Donna Jones - Angel Mummy to Charlie & Kian - both HLHS Warriors
On 15th January 2009, we excitedly went along for our 20 week scan at the Princess Anne Hospital, Southampton. All we were concerned about was seeing the baby again and possibly finding out the sex. We never paid any heed to the sonographer when she said she couldn't see all four chambers in the heart and to come back the next day to see a consultant who would be able to scan me better.
Our life changed forever. They sat us down and said there was a problem with our baby's heart. The said that the left ventricle was smaller than it should be and they believed it was due to a Coarctation in the Aorta (COA).
They said there was also the problem of a genetic disorder called DiGeorge's Syndrome or 22q deletion. They assured us that the Coarctation would be a minor operation, possibly a couple of days or weeks after birth and then we will go home and have our happy ever after.
Like most heart parents, I am sure, we went home and did all the research under the sun and went armed with questions for our follow up appointment, the following week.
The consultants were able to answer everything and again we were given reassurance. We were scheduled for a re-scan at 28 weeks – 11th March 2009.
Whilst we went with the knowledge at the back of our heads that we were going for a check up on baby's heart, we also went with excitement at seeing our baby again. I wish this was good news but they said the left ventricle was smaller again since they last saw us and they were pretty sure it was now Aortic Stenosis (AS) with a possible COA.
Again, we were reassured that it was a simple procedure, with balloon catheterization. Again, it would be a couple of days in hospital, a week at most. We accepted this and moved on to the next milestone – 36 week scan on 6th May 2009.
During the next 8 weeks, I grew bigger by the second it feels like and go on Maternity leave. By the time the 6th May comes around, I am beginning to get fed up with being pregnant and want to have this baby out. I also go to the doctor at around 30 weeks as I was beginning to feel depressed I guess and anxious about the baby. Apparently, it was all normal because of the news we had been given and it didn't last that long.
There are dates that heart parents will never forget and from this point on it is all about dates and they are all tied to significant events.
6th May – we are told that baby's heart had changed significantly since the 28 week scan and that the doctors were concerned. So much so they sent us away to talk about inducing me. Me being me, fed up of being huge, not being able to bend down, to sleep comfortably etc and almost begged them to induce me... how I wish I didn't.
7th May – got a phone call at 11.00am from the Fetal Medicine Unit (FMU) to say they were inducing me on Monday 11th May at 9.00am. I was all excited; it was an omen, our 9 year anniversary!!
On the night before induction, I had a shower and whilst in the shower I notice I have a show... was I about to go it on my own? No it wasn't meant to be.
We arrive at the FMU at 9.00am and after a quick scan, I am taken down to the Induction Suite. After an examination, I was already found to be 1cm – a good start for me! The pessary goes in and off we went on some walking to encourage baby to do something. And nothing happened. We returned for some lunch and then decided to go over to the main hospital to have a look around the shops. I will never look at WH Smiths in the same light again... the first contractions began and they didn't stop.
At roughly 5pm, they examined me and found that I was a good 2-3cm and the midwife brokemy waters and took me round to delivery. The contractions came thick and fast but after another examination at 8pm, I was found to have only gone to 3-4cm even though the contractions were appearing regular.
After discussion with the doctors and midwife, it was decided I needed to go on the Syntocin drip and I took the opportunity of being bed ridden to take the epidural too. Fast forward to 4am and they finally examined me and I was almost 9cm!!! At 5am I was fully dilated and left for an hour to see if baby would descend a bit further before pushing.
After an hour of pushing, baby really didn't want to come out despite my best efforts so we have forceps and at 8.14am, Charlie Eoin James Jones was born on 12th May 2009.
He came out blue and it took what felt like a lifetime to get him to respond. The doctors worked on him and got him stable and brought him round to me, long enough for me to stroke his head and to tell him that I loved him. He was then whisked away to the Neo-Natal Unit (NNU).
At 2pm, a cardiologist came to see me and Dean, my husband. They need to operate on Charlie... NOW... and they needed our permission. He told us that Charlie was now over in the main hospital on the PICU unit but he needed urgent surgery to help save him.
I cried as I hadn't seen him apart from those precious seconds after birth and hadn't even held him. Never mind, the fact that my family were en route to me from London to see me and him. But needs must and Dean signed the consent form as I was still too weak to do so.
We agreed to catheterization for Bi-Lateral Pulmonary Artery Banding and Atrial Septostomy (tiny rubber bands placed on the two main tubes coming out of the heart to restrict the blood flow and a hole made between the 2 upper chambers of the heart); if needed he would go for open heart surgery (OHS) to complete the procedure.
However you want to look at it, Charlie didn't end up going down until around 8pm. Long enough for his daddy and family to see him. Me, well I had to wait until the epidural had worn off and as I lost a lot of blood I had to fulfil certain criteria before I could even be wheel chaired over.
