Nutritional Management of PFIC

By Dr. Laura Bull, PhD, RD

This article is intended to provide an overview of nutritional considerations for patients with PFIC and to give examples of products that are available to support nutritional needs. Nutrition products may vary between countries and with product availability. Please consult with your/your child’s doctor or registered dietitian about individual nutrition recommendations.

1. Why is nutrition important in PFIC?

In intrahepatic cholestasis, the flow of bile from the liver to the intestine is impaired. Bile salts are powerful detergents that help the body emulsify and absorb fat, and are an important part of bile. Due to the decreased flow of bile and bile salts into the small intestine, PFIC patients may have problems with absorption of fat and fat-soluble vitamins (A, D, E, K).

Furthermore, cholestatic liver disease can cause a hypermetabolic state, in which a patient’s body burns energy and breaks down body protein at an increased rate. Meeting energy and protein needs is important for growth and nutritional status, so more energy and protein may be recommended.

Over time, patients with PFIC may develop side effects of advanced liver disease such as ascites, or gastrointestinal problems such as nausea or diarrhea, that can further contribute to poor intake of food, and negatively affect growth and development in children, necessitating increased nutritional support.

2. Possible consequences of nutrition challenges in PFIC

Fat malabsorption, vitamin deficiencies and inadequate dietary intake can lead to:

1. Faltering growth and poor weight gain (so called “failure to thrive”)
2. Impaired immunity (increased susceptibility to infection)
3. Loss of muscle mass (sarcopenia)
4. Symptoms associated with specific vitamin deficiencies, such as vision problems (vitamin A deficiency), loss of bone density (with deficiencies in vitamin D, vitamin K, calcium or phosphorus), problems with bruising and bleeding (vitamin K deficiency), or problems with balance and coordination (vitamin E deficiency).

Decreased nutrient intake can happen for a variety of reasons, such as:

1. Altered taste perception that decreases desire to eat and appetite
2. Feed intolerance. This can be due to nausea, vomiting, and/or early satiety (feeling full with a small amount of food), due to ascites, enlarged organs, or slow movement of food through the GI tract (dysmotility)
3. Decreased appetite due to altered nutrient metabolism
4. Itching, which may distract a child from eating

Even when food is eaten, decreased digestion and absorption can occur, due to:

1. Fat malabsorption (and consequent fat-soluble vitamin malabsorption) due to insufficient bile salts in the intestine
2. Secondary consequences of cholestasis, such as changes in intestinal bacteria and bile salts, as well as impairment of normal function of the intestines (enteropathy)

3. General management of nutrition challenges

Minimizing malnutrition in patients with cholestasis improves patient and caregiver quality of life, supports immune system function reducing risk of infections, and can improve outcomes of liver transplantation; in children, physical and cognitive development are also improved.

Since people with cholestasis can have trouble absorbing some foods they eat, and need more energy for normal bodily functions when compared to people without cholestasis, it can be helpful to aim for a daily calorie intake above estimates for people without cholestasis, so as to maintain good nutritional status.

Nutrition goals and approaches will vary by patient, depending on their age and other factors, such as physical activity. There are three main approaches to managing nutritional needs in PFIC patients:

1. Nutrient supplementation (sections 5 and 6 below)– in particular, due to the nature of PFIC, some supplementation is usually necessary to achieve adequate fat-soluble vitamin levels
2. Oral dietary intake (section 7 below)– eating a diet that supports nutrition goals
3. Nutrition support (section 8 below)– When necessary or helpful, oral intake can be supplemented or replaced through enteral nutrition (‘tube feeding,’ directly into the stomach or intestine through a tube), or parenteral nutrition (intravenous nutrition, directly into the blood)

In general, nutrition goals will include avoiding or minimizing vitamin deficiencies, and achieving adequate intake and absorption of calories and a variety of nutrients.

