Ubuthakathaka beCreutzfeldt-Jakob Disease? Izimpawu, Izimbangela, & Ukwelashwa

I-Creutzfeldt-Jakob disease (CJD) yisifo esingavamile sobuchopho esibangela ukwehla okusheshayo kwengqondo nokukhohlwa. Lesi simo sithinta amangqamuzana ezinzwa zobuchopho, sibangela ukuba aphule ngokuhamba kwesikhathi, okubangela izinkinga ezinzima zezinzwa.

Nakuba i-CJD ingavamile kakhulu, ithinta kuphela omunye umuntu ngamanye ayizigidi ngonyaka, ukuqonda lesi simo kungakusiza ukuthi uqaphele izimpawu zaso futhi wazi ukuthi nini kufanele ufune ukunakekelwa kwezokwelapha. Lesi sifo siyingxenye yeqembu elibizwa ngokuthi izifo zeprion, lapho amaprotheni angavamile enyakaza khona ithishu yobuchopho.

Iyini i-Creutzfeldt-Jakob Disease?

I-Creutzfeldt-Jakob disease yisimo sobuchopho esonakalayo esibangelwa amaprotheni angavamile abizwa ngamaprion. La maprotheni aqhekekile aqina kakhulu ethishwini lobuchopho, ebangela ukuba amangqamuzana ezinzwa afe futhi enze imingxuma efana nesipongu ebuchophini.

Lesi sifo siqhubeka ngokushesha, ngokuvamile ngezinyanga kunokuba kube ngeminyaka. Ngokungafani nezinye izimo zobuchopho ezithuthuka kancane, i-CJD ibangela ukushintsha okusheshayo ekucabangeni, ekukhumuleni, nakho konke ukusebenza komzimba okuqaphela emavikini noma ezinyangeni.

Abantu abaningi abane-CJD baphila cishe unyaka owodwa ngemuva kokuba izimpawu ziqale, nakuba lokhu kungashintsha. Lesi simo sithinta abantu bazo zonke izizinda, kodwa sivame ukuxilongwa kubantu abadala abaneminyaka engama-45 kuya kwengu-75.

Ziyini izinhlobo ze-Creutzfeldt-Jakob Disease?

Kunezinhlobo ezine eziyinhloko ze-CJD, ngayinye inezimbangela nezindlela ezahlukene. Ukuqonda lezi zinhlobo kusiza odokotela ukuthi banqume indlela engcono kakhulu yokuxilonga nokunakekelwa.

I-Sporadic CJD yiyona ndlela evamile kakhulu, enza amaphesenti angama-85% azo zonke izimo. Lolu hlobo lubonakala ngokungahleliwe ngaphandle kwesizathu esaziwayo noma umlando womndeni, ngokuvamile luthinta abantu abaneminyaka engama-60 kuya kwengu-70.

I-Familial CJD imele amaphesenti angama-10-15% yezimo futhi iqhubeka emindenini ngenxa yezinguquko zezinto zakwa-genetic. Abantu abalolu hlobo ngokuvamile bathuthukisa izimpawu besebancane, ngezinye izikhathi abaneminyaka engama-40 noma ama-50.

I-Acquired CJD ingavamile kakhulu futhi ivela ekubeni ukhombiswe ithishu eyenziwe yi-infections, ngokuvamile ngezinqubo zezokwelapha. Lokhu kufaka izimo ezihlotshaniswa nezinto zokusebenza ezingcolile noma ukuhlinzwa kwethishu kubanikazi abathintekile.

I-Variant CJD yiyona ndlela engavamile kakhulu, ehlotshaniswa nokudla inyama yenkomo evela ezinkomeni ezine-mad cow disease. Lolu hlobo ngokuvamile luthinta abantu abasebancane futhi lunezimpawu ezahlukene uma kuqhathaniswa nezinye izinhlobo ze-CJD.

Ziyini izimpawu ze-Creutzfeldt-Jakob Disease?

Izimpawu zokuqala ze-CJD ngokuvamile zifana nezinye izimo, okwenza ukuxilonga kube nzima ekuqaleni. Ungase uqaphele ukushintsha ekukhumuleni, ekucabangeni, noma ekuziphatheni okubonakala kuwohloka ngokushesha emavikini noma ezinyangeni.

