Unyango lwe-nemaline myopathy

Umxholo

• Iimpawu ze-nemaline myopathy
• Iimpawu zokuphucula i-nemaline myopathy
• Iimpawu zokuba mandundu kwemaline myopathy

Unyango lwe-nemaline myopathy kufuneka lukhokelwe ngugqirha wabantwana, kwimeko yosana nomntwana, okanye ugqirha wamathambo, kwimeko yomntu omkhulu, eyenziwayo ingekunyanga esi sifo, kodwa ukukhulula nokunyanga iimpawu, ukuphucula umgangatho wobomi.

Ngokwesiqhelo, unyango luqaliswa ngeeseshoni ze-physiotherapy ukunceda ukomeleza izihlunu ezibuthathaka ngokwenza imithambo ethile ehlengahlengiswe ngumzimba womzimba.

Ukongeza, kwaye kuxhomekeke kwiimpawu ezinokuthi zivele, unyango lunokwenziwa kwakhona:

• Ukusetyenziswa kwe-CPAP: sisixhobo esinemaski esetyenziswe kwimeko ezimodareyithayo nezinzima ukuququzelela ukuphefumla, ngakumbi ngexesha lokulala. Funda ngakumbi kwi: CPAP;
• Ukusetyenziswa kwesihlalo esinamavili: Kuyimfuneko kwiimeko ze-nemaline myopathy ebangela ubunzima ekuhambeni ngenxa yobuthathaka bemisipha yomlenze;
• Ukubekwa kwetyhubhu ye-gastrostomy: Iqukethe ityhubhu encinci efakwe ngqo kwisisu esivumela ukondla kwiimeko ezinzima kakhulu;
• Ukungenwa kwee-antibiotics: zisetyenziswa kwezinye iimeko ukunyanga izifo zokuphefumla, ezinje nge-pneumonia, exhaphake ngenxa yeengxaki zokuphefumla ezibangelwa yi-myopathy.

Kwiimeko ezinzima kakhulu, kunokuba yimfuneko ukuhlala esibhedlele ukuze wenze unyango olufanelekileyo kwaye uphephe iingxaki ezinzulu, ezinjengokubanjwa ngokuphefumla, okubeka ubomi besigulane engozini.

Iimpawu ze-nemaline myopathy

Iimpawu eziphambili ze-nemaline myopathy zibandakanya:

• Ubuthathaka bemisipha, ngakumbi ezingalweni nasemilenzeni;
• Ubunzima bokuphefumla okanye ukugwinya;
• Ukulibaziseka kophuhliso;
• Kunzima ukuhamba.

Ukongeza kwezi mpawu, kuqhelekile ukuba ezinye izinto zibonakale, ezinje ngobuso obucekeceke, umzimba omxinwa, umlomo ovulekileyo, inyawo elingenanto, isifuba esinzulu kunye nokukhula kwescoliosis okanye i-osteoporosis.

Iimpawu zihlala zibonakala kwakamsinya emva kokuzalwa kuba sisifo semfuza, kodwa kwezinye iimeko, iimpawu zokuqala zisenokukhula kuphela ebudaleni.

O Uxilongo lwe-myal nemopathy kwenziwa nge-biopsy yemisipha xa kukho iimpawu zokukrokrela isifo, ngakumbi xa ulibaziseko lokukhula kunye nobuthathaka obuqhubekayo bemisipha buvela.

Iimpawu zokuphucula i-nemaline myopathy

Akukho zimpawu zokuphucula i-nemaline myopathy, kuba esi sifo asiphucuki. Nangona kunjalo, iimpawu zinokulungiswa ngonyango, ukuvumela umgangatho wobomi obungcono.

Iimpawu zokuba mandundu kwemaline myopathy

Iimpawu zokonyuka kweemaline myopathy zinxulumene neengxaki, ezinje ngokusuleleka kunye nokubanjwa kokuphefumla, kwaye ke zibandakanya umkhuhlane ongaphezulu kwe-38ºC, ubunzima bokuphefumla, ukuphefumla nzulu, iminwe eluhlaza kunye nobuso.