9/12/2014. BVN-SBN Core Curriculum Course in Nephrology Introduction. em. prof. dr. R. Van Damme-Lombaerts KU Leuven

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1 BVN-SBN Core Curriculum Course in Nephrology Pediatric-adult interface Clinical management, standard of care, counselling December 13th, 2014 em. prof. dr. R. Van Damme-Lombaerts KU Leuven Introduction Number of young patients living with ESRD (transplantation / dialysis/ckd4 5) is increasing over the past decades Transfer to adult care or transitional care is a hot topic not only for children with a renal disease also for young patients with mucoviscidosis, diabetes mellitus, Down syndrome etc. The long term care of these patients presents a challenge 1

2 Transplant journey is a Developmental process that poses challenges for patients, families and clinicians 1. Referral for a pediatric solid organ Tx 2. Pretransplant psychosocial evaluation 3. Psychosocial impact of awaiting Tx in the context of worsening illness 4. Issues in the early post transplant period (school activity family functioning) child psychosocial adjustment and late post Tx period: non adherence autonomy-transition to adult care maturity 5. End of life care Complicated and potentially unpredictable process that requires a long term view of patient and family needs Global concept of transition of pediatric patients to adult renal patients was evolved in Berlin in 2003 (World Congress of Nephrology) 8000 adult delegates 400 pediatric delegates prevalence RRT in UK 566 pmp prevalence RRT in UK 8,96 pmp The adult patients in the service have the age of grand parents of the adolescents!! A few adolescents are transferred to the adult clinic each year 2

3 Major concern after transfer Increasing serum creatinine / graft loss Watson AR Ped Nephrol 2000; 14: Difficulties of transition 1. Generic issues 2. Treatment modality related issues 3. Disease specific related issues Generic issues Timing of transfer (1/3 of pt is always against transfer) Preparation and education Coordinated transfer process Primary care involvement Models for transition 3

4 Treatment modality related issues RRT: dialysis or transplantation Progressive renal impairment prior or after Tx CKD 4-5 (GFR < 30/ <15) Complex urology (neurological bladder; urethral valves; etc.) needs multidisciplinary approach Transplantation by recipient age The outcome of pediatric cadaveric renal transplantation in the UK and Eire The graft survival is worse in adolescent age group: (15 17 yrs) Postlethwaite RJ et al. Pediatr Transplantation 2002; 6, 367 Disease related specific issues Metabolic disorders Cystinosis Lowe syndrome etc. 4

5 Metabolic disorders e.a. Cystinosis Lysosomal storage disease Defect in lysosomal cystine transporter Intracellular lysosomal accumulation of cystine In proximal tubular cell of the kidney Proximal tubulopathy Fanconi Clinical symptoms: polyuria, polydipsia, aminoaciduria, glycosuria, phosphaturia, RTA, hypophosphatemic rickets Eye: Corneal crystal deposition with photophobia Corneal erosion, retinal deposition Defective colour vision Mild blindness to blindness Untreated ESRD by 10 years Cystinosis Gene for cystinosis is located on short arm of chromosom 17 Cystinosin: gene product (367 aminoacids) Cysteamine treatment is effective also in adults but compliance is low since taste nausea smell vomiting Cystagon dose = 50 mg/kg/day recommended Dosage in WBC is possible and is if treatment is effective to be stopped during pregnancy (teratogenicity) Complications in cystinosis Eye disorders Hypothyroidism Diabetes mellitus Encephalopathy / distal myopathy Swallow defects Portal hypertension But graft function is usually excellent!! 5

6 Pregnancy and cystinosis Counselling Partner testing genetic analysis Cephalopelvic disproportion during pregnancy Transplant function Stop cysteamine during pregnancy Placental function to be followed crystal deposition Risk of transfer Puberty delay and personal maturation Changing of metabolism during puberty Shift of medical care Structures of adult care giving versus pediatric clinic is different Pubertal development changing during puberty Delay of puberty / maturation Mean age is in male 16,4-18,7 years female 16,6-18,2 years Sex hormones : androgens and oestradiol produced but delayed metabolism of cyclosporin A / tacrolimus changes during puberty need of IS decreases Mental maturation delay 6

