Course
Pediatric End Stage Renal Disease
Course Highlights
- In this Pediatric End Stage Renal Disease course, we will learn about the epidemiology, etiology, and pathophysiology of pediatric end stage renal disease (ESRD). .
- You’ll also learn the signs and symptoms of ESRD in pediatric clients.
- You’ll leave this course with a broader understanding of the types of treatment for pediatric ESRD.
About
Contact Hours Awarded: 3
Course By:
Devon Capristo, MSN, ARNP
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The following course content
Introduction
Pediatric end-stage renal disease (ESRD) can present unique challenges for the client, parents and/or guardians, and healthcare providers, particularly nurses, who play an integral role in the care of these children. ESRD in children can greatly affect their development, growth, and quality of life, all requiring custom approaches to management and support. Nurses must skillfully address the complexities of pediatric ESRD by understanding its causes, risk factors, evaluation, and treatment while providing compassionate care.
Definitions
Understanding the definitions of (ESRD) is important for nurses to determine what type of progression, treatment and care a pediatric client may need. ESRD can also be known as end-stage kidney disease (ESKD) (14). End-stage renal disease (ESRD) is also known as chronic kidney failure (1). This can occur when the pediatric client’s kidney becomes increasingly unable to perform all its functions such as filtering and clearing the toxins and waste from the blood and regulating the level of red blood cells (RBCs) that the body produces. ESRD is a disease of the affected kidney that is past the point of healing or treatment, therefore the affected kidney is no longer able to perform its priority functions (1).
ESRD is typically defined as chronic kidney disease (CKD) stage 5, and it is important to understand the staging of chronic kidney disease as well as to see how the disease progresses and how a pediatric client progresses to ESRD.
CKD is a state of permanent kidney damage and/or a decrease in the glomerular filtration rate (GFR) relates to an advanced loss of kidney function over time. The degree of kidney function loss can lead to a wide range of complications which become more evident with a progressive decline in kidney function. CKD can be defined by the presence of functional or structural kidney damage that persists over a minimum of 3 months (10). Functional damage is defined by a sustained reduction of the estimated glomerular filtration rate (eGFR), persistent increases in urinary protein excretion, or a combination of both (10). The staging of CKD in pediatrics can be seen below in the following table.
Stages of Chronic Kidney Disease |
||
GFR Category |
GFR (ml/min/1.73 m²) |
Terms |
G1* | 90+ | Normal or high |
G2* | 60-89 | Mildly decreased |
G3a | 45-59 | Mildly to moderately decreased |
G3b | 30-44 | Moderately to severely decreased |
G4 | 15-29 | Severely decreased |
G5 | <15 | Kidney failure |
*In the absence of evidence of kidney damage, neither GFR Category 1 nor 2 fulfill the criteria for CKD |
Table 1. Stages of Chronic Kidney Disease for Pediatric Clients (14)
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Self Quiz
Ask yourself...
- Have you taken care of a pediatric client with end-stage renal disease in your nursing area?
- What do you hope to learn from this course regarding pediatric clients with end-stage renal disease (ESRD)?
- Why is it important to differentiate between ESRD and CKD in a clinical setting?
- How many stages of chronic kidney disease (CKD) are there?
- What stage of CKD is ESRD typically defined as?
Epidemiology
ESRD is relatively uncommon worldwide, with reported incidence rates varying by country, ranging from 1.9 to 18 cases per million in children up to 18 years old (14). This variability can be due to differences in the ability to manage and diagnose children with significant kidney impairment as well as environmental and genetic factors. The prevalence of ESRD in pediatrics in the United States is rare as well and the National Health and Nutrition Examination Survey (NHANES) showed that less than 1% (0.51%) of US Children from the ages of 12-17 have a low eGFR of less than 60 mL/min/1.73 m2. According to the North American Pediatric Renal Transplant Cooperative Study (NAPRTCS) chronic renal insufficiency database, 5,651 clients aged 2–17 years had an estimated eGFR below 75 mL/min/1.73 m² (4).
Furthermore, over the past 20 years, the incidence of ESRD has steadily risen across all ethnic groups. In the United States, the incidence and rate of progression to ESRD are equal in both male and female clients. ESRD rates in black individuals are noted at 2.7 times higher than in white individuals which could be due to genetic susceptibility, limited access to quality medical care, or other socioeconomic problems (4). As of December 2020, 1,092 pediatric clients were awaiting kidney transplants in the United States. The youngest clients with ESRD typically have the least favorable outcomes and those younger than one year old will have a 5-year survivability of 76% (5). Ongoing education and a deeper understanding of ESRD in the pediatric population enable healthcare providers to deliver more effective and informed care.
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Self Quiz
Ask yourself...
- How does the reported prevalence of ESRD in pediatric clients in the U.S. compare to global trends?
- Where does the variability in the epidemiology of ESRD stem from?
- Why might ESRD rates in Black individuals be 2.7 times higher than in White individuals?
- Why might the youngest clients with ESRD, particularly those under age one, have the least favorable outcomes?
