Throughout Heart Month, I’ll be sharing various stories from heart families about their experiences. Today’s post is the final guest post of the month and comes from Gemma who blogs over at Heart Mammi. Gemma is a mum of three children and her youngest daughter Martha Grace was born with an interrupted aortic arch and large ventricular septal defect which was diagnosed at her 20 week scan. Thank you Gemma for sharing Martha’s journey so far.
“I have an 18 month old daughter; she calls me Mammi. She’s not walking yet but she can pull herself up using the furniture and can walk around the sofa and coffee table. Believe me; there were days where I didn’t think I’d ever say a sentence like that.
We first found out our baby had a problem with her heart at our 20 week scan. After a couple of appointments at Liverpool Women’s Hospital we weren’t happy with their prognosis – we were then referred to Great Ormond Street where it was confirmed that our baby had an Interrupted Aortic Arch Type B and a large Malaligned Overriding Ventricular Septal Defect. Plans were put in place for me to be induced at UCLH when I was 38 weeks, she would immediately be started on an IV of prostin to keep her Arterial Duct patent. She would be transferred to GOSH within her first week for life saving open heart surgery. We knew the risks, either she didn’t have the operation and died, she had the operation and died on the operating table or in recovery – or by some miracle she survived.
Martha’s 1st Operation took place on Tuesday 27th August 2013 when she was just 5 days old. I was still physically recovering from childbirth and I was facing losing my child. She was in theatre for just over 6 hours, they managed to repair the interruption to her aortic arch but as her VSD was in such a difficult position they weren’t able to close it so they left her with PA bands, meaning she would need another operation a few months later. It really took its toll on Martha’s little body. During the first few days Martha’s kidneys really struggled with the shock of being on by-pass, they were failing and she was badly overloaded with fluid so she needed Peritoneal Diaslysis for a couple of days. The fluid overload meant the chest closure was delayed – but the PD worked its magic in a couple of days and our little puffy baby looked like her beautiful dainty dolly self. Finally 5 days post-op they closed her chest. There was no stopping Martha then – they removed her chest drains later that day, she was extubated the next day and a few days after that she was moved out of ICU and up to High Dependency.
Exactly two weeks after her open heart surgery we were told they would be kicking us out to our local hospital as Martha had been stable from a cardiac point of view and they couldn’t keep a bed for her just for her feeding issues – but we wanted to be discharged home and not to our local hospital. Our last few days at GOSH were spent being trained so that we could be signed off on NG tube feeding Martha Grace – a skill which came in handy during her next admission.
18 days after major open heart surgery – we brought Martha Grace home to Anglesey. I settled in to a routine of being a Mammi of three. Martha was a dream and within three weeks she was exclusively bottle feeding and didn’t need her NG tube. We had an outpatients appointment at GOSH when she was 7 weeks old where Dr Sullivan told us she should probably have her second operation for PA de-banding and VSD closure within a couple of months. By then it was October and my main fear was of being in hospital over Christmas… looking back now – that was the least of my worries.
The phone call came at the end of October that they had Martha Grace listed for theatre on Thursday 14th November with Dr Victor Tsang. I’ll never forget meeting Dr Tsang on the morning of surgery; he walked around the bed and kept repeating “This is a big operation, on a small baby” when I asked him how long he thought the operation would take – he told me he had cleared his whole day for Martha. He left us by saying “So we’ll call you when we’ve finished and hopefully we’ll have good news”…all of us too scared to ask if he meant “good news – we did everything we needed” or “good news – your baby is still alive”
We left Martha in the anaesthetic room just before 10am, it was 2:30pm before we had a phone call to say it was finished – she’d made it. This is when our nightmare began.
Martha had become unstable at the end of the operation and was being paced by an external pacemaker; at that point she was in complete heart block – they were hopeful she’d return to a sinus rhythm but if she didn’t then she’d need a pacemaker.
While we slept in our bed at the parents’ accommodation that night Martha had become really unstable – it was a total shock to us as she had held her own the first night after her first operation. Three cardiologists spent well over two hours performing an ECHO on Martha that afternoon (which wasn’t easy as her chest was still open) trying to figure out why she was so poorly. She remained in a critical but stable condition all weekend, but by Tuesday they were ready to close her chest. I imagined the same thing as last time would happen again – that she’d start to improve once her chest was closed. Unfortunately things got a lot worse before they got better.
Martha developed pneumothorax which delayed her extubation, then chylothorax so she was fed Monogen. By the time she was well enough to consider extubation again – she was listed for theatre to have her pacemaker fitted. When she came back to ICU after having her pacemaker implanted – she needed several doses of insulin as her blood glucose level sky rocketed from the trauma.
I thought that once the pacemaker was in then we’d be on the home stretch, but Martha had other ideas. She was extubated the next day but after an hour or so I could see she wasn’t happy and I begged our nurse to call the doctor over – pretty soon we were in the middle of a scene from Casualty as everyone crowded around her bed – they thought she couldn’t breathe. But in fact it was worse than that – Martha was in atrial fibrillation. She was prescribed Amiodarone to try to slow her heart down and Heparin to thin her blood so that it wouldn’t clot at the bottom of her ventricle. Her heart was going so fast that the Electrical Physiologist couldn’t even programme her pacemaker to override it. There were a few things they could then try, first on the list was cardioversion where they would try to capture her rhythm and shock her heart back in to sinus rhythm. This of course meant she needed to be fully sedated and ventilated again… I remember asking if they really had to ventilate and sedate her… I’ll never forget Dr Mangat’s reaction “It would be very cruel to do that, we’re sending 20j of electricity in to her body” – I had no idea what cardioversion meant.
