I-HD inezinhlobo eziningi zezimpawu ezingase zithuthuke, ezinye zazo zingaphathwa ngempumelelo ngemithi kanye nezindlela ezingezona izidakamizwa. Ngaphambi kokuthola ukwelashwa, izimpawu zokuqonda njengengxenye yesifo zingasiza futhi ukubhekana nazo futhi uthathe indima ekhuthele kakhulu ekunakekeleni ilungu lakho noma lomndeni.
Izinhlobo zezibonakaliso
Njengesimo sezinzwa, izimbangela zikaHuntington zikhombisa izimpawu ezivame ukuwela kwesinye sezigaba ezintathu: izinguquko zomzimba / ukunyakaza, izinguquko zengqondo, nezinguquko ezingokomzwelo / zokuziphatha.
I-HD ingenye yezimiso eziningana ezibangela ukucindezeleka komqondo .
Izimpawu ngokuvamile zikhula phakathi kuka-30 no-50 ubudala. Kodwa-ke, abanye abantu abancane kuneminyaka engama-20 bahlakulela uhlobo lwesifo sikaHuntington esibizwa ngokuthi yisifo sabantwana baseHuntington.
Izinguquko zomzimba
Esinye sezibonakaliso eziphawulekayo ze-HD yi-chorea. I-Chorea ibhekisela ekulahlekelweni kwekhono lokulawula ukunyakaza okungokwenyama. Iveza ukunyakaza okungazibandakanyi, okweqile, futhi ngokuzumayo. Lezi zinyama ngokuvamile zivela emzimbeni ongaphezulu futhi zifaka izingalo ezingaphezulu, isiqu, ikhanda, intamo, nobuso. Kungenzeka futhi emilenzeni. I-Chorea ikhona cishe ngamaphesenti angu-90 abantu abane-HD futhi ingenye yezimpawu ngokuvamile ezihloselwe imithi.
Ezinye izibonakaliso ze-HD zihlanganisa ukwehla ekuhambeni nasekukhulumeni, ukungabi nokusebenzisana, ukukhubazeka ekugwileni ukudla nokuphuza, futhi ngenxa yalokho, ukulahlekelwa isisindo esikhulu. Ngenxa yalezi zimpawu, ingozi yokuwa iphezulu kubantu abane-HD, kodwa ukusebenza nochwepheshe kungasiza ukunciphisa ingozi.
Izinguquko ezingqondweni
I-HD ingumphumela wezinguquko ebuchosheni, ngakho-ke kuyaqondakala ukuthi amakhono okuqonda anethintekile. Nakuba imemori ingathinteka, ezinye izindawo zokuqwashisa nazo zithinteka futhi. Lokhu kufaka phakathi ukusebenza komsebenzi ongasebenzi kahle (njengokukwazi ukuhlela nokwenza izinqumo), ukugxila okuncane, ukungabikho kokugxila, ukwahlulela okungalungile kanye nokungaqondi kokuziphatha kwakho.
Ukuntuleka kokuvimbela kungase futhi kuthuthuke. Isibonelo, uma unayo i-HD, ungase wenze okuthile ongavamile ukuzizwa kulungile ngoba ukulawulwa kwakho kokucindezela kunciphile.
Izinguquko Zomzwelo Nezimo
Izimpawu ze-HD zifaka izinguquko eziningana nezokuziphatha. Ungase uzizwe ukhathazeke ngokweqile nentukuthelo, kanye nokuziphendulela okungenakwenzeka. Ukuhlukumezeka komlomo kanye nomzimba kungase kuthuthuke. Eqinisweni, ucwaningo oluthile luphetha ukuthi amaphesenti angu-22 kuya kuma-66% abantu abane-HD bazobonisa ukuhlukunyezwa, ngokuvamile ezikhathini zokuqala zesifo.
Njengazo zonke izinhlobo zokudemeka kwengqondo , ukunganakwa kuvamile ku-HD. Ukucindezeleka (okuyinto efana nokunganakwa kodwa ngokuvamile kuhilela imizwa yokudabuka nokuphelelwa ithemba) kungabonakala ngokuphawulekayo futhi kuyisimo esiqinile sokucabanga noma ukucabangela ukuzibulala. Ucwaningo lubonisa ukuthi kunengozi enkulu yokuzibulala kulabo abaphila ne-HD, ngezifundo ezithile ezibonisa ukuthi kukhona ukucabanga okuzibulala ezimpikeni ezingaba ngu-19 zabahlanganyeli ocwaningo.
