Daniel & Riley’s Story

After a healthy pregnancy we were very excited to meet our first born child. Daniel was born at Hornsby Hospital on the 18th August 1994 via caesarean section and within moments it was apparent something was wrong.  His little chest was caving in, he was making grunting sounds and it was clear he was having breathing difficulties. He was whisked away and placed straight under oxygen. As the day wore on his oxygen needs were getting greater and it was decided NETS needed calling. They immediately ventilated Daniel and he was transferred to RNSH NICU. He was subsequently diagnosed with Hyaline membrane disease of the lungs. We were told he was a very sick little boy.

He remained ventilated for 4 days and on day 6 required phototherapy for Jaundice. At this point he was getting better and we could finally see light at the end of the tunnel.

A routine check later that day found that he had a strong heart murmur and would need to be checked by a cardiologist. This was an agonising wait, we were hoping desperately for good news and it wasn’t to be. We were told our little baby had heart disease and would require open heart surgery.  Our world came tumbling down again. His diagnosis was Double outlet  right ventricle, and VSD (Ventricular Septal defect). Daniel remained in hospital for 3 weeks before we were allowed to bring him home.

 

At 4 months of age Daniel had his first surgery at Camperdown Children’s Hospital. It was a pulmonary banding of his Pulmonary Artery. He had too much blood going to his lungs and this would regulate that blood flow better. This surgery had complications, 3 of his 5 wires holding his chest together broke. This was extremely distressing for me to see him in so much pain; he went back to surgery 6 days later to have his chest rewired. A few days later we were able to take him home.

 

Daniel was very precious and we wrapped him in cotton wool. We were told to treat him normally, but what was normal, he was our first child. He progressed well and at 22 months had his final repair of his heart at the New Children’s Hospital Westmead. He was in hospital for only one week but we did not leave his side, not for 1 second, taking it in shifts around the clock. He had made a remarkable recovery, as kids do, and this time without any complications.

 

Within days of bringing Daniel home I was changing his nappy and suddenly found his scrotum the size of a lemon. Well naturally I was very concerned and after a trip to the doctor it was thought he had a strangulating inguinal hernia, which is potentially nasty. He was then nil by mouth until we got back out to Westmead and seen by a surgeon, where an ultrasound diagnosed bilateral hydroceles.  This required more surgery but was done about a month later to give him time to recover from his open heart surgery.

 

Daniel cruised, he had no issues with his heart and everything seemed to be going well until 5 years of age we noticed his heart was racing very fast. It wasn’t hard to miss because it was easily heard when lying next to him. It only happened a few times and we thought nothing of it.  That was until his next episode when his heart rate was much faster and he was nearly unrousable on the bed.  After several trips to the hospital and a little monitor called a ‘King of hearts monitor’ was used to diagnose him with Supra Ventricular Tachycardia (SVT). SVT is a condition where the heart beats very fast often double what it is supposed to.  Daniel was put on medication to control his SVT. He still had many episodes of SVT, some required him to go to hospital for his heart to be converted back to a normal rhythm and others were managed at home because his heart returned to a normal rhythm. In an attempt to fix this problem EPS studies were carried out under General anaesthetic but were unsuccessful.

 

At 7 years of age his SVT was becoming very problematic. After a nasty turn at school it was decided they would do another EPS study in an attempt to ablate the problem area in his heart. 5 hours under general anaesthetic and again it was unsuccessful. They had run out of options and it was decided to attempt again when Daniel was close to adult size at age 15 and more sophisticated adult equipment could be used.

 

Two months later while on a weekend away with friends in the Blue Mountains our beautiful Daniel aged 7 years and 8 months passed away very suddenly and very unexpectedly from another nasty abnormal heart rhythm, which this time was fatal. There was no way we were to know this would happen. That day in the Blue Mountains our lives changed forever.  We were devastated, and absolutely shattered, something I struggled with for a long time and still do today. He was my baby.

 

Daniel could never be replaced but his passing left a big void in our family. 13 months later we found out we were expecting again, our fourth child.  With this came a flood of all sorts of emotions, excitement that we were having another baby and sadness because Daniel would never know his little brother or sister.

