Compassionate Use of Omegaven IV Fat Emulsion
Condition: Cholestasis; Total Parenteral Nutrition-induced Cholestasis
MeSH terms: Cholestasis
Intervention: Omegaven
Intervention description: Omegaven® will be initiated at a dose of 0.5 gram/kg/day and is infused over 24 hours for 1-2 days, and then advanced to 1 gram/kg/day. Omegaven® will be infused intravenously through either a central or peripheral catheter alone or in conjunction with parenteral nutrition. Omegaven® will continue until weaned from PN. Monotherapy with Omegaven® can continue as an additional source of calories after the dextrose/protein portion of PN is discontinued. Omegaven may be restarted within seven days of discontinuing therapy. After seven days, and meeting inclusion criteria, Omegaven can resume at the initial dose of 0.5 grams/kg/day, advancing to 1 gm/kg/day.
Phase: Phase 2
Study type: Interventional
Primary purpose: Treatment
Intervention model: Single Group Assignment
Masking: None (Open Label)
Enrollment: 15.0
Sex: All
Minimum age: 2 Weeks
Maximum age: 5 Years
Healthy volunteers: No
Keywords: Cholestasis; Parenteral Nutrition Associated Liver Disease

Summary:

      This is a compassionate use protocol to use intravenous fish oil infusion, Omegaven®, to
      infants and children with parenteral nutrition-associated liver disease to enable reversal of
      elevated serum liver enzymes and direct bilirubin (cholestasis).
    

Eligibility criteria:

        Inclusion Criteria:

          -  Two consecutive direct bilirubin levels of 2 mg/dl or more in a parenteral nutrition
             dependent infant or child (unable to meet nutritional needs solely by enteral
             nutrition)

          -  Other causes of liver disease have been excluded. A liver biopsy is not necessary for
             treatment.

          -  The patient must have utilized standard therapies to prevent the progression of the
             liver disease including reduction/removal of copper and manganese from daily PN, trial
             of enteral feeding if possible, and the use of ursodiol (i.e., Actigall®).

        Exclusion Criteria:

          -  Documented causes of chronic liver disease other than parenteral nutrition associated
             liver disease

          -  Proven severe advanced liver disease including cirrhosis on biopsy, varices, ascites.

          -  An allergy to any seafood product, egg protein, and/or previous allergy to Omegaven®

          -  Active coagulopathy characterized by ongoing bleeding or by a requirement for clotting
             factor replacement (e.g. fresh frozen plasma or cryoprecipitate) to maintain
             homeostasis

          -  Impaired lipid metabolism or severe hyperlipidemia with or without pancreatitis

          -  Unstable diabetes mellitus or hyperglycemia

          -  Stroke, embolism, collapse and shock, recent MI

          -  Cholestasis due to any reason other than parenteral associated liver disease

          -  Active new infection at time of initiation of Omegaven®

          -  Hemodynamic instability

          -  The patient may not be enrolled in any other clinical trial involving an
             investigational agent (unless approved by the designated physicians on the
             multidisciplinary team).