Omegaven as Alternative Parenteral Fat Nutrition
Condition: Cholestasis; Cholestasis of Parenteral Nutrition
MeSH terms: Cholestasis
Intervention: Omegaven
Intervention description: Omegaven® fat emulsion will be used as a compassionate use treatment for critically ill infants with parenteral nutrition associated liver injury
Phase: Phase 3
Study type: Interventional
Primary purpose: Treatment
Intervention model: Single Group Assignment
Masking: None (Open Label)
Enrollment: 2.0
Sex: All
Minimum age: 14 Days
Healthy volunteers: No
Keywords: Omegaven; parenteral nutrition; Liver disease; Cholestasis

Summary:

      To provide Omegaven® as a compassionate treatment for critically ill infants with parenteral
      nutrition associated cholestasis
    

Eligibility criteria:

        Inclusion Criteria:

          -  Greater than 14 days old

          -  Has parenteral nutrition associated cholestasis defined as at least 2 consecutive
             direct bilirubin >2 mg/dL with anatomical or functional short gut (OR >4 mg/dL if
             intact intestine) obtained at least 1 week apart with a ratio of direct: total
             bilirubin > 0.4

          -  Patient is parenteral nutrition dependent (unable to meet nutritional needs solely by
             enteral nutrition) and are expected to require parenteral nutrition for at least 3
             more weeks

          -  Patient has not responded to other therapeutic approaches for parenteral
             nutrition-associated liver disease such as : cycling of parenteral nutrition, avoiding
             overfeeding, reduction/removal of copper and manganese from parenteral nutrition,
             advancement of enteral feeding, and use of Ursodiol

          -  Patient has been on at least 2 weeks of SMOFLIPIDs with no improvement or worsening of
             direct bilirubin levels

          -  Signed patient informed consent

          -  The patient is expected to have a reasonable possibility of survival

          -  No other known etiology of cholestasis other than parenteral nutrition-associated
             liver disease at the time of Omegaven® initiation

        Exclusion Criteria:

          -  Known causes of cholestasis other than parenteral nutrition-associated liver disease
             including but not limited to Hepatitis C, Cystic fibrosis, biliary atresia, and alpha
             1 antitrypsin deficiency are present, prior to Omegaven® initiation

          -  Known fish or egg allergy

          -  Any of the contraindications to use of Omegaven®:

               1. Active new infection at the time of initiation of Omegaven®

               2. Hemodynamic instability

               3. Recent use of medications with associated risk of bleeding, including NSAIDs

               4. Active coagulopathy or bleeding

               5. Platelet counts persistently under 30,000 despite transfusions

               6. Unstable hyperglycemia

               7. Impaired lipid metabolism (triglycerides >1000 mg/dL) while on 1 g/kg/day or less
                  of Intralipid

               8. History of severe hemorrhagic disorders (ie. hemophilia, Von Willebrand disease,
                  etc.)

               9. Unstable diabetes mellitus

              10. Collapse and shock

              11. Stroke/ Embolism

              12. Cardiac infarction within the last 3 months

              13. Undefined coma status