Interrupted Aortic Arch type B, Large VSD, Sub Valvar Aortic Stenosis, Aortic Valvar Stenosis, Dysplastic Pulmonary Valve.
Baby Lagan was diagnosed with Complex Heart Defects in September 2010 during an intrauterine Fetal Medicine Scan at 22 weeks.
We were taken to a small counselling room and given our options.
Continue with the pregnancy and hope for a repair,
Continue with the pregnancy and provide palliative care
Terminate the pregnancy.
Lagan was so active we agreed to give her the chance to prove her strength. On the 11th January 2011, Lagan was born in St Mary’s Hospital, Manchester, a brilliant 7lb 5oz. She was whisked away to NICU where she was looked after incredibly well. When we went to visit her we could not believe there was anything wrong she was perfect. But what we didn’t know was along with the complex defects we had been told about, Lagan also had an interrupted aorta type B. We were also told due to the interrupted arch that Lagan had a high chance of having Di-George Syndrome, they said she also presented soft features of this syndrome.
On the 12th January we were told they were going to transfer her by blue light to Alder Hey Children’s Hospital in Liverpool for surgical review. That evening Lagan left the comfort of Saint Mary’s and embarked on her 30 minute journey to Alder Hey Children’s Hospital, Liverpool. I returned back to my room on Maternity and sobbed while we waited desperately to hear that Lagan had arrive safely in Alder Hey and had been transferred on to K2 Ward.
After a long 24 hours Lagan, her dad and I were reunited at 7pm on Thursday 13th January. A long week passed and Lagan began to deteriorate throwing very odd blood gas results and rapid breathing. After a week of nil by mouth Lagan was just 6lb 2oz on the morning of her surgery.
Nelson Alfonso informed us that he would be performing the Modified Sano Norwood Stage 1 repair due to the stenosis in the aortic valve and area just below the aortic valve. He would also use a Gortex patch to repair the aortic artery. We were told that this was a risky procedure and due to Lagan’s frail state this posed even more risks. The estimated time the procedure was 6 – 10 hours and would take 2 hours to prepare Lagan for surgery in the anaesthetists area. We arrived on the ward at 7am after spending a restless night in McDonald House and sat with Lagan hoping these were not our last hours. The nurses began to prepare Lagan for her surgery, it was all too much for me and I was exhausted, I told Barry I could no longer cope and left Lagan in Barry’s arms. I returned back to Mac House and tried to sleep.
Barry returned to Mac house at 9am and immediately broke down. Were we going to get our beautiful little girl back? We could only hope.
My dad came over to Mac House to try to keep us comfort, he bought us lunch at a pub local to the hospital, I don’t think Barry or I had eaten for days…. surprisingly we ate what we ordered despite the emotional trauma we were going through. Because we kept our selves occupied the time didn’t drag too much. At 3pm my dad said he would drive home back to Bolton. Typically just 30 minutes after my dad left us we received our first call from anaesthetics to confirm Lagan had successfully come through her operation and had been taken to PICU. Just 3 minutes after receiving that call PICU rang to confirm she had arrived in PICU and we could go and see her.
We rush to PICU and saw Lagan immediately. I was prepared for the worst so just seeing her was a relief. She still had her chest open although this had been covered and the nurses had written a little note on a napkin to cover the wound saying “My Chest is Open” There were lots of noises and wires and pumps and the ventilator dominated her tiny face and had three huge drains hung from the bed. It was a lot to take in but we understood all of this was all helping our little girl.
After 7 days Lagan finally came off the ventilator, this was probably the most stressful time as we had to will her to take over the job of breathing by herself again.
The same day Lagan was transferred back to K2 and started her long road to recovery. It became evident quite quickly Lagan couldn’t cry. Despite constantly asking why she could not cry we were told that it was just bruising from the vent and this would improve shortly, however it didn’t. One day one of the ST Doctors who had just returned from holiday, raised the alarm that she may have had vocal chord damage due to the operation. Something that had not been discussed with us as a possibility. Immediately, Lagan was placed as nil by mouth again and a Nasal Gastric (NG) tube placed to minimise any chance of aspirating should the vocal chord indeed be damaged.
