Usenokuba awuzange uve ngezifo ezenzeka xa uhlobo oluthile lweproteni emzimbeni wethu luqokelelana ngendlela engafanelekanga luze lubekwe kwizitho ezibalulekileyo ezifana nentliziyo kunye nemithambo-luvo. Ezi zizifo ezingaqhelekanga. Kwizonyango, olu hlobo lokufakwa kweproteni lubizwa ngokuba yi -amyloidosis . Namhlanje, siza kuthetha ngesifo esingaqhelekanga esibizwa ngokuba yi-ATTR-CM, esichaphazela ngqo intliziyo, oko kukuthi, apho ezi proteni zifakwa kwimisipha yentliziyo, kwaye iyeza lanamhlanje elibizwa ngokuba yi-Amvuttra lisetyenziselwa lona.
Yintoni kanye kanye i-ATTR-CM?
Ngamafutshane, i-ATTR-CM yimeko apho, ngenxa yesiphene semfuza okanye esenzeka ngokwendalo xa umntu ekhula, iproteni i-TTR, eveliswa emzimbeni wethu, itshintsha imo kwaye ihlangana ize izibeke eludongeni lwemisipha yentliziyo. Igama elipheleleyo lale nto yiTransthyretin Amyloid Cardiomyopathy.
Cinga nje, iindonga zethu zentliziyo kufuneka ziguquguquke ukuze zikwazi ukupompa kakuhle. Kodwa xa ezi proteni ziqokelelana, iindonga zentliziyo ziba nkulu kwaye ziqine . Oku sikubiza ngokuba yi-cardiomyopathy. Oku kwenza intliziyo ingakwazi ukupompa igazi ngokufanelekileyo. Ekuhambeni kwexesha, oku kunokuqhubela phambili ekubeni kuphele intliziyo.
Abanye abantu abane-ATTR-CM abanazo naziphi na iimpawu, kodwa abanye bafumana iimpawu ezifana nezo zokuhlaselwa yintliziyo okuqhelekileyo.
- Ukuphefumla kancinci (i-dyspnea)
- Ukudumba, ingakumbi emilenzeni nasemaqatheni
- Ukudideka okanye ukuphelelwa lithemba
- Ukunyuka kwesantya sentliziyo okanye ukubetha kwentliziyo okungaqhelekanga
- Iintlungu zesifuba
Oku kunokukwenza uzive udiniwe ngokukhawuleza xa usenza izinto ezifana nokunyuka izinyuko, ukwenza imisebenzi yasekhaya, okanye ukuhamba umgama omfutshane.
Ukongeza, ezinye iimpawu ezingahambelaniyo nentliziyo nazo zinokuvela. Umzekelo, ubuthathaka, ukungaziva okanye iintlungu ezingalweni nasemilenzeni, ukubila kakhulu, isiyezi okanye ukutyhafa, kunye neengxaki zokugaya ukutya.
Isebenza njani iyeza lase-Amvuttra?
Ukuze sikuqonde oku, kufuneka siqale sazi kancinci ngeproteni ebizwa ngokuba yi-TTR. I-TTR (iTransthyretin) yiproteni eveliswa kakhulu esibindini sethu. Umsebenzi wayo ophambili kukuhambisa iihomoni ze-thyroid kunye ne-vitamin A emzimbeni wonke.
Kubantu abane-ATTR-CM, isiphene semfuza sibangela ukuba iproteni ye-TTR iveliswe ngendlela engalunganga. Le protein ingalunganga iqokelelana ize ihlale entliziyweni.
Eli yeza i-Amvuttra lisebenza njengesithinteli - zintsholongwane zezakhi zofuzo. Oko kukuthi, eli yeza liya ngqo esibindini lize livale iproteni ye-TTR engalunganga.Iyanciphisa umsebenzi wejini edalwayo. Kufana nokunciphisa isandi serediyo.
Oku kunciphisa ubungakanani beprotheyini ye-TTR engalunganga eveliswa sisibindi, nto leyo enciphisa ukubekwa kweeprotheyini ezintsha kwizitho ezifana nentliziyo.
I-Amvuttra yinaliti efakwa ngaphantsi kwesikhumba. Olu gonyo ludla ngokunikezelwa yingcali yezempilo rhoqo emva kweenyanga ezintathu .
Impumelelo yale mayeza yavavanywa njani?
Njengalo naliphi na iyeza elitsha, ukusebenza kakuhle kwe- AMVUTTRA kuqinisekisiwe ngophando olukhulu. Kolu phononongo, izigulane ezingama-655 ze-ATTR-CM zanikwa igonyo le-Amvuttra kwaye elinye iqela lanikwa igonyo le-placebo elingenayo iyeza rhoqo kwiinyanga ezili-12 kangangeminyaka emithathu.
Abaphandi bajonge izinto ezininzi ukuze balinganise ukuba eli yeza liyasebenza na.
- Uvavanyo lokuhamba ngemizuzu emithandathu: Olu vavanyo lulinganisa ukuba ungahamba umgama ongakanani ngesantya esiqhelekileyo kwimizuzu emi-6. Lo mgama uyancipha njengoko imeko yentliziyo yakho isiba mandundu.
- Udidi lweNew York Heart Association (NYHA): Olu luhlu lulinganisa ubukhali beempawu zokusilela kwentliziyo. Kukho iindidi ezine (I, II, III, IV). Okukhona inani leklasi liphezulu, kokukhona isifo siba sibi kakhulu.
| Iklasi ye-NYHA | Inkcazo |
|---|---|
| Iklasi yoku-1 | Akukho miqathango kwimisebenzi eqhelekileyo yomzimba. Akukho kudinwa, ukuphefumla nzima, okanye iintlungu zesifuba. |
| Iklasi yesiBini | Umsebenzi womzimba ulinganiselwe kancinci. Akukho ntlungu xa uphumla, kodwa ukudinwa nokudinwa kunokwenzeka ngexesha lemisebenzi eqhelekileyo. |
| Iklasi yesi-3 | Umsebenzi womzimba ulinganiselwe kakhulu, kwaye iimpawu ziyenzeka nokuba umsebenzi ulula kunesiqhelo. |
| Iklasi yesi-IV | Iimpawu ziyabonakala nokuba uphumle. Nawuphi na umsebenzi womzimba wenza imeko ibe mandundu. |
- Uvavanyo lweMigangatho yoBomi (KCCQ-OS): Olu vavanyo lulinganisa impembelelo yesifo kumgangatho wobomi bomguli.
Yintoni efunyenwe luphando?
Ekupheleni kwexesha lonyango lweminyaka emithathu, abaphandi bahlalutya iziphumo.
Isiphumo esiphambili yayikukuba izigulane ezifumene i-Amvuttra zazinomngcipheko ophantsi ngama-28% wokufa okanye ukulaliswa esibhedlele ngequbuliso okanye ukutyelela kwigumbi likaxakeka (ETU) ngenxa yokusilela kwentliziyo , xa kuthelekiswa nezo zifumene i-placebo (ugonyo olungelulo olokwenyani).
Enye into ebalulekileyo kukuba ekupheleni kweenyanga ezingama-42, umngcipheko wokufa ngenxa yesizathu esithile wehliswa ngama-35% kwiqela elafumana i-Amvuttra.
Ukongeza, izigulane ezifumene i-Amvuttra zazinokwehla okulawulekayo kwindlela ezihamba ngayo kunye nomgangatho wobomi.
Kodwa khumbula, ezi ziphumo zophando zisenokungafani kuye wonke umntu. Wena nogqirha wakho kuya kufuneka nigqibe kunye ukuba i-Amvuttra ikulungele na kwaye ingaba izibonelelo zingaphezulu kuneziphumo ebezingalindelekanga.
Masifunde ngeziphumo kunye nemiphumo emibi.
Kuthatha ixesha elingakanani ukubona iziphumo?
I-Amvuttra liyeza elisebenza ngokuhamba kwexesha. Ngenxa yoko, iziphumo azibonakali kwangoko. Kungathatha iinyanga ezininzi okanye ngaphezulu ukubona naluphi na utshintsho kwiimpawu.
Ungayilawula njani imiphumo emibi?
Esinye seziphumo ebezingalindelekanga ze-Amvuttra kukwehla kwamanqanaba e-vitamin A egazini. Oku kungenxa yokuba iproteni ye-TTR yiyo ethwala i-vitamin A emzimbeni wonke. Xa iyeza linciphisa ukuveliswa kwe-TTR, ukuthuthwa kwe-vitamin A nako kuyancipha.
- Ugqirha wakho uza kuyalela rhoqo uvavanyo lwegazi ukujonga amanqanaba akho evithamini A. Kubaluleke kakhulu ukwenza olu vavanyo ngexesha.
- Yongeza ukutya okutyebileyo kwi-vitamin A (umz. iminqathe, iitapile ezimnandi, ispinatshi, amaqanda, ubisi, iipepile ezibomvu ) ekutyeni kwakho.
- Ukuba ubona naluphi na utshintsho embonweni wakho (ingakumbi ukwehla kombono wasebusuku), yazisa ugqirha wakho ngoko nangoko.
- Ngokuxhomekeke kwinqanaba le-vitamin A yakho, ugqirha wakho unokucebisa ukuba uthathe isongezelelo se-vitamin A.
Ezi azizo zodwa iziphumo ebezingalindelekanga ze-Amvuttra. Ukuba unezinye iimpawu ezikukhathazayo, qiniseka ukuba uthetha nogqirha wakho .
Umyalezo Wokuya Ekhaya
- I-ATTR-CM sisifo sentliziyo esingaqhelekanga kodwa esibi kakhulu esibangelwa kukugcinwa kweeproteni ezigobileyo entliziyweni.
- I-Amvuttra liyeza lanamhlanje elisetyenziselwa le njongo kwaye lifakwa inaliti rhoqo emva kweenyanga ezintathu.
- Eli yeza lisebenza ngokunciphisa imveliso yeproteni enesiphene esibindini.
- Uphando lubonise ukuba oku kunokunciphisa ukufa, ukulaliswa esibhedlele, nokuphucula umgangatho wobomi bezigulana.
- Ekubeni oku kunokukhokelela ekubeni amanqanaba e-vitamin A aphantsi, kubalulekile ukuba uhlale unxibelelana nogqirha wakho rhoqo kwaye wenze uvavanyo rhoqo.
- Ngaphambi kokuba uqalise naluphi na unyango, xoxa ngononophelo ngezinto ezilungileyo nezingalunganga nogqirha wakho.
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