Ngaba nawe uvakalelwa kukuba umde kakhulu kwaye uneengalo nemilenze emide kunabahlobo bakho? Okanye amalungu akho aphuka lula? Ngaba kuye kwafuneka unxibe iiglasi ukususela ebuntwaneni? Ukuba enye okanye ezingaphezulu kwezi zinto ziyasebenza kuwe, kunokuba kubaluleke kakhulu ukuba uqaphele imeko esithetha ngayo namhlanje, ebizwa ngokuba yiMarfan Syndrome. Nangona oku kungaqhelekanga kancinci, masithethe ngayo ngokulula.
Ngamafutshane, yintoni iMarfan Syndrome?
Cinga ngemizimba yethu njengesakhiwo esakhiwe kakuhle. Izitena, iindonga, kunye nophahla lwesi sakhiwo zibanjwe kunye kwaye zenziwe zomelele ngesamente. Ngokufanayo, kukho uhlobo olukhethekileyo lwezicubu ezidibanisa yonke into emzimbeni wethu, njengezitho zomzimba, amathambo, izihlunu, kunye nemithambo yegazi, kwaye zinika amandla kunye nokuguquguquka ezikufunayo. Kwizonyango, oku sikubiza ngokuba "yizicubu ezinxibelelanayo." Ezi zifana 'nentsini' emzimbeni wethu.
I-Marfan Syndrome yimeko yemfuza. Umntu onesi sifo unezicwili ezibuthathaka, okanye ngokuthe ngqo, ezikhululekileyo kakhulu. Oku kuthetha ukuba izicwili ezidibeneyo azinamandla kwaye aziguquguquki. Ngenxa yokuba ezi zicwili zifumaneka kuwo wonke umzimba, i-Marfan Syndrome inokuchaphazela iindawo ezininzi zomzimba wethu. Ingachaphazela kakhulu intliziyo, imithambo yegazi, amehlo, amathambo kunye namalungu .
Nangona esi sisifo sokuzalwa, ngamanye amaxesha iimpawu ziyavela kwaye ukuxilongwa kwenziwa ebuntwaneni.
Zingaba yintoni iimpawu zoku?
Into ebalulekileyo ekufuneka uyiqonde apha kukuba ayinguye wonke umntu oneMarfan Syndrome oza kuba neempawu ezifanayo. Ziyahluka kakhulu kumntu nomntu. Abanye abantu banokuba neempawu ezininzi, ngelixa abanye banokuba nezimbalwa. Yiyo loo nto oogqirha bebiza le meko ngokuthi "yimeko eguquguqukayo."
Nangona kunjalo, kukho iimpawu ezifanayo ezinokubonwa. Masizihlukanise zibe ngamacandelo amabini.
| Iimpawu ezimbini eziphambili zeMarfan Syndrome | |
|---|---|
| I-Aortic root aneurysm | I-aorta yeyona mthambo wegazi mkhulu othwala igazi lisuka entliziyweni yethu liye kuwo wonke umzimba wethu. Inxalenye yokuqala yayo, edibana nentliziyo, inokuba buthathaka kwaye idumbe kwaye ivuleke njengebhaluni. Olu lolona phawu luyingozi kakhulu lwesi sifo. |
| I-Ectopia lentis (ukususwa kwelensi yeliso) | Ilensi engaphakathi kwamehlo ethu, ngenxa yokuba buthathaka kweefayibha ezithambileyo eziyibambayo, inokuhamba kancinci ukusuka apho kufanele ukuba ikhoyo. Oku kubangela ukuphazamiseka kokubona. |
Ukongeza kwezi mpawu zimbini ziphambili, ezinye iimpawu ezinxulumene nenkangeleko yomzimba zihlala zibonakala.
| Iimpawu eziqhelekileyo ezinxulumene nenkangeleko yomzimba | |
|---|---|
| Uhlobo lomzimba | Unomzimba omde ngokungaqhelekanga, obhityileyo. |
| Izandla, iinyawo, iminwe | Iingalo, imilenze, izandla, kunye neminwe ezinde ngokungaqhelekanga xa kuthelekiswa nezinye iindawo zomzimba. |
| Ubuso | Ubuso obude, obumxinwa. |
| Umqolo | I-Scoliosis. |
| Isifuba | Ithambo lesifuba elitshone ngaphakathi (Pectus excavatum) okanye eliphumela ngaphandle (Pectus carinatum). |
| I-Junction | Amalunga aguquguquka kakhulu kwaye athambekele ekuqhekekeni. |
| Iinyawo | Iinyawo ezithe tyaba. |
| Amazinyo | Amazinyo axineneyo ngenxa yokungabikho kwesithuba emlonyeni. |
| Ulusu | Amabala okuzolula abonakala kumphezulu wesikhumba nangona kungekho tshintsho kubunzima bomzimba. |
Ziziphi iingxaki ezinokubakho ngenxa yeMarfan Syndrome?
Ukuba le meko ayiphathwa kakuhle, iingxaki ezinkulu zinokuvela ngokuhamba kwexesha.
