CHILDREN AND YOUNG PEOPLE’S

SPECIALISED SERVICES PROJECT (CYPSSP)

All Wales Endocrine Standards for

Children and Young People’s Specialised Healthcare Services

Consultation Document

2008

Foreword

Edwina Hart, AM, MBE, Minister for Health and Social Services

The best investment we can make in the future of Wales is ensuring that high quality and equitable services are provided for our children and young people. The Welsh Assembly Government is committed to ensuring these services are in place.

This document is one of a series, which address the specific needs of the children and young people who require specialised healthcare services. This series has been designed to compliment the National Service Framework for children, young people and maternity services in Wales.

I encourage you to participate in this consultation to help us ensure that these standards have the utmost impact on the health and wellbeing of our children and young people with specialised healthcare needs.

Edwina Hart AM, MBE

Minister for Health and Social ServicesCONSULTATION QUESTIONS

  1. Please state your name, title and place of work (please also state if you want your name withheld from the publication of the results of this consultation).
  1. Some of the key actions within this document have been identified for delivery within 1 year of publication of the standards. Implementation of these key actions has been estimated to be low or no-cost because they are organisational issues or are already being planned for, or implemented, in many areas.

Do you agree that the flagged early implementation key actions are the right ones to prioritise for early delivery? Please state which of the flagged key actions you do not believe can be delivered by this deadline and why. Please also state if there are any other key actions which you believe could be delivered by the end of the first year following publication.

  1. Each key action has the organisations which are responsible for their delivery clearly identified.

Are there any key actions which you feel that the organisation you work for is not responsible for delivering? Are there any key actions which you feel your organisation or another organisation could contribute to delivering but has not been listed next to a key action?

  1. When you read both the universal and service specific documents, please consider the following;

Are there are important universal or service specific NEEDS of children, young people and their families which you feel have not been addressed by the key actions in these standards document?

  1. Every attempt has been made to make each key action clear, specific and measurable to allow for easier audit.

Are there any key actions which you feel do not meet the above criteria? If so, can you suggest a form of wording that would improve the key action?

  1. These documents are aimed at service commissioners and providers, however we have developed the documents with the help of children, young people and their families and therefore hope that they are easily understandable. We have identified some key words in the glossary of each standards document.

Are there any terms or phrases used within this document that have not been included in the glossary, but which you feel require definition?

  1. Can you foresee any barriers to the implementation of these standards? If so, are there any actions which the Welsh Assembly Government should take to help overcome these barriers?
  1. From those key actions which are developmental key actions please select the three that you feel will have the greatest impact on the specialised services for children and young people or are essential to develop and rank them in order of priority.
  1. Do you have any other comments that you would like to make about these document?

The consultation period will come to an end on the 22nd September 2008. Please send comments to the address below by this date.

Michelle Grey

Children’s Health and Wellbeing Branch

Health and Social Services

Welsh Assembly Government

2nd Floor

Cathays Park

Cardiff

CF10 3NQ

or to

Children and Young People’s Specialised Services

Introduction

In 2002, the Specialised Health Service Commission for Wales undertook a review of specialised healthcare services for the children and young people of Wales, which identified that these services were delivered in an ad hoc and fragmented way.1, 2 Following this review, the Minister for Health and Social Services announced that Managed Clinical Networks (MCNs) would be developed to deliver specialised healthcare services for children and young people. 3

The Children and Young People’s Specialised Services Project (CYPSSP) was established by the Welsh Assembly Government (WAG) to take this work forward. The project’s remit was to:

Develop high quality, equitable and sustainable specialised children’s health services across Wales based upon the best available evidence and with children and their carers at the centre of all planning and provision.

This would be achieved by the following aims:

  • To develop service specific standards for Children’s specialised services for the children and young people of Wales
  • To enable equity of access through effective managed clinical network models for all children and young people in Wales requiring specialised services.

The agreed specialised services for the project are:

Paediatric Critical Care (standards already published)

Neonatal Services

Paediatric Neurosciences

-Neurosurgery

-Neurology

-Neurodisability

Paediatric Oncology

Paediatric Palliative Care

Paediatric Anaesthetics and Surgery

- Anaesthetics

-General surgery

-Trauma and Orthopaedics

-Ear Nose and Throat

-Ophthalmology

-Maxillofacial

-Cleft Lip and Palate

-Plastic Surgery

-Burns

Nephrology

Cardiology and Congenital Cardiac Services (including access to Cardiac Surgery)

Endocrinology

Gastroenterology/Hepatology and Nutrition

Inherited Metabolic Disease

Respiratory

The Standards Documents

There is a standards document for each particular specialised service containing key actions (KA) related to that specific service. There is also a universal standards document, which applies to all specialised services included in the project. The standards documents are written from an All Wales perspective and apply to all children and young people with that particular health need, wherever they live in Wales. 4, 5, 6

These standards should be read and used in conjunction with the National Service Framework for Children, Young People and Maternity Services in Wales (Children’s NSF), 7 in particular Chapter 2, “Key actions universal to all children” which is relevant to all services and all children and young people.

The standards and key actions within the CYPSSP documents apply to all children and young people accessing that specific specialised service who are between the ages of 0-18 years of age. However, key actions that relate to transition apply to all young people who may require ongoing services beyond this age range. The age for transition to adult services must be flexible to ensure that all young people are treated by the most appropriate people and in the most appropriate setting. This will also depend on the young person's own mental, emotional and physical development.

To aid effective communication across primary, secondary and specialist healthcare, it is proposed that a directory of specialised services will be developed which will be updated annually.

Purpose of Standards

The standards and their key actions have been developed to provide a basis for service commissioners and providers to plan and deliver effective services. 8. 9 They are to be used to benchmark current services and then inform the development of robust future services to meet the specialised health needs of children and young people across Wales. 10

Developing the Standards

The standards for each service have been developed by an External Working Group (EWG) representative of key stakeholders. Membership details are included as Appendix One in each of the service specific standards documents.

The contribution made by EWG members is greatly appreciated. We are particularly grateful to all the children, young people and parents who have been involved in the development of this work. 11, 12

The standards have been Quality Assured by a Project Steering Group (SG) comprising of strategic stakeholders, the details of which are included as Appendix Twoin the service specific standards documents (or Appendix One of the Universal standards document).

Since the CYPSSP commenced in 2003, three project managers have successfully managed and facilitated the development of each of the standards documents. We would like to extend our grateful thanks to each of the Project Managers, namely Eiri Jones, Sian Thomas and Mary Francis for their contribution to this work.

Delivering the standards

Some of the key actions can be delivered within the first year, however due to workforce and financial constraints others will take a number of years to achieve. Thus each key action has a timescale for delivery between one and ten years.

Every attempt has been made to ensure that the key actions are clear and measurable, however when terms which cannot be measured, such as ‘timely’ and ‘appropriate’ have been used it will be for the specific MCN to agree on the acceptable definition of the term. This will allow each standard and key action to reflect the particular needs of the individual specialised service.

Whenever "children" are referred to in this document it should be accepted that this also includes young people. Reference to "parents" includes mothers, fathers, carers and other adults with responsibility for caring for the children.

Monitoring the standards

Standards will be monitored and audited annually. Audit programmes will be funded as part of the MCN arrangements and will include comprehensive audit of training, practice and compliance with pathways, protocols and agreed outcomes.

Managed Clinical Networks (MCNs)

MCNs can be defined as:

“Linked groups of health professionals from primary, secondary and/or specialist care, working in a co-ordinated manner, unconstrained by existing organisational boundaries, to ensure equitable provision of high quality and clinically effective services.” 13

Through the formal establishment of an MCN, children and young people requiring specialised healthcare will do so based on the following principle:

Age appropriate, safe and effective (high quality) care delivered as locally as possible, rather than local care delivered as safely and effectively as possible.14.

Dental Care

Although dental care has not been addressed separately it is important to recognise that oral healthcare is a significant consideration for all children and young people but because of their medical conditions many of the children and young people requiring specialised healthcare services will:

  • be at higher risk of oral disease and oral complications than the average child population e.g. children who are unable to feed by mouth and those on certain medications, which can cause side effects in ‘the mouth’.
  • be at higher risk when treated for oral disease e.g. children with respiratory disorders requiring general anaesthetics and children who have had cardiac surgery.

They may also find the management of their dental treatment difficult, for example, because of associated learning disability or general anxieties towards medical/ dental intervention.

Prevention of oral and dental disease is therefore highly desirable for this group of children and thus preventative oral healthcare advice should be part of every child’s overall care plan so that families and carers are well informed as to the specific risks for each child. Specific oral assessment and care should also be available where appropriate.

To facilitate this it is essential that the dental team is considered an integral part of the multidisciplinary approach advocated throughout this project. There should also be a named dentist with specialised skills and knowledge in the oral healthcare of children e.g. a Specialist in Paediatric Dentistry linked to each large District General Hospital (DGH) to provide support and advice to the broader teams and ensure referral of children for appropriate healthcare.

PAEDAITRIC ENDOCRINE SERVICES

Paediatric endocrinology is concerned with the diagnosis and management of children and young people with hormonal disorders (including growth problems). Many specialist paediatricians also care for patients with diabetes mellitus, as unlike adult diabetes services, paediatric diabetes is never managed exclusively in primary care.

