Heidi Puelzl Quinn, MS, RD
Developmental disabilities (DD) is a term used to describe a collection of disorders that cause an impairment in normal development and body function.' There are a wide range of disabilities, with varying degrees of impact on growth and nutritional status. It is estimated that the incidence of developmental disability in the pediatric population is approximately 37r.: Approximately 90% of children with developmental disabilities have nutritional concerns.- Some of these are outlined in Table 22-1.
Oral-motor and feeding difficulties are common in children with developmental disabilities. It is generally helpful to have an interdisciplinary feeding evaluation performed to establish an appropriate feeding plan that optimizes diet intake as well as feeding skill development. Ideally, the team should consist of the following clinicians: nutritionist, speech therapist, occupational therapist, physical therapist, behavioral management specialist, developmental pediatrician, nurse. Some children with DD may require support with supplemental tube feedings to meet their fluid and nutrient needs for adequate growth and good health. Coordination of tube feeding and oral feeding to maintain oral motor skills while insuring good growth and health is recommended, provided there are no contraindications to oral feeding, such as aspiration. Regular reassessment of the feeding plan is essential as the child grows and develops.
Table 22-1. Nutritional Risk Factors for Children with Developmental Disabilities
Obesity (Prader-Willi, Laurence-Moon-Biedl. Carpenter's, and
Down syndromes) Failure to thrive (Rett syndrome, cerebral palsy [CP]) Short stature (Down, Hurler's, Russell-Silver, and Cornelia de Lange's syndromes) Gastrointestinal symptoms Diarrhea Constipation
Vomiting/gastroesophageal reflux Oral-motor difficulties Discoordination of suck/swallow Structural abnormalities (cleft lip/palate: dentition) Poor oral containment (food/fluid loss) Tone abnormalities (hypo/hypertonic) Altered oral sensory response (hypo/hyper-responsive) Delayed oral motor skill development Aspiration
Restricted intake (metabolic disease, food allergies, food texture aversion) Inadequate intake (poor appetite, poor oral motor control, malabsorption) Increased calorie requirement (athetoid CP, spasticity) Inadequate fluid intake Positioning for feeding
Adaptive seating devices Behavior Oral aversion Pica
Rumination Hyperactivity Distractibility Perseverative behaviors Binge eating/overeating Feeding skill development Sell-feeder vs. dependent feeder Adaptive feeding equipment
Adapted from Hendricks K, Walker WA. Manual of pediatric nutrition. 2nd ed. Toronto: B.C. Decker. Inc.; 1990. p. 211-215.
Special Aspects of Nutritional Assessment and Management
There should be a complete review of birth, medical, and feeding history to determine the potential effects of long-term hospitalization, surgery, and medical procedures (ie. intubation, supplemental tube feedings) on overall development as well as on oral feeding and feeding skill development. Harly medical/feeding history can provide information regarding development of feeding problems such as oral aversion.
Obtaining accurate weight, length/height, and head circumference measurements, and plotting serial points over time, provide critical information on growlh adequacy. Growth should be plotted on National Center for Health Statistics (NC'HS) growth charts or specialized syndrome-specilic growth charts if available. Specialized growth charts are currently available for various diagnoses, including Down syndrome. Turner's syndrome. Prader-Willi syndrome, myelomeningocele, sickle cell disease, and achondroplasia (sec Appendix K).1 Accurate measurement of linear growth in children w ith DD may be compromised by the presence ol contractures, scoliosis, kyphosis, or an inability to stand. Alternative methods of linear measurement include crown-rump length or sitting height, arm span, tibial length or segmented body length.4-> Use of these methods may also be compromised by contractures and/or scoliosis.
