Maralixibat

How it works

It inhibits the apical sodium-dependent bile acid transporter in the terminal ileum, reducing reabsorption of bile acids and increasing their faecal elimination, which lowers serum bile acids and relieves cholestatic itch.

Prescribing in practice

• Diarrhoea and disturbances of liver biochemistry can occur, so monitor liver tests and bile acids and follow the SPC guidance on interrupting or adjusting therapy if these worsen.
• Because it reduces bile acid recycling, it may impair absorption of fat-soluble vitamins, which should be monitored and supplemented as needed.
• It is used as a specialist treatment for cholestatic pruritus; consult a children's formulary and metabolic or hepatology specialists for dosing in the paediatric population.

Monitoring

Monitor liver function tests and serum bile acids at baseline and during treatment, together with fat-soluble vitamin status, under specialist supervision.

Counselling the patient

• This medicine is given to relieve itching caused by liver disease; report worsening tummy upset, diarrhoea or yellowing of the skin or eyes.
• Attend the scheduled blood tests so the team can monitor the liver and vitamin levels.

Evidence & guidelines

Ileal bile acid transport inhibitors are established for cholestatic pruritus in Alagille syndrome, having reduced serum bile acids and pruritus in controlled clinical trials.