4. Monitoring of nutrition status

Malnutrition (or undernutrition) has different definitions, but in general is an imbalance between nutrient requirements and intake/absorption of nutrients that results in deficits of energy, protein, or micronutrients (vitamins and minerals) that may negatively affect health, and in children, growth and development. Monitoring of nutritional status identifies patients at risk of malnutrition.

Factors evaluated as part of monitoring nutritional status include (A, B, C, D’s);

• Anthropometric measurements (growth measurements)
  • Length/height, weight, weight-for-length/body mass index, and head circumference (most often in children up to 3 years of age).
  • These measurements are compared to data collected in healthy children in order to assess growth in children.
  • To assess whether weight gain is due to growth (including adequate muscle and fat), rather than largely due to fluid accumulation (e.g., ascites) or organ enlargement, mid-upper arm circumference (called MAC or MUAC) and triceps or subscapular skinfold thickness (TSF or SSF) are monitored to assess muscle and fat tissue accumulation.
• Biochemical review (lab tests)
  • Used to screen for nutritional deficiencies.
  • Types and frequencies of lab tests vary between centers and depending upon the current condition of the patient.
• Clinical review
  • Including asking about any symptoms of concern, such as vomiting or abdominal pain.
• Diet review
  • Includes asking about usual intake of food, drinks, and nutrient supplementation and comparing the intake to nutritional needs.

5. Nutrient supplementation in more detail: Medium-chain triglycerides (MCTs)

PFIC patients often need additional MCTs in their diet. MCTs are fat molecules that are shorter than the more common long-chain triglycerides (LCTs), and are easy for the body to break down and absorb, without help from bile salts. There are special formulas (infant and pediatric) with added MCT that may be recommended. As well, there are MCT supplements that may be better suited for older children and adults. Up to 60% of the fat in a formula can be MCTs, with the remaining fats being usual long-chain triglycerides. (Long-chain fats are included to prevent deficiencies of essential fatty acids.)

MCT oil can be taken from a dropper like a medication or added to food. Due to the popularity of ketogenic diets, there are many varieties of MCT oil commercially available at groceries or health food stores, and even online. Look for an oil with a high percentage of C8 and C10 MCT oils.

 *MCT oil has a low smoke point so it is not recommended as a cooking or frying oil substitute.

There are many ways to add MCT oil to the diet, such as:

• Add to formula, shake or smoothie
• Add to salad dressings
• Drizzle on top of food (such as on toast)
• Stir into soup or oatmeal
• Add to coffee (frequently known as “Bulletproof” coffee) or other hot beverages

 These are just a few of the many ways to add MCT oil to foods. Be inspired by your own food preferences and culture and try different ways to add MCTs to meals and snacks. Also, there are many free recipes on the internet featuring delicious ways to incorporate MCT oil into your diet. If you want to add MCT oil to liquids, there are varieties available in powdered form, or emulsified in water for easier mixing.

Note for infants: Adding MCT oil to formula or breastmilk can be challenging because the oil does not mix well with liquids, and the taste or texture can be affected, affecting acceptance of the bottle feed. Also, MCTs can stick to the sides of glass or plastic bottles or inside bottle nipples, decreasing the amount of MCT oil the baby actually drinks. Providing MCT oil as a bolus as if it were a medication may sometimes be more effective. One caution is the risk of aspiration into the lungs of any oil (including MCT oil) if the child has significant gastroesophageal reflux/spitting up or difficulty swallowing. Speak to your doctor about pros and cons of an MCT oil supplement if this is the case.

6. Nutrient supplementation in more detail: Fat-soluble vitamins (FSV: A, D, E, and K)

PFIC patients often require supplementation of fat-soluble vitamins (FSV) to avoid deficiencies. To aid with vitamin absorption, it can be helpful to consume vitamin-containing foods together with fats. However, due to challenges in absorbing fats and FSV, it is typical to rely more on supplements than diet to obtain sufficient amounts of FSV. Standard over-the-counter multivitamin formulations are frequently not effective in managing FSV deficiencies in PFIC. Improvement in vitamin status is substantially greater when water-miscible versions of FSV vitamins are used; these are designed to dissolve or mix easily in water, enhancing absorption. An example of water-miscible FSV is alpha tocopherol polyethylene glycol succinate (called TPGS or tocofersolan) Vitamin E. Use of TPGS can improve absorption of vitamin E but also of other fat-soluble vitamins administered with it. Dosing may be high initially, to correct deficiencies, then be adjusted over time, depending on follow-up blood tests. It is important to give the correct dose of the vitamins as different preparations may have different concentrations.

Despite oral supplementation, FSV deficiencies may persist. In some such cases, intravenous or intramuscular FSV may be medically advised, if available. It is very important to have FSV status monitored routinely, to ensure good vitamin stores, but also to meet needs that may change as a child grows or disease progresses. 

When and how often should FSV status be assessed?

• Serum FSV levels should be assessed at diagnosis of cholestasis.
• During the 1^st year after diagnosis, FSV levels should be monitored every 2-4 weeks initially (if abnormal) and then usually about every 3 months, depending on previous levels, changes in doses of supplements and disease severity.
• Once stable, adequate levels of FSV are achieved, levels should be monitored every 3-6 months, to ensure that adequate, but not excessive, FSV levels are maintained.

Some situations when FSV management is particularly vulnerable:

• A child may already have FSV deficiencies at the time they are diagnosed, so assessment and supplementation as needed should start as soon as feasible after diagnosis or in some cases, even when diagnosis is suspected but not yet confirmed.
• If a person with cholestasis is seen by non-liver healthcare practitioners, such as in the emergency room, sometimes such practitioners may recommend lowering vitamin dosages, due to not being liver disease specialists, and so not understanding the reason for the high doses that are given. In such situations, it is helpful to share the reason for the high dosages.
• Sometimes, FSV management may take a back seat to more urgent medical concerns when a child is hospitalized. FSV management may need adjustment after procedures such as biliary diversion, or when starting new medications, such as IBAT inhibitors.
• When a patient transfers from pediatric to adult care, or transfers to management at a new center, care should also be taken to ensure that issues related to nutrition are communicated during handover.

ABOUT THE INDIVIDUAL FAT-SOLUBLE VITAMINS (FSV)

Vitamin A

‘Preformed’ vitamin A (retinol and retinyl esters) is found in animal products. Our bodies can also generate vitamin A from ‘provitamin A carotenoids,’ such as alpha- and beta-carotene, found in plant foods.

• Blood tests
  • Vitamin A levels can be assessed using tests for serum retinol and/or serum retinol binding protein (RBP), or a ratio of these 2 tests.
  • Levels of retinol and RBP may temporarily decrease during an acute infection, and can also be low due to iron or zinc deficiency.
• Roles of Vitamin A
  • Vitamin A plays a role in many important processes in the body.
  • It is essential for healthy vision, plays roles in growth, development and maintenance of many organs, and helps with function of the immune system and in wound healing.
• Possible effects of vitamin A deficiency
  • Most commonly, inadequate vitamin A negatively impacts eye health, including difficulty seeing in low light, and can lead to permanent vision impairment if not treated.
  • Inadequate vitamin A can also impair wound healing, and result in greater susceptibility to infection and increased risk of anemia.
• Good food sources.
  • Animal sources
    • Meats, poultry, liver, cod liver oil, dairy foods, eggs and fish.
  • Plant sources
    • Plants containing carotenoids, such as beta-carotene, especially yellow and orange foods (such as carrots, yam, sweet potato, pumpkin, cantaloupe, apricot, mango) and dark green leafy vegetables (such as broccoli, spinach)
• Supplementation oral dosing, or intramuscular injection.

Vitamin D (ergocalciferol=D₂, cholecalciferol=D₃).

Vitamin D can by synthesized in the skin when exposed to ultraviolet (UV) B rays from sunlight. Synthesis of vitamin D in the skin is impacted by latitude (how far north or south of the equator one lives, further away being associated with greater risk of deficiency), season (greater risk of deficiency during colder seasons when people have more skin covered) and skin tone (darker skin tones are associated with greater risk of deficiency). However, many people do not get sufficient sun exposure to produce enough vitamin D, and so must obtain some from food and/or supplements.

