I-Myasthenia gravis

I-Myasthenia gravis yisifo se-neuromuscular disorder. Izinkinga ze-Neuromuscular zifaka imisipha nezinzwa ezizilawulayo.

I-Myasthenia gravis ikholelwa ukuthi iyinhlobo yokuphazamiseka okuzenzakalelayo. Isifo sokuzivikela komzimba senzeka lapho amasosha omzimba ehlasela ngephutha izicubu ezinempilo. Ama-antibody amaprotheni enziwa amasosha omzimba omzimba lapho ethola izinto eziyingozi. Ama-antibody angakhiqizwa lapho amasosha omzimba ngephutha ethatha izicubu ezinempilo njengezinto eziyingozi, njengasesimweni se-myasthenia gravis. Kubantu abane-myasthenia gravis, umzimba ukhiqiza amasosha omzimba avimba amaseli emisipha ekutholeni imiyalezo (ama-neurotransmitters) avela kumaseli ezinzwa.

Kwezinye izimo, i-myasthenia gravis ixhunyaniswe nezicubu ze-thymus (isitho samasosha omzimba).

I-Myasthenia gravis ingathinta abantu nganoma yisiphi isikhathi. Kuvame kakhulu kwabesifazane abasebasha nakwabesilisa abadala.

I-Myasthenia gravis ibangela ubuthakathaka bemisipha yokuzithandela. Le yimisipha ongayilawula. Imisipha yokuzimela yenhliziyo kanye nomgudu wokugaya ukudla akuvamisile ukuthinteka. Ubuthakathaka bemisipha ye-myasthenia gravis buyaqina ngomsebenzi futhi buthuthuke ngokuphumula.

Lokhu buthakathaka kwemisipha kungaholela ezinhlotsheni ezahlukahlukene zezimpawu, kufaka phakathi:

• Ukuphefumula kanzima ngenxa yobuthakathaka bemisipha yodonga lwesifuba
• Ukuhlafuna noma ukugwinya ubunzima, kubangele ukuklinya kaningi, ukuklinya noma ukukhipha amathe
• Kunzima ukukhuphuka izitebhisi, ukuphakamisa izinto, noma ukukhuphuka endaweni yokuhlala
• Kunzima ukukhuluma
• Ikhanda elinciphisayo namajwabu amehlo
• Ukukhubazeka ebusweni noma ubuthakathaka bemisipha yobuso
• Ukukhathala
• Ukubola noma ukushintsha kwezwi
• Umbono ophindwe kabili
• Kunzima ukugcina iso lingaguquguquki

Umhlinzeki wezokunakekelwa kwempilo uzokwenza ukuhlolwa komzimba. Lokhu kufaka ukuhlolwa okuningiliziwe kwesistimu yezinzwa (izinzwa). Lokhu kungakhombisa:

• Ubuthakathaka bemisipha, nemisipha yamehlo ivame ukuthinteka kuqala
• Ukucabanga okujwayelekile nokuzwa (ukuzwa)

Ukuhlolwa okungenziwa kufaka phakathi:

• Ama-antibodies e-Acetylcholine receptor ahambisana nalesi sifo
• I-CT noma i-MRI scan esifubeni ukubheka isimila
• Ucwaningo lokuqhuba imizwa ukuhlola ukuthi amasiginali kagesi ashesha kangakanani ngokusebenzisa inzwa
• I-Electromyography (EMG) yokuhlola impilo yemisipha nezinzwa ezilawula imisipha
• Ukuhlolwa komsebenzi wePulmonary ukukala ukuphefumula nokuthi amaphaphu asebenza kahle kanjani
• Ukuhlolwa kwe-Edrophonium ukubona ukuthi lo muthi uziguqula yini izimpawu okwesikhashana

Alikho ikhambi elaziwayo le-myasthenia gravis. Ukwelashwa kungakuvumela ukuba ube nezikhathi ngaphandle kwezimpawu (ukuthethelelwa).

Izinguquko zendlela yokuphila zingakusiza uqhubeke nemisebenzi yakho yansuku zonke. Kungalandelwa okulandelayo:

• Ukuphumula usuku lonke
• Sebenzisa isichibi seso uma ukubona kabili kuyakhathaza
• Ukugwema ukucindezeleka nokuchayeka kokushisa, okungenza izimpawu zibe zimbi kakhulu

Imithi engabhalwa ifaka phakathi:

• I-Neostigmine noma i-pyridostigmine yokuthuthukisa ukuxhumana phakathi kwezinzwa nemisipha
• I-Prednisone nezinye izidakamizwa (njenge-azathioprine, cyclosporine, noma i-mycophenolate mofetil) ukucindezela ukuphendula kwamasosha omzimba uma unezimpawu ezinzima kanti eminye imithi ayisebenzi kahle.