 

Our first scan went well at 12 weeks. There was no reason to think anything was wrong. I ate well, didn’t smoke or drink and kept a positive outlook. The scan at 20 weeks was routine, I had done it 3 times before with 2 healthy boys after Daniel. The stenographer was asking a lot of questions about Daniel and his heart and I thought nothing of it. I just thought it was relevant past medical history and she was curious for that reason only. After about 10 minutes she stopped. She bent across to me and said, “It’s the same as Daniel”, and not sure what she meant I needed clarification. She then said this baby had the same heart condition as Daniel. These words were shattering, words I was not expecting to hear. I can only describe that moment similar in feeling to loosing Daniel because I thought we would now loose this baby that was very much alive inside of me. Another little boy, Riley, how could we possibly do this all over again.

 

Later that day I had an amniocentesis to rule out any chromosomal abnormalities and a visit to Daniel’s cardiologist for a more thorough foetal echo. The news only got worse. His heart condition was in fact worse than Daniel’s, and no guarantee could be given on his survival at birth. Riley’s diagnosis was Double Outlet, Hypoplastic Right Ventricle, Ventricular Septal Defect, (VSD), Pulmonary Atresia and Dextrocardia. There were surgical options for Riley and we decided to give him every chance at life.

 

The next few weeks were very difficult; we were still living with the grief of Daniel and now the grief of this news. Did I do something wrong to cause this? Could I have done anything differently? My guilt was overwhelming; we must do everything to protect our baby.

 

The pregnancy progressed normally and a Caesarean Section was booked. The day arrived and up until that point we couldn’t wait for Riley’s arrival. Well now I didn’t want him to come out, he was happy and healthy but that would change after he was born.

 

Riley was born and cried straight away, he looked remarkably well considering the seriousness of his heart condition. He was transferred to Westmead Children’s Hospital Neonatal Intensive Care Unit where he was stabilised. He remained in a stable condition with help from a drug called Prostin until he was 4 days old. Riley needed a Blalock Shunt (BT Shunt) which was the first of 3 major surgeries he would require. Handing Riley over to the theatre staff was heart breaking, we had done this all too many times before with Daniel.  We prayed that he would make it out the other end. It was a long anxious wait and Riley had come through with flying colours. Ventilated, and with multiple tubes coming out of his tiny body we felt helpless and wishing it was us lying there instead of him.

 

Over the next 2 weeks Riley lost all of his tubes one by one and was learning to breast feed. If he could regain his birth weight we could take him home finally. That day arrived when he was 2 and half weeks old. Our Joy didn’t last long, Riley was only home for 36 hours when he spiked a high fever and had become lethargic and floppy like a rag doll. We rushed him to RNSH where after a few tests he was transferred back to Intensive care at Westmead Children’s Hospital. More tests revealed he had a bad urinary tract infection (UTI). He remained in hospital for another week and then allowed home. We had to return in 2 weeks’ time for investigations into what caused this UTI. He was later diagnosed with Bilateral Ureteric Reflux of his Kidneys and may require surgery at a later date for this. He was put on Antibiotics for the long term as a preventative measure.

 

Riley had always been temperamental with feeding, some days were better than others.

By 3 months of age Riley was refusing to feed most of the time despite being hungry. It didn’t make sense. By 4 months of age he was hospitalised in an attempt to work out why he wasn’t feeding. We were unable to find any answers. He had been positing for a little while and this was becoming more frequent. Then he started vomiting blood, it wasn’t a huge amount but a huge concern. Feeding was now impossible, it totally consumed me 24/7 and I was at my wits end. I had to take control and instigated tube feeding because there was no other way. Riley was in complete refusal. What milk did reach his stomach he would often vomit back again. Riley was now 5 months old and being totally tube fed. This gave us some relief until the vomiting started to worsen, whole stomach contents and projectile.

 

In a desperate state and in tears to the feeding clinic at Westmead, they managed to squeeze us in within 2 weeks instead of a 4 month wait. I was hoping for some miracle answer but what was suggested I was already doing for Riley anyway. If this was to persist a surgical option was available and that involved putting a tube directly into Riley’s stomach and feeding him that way. More surgery?? No way, we decided to continue with the tube feeding via his nose, surgery wasn’t an option. So we set the alarm clock and started feeding Riley round the clock because that’s what we had to do to ensure he got adequate fluids and nutrition and also to keep his kidneys flushing. It became a 24 hr. job feeding Riley because every time he would vomit, he required feeding again, and simply crying was enough of a trigger for him to vomit his whole stomach contents. He could do this up to 4 – 5 times a day.

