Cabanga nje, ngokuzumayo uba nobunzima bokuhamba, ulahlekelwe yibhalansi yakho, futhi uzizwe unesizungu bese uwa lapho usukuma uhleli. Kuvamile ukuzizwa wesaba kakhulu lapho lezi zinto ziqala ukwenzeka ngokuzumayo. Nakuba ngezinye izikhathi singazinaki kakhulu lezi, zingaba izimpawu zokuqala zezifo ezithile ezingavamile. Namuhla sizokhuluma ngesifo esingavamile, kodwa esibi kakhulu sezinzwa. Leso yi- Multiple System Atrophy , isifo thina odokotela neziguli esisibiza ngokuthi (MSA) ngamafuphi. Nakuba lesi kuyisihloko esiyinkimbinkimbi kancane, sizokhuluma ngaso kalula, ngendlela ongayiqonda.
Kalula nje, iyini le MSA?
I-MSA yisifo esingavamile sezinzwa lapho izingxenye zobuchopho bethu ziba buthaka kancane kancane futhi zife. Ngokuhamba kwesikhathi, amakhono omzimba kanye nemisebenzi elawulwa yilezi zingxenye zobuchopho ezonakele iyanyamalala kancane kancane. Uma sikhuluma iqiniso, lesi yisimo esidabukisayo kakhulu, ngoba lesi sifo sigcina siphelile ngokufa.
Esikhathini esidlule, odokotela babebiza leli qoqo lezimpawu ngamagama amathathu. Lawa kwaku:
- I-Shy-Drager syndrome
- Ukuwohloka kwe-olivopontocerebellar okungahleliwe
- Ukuwohloka kwe-Striatonigral
Kodwa kamuva, abacwaningi baqaphela ukuthi lezi zifo ezintathu zinezici eziningi ezifanayo. Ngakho-ke, bazihlanganisa zonke baziqamba ngokuthi "i-Multiple System Atrophy" (MSA). Igama elithi "Multiple System" lisho "izinhlelo eziningana." Lokhu kusho ukuthi lesi sifo sithinta izinhlelo eziningana emzimbeni wethu. Izimpawu zincike ekutheni yiziphi izingxenye zobuchopho ezilimele. Yingakho umuntu ngamunye eba nenhlanganisela ehlukile yezimpawu.
Kamuva, lesi sifo sahlukaniswa kabanzi, ikakhulukazi ngokusekelwe ezimpawini esizivezayo. Ngakho-ke, kunezinhlobo ezimbili ze-MSA.
| Uhlobo lwe-MSA | Incazelo kanye nezici eziyinhloko |
|---|---|
| I-MSA-C | U-'C' lapha umele 'i-Cerebellar'. I-cerebellum yingxenye yobuchopho eqondisa ukunyakaza kwethu. Izimpawu eziyinhloko zalolu hlobo ukulahlekelwa yibhalansi (i-ataxia) . Lokhu kusho ukuthi asikwazi ukulawula izitho zethu ngendlela efanele, njengalapho sihamba. Ngaphezu kwalokho, kunezinkinga ngemisebenzi yomzimba ezenzakalelayo (ukungasebenzi kahle kwe-autonomic) kanye nokuwa njalo. |
| I-MSA-P | I-'P' lapha imele i-'Parkinsonism'. Lolu hlobo lwe-MSA-C lunezimpawu ezifana kakhulu nesifo sikaParkinson. Isibonelo, ukuthuthumela, ukunyakaza okuhamba kancane, kanye nokuqina. Nakuba lezi zimpawu zikaParkinson ziyizona eziyinhloko ezigabeni zokuqala, ngokuhamba kwesikhathi, izimpawu ezifana nezezinye i-MSA-C (ukulahlekelwa ibhalansi, izinkinga ngemisebenzi ezenzakalelayo) nazo zingavela. |
Ubani osengozini enkulu yokuthola i-MSA?
I-MSA ivame ukuthinta abantu abadala, ikakhulukazi ngemva kweminyaka engama-30. Izimpawu zivame ukuvela phakathi kweminyaka engama-50 nengama-59. Lesi sifo singathinta noma ubani, kungakhathaliseki ubulili.
Lesi yisifo esingavamile kakhulu. Ngokwezibalo, kunezimo ezintsha ezingu-0.6 kuya ku-0.7 kuphela kubantu abayi-100,000 ngonyaka. Lokhu kusho ukuthi lesi akusona isifo esivamile emphakathini wethu.
