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About PFIC

Understanding PFIC

Progressive familial intrahepatic cholestasis (PFIC) is a spectrum of rare, inherited, liver diseases that can take so much from children and their families because of severe itching.

  • The itching (pruritus) can be so severe that infants and children may scratch through their skin1
  • Many patients turn to surgery, including liver transplant, to relieve the itching2,3

What Happens in PFIC?

The body naturally makes bile acids that help with digestion, including absorbing fats and some vitamins. In people with PFIC, the normal flow of bile acids is disrupted. Bile acids build up in the body, damaging the liver.5,6

1. Srivastava A. J Clin Exp Hepatol 2014;4:25-36.
2. Bjørnland K et al. Eur J Pediatr Surg. 2020.
3. van Wessel DBE et al. Hepatology. 2021;10.1002/hep.31787.
4. Davit-Spraul A et al. Orphanet J Rare Dis. 2009;4:1
5. Kamath BM et al. Liver Int. 2020;40(8):1812-1822.
6. Karpen SJ. Clin Liver Dis. 2020;15(3):115-119.

IMPORTANT SAFETY INFORMATION AND INDICATION

IMPORTANT SAFETY INFORMATION

Speak with your healthcare provider if you experience: abdominal pain, vomiting, diarrhea, and dehydration as these have been reported with the use of Bylvay. Patients should contact their healthcare provider if they experience new onset or worsening of diarrhea.

Elevations in liver tests (for example, AST, ALT, TB) have been observed with use of Bylvay. The patient’s health care provider will obtain liver tests before starting Bylvay and periodically during treatment with Bylvay. Patients should report to their healthcare provider any symptoms of liver problems (for example, nausea, vomiting, skin or the whites of eyes turn yellow, dark or brown urine, pain on the right side of the abdomen, loss of appetite).

Bylvay may impair absorption of fat-soluble vitamins (FSV), which include vitamins A, D, E and K (vitamin K is assessed by measuring INR). The patient’s health care provider will obtain serum levels of vitamins A, D, E, and INR (for vitamin K) at baseline and periodically during treatment to assess for worsening of FSV deficiency.

Do not mix Bylvay with liquids.

Do not swallow the 200 mcg or 600 mcg capsule(s) containing Oral Pellets whole. These are intended to be opened and the contents mixed into soft food. Take Bylvay in the morning with a meal.

For patients taking bile acid binding resins, take Bylvay at least 4 hours before or 4 hours after taking a bile acid binding resin.

INDICATIONS AND USAGE

Bylvay is indicated for the treatment of pruritus in patients 3 months of age and older with progressive familial intrahepatic cholestasis (PFIC).

Limitation of Use

• Bylvay may not be effective in PFIC type 2 patients with ABCB11 variants resulting in non-functional or complete absence of bile salt export pump protein (BSEP-3).

Please see full Prescribing Information.

IMPORTANT SAFETY INFORMATION AND INDICATION

IMPORTANT SAFETY INFORMATION

Speak with your healthcare provider if you experience: abdominal pain, vomiting, diarrhea, and dehydration as these have been reported with the use of Bylvay. Patients should contact their healthcare provider if they experience new onset or worsening of diarrhea.

Elevations in liver tests (for example, AST, ALT, TB) have been observed with use of Bylvay. The patient’s health care provider will obtain liver tests before starting Bylvay and periodically during treatment with Bylvay. Patients should report to their healthcare provider any symptoms of liver problems (for example, nausea, vomiting, skin or the whites of eyes turn yellow, dark or brown urine, pain on the right side of the abdomen, loss of appetite).

Bylvay may impair absorption of fat-soluble vitamins (FSV), which include vitamins A, D, E and K (vitamin K is assessed by measuring INR). The patient’s health care provider will obtain serum levels of vitamins A, D, E, and INR (for vitamin K) at baseline and periodically during treatment to assess for worsening of FSV deficiency.

Do not mix Bylvay with liquids.

Do not swallow the 200 mcg or 600 mcg capsule(s) containing Oral Pellets whole. These are intended to be opened and the contents mixed into soft food. Take Bylvay in the morning with a meal.

For patients taking bile acid binding resins, take Bylvay at least 4 hours before or 4 hours after taking a bile acid binding resin.

INDICATIONS AND USAGE

Bylvay is indicated for the treatment of pruritus in patients 3 months of age and older with progressive familial intrahepatic cholestasis (PFIC).

Limitation of Use

Bylvay may not be effective in PFIC type 2 patients with ABCB11 variants resulting in non-functional or complete absence of bile salt export pump protein (BSEP-3).

Please see full Prescribing Information.