Children with ESRD:

They’re Not Little Adults!

Beth A. Vogt, MD

Rainbow Babies and

Children’s Hospital

Conflict of Interest/

Disclosure Statement

• I have no conflicts of interest to disclose

• I will not discuss off label use of drugs in my

presentation

Case: Ian

• 14 yr old teenage boy

• Born with posterior urethral valves and renal dysplasia

• Medically managed CKD until started PD at age 2 yrs

• Supported with G-tube feedings until age 6 yrs

• Living related donor transplantation from Mom at age 4.5 yrs

• Complications

– Rejection x 2

– Chronic low level EBV titer

– Recurrent sinusitis/URIs

– Short stature (height 5 ft, weight 86 lbs)

– Behavioral issues, school avoidance, adherence concerns

– Gradual loss of kidney function (Cr 2.2, eGFR 29 ml/min)

• Starting to plan for next step as allograft is failing after 10 yrs

Outline

• Causes of CKD/ESRD in children

• Medical issues

• Psychosocial issues

• Treatment options for children with ESRD

– Peritoneal dialysis

– Hemodialysis

– Transplantation

ESRD in Children

• Rare in children (9 per million)

• High burden of care

• Underlying causes of ESRD are very

different in children than in adults

– NOT diabetes and hypertension!

– Congenital conditions

– Glomerulonephritis

– Other

Congenital Kidney Disease

• 40% of pediatric ESRD

• Renal dysplasia– Poorly developed kidneys in utero

– May be associated with obstruction• Posterior urethral valves

– May be associated with a syndrome• Eagle Barrett syndrome (prune belly

syndrome)

– May be an isolated finding• Sarah Hyland (Haley Dunphy of Modern

Family)

Glomerular Disease

• 25% of pediatric ESRD

• Glomerulonephritis– Lupus nephritis

– MPGN

– IgA nephropathy

– HSP nephritis

– Vasculitis

• Steroid-resistant nephrotic syndrome– Infantile NS

– Focal segmental glomerulosclerosis• Alonzo Mourning, Sean Elliott, Gary Coleman

• Hereditary nephritis– Alport syndrome

Other Conditions

• Genetic disorders

– ARPKD

• h/o severe AKI– Former preemies

– ICU care• Sepsis

• BMT

• Cardiac surgery

• Cancer

Medical Issues

Measurement of

Kidney Function

• Serum creatinine (Cr)

• Affected by

– Age

– Muscle mass

– Creatinine supplements

• Cannot use MDRD, CKD-EPI formulas for eGFR

• Use Schwartz formula

Blood Pressure

• Adult norms

– < 120/80 Normal

– > 140/90 HTN

• Pediatric BP norms

– Age

– Height

– Gender

• Fourth Report BP table

• BP > 120/80 is always high in a child

5 yr old boy with

height at 5th

percentile

Normal:

BP < 104/65

PreHTN:

BP 104/65-

108/69

Stage 1 HTN:

BP > 108/69-

120/82

Stage 2 HTN:

BP > 120/82

Average BP:

90/50

Fourth Report on High BP in Children, Pediatrics 2004.

Anemia

• Lack of erythropoietin, +/- iron

• May be exacerbated by– Frequent lab tests

– Residual blood loss in HD circuit

– Uncontrolled CKD-MBD

• Microtainers can reduce blood loss

• EPO and Venofer (dosed per kg)

• Younger children may need higher doses of EPO

• Goal Hb 10-12 g/dl

Malnutrition

• Weight loss OR failure to

gain weight

• Multifactorial

• Often need oral, NG or G-

tube feedings

• Can help with catch up

growth

• Caution: obesity can occur if

weight gain without good

linear growth

Weight Gain Improves

with GT Feeds

CKD-MBD

• Usually high-turnover bone

disease with high PTH

• Use binders, vitamin D,

paracalcitol, cinacalcet (off label),

and/or parathyroidectomy

• Fractures, bone/muscle pain

• Disease specific to growing bone

– Rickets

– Short stature

Short Stature

• >1/3 of kids on dialysis have short stature

• Multifactorial problem

– CKD-MBD

– Malnutrition

– Steroids

– Growth hormone (GH) resistance

• More of an issue with early onset CKD

• Treatment: nutrition, treat PTH and other

metabolic issues

• Recombinant GH

• Daily SQ injections given x years

• Track growth curve, height Z scores

Growth Improves with rGH

Cardiovascular Disease

• Growing focus of attention

• Usually does not manifest in

childhood years, but CV risk is

silently building

• 50% of kids on dialysis have LVH

• Treatment

– Tight BP control

– Fluid balance

– Treat anemia and CKD-MBD

– Surveillance echocardiograms

Psychosocial Issues

Quality of Life

• Multiple challenges for kids with ESRD

– Physical changes

– Need for medications

– Need for treatments

– Time spent away from school/peers

– Dependence on medical equipment

• Peds QOL scores are low and equal to

those of children with cancer!

