Kuyini i-Systemic Mastocytosis (SM)? Ake sifunde ngomuthi i-Ayvakit.

Ingabe udokotela wakho ukutshelile ukuthi unesifo esingavamile esibizwa ngokuthi i-Systemic Mastocytosis ? Ungase uzizwe wesaba futhi ukhathazekile kancane uma uzwa lokho. Kujwayelekile kakhulu. Kodwa ungakhathazeki. Manje kunezindlela zokwelapha ezinhle kakhulu nezisebenzayo zalezi zimo. Namuhla sizokhuluma ngomuthi i-Ayvakit (avapritinib) owawunikezwe wona. Ake siqonde konke ngalesi sifo kanye nomuthi kalula nangokucacile.

Iyini ngempela i-Systemic Mastocytosis (SM)?

Kalula nje, i-Systemic Mastocytosis yisimo esingavamile lapho uhlobo lweseli olubizwa ngokuthi 'amaseli e-mast' emzimbeni wethu lukhula ngokweqile. Lawa maseli e-mast ayingxenye yesimiso sethu somzimba sokuzivikela. Njengamandla okuzivikela ezwe lethu. Uma into yangaphandle ingena emzimbeni, lawa maseli asebenza futhi akhiphe amakhemikhali afana ne-'histamine'.

Cabanga ukuthi uma umiyane ukuluma, indawo iba lusizi futhi ibe bomvu, akunjalo? Yilokho okwenzekayo ngenxa yale histamine. Kodwa kumuntu one-Systemic Mastocytosis, lawa maseli e-mast aqoqana ngendlela engalawuleki emzimbeni. Aqoqana esikhumbeni, emnkantsheni, noma ezithweni zangaphakathi njengesibindi kanye ne-spleen. Ngenxa yalokhu, umzimba ungabhekana nezimpawu zokungezwani komzimba, njengokulunywa, ukubomvu kwesikhumba, ubuhlungu besisu, kanye nesiyezi, ngaphandle kwesizathu esibonakalayo.

Igama elithi "systemic" lisho ukuthi lingathinta umzimba wonke, hhayi indawo eyodwa kuphela. Kunezinhlobo eziningi ezahlukene zalesi sifo, kusukela ezinhlotsheni ezincane, ezikhula kancane (ezingasebenzi kahle) kuya ezinhlotsheni ezithuthukile, ezinomdlavuza.

Ngingazi kanjani ukuthi i-Ayvakit ingifanele?

Udokotela wakho uzokwenza uchungechunge lwezivivinyo ukuze anqume ukuthi hlobo luni lwe-Systemic Mastocytosis onayo. Imiphumela yalezo zivivinyo kanye nezimpawu zakho kuzonquma ukuthi i-Ayvakit ikulungele yini. I-Ayvakit okwamanje ivunyelwe ukwelapha izinhlobo eziningana ze-Systemic Mastocytosis kubantu abadala.

Lo muthi we-Ayvakit usebenza kanjani emzimbeni?

I-Ayvakit umuthi ongoweqembu lemithi ebizwa ngokuthi 'i-kinase inhibitors'. Ama-Kinase awuhlobo lweprotheni yama-enzyme esiza ukuthumela izimpawu phakathi kwamaseli emzimbeni wethu. Lezi zimpawu zibaluleke kakhulu ezintweni ezifana nokukhula kwamaseli nokuhlukana kwawo.

Abantu abaningi abane-Systemic Mastocytosis banoguquko kuphrotheni ebizwa ngokuthi i-KIT etholakala kumaseli e-mast . Lolu guquko lubangela ukuthi iphrotheni ye-KIT isebenze ngokweqile, okubangela ukuba amaseli e-mast akhule ngokungalawuleki.

Umuthi we-Ayvakit uhlose ngqo leyo phrotheni ye-KIT eguqukile futhi uyivimbele ekusebenzeni.Njengokuthulisa umbhoshongo okhipha isignali engalungile. Lokhu kuvimbela ukukhiqizwa ngokweqile kwamaseli e-mast, kunciphisa izimpawu, futhi kulawula ukulimala kwezitho zomzimba.

Kufanele ngisebenzise kanjani amaphilisi e-Ayvakit?

