Joel’s Story: A life with Aortic Stenosis

United Kingdom

Joel arrived quietly at 5.35pm in the middle of July 2008, the labour was quick, quiet and peaceful with just myself, Tony and our midwife there. It was everything I had hoped for. We burst out laughing as soon as we saw him - he was the double of his big brother Lucas !

The next morning whilst Tony was on his way into the hospital with Lucas, I rang my mum whilst we were waiting for them. I remember telling her that I had never in my whole life been so happy, for the first time I felt totally complete, I was fit to burst with elation.

Within an hour we were packed & ready to go home, the paediatrician came to do his final checks - but it took a lot longer than it did with Lucas and he spent all his time concentrating on listening to Joel’s heart and feeling the pulse at the top of his thigh. He turned to us after minutes of silence, ’I think little Joels got a heart murmur’ was all he said. Our world started to collapse around us.

Home was out of the question and we were transfered to SCBU, we were told Joel would probably go the next day to Leeds General Infimary (LGI) for an ECHO, but within half an hour a scanner was brought to him and he was taken into a room and scanned whilst various doctors whispered around him. I knew then that it wasn’t just a murmur. Minutes after that scan, we were told Joel would be transferred to LGI that day, that an ambulance was on standby and a special care nurse was coming in to be with him in the ambulance. Lucas was taken home by my best friend and Tony & I had to follow in our car.

When we got there, Joel had already been taken to HDU on ward 10, he’d been scanned and the nurses were settling him in. He was pink and warm and wriggling and didn’t look unwell at all. We looked at all the machines and the other babies and children in the unit and it seemed like a bad dream.

Shortly after we were taken into a room, there was an empty box of tissues on a table and dim lighting. I had spent most of the day sobbing and as I looked at Tony I think I was stunned into silence. ‘This doesn’t look good, does it?’ I said. ’No sweetie, not at all’ Tony answered quietly.

We sat down as a doctor and a cardiac nurse came in. He pulled out a piece of paper, started drawing a heart diagram and told us that Joel had Critical Aortic Stenosis. Because Joel was newborn, nothing could be done for him - he was too small for surgery and there weren’t any valves small enough for him anyway. We were told that Joel would die, he was surprised he had survived this long and nothing could be done for him. We were broken, utterly destroyed, unable to speak. The pain was physical, and nothing in my life had ever hurt so much. Tony and I clung onto each other, uncontrollably sobbing-but couldn’t look at each other. Eventually we came out and went back into see our small soldier, still not able to believe what they had said, after all apart from the wires and machines he looked so well.

Later that night, after another scan and another venture into the room with a different consultant -to be told the same thing, - we were asked to leave him so the HDU team could do what they needed to. It was 1.00 am and we went to the parents accommodation on the floor below - on the condition that they would get us immediately if we were needed. For four hours we looked at the ceiling, barely speaking - clutching each others hands but unable to reach other. I was sore and uncomfortable after giving birth, exhausted physically and emotionally - but nothing was going to keep me away from Joel any longer. I managed to get up to HDU to sit with him again. He had an NG tube for milk as he was now too tired to feed, and he was on prostin to keep his arterial duct open - the one thing that was currently keeping him alive. Other parents in the room kept smiling at me, the sad slow smile that only parents who have lived this can give. Machines were beeping, the nurses were quietly busy but always took time to explain what each machine, wire, drug, procedure was.

Later that morning, Joel was scanned yet again, it made me so angry. He would be scanned, in silence, as if we weren’t there. I realise now that they were only concentrating on what they were doing, but to us who had been told we had days left at most with him, it was very upsetting.

We were taken once again back to ’that room’ as we now call it. We met John Thompson, Joels cardiologist who once again ran over the condition Joel had, the implications it meant for us all. He said “he was prepared to try a Balloon catheter, to stretch Joels aortic valve”. There were risks - it might not work, he might have to have a limb amputated, he may not survive the surgery. But it was his only chance. We signed the consent form, and his surgery was booked for 1.00 pm that afternoon, two hours after we signed the form.

