Scottish Paediatric Cystic Fibrosis MCN

Protocols / Guidelines

Protocol for Annual Review Assessments

Author: SPCF MCN
Accountable Lead / Review Group:
Scottish Paediatric Cystic Fibrosis Managed Clinical Network Steering Group
Review Date: February 2013 / Last Update: March 2012
Contents: / Page Number

1. Background 3

2. Scope of the protocol 3

3. Key areas 3

3.1 Administration of annual review service 4

3.2 Place and timing of annual review 4

3.3 Medical and Nursing assessment 4

3.4 Blood Investigations 4

3.5 Radiology 4

3.6 Lung Function 5

3.7 Physiotherapy 5

3.8 Dietetic assessment 5

3.9 Psychology 5

3.10 Pharmacy 5

3.11 Annual review meeting and Report feedback 6

4. References 6

5. Stakeholders 6

Appendix 1 8

Appendix 2 10

Appendix 3 11

Appendix 4 12

Appendix 5 13

1.  Background

Annual reviews play an important part in the care of children with cystic fibrosis (CF). They allow a detailed assessment of every aspect of the children’s treatment and condition, and identify areas where treatment can be improved.

The purpose of this Protocol is both to ensure that all children with CF in Scotland have an annual review and to offer guidance on the content of the review process. Recommendations are made of investigations to be carried out and assessments to be made by CF Team members. Methods of feedback and communication are also discussed.

2.  Scope of the protocol

This Protocol relates to:

·  All children with CF in Scotland

·  All members of individual CF teams

o  Medical

o  Nursing

o  Physiotherapists

o  Dietitians

o  Psychologists

o  Social Work

o  Pharmacy

3.  Key areas

3.1 Administration of annual review service

3.2 Place and timing of the annual review

3.3 Medical and nursing assessment

3.4 Blood investigations

3.5 Radiology

3.6 Lung function

3.7 Physiotherapy

3.8 Dietetic assessment

3.9 Psychology

3.10 Pharmacy

3.11 Annual review meeting and report feedback

3.1 Administration of annual review service

Each CF centre will have a person responsible for coordinating the annual review appointments.

The members of the CF team will be informed in good time when the annual review is due to take place.

The annual review should take place no more than two months prior to the Joint CF multidisciplinary discussion with the Specialist Centre CF Team.

3.2 Place and timing of annual review

The annual review assessments and investigations will, whenever possible, take place in the local Centre as will the annual review follow up meeting. Some Centres will incorporate annual review investigations and assessments into the patients’ CF Clinic visits, while other Centres will offer separate appointments specifically for annual review. The date of the first assessment or investigation will determine the beginning of the annual review process.

3.3 Medical and Nursing assessment

·  History and clinical examination

·  Review of microbiology culture results in the previous 12 months

·  Review of number of oral and intravenous (IV) antibiotic courses

·  Discussion of fertility (from age 13 years)

·  Audiology assessment - as per local screening Protocol

·  Sputum (spontaneous or induced) will be collected for routine microbiological and atypical mycobacterial culture

·  Review of pubertal status as appropriate

·  Review of pancreatic function for PS patients, faecal elastase if indicated

3.4 Blood Investigations- (see Appendix 1)

3.5 Radiology

For all children:

·  Chest x-ray

There will be a CXR done annually which will be scored using the Northern Score at the Annual Review meeting. In addition, local teams may also wish to use other scoring systems e.g. modified Crispin-Norman score. Scores will be compared over time.

·  Ultrasound of liver and spleen, asking for assessment of portal blood flow

From 10 years:

·  Dual-Energy X-ray Absorptiometry

This will be at two yearly intervals unless abnormal results are found where the results will be discussed at the local bone health meeting.

3.6 Lung Function - For children age > 5 years

·  Full spirometry with Plethysmography

·  Incremental externally paced maximum exercise test

3.7 Physiotherapy

Detailed assessment by a CF-experienced physiotherapist (see Appendix 3)

3.8 Dietetic Assessment

Full assessment by CF-experienced dietitian (see Appendix 4)

3.9 Psychology

All patients should be offered a psychology review as part of the annual review process.

The exact nature of the assessment and who is involved will vary depending on the age and individual circumstances of the patient. It is recommended that the psychology review include an assessment of behaviour, mood and family functioning in order to assess both the psychological needs of the patient and the wider family system. It is recommended that this information be gathered by direct clinical interview and/or psychometric questionnaires.

Where clinically appropriate, information and recommendations from a psychological perspective should be communicated back to the CF Team (with respect to the boundaries of confidentiality) and incorporated into future treatment plans.

Appendix 5 – Psychology Assessment Pro-forma Guidelines

These guidelines have been developed in consultation with the Scottish CF Psychologists Group.

3.10 Pharmacy

All patients should be reviewed by a paediatric pharmacist at annual review. Where this is not possible aspects of the pharmacist review may be carried out by another member of the multidisciplinary team but each team will have a named pharmacist to contact if further information is required.

3.11 Annual review meeting and Report feedback

Once all the blood tests and reports are available the local CF team will meet with members of the Specialist Centre CF team to discuss the results, score the CXR and formulate a Final Report. This meeting should take place immediately before the next Joint clinic appointment for the patient.

The Port CF annual review page will be filled in before the CF Registry end of year deadline.

The formal report, including Key Recommendations and Action Points, will be discussed with the child and their family at the next Joint clinic appointment for the patient or within 3 months of the annual review date.

