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Lisa'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. 

 

 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 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 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. You never know how strong you are until you have no choice. As a family we have Hope, love and faith.

 

 

Amelia’s Fontan 23rd August 2010.

It’s that time again, that very unsure, heartbreaking time. Our letter came to say Amelia was due in for her fontan. After receiving our initial date we were cancelled twice in one week. At first we were angry as we have so much to put into place with four other children. Family took time off work and lost that week to help us out. Looking back it was more time with Amelia and we needed that for our whole family.

 

So Sunday 22nd August and we are on our way to Bristol Children’s Hospital, I have this deep feeling of fear in my heart and both Paul and I again wish we could close our eyes and this all be over for Amelia. We met the anaesthetist and the team who would be looking after Amelia during surgery. Mr Cuputo Amelia’s amazing surgeon came to speak to us about the risks and for us to sign the consent form. Amelia was very upbeat that evening, little did she know what exactly was to come. That was the last time I saw my sweet Amelia’s sparkle. If I could have made one wish that night, I would have done in a heartbeat…….

 

Paul and I stayed with Amelia and were awake early, the nurses came with the sleepy drug which Amelia took and was very very sleepy then at 8.30 they came with the theatre bed for us to take Amelia to theatre. Up to this time we held Amelia so close and didn’t want to let her go. Our brave little heart hero, it was time to say our good-byes. After holding Amelia down for the gas mask she fought and cried and cried even after she was asleep. We kissed her little cheeks and said we will see you later. Leaving Amelia was for both Paul and I so very hard and both of us broke down in tears. We made our way back to the ward to collect our bits. Paul and I walked very slowly to the flat we had rented, which was only a 5 minute walk away. The feeling was even worse than before, pain in our chest, sickness in our stomachs. Any heart parent could relate to this feeling. Our other children apart from baby Phoebe with my mum came to be with us, so we all waited and waited and waited……we were told a 6 hour surgery, 6 hours came and went and by this time we were back at the hospital waiting to be called to see Amelia. 8 hours later we were called into PICU. At 5 o’clock we went into see her.

 

At her bedside the doctor on duty that evening asked if anyone had spoken to us about what happened during surgery. My body went cold and my heart was beating so fast. Amelia was still asleep, they kept her that way so her heart could rest. We had not spoken Mr Cuputo about surgery so Michelle the anaesthetist came to speak with us and asked if we could go to the quiet room with her and a nurse. I was shaking with fear holding tight onto Paul’s hand. During the next 10 minutes all I can remember was Michelle’s mouth moving and a loud white noise surrounding me, the nurse moving a box of tissues to the edge of the table. I didn’t cry, I didn’t want to believe what had happened to our precious daughter. My older daughter’s and my mum were in the family room waiting to hear from us and to see Amelia. It was then when they said what happened I broke down in tears; on that day another part of my heart was broken.

 

We went back in to be with Amelia, stroking her head, kissing her hand I thanked God he let her live by giving back her life during surgery. Amelia was touched by an angel that day. On opening her sternum her heart was torn as it had fused itself to the scar tissue of her previous operation. It bled profusely and at that point she lost all her blood clotting properties, they stitched her heart and carried on with surgery. Again they came across another problem, where the gortex tube was meant to be going they couldn’t see as a canula was blocking the heart and they were sure it had torn in another area. So they made the decision to freeze her body to 18* which in itself very dangerous, so they could see better on how to carry on. Amelia then went into cardiac arrest, she died for 12 minutes; they revived her and dealt with the heart. She was then put back onto the bypass machine for the second time during surgery. They then mentioned there may be a chance of brain damage because of the body freezing but they wouldn’t know until Amelia woke up. They completed the surgery eventually and Amelia was very poorly in PICU.

 

Amelia in HospitalThe first night Amelia was on lots of blood clotting agents such as platelets, cryo, tranexamic acid. Pain relief- milrinone, morphine, midazolam, dopamine. This night Amelia was on the verge of being taken back into theatre as her chest drains were bleeding too much. The doctor in charge called her surgeon and they were all ready to take Amelia back to stop the bleeding, they thought her stitches had not held from the tear in her heart. My heart was in my throat so much during this whole journey and my husband Paul also. I wanted just to hold her and keep her safe. They gave her more tranexamic acid and within the next 5 minutes they worked and the blood flow was lessened, the urgency was gone. For them, for us WHAT JUST HAPPENED!!! We left her at 10.30pm that night and could not sleep, we were back with her at 4.45am. 

