what-when-how
In Depth Tutorials and Information
CHAPTER
40
Nutrition
in Osteogenesis I
mperfecta
Elisabeth Enagonia
Kennedy Krieger Institute, Baltimore, MD, USA
INTRODUCTION: COMMON NUTRITION
RELATED PROBLEMS IN OI
balance of proteins, carbohydrates and fats, meeting
the Dietary Reference Intakes (DRIs) for vitamins and
minerals, taking in the recommended amount of fiber
and taking in enough fluid to meet hydration needs. In
addition, infants, children and adolescents with OI need
to meet their nutrient needs for appropriate growth.
Depending on the severity of OI, mobility and activ-
ity level, and body size and height, individuals with
OI may need to reduce their energy intake by decreas-
ing portion sizes and choosing lower fat, lower kcalorie
foods. If energy needs are unusually low, care must be
taken to ensure adequate intake of protein and micro-
nutrients. Suggestions are included in this chapter.
Specific nutrient needs of individuals with osteogen-
esis imperfecta (OI) are not well researched.
1
Obesity
may be a problem for individuals with OI (
Figure 40.1
).
Limited physical activity, small body size and/or
short stature, and intake of excess kcalories can cause
excess weight gain. Overweight and obesity increase
the risk for chronic diseases such as cardiovascular dis-
ease (CVD) and type 2 diabetes. Excess bodyweight can
limit mobility, increase stress on joints and bones, cause
skin breakdown and pressure ulcers, and impair quality
of life.
Gastrointestinal problems may occur in some per-
sons with OI. Scoliosis and other truncal or pelvic
abnormalities may cause gastroesophageal reflux (GER)
or constipation. Persons with OI may have lactose intol-
erance. Although medical management may be needed,
avoiding foods that exacerbate GER may be helpful,
and adequate fluid and a diet high in fiber may help
alleviate constipation.
Poor nutrition can occur in both adults and children
with OI. Causes may include poor dietary choices, low
kcaloric intake in an effort to manage bodyweight, dif-
ficulties with eating solid food, and decreased appetite
secondary to medication, bone pain, surgeries, or other
physical and mental health concerns.
Kcalories
Energy requirements are defined as the amount of
energy intake from food that balances energy expendi-
ture, while also providing the energy needs for tissue
maintenance, growth, and supporting higher demand
states such as pregnancy, lactation or recovery from ill-
ness or injury.
2
Estimates for energy needs of healthy persons are
included in the DRIs published by the Institute of
Medicine.
3
The estimated energy requirement (EER)
is the average kcaloric intake that is estimated to meet
the needs of a healthy person to maintain health and
participate in physical activity consistent with health.
The EER takes into account age, gender, weight and
height. In addition, there are calculations that factor in
physical activity at a sedentary, moderate and active
level. The EER, stratified by age, gender and activity
dri-tables
.
4
Energy balance is achieved when an individual's
intake balances energy expenditure. Energy intake that
KCALORIE AND
MACRONUTRIENT NEEDS
The nutrition goals for individuals with OI are the
same as for any individual: to achieve optimal health
by taking in adequate kcalories to achieve and main-
tain a healthy weight, eating a diet with an appropriate
