Management of growth and nutrition

Question and answers with Dietician Dee Madore

Written June 2018

Growth in children with ASXL syndromes is different from healthy children. Our children are characterized with IUGR (Intrauterine Growth Retardation) and severe feedings problems, as well as failure to thrive. With this in mind, it is important to take a different approach for managing growth and nutrition.  Due to the limited published knowledge, often the expectations of how these children “have to grow” and the concerns and pressure on parents and caregivers if they did not reach the growth standards can be overwhelming.

We asked Dee Madore five questions how to "Gain a Healthy Weight"! Dee Madore has received her Master’s and Bachelor’s Degrees in Human Nutrition from the University of Maine, and she has been practicing as a Registered Dietitian for over 17 years. Her career has been heavily clinical, and focused in pediatrics, but in the recent years, her scope has expanded to include mostly outpatient counseling. Dee Madore saw the need for this type of practice and started Nourish Me Well.

Question 1: Since there are so few cases of ASXL syndromes and variation in their clinical presentations, growth charts are not available to help benchmark averages.  What are some of the metrics that you track to determine a healthy weight?  What type of trends do you track?

"Giving lacking growth charts for ASXL syndromes, it becomes important to monitor weight for length, weight for height or Body Mass Index (BMI).  In children aged 0-24 months, the World Health Organization weight for length chart is generally used.  For children aged 2-20, the Centers for Disease Control BMI or weight for height charts are used. On the weight for length or height charts, length or height is on the X axis and weight is on the Y axis.  Weight for length or height is considered normal from 5th percentile to 95 percentile. This gives the practitioner an idea for assessing whether weight for length is too low or too high. The BMI chart has age on the X axis and BMI on the Y axis.  BMI is calculated by weight in kilograms divided by height in meters squared.  There is certainly not an expected or ideal weight for length or height or BMI, as there are several factors that influence what is ideal, such as the presence of hyper or hypotonia. 

I generally aim for the weight for length or height or BMI to fall between the 10th and 75th percentile. However, if a child appears too thin with a weight for length of 10th percentile, the goal then becomes a higher percentile.  Conversely, the goal weight for height may be less than the 50th percentile if a child appears too heavy at a given percentile.  Given the unpredictability of linear growth (gains in height), there is no standard expected rate of weight gain in terms of ounces per month or grams per day.  The goal is for the rate of weight gain to not exceed the rate of linear growth. In order to feel confident that the child is growing in length as well as they possibly can, the dietitian makes certain that the food and or formula intake is meeting protein and vitamin/ mineral needs. 

Calorie needs vary, so determining calorie needs is not as simple as using an equation for the typically developing child.  The equations can be used as a baseline, but it takes experience over time to fine tune.  Often, the child’s weight age is used rather than chronological age to determine calorie needs when using existing equations for typically developing children. However, delays in mobility are considered and deductions are taken accordingly. Plotting on standard growth charts also allows the practitioner to compare the growth curve of the child against the growth standards. It’s not expected that the growth trend of the child follow the slope of the standard curve, but it provides a helpful visual to assess trends."

Question 2: For children who struggle with weight gain, what are some of the strategies their caregivers can implement to assist them (e.g. increased frequency of feeds, fat boosted diet, Better than Milk, etc.)?

"To increase calorie intake in children who struggle to gain weight, it’s about increasing natural healthy fats and carbohydrates. Healthy fats include olive or safflower oils, flax meal, avocado, olives, nut butters, nut meal, salad dressings, and coconut oil. Butter, mayonnaise, cream cheese and sour cream are not generally considered healthy fats, but children can afford to have animal-based saturated fats as part of a healthy diet. Using some additional natural carbohydrates in the diet to increase calories is also an option, but they have fewer calories than fats do. Examples would be banana puree, honey, real maple syrup, molasses and powered calorie boosters such as Better Than Milk or Duocal, which consist of both carbs and fat. Also, small frequent feedings are helpful to promote an increase in calorie intake. Children should be fed about every 2-3 hours, about 5 or 6 meals and snacks per day."

Question 3: Some children with ASXL syndromes struggle with gastric dysmotility, cyclic vomiting, and constipation. What are some foods/recipes that can assist with these symptoms?

"For GI dysmotility, cyclic vomiting, and constipation, multiple nutrition adjustments can be made to help ameliorate the problem. Children on standard commercial formulas tend to improve from a GI standpoint on whole food based formulas, either commercial brands or homemade blenderized diets. Vomiting can be decreased by decreasing the size of feeds and feeding more frequently or decreasing fat content of the feedings without compromising overall calorie intake. Increasing soluble fibers in the diet, which bring fluid into the intestine, can help with constipation by adding bulk to the stool. Oats, beans, and blueberries are examples of high soluble fiber foods. Recipes using ingredients such as prunes, prune juice, figs, dates, and raisins can be helpful as natural laxatives."

For constipation and eventual possible Miralax weaning, try the following recipe:

Power Pudding: Mix one cup applesauce with one cup coarse unprocessed bran and 3/4 cup prune juice. This thick paste should be refrigerated. Take a tablespoon daily, washed down with eight ounces of water.

Question 4: How do you balance calorie, protein, and fluid needs?  What is the impact of disproportionate amounts of any of these?

"For children, a balanced diet provides the correct amounts of protein, calorie and fluids. Excessive proteins may be harmful to the child’s organs, particularly if the child also struggles to meet fluid needs. Excessive fluid intake in the form of juice can “fill up” the child with carbohydrates and lead to decrease in solid food intake, which makes the diet unbalanced. This can either result in excessive weight gain, or poor weight gain. Adding too many empty calories to the diet in the form of sugars and fats may decrease the child’s intake of healthy fruits, vegetables and whole grains, which can lead to vitamin and mineral deficiencies."

Question 5: For children who are primarily G-tube fed, do you have any recommendations or resources to help with weight gain and a balanced diet?

"Children who are primarily G-tube fed should be monitored closely by a practitioner who can assess the adequacy of the child’s growth and intake.  If weight gain is poor, but the child can not tolerate more volume, there are more concentrated formulas that contain more calories.  On the other hand, if weight gain is excessive, there are lower calorie formulas that do not have diluted protein or vitamins and minerals. 

Blenderized or whole food-based diets are superior to standard formulas, so they should be considered if possible.  Parents can get advice from chat sites that include parents of children on tube feedings, but recipes should be provided by a Registered Dietitian who understands the child’s unique nutritional needs.  Eatright.org is a reputable website that will help parents find a qualified RD or RD/N in their area.  Medical Nutrition Therapy provided by RDs and RD/Ns is generally a covered service under medical insurance plans."

Children's BMI-percentile-for-age calculator

Use this body mass index (BMI) calculator to determine whether a child is at a healthy weight for his/her height, age, and gender. The BMI-percentile-for-age calculator automatically adjusts for differences in height, age and gender, making it one of the best tools for evaluating a growing child's weight.

Medical disclaimer: This website does not provide medical advice. Our website contains general medical information about congenital ASXL mutations. This information is not medical advice and should not be treated as such. You should always talk to your or your family member’s health care professionals for diagnosis and treatment, including information regarding which drugs or treatment may be appropriate for you or your family member. We do not represent or warrant that any particular treatment is safe, appropriate or effective for you or your family member. In the event of a medical emergency, you should contact your doctor immediately or call 911.