Living with ARPKD

How will my child feel living with ARPKD?

As your child grows, the initial abdominal enlargement will decrease.  It is unusual to have any signs or symptoms related to the kidneys unless the kidney function is severely reduced.  The kidneys are not painful but there is an increased possibility of developing urinary tract infection (UTI). The liver may develop scarring causing pressure on the blood vessels of the stomach and spleen.  This may result in enlargement of the spleen and rarely the tendency for bleeding into the stomach.

How often should my child see a paediatric nephrologist?

Children are usually seen frequently initially to stabilise blood pressure but will then have tests every 3-6 months depending on the severity of the kidney dysfunction.

What can I do to slow the progression of ARPKD?

The most important aspect to slowing the rate of kidney deterioration is excellent control of blood pressure.  Children have lower blood pressure ranges than adults with a goal blood pressure less than 110/75.