Ngaba ngamanye amaxesha uziva unesizungu nangona uhamba umgama omfutshane? Ngaba uziva uphelelwa ngumoya xa usiya kulala? Ngaba imilenze namaqatha akho ayavuvukala? Ngaba intliziyo yakho ibetha ngendlela engaqhelekanga okanye ngokukhawuleza? Nangona sihlala sicinga ukuba ezi zinto zizinto eziqhelekileyo ezenzeka njengoko sikhula, ngamanye amaxesha emva kwezi mpawu kunokubakho imeko esingazivanga kangako ngayo kwaye enokuba yingozi ukuba ayifunyaniswanga ngokufanelekileyo. Esinye sezifo ezinjalo yiTransthyretin Amyloidosis, okanye i-ATTR-CM ngamafutshane.
Ngamafutshane, yintoni iTransthyretin Amyloidosis (ATTR-CM)?
Eli gama liyinkimbinkimbi kancinci, ngoko masilichaze igama negama. Emva koko uza kuliqonda lula kakhulu.
- I-Transthyretin (TTR): Olu luhlobo lweproteni emizimbeni yethu. Yenziwa sisibindi sethu. Umsebenzi wayo ophambili kukuthwala i-vitamin A kunye ne-thyroid hormone i-thyroxine egazini lethu ukuya kwiindawo ezifunekayo emzimbeni. Ifana nomntu ohambisa impahla ethwele iipasela.
- I-Amyloidosis: Oku kwenzeka xa uhlobo lweproteni, njengoko kukhankanyiwe apha ngasentla , lugoba ngendlela engafanelekanga, luhlangana, kwaye lwenze ii-fibrils, ezibonakala ngathi ziibhola ezincinci zentambo, kwaye zifakwa kwizitho zethu zomzimba. Xa zifakwa ngale ndlela, ukusebenza okuqhelekileyo kwezo zitho kuyaphazamiseka.
- I-Cardiomyopathy (CM): Oku kubhekisa kwisifo semisipha yentliziyo .
Ngoku, xa sidibanisa konke oku, iTransthyretin Amyloidosis (ATTR-CM) sisifo apho iproteni iTTR, eyenziwa sisibindi sethu, idityaniswe ize ihlangane, ingakumbi kwimisipha yentliziyo yethu, nto leyo ebangela ukuba imisipha yentliziyo iqine ize iqine, nto leyo eyenza ingakwazi ukupompa igazi ngokufanelekileyo.
Khawuthelekelele ukuba inxalenye ephambili yokupompa igazi yentliziyo yakho (i-ventricle yasekhohlo) ifana nebhola yerabha eguquguqukayo. Ifinyela lula kwaye ikhule ukuze ipompe igazi emzimbeni wonke. Ngoku, xa ezo ziqu zeproteni zifakwa kuloo msipha wentliziyo, loo nxalenye ifana nerabha iba rhabaxa kwaye ixinene. Iba njengebhola yeqokobhe lekhokhonathi endaweni yebhola yerabha. Emva koko ayinakufinyela kwaye ikhule lula, akunjalo? Yiloo nto kwenzeka entliziyweni kwesi sifo. Yiyo loo nto iimeko ezifana nokusilela kwentliziyo zenzeka.
Kukho iintlobo ezimbini eziphambili zesi sifo.
Isifo se-ATTR-CM sinokwahlulwa sibe ziintlobo ezimbini eziphambili.
1. I-ATTR-CM yoSapho okanye yelifa:
Oku kubangelwa kukuguquka kwezakhi zofuzo zethu. Oku kuthetha ukuba sisenokuba sifumene i-gene yesi sifo kumama, utata, okanye umntu okwintsapho yethu. Kolu hlobo, ii-protein clumps zinokubekwa kungekuphela nje entliziyweni, kodwa nakwiindawo ezifana nenkqubo yemithambo-luvo kunye nezintso.