7 Ex pediatric population: specification Risk of urinary infection increased especially in females Incidence of hypertension is elevated Occurrence of disabilities is increased Disturbed lipid profile Incidence of diabetes mellitus increased Adult height is lower compared to healthy population 169 cm ± 10 (SDS 1.57) / 159 cm ± 8 (SDS 1.35) Psychological: Risk taking behaviour Non compliance Difficulty in the abstract concepts concerning future consequences Development identity separate from parents Pediatric clinic / Adult clinic Pediatric clinic Follow-up by a pediatric nephrologist Adult clinic Family doctor is integrated Different nephrologists / assistants Interval 1 4 weeks between control visits Team: psychologist, nurse specialist, social worker Consultation with parent/carer Fewer patients No waiting list for dialysis Self help groups Interval of visits: 3 months No additional care giver Individual consultation with patient Large number of patients Pressure on dialysis spaces Quality of life / compliance Communication by chronic ill children Dietician Psychologist Pastor / Counsellor All the other doctors The doctor The parent The nurse Social worker The taxi driver The child School J. Steyaert, EWOPA

8 Adolescent-young adults (17-24 yrs) have the highest risk of graft failure irrespective of age at transplant Foster BJ et al Transplantation 2011; 92 (11): Age related kidney transplant outcomes Health disparities amplified in Adolescents K. Andreoni et al Jama Int Med 2013; 173: Graft survival according to age at transplant K. Andreoni et al Jama Int Med 2013; 173:

9 Age related kidney transplant outcomes Andreoni KA et al Jama Intern Med 2013, 173: renal first transplants Age years at Tx: highest risk of graft loss Highest risk!! Black adolescents Patients with government insurance Transition protocol IS A MUST!!!!!!!!! Transition protocol More joint clinics ped/adult are necessary Education of adult nephrologist in specific pediatric diseases Educational programs for patients Data sharing - registry 9

10 Consensus statement : Watson AR et al Ped Nephr 2011; 26: Transition to transfer from pediatric to adult nephrology Only after efforts to prepare the adolescent aged 14 to 24 years Sufficient patient care information 2. Transfer: Individualized after he/she has completed a transition plan Agreed upon jointly During period without crisis Treatment plan by other subspecialities ea urology Financial factors 3. Transition process: Early adolescence (12 14 years) Information by transition champions Individualized plan for each patient Consensus statement (2) Include parents, family, friends Informed visit to adult service Peer support Self-management skills TRx ANSITION SCALE 4. Transition or transfer clinic Nephrologist should be trained in managing young people with CKD4-5 and trained in specific pediatric diseases e.a. cystinosis 5. Continuity of care Transition scale Type (of illness) Rx Adherence Nutrition Self-management skills Informed reproductive health Trade/school Insurance Ongoing support New health care providers 10

11 Coaching protocol (1) Training for small groups adolescents during 1 week Sport, physical and psychological information, career guidance Workshops during weekend Parents education Internet contact possibilities after the training Coaching protocol (2) Participation is self determined Full acceptance of location Continuation of arised contacts Information should be understandable, profitable and helpful Other initiatives Camp COOL with buddies Op eigen benen Website Leuven: 11

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13 Solutions to improve the transition process Transition coordinator Integrated pediatric young adult joint transition clinic Camps and mentors (after mentor training session) Holiday Youth worker Nurse practitioner: APN (advanced nurse practitioner) Transition coordinator A pilot study of using a transition coordinator to impose transfer from pediatric to adult services Annunziato R. J Ped 2013; 163 (6): Year before and after year transfer Tacrolimus TL SD: 1,98 3,25 : Higher in the group without coordinator! Adherence to transition guidelines in 15 pediatric nephrology units Forbes TA et al Ped Nephrol 2014; 9: Only 1/3 of 15 units integrated the guidelines 2 years after publication of guidelines 73 % of units transfers are government-and-hospital aged based driven Adult nephrologists receive transferred children infrequently 13