Neurocognitive Concerns

• May have developmental delay, learning

disabilities

• IQ lower than in non-CKD/ESRD siblings

• Lower scores in

– Verbal performance

– Memory

– Executive function

– Problem solving

Psychological Concerns

• Issues with short stature, looking younger

than stated age

• Depression

• Fear/anxiety

• Needle phobia

• Family needs

• Non-adherence

Transition of Care

• Movement of patients from pediatric to

adult healthcare services

• Occurs between 18-21 years of age

• New problem created by improvement in

pediatric patient survival

• High risk time; requires a lot of

preparation; optimally “Transition Clinic”

Treatment Options for

Pediatric ESRD

Choosing an Option

for ESRD Treatment

• Pediatric Nephrologists usually follow their

patients from early stages of CKD

• Formal “options meeting” when patient

enters CKD 4 (GFR <30 ml/min)

– Discuss both dialysis options AND transplant

• Transplant almost always preferred in

children

Transplantation

Transplantation

• Preemptive transplantation is

treatment of choice!

• Only 20% of children are able

to get a preemptive transplant

– Size

– Lack of donor

– Late diagnosis

• Dialysis should be used as a

“bridge to transplant”

Benefits of Renal

Transplantation in Children

• Freedom from dialysis!

• Lower mortality/morbidity

• Improved growth

• Improved cognition and school performance

• More normal lifestyle

Special Considerations in

Pediatric Transplantation• Shorter waiting times

– Prioritized allocation to pediatric recipients

– Living donors

• Technical issues

– Smaller children

• Increased risk of infection (EBV, CMV, BK)

• Recurrent disease

• Adherence concerns (teens)

Peritoneal Dialysis

Peritoneal Dialysis

• Preferred dialysis modality for most children, particularly infants

• Technically easier, no needles

• Minimal interference with school and after-school activities

• Less restrictive diet

• Allows parents to provide their child’s care

Peritoneal Dialysis

• CCPD (rarely CAPD)

• Children are usually

high transporters

• Dwell volume

– 40 ml/kg

– 1100 ml/m2

• Treatment time

– 8 hrs; can be much

longer (12-13 hrs) in

infants

Peritonitis

• Peritonitis rate highest in youngest children

– < 1 yrs--1:15.3 months

– > 12 yrs--1:21.2 months

• Young children more likely to be hospitalized

• G-tube considerations

– Placement at a different time than PD catheter

– Placement as far as possible from PD catheter

Hemodialysis

Hemodialysis

• Less desirable dialysis

choice for children

– More restrictive diet

– Interrupts normal activities

– Access challenges

– Travel challenges

• Reserved for pediatric

patients with:

– PD failure

– Noncompliance issues

– Patient/family preference

Fistula

• Preferred access unless

– Weight < 20 kg

– Bridging to peritoneal

dialysis

– Expectation of transplant in

1 year

• Takes longer to mature

than in adults (> 4 months)

Graft

• Synthetic material

• Can be used in a

couple of weeks

• May be useful in

children with

smaller vessels

Tunneled Catheter

• Least desired access

• May be used in

– Children < 20 kg

– Bridging to PD

– Transplant expected in 6-12 mos

– Exhausted access

• Complications

– Infection

– Thrombosis

– Vessel stenosis

– Pro-inflammatory state

8F Medcomp 8-20 kg10F Medcomp Split 20-40 kg14F Medcomp Split >40 kg

Hemodialysis Equipment• Dialyzers

– F4 42 ml <20 kg

– F160 83 ml >20-80 kg

– F180 99 ml >80 kg

• Dialysis tubing– Neonatal 24 ml <10 kg

– Pediatric 67 ml 10-20 kg

– Adult 130 ml >20 kg

• Volume of dialyzer + tubing should be <10% of patient’s blood volume (80 ml/kg x wt x 0.1)

• 80 ml/kg x 18 kg x 0.1 = 144 ml

• F4 dialyzer (42 ml) + peds tubing (67 ml) = 109 ml

Circuit Priming• Needed in patients < 10 kg or

when circuit volume is >10%

of blood volume

• Prevents instability during

initiation of treatment

• Priming options

– Heparinized, diluted prbcs

from Blood Bank (hct 35%)

