Seasons Greetings

Merry Christmas to all blogreaders and a happy healthy 2009!

People you meet along the way...

I was thinking about a friend of mine and her daughter today and that got me thinking about other people we've met over the years too.



Corinna was the first person I found whose child had the same heart condition as Emily. Tis a very rare defect, around 1 in 300,000 live births, and I've not been able to find a child older than Em although I know there are some out there. It was Corinna who introduced me to a couple of online support groups and the friends I've made through them have been with us through thick and thin. Corinna's daughter Nia has been very unlucky in contracting leukaemia (again a very rare strain). She doesn't do things by halves, Nia. At some point over the next couple of weeks she's due to have a bone marrow transplant and they are on my mind alot.

The appointment.

The results of the MRI were inconclusive.



This is more than a little disappointing as it means that Emily has to have a cardiac catheter to clarify what's going on. This issue is with her pulmonary artery which is narrowed where the left coronary passes through it. On echo the pressures are high, the stenosis is graded as severe and would normally require surgery before causing permanent damage to the overworked ventricle. That said, past catheters have shown that the area is difficult to measure on echo and measurements taken once inside have been more acceptable. Just hoping that this is the case now and the cath will just show that ongoing monitoring is required rather than intervention.



I'm trying to be hopeful but my pessimistic side is currently winning the battle going on in my head.

Hello.....

I've rather neglected the blog haven't I? It took me years to start it and now it's only half told. Still, it got it out of my system for a while.

I'm back because Emily went to Birmingham for an MRI this week. It's not something she was looking forward to but she did so well. It was a very long scan, with lots of breath-holding. The machine is very claustrophobic and noisy and it's not something she wants to repeat so I hope the pictures are in focus!

The follow up appointment is in four weeks. So now we wait.......

Fast forward.....for a moment...

Ok - so whilst this is really a 'historical' blog there may be the odd foray into real-time for relevant updates. Confused?!

Today we had an appointment with Emily's orthopaedic surgeon.

Following on from the clot that I posted about previously, the blood flow in the leg was compromised and it didn't grow properly. By the time she walked at around 18 months it was obviously much thinner and a good bit shorter. She has had very weak pulses in that leg all her life. We had some physio whilst she was small to help with strengthening the muscle and she has always worn a raise on her shoe in order to maintain her back and hips in alignment. Her surgeon was always hopeful that her would be able to level her out by a procedure called epiphysiodesis. The surgery involves putting pins (big fat Frankenstein bolts) in in the leg around the growth plates at the lower end of the femur and the uppper end of the tibia/fibia. The pins restrict the growth of the bone and allow the good leg to catch up. This all went ahead when she was 12 and the pins removed 2 years later.

The upshot of today's appointment was that the wounds are well healed, she's fully grown and is as good as level. Discharged. Success!!

Escape from PICU

Finally we made it to the ward. Not far physically - PBG ward which was right next door to ICU in the old Children's Hospital - but it was a massive step forward and I did finally start to relax a little and believe that I would bring her home eventually.

I'm not sure that anyone would be interested in a blow-by-blow account of the next few months. We continued to have some bad days but they were more than outweighed by the good. Emily had had no proper nutrition during her time in ICU - only maintenance fluids as she couldn't seem to tolerate even the tiniest amount of feed. There was a short foray into TPN but that was discontinued when there was a concern about infection. So she left ICU at barely over her birth weight despite now being around 20 weeks old. She looks so skinny in this picture with Josh. Look at her stick legs! And the bald patch on the side of her head where she had a line in after all her other veins gave up the ghost.

Feeding, putting some weight on, and sorting out her long-term drugs were the next goals. She was still in quite severe heart failure and this was likely to continue for some months at least, still couldn't suck so all her feeds went down the tube and we gradually increased from 10ml an hour to something more reasonable. The feed had so many additives to increase the calories that it was as thick as custard and couldn't be gravity fed. It had to be pumped or syringed via the tube. We were patient though and she gradually gained a little weight.

A cuddle at last

Emily continued to give us some scary moments but on the whole began to improve. She did not want to be weaned from the vent though so it was slow going. Towards the end of the fourth week post surgery she finally got extubated and into a 'headbox'. It was fabulous to see her face again without the tubes although she still had her nasogastric tube which was to become almost a permanent fixture for several months. And better still she was beginning to be weaned off some of her drugs. The morphine and sedation withdrawal made her particularly miserable; unsurprisingly as she'd been on it for quite some time. She worried me for a time as seemed unable to focus on anything but that slowly got better over the weeks.

Being able to hold her in my arms was wonderful. If you've ever heard the phrase 'my arms ached for her' then I can tell you that it is almost a physical pain.

Worries averted.

It took some time for her to become settled again - she was having periods of tachycardia and desaturating. They wondered if she'd had a brain bleed and was fitting despite being on full support so there were more tests to endure; scans, ultrasound and an EEG. All proved to be clear and during the next few days she was finally listed as stable as opposed to critical. At last something to celebrate!

