Ngaba ugqirha wakho ukuxelele ukuba unesifo esingaqhelekanga esibizwa ngokuba yiSystemic Mastocytosis ? Usenokuziva usoyika kwaye ukhathazekile xa usiva loo nto. Kuqhelekile kakhulu. Kodwa ungakhathazeki. Ngoku kukho unyango oluhle kakhulu nolusebenzayo lwezi meko. Namhlanje siza kuthetha ngeyeza i-Ayvakit (avapritinib) owalinikwayo. Masiqonde yonke into ngesi sifo kunye neyeza ngokulula nangokucacileyo.
Yintoni kanye kanye iSystemic Mastocytosis (SM)?
Ngamafutshane, i-Systemic Mastocytosis yimeko engaqhelekanga apho uhlobo lweseli olubizwa ngokuba 'ziiseli ze-mast' emzimbeni wethu lukhula kakhulu. Ezi seli ze-mast ziyinxalenye yenkqubo yethu yokuzikhusela. Njengemikhosi yokhuselo yelizwe lethu. Xa into yangaphandle ingena emzimbeni, ezi seli ziyavuselelwa kwaye zikhuphe iikhemikhali ezifana 'ne-histamine'.
Khawucinge ukuba xa ingcongconi ikuluma, indawo iyarhawuzelela kwaye ibe bomvu, akunjalo? Yiloo nto eyenzekayo ngenxa yale histamine. Kodwa kumntu one-Systemic Mastocytosis, ezi mast cells ziqokelelana ngendlela engalawulekiyo emzimbeni. Ziqokelelana eluswini, kumongo wethambo, okanye kwizitho zangaphakathi ezifana nesibindi kunye ne-spleen. Ngenxa yesi sizathu, umzimba unokufumana iimpawu ze-allergy, ezinje ngokurhawuzelela, ukubomvu kolusu, iintlungu zesisu, kunye nesiyezi, ngaphandle kwesizathu esicacileyo.
Igama elithi "systemic" lithetha ukuba linokuchaphazela umzimba wonke, kungekuphela nje indawo enye. Kukho iintlobo ezininzi ezahlukeneyo zesi sifo, ukusuka kwiintlobo ezibuthathaka, ezikhula kancinci (ezingasebenzi kakuhle) ukuya kwiintlobo eziphucukileyo nezinomhlaza.
Ndingazi njani ukuba i-Ayvakit ilungile kum?
Ugqirha wakho uza kwenza uthotho lweemvavanyo ukuze afumanise ukuba loluphi uhlobo lweSystemic Mastocytosis onayo. Iziphumo zolo vavanyo kunye neempawu zakho ziya kuqinisekisa ukuba iAyvakit ikulungele na. I-Ayvakit okwangoku ivunyiwe ukunyanga iintlobo ezahlukeneyo zeSystemic Mastocytosis kubantu abadala.
| Uhlobo lwesifo | Ingcaciso elula |
|---|---|
| I-Advanced Systemic Mastocytosis (AdvSM) - uhlobo olunzima nolusasazeka ngokukhawuleza | |
| I-Mastocytosis Enobundlongondlongo Ecwangcisiweyo | Ukuqokelelana kakhulu kwee-mast cells kukhokelela ekungasebenzi kakuhle kwamalungu omzimba afana nesibindi, indlela yokugaya ukutya, kunye namathambo. |
| I-SM enesifo segazi esihambelanayo (i-hematological neoplasm) | Njengoko iiseli ze-mast zisanda, ezinye izifo ezinxulumene negazi (umz. i-myelodysplastic syndromes) zinokuvela. |
| I-leukemia yeseli ye-mast | Lo ngumhlaza ongaqhelekanga kakhulu, okhula ngokukhawuleza apho iiseli zemast ziqokelelana ngamanani amakhulu (ngaphezulu kwama-20%) kwizicubu. |
| Indolent Systemic Mastocytosis (ISM) - uhlobo oluthambileyo nolukhula kancinci | |
| I-Indolent Systemic Mastocytosis (ISM) | Lo asingomhlaza. Iiseli zesisu zikhula kancinci. Nangona zinokubangela iimpawu ezingathandekiyo, ukungasebenzi kakuhle kwamalungu omzimba akuqhelekanga. |
Kubalulekile: Kunokuba yingozi ukusebenzisa i-Ayvakit ukuba inani leeplatelet zakho zegazi liphantsi kakhulu. Iiplatelet luhlobo lweeseli ezinceda ekumiseni ukopha. Ugqirha wakho uza kujonga inani leeplatelet zakho rhoqo ngaphambi kokuba uqale ukuthatha eli yeza kwaye ngelixa ulisebenzisa. Oku kwenzelwa ukunciphisa umngcipheko weziphumo ebezingalindelekanga ezinzulu, ezifana nokopha ebuchotsheni.