Izimpawu zokuqala ezivamile kakhulu zihlanganisa:

• Izinkinga zokukhumbula nokudideka
• Ubunzima bokuxhumanisa nokuqondana
• Ukushintsha ubuntu noma ukuziphatha
• Izinkinga zokukhuluma noma ukuthola amagama
• Ukushintsha kwesimo sengqondo njengokucindezeleka noma ukukhathazeka
• Ubunzima ngokubona noma ukunyakaza kwamehlo

Njengoba lesi sifo siqhubeka, izimpawu ziyaqina futhi zingase zihlanganise ubunzima bemisipha, ukunyakaza okungapheli, nobunzima bokuginya. Lezi zimpawu kamuva zithuthuka ngoba izindawo eziningi zobuchopho ziyaqina ngokuhamba kwesikhathi.

Abanye abantu nabo bahlangabezana nezinkinga zokulala, ukukhahlela kwemisipha ngokuzumayo, noma ubunzima ngemisebenzi eyisisekelo njengokuhamba noma ukudla. Ukuqhubeka okusheshayo kwalezi zimpawu yilokho okuvame ukuxwayisa odokotela ukuthi bacabange nge-CJD njengokuxilongwa okungenzeka.

Yini ebangela i-Creutzfeldt-Jakob Disease?

I-CJD ibangelwa amaprotheni angavamile abizwa ngamaprion ashintsha ukuma futhi enyakaze amangqamuzana ebuchopho. La maprotheni aqhekekile asebenza njengesampula, ebangela amaprotheni ajwayelekile ukuba aqhekeke ngokungalungile futhi asakazeke kulo lonke ubuchopho.

Ku-sporadic CJD, eyona ndlela evamile kakhulu, amaprotheni eprion abonakala eqhekeka ngokungahleliwe ngaphandle kwesizathu esicacile. Ososayensi abakuqondi ngokugcwele ukuthi lokhu kwenzeka kanjani, kodwa kubonakala sengathi kuyisenzakalo esingahleliwe esenzeka njengoba abantu bekhula.

I-Familial CJD ithuthuka lapho abantu bezuza izinguquko zezinto zakwa-genetic ezenza amaprotheni abo abe nomkhuba wokuba aqhekeke. Uma umzali ethwala enye yalezi zinguquko, umntwana ngamunye unethuba elingu-50% lokulithwala, nakuba akuwona wonke umuntu ozuza i-gene ozoba nesifo.

I-Acquired CJD yenzeka lapho othile ekhombiswe ithishu eyenziwe yi-infections evela komunye umuntu. Lokhu kungenzeka ngezinto zokusebenza ezingcolile, ukuhlinzwa kwethishu, noma ezinye izinqubo zezokwelapha ezihilela ithishu yobuchopho noma yomgogodla.

I-Variant CJD ivela ekudleni imikhiqizo yenyama yenkomo evela ezinkomeni ezithintekile yi-bovine spongiform encephalopathy (isifo senkomo esibuhlungu). Amaprion avela ezinkomeni ezithintekile angadlulela kubantu futhi abangele lolu hlobo lwesifo.

Nini kufanele ubheke udokotela nge-Creutzfeldt-Jakob Disease?

Kufanele ubheke udokotela uma uqaphela ukushintsha okusheshayo ekukhumuleni, ekucabangeni, noma ekuqondiseni okubi kakhulu emavikini noma ezinyangeni. Nakuba lezi zimpawu zingaba nezizathu eziningi, ukuqhubeka okusheshayo kuyisibonakaliso esibalulekile sokuqaphela esidinga ukunakekelwa kwezokwelapha.

Xhumana nodokotela wakho uma uhlangabezana nokushintsha okungaqondakali kobunzima, ubunzima bokuqondana noma ukuhamba, noma izinkinga zokukhuluma ezithuthuka ngokushesha. Lezi zimpawu, ikakhulukazi lapho zivele ndawonye, zidinga ukuhlolwa okusheshayo.