- How might ongoing education about ESRD in the pediatric population improve care delivery?
Etiology
Nurses need to understand the etiology of ESRD in pediatric clients because this knowledge directly influences client care and outcomes. There are multiple causes of ESRD in pediatric clients which can include congenital abnormalities of the kidneys and urinary tract (CAKUT). CAKUT counts for around 30-35% of the causes of ESRD in pediatrics which require kidney transplantation (2)(16). Conditions that commonly fall under this group include;
- Hydronephrosis (swelling in kidneys due to buildup of urine)
- Vesicoureteral reflux (VUR) (urine backflows from the bladder into the ureters and kidneys)
- Hypoplastic or dysplastic kidneys (kidneys that do not fully develop while the fetus is in the womb, or a kidney is smaller than the average size)
- Multi-cystic dysplastic kidneys (abnormal kidney development while the fetus was still in the womb) (2).
- Outside of CAKUT, there are additional causes of ESRD in children including;
- Focal segmental glomerulosclerosis (FSGS) (scarring in the glomeruli)
- Hemolytic uremic syndrome (small blood vessels of the kidneys become damaged and inflamed)
- Secondary glomerular disease (i.e., systemic lupus nephritis). This etiology plus primary glomerular disease, accounts for up to 1/3 of children with kidney failure (29.5% of clients with ESRD)
- Genetic disorders such as oxalosis, cystinosis, and Alport syndrome
- Interstitial nephritis (causes inflammation in the renal interstitium) (4.7% of clients with ESRD)
- Unidentified or unknown primary underlying etiology (6.2% of clients with ESRD) (16).
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Self Quiz
Ask yourself...
- Why is it essential for nurses to understand the specific causes of ESRD in pediatric clients?
- What are some common congenital abnormalities that lead to ESRD in pediatric clients?
- Why do congenital abnormalities of the kidneys and urinary tract (CAKUT) related conditions account for such a significant proportion of ESRD cases requiring kidney transplantation?
- In addition to CAKUT-related conditions, what are other causes of ESRD in pediatric clients?
Pathophysiology
Regardless of the cause of the kidney injury, once the CKD develops, the course is similar. In the beginning, the kidney will adapt to the damage by increasing the filtration rate in the healthy nephrons which is called adaptive hyperfiltration. These changes will maintain clients in a normal or near-normal serum creatinine concentration and the body will adapt and allow the concentrations of potassium, sodium, calcium, phosphorus, and total body water to remain within normal limits, particularly in those clients with mild or moderate stages of CKD. This increase in filtration can potentially damage the glomeruli of the remaining nephrons which is seen later in proteinuria and progressive insufficiency of the kidneys. The irreversibility of these changes is responsible for the development of ESRD in clients with inactive or cured original illness (4).
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Self Quiz
Ask yourself...
- What is adaptive hyperfiltration, and how does this lead to chronic kidney disease (CKD)?
- How does proteinuria signal the progression from CKD to ESRD?
- What factors might accelerate or slow the progression from CKD to ESRD in pediatric clients?
Clinical Signs and Symptoms
Nurses who recognize the clinical signs and symptoms of pediatric clients with ESRD can develop early recognition and intervention, implement comprehensive client care, support client growth and development, and adapt client education. ESRD in the pediatric population can present itself in multiple ways. Complications and symptoms often appear well before an ESRD diagnosis is established. Comorbidities of kidney disease in children may include but are not limited to: (12)
- Cardiovascular disease
- Anemia
- Electrolyte imbalances, especially potassium
- Growth and development issues (i.e., stunted growth)
- Hypertension
- Infection
- Urinary incontinence
- Cognitive issues
- Mineral and bone disorders
- Metabolic acidosis
- Relationship, behavioral, and self-esteem problems
Due to the previously discussed adaptive hyperfiltration, children in the beginning stages of kidney disease may have few or no symptoms at all. As the disease progresses, symptoms may include (10, 12)
- Edema (leading to weight gain)
- Polyuria
- Oliguria
- Proteinuria (leads to foamy urine)
- Hematuria
- Malaise
- Decreased appetite
- Fever
- Hypertension
- Urticaria
- Nausea or vomiting
- Shortness of breath
- Difficulty concentrating
- Weight loss
- Weakness
- Fluid and electrolyte imbalances such as metabolic acidosis, hypokalemia, hyperkalemia, sodium and water homeostasis
- Hyperlipidemia
- Endocrine abnormalities
- Uremia (a decreased clearance of waste excreted by the kidneys) which can cause uremic bleeding or uremic pericarditis
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Self Quiz
Ask yourself...
- Why is it essential for nurses to understand the specific causes of ESRD in pediatric clients?
- How can nurses differentiate between early signs of chronic kidney disease and symptoms of ESRD?
- What are the most common comorbidities associated with ESRD in pediatric clients, and why might they develop?
- How could growth and development issues impact children with ESRD in comparison to adults with ESRD?
- Why in the beginning stages of ESRD might children show few clinical signs and symptoms?
- What symptoms would you expect a pediatric client to have with ESRD?