Iziphumo ezinokubakho entliziyweni nakwimithambo yegazi
Ezi zezona ngxaki zifuna ingqalelo enkulu kwiMarfan Syndrome.
- Ukuqhekeka kwe-aorta: Le yeyona meko iyingozi kakhulu. Udonga lwe-aorta lwenziwe ngamaleya aliqela. Ukukrazuka ngequbuliso kumaleya angaphakathi kolu donga kunokubangela ukuba igazi livuza phakathi kwamaleya. Le yimeko esongela ubomi efuna utyando olungxamisekileyo.
- Isifo sevalvu yentliziyo: Iivalvu entliziyweni ziba buthathaka kwaye zisenokungavaleki kakuhle, nto leyo evumela igazi ukuba livuze umva.
- Intliziyo ekhulisiweyo: Izihlunu zentliziyo zinokukhula zibe buthathaka kuba intliziyo kufuneka isebenze nzima ngokuhamba kwexesha.
- Ukuphazamiseka kwentliziyo (i-Arrhythmia): Ukubetha kwentliziyo kusenokuba yinto engaqhelekanga.
- Ii-aneurysms zobuchopho: Indawo ebuthathaka kwimithambo yegazi engaphakathi okanye ejikeleze ingqondo inokudumba njengebhaluni.
Iziphumo emehlweni
- IiCataracts
- I-Glaucoma
- Ukuqhekeka kwe-retinal
Iziphumo kwimiphunga
Ubuthathaka bezicubu ezidityanisiweyo bunokuchaphazela nemiphunga.
- Isifuba
- Usulelo lwemiphunga (iBronchitis, iPneumonia)
- Umphunga oqutyulweyo (i-Pneumothorax)
Kubaluleke kakhulu ukuhlala uphaphile kwaye uhlolwe ngugqirha ukuze ufumane iingxaki ezinxulumene nentliziyo kunye ne-aorta kwiMarfan Syndrome.
Kutheni iMarfan Syndrome isenzeka? Yintoni unobangela?
Isizathu esiphambili soku kukuguquka kwezakhi zofuzo, iFibrillin-1, efumaneka kwizicwili zomzimba wethu.Kukho i-gene elawula ukuveliswa kweproteni ebizwa ngokuba yi-``fibrillin.'' Ibizwa ngokuba yi-``FBN1'' gene. Abantu abane-Marfan Syndrome banotshintsho oluthile, okanye isiphene (utshintsho), kule gene ye-``FBN1''. Oku kubangela ukuba umzimba uvelise iproteni ye-``FBN1'' ebuthathaka.
- Amaxesha amaninzi (malunga nama-75%) i-gene enesiphene ifunyanwa ngumzali omnye. Ukuba umzali ngamnye unale meko, ngamnye kubantwana bakhe unethuba elingama-50% lokufumana le meko.
- Kwi-25% eseleyo yamatyala, umntwana unokuba nolu tshintsho lwemfuza nokuba abazali abanalo olu tshintsho. Isizathu esichanekileyo asikafunyanwa.
Oogqirha bayifumana njani le nto?
Ngenxa yokuba iMarfan Syndrome ichaphazela amalungu amaninzi omzimba, kusenokufuneka iqela loogqirha abavela kwiingcali ezahlukeneyo ukuze benze uxilongo oluchanekileyo. Ugqirha wakho uhlala elandela la manyathelo:
- Ukubuza ngembali yakho yezonyango kunye neempawu: Ukubuza malunga nokungonwabi okufumanayo, iingxaki zokubona, okanye iintlungu zamalungu.
- Uvavanyo olupheleleyo lomzimba: kulinganisa ubude, ubunzima, ubude bamalungu omzimba, ukuba kukho intlungu emqolo, kunye nokuma kwesifuba.
- Ukubuza ngembali yezonyango zosapho: Ukubuza ngezinto ezifana nokuba kukho umntu kusapho okhe waba nezi mpawu, okanye ngaba kukho umntu oye wasweleka ngenxa yokuma kwentliziyo ngequbuliso.
- Ukuthunyelwa kwiimvavanyo ezikhethekileyo:
- I-Echocardiogram (Uvavanyo lwe-Echo): Olu lolona vavanyo lubalulekileyo. Lunokujonga imeko yentliziyo kunye ne-aorta, kunye nokusebenza kweevalvu.
- I-Electrocardiogram (ECG): Jonga ukuba akukho kuphazamiseka kwesigqi sentliziyo.
- Iiskeni ze-CT okanye ze-MRI: Jonga ubude bonke be-aorta kunye neminye imithambo yegazi ngokweenkcukacha.
- Uvavanyo lwamehlo: Chonga indawo yelensi yeliso kunye nezinye iingxaki.
- Uvavanyo lwemfuza: Isampuli yegazi ingathathwa ukuze kuchongwe ukuba kukho isiphene kwimfuza ye-`FBN1`.
Zithini iindlela zonyango zoku?
