1. Psychological Considerations in Nutrition and Growth

2. Medical conditions increase the risk of feeding problems… Interruption of normal feeding Interruption of normal feeding History of physical discomfort during and following eating (Mathisen et al., 1999) History of physical discomfort during and following eating (Mathisen et al., 1999) Effects development of self-efficacy, tolerance for texture, oro-motor control Effects development of self-efficacy, tolerance for texture, oro-motor control Removes opportunities to develop autonomy and negotiate dependence in relation to caregiver Removes opportunities to develop autonomy and negotiate dependence in relation to caregiver

3. Fear Through Aversive Experiences Choking due to limited tongue and jaw mobility, oesophageal strictures Choking due to limited tongue and jaw mobility, oesophageal strictures Regurgitation and pain via reflux Regurgitation and pain via reflux Pain from trauma to skin Pain from trauma to skin Bone and tooth pain Bone and tooth pain Unfamiliarity with textures Unfamiliarity with textures

4. Iatrogenic Resistance Supplements/medicines taste bad Supplements/medicines taste bad Unpleasant treatments eg n-g tubes Unpleasant treatments eg n-g tubes Complicated regimes Complicated regimes Appetite loss through treatment side- effects Appetite loss through treatment side- effects Hopelessness through repeated failures Hopelessness through repeated failures Reclaiming control Reclaiming control

5. Parental Disagreement with Professional Advice Different priorities Different priorities (adaptive non-adherence) Trying but not believed Trying but not believed Desire to maintain “normal” feeding Desire to maintain “normal” feeding

6. Consequences of Ongoing Feeding Difficulties Chewing and swallowing difficulties Chewing and swallowing difficulties Loss of family routine Loss of family routine Unfavourable comparisons with siblings and other children Unfavourable comparisons with siblings and other children Relationship between professionals and family Relationship between professionals and family Different rules and boundaries Different rules and boundaries

7. Consequences for parents Parental guilt/failure Parental guilt/failure Parental stress Parental stress Parenting difficulties Parenting difficulties Loss of parenting confidence Loss of parenting confidence

8. Consequences for patient Sense of difference Sense of difference Delays in growth and motor development Delays in growth and motor development Mood Mood Appearance; small, thin, pre-pubertal Appearance; small, thin, pre-pubertal Physical ability; weak, lack of endurance, sleepiness at school Physical ability; weak, lack of endurance, sleepiness at school -> Low self esteem, low self-efficacy, poor social integration Long term consequences Long term consequences

9. Psychosocial Effect of Gastrostomy Feeding in EB  No studies in EB  Studies looking at other conditions : - carers’ feelings of inadequacy - insufficient attention to carers’ psychosocial needs - and consequent poor compliance with the feeding regimen Pederson et al (2004), Enrione et al (2005)

10. Child Protection Issues Consent: Is child capable for making an informed decision? Consent: Is child capable for making an informed decision? Neglect: “Failure of child to grow within normal limits” Neglect: “Failure of child to grow within normal limits” Abuse: “Force feeding” Abuse: “Force feeding” “Deliberate failure to follow medically recommended regimes” “Deliberate failure to follow medically recommended regimes” “Misapplication of programmes/regimes” “Misapplication of programmes/regimes”

11.  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?