I-Amyotrophic lateral sclerosis (ALS)

I-Amyotrophic lateral sclerosis, okanye i-ALS, sisifo seeseli zemithambo-luvo kwingqondo, isiqu sobuchopho kunye nomqolo olawula intshukumo yokuzithandela yemisipha.

I-ALS ikwabizwa ngokuba sisifo sikaLou Gehrig.

Ityala elinye kwi-10 le-ALS kungenxa yesiphene semfuza. Isizathu asaziwa kwezinye iimeko ezininzi.

Kwi-ALS, iiseli zemithambo-luvo (iiseli zemithambo-luvo) ziyamosha okanye zife, kwaye azisenako ukuthumela imiyalezo kwizihlunu. Oku kukhokelela ekutyhafeni kwezihlunu, ukujija, kunye nokungakwazi ukuhambisa iingalo, imilenze kunye nomzimba. Imeko iya isiba mandundu. Xa izihlunu kwindawo yesifuba ziyeke ukusebenza, kuba nzima okanye kungenzeki ukuphefumla.

I-ALS ichaphazela phantse abantu aba-5 kwabali-100 000 ehlabathini lonke.

Ukuba nelungu losapho elinoluhlobo lwesifo sisifo esingumngcipheko we-ALS. Eminye imingcipheko ibandakanya inkonzo yasemkhosini Izizathu zoku azicacanga, kodwa zinokuhambelana nokuchazwa kwetyhefu kwindalo esingqongileyo.

Iimpawu zihlala zingakhuli de kube emva kweminyaka engama-50, kodwa zinokuqala kubantu abancinci. Abantu abane-ALS baphulukana namandla emisipha kunye nokulungelelanisa okuya kusiba mandundu kwaye kwenza ukuba bangakwazi ukwenza imisebenzi yesiqhelo njengokunyuka amanyathelo, ukuphuma esihlalweni, okanye ukuginya.

Ubuthathaka bunokuchaphazela iingalo okanye imilenze, okanye amandla okuphefumla okanye okuginya. Njengoko esi sifo siya sisiba mandundu, amaqela ezihlunu amaninzi avelisa iingxaki.

I-ALS ayichaphazeli izivamvo (ukubona, ukunuka, ukungcamla, ukuva, ukuchukumisa). Uninzi lwabantu luyakwazi ukucinga ngesiqhelo, nangona inani elincinci liphuhlisa isifo sengqondo esixhalabisayo, sibangela iingxaki kwimemori.

Ubuthathaka bemisipha buqala kwilungu elinye lomzimba, njengengalo okanye isandla, kwaye ngokuthe ngcembe uya usiba mbi de ukhokelele koku kulandelayo:

• Ubunzima bokuphakamisa, ukunyuka izinyuko, kunye nokuhamba
• Kunzima ukuphefumla
• Kunzima ukugwinya-ukukrwitshwa ngokulula, ukuntywila ngamathe, okanye ukubamba umlomo
• Ukuwa kwentloko ngenxa yobuthathaka bemisipha yentamo
• Iingxaki zentetho, ezinje ngenkqubo yokuthetha engacacanga okanye engaqhelekanga (ukutswina kwamagama)
• Ukutshintsha kwelizwi, ukurhawuzelela

Ezinye iziphumo zibandakanya:

• Uxinzelelo
• Iintlanzi zemisipha
• Ukuqina kwemisipha, ebizwa ngokuba kukungxama
• Izihlunu zemisipha, ezibizwa ngokuba zii-fasciculations
• Ukuhla ukusinda

Umboneleli wezempilo uya kukuvavanya kwaye akubuze ngembali yakho yezonyango kunye neempawu.

Uvavanyo lomzimba lunokubonisa:

• Ubuthathaka, obuhlala buqala kwindawo enye
• Ukungcangcazela kwemisipha, ukuphazamiseka, ukujija, okanye ukulahleka kwezihlunu zemisipha
• Ukutswina kolwimi (okuqhelekileyo)
• Ingqondo engaqhelekanga
• Ukuhamba ngokungqongqo okanye ukungahambi kakuhle
• Ukuhla kwehlise okanye ukunyusa amandla kumalungu
• Ubunzima bokulawula ukukhala okanye ukuhleka (ngamanye amaxesha kubizwa ngokuba kukungakwazi ukuzibamba)
• Ukuphulukana ne-gag reflex

Uvavanyo olunokwenziwa lunokubandakanya:

• Uvavanyo lwegazi ukulawula ezinye iimeko
• Uvavanyo lokuphefumla ukubona ukuba izihlunu zemiphunga ziyachaphazeleka
• Umlomo wesibeleko i-CT okanye i-MRI ukuze uqiniseke ukuba akukho sifo okanye ukwenzakala entanyeni, okunokulinganisa i-ALS
• I-Electromyography ukubona ukuba yeyiphi imithambo okanye izihlunu ezingasebenzi kakuhle
• Ukuvavanywa kwemvelo, ukuba kukho imbali yosapho ye-ALS
• Intloko ye-CT okanye i-MRI yokulawula ezinye iimeko
• Ukuginya izifundo
• Impompo yomqolo (i-lumbar puncture)

Akukho lunyango lwe-ALS. Iyeza elibizwa ngokuba yi-riluzole linceda ukucothisa iimpawu kwaye linceda abantu ukuba baphile kancinci.

