Paediatric Cystic Fibrosis team celebrates 25 years of service
The NNUH Paediatric Cystic Fibrosis (CF) team is celebrating 25 years since its foundation. It’s also the 40th anniversary of the launch of the world’s first successful newborn screening service for cystic fibrosis, which started here in East Anglia with support and participation from NNUH and other regional hospitals.
The two landmarks were celebrated at a special event at Dunston Hall on 5 March, organised by Dr Anjay Pillai, Consultant in Paediatric Respiratory Medicine and director of the paediatric CF service at the Jenny Lind Children’s Hospital. It was attended by the Cystic Fibrosis Trust, Chief Nurse Nancy Fontaine and around 100 delegates from across the country.
Speakers included Alison Betteridge, a Cystic Fibrosis Nurse who has just retired after 38 years; Dr Chris Upton, the first dedicated CF paediatrician appointed by the trust; Dr Caroline Kavanagh, Consultant in paediatric respiratory medicine, and a panel of expert speakers from other CF centres who reflected on the past, present and future of care and newborn screening.
Dr Anthony Heeley, retired head of the regional biochemical genetics laboratory, spoke about his pioneering work in setting up the newborn screening service in 1980 – a world-first model adopted across this country and many others. Polly Crosby, the first “baby” diagnosed with cystic fibrosis as a result of newborn screening and now a successful author, also talked about her experience of growing up with the condition.
“Before newborn screening, children were usually diagnosed later in the course of the illness, when they presented with symptoms,” said Dr Pillai. “This screening, adopted nationally in 2007, detects nearly 97% of cases within two weeks of birth and provides an opportunity for early intervention and preventing complications. Together with specialist multi-disciplinary care and multiple medical advances, this has significantly improved outcomes and markedly enhanced health span and life expectancy.”
What was once a lethal disease is now a well-controlled chronic condition for most children and more advances are being made all the time.
“In addition to well established multi-pronged management strategies addressing lung and gut problems, this year we started rolling out the new generation of gene modifying treatments called ‘CFTR modulators’ for those with eligible mutations,” said Dr Pillai. “Gene therapy is not here yet but the future looks promising.”
Historically, there was no specialist care for patients with cystic fibrosis. As awareness grew, CF care was supervised by regional specialist teams and today patients are looked after by a highly specialised team that includes doctors, specialist nurses, physiotherapists, dieticians, psychologists, pharmacists and social workers – such a team has been in place here at NNU for 25 years.
“Today, data from consenting patients is sent to a national database to build a nationwide picture, and we have always performed well for patient outcomes,” said Dr Pillai.“
“We are one of two specialist paediatric CF centres in East Anglia, the other being at Addenbrooke’s Hospital, Cambridge. We provide specialist outreach services to other hospitals in the region and are actively involved in CF research as part of the National Clinical Trials platform.
“We’re proud to mark our two special anniversaries with the event at Dunston Hall and are proud of our role as a specialist centre that’s continuing to help improve the lives of children and adults with cystic fibrosis.”
- Cystic fibrosis is a genetic condition that affects more than 10,500 people in the UK. It’s caused by a faulty gene, carried by one in 25 of us, which affects the movement of salt and water in and out of cells which, alongside recurrent infections, causes sticky mucus to build up in the body’s tubes and passageways, particularly the lungs and digestive system. Around 75 children are currently being treated at the NNUH paediatric CF centre, with 2-3 new diagnoses a year. For adults with cystic fibrosis, there is a separate adult CF service at NNUH which works closely with the paediatric service.