This is a true account about the bravest person I have ever had the honour to meet, my son, Harvey Castel-Nuovo, born October 2009
When my husband and I found out that I was pregnant after 6 months of trying we were over the moon. I was nervous for the first few months having experienced a few miscarriages in the past, but when the date came through for the detailed scan, I started to feel less anxious and more excited as we were past the “danger period”. On the day of the scan we were sat in the waiting room, appointments running late as they often did, and I suddenly had an overwhelming feeling of anxiety again, I can’t explain it but I just felt that I was going to have some bad news, before we were even called through, I suppose looking back it was mothers intuition. I brushed the feeling to one side and we were called through. My husband was beaming as we watched our little baby wriggle and scratch his head. The sonographer was showing us the spine, head etc., saying everything is fine. I felt so relieved seeing my baby alive and well. But then she started to be quiet, and asking me to turn to one side as she couldn’t get a good view. She said that the baby wasn’t in a very good position. I asked what she couldn’t get a good view of, she didn’t reply. I felt a shudder down my spine as I looked at the screen, she was scanning his heart. I asked if there was a problem, she didn’t reply. (she was concentrating). Finally she said to me that she couldn’t get a clear four chamber view of the heart. I asked what that meant exactly, and she told us to take a seat in a side room and some one would be in to explain. I was in shock, my husband tried to comfort me telling me not to worry. As we waited I felt numb. Another nurse came in and repeated the conclusion of the ultrasound, stating that it seems that there may be a problem with the heart but they needed a second opinion and referred us to see fetal echo specialist in the fetal care department.
As it was the Friday before bank holiday Monday we had to wait until the Tuesday. It was an agonising weekend. We attended the appointment at the fetal care unit, the sonographer was very pleasant but quiet as she took dozens of images of our little ones heart. She asked us not to question her findings as she would speak to us afterwards. We waiting in a side room again and she came through, she explained that our baby’s heart had not formed properly, and that she could not see all four chambers or the tricuspid valve. She thought that the condition could be tricuspid atresia, with possibly an Atrial Septal Defect (ASD) or Ventricular Septal Defect (VSD) but she would need to have a heart specialist confirm if she was right. We were referred again to a specialist heart hospital 40 miles away in Leicester to see a fetal cardiologist to confirm the diagnosis the next day. My head was spinning, I went home and researched the condition on the internet. I could not believe that my poor tiny baby in my belly who was so active kicking and hiccuping could have a poorly heart and would need an operation. It seemed so unfair and surreal.
The next day we saw the wonderful Frances Bu’lock at Glenfield. She was straight talking and told us how it really was. His condition was actually complex Pulmonary Atresia with intact ventricular septum, an acutely hypo plastic right ventricle and a coronary artery fistula. She told us straight away that they would not be able to open up the right ventricle, it was practically non existent and even if it wasn’t the fistula would compromise its function. She did a diagram of a healthy heart and our baby’s hear to show the difference and explained that he had no pulmonary valve to pump blood to his lungs, which wasn’t a huge problem in utero as the blood was flowing through a duct which would naturally close off after birth when his lungs should take over. He would need to be put on a drug as soon as he was born to keep the duct open until a gortex shunt could be out in to take over so that he could be weaned off the drug. The only option was palliative surgery to create a single ventricle circulation. This would be done over 3 stages, a BT shunt within the first few weeks of life, the glen shunt sometime between 4 months and a year, and the complete fontan some time between 18 months and 5 years. She advised us to consider termination as an option if we felt that we would not be able to cope with it, and also told us plainly that statistically only 50% of babies with condition are alive at age 5 and the long term prognosis is guarded as until 20 years ago it was entirely fatal ( prior to the fontan surgery being introduced). It was so much to take in, on top of that there is a high correlation between severe heart conditions and trisomy deletion such as down syndrome so we were advised to have our baby tested. Despite all this heart wrenching news my husband and I felt so strongly about our baby that we were in full agreement that we wanted to continue with the pregnancy.
The next four months were filled with dozens of appointments at our local hospital and Glenfield with student doctors and trainees present at every visit as its such a rare condition that they had to learn from the scans, as well as visits to PICU at both hospitals as I was going to be delivering at my local maternity unit and have him transferred by a neo natal ambulance to Glenfield. I was told that we could not travel in the ambulance but if I had a simple delivery and was discharged we could follow and meet him there. I had so much going through my head, what if I DIDN’T have a straight forward delivery, what if I COULDN’T be with my baby, what if I had to stay at hospital alone while my husband went with him, how would I cope being parted from both of them and how would he cope without me there. So many uncontrollable variables, no one ever knows what will happen in labour. I decided that the best thing I could do was stay calm and focus on the positives and deal with stuff that I could control. I had to make child care arrangements etc. for our boys, again this was difficult as we didn’t know when he would arrive.
