A Testing Summary of Nice Guidance 3

Public Health Wales / Coeliac Disease
Coeliac Disease
Quality improvement toolkit - Appendices
Author: Primary Care Quality and Information Service
Date: January 2011 / Version: 1
Status: Final
Intended audience:
Public (Internet) / NHS Wales (Intranet) / Public Health Wales (Intranet)
The former Public Health Wales Primary Care Quality Team, now incorporated within the Primary and Community Care Development and Innovation Hub, developed a series of quality improvement toolkits to assist practices in collating and reviewing information. From information received, practices still find these toolkits useful, therefore they will remain on this webpage for your ease of reference. Please note, however, that the date of publication is clearly stated in the toolkit and that the evidence within may have changed since publication
Purpose and summary of document:
This document is for use by general practices. The main aims of the toolkit are to raise awareness of Coeliac Disease and to improve identification, treatment and care of patients with the condition.
The audit toolkit will provide the user with a summary of the current evidence directing the safe provision of service, and a schedule of patient review criteria to compare current practice against those evidence based criteria. It will support practices to review and improve where necessary, the appropriate recording of information for care of patients with coeliac disease
Also included is a practice review section designed to encourage a whole practice response to the audit findings and an evaluation of the quality and usefulness of the audit itself.
Publication / distribution:
· Publication in PHW Document Database (Primary Care Quality and Information Service)
· Link from PHW e-Bulletin

Contents

Appendix Page

A Testing – Summary Of Nice Guidance 3

(Clinical Guideline 86)

B Care Pathway (NICE) 5

C Dietary Advice and Ongoing Management of 8

Patients with Coeliac Disease (Patient UK)

D Associated Conditions 11

E Further Information 12

F READ Codes 13

G Reflection Sheet 16

H Gluten Free Foods on Prescription 18

References 19

Material contained in this document may be reproduced without prior permission

provided it is done so accurately and is not used in a misleading context.

Acknowledgement to the Public Health Wales to be stated.

Appendix A

TESTING – SUMMARY OF NICE GUIDANCE (Clinical Guideline 86)

When to offer testing - Offer serological testing for coeliac disease to children and adults with any of the following signs and symptoms:

· Chronic or intermittent diarrhoea

· Failure to thrive or faltering growth (in children)

· Persistent or unexplained gastrointestinal symptoms including nausea and vomiting

· Prolonged fatigue (‘tired all the time’)

· Recurrent abdominal pain, cramping or distension

· Sudden or unexpected weight loss

· Unexplained low ferritin

· Other unspecified anaemia

Offer serological testing for coeliac disease to children and adults with any of the following conditions:

· Autoimmune thyroid disease

· Autoimmune parathyroiditis

· Dermatitis herpetiformis

· Irritable bowel syndrome

· Type 1 diabetes

· First-degree relatives (parents, siblings or children) with coeliac disease.

Serological testing may also be considered in the following:

· Addison's disease

· Amenorrhoea

· Aphthous stomatitis (mouth ulcers)

· Autoimmune liver conditions

· Autoimmune myocarditis

· Chronic thrombocytopenia purpura

· Dental enamel defects

· Depression or bipolar disorder

· Down’s syndrome

· Epilepsy

· Low-trauma fracture

· Lymphoma

· Metabolic bone disease (such as rickets or osteo-malacia)

· Microscopic colitis

· Persistent or unexplained constipation

· Persistently raised liver enzymes with unknown cause

· Polyneuropathy

· Recurrent miscarriage

· Reduced bone mineral density

· Sarcoidosis

· Sjögren's syndrome

· Turner syndrome

· Unexplained alopecia

· Unexplained sub-fertility.

Dietary considerations before testing for Coeliac Disease (CD)

Do not use serological testing for CD in infants unless gluten included in diet.

Testing for CD requires the patient’s diet to include gluten during the diagnostic process.

Patients should not start a gluten-free diet until diagnosis is confirmed by intestinal biopsy, even if a self-test or other serological test is positive.

Those following a gluten containing diet should eat some gluten in more than one meal every day for 6 weeks (min) before testing (the exact amount is not known).

Patients failing to reintroduce gluten into their diet before testing should be referred to a gastrointestinal specialist to confirm diagnosis of CD via intestinal biopsy.

Other information before serological testing

Inform people considering, or have undertaken, self-testing for CD (or parents or carers) that any result needs to be discussed and confirmed by laboratory-based tests. Before seeking consent to take blood for serological tests, explain:

· What coeliac disease is.

