Cystic Fibrosis Information for Families

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Looking after children with cystic fibrosis (CF) requires a number of different health professionals working together as a team, often called a multi-disciplinary team (MDT) This consists of a consultant paediatrician, specialist CF and respiratory nurses, physiotherapists and a dietician. Further information regarding the CF team and what they do are included within this booklet.
We hope the following information will be helpful, If you have any questions please do not hesitate to contact us on one of the telephone numbers printed at the end of this booklet.

The Cystic Fibrosis Clinic
All children with CF should be seen routinely in an outpatient clinic every 2 months or so, extra visits maybe necessary if your child becomes unwell in-between visits. The purpose of a routine
outpatient visit is for the clinical team to check that children are as healthy as possible, particularly focusing on chest infections and nutrition. It is also an opportunity to check whether there are any problems with treatments.

The clinic visit includes:
• A consultation with a doctor
• Height and weight measurements
• A review with the Physiotherapist, including a cough swab or sputum sample
• An evaluation of your child’s diet with the Dietitian.
• A review by the CF specialist nurse. This could include a lung function test from the age of 5 years and a discussion of any issues at home or school.

If your child is well when they are seen we will usually make no changes to their treatment at the time of the clinic appointment. If their cough swab or sputum sample grows some bacteria we will
contact you by phone after the appointment and organise for them to start a course of antibiotics. If there has been a change to your child’s health, they may have a cough, or their lung function is not as good as it was, or their weight gain is not ideal, then treatment may be changed at the clinic appointment. This could be starting antibiotics straightaway, a change to how they are doing their chest physiotherapy, or a change in their diet. Sometimes if changes are made we may ask children to come back for a review before their next routine appointment to check that they are getting better.

Children up to the age of around 13 will be seen by the consultant with a parent present, when your child is aged around 13 years we like to introduce an initial 5 minute session when we will see your child alone, so that he/she can start to take on some responsibility for their own health. Parents will then be welcomed back and informed of their child’s progress. Please take advantage of your clinic appointment to air your concerns, collect supplies or arrange further appointments with the team.

Respiratory Nursing Team
The nursing team consists of two nurses, Terri James, an Advanced Respiratory Nurse Practitioner and Lorna Bass Paediatric Respiratory Nurse. Like our colleagues, our aim is to make life easier for you as a parent of a child with CF.

Although our role is extremely varied, an overview of our services are:

• To provide support, treatment and advice to children and families in their home.
• Inform and teach parents how to include CF treatment into their daily lives with minimal disruption.
• Liaise with all members of the CF team so that continuity of care is maintained.
• Support parents and carers through the difficult times e.g. on diagnosis, when the child is unwell, transition to adult care, etc.
• Maintain regular contact with the child so that any changes in health are identified early and appropriate treatment given.
• Act as a resource for information about CF to all involved in the child’s care, including the child themselves.
• Train and supervise you to administer medication as and when the need arises, including home intravenous therapy.

Terri James, Advanced Respiratory Nurse Practitioner Tel: 01908 996572 Bleep 1171
Lorna Bass, Paediatric Respiratory Nurse Tel: 01908 996572 Bleep 1171

Dietetic Service
My name is Jane Stanger. I am the Paediatric Dietitian who looks after children with CF.

As part of the CF team, I am involved in your child’s care from diagnosis and you will see me on most visits to the CF clinic. I am available to discuss all aspects of your child’s nutrition. I can give advice on Creon doses and how to maximise your child’s calorie intake to optimise normal growth and weight gain. If you have any questions about your child’s diet you can ask the CF Nurse to contact me. If I am not available, please ask for another Paediatric Dietitian to help.

Dietetic department
Tel: 01908 996004

The Physiotherapists look after all the patients with CF and are usually available in CF Clinic. There is always a physio on the Children’s Ward available to give your child his/her physiotherapy.
Exercise is really important as a child with CF gets older. Whenever your child is hospitalised we will encourage increasing the amount of physio given and the treatment sessions may be longer.
Physiotherapy is often seen as the most challenging part of having CF. We try to make it fun as it is something that has to be done every day. We encourage children to get into a routine as soon as
possible, which generally helps them to accept their physio as they get older.

If you have any worries or questions about physiotherapy, please do not hesitate to contact the Physiotherapist on 01908 995532.

Consultant Paediatrician
The Consultant Paediatrician involved in your child’s care is Dr Abraham Oommen. Dr Oommen will be involved in all aspects of your child’s CF care until he/she reaches 16. Your Consultant’s role includes the following:

 To take overall responsibility for the care of all children diagnosed with CF.

• To liaise with specialist CF centres to seek advice and provide appropriate care for your child.
• To regularly assess and monitor your child’s progress in the CF clinic or the Children’s Ward and start appropriate treatment.
• To provide support and advice to yourself and other members of your family.
• To prescribe and supervise treatment when your child is unwell.
• To liaise regularly with all members of the CF team to maintain a record of your child’s progress

Ward 4
There will be times when you will be visiting the Paediatric Assessment Unit (PAU) at Milton Keynes Hospital (Ward 4). This is usually if the Consultant or CF specialist nurse wishes to see you, or
if your child is unwell and you are unable to contact the CF specialist nurse.

You will have access to ward 4 at all times please phone the ward prior to your visit this will allow us to make appropriate arrangements. The ward staff will liaise closely with the CF team regarding the best treatment plan for your child. During your stay we hope to make you as comfortable as possible whether this is for a short visit or a longer stay (e.g. requiring admission for intravenous antibiotics) when you will be admitted to the Children’s Ward (Ward 5).

If there are times when none of the CF Team is available, any member of the nursing staff on ward 4 will be happy to see you.

Ward 4 (PAU)
Tel: 01908 996367 / 01908 996368
If your child has a clinical problem related to his/her CF please contact us on 01908 996574 and a member of the CF Team will contact you and arrange to see you as required. On weekends and
out of hours please phone the Children’s Ward on 01908 996368

CF Service Dr A. Oommen, Consultant Paediatrician 01908 996502
Terri James, Respiratory Advanced Nurse Practitioner 01908 996574 Bleep 1171
Lorna Bass Paediatric Respiratory nurse 01908 996574 Bleep 1171
Physiotherapists 01908 995432
Jane Stanger, Dietitian 01908 996004

Other Information
For the benefit of our patients, visitors and staff, Milton Keynes Hospital operates a smoke-free policy. This means smoking is not allowed on the Trust site, including all buildings, grounds and car
• Parking – Multi-storey Car Park close to the Luing Cowley Outpatient building.
• The Trust operates a Pay and Display Car Parking system.