2.Uhlobo lwe-ATTR-CM lwasendle:
Unobangela ochanekileyo wolu hlobo awukafunyanwa. Awulofuzo. Olu hlobo lubonakala kakhulu kumadoda angaphezulu kweminyaka engama-65 ubudala. Luchaphazela kakhulu intliziyo kunye nenkqubo yemithambo-luvo.
Zithini iimpawu? Siyibona njani le nto?
Iimpawu zesi sifo zingahluka kumntu nomntu. Ngamanye amaxesha, abantu abanohlobo 'lwe-wild-type' basenokungabonakalisi zimpawu kwaphela kwizigaba zokuqala. Nokuba iimpawu ziyavela, zifana kakhulu nezo zesifo sentliziyo esiqhelekileyo, ngoko ke ngamanye amaxesha kunokuba nzima ukufumanisa esi sifo.
Ezi ziimpawu ezibonakala rhoqo.
| Uphawu | Ingcaciso elula |
|---|---|
| Ukuqhawukelwa ngumphefumlo | Ubunzima bokuphefumla, ingakumbi xa usenza umsebenzi olula, uhamba, okanye ulele ebhedini. |
| Ukudumba kwemilenze, amaqatha, iinyawo (i-Edema) | Ukudumba kwemilenze ngenxa yokugcina ulwelo oluninzi emzimbeni. Kungavakala ngathi kukunamathela xa unxiba isihlangu. |
| Ukungahambi kakuhle kwentliziyo (i-Arrhythmia) | Iimvakalelo zokubetha kwentliziyo ngokukhawuleza, iintlungu zesifuba (ukubetha kwentliziyo), okanye ukubetha kwentliziyo okungahambelaniyo, ingakumbi imeko ebizwa ngokuba yi-Atrial Fibrillation (Afib), ziqhelekile. |
| Ukudinwa | Ukuhlala ndiziva ndidiniwe kakhulu ngaphandle kwesizathu. |
| Ukudinwa okanye ukuquleka | Ukuziva unesizungu okanye uphelelwe ngamandla xa umi okanye uvuka ngequbuliso. |
| Isisu esidumbileyo | Ukuziva udumbile ngenxa yokuqokelelana kolwelo esiswini. |
Ezinye iingxaki ezinokubakho ngenxa yesi sifo
Le protein ayigcinwanga entliziyweni kuphela, kodwa inokubangela nezinye iingxaki ngokuhlala kwinkqubo yemithambo-luvo nakwezinye izicwili.
- I-Carpal Tunnel Syndrome: Le yimeko apho umthambo ohamba esihlahleni ucinezelwa khona. Abantu abanale meko banokuhlangabezana nale meko ngezandla zombini . Iimpawu ziquka ukungaziva mnandi kunye nentlungu kwiminwe.
- I-Peripheral Neuropathy: Oku kubangela umonakalo kwimithambo-luvo emilenzeni. Ngenxa yoko, usenokuba nentlungu, ukurhawuzelelwa, kunye nentlungu emilenzeni.
- I-Spinal Stenosis: Ukuncitshiswa komjelo womqolo. Oku kunokubangela iintlungu zomqolo kunye nentlungu yomlenze.
- Ukuqhekeka kweTendon: Ukuqhekeka kwetendon engalweni okanye emlenzeni okuzenzekelayo.
Okubalulekileyo kukuba, ukuba unyangwa uxinzelelo lwegazi oluphezulu kwaye une-carpal tunnel syndrome ezandleni zombini, ugqirha wakho unokurhanela i-ATTR-CM. Ke ngoko, kubalulekile ukuxelela ugqirha wakho ngeempawu zakho ezipheleleyo.
Ugqirha ufumanisa njani esi sifo kanye kanye?
Ngenxa yokuba iimpawu zesi sifo zifana nezifo zentliziyo eziqhelekileyo, kufuneka uvavanyo olukhethekileyo oluninzi ukuze kuchongwe ngokuchanekileyo.
- I-Electrocardiogram (ECG): Uvavanyo lomsebenzi wombane wentliziyo.
