Amelia's story - part 2 'The Fontan'

Amelia’s journey - Fontan

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.

The 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.
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.

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.

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. We went home with our brave heart hero Amelia.

So it's been a while but I finally think I'm ready to post the most precious story to little sister Amelia Belle Hine! Part 1 of her journey

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.

Yvonne Richardson's story about her son Chris...

Chris was born on the 1 November 1984 he was my second and last child. Chris was sent home from the hospital a healthy baby - so we were told . He never woke for his feeds and slept all the time, we tried our best to force the milk into him but he just slept through are sucked a couple of times and fell straight back to sleep bless him.
On the 10th day my midwife said I will get your GP to come and check him over, sure its nothing to worry about . At 5 pm that night our GP came out and then phoned the freeman hospital , that was when our lives would change forever . Chris was giving an echo , we were then took into a room and the cardio docs broke the worst news ever that our baby was dying.
They put him on a drug to keep his pda open the only thing that was keeping him alive, in a normal baby it usually closes of about 10 days old here we were with our 10 day old baby ..That night he had 2 cardiac arrests a side affect of the drug , but thanks to the skills of the staff at the freeman that night they saved my baby . Chris went to theatre 2 days later for an op to put a shunt in to keep him alive we were told that this was the only op that would be available because there was nothing else they could do for him . We were told that they could not tell us how long he would live because not that many kids do well living with a single pump . I could tell you a thousand stories of how hard we struggled with tube feeding and fast heart rates many other ops because of various other pr obs over the years , but at the end of the day chris defied all the odds and lived . Chris spent all of his life in a wheelchair from coming out of a child's buggy he never had the strength to walk . Chris began to really go down hill in his teens always so tired due to the pressures in his lungs caused by the huge shunt put in his heart at 10 days old ,it saved his life but at the same time caused the demise of his lungs . The worst times were his collapses when we thought we had lost him his heart just did not know how to beat properly any more it would go from about 80 beats per min to 280 beat in a second just swiping him of his feet as he tried to get out of bed for help .
The worst time we just heard a huge bang at 5 in the morning ran in his room and he was on the floor eyes wide open white as a sheet this must be it i thought running for the oxygen and slapping it on his face hands shaking and thinking that i was going to collapse myself , but after a few Min's he came around the bang had not only put his heart back into a normal rhythm but had also severely winded him hence he could not breath or move . from that day we started the process of transplant having put of so long now was time .. Chris, his dad and myself spent the five days together at the freeman for his assessment to see if he would make it on to the list . On the last day all the transplant team called us into a room to give chris and us their decision . they told us as from that min chris was on the list for a heart and double lung transplant , we did not know weather to laugh or cry such a surreal day . 15 th April 2007 was a new saga in Chris's life now we were waiting , waiting for someone else to die- omg how were we going to get through this without falling apart .................. On The 26 July 2007 i received a call from the transplant coordinator they may have a suitable donor for chris could we be there by 10pm it was now 8.30 pm . Chris, his brother and dad were sitting watching Newcastle play on sky TV , they had heard my conversation on the phone , chris just sat there and looked lost we never thought this might be a false call don't ask us how we just knew that tonight this was going to happen .The call came on the 26th , but it went until the early hours before we were told it was a go, 27 -7-07 we took our boy to theatre only one person was allowed to go in the pre med room with chris we asked who he wanted he looked into my eyes and said you mam .
Chris said his goodbyes to his dad and brother and sister-in-law as they pushed him through on his bed he put his thumbs up to them and said bring this on it has to be done . I kissed and hugged him and did not want to ever let go we had fought for 22 years to keep him and i felt i might never see him again . I walked back with Kristy the transplant co , who i can only thank for putting up with a mother who was totally not there i can not remember rejoining my family the next 9 hours just passed in a haze until my mobile went saying chris was coming out of theatre he was alive that's all i could think . I wont go on about his recovery to much you can imagine what he looked like he had just had his heart and lungs took out and replaced . Will just say he did amazing he came of life support after just 12 hours fighting the ventilator . the next day we walked in and looked at chris he was amazing he was pink, he was so drugged up cant remember the 5 days in ICU but he looked great .he spent the next 3 weeks on the transplant ward had a few hiccups and a bout of rejection which was handled very swiftly . He came home at the start of week 4 wow . Chris continues to do well has had a few hiccups nothing major one more rejection episode which was dealt with again . 3 and a half years post transplant and we all are so so grateful to Chris's donor and family . CHRIS is walking and breathing and is living his life , we were told he might not live past one then his teens but here we are today 26 years on . i wanted to share Chris story to those of you who are on this journey now, there can be a future even if you are given the worst prognosis like us .xxxxx

