Ingaba icala lasekhohlo lomntwana wakho libuthathaka? Masithethe ngeHypoplastic Left Heart Syndrome (HLHS)

Kunzima ukuchaza indlela ovakalelwa ngayo xa ugqirha wakho ekuxelela ukuba umntwana wakho osandul’ ukuzalwa unesifo sentliziyo esinzima. Kunye noloyiko, ukothuka, kunye nosizi, umbuzo othi “Kuza kwenzeka ntoni kumntwana wethu?” uvakala engqondweni yakho. Lixesha elinzima kakhulu. Kodwa eyona nto ibalulekileyo ngexesha elinje kukwazi ngokupheleleyo esi sifo. Namhlanje siza kuthetha ngeHypoplastic Left Heart Syndrome (HLHS), isifo sentliziyo esinzima nesingaqhelekanga esibangelwa kukuzalwa. Kweli nqaku, siza kuthetha ngale nto ngokulula, ngendlela onokuyiqonda.

Yintoni kanye kanye iHypoplastic Left Heart Syndrome (HLHS)?

Ngamafutshane, iHypoplastic Left Heart Syndrome (HLHS) yimeko eyenzeka xa icala lasekhohlo lentliziyo lingakhuli kakuhle ngelixa umntwana ekhula esibelekweni. Esi sisifo sentliziyo esizalwa naso. Igama elithi "hypoplastic" lithetha "ukukhula okungaphelelanga."

Intliziyo yethu ifana nendlu encinci enamagumbi amane. Amagumbi amabini phezulu namagumbi amabini phantsi. Iindawo zecala lasekhohlo lentliziyo yomntwana one-HLHS, ngokukodwa:

• I-ventricle yasekhohlo: Le yeyona ndawo iphambili yokumpompa intliziyo. Ipompa igazi elicocekileyo, elineoksijini emzimbeni wonke. Kwi-HLHS, lincinci kakhulu kwaye aliphuhli kakuhle.
• I-Aorta: Umthambo wegazi oyintloko othwala igazi ukusuka kwi-ventricle yasekhohlo ukuya kuwo wonke umzimba. Lo mthambo uhlala umxinwa kwaye uncinci.
• Iivalvu zeMitral neze-aortic: Ezi zifana neengcango. Zivumela igazi ukuba lihambe kwicala elinye kuphela. Kwi-HLHS, ezi valve zisenokungaphuhli kakuhle okanye zingavalwanga ngokupheleleyo.

Ngenxa yokuba la malungu aphambili awakhuli kakuhle, icala lasekhohlo lentliziyo alikwazi ukupompa igazi elineoksijini eyaneleyo emzimbeni wonke. Le yimeko embi kakhulu. Ngamanye amaxesha, kunokubakho umngxuma omncinci eludongeni phakathi kwamagumbi amabini aphezulu entliziyo, obizwa ngokuba yi- atrial septal defect .

Yintoni umahluko phakathi koku nentliziyo ephilileyo?

Kwintliziyo ephilileyo, amacala omabini asekunene nasekhohlo asebenza kunye. Khawuthelekelele ukuba icala lasekunene lithatha igazi elingenaoksijini emzimbeni lize lilithumele emiphungeni ukuze lifumane oksijini. Igazi elineoksijini elivela emiphungeni libuyela kwicala lasekhohlo lentliziyo. Emva koko ipompo enamandla kwicala lasekhohlo (i-ventricle yasekhohlo) ipompa elo gazi liye kuwo wonke umzimba.

Kodwa kumntwana one-HLHS, icala lasekhohlo lentliziyo libuthathaka kakhulu. Ngoko ke alikwazi ukwenza loo msebenzi. Ngoko ke kwenzeka ntoni? Icala lasekunene lentliziyo kufuneka lenze yomibini le misebenzi ngokwalo . Oko kuthetha ukuba i-ventricle yasekunene kufuneka ipompe igazi liye emiphungeni, ngelixa ipompe igazi liye emzimbeni wonke.

Kwenzeka njani oku? Xa umntwana esesibelekweni, kukho unxibelelwano lwexeshana phakathi kwemithambo yegazi emibini ephambili entliziyweni. Lubizwa ngokuba yi-ductus arteriosus.Kufana 'nendlela emfutshane'. Le 'ndlela emfutshane' ibalulekile kumntwana one-HLHS ukuze aphile emva kokuzalwa. Kuba igazi lihamba ngale tyhubhu liye emzimbeni. Ngokwesiqhelo, le tyhubhu ivala emva kweentsuku ezimbalwa umntwana ezelwe. Kodwa ukuba le tyhubhu ivala kumntwana one-HLHS, akukho ndlela yokuba igazi lihambe liye emzimbeni. Ke ngoko, ukuba le meko ayinyangwa, umntwana uya kuba neentsuku ezimbalwa zokuphila.

Uyazi njani ukuba umntwana wakho unale meko? Zithini iimpawu?

