I-Amyotrophic lateral sclerosis (ALS)

I-Amyotrophic lateral sclerosis, noma i-ALS, yisifo samaseli ezinzwa ebuchosheni, esiqwini sobuchopho nasentanjeni yomgogodla elawula ukunyakaza kwemisipha ngokuzithandela.

I-ALS yaziwa nangokuthi isifo seLou Gehrig.

Icala elilodwa kwezingu-10 ze-ALS kungenxa yokukhubazeka kwezakhi zofuzo. Imbangela ayaziwa kwezinye izimo eziningi.

Ku-ALS, amangqamuzana ezinzwa zemoto (ama-neuron) ayachitha noma afe, futhi awasakwazi ukuthumela imiyalezo ezicutshini. Lokhu kugcina kuholele ekutheni izicubu zomzimba zibe buthakathaka, zishwabane, futhi zingakwazi ukunyakazisa izingalo, imilenze nomzimba. Isimo siya ngokuya siba sibi. Lapho imisipha esendaweni yesifuba iyeka ukusebenza, kuba nzima noma kungenzeki ukuphefumula.

I-ALS ithinta cishe abantu abahlanu kwabangu-100 000 emhlabeni wonke.

Ukuba nelungu lomndeni elinofuzo kulesi sifo kuyingozi ye-ALS. Ezinye izingozi zifaka phakathi inkonzo yezempi. Izizathu zalokhu azicaci, kepha kungenzeka ukuthi zihlobene nokuchayeka kwimvelo kubuthi.

Izimpawu ngokuvamile azikhuli kuze kube ngemuva kweminyaka yobudala engama-50, kepha zingaqala kubantu abasha. Abantu abane-ALS balahlekelwa amandla emisipha nokuhlangana okugcina kubi kakhulu futhi kwenza kube nzima kubo ukwenza imisebenzi ejwayelekile njengokukhuphuka izitebhisi, ukuphuma esihlalweni, noma ukugwinya.

Ubuthakathaka bungathinta izingalo noma imilenze kuqala, noma amandla okuphefumula noma okugwinya. Njengoba lesi sifo siba sibi kakhulu, amaqembu emisipha amaningi aqala ukuba nezinkinga.

I-ALS ayithinti izinzwa (ukubona, ukuhogela, ukunambitha, ukuzwa, ukuthinta). Iningi labantu liyakwazi ukucabanga ngokujwayelekile, yize inani elincane likhula ukuwohloka komqondo, lidale izinkinga ngememori.

Ubuthakathaka bemisipha buqala engxenyeni eyodwa yomzimba, efana nengalo noma isandla, bese buya ngokuya buba bubi kuze kuholele kokulandelayo:

• Kunzima ukuphakamisa, ukukhuphuka izitebhisi, nokuhamba ngezinyawo
• Kunzima ukuphefumula
• Kunzima ukugwinya - ukuminyanisa kalula, ukukhipha amathe noma ukugoqana umlomo
• Ukwehla kwekhanda ngenxa yobuthakathaka bemisipha yentamo
• Izinkinga zokukhuluma, njengephethini yokukhuluma engasheshi noma engajwayelekile (ukuthopha kwamagama)
• Izinguquko zezwi, ukushaqeka

Okunye okutholakele kufaka:

• Ukucindezeleka
• Amajaqamba emisipha
• Ukuqina kwemisipha, okubizwa ngokuthi ukuqina
• Ukuphambana kwemisipha, okubizwa ngokuthi ama-fasciculations
• Ukwehla kwesisindo

Umhlinzeki wezokunakekelwa kwempilo uzokuhlola abuze ngomlando nezimpawu zakho zezokwelapha.

Ukuhlolwa komzimba kungakhombisa:

• Ubuthakathaka, obuvame ukuqala endaweni eyodwa
• Ukudlidliza kwemisipha, ukudumba, ukudikiza, noma ukulahleka kwezicubu zemisipha
• Ukudikiza kolimi (okuvamile)
• Izimo ezingejwayelekile
• Ukuhamba okunzima noma okungahambi kahle
• Ukunciphisa noma ukukhuphuka kwengqondo kumajoyini
• Kunzima ukulawula ukukhala noma ukuhleka (kwesinye isikhathi kubizwa ngokuthi ukungalawuleki okungokomzwelo)
• Ukulahlekelwa kwe-gag reflex

Ukuhlolwa okungenziwa kufaka phakathi:

• Ukuhlolwa kwegazi ukukhipha ezinye izimo
• Ukuhlola ukuphefumula ukubona ukuthi imisipha yamaphaphu iyathinteka yini
• Umgogodla womlomo wesibeletho i-CT noma i-MRI ukuze uqiniseke ukuthi asikho isifo noma ukulimala entanyeni, okungalingisa i-ALS
• I-Electromyography ukubona ukuthi iziphi izinzwa noma imisipha engasebenzi kahle
• Ukuhlolwa kofuzo, uma kunomlando womndeni we-ALS
• Inhloko ye-CT noma i-MRI ukukhipha ezinye izimo
• Ukugwinya izifundo
• Thepha umgogodla (ukubhoboza i-lumbar)

Alikho ikhambi elaziwayo le-ALS. Umuthi obizwa nge-riluzole usiza ukunciphisa izimpawu futhi usiza abantu ukuthi baphile isikhathi eside.

