Nutrition in FA

Many individuals with FA experience poor growth, proportionately lower weight, and have short stature. Parents of children with FA are often concerned about their child’s poor weight gain and “picky eating.” This is a quick guide to help families understand nutrition issues in children with FA.

How is growth affected by FA?

  • Individuals with FA may be shorter than expected based upon the genetic condition itself, non-FA related genetics, hormonal abnormalities, malnutrition, malabsorption, or growth suppression due to inflammation associated with infection.
  • Malnutrition, whether the result of poor food intake, high energy utilization, utilization of fat and protein over glucose, or excessive stool loss, initially results in a growth curve demonstrating low weight relative to height or low BMI relative to age.

What is a “normal” growth curve and what if my child’s measurements do not fall within “typical” growth percentile ranges

According to Cynthia Taggart, RD at Cincinnati Children’s Hospital:

  • Growth curves allow physicians to monitor a person’s growth over time in comparison with other individuals of the same age and gender.
  • Dieticians and physicians monitor trends in weight, height, weight/height comparison (BMI) in children with FA.
  • Weight and height should be measured at each clinical visit and the child’s measurements should be plotted on a graph called a growth curve.
  • Typical growth range for a typical child is between 3rd and 97th percentile; however, there are conditions and diseases where children will never grow between the 3rd and 97th percentile.
  • Oftentimes, the growth curve of an FA child will mimic a non-FA child’s growth curve but remain significantly below the typical growth curve. This may be okay, as long as there is some growth!
  • Dieticians who see children with FA look to see that the child has established their own growth curve. They may not gain weight or height like a non-FA child, but it is important to examine whether they have established their own growth curve regardless of where that growth appears on the growth chart.
Nutrition in FA overview (June 2020)

What if my child is a picky eater?

Poor oral intake can result from many factors, including complica­tions of anatomic gastrointestinal abnormalities (narrowing of the digestive tract or complications of repair), chronic inflammation and/or infection, medication side effects, or neuro­logic/behavioral problems.

Individuals who are “picky eaters” may benefit from behavioral therapies, such as family-based and cognitive-behavioral (CBT) approaches to increase the variety of foods eaten. According to Essayli & Vitousek (2020), CBT is recognized as an effective treatment for adults who struggle with restrictive food behaviors, however additional research is needed to show efficacy for younger individuals. To date, there is no research pertaining specifically to the efficacy of behavioral therapies for children with FA, however, behavioral therapies have been effective in other patient populations with poor food intake. For example, in patients with cystic fibrosis, behavioral modification has demonstrated long-term improvements in food intake.

Dietary counseling, with or without evaluation by a feeding specialist, may be enough to improve oral intake; however, if food intake does not increase, counseling should be aimed at maximizing calories by the addition of high-calorie foods to diet and liquid or powder supplements (e.g., bananas, avocados, nut butters, hummus, cheese, oil, butter, dairy, dressing, medical-grade calorie additives, etc.).

Aggressively trying to increase the individual’s food intake will not increase their height or overall health and may even encourage disordered or unhealthy eating problems.

Healthy eating information & tips

Provided by Cynthia Taggart, RD at Cincinnati Children’s Hospital

The gold standard for healthy eating can be found on MyPlate.gov. It replaced the traditional food pyramid and was put out by the United States Department of Agriculture (USDA). These standards dictate that half of a plate of food should contain fruits and vegetables, a quarter of the plate should be protein, a quarter grains, and dairy as a supplement to the meal.

  • Eat in-season fruits/vegetables
  • Eat natural sugars and avoid processed sugars (e.g., eat dried fruit, fresh fruit)
  • Add variety to meals by adding a new vegetable to meals each meal every day
  • Dips, dressings, and dairy can be a great source of extra calories
  • Many processed foods contain high amounts of sodium. Choose fresh fruits and vegetables when possible. Try using spices and herbs to add flavor in place of salt.
  • Purchase lean meat to stay within saturated fat limits (e.g., chicken, fresh tuna, venison, etc.).
My Plate Chart Healthy eating standards as defined by the USDA

Organic foods vs. non-organic

According to Cynthia, “There have not been any direct studies on humans to show that organic foods can prevent cancer or other diseases any more effectively than conventionally grown foods. So far, there is also no consistent evidence that organic food is more nutritious than conventionally grown foods.”

Eating organic is a family choice and preference.

  • Dirty Dozen (2020): Every year the Environmental Working Group (EWG) organization publishes a list of foods that have been tested and found to have high amounts of pesticides, AKA the “Dirty Dozen.”
  • Clean Fifteen (2020): the EWG also produces a list of foods tested and found to have low amounts or no pesticides, AKA the “Clean Fifteen.”