– 5% albumin

• Blood should not be returned

to patient at end of treatment

Hemodialysis Prescription

• Pediatric blood flow rate is

usually much lower than adult

blood flow rate

• For 18 kg patient, blood

flow18 x 5 = 90 ml/min

• Dialysate flow is usually 500

or 800 ml/min

• Treatments 3.5-4 hrs x 3

(may need 4x/week in

infants/toddlers)

Fluid Balance

• Dry weights are smaller and more

unusual values

– Ex. 10.5 kg

– Dry weights change with growth

• Fluid removal goals smaller and unusual

– Max removal for infant

– 10.5 kg x 13 ml/kg/hr x 3.5 hr = 480 ml

• Pre/post weights may involve

– Infant scale

– Staff supervision

• Errors have greater risk

• Blood volume monitoring helpful

Anticoagulation

• Heparin bolus

– Same scheme as adults

• Heparin infusion

– Occasionally used in infants, acute setting

• NS boluses q 30 min

– F4 100 ml flush

– All others 200 ml flush

Remember, smaller child, smaller dialyzer, smaller

flush volume

Medication Dosing

• Cannot use standard adult doses in children

• Children vs. adults

– Smaller size

– Increased volume of distribution

– Increased drug metabolism

• Drug dosing is usually in mg/kg

– Vancomycin 20 mg/kg x 18 kg = 360 mg

– Venofer load 1.4 mg/kg x 18 kg = 25 mg

Hemodialysis Adequacy

• KT/V goal > 1.2

• URR goal > 65%

• PCR goal higher in children than adults because of higher protein requirement for growing children

– < 6 mos >2.2

– 6 mos-3 yrs >1.8

– 4-6 yrs >1.5

– 7-10 yrs >1.2

– 11-14 yrs >1.0

Conclusions

• Children with ESRD are not little adults!

• Pediatric ESRD is due to congenital disorders and GN, not HTN and diabetes

• Cr and BP have age-adjusted norms

• Transplant is first choice, followed by PD then HD

• Medication doses, HD and PD Rx are all dosed by weight

• Special issues: growth, development, short stature, transition

• Risk of error is higher, particularly in infants

• Kids are resilient, and generally do well!

Questions

Question 1

• The most likely cause of ESRD in a 4 year

old boy is:

– A. Hypertensive nephrosclerosis

– B. Congenital kidney disease (renal dysplasia)

– C. Lupus nephritis

– D. Diabetic nephropathy

Question 2

• A 5 year old girl has a serum Cr of 1.0 mg/dl.

She most likely has:

– A. Normal kidney function

– B. End stage kidney disease

– C. Lab error

– D. Impaired kidney function

Question 3

• Which statement is true regarding pediatric

blood pressure?

– A. 120/80 is a normal BP for a 4 year old girl

– B. Children on dialysis can develop target organ

damage (LVH) but rarely have cardiac events

– C. Goal BP for all pediatric dialysis patients is

under 140/90

– D. Hypertension in a child is defined as BP > 50th

percentile

Question 4

• Which of the following is a manifestation of renal bone disease (CKD-MBD) seen only in children?

– A. Brown tumor

– B. Rickets

– C. Fractures

– D. Osteopenia

Question 5

• Which statement is correct regarding growth/nutrition in pediatric dialysis patients?

– A. Oral and G-tube dietary supplements may be needed to achieve nutritional goals

– B. Growth hormone injections are used to improve weight gain

– C. Dialysis patients cannot become obese

– D. Phosphate binders such as Renvela are not indicated in children

Question 6

• Which statement is true about pediatric dialysis patients?

– A. A mature 17 year old boy may sign himself off treatment early

– B. All pediatric HD patients have developmental delay

– C. Children on dialysis may transition to adult providers at age 16 years

– D. Pediatric dialysis patients have low QOL scores

Question 7

• The treatment of choice for children with

ESRD is:

– A. Chronic ambulatory peritoneal dialysis (CAPD)

– B. In center hemodialysis

– C. Kidney transplantation

– D. Chronic cycling peritoneal dialysis (CCPD)

Question 8

• Which of the following is true regarding pediatric hemodialysis access:

– A. Catheters are the preferred access in children to avoid needlestick pain

– B. Catheter choice is based on the child’s size/weight

– C. Fistulas mature more quickly in children than adults

– D. Fistulas are the preferred access in children < 20 kg

Question 9

• To prevent instability when initiating

hemodialysis, the total volume of the circuit

(dialyzer + tubing) should be:

– A. Less than 200 ml

– B. Less than 10% of the patient’s blood volume

– C. 10 ml/kg

– D. Less than 2% of the patient’s blood volume

Question 10

• All of the following are advantages of PD over

HD in children EXCEPT:

– A. Better school attendance

– B. No needles

– C. Fewer hours on dialysis machine

– D. Less restrictive diet