Ends at: 09 June 2021
Ignacy’s heart is still beating, but the day will come, when it will be no more... We’re living on the ticking bomb. Once it explodes, there will be nothing left. Every time I speak about this tears flood my eyes, but I know I have to stop them... Next to me I have my lovely, joyful 3 years old and if he sees me crying, he will share my sadness and that can lead to tragedy. With such significant heart defect and congenital pleural hypertension every sudden oxygen drop may cause unconsciousness. Continues fear cannot go away, because I always see his blue fingers from cyanosis, sweat, breathlessness and tiredness even after not demanding play with his siblings and friends. Every moment can bring the last beats of his weak hearth...
Ignaś was born with the congenital heart defect with critical aortal stenosis (AS) with impaired function of the left ventricle (fibroelastosis) and congenital pleural hypertension (HP). I learned about my dear son illness during the USG test in 26th week of the pregnancy. I knew already, that there would be no miracle, that this was like a sentence, although not clear yet, how severe. In that moment, I just wanted to cry out loud from my powerlessness. But then a spark of hope came to us, with the advanced treatment of modern medicine. In 27th week of pregnancy cardiologists conducted successful cardiac catheterization. Aortic valve of my son got widen and after it gave promising prognosis for future.
Ignaś was born on Thursday, January 11th 2018. First time I saw him I knew, it was love for life. I only had no clue how long his life would last. I took him into my arms, hugged, fed, deeply enjoyed the amazing feeling of giving life for the third time. I wanted him to be with me as long as possible, so he could hear the beating of my heart, feel warm of mother’s body and we could be there together and forget about the fact, that his dearest life is supported by so weak heart. Unfortunately, I soon hit the wall of the harsh reality. Doctors took my son away, as they were well aware he was actually dying with each breath. They gave him lifesaving drug. I was left there with overwhelming longing for my boy and unimaginable fear. I was left with no illusions, but deep in my heart I still hoped, that this nightmare will soon be over.
So many procedures with no results
In second day of Ignacy’s life he had the balloon aortic valvuplasty. It was necessary, because his aortic valve is two-leaflet (instead of three well developed leaflets, he has only two), bolden and narrowed, which significantly reduces the blood outflow from left ventricle. Sadly, this surgery did not bring expected results and it had to be repeated in 7th day of my little boy life. After that there were couple of heart catheterizations. Additionally, he had stent implanted to the arterial duct (PDA) and went through the Rashkind procedure. The latter was followed by the stay in the intensive care unit because of pulmonary atelectasis and sepsis. Finally, after three long and exhausting months, Ignaś arrived at his home and met his siblings for the first time. Still, the most important for me was that our fight was won. I was not expecting at all, that we would be back in the hospital so soon...
First year of his life Ignaś spent on continuous trips between home, hospitals and doctors. It turned out that the hearth defect is not the only enemy. I had to fight for every gram of his weight gain, I had to be there every night to make sure son kept breathing. Next the infections have come. Every single of them was disastrous for this young and weak body of Ignaś. I prayed for him to make it to the scheduled procedure – replacement of aortic valve. Unfortunately, it did not happen. Another infection forced us to fight for Ignacy’s life once again. Infection led to the collapse of right lung and on the morning of the Christmas Eve day he had the bronchoscopy done. Two days later he was moved to the intensive care unit and connected to the ventilator, due to the severe pneumonia caused by RSV virus. All the pipes, cables, drains, electrodes and set of infusion pumps continuously providing drugs... There we were again in the worst of the nightmares. Doctors prognosis were far from promising and they had no idea what else to do to save Ignacy’s. But my little boy decided to show everyone his determination, he simply de-intubated himself after two weeks!
The hope died, but Ignaś did not!
Our situation calmed down a bit in the second year of Ignacy’s life. We were waiting for the date of surgery. Dr Rudolf Mair was supposed to come from Austria. He is well known expert in this kind of hearth defects that Ignaś has. My son was taking his time to make up for the time lost in the hospitals. He started to gain weight, develop himself and learn things already mastered by his peers. Finally I got a phone call, that left me speechless and hopeless at the same time. Austrian doctor would not come and Polish doctor’s would not dare to try themselves conduct the surgery. It turned out, that regardless of being calmed down by them and their assurance of still having time, we did not have any left. The last things done were the dilatation of interatrial communication and implantation of the restrictor.
To add to this misery, Ignacy’s dad, the only provider for the family, had a very dangerous car accident. Drunk driver hit his properly driving car. My husband survived but with damaged spine and is still recovering from the accident.
My boy has chance to live!
Ignaś has had no real cardiac surgery so far and his hearth is now in critical condition. Systolic function of heart is deteriorating, the left ventricle works on the edge of limits. The echocardiography gives very bad results. Heart is enlarged, exhausted, all the parameters are bad. On top of it the congenital pleural hypertension is additionally demolishing it. Every doctor’s visit ends with new medications and higher doses of old ones. My boy takes 8 different drugs. He wouldn’t be here, if it weren’t for them. We have reached dead end in Poland, the only proposal is palliative care, as certainly Ignaś will not make it to have a chance for heart-lung transplantation.
The doctor told us though, that there is one hospital specialized in this kind of conditions. It is Boston Children’s Hospital in USA, where since many years there is established program of treating the two-ventricle hearts. I reached out to them and received positive response. The quote I got states 250 251 US dollars, which is close to 1 000 000 Polish zloty.
Lots of money and no time left!
My boy cannot wait any longer, soon the effects of his illness will be too severe to be reverted by any doctor! The most terrifying thought for me is that I may hear, that even experts in Boston cannot do anything. That it is simply too late... I wouldn’t survive this, not me, not his loving father, his sister Ewa or brother Antoś. Our family would die together with Ignaś heart. Please, help us to not let this happen! Help us save our most precious treasure! Please give Ignaś chance to live!
Monika Lenartek-Rapacz, mother