programme Pipilotta und der Nierendetektiv Marcus R. Benz 1, E.-M. Rüth 2, F. Kusser 1, T. Volkmar 1, H. Fehrenbach 3, D. Kleinert 3, J. Dötsch 2, L.T. Weber 1 1 Paediatric Nephrology, Dr. von Haunersches Kinderspital, children s university hospital, Munich, Germany 2 Paediatric Nephrology, children s university hospital, Erlangen, Germany 3 Paediatric Nephrology, children s hospital, Memmingen, Germany
Nephrotic syndrome in childhood heavy proteinuria (> 1g/m 2 x d) hypoalbuminaemia (<25 g/l)
Relapsing disease >50% 80-90% - 35-50% frequent relapser or steroid dependent persistent disease in adulthood: 7-42%
Health related quality of life (HRQoL) Rüth EM et al., 2004, J Pediatr
Psychosocial adjustment Rüth EM et al., 2004, J Pediatr
Family education programme Pipilotta und der Nierendetektiv
Knowledgetransfer Early detection of relapse Management in remission Coping in the family Interdisciplinary team Pragmatic coping strategie ge-appropriate didactics Family as important networ Acceptance of nephrotic syndrom as a chronic-relapsing disease Improvement of family interaction Enhancement of selfconfidence less side-effects of the drugs less hospitalization less days absent of school Improvement of HRQoL Improvement psychosocial adjustment Improvement of state of health
Organisation 4-6 children (similar age) + parents + siblings team -doctors - psychologists - nurses all-day 4 units (children/ parents)
Sieve-model Sieve with small wholes, but proteins do not pass. Sieve with big wholes, little amount of proteins passes. Sieve with more bigger wholes, big amount of proteins passes.
protein negativ trace + ++ +++ ++++ Sieve-model Sieve with small wholes, but proteins do not pass. Sieve with big wholes, little amount of proteins passes. Sieve with more bigger wholes, big amount of proteins passes.
Detective Mr. Kidney is after the protein Family education These are his tools: 1. Urine dipstick Is there protein in the urine? Measurement: Daily in the morning 2. scales: Gain of weight? Measurement: daily, if albustix is positive 3. mirror: Are there any oedema visible? 4. protocol: All results will be collected and written down in here Very important!!!
kidney-sieve (=glomerulus) vessel vessel protein urinary bladder kidney-sieve (=glomerulus) vessel vessel rotein urinary bladder
Evaluation before and 6 months after intervention HRQoL (TACQOL-PF und TACQOL-CF) psychosocial adjustment children (CBCL) psychosocial adjustment children in school (TRF) psychological adjustment parents (BSI) disease specific questionnaire life events of family family relationship index
Participants n = 14 Gender Age at diagnosis Age at programme 7 male 4,6 (1,8-10,1) 9,0 (5,8-11,9) 7 female Interval: diagnosis to programme Clinical course 4,3 (0,6-8,4) infrequent relapser frequent relapser steroid dependent steroid resistant 5 3 4 2
*Wilcoxon-Test **p<0,05 Family education HRQoL (TACQOL-PF) mothers before intervention 6 months after intervention p * n mean SD mean SD Body 14 24,3 4,3 26,3 2,9 0,11 Motor 14 27,4 4,2 30,4 1,74 0,01** Autonomy 14 30,8 1,7 31,2 2,0 0,35 cognition 14 25,9 5,6 27,0 5,5 0,26 Social 14 29,1 2,9 29,9 2,4 0,24 Emotion (positive) 14 13,7 2,3 13,8 2,43 0,82 Emotion (negative) 14 11,0 2,9 12,1 2,3 0,03**
thoughts of relapse (mother) before intervention Psychological adjustment parents (BSI) never rarely sometimes frequently (e.g. infections) (e.g. daily dipstick) Before intervention n mean 6 months after mean p * 6 months after intervention mother father 14 13 48,7 49,0 43,6 44,6 0,01** 0,05** *Wilcoxon-Test **p<0,05 never rarely sometimes frequently
CBCL - mother Internalizing score: Externalizing score: withdrawn, somatic complaints, anxiety/depression, thought problems social problems, attention problems, delinquent and aggressive behaviour mother Before intervention 6 months after intervention p * n mean SD mean SD Total-Score 13 55,9 10,5 50,8 8,2 0,02** Internalizing-Score 13 57,5 12,1 51,0 8,1 0,03** Externalizing-Score 13 52,6 8,3 47,7 8,2 0,11 *Wilcoxon-Test **p<0,05
Conclusion Familiy education programme : empowerment as aim improves HRQoL improves psychosocial adjustment possible new component in the integrated treatment of in childhood
T. Volkmar M. R. Benz F. Kusser E. M. Rüth München München München Erlangen foundation: