Ingaba umntwana wakho unomthambo entliziyweni yakhe ofana nekrele? Masifunde ngesi sifo singaqhelekanga (Scimitar Syndrome)!

"Owu, umntwana wethu omncinci ubonakala enengxaki yokuphefumla, uyaphefumla nangona encancisa... ngamanye amaxesha ulusu lwakhe lubonakala luluhlaza okwesibhakabhaka..." Ngaba wakha wahlangabezana nento efana nale? Okanye wakha wayibona le nto isenzeka kumntwana womntu omaziyo? Ngamanye amaxesha ezi mpawu zinokubangelwa ziingxaki ezithile zentliziyo ezizalwa nazo iintsana. Namhlanje siza kuthetha ngesifo sentliziyo esingaqhelekanga, kodwa esibalulekileyo ekufuneka sazi ngaso.

Yintoni iScimitar Syndrome?

Oku kubizwa ngokuba yiScimitar Syndrome . Eli gama livakala lingaqhelekanga, akunjalo? 'Scimitar' ibhekisa kwikrele elinencam egobileyo, elalisetyenziswa mandulo kuMbindi Mpuma. Kule meko, ngamanye amaxesha umthambo wemiphunga, othwala igazi ukusuka kumphunga wasekunene ukuya entliziyweni, ubonakala ufana nekrele eligobileyo kwi-X-rays. Akumangalisi ukuba eli gama lavela njani?

Ngamafutshane, esi sisifo sentliziyo esizalwa naso . Oko kukuthi, imeko eyenzeka ngelixa umntwana esesesibelekweni. Oogqirha babiza esi sifo ngohlobo lwesifo olubizwa ngokuba yi-Partial Anomalous Pulmonary Venous Return (PAPVR). Oko kukuthi, kukho isiphene kwindlela imithambo yegazi evela emiphungeni edibana ngayo nentliziyo.

Kukho iimpawu eziphambili ezininzi ezinokubonakala kule meko:

• Umphunga wasekunene awukakhuli ngokupheleleyo (i-hypoplastic right lung). Oku kuthetha ukuba mncinane kwaye awusebenzi kakuhle kunomphunga kwelinye icala.
• Umthambo oyintloko othwala igazi uye kwimiphunga yasekunene (umthambo wepulmonary wasekunene) awuphuhliswanga kakuhle (umthambo wepulmonary wasekunene ongaphantsi kwe-hypoplastic). Oku kunokunciphisa nobungakanani begazi elifikelela kwimiphunga yasekunene.

Ngaba singayiqonda kancinci indlela ezisebenza ngayo iintliziyo zethu ngendlela emangalisayo?

Ngoko ke, ukuze siqonde ngokwenene okwenzekayo kwiScimitar Syndrome, masiqale sijonge indlela esebenza ngayo intliziyo esempilweni, akunjalo? Cinga ngentliziyo yethu njengepompo esebenza kakuhle kakhulu. Ipompa igazi kuwo wonke umzimba wethu, emini nasebusuku.

Oku kwenzeka rhoqo:

1. Igazi elisetyenzisiweyo, elingenayo ioksijini emzimbeni wonke liyaqokelelwa lize libuyiselwe kwicala lasekunene lentliziyo.

2. Eli gazi lingenawo umoya-mpilo ofanelekileyo elivela kwicala lasekunene lentliziyo lithunyelwa emiphungeni, ngemithambo yemiphunga .

3. Ngaphakathi emiphungeni, eli gazi `lifakwe ioksijini`, kanye njengokuba amanzi angcolileyo ehluzwa aze acocwe.

4. Emva koko, eli gazi lizele yioksijini libuyela kwicala lasekhohlo lentliziyo ngemithambo yemiphunga, eyimithambo ethwala ioksijini esuka emiphungeni iye entliziyweni .

5. Icala lasekhohlo lentliziyo limpompa eli gazi lizele yioksijini liye emzimbeni wonke, ngomthambo wegazi omkhulu , i-aorta .

Le yindlela iseli nganye emzimbeni wethu efumana ngayo ioksijini. Ayisiyonkqubo inzima kakhulu nemangalisayo le?

Ngoko ke kwenzeka ntoni kwiScimitar Syndrome?

Ngoku mhlawumbi uyaqonda indlela esebenza ngayo intliziyo ephilileyo. Okwenzekayo kumntu one-Scimitar Syndrome kukuba igazi elifanele ukuba livela emiphungeni ukuze liqokelele ioksijini liye kwicala lasekhohlo lentliziyo, endaweni yokuba lize ngaloo ndlela , lihamba ngendlela engalunganga lize liye kwicala lasekunene lentliziyo.