After investigation it was confirmed, Lagan had left vocal chord damage. We were unsure if this would be permanent. But for now she could only be fed via the tube. Up to this point Lagan had been breast feeding. I was devastated.
Lagan’s voice slowly grew and we were moved on to thickened fluid to allow her to feed orally. During this time of adjustment we were told Lagan did not have Di-Georges. Relief passed over us.
Eventually after 6 more weeks we were discharged.
We arrived home early afternoon and waited for everyone to return home from school and work. Everyone was so happy. This was to be short lived, just three days at home Lagan woke up in the middle of the night with breathing difficulties. We immediately rang 999. She was taken to Royal Bolton Hospital and treated for RSV. Tests confirmed she did not have RSV but everyone played it safe. After 3 more days there we were discharged and went to Alder Hey to attend the Cardiac / Feeding Clinic, where surprisingly Lagan was readmitted. I was completely unprepared and had no clothes, milk etc. Mark Dalzell the Gastroenterologist decided Lagan required a PEG. She was failing to thrive. The operation was done the next day and only took 20 minutes. Amazingly after that Lagan began to pile on the weight. I hated using it but knew it was the best thing for her. A couple of days later Lagan was discharged. We had to return to clinic in 2 weeks.
Two weeks passed and Lagan’s reflux began to get worse and worse. At home I panicked, I had no one who could advise me. Was this normal? Were we feeding her too much? Etc? No one could help me. We return to clinic and the Gastro team readmitted us to observe the reflux. They changed her medications and eventually after a fight they discharged her. Alder Hey only wanted the very best for Lagan.
Life got more and more difficult. The reflux took over our feeding regime. I hated being out of the house too long and therefore found myself house bound. Lagan was fed every 3 hours and each feed would take an hour and then I would have to sit quietly with her for an hour leaving one hour free to prepare for the next feed. Was this my life now? Everything had been turned upside down!
Unknown to me, Lagan was supposed to have a paediatric community nurse visit her weekly. The only time we received a visit was after each discharge from hospital. I now feel extremely let down by the community team as they could have alerted me to things to come.
Easter approached, and on the Friday Lagan just didn’t seem herself. She had not long since had her 1st injection and I just thought she was under the weather from this. On the Saturday she was very clingy and cried quite a lot. I just couldn’t work out what was wrong and put her on long feeds and gave her lots of cuddles. Easter Sunday arrived and so did all the family. She still seemed out of sorts but not quite as bad. Lagan received lots of nice clothes instead of chocolate and a lovely day was had by all.
On Easter Monday we had another visit from friends and again Lagan was still a little off! She slept quite well and took her milk via the PEG only. At midnight it was time for her final feed. We were all shattered. I lay in bed with her on my chest and she took her milk without a problem and slept through it all (Something I wonder about now. Had this been a serious sign I missed?). I placed her in bed and she seemed so content. Exactly 1 hour from the finish of the feed at 2.30am Lagan woke up with the most terrifying scream. She was inconsolable. We thought she had nappy rash? No, Colic? No. And quite quickly she started showing signs of respiratory distress. I took Lagan downstairs and rang 999. They arrived in 8 minutes and rushed us towards the Royal Bolton Hospital. We arrived at the hospital at about 3.10am. Unfortunately, whilst on the ambulance Lagan crashed and the paramedic commenced CPR. Something I had always dreaded.
On arrival at the hospital everything became a blur. Barry was still at home and I was at the hospital. I knew she was dying and felt hopeless….
After 30 long minutes the medical team worked on Lagan solidly desperately hoping something would work and sadly at 3.41am on the 26th April 2011 Lagan was pronounced dead. She had gained her wings in the most distressing way.
When I look back now I always ask, why didn’t I take her earlier, why didn’t I have help at home. Was she presenting signs of heart failure? Was I labelled as a paranoid parent and due to all the funny looks and lack of support became frightened of taking her to our local hospital? All questions that just will never be answered.
No parent deserves to go through that and therefore Lagan’s Foundation was born.
I vow to bring to parents the information they ask for, the support at home they need and the sensitivity and passion every heart parent has.
Our story is tough but true. I hope through Lagan’s Foundation, Lagan will be remember and many more people will be helped.
