132–142 Potassium (mmol/l) <1 month 3.0–6.6 >1 month 3.0–5.6 Bicarbonate (mmol/l) 22–32 Urea (mmol/l) 2.5–6.5 Albumin (g/l) 34–45 Calcium (mmol/l) <1 year 2.4–2.8 1–2 years 2.3–2.7 3–16 years 2.2–2.6 Phosphate (mmol/l) <4 wks 1.2–3.1 5 wks–6 mths 1.5–2.4 6 mths–1 year 1.5–2.1 1–3 years 1.2–2.0 3–6 years 1.0–1.8 6–15 years 1.0–1.7 Adult 0.8–1.4 Creatinine (μmol/l) <5 years <44 5–6 years <53 6–7 years <62 7–8 years <71 8–9 years <80

Blood pressure increases with size/age. Height centile charts should be used to monitor blood pressure in children with short stature for accuracy. This will include many children with renal disease (National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents 2005)

Chronic Kidney Disease

The common causes of chronic kidney disease (CKD) in children are different from those experienced in later life, as indicated in Box 12.1. In comparison with the numbers seen in the adult population, it is difficult to gain accurate figures. In 2009 data suggested that the incidence of established renal disease (ERD) was 56.1 pmarp (per million age related population) and 9.6 pmp (per million population) as a whole. This rate was estimated to be 774 pmp in the adult population (UK Renal Registry 2012).

The presenting symptoms of CKD commonly seen in children are;

• Failure to thrive or anorexia.
  
• Nausea, vomiting and loss of appetite.
  
• Lethargy.
  
• Headaches and high blood pressure.
  
• Reduced urine output or polyuria/polydipsia, possibly wetting.

Children can present at any age and at different stages of renal disease. It is important that investigations are undertaken to determine cause and therefore the interventions required. Dialysis may be necessary in some cases, however many children can be treated conservatively.

Some conditions are familial and therefore siblings may also need to be assessed, even if they are asymptomatic. In rare cases a parent and one or more children may be dialysis dependent and a thorough plan of psychosocial support is a necessity in this situation.

Conservative Management

Providing the biochemistry is stable, children can be maintained without dialysis for some time. Several interventions can be incorporated into conservative management. These interventions continue into the established phase of chronic kidney disease, and may be temporarily used to support children in an acute phase of renal disease.

Diet (also see Chapter 13)

Poor appetite, gastro-intestinal reflux and vomiting are common problems seen in children and infants with chronic kidney disease. Parents often see feeding their child as an important aspect of their role, and therefore this is potentially an area of great stress (Royle 2007).

It is important that a paediatric renal dietitian is involved in the management of children with renal disorders, providing support in this area of anxiety (Department of Health 2006a). Dietary control can assist in delaying the need for dialysis and all of the medical and psychosocial problems associated with it, as discussed further below. Adequate nutrition is also important for growth and neurological development.

Energy requirements should be based upon EAR (estimated average requirement) for chronological age, or height age if the child falls below the second percentile (Royle 2007). Modified mild formulas may be necessary for infants, dependent upon biochemistry, as standard formulations may have inappropriate protein and electrolyte concentrations.

Individualised dietary guidance is needed for each family, based on the child’s biochemistry. Negotiation with food allowances is often needed with older children, to improve adherence to the nutritional plan. Clear education is needed for the child and their parents, to ensure understanding of the necessity for restricting many favourite childhood foods, such as chips and pizza. Sodium, potassium and phosphate intakes often need to be altered. A small number of patients with tubular disorders may require supplementation (Rees and Shaw 2007). Protein intake may also need to be modified to balance growth requirements against potential uraemia (Royle 2007).

High-energy food and drinks are encouraged, as it can be difficult to achieve EAR when on an altered diet. Good nutrition is very important in reaching the minimum weight to be placed on the transplant list (commonly 10 kg).

It is common for younger children and infants, in particular, to refuse to eat, and enteral feeding via the nasogastric or gastrostomy route is often required. Gastrostomy feeding has been shown to be a valuable tool in nutritional support and has also been associated, with other factors, in improved growth of young children (Rees et al. 2011).

Socialisation with food is still important, with the end goal of transplantation in mind. Feeding problems can remain following a successful transplant and speech-and-language therapy can be required. Conversely, in older children, advice is often needed to prevent excessive weight gain, particularly when taking corticosteroids as immunosuppression.

Fluid management

The approach to fluid management will be dependent upon whether the child concerned has a reduced urine output or is polyuric.

Patients who have a reduced urine output will usually be given a fluid allowance, and placed on a diuretic regimen. The common guideline used is to add 400 ml/m2 surface area/day to the average daily urine output, to allow for insensible losses (Royle 2007).