Extra details: vitamin D undergoes 2 hydroxylations- chemical modifications- to produce the active form. The 1^st hydroxylation occurs in the liver and produces 25-hydroxy-vitamin D (25-(OH)D)=calcidiol.The 2^nd hydroxylation occurs in the kidney, to produce 1,25-dihydroxy-vitamin D (1,25(OH)₂D)=calcitriol.

• Blood tests
  • Measurement of serum 25-hydroxy vitamin D (25-(OH)D) (also called calcidiol) is used to assess Vitamin D levels.
• Roles of Vitamin D:
  • Vitamin D facilitates calcium absorption in the intestine, and helps maintain healthy levels of calcium and phosphorus in our bodies.
  • Vitamin D allows normal mineralization, growth, and healing/remodeling of bone.
  • Vitamin D also has roles in a variety of other processes in the body, such as glucose metabolism and immune function, including helping to moderate inflammation.
• Possible effects of vitamin D deficiency
  • Rickets
  • Abnormal bone development and structure with low bone mass and increased fracture risk
  • Impaired muscular strength, stunted growth, immune system impairment.
• Good food sources
  • Vitamin D is only naturally found in a few foods, including fatty fish, fish liver oils, egg yolks, and  beef liver.
  • Some mushrooms may be a good source, if they have been exposed to UV light.
  • In many countries, foods fortified with vitamin D, such as cow’s milk and breakfast cereals, provide most of the vitamin D in the diet.
• Supplementation
  • Oral dosing, preferably in a TPGS-based fat-soluble vitamin supplement to enhance absorption.
  • In some locations, vitamin D may also be administered via intramuscular injection or intravenous infusion.

Vitamin E (tocopherols and tocotrienols)

While it can be difficult to achieve normal levels of vitamin E in people with cholestasis, with proper dosing of TPGS, most patients can achieve normal vitamin E status.

• Blood tests
  • Measurement of serum alpha-tocopherol, and determination of the ratio of serum alpha-tocopherol to total serum lipids, allows for optimal monitoring of vitamin E status.
• Roles of Vitamin E
  • Vitamin E contributes to many processes in our bodies.
  • It is an antioxidant, protecting our cells from damage by free radicals, such as reactive oxygen species (ROS), including those produced by the body during normal metabolism.
  • Vitamin E also plays roles in the function of the immune system, and a variety of other biological processes.
• Possible effects of vitamin E deficiency
  • Lack of vitamin E can impair development and function of the nervous system and skeletal muscles.
  • Early signs of deficiency can include reduced reflexes and impaired coordination or tremor.
  • Immune responses may also be impaired.
• Good food sources
  • The highest amounts of vitamin E are provided by plants in the diet, especially nuts, seeds, and vegetable oils.
  • Leafy green vegetables and fortified cereals can also provide vitamin E.
• Supplementation
  • Oral dosing, preferably using the water-soluble form, TPGS.

Vitamin K

K₁=phylloquinone, largely from plants; K₂=menaquinones, mainly from animal foods, and can also be produced by bacteria in the human gut.

• Blood tests
  • As levels of vitamin K in blood fluctuate greatly, adequacy of vitamin K levels is most commonly and reliably assessed by screening for impairment of blood clotting, by determining the prothrombin time (PT) or International Normalized Ratio (INR).
  • The INR can be calculated from the PT.
• Roles of Vitamin K
  • Vitamin K is especially important for blood clotting and bone metabolism, amongst many other bodily functions.
• Possible effects of vitamin K deficiency
  • Impaired blood clotting
  • Increased risk of bleeding
  • Reduced bone mineralization.
• Food sources
  • Plant sources
    • Green leafy vegetables like spinach, collard or turnip greens, and kale, provide vitamin K₁, the main dietary form of vitamin K.
    • Other good sources include broccoli, lettuce, and vegetable oils, soybeans and some fruits.
  • Animal sources
    • Liver, fermented dairy products, eggs and meat can provide a modest amount of vitamin K₂.
    • Natto (made from fermented soybeans) is an excellent plant source of vitamin K₂.
• Supplementation
  • Oral dosing, preferably in a TPGS-based supplement, and/or through intramuscular or intravenous dosing.