Imizwa yokukhathazeka ivame ukuhamba nokucindezeleka. Ucwaningo lwezokwelapha lubonisa ukuthi noma yikuphi okuvela ngamaphesenti angama-34 kuya kwangu-61 alabo abane-HD abanemizwa yokukhathazeka.
Ukukhuthazela , lapho uthola "ukunamathela" ngegama, ucabanga noma isenzo, akuvamile.
Lokhu kungahlanganisa ukuphoqa nokucindezelwa futhi kwenze kube nzima ukuqhubekela emsebenzini omusha. Kungase kubangele ukuziphatha okungalungile kwezenhlalo okwenze kube nzima kwabanye ukwazi ukuchitha isikhathi nomuntu ono-HD.
Uhlolojikelele Ukwelashwa
Ngenkathi kungekho ukwelashwa okutholakala i-HD ngalesi sikhathi, kunemithi embalwa kanye nezindlela ezizwakalayo ezingasiza ukukhulula ezinye izimpawu okwesikhashana. Khumbula ukuthi akufanele usebenzise izinto ezizimele noma ezengeziwe ngaphandle kokuhlola nodokotela wakho, njengoba abanye bengaba nemiphumela emibi kakhulu emibi noma ukusebenzisana kwezidakamizwa neminye imithi yakho.
Njengoba kungekho ukwelashwa, umgomo wokwelashwa ku-HD ukuthuthukisa ikhwalithi yokuphila nokugcina ukusebenza isikhathi eside ngangokunokwenzeka.
Imithi
I-Xenazine (tetrabenazine)
U-Xenazine wamukelwa ngo-2008 yi-US Food and Drug Administration (FDA) ukuphatha i-chorea ku-HD. Kuye kwaboniswa ukusiza ukunciphisa ukunyakaza okungazibandakanyi futhi ngokuvamile kuthathwa njengendlela yezindlela ezivame kakhulu nezisebenzayo zokuphatha i-HD.
Nokho, i-Xenazine yenza isixwayiso mayelana nokusetshenziswa kubantu abanokucindezeleka ngoba kubonakala sengathi kwandisa ukucindezeleka nokucabanga okuzibulala. Imiphumela emibi ingafaka futhi kokubili ukulala nokulala.
I-Austedo ( deutetrabenazine )
I-Austedo ivunyiwe yi-FDA ngo-2017. Inqunyelwe futhi ukuba iphathe ukunyakaza okungazibandakanyi (chorea) esifweni sikaHuntington.
U-Austedo unamakhemikhali afana ne-Xenazine kodwa ukusebenza kwawo kuqhubeka isikhathi eside. Ngenxa yalokho, i-Austedo ivame ukuba yenziwe kanye noma kabili ngosuku, kuyilapho i-Xenazine ivame ukuba yenziwe kathathu ngosuku ngalunye.
U-Austedo ubonise ukuphumelela ekunciphiseni izimpawu ze-chorea, kodwa njengo-Xenazine, le mithi ifika ngesixwayiso esinamandla esibonisa ukuthi akufanele isetshenziselwe abantu abane-HD abazwa imizwa yokucindezeleka noma imicabango yokuzibulala, njengoba kungakhuphula leyo mizwa.
Imishanguzo ye-Antipsychotic
Imishanguzo ye-antipsychotic, ebizwa nangokuthi i-neuroleptics, ngezinye izikhathi iqondiswe ukuphatha ukhetho. Lokhu kuyindlela yokusebenzisa i-off-label yemithi, okusho ukuthi i-FDA ayizange ivume le mithi ngokukhethekile kule njongo; Nokho, ezinye zazo ziye zabonisa inzuzo ethile kule ndawo.
Imiphumela yocwaningo iyahlukahluka, kepha ama-antipsychotics ase-atypical asetshenziselwa ukuphatha i-HD afaka i-Zyprexa (i-olanzapine), i-Risperdal (risperidone), ne-Seroquel (quetiapine). Ama-antipsychotics asebekhulile afana ne-Haldol (haloperidol) ne-Clozaril (clozapine) nawo anqunywe kodwa abe nemiphumela emibi yokuthuthumela kanye ne-tardive dyskinesia, kokubili okubangela ukunyakaza okungazibandakanyi futhi ngaleyo ndlela kungahle kube yinto engenzi lutho.
Ukwengeza, imishanguzo ethile ye-antipsychotic efana ne-Abilify (aripiprazole) ibonise ukuphumelela ekunciphiseni izimpawu zokucindezeleka ku-HD, kanye nokwenza ngcono ukuqonda.