Isifo se-MSA sithinta kanjani imizimba yethu?
I-MSA ibangela ukuthi amangqamuzana afe ezingxenyeni ezahlukene zobuchopho. Izimpawu zincike ekutheni yiziphi izingxenye zobuchopho ezilimele. Izingxenye eziyinhloko zobuchopho ezithintekile yilezi:
- I-Basal Ganglia: Lezi zitholakala phakathi kobuchopho. Zifana nesikhungo esixhumanisa izingxenye ezahlukene zobuchopho. Kungale ngxenye lapho izingxenye ezahlukene zobuchopho zihlangana khona futhi zisebenze ndawonye.
- I-Brainstem: Yilapho izinqubo ezizenzakalelayo emzimbeni wethu ezibalulekile ukuze siphile zilawulwa khona. Isibonelo, izinto ezifana nokuphefumula, ukushaya kwenhliziyo, kanye nomfutho wegazi. Lezi zinto zenzeka ngokuzenzakalelayo ngaphandle kokuthi sicabange ngazo, akunjalo? Lesi yisikhungo sokulawula.
- I-Cerebellum: Lokhu kutholakala ngemuva kwekhanda, eduze kwesisekelo. Lena yingxenye eyinhloko yobuchopho bethu eqondisa ukunyakaza kwemizimba yethu futhi igcine ibhalansi yethu. Ngaphezu kwalokho, ucwaningo seluthole ukuthi le ngxenye nayo ihilelekile emizweni yethu nasekuthatheni izinqumo.
Ngakho-ke, uma lezi zingxenye zobuchopho zonakele, zonke izinqubo ezilawulwa yilezo zingxenye ziqala ukuba yinkinga. Isibonelo, lapho `(i-brainstem)` yonakele, kuba nezinkinga ezinkulu ngezinqubo ezizenzakalelayo njengomfutho wegazi.
Yiziphi izimpawu eziyinhloko ze-MSA?
Ku-MSA, ezinye izimpawu zivamile kuzo zombili izinhlobo. Ngaphezu kwalokho, kunezici ezithile zohlobo ngalunye. Isici esiyinhloko esivamile kuzo zombili izinhlobo ukungasebenzi kahle kokuzimela . Kalula nje, izinqubo ezenzeka ngokuzenzakalelayo emzimbeni wethu azisebenzi kahle.
Izimpawu Zokungasebenzi Kahle Kokuzilawula
- Ukucindezeleka Okungapheli Kwegazi: Lesi yisibonakaliso sokuqala abantu abaningi abasibonayo. Uma usukuma ngokuzumayo uhleli noma ulele phansi , umfutho wegazi wakho uyehla ngokuzumayo . Lokhu kungabangela isiyezi, ukubona okufiphele, ngisho nokuquleka.
- Ukungakwazi ukulawula umchamo nendle: Ukungakwazi ukuzibamba komchamo kanye nokungakwazi ukuzibamba indle kungenzeka ngesikhathi ongasilindele.
- Ukungasebenzi kahle kocansi: Izinkinga ezifana nokungasebenzi kahle kobulili, ikakhulukazi kwabesilisa, zenzeka ngesikhathi socansi.
- Izinkinga zokulala: Ikakhulukazi, isimo esibizwa ngokuthi "(REM) sleep behavior disorder" singabonakala. Kulokhu, uma uphupha, ushukumisa umzimba wakho, umemeza, futhi ugoqa izitho zakho ngenxa yamaphupho akho.
- Ukwehla kokujuluka (i-Anhidrosis): Amandla omzimba okujuluka ancipha kakhulu.
- Izimpawu ezifana nezinkinga zokubona, umlomo owomile, i-sleep apnea, kanye nokuqunjelwa nazo zivamile.
Okubalulekile, lokhu kungasebenzi kahle kwe-autonomic kungenzeka ezinyangeni, noma ngisho neminyaka, ngaphambi kokuqala kwezimpawu zokunyakaza (njengokuphazamiseka kokuhamba kanye nokuthuthumela). Lokhu kwenzeka phakathi kuka-20% no-75% weziguli ze-MSA.
Izici zengqondo nezingokomzwelo
Cishe ingxenye yesithathu yeziguli ze-MSA ihlangabezana nezinkinga ngekhono lazo lokucabanga nokugxila. Ziphinde zibe nobunzima bokulawula imizwa yazo, okungaholela ezinkingeni ezahlukahlukene zempilo yengqondo.
