwe `(LGMD)`?
• Zviratidzo zvakatanga pazera ripi uye chirwere ichi chinokurumidza kukura zvakadii.
• Une nzvimbo dzakanaka dzekurapa nekurapa zviratidzo.

Mamwe ematambudziko anogona kuitika ndeaya:

• Kana LGMD ikatanga muhudiki, inogona kukonzera kunonoka kwehunyanzvi hwekufamba kwemuviri hwakadai sekufamba .
• Mamwe marudzi anogona kukonzera matambudziko epfungwa nematambudziko ekudzidza .
• Kurwara kwemutsipa (kyphosis) uye/kana scoliosis zvinogona kuonekwa, kunyanya muLMD inotanga muhudiki.
• Kushanda zvakanaka kwemapapu kunogona kukanganisa mashandiro emapapu, zvichikonzera chirwere chemapapu chinodzvinyirira , pamwe kutadza kufema zvakanaka kana kutadza kufema zvakanaka .
• Kushaya chikafu chinovaka muviri kunogona kuitika nekuda kwekutadza kudya nekumedza.

Sei izvi zvichiitika? Chikonzero chacho ndechei?

Chikonzero chikuru cheLimb-Girdle Muscular Dystrophy ikuchinja kwemajini.. Majini aya ane basa rekuchengetedza chimiro chetsandanyama nekushanda zvakanaka. Saka kana paine shanduko mumajini aya, masero anofanirwa kuchengetedza tsandanyama haagone kuita basa iroro nemazvo. Nekuda kweizvozvo, tsandanyama dzinopera simba zvishoma nezvishoma nekufamba kwenguva. Kune shanduko dzemajini dzakananga dzine chekuita nerudzi rwese rwe `(LGMD)`.

Kuchinja kwemajini uku kunobva kuvabereki vedu. LGMD yakakamurwa kuita zvikamu zviviri zvikuru, zvichienderana nekuti majini acho anogarwa nhaka sei:

• LGMD Group D : Izvi zvinoitika nenzira inonzi ``autosomal dominant inheritance``. Izvi zvinoreva kuti genetic mutation inokonzera chirwere ichi inogona kukura kunyangwe mubereki mumwe chete ndiye akaitora nhaka.
• Boka reLMD R : Izvi zvinoitika nenzira inonzi ``autosomal recessive inheritance``. Izvi zvinoreva kuti genetic mutation inokonzera chirwere inofanira kugara nhaka kubva kuvabereki vese.

Kune mhando dzakasiyana dzeLMD here?

Ehe, kune mhando dzakasiyana-siyana dze `(Limb-Girdle Muscular Dystrophy)`. Vaongorori navanachiremba vanoshandisa chimiro chakakosha chekutumidza mazita kuti vagadzire mhando idzi. Chine mavara ekuti "LGMD" nezvimwe zvinhu zvishoma:

• Maitiro ekugara nhaka majini : Vara "D" rinomirira `(autosomal dominant inheritance)`. Vara "R" rinomirira `(autosomal recessive inheritance)`.
• Kurongeka kwekuwanikwa : Vaongorori vanopa mhando idzi nhamba zvichienderana nekurongeka kwadzakawanikwa.
• Mapuroteni kana jini rakakanganiswa : Izvi zvinoratidzwa se "[jini kana zita reprotein]-zvine chekuita". Semuenzaniso, `(zvine chekuita necalpain3)`.

Kune mhando dzakasiyana dzeizvi. Zvakaoma kutsanangura chimwe nechimwe, saka hatizonyatsotsanangura zvakadzama. Asi chiremba wako anogona kukuudza zvakawanda nezvazvo.

Vanachiremba vanoona sei izvi?

Kana iwe kana mwana wako muchifungirwa kuti mune zviratidzo zveLimb-Girdle Muscular Dystrophy, chiremba angangoita bvunzo yemuviri , bvunzo yetsinga , uye bvunzo yetsandanyama . Vachakubvunza nezvezviratidzo zvako uye kana paine mumwe munhu mumhuri mako akambova nematambudziko aya.

Kana uchifungidzira kuti une LGMD, imwe kana kupfuura yemiedzo inotevera inogona kukurudzirwa:

• Kuongororwa kweropa reCreatine kinase : Kana tsandanyama dzakuvara, enzyme inonzi ``creatine kinase`` inoburitswa muropa. Saka kana huwandu hwayo hwakwira, inogona kunge iri chiratidzo chechirwere chinonzi ``muscular dystrophy.``
• Kuongororwa kwemajini : Kuongororwa uku kunogona kuona shanduko dzemajini dzine chekuita nemamwe marudzi eLGMD. Iyi ndiyo nzira chete yekusimbisa kuti ndeipi mhando yeLGMD yaunayo .
• Kuongororwa kwetsandanyama: Izvi zvinosanganisira kutora sampuli diki yenyama yako yetsandanyama uye kuita kuti nyanzvi iiongorore pasi pemaikorosikopu. Izvi zvinogona kubatsira kuona kana uine zviratidzo zve muscular dystrophy.
• Electromyography (EMG) : Iyi bvunzo inoyera mashandiro emagetsi emhasuru netsinga.

