Ingabe ngezinye izikhathi uzizwa unesizungu ngisho nalapho uhamba ibanga elifushane? Ingabe uzizwa uphelelwa umoya uma uya embhedeni? Ingabe imilenze namaqakala akho ayavuvukala? Ingabe inhliziyo yakho ishaya ngendlela exakile noma esheshayo? Nakuba sivame ukucabanga ukuthi lezi zinto ziyizinto ezivamile ezenzeka njengoba sikhula, ngezinye izikhathi ngemuva kwalezi zimpawu kungase kube nesimo esingakazwa kakhulu ngaso futhi esingaba yingozi uma singahlolwanga kahle. Esinye sezifo ezinjalo yi-Transthyretin Amyloidosis, noma i-ATTR-CM ngamafuphi.
Kalula nje, iyini i-Transthyretin Amyloidosis (ATTR-CM)?
Leli igama eliyinkimbinkimbi kancane, ngakho-ke ake silichaze igama negama. Khona-ke uzoliqonda kalula.
- I-Transthyretin (TTR): Lolu uhlobo lweprotheni emizimbeni yethu. Lwenziwa yisibindi sethu. Umsebenzi walo oyinhloko ukuthwala i-vitamin A kanye ne-thyroid hormone i-thyroxine egazini lethu iye ezindaweni ezidingekayo emzimbeni. Kufana nomuntu oletha impahla ethwele amaphasela.
- I-Amyloidosis: Lokhu kwenzeka lapho uhlobo lweprotheni, njengoba kushiwo ngenhla , lugoqeka ngendlela engafanele, luhlangana ndawonye, bese lwenza ama-fibrils, abukeka njengamabhola amancane entambo, bese efakwa ezithweni zethu zomzimba. Uma efakwa ngale ndlela, ukusebenza okuvamile kwalezo zitho kuyaphazamiseka.
- I-Cardiomyopathy (CM): Lokhu kubhekisela esifweni semisipha yenhliziyo .
Manje, ukuhlanganisa konke lokhu, i-Transthyretin Amyloidosis (ATTR-CM) yisifo lapho iphrotheni i-TTR, eyenziwa yisibindi sethu, ihlukana futhi ihlangane ndawonye, ikakhulukazi emisipheni yenhliziyo yethu, okubangela ukuba imisipha yenhliziyo iqine futhi iqine, okwenza ingakwazi ukupompa igazi kahle.
Cabanga ukuthi ingxenye eyinhloko epompa igazi yenhliziyo yakho (i-ventricle yesobunxele) ifana nebhola lerabha eliguquguqukayo. Ifinyela kalula futhi ikhule ukuze ipompe igazi emzimbeni wonke. Manje, lapho lawo maqoqo amaprotheni efakwa kulowo msipha wenhliziyo, leyo ngxenye efana nerabha kancane kancane iba nzima futhi iqine. Iba njengebhola legobolondo likakhukhunathi esikhundleni sebhola lerabha. Ngemuva kwalokho ayikwazi ukufinyela futhi ikhule kalula, akunjalo? Yilokho okwenzeka enhliziyweni kulesi sifo. Yingakho kwenzeka izimo ezifana nokwehluleka kwenhliziyo.
Kunezinhlobo ezimbili eziyinhloko zalesi sifo.
Isifo se-ATTR-CM singahlukaniswa ngezinhlobo ezimbili eziyinhloko.
1. I-ATTR-CM Yomndeni noma Yefa:
Lokhu kubangelwa ukuguquka kwezakhi zofuzo zethu. Lokhu kusho ukuthi kungenzeka ukuthi sithole ifa lesi sifo kumama, kubaba, noma komunye umuntu emndenini wethu. Kulolu hlobo, amaqoqo amaprotheni angabekwa hhayi enhliziyweni kuphela, kodwa nasezindaweni ezifana nesimiso sezinzwa nezinso.
