Anna was born with a giant exomphalos in May 2005 and it
was treated conservatively. She has suffered from respiratory
problems which is why I have updated this just before her
I was sixteen weeks pregnant with my second child 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, Andy, and I met with our (fantastic) consultant,
Dr Steve Sturgiss. Following a scan, he told us there was
indeed a serious a 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
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
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.
Anna 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 Anna'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 Anna 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 Andy following close behind.
We had decided that I would have my mum with me - so that
Andy would concentrate on Anna - and that worked well. The
Special Care team took some instant photos of Anna for me
and Andy was also armed with our digital camera.
Some hours later I was well enough to get into a wheelchair
to visit Anna 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), but Andy had to go home to see our eldest
daughter. I couldn't sleep and just wanted to be with my baby.
At 2am a midwife rang PICU to be assured that Anna was fine.
However, when Andy and I went down to the ward early the next
morning Anna 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 Anna on a ventilator. We were relieved as she
was clearly getting worn out. Anna 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 Anna'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.
Anna had to lie on her side as her 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 Anna 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 Anna. 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
Anna 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
We moved into the Paediatric Surgical unit where we were really
able to get hands on with Anna. 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 Ella and Andy) was finally able to go home.
Initally, a paediatric nurse came in daily to help me with
her bath, but as Anna's 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. Anna's dad was very hands on
and always took care of her baths every weekend. 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. Anna's surgeon
also said that as she had confounded all expectations and
was doing so well he might look at operating at six months.
Anna 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.
Anna 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 when she is nine or ten, but this is yet
to be decided.
Unfortunately, just when we thought that everything was calm
after her operation, Anna started to suffer from respiratory
problems. Spring and summer 2006 involved lots of dashes to
the RVI and many admissions. In August, the respiratory team
at the hospital discovered that Anna had problems with her
lungs - tracheomalacia and bronchomalacia. In basic terms,
her lungs and wind pipe are too floppy (this is because of
her exomphalos) and as a result she does not shift infection
as she should. She also has asthma which adds to her difficulties.
However, this situation is slowly improving with age and we
have had fantastic support from Dr Chris O'Brien and Dr Mike
McKean at the RVI. Thanks to them (and many, many other NHS
folk) Anna should lead a long and healthy life.
The past five years have been very tough at times, but our
youngest daughter is an absolute joy to us and we still can't
quite believe that she is here. We also had wonderful support
from friends and family, which has made all the difference
in the world.
Lots of love to anyone reading this who is expecting a baby
with exomphalos. If you would like to read Anna's blog you
can go to:
Jane (Anna's mum)