Lesley Haynes RD

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Nutrition in epidermolysis bullosa
Santiago November 2006
Patients photographs have been removed from this
presentation

• Lesley Haynes RD
• Specialist Paediatric Dietitian GOSH, London

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Chile would be a wonderful place to have an EB
conference !
Maybe we should have an EB conference in Chile !
GOSH main entrance
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Two main factors compromisenutritional status in
severe EB

• Decreased nutritional intake as a result of
  oral, oesophageal and GI complications
• Increased nutritional requirements in response
  to loss of blood and serum, infection and sepsis

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Aims of nutrition support(modify for Herlitz JEB)

• Alleviate under-nutrition and stresses of
  feeding
• Minimise nutritional deficiencies
• Promote growth ( catch-up if necessary)
• Promote normal bowel function
• Optimise immune status
• Promote wound healing

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Causes and effects of nutritional problems in
severe EB
Painful BO /-constipation Diarrhoea
Anorexia Apathy
Oral, pharyngeal oesophageal blistering Dental
caries G-O-R Fixed tongue Oesophageal
stricture Hand deformity
Inadequate food intake
Growth failure Osteoporosis/osteopaenia Compromise
d immunity Poor wound healing
Nutrient losses via skin lesions
Macro- micro- nutrient deficiencies
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Causes and effects of nutritional problems in
severe EB
Painful BO /-constipation Diarrhoea
Anorexia Apathy
Oral, pharyngeal oesophageal
blistering Dental caries G-O-R Fixed
tongue Oesophageal stricture Hand deformity
Inadequate food intake
Growth failure Osteoporosis/osteopaenia Compromise
d immunity Poor wound healing MISERY
Nutrient losses via skin lesions
Macro- micro- nutrient deficiencies
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Oral, pharyngeal oesophageal blistering Dental
caries G-O-R Oesophageal stricture Hand deformity

• Soft, moist foods nutrient-dense /-
  fortified
• Sugary foods (ideally restricted to mealtimes)
• Soft toothbrush, mouthwashes
• Anti-reflux medications
• Dilatation /- gastrostomy
• Surgery

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Causes and effects of nutritional problems in
severe EB
Painful BO /-constipation Diarrhoea
Anorexia Apathy
Oral, pharyngeal oesophageal blistering Dental
caries G-O-R Fixed tongue Oesophageal
stricture Hand deformity
Inadequate food intake
Growth failure Osteoporosis/osteopaenia Compromise
d immunity Poor wound healing MISERY
Nutrient losses via skin lesions
Macro- micro- nutrient deficiencies
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Painful BO /-constipation Diarrhoea
Anorexia Apathy

• Increased fluids and fibre, laxatives

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Causes and effects of nutritional problems in
severe EB
Painful BO /-constipation Diarrhoea
Anorexia Apathy
Oral, pharyngeal oesophageal blistering Dental
caries G-O-R Fixed tongue Oesophageal
stricture Hand deformity
Inadequate food intake
Growth failure Osteoporosis Compromised
immunity Poor wound healing
Nutrient losses via skin lesions
Macro- micro- nutrient deficiencies
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Growth failure Osteoporosis/osteopaenia Compromise
d immunity Poor wound healing

• Globally improved diet
• Calcium Vitamin D, mobility
• Dressings, pain relief
• Skin care, infection control

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Causes and effects of nutritional problems in
severe EB
Painful BO /-constipation Diarrhoea
Anorexia Apathy
Oral, pharyngeal oesophageal blistering Dental
caries G-O-R Fixed tongue Oesophageal
stricture Hand deformity
Inadequate food intake
Growth failure Osteoporosis/osteopaenia Compromise
d immunity Poor wound healing MISERY
Nutrient losses via skin lesions
Macro- micro- nutrient deficiencies
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Macro- micro- nutrient deficiencies

• Globally improved diet inc. supplements
• Dilatation /- gastrostomy

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Benefits ofgastrostomy placement

• Relatively straightforward surgery
• Discreet device (unlike NG tube)
• Improved quality of life for child and family
• Improved compliance with supplements ( fibre)
• Can be used for pain relief / other meds
• Reversible procedure

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Negative aspects of gastrostomy placement

• Bedwetting
• Bowel movement during night
• Leakage
• Infection
• Decreased oral intake g? increased frequency of
  OD
• When audited, 90 parents said they would
• recommend gastrostomy placement to other families

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Nutrition support can only work when
a multi-disciplinaryapproach is taken
nutrition
quality of life
pain
mobility
immunity

wound healing
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Dietitians cannot work in isolation nutrition
support must be everybodys business !

• Anaesthetist
• Cardiologist
• Dentist
• Dermatologist
• Dietitian
• Endocrinologist
• Gastroenterologist
• Haematologist biochemist
• Interventional radiologist
• Nurse

• Occupational therapist
• Ophthalmologist
• Pain specialist
• Physiotherapist
• Podiatrist
• Psychologist
• Social worker
• Speech language therapist
• Surgeon
• Urologist

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Nutrition support should be evaluated regularly

• Consider -
• Growth
• Chewing and swallowing capability
• Food intake
• Extent of lesions, infection and rate of healing
• Defaecation
• Biochemistry and haematology

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Blood tests

• Important aspect of monitoring
• But often difficult to interpret
• Iron, zinc, selenium, bone status
• Hormonal status

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What are nutritional requirementsin EB

• (1) for growth
• Vary depending on -
• Age
• Gender

• (2) for wound healing
• Vary depending on -
• Extent of blisters
• Presence of infection

• Many nutrients involved -
• Protein
• Calories
• Iron
• Zinc
• Vitamin C
• etc, etc

No guidelines but coming ..
National guidelines for healthy children
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Clinical Practice Guidelines for Nutrition
Support in Infants and Children
with Epidermolysis Bullosa (EB)
Lesley Haynes RD Specialist Paediatric Dietitian
for EB Great Ormond Street Hospital for Children
NHS Trust (GOSH) Great Ormond Street, London WC1N
3JH, UK
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Scoring System to Assess Nutritional Risk in EB

• Growth
• Chewing and swallowing capability
• Dysphagia / G-O-R
• Bowel habit
• Presence / absence of gastrostomy
• Extent of lesions, infection and rate of healing

• Each category scored
• Maximum possible total score 100
• The lower the score, the lower the risk
• Recommended action
• Algorithm

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THINC about nutrition ! This is a . T ool
to H elp I dentify N utritional C
ompromise in EB for professionals by Lesley
Haynes RD

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• Guidelines must be read first
• The aim of THINC is to highlight the child at
  actual or potential risk of nutritional
  compromise, so that a pro-active treatment plan
  can be drawn up which minimizes the impact of the
  disease process. THINC is intended to be used by
  the health professional who is supervising the
  childs medical care in association with other
  involved professionals THINC should aid, not
  replace, clinical judgement. Please refer to the
  accompanying algorithm, clinical guidelines and
  references for guidance re management strategies
• The scoring chart is intentionally brief to aid
  clarity and to simplify the completion process.
  Scores for some aspects will be unavoidably
  subjective, however they have been weighted to
  allow for this
• To maximize the accuracy of the final score,
  please take time to read the following notes.
  They explain the relevance of the aspect rated
  and offer guidance regarding the questions to ask
  in order elicit the most authentic picture of the
  child's condition and the likelihood of his / her
  developing future problems which affect
  nutritional status

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Thank you !