14 Different models of transition to adult care (around 16 years of age) Pape L et al Ped Transpl 2013; 17: Group 1: Transition to a specialized young adult clinic on same campus led by one specialised adult nephrologist Control : 1 x /4 weeks Group 2: Transition to an adult nephrologist in the private practice with alternate appointments 2 yr before transfer visit 3 x/yr to the adult Tx unit of the university appointments at family doctor Group 3: Transition to adult nephrologist in private practice 2 yr alternate appointments before transfer, no connection in the university Control: 1 x /4-12 wks Results (Pape L et al) Group 1 More satisfied Fewer changes in IS or antihypertensive therapies No influence on graft function or graft loss in the 3 groups Mean number of steroid doses increased in the 3 groups!! Conclusion Importance of single fixed doctor!! Specialised young adult clinic improves satisfaction rate after transfer The different roles and availabilities within the pediatric and adult units, and of the young person Watson AR et al British Journal of Renal Medicine 2011; 16(1): 17 14

15 Example of competencies I understand my condition and can describe it to others I know my medications and what they are for Watson AR et al Pediatr Nephrol 2011; 26: I can make decisions for myself about my treatment I know what the adult clinic arrangements are and who will be reviewing me in clinic I know how to make my appointments I can make my own transport arrangements to get to the hospital for appointments I know who to call in a medical emergency I am able to talk about my worries concerning blood tests and other treatments I know the dietary advice that I have to follow and the importance of activity I have appropriate knowledge about sexual health matters I have discussed alcohol, smoking and drug issues I know how to contact my primary care physician 15

16 Transition protocol UZ Leuven (1) Inclusion: Pediatric transplanted children from age 12 years with normal intelligence and without psychiatric problems Exclusion: Mental handicap / Psychiatric disorders Severe motor handicap, incapable of self-management Language problems Transition protocol UZ Leuven (2) Specific brochure was developed in yrs: Concept transition in introduced Advanced nurse specialist (APN): contact with patient without parents Non-adherence is discussed Information appropriate to patient developmental stage and intellectual ability about Tx/medication 16 years: Patient / doctor contact without parents Medical information: growth, sexuality, fertility, puberty APN more responsibility Contact with dietician, psychologist, social worker to discuss transfer 16

17 Transition protocol UZ Leuven (3) Year before transfer Critical evaluation of medication adherence Informed visit to the adult service Joint consultation pediatric / adult specialist 1 x Data transfer years: transfer to the adult service APN and transition physician are involved Selection of the moment of transfer Post transfer Contact APN and patients/patient 6 months of 1 year after transfer Feedback from the findings Transition protocol UZ Leuven (4) (Non) Adherence: BAASIS questionnaire Quality of life questionnaire Graft outcome Check list are developed for each age group concerning skills 8 12 yrs 13 yrs yrs yrs yrs Check list of diet information Role of water Role of physical activity Role of healthy food etc. Check list psychosocial aspects School, work, friends Relation with parents Sexuality pregnancy partner Responsibility Transition anxiety etc. Insurance - special features Key points Transition to the adult renal unit provides the young patient with the knowledge and skills to move from child/caregiver-directed care to an adult setting Transfer takes place at the end of a transition process, which should be individualised depending on a transition plan, and patient choice an maturity Improved communication and direction for transition should be promoted by transition champions Transition does not stop with transfer to the adult unit, but encompasses the ages 14 to 24 17

18 Literature 1. Optimal care of the infant, child, adolescent on dialysis 2014 Warady BA et al Am J Kidn Des 2014; 64: Transition with formal transition program 2. Bridging the gap: an integrated pediatric to adult clinic service for young adults with kidney failure Harden PN et al BMJ 2012; 344 Integrated pediatric-young adult joint transition clinic 3. Transition from pediatric to adult renal services: a consensus statement by ISN and IPNA. Watson AR et al Ped Nephrol 2011; 26: 1753 Literature (2) 4. Therapeutic recreation camps to provide a residential experience for young people in transition to adult renal units Watson AR et al Ped Nephrol 2010; 25: Evolution of youth work service in hospital Hilton D et al Nurs Child Young People 2012; 24: Adherence to transition guidelines in European pediatric nephrology units Watson AR et al Ped Nephrol 2014; 29: Growing pains: non adherence with the immunosuppressive regimen in adolescent transplant recipients Dobbels F, Van Damme-Lombaerts R Pediatr Transplant 2005; 9: