Fiona was just 10 days old when she had an emergency tracheostomy to save her life. She has faced a difficult series of operations in the five years since. You can support patients like Fiona by donating to our appeal for two new operating theatres, due to be completed in 2016.
Fiona was just a few minutes old and about to have her first feed with her mother Emi when she was rushed away to special care.
Emi and Fiona’s dad Andrew were shocked and very worried when doctors told them Fiona may have something called Pierre Robin syndrome, where a smaller than usual lower jaw causes breathing problems.
It transpired that Fiona’s condition was in fact a much rarer disorder called Nager acrofacial dysostosis, or Nager syndrome.
When Fiona was just 10 days old she had an emergency tracheostomy, meaning a hole was made through the front of her neck and into her windpipe for her to breathe through. It was almost 3 months before she went home for the first time.
“The hospital and staff have been wonderful”
When Fiona was 18 months old she came to Great Ormond Street Hospital for distraction surgery. This surgery was traditionally used to lengthen arms and legs, but in the 1990s smaller devices were developed to allow craniofacial surgeons to lengthen the jawbone.
The process for Fiona began in 2010, when she was three years old. “They cut the jaw in half and put in bolts and a screw that we had to turn every day so new bone is formed in the gap as it widens – it was very painful for her,” says Andrew.
“It’s such a difficult operation but the hospital and staff have been wonderful, they make us feel very relaxed and Fiona is never nervous before operations,” says Andrew.
Nager syndrome has also meant Fiona has had two lots of surgery to correct problems with her thumbs. She is seeing a specialist because she can’t straighten her elbows and a bone growing across the inside of her ear blocks sound so she’ll have a permanent bone-anchored hearing aid. She may need further surgery on her jaw to reconstruct the joint as her mouth doesn’t open very far.
These kinds of operations will be carried out in two new operating theatres we are planning to build at the hospital, in the second part of the Mittal Children’s Medical Centre, due to open in 2016.
A positive outlook
Fiona currently uses sign and symbol language Makaton to communicate and Andrew hopes that once all the operations are finished they can look at having the tracheostomy removed.
The many operations Fiona has had mean she has missed 80 per cent of school. “She had made very good friends but because she wasn’t going, they formed other bonds,” says Andrew. But, he says: “Fiona has a very positive outlook on life, and what we are most proud of is Fiona’s confidence.”