Kunemithi emibili esiza ukubambezela ukuqhubeka kwezimpawu futhi ingasiza abantu ukuthi baphile isikhathi eside kancane:

• Isi-Riluzole (Rilutek)
• I-Edaravone (Radicava)

Ukwelashwa ukulawula ezinye izimpawu kufaka:

• I-Baclofen noma i-diazepam ye-spasticity ephazamisa imisebenzi yansuku zonke
• I-Trihexyphenidyl noma i-amitriptyline yabantu abanezinkinga zokugwinya amathe abo

Ukwelashwa ngokomzimba, ukuvuselelwa, ukusetshenziswa kwezinsimbi noma izihlalo zabakhubazekile, noma ezinye izinyathelo kungadingeka ukusiza ngokusebenza kwemisipha nempilo ejwayelekile.

Abantu abane-ALS bathambekele ekunciphiseni isisindo. Ukugula uqobo kukhulisa isidingo sokudla namakhalori. Ngasikhathi sinye, izinkinga zokuklinyeka nokugwinya zenza kube nzima ukudla ngokwanele. Ukusiza ngokudla, ithubhu lingafakwa esiswini. Isazi sokudla esikhethekile ku-ALS singanikeza izeluleko ngokudla okunempilo.

Imishini yokuphefumula ifaka imishini esetshenziswa ebusuku kuphela, kanye nokungenisa umoya okuqhubekayo.

Imithi yokudangala ingadingeka uma umuntu one-ALS ezizwa edabukile. Kufanele futhi baxoxe ngezifiso zabo maqondana nomoya wokufakelwa nemindeni yabo kanye nabahlinzeki.

Ukwesekwa ngokomzwelo kubalulekile ekubhekaneni nalesi sifo, ngoba ukusebenza kwengqondo akuphazamiseki. Amaqembu afana ne-ALS Association angatholakala ukusiza abantu ababhekene nalesi sifo.

Ukusekelwa kwabantu abanakekela umuntu one-ALS nakho kuyatholakala, futhi kungasiza kakhulu.

Ngokuhamba kwesikhathi, abantu abane-ALS balahlekelwa amandla okusebenza nokuzinakekela. Ukufa kuvame ukwenzeka kungakapheli iminyaka emithathu kuya kwemihlanu yokuxilongwa. Cishe umuntu oyedwa kwabane usinda iminyaka engaphezu kwengu-5 ngemuva kokuxilongwa. Abanye abantu baphila isikhathi eside, kepha badinga usizo lokuphefumula kusuka kumshini wokuphefumula noma kwenye idivayisi.

Izinkinga ze-ALS zifaka:

• Ukuphefumula kokudla noma uketshezi (ukufisa)
• Ukulahleka kwekhono lokuzinakekela
• Ukwehluleka kwamaphaphu
• Inyumoniya
• Izilonda zengcindezi
• Ukwehla kwesisindo

Shayela umhlinzeki wakho uma:

• Unezimpawu ze-ALS, ikakhulukazi uma unomlando womndeni walesi sifo
• Wena noma omunye umuntu kutholakale ukuthi une-ALS futhi izimpawu ziya ngokuya ziba zimbi kakhulu noma kuvela izimpawu ezintsha

Ubunzima bokugwinya, ukuphefumula kanzima, kanye neziqephu ze-apnea yizimpawu ezidinga ukunakwa ngokushesha.

Ungahle uthande ukubona umeluleki wezofuzo uma unomlando womndeni we-ALS.

Isifo seLou Gehrig; ALS; Isifo se-neuron esiphezulu nesiphansi; Isifo se-motor neuron

• Isistimu yezinzwa emaphakathi nesistimu yezinzwa ezungezayo

UFearon C, uMurray B, uMitsumoto H. Ukuphazamiseka kwama-motor neurons aphezulu nangaphansi. Ku: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, ama-eds. I-Neurology kaBradley ekwenziweni kwemitholampilo. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2016: isahluko 98.

UShaw PJ, uCudkowicz ME. I-Amyotrophic lateral sclerosis nezinye izifo zemoto neuron. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 391.

van Es MA, Hardiman O, Chio A, et al. I-Amyotrophic lateral sclerosis. I-Lancet. 2017; 390 (10107): 2084-2098. I-PMID: 28552366 pubmed.ncbi.nlm.nih.gov/28552366/.