- Ukukhathazeka
- Ukucindezeleka
- Ukukhala noma ukuhleka ngokungafanele
- Ukuhlaselwa uvalo
- Imicabango yokuzilimaza noma yokuzibulala
Izimpawu Ezihlobene Nokunyakaza
Lezi zici ziyahlukahluka kuye ngezinhlobo ezimbili ze-MSA esixoxe ngazo ekuqaleni.
| Izici ze-MSA-C (Cerebellar) | Izimpawu ze-MSA-P (Parkinsonism) |
|---|---|
| Uphawu oluyinhloko yi -ataxia , okusho ukulahlekelwa ukuhambisana. | Izimpawu eziyinhloko yi -Parkinsonism , okusho izimpawu ezifana nesifo sikaParkinson. |
| - Ukunyakaza okungalawuleki nokungahlelekile kwamalungu. | - Ukunyakaza kuhamba kancane kakhulu (bradykinesia) . |
| - Ukuthuthumela okukhulu kwamalungu lapho uzama ukwenza okuthile (Ukuthuthumela kwesenzo). | - Ukuzizwa uqinile futhi uqinile emzimbeni, kanye nesimo somzimba esibonakala sengathi sigobile phambili. |
| - Ukuhamba ngezinyawo ziqhelelene ngendlela engavamile, njengokuhamba okuguquguqukayo. | - Ukuwa njalo lapho uhamba. |
| - Ukuqhaqhazela kwamehlo okungalawuleki kanye nokunyakaza (i-nystagmus) . | - Ukungakhulumi kahle nokungakhulumi kahle amagama uma ukhuluma. |
Yini ebangela i-MSA?
Imbangela eqondile ye-MSA ayikakaziwa . Kodwa-ke, ososayensi basola ukuthi ibangelwa iphrotheni ebizwa ngokuthi i-alpha-synuclein . Le phrotheni kucatshangwa ukuthi iqoqana ngendlela engavamile ezingxenyeni ezahlukene zobuchopho, ibangele umonakalo emaseli obuchopho. Ngokumangazayo, le phrotheni efanayo nayo kusolwa ukuthi iyimbangela yesifo sikaParkinson.
Amaprotheni abalulekile ekusebenzeni kwemizimba yethu. Kodwa-ke, uma lawa maprotheni ehlangana ezindaweni ezingafanele, ngendlela engafanele, angalimaza amaseli. Yilokho okwenzekayo ku-MSA.
Ucwaningo lusaqhubeka mayelana nokuthi kungani le phrotheni ye-alpha-synuclein iqoqana ebuchosheni. Kusolwa ukuthi kungenzeka ukuthi kungenxa yokuguquka kwezakhi zofuzo. Ikakhulukazi, kunobufakazi bokuthi uhlobo lwe-MSA-C lungadluliselwa kusuka esizukulwaneni kuya kwesinye ngezinga elithile. Kodwa-ke, akukho ukuxhumana okunjalo kwezakhi zofuzo okutholakale okwamanje kohlobo lwe-MSA-P.
Okubalulekile: I-MSA ayisona isifo esithathelwanayo. Ayidluliselwa kusuka komunye umuntu iye komunye nganoma iyiphi indlela.
Itholakala kanjani i-MSA?
Lena yingxenye eyinselele kakhulu yalesi sifo. Yiyona ndlela kuphela yokuqinisekisa i-MSA ngokuqiniseka okungu-100%.ukuhlola izicubu zobuchopho ngemva kokufa komuntu. Isizathu salokhu ukuthi okwamanje abukho ubuchwepheshe bokubona ukuthi iphrotheni ye-alpha-synuclein ibekwe yini ebuchosheni bomuntu ophilayo.
Kodwa-ke, ngesikhathi isiguli sisaphila, odokotela bangasola lesi sifo. Basebenzisa izinto ezifana nezimpawu, umlando wezokwelapha wesiguli, umlando womndeni, kanye nokusabela kwezinye izindlela zokwelapha. Ngokuvamile, odokotela bangase baqale baxilonge isifo sikaParkinson. Kodwa-ke, njengoba ezinye izimpawu zikhula ngokuhamba kwesikhathi, noma lapho imithi kaParkinson iyeka ukusebenza, ukuxilongwa kungadingeka kushintshwe kube yi-MSA.