We were blessed and the cardioversion worked. We let her rest for a few days before attempting another extubation. Again I thought we’re on the home stretch now, it’s the end of November so hopefully we’ll be home in time for Christmas. On the night of 6th December Martha was given an ECHO that we thought was routine, but later on that night Dr Graham Derrick came to visit us, he thought the stall in Martha’s progress was down to the narrowing in her aorta and the shunting through her VSD. He thought they should re-intervene – but her surgeon didn’t agree. They had apparently compromised and Martha was listed for an investigative catheter on Monday… then they could plan the best course of action for her.
The procedure took just over two hours and she was wide awake and crying when she came back to the ward. Then Dr Derrick came to see us… the narrowing below her supraaortic valve was worse that the ECHO was showing, the shunting through the residual VSD was also more significant. She needed to go back to theatre… she was facing her third Open Heart in her four months of life. She was second on the list for Thursday 12th December 2013.
Dr Tsang was late arriving that morning so the anaesthetists took Martha Grace to get started as they needed an hour with her before surgery could start… we reasoned that the sooner she went down then the sooner she’d be back. When we did see Dr Tsang he told us “don’t worry, this operation is a lot smaller than the last one” – and we know from experience that he’s the type to tell you it how it is.
I wasn’t even scared of her going in to theatre for the third time, as far as I’m concerned she’s safe there as everything is controlled – it’s the recovery that worries me, when she has to do everything for herself. We were relieved that she hadn’t suffered any complications and behaved well when coming of by-pass. Me and my mam decided to stay up all night as she had been unstable after her previous operation… I can handle the situation better if I’m there as it’s developing than if I go away for a few hours and bombarded with all the bad news in one hit.
We went to bed at 6am and Mark took over until we came back in at lunchtime. She was an angel, her fluid output was brilliant, she wasn’t as reliant on milrinone or adrenalin as she had been after previous surgeries. She had her chest closed three days post-op, she’s never had it done that soon after surgery before. She was extubated after four days – I was amazed, she was behaving and I even let myself believe that we might be home before Christmas. But I should have known better.
The next day Martha was having a planned 1/2 CPAP break, but I saw her starting to struggle so I had to leave her bedside. It was happening again. Martha vomited and aspirated her vomit, she was essentially drowning.
They quickly re-intubated her… and with that any hope I’d had for being home for Christmas were crushed. The plan for then was to take it slowly; rest her on the ventilator for a week and take it from there. The next few days were quiet ones and Martha behaved, Christmas came and went and on Boxing Day we attempted extubation again. This was her last time – if she failed she would be fitted with a Tracheostomy. We went back to the accommodation rather than stay to watch the procedure; we didn’t get a phone call to say anything had gone wrong so we braved it and headed back to ICU. We could hear the whooshing of the Fabian as we got to her bed space but it wasn’t long before we noticed it was going wrong… there was major recession and she was as deathly gray colour. I begged them to re-intubate her but they were adamant they were going to give her a chance to prove us wrong. At first I agreed to give her the 20mins they suggested, then they suggested leaving her another half an hour, then they wanted to leave her another half an hour. Each time they measured her blood gasses they improved, the recession decreased and she returned to her normal colour and she looked happier.
She was given a week on the Fabian without any breaks while her heart got used to taking on the job of Martha breathing for herself. On New Year’s Day Martha did well for her first CPAP break which lasted 5 hours… her second break was planned for 4pm and it was at this time that it was decided that she wouldn’t be going back on the CPAP unless she showed signs of needing it.
She finally moved up to High Dependency later that week, it wasn’t any easier once we got there as Martha still had a distended liver (it measured 5cm at one point) but we re-introduced bottle feeding, we were able to take her for walks around the hospital in her pram and I was able to take more responsibility in her day-to-day cares.
On Sunday 19th January we were told that pending the results of an ECHO and Pacemaker test – Martha Grace would be discharged that week. On Wednesday 22nd January 2014, 10 whole weeks after bringing Martha Grace to London – we finally boarded a train at London Euston and brought her home to Anglesey.
In the early days we struggled to control her reflux and with getting her to take more than 30ml orally, she’s been admitted to our local hospital twice with a tummy bug. From a cardiac point of view she has been stable, in fact when we last took Martha to GOSH in September she was put on 6 monthly check-ups… something I couldn’t have imagined in November or December 2013.
Our next appointment is just around the corner and I’d be lying if I said I wasn’t worried, I think I’ll always be nervous for our outpatient appointments – it kind of goes with the territory when you’re a Heart Parent. But to look at Martha Grace, crawling around the house at speed, stealing toys off her big sister and crawling all over her big brother – you’d struggle to believe she’s the same baby.
As all our Heart Warriors are – she is simply amazing.