This page provides an overview of fat-soluble vitamins in PFIC and examples of supplement products. Product availability and affordability vary by country. PFIC Network does not endorse any specific products or brands. Please consult your doctor or dietitian about the right supplements and dosing.
Why are fat-soluble vitamins a challenge in PFIC?
Vitamins A, D, E, and K are fat-soluble, meaning they depend on dietary fat — and bile salts — to be absorbed in the small intestine. In PFIC and other cholestatic diseases, reduced bile flow impairs fat absorption, which in turn makes it difficult to maintain adequate levels of these vitamins even when eating well.
This means that many PFIC patients require vitamin supplementation, and that the type of supplement matters. Standard over-the-counter multivitamins are frequently not effective for people with cholestasis.
Water-miscible (water-soluble) formulations are recommended.
Water-miscible vitamins are specially formulated to dissolve or mix in water, which greatly improves absorption in people with cholestasis. One example is TPGS vitamin E (tocopherol polyethylene glycol succinate) — also called tocofersolan. Taking TPGS vitamin E at the same time as other fat-soluble vitamins has been shown to improve the absorption of all of them, not just vitamin E.
Monitoring Vitamin Levels
Because fat-soluble vitamin deficiencies can develop quickly, regular monitoring is important. The general schedule recommended for most patients is:
- At diagnosis: Fat-soluble vitamin levels should be assessed as soon as possible after a diagnosis of cholestasis, or even when diagnosis is suspected but not yet confirmed.
- First year: Levels should be checked every 2 weeks to 3 months, depending on previous vitamin levels and disease severity.
- Once stable: After adequate levels are reached and maintained, monitoring every 3–6 months is typical.
Times to pay extra attention to fat-soluble vitamin management:
Fat-soluble vitamin management can be disrupted at key moments. It is worth being proactive about these situations:
- If you or your child are seen in the emergency room or by a non-liver specialist, they may not be familiar with why high vitamin doses are needed. It can help to explain this proactively or bring documentation from your liver team.
- FSV management may need to be adjusted after biliary diversion surgery, or when starting new medications such as IBAT inhibitors.
- When transitioning from pediatric to adult care, or transferring to a new center, make sure nutrition and vitamin management are explicitly communicated during handover.
During hospitalization, FSV management can sometimes take a back seat to more urgent concerns but it is worth keeping it on the radar.
The Four Fat-Soluble Vitamins: A, D, E, K
Vitamin A
| Why it matters | Essential for healthy vision, immune function, wound healing, and the growth and maintenance of many organs. |
| If levels are low | ݀• Most commonly affects eye health — early signs include difficulty seeing in low light. ݀• Can also impair wound healing and increase susceptibility to infection. |
| Food sources | ݀• Animal sources: liver, dairy, eggs, fish. ݀• Plant sources: orange and yellow foods (carrots, sweet potato, mango, apricot) and dark leafy greens. |
Vitamin D
| Why it matters | ݀• Essential for calcium absorption and bone health. ݀• Also plays roles in immune function, muscle strength, and moderating inflammation. |
| If levels are low | ݀• Rickets and abnormal bone development in children ݀• Low bone mass and increased fracture risk ݀• Impaired muscle strength, stunted growth |
| Food sources | ݀• Naturally found in few foods: fatty fish, fish liver oils, egg yolks, beef liver. ݀• Many countries fortify cow’s milk and cereals with vitamin D. |
Vitamin E
| Why it matters | ݀݀• An antioxidant that protects cells from damage. ݀• Also important for immune function and nervous system health. ݀• Particularly difficult to maintain in cholestasis even with supplementation. |
| If levels are low | ݀• Impaired development and function of the nervous system and muscles. ݀• Early signs may include reduced reflexes and impaired coordination. |
| Food sources | ݀݀• Highest amounts from nuts, seeds, and vegetable oils. ݀• Also found in leafy green vegetables and fortified cereals. |
Vitamin K
| Why it matters | Essential for blood clotting and bone metabolism. |
| If levels are low | ݀݀• Impaired blood clotting ݀• Increased risk of bleeding ݀• Reduced bone mineralization݀ |
| Food sources | ݀• Vitamin K1 (plant form): green leafy vegetables (spinach, kale, broccoli), vegetable oils, soybeans. ݀• Vitamin K2 (animal form): liver, fermented dairy, eggs, meat. Natto (fermented soybeans) is an excellent plant source of K2. |
Tips for Taking Fat-Soluble Vitamins
Getting the most out of your supplements
- Take TPGS vitamin E at the same time as your other fat-soluble vitamins. The TPGS form of vitamin E helps improve the absorption of all fat-soluble vitamins taken together — not just vitamin E.
- Take vitamins after a meal, so that food is eaten first. While fat-soluble vitamins in cholestasis depend mainly on water-miscible formulations rather than dietary fat for absorption, taking them with food can still be helpful.
- Pick a consistent time and stick to it. Compliance is much better with a fixed daily routine.
- Don’t reduce doses without consulting your liver team. The doses prescribed for PFIC are often much higher than typical recommendations, for good reason. If another provider suggests lowering them, check with your liver team first.
If deficiencies persist despite oral supplementation, ask your doctor about intravenous or intramuscular vitamin options, which may be available and appropriate in some cases.
PRODUCT LIST
Water-Miscible Vitamin Supplement Products
The following are examples of water-miscible vitamin supplement products commonly used in PFIC management. This is not an exhaustive list. Product availability, cost, and insurance coverage vary significantly by country and health plan.
PFIC Network does not endorse any specific products or brands. Always consult your care team before starting, stopping, or changing any supplement.
| Product | What It Contains | Notes |
|---|---|---|
| DEKAS Plus | Vitamins A, D, E (TPGS), K, plus additional vitamins and minerals | -A combination supplement specifically formulated for cholestatic liver disease. -Covers all four fat-soluble vitamins in one product. |
| DEKAS Essentials | Vitamins A, D, E (TPGS), K only | -Same as DEKAS Plus but without the additional vitamins -For patients who need fat-soluble vitamins only, at doses targeted for cholestasis. |
| Aqua-E Concentrate | Vitamin E (TPGS, water-soluble form) | -Single-vitamin supplement |
| Aqua-D | Vitamin D (water-soluble form) | -Single-vitamin supplement |
| Aqua-K | Vitamin K (water-soluble form) | -Single-vitamin supplement |
| Water-soluble Vitamin A palmitate | Vitamin A (water-soluble palmitate form) | -Single-vitamin supplement -Available from various brands. |
In the U.S., water-miscible vitamin preparations effective for cholestasis can be expensive and are often not covered by health insurance. Health plan formularies may also affect which specific forms of vitamin D (D2 vs. D3) are available and in an appropriate concentration. If you are having difficulty accessing or affording supplements, talk to your liver team or dietitian — they may be able to help with prior authorizations, peer-to-peer reviews, or identifying patient assistance programs.
ABOUT THIS PAGE
The content on this page is based on “Nutritional Management of PFIC,” an article prepared by Dr. Laura Bull, PhD, RD, from the University of California San Francisco. In addition to her long career researching the genetics of PFIC, Dr. Bull is a registered dietitian and a member of the PFIC Network Scientific and Medical Advisory Board. Dr. Bull consulted with registered dietitians and clinician experts in the field to pull together this page as a resource for families, taking into consideration variability of practice amongst centers and across borders.
Resources
Learn More
The full clinical article that this web page is based on, for those who want additional information.
The PFIC IMPACT Treatment Experience App (TEA) includes a Vitamins & Nutrition section where you can explore community experiences with treatments such as MCT oil and TPN and share your own.
Panel presentation and discussion video from the 2024 PFIC Family and Scientific Conference featuring Dr. Ron Sokol, Elizabeth Tamayo RD, Sharon Munn, and Dr. Laura Bull.
This overview webinar includes a section on nutrition management with clinicians from Children’s Hospital Colorado