2.I-ATTR-CM yohlobo lwasendle:
Imbangela eqondile yalolu hlobo ayikatholakali. Akulona ufuzo. Lolu hlobo luvame ukubonakala kakhulu kwabesilisa abangaphezu kweminyaka engu-65. Luthinta kakhulu inhliziyo kanye nesimiso sezinzwa.
Ziyini izimpawu? Sikubona kanjani lokhu?
Izimpawu zalesi sifo zingahluka kumuntu nomuntu. Ngezinye izikhathi, abantu abanohlobo 'lohlobo oluhlala njalo' bangase bangakhombisi nhlobo izimpawu ezigabeni zokuqala. Ngisho noma izimpawu zivela, zifana kakhulu nezesimo esivamile sokwehluleka kwenhliziyo, ngakho-ke ngezinye izikhathi kungaba nzima ukuthola lesi sifo.
Lezi yizimpawu ezivame ukubonakala.
| Isibonakaliso | Incazelo elula |
|---|---|
| Ukuphelelwa umoya | Ubunzima bokuphefumula, ikakhulukazi lapho wenza umsebenzi olula, uhamba, noma ulele embhedeni. |
| Ukuvuvukala kwemilenze, amaqakala, nezinyawo (i-Edema) | Ukuvuvukala kwemilenze ngenxa yokugcinwa koketshezi emzimbeni. Kungazwakala sengathi kuyalingana uma ugqoka isicathulo. |
| Ukuphazamiseka kwenhliziyo (i-Arrhythmia) | Imizwa yokushaya kwenhliziyo okusheshayo, ubuhlungu besifuba (ukushaya kwenhliziyo), noma ukushaya kwenhliziyo okungajwayelekile, ikakhulukazi isimo esibizwa ngokuthi i-Atrial Fibrillation (Afib), ivamile. |
| Ukukhathala | Ukuzizwa ukhathele kakhulu njalo ngaphandle kwesizathu. |
| Ukuzizwa ulele noma ukuphelelwa yithemba | Ukuzizwa unesizungu noma uqulekile uma umi noma uvuka ngokuzumayo. |
| Isisu esivuvukele | Ukuzizwa uvuvukele ngenxa yokuqongelela koketshezi esiswini. |
Ezinye izinkinga ezingase zivele ngenxa yalesi sifo
Le phrotheni ayigcini nje ngokuhlala enhliziyweni. Ingabangela nezinye izinkinga ngokuhlala ohlelweni lwezinzwa nakwezinye izicubu.
- I-Carpal Tunnel Syndrome: Lesi yisimo lapho inzwa edlula esihlakaleni icindezelwa khona. Abantu abanalesi simo bangabhekana nalesi simo ngezandla zombili . Izimpawu zifaka phakathi ukungazweli kanye nobuhlungu eminweni.
- I-Peripheral Neuropathy: Lokhu kubangela umonakalo ezinzwa ezithweni. Ngenxa yalokho, ungase uzwe ukungabi namandla, ukuqaqamba, kanye nobuhlungu ezithweni.
- I-Spinal Stenosis: Ukuncipha komsele womgogodla. Lokhu kungabangela ubuhlungu emhlane kanye nobuhlungu emilenzeni.
- Ukuqhekeka kwe-tendon: Ukuqhekeka okuzenzekelayo kwe-tendon engalweni noma emlenzeni.
Okubalulekile, uma welashwa umfutho wegazi ophakeme futhi une-carpal tunnel syndrome ezandleni zombili, udokotela wakho angase asole i-ATTR-CM. Ngakho-ke, kubalulekile ukutshela udokotela wakho ngezimpawu zakho eziphelele.
Udokotela usithola kanjani lesi sifo ngqo?
Ngenxa yokuthi izimpawu zalesi sifo zifana nezifo zenhliziyo ezivamile, kudingeka izivivinyo eziningana ezikhethekile ukuze zixilongwe ngokunembile.
- I-Electrocardiogram (ECG): Ukuhlolwa komsebenzi kagesi wenhliziyo.
- I-Echocardiogram: Lokhu ukuskena kwenhliziyo. Kungaqapha ngokucophelela izinto ezifana nokujiya kanye nokusebenza kwemisipha yenhliziyo.
