One of the serious effects of PFIC is itching (pruritus). The level of itch can vary from child to child. It can range from being mild to being very disruptive and causing severe discomfort.

Itching is usually felt all over the body. It often 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.

Severe itching can lead to:

• skin damage (due to scratching)
• disturbed sleep
• fatigue (tiredness)
• irritability
• poor attention
• loss of appetite
• feeling sick (nausea)
• being sick (vomiting

See our page on pruritus for more information. This gives details of the treatments available and includes advice from other parents on how to deal with severe itching.

This happens when bilirubin trapped in the liver passes back into the bloodstream. It moves around the body and causes yellowing of the whites of the eyes (sclera) and skin.

“I used to be jaundiced some days and I even came up with a yellow superhero called Banana Girl to describe myself!”

A type of bilirubin called conjugated bilirubin passes out in the wee (urine) and can make it appear darker than normal.

This can happen when jaundice is present while the teeth are forming and growing. It can also be caused by the high calorie diet and/or medicines used to manage PFIC.

Poo (stools) will often be watery and loose (diarrhoea). They may float in the toilet and be more difficult to flush away. This is because they contain more undigested fat. This is due to less bile flowing from the liver into the intestine.

This refers to poor weight gain and slower growth. In PFIC this can be caused by a lower absorption of fats and other important nutrients.

The vitamins A, D, E and K need good fat absorption to be taken into the body. In PFIC, fat absorption is poor, leading to a lack of these vitamins. This can cause problems, because the vitamins each play an important role in the body.

This is a larger than normal swelling of the liver. It may cause tummy (abdominal) discomfort or lead to your child “feeling full”.

This is a larger than normal swelling of the spleen. It may happen as a result of a condition called portal hypertension. It can cause tummy (abdominal) discomfort or lead to your child “feeling full”. 

These are small stones that form in the gallbladder or bile ducts. In most cases gallstones are present but cause no problems. However, if a stone blocks a bile duct this can cause severe pain and jaundice.

Gallstones also increase the risk of infection in the bile ducts (cholangitis).