· Serological tests don’t diagnose coeliac disease, but indicate if further tests needed

· Implications of a positive test (need to refer for intestinal biopsy/familial possibilities)

· Implications of a negative test (that CD is unlikely but could present at a later date).

· Patients / parents / carers should know that a delayed diagnosis of CD can result in:

· Continuing ill health

· Long-term complications, including osteoporosis and increased fracture risk, risks in pregnancy / new babies and a modest increased risk of intestinal malignancy

· Growth failure, delayed puberty and dental problems (in children).

Serological tests

All tests should be undertaken in laboratories with clinical pathology accreditation (CPA).

Do not use immunoglobulin G (IgG) or immunoglobulin A (IgA) anti-gliadin antibody (AGA) tests in the diagnosis of coeliac disease. Do not use if self-tests and/or point-of-care tests for CD in place of laboratory-based testing. When clinicians request serology, laboratories should: (effective liaison between the practice and their supporting laboratory will assist with compliance)

· Use IgA tissue transglutaminase (tTGA) as the first choice test

· Use IgA endomysial antibodies (EMA) testing if the result of the tTGA test is equivocal

· Check for IgA deficiency if the serology is negative 1

· Use IgG tTGA and/or IgG EMA serological tests for people with confirmed IgA deficiency

· Communicate results clearly in terms of values, interpretation and recommended action.

Investigation for IgA deficiency should be done if the laboratory detects a low or very low optical density on IgA tTGA test or low background on IgA EMA test.

Do not use human leukocyte antigen (HLA) DQ2/DQ8 testing for initial diagnosis of CD, although its high negative predictability may assist gastrointestinal specialists in specific clinical situations.

After a positive serological test - Offer referral to a gastrointestinal specialist for intestinal biopsy to confirm or exclude coeliac disease to people with positive serological results from any tTGA or EMA test.

Referral - If serology tests are negative, but CD is still clinically suspected – offer

a referral to a gastrointestinal specialist for further assessment

Author: Primary Care Quality and Information Service / Date Dec 2010 / Status; Final
Coeliac Disease; Version 1 / 2 / Intended Audience; Public (internet) / NHS / PHW / PCQIS
Public Health Wales / Coeliac Disease

Appendix B

Care Pathway (NICE)

Appendix C

Dietary Advice and Ongoing Management of Patients with Coeliac Disease (Patient UK)

Starting a Gluten Free Diet rapidly induces clinical improvement, which is mirrored by the mucosa. The diet consists of no wheat, barley, rye, or any food containing them (e.g. bread, cake, pies). Moderate quantities of oats (free from other contaminating cereals) can be consumed as recent studies suggest that they do not damage the intestinal mucosa. Rice, maize, soya, potatoes, sugar jam, syrup and treacle are all allowed.

Gluten-free biscuits, flour, bread and pasta are obtainable through the NHS via prescription. Coeliac UK produces a prescribing guide 2
Patients should be encouraged to attend regular dietitian appointment. Even minor dietary lapses may cause recurrence. GFD should be life long, as relaxation of diet generally brings a return of symptoms and increased incidence of complications. Add supplements as necessary (e.g. folic acid, iron, calcium and vitamin D). Serial tTGA or EMA antibodies can be used to monitor response to diet.
Oral proteases are being developed (which digest gluten) and these may offer therapeutic options in the future

Continuous monitoring and treatment of patients with Coeliac Disease is vital to ensure people suffering from the condition stay well throughout. Patients should be monitored for life.

· The evidence indicated that patient compliance is poor with between 45-87% of those affected maintaining a gluten free diet. The long-term health risks of poor compliance include nutritional deficiency and reduced bone mineral density. Around 25% of patients with Coeliac Disease have osteoporosis of the lumbar spine, however bone mineral density improves significantly for those who maintain a gluten free diet 2

· Dietary compliance positively correlates with regular follow-up and knowledge of the condition.

· Half of all coeliac patients have an inadequate energy intake, 10% have inadequate intake of calcium and vitamin B6. 80% of elderly patients have inadequate intake of vitamin D.

· GPs are responsible for the appropriate prescription of gluten-free products

· Regular follow-up is an opportunity to provide patient-centered care that is sensitive to the individual's life circumstances.

· After diagnosis, the patient should be reviewed at the gastroenterology clinic after 3 months and 6 months to ensure they are making satisfactory progress and managing the diet.

