I-Duchenne muscular dystrophy: kuyini, izimpawu nokwelashwa

-Delile

• Izimpawu eziyinhloko
• Ungakuqinisekisa kanjani ukuxilongwa
• Yelashwa kanjani
• 1. Ukusetshenziswa kwemithi
• 2. Izikhathi ze-Physiotherapy
• Siyini isikhathi sokuphila
• Izinkinga ezivame kakhulu
• Yini ebangela lolu hlobo lwe-dystrophy

I-Duchenne muscular dystrophy yisifo esingajwayelekile sofuzo esithinta abesilisa kuphela futhi esibonakala ngokushoda kweprotheni ezicutshini, esaziwa nge-dystrophin, esisiza ukugcina amaseli emisipha ephilile. Ngakho-ke, lesi sifo sibangela ukuba buthakathaka okuqhubekayo kwawo wonke ama-musculature omzimba, okwenza kube nzima ngokwengeziwe enganeni ukufinyelela ezingeni elibalulekile lokukhula, njengokuhlala, ukuma noma ukuhamba.

Ezimweni eziningi, lesi sifo sikhonjwa kuphela ngemuva kweminyaka emithathu noma emi-4 lapho ingane iba nezinguquko endleleni yokuhamba, ukugijima, ukukhuphuka izitebhisi noma ukukhuphuka phansi, ngoba izindawo ezithintekile kuqala yizinqe, amathanga namahlombe. Ngokukhula, lesi sifo sithinta imisipha eminingi futhi izingane eziningi zigcina zincike esihlalweni sabakhubazekile lapho sezineminyaka engama-13 ubudala.

I-Duchenne muscular dystrophy ayinalo ikhambi, kepha ukwelashwa kwayo kusiza ukubambezela ukukhula kwesifo, ukulawula izimpawu nokuvimbela ukuqala kwezinkinga, ikakhulukazi emazingeni enhliziyo nawokuphefumula. Ngakho-ke, kubaluleke kakhulu ukwelashwa nodokotela wezingane noma omunye udokotela ogxile kulesi sifo.

Izimpawu eziyinhloko

Izimpawu eziyinhloko zeDuchenne muscular dystrophy zivame ukubonakala kusukela onyakeni wokuqala wokuphila kuze kufike eminyakeni yobudala engama-6, ziqhubeka ngokuya ziba zimbi ngokuhamba kweminyaka, kuze kube yilapho, cishe eneminyaka eyi-13, umfana ancike esihlalweni esinamasondo.

Ezinye zezimpawu nezimpawu ezivame kakhulu zifaka:

• Ikhono elibambezelekile lokuhlala, ukuma noma ukuhamba;
• Ukudiyazela noma ukuba nobunzima bokukhuphuka izitebhisi noma ukugijima;
• Umthamo owandisiwe ematholeni, ngenxa yokushintshwa kwamaseli emisipha ngamafutha;
• Kunzima ukuhambisa amalunga akho, ikakhulukazi ukugoba imilenze yakho.

Kusukela ebusheni, izinkinga zokuqala ezinzima kakhulu zesifo zingaqala ukuvela, okungukuthi, ubunzima bokuphefumula ngenxa yokwehla kwesithanjana neminye imisipha yokuphefumula, kanye nezinkinga zenhliziyo, ngenxa yokwehla kwemisipha yenhliziyo.

Lapho izinkinga ziqala ukuvela, udokotela angaguqula ukwelashwa ukuzama ukufaka ukwelashwa kwezinkinga nokwenza ngcono izinga lempilo. Ezimweni ezimbi kakhulu, ukwelashwa kungadingeka.

Ungakuqinisekisa kanjani ukuxilongwa

Ezimweni eziningi, udokotela wezingane usola i-Duchenne muscular dystrophy kuphela ngokuhlola izimpawu nezimpawu ezethulwe ngesikhathi sokukhula.Kodwa-ke, zingenziwa futhi ngegazi ukukhomba inani lama-enzyme athile, njenge-creatine phosphokinase (CPK), ekhishelwa egazini lapho kunokonakala kwemisipha.

Kukhona nokuhlolwa kofuzo, okusiza ukufika ekutholeni ukuxazululwa okuphelele, futhi okubheka ushintsho kuzakhi zofuzo ezibhekele ukuqala kwesifo.