Uma unomlando womndeni we-CJD noma izimo ezihlobene, xoxa nodokotela wakho ngisho noma izimpawu zakho zibonakala zingenamandla. Ukuhlolwa kwezokwelapha okusheshayo kungasiza ukukhipha ezinye izimo ezilawuleka futhi kunikeze ulwazi olubalulekile ngomndeni wakho.

Ungalindi uma uhlangabezana nezinkinga zobona, ukukhahlela kwemisipha, noma ubunzima bokuginya kanye nokushintsha kwengqondo. Lezi zihlotshaniso zezimpawu zidinga ukuhlolwa okusheshayo kwezokwelapha ukuze kutholakale imbangela nokunakekelwa okufanele.

Ziyini izinto ezingase zikhulise ingozi ye-Creutzfeldt-Jakob Disease?

Izinto eziningi zingase zikhulise ingozi yakho yokuthuthukisa i-CJD, nakuba lesi sifo sisalulamile kakhulu ngokujwayelekile. Ukuqonda lezi zinto ezingase zikhulise ingozi kungakusiza ukuba ube nezingxoxo ezilwemi nodokotela wakho.

Izinto eziyinhloko ezingase zikhulise ingozi zihlanganisa:

• Ubudala - izimo eziningi zenzeka kubantu abangaphezu kweminyaka engama-60
• Umdlalo womndeni we-CJD noma izinguquko zezinto zakwa-genetic ezihlobene
• Izinqubo zezokwelapha ezidlule ezihilela ubuchopho noma ithishu yomgogodla
• Ukukhombiswa izinto zokusebenza ezingcolile
• Umdlalo wokwamukela ukuhlinzwa kwethishu noma ezinye izindlela zokwelapha

Ukuba nomuntu womndeni onayo i-familial CJD kusho ukuthi ungase uthwale inguquko yezinto zakwa-genetic, kodwa lokhu akuqinisekisi ukuthi uzoba nesifo. Abantu abaningi abane-mutation ababonisi izimpawu, futhi ukwelulekwa kwezinto zakwa-genetic kungakusiza ukuthi uqonde ingozi yakho ethile.

Izinqubo zezokwelapha ezenziwe ngaphambi kwezindlela zanamuhla zokubulala amagciwane zinezinhlobo ezincane zengozi, ikakhulukazi ukuhlinzwa kobuchopho noma izinqubo ezihilela uketshezi lomgogodla. Noma kunjalo, izinqubo zanamuhla zokuphepha kwezokwelapha zisuse le ngozi emazweni athuthukile.

Ziyini izinkinga ezingase zenzeke ngenxa ye-Creutzfeldt-Jakob Disease?

I-CJD iholela ezinkingeni ezinzima njengoba lesi sifo sithinta izindawo eziningi zobuchopho ngokuhamba kwesikhathi. Lezi zinkinga zithuthuka ngoba ithishu yobuchopho iyalimaza ngokwanda futhi ayikwazi ukulawula imisebenzi yomzimba evamile.

Izinkinga ezibaluleke kakhulu zihlanganisa:

• Ukulahlekelwa okuphelele kwengqondo nokubona
• Ukungaqondi ukukhuluma noma ukuxhumana
• Ukulahlekelwa kokulawula imisipha nokuqondana
• Ubunzima bokuginya, obuholela ekukhahleleni noma e-pneumonia
• I-infections ngenxa yesistimu yokulwa nezifo ebuthakathaka
• Izinkinga zenhliziyo nokuphuma komoya

Abantu abaningi abane-CJD ekugcineni badinga ukunakekelwa okugcwele njengoba beyahluleka ukwenza imisebenzi eyisisekelo njengokudla, ukuhamba, noma ukuqaphela amalungu omndeni. Lokhu kuqhubeka ngokuvamile kwenzeka ngezinyanga eziningi.

Imbangela evamile kakhulu yokufa ku-CJD yi-pneumonia, ngokuvamile ivela ebunzimeni bokuginya noma ekuphumuleni isikhathi eside embhedeni. Izinkinga zenhliziyo nezinye i-infections zingase zibe yingozi ekuphileni njengoba lesi sifo siqhubeka.