Thank you so much Yvonne...Chris is an inspiration to us all <3

Michelle Leigh Padilla-Hanna's story...

Written in the words of Michelle Leigh Padilla-Hanna...

Hi my name is Michelle Leigh Padilla-Hanna.
I was born in Tuscan Arizona, after only living there for two months my mom and dad decided to come California were they have, at the time, best doctors to deal with a little baby who had lack of oxygen that went to the brain and that point I turned blue. I almost died a couple times. I had my first heart surgery when I was 2 at St. Vicente hospital, my second one when I was 5 years also at St. Vicente hospital,my third heart surgery when I was 13 at St. Vicente hospital.
I had my mouth and ear surgery at Torrance Memorial hospital.

When I was growing up I was in special program through out elementary, Jr High School, High School. I had my seizure when I was 18 i dont remember much because I was out for 2 weeks. Thank god my mom was there. I just recently graduated from college. The school that I went to is Harbor College it is a community college. I have had jobs but was not able to keep any of them because I cant pass a test to keep my job also I was getting sick with colds. I just had sinus surgery a few months ago. I just recently found out that I have Di George Syndrome. I never knew that I had it neither did my mom or dad. I am the only child. The doctors told me that it is a 50-50 percent chance that my kids could have this syndrome, so my husband and I decided not to have any kids. I will always have more surgeries dont know when though.Thanks so much in helping me and getting the word out. I trying to to get my benefits back SSI but they keep turning me down.

Sincerely yours,
Michelle Leigh Padilla-Hanna

Sairah Grace Casto-Hodge's story...

This story is written in the words of someone with CHD...

My name is Sairah Grace Casto-Hodge. I am 18 years old. I was born with a congenital heart defect called Transposition of the greater arteries & pulmonary stenosis.

My mom said that her pregnancy went fine and that the doctors said I was healthy and a good size. Then on October 19, 1992 at 6:57pm I was born and within 2 hours they told my family I was turning blue and I had something wrong with my heart. They transported me to Long Island Jewish Hospital in New York. There is where the Cardiologist decided I needed open heart surgery. Dr. Joseph Amato was my Pediatric Cardiologic surgeon. My family said he was an amazing Doctor and he was very caring and gave my parents lots of information and so when I was 7 days old I had the open heart surgery called the Switch, which took 10 hours in the operating room. I was given a 50/50 chance and I was not promised tomorrow.

My years growing up I have had a few heart caths, balloons and stints. I had my 2nd open heart surgery in April of 2008. So I did not finish my freshman year. One of the common sides affects of having a heart condition and having open heart surgery is Scoliosis of the spine. They say due to the fact of the heart and also ribs being cracked that it pulls you forward. I had to wear a full time back brace. Then I had to have 2 spinal fusion surgeries in 2009 and now I have 2 rods in my spine and 24 screws. I had the first spine surgery with 2 rods 18 screws in Aug. of 2009, but then due to complications I had to have another spine surgery which left me now with 2 rods and 24 screws and only 2 discs left in my back. This caused me to miss the first half of my junior year. I always seem to get sick easily so I miss a lot of school, but I push to stay on top of things. As far as my heart I still have issues, but I do not let it stand in my way. With medication and my desire to be strong and never give up I enjoy life.