Amayeza amabini afumanekayo anceda ukucothisa ukuqhubela phambili kweempawu kwaye anokunceda abantu ukuba baphile kancinci:

• Riluzole (Rilutek)
• Edaravone (Radicava)

Unyango lokulawula ezinye iimpawu lubandakanya:

• I-Baclofen okanye i-diazepam ye-spasticity ephazamisa imisebenzi yemihla ngemihla
• I-Trihexyphenidyl okanye i-amitriptyline yabantu abaneengxaki zokuginya amathe abo

Unyango lomzimba, ukubuyisela kwimeko yesiqhelo, ukusetyenziswa kwamabhanti okanye isitulo esinamavili, okanye amanye amanyathelo anokufuneka ukunceda ekusebenzeni kwemisipha kunye nakwimpilo ngokubanzi.

Abantu abane-ALS badla ukunciphisa umzimba. Isigulo ngokwaso sonyusa isidingo sokutya kunye neekhalori. Kwangelo xesha, iingxaki zokukrwitshwa nokuginya zenza kube nzima ukutya ngokwaneleyo. Ukunceda ukondla, ityhubhu inokufakwa esiswini. Ingcali yezidlo egxile kakhulu kwi-ALS inokunika iingcebiso ngokutya okusempilweni.

Izixhobo zokuphefumla zibandakanya oomatshini abasetyenziswa kuphela ebusuku, kunye nokungenisa umoya rhoqo.

Unyango kuxinzelelo lunokufuneka ukuba umntu one-ALS uziva elusizi. Kananjalo kuya kufuneka baxoxe ngeminqweno yabo malunga nomoya wokufakelwa okungengowabo kunye neentsapho zabo kunye nababoneleli.

Inkxaso yeemvakalelo ibalulekile ekujonganeni nesi sifo, kuba ukusebenza kwengqondo akuchaphazeleka. Amaqela afana noMbutho we-ALS anokufumaneka ukunceda abantu abahlangabezana nengxaki.

Inkxaso yabantu abakhathalela umntu one-ALS ikwafumaneka, kwaye kunokuba luncedo kakhulu.

Ixesha elingaphezulu, abantu abane-ALS baphulukana namandla okusebenza nokuzikhathalela. Ukufa kuhlala kwenzeka kwiminyaka emi-3 ukuya kwezi-5 yokufunyaniswa kwesifo. Phantse umntu omnye kwabane usinda ngaphezulu kweminyaka emi-5 emva kokuxilongwa. Abanye abantu baphila ixesha elide, kodwa badinga uncedo lokuphefumla kumatshini wokuphefumla okanye esinye isixhobo.

Iingxaki ze-ALS zibandakanya:

• Ukuphefumla kukutya okanye kwi-fluid (aspiration)
• Ukuphulukana nokukwazi ukuzikhathalela
• Ukungaphumeleli kwemiphunga
• Ukukrala kwemiphunga
• Izilonda zoxinzelelo
• Ukuhla ukusinda

Fowunela umnikezeli wakho ukuba:

• Unempawu ze-ALS, ngakumbi ukuba unembali yosapho kwesi sifo
• Wena okanye omnye umntu uye wafumanisa ukuba une-ALS kwaye iimpawu ziba mandundu okanye ukukhula kweempawu ezintsha

Ukwanda kobunzima bokuginya, ukuphefumla nzima, kunye neziqendu ze-apnea ziimpawu ezifuna ingqalelo kwangoko.

Unokufuna ukubona umcebisi wemfuza ukuba unembali yosapho ye-ALS.

Isifo seLou Gehrig; ALS; Isifo se-neuron esiphezulu nesisezantsi semoto; Isifo se-neuron

• Inkqubo ye-nervous central kunye ne-peripheral system

UFearon C, uMurray B, uMitsumoto H.Ukuphazamiseka kwee-motor neurons ezisezantsi nezisezantsi. Ku: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, ii-eds. I-Neurology kaBradley kwiKlinikhi yokuziqhelanisa. Umhla wesi-7. IPhiladelphia, PA: Elsevier; Ngo-2016: isahluko 98.

UShaw PJ, uCudkowicz ME. I-Amyotrophic lateral sclerosis kunye nezinye izifo ze-motor neuron. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 391.

van Es MA, Hardiman O, Chio A, okqhubekayo. Amyotrophic lateral sclerosis. ILancet. Ngo-2017; 390 (10107): 2084-2098. IINKCUKACHA: 28552366 pubmed.ncbi.nlm.nih.gov/28552366/.