As we waited for the time to pass, I asked for Tony to take photos of him, as many as we could - just in case. Tony’s parents were travelling up to Leeds from Northampton - not knowing what they would be met with when they arrived. My mum was back at the hospital with us, she had to leave HDU when I asked for the camera, she couldn’t bear to watch as we prepared ourselves for him leaving us.

John Thompson and an anaesthetist came to go over again what would happen. They must have thought I was mental, all I could say was ’ I know you’ll do your best but please, please bring him back to us, he’s our world’. Of course they would do their best, but I had to make sure they knew how desparately we loved him, how we needed to have him back and safe with us.

Finally, they took him - a lovely team, they were smiling and seemed confident which in turn gave us hope. We walked behind them as they wheeled him to the doors of the ward, then let them take him to the theatre. The doors closed, I crumbled. We were told it would take around two hours for the op, and depending on how it went, that he’d probably have to go to PICU for recovery.

The three of us went down the café in the entrance of the hospital. We must have looked a sight, and quite a lot of people stared at us. There was me, with the puffiest most swollen and painful eyes you can imagine, alternating between crying and laughing. Yes, we laughed. We had reason to, we had a chance to be positive that something was being done to save him. I switched on my phone and around 60 messages flashed up for us - all sending us love, people praying for us, asking if there was anything that could be done. People can be so kind.

At 2.45 pm we went back up to the ward - there was at least another 15 minutes before they said he would be out of theatre, but coming out of the lift - there they were, wheeling our treasure back to the ward - he was wide awake, kicking his little legs and waving for all the world. They smiled as they told us ‘what a little trooper we had’. Back in the ward with him, Mr Thompson came to see us - ’you have a remarkable little man here’ he told us. Apparently, the catheterisation couldn’t have gone any better, the pressures around his little heart had stabilised almost immediately, and he had woke up remarkabley well, not needing to go to PICU. Tonys parents arrived to find us in the highest spirits, the switch from devastation to jubilation couldn’t have been greater. Three days later, we finally brought our baby home - albeit with bottles of
medicines and minds boggling about what to do and when!

Happiness turned to doubt over the next few days though, he seemed to be sleeping constantly and wasn’t feeding well at all. There was a sharp and obvious line between his chest and his tummy when he was breathing, I wasn’t convinced all was well. Five days after bringing him home, he was re-admitted to our local hospital and treated for an infection. After five days in there, we were allowed to drive Joel back to LGI, (complete with canula in his hand!) for an ECHO on his heart just to double check all was fine. We were told it was OK and that we could take him home, however I did ask on the way out how his ECHO compared to the one we had on discharge the previous week - thank god I asked - within a few minutes we were told Joel couldn’t go home, his left ventricle had almost stopped pumping and it was very unclear what his survival chances were.

It was the start of more heartbreak and the discovery that you are given no hope. The start of the ’wait and see’, the ’lets just see how this goes’. I understand now why you aren’t given hope, but I do believe you need hope and faith to get you through these times. Most of the week we were there, the four of us stayed in Joel’s room. Lucas didn’t want to go home, he needed to be with us. So we took it in turns, one of us in the single bed with Lucas, the other in the chair next to Joel. The days and nights were endless.

During the days, when the ward was busy it was easy to be optimistic. Medical staff were in and out constantly checking him -a reason to be optimistic. We needed Lucas to be the happy 4 year old we loved, so fought to keep as much normality and fun for him as possible. But the nights came, and the ward went quiet and the lights dimmed. Then a wave of pain and desperation hit me, I felt like I was dying inside myself. I would hold him, crying, silently begging anyone above who would hear, ‘please, please, please don’t take him’.

Finally, after seven days and when Joel had started to gain weight, we were yet again allowed to bring him home. This time there was no celebration, just a quiet journey home and the four of us settling into a different kind of new baby routine.

Things at home were steady and we had established what needed to be done when. We came to the conclusion that this was our life, but it was just different to what we expected it to be. Even so, we thought we ought to try to adapt to it as quickly as possible. We soon learned to get by on five broken up hours of sleep each night - taking it in turns to check him in the night and to spend a good hour or so trying to get him to take milk. Several nights were spent syringing milk into his tiny mouth in the early hours.