Ama-Antipsychotics asetshenziswe ngezikhathi ezithile ngenhloso yokunciphisa ukuziphatha okuyinselele (njengokuhlukumeza) okungathuthuka ku-HD; Kodwa-ke, kunezinto eziningana ezingase zibe nemiphumela emibi ngokusetshenziswa kwe-antipsychotics, ngakho-ke ukuqapha kuyadingeka.
I-Symmetrel
Ucwaningo mayelana nokusebenza kwe-Symmetrel (i-amantadine) kubonise imiphumela ephikisanayo. I-Symmetrel iyisidakamizwa esilashwa yi-anti-virus esetshenziselwa ukuphatha ukuthuthumela okungazibandakanyeki okwenzeka ngezinye izikhathi kulesi sifo sikaParkinson , ngakho-ke ukukhomba kwayo ku-HD kuyisimo se-chorea. Kubonakala inenzuzo kwabanye abantu abane-HD.
SSRIs
I-serotonin reuptake inhibitor (SSRI) ekhethiwe yokunakekelwa kwezidakamizwa ihlelwe ngezinye izikhathi ukuphatha ukucindezeleka kanye nokuphoqa nokucindezelwa ngezinye izikhathi okuhlangenwe nakho kwalabo abaphila ne-HD. Njengeminye imithi, ukuphumelela kuyahluka.
I-Mood Stabilizers
Ama-mood stabilizers afana ne-Depakote (divalproex) angase asetshenziselwe ukubhekana nokuhlukahluka okukhulu kwemizwelo engahambisana ne-HD, kanye nokuhlukumezeka, ukungacabangi, nezimpawu ezicindezelayo.
Eminye Imithi
Ephendula izimpawu ezihlukahlukene isiguli esibikeza odokotela babo, ezinye izidakamizwa zingabuye zenziwe ukuba zixazulule lezo zinkinga ezithile. Isibonelo, uma ukulala nokukhathazeka kungukukhathazeka okuyinhloko ku-HD, udokotela uzovame ukubeka imithi yokuzikhomba. Ngakho-ke, kubalulekile ukuvuleka neqembu lakho lokunakekelwa kwezempilo futhi ubazise ngakho konke okukukhathazayo. Bangase babe nokukhetha okuzokwenza ngcono isimo sakho.
Izindlela ezingezona izidakamizwa
Njengoba imishanguzo okwamanje ilinganiselwe ukuzama ukuphatha izimpawu ezithile kwi-HD, ezinye izindlela ezingezona izidakamizwa zinconywa zinconywa.
Ukukhulumisana nokukhuluma kolimi
Inkulumo kanye nodokotela wezolimi bangasebenza nawe ukusiza ekukhulumeni izidingo zakho nezintandokazi zakho. Ukuzivocavoca okunye kungakusiza uqinise ulimi lwakho nemisipha yomlomo ukuze ugcine ukusebenza kwakho isikhathi eside ngangokunokwenzeka.
Abahlinzeki bezinkulumo bangabuye bahlole ikhono lakho lokugwinya futhi banqume ukuthi yimiphi imishini noma ukungenelela kuyokusiza kakhulu kuwe. Lokhu kungabalulekile ngoba, njengoba lesi sifo siqhubeka, kungase kube nzima kakhulu ukugwinya ukudla noma amanzi ngaphandle kokukhipha.
Ukwelashwa Kwemvelo Nokusebenza
Ukwelapha ngokomzimba kanye nokwelapha emsebenzini kungakusiza ngezindlela eziningana. Ezingxenyeni zokuqala ze-HD, ukwelashwa ngokomzimba kungasiza ekuthuthukiseni nasekugcineni amandla nokusebenza jikelele. Njengoba i-HD iqhubeka, imishini ingahle ilandiswe futhi ifanelane nezidingo zakho ezithile, futhi uhlelo lokuzivocavoca ekhaya lungakhiwa ukwandisa impilo yomzimba.
Udokotela osebenzayo angasebenza nawe ekunqumeni indlela engcono kakhulu yokwenza imisebenzi yokuphila kwansuku zonke , njengokugqoka nokugqoka. Ochwepheshe bezisebenzi bangakwazi ukubona ukuvivinya kwengqondo , ngenhloso yokugcina ukusebenza kwakho kwengqondo.
Abahlinzeki bangasebenza futhi nabanakekeli bakho njengoba lesi sifo sithuthuka ukubasiza ukuthi bazi kangcono kanjani ukunakekela.