Mealtime routine tips

The “Parents provide, kids decide” method:

  • Parents serve a variety of healthy foods and children get to choose from those foods offered
  • Offer new foods with favorite foods (avoid offering all new foods on the plate)
  • When introducing new foods, it can take 12-20x for a child to accept that new food. This is frustrating but keep at it!
  • Avoid becoming a short-order cook
  • Prepare the same foods for everyone in the family
  • Stick to regular meals and snack routines (every 2-4 hours). Allow 20-30 minutes for a meal and 15-20 minutes for a snack.
  • Offer water between meals, avoid offering juice or milk between meals/snacks.
  • Have a dedicated place to eat meals and snacks and attempt to create a pleasant eating environment.
  • Limit distractions during meals
  • Eat together when possible and model positive eating habits.
  • Avoid centering conversations around how much or how little your child is eating. Expect your child’s appetite to vary from day to day.

It may become a concern if your child shows a decline in eating over a week-long period or isn’t eating like they previously did. If this is happening consult a physician.

Supplemental feeding

Supplemental feeding via a feeding tube (enteral supplementation) may be needed to achieve a healthy nutritional status in children with FA who are persistently less than 85% of the expected weight for their height, who have a BMI that is persistenly less than the 3rd percentil for their age, or who have failed to gain weight over a 3-to-6 month period and when deemed necessary by a physician.

  • An NG tube is inserted into the nose, goes through the esophagus and into the stomach. These feeding tubes are typically meant to be short-term.
  • G-tube requires a surgical procedure and is placed into the stomach through the abdominal wall. G-tubes are often used for long-term nutritional needs.

Pros of feeding tubes include helping to meet nutritional needs and mitigating the pressure of mealtimes. In some cases, they may assist with stretching the stomach and can be used if there are esophageal or swallowing issues. Finally, G-tubes may also be used for medication administration. Cons of feeding tubes include discomfort with placement and the care and maintenance of the tube.

There are many different products and companies that provide formula for tube feeding (e.g., higher calorie, lower-calorie, broken down proteins, free of allergens, vitamins, organic, etc.). Things to consider when choosing tube feed formula:

  • What are the ingredients?
  • Will my child tolerate this formula?
  • What is the cost and is it covered under my insurance plan?
  • Is it available through my durable medical equipment (DME) company?

Appetite stimulants: Before utilizing appetite stimulants, physicians must first investigate and appropriately manage diagnosable causes of poor appetite and inadequate growth in FA patients. Appetite stimulants will not treat delayed gastric emptying, depres­sion, chronic infection, or other treatable causes of inadequate weight gain and growth. It remains unclear whether any weight gained while taking appetite stimulants will be maintained after the medication has been stopped. Nonetheless, there are several medications that have appetite-stimulating side effects (e.g., cyproheptadine, megestrol acetate, and the atypical antipsychotic agents, olanzapine and mirtazapine).

What about vitamins and supplements? Are they safe to use?

Consult with a specialist before using any vitamins and supplements. It is important to know what is in each supplement, where it was produced and how, in order to limit exposure to environmental toxins.

  • All individuals with FA should be screened for vitamin D deficiency at least once a year, preferably during the winter. Furthermore, individuals with FA may also have deficiencies in or increased need for other specific vitamins and minerals, including folate, and zinc. It is important to note that large studies in the general population have shown that both vitamin A and vitamin E supplements are associated with an increased risk of some cancers; therefore, FA patients should avoid additional supplementation with these vitamins until further study indicates otherwise.
  • A complete multivitamin can be incorporated into an individual’s routine, however not all gummy multivitamins are complete. Discuss with a physician or dietitian before purchasing multivitamins.
  • If the individual with FA receives blood transfusions, iron supplementation is typically not recommended.
  • Iron deficiency anemia often requires iron supplementation and removing certain foods from the individuals’ diet.

What if my child is overweight and has FA?

As with the general population some patients with FA are overweight or obese. Both a diagnosis of being overweight or obese need to be confirmed by a physical exam. Significant complications may result from overweight and obesity, including elevated levels of fat and cholesterol in the blood, diabetes, obstructive sleep disorder, and other aspects of metabolic syndrome—a combination of disorders that increase the risk of developing cardiovascular disease and diabetes.

Physicians may ask patients to keep a 6-day diary of diet and daily activity to provide the foundation for counseling regarding dietary and exercise changes. Families may require monthly counseling sessions to ensure achievement of the appropriate weight. Psychological counseling may also help, especially if an eating disorder is suspected. Management of overweight and obesity is a long-term process, requiring the commitment of the entire family for success.

Individuals with FA should be urged to avoid fad diets and over-the-counter weight loss preparations and to focus on healthy lifestyle modifications.

Remember

The overall nutritional status of individuals with FA can be determined during each routine physical exam by assessing muscle mass, skin and mucus membrane health, and energy and activity levels.

Just like all of us, children with FA have different body types and needs. Your child may grow at different rate than a non-FA child or even other children with FA; this is not necessarily a negative thing. Remember that physicians and dieticians familiar with treating FA will look at whether a child has established their own growth curve. Evaluation by a pediatric endocrinologist may be needed for children with FA who exhibit poor growth and/or stop growing and do not develop their own curve.

You may find additional information on nutrition, the endocrine system, and metabolism in FA below: