Great Ormond Street Hospital for Children NHS Foundation Trust: Information for Families
Chronic renal failure in infants
This information sheet explains chronic renal
failure in infants under two years old, which
is often caused by different things to chronic
20 children in the UK are born who will require
How does the
urinary system work?
dialysis in the first two years, and some of
The urinary system consists of the kidneys,
renal failure in older children. Each year around
them will need it from when they are born. This
information sheet also explains the options for
the bladder and ureters. The kidneys filter
the blood to remove waste products and
form urine. The urine flows from the
treatment and what you can expect when your
kidneys down through the ureters to the
child comes to Great Ormond Street Children’s
bladder.
Hospital for assessment and treatment.
What is
chronic renal failure?
Chronic renal failure (CRF) is a term used
to describe when the kidneys are not
working as well as they should. The term
implies that both kidneys are affected. This
is because one normal kidney is enough
to maintain normal kidney function
throughout life. When both kidneys are
damaged, they cannot keep up with the
Kidneys
work that is asked of them, so that they
may slowly fail over the years. When their
function becomes very poor, terms other
than CRF are used, like ‘end-stage renal
Ureters
failure’ (ESRF). This term is not a very good
one as it implies that there is nothing
more that can be done, but in fact it
means that there is a need for dialysis or
transplantation to maintain good health.
Bladder
Dialysis and transplantation are often
Sphincter
Urethra
Sheet 1 of 5
called ‘renal replacement therapy’ (RRT).
Ref: 2014F0771
© GOSH NHS Foundation Trust September 2014
How is chronic renal
What can cause chronic
failure different in infants? renal failure in infants?
The main differences are when chronic
The most common diagnosis is renal
renal failure is diagnosed and its cause.
dysplasia, which means that the kidney
Problems with the kidneys or their
tissue has not formed normally. Other
plumbing can be diagnosed before birth,
causes might be a blockage affecting
which can lead to many families having
urine drainage. This most often affects
months of anxiety before their baby is
boys with a condition called posterior
born. Around half of affected infants are
urethral valves (PUV). More information
diagnosed before birth although some of
about this is in our Posterior urethral
these may be missed if the mother does
valves leaflet. The next most common
not have a scan during the last part of
cause is congenital nephrotic syndrome,
pregnancy.
where the kidneys leak protein so that
Sometimes it is possible to predict poor
kidney function if the amount of fluid
around the baby in the womb (amniotic
fluid) is very low. This can be caused by a
genetic condition, so genetic counselling
should be offered to assess the chance
of the same thing happening in future
pregnancies. In some cases, the kidney
problems may be linked to a blockage
the baby becomes swollen. For more
information about congenital nephrotic
syndrome, please see our leaflet. Finally,
there are other causes affecting small
numbers of children. These include
kidney damage due to problems that
happen during birth and some inherited
kidney disorders that cause cysts to form
in the kidney tissue.
stopping urine draining from the bladder.
In these cases, it may be possible to carry
there is no guarantee that this will stop
How can chronic
renal failure in
infants be treated?
the kidney failure. The important thing
There are many factors that influence
to remember is that one affected kidney
the treatments available and the
does not necessarily mean kidney failure
nephrologists (doctors specialising in
as the other kidney can continue to work
kidney disorders) will discuss these with
well throughout life.
you. The main options are palliative and
out surgery to relieve the blockage while
the baby is still in the womb. However,
supportive care or intensive treatment
with renal replacement therapy (RRT).
More information about each of these
options follows.
Sheet 2 of 5
Ref: 2014F0771
© GOSH NHS Foundation Trust September 2014
Palliative and support care
There may be circumstances when the
family together with the medical and
nursing team feel that the best option
is palliative or supportive care to avoid
any further pain or suffering for the
infant. This involves making the infant
comfortable without carrying out any
intensive, invasive or painful procedures.
This is more often the case when the
infant has other conditions causing severe
Deciding which
option to take
The main difficulty is that it is impossible
to predict whether the infant’s kidney
function will continue to get worse or
may have a period of improvement. The
level of kidney function in infants with
normal kidneys often improves during the
first year of life and the same can happen
in infants with chronic renal failure.
medical problems. In our experience,
Another reason for uncertainty is that
infants with severe kidney failure
many infants with chronic renal failure
receiving palliative care do not survive
cannot concentrate their urine. This
longer than a year after birth. Families
means that they lose large amounts of
will be supported during this time both at
water and minerals daily so can manage
GOSH and in their local area.
for many months without dialysis,
although growth and development is
Intensive treatment with renal
replacement therapy (RRT)
The aim of intensive treatment with renal
replacement therapy (RRT) is to enable an
infant to grow as much as possible so that
he or she can receive a kidney transplant
as soon as possible. This involves dialysis
and diet therapy, leading to a kidney
likely to be affected.