· If well, they should be reviewed annually or sooner if problems arise - follow-up assessments are currently being carried out by dietitians, nurses, general practitioners and gastroenterologists in primary and secondary care

Annual Review should include;

Assessment of Disease status: weight, height, body mass index

Symptom assessment;

Bowel function (stool frequency and consistency, blood) abdominal pain

Investigations:

· Haemoglobin

· Red cell folate

· Serum ferritin

· Serum albumin

· Alkaline phosphatase.

Patients with Coeliac Disease who adhere to a gluten free diet often eat inadequate intakes of folic acid and iron. Low haemoglobin, red cell folate, and serum ferritin may suggest persisting mal-absorption and warrant further assessment.

Antibody tests can be used to monitor significant dietary gluten ingestion

Disease prevention:

· Osteoporosis risk assessment and management: Consider measuring bone mineral density (DEXA scan) at diagnosis (depending on age), and the test should then be repeated:

· For women – at the menopause

· For men – at the age of 55

· At any age if a fragility fracture is suspected (follow osteoporosis guidelines).

Advice should include;

· Regular physical activity

· Reduce smoking and alcohol consumption

· Calcium and / or vitamin D supplementation – calcium and Vitamin D can be prescribed if dietary intake is inadequate or the patient is housebound

· Bisphosphonates or strontium etc. should be considered if the patient is osteoporotic - see osteoporosis guidelines.

Hyposplenism

Some degree of splenic atrophy is present in most patients with Coeliac Disease, and is sufficiently severe to cause peripheral blood changes in about a quarter. Patients should be considered for:

· Vaccination against pneumococcus and Haemophilus influenzae (type b)

· Vaccination against influenza.

· Guidance about the increased risks attached to tropical infections, e.g. malaria .

· Life-long prophylactic antibiotics are not recommended.

Medical care:

The management of associated medical problems should include;

· A discussion of familial risk if required (First-degree relatives of people with CD have a 1 in 10 chance of developing the disease, and should be screened).

· Review prescription items (Primary Care Society for Gastroenterology Guidelines suggest minimum monthly prescription requirements, so discuss prescribable

Items with any patients using fewer than these recommendations

Self-care: Patient discussion should include;

· Gluten free diet - compliance and advice.

· Membership of the Coeliac Society.

· Use of the Coeliac Society's Gluten-Free Food and Drink directory.

· Dietary advice on weight, macronutrients, calcium, vitamin D, iron and fibre intake as required.

Specialist care, patients should be referred for specialist care where there is;

· A poor response to a gluten free diet

· Weight loss on a gluten free diet

· Blood in stools

· Onset of unexplained abdominal pain

· Other clinical concerns

Screening

Coeliac Disease appears to be under-diagnosed in primary care

· GPs can start to reverse this trend by screening for Coeliac Disease by targeting patients with autoimmune disease, unexplained anaemia, unexplained osteoporosis, chronic abdominal pain or other bowel symptoms (e.g. IBS), or family history of the condition.

· Childhood screening is currently being considered. NICE recommends screening children with type 1 diabetes for Coeliac Disease every 3 years until adulthood, and subsequently if adults have a low body mass index or develop unexplained weight loss.

Complications

· Osteoporosis

· Cancer risk - Some research fails to show any increased risk, whilst other papers have shown a very modest increase in overall risks of developing malignancy, e.g. gastrointestinal cancers and several types of lymphoma (risk greatest in first year after diagnosis). Paradoxically patients with Coeliac Disease may have a reduced incidence of breast and lung cancers – the reasons for which are not known. Intestinal lymphoma usually presents with return of bowel symptoms, although usually responds poorly to treatment. http://www.patient.co.uk/doctor/Coeliac-Disease-(CD).htm

Appendix D

Associated Conditions

Consider offering serological testing to children and adults with any of the following
Addison's disease
Amenorrhoea
Aphthous stomatitis (mouth ulcers)
Autoimmune liver conditions
Autoimmune myocarditis
Chronic thrombocytopenia purpura
Dental enamel defects
Depression or bipolar disorder
Down’s syndrome
Epilepsy
Low-trauma fracture
Lymphoma
Metabolic bone disease
(such as rickets or osteomalacia) / Microscopic colitis
Persistent or unexplained constipation
Persistently raised liver enzymes with unknown cause
Polyneuropathy
Recurrent miscarriage
Reduced bone mineral density
Sarcoidosis
Sjögren's syndrome
Turner syndrome
Unexplained alopecia
Unexplained sub-fertility

Appendix E – Further Information