How The Exeter Service Runs – Paediatrics

The children’s cystic fibrosis team see children and young people with Cystic Fibrosis from diagnosis until it is time for them to complete transition to adult CF care leading up to their 18^th birthday.

Weekly Paediatric MDT CF clinic are held on Monday mornings. These take place in the Paediatric Outpatient department, Royal Devon and Exeter Hospital, Wonford site, Level 1, Area J.

In response to the COVID pandemic we have learnt from the experience and adapted the way we run our CF clinics. We now offer both Face to Face and Virtual appointments depending on clinical need and circumstance. Virtual appointments ( held by ‘Attend anywhere’ video link) have been welcomed by many of you allowing flexibility and avoiding the need to attend hospital so regularly when well. We currently aim for alternate virtual and face to face appointments but we will guide you depending on age, clinical need and ability to perform accurate home spirometry as there are particular benefits of face to face apppointments in young children.

From the age of 14 years, young people with CF will be offered  some of our Adolescent clinics held on Wednesdays (currently both morning and afternoon clinics held intermittently) and are an opportunity to start meeting some of the adult team. These take place in the adult outpatient area at the Wonford site, just to the left of the main entrance. As you near transition to adult services,  you will be invited to attend 2 joint clinics here with  members of your paediatric team and future adult team  to ensure a smooth transition for you.

If there are specific infection control issues relevant to your child’s care, or if it is important to see you at other times then arrangements can be made for ad hoc slots to meet your needs

Please allow plenty of time for parking prior to your appointment.

Face to Face clinics:

Children with CF are at risk of cross-infection with bugs from other people with CF. Face to face clinics are therefore organised to minimise the risk of this happening. We allocate you a specific appointment time to help with this and aim to keep you separate from other patients. On arrival, you are allocated your own clinic room and then (apart from height and weight measurements) all other activities take place within that room. We encourage you to bring your own  child’s toys and activities to appointments.Each room is cleaned before and after use to CF standards of care.

Virtual Clinics:

Virtual clinic appointments by ‘Attend anywhere’ video consultation have been introduced during the COVID pandemic with home monitoring and will continue to form part of your CF care between face to face appointments and we hope you find these a safe and convenient way to access your CF care. The link will be sent to you to access appointments. An appointment link will be sent to you before your appointment and we will join you virtually!

For all Face to Face and virtual clinics  , please have your recent spirometry readings ( if appropriate)  available ahead of the clinic ( see Tips below!)

Tips for a successful virtual appointment!

Please ensure you have any spirometry data , oxygen saturation data, any BM readings and recent height/ weight measurements available to maximise the effectiveness of your appointment.

Please  send a cough swab/ sputum sample in a week before your appointment date so results are available for you – contact the team via email if you haven’t got any testing kits.

Think about where to take the Video call – have you got good wifi reception?, good lighting is helpful, ensure you will not be interrupted during important and sensitive discussion.

If you can’t access us we can problem solve by telephone and usually resolve this with you!

Each clinic is attended by at least one CF Doctor and members of the Multidisciplinary team.

You will meet:

• Clinic receptionist – to check you/ your childs details and inform the team of your arrival
• Clinic nurse – they will measure height, weight and an oxygen level
• CF Specialist nurse – they will discuss any specific issues and provide support, perform lung function on children old enough to do so and perform any routine CF care such as flushing of devices.
• CF Specialist Dieticians – to support nutritional needs
• CF Specialist Physiotherapist – may take respiratory samples, review chest and airway clearance techniques, adherence and nebuliser use, musculoskeletal and pelvic floor screening, exercise testing and offer advice on exercise.
• CF Doctor- to review the information gathered by the multidisciplinary team, undertake a physical examination and make any specific decisions about further investigations and care.
• A CF Specialist Psychologist is available to offer support as needed.

The Royal Devon University Hospital (Exeter)  is a teaching hospital so you may meet medical students and junior doctors during your visit to the CF clinic.

We ask that you keep a record of your future appointments with us, and let us know with as much notice as possible if you are unable to attend – at least 72 hours notice for routine appointments, and at least one week for an annual review. By cancelling your appointment ahead of time we will be able to offer the appointment to another person and ensure a smooth and efficient running of the service. Call  CF appointments  directly on 01392 406493 or email the team . Thank you for your help with this.

Children with cystic fibrosis may be admitted to hospital for a variety of reasons including:

• A more severe chest infection
• A planned course of intravenous antibiotics
• Minor surgical procedures
• General support and education about treatments to help establish good routines
• Other complications of cystic fibrosis.