My family is a very important part of my life. My parents are always by my side when I go through all my medical issues. I also have a 10 year old sister, Sierra who calls me her hero, because she says I am a fighter and she is proud to be my sister. Even at school my sister did a report on me. I sometimes feel bad that I take my parents away from her when I am in the hospital, but she understands and we have a close bond. I love her to pieces.

I am a senior at Orange Park High School. My future goal is to be a trauma nurse in a pediatric hospital or sports medicine. I love working with children and I want to be able to help them heal and also I will be able to understand what they are going through. It is hard for children with medical conditions, especially for teenagers. I can give them support in different ways.

My family and I are very actively involved with different organizations in my area. I am a part of Mended Little Hearts, Starlight Children’s Foundation, American Heart Assoc., Dreams Come True and Songs of Love. We are active members because I am a teenager that was born with a congenital heart defect and I am also involved by doing community service for them. I am a singer and I sing a lot for the community by singing the National Anthem for different organizations and I also do concerts. I did my very own concert at the local Fair this year. I even got to be one of the opening singing acts for the country singer “Linda Davis”, who happens to be the mother of Hilary Scott from Lady Antebellum. So I opened for Linda Davis and then had lunch with Linda Davis and Hilary Scott. It was amazing.

I also just found out that the American Heart Association made me the face of the First Coast for the “Go Red” the fight against heart disease for 2011. I will have to speak at events, be in a fashion show and go for a photo shoot so I can be on the advertisement for Go Red. I am so honored and excited because I want EVERYONE in the world to under stand how important the fight for heart disease is.

Another way I have tried to help others is for my Sweet 16 and my 18th Birthday they were Birthday Parties that were also a fundraiser for Dreams Come True and instead of people bringing gifts they donated to Dreams Come True in my honor and 2 little girls that were also born with CHD benefited from my parties. It made me feel so good to see the smiles on these little girls when they had their Dreams.

I love to hang out with my family and friends. I enjoy going shopping, watching movies and I love to sing. Did I mention I love to shop???

My advice to all CHD children and their families is to be a kid. Unless the Doctors say you can NOT do something, then do NOT sit back and waste life away. Enjoy it and live it to the fullest. I have done many sports. I have figured skated for 5 years and I am a singer. I have future plans to be in the medical field. This is how my parents raised me. They told me “I was never promised tomorrow, so live and enjoy life today”. I live outside the bubble.

God Bless you all.

Sairah Grace Casto-Hodge

Amanda Bell's story...

This is Amanda's story about her little girl Tanesha Mae...

