Scottish Paediatric Cystic Fibrosis Managed Clinical Network

Minutes from above meeting held at 1.00 pm – 3.00 pm on Friday 24 August 2012 in The Board Room, Perth Royal Infirmary.

Present: Dr Richard Brooker MCN Clinical Lead, RACH, Aberdeen Dr Julie Stephen Clinical Psychologist Dr Alison Coates CF Dietician, RHSC, Edinburgh Dr Alan Webb Paediatrician, Raigmore, Inverness Susan Grant Parent, Edinburgh Greg Fearn PCF MCN Manager Dr Andrew Fall CF Associate Specialist, RHSC, Edinburgh Dr Tim Adams Paediatrician, Ayrshire & Arran Dr Jane Wilkinson CF Associate Specialist, RHSC, Glasgow Helen MacFarlane Director, The Butterfly Trust

Apologies: Gail Milne CF Nurse Specialist, Dundee Katharine Fok Pharmacist, RHSC, Edinburgh Dr Marzi Davies Radiologist, RAH, Paisley Kathryn Sharp CF Physiotherapist, RHSC, Edinburgh Dr Ian Gould CF Microbiologist, ARI, Aberdeen Dr Edward Doyle Medical Director, SEAT PRG Dr Alastair Innes Respiratory Physician, Western Infirmary, Edinburgh Dr Greg Hunt Paediatrician, RAH, Paisley Dr Tom Marshall Respiratory Paediatrician, RHSC, Edinburgh Yvonne Hughes CF Expert Patient Advisor, CF Trust Elizabeth Johnston Parent, Paisley

In Attendance: Catriona Johnson Programme Manager, National Services Division James Thom Data Manager, Glasgow Becky Bolger Administrator, National Managed Clinical Networks

Chair: Dr Richard Brooker

1. Welcome to new members / apologies Action Apologies were received and noted as above.

R Brooker introduced James Thom, Data Manager for the Clinical Audit System (CAS) project, to the group.

2. Minutes from meeting 11 May 2012 Minutes from the previous meeting were accepted as an accurate record of proceedings.

3. Matters Arising

3.1 Newborn Screening The screening laboratory is revising its communication processes as there is currently no mechanism for parents to be informed of normal second IRT results. The group discussed whose responsibility it is to communicate with the parents,

MCN/Paeds CF/CF Steering Group/2012/240812/Minute 1 whether primary or secondary care. Dr Stephen commented that parental anxiety could be raised if a CF specialist is involved when CF is not diagnosed. In such cases it would be better communicated by a GP. The group suggested that the CF centre be copied in on the letter that goes to the GP. C Johnson will translate the suggestions and comments into a pathway and forward to the CJ group for approval.

Microbiology Update 3.2 Dr Gibb, clinical lead for the Microbiology MCN, has identified a team leader for a subgroup of their network to develop guidelines on laboratory processing of samples, but SPCF MCN is not aware of further progress. S Grant felt that this is a priority and that progress appeared to be slower than anticipated, to which Greg Fearn concurred. C Johnson will contact the Scottish Government to ascertain if this remains part of the Microbiology MCN workplan. CJ

Training Needs Analysis 3.3 The MCN will distribute the NES generic TNA to as many people as possible involved with paediatric CF. Dr Brooker requested that members of the steering group cascade the TNA to colleagues, who may not be captured in the email addresses known to the MCN, as a high response rate is desirable. BB Service Mapping 3.4 Apart from the very small units, all of the CF clinics and centres have been visited (proformas have been sent to the small units and a response is awaited from one). Each centre will receive a draft report for comments in due course following which an overall report will be drafted. There was a short discussion on RB/GF where this report should be sent, but this needs to be considered further at a later date. Dr Brooker explained that the initial findings show improvement in the CF clinics but there are difficulties, particularly in the CF centres, and thanked the group for their contributions to the project. A spreadsheet summarising the staffing levels, corrected for case load, and BB service provision is being developed which will be available along with the report. The spreadsheet will allow comparisons between the CF clinics and centres

3.5 Protocols i. IV antibiotics for pulmonary exacerbations – Dr Devenny has emailed a RHSC(G) report on ‘Hospital versus Home IV Treatment’ to Dr Hunt and offered assistance in progressing this guideline. AD ii. Pseudomonas aeruginosa eradication - Dr Brooker has emailed all paediatric and adult clinics/centres for copies of their current eradication guidelines with a good response rate. This is an outcomes driven protocol and audit data will also be sought before RB developing the guideline. Dr Fall clarified that Edinburgh are taking part in the Torpedo study. iii. Central Venous Access Device – Dr Wilkinson has met with G JW Milne and G Fearn. Due to difficulties in agreeing a consensus of minimum standards an audit of current practice will be initiated in the first instance. Dr Wilkinson will forward communication to G Fearn from G Milne. iv. Psychology Protocol – Dr Stephen explained that a provisional protocol on the role of psychology in CF which will be circulated to the Scottish CF psychology group for comment in the first instance. v. Dietetics Protocol – A Coates will finalise the dietetics protocol on AC Dietetic Assessment of Children with CF and Nutritional Interventions for Children with Cystic Fibrosis.

MCN/Paeds CF/CF Steering Group/2012/240812/Minute 2 vi. Scottish CF formulary – Dr Wilkinson will contact K Fok for an JW update on the development of a CF formulary. It was noted that Aberdeen had supplied their antibiotic formulary for possible incorporation.

4. Ivacaftor The steering group has been asked to provide a position statement on the possible use of Ivacaftor. This new treatment, for patients over the age of 6 years with the G551D mutation, has now received European Medicines Agency approval. Ivacaftor will not be appraised by NICE but the Specialised Commissioning Groups in England have commissioned a clinical and economic evaluation with the report expected in the early autumn 2012. Ivacaftor is expensive at £14k per patient per month and Scotland has a higher prevalence rate for the G551D mutation than England.

C Johnson has received an email from Mike Winter, Medical Director NSD, asking for the input of the MCN on three possible outcomes in Scotland:  Approved with restricted access  Patient Access Scheme Assessment Group (PASAG) approved  Scottish Medicines Consortium (SMC) do not recommend its use

Prior to the steering group meeting an email was sent to all of the lead clinicians asking for their views but only one response was received. The unanimous view was that the clinical benefits of the drug were in favour of it being approved. As the MCN looks to represent the patient’s interests, it was agreed to support the use of Ivacaftor, for children over 6 years of age, who have the specific G551D mutation. There was further discussion about the high cost of Ivacaftor and how new funds would need to be identified by the Scottish Government Health Department. Dr Brooker will draft a position statement for consideration by the RB steering group.

5. Audit Sub-Group G Fearn circulated an audit subgroup proposal for consideration (appendix 1). This item was deferred due to lack of time.

6. Clinical Audit System and Port CF Dr Brooker and G Fearn explained to the group that NSD require networks to make faster progress in producing data to evidence their effectiveness. This stems from demands from the Scottish Government for networks to be able to demonstrate that they are having a positive impact on services.

The CF Registry has recently made changes to the Port CF data fields and the CF Trust is beginning to consider other data fields that could inform on the quality of CF services across the UK. This could potentially be an opportunity for the MCN to influence the CF Trust to meet the MCN’s need to provide Clinical Quality Indicator (CQI) reports in the future, but not in the short term. Currently the CF Registry could provide data for 4 of the 9 CQIs.

The group discussed the difficulties of individual centres entering data into the CF Registry. Consideration was given to whether the Scottish paediatric clinics/centres should stop entering any data into the CF Registry, but this was rejected as there is an ongoing need to be able to compare Scottish CF health outcomes to the rest of UK. Therefore, it was agreed that from now onwards only annual review data should be entered into the CF Registry .