Kana iwe kana mwana wako mukaonekwa kuti mune chirwere cheLMD, chiremba wenyu anowanzo kuraira kuongororwa kwebasa remwoyo nemapapu kuti aone kuti mwoyo nemapapu zviri kushanda zvakanaka sei. Zvakakosha kuziva kana chirwere chacho chakakanganisa nhengo idzodzo zvakare.

Ndezvipi zviri kurapwa?

Zvinosuwisa kuti pari zvino hapana mushonga weLimb-Girdle Muscular Dystrophy, asi vaongorori vari kuramba vachishanda pairi.

Chinangwa chikuru chekurapa kuripo parizvino ndechekubatsira kudzora zviratidzo uye kuvandudza hupenyu . Sarudzo dzekurapa dzinogona kusiyana zvichienderana nerudzi rweLMD yaunayo. Dzimwe dzacho dzinosanganisira:

• Kurapa kwemuviri uye kwemabasa : Izvi zvinonyanya kubatsira kusimbisa tsandanyama uye kuita maekisesaizi ekutambanudza. Zvinokubatsira kuwana nzira dzekuita mabasa ezuva nezuva zviri nyore uye kuchengetedza kufamba-famba.
• Corticosteroids : Corticosteroids dzakadai seprednisolone nedeflazacort dzinogona kubatsira kuderedza kushaya simba kwetsandanyama, kuvandudza mashandiro emapapu, kuderedza marwadzo emusana, uye kuderedza kufambira mberi kwechirwere chemwoyo. Zvisinei, hazvishande kune marudzi ese eLGMD. Anonyanya kubatsira kune mamwe marudzi, akadai seLGMD R9 FKRP-related.
• Zvishandiso zvekufambisa : Zvishandiso zvakaita setsvimbo, mabraces, ma walker, uye ma wheelchairs zvinoita kuti kufamba nekufamba-famba zvive nyore, zvinobatsira kudzivirira kudonha, uye zvinobatsira kuderedza kuneta.
• Kuvhiyiwa : Vamwe varwere veLMD vangada kuvhiyiwa kuderedza kushushikana kwetsandanyama dzakasimba uye kugadzirisa scoliosis.
• Kutarisirwa kwekufema : Midziyo yekubatsira pakukosora nemidziyo yekufema inogona kubatsira kuita kuti kufema kuve nyore. Kana kutadza kufema zvakanyanya, tracheostomy (kuburitsa buri mumutsipa kuti ubatsire kufema) uye rubatsiro rwekufema zvinogona kudiwa.
• Kuchengetedza moyo : Mishonga yakaita seACE inhibitors uye/kana beta-blockers, kana ikatanga nekukurumidza, inogona kunonoka kufambira mberi kwechirwere chemwoyo uye kudzivirira kukura kwekutadza kushanda kwemoyo. Mishonga inodzora mafambiro emoyo inogona kubatsira kurapa matambudziko emwoyo uye kutadza kushanda kwemoyo.
• Kurapa nekutaura: Kurapa uku kunogona kubatsira kune avo vanonetseka nekumedza.
• Miedzo yekiriniki : Zvichienderana nerudzi rweLMD yaunayo uye kwaunogara, unogona zvakare kuwana mukana wekutora chikamu mumiedzo mitsva yekiriniki. Kune huwandu huri kukura hwemiedzo yekiriniki iri kuitwa pari zvino yeLMD.

Chikwata chenyanzvi chinowanzo diwa kuti chitarisire LGMD, iwe nemwana wako. Izvi zvinogona kusanganisira vanachiremba vetsinga, vanachiremba vemuviri, vanachiremba vemajini, vanachiremba vemapfupa, vanachiremba vemuviri, vanachiremba vezvemabasa, vanachiremba vemwoyo, vanachiremba vemapapu, vanachiremba vepfungwa, uye vashandi vezvemagariro evanhu.

Chii chiri kutarisira munhu ane LGMD?

Zvakaoma kuti vaongorori navanachiremba vataure kuti fungidziro yacho ndeyei kune munhu ane LGMD. Asi timu yenyu yezvekurapa ichakupai ruzivo rwakawanda sezvinobvira nezvezvamunotarisira.

