Yintoni kunye nendlela yokuphatha i-Becker muscular dystrophy

Umxholo

• Unyango lwenziwa njani
• Iimpawu eziphambili
• Uxilongo lwenziwa njani
• Yintoni enokubangela i-dystrophy

Becker muscular dystrophy sisifo semfuza esibangela ukutshatyalaliswa ngokuthe ngcembe kwezihlunu zokuzithandela, oko kukuthi, izihlunu esikwaziyo ukuzilawula, ezinjengezinqe, amagxa, imilenze okanye iingalo, umzekelo.

Ihlala ixhaphake emadodeni kwaye iimpawu zokuqala zivela ebuntwaneni okanye ngexesha lokufikisa, ukuqala ngokuncipha kancinci kancinci kwamandla phantse kuzo zonke izihlunu zomzimba, kodwa ngakumbi emagxeni nasezinqeni.

Nangona esi sifo singenalo unyango, kunokwenzeka ukuba ufumane unyango ukuze ukhulule iimpawu kwaye ube nomgangatho olungileyo wobomi kunye nolindelo lobomi ukuya kuthi ga kwiminyaka engama-50.

Unyango lwenziwa njani

Unyango lwe-Becker muscular dystrophy lwenziwe ukunciphisa iimpawu zomntu ngamnye kwaye, ke, zinokwahluka kwimeko nganye. Nangona kunjalo, ezona ndlela zixhaphakileyo zonyango zibandakanya:

• Unyango lweCorticosteroid, ezinje ngeBetamethasone okanye iPrednisone: ukunceda ukunciphisa ukudumba kwezihlunu ngelixa ukhusela imicu yemisipha kunye nevolumu yazo. Ngale ndlela kunokwenzeka ukugcina umsebenzi wemisipha ixesha elide;
• Umzimba wonyango: inceda ukugcina ukuhamba kwezihlunu, ukuzolula nokuzithintela ukuba zingabinamandla. Ke, kunokwenzeka ukunciphisa inani lokonzakala kwimisipha yemisipha kunye namalungu;
• Unyango emsebenzini: ziiseshoni ezifundisa indlela yokuphila nemida emitsha ebangelwa sesi sifo, ukuqeqesha iindlela ezintsha zokwenza imisebenzi esisiseko yemihla ngemihla njengokutya, ukuhamba okanye ukubhala, umzekelo.

Ukongeza, kusenokufuneka kwenziwe utyando, ngakumbi ukuba izihlunu ziba mfutshane okanye ziqine, ukuzikhulula kunye nokulungisa ukufinyezwa. Xa iikhontrakthi zivela kwiimisipha zamagxa okanye zangasemva, zinokubangela ukukhubazeka emqolo ekufuneka kulungisiwe ngotyando ngokunjalo.

Kwinqanaba elibi kakhulu lesi sifo, kuqhelekile ukuba iingxaki ezinzulu zivele, ezifana neengxaki zentliziyo kunye nokuphefumula kanzima, ngenxa yokutshatyalaliswa kwemisipha yentliziyo kunye nemisipha yokuphefumla. Kwiimeko ezinjalo, i-cardiologist kunye ne-pulmonologist inokuqeshwa ukuba incedise unyango.

Iimpawu eziphambili

Iimpawu zokuqala zeBackker muscular dystrophy zihlala zivela phakathi kweminyaka emi-5 ukuya kweli-15 ubudala, kwaye zinokubandakanya iimpawu ezinje:

• Kancinci kancinci ukuhamba nokunyuka kwezinyuko;
• Ukuwa rhoqo ngaphandle kwesizathu;
• Ukuphulukana nobunzima bemisipha;
• Ukudodobala kwezihlunu zentamo neengalo;
• Ukudinwa kakhulu;
• Ukuphulukana nokulingana kunye nokulungelelanisa;

Kwiimeko ezininzi umntwana angayeka ukuhamba de abe li-16 leminyaka, kuba esi sifo siqhubela phambili ngokukhawuleza kumalungu asezantsi. Nangona kunjalo, xa iimpawu zibonakala mva kunesiqhelo, amandla okuhamba anokugcinwa nokuba phakathi kweminyaka engama-20 nama-40.

Uxilongo lwenziwa njani

Kwiimeko ezininzi, ugqirha wabantwana unokulrhanela olu hlobo lwe-dystrophy kuphela ngokuvavanya iimpawu kunye nokujonga ukulahleka kwezihlunu zemisipha, umzekelo. Nangona kunjalo, ezinye iimvavanyo zokuqonda isifo ezinje nge-biopsy yemisipha, iimvavanyo zentliziyo kunye nee-X-reyi zinokunceda ekuqinisekiseni ubukho be-Becker Muscular Dystrophy.

Yintoni enokubangela i-dystrophy

I-Becker's Muscular Dystrophy ivela ngenxa yotshintsho kwimfuzo ethintela ukuveliswa kweprotein ye-dystrophin, into ebaluleke kakhulu ukugcina iiseli zemisipha ziqinile. Ke, xa le proteni ikwixabiso eliphantsi emzimbeni, izihlunu azikwazi ukusebenza kakuhle, ziqala ukubonakala njengezilonda ezitshabalalisa imicu yemisipha.

Njengesifo semfuza, olu hlobo lwe-dystrophy lunokudluliselwa kubazali luye ebantwaneni okanye luvele ngenxa yenguqu ngexesha lokukhulelwa.