Ukuba umntwana osandul’ ukuzalwa une-HLHS, ngamanye amaxesha iimpawu azibonakali ekuqaleni. Kodwa ezi mpawu zinokubonakala kwiiyure okanye kwiintsuku ezimbalwa. Kubaluleke kakhulu ukuba abazali bazi ngale nto.

Kutheni oku kusenzeka kwiintsana? Yintoni isizathu?

Lo mbuzo usemqondweni womzali ngamnye. Kuqhelekile ukuzibuza, "Kutheni oku kwenzeke kumntwana wethu? Ngaba senze into engalunganga?"

Kodwa inyaniso kukuba, kwiimeko ezininzi, akukho sizathu sithile se-HLHS . Oko kukuthi, asiyonto ibangelwa ngumama okanye ngutata. Ngamanye amaxesha, izinto ezibangela imfuza zinokudlala indima. Oko kukuthi, utshintsho oluthile lwemfuza kusapho lunokwandisa umngcipheko. Iintsana ezinezinye iimeko zemfuza, ezifana ne-Turner syndrome okanye i-trisomy 18, nazo zisengozini ye-HLHS.

Oogqirha bayixilonga njani ngokuchanekileyo le sifo?

Ngethamsanqa, ngobuchwepheshe obuphambili bale mihla, esi sifo sinokufunyaniswa ngaphambi kokuba umntwana azalwe, oko kukuthi, ngexesha lokukhulelwa . Ukuba kukho into engaqhelekanga entliziyweni ngexesha le-ultrasound scan eqhelekileyo ngaphambi kokukhulelwa, ugqirha wakho uza kukuthumela kwingcali aze akucele ukuba wenze i- echocardiogram ye-fetal . Oku kukuvumela ukuba ubone ulwakhiwo kunye nomsebenzi wentliziyo yomntwana ngokucacileyo.

Ukuze axilonge imeko emva kokuba umntwana ezelwe, ugqirha uza kujonga iimpawu zomntwana aze amamele isifuba sakhe nge-stethoscope ukuze abone ukuba kukho isandi sentliziyo esingaqhelekanga (ukugungqa kwentliziyo). Emva koko, kuya kwenziwa iimvavanyo ezininzi ukuqinisekisa imeko, ezinje:

• I-X-reyi yesifuba: I-X-reyi yesifuba inokujonga ubungakanani kunye nokuma kwentliziyo kunye nemiphunga.
• I-Echocardiogram: Olu lolona vavanyo lubalulekileyo. Lufana nokuskena kwevidiyo yentliziyo. Amagumbi entliziyo, iivalvu, kunye nemithambo yegazi zibonakala ngokucacileyo. Oku kuqinisekisa ukuba kukho i-HLHS.
• I-Electrocardiogram (EKG): Uvavanyo olulinganisa umsebenzi wombane wentliziyo.
• Uvavanyo lwe-pulse oximetry: Isixhobo esincinci esifana ne-clip sibekwa emnweni okanye endlebeni yomntwana ukulinganisa ubungakanani beoksijini egazini. Eli xabiso liphantsi kwiintsana ezine-HLHS.

Ngaba ikhona indlela yonyango lwale meko? Ngaba umntwana angasindiswa?

Ewe, le yimeko enzima kakhulu, kodwa kukho unyango. Kodwa esi ayisosifo esinokunyangwa ngamayeza. Ukuze kusindiswe ubomi bomntwana, kufuneka kwenziwe uthotho oluntsonkothileyo lotyando olunezigaba ezahlukeneyo .

Inyathelo Lokuqala: Ukuzinzisa Umntwana

Ngaphambi kotyando, umntwana kufuneka azinziswe. Inyathelo lokuqala kukugcina i-ductus arteriosus , umthambo wegazi 'omfutshane' esithethe ngawo, uvulekile. Iyeza elibizwa ngokuba yi -prostaglandin linikwa ngemithambo yegazi. Amanye amayeza angasetyenziswa ukunceda ukubetha kwentliziyo yomntwana, kwaye kungasetyenziswa ne-ventilator ukunceda umntwana aphefumle.

Inyathelo Lesibini: Uthotho Lotyando Olunezigaba Ezintathu

Injongo ephambili yolu luhlu lweenkqubo zotyando kukudlula ngokupheleleyo icala lasekhohlo elibuthathaka kwaye unike icala lasekunene eliqinileyo lentliziyo (i-ventricle yasekunene) umsebenzi wokupompa igazi liye emzimbeni wonke. Kufana nokuqondisa ukuhamba kwegazi kwicala elahlukileyo ngokupheleleyo.

1. Inkqubo yaseNorwood: Oku kwenziwa kwiiveki ezimbini zokuqala zokuzalwa komntwana. Olu lutyando olunzima kakhulu nolunzulu. Apha, oogqirha botyando:

• I-aorta engaphuhliswanga kakuhle iyakhiwa kwakhona.
• Kufakwa ityhubhu ekhethekileyo (i-shunt) ukuthumela igazi ukusuka kwi-ventricle yasekunene ngqo emiphungeni.
• Ngale ndlela, "inkqubo yetyhubhu" yentliziyo iyaguqulwa ukuze i-ventricle yasekunene ikwazi ukupompa igazi liye kuwo wonke umzimba kunye nemiphunga.