 

Amelia in Hospital

On arriving the nurse Laura told us that Amelia decided at 4am to climb onto all fours and tried to get out of bed, in a complete daze. Then went back to sleep. The rest of the day Amelia did not wake and they were getting concerned, we were concerned, the liaison nurse Cathy was concerned. She was still on bypap and all the drains and tubes stayed put. Laura then told us Amelia had the pacing box on at 100% as her heart couldn’t find its rhythm. We stayed with her all day all night just hoping for a glimpse of Amelia. The night staff came on and we asked if they decided to do anything drastic to call us and we would be come to PICU. We stayed as late as we were able and during the night we assumed things stayed un-changed.

 

We had a call at 4am to go to PICU, we walked in and Amelia was awake but looked straight through us, no response at all, she wouldn’t even look at us, she then went in and out of sleep all day. During the night after saying not to do anything without us they decided to take her of bypap and put nasal oxygen tubes and an extra mask as it wasn’t enough for her. Her feeding tube came out also. They also took her off midazolam and dopamine. She was still being paced at 100% no improvement and her saturation levels started to drop into the 70’s. Everything seemed to be condensed into one whole painful day. We watched on as she didn’t make the improvements they were expecting. She was put on heparin, and they were still concerned. Her catheter was taken out on this day and Amelia did not go to toilet for 15 hours from this point. So the catheter went back in and the feeding tube went back in. She had an ultrasound scan on her left lung, they found a build up of fluid around it and the base of her lung had collapsed. We were also told she had pneumonia. No wonder she wasn’t improving. So I think we were on day 4 now in PICU, gentomisin, estatin and other antibiotics were being given to Amelia.

 

This day was when I felt even more helpless, the physiotherapist came to help Amelia’s collapsed lung by thumping her back, she just led there no emotion, no protesting, nothing……..This was also the day Paul could take no more, seeing his little daughter being so brave and having to go back to have another drain put in for her left lung fluid. She made no improvements still, still paced but down to 80% a slight improvement, still on oxygen of 3 litres, nothing taken away apart from some pain relief. This was the longest day, we just wanted to see Amelia and for her to acknowledge us.

 

Amelia in Hospital

When would Amelia start to improve and the treatment lessen? After a good night’s sleep the decision was made to remove her chest drains, even though it seemed her chest was still seeping. Her oxygen was reduced to 1 litre and her pacing machine turned down but left connected. It seemed too good to be true, was this the day she

would make the turn toward the finish line? No. X-rays were carried out three times daily and this detected the next set back. After another ultrasound scan it was verified she had another build up of fluid, but this time on her right lung. So another lung drain in which meant 2 pots on the floor either side of her bed. She looked far from well but we were told we were heading up to ward 32 that evening. On moving Amelia all her wound sites and nose started to bleed, what next??

Amelia was put in a high dependency room, 2 lung drains intact, an IV line still in her groin, a line of heparin into her left hand and still taking oramorph to relieve her pain. We took alternate shifts to sleep with Amelia in her room. Her lung drains were still in but her catheter had been removed, trying to get her on a bed pan in the middle of the night was such hard work, trying to lift her carefully whilst keeping her drains out of the way, the bed always got wet. The following days seemed to merge into one, Amelia was so frail and weak. The IV line from Amelia’s groin was removed as they felt it wasn’t needed any more. The following day they needed to test her blood so we had to hold her firmly on the bed, crying her eyes out while the nurses drew blood from the inside of her arm. They did the same the following day.

 

Amelia was starting to improve, although very slowly. Paul managed to walk her to the toilet one morning with the aid of one of the nurses, carrying her lung drains and her heparin drip on a mobile stand. It was only a 10 yard walk for us but must have felt like 100 yards to Amelia, our brave little warrior did so well after being lead in bed for so long. The return journey was too much for her, the pain was too much to bear, especially with her lung drains pulling on her sides. These were removed on day 9 or 10 I think, nothing was too clear, following a trip to x-ray in a wheelchair. The heparin line from her hand was also taken out. Over the next couple of days we tried walking to the toilet a few more times, it was such a relief not to have to try and put her on the bed pan and so much easier for Amelia with all of the tubes and lines taken out. She was taken off her antibiotics and her nose tube was removed too. The only thing she was taking now was calpol as pain relief, diuretics to help remove any more excess fluid remaining in her body and of course the one thing she can’t do without, warfarin.

 

Amelia in Hospital

The pain and screams that we saw Amelia endure was something we will never forget. On the last day in Ward 32, we were told that maybe Amelia would be better off recovering at home but had to go through a series of tests. Her x-ray came back ok, her heart rhythm, she was put on a 24 hours monitor; she was still grey and barley walking. The echo…well as soon as I saw the Dr’s face I knew we weren’t going home. Amelia had a pocket of fluid around her heart. One Dr said I am afraid you won’t be going home until Monday at least. On that being said Amelia broke down and sobbed and sobbed and wouldn’t stop. This day we went home Dr Allison made the decision to let us go home only to return on the Monday for another echo.