I-Creutzfeldt-Jakob Disease ixilongwa kanjani?

Ukuxilonga i-CJD kungaba nzima ngoba izimpawu zokuqala zifana nezinye izimo zobuchopho. Udokotela wakho uzoqala ngomlando wezokwelapha ophelele nokuhlolwa komzimba, egxila ezimpawu zezinzwa nokuthi zithuthukile kangakanani ngokushesha.

Izivivinyo eziningi zisiza odokotela ukuba benze ukuxilongwa, nakuba ukuxilongwa okuqinisekile ngokuvamile kudinga ukuhlolwa kwethishu yobuchopho. Izivivinyo zegazi zingase zithole amaprotheni athile ahlobene ne-CJD, kuyilapho ukuhlolwa kwezinto zakwa-genetic kungase kuqonde izinguquko ezihlobene nezinhlobo ze-familial.

Ukubonwa kobuchopho njenge-MRI scans kungase kubonise ukushintsha okubonakalayo ethishwini lobuchopho okuphakamisa i-CJD. Lezi zi-scans zibheka izindlela ezithile zomonakalo ezisiza ekuhlukaniseni i-CJD kwezinye izimo njenge-Alzheimer's disease noma i-stroke.

I-electroencephalogram (EEG) ilinganisa amaza obuchopho futhi ngokuvamile ibonisa izindlela ezingavamile kubantu abane-CJD. Ukugaya umgogodla ukuhlola uketshezi lwe-cerebrospinal kungase futhi kunikeze izinkomba ezibalulekile mayelana nokuxilongwa.

Ngezinye izikhathi odokotela benza i-biopsy yobuchopho, lapho ingxenye encane yethishu yobuchopho ihlolwa ngaphansi komkhiqizo. Le nqubo yenziwa kuphela lapho ukuxilongwa kungaqiniseki futhi ulwazi lungashintsha izinqumo zokwelapha kakhulu.

Iyini ukwelashwa kwe-Creutzfeldt-Jakob Disease?

Okwamanje, ayikho imithi ye-CJD, futhi ayikho imithi engase ibe nokuthuthukisa noma ukuyeka ukuqhubeka kwalesi sifo. Noma kunjalo, ukunakekelwa kwezokwelapha kugxila ekulawuleni izimpawu nokugcina ukhululekile ngangokunokwenzeka kulo lonke ubuhlungu.

Imithi ingasiza ekulawuleni ezinye izimpawu njengokukhahlela kwemisipha, ukukhathazeka, noma ubuhlungu. Imithi yokulwa ne-seizure ingase inciphise ukunyakaza okungapheli, kuyilapho imithi yokucindezeleka noma yokukhathazeka ingasiza ekushintsheni kwesimo sengqondo nokukhathazeka.

I-physical therapy ne-occupational therapy zingasiza ekuqondiseni nokuqhubeka kokusebenza isikhathi eside ngangokunokwenzeka. Lezi zindlela zokwelapha zigxila ekuphepheni, ukuvimbela ukuwa, nokwenza indawo yakho ibe lula ukuze wenze imisebenzi yansuku zonke ibe lula.

Njengoba lesi sifo siqhubeka, ukunakekelwa kuguqukela ekuqinisekiseni ukududuzeka nokuhlonipha. Lokhu kungase kuhlanganise usizo ekudleni, ukugeza, nezinye izidingo zokunakekelwa komuntu siqu, kanye nokulawula ubuhlungu nezinye izimpawu ezingathandeki.

I-speech therapy ingasiza ngezinkinga zokuxhumana nokuginya, kuyilapho ukusekelwa kokudla kuqinisekisa ukuthi uthola ukudla okufanele ngisho noma ukudla kuyaqina. Imindeni eminingi izuza nasekusekelweni nasekusekelweni ngalesi sikhathi esinzima.

Indlela yokuphatha ukunakekelwa ekhaya ngesikhathi se-Creutzfeldt-Jakob Disease?

Ukunakekelwa ekhaya kwe-CJD kugxila ekuphepheni, ukududuzeni, nokugcina ikhwalithi yokuphila isikhathi eside ngangokunokwenzeka. Ukudala indawo ethulile, ejwayelekile kungasiza ekuqedeni ukudideka nokukhathazeka njengoba izimpawu ziqhubeka.