Ukuzivocavoca umzimba
Ukuzivocavoca umzimba kuhlotshaniswa kakhulu namakhono azinzile-noma ngisho nokuthuthukiswa kwezifo eziningi ezibangelwa ukugula komqondo, futhi lokhu kuqinisile ku-HD. Ucwaningo luye lwabonisa ukuthi amazinga aphezulu okuzivocavoca umzimba ahlolisisa izilinganiso ezingcono ekuhlolweni kokucubungula nokusebenza kwansuku zonke okuthuthukisiwe.
I-Psychotherapy / Ukululekwa okusekelayo
Ukukhuluma nesisebenzi senhlalakahle yomtholampilo noma isazi sokusebenza kwengqondo singabalulekile njengoba ulungisa izinguquko i-HD eza nazo bese uthola amasu akho okubhekana nokubhekana nazo. Ukwelapha okukhulumayo nakho kungaba nenzuzo kakhulu kwabashade nabalingani, kanye nezingane kanye nemindeni.
I-HD iletha izinguquko eziphawulekayo ezingeni lakho lokusebenza, futhi ingabangela ukukhathazeka emalungwini omndeni mayelana nobungozi babo bokuthuthukisa i-HD kusukela ngokudluliselwa kwezakhi zofuzo ezinganeni. Udokotela angakusiza wena nomndeni wakho ukuthi nisebenze ngokusebenzisa izinguquko ezenzekayo, ingozi yezofuzo ze-HD, futhi ngikuxhumanise nezinsiza emphakathini wakho nasendlini yakho.
Ukwelashwa Okubalulekile
Ezinye izindlela zingase zizuze nalabo abahlala ne-HD. Isibonelo, umculo , ubuciko, kanye nokwelashwa kwedrama konke sekusetshenzisiwe. Ngenkathi bengeke bashintshe izimpawu ze-HD, bangakwazi ukuthinta inhlalakahle jikelele futhi bathuthukise izinga lokuphila.
Izinsizakalo zeDietitian
Ukudla ukudla okunempilo kubalulekile kithi sonke, futhi ngisho nangaphezulu uma uhlangabezana ne-HD. Ukuqinisekisa ukuthi umzimba wakho uthola izakhi ezidingekayo kungasiza ukugcina amandla akho. Lokhu kungaba nzima, ikakhulukazi njengoba i-HD iqhubeka, ngakho-ke ungase uzuze ekusizeni kwesidlo sokudla esibhalisiwe.
Izwi elivela
Okubaluleke kakhulu, abantu abane-HD nemindeni yabo banengozi yokuzihlukanisa. Yazi ukuthi kunezinsiza kanye nokwesekwa okukhona ukuze kukukhuthaze futhi kukusize ubone izinyathelo ezilandelayo. Uma ungaqiniseki ukuthi ungenzani noma udinga usizo, i-Huntington's Disease Society of America inezigaba zendawo kanye namaqembu okusekela inthanethi angakhuthaza wena, uphendule imibuzo yakho noma uhambe nawe noma nje ube indlebe ekulalela njengoba uhlala I-HD.
> Imithombo:
> Arora, G. (2015). Ukuphatha Ukuhlukumezeka Emzimbeni KaHuntington. http://hdsa.org/wp-content/uploads/2015/07/Managing-Aggression-in-HD_Garima-Arora_ver005.pdf
> Coppen, E. noRoos, R. (2016). Izindlela Zokuphila Zamanje Zokunciphisa I-Chorea Esiguleni SaseHuntington. Izidakamizwa , 77 (1), i-pp.29-46. 10.1007 / s40265-016-0670-4
> Dale, M. no van Duijn, E. (2015). Ukukhathazeka ngezifo zikaHuntington. I-Journal of Neuropsychiatry ne-Clinical Neurosciences , 27 (4), iphep.262-271.
> Wallace M, Downing N, uLourens S, et al. Ingabe Kukhona Inhlangano Yomsebenzi Wokuzivocavoca nge-Brain volume, Ukuziphatha, nokusebenza Kwansuku zonke? I-Cross Sectional Design ku-Prodromal and Early Huntington Disease. I-PLoS Currents . 2016; 8.
> Wetzel HH, Gehl CR, Dellefave L, et al. Isifiso sokuzibulala esifubeni saseHuntington: Indima ye-comorbidity. Ucwaningo lwe-Psychiatry . 2011; 188 (3): 372-376. doi: 10.1016 / j.psychres.2011.05.006.