This story is so close to my heart, I’ve put off sharing it with you all for months now. But for you to fully understand CHD; you need understand families up and downs and how SO many times we’ve thought we were going to lose beautiful Amelia.Part 1;
Amelia’s journey – Lisa and Paul’s words
Amelia’s congenital heart condition is very rare: Tricuspid Atresia, (she has no tricuspid valve and she has a hypoplastic right ventricle) also a VSD(Ventricular Septal Defect) large ASD (Atrial Septal Defect) Mild Pulmonary Stenosis, (narrowing of the pulmonary valve). Pulmonary obstruction and pinch. Mitral Regurgitation. Type 1b Tricuspid Atresia with normally related
great arteries. (Statistics show 5 in 100,000 have TA – very rare)
In January 2006 I had the amazing news of being pregnant with my 4th child and my husband’s first. We were over the moon and so excited, there was nothing better we could be blessed with. I had all the normal experiences such as morning sickness and headaches. In February that year my husband and I had a break away from home to New York, I was 9 weeks pregnant at that time, we in fact had to cut the holiday short as I was very ill, and on returning I suffered with even worse headaches.
During the weeks to follow I felt something was wrong, I kept having dreams about the baby, she was not well. All the time I knew. At the 20 week scan, I sat with Paul and held his hand so tightly, we went into the room and the radiographer took a long time, longer than normal, then excused herself from the room. On returning she came back with a Doctor, Dr Brook. She scanned me again which seemed like it went on forever. From that moment on I felt like I was in a room where they were talking and I couldn’t hear them, I could just see their mouths moving. Our baby had a broken heart.
We were sent back to the anti-natal department and spoke to the nurse in charge who referred us to The Heart Unit, Cardiff. I sat and felt numb I kept hoping that they were wrong. After that, one day seemed to merge into another and we went to see an amazing neonatal cardiologist Dr Uzon, on the 19th May 2006, who scanned me again and sat us down with Wendy Williams the cardiac liaison nurse. He began to tell us what was wrong with our baby.
She had Tricuspid Artesia, with hypoplastic right ventricle, VSD and normally related great arteries. Dr Uzon drew her heart and the three surgeries she would need and how in the future she will have to have a heart transplant. He also said there is no cure for this heart defect. I remember going home; Paul and I looked at each other and cried and cried. Why us, did I do something wrong, I blamed myself so much. We were also asked if we wanted to continue with the pregnancy because if there was anything else wrong they would not offer surgery, such as coronary artery abnormalities or pulmonary venous obstruction. Amelia has only half a heart. This was the first time my heart was broken.
After weeks of tears and fears for our baby, I had an amnio test which proved I had no other anomalies thank God, but unfortunately I had developed diabetes and was put on insulin which could have affected the baby. She was safe inside me and I wanted her to stay put. At week 38 I was admitted to St Michael’s Hospital, Bristol and our experience in theatre (a planned section) was surreal, they played music & danced.
Amelia was delivered at 5.56pm on the 29th September 2006, I was able to see her briefly and she was beautiful, she weighed 7lb 4oz. I longed to hold her then but she was taken away to NICU. I spent 4 hours having a blood transfusion before I was able to see our darling Amelia.
The love I felt for that little girl was immense, I would do whatever I could to protect her and keep her from harm. She was perfect in my eyes, Amelia Isabel Hine. So quiet and blue in colour, so special from that day on.
Amelia spent a week in NICU, after scanning and x-rays they found that she also had a large ASD. We came home briefly, back and forth to Cardiff heart unit and Bristol, where we were admitted on 20th October 2006 to Ward 32 for Amelia’s stage 1 surgery, the PA banding. We had no idea what was happening to us, we had never heard of Congenital Heart Disease before so while we were at the hospital we had Amelia christened in her room by the Chaplain. A bittersweet feeling for Paul and me, we said if Amelia was well enough we would have her blessed at our church, so all our children and closest friends could share that moment. We spent the weekend in hospital only to be told that Amelia had a virus and they could not operate, so we came back home with medication furosemide and spironolactone.
