Jane's experience

Elizabeth was born with a giant exomphalos in 2005 and it was treated conservatively. She is now in her teens.

I was sixteen weeks pregnant with my second baby when I discovered that there was a major problem when the results of my '16 week' blood tests came back. My 12 week scan had looked fine, but I felt peculiar and was convinced that the blood tests would be positive. I remember apologising to the baby for having them! Three days before Christmas 2004, my midwife called to ask me to go to the Royal Victoria Infirmary (RVI) in Newcastle as the tests had come back with some worrying results and the baby could possibly have Spina Bifida. 

The midwife called at 4pm and our appointment at the RVI was at 9.30am the next morning at the Fetal Medicine Unit. My husband and I met with our (fantastic) consultant, Dr Steve Sturgiss. Following a scan, he told us there was indeed a serious problem with the baby. We were told that the baby had exomphalos - something we had never heard of - and that he/she could also have the chromosomal disorder called Edwards Syndrome. We were offered a CVS test immediately to check for this and we decided to go ahead. The test took place within an hour or so and was painless. The fact that things happened so quickly that day was an enormous help to us as there was no time to get really anxious.

From the start, we were treated with enormous kindness and sensitivity by the team at the RVI and we will always be very grateful for that. 

Unfortunately, because of the Christmas break, we had to wait longer than usual for the results of the CVS test. The normal two days became six and they were the longest of our lives. We spent the period between Christmas and New Year investigating the implications of Edward's Syndrome and finding out more about exomphalos. Thankfully the results for Edward's Syndrome were negative. 

The first few weeks following diagnosis were really difficult. Expecting a baby is normally such a happy time, but we had to tell people about the exomphalos. Being able to direct people to the GEEPS site was a great help as that covered 'the science bit'. 

We eventually decided to find out the sex of our baby as it seemed weird that all the RVI team knew what we were having from the results of the CSV, but we didn't. Knowing that we were expecting a little girl gave us a real boost and a little 'person' to focus on. 

We attended the RVI every three weeks and as time went on more was discovered about our baby. Her exomphalos was huge, but before she was born the Foetal Medicine team were pretty certain that her heart and lungs were good. I felt such mixed feelings during the scans as I felt enormous love for my baby mixed with real fears about how long she would be with us. I was also very concerned about my eldest daughter and how she would feel if her sister died. 

Elizabeth was born by C-section at 38 weeks. Our consultant had been encouraging about trying for a vaginal delivery, but because of the position of the baby's liver in the sac he finally opted for surgery and carried out the operation himself. I found the whole experience quite surreal. I heard the baby cry (my mum could hear her down the corridor her cry was so loud!), saw her for a few moments, held her hand and then she was whipped off to Special Care with my husband following close behind. We had decided that I would have my mum with me - so that my husband could concentrate on Elizabeth - and that worked well. The Special Care team took some instant photos of the baby for me and my husband was also armed with our digital camera. 

Some hours later I was well enough to get into a wheelchair to visit Elizabeth in Paediatric Intensive Care. She looked dreadful, but was breathing for herself and I managed to breastfeed her. To feed her and cuddle her on that first day was incredible as it was so unexpected. 

Unfortunately, the good times did not last. That night I was taken back to my room (midwifery had very kindly given me a private room, so that I didn't have to listen to other people's babies crying) and my husband went home. I couldn't sleep and just wanted to be with my baby. At 2am a midwife rang PICU to be assured that Elizabeth was fine. However, when we went down to the ward early the next morning Elizabeth was very unwell and making some awful grunting sounds. All day the medical team tried to get her breathing back in line, but eventually they told us that they would have to put Elizabeth on a ventilator. We were relieved as she was clearly getting very worn out. Elizabeth remained on the ventilator for eight long days during which time she was fed mainly on expressed breast milk. 

The nurses on PICU were wonderful. They really encouraged us to get involved with Elizabeth's 'cares', so we changed her nappies, wiped her eyes and so on. She was very, very swollen during this period and could barely open her eyes most days. It was heartbreaking to see her cry - unable to make a sound because of the tubes. 

Elizabeth had to lie on her side as her huge exomphalos put so much pressure on her chest. Her dressing was changed daily and the nursing staff suggested using a surgical stocking to hold the dressings on to contain it a little. All we could do was spend as much time as possible with our little girl and give her some comfort. The nurses were very gentle with her. 

We met with her surgeon (Mr Bruce Jaffray, another wonderful RVI man) who told us that he had decided to take a conservative approach with Elizabeth. No surgery would take place until she was at least 12 months old and that she might be in hospital for at least six of those. The exomphalos would be bathed daily and treated with a burns cream called Flamizine to encourage skin to 'heal' over

Elizabeth came off the ventilator at nine days old and came out of PICU five days later. She still needed oxygen 24 hours a day, but she was much less swollen and, to our delight, we started to see that she looked just like her gorgeous big sister. 

We moved into the Paediatric Surgical unit where we were finally able to get hands on with our daughter. She started to have a daily bath with disinfectant solution and then her exomphalos was treated with Flamazine and dressed. Over the next few weeks I gradually took over the lead in this from the nurses. Anna did really well and and, bar some nasty infections, she thrived from this point on. Her exomphalos started forming 'skin' over it and began to reduce in size which in turn helped her breathing to improve. 

When she was six weeks old we were told that we could nurse her at home with the support of the Community Nursing Team. We were very worried about this, but were persuaded that things would be fine and they were. It took me some time to get my head around ordering oxygen canisters, prescriptions etc, but all the difficulties were worthwhile as we were able to start living as a family again. It was wonderful to see my eldest daughter every day and my mum - who had stayed to look after her - was finally able to go home. Oxygen was piped in around the house and I had to take mobile canisters with me wherever the baby went.

Initially, a paediatric nurse came in daily to help me with baths, but as the exomphalos shrank in size this went down to twice a week. The nurses were all lovely people and it was a pleasure to see them. We visited the out-patients clinic at the RVI every couple of months and were thrilled when we were told that Anna's organs had gone back into her abdomen of their own accord. Elizabeth's surgeon also said that as she had confounded all expectations and was doing so well he might look at operating at six months. 

Elizabeth went into the RVI for her closure operation at the beginning of December 2005 - at the age of just over six months. I expected to feel very nervous, but by this stage I had developed such faith in the team at the RVI that I was strangely calm about the whole process. 

Elizabeth took the operation in her stride and came down from theatre with a wonderful flat tummy. She was well enough to come home within two days. It took her a little while to catch up on sitting up, rolling over and so on. The muscle coverage on her tummy is weak and she has a little bulge, so she may have another repair op, but this is yet to be decided.

Elizabeth has not had the easiest of times healthwise since then. She had respiratory problems as a toddler as a result of her exomphalos, which required several emergency admissions to hospital. She has asthma and allergies.  At the age of nine, she developed ME/CFS - however, this doesn't appear to have any link to her problems in the womb. She also has scoliosis, but we have not seen the consultant yet and do not know whether this is linked. She is a lovely young woman who has been through an awful lot for her young age. I can't tell you how much I love her.


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