Eli yeza le-Ayvakit lisebenza njani emzimbeni?
I-Ayvakit liyeza elikwiqela lamayeza abizwa ngokuba yi-'kinase inhibitors'. IiKinases luhlobo lweprotheyini ye-enzyme enceda ukuthumela imiqondiso phakathi kweeseli emzimbeni wethu. Ezi miqondiso zibaluleke kakhulu kwizinto ezifana nokukhula kweeseli kunye nokwahlukana kwazo.
Abantu abaninzi abaneSystemic Mastocytosis banenguqu kwiproteni ebizwa ngokuba yiKIT efumaneka kwiiseli zemast . Olu tshintsho lubangela ukuba iproteni yeKIT isebenze kakhulu, nto leyo ebangela ukuba iiseli zemast zikhule ngokungalawulekiyo.
Iyeza le-Ayvakit lijolise ngqo kuloo protein ye-KIT eguqukileyo kwaye limise ukusebenza kwayo.Njengokuthulisa inqaba ekhupha isignali engalunganga. Oku kuyayekisa ukuveliswa kakhulu kweeseli zemast, kunciphisa iimpawu, kwaye kulawula umonakalo kwizitho zomzimba.
Ndingayisebenzisa njani iipilisi ze-Ayvakit?
I-Ayvakit yipilisi. Eyona nto ibalulekileyo xa uyithatha kukuyithatha ungenanto esiswini . Oko kuthetha ukuba, kufuneka uyithathe ubuncinane iyure ngaphambi kokutya, okanye iiyure ezimbini emva kokutya. Kulapho iyeza lifunxwa ngcono ngumzimba.
Iidosi ezimiselweyo ngokuqhelekileyo zezi zilandelayo:
- Kwi-Advanced Systemic Mastitis TOS (AdvSM): 200mg kanye ngemini.
- Kwi-Indolent Systemic Mastocytosis (ISM): 25mg kanye ngemini.
Olu lunyango oluhlala luthatha ixesha elide. Ugqirha wakho uza kukuxelela ukuba uqhubeke usela iyeza ude ufumane iziphumo ezilungileyo kulo kwaye ungabi naziphumo zimbi kakhulu. Ukuba uneziphumo ebezingalindelekanga, ugqirha wakho angagqiba ekubeni anciphise umthamo wakho, awuyeke okwethutyana, okanye awuyeke ngokupheleleyo.
Landela imiyalelo kagqirha wakho ngokuchanekileyo. Ungaze uyeke ukuthatha amayeza akho okanye utshintshe umthamo ngaphandle kokuthetha nogqirha wakho. Funa ingcebiso kagqirha ngokukhawuleza ukuba ufumana naziphi na iziphumo ebezingalindelekanga ezibonakala zinzima.
Uphando luthini ngempumelelo ye-Ayvakit?
Kukho ulwazi malunga nezilingo ezintathu zeklinikhi ezenziweyo ukuphanda ukusebenza kakuhle kunye nokhuseleko lweyeza le-Ayvakit.