7. Oral dietary intake

Eat a diet rich in fat, protein, and calories. While supplements may help with PFIC-related nutritional challenges, it is also important to eat a healthy diet that provides an adequate amount of calories, including enough enough fat and protein. A balanced diet including many foods will help ensure that a variety of nutrient needs are met, including adequate intake of nutrients such as essential fatty acids, iron, zinc, calcium, B vitamins and vitamin C.

For infants:

• Breast milk as expressed breast milk (EBM) can be fortified and fed in addition to breastfeeding
• Any combination of fortified formula or EBM via bottle with breastfeeding can be considered
• Fortified bottle feeds can include specialized formula that is rich in MCTs to support growth
• Frequent, small feedings can increase intake
• When bottle feeding is difficult or a baby experiences feeding intolerance, nasogastric (NG) tube feeds can provide enteral nutrition support, in addition to bottle or breastfeeding.
• Occasionally, when continued breastfeeding (without pumping) is strongly preferred, a supplemental nursing system (SNS) can be used.

For older children:

• Offering more frequent, smaller volumes of food or formula, including a bedtime snack, may help increase calorie intake.
• Try formula, shakes or smoothies- some children have an easier time taking in sufficient calories if they are in liquid form and can be quickly and easily drunk through a straw.
• Calorie boosting, by adding calorie-dense foods (such as MCT oils, butter, nut butters,  or mayonnaise) to their preferred foods, can help increase energy intake.
• For children who tend to eat lots of carbohydrates but insufficient protein, encourage snacks that pair carbohydrates and protein (such as cheese and crackers, tortillas and bean dips, eggs on toast) and limit the intake of sugary drinks (like juices and soda pops).
• As itching (pruritus) can distract a child from eating, treatment that decreases pruritus may help increase oral intake.
• To maintain a child’s energy and blood sugar levels, eating often (up to 6 small balanced meals per day) can help.

8. Difficulties with feeding, and use of Nutrition Support

Sometimes children are not able through oral intake alone to take in enough nutrients to make up for the malabsorption and effects of PFIC on the liver. In this case, they can receive additional nutrition through enteral and/or parenteral nutrition support (see below). Even when such means are used, it is strongly recommended to continue some oral intake of foods, and provide oral stimulation (especially for infants), to prevent development of feeding aversions, and minimize risk of developing food allergies.  

In cases where a child must get significant nutrition from tube feeding (enteral nutrition) or parenteral nutrition, encourage the child to eat at regular meals, and try to make oral intake a fun, positive experience. Some children respond well to things like reward/sticker charts. It is not uncommon for children with PFIC to have difficulties with eating. It is preferable, if difficult, to avoid having eating become a chronic battle between caregiver and child. Consultation with a health/food psychologist can be very helpful in making oral intake less stressful for the patient and family.

• Enteral Nutrition (EN or ‘Tube Feeding.’)
  • Formula or other liquid food is administered through a tube.
  • The tube can be a short-term soft tube that goes through the nose to the stomach (nasogastric; NG) or intestine (nasojejunal; NJ).
  • The tube can also be a long-term tube that is inserted in the stomach through the skin (gastrostomy; G-tube), if medically needed and if it can be safely inserted in the abdomen of the patient.

• Parenteral Nutrition (PN)
  • A solution containing macronutrients (carbohydrates, proteins, fats) and micronutrients (vitamins and minerals) is provided directly into a vein through an intravenous catheter (tube), bypassing the digestive system.
  • PN can be especially useful in helping to maintain nutritional status in a child awaiting liver transplantation (LT).
  • The amount of nutrition a patient obtains by PN varies. PN can be ‘partial’- providing supplementary nutrition to someone who is able to take in some food by other means, but not enough to avoid malnutrition- or ‘total,’ in which all nutrition is provided by PN.
  • PN can be given only at night while the patient is sleeping, or throughout the day if needed. 
  • A larger catheter inserted into a large, central vein allows partial or total PN, and may remain in place for long periods of time (many weeks to months)
  • A smaller catheter inserted into a smaller, peripheral vein (peripheral intravenous line, or PIV) allows partial PN, and is typically for short-term use.

While these options may sound invasive, they often substantially improve the quality of life, growth and development of children who otherwise struggle to get adequate nutrition.

9. Nutritional needs after liver transplantation

There are no general dietary restrictions or increased needs for patients after a liver transplant. Supplementation with special FSV formulations is generally no longer needed. Children often experience relatively rapid, catch-up growth in the period after recovery from transplant. Some children may have developed oral aversions and trouble eating orally, if they were mainly nourished through tube feeding or parenteral nutrition support before transplant. In such cases, working with a speech/language pathologist, occupational therapist, or health/food psychologist can sometimes help improve oral intake. Some children had little dietary variety pre-transplant, and should be encouraged to try a greater variety of foods. Some post-transplant patients may actually be at risk of gaining too much weight and are monitored for overweight and obesity to prevent poor liver and other health outcomes.

10.Some suggestions and examples of products that may help with nutritional management in PFIC.

Here, we outline some common products and approaches used in nutritional management of PFIC. Product availability and affordability varies between countries and centers around the world.  As such, approaches to managing nutrition among PFIC patients may vary. For example, in the U.S., FSV preparations effective in cholestasis can be quite expensive, and are often not covered by health insurance.

Formulas and nutrient supplements are typically prescribed by the patient’s care team. Detailed plans regarding diet, supplements, and/or nutrition support should be developed in collaboration with your child’s care team, which may include their liver doctor and dietitian or similar professionals on staff. In some cases when feeding difficulties arise, working with a speech/language pathologist, occupational therapist and/or health/food psychologist can also be very helpful.

Sources of MCTs

MCT-enriched Formulas

For more information on formulas, foods and vitamin supplements for PFIC patients, check out this PFIC 101 webinar, which features a section on nutrition management.

Whole Food Sources of MCTs

• Coconut oil is by far the best whole food source of MCTs, and is widely available. Consider using it as your primary cooking oil or fat.
• Palm kernel oil is also a good source of MCTs.
• Other coconut products, including coconut meat, cream and milk, also contain appreciable amounts of MCTs.
• Dairy products may contain modest amounts of MCTs

MCT Oil (such as that manufactured by Nestle Health Sciences or Nutricia North America).

If you want to add MCT oil to liquids, there are varieties available in powdered form or specially formulated to be emulsified (mixed) in water. Examples include:

• MCTprocal (powdered)
• Betaquik (emulsified)
• Liquigen (emulsified)

Note: Purified MCT oil has a lower smoke point than coconut or palm kernel oil, and so should not be used as a cooking or frying oil.

FSV Supplements

Water-miscible versions of vitamins are more easily absorbed than other forms of vitamins in people with cholestasis. Water-miscible vitamins include TPGS vitamin E, but also other forms, such as water-soluble palmitate (or vitamin A). Some FSV formulations used in PFIC include:

• Vitamin E (TPGS)
  • Single vitamin supplement
• DEKAS Plus
  • Contains Vitamins A, D, E, K, plus additional vitamins and supplements
• DEKAS Essentials
  • A, D, E, K only (at doses targeted for cholestasis)
• Aqua-E Concentrate, Aqua-D, Aqua-K
  • Other single vitamin supplements

Note: Health plan formularies may determine whether Vitamin D₂ and/or Vitamin D₃ is available and in an appropriate concentration for a child’s needs.