Due day came and went, I finally went into labour 8 days late, when we sorted the kids out we made our way to the hospital. We arrived about 9.30pm, the mid wife put me on a fetal monitor tracing the baby’s heart beat and checked me, I was only 4cm dilated and the contractions were getting stronger and coming every minute. I was worried that I was in for a long painful labour,I couldn’t move about as I was attached to the monitor. We were introduced to the paediatrician, the neo natal doctor, another doctor and a second mid wife all on stand by ready for delivery. I felt robbed of the happy natural birth that I had planned in my head, I didn’t want any pain relief encase it affected my baby or delayed my recovery and I couldn’t be with him. I kept looking at the fetal monitor and asking if he was OK. I was checked again at 11 o’clock, I was 8.5cm and my waters were still intact. By 11.45 I was feeling the urge to push,she checked me again, 9.5cm water not broken, they decided to break my waters so that I could start to deliver. By 12.20 I was pushing, his head was out but his shoulders were stuck, the mid wife told me that if I didn’t get him out in the next push I would need surgical assistance (I later found out it was shoulder dystocia). I knew I had to get him out safely so I used all my energy to give a huge push to get him out, he was born at 12.34am. My husband cut the cord,he was placed on me me literally for a second, he looked blue but beautiful,then he was gone, whisked away the neo natal intensive care. I didn’t have time to get upset, I had a cup of tea and toast then a shower, by 1.20 we were visiting our gorgeous helpless new baby. They had just weighed him, a rather impressive 8lb 1. He looked so beautiful, he had been put on progastrian and had all sorts of wires and monitors beeping around his incubator. He was trying to pull his wires out, feisty little man! We put our hands through the little windows on the incubator and touched him gently. They told us a transfer had been arranged for 9am and were told we could sleep in a side room. I had some blood taken in case they needed to do an emergency transfusion as they were in the process of checking his blood group (I am rhesus negative, a universal donor).
We stayed with him until 4.30 then went to get some sleep.
The transfer team arrived at 9.15 and told us that they would not be using the blue lights and going at normal speed. As he was loaded they told us that they would use the lights to get through the traffic and lights and not to follow them. This panicked us. Ironically we did not follow them but went the route we knew and arrived 10 minutes before them.
We had named our son Harvey. dozens of doctors and nurses came to do tests and monitor him, they took blood, put cannula’s in, did scans, ECG’s. The poor little guy was fretting, and he was starving as he was nil by mouth, I couldn’t even feed him, although I did express some ready for after his op. Our cardiologist Dr Duke spoke to us to say that Harvey had been put on the list for the next day but wasn’t sure what time as he was waiting for confirmation of theatre slots. I didn’t realise he would be operated on so soon after birth, it just compounded the seriousness of the situation. We stayed in a room at the hospital,the next day we just waited with him, trying to comfort him, holding his finger and stroking his head. We met Dr. Firman the surgeon who would be operating on Harvey who explained the operation and risks and got us to sign the consent forms. It was about midday when we were called to theatre with him, the anaesthetist asked for his date of birth, and I just mumbled “yesterday” and burst into tears, reality had hit home that our one day old baby boy was about to have a major operation. We stayed with him until he was asleep then kissed him goodbye. It was the hardest thing we had ever done, walking away and leaving him and his fate in the hands of the surgeons. We were told that it would take approx 6 hours until he was in recovery. The wait was the worst gap in my life ever. We tried to eat, we were so tired we hardly spoke to each other, just sat holding hands, both understanding how the other felt but unable to speak in case the tears started again. We finally got to see him in PICU, it was shocking, he was covered in red dye with lines drawn on him his mouth had a huge ventilator down his throat and his nosed was taped up, an ng tube up his nose, wires and monitors everywhere. He was on a long list of drugs via a total of 5 cannula’s in all four of his little limbs, had a chest drain and a catheter. Poor brave little man! It was a waiting game. The next 24 hours were crucial. I didn’t want to leave his side, my husband had to make a quick visit home to check on the dogs/kids etc. and pick up some things. I sat watching him on life support, willing him to be strong. The senior nurse on duty kept asking me if I wanted a drink, and telling me I should get some rest, trying to convince me to leave him, but I couldn’t and wouldn’t. She said that I needed to think of myself as I had just given birth, but all I could think of was that is nothing compared to what my little ones been through and I needed to be strong and be with him. While my husband was gone there were a few wobbly episodes when his alarms all went off and the staff had to use a bag to get his breathing back on track and suction to remove mucus blockages from his ventilator, his sats kept dropping really low, it was really worrying. A few hours later my husband returned, I was so pleased to see him, we stayed by Harvey’s side all night.