- I-Echocardiogram: Olu luvavanyo lwentliziyo. Lunokujonga ngokusondeleyo izinto ezinje ngobukhulu kunye nomsebenzi wemisipha yentliziyo.
- I-MRI yentliziyo: Olu lolunye uvavanyo olunzulu lwentliziyo.
- I-Bone Scintigraphy (Bone Scan): Olu luvavanyo olukhethekileyo. Olu vavanyo, oludla ngokwenziwa ukuze kuhlolwe amathambo, lunokufumanisa ukuba iproteni ye-amyloid ibekwe entliziyweni na.
- Uvavanyo lwegazi: Uvavanyo lwemfuza lungenziwa ukuze kubonwe ukuba kukho naluphi na utshintsho kwi-TTR gene.
- I-Biopsy yeNtliziyo: Ngamanye amaxesha, kufuneka kuthathwe iqhekeza elincinci kakhulu lemisipha yentliziyo lihlolwe ukuqinisekisa isifo.
Ngaba ikhona indlela yonyango? Ngaba le nto inganyangeka ngokupheleleyo?
Okwangoku, akukho nyango lwe-ATTR-CM, kwaye akukho ndlela yokususa ii-clumps zeproteni esele zakhekile entliziyweni.
Kodwa ungakhathazeki. Ngoku kukho amayeza asebenzayo kakhulu anokulawula esi sifo, oko kukuthi, ayeke okanye acothise ukwakheka nokubekwa kweeproteni ezintsha. La mayeza anokuthintela isifo ukuba singabi mandundu.
- IziQinisekisi zeTTR: Amayeza afana neTafamidis (Vyndaqel®, Vyndamax®) abopha kwiproteni yeTTR aze ayithintele ekusongeni ngendlela engafanelekanga.
- Izithulisi zeTTR: Amayeza afana nePatisiran (Onpattro®) kunye ne-Inotersen (Tegsedi®) anciphisa ukuveliswa kwale protein ineengxaki zesibindi.
Ukongeza kula mayeza, unganikwa nonyango lokulawula iimpawu zakho. Umzekelo:
- Amayeza okulawula isifo sentliziyo.
- Amayeza okunceda abantu abanengxaki yentliziyo.
- Amayeza okugcina ulwelo.
Kunqabile kakhulu, kwiimeko ezinzima kakhulu zesifo, kunokufuneka ukuba usebenzise into efana nokufakelwa isibindi, ukufakelwa izintso, okanye ukufakelwa intliziyo.
Eyona nto ibalulekileyo xa uphila nesi sifo kukugcina ulwalamano oluqhelekileyo nogqirha wakho wentliziyo kwaye uthathe amayeza akho owanikwe ngugqirha wakho ngexesha. Ngophando oluqhubekayo ngamayeza amatsha kunye nonyango, kukho ithemba ngekamva.
Umyalezo Wokuya Ekhaya
- I-ATTR-CM sisifo apho uhlobo oluthile lweproteni lufakwa entliziyweni, nto leyo ebangela ukuba izihlunu zentliziyo ziqine, ziqine, kwaye zenze buthathaka ukusebenza kwazo.
- Ukuphefumla kancinci, ukudumba kwemilenze, ukubetha kwentliziyo okungahambelaniyo, kunye nokudinwa kakhulu zezona mpawu ziphambili. Musa ukuzityeshela ezi njengeempawu eziqhelekileyo zokwaluphala.
- Esi sifo sinokufunyanwa njengelifa (sentsapho), okanye singenzeka ngaphandle kwesizathu (sendalo).
- Ukuba uneCarpal Tunnel Syndrome ezandleni nasezintliziyweni, lumka kakhulu.
- Nangona esi sifo singenakunyangwa ngokupheleleyo, ngoku kukho amayeza asebenzayo kakhulu anokuthintela esi sifo ukuba singaqhubeki phambili.
- Ukuba unale mpawu, kubaluleke kakhulu ukubona ugqirha wakho ngokukhawuleza kwaye wenze uvavanyo olufunekayo.
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