- I-MRI Yenhliziyo: Lokhu kungenye indlela yokuhlola inhliziyo ngokujulile.
- I-Bone Scintigraphy (Bone Scan): Lolu vivinyo olukhethekile kakhulu. Lolu hlolo, oluvame ukwenziwa ukuhlola amathambo, lungabona ukuthi iphrotheni ye-amyloid ifakiwe yini enhliziyweni.
- Ukuhlolwa kwegazi: Ukuhlolwa kofuzo kungenziwa ukuze kubonakale ukuthi kukhona yini izinguquko ku-TTR gene.
- Ukuhlolwa Kwenhliziyo: Ngezinye izikhathi, kudingeka kuthathwe ingxenye encane kakhulu yomsipha wenhliziyo futhi ihlolwe ukuqinisekisa isifo.
Ingabe ikhona indlela yokwelapha? Ingabe lokhu kungelapheka ngokuphelele?
Okwamanje, alikho ikhambi le-ATTR-CM, futhi ayikho indlela yokususa amaqoqo amaprotheni asevele akhekile enhliziyweni.
Kodwa ungakhathazeki. Manje kunemithi ephumelela kakhulu engalawula lesi sifo, okungukuthi, ukuvimba noma ukulibazisa ukwakheka nokubekwa kwamaqoqo amasha amaprotheni. Le mithi ingavimbela lesi sifo ukuthi singabi sibi kakhulu.
- Ama-TTR Stabilizers: Izidakamizwa ezifana ne-Tafamidis (Vyndaqel®, Vyndamax®) zibopha iphrotheni ye-TTR futhi ziyivimbele ukuthi ingagoqeki kahle.
- Izithulisi ze-TTR: Izidakamizwa ezifana ne-Patisiran (Onpattro®) kanye ne-Inotersen (Tegsedi®) zinciphisa ukukhiqizwa kwale phrotheni enephutha yisibindi.
Ngaphezu kwale mithi, unganikezwa nokwelashwa ukulawula izimpawu zakho. Isibonelo:
- Imithi yokulawula izifo zenhliziyo.
- Imithi yokwelapha izinkinga zesigqi senhliziyo.
- Imithi yokugcina uketshezi.
Akuvamile kakhulu, ezimweni ezimbi kakhulu zesifo, kungadingeka ukuthi usebenzise into efana nokufakelwa isibindi, ukufakelwa izinso, noma ukufakelwa inhliziyo.
Into ebaluleke kakhulu uma uphila nalesi sifo ukugcina ubuhlobo obuvamile nodokotela wakho wenhliziyo futhi uphuze imithi oyinikezwe udokotela ngesikhathi. Njengoba kuqhubeka ucwaningo ngemithi emisha kanye nokwelashwa, kunethemba ngekusasa.
Umlayezo Wokuya Nawe Ekhaya
- I-ATTR-CM yisifo lapho uhlobo oluthile lweprotheni lufakwa enhliziyweni, okubangela ukuba imisipha yenhliziyo iqine, iqine, futhi yenze buthaka ukusebenza kwayo.
- Ukuphefumula kanzima, ukuvuvukala kwemilenze, ukushaya kwenhliziyo okungajwayelekile, kanye nokukhathala okukhulu yizimpawu eziyinhloko. Ungakushayi indiva lokhu njengezimpawu ezivamile zokuguga.
- Lesi sifo singazuzwa njengefa (esivela emndenini), noma singavela ngaphandle kwesizathu (esivela endle).
- Uma une-Carpal Tunnel Syndrome ezandleni nasezinhlungwini zenhliziyo, qaphela kakhulu.
- Nakuba lesi sifo singelapheki ngokuphelele, manje kunemithi ephumelela kakhulu engavimba lesi sifo ukuthi singaqhubeki phambili.
- Uma unalezi zimpawu, kubaluleke kakhulu ukubona udokotela wakho ngokushesha okukhulu bese wenza izivivinyo ezidingekayo.
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