GASTROENTEROLOGIST CONNECT

We have a robust interdisciplinary/multidisciplinary team taking care
of Alagille Syndrome patients at my institution. I would suggest that hepatologists
are more involved in the care of ALGS patients than gastroenterologists. Most of my
ALGS patients were initially referred by pediatricians. We have used a variety of
medications for pruritus, including antihistamines, cholestyramine, rifampicin, and
others, none of which were very successful. Not too long ago we began using
Livmarli (maralixibat), the first IBAT inhibitor (ileal bile acid transporter inhibitor)
approved for the treatment of ALGS-related pruritus and, more recently, (Bylvay)
odevixibat, which was approved for ALGS only recently. Although there is some
recent evidence that the IBATs slow the progression of the chronic liver disease,
none of these therapies directly address the underlying disease, so the
multidisciplinary approach to the treatment of ALGS patients remains.