My usual routine was to get up pretty early and visit Emily before breakfast, around the time that the staff changed over. On this morning it was my favourite nurse Suzie who was to look after her. There had been concerns overnight as Emily had been very up and down. One of her main lines had 'blown' and tissued into the arm, her monitoring was all over the place and it was noted that some of her old puncture wounds had started to bleed. I was reading through her overnight notes and started to ask Suzie about the fact that her suctioned secretions had been blood-stained - at almost the same moment Suzie had realised that this wasn't just Em being difficult - but that there was something fairly seriously wrong. Things developed pretty quickly, Suzie took down the heparin immediately and called the team over. Her clotting was way off (they couldn't clot the sample at all in the ICU machine and the labs which came back later noted the same) It transpired that the heparin pump had been refilled overnight and because of a lack of clarity in the prescription Emily had been given 10x her normal dose.

It shook my faith quite a bit as you can imagine. But I have to say that it was well handled by the Head of Intensive care and the Head of Nursing Services. They were very 'upfront' with regards to what went wrong and I saw the prescription for my myself immediately after the incident. Thankfully it led to protocols being changed surrounding the writing of prescriptions for that particular drug. I was saddened to learn though that another child died from the exact same overdose following open heart surgery in another children's unit just two years ago. This led me to speak with PICU again recently to confirm that the protocols remain in place.

Taken shortly after the heparin incident. You can see what a state her arm was in.

Life on PICU

The next couple of weeks went by slowly, punctuated by crises. She took it upon herself to let us know when one was approaching - temp and HR would soar, BP and SAT's plummet, usually giving enough time for the nurse to summon enough hands to help 'bag' her through. Around the middle of week three we were moved on to the main ICU ward. We'd been in a little cubicle up until this point and to be out onto the unit was seen as an improvement.

This heralded another of my little enlightened moments (residing in my little self-made cocoon of denial I came to have quite a few) . I'd assumed that we were in the cubicle because Em had bronchiolitis.; it became evident over the next couple of weeks that that wasn't the reason at all. Every patient who went into the cubicle after us died and it turned out that patients were placed there because it offered more privacy for the families who were expected to experience a loss. I am eternally grateful that Em decided that she wasn't going anywhere.

The leg was still giving cause for concern but the heparin was thankfully preventing more clots. However a few days later it was the heparin itself that was the cause of some problems.

A good friend.

I mentioned my friend Caroline a little earlier and I need to say something about her. She hates hospitals - has a real phobia of them - but she was such a support to me during those early days at Birmingham. She put aside her panic to be with me and I can't thank her enough - I don't think I ever thanked her properly. She drove me up and down the motorway when I wasn't fit to do it myself and she even accompanied me on a mission back to the children's ward at our local hospital to retrieve one of Emily's baby blankets. Emily had been sick on it the day she was diagnosed and it had remained under the sink in the sluice room for several more days. She must have thought I was nuts to contemplate a round trip of over 100 miles to find a stinky blanket, but in my odd mental state at the time I just had to get it back. She never once commented on my madness. Bless her!

This photo was taken by one of our fab nurses, Suzie, after she came back from theatre the second time. She covered her with a little dress because her chest was such a mess.

I didn't realise the significance of it at the time - I assumed that they did this for everyone but it was only when we were leaving PICU some weeks later that I understood that they really didn't believe that she would make it through the night.

Back to theatre

She had many ups and downs. Being called to ICU by one of the nurses on the Sunday was probably the worst moment. When I got there, with my friend Caroline who had come to see us, they wouldn’t let me in. It was dreadful just waiting there and knowing that when they came out it could be with the worst news. She was just so unstable and several times over the next couple of weeks they had to work hard on her to keep her with us. On this occasion she had suffered a clot in her leg following surgery, it was causing some problems and they were concerned that any break off could cause a stroke or be carried to her heart/lungs. Her right leg was cold and mottled and there was no pulse, so they had to take her back to theatre where they removed an embolism from the femoral artery at her groin. They also closed her chest at the same time as she was on maximum heparin, they needed to prevent her bleeding more than necessary and they wanted to do chest physio to help her shift the bronchiolitis.

The longest night

It was the longest night - we remained in the PICU parents room - there was nowhere to sleep and we napped on the uncomfortable chairs. It was pretty much light when Mr Sethia came to see us. She was on her way back to PICU and we'd be able to see her shortly. They'd had a hard time getting her off bypass as her LV was only functioning at 9%; it was the start of a long & bumpy road - she just had to fight.