2. Utyando lukaGlenn (Utyando lwe-Bidirectional Glenn shunt): Oku kwenziwa xa umntwana ephakathi kweenyanga ezi-4 nezi-6 ubudala . Apha:

• Ityhubhu (i-shunt) efakwe ngexesha lotyando lokuqala iyasuswa.
• Igazi elingenaoksijini elivela kwinxalenye ephezulu yomzimba (elivela kumthambo obizwa ngokuba yi-vena cava ephezulu) liqhagamshelwe ngqo emiphungeni.
• Oku kunciphisa kakhulu umthwalo kwi-ventricle yasekunene, kuba ngoku akufuneki ipompe igazi elivela emzimbeni ongaphezulu, liya ngqo emiphungeni.

3. Inkqubo yeFontan: Eli linqanaba lokugqibela. Lenziwa phakathi kweenyanga ezili-18 kunye neminyaka emi-4 ubudala . Apha:

• Igazi elingenaoksijini elivela kwinxalenye esezantsi yomzimba (elivela kumthambo obizwa ngokuba yi-vena cava engaphantsi) liqhagamshelwe ngqo emiphungeni.
• Emva kolu tyando, lonke igazi elingekho kakuhle kwi-oxygen emzimbeni liya ngqo emiphungeni. I-ventricle yasekunene yentliziyo kufuneka ipompe igazi elicocekileyo nelinomoya-mpilo elivela emiphungeni liye emzimbeni wonke.

Emva kokuba olu tyando luthathu lugqityiwe, inkqubo yokujikeleza kwegazi yomntwana iqala ukusebenza ngendlela entsha ngokupheleleyo.

Zithini iingxaki zonyango?

Nangona olu tyando lusindisa ubomi, lunzima kakhulu kwaye lunokuba neengozi kunye neengxaki. Iingxaki zinokuvela ngexesha nasemva kotyando.

• I-ventricle yasekunene iba buthathaka ngenxa yomthwalo onzima ekufuneka iwuthwale.
• Ukuvuza kwegazi kwiivalvu zentliziyo.
• Izifo zesibindi.
• Izingqisho zentliziyo ezingaqhelekanga.
• Ukuqunjelwa kwegazi.
• Iintsholongwane.
• Ubunzima bokutya.
• Ukuxhuzula.
• Iziphumo kwizintso.

Ugqirha uza kuxoxa nawe ngazo zonke ezi ngozi ngokweenkcukacha. Iqela lezonyango liza kumjonga umntwana ngokusondeleyo njengoko besenza olu hambo.

Ngaba ukufakelwa intliziyo yindlela elungileyo?

Kwezinye iimeko, ukuba imeko yentliziyo yomntwana inzima kakhulu okanye ukuba oogqirha bacinga ukuba umntwana akayi kusinda kolu tyando, banokucebisa ukuba afakelwe intliziyo . Kodwa kufuneka balinde de kubekho intliziyo ehambelanayo. Kwakhona, emva kokufakelwa intliziyo, umntwana kuya kufuneka athathe amayeza okunciphisa umzimba ubomi bakhe bonke.

Ukuba umntwana unale meko, yintoni esifanele siyazi thina njengabazali?

Olu hambo lunzima, kodwa ngokulawulwa ngokufanelekileyo kwezonyango kunye nokhathalelo, abantwana abane-HLHS banokuphila ubomi obuhle.

• Ukuhlolwa kwezonyango ubomi bonke: Emva kotyando, umntwana kuya kufuneka aye kugqirha wentliziyo ubomi bakhe bonke. Kufuneka ahlolwe ubuncinane kanye ngonyaka.
• Amayeza: Abantwana abaninzi kuya kufuneka bathathe amayeza entliziyo ubomi babo bonke.
• Ii-antibiotics ngaphambi kotyando oluthile: Ngaphambi kotyando olunye, olufana nokukhupha amazinyo, kufuneka uthathe ii-antibiotics ukuthintela usulelo lwentliziyo (endocarditis).
• Umsebenzi womzimba: Umsebenzi womzimba womntwana wakho unokufuneka ulinganiselwe. Imisebenzi enzima, efana nemidlalo yokhuphiswano, ayikhuthazwa ngokubanzi. Cela ingcebiso kugqirha wakho ngale nto.
• Impilo yakho yengqondo: Olu hambo ludinisa kakhulu kubazali. Lunokuba luxinzelelo. Ngoko ke, thetha ngeemvakalelo zakho nomyeni/umfazi wakho kunye nosapho. Funa ingcebiso ukuba kuyimfuneko. Kuphela xa unamandla apho uya kukwazi ukumnyamekela kakuhle umntwana wakho.