Ingabe unomngane omude kakhulu, onezingalo nemilenze emide, futhi onomzimba omncane? Uma sibona umuntu onjalo, sicabanga ukuthi angaba kuhle emdlalweni ofana ne-basketball. Kodwa namuhla sizokhuluma ngesimo sempilo esihambisana nalezi zici zomzimba, esingakhulumi kakhulu ngazo, kodwa esingaba sibaluleke kakhulu. Leyo yi-Marfan Syndrome. Kubaluleke kakhulu ukuqaphela lokhu ngoba kungathinta inhliziyo ikakhulukazi.
Kalula nje, iyini i-Marfan Syndrome?
I-Marfan syndrome yisimo sezakhi zofuzo esenzeka ezizakhini zethu zofuzo. Lokhu kubangelwa 'izicubu ezixhumene' emzimbeni wethu ezingakhuli kahle. Cabanga ngemizimba yethu njengesakhiwo. Konke okukulesi sakhiwo, njengezitini, izindonga, nophahla, kubanjwa ndawonye futhi kugcinwe kuqinile ngesamente. Ngokufanayo, izicubu ezixhumene 'ziyiglu' esiza ukubamba konke okusemzimbeni wethu, njengezitho zomzimba, amathambo, nemithambo yegazi, ndawonye futhi kuzigcine ziqinile.
Kumuntu one-Marfan syndrome, 'insini', noma izicubu ezixhumeneyo, emzimbeni zibuthakathaka. Kufana nokwakha indlu ngesimende esishibhile. Lokhu kungathinta izindawo eziningi, okuhlanganisa amehlo, amaphaphu kanye nohlelo lwamathambo. Kodwa imiphumela emibi kakhulu ingaba senhliziyweni yethu kanye nasemithanjeni yegazi emikhulu.
Kungani lokhu kuthinta inhliziyo kangaka? Izinkinga ezimbili eziyinhloko!
Izicubu ezixhumeneyo ezibuthakathaka zingalimaza izingxenye ezimbili eziyinhloko zenhliziyo.
1. I-Aorta: Lona umthambo wegazi omkhulu kunayo yonke othwala igazi lisuka enhliziyweni yethu liye emzimbeni wonke. Njengesistimu yamapayipi eyinhloko ethwala amanzi esuka ethangini lamanzi endlini yethu aya yonke indawo.
2. Ama-valve Enhliziyo: Lawa afana neminyango emincane ngaphakathi kwenhliziyo. Lawa ma-valve aqinisekisa ukuthi igazi ligeleza ohlangothini olulodwa kuphela.
Manje ake sibone ukuthi imiphumela ithini kulezi zingxenye ezimbili.
1. Kwenzekani ku-aorta?
Umuntu one-Marfan syndrome unezicubu ezibuthakathaka ezindongeni ze-aorta yakhe. Lokhu kungabangela izinkinga ezimbili eziyinhloko:
- Ukwanda kwe-Aortic: Ngokuvamile, umthambo wegazi uyaguquguquka. Kodwa-ke, ngenxa yalobu buthakathaka, i-aorta iqala ukuvuleka kancane kancane futhi ikhule, ingakwazi ukumelana nengcindezi ebangelwa ukushaya kwenhliziyo ngakunye.
- I-Aortic Aneurysm: Ngezinye izikhathi lokhu kukhula akugcini nje ngokuma. Ingxenye ebuthakathaka kakhulu yomthambo iqala ukukhukhumala njengebhaluni. Lokhu sikubiza ngokuthi "i-Aortic Aneurysm". Lena yinkinga eyingozi kakhulu ye-Marfan syndrome. Ngoba lokhu kukhukhumala okufana nebhaluni kungaqhuma (kuqhekeke) noma kudabuke (kuhlukaniswe) nganoma yisiphi isikhathi. Kuyisimo esiphuthumayo esisongela ukuphila.
2. Kwenzekani kuma-valve enhliziyo?
Izicubu ezibuthakathaka ezixhumanisayo nazo zingabangela ukuthi ama-valve enhliziyo angasebenzi kahle. Angaba buthakathaka futhi angavaleki kahle. Yingakho:
- Ukubuyiselwa Kwevalvu: Uma ivalvu ingavali kahle, igazi lingavuza libuyele enhliziyweni. Lokhu kubangela ukuthi inhliziyo isebenze kanzima ukupompa igazi liye emzimbeni. Ngokuhamba kwesikhathi, lokhu kungaholela ekuhlulekeni kwenhliziyo. Amavalvu avame ukuthinteka yi-Aortic Valve kanye ne-Mitral Valve.
- Ukuwa kwe-Mitral Valve: Kulesi simo, i-mitral valve engakwesobunxele senhliziyo iba buthakathaka futhi ayivali kahle, kodwa kunalokho igoba ibuyela emuva.
Into ebalulekile ukuthi cishe abantu abayisishiyagalolunye kwabayishumi abane-Marfan syndrome bazoba nezinkinga zenhliziyo noma ze-aorta, ngakho-ke kubalulekile ukuqaphela lokhu.
Yiziphi lezi zimpawu zesifo senhliziyo? Sizibona kanjani?
Ezimweni eziningi, kungase kungabi khona zimpawu ezigabeni zokuqala. Kodwa-ke, lezi zimpawu zingase zivele njengoba i-aorta iba banzi noma izinkinga zevalvu ziba zimbi kakhulu. Uma unesinye noma ngaphezulu kwalezi, kubaluleke kakhulu ukubona udokotela.
| Isibonakaliso | Incazelo elula |
|---|---|
| Ubuhlungu esifubeni noma emhlane ongaphezulu | Kuyingozi kakhulu uma ubuhlungu buvele bube bukhulu futhi busheshe bube khona. |
| Ukuphefumula kanzima noma ubunzima bokuphefumula | Ukukhathala okuhambisana nokuhamba kancane noma ukwenza umsebenzi. |
| Ukushaya kwenhliziyo (ukuzwa sengathi inhliziyo yakho ishaya ngokushesha) | Ukuzizwa sengathi ukushaya kwenhliziyo yakho kuyashintsha noma isifuba sakho siyashaya. |
| Ukuzizwa udidekile noma unekhanda elibuhlungu | Ukuzizwa unesizungu lapho umi noma uvuka ngokuzumayo. |
| Ukukhathala okungavamile kanye nobuthakathaka | Ukuzizwa ukhathele njalo ngaphandle kwesizathu. |
| Ukuvuvukala kwemilenze nezinyawo | Kungaba uphawu lokungasebenzi kahle kwenhliziyo. |
Udokotela usithola kanjani lesi sifo ngqo?
Ukuhlonza i-Marfan syndrome kungaba nzima kancane ngoba akuwona wonke umuntu onezimpawu ezifanayo, ngakho udokotela uzobheka izici eziningana.
- Ukuhlolwa komzimba: Udokotela uzohlola ubude bakho, ubude bengalo nomlenze, ubude bomunwe, ukuma kwesifuba, amehlo, nomgogodla.
- Umlando wezokwelapha womndeni: Njengoba lesi kuyisifo esizuzwe njengefa, uzobuzwa ukuthi ukhona yini emndenini wakho oke waba nalezi zimpawu noma washona ngenxa yokumiswa kwenhliziyo ngokuzumayo.
- Ukuhlolwa okukhethekile: Kuhlolwa izivivinyo eziningana ukuze kutholakale isimo esiqondile senhliziyo.
- I-Echocardiogram: Lokhu kufana nokuskena kwenhliziyo. Kungabona ngokucacile izinto ezifana nosayizi we-aorta kanye nomsebenzi wama-valve.
- I-Electrocardiogram (EKG): Lokhu kusiza ukuhlola ukusebenza kukagesi kwenhliziyo, okungukuthi, ukubona ukuthi kukhona yini izinkinga ngesigqi sokushaya kwenhliziyo.
- Ukuhlolwa Kwezakhi Zofuzo: Ukuhlolwa kwegazi ukuze kutholakale ukuthi une-mutation yezakhi zofuzo ebangela i-Marfan syndrome.
- Ukugwema imisebenzi ecindezela inhliziyo kakhulu. Isibonelo, imidlalo enomthelela omkhulu njengokuphakamisa izinsimbi, i-rugby, kanye nebhola ayifaneleki.
- Uma i-aorta iba banzi ngendlela eyingozi, kwenziwa ukuhlinzwa ukuze kuvinjelwe ukuthi ingadabuki (ukusikwa). Kungcono ukuhlela lokhu ngaphambi kokuba kube yisimo esiphuthumayo.
- Udokotela angase anikeze imithi efana ne-"Beta-blockers" noma "ARBs". Le mithi isebenza ngokulawula umfutho wegazi nokunciphisa umfutho wegazi e-aorta. Lokhu kunciphisa izinga lokukhula komthambo wegazi.
- Ukususa ingxenye eyonakele ye-aorta bese uyilungisa ngepayipi lokwenziwa (i-graft).
- Ukulungisa noma ukufaka esikhundleni amavalvu enhliziyo angasebenzi kahle ngevalvu yokwenziwa.
- Kubalulekile ukwenza i-scan efana ne-echocardiogram okungenani kanye ngonyaka ukuze kuqashwe ubukhulu be-aorta. Lokhu kungasiza ekuboneni noma yiziphi izinkinga kusenesikhathi uma zivela.
- Uma ububanzi be-aorta budlula amasentimitha angu-5.
- Uma umthambo ukhula ngokushesha okukhulu phakathi nonyaka.
- Uma othile emndenini wakho enomlando wokusikwa kwe-aorta.
Uma othile emndenini wakho enalesi sifo noma enezimpawu ezifanayo, ukutshela udokotela wakho ngaso kuwusizo olukhulu ekuxilongeni lesi sifo.
Kulungile, manje yiziphi izindlela zokwelapha lokhu?
I-Marfan syndrome ayinakwelapheka ngokuphelele. Ngoba yinto esezakhini zethu zofuzo. Kodwa-ke, kunezindlela zokwelapha ezinhle kakhulu zokulawula umonakalo ewubangela enhliziyweni, ukuvimbela izimo ezibucayi, nokuvumela abantu ukuba baphile impilo evamile. Ukwelashwa kungahlukaniswa izingxenye ezimbili eziyinhloko.
| Ukwelashwa Okungeyona Eyokuhlinzwa | Ukwelashwa ngokuhlinzwa |
|---|---|
Izinguquko zendlela yokuphila: | Kungani ukuhlinzwa kuyadingeka?: |
Imithi: | Izinhlobo zokuhlinzwa: |
Ukuhlolwa njalo kwezokwelapha: | Kunconywa ukuhlinzwa kwalokhu okulandelayo: |
Izimo eziphuthumayo ezidinga ukungeniswa esibhedlela ngokushesha! (Izimpawu Zesexwayiso)
Lezi zimpawu zingase zibe izimpawu zokuqhekeka komthambo wegazi noma ukuqhekeka komthambo wegazi. Uma kwenzeka noma yikuphi kwalokhu, hamba uye eMnyangweni Wezimo Eziphuthumayo (ETU) oseduze ngokushesha.
| Uma unalezi zimpawu, iya ku-ETU ngokushesha! | |
|---|---|
| - Ubuhlungu obungazelelwe, obungabekezeleleki esifubeni, emhlane, noma esiswini . | - Ubunzima obukhulu bokuphefumula. |
| - Ukulahlekelwa ingqondo. | - Ukuba ndikindiki noma ukuqaqamba engxenyeni yomzimba. |
| - Ukujuluka ngokweqile, isikhumba esibandayo. | - Isicanucanu nokuhlanza. |
Kungenzeka yini ukuphila kahle ne-Marfan syndrome?
Yebo impela! Eminyakeni edlule, isikhathi sokuphila sabantu abanalesi simo sasifushane. Kodwa namuhla, ngenxa yokwelashwa okuthuthukisiwe, ukuhlolwa okuvamile, kanye nokuhlinzwa, umuntu one-Marfan syndrome angaphila impilo enempilo, ngisho noma eneminyaka engu-70 noma engu-80, njengomuntu ovamile.
Into ebaluleke kakhulu ukuxhumana njalo nodokotela wakho, ukulandela imiyalelo yakhe ngqo, ukuhlolwa ngesikhathi, nokusebenzisa imithi yakho ngendlela efanele.
Ikakhulukazi, uma owesifazane one-Marfan syndrome ehlela ukukhulelwa, kufanele nakanjani axhumane nodokotela wenhliziyo kanye nodokotela wezifo zabesifazane ngaphambi kokukhulelwa. Ngenxa yokuthi ukucindezeleka enhliziyweni kuphezulu ngesikhathi sokukhulelwa, kufanele kuthathwe ukunakekelwa okukhethekile.
Ngokuqonda lesi simo, ukuthatha izinyathelo ezidingekayo, nokuphila ngokuqaphela, ungavikela inhliziyo yakho futhi uphile impilo enempilo nende.
Umlayezo Wokuya Nawe Ekhaya
- I-Marfan syndrome iyisifo esidluliselwa ngofuzo esenza buthaka izicubu zomzimba ezixhumeneyo.
- Lokhu kungaba nemiphumela emibi kakhulu enhliziyweni nasemzimbeni wegazi oyinhloko, i-aorta.
- Ukwanda kwe-aorta kanye ne-aneurysm yizinkinga eziyingozi kakhulu.
- Kubalulekile ukubona udokotela ngesikhathi esihleliwe bese kwenziwa ama-scan njenge-echocardiogram.
- Gwema imisebenzi ecindezela inhliziyo, njengokuphakamisa izinto ezisindayo.
- Qaphela izimpawu eziyisixwayiso, njengobuhlungu besifuba obungazelelwe nobunamandla.
- Ngokuphathwa kahle nokwelashwa, ungaphila impilo evamile nende.
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