Ingabe ngezinye izikhathi uzizwa sengathi imilenze nezingalo zakho ziya ngokuya ziba ndikindiki kancane kancane, noma amandla akho ayancipha? Ingabe uzizwa sengathi awusenamandla njengakuqala lapho uhamba, futhi mhlawumbe ulahlekelwa ngisho nokulinganisela kwakho? Nakuba lezi kuyizimpawu ongase ungazinaki kakhulu, ngezinye izikhathi kungaba nesizathu esingathi sína ngemuva kwazo. Yilokho okuyi-Primary Lateral Sclerosis, noma i-PLS ngamafuphi.

Ake sibone ukuthi lokhu kusho ukuthini?

Kalula nje, i-PLS isifo esiqhubekayo se-neuromuscular. Sibangela ukuba imisipha yakho ibe buthaka kancane kancane futhi/noma iqine. Izimpawu zivame ukuqala emilenzeni yakho. Ngokuhamba kwesikhathi, lobu buthakathaka nokuqina kungasakazekela kwezinye imisipha emzimbeni wakho.

Cabanga ngale ndlela: imisipha yethu isebenza njengedivayisi kagesi. Idinga ukwamukela 'ugesi', umyalezo ovela ebuchosheni, njengoba kufanele. Ku-PLS, amangqamuzana ezinzwa abizwa ngokuthi ama-Upper Motor Neurons (UMN), afana 'nezintambo' ezithwala le miyalezo, ayalimala.

Ngeshwa, okwamanje akukho ukwelashwa kwe-PLS. Ngakho-ke ukwelashwa kugxile kakhulu ekulawuleni izimpawu zakho nasekukusizeni ukuthi wenze imisebenzi yansuku zonke kalula. Isibonelo, ukukusiza ukuthi usebenzise udondolo noma i-walker.

I-PLS ivame kangakanani?

I-PLS empeleni iyisimo esingavamile kakhulu. Azikho izibalo okwamanje zokuthi bangaki abantu abayihlakulelayo.

Uyini umehluko phakathi kwe-PLS ne-ALS? Ingabe i-PLS ingaba yi-ALS?

Cishe uke wezwa nge-ALS, noma i-Amyotrophic Lateral Sclerosis. Zombili i-PLS ne-ALS ziyizifo zemizwa nemisipha. Kodwa kunomehluko obalulekile phakathi kwalokhu okubili.

Umehluko omkhulu ukuthi i-PLS ithinta kuphela ama-neuron akho aphezulu e-motor (ama-UMN) . Lawa yizinzwa ezithwala imiyalezo esuka ebuchosheni iye emgogodleni. Ku-ALS, la ma-UMN nawo ayathinteka, njengoba kunjalo nama-neuron aphansi e-motor (ama-LMN) , athwala imiyalezo esuka emgogodleni iye emisipheni.

Manje ungase uzibuze, 'Ngakho-ke, ingabe umuntu one-PLS angaba ne-ALS kamuva?' Yebo, kunethuba lokuthi ingase ivele. Ngezinye izikhathi izimpawu zokuqala ze-ALS zingafana ne-PLS. Uma unezimpawu ezithinta i-UMN kuphela, ungase utholakale une-PLS ekuqaleni. Kodwa uma kamuva uba nezimpawu ze-ALS ezithinta i-UMN kanye ne-LMN, udokotela wakho angase ashintshe ukuxilongwa kwakho kube yi-ALS.

Eqinisweni, amacala amaningi abukeka njenge-PLS angaba izimpawu zokuqala ze-ALS ezithinta i-UMN (`i-UMN-predominant ALS`). Yingakho odokotela bethi ukuze utholakale une-PLS, kudingeka ube nezimpawu okungenani iminyaka emithathu kuya kwemine .

Ziyini izimpawu ze-PLS?

Izimpawu ze-PLS zibonakala kancane kakhulu.Ngeke uzwe umehluko omkhulu ngokushesha.

Izimpawu ezingase zibonakale ezigabeni zokuqala:

Ekuqaleni ungase uzizwe izinto ezinjengalezi:

• Ukuqina kwemilenze. Kungase kuzwakale sengathi imilenze yakho inamathele.
• Imisipha emilenzeni yakho iba buthakathaka. Ungase uzizwe sengathi awusenamandla njengakuqala.
• Kuba nzima ukuhamba, noma ukugcina ibhalansi yakho. Ungase ukhubeke noma uwe.
• Kuvela ukuqaqamba kwemisipha noma ukuqaqamba okubuhlungu.

Izimpawu ezingase zivele njengoba isifo siqhubeka:

Njengoba lesi sifo siqhubeka, kungase kuvele izimpawu ezengeziwe ezifana nalezi:

• Imisipha yeminwe, izandla, kanye nezingalo nayo iyaqina futhi ibe buthakathaka. Kungaba nzima ukubamba izinto ezincane noma ukubhala.
• Ubunzima bokulawula ukuchama. Lokhu kungaholela ekuphuthumeni komchamo kanye nokungakwazi ukuzibamba.
• Ubuhlungu buvela emhlane ongezansi nasentanyeni.