Well where can I start? As soon as I hit 30, the clock was ticking and I wanted another child; boy or girl, I wasn’t too bothered just like most parents but I thought the baby thing wasn’t to be till I reached 35 and found out I was 7 weeks pregnant. I had the most horrendous morning sickness ever (if you can even call it morning sickness it lasted all day) I also had bleeding at 8 weeks and thought I’d miscarried but no, I knew this baby was strong then; this tiny human hung in there. I went to my 12 week scan and everything was fine, I carried on with everyday life like normal, then came my 20 week scan and my other 2 children came with me and again was told everything looked fine! So I went home and just prepared for my baby girl to arrive - you see, I wanted a girl and everybody in my family said it was a girl and they were right, it was a girl! I was so happy and couldn’t wait; I picked a name straight away and stuck with it. “Her name is Tanesha Mae” I said to all my family. Now my due date came and went with no signs of labour, even had a sweep at 41 weeks and had horrendous pain for the next week and after that my waters finally broke and off I went screaming to hospital. This was the worst labour I had had you see, I also have 2 teenagers and their birth was much easier compared to this. I had forceps because she got stuck and ended up with 32 stitches. Tanesha weighed 9lb 10 oz - ouch!!! Anyway the moment I saw her she was my world and I bonded with her long before I gave birth as I wanted her for so long! I stayed in hospital for 4 days due to amount of blood I had lost and we were both discharged and sent home. She seemed to be an easy baby; sleeping all the time; only waking for 5 minute feeds then going back asleep. This was constant and I began to worry as she also seemed to be breathing too quickly so the next morning I told my midwife about it and she checked her and said it may settle in a day or two so I said ok and carried on as normal. As the days passed all she seemed to do was sleep and feed then after about 2 weeks she started vomiting so again I got the midwife to check her and she decided to weigh her for the next few days to see how she goes on. The beginning of the third week she had lost well over 70g so I was sent to the local hospital to get her checked out. They did all the checks and I was sent home with gaviscon for reflux. I tried this over the weekend then took her back on the Monday because she had lost more weight and her breathing was becoming worse. I waited for hours to see a doctor and when the doctor came out he did all the checks and asked a lot of questions. He then left the room and in came another doctor; at this point I was getting anxious as the same questions were getting asked over and over again. He then turned and said they wanted to get a senior doctor to come see her and talk to me. I waited 5 minutes for the registrar to come, he examined her and turned to me and said has anybody mentioned that she has a heart murmur? I replied with ‘no what does that mean?’ and from this point on, it was the worst time of my life as something inside me was ripped apart. I was taken to the ward while x-rays and ECG’s were carried out on my beautiful baby girl. Blood test after blood tests were done whilst I sat there and cried. After all her tests had done, a nice doctor came to see me and told me the x-rays and ECG weren’t normal and said they wanted to do a lumbar puncture but I told them no so they went to get a different doctor to come and see me. After an hour or so a nice curly haired doctor came to see me and said they don’t need to do a lumbar test as he had looked at then x-rays and was sure it was her heart that was the problem. He was sending me to Leeds heart unit for her to have an echo; I was so scared for my baby. The next morning we packed up early and waited for an ambulance to transfer us to LGI ward 10 heart unit; it arrived 10 minutes later and off we went. It took a good hour to get there and when we arrived I was sent into this room on ward 10 with a big machine that takes an echo of your baby’s heart and vessels. I sat there holding her head still while the scan was done. I couldn’t tell you the fear I felt inside as I watched the doctor scanning over my baby’s chest. After it was done she turned to me and said there is something wrong with your baby’s heart, I will draw it to explain to you and as she started drawing the diagram, I knew it was serious; she turned to me and said your baby has a complete Atrioventricular Septal Defect and without surgery she will die. My world right there and then just fell before me. I