Combined Nutrition, Combined Nutrition, Nurses’ and Nurses’ and

Psychosocial Care Psychosocial Care

ForumForum

AvignonAvignon2 November 20072 November 2007

Nutrition and Nutrition and GrowthGrowth

Patients’ photographs have been removedfrom 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

Dental / gum diseaseOral, pharyngeal &

oesophageal blisteringMicrostomia *, fixed tongue *

DysphagiaOesophageal stricture *

Gastro-oesophageal reflux (GOR)Painful defaecation +/- constipation

GI tract involvementAnal fissures

Hand deformity *PAIN

food intake? malabsorptionmobilityweight-bearingsunlight exposure

Growth failureNutrient losses via blisters

& woundsNutritional deficiencies

Compromised wound healingCompromised immunityIncreased infection ratesPubertal delay / failure

Osteoporosis / osteopenia

Anorexia, Apathy, MISERY

* Generally confined to RDEB

Causes and effects of nutritional Causes and effects of nutritional problems in severe EBproblems in severe EB

But the complexity of some cases means that they still pose great challenges to MDT and carers alike

So, nutritional status is very So, nutritional status is very important and the main important and the main ways of ways of monitoring it are growth and blood monitoring it are growth and blood teststests

Children with Children with RDEBRDEB are of significantly are of significantly lower birthweight than unaffected lower birthweight than unaffected children, and the compromise in growth children, and the compromise in growth seen throughout life in RDEB appears to seen throughout life in RDEB appears to begin in utero begin in utero

Fox AT, Alderdice F, Atherton DJ (2003)Fox AT, Alderdice F, Atherton DJ (2003)

What are we aiming for?What are we aiming for?

What is optimal growth?What is optimal growth?

Different types of EB : different growth expectationsDifferent types of EB : different growth expectations

Is this optimal growth?

Dowling-Meara EB Simplex

Is this optimal growth?

Summer 2006 12½ years old, withrole model and Ducati 999R

6 months later

Recessive dystrophic EB1

2

The greater the number of professionals that are involved, the more interventions there are with which parents are expected to comply.

The more severe the child’s EB, the greater the number of professionals that are involved in his/her care …………..

AnaesthetistAnaesthetistCardiologistCardiologistDentistDentistDermatologistDermatologistDietitianDietitianEndocrinologistEndocrinologistGastroenterologistGastroenterologistHaematologist & Haematologist &

biochemistbiochemistInterventional Interventional

radiologistradiologistNurseNurse

Occupational therapistOccupational therapistOphthalmologistOphthalmologistPain specialistPain specialistPhysiotherapistPhysiotherapistPodiatristPodiatristPsychologistPsychologistSocial workerSocial workerSpeech & language Speech & language

therapisttherapistSurgeonSurgeonUrologistUrologist

Is it any wonder that families don’t / can’tIs it any wonder that families don’t / can’timplement everything we advise ?implement everything we advise ?

So many professionals

20 Medications / supplements

ItemItem TimingTiming ItemItem TimingTiming

Sodium Sodium feredetateferedetate

bd after mealsbd after meals CodeineCodeine prnprn

Zinc sulphateZinc sulphate od after mealod after meal MorphineMorphine prnprn

SeleniumSelenium od before feedod before feed MidazolamMidazolam prnprn

Calcium & Vit DCalcium & Vit D od before feedod before feed GabapentinGabapentin tdstds

RanitidineRanitidine bd before feedbd before feed CalpolCalpol prnprn

DomperidoneDomperidone qds before feedqds before feed DoxepinDoxepin noctenocte

OmeprazoleOmeprazole od before feedod before feed

BecotideBecotide bd bd LactuloseLactulose od before feedod before feed

MesalazineMesalazine bd after mealsbd after meals Sodium picosulphateSodium picosulphate od after feedod after feed

KetotifenKetotifen bd after feedbd after feed

PiroxicamPiroxicam od before feedod before feed

Pepti JuniorPepti Junior Overnight 45ml Overnight 45ml x 1 x 10x 1 x 10

As dietitians we have so much to offer, but does As dietitians we have so much to offer, but does addressing sub-optimal nutrition just reinforce addressing sub-optimal nutrition just reinforce problems and increase parental guilt?problems and increase parental guilt?

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

Nutrition, a “poisoned chalice”?Nutrition, a “poisoned chalice”?

Not my words, but those of a Not my words, but those of a non-dietetic colleaguenon-dietetic colleague

Not that the chalice is poisoned, but that by Not that the chalice is poisoned, but that by addressing the EB child’s nutritional intake, addressing the EB child’s nutritional intake, status and growth, the chalice-bearer status and growth, the chalice-bearer (dietitian) is touching on very sensitive and (dietitian) is touching on very sensitive and fundamental and sensitive parenting issues – fundamental and sensitive parenting issues – ie parents’ ability to nourish their child. ie parents’ ability to nourish their child.

Age 2 years Age 7 years Age 9 years

(~ 6 months before gastrostomy placement)

16 years

Gastrostomy placementGastrostomy placement

Gastrostomy – a patient’s opinionGastrostomy – a patient’s opinion

Before, weak and skinny

After, strong and curvy

Why should Why should thisthis

Become like Become like thisthis

??

A tight stricture (2mm) typically located in the thoracic oesophagus in severe RDEB

The dilated stricture

Oesophageal dilatationOesophageal dilatation

Where does/should nutrition lie in the list of Where does/should nutrition lie in the list of priorities for care of severely-affected priorities for care of severely-affected children?children?

How hard should we push severely-affected How hard should we push severely-affected children (or adults) who don’t want to eat children (or adults) who don’t want to eat when life expectancy is short regardless of when life expectancy is short regardless of what we do?what we do?

Nutrient losses via blisters & woundsNutrient losses via blisters & woundsNutritional deficienciesNutritional deficienciesCompromised wound healingCompromised wound healingCompromised immunityCompromised immunityInfectionsInfectionsPubertal delay / failurePubertal delay / failureOsteoporosis / osteopeniaOsteoporosis / osteopeniaGrowth failureGrowth failure

Consequences ofConsequences ofcomplications of severe EBcomplications of severe EB

With difficulty, in severe EB, the tools we have are With difficulty, in severe EB, the tools we have are often associated with problems :-often associated with problems :-

WeightWeight HeightHeight Body Mass Index (BMI) = weight (kg) / height (mBody Mass Index (BMI) = weight (kg) / height (m2 )2 )

Waist circumferenceWaist circumference Skinfold thickness (calipers)Skinfold thickness (calipers) Mid upper arm circumferenceMid upper arm circumference Individual limb measurementsIndividual limb measurements Measurement of body compositionMeasurement of body composition

How to monitor growth ?How to monitor growth ?