Angel Lagan’s Mummy
Hypoplastic Left Heart Syndrome. (Half a Heart)
We found out something was wrong with David when I was 38 weeks pregnant. Up to then his heartbeat had sounded fine at all of my check-ups, including the one at 36 weeks, but two weeks later I and my midwife could tell something wasn’t right.
A week of scans and appointments followed, first at University Hospital South Manchester then at St Mary’s. We were told that he had Hypoplastic Left Heart Syndrome and were given the options of terminating the pregnancy, having him and keeping him comfortable but letting him slip away, or having him and getting him treatment which involved open heart surgery. These were the dark times, the days before he was born. The happy anticipation of our second baby was taken away, replaced by dread of what the future held. Having to explain to our eldest, Edward, that he might not get a baby brother after all was dreadful. Luckily I was already following Lagan’s Foundation as its founder, Carren, was a former colleague of mine, so I knew where to go for support and advice. Some of the terms the doctors were using were already familiar to me from the Lagan’s page. Carren was fantastic and gave me hope when I really thought there wasn’t any. The doctors had given us the stats, and of course the stats show that far more babies survive with HLHS than don’t. But you don’t take that in, all you hear is “your baby has an extremely serious heart condition and he might die”.
Carren reassured me that she knew lots of hypoplasts and David would make it. After much soul searching we knew we only had one option: to give David every chance to survive. And he did. From the day he was born, 20 September 2013, David has done everything right. He came out pink and screaming when we had been warned he could be blue and sickly. He recovered incredibly well from his first surgery at Alder Hey, which he underwent at 5 days old. He was able to feed orally so was discharged from hospital at 3 weeks old with no tubes and only aspirin for medication. Having been on expressed breast milk from the start, at 4 weeks old he latched on to me and has been breastfed ever since. Second surgery took place on February 19th, the day before David turned 5 months. He gave his ICU nurse the runaround on day 2 fighting his sedation, but he was home on day 6. We really could not have asked for more from him. Without the support from Lagan’s Foundation we would have found it much harder to make the decision to give David the chances he has had. We did not know what the future held, we were frightened, we had an older child to consider. Lagan’s gave us the confidence to go down the surgical route, knowing we would be supported every step of the way. On behalf of my beautiful boy, I thank them from the bottom of my heart.
Down Syndrome With Complete Atrioventricular Septal Defect
We knew from the 20 week scan that our baby had a heart problem. Alex was born with Down Syndrome and Complete Atrioventricular Septal Defect.
When Alex was born he was well but didn’t feed well and failed to thrive. Then began the whole alternative world of the stress of CHD, severe reflux and all that went with it. Alex is now fed by Gastrostomy Mic-Key Button and is on a blended diet.
I, Alex’s mummy, was constantly depressed. Lagan’s came into our lives in 2013 and continue to help me with managing feeding and care as well as giving me precious time for other things. Not to mention reducing my sense of isolation by giving me something/someone to look forward to every week. Thank you Lagan X
Tetralogy of Fallots
Luke was born with Tetralogy of Fallots, Pulmonary Stenosis and a possibility of Di George Syndrome. My baby boy entered the world on the 8th of march 2011.
It was confirmed he had ToF and Pulmonary Stenosis but he didn’t have Di George Syndrome. So eventually we brought our boy home. Things moved along and Luke got sick so at 6 weeks we went to Alder Hey for his first surgery, after 2 months we got home!
Life was tricky, I was so tired from everything going on with Luke and looking after my daughter. Eventually I received funding for Luke to go to nursery this is where i met Vicky. Vicky had just been through her training with Lagan’s she explained how they help children with heart conditions and feeding difficulties. Luke ticked all the boxes but before I could get in touch with Lagan’s Luke got sick and we had to go back to Alder Hey for more surgery. It was a rocky road but finally he pulled through and eventually we got home. So after more appointments and sleepless nights days and pure stress Vicky approached me again and suggested that I get in touch with Lagan’s. So I did. Carren’s Mother-in-Law came out to meet up to see Luke and to fill out the paper work. She told me how the Foundation came to be set up.I told her all about Luke and how difficult I found things at times. I cried got hugs and reassured that things would get better. Vicky would be my saving grace. She would come every week for a couple of hours and in this time I could sleep, get a shower, do my house work while Luke would be safe played with and looked after. I couldn’t be without my saving grace on a Friday I would be in the nut house by now! Not only do I get fab support from Vicki, who will listen and talk to me as well as play with Luke, the help and support I have received from Carren and team Lagan have been amazing from the face book page to private chats with Carren or a member of the team. I never feel stupid asking the hard questions or advice from them and they are always there when I need them. If asked would I recommend Lagan’s my answer would be absolutely. It doesnt make you a bad parent asking for help it makes you a better one. With Lagan’s you not only get help when you need it you get a friend to share your journey with!