Some patients are polyuric and great care needs to be taken to ensure that fluid intake is sufficient to prevent dehydration.

Along with dietary changes, it is the reduction in fluid allowance that older children often find the most difficult to adhere to. It is important to give advice on ways of managing fluid. Fluid overload is a contributor to hypertension in many patients, and therefore can impact in cardiovascular health (Wright 2004).

Blood pressure

As with adults, hypertension is a complication that children with renal disease may experience. It is essential that accurate measurements are taken, to enable effective treatment of hypertension. Cardiovascular complications are a major cause of mortality and morbidity in adult patients (UK Renal Registry 2012). Slowing the progression of CKD, avoiding long-term dialysis and, if possible, conducting pre-emptive transplantation may represent the best strategies to decrease the risk of premature cardiac disease (Mitsnefes 2012).

Prevention of early complications is therefore essential, with hypertension being one of the most modifiable. Hypertension has also been associated with acceleration of renal disease and there is evidence that intensified blood-pressure control, with target 24-hour blood-pressure levels in the low range of normal, confers a substantial benefit in terms of slowing kidney disease progression (ESCAPE trial group et al. 2009).

Blood pressure decreases with age, and there are reference guidelines to assist in patient management. As children with renal insufficiency are often short in stature, their height should be used as a reference, rather than chronological age. It is recommended that systolic blood pressure should be maintained below the 90th percentile for height and sex (UK Renal Registry 2012). There are charts based upon gender, age and height, which should be used (National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents 2007).

The British Hypertension Society has guidelines on blood-pressure measurement, which include some guidance on children www.bhsoc.org/ (accessed 20 May 2013). Blood-pressure measurement in children is difficult and many inaccuracies are seen because of the method of measurement of the blood pressure cuff and the choice of apparatus with which to measure the blood pressure See Box 12.2 for a summary of blood pressure measurement guidelines.

There are other issues to consider in very small children. Firstly they can be uncooperative. It can also be difficult to hear Korotkoff sounds in small children using a stethoscope. As a result, Doppler devices are recommended in children under 2 years of age. Single readings should not be used to direct management, because white-coat hypertension can be particularly problematic in children. Ambulatory blood-pressure monitoring is often used to assist in correct treatment of hypertension enabling monitoring away from the hospital environment (Flynn 2011).

Anaemia

Anaemia is also associated with an increased risk of cardiac morbidity and mortality, and also affects growth and development (Koshy and Geary 2008). Haemoglobin should be maintained above or equal to 11 g/dl, ferritin 100–500 ng/ml and TSATS (Transferrin Saturation Rate) >20%. Initially oral iron supplementation with nutritional management is sufficient in managing anaemia. However as renal function deteriorates erythropoietin stimulating agents may need to be introduced. Most children with established kidney disease require treatment.

Intravenous iron is beneficial in increasing ferritin, TSATS and haemoglobin (Albaramki et al. 2012). This is usually given on an as required basis, however weekly doses for children on haemodialysis can be effective in counteracting the blood loss experienced. Whenever possible, blood transfusions are to be avoided.

Growth and bone health

Growth problems are common in children with renal disease, and monitoring is essential. This becomes a particular issue when GFR decreases below 25% (Hodson et al. 2012). Height/length, weight and head circumference in the under 2s should be measured and recorded on a growth chart (Royle 2007).

Renal bone disease is a major contributor to growth problems (Wesseling et al. 2008). Close monitoring of phosphate levels and parathyroid hormone is essential. Nondominant hand and wrist x-ray is also used to monitor growth, enabling bone age to be compared with chronological age.

Diet has a major impact on growth, including control of calcium and phosphate levels. Phosphate binding medication is often required (Royle 2007). In infants and young children this is added as a suspension into feeds, whereas older children follow similar regimes to adults. Again adherence can be a problem, and education and negotiation is essential to identify a regime that is realistic.

Vitamin D analogue treatments may also be required. Combining these treatments can control serum calcium levels and prevent the development of hyperparathyroidism (Bacchetta et al. 2012). Progression to tertiary hyperparathyroidism can make long-term treatment very difficult, and may ultimately lead to the need for a parathyroidectomy. Vascular calcification is also a major concern, and can be a contributing factor in cardiovascular complications (Bacchetta et al. 2012).

Growth problems in children with renal disorders have been associated with deranged secretion of growth hormone and resistance. Recombinant growth hormone can be effective (Hodson et al. 2012) and this treatment should be considered. This should be carefully managed in conjunction with an endocrinologist to minimise complications.