Izimo ezibucayi ukuhlaselwa kobuthakathaka bemisipha yokuphefumula. Lokhu kuhlasela kungenzeka ngaphandle kwesixwayiso uma kuthathwa umuthi omningi noma omncane kakhulu. Lokhu kuhlasela kuvame ukuhlala isikhathi esingaphezu kwamasonto ambalwa. Ungadinga ukuthi ungeniswe esibhedlela, lapho ungadinga khona usizo lokuphefumula ngomshini wokuphefumula.

Inqubo ebizwa nge-plasmapheresis nayo ingasetshenziswa ukusiza ukuqeda inkinga. Le nqubo ihilela ukususa ingxenye ecacile yegazi (i-plasma), equkethe amasosha omzimba. Lokhu kufakwa esikhundleni se-plasma enikelwe engenawo amasosha omzimba, noma nolunye uketshezi. I-Plasmapheresis ingasiza futhi ekunciphiseni izimpawu zamasonto amane kuya kwayisithupha futhi ivame ukusetshenziswa ngaphambi kokuhlinzwa.

Umuthi obizwa nge-intravenous immunoglobulin (IVIg) nawo ungasetshenziswa

Ukuhlinzwa ukususa i-thymus (i-thymectomy) kungaholela ekuxolelweni unomphela noma ngaphansi kwesidingo semithi, ikakhulukazi uma kukhona isimila.

Uma unezinkinga zamehlo, udokotela wakho angase asikisele ama-lens prism ukuze athuthukise umbono. Ukuhlinzwa kungabuye kunconywe ukwelapha imisipha yakho yamehlo.

Ukwelashwa ngokomzimba kungasiza ukugcina amandla emisipha yakho. Lokhu kubaluleke kakhulu emisipheni esekela ukuphefumula.

Eminye imithi ingaba zimbi izimpawu futhi kufanele igwenywe. Ngaphambi kokuthatha noma imuphi umuthi, buza udokotela wakho ukuthi kulungile yini ukuthi uwuphuze.

Unganciphisa ukucindezela kokugula ngokujoyina iqembu le-myasthenia gravis. Ukwabelana nabanye abanokuhlangenwe nakho okuvamile nezinkinga kungakusiza ungazizwa uwedwa.

Alikho ikhambi, kepha ukuxolelwa kwesikhathi eside kungenzeka. Kungadingeka ukhawulele imisebenzi ethile yansuku zonke. Abantu abanezimpawu zamehlo kuphela (i-ocular myasthenia gravis), bangakhula i-myasthenia ejwayelekile ngokuhamba kwesikhathi.

Owesifazane one-myasthenia gravis angakhulelwa, kepha ukunakekelwa ngaphambi kokubeletha kubalulekile. Ingane ingaba buthakathaka futhi idinge imishanguzo amasonto ambalwa izelwe, kepha imvamisa ayizukuthola isifo.

Lesi simo singadala izinkinga zokuphefumula ezisongela impilo. Lokhu kubizwa ngenkinga ye-myasthenic.

Abantu abane-myasthenia gravis basengozini enkulu yezinye izifo ezizimele, njenge-thyrotoxicosis, i-rheumatoid arthritis, ne-systemic lupus erythematosus (lupus).

Shayela umhlinzeki wakho wezokunakekelwa kwezempilo uma uthuthukisa izimpawu ze-myasthenia gravis.

Iya egumbini labezimo eziphuthumayo noma ushayele inombolo yendawo ephuthumayo (efana ne-911) uma unenkinga yokuphefumula noma unenkinga yokugwinya.

Isifo se-Neuromuscular - myasthenia gravis

• Imisipha yangaphandle engaphezulu
• I-Ptosis - ukujula kwejwabu leso
• Isistimu yezinzwa emaphakathi nesistimu yezinzwa ezungezayo

I-Chang CWJ. I-Myasthenia gravis ne-Guillain-Barré syndrome. Ku: Parrillo JE, Dellinger RP, ama-eds. Imithi Yokunakekelwa Okubalulekile: Izimiso Zokuxilongwa Nokuphathwa Kwabantu Abadala. Umhlaka 5. IPhiladelphia, PA: Elsevier; 2019: isahluko 61.

ISanders DB, iGuptill JT. Ukuphazamiseka kokudluliswa kwe-neuromuscular. Ku: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, ama-eds. I-Neurology kaBradley ekwenziweni kwemitholampilo. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2016: isahluko 109.

USanders DB, uWolfe GI, uBenatar M, et al. Umhlahlandlela wokuvumelana kwamazwe omhlaba wokuphathwa kwe-myasthenia gravis: isifinyezo esiphezulu. Neurology. 2016; 87 (4): 419-425. I-PMID: 27358333 www.ncbi.nlm.nih.gov/pubmed/27358333.