picked up my baby and held her so close and cried and at that point I gave my daughter to the nurse that came with me and I ran to the toilet to be sick. When I returned they had put my daughter on the oxygen and put an NG feeding tube in. They said her ‘sats’ were low and she needed help. They told me I might have to stay for 4 days depending on how she did on meds. The next morning the doctors did their rounds and they got to Tanesha’s bed and said ‘late diagnosis of complete AVSD’ and talked through her short history as I eagerly listened and as the days went by her breathing got worse she started vomiting at every feed even though she was tube fed and she was sweating and clammy; it was obvious she needed the operation soon. That’s what the surgeon confirmed and that it was to be done on the following Monday. Monday morning came and it was the worst day of my life; I was taken down to the theatre until she was put to sleep and with tears rolling down my face I gave her a kiss and then I went with my sister into town; looking at my phone every 5 minutes trying to pass time on as they told me it would be an 8 hours operation when in fact it took over 9 hours to get her stable so I could go see her in ICU, I was led into a room to wait for the surgeon to come talk to me. I sat there crying with my sister when in walked the surgeon, he looked at me and said "things went well but it was very difficult; her valve was paper thin and transparent but I succeeded to separate it and I patched the holes but the next 48 hrs are critical". When I saw her it wasn’t what I expected; she was swollen and her chest was left open because her heart was very enlarged. There were so many wires coming from her tiny body and all I felt at that time was guilt; I blamed myself, always asking why me? But nobody knows why - I wanted to be there instead of her. It was an eventful week watching the doctors and nurses giving meds to keep her blood pressure stable and cloral to keep her sedated. After a week they closed her chest and started to wake her up and slowly take her off the ventilator and I cannot tell you how happy I was to hear her cry (well more like a little lamb noise than a cry) but it was brilliant to hear and as the days passed they prepared her to go back to HDU upstairs which was a step closer to home, but when she got to HDU, the morning after she didn’t look right so I got a nurse to get the doctor. They started her on antibiotics for an infection but at this time they didn’t know where it was. They sent off bloods and we had to wait for the results. After a day she was getting no better so the doctor came and said she got a nasty infection of the blood caused by the arterial line in her neck so it was removed and she was put on very strong antibiotics; she also was struggling to eat as her stomach wasn’t digesting her food and it became very distended so I was told that it was suspected nec of the bowel and she had to be nil by mouth for 7 to 10 days and I just cried most of that time because I was breast feeding and every time I held her she wanted to feed and I couldn’t; it was so upsetting. She was also on a pacemaker for 2 weeks as her heart wasn’t beating at the correct speed on its own so to give it time to heal where the patches are they kept her on a external pacemaker. I was so scared everyday as the doctor came and switched it off to see if there was any change now. I sat by her cot morning and night willing and praying to god that she will pull through, it affected me really badly and I cried all the time. I didn’t eat or sleep until one of the nurses said if you don’t eat or sleep you will get ill then they won’t let you sit with your baby. So I started to eat and sleep but the crying didn’t stop, I couldn’t help it. Then one day Tanesha looked at me and gave me her first smile; I was sooo happy that day, not a tear shed because I knew there was no brain damage and that she will be going home! After 2 weeks her pacing wires were removed and we were taken out of HDU and put on the ward. We were transferred back to our local hospital for feeding issues as she still was been fed by NG tube through her nose but after a week in our local hospital without the tube, we made it home and she is my little warrior princess who is doing very well at the moment. She has a tiny VSD and Mitral Valve regurgitation and we don’t know what the future holds or if she will need more surgery but we live 1 day at a time and hope it’s far off. I would give anything I had to the surgeon and staff that took care of her without them she wouldn’t be here so I thank them all from the bottom of my heart xxx

