Cholestatic pruritus is a term used to describe itching caused by liver disease. Itching can occur when there is poor bile flow within and from the liver (cholestasis). There are some liver diseases in which itching can be particularly troublesome such as progressive familial intrahepatic cholestasis (PFIC) and Alagille syndrome.
Poor bile flow leads to high levels of bile acids in the blood stream. It is thought that these bile acids lead to itching. Jaundice is not always present in people with pruritus. Itching is usually felt all over the body but most severely affects the palms of the hands, the soles of the feet and the upper part of the body (the trunk). The itching may come and go in phases or in different seasons.
Sometimes medicines can work well to stop the itch; however, in other cases they may work for a short time but then stop working and need to be altered.
In other cases it can be difficult to find a combination that works for a particular person.There are operations that may be considered such as biliary diversion but success is very variable. Research is being carried out looking at better treatments for the future.
Sometimes itching may have more than one cause, such as liver disease and eczema, so more than one treatment approach may be needed.
Here are some of the most common medicines used:
Ursodeoxycholic acid (Urso)This is a type of bile acid. It works by increasing the proportion of watery bile acids in bile. This helps bile to flow more easily.
It has been shown to improve bile flow in children with liver disease and can prevent further damage to their liver cells.Some people find their itching becomes worse in the first or second week after taking the medicine. Another potential side effect is diarrhoea.
Rifampicin is an antibiotic. Normally a low dose is given at the start of the treatment and is gradually increased to stop the itch. It can cause urine, saliva and tears to turn an orange-red colour; this is harmless.
(Questran)This medicine combines with bile acids in the small intestine and reduces their reabsorption. The medicine can also bind with fat-soluble vitamins (vitamins A, D, E and K) and reduce their absorption.
For this reason some vitamins (and some other medicines) shouldn’t be taken within two hours of taking cholestyramine.
This medicine has an unusual taste and texture. If a child is having difficulty taking it then ask your hospital team for advice.SedativesA sedative is a medication which can help with sleep. This may be given if pruritus is affecting sleep.
When children are very young they won’t be able to tell you what the problem is.
Here are some hints and tips from other parents to help to deal with pruritus:
Keep your child’s nails short
Encourage rubbing rather than scratching
Use moisturisers and ointments to try and soothe your child’s skin.
Some parents suggest cold moisturiser from the fridge can be especially soothing and other suggest massaging oils into the skin
Keep your house cool as the heat can make children itchier
A cool bath can help.
Keep the water cooler than normal and add baby oil to the water
In very young children try and use clothes that cover the whole body, e.g. babygrow at night, dungarees in the day to prevent the child from scratching their skin
Scratch mitts and scratch sleeves can also be used
You can sew socks onto pyjamas to prevent scratching at night
Use a wool or cotton underblanket to make the mattress feel softer and use cotton bedding
Dress children in loose cotton clothes and avoid wool, acrylics and polyester
Distract your child with a favourite toy, a move to another room or finding other people to play with (brothers or sisters can help here)
Older children may find that watching TV or talking to friends online is a good distraction
Speak to your child’s school regarding exams and the potential for extra thinking time as itching can be a real distraction