Diabetes can be managed in secondary care, as long as clinics have an appropriate caseload and contain trained medical, nursing, dietetic and psychology staff. As treatment and follow-up are life-long, planned transition to adult services (via joint and/or hand-over clinics) is necessary. Specialised services may be required for the following:

  • Diabetes complications in childhood
  • Type 2 or rare forms of diabetes (e.g. Maturity onset diabetes MODY)
  • Diabetes associated with Chronic Disease e.g. cystic fibrosis or high dose steroid usage e.g. in the treatment of some cancers. 15

Paediatric endocrine care is delivered by professionals working in many different areas of child health services. Most common conditions are managed in a primary or secondary care setting. Some of the more complex and rare conditions need to be managed in conjunction with a specialised children’s endocrine service.

It is noted that a significant number of cases are variations of normal development and can be managed locally. In some cases local investigations may reveal more complex conditions requiring referral to specialist services.

Common Endocrine Disorders

Common endocrine disorders include the following, as defined by the Royal College of Paediatrics and Child Health;16

  • Short and tall stature
  • Delayed puberty in boys
  • Obesity
  • Variations in pubertal development in girls
  • Hypothyroidism
  • Idiopathic isolated growth hormone deficiency.17

A minority of these children may require further detailed investigation and the more complex or difficult cases will require the expertise of the specialised service.

Uncommonor Complex Endocrine Disorders

A number of rare or more complex conditions require specialised expertise and should be managed by, or in conjunction with, an endocrinologist. Examples of these include:15

  • Adrenal disorders (including CAH)
  • Thyrotoxicosis
  • Turner Syndrome
  • Hypopituitarism
  • Precocious puberty in boys
  • Rare endocrine tumours
  • Disorders of calcium and bone metabolism
  • Hypoglycaemia (non- Diabetes related)
  • Disorders of sexual development
  • Ambiguous genitalia
  • Endocrine Disorders Associated with Chronic Disease

-Growth and pubertal problems associated with chronic renal failure and inflammatory bowel disease

-Care of endocrine problems in cancer survivors

-Cystic fibrosis related diabetes.

Adult Endocrine

It is vital that close relationships exist between paediatric and adult endocrinology services to ensure successful collaboration and transition planning.

Clinical Biochemistry

It is recognised that close liaison with specialist clinical biochemistry is integral to paediatric endocrinology.

As with other documents, many diseases involve more than one system. There is a significant interface with other specialised services, in particular:

  • Cardiology
  • Metabolic
  • Dermatology
  • Haematology
  • Gastroenterology
  • Neurology
  • Nutrition
  • Neonatal
  • Critical Care
  • Genetics
  • Palliative Care
  • Nephrology
  • Paediatric Surgery
  • Gynaecology
  • Neurosurgery
  • Oncology
  • Respiratory
  • Radiology
  • CAMHS/ Psychosocial Support

Standards documents from these services may therefore apply here.

Current key documents have informed the development of this document. These include:

  • Commissioning Tertiary and Specialised Services for Children and Young People (2004), Royal College of Paediatrics and Child Health, London16
  • Type 1 diabetes: diagnosis and Management of Type 1 Diabetes in Children and Young People, (2004) NICE 18
  • Consensus Guidelines for the Management of Type 1 Diabetes in Children and Young People, (2006) Brecon Diabetes Group (draft) 15
  • Obesity: the prevention, identification, assessment and management of overweight and obesity in adults and children, (2006), NICE. 19

Some children will need to access services identified as “supra-regional”. These are highly specialised Endocrine services. Access to these services for the children of Wales will be through the identified lead centre.

PAEDIATRIC ENDOCRINE SERVICE MODEL

Current Service Model

Shared care and informal networks in endocrine services are well established across secondary and tertiary services for the children and young people in Wales.

South & Mid Wales

Common Endocrine Disorders

Children and young people are seen at their local district general hospital (DGH) and referred on to the lead centre (UHW, Cardiff) if necessary. Children with diabetes are looked after within their own local services

Uncommon or Complex Endocrine Disorders

Children and young people requiring specialised care for complex endocrine disorders are seen at their local DGH and / or at UHW. If seen locally, the lead centre clinicians support this care through outreach clinics; but at present other members of the MDT do not accompany them.

The lead centre also offers the following services:

  • Telephone advice service – 24 hr telephone advice from the consultant in the lead centre already exists.
  • Access to specialised nurse advice is currently available three days a week during the hours of 9am-5pm.
  • Transfer Clinic- this is a handover service for young people with endocrine disorders entering the adult services. This service currently only exists at the lead centre.
  • Specialist clinics for young people with disorders of growth associated with renal and gastrointestinal disease, cancer, adolescent gynaecology, genetic disorders and metabolic bone disease are currently held in the lead centre.

North Wales