Kwi-AdvSM (enzima): Kwiizifundo ezimbini, i-EXPLORER kunye ne-PATHFINDER, ama-57% abantu abathathe i-Ayvakit, okanye ngaphezulu kwesiqingatha, babeneempawu ezinciphileyo kunye nokubuyela emsebenzini kwizitho ezichaphazelekayo. Isiqingatha sabo babonise iziphumo baqhubeka nolo phuculo kangangeenyanga ezingama-38, okanye ngaphezulu kweminyaka emi-3.
Kwi-ISM (uhlobo oluphantsi): Kuphononongo lwe-PIONEER, abo bathathe i-Ayvakit babeneempawu ezimbalwa kakhulu (ezifana nokurhawuzelelwa, iintlungu zesisu, kunye nokudinwa) kunabo bathathe i-placebo. Oku kuthetha ukuba eli yeza libonelele ngoncedo olukhulu ekuphuculeni umgangatho wobomi.
Ezi zifundo zibonisa ukuba i-Ayvakit yindlela yonyango esebenzayo kakhulu kwi-Systemic Mastocytosis. Nangona kunjalo, khumbula ukuba ayinyangi ngokupheleleyo esi sifo, kwaye iziphumo aziyi kufana kuye wonke umntu.
Ngaba kukho iingxaki ngamanye amayeza? (Ukusebenzisana kwamayeza)
Ewe, i-Ayvakit inokusebenzisana namanye amayeza. Ingakumbi lawo achaphazela i-enzyme ye-CYP3A esibindini sethu. Amanye amayeza anokunyusa izinga le-Ayvakit emzimbeni kwaye anyuse iziphumo ebezingalindelekanga, ngelixa amanye amayeza anokunciphisa izinga le-Ayvakit kwaye anciphise ukusebenza kwayo.
Ngoko ke, qiniseka ukuba wazisa ugqirha wakho ngayo yonke imithi oyithathayo (amayeza ezinye izifo, iivithamini, amayeza esintu, izongezo). Emva koko unokujonga naziphi na iingxaki aze, ukuba kuyimfuneko, atshintshe amanye amayeza akho okanye atshintshe umthamo we-Ayvakit.
Kwakhona, ukuba uthatha amayeza okunciphisa igazi, afana ne -warfarin (Coumadin, Jantoven) , umngcipheko wakho wokopha igazi engqondweni evela kwi-Ayvakit unokwanda kancinci. Ke ngoko, ugqirha wakho uza kukujonga ngokusondeleyo ukuba usebenzisa amayeza anjalo.
Ungayifumana njani iyeza le-Ayvakit?
I-Ayvakit liyeza elinikwa kuphela ngugqirha, elidla ngokunikwa yingcali ye-hematologist okanye ingcali yomhlaza.
Eli asiloyeza onokulifumana kwikhemesti eqhelekileyo . Lifumaneka 'kwikhemesti ekhethekileyo'. Nje ukuba ugqirha wakho athumele iphepha leyeza kwikhemesti ekhethekileyo, angalithumela ekhayeni lakho okanye kwiofisi kagqirha wakho.
Umyalezo Wokuya Ekhaya
- I-Systemic Mastocytosis (SM) yimeko engaqhelekanga ebangelwa kukuqokelelana kakhulu kweeseli zemast emzimbeni.
- I-Ayvakit liyeza lanamhlanje elisebenza kakhulu nelijongana nonobangela wesi sifo kwaye lisinyange.
- Eli yeza ngokuqinisekileyo lifanele lithathwe esiswini esingenanto (iyure enye ngaphambi kokutya okanye iiyure ezimbini emva kokutya).
- Kubaluleke kakhulu ukwazisa ugqirha wakho malunga nawo onke amanye amayeza, iivithamini, kunye nezongezo ozithathayo.
- Akufuneki uyeke ukuthatha amayeza okanye utshintshe umthamo ngaphandle kwengcebiso kagqirha.
- Thetha nogqirha wakho ngoko nangoko malunga nokungonwabi okanye iziphumo ebezingalindelekanga. Musa ukoyika, yazi ulwazi olufanelekileyo, kwaye ngonyango olufanelekileyo, ungaphila ubomi obusempilweni.
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