Diagnosis and Assessment
Nurses must understand the diagnosis and assessment of pediatric clients with ESRD to provide timely interventions, manage complications effectively, and support the child’s overall growth, development, and quality of life. The diagnosis of CKD in children, including whether it has progressed to ESRD, can be determined through various methods. The diagnosis of CKD can be approached in two ways: identifying structural problems with the kidneys (diagnostic) or evaluating how well the kidneys are functioning (functional).
Primary testing in pediatric clients with suspected CKD needs to include a urine examination as well as an estimation of the glomerular filtration rate. An essential part of the initial evaluation and assessment is to determine the length of the disease duration (4). The initial assessment is best completed by comparing the current plasma creatinine concentration (PCr) or urinalysis with previous results (4).
In addition, imaging techniques like ultrasound and radionuclide studies can help confirm a CKD diagnosis while providing insights into its underlying cause (4). The diagnosis of CKD, and ultimately ESRD, is achieved through a multifaceted approach involving the entire clinical care team managing the pediatric client.
As previously noted, pediatric clients may not exhibit symptoms until the later stages of disease progression due to the kidneys’ compensatory mechanisms. In most cases, clients remain asymptomatic during Stage 1 and Stage 2 kidney disease. An abnormal urinalysis or elevated blood pressure is often among the earliest indicators of CKD, prompting further assessment and evaluation in pediatric clients (4).
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Self Quiz
Ask yourself...
- Why must nurses understand the steps of diagnosis and assessment in pediatric clients?
- What two ways is the diagnosis of kidney disease approached?
- What 2 modalities are involved in the primary testing in the diagnosis of chronic kidney disease (CKD) in children?
- What is the role of serum chemistry and CBC count in the diagnostic work-up for chronic kidney disease (CKD) and end-stage renal disease (ESRD)?
- In what stages do most CKD clients remain asymptomatic?
Blood Studies
Important aspects of CKD staging and ESRD diagnosis include multiple laboratory tests that include but are not limited to: (4)
- eGFR (estimated glomerular filtration rate)
- CBC (to rule out anemia)
- Serum chemistry (BUN, serum creatinine, sodium, potassium, calcium, phosphorus, alkaline phosphatase, bicarbonate, parathyroid hormone, cholesterol)
- Lipid panel
Additionally, after a diagnosis of either CKD or ESRD is made, intervals of blood testing should be performed on the pediatric client. Please see the table below.
Laboratory Tests and Frequency for Children with Chronic Kidney Disease(Stages 2 to 5) |
||||
Laboratory Test |
Frequency of Testing |
|||
CKD Stage 2 |
CKD Stage 3 |
CKD Stage 4 |
CKD Stage 5 |
|
Electrolytes | 12 months | 6 to 12 months | 3 to 6 months | 1 to 3 months |
Calcium/Phosphorus | 12 months | 6 to 12 months | 3 to 6 months | 1 to 3 months |
Parathyroid hormone | 6 months | 6 to 12 months | 3 to 6 months | |
Alkaline Phosphatase | 12 months | 12 months | ||
25-Hydroxyvitamin D | 6 to 12 months | 6 to 12 months | 6 to 12 months | 6 to 12 months |
Lipid Profile | 12 months | 12 months | 12 months | 12 months |
Hemoglobin | 12 months | Every 6 months | Every 6 months |
Table 2. Laboratory Tests and Frequency for Children with CKD Stages 2 to 5 (10)
The eGFR plays a crucial role in the staging and diagnosis of CKD and ESRD in pediatric clients and should not be underestimated. The glomerular filtration rate (GFR) shows how well the kidneys are filtering the toxins and waste out of the blood (3). Obtaining an accurate GFR level proves to be challenging because the measured GFR (mGFR) is an extensive and tedious process. The mGFR measures how the kidneys are filtering certain chemicals not produced by the body such as inulin and iohexol. This test is not widely available and is more expensive (3). Due to this, clinicians use a formula called the estimated GFR (eGFR) with the use of a simple blood test (3).
The estimated GFR comes from a calculation that determines how well the kidneys are filtering certain agents that are produced by the body such as cystatin C (a protein that slows the breakdown of protein cells) and creatinine (a waste product that comes from the normal breakdown of muscles). This test is widely available, and less expensive than its counterpart, however, it can be less accurate and less precise (3). In pediatric clients, eGFR needs to be calculated from either the Counahan-Baratt prediction or Schwartz equation in children due to it being reasonably precise, practical, and convenient (4).
Recent advancements in kidney research have incorporated cystatin C alongside creatinine measurements in eGFR calculations. Previously, eGFR was primarily determined using serum creatinine levels. However, the newer “CKiD under 25” equations have improved accuracy by including cystatin C as a reliable marker of kidney function. This updated method accounts for both age- and gender-specific constants, utilizing measurements of both creatinine and cystatin C (4).
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Self Quiz
Ask yourself...
- What blood studies are included in the diagnosis of CKD in pediatric clients?
- Why is the estimated GFR so important in the diagnosis of CKD in pediatric clients?
