Nutritional Guide
Affecting more than 50% of people with achondroplasia, obesity is a common problem among people with this condition that can lead to or exacerbate a series of other complications associated with the disease [1].
Obesity is generally accepted as being a risk factor for cardiovascular disease in the general population and may be a contributing factor for the increased risk for heart disease-related death associated to achondroplasia [2]. Also, by being a contributing factor for other common complications, such as obstructive sleep apnea, genu varus, spinal stenosis and hyperlordosis it is important to monitor weight gain to maintain health and avoid other complications [3].
The recommendation to avoid obesity is to monitor weight from childhood, using weight-for-age charts based in the north american population and to seek nutritional counseling as early as when the child reaches 75 cm of height [1, 3, 4].
However, the lack of a Food Guide Pyramid made nutritional counseling for people with achondroplasia difficult until 2012, when Dr. Katheryn Lamb developed a Food Guide Pyramid specific for people with this condition, which can be seen below [5].
Credits: Dr. Kathryn Lamb in "Development Of Food Guide Pyramid For Individuals With Achondroplasia" |
These recommendations are based on the resting metabolic rates (RMR, the amount of energy spend while in a resting state) calculated by Owen et al., resulting in 3 meal plans based on the necessary energy levels, for the smallest, middle and highest RMR within the group: 1000, 1400 and 1800 calorie meal plans, respectively [5, 6]. These can be consulted in the table below:
Daily Meal Plans for the three energy levels. Credits: Dr. Kathryn Lamb in "Development Of Food Guide Pyramid For Individuals With Achondroplasia" |
Energy Level (kcal) | Food Group |
Number of Exchange Servings |
Estimated Total Serving Size |
1000 | Grains | 4 | 4 oz. (113 g) |
Vegetables | 2 | 1 cup cooked, 2 cups fresh | |
Fruits | 3 | 1.5 cups | |
Dairy | 1 | 1 cup | |
Protein | 4 | 4oz. (113 g) | |
1400 | Grains | 6 | 6 oz. (170 g) |
Vegetables | 2 | 1 cup cooked, 2 cups fresh | |
Fruits | 3 | 1.5 cups | |
Dairy | 2 | 2 cups | |
Protein | 5 | 5 oz. (142 g) | |
1800 | Grains | 7 | 7 oz. (198 g) |
Vegetables | 4 | 2 cups cooked, 4 cups fresh | |
Fruits | 4 | 2 cups | |
Dairy | 3 | 3 cups | |
Protein | 6 | 6 oz. (170 g) |
Sources:
- Unger, S., L. Bonafé, and E. Gouze, Current Care and Investigational Therapies in Achondroplasia. Current Osteoporosis Reports, 2017. 15(2): p. 53-60.
- Wynn, J., et al., Mortality in achondroplasia study: A 42-year follow-up. American Journal of Medical Genetics Part A, 2007. 143A(21): p. 2502-2511.
- Ireland, P.J., et al., Optimal management of complications associated with achondroplasia. The Application of Clinical Genetics, 2014. 7: p. 117-125.
- Hoover-Fong, J.E., et al., Weight for age charts for children with achondroplasia. American Journal of Medical Genetics Part A, 2007. 143A(19): p. 2227-2235.
- Lamb, K.E., Development Of Food Guide Pyramid For Individuals With Achondroplasia, in Food and Nutrition2012, University of Carbondale: Southern Illinois. p. 80.
- Owen, O.E., et al., Resting metabolic rate and body composition of achondroplastic dwarfs. Medicine (Baltimore), 1990. 69(1): p. 56-67.