MCN/Paeds CF/CF Steering Group/2012/240812/Minute 3 The following options for data collection were considered:

1. Developing a new database based on the one used by Dr Adams in Ayrshire & Arran. 2. Using the Clinical Audit System (CAS). G Fearn and J Thom gave a demonstration of the latest version of CAS. J Thom described the background to the system and that support is also offered by the data manager in terms of entering initial data and data analysis. The steering group agreed that CAS would need to be piloted prior to a decision being made but the time constraints would not allow this to take place. 3. Using the relevant CF Registry data that is currently entered at annual review and supplementing this with the small amount of additional information required.

It was agreed that the MCN will not continue to develop CAS and that option 3 was the best interim solution . A new proforma will be created and this, together with copies of the Port CF annual review proformas, will be returned to the MCN administrator for data entry into a simple spreadsheet that will be sufficient to RB/GF provide statistical analysis for each of the CQIs.

7. Public Involvement S Grant updated the group regarding the progress of the parent / carer group in the East of Scotland. The first meeting is to be held on 25 September at the Sick Kids in Edinburgh. Those invited were those who expressed an interest in such a group at the EoS Education Evening held in May. S Grant is hopeful that between 20-25 carers will attend. The meeting will be structured in two parts with a guest speaker attending the first half of the meeting and the remainder comprising informal discussion.

8. Primary Care Dr Brooker explained the MCN should cross all levels of care including primary care. GPs have been identified in Tayside and Grampian and the steering group should forward the names of GPs they know who would be interested in assembling a primary care sub-group. Dr Fall mentioned an interested GP from Stockbridge in Edinburgh. G Fearn will follow this up. GF/AF

9. AOCB G Fearn read to the group a statement from the Scottish Newborn Blood Spot Screening Programme concerning the unexpected decline in the number of new CF cases detected since October 2011. A short life expert group has investigated this and can find no evidence of laboratory error in the Immune Reactive Trypsin testing process. There has been no evidence from the CF clinics and centres of cases being missed. The statement closed by reporting that the detection rate had picked up since June 2012 but clinicians should remain vigilant for late presentations.

10. Date and Time of Next Meeting Friday 23 November 2012, 1pm-3pm, the Boardroom PRI. Videoconferencing will be available.

MCN/Paeds CF/CF Steering Group/2012/240812/Minute 4 (Appendix 1)

Proposal for a PCF MCN Audit Subgroup

It is proposed that the MCN develops some form of subgroup to oversee and undertake work relating to clinical audit. The format of this group needs to be discussed and decided by the Steering Group. This could be a formal subgroup, work led and coordinated by a single clinician or part of an extended Steering Group a couple of times a year. The remit of the group will need to be decided by the Steering Group but could include audits, standard and protocol development, data collection and clinical system development. Below are some suggested areas of work that are currently underway in some form, but could come under the remit of the subgroup.

Suggested Area of Work Progress to date Desired Outcome 1 Develop a robust approach to data collection, It has been agreed that the CAS implementation will The MCN will have a particularly in relation to the Clinical Quality focus on robust collection of data at annual review, in structured approach to data Indicators agreed by the network. line with our new protocol and agreed CQIs. A collection, analysis and dataset to aid data collection has been agreed. reporting, based on the agreed CQIs. 2 Auditing the Clinical Quality Indicators The network can currently audit 4 of our 9 CQI’s The MCN will have robust from Port CF. The rest will be done via a audit data from all treatment structured audit of 20-25% of all records from centres in Scotland, which each treatment centre. will be reported nationally. 3 Protocol Audits The network has been active in the production of Specific protocol audits protocols covering our relevant specialties. There should be developed by is a requirement to measure the effectiveness of each specialty subgroup the protocols via clinical audit

4 Central Venous Line Audit It has been agreed to look at practice throughout Establish a national Scotland in order to inform the development of overview of central line the guidelines which the Nursing Subgroup has management. been working on.

MCN/Paeds CF/CF Steering Group/2012/240812/Minute 5