Khawucinge ukuba ubunokuphinda uqhagamshele umbhobho ozisa amanzi endlwini kwindawo engafanelekanga, mhlawumbi kwitanki yamanzi esele isetyenzisiwe. Oko kufanele kwenzeke akuyi kwenzeka ngendlela efanelekileyo. Imeko inokuvela apho kuxubana amanzi acocekileyo namanzi angcolileyo.

Kule meko, imithambo-luvo enye okanye ezingaphezulu (imithambo-luvo yemiphunga) edla ngokuthwala igazi elineoksijini ukusuka kwimiphunga yasekunene iqhagamshela kwicala lasekunene lentliziyo endaweni yecala lasekhohlo. Oku kuvumela ukuba elinye igazi elineoksijini libuyele kwimiphunga. Kwakhona kwenza icala lasekunene lentliziyo lisebenze nzima.

Ngamanye amaxesha, umphunga wasekunene awufumani igazi kwimithambo yegazi yemiphunga , kodwa liphuma ngqo kwi-aorta . I-aorta ngumthambo wegazi oyintloko othwala igazi liye emzimbeni wonke. Ngoko ke kukho ingxaki encinci apho. Oku kunokubangela uxinzelelo olungeyomfuneko entliziyweni.

Ixhaphake kangakanani le meko? Ngaba kufuneka soyike ngokungeyomfuneko?

Oku kusenokuvakala kusoyikisa kuwe, kodwa iScimitar Syndrome sisifo esingaqhelekanga kakhulu. Ngokwezibalo, sichaphazela usana olunye ukuya kwabathathu kuphela kwi-100,000 nganye. Kwakhona sifumaneka sixhaphake kabini kumantombazana kunamakhwenkwe.

Ngoko ke, musa ukoyika ngokungeyomfuneko, kuba oku kunqabile kakhulu. Nangona kunjalo, kubaluleke kakhulu ukukwazi oku.

Zithini izizathu zoku? Kutheni oku kusenzeka?

Amaxesha amaninzi, noogqirha banengxaki yokufumanisa unobangela weScimitar Syndrome. Nangona kunjalo, kukho izinto ezinokubangela umngcipheko wokukhula kwentliziyo kumntwana. Ezi ziquka:

• I-genetics: Ngamanye amaxesha iimeko ze-genetic ezidluliselwa kwizizukulwana zinokuba nefuthe.
• Amanye amayeza asetyenziswa ngexesha lokukhulelwa: Umzekelo, amanye amayeza e-acne afana ne-`(Isotretinoin - Accutane®)` anokuba yingozi. Yiyo loo nto kucetyiswa ukuba ungasebenzisi naliphi na iyeza ngexesha lokukhulelwa ngaphandle kwengcebiso kagqirha.
• Izinto ezichaphazela imeko-bume: Ukuba umama utshaya okanye usela utywala ngexesha lokukhulelwa, ezo zinto zinokuchaphazela ukukhula kwentliziyo yomntwana.
• Usulelo oluthile kumama: Umzekelo, ukuba umama ufumana usulelo olufana ne -rubella ngexesha lokukhulelwa, lunokuchaphazela ukukhula kwentliziyo yomntwana.
• Izifo ezingapheliyo zikamama ezingalawulekiyo: Ukuba izifo ezifana nesifo seswekile azilawulwa kakuhle ngexesha lokukhulelwa, zinokuchaphazela nomntwana.

Eyona nto ibalulekileyo kukuba, uninzi lwexesha, oku akulotyala lomntu. Ngoko ke ungazibeki tyala.

Zeziphi iimpawu ezikhoyo?

Iimpawu zeScimitar Syndrome azifani kuye wonke umntu. Abanye abantwana banokubonisa iimpawu emva nje kokuzalwa . Izinto ekufuneka ziqwalaselwe ngamaxesha anjalo ziquka:

• Ubunzima bokuphefumla nokukrwitshwa xa uncancisa.
• Ukutshintsha kombala wesikhumba kunye nemilebe okwesibhakabhaka/ngwevu (cyanosis): Oku kubangelwa kukungafumani ioksijini eyaneleyo emzimbeni.
• Ukuphefumla ngokukhawuleza.
• Ukudumba (ukudumba) emilenzeni, esiswini, nasemjikelezweni wamehlo.

Nangona kunjalo, ezinye iintsana zisenokungabonakalisi zimpawu de zibe zikhulile. Ezinye zisenokuhlala zingenazo iimpawu de zibe zikhulile. Nangona kunjalo, ukuba iimpawu ziyavela xa zikhulile, zinokuba nezi zinto zilandelayo:

• Usulelo oluqhelekileyo lwemiphunga.
• Ubunzima bokuphefumla (ukuphelelwa ngamandla) ngexesha lokuzilolonga okanye lokuzilolonga.