4 February 1994 - The bad news

Again she quickly went down bank and after a couple of days, back I went to the consultant’s clinic where another doctor said he thought she had RSV. He watched her feed and said if she was worse the next day to come back. We got in the car and she was promptly sick again and I burst into tears. She was about 14 weeks old by now.
The next day she looked just awful. She was grey and struggling for breath. It sounded like she was drowning. The cardiologist thinks she’d had a heart attack during the night. We rushed her back to the ward where they put her on a SATs monitor and did some tests for RSV. The nurse commented that the SAT’s machine wasn’t working properly because the oxygen levels were 100% and her heart rate was 200 (& that couldn’t be right ??). We went for a chest x-ray and when we got back they put her on another monitor. Within 10 minutes of us getting back from x-ray all hell broke loose. The film showed that her heart was so enlarged that it was collapsing her left lung. And the new monitor showed her HR was still 200. The paed came to tell us that she had a problem with her heart, possibly a virus, and she would need a scan. At this point I just shut off I think, they were asking me if I needed to know anything but I just said ‘No, its fine’!! Even when they talked about going to the Children’s Hospital (Birmingham) it just didn’t register with me. They said she couldn’t go for the scan for a while, so I left her with her dad and went to collect the boys from school and nursery. When I got back, he was on the phone. The ambulance had arrived to take her so he had to go and he said he would ring me. We were told that she’d be coming straight back and the ambulance would wait.

I truly regret leaving her with Gary that day. I was in complete denial of the seriousness of the situation I think. I don't regret it because he wasn't up to the job - he was and is more than up to the job. It's just that I wasn't there for him or for Emily at possibly the worst moment......


Well when he got to BCH it was a different story. He says that when the doors opened a sea of people greeted him, a full PICU team, who took Emily from him. Even before the echo they said that from the ECG they could tell that she either had a serious congenital defect or a virus and either way they weren’t sure they could do anything for her. They wanted me there immediately.

Gary doesn't speak about how he felt at that point but I know he was badly shocked, close to breaking down I think. I heard it in his voice. It must have been a dreadul call to make as well as to receive.

You can imagine how I felt; they were 50 miles away. My dad drove me and I thought it was the worst hour of my life (little did I know). She'd gone into cardiogenic shock and when I arrived they had ventilated her and were basically waiting for me to say goodbye. So I had two precious minutes with her before they went to catheter and then hopefully to surgery. After a couple of hours they came to say that she had Anomlaous Left Coronary Artery from Pulmonary Artery ALCAPA and she’d gone to theatre, plus she did have bronchiolitis which was an added risk. Her LV function was so poor they could only hope that she’d get through. I vaguely remember someone bringing us tea and explaining about the defect but apart from that the night is a complete blur.

Over anxious

Once there they took more bloods and a urine sample and watched her feed. She was fine (aren’t they always?) and I think they decided that I was an over anxious mum. The tests showed a UTI and anaemia and we were sent home with anti-b’s and iron. Next day it was back to normal, Emily feeding very little and vomiting it all back a few minutes later. It was the weekend so we called the emergency doctor who said it was probably the iron or the anti-b’s and to stop giving them and take her back to the GP on Monday. We went back and he changed the meds but wasn’t willing to refer her again. On our way out I saw the HV who weighed her and as she had lost more weight, went over his head and sent us back to the ward. (I have to say that she was brilliant throughout and probably saved Emily’s life as she was so persistent).
So, back we went and she was put on IV meds and had an NG tube passed. We stayed for a few days and she fed okay, although she never seemed really settled. During this stay she had a funny turn which turned out to be the first of her two probable heart attacks. She had been in a bouncy chair on the ward floor when she suddenly went a horrid grey colour, sweaty and started to cry. I asked the nurse to watch her while I went for a bottle, thinking she was probably hungry. When I got back she was floppy in the nurse’s arms. She began to cry again and then fell asleep for about 3 hours. They noted the episode in her records and a couple of days later discharged us again with more iron and anti-bs.

And so it began......

Everything was fine until, at about 6 weeks her slow weight gain became non-existent and the HV asked me to wake her and feed more often. Despite this she began to lose weight and I changed to mixed breast/bottle feeding to see if it helped. All that did was prove just how little she took and within a few days she began to vomit back most of that. At about the same time she also began to get breathless and sweaty at feeds. My HV wasn’t happy and at the next baby clinic we saw the paed who did a monthly clinic. She did a full exam and took some bloods. A few days later she rang me at home with the results (why didn’t alarm bells ring at this point?) and asked me to take Emily to the GP and give him the results. Our GP didn’t seem concerned at all but at my insistence referred her to our local children’s ward.

24 October 1993 The New Arrival

Emily presented herself happy and healthy (?) on 24.10.1993. She was a few days late, labour was very fast, her heart rate dipped at one point and they panicked a little about getting her delivered immediately but it was otherwise uneventful. She weighed in at almost 10lbs and when we took her home we congratulated ourselves on what a contented child she was. She slept all the time and had to be woken to feed; with two boisterous brothers already at home she fitted right in.