Strategies for improving compliance with FSV supplement intake in children. It is best to take TPGS Vitamin E at the same time as the other FSV, as the TPGS form of vitamin E helps with absorption of all of the FSV. The optimal time of day to take FSV is whatever time works best for the family, in order for the child to reliably take the vitamins; compliance works better with a fixed time. Ideally, FSV supplements should be taken after a meal such as breakfast, so that food is eatenfirst to stimulate bile flow into the intestine.

Dietary Intake.

While formula or nutrient supplements play a key role in addressing nutritional challenges in PFIC, in order to obtain a variety of important nutrients, it is also important to eat a healthy diet, incorporating enough fat, protein and calories. A typical recommendation might be for a diet in which 40-60% of calories can come from carbohydrates, with the remainder comprised of fats and proteins. Suggested goals for intake of protein in children with PFIC are often 3-4 grams of protein per kg body weight per day; for calories, in infants, as much as 130-150 kilocalories per kg body weight per day, and for children, as much as 1.5 times estimated energy needs.

Below are some suggestions for foods to incorporate to help attain intake goals for calories and protein.

High Calorie Food Ideas:

• Coconut Oil
  • Use as your primary cooking oil
  • Stir additional oil into pasta, soup or vegetables after cooking
  • Add to baked goods
• Full Fat Coconut Milk
  • Stir into smoothies, sauces or soups
  • Add to coffee or hot beverages
• Pure Maple Syrup
  • Use to sweeten hot cereal, yogurt or smoothies
  • Add to hot beverages
  • Top pancakes, waffles or toast
• Dried Fruit
  • Add to trail mix, yogurt or cereal
  • Use as a salad topping
  • Eat plain as a snack
• Avocado
  • Slice and add to sandwiches, toast or tacos
  • Make guacamole for chips or vegetables
  • Add to shakes and smoothies
  • Avocado Chocolate pudding
• Dressings/Condiments
  • Mayonnaise
  • Salad dressing

High Protein Food Ideas:

• Poultry, Meat and Fish
  • Red meat
  • Chicken with the skin on
  • Salmon or other oily fish
  • Pork
• Full fat high-protein yogurts (such as Greek yogurt, skyr)
  • Yogurt parfaits with nuts, fruit, granola or maple syrup
  • Add to smoothies or shakes
  • Stir into savory dishes like curry
  • Dipping sauces such as tzatziki or raita
  • Add to baked goods
  • Topping for chili or baked potatoes
• Nut and Seed Butters (such as peanut, almond, sunflower, cashew)
  • Spread on crackers or bread
  • Dip for apple slices
  • Stir into hot cereal
  • Add to shakes or smoothies
  • Add to baked goods
• Whole nuts and seeds (for older children*)
  • Plain as a snack or as trail mix
  • Sprinkled on top of yogurt bowls
  • Added to baked goods
  • Add to salads

(*Infants and young children should not eat nuts because of the risk of pulmonary aspiration)

• Dips (including legume dips, such as bean dips or hummus)
  • Dips for vegetables or pita chips
  • Add to sandwiches or tacos
• Eggs
  • Hard-boiled as a snack
  • Egg salad (plain or as a sandwich)
  • Add to baked goods
  • Scrambled eggs
  • Breakfast tacos or burritos

Suggestions for managing diarrhea. Diarrhea can be a problem in PFIC, and it can sometimes worsen after biliary diversion or liver transplantation. If the diarrhea has been evaluated by a patient’s medical team and determined not to be due to infection, changes to usual intake may help. Sometimes decreasing the amount of fat and/or increasing the amount of fiber in the diet can help manage diarrhea. Reducing the intake of simple sugars (like juices, some fruits) may also help reduce the diarrhea. Adding pureed green beans to formula (with oral intake or tube-feeding), or changing formulas to a hydrolyzed (proteins broken down to peptides) or elemental formula (proteins as individual amino acids) may also help. Occasionally, removing particular foods from the diet and assessing for food allergies may help. For some patients, diarrhea can be an ongoing challenge to quality of life, and working with a practitioner experienced in this area is strongly recommended. In some forms of PFIC, pancreatic insufficiency, and resulting diarrhea, can occur.