Oogqirha bayixilonga njani le nto? (Ukuxilongwa)

Oogqirha baneemvavanyo ezininzi zokunceda ekuxilongeni iScimitar Syndrome. Ezi zinokujonga izinto ezinje ngobungakanani bomphunga wasekunene kumntwana okanye kumntu omdala kunye nemeko yomthambo wepulmonary wasekunene.

• I-X-reyi yesifuba: Oku kunokunika umbono ocacileyo wentliziyo, imiphunga, iindlela zomoya, kunye nemithambo yegazi. Ungabona imo 'yeScimitar' apha.
• I-CT scan: Oku kuthatha ii-X-ray kwiikona ezahlukeneyo ukuze kudalwe imifanekiso eneenkcukacha ezibanzi zangaphakathi lomzimba.
• I-MR angiography: Olu luvavanyo olusebenzisa amaza emagnethi kunye nekhompyutha ukujonga imithambo yegazi ngokweenkcukacha.
• I-Transthoracic echocardiogram (TTE): Oku kufana ne-ultrasound scan. Ingajonga ngokusondeleyo ukusebenza kwentliziyo, amagumbi, iivalvu, kunye nokuhamba kwegazi.
• Ukufakwa kwe-catheter kwintliziyo: Kule nkqubo, kufakwa idayi ekhethekileyo kwimithambo yegazi kwaye kuthathwa i-X-ray ukuze kubonwe indlela igazi elihamba ngayo kwimithambo yegazi. Ikwasetyenziselwa ukulinganisa uxinzelelo entliziyweni nasemiphungeni.

Musa ukoyika ukubuza ngezi mvavanyo. Oogqirha benza oku ukuze baxilonge ngokuchanekileyo imeko kwaye banike unyango olungcono kangangoko.

Ngaba ikhona indlela yokunyanga oku? (Iindlela zonyango)

Ewe, unyango oluphambili lweScimitar Syndrome lutyando.

• Kwiintsana, ukuba oogqirha bayayibona le meko, badla ngokulingeka ukuba benze utyando kwiinyanga ezimbini zokuqala zobomi . Kuba okukhona esi siphene silungiswa ngokukhawuleza, kokukhona umntwana ekhula kakuhle kwaye impilo yakhe yekamva ingcono.
• Kwabantu abadala,Ayinguye wonke umntu one-Scimitar Syndrome oya kufuna utyando ngaphandle kokuba uneempawu eziqatha (umz., usulelo oluqhelekileyo lwemiphunga, ukuphefumla okungapheliyo). Nangona kunjalo, ukuba iimpawu ziqatha, utyando lunokufuneka.

Kukho izinto ezininzi ezinokwenziwa ngugqirha wentliziyo ngexesha lotyando:

• Umthambo wemiphunga, odibene kwindawo engafanelekanga , udluliselwa kwicala lasekhohlo lentliziyo. Ngamanye amaxesha kusetyenziswa amabala akhethekileyo koku.
• Ngaphandle koko, umthambo ufakwa ngqo kwicala lasekhohlo lentliziyo.
• Kunqabile kakhulu ukuba kwenziwe i-pneumonectomy ukuze kususwe ngokupheleleyo umphunga wasekunene ongakhulanga kakuhle, ongasebenzi kakuhle. Nangona kunjalo, oku kwenziwa kuphela xa kungekho ndlela yimbi.

Ngaba ikho indlela esinokuyithintela ngayo le nto?

Enyanisweni, akukho ndlela yokuthintela ngokupheleleyo isiphene sentliziyo esizalwa naso njengeScimitar Syndrome, njengoko sikwaphenjelelwa zizinto zemfuza.

Nangona kunjalo, kukho izinto umama anokuzisebenzisa ngexesha lokukhulelwa ukuze andise amathuba akhe okuba nomntwana osempilweni:

• Ziphephe ngokupheleleyo utywala, icuba, nezinye iziyobisi ngexesha lokukhulelwa.
• Ngaphambi kokuba usebenzise naliphi na iyeza, thetha nogqirha wakho malunga neengozi kunye neenzuzo zalo.
• Ukuba awukafumani ugonyo lwe-rubella , kubalulekile ukuba ulufumane ngaphambi kokuba ukhulelwe, njengoko ukosulelwa yi-rubella ngexesha lokukhulelwa kunokuchaphazela intliziyo yomntwana.
• Ukuba unezifo ezingapheliyo ezifana nesifo seswekile , zilawule kakuhle ngexesha lokukhulelwa.
• Thatha i-folic acid kunye nezinye iivithamini zokukhulelwa njengoko ugqirha wakho ekuyalele.

Buza kuba njani ubomi kule meko? (Umbono wexesha elizayo)

Abantu abenza utyando lweScimitar Syndrome badla ngokuba neziphumo ezihle kakhulu zexesha elide . Uninzi lwabantu aluphindi lubone zimpawu emva kotyando.

Nangona kunjalo, ngamanye amaxesha, ingakumbi kwiintsana nakubantwana abancinci, umthambo wemiphunga otshintshiweyo unokuba mxinwa. Ukuba oku kuyenzeka, kunokufuneka unyango olongezelelweyo.

Abanye abantu abadala abaneScimitar Syndrome banokuphila ubomi obusempilweni ngaphandle kweempawu. Nangona kunjalo, kwabanye abantu abadala, le meko inokukhokelela kwezinye iingxaki zentliziyo. Kwiimeko ezinjalo, umgangatho wobomi unokuchaphazeleka. Ukuba iimpawu ziyavela (ezifana nokuphefumla okungapheliyo, usulelo lwemiphunga), utyando lunokufuneka. Kusenokufuneka wenze uvavanyo olukhethekileyo ukujonga uxinzelelo lwegazi oluphezulu kwimithambo yegazi emiphungeni yakho.

Ngaba zikhona ezinye izifo zentliziyo ezinxulumene neScimitar Syndrome?

Ewe, abantu abaninzi abaneScimitar Syndrome banokuba nezinye iingxaki zentliziyo. Abanye bazalwa beneziphene ezininzi zentliziyo. Okanye, iScimitar Syndrome inokubangela ezinye iingxaki.

Ezinye iimeko ezinokuthi zihambelane nazo ziquka:

• I-Atrial Septal Defect (ASD): Umngxuma ophakathi kwamagumbi amabini aphezulu entliziyo.
• Umphunga weHorseshoe: Unxibelelwano olungaqhelekanga phakathi kwamacandelo amabini omphunga.
• Uxinzelelo oluphezulu lwegazi oluphuma emaphaphu: Uxinzelelo oluphezulu kwimithambo yegazi yemiphunga.
• Ukususwa kwemithambo yegazi (umphunga oncedisayo): Iqhekeza lezicubu zemiphunga elingasebenziyo nelihlukanisiweyo elinokuhambisa igazi okungaqhelekanga.
• I-Tetralogy of Fallot: Isifo sentliziyo esiqhelekileyo esifuna unyango lokupompa igazi liye emiphungeni kwiintsana.
• I-Ventricular Septal Defect (VSD): Umngxuma ophakathi kwamagumbi amabini asezantsi okupompa entliziyo.

Ukuva ezi zinto kunokuba yinto eyoyikisayo nangakumbi. Nangona kunjalo, kukho unyango lwezonyango lwazo zonke ezi meko. Eyona nto ibalulekileyo kukuthetha nogqirha wakho, uqonde imeko yakho ngqo, kwaye ufumane unyango olufunayo.

Imibuzo ebalulekileyo yokubuza ugqirha wakho

Ukuba ufumanisa ukuba wena okanye umntwana wakho une-Scimitar Syndrome, ungabuza ugqirha wakho imibuzo efana nale:

• Yintoni enokubangela ukuba i-Scimitar Syndrome ibe yimbangela enkulu?
• Ingaba mna/umntwana wam kufuneka ndityandwe?
• Kuza kwenzeka ntoni ukuba awutyandwanga?
• Ungayilawula njani impilo yentliziyo ngeScimitar Syndrome?
• Ukuba ndinale meko, mangakanani amathuba okuba nomntwana wam abe nayo?
• Ukuba ndinomntwana wokuqala one-Scimitar Syndrome, mangaphi amathuba okuba nomntwana wam wesibini abe nayo?

Ungaze woyike okanye ube neentloni ukubuza le mibuzo. Kubaluleke kakhulu ukususa onke amathandabuzo engqondweni yakho.

Okokugqibela, izinto ekufuneka uzikhumbule (Umyalezo Wokuya Ekhaya)

I-Scimitar Syndrome sisifo sentliziyo esingaqhelekanga, esizalwa naso esichaphazela kakhulu imiphunga yasekunene kunye nemithambo yegazi eyidibanisa nemiphunga.

Abanye abantwana basenokungabonakalisi zimpawu konke konke, ngelixa abanye banokufuna utyando kwiinyanga ezimbini zokuqala zobomi.

Iintsana ezifuna utyando kwangethuba zinokufuna unyango olungakumbi kamva kuba umthambo wegazi ophambukisiweyo uyancipha. Nangona kunjalo, abantu abadala abaninzi nabantwana abenza utyando lweScimitar Syndrome baphila ubomi obusempilweni kwaye baphila ubomi obuqhelekileyo.

Siyathemba ukuba olu lwazi luza kukunceda. Ukuba unemibuzo engaphezulu malunga noku, qiniseka ukuba uthetha nogqirha wakho. Hlala usempilweni!

"I-Scimitar Syndrome, isifo sentliziyo, iziphene zokuzalwa, imiphunga, ukujikeleza kwegazi, iimpawu zeentsana, utyando