Update 28/02/2010 Tanesha’s scan went well; they took off all meds to see how she goes; her blood pressure a little high for her but they want to see her again in 2 months and see how it is then her valves still leaky but not as much as before.

Update 27/5/10 Mitral Valve still leaking and a small VSD but no worse than before so see him again in November in 6 months

Update 20/08/10 Tanesha started making grunting noises while moving around so I checked her sats on my pulseoximeter and her o2 was 98 and heart rate was 79 so I took her to A&E by ambulance. They observed her at my local hospital then transferred us to LGI where she was given ECG’s, an x-ray and an echo it was then confirmed that she was in complete heart block and needed a permanent pacemaker. She had her surgery on Wednesday 23 August 10 and all went well she stayed in ICU over night then back on HDU the next morning.
Her recovery was brilliant and we were able to go home on Friday and she is going from strength to strength and the pain seems to be easing too. So thank you again ward 10 and Dr Waressena for making her life better.

Written by Amanda Bell, mummy to Tanesha Mae Ives 31/05/09 Complete AVSD

Emma Grimshaw's Story...

This is Emma Grimshaw's story about her son Finlee...

When I found out I was pregnant it was a bit of a shock. I wasn’t in a good place at the time, but I thought I couldn't end it so I started to turn my life round. The pregnancy went well and the 1st scan was great. I could see this tiny little baby inside me growing away, I was very lucky as I didn't show that much when I was carrying Finlee and the midwife said I had good tummy muscles. The 2nd scan came and there was two of the Drs sat in the room and said everything was going great and they could see four chambers of the heart and that was that. They did all the other stuff they normally do in the scans and they then said they were happy and could I go. The last few weeks seem to drag and then the time had come - it all started and I was in labour for 4 hours (seemed like a life time) and then Finlee was here and I met my little boy for the 1st time but he was blue and he didn't hold his temp very well but they didn't seem to worried. He didn’t even feed from me for over 12 hours! All they did was tell me to put more clothes on him and put him in an incubator. After a while his temp picked up and they brought him out but his temp dropped again, so they just put him back in - that happened a few more times and then he did hold his temp but was still blue. After 2 days in hospital we were allowed home, which was great. My mum also booked time off work to help me out - being a new mum that couldn't even change a nappy before I had Finlee! A few days passed and he was still blue but his body was warm. I had a health visitor come round to do me a ‘show bath’ and I asked her why my son was so blue and all she told me was that he was fine in his body temp so not to worry. Then it was day 9 and in the morning Finlee let out this cry which was not right; I told my mum and she said see how it goes but he was then sick as well (he was not a sicky baby) I was just worried and after dinner that night I was at my mum and dad’s and called the out of hours doctors and they said if he was blue then call 999 and if you don’t do that then make your way to Torbay hospital but as we were told he was fine we did not call 999. When we got there the doc looked at him and said I want someone else to see him, that was it - they had him in there arms running through A&E to resus and there were 6 to 8 doctors and nurses round him and I was just stood there in shock. What the hell was going on with my son? At this time my dad was in the waiting room with all mine and mums stuff and the nurses started talking to me but I couldn't take it in - all I heard was his heart, I was in shock I left mum with Finlee and the doctors and walked to my dad. I was in tears and couldn't talk, my dad was saying what is going on and the A&E waiting room was full and all I could say was it is his heart, dad left me and went to find my mum and Finlee. We were put in to a side room and a doctor came after a few hours to say what was going on but I still couldn’t take it in, he was sent to ICU over night and was taking by blue light to Bristol Children’s hospital the next day. I didn't sleep that night just sat next to him in ICU, my mum and dad picked me up the next day and we had to meet Finlee at Bristol as I was not allowed to go with him. When we got there we were sat in a side ward with Finlee and a doctor came though and told us that Finlee had pulmonary atresia with vsd and he was sick and that if we had left it another 24 hours he would not be here. And that was the start of our CHD journey...
AT 13 days old, Finlee went to have his right BT shunt. We took him down at about 12pm and we didn't see him till about 7.30 pm as they got one number wrong in my phone number. He had a short episode of bradycardia with a drop in bloody pressure, immediately after the op CPR was carried out for 2-3 min, he also needed a blood transfusion. He was in PICU for 5 days when he should have only been there for 2 or 3 days, when up on the ward Finlee was not able to put any weight on and dropped some days but after 2 weeks he started to put on and we were allowed home. I went back to my mum and dad’s for a few months so they could help me out. In March he was started to get really sleepy again and going blue so we went back down to Torbay and his saturation levels had dropped from 80s to 50 and 60s again so he was taken back to Bristol, but because he had a slight cold he could not go on the heart-lung machine and he needed another shunt - this time on the left. After the op he was a lot better and only in PICU for 3 days, but it nearly didn’t last as we went back up on to the ward the 1st night was fine but the 2nd he was being sick all the time I asked if this was right but they didn’t seem too bothered but by the morning he was really bad and was put in to his own room. Then it got really bad and his temp hit 40s and was not keeping anything down. I had new doctors in and even the team from PICU in and out the room for 24 hours, he had got rota virus and almost ended up back down in PICU as he was that ill and only being 48hrs after coming back up from there. He settled after 5-6 days on supportive therapy, again with this his heart rate went up to 196. Since he has been home he has been fine but is now due for his OHS - he was due this on the 20th Sep but he got taken ill and spent a few days in Torbay hospital, but we now have a new date for 8th Nov and so far he has been well.

Thank you so much for sharing your special story!!