- Explain the difference between an estimated glomerular filtration rate (eGFR) and a measured glomerular filtration rate (mGFR). Which one is preferred and why?
Urine Studies
These studies can be deemed the most important test in determining the severity of CKD in pediatric clients. The following urine studies used in the diagnosis of CKD and eventual ESRD include but are not limited to:
- Urine microscopy via dipstick (RBCs, WBCs, casts)
- Spot urine specimen (useful in detecting protein, albumin)
Novel Biomarkers
While there are important biomarkers in the diagnosis of CKD (serum creatinine and proteinuria), these typically do not show up until much later in the progression of the disease. Research shows that there are additional biomarkers that can show an ESRD risk factor correlation. These include tumor necrosis factor receptor 1 (TNFR1) and tumor necrosis factor receptor 2 (TNFR2). These both show a strong correlation with a progression to ESRD even after the eGFR and albuminuria are controlled (4).
Ultrasonography
A commonly used radiographic technique in clients with ESRD is ultrasound. All clients who present with potential chronic or acute kidney failure should have a US performed due to the ability to rule out obstructive diseases. Ultrasounds can additionally be useful in determining a simple benign cyst from a more complex cyst or solid tumor or they can be used as a screening tool for different types of polycystic kidney disease (4).
CT Scan
Additional use of computed tomography (CT) scans can prove useful in the diagnosis of CKD and ESRD. CT scans allow for a high anatomic definition of the kidneys and provide a panoramic image of the urinary tract and kidneys. These exams are fast and volumetric, meaning three-dimensional images are created, which allow a thorough assessment of the organs and tissues throughout the body (13).
Radionuclide Studies
Radionuclide Studies prove to be very beneficial in determining the potential cause of ESRD. The detection of renal scarring in the early stages of CKD can be possible with radioisotope scanning with 99m (99m)-technetium dimercaptosuccinic acid (DMSA). This initial detection can lead to the early diagnosis of reflux nephropathy (4). With the use of radionuclide tracer studies, voiding cystourethrography can detect vesicoureteral reflux while retrograde or antegrade pyelography can be used to diagnose and treat urinary tract obstructions. However, consultation with a pediatric Urologist is suggested before obtaining this testing on a pediatric client with suspect obstructions (4).
Kidney Biopsy
Additional information can be obtained from kidney biopsy on potential CKD clients. If glomerulonephritis, vasculitis, unexplained CKD, or acute kidney failure is suspected, a renal biopsy can be performed to determine the probable cause of the CKD (4). In the late stages of CKD (stages 4 and 5) regardless of the cause, the findings of the biopsy are typically consistent with segmental and globally sclerosed (hardened) glomeruli and tubulointerstitial atrophy (4).
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Self Quiz
Ask yourself...
- What urine tests are useful in the diagnosis, evaluation, and management of CKD and ESRD?
- Which biomarkers have a strong correlation showing the progression from CKD to ESRD in pediatric clients?
- How can ultrasonography be useful in the diagnosis of clients with CKD and ESRD?
- Who should be consulted before performing radionuclide studies on a pediatric client with suspected obstructions due to CKD or ESRD?
- In the late stages of CKD, what diagnoses are detectable with the use of a kidney biopsy in pediatric clients?
Treatment
Understanding the progression of treatment in a CKD and ESRD pediatric client is important to provide client-centric care to the client and the parents and/or guardians. Because chronic kidney disease (CKD) causes a gradual decline in kidney function and can progress to end-stage renal disease (ESRD), evaluating individual factors for each pediatric client is essential to potentially slow its progression.
Factors in all ages: (16)
- Hypertension: Elevations in blood pressure (BP) are associated with a decline in GFR independent of proteinuria.
- Proteinuria: Elevated urinary protein excretion and hypoalbuminemia reflect glomerular permeability and injury. The rate of progression to higher stages of CKD increases with the level of proteinuria even in clients who have normal blood pressures.
- Genetic predisposition: The genotype APOL1 increases the vulnerability to CKD and can contribute to a faster rate of progression. Additionally, genetic conditions like cystinosis and Alport syndrome contribute to the higher incidence of CKD in children compared to adults.
- Recurrent pyelonephritis: This is a risk factor for CKD and is especially seen in children with CAKUT
- Anemia
- Hypovitaminosis D
- Hyperphosphatemia
- Hypocalcemia
- Metabolic acidosis
- Hyperlipidemia
Additional factors relevant to children: (16)
- Neonatal factors: Fetal growth restriction and prematurity are risk factors for CKD, as kidney injury can occur during the critical period of nephron development, which typically continues until around 35–36 weeks of gestation.
- Growth: CKD typically accelerates during rapid growth, so children with CKD should be monitored more closely during adolescence for rapid progression of CKD.
- Underlying primary disease: accelerated CKD progression is associated with pediatric clients with glomerular disease (proteinuria and hypertension association) vs. non-glomerular disease
- Novel biomarkers: Currently there are several plasma and urinary proteins under active study to identify novel biomarkers that predict CKD progression in children (16).