Umehluko obalulekile phakathi kwe-MSA nesifo sikaParkinson
| Isici | Ukuwohloka Kwesistimu Eningi (i-MSA) | Isifo sikaParkinson |
|---|---|---|
| Isivinini sokusabalala kwesifo | Izimpawu ziya ziba zimbi kakhulu ngokushesha okukhulu . | Lesi sifo siqhubeka kancane kancane . |
| Ubuthakathaka obuzenzakalelayo | Izinkinga ezinkulu (isib. izinkinga zomfutho wegazi) zivame ukuvela phakathi nonyaka wokuqala wokugula. | Kungathatha iminyaka ukuthi lezi zimpawu zivele. |
| Ukuthuthumela | Ukuthuthumela kuncane, mhlawumbe akukho nhlobo . | Ukuthuthumela okwenzeka lapho umuntu ephumule kuyisibonakaliso esikhulu . |
| Impendulo ku-Levodopa | Bambalwa kakhulu abasabela kulo muthi.Noma cha nhlobo. | Isabela kahle kakhulu kulo muthi. |
Ukuhlolwa kokuxilonga
Zimbalwa kakhulu izivivinyo ezingaxilonga i-MSA ngqo. Ngokuvamile, kwenziwa uchungechunge lwezivivinyo ukuze kukhishwe ezinye izimo futhi kuqoqwe ubufakazi bokusekela izinsolo zokuthi i-MSA ingaba yimbangela.
- Iskeni se-MRI (Magnetic Resonance Imaging): Lokhu ngezinye izikhathi kungabonisa umonakalo ezingxenyeni zobuchopho. Ku-MSA-C, ikakhulukazi, iphethini ebizwa ngokuthi uphawu lwe-"hot cross bun" ingabonakala engxenyeni yobuchopho. Ibukeka njenge-hot cross bun. Kodwa-ke, lolu phawu lungabonakala nakwezinye izifo, ngakho-ke akuyona indlela eqondile yokuthola i-MSA.
- Ukuhlolwa kwezakhi zofuzo: Lokhu kungahlola izinguquko zezakhi zofuzo ezihlobene neprotheni ye-alpha-synuclein.
- Ukuhlolwa kwesikhumba: Ucwaningo olusha lubonisa ukuthi lolu vivinyo lungasiza ekunqumeni ukuthi i-alpha-synuclein ikhona yini ezicutshini zemizwa yesikhumba. Kodwa-ke, lokhu kusesesigabeni socwaningo.
Udokotela wakho uzochaza ukuthi yiziphi izivivinyo acabanga ukuthi ziyadingeka, ngokusekelwe esimweni sakho, nokuthi yini ongayilindela kuzo.
Ingabe ikhona indlela yokwelapha i-MSA?
Ngeshwa, okwamanje alikho ikhambi le-MSA . Ngakho-ke, umgomo oyinhloko wokwelashwa ukulawula izimpawu nokugcina izinga lempilo elihle isikhathi eside ngangokunokwenzeka.
Ukwelashwa okunikezwayo kuncike ezimpawini zesiguli kanye nobunzima bazo. Isibonelo, imithi yokucindezela kwegazi inikezwa ukwelashwa kwe-orthostatic hypotension, imithi yokukhulula imisipha yokuqina, kanye neminye imithi yokulawula umchamo. Futhi, izinto ezifana ne-physiotherapy kanye nokwelashwa ngenkulumo zibaluleke kakhulu.
Uma wena noma othile omaziyo enalesi simo, ungazami ukuzelapha noma ukulawula izimpawu. Njalo bona udokotela ofanelekayo bese ulandela iseluleko sakhe.
Hlobo luni lwekusasa umuntu onesifo angalulindela?
I-MSA iyisifo esiqhubekayo. Cishe ingxenye yalabo abathintekile izodinga usizo lokuhamba (njengodondolo noma i-walker) zingakapheli iminyaka emi-5 kusukela izimpawu ziqale. Cishe ama-60% azodinga isihlalo sabakhubazekile zingakapheli iminyaka emi-5. Eminyakeni eyi-6 kuya kweyi-8, iziguli eziningi zisuke zilele embhedeni.
Njengoba lesi sifo siqhubeka, kudingeka izinqubo ezahlukene zezokwelapha ukuze kugcinwe ukusebenza komzimba.