Ten days after we came home I was really concerned again about Joel’s breathing so I rang the ward just to check what to do. I was told that so long as he wasn’t gulping air then his breathing was fine. To me, he seemed not right, but everyone said ‘I was just being paranoid’, understandable apparently, but paranoid all the same.

On the Saturday, we decided to be brave and have a family day out. We thought we’d go see Tony’s parents in Northampton which is a two and half hour drive away, stopping halfway down to feed and change Joel. We thought being ’normal’ would be good for all of us.

As we neared Northampton, Joel had started whinging so I told Tony to stop the car. I got out and stepped to open Joel’s doors and shouted to Tony that we needed ambulance. Joel was cold, clammy and almost black. I lifted him out of his seat and passed him to Tony whilst he passed me the phone. Calling 999, I told them all I could but had no idea where we where. I ran into a pub, shouting to the bar staff ‘ please tell me where I am, I need an ambulance’ they must have thought I was mental. I ran back outside as I was told the ambulance was on its way to us, but to stay on the line and keep them informed of what Joel was doing. Tony was holding him, talking gently in his ear, trying not to cry. Lucas was sat in his car seat, ’ is Joel going to heaven ?’ was all he said.

A few minutes later and we heard the sirens and saw the ambulance flying towards us. The two paramedics were fantastic, and soon had Joel on oxygen and told Tony where he needed to go. When we arrived at Northampton General, the doors were flung open as Joel was handed to a team of doctors who were waiting for him, a security guard waited for Tony to tell him where to park and a lady waited in the entrance to bring Tony & Lucas straight to Joel & I.

After an hour in A&E, Joel was then transferred to HDU and hooked up to various machines again. More tests were done, they had him undressed and getting cold for an hour and couldn’t understand why they couldn’t get any blood from him or get a canula into him. Finally, when they decided to try get a canula into his tiny head, I snapped. I refused to let them do anything until they had him warm when it was obvious that his veins would be easier to get to.

Twenty minutes later, after being warmed through they managed to get what they needed. Joel had been taken off his diuretics in LGI when he was started on the Captipril, it was thought his body would be able to do the job on its own but it couldn’t. Joels body couldn’t get rid of his waste and in turn his liver become full of toxins and his vital organs were failing. All the blood had rushed to preserve his vital organs and that was why he has turned almost black. The most frightening thing was that we were told that had we finished our 15 minutes remaining of our journey, then Joel would have died.

That night, Tony took Lucas to stay with his parents whilst I stayed next to Joel in a chair. One of the night doctors came to chat with me in the early hours, saying what a fright the day staff had had with Joel. She started talking about Joel being in cardiac failure - it seems obvious now but at the time it was the first time anyone had ever put it that way. She told me that we ought to speak to Leeds about what would happen if Joel ’totally crashed’ and that we needed to think whether resuscitation would be the right thing to do for him. Also, to make plans for Joel not coming through this, talk about organ donation, funeral idea’s and be prepared again for losing him.

I looked at Joel. My tiny five week old precious baby, so fragile and vulnerable. Drips into him, and monitors everyway, wires attached to him. We had taken part in ovulation prediction trials just to conceive him, there was no baby more wanted than him. Yet once again, he was dying.

In the early hours of the morning, Joel was allowed some milk - just 20 mls and he was quite clearly starving, when the milk ran out, he screamed for more. It was music to my ears. Over the next few hours, Joel’s colour returned to pink, he started to have wet nappies and his little arms and legs were more active than they had been for days. We spent the following day in a side room, and were discharged the next. We drove back up the M1, steadily, stopping frequently to check him and couldn’t wait to get our beautiful boys home.