A massive Thank You to you all at Lagan’s Foundation I would be lost without you.
Lots of love Sarah xxx
Tetralogy of Fallot
We were told at our 20 week scan that something wasn’t quite right with our baby’s heart. Almost a week later (that week felt like a decade), it was confirmed by Dr Gladman that our son had Tetralogy of Fallot.
Born 4 days late via emergency c-section, weighing a whopping 10lb 11oz, Benjamin was born. We were told that they didn’t think he would have any problems and surgery would be elective at around 6-9 months of age. Ben started having blue spells at 3 weeks old. After numerous hospital admissions, Ben was transferred to Alder Hey on the 4th December 2013, aged 7 weeks.
They decided because of Ben’s size and the anatomy of his heart, they would do a full Fallots repair. Ben underwent 5 hours of open heart surgery was taken onto Intensive Care Unit. His first night was comfortable and we thought he was through the worst.
Unfortunately, Ben went into cardiac arrest the following morning. After 15 minutes of CPR and being unable to revive him, they decided to put him onto ECMO. Ben remained relatively stable and ECMO and apparently you could see his heart trying to pump and this made the doctors believe he could be trialed off ECMO the next day.
Thankfully they managed to get him off ECMO and he made a slow recovery after encountering various complications along the way e.g. Chylothorax – he had his chest drains in from 10.12.13 to 01.01.14. The drain sites became infected. Eventually he was discharged home on the 7th January 2014……for one night!
We took him to our local hospital because of reduced feeds and increased work of breathing. Ben then had an hypothermic episode and was admitted into High Dependancy Unit with Bronchiolitis. He was discharged 5 days later. He was again home for one night before being admitted again. The re-admissions continued until they decided there was a reason for his constant need for oxygen. Ben was sent to alder hey for a cardiac catheter iPad the end if January which showed that he had a large 7-8mm VSD which would need another OHS. It was decided this would take place at the end mid march.
After numerous trips to our local hospital, with increased work of breathing and inability to feed we were told that Ben was in heart failure and would need to be transferred to Alder Hey as an urgent in patient to have the VSD repaired.
Ben had this op 26.02.14 and unlike last time he made a swift and smooth recovery and was home less than a week later. Thankfully he had had no heart related issues since and is now a healthy,thriving little boy who is full of life and brings us joy even on his grumpiest days.
Emanuel Syndrome, Hypoplastic Right Heart Syndrome and has a tracheostomy
April Eve Karmazyn-Wall was at 38 weeks by emergency c section on 20/11/12 weighing 4 lb 10 oz at the RVI in Newcastle. We found out at 23 weeks that she had pulmonary atresia with small right ventricle. They also advised us to have a test as she was small for dates and they thought it may be genetic. She was taken straight to the Freeman hospital as soon as she was born. We were told she had a lot of other problems, cleft palate, talapies, coanal stenosis, hypoplastic kidneys, anterior anus. She was not passing much stool and was panting heavily. After a few days we received a call in the middle of the night advising she had been rushed to intensive care. We were told she had to have cpr and they thought she had necrotising entrocolitis. This is when the bowel starts dying. She had 4 surgeries to remove most of her large bowel and create an ileostomy. She also had her open heart surgery to place a 3 mm BT shunt. We were told a few times that they only gave her 50/50 chance to survive. Following the surgery we found out April had some Emanuel Syndrome. We had to make a decision to give April a tacheostomy as they were unable to wean ventilation. We were transferred back to RVI PICU in January who had the long hard task of establishing feeds and weaning ventilation. April went into liver failure due to the TPN that was giving her the nutrients she needed while she could not feed. She had several infections that set us back but in may 2013 we could move out of a ward and start planning for home. We had one last trip to the Freeman for a cardiac catheter to open her pulmonary artery. Not long after that we were discharged on 20/7/13. 7 month after she was born.