On the 14th November we had to attend the radiology department to have her kidneys, bladder, abdomen, brain, liver, pancreas, spleen, gall bladder and tubes that drain from the kidneys x-rayed and scanned. We had confirmation on the 27th November all is normal. What a relief so much to go through for such a brave baby girl.
We went to BCH again the 19th November 2006, we were told she did not need the PA banding as the ASD was large enough to keep the blood flow going. She would go straight to Stage 2, the Bi-directional Glenn Shunt. Also our Dr, Dr Onuzo at Cardiff wrote to us telling us Amelia now has mild right ventricular outflow tract obstruction, her left ventricle was dilated and volume loading. She was also now in mild heart failure. I felt like we were losing everything, Amelia was getting worse and worse. I was not going to let my baby girl slip away from us. BREATHE…………….
We were seen again in Cardiff and Dr Onuzo was concerned Amelia was deteriorating quicker than they would have expected, so on the 5th December we had a letter admitting Amelia to Cardiff for her Cardiac catheterization on 24th January 2007. We attended 3 more appointments at Cardiff Heart Unit before the end of the year. She also had another complication mild pulmonary stenosis.
We had Christmas at home which was wonderful, the feeling of having my family all together. In January we had Amelia blessed in our local church, what a wonderful day. The week after we were called into the Cardiff Heart Unit for Amelia to have a cardiac catheter (a camera from her groin through a vein to look into her heart). We went in again so very nervous and felt helpless, she was sedated and taken to theatre. She came back to us about an hour later seemingly unaware of what had happened, we stayed a couple of nights with Amelia, the entry point which they had taken the camera through (a main artery) bled like a shot of blood to the ceiling and her saturation levels dropped so they needed to keep Amelia in to just be safe as there is a risk of blood clots.
After having this we saw our cardiologist Dr Onuzo, who tells us Amelia now had, as well as everything else, mild narrowing in the origin of the left pulmonary artery. We could go home but we needed to be vigilant with Amelia as her mild heart failure could become full heart failure, at that point nothing more could be done.
So we came home and for the next two months and watched as our daughter was growing but her heart was failing. I lived in fear every second of every day, we had weekly checkups at Cardiff, and finally with a huge sigh of relief were called into Bristol, thanks to Dr Wilson, who felt Amelia was an emergency case due to her saturation levels dropping into the low 70’s and heart failure. During the months up to then Amelia smiled and slept lots and was such a happy little baby, feeding well. My baby with the broken heart.
On April 15th 2007 we were once again admitted to Bristol Children’s Hospital for the Bi-directional Glenn shunt. We spent the night in ward 32, we had a room of our own, I slept in the fold down bed and Paul slept in the chair. The next morning was one we weren’t looking forward to, the reality of it all hit home so suddenly. Amelia was given some medicine to make her drowsy; she would put up less of a fight when given gas. We were visited by the anesthetist and the surgeon to sign the consent forms. Our time was called and off we headed to theatre, it was the longest walk ever. Paul held Amelia very gently but tightly as she was put to sleep with gas, I looked on in tears feeling so helpless. It felt like our little angel was being taken away from us. We were told to keep ourselves occupied so we headed into town to try and take our mind off things. Amelia was now having Open heart bypass surgery. The surgery was expected to take five hours, we were called after four to say she had been in intensive care the last hour. We headed back to the PICU not knowing what to expect.
On entering PICU, we saw several nurses around Amelia, they apologized as they could not sedate Amelia she was throwing herself up and down the bed in pain. She should be paralyzed so her heart could recover from surgery. Not our Amelia, I just watched and wanted to hold her so desperately but she had so many wires and tubes it was not an option so I just held her hand and stroked her head to try and calm her. They kept reviewing her and increasing her medications of morphine, midazolam and a third one and even during the evening she did not settle, they were getting very concerned at this point, her heart needed to rest.
