Ukukhubazeka kwemisipha

I-Muscular dystrophy iyiqembu lokuphazamiseka okuzuzwe njengefa okubangela ukuba buthakathaka kwemisipha kanye nokulahleka kwezicubu zemisipha, eziya ziba zimbi ngokuhamba kwesikhathi.

Ama-dystrophies emisipha, noma i-MD, ayiqembu lezimo ezizuzwe njengefa. Lokhu kusho ukuthi badluliselwa emindenini. Zingavela ebuntwaneni noma ebudaleni. Kunezinhlobo eziningi ze-muscular dystrophy. Kubandakanya:

• I-Becker dystrophy yemisipha
• I-Duchenne muscular dystrophy
• U-Emery-Dreifuss muscular dystrophy
• I-Facioscapulohumeral dystrophy yemisipha
• I-limb-bhande imisipha yokukhubazeka
• I-Oculopharyngeal muscular dystrophy
• I-Myotonic dystrophy yemisipha

I-dystrophy yemisipha ingathinta abantu abadala, kepha izinhlobo ezinzima kakhulu zivame ukwenzeka zisencane.

Izimpawu ziyahlukahluka phakathi kwezinhlobo ezahlukahlukene ze-muscular dystrophy. Yonke imisipha ingathinteka. Noma, amaqembu athize kuphela emisipha angathinteka, njengalawo azungeze i-pelvis, ihlombe noma ubuso. Ubuthakathaka bemisipha buya buba bucayi futhi izimpawu zingafaka:

• Ukuthuthukiswa okulibazisekile kwamakhono emisipha yemisipha
• Kunzima ukusebenzisa iqembu elilodwa noma amaningi emisipha
• Ukumunca amathe
• Ukudonsa ijwabu leso (ptosis)
• Ukuwa njalo
• Ukuphelelwa amandla emisipheni noma eqenjini lezicubu njengomuntu omdala
• Ukulahleka ngosayizi wemisipha
• Izinkinga zokuhamba (ukubambezeleka ukuhamba)

Ukukhubazeka kwengqondo kubakhona kwezinye izinhlobo ze-muscular dystrophy.

Ukuhlolwa ngokomzimba nomlando wakho wezokwelapha kuzosiza umhlinzeki wezokunakekelwa kwezempilo ukuthi anqume uhlobo lwe-dystrophy yemisipha. Amaqembu emisipha akhethekile athintwa yizinhlobo ezahlukene ze-muscular dystrophy.

Ukuhlolwa kungakhombisa:

• Umgogodla ogobile ngokungajwayelekile (scoliosis)
• Izinkontileka ezihlangene (i-clubfoot, uzipho-isandla, noma abanye)
• Ithoni yemisipha ephansi (hypotonia)

Ezinye izinhlobo ze-muscular dystrophy zibandakanya imisipha yenhliziyo, ibangele i-cardiomyopathy noma isigqi senhliziyo esingajwayelekile (i-arrhythmia).

Imvamisa, kukhona ukulahleka kwemisipha (ukumosha). Lokhu kungaba nzima ukukubona ngoba ezinye izinhlobo zemisipha dystrophy zidala inqwaba yamafutha nezicubu ezixhuma ezenza imisipha ibonakale inkulu. Lokhu kubizwa ngokuthi i-pseudohypertrophy.

I-biopsy yemisipha ingasetshenziselwa ukuqinisekisa ukuxilongwa. Kwezinye izimo, ukuhlolwa kwegazi le-DNA kungaba yikho konke okudingekayo.

Ezinye izivivinyo zingafaka:

• Ukuhlolwa kwenhliziyo - i-electrocardiography (ECG)
• Ukuhlolwa kwemizwa - ukuqhutshwa kwemizwa kanye ne-electromyography (EMG)
• Ukuhlolwa komchamo nokuhlolwa kwegazi, kufaka phakathi izinga le-CPK
• Ukuhlolwa kofuzo kwezinye izinhlobo ze-muscular dystrophy

Akukho ukwelashwa okwaziwayo kwama-dystrophies ahlukahlukene emisipha. Umgomo wokwelashwa ukulawula izimpawu.

Ukwelashwa ngokomzimba kungasiza ekugcineni amandla nokusebenza kwemisipha. Izinsimbi zomlenze nesihlalo esinamasondo kungathuthukisa ukuhamba nokuzinakekela. Kwezinye izimo, ukuhlinzwa emgogodleni noma emilenzeni kungasiza ekuthuthukiseni ukusebenza.

Ama-Corticosteroids athathwe ngomlomo kwesinye isikhathi anikezwa izingane ezinama-dystrophies athile emisipha ukuzigcina zihamba isikhathi eside ngangokunokwenzeka.

Umuntu kufanele asebenze ngangokunokwenzeka. Awukho umsebenzi nhlobo (onjenge bedrest) ongenza lesi sifo sibe sibi kakhulu.

Abanye abantu abanobuthakathaka bokuphefumula bangazuza kumadivayisi okusiza ukuphefumula.

Unganciphisa ukucindezela kokugula ngokujoyina iqembu lokwesekwa lapho amalungu abelana ngokuhlangenwe nakho okuvamile nezinkinga.

Ubukhulu bokukhubazeka buxhomeke ohlotsheni lwe-muscular dystrophy. Zonke izinhlobo zemisipha yokuwohloka kwemisipha ziya ngokuya ziba zimbi, kepha ukuthi lokhu kwenzeka ngokushesha okungakanani kuyehluka kakhulu.

Ezinye izinhlobo ze-muscular dystrophy, njengeDuchenne muscular dystrophy kubafana, ziyabulala. Ezinye izinhlobo zidala ukukhubazeka okuncane futhi abantu banempilo ejwayelekile.

Shayela umhlinzeki wakho uma:

• Unezimpawu zokuwohloka kwemisipha.
• Unomlando womuntu siqu noma womndeni we-muscular dystrophy futhi uhlela ukuba nezingane.

Ukwelulekwa ngofuzo kuyelulekwa lapho kunomlando womndeni we-muscular dystrophy. Abesifazane kungenzeka bangabi nazimpawu, kepha basaphatha isakhi sokuphazamiseka. I-Duchenne muscular dystrophy ingatholwa ngokunemba okungaba ngu-95% ngezifundo zofuzo ezenziwa ngesikhathi sokukhulelwa.

I-myopathy eyifa MD

• Imisipha yangaphandle engaphezulu
• Imisipha yangaphakathi ejulile
• Ama-tendon nemisipha
• Imisipha yomlenze engezansi

I-Bharucha-Goebel DX. Ama-dystrophies emisipha. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Incwadi kaNelson Yezingane. Umhlaka 21. IPhiladelphia, PA: Elsevier; 2020: isahluko 627.

Selcen D. Izifo zemisipha. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 393.