Transcript Welcome to the INDEB Forum

Combined Nutrition,
Nurses’ and
Psychosocial Care
Forum
Patients’ photographs
Nutrition and
Growth
Avignon
2 November 2007
have been removed
from this presentation
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
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
food intake
? malabsorption
mobility
weight-bearing
sunlight exposure
Growth failure
Nutrient losses via blisters
& wounds
Nutritional deficiencies
Compromised wound healing
Compromised immunity
Increased infection rates
Pubertal delay / failure
Osteoporosis / osteopenia
Anorexia, Apathy, MISERY
* Generally confined to RDEB
Causes and effects of nutritional
problems in severe EB
So, nutritional status is very
important and the main ways of
monitoring it are growth and blood
tests
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?
Different types of EB : different growth expectations
Is this
optimal
growth?
Dowling-Meara EB Simplex
Is this optimal
growth?
Recessive dystrophic EB
1
Summer 2006 12½ years old, with
role model and Ducati 999R
2
6 months later
The more severe the child’s 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.
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 don’t / can’t
implement everything we advise ?
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
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
nocte
As dietitians we have so much to offer, but does
addressing sub-optimal nutrition just reinforce
problems and increase parental guilt?
We work in MDT’s 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
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 child’s 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.
Gastrostomy placement
Age 2 years
Age 7 years
Age 9 years
16 years
(~ 6 months before
gastrostomy placement)
Gastrostomy – a patient’s opinion
Before,
weak and
skinny
After,
strong and
curvy
Become like
this
Why should
this
?
Oesophageal dilatation
A tight stricture (2mm)
typically located in the
thoracic oesophagus in
severe RDEB
The dilated
stricture
 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 don’t want to eat
when life expectancy is short regardless of
what we do?
Consequences of
complications of severe EB
Nutrient losses via blisters & wounds
Nutritional deficiencies
Compromised wound healing
Compromised immunity
Infections
Pubertal delay / failure
Osteoporosis / osteopenia
Growth failure
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