Presently, there is an online prediction tool that can estimate when a child with CKD may progress to ESRD and require a kidney transplant. The estimated time is based on variables measured in clients including but not limited to CKD diagnosis, proteinuria, GFR, blood pressure category, serum albumin, anemia, serum bicarbonate, and serum chloride. (7). The calculator is based on the data from the Chronic Kidney Disease in Children (CKiD) study in addition to other CKD publications (7).
Before the full development of ESRD (CKD Stage 5), treatments of CKD should include: (4)
- Evaluation and management of reversible causes of kidney dysfunction
- Specific treatments based on diagnosis and etiology
- Prevention and treatment of comorbidities of decreased renal function such as bone disease, hypertension, anemia, growth failure, and cardiovascular symptoms
- Slowing the loss of kidney function
- Preparing the client and family for kidney failure treatment
- Replacing kidney function with dialysis and transplantation if signs and symptoms of uremia are present
- Managing complications
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Self Quiz
Ask yourself...
- What is included in treating and evaluating chronic kidney CKD and ESRD in pediatric clients?
- What are factors present in all ages that are crucial to understand in the slowing of the progression of CKD?
- What are factors specific to children that can accelerate the progression from CKD to ESRD?
- Before the full development of CKD to ESRD, what treatment modalities should be implemented?
Kidney Failure Treatment Plans
As healthcare providers, nurses must be thoroughly informed about the treatment and management plan following a kidney failure diagnosis. In pediatric clients with CKD, kidney replacement therapy (KRT) is typically needed when the GFR falls below 15 mL/min per 1.73 m2 (stage G5, CKD, kidney failure). In certain circumstances, KRT is required before this stage. Pediatric kidney specialists will typically start to prepare the client and family for KRT when the eGFR falls to <30 mL/min per 1.73 m2 (stage G4). The family should be educated about the timing and choice of KRT (preemptive kidney transplantation, hemodialysis, and peritoneal dialysis (10).
It should be noted that it is rare for parents and/or guardians of a child with ESRD to choose conservative management and death over lifelong dialysis and KRT. On occasion, this should be considered a choice that is legally, ethically, and medically acceptable. If a decision is made to forego KRT, the family must be supported emotionally and provide the child with all the care necessary to remain pain-free (10). Nurses have the ability to be a supportive and helpful client advocate during these decisions and assist in ways to keep the client as comfortable as possible.
Incidence and Prevalence of Kidney Replacement Therapy
Around the world, most children who receive KRT live in the United States, Japan, Europe, and Australia/New Zealand. In the United States, the incidence of KRT in pediatric clients with ESRD is approximately 12 clients per million children. In 2020, this represented 912 clients from ages 0 to 17 years old. Globally, the prevalence of KRT has risen due to improved survival rates with reported figures ranging from 18 to 100 per million in age-specific populations (14).
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Self Quiz
Ask yourself...
- Why is it important for nurses to be involved in the planning of late-stage kidney disease?
- When is kidney replacement therapy (KRT) typically initiated?
- What therapies are included in kidney replacement therapy?
- In the event a family chooses conservative management, what resources should the healthcare team provide?
- What factors might contribute to the rising global prevalence of kidney replacement therapy (KRT) among pediatric clients?
Kidney Replacement Therapy Modalities
There are multiple adjunctive therapies used in a client with ESRD. The therapy options for children with ESRD include kidney transplantation, peritoneal dialysis (PD), and hemodialysis (HD). The type of modality used with each client can depend on age and availability of resources. Global data has shown that PD is commonly the initial KRT therapy in children <9 years of age while HD is commonly used in children >10 years old. In the United States PD is most common in children < 5 years old. Over time, kidney transplantation becomes the preferred method of KRT as clients shift from initial dialysis to transplantation (14).
Of the 3 modalities used for KRT, kidney transplantation is preferred over dialysis (both PD and HD) for several reasons. All-cause mortality rates are lower for children who received a kidney transplant than for those on dialysis (14). The survival probability at 5 years for children who received a transplant was 0.96 while it was at 0.81 and 0.86 for HD and PD respectively (11). In addition, kidney transplant is associated with better outcomes concerning quality-of-life measures, growth, and development in comparison to HD and PD. Transplantation is less disruptive regarding schooling, social interactions, and family lifestyle than dialysis. Additionally, transplantation avoids the fluid and dietary restrictions that are required while on dialysis. Lastly, there are increased risks of dialysis including a higher risk of cardiovascular disease (cerebrovascular disease, cardiomyopathy, ischemic heart disease, heart failure, cardiac arrhythmias/arrest) (14).
Kidney Transplant Timing
As mentioned previously, mortality is known to be lower in pediatric clients who receive preemptive transplantation than in those clients who have been on chronic dialysis. Additionally, preemptive transplantation is easier to perform in children than in adults because of the availability of parent donors who in most cases are a haplotype match. The parents also are usually young and healthy and more willing to donate their kidneys. If a living donor is not available, numerous countries prioritize children on the deceased donor transplant list (14).
Kidney Transplant Surgery
Preparing clients and their family members for the actual kidney transplant is vital for a healthy life for the client with an improved prognosis. In the United States in clients under 18 years old, about 800 kidney transplants are performed annually (8). As previously noted, preemptive or primary transplantation is the preferred form of kidney replacement therapy (KRT) because it reduces client mortality and supports better growth outcomes, particularly in children aged six years and under. However, preemptive transplants are not possible in the following circumstances: (8)
- ESRD from autoimmune disease with consistently high titers of autoantibodies
- Need for transplant nephrectomies (i.e., chronic pyelonephritis, malignant renovascular hypertension, nephrotic syndrome with complications from hypercoagulability)
- Underlying kidney disease that is active and associated with a rapidly progressive disease
- Ongoing active infections
- If the client or parents/guardians are not able to deal with the care necessary post-transplantation
In pediatric clients, the contraindications to preemptive kidney transplantation include:
- Overwhelming systemic septic infection
- Uncontrolled malignancies outside of the kidneys (extrarenal)
- Severe pulmonary or cardiac dysfunction
- Life-threatening disorder of extrarenal origin that would not be corrected by an organ transplant
- Severe multisystem organ failure not corrected by organ transplant
- Increased levels of circulating anti-glomerular basement membrane antibodies
Before the surgery, the client will need to complete a thorough pretransplant evaluation. This can include the recognition of anti-human leukocyte (HLA) antibodies of the donor client, detection and treatment of any infections, completion of childhood immunizations (including varicella), correction of urinary tract abnormalities, review of whether or not the kidney replacement would be beneficial long term (8). Nurses play an important role in the education of the client and family before surgery. The majority of client education will happen during this time which can in turn provide better outcomes for the client and their family.
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Self Quiz
Ask yourself...
- What is the recommended KRT in children < 9 years old? What is the recommended KRT in children >10 years old?
- What makes kidney transplantation the preferred KRT over hemodialysis?
- What advantages might preemptive transplantation offer in managing ESRD in pediatric clients?
- Why is preparing clients and their families for kidney transplantation important for ensuring a healthy life and improved prognosis?
- In what circumstances are preemptive transplants not allowed/contraindicated in pediatric clients?
Kidney Transplant Outcomes
Knowing how to take care of a pediatric client and their caregiver’s post-kidney transplant is vital to clients’ proper healing and prognosis. Overall, the outcomes of kidney transplantation in children have improved over the last several years. The 1-year graft survival rates for transplants performed in 1987 vs. 2010 were 81% vs. 97%. The 5-year graft survival rates for the transplants performed in 1987 vs. 2006 were 59% vs. 78% and lastly, the 10-year graft survival for transplants done in 1987 vs. 2001 was 47% vs. 60% (9). Several factors can affect allograft survival in pediatric clients including: (9)
- Source (deceased vs. living donor) and age of donor
- Improved immunosuppressive regimen
- Preemptive transplantation before the initiation of dialysis
- Recipient factors (race and age)
- HLA compatibility
- Acute rejection
- Infections
- Underlying primary disease
- Repeat transplant
- Delayed graft function (DGF)
- Adherence to immunosuppressive medication regimen
Post-transplant aftercare is crucial for nurses to understand to provide appropriate client education. Clients typically recover in the hospital for about a week, though longer stays may be required if complications arise. Blood work will be conducted to monitor the new kidney’s function, and some clients, particularly those receiving a kidney from a deceased donor, may need temporary dialysis until the kidney begins functioning independently. Nurses play a key role in educating families on post-transplant care, including limiting physical activity until recovery, promoting movement to build strength and support healing, recognizing signs of infection or rejection, and understanding the lifelong need for immunosuppressive medications (6). Client-specific education during this critical period is essential
Dialysis
When preemptive transplantation is not a viable option, a choice needs to be made between HD and PD. To make this decision, a client’s multi-professional team—which includes the family physician, dialysis nurse, nephrologist, social worker, and psychologist—assesses various factors, such as the family’s psychological, social, and economic background (10). The factors evaluated include: (10)
- Client age and size: PD is preferred in young children and infants as it is easier to perform due to not requiring vascular access.
- Comorbidities: PD is preferred over HD when there are contraindications to the use of anticoagulants and in clients who have cardiovascular instability.
- Technical, social, and compliance issues: Home dialysis (HD or PD) is preferred due to less impact on schedules, schools, etc. However, if home dialysis is not possible, the choice is managed based on availability, vascular access, etc.
- Client and parent/caregiver preference
- Preservation of dialysis access
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Self Quiz
Ask yourself...
- What are the survival outcomes for pediatric clients post-kidney transplant?
- What factors can affect allograft survival in pediatric clients status-post kidney transplant?
- What are the essential duties of a nurse during the post-transplant period?
- What factors are involved in the decision to place a pediatric client on dialysis?
- In what ways could home dialysis positively or negatively impact a client’s daily life and responsibilities, such as school or work?