Yelashwa kanjani

Yize i-Duchenne muscular dystrophy ingenakho ukwelashwa, kunezindlela zokwelapha ezisiza ukuvimbela ukwanda kwayo ngokushesha futhi okuvumela ukulawula izimpawu, kanye nokuvela kwezinkinga. Ezinye zalezi zindlela zokwelapha zifaka:

1. Ukusetshenziswa kwemithi

Ezimweni eziningi, ukwelashwa kwe-Duchenne muscular dystrophy kwenziwa ngokusetshenziswa kwemithi ye-corticosteroid efana ne-prednisone, prednisolone noma i-deflazacort. Le mithi kufanele isetshenziselwe impilo yonke, futhi ibe nesenzo sokulawula amasosha omzimba, esebenza njenge-anti-inflammatory nokubambezela ukulahleka kokusebenza kwemisipha.
Kodwa-ke, ukusetshenziswa isikhathi eside kwe-corticosteroids kuvame ukudala imiphumela emibi eminingi njengokunyuka kwesifiso sokudla, ukukhuluphala, ukukhuluphala, ukugcinwa kwamanzi, i-osteoporosis, isiqu esifushane, umfutho wegazi ophakeme kanye nesifo sikashukela, futhi kufanele kusetshenziswe kuphela ngaphansi kokuqondisa kukadokotela. Bheka okuningi mayelana nokuthi ama-corticosteroids ayini nokuthi ayithinta kanjani impilo.

2. Izikhathi ze-Physiotherapy

Izinhlobo ze-physiotherapy ejwayele ukusetshenziselwa ukwelapha i-Duchenne muscular dystrophy yimoto kanye ne-kinesiotherapy yokuphefumula kanye ne-hydrotherapy, ehlose ukubambezela ukungakwazi ukuhamba, ukugcina amandla emisipha, ukuqeda izinhlungu nokuvimbela izinkinga zokuphefumula kanye nokuqhekeka kwamathambo.

Siyini isikhathi sokuphila

Isikhathi sokuphila seDuchenne muscular dystrophy sasineminyaka ephakathi kwengu-16 kuya kwengu-19 ubudala, kodwa-ke, ngokuthuthuka kwemithi nokuvela kwemithi nokunakekelwa okusha, lokhu kulindelwa kuye kwandiswa. Ngakho-ke, umuntu othola ukwelashwa okunconywe ngudokotela angaphila ngaphezu kweminyaka engama-30 futhi abe nempilo ejwayelekile, ngamacala amadoda aphila iminyaka engaphezu kwengu-50 enalesi sifo.

Izinkinga ezivame kakhulu

Izinkinga ezinkulu ezibangelwa iDuchenne muscular dystrophy yilezi:

• I-scoliosis enamandla;
• Kunzima ukuphefumula;
• Inyumoniya;
• Ukungakwazi ukusebenza kwenhliziyo;
• Ukukhuluphala ngokweqile noma ukungondleki.

Ngaphezu kwalokho, iziguli ezinale dystrophy zingase zithole ukukhubazeka kwengqondo okulingene, kepha lesi sici asixhumene nobude besikhathi noma ukuqina kwalesi sifo.

Yini ebangela lolu hlobo lwe-dystrophy

Njengesifo sezakhi zofuzo, iDuchenne's muscular dystrophy yenzeka lapho kuguquka okuthile kolunye zofuzo olubhekele ukudala umzimba ukuthi ukhiqize iphrotheni ye-dystrophin, isakhi sofuzo i-DMD. Le phrotheni ibaluleke kakhulu ngoba isiza amangqamuzana emisipha ukuthi ahlale enempilo ngokuhamba kwesikhathi, ewavikela ekulimaleni okubangelwa ukusikwa kwemisipha okujwayelekile nokuphumula.

Ngakho-ke, lapho ugu lwe-DMD luguqulwa, akhiqizwa amaprotheni anele futhi imisipha igcina ibuthaka nokulimala ngokuhamba kwesikhathi. Le phrotheni ibalulekile emisipheni elawula ukunyakaza, kanye nangensipha yenhliziyo.