I-Ayvakit iyiphilisi. Into ebaluleke kakhulu uma uyithatha ukuyithatha esiswini esingenalutho . Lokho kusho ukuthi, kufanele uyithathe okungenani ihora ngaphambi kokudla, noma amahora amabili ngemva kokudla. Yilapho umuthi umuncwa kahle khona emzimbeni.

Imithamo evame ukunqunywa yilena elandelayo:

• Kwe-Advanced Systemic Mastitis TOS (AdvSM): 200mg kanye ngosuku.
• Nge-Indolent Systemic Mastocytosis (ISM): 25mg kanye ngosuku.

Lokhu kuvame ukuba ukwelashwa kwesikhathi eside. Udokotela wakho uzokutshela ukuthi uqhubeke uthatha umuthi uze uthole imiphumela emihle kuwo futhi ungabi nemiphumela emibi kakhulu. Uma unemiphumela emibi, udokotela wakho angase anqume ukwehlisa umthamo wakho, awuyeke okwesikhashana, noma awuyeke ngokuphelele.

Ucwaningo luthini ngempumelelo ye-Ayvakit?

Kukhona ulwazi mayelana nezivivinyo ezintathu zemitholampilo ezenziwe ukuze kuhlolwe ukusebenza kahle nokuphepha komuthi we-Ayvakit.

Nge-AdvSM (enzima): Ezifundweni ezimbili, i-EXPLORER kanye ne-PATHFINDER, ama-57% abantu abathathe i-Ayvakit, noma ngaphezu kwengxenye, babe nokwehla kwezimpawu kanye nokululama okuthile ekusebenzeni kwezitho ezithintekile. Ingxenye yalabo ababonise imiphumela balondoloze lokho kuthuthuka izinyanga ezingama-38, noma iminyaka engaphezu kwemi-3.

Nge-ISM (uhlobo oluthambile): Ocwaningweni lwe-PIONEER, labo abathatha i-Ayvakit babenezimpawu ezincane kakhulu (njengokulunywa, ubuhlungu besisu, nokukhathala) kunalabo abathatha i-placebo. Lokhu kusho ukuthi lo muthi unikeze impumuzo ebalulekile ekuthuthukiseni ikhwalithi yokuphila.

Lezi zifundo zibonisa ukuthi i-Ayvakit iyindlela yokwelapha ephumelela kakhulu ye-Systemic Mastocytosis. Kodwa-ke, khumbula ukuthi ayilaphi ngokuphelele lesi sifo, futhi imiphumela ngeke ifane kuwo wonke umuntu.

Ingabe kungaba nezinkinga ngeminye imithi? (Ukusebenzisana kwezidakamizwa)

Yebo, i-Ayvakit ingasebenzisana neminye imithi. Ikakhulukazi leyo ethinta i-enzyme i-CYP3A esibindini sethu. Eminye imithi ingakhuphula izinga le-Ayvakit emzimbeni futhi yandise imiphumela emibi, kanti eminye imithi inganciphisa izinga le-Ayvakit futhi inciphise ukusebenza kwayo.

Ngakho-ke, qiniseka ukuthi wazisa udokotela wakho ngayo yonke imithi oyithathayo (imithi yezinye izifo, amavithamini, imithi yendabuko, izithasiselo). Angabe esehlola noma yiziphi izinkinga, futhi uma kudingeka, ashintshe eminye imithi yakho noma ashintshe umthamo we-Ayvakit.

Futhi, uma uthatha imithi yokunciphisa igazi, njenge -warfarin (Coumadin, Jantoven) , ingozi yakho yokopha igazi ebuchosheni evela ku-Ayvakit ingase ikhuphuke kancane. Ngakho-ke, udokotela wakho uzokuqapha kakhulu uma uthatha imithi enjalo.

Ungawuthola kanjani umuthi we-Ayvakit?

I-Ayvakit umuthi onikezwa udokotela kuphela, ngokuvamile onikezwa udokotela wegazi noma udokotela wezimila.

Lona akuyona imithi ongayithola ekhemisi elivamile . Uyatholakala 'ekhemisi elikhethekile'. Uma udokotela wakho esethumele umuthi kulelo khemisi elikhethekile, angawuthumela ekhaya lakho noma ehhovisi likadokotela wakho.