Planning for Dialysis
There are important aspects of planning for dialysis (either HD or PD) for pediatric clients that need to be taken into consideration. For HD, an evaluation needs to occur for vascular access options which include arteriovenous fistula (AV fistula) or a central venous tunneled catheter. If an AV fistula is chosen, the time it takes for the maturity of the fistula needs to be taken into account as this can take several weeks to several months. If a central venous catheter is chosen, the parents and the client need to be aware these are typically reserved for clients too small for the surgery required for an AV fistula. Additionally, it should be noted that using central venous catheters has a higher incidence of infection complications (10).
Pediatric clients who choose to use PD must undergo abdominal surgery for catheter placement with the use of infection prevention precautions such as the use of preoperative antibiotics and double cuff catheters. Allowing 2 weeks after the surgery for wound healing can help prevent complications before the initiation of PD (10).
Dialysis Initiation
Nurses caring for pediatric clients with ESRD should understand the typical timing for initiating dialysis. The decision of when to start dialysis takes into account numerous variables, however, the most important include the eGFR and signs/symptoms of uremia. Once the eGFR drops below 15 ml/min per 1.73 m2 (CKD stage 5), the indications for initiation of dialysis include: (10)
- Decline in nutritional status and/or growth failure, marked by decreasing weight and/or height percentiles.
- Fluid overload
- Complaints from the client (i.e., nausea, loss of appetite, fatigue, perceived poor quality of life, inability to concentrate)
- Uncontrolled metabolic abnormalities (i.e., acidosis, hyperkalemia, hyperphosphatemia)
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Self Quiz
Ask yourself...
- What factors influence the choice between an arteriovenous (AV) fistula and a central venous tunneled catheter for vascular access in pediatric clients?
- To undergo peritoneal dialysis (PD) what steps must be taken?
Prognosis
As can be expected, children with kidney failure have a shortened life expectancy than children without CKD. Around 75% of children diagnosed with ESRD will undergo dialysis and the mortality rate for these children is around 30 times higher than in the general pediatric population. Infection and cardiovascular disease are the primary causes of death in these clients; however, mortality rates have decreased due to advancements in clinical expertise and improved pre-dialysis care. If a client is placed on KRT before the age of 15, they do have a greater mortality and morbidity risk than their age-matched peers. These individuals are more likely to have severe fatigue, skin cancer, and motor disabilities in comparison to others their age (10).
Research in ESRD
As with most clinical diagnoses, research is always ongoing, and pediatric ESRD is no different. One institute that is continually doing research in this area is the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). The NIDDK performs clinical trials in this population by looking for new ways to prevent, detect, and treat kidney disease to improve the quality of life in the pediatric population (12). Since pediatric clients respond to treatments and medicines differently than adults, clinical trials are necessary to find appropriate treatment and management modalities. Specifically in this population, scientists are researching many aspects of kidney disease in children such as earlier and more accurate methods of diagnosis in acute kidney failure and ways to improve client outcomes post-kidney transplant (12).
A valuable resource that nurses can give to the families of clients with ESRD is the clinical trial finder. This can be found at www.ClinicalTrials.gov. The families can narrow or expand the results to include studies from individuals, universities, or industries. It is best to educate the family members that once they find a study they are interested in, they should discuss it with the client’s healthcare provider before enrolling (12). Nurses who work with pediatric clients with kidney disease must stay at the forefront of new research that could have a substantial impact on their client population.
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Self Quiz
Ask yourself...
- What are the primary causes of death in ESRD pediatric clients?
- What areas of research are occurring about pediatric ESRD?
- What is a resource that families can use to find clinical trials?
Nursing Care of Clients with ESRD
The nursing care of these clients requires a multidisciplinary approach to ensure successful outcomes for the client population. A multidisciplinary team will meet often to monitor and review care for the pediatric client. Members of this team could include but are not limited to:
- Nephrologists
- Surgeons
- Nurses
- Dieticians
- Child-life specialists
- Social workers
- School teachers
Nurses play a crucial role in influencing the health and well-being of pediatric clients throughout the various stages of ESRD. By working closely with clients and their families, nurses can perform a wide range of essential duties, including:
- Preventing and managing complications
- Supporting the kidney transplantation or dialysis processes
- Educating on chronic condition management
- Implementing fluid restrictions and dietary recommendations
- Promoting physical and psychosocial health
- Enhancing the client’s overall quality of life
- Guiding lifestyle modifications
- Providing emotional and realistic support to clients and families
- Collaborating with nephrologists and other healthcare team members
These contributions help ensure comprehensive care and better outcomes for pediatric clients with ESRD.
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Self Quiz
Ask yourself...
- What are some commonly used names for end-stage renal disease (ESRD)?
- How long does structural or functional kidney damage occur before a kidney disorder can be labeled as chronic kidney disease (CKD)?
- Why is a multidisciplinary approach critical during the care of a pediatric ESRD client?
- How does the nurse’s role in managing complications and educating families impact the client’s long-term health outcomes?
- What key things can you take away from this course that can help you in taking care of pediatric clients with ESRD?