At 4am in the morning, we got the call that in the end he had to have OHS but he was back on PICU and so far stable.
At 7am, my wonderful midwife got permission to push me across to the main hospital so I could meet my son properly. In bed 6 of the PICU, I never saw all the tubes or wires or the ventilator, all I saw was my beautiful boy laying there. When I had composed myself, the nurses explained the different medicines he is on. Prostaglandin to keep the duct open, Adrenline, Morphine, Sedative, and many others. He had a feeding tube in but wasn't fed for a while. I spent most of the day at his side, only returning to the maternity hospital for lunch, medication and dinner. Before leaving Charlie's bedside that night, I will never forget this for the rest of my life. Dean and I were sat on the right side of Charlie and one of the consultants was sat on the left side on a stool, just staring at the monitor... he turned to us and said "It's not looking good, I am not sure if he will make the night." At 36 hours old, we were told that our precious
baby might not survive.
Well, he proved him wrong. In the 3 ½ weeks that ensued our stay in PICU, the doctors slowly learnt that Charlie liked doing things his own way. He dictated when he was happy for medicines to be weaned, how much milk he would absorb, he would give the impression he was going downhill and we were prepared for his Norwood (and at one point we were skipping the Norwood and going to the Glenn) as an emergency procedure but eventually we were only on oral medicines (except for Prostin) and he was absorbing milk. But there were also a lot of highs too; the first time he opened his eyes, squeezed our fingers, having his chest closed, being taken off the ventilator (he looked so pleased with himself) and the biggest high was when he was exactly 2 weeks old, I got to hold him for the first time. We also were told there was nothing genetically wrong with him, no DiGeorge’s. We had decided before he was born to have his cord blood tested and they came back clear.
I can’t remember exactly when we found out the extent of his heart problem but I remember distinctly how. We were sat there one Saturday, doing a bedside vigil. Being a weekend, the unit was quieter and everyone was more relaxed. The nurses were discussing Charlie between themselves and they mentioned that he had a ‘Hypoplastic Left Heart’.
Our ears pricked and we questioned them. Charlie had Hypoplastic Left Heart Syndrome (HLHS).
The nurses then became acutely aware that no one had told us the extent of his problems. Meanwhile, I had text my best friend and asked her to do research for me and bring it in when she came to visit. Only, when she arrived, she pulled Dean to one side as she was worried about what she found out. She told him that from what she found out was some children may not live until they are 5 years old and handed him the print outs she had. When he told me, of course I was upset but I vowed that if that’s all we had then it would be the best 5 years of my life. I guess I also said it with naivety as I thought my little boy would fight and I would have him to eternity. I guess those words will come back to haunt me. On the Monday, one of the PICU consultants, took us to a side room and sat us down and talked us through HLHS, surgery and the future.
We made it up to the children’s cardiac ward on Thursday 4th June 2009, a huge milestone for us. We went to grow and get stronger; a week later they came to tell me that they were going to do the Norwood on Monday 15th June 2009. We told no one. Due to previous disappointments, we didn’t want it to be cancelled again.
The weekend before was so quiet on the ward, there was only 3 patients (including Charlie) and they sent the other 2 home on weekend leave. They moved us into one of the side rooms and let both of us stay the night at his bedside.
The night before the Norwood, we were so nervous, it was a hot summer. And it was hot in the room that night. When they took Charlie’s temperature at 5am it was a bit high and they thought he might be running a temperature but after stripping him down it came down, he was obviously just hot.
So the time comes where we are wheeled down to theatre. It was the worst journey of my life. I had to hand over my baby boy and had no idea whether he would be coming back to me. Charlie had cried the whole time prior to leaving the ward, he was hungry, but as soon as we left, it was as if he knew and stopped crying and just stared at me. Whether it was just him saying it will be okay mummy, I will never know. Whilst sat in the pre-op area, we see one of the PICU consultants and he says to us he will be in there with Charlie and that gives us some peace.
A long 8 hours later, the surgeon came to see us. It was a success. We can go and see him on PICU in half hour. I never felt so relieved in all my life. The phone calls followed to family and friends to tell them that a) he had surgery and b) he was still with us.
I remember walking into the PICU, an all too familiar environment and not hearing what the doctors were telling me regarding the surgery and just spying my baby boy lying there in bed space 2. I just wanted to hold him tight and kiss him all over and thank god he came back to me. This time there was no hanging about and 5 days later we were back on the cardiac ward. 10 days later (15 days post op) we are finally discharged with the NG tube in place.