Okokuqala, akukho nyango lweMarfan Syndrome. Kodwa ungakhathazeki. Kunokwenzeka ukuyilawula kakuhle, uthintele iingxaki, kwaye uphile ubomi obuqhelekileyo nobusempilweni. Injongo ephambili yonyango kukulawula iimpawu kunye nokuthintela iingxaki eziyingozi.
Amayeza
- Ii-Beta-blockers: La mayeza anciphisa isantya sentliziyo kancinci, anciphise uxinzelelo lwegazi, kwaye anciphise uxinzelelo kwi-aorta. Oku kunokuthoba isantya apho i-aorta ikhula khona.
- Ii-Angiotensin II Receptor Blockers (ARBs): La mayeza anceda ukukhusela i-aorta.
Ukubeka iliso rhoqo
Le yeyona nxalenye ibalulekileyo yolawulo. Kufuneka ubone ugqirha rhoqo kwaye uhlolwe.
- Intliziyo nemithambo yegazi: Uvavanyo lwe-echo lwenziwa ubuncinane kanye ngonyaka ukujonga utshintsho kubukhulu be-aorta.
- Amehlo: Ufanele uhlolwe amehlo akho rhoqo ngugqirha wamehlo.
- Inkqubo yamathambo: Kufuneka uqwalasele nezinto ezifana nomqolo wakho.
Iingcebiso malunga nomsebenzi womzimba
Ukuzilolonga kulungile kumntu oneMarfan Syndrome, kodwa ayizizo zonke iindlela zokuzilolonga ezilungileyo. Ezinye iindlela zokuzilolonga ezinzulu zinokubangela uxinzelelo kwi-aorta. Ngoko ke, kubalulekile ukuthetha nogqirha wakho ukuze ufumane ukuba zeziphi iindlela zokuzilolonga ezifanelekileyo kuwe.
| Imisebenzi efanelekileyo (ngesiqhelo) | Izinto ekufuneka uziphephe/ongazenziyo |
|---|---|
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Utyando lwentliziyo
Ukuba i-aorta iba banzi ngendlela eyingozi, oogqirha banokucebisa utyando lokususa inxalenye ebuthathaka nokufaka i-graft ngaphambi kokuba iqhekeke. Ukuba iivalvu zentliziyo zonakele, utyando lunokwenziwa ukuze zilungiswe okanye zitshintshwe.
Ubomi kunye nempilo yengqondo kunye neMarfan Syndrome
Ukuphila neMarfan Syndrome kunokuba nzima ngamanye amaxesha. Ukuhlala ubona oogqirha, usela amayeza, kunye nokwenza utshintsho kwindlela ophila ngayo kunokuba yinto edinisayo.
Kwaye kwakhona,
- Ukucinga ngembonakalo yomzimba wakho kunokubangela ixhala.
- Iintlungu zomzimba rhoqo kunye nokudinwa.
- Xa ungakwazi ukudlala imidlalo, ukubaleka, nokudlala njengabanye abantu, ungaziva ngathi ukhe wedwa.
- Izinto ezifana nekamva kunye nokuqalisa usapho zinokubangela uloyiko.
Ngenxa yezi zizathu, kukho umngcipheko wokuba neengxaki zengqondo ezifana nokuxhalaba kunye nokudakumba .
Khumbula, impilo yakho yengqondo ibaluleke njengempilo yakho yomzimba. Ukuba uziva uxinezelekile okanye uxhalabile, thetha nomntu omthembayo. Ukuba kuyimfuneko, ungaze ungathandabuzi ukufuna uncedo kugqirha wengqondo okanye kumcebisi.
Ngenxa yolwazi esinalo malunga neMarfan Syndrome kunye nonyango olutsha, ubomi bomntu onesi sifo ngoku bufana kakhulu nobomntu oqhelekileyo. Eyona nto ibalulekileyo kukubona isifo kwangethuba kwaye usilawule ngokufanelekileyo.
Umyalezo Wokuya Ekhaya
- I-Marfan Syndrome yimeko yemfuza ebangela ukuba izicubu zomzimba wethu zibe buthathaka.
- Iimpawu eziphambili zimde ngokungaqhelekanga, umzimba omncinci, amalungu amade, amalungu akhululekileyo, kunye neengxaki zokubona.
- Eyona ngxaki iyingozi yesi sifo kukwanda nokuqhekeka kwe-aorta.
- Nangona kungekho nyango lupheleleyo loku, lunokulawulwa kakuhle kwaye ubomi obusempilweni bunokukhokelwa ngamayeza, ukujonga rhoqo ugqirha (ingakumbi iimvavanyo ze-echo), kunye nokutshintsha indlela yokuphila.
- Ukuba ukrokrela ukuba wena okanye umntu osapho lwakho unale mpawu, bonana nogqirha wakho ngoko nangoko ukuze nixoxe ngayo. Ukufunyaniswa kwangoko kubaluleke kakhulu.
- Nyamekela impilo yakho yengqondo kunye nempilo yakho yomzimba. Fumana uncedo ukuba uyalufuna.
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