Kazhinji, kana LGMD ikatanga muhudiki, chirwere ichi chinokurumidza kukura uye matambudziko anogona kuitika . Zvisinei, kana LGMD ikatanga muhudiki kana kuti muhukuru, kazhinji inenge isina kunyanya kuipa uye chirwere chinokura zvishoma nezvishoma .

Ungagara kwenguva yakareba sei uine izvi?

Avhareji yehupenyu hwemunhu ane LGMD inoenderana zvikuru nekuti vane rudzii rweLMD uye kuti inokurumidza sei kukura . Kazhinji, vanhu vane LGMD vanotambura nematambudziko akadai sechirwere chemwoyo uye matambudziko ekufema, vanogona kurarama kwenguva pfupi kupfuura avo vasina matambudziko akadaro.

Pane nzira yekudzivirira izvi here?

Nekuti Limb-Girdle Muscular Dystrophy chirwere chinokonzerwa nemajini, pari zvino hapana chinogona kuitwa kudzivirira chirwere ichi .

Kana uchironga kuva nevana uye uchinetseka kuti ungangove uchipfuudza LGMD kana zvimwe zvirwere zvemajini, zvakanaka kutaura nachiremba wako nezvemazano ezvemajini .

Kana uine LGMD, pane zvinhu zvakawanda zvaunogona kuita kudzivirira kana kunonotsa matambudziko uye kuvandudza hupenyu hwako:

• Idya chikafu chakanaka uye chinovaka muviri kudzivirira kushaya chikafu chakakwana.
• Inwa mvura yakawanda kudzivirira kupera mvura mumuviri uye kuvimbiwa.
• Ita maekisesaizi ari nyore kusvika ane mwero sezvakakurudzirwa nechikwata chevanachiremba vako.
• Ramba uine huremu hwakanaka .
• Dzivisa kusvuta kuti udzivirire mapapu ako nemoyo.
• Wana majekiseni panguva .

Ndingabatsira sei munhu ane chirwere cheLMD? Kana kuti ndoita sei kana ndiinacho?

Kana uine chirwere cheLimb-Girdle Muscular Dystrophy, kana kuti uri kutarisira mumwe munhu ane chirwere ichi, zvakakosha kutsigira nekutsvaga rubatsiro rwekurapa rwakanakisisa . Kuita izvi kuchakubatsira kuchengetedza hupenyu hwakanaka.

Iwe nemhuri yako munogonawo kubatana nemapoka ekutsigirana . Izvi zvichakupai mukana wekusangana nevamwe vanhu vakambosanganawo nezvinhu zvakafanana nemi, kutaura navo, uye kugovana mazano. Zvichava simba guru.

Ndinofanira kuona chiremba rini?

Kana uine Limb-Girdle Muscular Dystrophy, unofanira kuonana nechikwata chako chekurapa nguva nenguva kuti uwane kurapwa uye kutarisa zviratidzo zvako.

Kunzwisisa kuongororwa kwako kweLimb-Girdle Muscular Dystrophy (LGMD) kunogona kukuomera. Asi timu yako yezvekurapa inogona kukubatsira kugadzira hurongwa hwekutarisira hunoenderana nezviratidzo zvako. Chinhu chinonyanya kukosha ndechekuva nechokwadi chekuti unowana rutsigiro rwaunoda uye kuramba wakatarisa pahutano hwako . Yeuka, timu yako yezvekurapa iripo nguva dzose kuti ikubatsire iwe nemhuri yako.

Pakupedzisira, zvinhu zvekurangarira

Kusashanda zvakanaka kweMuscular Dystrophy (LGMD) chirwere chinokonzerwa nemajini chinoita kuti tsandanyama dziri mumafudzi nemuhudyu dzishaye simba zvishoma nezvishoma. Kunyangwe chiri chirwere chisingawanzoitiki, zvakakosha kuziva zviratidzo zvacho.

• Zviratidzo zvikuru : Kushaya simba kwemhasuru dziri mumapfudzi, maoko ekumusoro, mahudyu, nemahudyu, kunetseka kufamba, uye kutadza kusimudza zvinorema.
• Chikonzero : Kusiyana kwemajini.
• Kurapa : Parizvino hapana mushonga. Chinangwa ndechekugadzirisa zviratidzo uye kuvandudza hupenyu. Kurapa kwemuviri, kurapwa kwebasa, uye, kana zvichidikanwa, mishonga nezvishandiso zvakakosha.
• Chinhu chinonyanya kukosha : kuongororwa nekukurumidza, mazano akakodzera ekurapa uye kurapwa. Rutsigiro rwemhuri nemapoka ekutsigirana zvakakoshawo zvikuru.

Kana muine mimwe mibvunzo nezvenyaya iyi, musatya kutaura nachiremba wenyu. Vachakubatsirai.

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