 

By 8 months of age I stopped the tube feeding, it had become too traumatic for Riley. He was vomiting so much he would often vomit the tube out and it needed replacing sometimes several times a day. What little we did manage to get down and what little did manage to stay down must have been enough.

 

An endoscopy at this time also gave us no answers. I came to a conclusion myself what could have been the cause for all Riley’s feeding problems. Riley was on Aspirin to keep his blood thin since birth, he had silent reflux which progressed to positing then to projectile vomiting. Aspirin would have irritated Riley’s Oesophagus eventually causing him discomfort. As his discomfort worsened so too did his refusal to feed. He was associating pain with feeding, so wouldn’t feed. This became habit over many months because medication would have alleviated any discomfort. It was a vicious cycle and extremely difficult time for the whole family. Our other 2 boys craved our attention.

 

 

At 10 months of age Riley went back into hospital for his Cardiac Catheterization, this was in preparation for his Bi Directional Glen Shunt planned for 1 year of age. All his heart/lung pressures were good and it was full steam ahead.

 

Riley was admitted days after his first birthday for his second heart surgery. It certainly doesn’t get any easier. The shock of walking into intensive care with your baby connected to multiple monitors and more tubes than you care to count is indescribable.

The journey in ICU can be a rough one. Sometimes it was 2 steps forward and 1 step backwards, which was really hard, and Riley was not immune to this. Riley’s Blood pressure was playing up which was a concern, but in time this came good. After 2 days in ICU he was transferred to the ward. He made a remarkable recovery here and was allowed home after only 1 week.

 

In the months after surgery the vomiting was starting to settle and the feeding was improving. By 18 months we saw light at the end of the tunnel. We could now finally enjoy Riley.

 

Riley progressed through the next few years without any incident. (Except for colds and the occasional fever) While he was blue, had no energy or stamina and tired very easily this was all normal for Riley due to his heart condition. This all became more pronounced as he was nearing the time for his final surgery. His oxygen saturations were slowly dropping from 80’s to 70’s, then high 60’s. It was time.

 

At 4 years of age Riley went into hospital again for another Cardiac Catheterization. This was necessary to ensure the pressures in his heart and lungs were okay, because if not, the outcome from the final surgery “The Fontan” would not be as good. Riley came out with really good pressures and this gave us the green light for his Fontan.

 

At 4 years and 8 months Riley was ready for his Fontan. After 2 weeks of isolation at home to keep him well, he went in for his final heart surgery. This was more difficult than the last surgery and there were more risks of complication. He could potentially be in hospital for up to 2 months so be prepared just in case. It was a long anxious wait, just like all the other ones, but this was the last. Riley came through surgery with flying colours again. The doctors were really pleased. He had 14 tubes coming out of him this time and that was a shock. He was taken off the ventilator within hours of the surgery. It was important for his lungs that he breathed for himself. This meant Riley was then aware and conscience enough to complain about his extreme thirst. He wasn’t allowed anything to drink and this was very distressing to watch. The following morning it looked like Riley would get an early mark and be able to leave ICU and go to the ward. A Chest X-ray revealed his right lung had half collapsed so he wasn’t going anywhere. He was draining a lot of fluid from his chest drains and it was this problem that could see him stay in hospital for up to 2 months. Riley stayed in ICU another 2 days before he was transferred out to the ward. He had all his tubes out except for his 2 chest drains which were still draining a lot of fluid. Over the next couple of days the fluid draining was getting less and eventually had both of these removed 2 days apart. Riley was making a remarkable recovery, especially when I thought we might be the ones to be in hospital for up to 2 months. On day 11 Riley was discharged. He was a champion and such a brave little boy.

 

Riley is now 7 years old and in year one at school. What a little miracle he is and an absolute joy. We are forever grateful to his team of surgeons and doctors who saved his life, and who continue to monitor his health. We can’t begin to thank them enough. His wonderful nurses out at Westmead Children’s Hospital for looking after him and us as a family, and to all our wonderful family and friends for continually picking up the pieces and for just being there.

 

HeartKids have been an integral part of our lives, they have been a constant source of support, and we have made many lifelong friends with wonderful people who share similar stories to us.

 

Riley is now in good health, he enjoys riding his bike and scooter, attends a recreational gymnastics class and actively plays with his older brothers. He is a very confident, loving little boy who is adored by many.

New South Wales