Dr Duke came to speak to us after the doctors ward rounds, he explained that although the surgery had gone really well there was still not as much flow as they would like, and he wanted to do a surgical procedure called a atrial septostomy. This involved a wire being inserted in Harvey’s groin, passed up to his heart, through the left atrium to the right, inflating a balloon attached to the end of the wire then pulling it back through the wall of the two chambers to “rip” it causing a defect known as an ASD to allow communication in the upper chambers. It seemed strange that they wanted to cause another defect to help his poorly heart, but we knew it had to be done, he explained the risks and said the longer we leave it the riskier it will be and Harvey’s condition could get worse in the mean time, plus if he did it that day it would be while Harvey was still under from the previous operation. We gave our consent, we just wanted our little boy to get better. It was scheduled for 12.30 lunch time. I couldn’t get my head round it, two days old, two major operations, so so unfair. We waited in the parents room while they carried out the procedure, it took a lot longer than expected, it should have been a 30 minute procedure and we waited 2 hours, we started to panic that something had gone wrong. It was such a relief to see Dr Duke, he told us he was fine the delay was due to the equipment taking a while to set up (plus they had to wait for it to come from another theatre). What a relief!
We stayed with Harvey as much as we could, took it in turns to go and make phone calls etc. to family, sometimes went together to get some food, sometimes my husband had to make flying visits home. After one he returned with the biggest teddy I have ever seen wearing a t-shirt with “Harvey” printed on it, unfortunately the bear was not allowed on PICU. We had a room with 2 beds and sink and TV down the corridor from PICU, with a shared shower room, it was clean and a space for us to lay our heads for a few hours at a time. I even tried to watch X factor for an hour to feel “normal” as I expressed some more milk but I just couldn’t stay away from him for long.
Gradually Harvey started to improve, and as he did he started to be weaned off some of his drugs, I can’t remember all the names but one was dopium for his blood pressure, potassium for his salt levels, some antibiotic, morphine for the pain (although he was also on dipostry paracetamol). They monitored how he coped with having his morphine turned down to see if he could be taken off his ventilator as he was starting to do some breaths himself over the ventilator. Eventually, 2 days after the 2nd op, they took out his chest drain and turned his sedation down.
The next day he showed signs of waking so they turned his sedation down again. He opened his big brown eyes and took a peak at the world and us. It was amazing! The nurses fed him some of my expressed milk down his ng tube. That night they decided to extribate him (take him off the ventilator). They said they may have to put him back on it if he doesn’t cope, but thankfully he did. They turned his sedation off completely. He was doing so well in just 4 days since his ops. We were so proud and thankful.
The next day at just a week old he was transferred to ward 30, a high dependency ward. It felt amazing to be leaving PICU, like a huge achievement for him, we knew he was moving in the right direction. The nurses allowed me to give him a bed bath and we had cuddles, it was so special for us to hold him. I was allowed to feed him breast milk down his tube, his appetite was growing rapidly and I asked if we could try him on a bottle of breast milk. It was so satisfying to hold him and give him an ounce of milk from a bottle. I was soon having to express an ounce an hour and gradually they turned down his oxygen. They had to leave his ng tube in as he couldn’t always manage all his milk orally and had to have whatever was left down his nose.
After a day of doing really well, we had a bit of a set back, his sats dropped really low and they had to turn his oxygen right up and he wasn’t feeding as well. They did all sorts of blood test and scans but they didn’t show a cause. The conclusion was that he may have a cold. Thankfully he started to pick up again after a few days and the oxygen was reduced again- his tube finally came out! They told us if he tolerated full feed from a bottle for a full 24 hours he would be discharged, we were so excited! The new born clothes we had packed for him didn’t fit so in between feeds we went shopping, excited at the prospect of taking him home, we checked that we could get the car seat in and packed his huge teddy. The next day after the ward rounds we were told he would be discharged as soon as his drugs were ready for us. He was on oral aspirin. It was the best news I HAVE EVER HAD.
Harvey has continued to do well, gaining weight, smiling lots. We had a scare before Christmas when he was rushed into hospital and his sats were really low, they gave him oxygen therapy for 4 days then he was discharged after numerous tests showed that again it was just a common cold that had affected his breathing. We have to take him for regular ward reviews and inoculations, one of his pulmonary arteries is smaller than the other and may need surgery for that, but otherwise we just on with things. Its hard knowing that a cold could cause him serious harm, we just have to make sure he’s wrapped up well ad avoid people who have been poorly for his sake. Harvey will be having further open heart surgery (Glenn shunt) sometime later in the year, not sure when yet. He will also have to have the man made ASD repaired at some point but they haven’t told us when. So all in all he will require quite a lot of surgery in the future, but they way he has handled it so far inspires me with faith that he will come through each one of them stronger. He is the happiest little baby, if you didn’t know what he had been through you would never be able to tell, he is my hero!
Thank you for reading our story x
Wrote by Nina Castel-Nuovo
Nina Castel-Nuovo has given CHD-UK permission to use the photographs on this page.