Infants with ESRD. challenges and pitfalls

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1 Infants with ESRD challenges and pitfalls

2 ESRD in an infant poses a major challenge - to the infant - to parents and families - to the multi-professional paediatric renal team

3 It is clear that the management of the infant with renal failure demands a special expertise. A closely co-ordinated team of paediatric nephrologist, urologist, radiologist, nursing staff, pathologists and dietician, all experienced in and equipped for the management of sick infants, is essential, and any compromise of this arrangement will result in less than optimal results.

4 RENAL FAILURE IN THE FIRST YEAR OF LIFE Martin Barratt British Medical Bulletin 1971

5 Overview Theme of uncertainty Early decision making Choosing not to start dialysis Outcome of infants who start dialysis Personal reflections

6 Uncertainty Whilst some advances in dialysis fluid and catheter design have contributed small improvements to the technical aspects of dialysis in infants, the huge burden of care, risk of complications, and uncertainty over treatment and outcome remain.

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8 Uncertainty antenatal diagnosis of renal disease, but uncertainty during pregnancy about how severe Once born, uncertainty over whether the poor kidney function might gradually improve, or get worse we often find ourselves telling parents that -we know the kidneys will never be normal -we think that renal replacement therapy will be needed at some stage -but for now there is uncertainty about when

9 C reatin ine Level Creatinine Level (um ol/l) umol/l H 0 L May 2009 Jun Jul COOPER, YUKI Aug Sep

10 C reatin in e L ev el Creatinine Level (um ol/l) umol/l H L H L 0 M ar 2010 Mon 22 Apr 1 Thu 8 Thu 15 BAKER, PATRICK Thu 22 M ay 1

11 Uncertainty uncertainty over survival of the infant uncertainty, in the minds of parents, and staff, over -whether it is in the best interests of the baby to commence renal replacement therapy -whether conservative treatment, resulting in death of the baby, may in some cases be the right choice

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13 The Ethics of Infant Dialysis Bunchman. Peritoneal Dialysis International 1996 Treatment of an infant with ESRD depends upon a number of factors including parental willingness to take on the task experience of the health-care team local and regional resources society's willingness to accept this support as a standard of care. For a family to agree to take on such a task, an understanding of the risks and long-term prognosis should be offered This "informed consent" is difficult to obtain in such a highly charged situation when emotions often dictate choice independently of logic.

14 Lesley Rees, 2002 (NDT) and 2007 (PDI) At no other time are there so many emotional and ethical dilemmas, both for the family and the medical team. The birth of such an infant may have been preceded by months of parental anxiety termination of pregnancy may have been discussed foetal interventions performed

15 Lesley Rees, 2002 (NDT) and 2007 (PDI) Parents are fearful for the outcome can only be successful if there is full commitment from themselves and a highly skilled and resourced medical team It is at this time of vulnerability that families and the medical team must discuss together the multiple factors affecting the management and potential outcome for their child, before making the decision to embark on long-term renal replacement therapy.

16 Lesley Rees, 2002 (NDT) and 2007 (PDI) Some families may decide that they do not wish to inflict further pain and suffering on their infant, and they choose conservative management. The decision to focus on palliative care often evolves gradually different members of staff and family may be working towards the decision at different rates time and discussion are essential.

17 Lesley Rees, 2002 (NDT) and 2007 (PDI) Many infants are polyuric, and such infants may survive many months even if untreated but at considerable cost to their growth and development. Parental bonding with an infant can lead to reversal of a decision for conservative management, by which time irreversible damage to growth and development may have occurred.

18 Journal of Pediatrics 1998 multinational survey of pediatric nephrologists in Canada (16), France (12), Germany (26), Holland (3), Italy (10), Japan (13), United Kingdom (27), and the United States (104)

19 Journal of Pediatrics 1998 multinational survey of pediatric nephrologists in Canada (16), France (12), Germany (26), Holland (3), Italy (10), Japan (13), United Kingdom (27), and the United States (104) asked whether the nephrologist offered RRT for infants with ESRD to any to all

20 Journal of Pediatrics 1998 multinational survey of pediatric nephrologists in Canada (16), France (12), Germany (26), Holland (3), Italy (10), Japan (13), United Kingdom (27), and the United States (104) asked whether the nephrologist offered RRT for infants with ESRD to any to all if parents rejected RRT for otherwise normal children with ESRD, was this acceptable to the nephrologist ever usually

