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.
According to Cynthia Taggart, RD at Cincinnati Children’s Hospital:
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” 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.
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.
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.
The “Parents provide, kids decide” method:
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 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 percentile 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:
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).
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.
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.
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 a 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 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: