Combined Nutrition, Nurses

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Combined Nutrition, Nurses and Psychosocial Care
Forum
Patients photographs have been removed from
this presentation
Nutrition and Growth

• Avignon
• 2 November 2007

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Severe EB has been described as
recalcitrant nutritional deprivation
unparalleled in all of clinical medicine. (Tesi
Lin, 1992)
Things have improved greatly in the last 15
years, thanks to MDT working
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But the complexity of some cases means that they
still pose great challenges to MDT and carers
alike
Dental / gum disease Oral, pharyngeal
oesophageal blistering Microstomia , fixed
tongue Dysphagia Oesophageal stricture
Gastro-oesophageal reflux (GOR) Painful
defaecation /- constipation GI tract
involvement Anal fissures Hand deformity PAIN

Growth failure Nutrient losses via blisters
wounds Nutritional deficiencies Compromised wound
healing Compromised immunity Increased infection
rates Pubertal delay / failure Osteoporosis /
osteopenia
äfood intake ? malabsorption ämobility äweight-bea
ring äsunlight exposure



Anorexia, Apathy, MISERY
Generally confined to RDEB
Causes and effects of nutritional problems in
severe EB
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• So, nutritional status is very important and the
  main ways of monitoring it are growth and blood
  tests

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What is optimal growth?

• Children with RDEB are of significantly lower
  birthweight than unaffected children, and the
  compromise in growth seen throughout life in RDEB
  appears to begin in utero
• Fox AT, Alderdice F, Atherton DJ (2003)
• What are we aiming for?

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Different types of EB different growth
expectations
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Is this optimal growth?
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(No Transcript)
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Dowling-Meara EB Simplex
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Is this optimal growth?
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Recessive dystrophic EB
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6 months later
Summer 2006 12½ years old, with role model and
Ducati 999R
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The more severe the childs EB, the greater the
number of professionals that are involved in
his/her care ..
The greater the number of professionals that are
involved, the more interventions there are with
which parents are expected to comply.
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So many professionals

• 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

Is it any wonder that families dont /
cant implement everything we advise ?
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20 Medications / supplements
Item Timing Item Timing
Sodium feredetate bd after meals Codeine prn
Zinc sulphate od after meal Morphine prn
Selenium od before feed Midazolam prn
Calcium Vit D od before feed Gabapentin tds
Ranitidine bd before feed Calpol prn
Domperidone qds before feed Doxepin nocte
Omeprazole od before feed
Becotide bd Lactulose od before feed
Mesalazine bd after meals Sodium picosulphate od after feed
Ketotifen bd after feed
Piroxicam od before feed
Pepti Junior Overnight 45ml x 1 x 10
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• As dietitians we have so much to offer, but does
  addressing sub-optimal nutrition just reinforce
  problems and increase parental guilt?

• We work in MDTs to agreed care plans for
  patients, but we may be seen as the chalice
  bearers and this can make relationships with
  patients difficult and we can be seen as the bad
  guys

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Nutrition, a poisoned chalice?
Not my words, but those of a non-dietetic
colleague
Not that the chalice is poisoned, but that by
addressing the EB childs nutritional intake,
status and growth, the chalice-bearer (dietitian)
is touching on very sensitive and fundamental and
sensitive parenting issues ie parents ability
to nourish their child.
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Gastrostomy placement
Age 9 years ( 6 months before gastrostomy
placement)
Age 2 years
Age 7 years
16 years
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Gastrostomy a patients opinion
After, strong and curvy
Before, weak and skinny
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Become like this
Why should this
?
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Oesophageal dilatation
A tight stricture (2mm) typically located in the
thoracic oesophagus in severe RDEB
The dilated stricture
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• Where does/should nutrition lie in the list of
  priorities for care of severely-affected
  children?
• How hard should we push severely-affected
  children (or adults) who dont want to eat when
  life expectancy is short regardless of what we
  do?

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Consequences ofcomplications of severe EB

• Nutrient losses via blisters wounds
• Nutritional deficiencies
• Compromised wound healing
• Compromised immunity
• Infections
• Pubertal delay / failure
• Osteoporosis / osteopenia
• Growth failure

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How to monitor growth ?

• With difficulty, in severe EB, the tools we have
  are often associated with problems -
• Weight
• Height
• Body Mass Index (BMI) weight (kg) / height (m2
  )
• Waist circumference
• Skinfold thickness (calipers)
• Mid upper arm circumference
• Individual limb measurements
• Measurement of body composition