We have continuing battles with feeding, weight and hospital stays. She gave us another fright in February when they went to do fundoplication, gastrostomy and ileostomy reversal. Another 3 month in hospital with stays between rvi and Freeman as BT shunt blocked causing various complications.
We are finally making progress. We discovered blended diet which has helped us with feeding, heart wise she is stable at the moment. I was recommended to join Lagan’s by another heart mum. I soon after looked into applying for a volunteer as we get little in home respite. Our volunteer was placed with us in June 2014 and I am able to have a few hours every week to use how I like.
Tetralogy of Fallots and Gastrostomy
This is Ted Rourke born 6/07/2012. After a few hours of being born we were told Ted had a heart murmur during the paediatric checks. Being our second child this came as a shock. After a lot of indecisiveness at our local hospital we decided to take him to Alder Hey ourselves. On day 10 of being born he was diagnosed with TOF.
He did well for 4 weeks, then dive bombed! Our local couldn’t stabilise him so we were blue lighted to Alder Hey. Here he stopped feeding entirely & got worse & worse. Finally after a scary moment he was taken to theatre at 8pm on 31st Aug 2012 & had an emergency bypass (BT shunt). This was a long and painful recovery for Ted. We went home with the NG in & I look back now & have no idea how I coped at home with it! It was just awful. So on 14th Oct 2012 he had a PEG fitted. He finally started to recover properly from the shunt and was gaining weight for the first time ever & that awful reflux finally started to subside. He lasted 18 months on the shunt & we had the PEG replaced with a Mic-Key button.
By this time last year he had become incredibly poorly again & it was time for his OHS as the shunt was starting to fail. Teds was so old having his fix due to his capillaries not sitting in the correct place in his heart, so it was too dangerous to operate earlier.
Finally on 21/01/2014 he had his full TOF fix. This time was much less stressful & he was done in 7 days! Since he arrived home he has gone from strength to strength, he has now also started to eat!! In my eyes an actual miracle, his aversion was that bad I never thought he would!!! But the Mic-Key is gone & to anyone passing him he looks a happy, healthy boy!!!
Through out the last 2 years we have had an amazing volunteer, Annastacia. Ted loves her dearly & she was a rock for me. Also Carren & the team have been incredibly supportive & always on the end of a phone with advice & words of encouragement. I could not of survived if it wasn’t for Lagan’s Foundation, thank you! Heart Felt Care xxxxx
Finely was born on 23rd January 2015 weighing 9lbs lOoz. Finley had been out of hospital a week when he started to turn blue whilst feeding. After being rushed to hospital and placed in intensive care Finley was seen by an ENT surgeon who explained Finley had something called Laryngomalcia and needed surgery.
Laryngomalacia is the most common cause of stridor in infancy, in which the soft, immature cartilage of the upper larynx collapses inward during inhalation, causing airway obstruction. Laryngomalacia is one of the most common laryngeal congenital disease in infancy and public education about the signs and symptoms of the disease is lacking.
Finley was taken down to theatre to correct the epiglottis, there were complication which left mum scared of any future operations as she had been told he has Pulmonary Stenosis and an extra membrane on his heart.
Mum was sent home afterwards with no support, Finley continued to be sick, turn blue and was always chesty. After another emergency dash with Finley the Drs discovered Finley has Chronic Lung Disease, he was sent home with a SATS monitor and Oxygen to be used when his 02levels dipped.
As Finley has grown his mobility hasn’t been able to follow, he currently uses a wheelchair and unable to get up the stairs on his own or walk very far, he becomes exhausted due to his heart and lungs having to work so hard.
Mum felt hopeless and exhausted. Lagan’s Foundation were contact by her family and soon a volunteer was placed to help. Mum explains that Julie her volunteer has been a huge support for her, and her eldest son. As well as helping with Finley Julie takes the time to talk and help Finley’s older brother who is 10. She doesn’t know what she would do without the help of Lagan’s Foundation.