Izimpawu ezingavamile ezithinta imisipha yolimi:

Lokhu akuvamile kakhulu, kodwa abanye abantu bangathinteka nemisipha yolimi lwabo. Uma lokhu kwenzeka:

• Inkulumo engacacile (i-dysarthria) iyenzeka. Kuba nzima ukuphimisela amagama ngokucacile.
• Ubunzima bokugwinya (i-dysphagia). Ungase uzizwe sengathi uyaminyana uma ugwinya ukudla noma isiphuzo.

Yini ebangela i-PLS?

Ezimweni eziningi, asikazi imbangela eqondile ye-PLS. Lelo yiqiniso.

Kodwa-ke, akuvamile kakhulu ukuba khona kohlobo lwe-PLS oluthinta izingane ezincane kanye nabantu abadala abasebasha (`i-juvenile primary lateral sclerosis`). Lokhu kubangelwa ushintsho ku-DNA yabo ngesikhathi sokukhulelwa.

Kwenzekani emizimbeni yethu ngenxa ye-PLS?

I-PLS yisimo se-neuromuscular. Ithinta kakhulu ama-motor neuron ebuchosheni bakho kanye ne-nerve fibers esukela kulawo ma-neuron iye emgogodleni. Lawa abizwa nangokuthi ama-upper motor neurons (UMN).

Cabanga nje, uma ufuna ukuhambisa umlenze wakho, ubuchopho bakho buthumela umlayezo. Lo mlayezo uhamba ngemicu yezinzwa uye kuma-neurons emgogodleni, bese usuka lapho uye emisipheni yokuzithandela emlenzeni wakho. Lapho imisipha ithola lo myalezo, iyanyakaza.

Ku-PLS, la maseli emizwa e-UMN ayafa kancane kancane (ukuwohloka). Bese, imisipha ayitholi imiyalezo edingekayo kahle, ngakho imisipha ayisebenzi njengoba kulindelekile.

Ingabe lesi isifo esizuzwe njengefa?

I-PLS ngokuvamile ayilona ifa. Lokhu kusho ukuthi ungayithola ngisho noma kungekho muntu emndenini wakho oke waba nayo. Esikhathini esiningi, yenzeka ngokungahleliwe, ngaphandle komlando womndeni walesi sifo.

Ubani osengozini enkulu yokuthola i-PLS?

I-PLS ingathinta noma ubani. Ivame ukutholakala ineminyaka engaba ngu-50. Kodwa-ke, ingathinta abantu abangaphansi kweminyaka engu-50, abantu abadala, ngisho nezingane. Ivame kakhulu kwabesilisa.

Yiziphi izinkinga ezingaba khona ngenxa ye-PLS?

Njengoba i-PLS iqhubeka, ungase ukuthole kunzima ukuhamba ngaphandle kosizo. Kungase kudingeke usebenzise induku, i-walker, noma isihlalo sabakhubazekile. Ngaphezu kwalokho, njengoba imisipha yakho ibuthakathaka, ungase ulahlekelwe ibhalansi yakho futhi uwe kalula. Uma wenza kanjalo, ungase ulimele.

I-PLS itholakala kanjani?

Udokotela uxilonga i-PLS ngemuva kokuhlolwa ngokomzimba, ukuhlolwa kwezinzwa, kanye nokunye ukuhlolwa okukhethekile. Bazobuyekeza izimpawu zakho futhi bazame ukuqeda ezinye izimo ezinezimpawu ezifanayo, njenge-ALS noma i-multiple sclerosis. Ukuhlolwa kungafaka:

• Ukuhlolwa kwegazi.
• Ukuhlolwa kwe-electrodiagnostic - Lokhu kuhlola ukuthi izinzwa zakho nemisipha yakho zisebenza kahle kangakanani. Izibonelo zifaka phakathi izifundo zokuqhuba izinzwa kanye nezifundo ze-needle electrode.
• Ukuhlolwa kwe-MRI (Magnetic Resonance Imaging) kobuchopho kanye nomgogodla.
• Ukuhlaziywa koketshezi lwe-cerebrospinal (CSF). Lokhu kwenziwa ngokuthatha inani elincane loketshezi emgogodleni wakho (ukubhoboza kwe-lumbar noma i-spinal tap) ukuze kubhekwe izinkinga ezithile eziphathelene nezifo ze-neuromuscular.

Yiziphi izindlela zokwelapha i-PLS?

Ukwelashwa kwe-PLS kuhloselwe ukukusiza ukuthi uphathe izimpawu zakho. Lokhu kungafaka:

• Imithi yokunciphisa ukuqina kwemisipha, ukuxhuga, kanye nobunzima bokugwinya.
• Ukwelashwa ngokomzimba ukunciphisa ubuthakathaka bemisipha, ukuthuthukisa ukuguquguquka kwemisipha, nokuthuthukisa ukunyakaza kwamalunga.
• Amadivayisi asiza ngemisebenzi yansuku zonke kanye nokuhamba ngezinyawo. Isibonelo, induku, inqola yokuhamba, noma isihlalo sabakhubazekile.
• Ukwelashwa ngenkulumo noma amadivayisi okusiza ngezinkinga zokukhuluma.

