I-Transthyretin Amyloid Cardiomyopathy (ATTR-CM): Iimpawu, unyango kunye nokunye

Umxholo

• Ziintoni iimpawu ze-ATTR-CM?
• Yintoni ebangela i-ATTR-CM?
• Ilifa (usapho) ATTR
• Uhlobo lwe-ATTR yasendle
• Ichongwa njani i-ATTR-CM?
• Iphathwa njani i-ATTR-CM?
• Ziziphi izinto ezinobungozi?
• Ithini imbonakalo ukuba une-ATTR-CM?
• Umgca wezantsi

I-Transthyretin amyloidosis (ATTR) yimeko apho iprotein ebizwa ngokuba yi-amyloid ifakwa entliziyweni yakho, nakwimithambo-luvo nakwezinye izitho zomzimba. Ingakhokelela kwisifo sentliziyo esibizwa ngokuba yi-transthyretin amyloid cardiomyopathy (ATTR-CM).

I-Transthyretin luhlobo oluthile lweprotheni ye-amyloid efakwe entliziyweni yakho ukuba unayo i-ATTR-CM. Ngokwesiqhelo ithwala ivithamini A kunye nedlala lengqula emzimbeni wonke.

Zimbini iintlobo ze-transthyretin amyloidosis: uhlobo lwasendle kunye nelifa.

Uhlobo lwasendle i-ATTR (ekwabizwa ngokuba yi-senile amyloidosis) ayibangelwa kukutshintsha kwemfuzo. Iprotein efakiweyo ikwimo engaguqukiyo.

Kwi-ATTR yelifa, iprotheni yenziwa ngokungachanekanga (isongelwe kakubi). Emva koko iyadibana kwaye kunokwenzeka ukuba iphele kwizicwili zomzimba wakho.

Ziintoni iimpawu ze-ATTR-CM?

Intliziyo yakho yasekhohlo yempompo yegazi emzimbeni wakho. I-ATTR-CM inokuchaphazela iindonga zeli gumbi lentliziyo.

Iidipozithi ze-amyloid zenza iindonga ziqine, ukuze zingakwazi ukuphumla okanye ukucudisa ngesiqhelo.

Oku kuthetha ukuba intliziyo yakho ayinako ukugcwalisa ngokufanelekileyo (ukunciphisa umsebenzi we-diastolic) ngegazi okanye ukumpompa igazi emzimbeni wakho (ukunciphisa ukusebenza kwe-systolic). Oku kubizwa ngokuba kukuthintela i-cardiomyopathy, eluhlobo lokusilela kwentliziyo.

Iimpawu zolu hlobo lokusilela kwentliziyo zibandakanya:

• ukuphefumla kancinci (iDyspnea), ngakumbi xa ulele phantsi okanye uzimisele
• ukuvuvukala kwimilenze yakho (i-peripheral edema)
• iintlungu zesifuba
• ukubetha okungaqhelekanga (arrhythmia)
• ukubetha
• ukudinwa
• isibindi esandisiweyo kunye ne-spleen (hepatosplenomegaly)
• ulwelo esiswini sakho (ascites)
• ukungathandi ukutya
• intloko ebukhaphukhaphu, ngakumbi xa imile
• ukuphelelwa ngamandla (syncope)

Uphawu olwahlukileyo olwenzeka ngamanye amaxesha luxinzelelo lwegazi oluphukayo ngokuthe chu. Oku kwenzeka ngenxa yokuba intliziyo yakho ingasebenzi kakuhle, ayinakupompa ngokwaneleyo ukwenza uxinzelelo lwegazi luphezulu.

Ezinye iimpawu onokuba nazo kwiidiphozithi ze-amyloid kwezinye iindawo zomzimba ngaphandle kwentliziyo yakho zibandakanya:

• Isifo seCarpal tunnel
• ukutshisa kunye nokubhabha ezingalweni nasemilenzeni (peripheral neuropathy)
• umqolo obuhlungu obuvela kwi-spinal stenosis

Ukuba unentlungu esifubeni, tsalela umnxeba ku-911 kwangoko.

Funa unyango ngokukhawuleza ukuba uvelisa ezi mpawu:

• ukwandisa ukuphefumla okufutshane
• ukudumba okunzima komlenze okanye ukukhawuleza ukutyeba
• ukukhawuleza kwentliziyo okanye okungaqhelekanga
• ikhefu okanye ukubetha kwentliziyo kancinci
• isiyezi
• isiqaqa

Yintoni ebangela i-ATTR-CM?

Zimbini iintlobo ze-ATTR, kwaye nganye inesizathu esahlukileyo.