Later that week, we took him back to LGI for his routine appointment. They were well aware of the Northampton episode and were surprised that he needed the diuretics as well as the Captipril. After scanning him again, we were told that Joel’s left ventricle hadn’t made any improvements in its pumping, that his mitral valve was leaking back into his lungs and had scar tissue in it which meant that couldn’t improve without surgery . Again, when we pushed for whether he was likely to come through it, we were not given any hope. Joel’s heart was likened to ’a car doing 80 mph down the motorway in second gear. That’s what just existing for Joel is like’. At five weeks old, he had been on the brink of death on three separate occasions and each time he had fought back from it, astounding everyone each time.

For us, it wasn’t surpising that his ventricle hadn’t improved, his heart was working overtime to do the job without the help of the diuretics. We were told that Joel’s only hope of survival was weight gain and time. That ventricles in worse states had been seen to improve but that was very, very rare. That surgery at this time wasn’t an option as he wasn’t strong enough to survive it.

John Thompson must have thought we didn’t understand what he was telling us, because we walked out smiling and saying ’he’ll be fine, he’ll show you’. We were probably in complete denial. But he had overcome so much and was clearly a tough little cookie.

The next few months were tough. The toughest of my life. I daren’t take Joel out in case he caught any infections, I spent my time feeding him little and often, just to get his weight up. I missed Lucas’ first day at school because I was too frightenend to take Joel to the classroom. I spent day after day crying, heartbroken and unable to speak to my friends as I was incapable of conversation. The only things that mattered to me were Tony and our boys, and keeping us all together. There was no help, in the day whilst Tony was at work, I was utterly alone. I suffered terrible stress migraines, and used to feel my way around the kitchen to make a bottle for him, as my eyes would have flashing lights in them. Those days taught me a lot, taught me who my friends were (and thank god, they came out in force for me when they could), and showed me those who said they would do anything, but then did nothing. It was a good life lesson.

The next few months were fraught and we basically lived in between the stress of the hospital appointments, trying to act as normally as we could for the sake of Lucas and the sake of our marriage.

In October, we arranged a charity night for the Children’s Heart Surgery Fund at LGI. The hardest part was not knowing whether Joel would be alive when it came to the event. Thankfully he was, and 120 friends and family came out to support us and we raised £3125.00 for the charity. Everyone says it was a brilliant evening, to be honest though it passed me by in a blur.

Time went on and gradually at each appointment things started getting better. The mitral valve leak had improved, the ventricle was pumping better……….the mitral valve leak had all but vanished - the ventricle was pumping well and synchronous.

At Joel’s appointment after Christmas, we were told that Joel was doing amazingly well. We knew how much weight he was gaining and that improvements were being made, but this was the first time we were given long-term hope. For the very first time, since we were told of Joel’s survival chances at six months old, we were told that Joel was expected to survive. For us, it was a miracle and the cue for many more tears !!!

As I write this, Joel is now two and half years old. The first two years of his life were spent being as protected as possible from illnesses and places that could be risky for him. It’s very different from having a healthy child and many people don’t understand why we felt it necessary to go to such lengths. Joel had such poor immunity anyway and we felt we gave him the best start we could. It wasn’t an easy time, being at home alone with a sick child is a lonely, stressful and isolating life but it was definitely the right thing to do for us and him.

Joel! I hear his name and and I smile. I think of him and I smile. He is a joy everyday, always smiling, always giggling and trying to assert his independence. I find this remarkable for any child, but for my beautiful heart child, I think it’s incredible. He chatters, laughs at funny things, has started playgroup for a morning session, runs, hides, swims in his warm-belly wetsuit, climbs up slides and throws himself off beds, scraps with his now six year old brother, and is a very cheeky little monkey - so normal you’d never guess what he’s been through or what he faces!!!!

He isn’t out of the woods as such, he still has aortic stenosis and must one day have a valve replacement - but we can deal with that as and when it happens. For now, we will enjoy our special little man and whilst we are able, will try to treat him like any regular healthy little boy.

We’ll never be the same people that we were before. He’s here for a reason, and we’ve learned so much already. He is truly an inspiration.

Wrote by Gaynor Bearder
February 2010

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Gaynor Bearder has kindly given CHD-UK permission to use the photographs on this page.