Ukwenza izinguquko zokuphepha ekhaya kuba yinto ebalulekile njengoba izinkinga zokuqondana nokuqondana zithuthuka. Lokhu kuhlanganisa ukususa izinto ezingase zibangele ukuwa, ukufaka ama-grab bars ezihlanzweni, nokuqinisekisa ukukhanya okuhle kulo lonke indlu.

Ukuqalisa izindlela zansuku zonke kungasiza ekuqondeni okujwayelekile nokunciphisa ukudideka. Izinhlelo ezilula, eziqondile zokudla, imithi, nemisebenzi isebenza kangcono njengoba amandla okuqonda eya.

Amalungu omndeni nabaphathi badinga ukusekelwa futhi, njengoba ukunakekela othile onayo i-CJD kungaba nzima ngokomzwelo nangokomzimba. Izinsizakalo zokunakekelwa kwesikhashana, amaqembu wokusekela, nokwelulekwa kungakunikeza usizo oluyigugu.

Ukuxhumana kungase kube nzima njengoba lesi sifo siqhubeka, kodwa ukugcina ukududuzeka komzimba ngokuthinta okuthambile, umculo ojwayelekile, noma izindawo ezithulile kungase kunikeze ukuxhumana nokududuzeka okunemqondo.

Kufanele ulungiselele kanjani i-aphoyintimenti yakho nodokotela?

Ngaphambi kwe-aphoyintimenti yakho, bhala zonke izimpawu ozibonile, kufaka phakathi ukuthi zaqala nini nokuthi zishintshile kanjani ngokuhamba kwesikhathi. Yiba ngokucacile ngangokunokwenzeka mayelana nezinsuku nokuqhubeka kwezimpawu.

Letha uhlu oluphelele lwemithi, izithasiselo, nama-vitamins owawathatha. Buye uqoqe ulwazi mayelana nomlando wezokwelapha womndeni wakho, ikakhulukazi noma yimiphi amalungu omndeni ayene-dementia, izifo zokunyakaza, noma izimo ezingavamile zezinzwa.

Cabanga ukuletha ilungu lomndeni noma umngani oseduze onganikeza ukubuka okwengeziwe mayelana nezinguquko ababeqaphele. Ngezinye izikhathi abanye bangabona izinguquko ongenakuzibona kuwe.

Lungiselela imibuzo mayelana nokuthi yini okulindeleke, izinketho zokwelapha, nezinsizakalo zokusekela. Zibhale phansi ngaphambi kwesikhathi ukuze ungakhohlwa izihloko ezibalulekile ngesikhathi se-aphoyintimenti.

Uma uke waba nezinqubo zezokwelapha ezidlule, ikakhulukazi noma yiziphi ezihilela ubuchopho noma ithishu yomgogodla, letha amarekhodi noma ulwazi oluqondile mayelana nalezi zindlela zokwelapha. Lolu lwazi lungabalulekile ekuqondeni izinto ezingase zikhulise ingozi yakho.

Iyini into ebaluleke kakhulu mayelana ne-Creutzfeldt-Jakob Disease?

I-Creutzfeldt-Jakob disease yisimo sobuchopho esingavamile kodwa esinzima esiqhubeka ngokushesha futhi okwamanje ayinalutho oluyimpumulo. Nakuba lokhu kuxilongwa kuyesabekisa, ukuqonda lesi simo kungakusiza ukuba wenze izinqumo ezilwemi mayelana nokunakekelwa nokusekela.

Into ebaluleke kakhulu okumele uyikhumbule ukuthi awuwedwa ekuhlanganyeleni nale nselelo. Amaqembu ezokwelapha, abasebenzi bezenhlalakahle, nezinhlangano zokusekela zingakunikeza izinsiza neziqondiso ezibalulekile kulo lonke uhambo lwakho.

