Combined Nutrition, Nurses’ and Psychosocial Care Forum Patients’ photographs have been removed from this presentation Avignon 2 November 2007 Nutrition and Growth
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 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-bearing äsunlight exposure 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?
Recessive dystrophic EB 1 2 6 months later Summer 2006 12½ years old, with role model and Ducati 999R
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.
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 So many professionals Is it any wonder that families don’t / can’t implement everything we advise ?
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 9 years (~ 6 months before gastrostomy placement) Age 2 years Age 7 years 16 years
Gastrostomy – a patient’s opinion After, strong and curvy Before, weak and skinny
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 ofcomplications 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