Cystic Fibrosis

Cystic Fibrosis (CF) is a condition that affects a child’s lungs, digestive system and other organs. The CF team cares for patients and their families from diagnosis (usually as a baby through Newborn Screening) through to transition to adult services.

Some children with CF can have some problems gaining weight or with chest infections, but the majority of our patients have near-normal lives. The team here at the Royal Hospital for Children and Young People (RHCYP) support children and young people living with this disease, as well as their families, through their early years and adolescence. Our aim is to keep children with CF as well as they can be so that they can achieve all the things that they want to do.

The CF team, based at the RHCYP, provides outpatient and in-patient care for children and young people with CF. We are a multidisciplinary team, which means a team of specialists all working together and can include consultants, specialist nurses, dietitians, physiotherapists and a psychologist. We also work with other teams and departments whenever they are involved in the care of a child with CF.

Newborn Screening & Diagnosis

CF can be diagnosed at any age. In the UK, all babies have the newborn blood spot screening test at around 5-8 days old, with parental consent. The team at the RHCYP is informed of all babies suspected to have a diagnosis of CF within the area they cover.

Two experienced members of the CF will arrange to visit the baby at home as soon as practically possible. This is usually a doctor and a nurse (and sometimes a health visitor if the family has already met them) who give the family information, guidance and support during the initial stages of diagnosis.

This can be a very difficult and challenging time for families and it is important that they are given reliable, accurate information at the right time.

An outpatient appointment is usually made for the baby and their family to come to the RHCYP and meet the rest of the CF team as soon as possible. Tests to confirm the diagnosis will be carried out as soon as possible.

These include blood tests for genetic testing and a sweat test. Download the sweat test PDF file.

After this first appointment, babies with a new diagnosis of CF will be seen at weekly clinic appointments where plans for their care and overall health monitoring will be made.

Clinic Appointments & Annual Review

Each year families are invited to an annual review, which is a longer appointment where we review each child’s health and talk about any events of the last year. We also try to set some goals for treatment in the coming year. We might carry out blood tests, detailed lung function tests (for children aged over 5) and x-ray tests at these appointments. We may also do extra tests such as an exercise test, an ultrasound of the tummy or a measurement of blood sugars. While we do our best to coordinate these different appointments and group them together wherever possible, it might be necessary to come to the hospital more than once, to get everything completed. Families will meet many different team members at their child’s annual review appointment, and it is a great time to bring any questions that you might have about CF or your child’s care, health and general well-being.

Blood Tests

Blood tests are usually carried out every year at a child’s annual review appointment. Sometimes, when children are unwell or starting a new medication, a blood sample may be taken more regularly. We understand that many children can feel scared or anxious about having a blood test. There are lots of different ways that we can help children who feel worried about having a blood test, which can include support from our Play Team specialists – please tell us if you would like to speak with someone from the team.