I-Transthyretin Amyloid Cardiomyopathy (ATTR-CM): Izimpawu, Ukwelashwa, nokuningi

-Delile

• Ziyini izimpawu ze-ATTR-CM?
• Yini ebangela i-ATTR-CM?
• Ifa (lomndeni) i-ATTR
• Uhlobo lwasendle i-ATTR
• I-ATTR-CM itholakala kanjani?
• Iphathwa kanjani i-ATTR-CM?
• Yiziphi izinto eziyingozi?
• Uyini umbono uma une-ATTR-CM?
• Okubalulekile

I-Transthyretin amyloidosis (ATTR) yisimo lapho kufakwa khona iprotheni ebizwa ngokuthi i-amyloid enhliziyweni yakho, kanye nasemithanjeni yakho nakwezinye izitho zomzimba. Kungaholela esifo senhliziyo esibizwa nge-transthyretin amyloid cardiomyopathy (ATTR-CM).

I-Transthyretin uhlobo oluthile lwamaprotheni we-amyloid afakwe enhliziyweni yakho uma une-ATTR-CM. Imvamisa iphatha uvithamini A nehomoni yegilo emzimbeni wonke.

Kunezinhlobo ezimbili ze-transthyretin amyloidosis: uhlobo lwasendle kanye nefa.

Uhlobo lwasendle i-ATTR (eyaziwa nangokuthi i-senile amyloidosis) ayibangelwa ukuguquka kwezakhi zofuzo. Amaprotheni abekiwe asesimweni sawo esingaguquki.

Ku-ATTR eyifa, amaprotheni akhiwa ngokungalungile (afingqiwe kahle). Bese iyahlangana futhi inamathuba amaningi okuthi igcine izicubu zomzimba wakho.

Ziyini izimpawu ze-ATTR-CM?

I-ventricle yenhliziyo yakho ipompa igazi emzimbeni wakho. I-ATTR-CM ingathinta izindonga zaleli gumbi lenhliziyo.

Ama-amyloid deposits enza izindonga ziqine, ngakho-ke azikwazi ukuphumula noma ukukhama ngokujwayelekile.

Lokhu kusho ukuthi inhliziyo yakho ayikwazi ukugcwalisa ngempumelelo (ukunciphisa umsebenzi we-diastolic) ngegazi noma ngokumpompa igazi emzimbeni wakho (kunciphise ukusebenza kwe-systolic). Lokhu kubizwa ngokuthi yi-cardiomyopathy evimbelayo, okuwuhlobo lokwehluleka kwenhliziyo.

Izimpawu zalolu hlobo lokwehluleka kwenhliziyo zifaka:

• ukuphefumula okuncane (i-dyspnea), ikakhulukazi lapho ulele phansi noma ngokuzikhandla
• ukuvuvukala emilenzeni yakho (i-peripheral edema)
• ubuhlungu besifuba
• ukushaya okungajwayelekile (arrhythmia)
• ukushaya kwenhliziyo
• ukukhathala
• isibindi esandisiwe kanye nobende (hepatosplenomegaly)
• uketshezi esiswini sakho (ascites)
• ukungathandi ukudla
• ikhanda elikhanyayo, ikakhulukazi lapho lime
• ukuquleka (i-syncope)

Uphawu oluyingqayizivele oluvela kwesinye isikhathi ingcindezi ephezulu yegazi ethi iba ngcono kancane. Lokhu kwenzeka ngoba njengoba inhliziyo yakho ingasebenzi kahle, ayikwazi ukupompa kanzima ngokwanele ukwenza umfutho wegazi lakho uphakame.

Ezinye izimpawu ongase ube nazo kusuka kuma-amyloid deposits kwezinye izingxenye zomzimba ngaphandle kwenhliziyo yakho zifaka:

• umhubhe we-carpal syndrome
• ukuvutha nokuba ndikindiki ezingalweni nasemilenzeni (i-peripheral neuropathy)
• ubuhlungu beqolo obuvela emgogodleni stenosis

Uma unezinhlungu esifubeni, shayela u-911 ngokushesha.

Funa usizo lwezokwelashwa ngokushesha uma uba nalezi zimpawu:

• ukukhuphuka komoya okwandayo
• ukuvuvukala okukhulu komlenze noma ukuzuza ngokushesha kwesisindo
• ukushaya kwenhliziyo okusheshayo noma okungajwayelekile
• ikhefu noma ukushaya kwenhliziyo okuhamba kancane
• isiyezi
• ukuquleka

Yini ebangela i-ATTR-CM?

Kunezinhlobo ezimbili ze-ATTR, futhi ngayinye inembangela eyingqayizivele.