For some this is a very rare occurrence and for others it may be a more regular event. One young person  who needs more regular treatment sees it as a ‘Spa treatment for her lungs’!  During your stay we encourage you/ your child to rest , sleep , eat and exercise , allowing us to support treatments when unwell and continue to educate you/ your child about all aspects of CF care during your stay.

If you/your child does need to come in, please bring all your medication, physio and nebuliser equipment with you, together with a small supply of any supplements taken and feed – this avoids any break in treatments if they are not immediately available from pharmacy. Any medication will need to be handed over to the nursing staff and will be kept in a locked cupboard. In particular CFTR modulator therapies will be difficult to get hold of quickly so please bring with you to avoid unnecessary treatment breaks.

Inpatient stays will be on Bramble ward (level 1, area H) in a dedicated side room with single use toileting facilities, usually en-suite. During the stay you will be seen by members of the CF team on a daily basis from Monday-Friday, with physio input available over the weekend.

Children requiring inpatient stay in North Devon will usually have a  planned  transfer to Exeter for ongoing inpatient care after stabilisation and discussion with the Exeter team.

All parents and  children will be advised on relevant infection control measures during their stay on the ward. Infection control is really important in keeping you and others safe so we encourage you to stay within your room , use good hand washing techniques and avoid chatting at the nurses desks. You will be allocated specific times to make use of the school room and play facilities to avoid contact with other CF children.

Meals cards will be available during your admission.

A small fridge will be available in your room for storage of  extra snacks.

Please be aware that our usual facilities are interrupted by the strict COVID regulations currently in place – we hope that usual services will restart as soon as possible to make your stay more comfortable.

Usual facilities include:

• Parents room with tea and coffee and a microwave
• School room and dedicated teachers who will liase with your school to support on going education
• Play specialists
• Play room for children – access will be limited to support infection control measures
• Physio gym

This is to make sure you are aware of how the ward is managed on day to day basis and how you can help with this.

Please click on the link to access our Code of Conduct. These documents make clear the professional standards you should expect from the CF Team and the conduct we expect from you and your child when your child is an inpatient

We know that the vast majority of patients and families already adhere to the statements in the Patient Code of Conduct but the documents available for everyone to avoid confusion. If you would like to discuss any related issues please do not hesitate to contact the CF team or Ward Matron.

All children with CF undergo a series of screening/monitoring investigations every year which are then discussed in a more detailed appointment called the Annual Review.  At this appointment  we assess progress over the previous year, screen for complications and conclude how things are currently. Treatment and any further investigation plans are then put together for the forthcoming year.

Leading up to your annual review we will request the following:

• A food diary (a detailed snapshot of everything eaten over a 3 day period) will be sent out well ahead of time (or can be accessed through the ‘Nutritional Resources’ link on the website) to allow our dieticians to assess your intake and nutritional needs.
• A Physiotherapy assessment to review your:
  • airway clearance method, nebuliser and inhaler technique – please bring all of your physio equipment to this appointment
  • postural and continence assessment
  • exercise review (including an exercise test so wear suitable clothing!)
  • A Cardiopulmonary excercise test (CPET) may be offered in older children giving  more detail  on how your lungs are functioning in exercise
• An annual Chest Xray
• Any additional imaging relevant for your individual care – this may include a liver ultrasound, (every few years in young infants, increasing to annually in older children), or a DEXA scan to look at bone strength
• Blood tests – these help to screen for evidence of allergic lung disease (ABPA),  liver and kidney function abnormailities and bone health, blood sugar abnormalities and CF related diabetes. Vitamins levels are checked to ensure we are giving you the correct amount of supplement. For many children and Parents this can be the hardest part of the annual review process, although others take it very much in their stride – we aim to work with you to find techniques that works best for you and  your child
• For those with concerns about CF related diabetes a continuous glucose recording may be requested.
• If you haven’t already met, our CF Psycologist will ask you to complete a screening questionairre for your annual review and will meet with you to talk over the impact CF is having for you and your child at this point in time and identify those who would like further support.. This is a really important part of the annual review process in supporting the whole family.
• We take this opportunity to review all aspects of care including school progress, research trials , microbiology results (germs on cough swabs/ sputums) over the year and number of days of IV antibiotic treatment

The annual review appointment itself will be a longer appointment  than usual so please allow extra time. We would encourage both parents and any additional  family members involved in care to attend if able.

At this appointment all the results are discussed and a clear plan of management for the following year is made. Children and families will of course have the opportunity to ask questions and raise any concerns and is often an important time for increasing young peoples understanding of their illness with maturity.

The review results, discussions and plan of care will be summarised in a letter and sent to you soon after the appointment.  Health details are added anonymously and with your consent, to the national CF registry database ,as part of the national audit of CF centre care