Gxilisa ukugcina ikhwalithi yokuphila nokuxhumana okunemqondo namalungu omndeni nabangane isikhathi eside ngangokunokwenzeka. Abantu abaningi abane-CJD nemindeni yabo bathola induduzo ekunakekelweni kokomoya, ukwelulekwa, namaqembu wokusekela aklanyelwe izifo ezingavamile.

Nakuba i-prognosis inzima, ukuthuthuka ekunakekelweni okusekela kusho ukuthi izimpawu zingaphathwa kangcono kunakuqala. Ukusebenzisana eduze neqembu lakho lezokwelapha kuqinisekisa ukuthi uthola ukunakekelwa nokusekela okungcono kakhulu.

Imibuzo evame ukubuzwa mayelana ne-Creutzfeldt-Jakob Disease

Ingabe i-Creutzfeldt-Jakob disease iyadluliseka?

I-CJD ayidluliseki ngendlela evamile futhi ayikwazi ukusakazeka ngokuxhumana okuvamile, ukukhahlela, noma ukwabelana ngokudla. Noma kunjalo, amaprion abangela i-CJD angase adluliselwe ngokuxhumana ngqo nethishu yobuchopho noma yomgogodla eyenziwe yi-infections, kungakho kuthathwa izinyathelo ezikhethekile ezindaweni zezokwelapha. Ukuxhumana okuvamile nabantu abane-CJD akubangeli ingozi kumalungu omndeni noma kubaphathi.

Ingabe i-Creutzfeldt-Jakob disease ingavimbeka?

Izinhlobo eziningi ze-CJD azikwazi ukumile njengoba zenzeka ngokungahleliwe noma zithwalwe. Noma kunjalo, unganciphisa ingozi yakho ye-variant CJD ngokugwema imikhiqizo yenyama yenkomo evela emazweni aye abe ne-mad cow disease outbreaks. Izindawo zezokwelapha manje zisebenzisa izindlela ezithuthukile zokubulala amagciwane nezinqubo zokuhlola ukuvimbela ukudluliselwa ngezinqubo zezokwelapha, okwenza i-acquired CJD ibe yingavamile kakhulu.

I-CJD ihluke kanjani ku-Alzheimer's disease?

Nakuba zombili izimo zithinta ukukhumbula nokucabanga, i-CJD iqhubeka ngokushesha kakhulu kunesi-Alzheimer's disease. Izimpawu ze-CJD ngokuvamile ziyaqina ngezinyanga, kuyilapho i-Alzheimer's ithuthuka eminyakeni. I-CJD futhi ngokuvamile ibangela ukukhahlela kwemisipha nezinkinga zokuqondana ekuqaleni kwesifo, okuvamile ekuqaleni kwe-Alzheimer's. Isikhathi esiphakathi sokuphila siphinde sifushane kakhulu nge-CJD.

Yini amalungu omndeni okufanele ayazi ngokuhlolwa kwezinto zakwa-genetic kwe-CJD?

Ukuhlolwa kwezinto zakwa-genetic kungase kuqonde izinguquko ezihlobene ne-familial CJD, kodwa ukuba ne-mutation akuqinisekisi ukuthi uzoba nesifo. Abanye abantu abane-mutation ababonisi izimpawu, kuyilapho abanye bengase bathuthukise lesi simo kamuva ekuphileni. Ukwelulekwa kwezinto zakwa-genetic ngaphambi kokuhlolwa kungakusiza ukuthi uqonde izimpikiswano zokuhlolwa futhi wenze isinqumo esilwemi mayelana nokuthi ukuhlolwa kufanele kuwe.

Ingabe kunokwelashwa okuvivinywayo okufundwa nge-CJD?

Abacwaningi bahlola izindlela eziningi zokwelapha i-CJD, kufaka phakathi imithi engase ibe nokuthuthukisa ukwakheka kwamaprion nemithi engase ivikele amangqamuzana ebuchopho ekubulaleni. Nakuba lezi zindlela zokwelapha zisavivinywa futhi azikafakazelwa ukuthi ziphumelela, ukuvivinywa kwemithi kungase kube khona kwabanye abantu. Udokotela wakho angakunikeza ulwazi mayelana nocwaningo oluqhubekayo nokuthi ungase ufaneleke yini ukuhlanganyela.