Nephrotic Syndrome

Nephrotic syndrome is the most common glomerular disease in childhood. The disease is predominantly seen in preschool children with an incidence of 2–4 cases per 100 000 in the United Kingdom (Krishnan 2012). It is 4–6 times more common in the UK Asian population (Lennon et al. 2009).

Nephrotic syndrome is characterised by a triad of symptoms: proteinuria (early morning protein to creatinine ratio greater than 200 mg/mmol), hypoalbuminaemia (plasma albumin less than 25 g/L), and generalised oedema (Lennon et al. 2009). Oedema develops when a loss of plasma protein leads to a fall in oncotic pressure and water leaks into the interstitial space. Elevated plasma cholesterol frequently occurs.

Acquired nephrotic syndrome can be secondary to infection, allergic disorders or systemic disease such as Henoch–Schönlein purpura, however it is most commonly idiopathic. Ninety-five per cent of paediatric patients respond well to corticosteroid therapy and can be classed as having minimal change nephrotic syndrome (MCNS).

The treatment of MCNS is with prednisolone, 60 mg/m²/day for 28 days, followed by 40 mg/m²/day on alternate weeks for a further 28 days. The child is considered to be in remission when the urine has been negative of protein for three consecutive days. Relapse occurs when there is proteinuria of 3+ or more on dipstick for three consecutive days after remission (Krishnan 2012). Relapse occurs in approximately 70% of children, requiring high-dose steroid treatment, which is weaned after 4 weeks. In children who frequently relapse, long-term low dose steroids (15–20 mg/m²) are recommended.

Renal biopsy is only indicated if remission is not achieved within 28 days of commencing treatment.

Nursing care of children with nephrotic syndrome

Accurate monitoring of fluid balance is essential along with daily weight and regular blood pressure measurement. Frequent assessment of oedema is necessary and mobilisation should be encouraged to disperse oedema. In children with significant oedema, a fluid allowance of 750 ml daily for children under 5 years and 1000 ml daily for those over 5 years should be considered. Although oedema may be present, hypovolaemia may occur due to the shift of fluid into the interstitial space. Diuretics should therefore be used with extreme caution and daily monitoring of serum electrolytes is necessary. In severe cases of oedema or hypovolaemia, 20% albumin infusions may be prescribed. Albumin should be administered with caution and frequent observations made during and after the infusion.

Urine should be checked for protein daily and a teaching programme should be arranged for children and carers, so that they can continue with urinalysis following discharge. Advice about steroid dosage and potential side effects should be given. Referral to a paediatric renal dietician is recommended. A healthy eating plan should be observed with a no-added salt diet. Monosaturated or polyunsaturated fats should be used to prevent hyperlipidaemia.

In cases of frequent relapse or steroid resistance, second-line nonsteroidal treatment is necessary. These children should be referred to a paediatric nephrologist. Minimal change nephrotic syndrome has a favourable prognosis. Children who present with this syndrome between one to eight years of age are likely to respond to steroid treatment. The disease usually burns out before childhood leaving no residual renal damage (Hodson et al. 2012).

Congenital nephrotic syndrome

This is a rare, inherited disorder, which usually presents in the antenatal stage or within the first three months of life (Jalanko 2009). It is characterised by heavy proteinuria, oedema and hypoalbuminaemia. Unlike childhood nephrotic syndrome, the disease cannot be controlled with the use of corticosteroids.

Daily albumin infusions are necessary and central venous line insertion is recommended to facilitate this. Ultimately, nephrectomy and renal replacement therapy is required. Early referral to a paediatric nephrology centre is crucial.

Renal Replacement Therapy

Transplantation

Transplantation is the treatment of choice for children with established renal disease. In 2009 it was reported that 76.7% of children with established renal disease were transplanted (UK Renal Registry 2012). Normal renal function provides children with better opportunities for growth and development. In addition, successful transplantation maximises the amount of education and normal childhood activities, which may be compromised during dialysis.

Allocation of deceased donor transplants currently gives priority to paediatric recipients (under 18 years of age) or young adults of 19 years of age who have been on the list since age 18 years. This was agreed as children are likely to require more than one kidney transplant in their life time and a good match at first transplant will mean less difficulty in finding a suitable donor in the future.

It is possible to transplant kidneys from adult donors depending on the size of the child’s abdominal cavity. If the donor kidney is disproportionately large, it may be placed intraperitoneally. However the extraperitoneal position is preferable as this preserves the peritoneum for dialysis and avoids long term complications.