Ifa (lomndeni) i-ATTR

Kulolu hlobo, i-transthyretin igoqeka kahle ngenxa yokuguquka kofuzo. Kungadluliselwa kusuka kumzali kuye enganeni ngokusebenzisa izakhi zofuzo.

Izimpawu zivame ukuqala kuma-50s akho, kepha zingaqala ekuqaleni kweminyaka yakho yama-20.

Uhlobo lwasendle i-ATTR

Ukusongelwa kahle kwamaprotheni kuyinto ejwayelekile. Umzimba wakho unezindlela zokususa la maprotheni ngaphambi kokuba abangele inkinga.

Njengoba ukhula, lezi zinqubo ziye zingasebenzi kahle, futhi amaprotheni asongelwe kahle angahlangana futhi akhe amadiphozi. Yilokho okwenzeka kuhlobo lwasendle i-ATTR.

I-ATTR yohlobo lwasendle akuyona inguquko yezakhi zofuzo, ngakho-ke ayinakudluliswa ngezakhi zofuzo.

Izimpawu zivame ukuqala kuma-60s noma 70s akho.

I-ATTR-CM itholakala kanjani?

Ukuxilongwa kungaba nzima ngoba izimpawu ziyefana nezinye izinhlobo zokwehluleka kwenhliziyo. Ukuhlolwa okuvame ukusetshenziselwa ukuxilongwa kufaka phakathi:

• i-electrocardiogram ukuthola ukuthi izindonga zenhliziyo zijiyile yini kusuka kumadiphozithi (imvamisa ugesi kagesi uphansi)
• i-echocardiogram ukubheka izindonga ezijiyile nokuhlola ukusebenza kwenhliziyo nokubheka izindlela zokuphumula ezingavamile noma izimpawu zokucindezela okwandayo enhliziyweni
• i-MRI yenhliziyo ukubheka ama-amyloid odongeni lwenhliziyo
• inhliziyo yemisipha biopsy ukubheka okufakwa kuma-amyloid ngaphansi kwesibonakhulu
• izifundo zofuzo ezifuna ifa le-ATTR

Iphathwa kanjani i-ATTR-CM?

I-Transthyretin ikhiqizwa kakhulu yisibindi sakho. Ngalesi sizathu, ifa le-ATTR-CM liphathwa ngokufakelwa kwesibindi lapho kungenzeka. Ngoba inhliziyo ivame ukulimala ngokungenakuphikwa lapho kutholakala isimo, ukufakelwa inhliziyo kuvame ukwenziwa ngasikhathi sinye.

Ngo-2019, imishanguzo emibili egunyaziwe yokwelashwa kwe-ATTR_CM: tafamidis meglumine (Vyndaqel) kanye ne-tafamidis (Vyndamax) capsules.

Ezinye zezimpawu ze-cardiomyopathy zingelashwa ngama-diuretics ukususa uketshezi oluningi.

Eminye imithi evame ukusetshenziselwa ukwelapha ukwehluleka kwenhliziyo, njenge-beta-blockers ne-digoxin (i-Lanoxin), ingaba yingozi kulesi simo futhi akufanele isetshenziswe njalo.

Yiziphi izinto eziyingozi?

Izici zobungozi zefa le-ATTR-CM zifaka:

• umlando womndeni walesi simo
• ubulili besilisa
• uneminyaka engaphezu kwengu-50
• Isizukulwane sase-Afrika

Izici zobungozi bohlobo lwasendle i-ATTR-CM zifaka:

• uneminyaka engaphezu kwengama-65
• ubulili besilisa

Uyini umbono uma une-ATTR-CM?

Ngaphandle kokufakelwa kwesibindi nenhliziyo, i-ATTR-CM izoba kubi ngokuhamba kwesikhathi. Ngokwesilinganiso, abantu abane-ATTR-CM baphila ngemuva kokuxilongwa.

Isimo singaba nomthelela owandayo kwikhwalithi yakho yokuphila, kepha ukwelapha izimpawu zakho ngemithi kungasiza kakhulu.

Okubalulekile

I-ATTR-CM ibangelwa ukuguquka kwezakhi zofuzo noma ihlobene nobudala. Kuholela ezimpawu zokwehluleka kwenhliziyo.

Ukuxilongwa kunzima ngoba kufana nezinye izinhlobo zokwehluleka kwenhliziyo. Kuya ngokuya ngokuya ngokuhamba kwesikhathi kepha kungelashwa ngokufakelwa kwesibindi nenhliziyo nemithi yokusiza ukulawula izimpawu.

Uma uhlangabezana nanoma yiziphi izimpawu ze-ATTR-CM ezibalwe ekuqaleni, xhumana nodokotela wakho.