Pruritus

Pruritus is the medical term for severe itching. 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.

The level of the itch can vary from person to person. It can range from being mild and more of an annoyance to being very disruptive and interfering with sleep and daily life. In rare cases pruritus can become so severe that a liver transplant is considered in order to improve the quality of a child’s life.

A number of medicines are available that can be used to relieve pruritus. Some people may take more than one medicine at a time and it can take some time to find the best combination.

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. It may 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.

Sometimes itching may have more than one cause, such as liver disease and eczema, so more than one treatment approach may be needed.

Some of the most common medicines used are listed here:

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 (Rimactane)

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.

Cholestyramine (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.

Other medications

For severe cases of pruritus, there may be options such as ondansetron and naltrexone that can be discussed with your medical team.

New medications have been developed called apical sodium dependent bile acid transporter (ASBT) inhibitors. They stop the uptake of bile acids in the gut and therefore reduce the amount in the body and the requirement of the liver to transport them. They are not currently licensed in the UK but hopefully will be available for treatment of PFIC and Alagille Syndrome in 2022. Trials for other conditions are ongoing. They don’t work on all patients but when they do it is likely they reduce pruritus as well as liver damage.

Sedatives

A sedative is a medication which can help with sleep. This may be given if pruritus is affecting sleep.

Itching can cause distress and discomfort. 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