Transplantation into very small children is rarely successful. Most transplant surgeons require children to weigh approximately 10 kg or have reached at least 22 months of age before transplantation is considered. This is due to the risk of infarction in small vessels post operatively. Children receiving renal replacement therapy from birth therefore require the maintenance of dialysis access up until this time. This places a significant burden on the carers and a challenge for professionals.

Living donor transplantation

With the decrease in the number of deceased donors, living donor transplantation is on the increase and is now routinely considered as an early option. In children, the donor is most frequently a parent, although donation from grandparents has been reported. UK law prohibits minors from donating a kidney and therefore siblings under 18 years are not considered. For children placed on the deceased donor waiting list, the avoidance of parental antigens will allow living related transplantation to be considered in the future.

Living related donation (LRD) is advantageous in that families are able to plan the timing of the transplant, which allows them to consider employment, education and family arrangements. It is recommended that children receiving kidney transplants must be cared for within a paediatric unit (Department of Health 2004) therefore there is the potential for the recipient and the donor to have surgery at different hospitals simultaneously. Professionals need to pay attention to the psychosocial needs of the family during this time, as often the nondonating parent must divide his or her time between the two sites. Supportive care could be provided in terms of video links and assistance with transport.

Pre-emptive transplantation

Transplantation prior to the need for dialysis is considered to be the gold standard for treatment of ERF and is achieved in up to 30% of transplanted children in the United Kingdom and United States (Williams 2012). The benefits of transplant before dialysis include: less interruption of schooling and family life, preservation of the peritoneum and vessels for future dialysis treatments, improved growth and development, and reduction in the symptoms of established renal failure

A child is generally considered for transplantation once the glomerular filtration rate has fallen below 10–15 ml/min/1.73 m2 and dialysis is anticipated within 12–24 months and/or a significant complication of growth failure is present (Webb 2003). In addition, pre-emptive transplantation should be considered if the child exhibits symptoms of established renal disease, i.e. renal bone disease, poor growth, fatigue and inability to take part in normal childhood activities.

Care of the child after kidney transplantation

Nursing care of the child following renal transplant must be in a designated high dependency nursing area in order to ensure close monitoring. Postoperative complications include bleeding, vascular thrombosis and delayed graft function (Williams 2012). After the first 48 h recovery is generally fast and, if there are no complications, children can be discharged 7–10 days following transplant.

Careful attention to kidney function and immunosuppression levels is necessary, and children are usually seen as an outpatient several times a week in the first four weeks. Due to the long travelling distances to the specialist centre for paediatric patients, close collaboration with local health centres and district hospitals is encouraged so that blood tests can be taken locally and results sent to the tertiary centre.

Children should be encouraged to return to school within 4–6 weeks of a successful transplant. School liaison is recommended in order to reassure teaching staff (Royal College of Nursing 2000).

Immunosuppression

There is currently no standard immunosuppression regimen for children and young people undergoing renal transplantation. However most children in the United Kingdom receive triple therapy with a calcineurin inhibitor (tacrolimus), a DNA proliferation inhibitor (azathioprine, mycophenolate mofetil) and a corticosteroid (NICE 2006). There are newer immunosuppressant agents targeting different sites in immune activation pathways, such as sirolimus and everolimus. Experience with these drugs in the paediatric population is evolving (Williams 2012) although potential nonadherence to the immunosuppressive regimen can be prevalent resulting in serious clinical consequences (Dobbels et al. 2010).

Complications

There are several potential complications following paediatric renal transplant. Hypertension is recognised as an important risk factor for cardiovascular morbidity and graft survival in transplanted children, with post-transplant hypertension occurring in 60–90% of transplanted children (Seeman 2007). Causes include: immunosuppression therapy, graft dysfunction, stenosis and weight gain. Ideally, hypertension should be confirmed using 24-hour automated blood pressure monitoring. The systolic blood pressure should be maintained at <90th percentile for age, gender and height. (+1.28SD = 90th percentile) or 130/– mmHg, whichever is lower.

Other complications include urinary-tract infection, rejection and recurrence of primary disease. Regular follow up and 24 hour access to the nephrology centre is essential.

Preparation for dialysis and transplant

Prior to commencing renal replacement therapy children, young people and their carers need to be given information regarding treatment options. In order for them to make an informed choice, information that is appropriate to age and understanding must be available. Hospital play specialists, trained to prepare children for procedures, are invaluable members of the paediatric multiprofessional team. Evidence shows that appropriate preparation for family members prior to treatment may prevent trauma later (Waby et al. 2005). Consideration must also be given to siblings who may feel distressed by the treatment and are concerned about family separation (Batte et al. 2006).

There are a number of investigations that are required within the preparation period. These are summarised in Box 12.3.

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