Cystic Fibrosis (Paediatric CF)
We are a Paediatric Specialist Cystic Fibrosis Centre and we had an extremely successful inspection by the Cystic Fibrosis Trust last year. We care for 40 children and families, and some of these were involved with our inspection.
Our children are seen regularly at the cystic fibrosis clinics. They also have open access to the Children's Assessment Unit where they can be seen quickly when they unwell.
We put basic information about our patients (with consent) onto the Cystic Fibrosis Trust PORTCF database. This provides the Cystic Fibrosis Trust with generalised data for the whole country and this is presented in an annual report for the country. They can also use the data to lobby the government for services for the Cystic Fibrosis community.
As a team we also visit the James Paget and Queen Elizabeth hospital's for their Cystic Fibrosis clinics to support the teams and the families as part of our role as a Specialist Cystic Fibrosis Centre.
We have a weekly paediatric Cystic Fibrosis clinic every Monday afternoon. This is a segregated clinic where the children are not able to meet. This is to reduce the risk of cross infection with each other as this has been recognised as a problem within the Cystic Fibrosis community.
Each member of the multidisciplinary team meets individually with the child and their family during their clinic appointment. At the end of clinic we have a multidisciplinary meeting involving the whole team. At the multidisciplinary meeting we discuss all of the children we have seen at the clinic that day and also any of the children not seen that day who have made contact with any of the team members during the previous week.
The vast majority of children are now diagnosed through newborn screening and parents are given the diagnosis when their child is very young. We recognise that this is a very difficult time for our families as they have to commence treatment for their child. We therefore review our newly diagnosed babies on a weekly basis initially to help our families adjust to the diagnosis of Cystic Fibrosis within their family unit. We continue to review them 4-6 times per year when they are children until they leave our service at around 16 years old.
If our patients have other complications related to cystic fibrosis then we arrange for them to see the relevant specialist team, for example the paediatric gastroenterology team at the Norfolk and Norwich University Hospital.
Children attend the annual review clinic once a year. This clinic involves a detailed review of the past year of the child's life. It looks at all aspects of the child's management within the past year. It includes management of their chest, nutrition, general wellbeing, other complications, social, family and schooling.
Their progress over the previous year is assessed and areas are identified where improvements can be made. Prior to this clinic attendance children have a full set of investigations to assist the assessment process. This includes blood tests, radiological investigations and dietary investigations. Again, they are seen by all members of the multidisciplinary team at this clinic.
We have a teenage transition clinic once a month for the young people with cystic fibrosis aged between 14 and 16yrs. At the NNUH we are fortunate that the adult Cystic Fibrosis Service exists on the same site.
At our transition clinic the teenagers get to meet members of the adult team on a regular basis with our paediatric team. This allows the young people and their families to become familiar with the adult team prior to their transfer to adult care.
Our transition service is fairly unique in that we have such regular joint clinics and the Cystic Fibrosis Trust asked us to write an article in their quarterly magazine to make families aware of the possibilities for teenagers with cystic fibrosis.
Norfolk, Suffolk and Cambridge Paediatric Cystic Fibrosis Network
The Norfolk, Suffolk and Cambridge Paediatric Cystic Fibrosis Network is a network of 8 hospitals looking after children with cystic fibrosis within the Eastern region. We meet twice yearly for a full day meeting. Dr Caroline Kavanagh is Co-director of the network and is the educational director of the network. Our group involves doctors, specialist nurses, Physiotherapy , dieticians and psychologists.
We have a morning business meeting which looks at guideline development for patient care within the region and also local, national and international issues affecting cystic fibrosis care. We meet as individual disciplines and later in the morning as the whole network to discuss these issues.
The afternoon sessions are educational meetings looking at specific issues affecting children with cystic fibrosis and other respiratory conditions and are excellent educational opportunities for keeping up to date.
We also have a network-wide website accessible only by the network members which is managed by Dr Caroline Kavanagh.
Our Network guidelines for the care of children and young people with Cystic Fibrosis are available on the hospital intranet site.
We are an enthusiastic team who often become involved with multicentre studies involving children with respiratory problems and those with Cystic Fibrosis. Some of the studies we have been recently involved with include:
- Carrier Testing for Cystic Fibrosis
- Mannitol Study in Cystic Fibrosis
- MASCOT - management of asthma in school age children on therapy
- TIDES - data collection, looking at depression and anxiety in people with CF and their carers.
- BTS Paediatric Asthma Audit
- BTS Paediatric Bronchiectasis Audit
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