Ilifa (usapho) ATTR

Kolu hlobo, i-transthyretin iphosakele ngenxa yokutshintsha kwemfuza. Ingadluliselwa ukusuka kumzali iye emntwaneni ngokusebenzisa iijethi.

Iimpawu zihlala ziqala kwi-50s yakho, kodwa zinokuqala kwangoko kwi-20s yakho.

Uhlobo lwe-ATTR yasendle

Ukusasazwa gwenxa kweeproteni yinto eqhelekileyo. Umzimba wakho uneendlela zokususa ezi proteni ngaphambi kokuba zibangele ingxaki.

Njengoko uneminyaka yobudala, ezi ndlela ziye zingasebenzi kakuhle, kwaye iiproteni eziphosakeleyo zinokuxinana kwaye zenze iidipozithi. Yiloo nto eyenzekayo kwi-ATTR yohlobo lwasendle.

Uhlobo lwasendle i-ATTR ayilulo uguquko kwimfuza, ngenxa yoko ayinakudluliswa ngemfuza.

Iimpawu zihlala ziqala kwi-60s okanye 70s yakho.

Ichongwa njani i-ATTR-CM?

Ukufumanisa isifo kunokuba nzima kuba iimpawu ziyafana nezinye iintlobo zentliziyo yokusilela. Uvavanyo oluqhelekileyo olusetyenziselwa ukuxilongwa lubandakanya:

• i-electrocardiogram ukumisela ukuba iindonga zentliziyo zixinekile ukusuka kwidiphozithi (ngesiqhelo umbane wombane uphantsi)
• i-echocardiogram ukukhangela iindonga ezomeleleyo kunye nokuvavanya ukusebenza kwentliziyo kunye nokujonga iipateni zokuphumla ezingaqhelekanga okanye iimpawu zoxinzelelo olwandileyo entliziyweni
• I-MRI yentliziyo ukujonga i-amyloid eludongeni lwentliziyo
• I-biopsy yentliziyo yokujonga iidipozithi ze-amyloid phantsi kwemicroscope
• izifundo zofuzo zikhangela kwilifa le-ATTR

Iphathwa njani i-ATTR-CM?

I-Transthyretin iveliswa ikakhulu sisibindi sakho. Ngesi sizathu, i-ATTR-CM yelifa iyanyangwa ngokufakelwa kwesibindi xa kunokwenzeka. Ngenxa yokuba intliziyo ihlala yonakele ingenakuguqulwa xa imeko ifunyaniswa, ukufakelwa kwentliziyo kuhlala kwenziwa ngaxeshanye.

Kwi-2019, amayeza amabini avunyiweyo kunyango lwe-ATTR_CM: tafamidis meglumine (Vyndaqel) kunye ne-tafamidis (Vyndamax) njengezingxobo.

Ezinye zeempawu ze-cardiomyopathy zinokunyangwa nge-diuretics ukususa ulwelo oluninzi.

Amanye amayeza aqhele ukusetyenziselwa ukunyanga ukusilela kwentliziyo, njenge-beta-blockers kunye ne-digoxin (Lanoxin), inokuba yingozi kule meko kwaye akufuneki isetyenziswe rhoqo.

Ziziphi izinto ezinobungozi?

Imiba yomngcipheko kwilifa le-ATTR-CM ibandakanya:

• Imbali yosapho yale meko
• isini esiyindoda
• ungaphezulu kweminyaka engama-50
• Inzala yaseAfrika

Imiba yomngcipheko kuhlobo lwasendle i-ATTR-CM ibandakanya:

• ungaphezulu kweminyaka engama-65
• isini esiyindoda

Ithini imbonakalo ukuba une-ATTR-CM?

Ngaphandle kwesibindi kunye nokufakelwa kwentliziyo, i-ATTR-CM iya kuba mandundu ngokuhamba kwexesha. Ngokomndilili, abantu abane-ATTR-CM baphila emva kokuxilongwa.

Imeko inokuba nefuthe elonyukayo kumgangatho wobomi bakho, kodwa ukunyanga iimpawu zakho ngamayeza kunokunceda kakhulu.

Umgca wezantsi

I-ATTR-CM ibangelwa kukutshintsha kwemfuza okanye inxulumene nobudala. Kukhokelela kwiimpawu zokusilela kwentliziyo.

Ukuxilongwa kunzima ngenxa yokufana kwayo nezinye iintlobo zokusilela kwentliziyo. Iya isiba mandundu ngokuhamba kwexesha kodwa inokunyangwa ngokufakelwa kwesibindi nentliziyo kunye namayeza ukunceda ukulawula iimpawu.

Ukuba ufumana naziphi na iimpawu ze-ATTR-CM ezidweliswe ngaphambili, nxibelelana nogqirha wakho.