You can discuss your options as frequently
as you need with various members of
the team. It is not impossible to change
and switch to the other option, although
this may have long-term effects on your
infant’s growth and development.
transplant. This is always an intensive
Our aim is to give you as much advice
process and a great deal of effort is
and information as possible to help
needed from both the medical team and
you understand the medical aspects of
family. It will involve frequent hospital
your child’s care. We will support you
visits and close monitoring at home, with
throughout this difficult time so that
a detailed diet to follow and medicines
the right form of care is offered to your
to take. This can be a difficult time for
child and that you can feel that the right
families and they will be supported by
choice was made for your child when you
GOSH and the local team. More detailed
look back years later.
information about intensive treatment
and RRT follows later in this information
sheet.
Sheet 3 of 5
Ref: 2014F0771
© GOSH NHS Foundation Trust September 2014
What does intensive
treatment involve?
There are various parts to intensive
treatment all of which are vital to
encourage your infant’s growth and
development until ready for transplant.
Haemodialysis
In our experience, haemodialysis is less
suitable for very young children but not
impossible. In this type of dialysis, there
needs to be access to the blood vessels
so that blood can be taken out of the
body, passed through a filter to clean
Dialysis
it and then returned to the body. In
There are two types of dialysis: peritoneal
young children, access is usually through
dialysis and haemodialysis. In both types,
a catheter placed into the blood vessels
the principal is the same: a cleaning
in the neck. Catheters can easily become
fluid (called dialysate) is used to take the
infected and may need to be replaced,
impurities, salt and water away from the
sometimes damaging the blood vessel
blood. The impurities pass from the blood
so that it cannot be used again. The
into the cleaning fluid, which then takes
haemodialysis machine needs to be
them away. There has to be a barrier
primed with blood, and due to the small
between the blood and the cleaning fluid
volume of blood in infants, donated
for this to happen. In haemodialysis, the
blood is usually used. This means that, in
barrier is the filter in the dialysis machine
effect, infants receive a blood transfusion
that the blood passes through and in
each session, which can lead to problems
peritoneal dialysis, the barrier is the layer
with antibodies. This increases the
of cells that lines the abdomen and covers
difficulties of finding a future kidney
the guts (the peritoneum).
transplant to match.
Peritoneal dialysis
Monitoring
Overnight peritoneal dialysis (continuous
Intensive treatment involves very close
cycling peritoneal dialysis or CCPD) is the
monitoring of the infant to make sure
preferred choice for infants with chronic
that the best levels of growth and
renal failure. A machine is used to push
development are possible. The success of
fluid in and out of the abdomen. This
dialysis is measured regularly, often every
dialysis takes place during the night while
week, and altered if needed. Your infant’s
your child is asleep. The machine is about
diet will also be checked every week to
the size of a suitcase and is transportable
make sure that he or she is receiving
to other houses if necessary. We usually
enough calories, and also changed if
prescribe 12 to 14 hours of dialysis to
needed. Medicines needed to prevent
remove the large fluid volume of the
long-term problems such as bone disease
infant’s diet. The catheter may need to be
will also be monitored closely.
replaced or other complications, such as
peritonitis, can happen, but the risks are
far less than with haemodialysis.
Sheet 4 of 5
Ref: 2014F0771
© GOSH NHS Foundation Trust September 2014
Diet
It is rare for an infant with chronic renal
failure to get enough goodness from his
or her diet to ensure the best growth
What is the outlook
for infants with
chronic renal failure?
and development possible. Many infants
Adequate growth and development is
have feeding problems, and some have
the primary aim of intensive treatment.
gastro-oesophageal reflux or vomiting
A closely monitored diet makes all
severe enough to need an operation to
the difference to the child’s eventual
tighten the top of the stomach (Nissens
height and weight, and many ‘catch
fundoplication). For more information
up’ once their diet is monitored closely
about this, please see our leaflet. A high-
and includes nutritional supplements.
calorie and high-protein diet is needed
However, if a child does not achieve
and the majority of children also need
adequate growth, growth hormone
supplements of minerals too. This is
treatment is a possibility if diet alone
more likely in infants with structural
does not help as the child gets older.
kidney problems who lose minerals easily.
In our experience, few children have
Many children may not be able to feed
developmental problems after chronic
adequately by mouth so need to be
renal failure, so most can attend
tubefed. This can either be through a
mainstream school successfully.
nasogastric tube, which is passed through
A successful kidney transplant is best
the nostril and down the food pipe into
possible outcome of intensive treatment.
the stomach, or through a gastrostomy,
There seems to be little difference in
which is passed through the skin directly
success rates between transplants in
into the stomach. For more information
infants and older children if the kidney
about these, please see our leaflets.
is donated from a parent, although the
Notes
chances of success are slightly lower
with a deceased donor kidney. In our
experience, there is no reason to hold
off from a kidney transplant until the
child is of a certain age or size, but
it is preferable to be sure that all the
immunisations are complete so it is rare to
go ahead before 18 months of age.
Compiled by Nephrology department in collaboration with the Child and Family Information Group.
Great Ormond Street Hospital for Children NHS Foundation Trust, Great Ormond Street, London WC1N 3JH
www.gosh.nhs.uk
Sheet 5 of 5
Ref: 2014F0771
© GOSH NHS Foundation Trust September 2014