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DEBRA Members’ Weekend 2017 Constipation and general Q&A

EB Specialist Clinical Panel

Constipation and general Q&A

EB Specialist Clinical Panel

Nutrition clinical practice guidelines

Chapter 1: Management of Constipation for children and

adults with EB

Background-Why constipation? • It affects people with different types of EB • It is a potential source of real pain and distress • Prevention & treatment are inexpensive and

effective with the correct advice on fibre, fluid and appropriate use of macrogols

• Identified as an issue from initial scoping meeting

Aim of guideline is prevention

• Reduce the risk of chronic constipation, faecal impaction, pain and rectal bleeding

• To promote increased oral intake of fluid and fibre-containing foods

• To promote increased bowel movement that is soft

• It is not a guideline for diagnosis or pharmaceutical management.

Who is it for?

• Clinicians- doctors, nurses, dietitians, allied health professionals

• People living with all types of EB • People involved in the care and support

of people living with EB

What is constipation? • Fewer than 3 bowel movements (BMs) per

week for adults1,2,3 • Fewer than 4 BMs per week for

children1,2,3,4 • Hard stool in more than 25% of BMs1,2,3 • A sense of incomplete evacuation in more

than 25% of BMs1,2,3 • Excessive straining in more than 25% of

BMs1,2,3 • A need for digital manipulation to facilitate

evacuation1,2,3

What is the incidence of constipation in EB?

• RDEB-SG 75% • RDEB other 40-54% • DDEB-9-46% • JEB-gen int- 20-33.3% • EBS-sev gen-25-37.9% • EBS-gen int-7-18.9% • KS=50% Ergun 1992, Freeman 2006, Fine 2008,

Horn 2002 Level 2+ evidence, level C.

What are the causes of constipation in people with EB?

1 Low fibre intake- •avoidance of acidic, hard, sticky husks, sharp or dry foods e.g. citrus fruit, peas, wholemeal bread. •Dental caries-reduced chewing and biting •Prioritising energy/protein dense foods Allman 1992 level 2+, grade C

2 Inadequate fluid intake

• Increased requirements from exudate loss or increased temperature

• Reduced intake from oral or oesophageal blistering

• In children parents reduce fluids so space for food, school may not encourage drinking, considerations of night time enuresis.

3 Fear of pain • Passage of a motion may cause abdominal pain, anal pain &

perianal blistering resulting in avoidance of defaecation • Cycle of constipation in adults and

children • In children with-holding the motion can

become an issue of control

Other causes

• 4 Medication- opiates & codeine reduce peristalsis. Amitriptyline and oral iron

• 5 Mobility- stimulates peristalsis & maybe reduced

• 6 Irregular eating pattern 2ndry to oral and oesophageal blistering

Prevention of constipation

• Optimise fibre intake using soft fibre rich foods, liquidised fruit and vegetables. Calculate fibre intake required for age and weight.

• If on supplementary drinks or enteral feed use fibre feed

• If begin on medication with known side effects start preventative treatment –macrogols.

Treatment of constipation

• Haynes (1997) reported using a supplementary drink containing fibre. Grade C

• Haynes (1998) reported on how constipation was resolved in 11 children after placement of gastrostomy tube resulting in adequate fibre and fluid. Grade C

• In clinical practice use of Optifiber added to food has been effective. Grade D

Treatment pathway

1 Calculate fibre and fluid requirements ↓ 2 Calculate current fibre intake & ↑ slowly if needed ↓ 3 Calculate current fluid intake and ensure meets required fluid intake based on age and weight

If blistering occurs

• If blistering in the perianal area is chronic also use a barrier cream

• The use of creams containing pain relief when a person is constipated is helpful e.g.ligonocaine gel, Instillagel

• If streptococcal infection is present treat.

Fear and control

• If with-holding out of fear becomes an issue or trying to assert control psychological input maybe helpful

This guideline is also a tool • Includes simple formula to calculate

fluid and fibre according to age and body weight

• An extensive appendices of fibre content of foods in order for a person with EB, carer or clinician to be able to assess their dietary intake

Summary of the process

• Convened a meeting of EB nurses, dietitians and people with EB scoping exercise-identified priority area

• Literature review • First draft written by lead and co-lead • Held meeting of EB nurses, EB dietitians,

parents, adults and children with EB at DEBRA members day-made additions to document

• Sent out draft 3 to adults with EB, parents and EB nurses for further comment. Contacted EB psychologist for input.

Conclusion

• We hope this provides access to and evaluation of the existing evidence regarding constipation in EB.

• A useful tool to assess if a person is taking enough fibre and fluid.

• Information on fibre containing foods to enable a clinician/person with EB to develop a treatment plan.

What next? Further chapters on nutrition are planned: • Assessment of requirements • Enteral and parenteral

feeding • Vitamins and trace element

requirements • Anaemia • Bone health

Finally we are there!

• Thank you for listening • Lynne Hubbard-Principal dietitian in EB, St

Thomas’ Hospital • Rosie Jones, Senior specialist dietitian in EB,

Birmingham Children’s Hospital • Kattya Mayre-Chilton, CPG co-ordinator & research dietitian

Updated Skin & Wound Care Best Practice Guidelines

https://www.debra.org.uk/downloads/community-support/woundcare-guidelines-2017.pdf

Premium sponsors:

DEBRA Members’ Weekend 2017 Constipation and general Q&A

EB Specialist Clinical Panel