The next day all of a sudden Amelia was still and finally sedated on an adult dose and slept for the whole of the next day(day2). On day three we were back down to Ward 32, as they felt Amelia was ready. After being on the ward for several hours Amelia took a turn for the worse, the alarms all went off, the nurses pulled the curtains around us and the registrars rushed over to Amelia’s bed. Her saturations levels dropped in the 40’s and heart rate was over 200. (A normal child would be 96-100 saturation and 60 heart rate) We were losing our baby girl, I looked on and could not move, the nurse sat me down while they dealt with Amelia. They managed to get her heart rate lower in the 100’s and saturation’s in the 70’s, they don’t know what caused this but she had to be put back under an oxygen box to help her breath. Day 4, Amelia was out of the oxygen box and hated being picked up or moved due to the pain of her chest scars. This day they removed her pacing wires and draining tubes, she was not eating much but drinking milk. It was not long after that they felt Amelia would recover better at home after a week on ward 32. She was on aspirin to thin her blood now.
So we brought our brave little heart warrior home and she was able to be with her brother and sisters again which made her smile. We had regular check up’s at the heart unit in Cardiff who kept a close eye on her. Amelia even taught herself to not sneeze as it hurt her chest too much. We could not pick Amelia up under her arms for months afterwards we would have to scoop her up in our arms.
As the months passed and our check up went to 6 months, (we always went back in between as Amelia was not well on occasions) Every checkup there was another part of her heart which was wrong, her axis and other parts of her ECG were getting worse. Still too soon for her next surgery. She had to be put on iron tablets Styron December 2008 for six months. We spent in all the past 3 years always keeping a close eye on Amelia and making lots of family memories with her until six months ago, Amelia’s heart was unable to let her have the life of a 3 ½ year old and with her saturation levels dropping in-between 58-78 her heart rate would reach 170. We had the awful wait to be called in by the surgeons, and this time it seemed more difficult to accept.
On the 31 March 2010 we were sent a letter admitting Amelia to Bristol, on the 6th June 2010 for her to have a diagnostic cardiac catheter(a fine camera that goes into her heart to measure pressure) and TOE, transoesphageal echocardiogram (scan probe down her throat to see the back of her heart). We arrived on Sunday and settled Amelia into her bed and surroundings. We met with the anesthetists and Dr Martin the surgeon who would be performing the catheter. We mentioned to the anesthetists that Amelia didn’t have good veins in her hands and gas would be preferred. So we slept in the ward Amelia in her bed and I beside her Paul slept on another bed in the room. We were second case of the day. On waking Amelia could not have any food and her turn was not until 12 midday. We did a lot of playing in the playroom and playing games, the nurses came and put the cream on the back of her hands, all this attention Amelia liked so far, mummy and daddy to herself (as I had another baby girl who at this time was 14mths old) so the extra attention Amelia loved. Paul carried her up to theatre and he sat down with Amelia on his lap, what was to follow was heart breaking.
They tried 3 times to get a line into Amelia’s hand without success; Amelia screamed and begged for them not to hurt her again. I was in tears seeing my precious little heart hero in so much pain. They finally decided just to put the gas mask over her face, at this point she was swollen facially and very purple. Within seconds she was asleep. We were taken to a room outside and again we both broke down in tears.
We were called by the nurse 2 ½ hours later that Amelia was in recovery and only one of us could collect her. Paul went and he said he could here Amelia crying as he got to the door. She was calling for us both. When she came back to the bed she could not swallow and was panicking so was very distressed and sore.
She was going to stay another night but the nurses again thought she would be better off at home, and they were short of beds. Amelia’s sisters and brother came into see her just as we were getting ready to come home but it was the right thing as she smiled at Jonathan and I knew then she would be ok.
We spent weeks chasing up secretaries and surgeons as Amelia didn’t look well and her saturation levels were dropping once again, all memories of 3 year ago came flooding back to us. We have recently been back to the Heart unit in Cardiff as Amelia has developed headaches and vomiting, which is caused from the pressure from her left ventricle, through the Glenn shunt into her neck causing her headaches. Again Dr Wilson intervened with Bristol and Amelia is now having her fontan (this operation joins the inferior vena cava to the pulmonary arteries) on the 18th August 2010.
Paul and I both want to close our eyes for this next operation to be over and Amelia is home recovering. Every morning I wake, I feel sick and tearful, but have my 5 children to be strong for so I start the day, thank God for Amelia.