21 Journal of Pediatrics 1998 multinational survey of pediatric nephrologists in Canada (16), France (12), Germany (26), Holland (3), Italy (10), Japan (13), United Kingdom (27), and the United States (104) asked whether the nephrologist offered RRT for infants with ESRD to any to all if parents rejected RRT for otherwise normal children with ESRD, was this acceptable to the nephrologist ever usually factors influencing the decision were recorded for infants <1 month: 41% of nephrologists offered RRT to all; 93% to some 1 month - 1 year: 53% offered RRT to all; 100% to some

22 Parental refusal of RRT for infants 1 month to 1 year 38% of nephrologists believed it was never ethically acceptable 25% believed it was usually ethically acceptable Parental refusal of RRT for infants <1 month 19% of nephrologists believed it was never ethically acceptable 50% believed it was usually ethically acceptable p < for both Factors most influencing the decision to initiate or withhold ESRD treatment were presence of coexistent serious medical disorders anticipation of morbidity for the child

23 Outcome of reaching end stage renal failure in children under 2 years of age Coulthard and Crosier, Archives of Diseases in Childhood 2002 UK data 1988 to patients When treatment continued, 71% survived 26/192 (14%) died because dialysis not started in 14 family and clinicians agreed not to treat 7 died within a month, 7 more by six months, 1 at 18 months dialysis withdrawn in 12 Significant co-morbidity not always diagnosed at start of Rx

24 Conservative treatment Not no treatment, but palliative treatment Symptom control includes Nutrition Bone, anaemia, acidosis management Family may change mind

25 Conservative treatment Not no treatment, but palliative treatment Symptom control includes Nutrition Bone, anaemia, acidosis management Family may change mind How well do we do it? What training do staff have in providing high quality expert palliative care?

26 Conservative treatment Not no treatment, but palliative treatment Symptom control includes Nutrition Bone, anaemia, acidosis management Family may change mind How well do we do it? What training do staff have in providing high quality expert palliative care? Is it as well developed and resourced as active treatment?

27 Conservative treatment Not no treatment, but palliative treatment Symptom control includes Nutrition Bone, anaemia, acidosis management Family may change mind How well do we do it? What training do staff have in providing high quality expert palliative care? Is it as well developed and resourced as active treatment? Not in my hospital recognised as area of need Much of the care needs to be community based Resources often concentrated in hospice care

28 What do we know about the outcome when dialysis starts in infancy?

29 Outcome of Infants on Chronic Peritoneal Dialysis (Ellis et al, Adv Perit Dial 1995) Reviewed 21 patients who began PD <1 year old 12/21 had non-renal abnormalities lung, heart, CNS Outcome: 7 received renal transplant 1 recovered renal function 4 continued on PD 9 died (43%) 7/11 with oliguria died 2/10 without oliguria 9/12 patients with non-renal abnormalities died 0/9 with isolated renal disease Conclusion: mortality in infants on PD appears to be associated with -oliguria -non-renal abnormalities.

30 Guy s Experience infants reaching ESRD during 1st year 15 survivors 15 died despite dialysis (mainly ) 10 had significant comorbidity 7 dialysis withdrawn, or never started

31 Cause of death 1 1 sepsis 1 9 ICH haemorrage resp+cardiac failure 2 post-cardiac surgery post-liver & kidney Tx 1 PICU: 10 Renal ward: 4 DGH: 1

32 M e d ia n a g e a t d e a th b y c a u s e age (months) N= a ll N=9 6.8 s e p s is cau se of d e ath N= o th e r

33 No treatment started, or treatment withdrawn 3 with CNS and rapidly deteriorating renal function 80% mortality by 1 year terrible quality of life for baby 3 with dysplasia 1. Dialysis withdrawn after 5 days at parents request 2. Strongly religious mother who entrusted baby to God s care 3. Female baby in large Asian family (5 other children) 1 with ARPKD Sent home from DGH to die, but by 3 weeks hadn t Respiratory collapse and worsening renal failure parents did not want ventilation and dialysis

34 Age at death for those not treated CNS 8.0 age (months) 7.0 ARPKD CNS 4.0 dysplasia CNS dysplasia dysplasia Home: Renal Ward: 3 DGH: 1 7

35 The GOS experience Shroff et al, Pediatric Nephrology 2006 Outcome since 1984 of all children receiving chronic dialysis for >3 months 98 children (61 boys) median age at start of dialysis 4.2 yrs (range: birth to 16.2 yrs) 21 started dialysis <1 year of age 54 started dialysis <5 years 30 children had significant co-morbidity

36 The GOS experience deaths 76% in association with co-morbid conditions Co-morbidity gave 7.5x risk of death 10 died on dialysis For those commencing dialysis aged <1 year, 5 year survival was 72% Overall patient survival 83%

37 The developmental progress of the surviving infants is encouraging, despite the great vulnerability of this group neuro-developmental outcome was more likely to be adverse if there was an associated disorder. Early hypoxia caused by pulmonary hypoplasia may be a particular risk factor rather than early ESRD.

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40 Neurodevelopmental outcome of children initiating PD in early infancy Warady et al, Pediatric Nephrology infants starting PD < 3 mths, /34 (18%) died < 12 mths Overall mortality 10/34 = 29.4% 23 of 28 survivors assessed at 12 months: 22/23 had developmental score in average range 19 retested at 4 yrs: 15/19 in average range; 3 borderline; 1 impaired 16 assessed 5 yrs: 15/16 in full-time age appropriate schooling

41 The Helsinki experience Laakkonen et al, NDT 2008 Review of 23 children between 1995 and 2000 starting dialysis before 2 yrs Diagnoses congenital nephrotic syndrome of the Finnish type (13) polycystic kidney disease (4) urethral valve (3) neonatal asphyxia (2) Prune-Belly (1)

42 The Helsinki experience % were anuric high % cf other centres Hernias diagnosed in 57% consider routine repair at catheter insertion Hospitalization time was 124 days/patient-year Only 2/23 (9%) died Conclusions results reassuring mortality low laboratory parameters were acceptable and growth was good

43 Comparisons with Registry data 5-year survival rate for children starting RRT by 1 year of age was 66% UK Renal Registry report 73% ANZDATA 3-year survival rate for children starting RRT by 1 year of age was 66% NAPRTCS

44 Personal Reflections

45 Personal Reflections Not possible to explain fully the burden of care Parents say We will do whatever it takes Staff try to paint a picture of how tough life can be This may not include every complication Cannot convey the impact on their relationship and on family life Parents may still react with anger and frustration We cannot say I told you so..

46 Personal Reflections Not every member of staff will give the right answer, or do the right thing Some parents shop around for the answer they want Ask many staff the same question, including junior nurses and doctors Sometimes play us off against each other: But Dr Smith said something different Compare notes with other families on ward, in clinic Wise to channel communication through senior medical and nursing staff

47 Personal Reflections When in hospital, may be critical of the way nurses perform care that they themselves do at home They are often experts Again, senior nursing staff are vital in dealing with this Wise to be clear about who is responsible for what aspects of care in hospital Drugs, feeds, setting up PD

48 Personal Reflections This is the start of a roundabout of treatment: Enter the roundabout with dysplasia, renal failure Exit the roundabout on PD Re-enter with peritonitis Exit on haemodialysis Re-enter for LRD transplant ESRD Exit into transplant clinic After years.

49 Personal Reflections We often cause a feeding and vomiting obsession Becomes the focus of every clinic visit Small weekly fluctuations in weight dominate discussion Expert, compassionate and common-sense dietician invaluable Wise to take the overview of growth

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53 Personal Reflections Pressure may grow to have transplant a.s.a.p. We try to predict how old child will be when big enough (eg 10kg) Can be wrong, both ways! Family desperate to stop dialysis Transplant seen as the answer but should never be hurried

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56 Personal Reflections It s often the little things. Being kept waiting longer than usual for clinic visit Problem with transport, or parking at the hospital But the real problem is lack of sleep/exhaustion Worry Anger/grief, not coming to terms with implications of diagnosis Failing relationship, effect on other children

57 Personal Reflections Core clinical team vital for consistent support senior doctor, nurse, dietician But, involve extended team in decisions over complex cases Avoid making decisions, in isolation, that may impact on whole team Open discussion vital when dealing with major decisions in complex infants with co-morbidity

58 Open for discussion