11. Some topics for scientific exploration

• Can the microbiome in PFIC be influenced by diet, probiotics, or other supplements or medications, to improve overall health and/or slow progression of liver disease.
• Are there any types of food or nutrients that tend to increase or decrease pruritus?
• When is use of other supplements, such as zinc, iron, or selenium, helpful in PFIC?

12. Your questions answered.

What herbal supplements are useful in PFIC? Please be cautious with use of herbal supplements as they are not regulated, it is hard to know if the product actually contains the supplement on the label (and it may also contain other things), and some supplements can be toxic to the liver. Often little or no research has been done on safety or efficacy in babies and children, or in patients with cholestasis.

Do we know what foods may intensify itching? No, we don’t know if particular foods affect itching due to cholestasis. We don’t have evidence that gluten-free or dairy-free diets decrease itching. Certainly, itching can affect whether a child is able to eat a sufficient, balanced diet. Food allergies and other allergies can also cause itching.

What to do, who to ask for help, when formula is denied by insurance? Talk to your dietitian or liver doctor. Formula coverage by private health insurance companies and by public health insurance programs varies between and within countries. In the U.S., your liver team may be able to do a peer-to-peer discussion with medical directors in the insurance company, which may result in approval. Often with private insurance, formula is an exclusion in the plan. In this case, families have sometimes gone to the employer, and had the employer talk to the insurance company to ask them to make an exception.

Which professionals can help us manage nutrition in our PFIC family member?

Your liver doctor can provide guidance. For more detailed nutrition management, many medical centers have a dietitian or similar professional on staff, with whom you can meet. In some situations, help from a speech/language pathologist, occupational therapist, or health/food psychologist can be very helpful.

13. Main sources I (Laura Bull) used while working on this document (Summer 2025-Summer 2026)

Lectures and slides:

• 2020 PFIC 101 lecture and slides
• Slides from Ron Sokol’s talk on nutrition at the 2024 PFIC meeting

Notes from my discussions with dietitians experienced in working with children with cholestasis:

• Stacey Beer at Texas Children’s Hospital- also reviewed the article
• Inez Martincevic at the Hospital for Sick Children in Toronto, Canada- also reviewed the article
• Elizabeth Tamayo at Cincinnati Children’s Hospital
• Members of the dietitian team at King’s College in London, UK- Karishma Manwani also reviewed the article

Emily Ventura, Executive Director of the PFIC Network helped in preparation and review of the article

Published manuscripts:

Boster JM, Rolfes PS, Sokol RJ, Sundaram SS. Nutritional Considerations for Children with Liver Disease. Gastroenterol Clin North Am. 2025 Dec;54(4):891-905. doi: 10.1016/j.gtc.2025.06.003. Epub 2025 Sep 12. PMID: 41238282.

Kamath BM, Alonso EM, Heubi JE, Karpen SJ, Sundaram SS, Shneider BL, Sokol RJ. Fat Soluble Vitamin Assessment and Supplementation in Cholestasis. Clin Liver Dis. 2022 Aug;26(3):537-553. doi: 10.1016/j.cld.2022.03.011. Epub 2022 Jun 25. PMID: 35868689.

Boster JM, Feldman AG, Mack CL, Sokol RJ, Sundaram SS. Malnutrition in Biliary Atresia: Assessment, Management, and Outcomes. Liver Transpl. 2022 Mar;28(3):483-492. doi: 10.1002/lt.26339. Epub 2021 Nov 10. PMID: 34669243; PMCID: PMC8857023.

NIH web pages:

https://ods.od.nih.gov/factsheets/VitaminA-HealthProfessional

https://ods.od.nih.gov/factsheets/VitaminE-HealthProfessional

https://ods.od.nih.gov/factsheets/VitaminD-HealthProfessional

https://ods.od.nih.gov/factsheets/VitaminK-HealthProfessional