- I-Tracheostomy inqubo lapho kwenziwa khona imbobo encane emphinjeni bese kufakwa ipayipi ukusiza ngezinkinga zokuphefumula.
- Ukondla ngepayipi kuwukunikeza ukudla ngepayipi lapho isiguli singakwazi ukugwinya.
- Ukufaka amapayipi noma ezinye izinqubo zokuhlinzwa zokulawula umchamo kanye nendle.
Ngokuvamile, isilinganiso sokuphila kwesiguli esine-MSA kusukela ngesikhathi sokuxilongwa siphakathi kweminyaka eyi-6 neyi-10. Kwezinye izimo ezingatheni kangako, kungenzeka ukuthi umuntu aphile iminyaka eyi-15. Kodwa-ke, uma lesi sifo sibi kakhulu, isikhathi sokuphila singaba sifushane kakhulu.
Ukufa kubangelwa kakhulu yizinkinga zesifo. Isibonelo:
- Inyumoniya.
- I-Sepsis (ubuthi egazini) ngenxa yokutheleleka komgudu womchamo.
- Ukufa okuzumayo okubangelwa izinkinga zokulawula ukuphefumula kobuchopho ngesikhathi sokulala.
Sibanakekela kanjani abathandekayo bethu?
Njengoba izimpawu zomuntu one-MSA ziba zimbi ngokuhamba kwesikhathi, angase agcine engakwazi ukuphila ngokuzimela. Angase futhi alahlekelwe ikhono lokucabanga, ukukhuluma, nokuzenzela izinqumo.
Ngakho-ke, kubaluleke kakhulu ukukhuluma ngekusasa nabathandekayo bakho ekuqaleni kwalesi sifo, isiguli sisaphila. Uma usesimweni lapho ungakwazi ukwenza izinqumo, kuyinto elula kuwo wonke umuntu uma ungaxoxa kusengaphambili ukuthi yiziphi izinqumo okufanele zenziwe mayelana nokwelashwa kwakho futhi ulungiselele amadokhumenti asemthethweni.
Kufanele ubonane nini nodokotela?
Izimpawu eziningi zokuqala ze-MSA yizinto okufanele uzixoxe nodokotela.
- Ukuziphatha okubi ngokocansi.
- Ukuzungeza okuqhubekayo kanye nokulahlekelwa ukwazi lapho usukuma.
- Izinkinga zokulala, ikakhulukazi i-sleep apnea.
Uma udokotela wakho ekutshelile ukuthi unesifo sokunyakaza esifana nesikaParkinson, kubalulekile ukukhuluma nganoma yiziphi izinguquko ezimpawu zakho. Ikakhulukazi uma umuthi (i-levodopa) wesifo sikaParkinson ungasebenzi, lokho kuyinkomba enkulu yokuthi ungase ube ne-MSA.
I-MSA iyisifo esibi kakhulu nesibulalayo. Kuyiqiniso ukuthi ayikho ikhambi laso. Kodwa-ke, izimpawu eziningi zingelashwa. Ngaleyo ndlela yokwelapha, izinga lempilo yesiguli lingagcinwa lisezingeni elihle iminyaka eminingi. Leso sikhathi siyithuba eliyigugu lokuchitha isikhathi nenjabulo nabathandekayo baso futhi sijabulele impilo ngokugcwele.
Umlayezo Wokuya Nawe Ekhaya
- I-MSA yisifo sezinzwa esingavamile nesibulalayo esibhubhisa kancane kancane izingxenye zobuchopho.
- Kunezinhlobo ezimbili eziyinhloko: i-MSA-C (ukulahlekelwa yibhalansi kuyanda) kanye ne-MSA-P (izici ze-Parkinsonism ziyanda).
- Ukungasebenzi kahle kokuzimela, njengokwehla komfutho wegazi lapho umi kanye nokulahlekelwa ukulawula umchamo, kubonakala kusenesikhathi.
- I-MSA ithuthuka ngokushesha kakhulu kunesifo sikaParkinson futhi ayizwani kahle nemithi kaParkinson.
- Nakuba kungekho ukwelashwa kwalesi sifo, kunezindlela zokwelapha zokulawula izimpawu nokuthuthukisa ikhwalithi yokuphila.
- Kubaluleke kakhulu ukuqaphela izimpawu kusenesikhathi, ukufuna iseluleko sezokwelapha, nokuhlela ikusasa.
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