We spend the next 13 weeks at home. On the 5th day of being home, Charlie was laid on the sofa with his daddy whilst I am cooking dinner and all I heard was "Charlie, what have you done?" I come rushing in and he had pulled his NG tube out. We laughed about it and phoned the ward, as he had been doing so well with his bottle feeds we were told we can keep it out until he began to get fussy with his feeds. Luckily, that day never came. Yes, we did have problems with feeds, but more so the fact that he had infantrini milk which was thicker than normal formula milk as it was high calorie and therefore, we had problems with teats. He still put on weight every week and his Community Paediatric Nurse was pleased with him every week. We stopped needing to go to cardiac clinic every week and were told he needed a cardiac catheter in August, just to check his heart function better than they could on an Echo.
We geared ourselves up for a 3 day, 2 night stay in hospital and went to the park before going into hospital. Charlie was duly starved the morning before and as I eventually got my hungry boy off to sleep, they came with bad news. The anaesthetist that was scheduled for Charlie‟s operation was not a consultant and they were not happy to proceed without one so the operation was cancelled and we went home.
Fast forward to another month and we went in again for the cardiac catheter. This time it took place and they informed us that his heart was enlarged and increase his dosage of Frusemide to try and shrink it back down. They told us to come to clinic in 10 days time for a follow up and they would tell us then about Stage 2 – Glenn.
We duly went along to clinic and apart from getting annoyed during the Echo, we were given a clean bill of health and told that the surgeons have yet to discuss further surgery for Charlie.
He wasn't his usual self that afternoon and wouldn't settle or anything but eventually he did and seemed happy enough.
On Thursday 1st October 2009, Charlie woke up as per normal in a great mood. I had cuddles with him and gave him his bottle, breakfast and medication then we went into wake Dean up, as it was his day off.
We all had fun cuddling and playing in our bed and when we got up, Charlie was playing whilst we had breakfast. Dean took him for his bath and he started to grizzle but it was coming up to another feed and nap time so we thought nothing of it.
Dean got him in and out as quickly as possible but he still didn't settle out of the bath, like he normally did. We thought he was just being clingy and wanted his mummy but I was going for a shower and he had to learn to be settled by daddy. When I came out of the shower he was still grizzling and wasn't taking his bottle from Dean, I went to him and told him not to be silly and to calm down. Then every heart parents' nightmare happened.
He started struggling to breathe and became raspy; he also went this funny grey colour. I wasn't happy and said to Dean who should we phone – in 10 seconds I made my mind up and phoned 999 and as I was on the phone to the operator, Dean screamed at me that he had gone floppy.
He had been walking around with Charlie, trying to get him to focus on something else. I screamed at the operator. It was then she told me we needed to do CPR, I took over from Dean so he could put the phone on loudspeaker and began giving my baby boy CPR.
I knew as I was doing it, it was too late, he was already gone but that still didn't stop me trying to get him to come back.
It felt like an eternity until the paramedic turned up. But he did and I distinctly remember asking him if he was breathing and he said to me "not right now my love". He told Dean to go and wait for the ambulance and show them up and told me I needed to get dressed as we were going as soon as the ambulance arrived.
I was still in my dressing gown following my shower; all I did was put my pyjamas back on. In the ambulance the whole way there, both Dean and I's eyes were on the heart monitor and every time they stopped to see if his heart was beating, there was just a flat line.
We got to A&E and they tried so hard to get him back. I sat there to one side and remember wanting to scream at them to stop so I could just hold my baby but also wanting them to bring him back. I turned to one of the PICU Consultants who was sat with us and said he's gone isn't he and she said yes it looks like it. They told us that they managed to get his heart beating briefly but his body had already gone into failure. I asked them to stop and put him in my arms to finally go to sleep. The time was 10.50am.
Due to the nature of Charlie's death, we had to have a post mortem. I had to know why he died. Was it my fault? Did I do something wrong? The answer, No. He died of heart failure due to HLHS.
His little heart was 3 ½ times the size it should have been.
6 weeks after his death, we found out we were pregnant again. By now, we already knew our chances of another HLHS baby was 2-4%. Surely, we couldn't have another child with HLHS?
How wrong could we be? At 17 weeks and 5 days old, Kian Jones was born sleeping on Friday 19th February 2010. His post mortem showed, he not only had HLHS but he had a degree of atrial isomerism, bi-lobed lungs and partial malrotation of the intestine.
Our chances of having another child with HLHS now lie at 10%. We are told it is only a small chance but our response is 2-4% was smaller but we still ended up in that category.
Where are we now
We are undergoing genetic counselling and will be having tests on our hearts to see if there is anything there. We are fortunate to be under the head of genetic testing and we are also being referred to a doctor who is doing specific research into causes of HLHS.
Every day is an uphill battle but for now, we hope that one day we will get our happy ever after.
In November 2009, Dean, myself and 4 friends founded the Charlie Jones Foundation (www.charliejonesfoundation.co.uk).
Our objectives are to raise awareness into HLHS and to raise money for the wonderful PICU at Southampton General Hospital. To date, we have raised just over £8,000 and have many more events in the pipeline.