A copy of the written report will be sent to:

o  Family

o  Case record

o  General Practitioner

o  Specialist Centre CF team

o  Community Paediatric Team (if appropriate)

4.  References

Standards of Care - Standards for the Clinical Care of Children and Adults with Cystic Fibrosis in the UK 2001. May 2001. (pages 17 – 19) http://www.cftrust.org.uk/aboutcf/publications/consensusdoc/[Accessed 29/04/2009]

Cystic Fibrosis Annual Review. Royal Brompton & Harefield NHS Trust http://www.rbht.nhs.uk/healthprofessionals/clinical depts/paediatrics/childrencf/service/annual-review/ [Accessed 29/04/2009]

Managing passengers with respiratory disease planning air travel. British Thoracic Society 2004 http://www.brit-thoracic.org.uk/Portals/0/Clinical%20Information/Air%20Travel/Guidelines/FlightRevision04.pdf [Accessed 17/05/2010]

Journal of the Royal Society of Medicine 1996;89(suppl):3-7; 2001;94 (suppl):12-16

Archives of Disease in Childhood 2002;87:518-521)

5. Stakeholders

·  Patients

·  Families

·  General practitioners

·  Specialist CF Team

·  Local CF Team

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Ratified June 2011

Appendix 1

Blood Investigations

CF annual review investigations:
Condition / Marker
Liver disease / GGT
ALT/APT
ALP
Prothrombin Time
Platelets
CF related DM / Random or fasting glucose from age 5
OGTT from age 10
PseudoBarrter's / Na
K
Renal disease / BUN
Creatinine
Bone demineralisation / Corrected Ca/Phos/Mg
PTH
Vit D
Nutrition / Cholesterol
Albumin
Total Protein
Vits A/D/E/K
Vit E/Cholesterol ratio
Iron status / Ferritin
Hb
ABPA / Total IgE
Asp specific IgE / IgG
Surrogate markers of infection / WBC
Total IgG

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Ratified June 2011

Appendix 2

Physiotherapy Assessment

All patients should be reviewed by a CF specialist paediatric physiotherapist at annual review.

The network physiotherapist at the regional centre should take part in the annual review discussion and patient planning.

The physiotherapy annual review should, as a minimum, consist of:

·  Full respiratory examination

·  Cough swab/sputum sample sent to network centre laboratory

·  Review of physiotherapy techniques and follow up input as appropriate

·  Review and advice on activity levels

·  Review of spirometry where appropriate

·  Review of oxygen saturation

·  Postural review and input from 8 years

·  Continence review / input from 8 years

·  Formal exercise testing from the age of 7 years

·  Review of physiotherapy adherence

·  Assessment of understanding of disease and reasons for treatment where appropriate.

Appendix 3

Dietetic Assessment

·  Assessment of growth centiles with comparison to previous year

·  Assessment of BMI and BMI centile with comparison to previous year

·  Full dietary assessment

·  Review of calcium intake with reference to bone health

·  Review of fluid and fibre intake

·  Review of enteral feeding and oral calorie supplements if appropriate

·  Review of pancreatic enzyme therapy dosing, bowel habit and adherence to PERT therapy

·  Review of timing and adherence of vitamin supplements

·  Review of fat soluble vitamin status

·  Review of CF related diabetes management where appropriate

·  Measurement of tricep skinfold and mid arm circumference of children with CF related liver disease and others as appropriate

Appendix 4

Psychology Assessment

The overall aim of the assessment is to establish if there are any clinically significant psychological issues that are impacting on the patient/families ability to manage their condition, and to ascertain any clinically significant problems that are a result of the diagnosis, treatments or course/duration of the illness. As such, the following areas should be considered as part of the assessment process:

·  Patients developmental progress (e.g. social, behavioural, emotional, cognitive)

·  Patients understanding of CF and it’s associated treatments

·  Patient/families psychological well-being and identification of factors impacting on presenting mood/anxiety difficulties

·  Patient/families ability to cope with treatments/side effects and associated changes this brings (e.g. surgery/transplant/end of life care)

·  Impact of transitional issues (e.g. home to hospital; primary to secondary; paediatric to adult) on mood/adjustment

·  Impact of CF on overall family functioning and quality of life

·  Identification of psychological barriers that inhibit CF management as well as identification of strengths/coping strategies that enhance CF management

·  Wider support systems available to patient/family (e.g. school, peers, extended family etc).

Where clinically appropriate, the use of a paediatric CF specific quality of life measure (e.g. CFQ-UK), as well as a paediatric mood assessment screening tool (e.g. PIED), should also be considered. In accordance with clinical judgement, adult psychometric measures may also be administered to screen for parental mental health difficulties such as anxiety or depression (e.g. HADS).

Appendix 5

Pharmacy Assessment

The pharmacist review should, as a minimum, consist of:

·  Establish accurate medication history (including OTC medications and immunisations)

·  Update community pharmacy details if necessary

·  Check all doses for age and weight

·  Check any amendments to formulation required e.g. liquids to tablets

·  Identify any potential side-effects from medications

·  Update adverse drug reaction/allergy information e.g. any reaction in past year/since last review

·  Ensure patient/carer aware of indications/doses of all current medication and how to administer them. Address any training needs

·  Address any compliance and concordance issues

·  Identify any potential drug interaction and ensure appropriate steps taken e.g. change dose, additional monitoring

·  Check renal and hepatic function and adjust doses if necessary

·  Check vitamin levels (once annual review bloods available) and adjust doses accordingly

·  Discuss any supply problems experienced and advise and/or assist where possible e.g. liaise with GP/community pharmacy

·  Update GP (or practice pharmacist) with any changes from the annual review

·  Update community pharmacist if required e.g. change in supplier.

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Ratified June 2011