Imithi enikezwa izimpawu ze-PLS:

Eminye yemithi evame ukunqunyelwa izimpawu ze-PLS ihlanganisa:

• Ukuqina kwemisipha: `(Baclofen)` kanye `(Tizanidine)`
• Ukuqaqamba kwemisipha: `(Quinine)`
• Ukukhulula imisipha: `(Diazepam)`

Ingabe ikhona imiphumela emibi yokwelashwa?

Yebo, njengayo yonke imithi, lo ungaba nemiphumela emibi. Imiphumela emibi iyahlukahluka kuye ngomuthi. Ngakho-ke, kubaluleke kakhulu ukukhuluma nodokotela wakho ngemiphumela emibi engaba khona ngaphambi kokuqala noma yimuphi umuthi.

Ingabe i-PLS ingavinjelwa noma yelapheke?

Njengoba imbangela eqondile ye-PLS ingaziwa, okwamanje ayikho indlela yokuyivimbela ukuthi ingakhuli.

Futhi, ngokudabukisayo, okwamanje akukho ukwelashwa okungayelapha ngokuphelele i-PLS.

Ingakanani iminyaka yokuphila komuntu one-PLS? Yini angayilindela?

I-PLS ayithinti ngqo isikhathi sakho sokuphila. Umuntu one-PLS angaba nesikhathi sokuphila esijwayelekile, njengomuntu ongenaso lesi sifo.

Ukuthi izimpawu zakho zibonakala ngokushesha kangakanani nokuthi zikhula ngokushesha kangakanani kuyahlukahluka kuye ngomuntu nomuntu. Ngokuvamile, i-primary lateral sclerosis iqhubeka kancane kakhulu, eminyakeni edlule, ngezinye izikhathi amashumi eminyaka. Kodwa uma uzizwa izimpawu zakho ziba zimbi ngokuzumayo, bona udokotela wakho ngokushesha.

Imithi ingasiza ekunciphiseni izimpawu zakho futhi ikusize wenze imisebenzi yakho yansuku zonke ngaphandle kokuphazamiseka. Ungase uthande ukucabanga ngokusebenzisa udondolo, i-walker, noma isihlalo sabakhubazekile ukuze uzizwe uphephile futhi uqiniseka kakhudlwana lapho uhambahamba.

Kufanele ubonane nini nodokotela?

Uma ubona noma yiziphi izinguquko eziqhubekayo emisipheni yakho, njengokuqina noma ubuthakathaka, khuluma nodokotela wakho. Uma kutholakale ukuthi une-PLS, uma kukhona imithi oyiphuzayo ebangela izimpawu zakho zibe zimbi kakhulu, noma uma ubhekene nemiphumela emibi, tshela udokotela wakho ngalokho futhi.

Okubaluleke kakhulu: Uma uwela noma ulimele engozini, hamba uye ekamelweni lezimo eziphuthumayo ngokushesha.

Imibuzo okufanele uyibuze udokotela wakho

Uma une-PLS, uzoba nemibuzo eminingi okufanele uyibuze udokotela wakho. Isibonelo:

• Yimiphi imithi yokwelapha engasiza ekulawuleni izimpawu zami?
• Iyini imiphumela emibi yokwelashwa?
• Ngingazi kanjani ukuthi isimo sami sizoba sibi kakhulu ngokuhamba kwesikhathi?
• Yimiphi imithambo engingayenza ukuze ngigcine imisipha yami isebenza kahle?
• Ingabe ngidinga ukusebenzisa udondolo noma i-walker?

Ekugcineni, Umyalezo Wokuya Ekhaya

Ukuzivumelanisa nesimo esiqhubekayo esithinta ikhono lakho lokunyakaza umzimba wakho kungaba nzima kakhulu. Ezinye zezinto obuzijabulela ukuzenza zingaba nzima kancane kancane. Nakuba isifo se-primary lateral sclerosis (PLS) siqhubeka kancane kancane, singathinta impilo yakho yansuku zonke.

Uma lezi zinguquko zomzimba zithinta nemizwa yakho, ungathola impumuzo ngokukhuluma nochwepheshe wezempilo yengqondo .Akuyona into yokuhlazeka, abantu abaningi badinga usizo ngezikhathi ezinje.

Uma ubona ushintsho emandleni emisipha yakho, bonana nodokotela ngokushesha. Bangakunikeza imithi noma amadivayisi okunyakaza ukuze bakusize uzimele futhi ukhululeke kakhudlwana. Khumbula, awuwedwa kulolu hambo.

I- Primary lateral sclerosis, i-PLS, ubuthakathaka bemisipha, i-neuropathy, ama-motor neurons, i-ALS, izimpawu