Conclusion
Pediatric ESRD presents unique challenges that require a whole-client, multi-disciplinary approach to care. Nurses are pivotal in this journey, offering compassion, clinical expertise, and education to clients and their families. By being able to recognize the etiology, epidemiology, pathophysiology, signs and symptoms, diagnosis, and treatment, nurses can significantly improve health outcomes and quality of life for pediatric clients. The commitment of nurses to their clients is essential in navigating the complexities of pediatric ESRD, supporting both the children and their families while advocating for the best possible outcomes.
References + Disclaimer
- Boston Children’s Hospital. (n. d.). Chronic kidney (renal) disease. https://www.childrenshospital.org/conditions/chronic-kidney-disease
- Children’s Hospital Colorado. (n.d.). End-Stage Renal Disease (ESRD). https://www.childrenscolorado.org/conditions-and-advice/conditions-and-symptoms/conditions/end-stage-renal-disease/
- National Kidney Foundation. (2022, July 13). Estimated Glomerular Filtration Rate (eGFR). https://www.kidney.org/kidney-topics/estimated-glomerular-filtration-rate-egfr
- Gulati, S. (2024, June 24). Chronic kidney disease in children. Medscape. https://emedicine.medscape.com/article/984358-overview#a1
- Gupta, R., Woo, K., & Yi, J. A. (2021). Epidemiology of end-stage kidney disease. Seminars in Vascular Surgery, 34(1), 71–78. https://doi.org/10.1053/j.semvascsurg.2021.02.010
- American Kidney Fund. (2023, January 3). Kidney transplant in children: Preparing, types, surgery, and life after. https://www.kidneyfund.org/kidney-donation-and-transplant/kidney-transplant-children-preparing-types-surgery-and-life-after#:~:text=Recovery%20in%20the%20hospital,medicines%20to%20help%20with%20pain
- Kim, E., Kuo, P., & Wang, F. (n.d.). KRT risk calculator. https://ckid-gfrcalculator.shinyapps.io/CKiD_KRT_Risk/
- McDonald, R. A. (2023, December 14). Kidney transplantation in children: General principles. UpToDate. https://www.uptodate.com/contents/kidney-transplantation-in-children-general-principles?search=pediatric+end+stage+renal+disease&topicRef=16179&source=related_link#H17417151
- McDonald, R. A. (2024, January 19). Kidney transplantation in children: Outcomes. UpToDate. https://www.uptodate.com/contents/kidney-transplantation-in-children-outcomes?search=kidney+transplant+rejection+pediatric&source=search_result&selectedTitle=2~150&usage_type=default&display_rank=2#H1
- Srivastava, T., & Warady, B. A. (2024, June 25). Chronic kidney disease in children: Overview of management. UpToDate. https://www.uptodate.com/contents/chronic-kidney-disease-in-children-overview-of-management?search=pediatric+chronic+kidney+disease&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1#H2
- Johansen, K. L., Chertow, G. M., Gilbertson, D. T., Ishani, A., Israni, A., Ku, E., Li, S., Li, S., Liu, J., Obrador, G. T., Schulman, I., Chan, K., Abbott, K. C., O’Hare, A. M., Powe, N. R., Roetker, N. S., Scherer, J. S., St Peter, W., Snyder, J., Winkelmayer, W. C., … Wetmore, J. B. (2023). US renal data system 2022 annual data report: epidemiology of kidney disease in the United States. American Journal of Kidney Diseases: The Official Journal of the National Kidney Foundation, 81(3 Suppl1), A8–A11. https://doi.org/10.1053/j.ajkd.2022.12.001
- U.S. Department of Health and Human Services. (2022b, August 1). Kidney disease in children – niddk. National Institute of Diabetes and Digestive and Kidney Diseases. https://www.niddk.nih.gov/health-information/kidney-disease/children#complications
- Viteri, B., Calle-Toro, J. S., Furth, S., Darge, K., Hartung, E. A., & Otero, H. (2020). State-of-the-Art Renal Imaging in Children. Pediatrics, 145(2), e20190829. https://doi.org/10.1542/peds.2019-0829
- Warady, B. A., & Rukshana Shroff. (2023, August 1). Overview of kidney replacement therapy for children with chronic kidney disease. UpToDate. https://www.uptodate.com/contents/overview-of-kidney-replacement-therapy-for-children-with-chronic-kidney-disease?search=end+stage+renal+disease+pediatrics&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1#H60773527
- Warady, B. A., Weidemann, D. K., & Srivastava, T. (2024, March 5). Chronic kidney disease in children: Complications. UpToDate. https://www.uptodate.com/contents/chronic-kidney-disease-in-children-complications?search=pediatric+end+stage+renal+disease&source=search_result&selectedTitle=3~150&usage_type=default&display_rank=3#H1941571480
- Warady, B. A., & Weidemann, D. K. (2024, October 16). Chronic kidney disease in children: Epidemiology, etiology and course. UpToDate. https://www.uptodate.com/contents/chronic-kidney-disease-in-children-epidemiology-etiology-and-course?sectionName=ETIOLOGY&search=end+stage+renal+disease+pediatrics&topicRef=16179&anchor=H6&source=see_link#H6
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