It is not uncommon for children with DD to be small for their age, w ith growth parameters below the 5th percentile on standard growth charts.1 Assessment of weight for length/height is especially important in this population as this indicates individual proportionality, which is a more appropriate way to evaluate adequacy of growth in children with DD. It is also important to note that alterations in head circumference (micro/macrocephaly) can skew the weight-for-age and weight-for-length parameters. Alterations in body composition with regard to muscle mass and body fat stores also impact the grow th assessment. The use of midarm circumference and skin fold measurements are helpful in assessing these parameters,"1
Alterations in activity level will also have an impact on weight goals. For nonambulatory individuals, weight-for-length of 10 to 25th percentile is generally an acceptable goal. In nonmobile individuals, additional weight is often accumulated as increased fat stores rather than muscle mass. Excessive weight can compromise care in terms of cardiorespiratory health as well as ease of transfers (bed. bath, wheelchair) and progression with gross motor skills. Given these considerations, a visual clinical assessment, in conjunction with growth history, is essential when assessing adequacy of growth in children with developmental disabilities.
Energy. Caloric requirements may be assessed in several ways: (1) calories per centimeter of body height/length (Table 22-2); (2) catch-up growth equations using height age instead of weight age; or (3) standard equation using Basal Energy Expenditure (BEE) x activity and injury factors.1 It is important to note, however, that these methods are merely guidelines and that individual calorie requirements should be assessed based on changes in weight over time and/or measurement of basal metabolic rate, if possible. Therefore, regular weight monitoring is an essential component in managing children with DD, Caloric requirements may be as low as 5 kcal/cm of height in children with severe central nervous system impairment.r>
Table 22-2. Guidelines for Estimating Caloric Requirements in Children with Developmental Disabilities
Ambulatory, ages 5-12 years Nonambulatory, ages 5-12 years Cerebral palsy with severely restricted activity Cerebral palsy with mild to moderate activity Athetold cerebral palsy, adolescence Down syndrome, boys ages 5-12 years Down syndrome, girls ages 5-12 years Myelomeningocele
13.9 kcal/cm height 11.1 kcal/cm height 10 kcal/cm height
15 kcal/cm height
Up to 6,000 kcal/d 16.1 kcal/cm height 14.3 kcal/cm height Approximately 50% of HDA for age after infancy. May need as little as 7 kcal/cm height 10-11 kcal/cm height for weight maintenance; 8-9 kcal/cm height for weight loss
Adapted from: Frick MS. Developmental disability. In: Perberton CN, Moxness KE, German MJ, et al, editors. Mayo Clinic diet manual. 6th ed. Toronto: B.C. Decker; 1988. p. 320; and from Frick MS. Other nutritional considerations. In: Nelson JK. Moxness KE, Jensen MD, Gastineau CF, editors. Mayo Clinic diet manual. 7th ed. St. Louis: Mosby; 1994. p. 457.
Catch-up growth equations using height age:
IBW lor height x RDA kcal/kg height age
IBW for height x RDA g protein/kg height age actual weight
Protein. Protein requirements are estimated using RDA for chronologic age or height age if growth parameters are significantly below chronologic age.1
Vitamins/Minerals. The most common nutrient deficiencies seen in children with DD are vitamins A. C. D. and folate, as well as iron and calcium.4
Fluid. Fluid requirements may be higher in some children with DD due to constipation, increased fluid losses (drooling, excessive sweating), and/or increased requirements. Standard guidelines for fluid based on body weight should be followed, with adjustment for special considerations as noted above (see Table 17-3, Fluid Requirements).
Some children with DD are on multiple medications, which can interfere with nutrient absorption, appetite, elimination patterns, and level of alertness for feeding. For example, children with seizures who are on multiple anticonvulsant medications should be monitored for adequate vitamin D and folic acid intake as requirements for these nutrients are increased with some seizure medications (eg. phenytoin [Dilantin]). Also, some medications can contribute to constipation, which often inhibits appetite. Due to the potential for inadequate diet intake, drug-nutrient interactions, and possibly decreased mobility, laboratory values reflecting iron, protein, vitamin D. calcium, and phosphorus status should be monitored on a regular basis (see Appendix B. Drug-Nutrient Interactions).
Children with DD are at increased risk for feeding difficulties due to alterations in motor and neurodevelopmental status. Nutritional management often involves dietary modifications such as enhanced calorie intake (Table 22-3). enhanced fiber intake, and texture modification (ie. pureed diets, thick ened liquids |T;thle 22 4|) to meet oral-motor skill level as well as nutrient needs. Natural thickeners are preferred over commercial cornstarch-hased thickeners as thev provide additional nutrients as well as calories and in some cases contribute to thud intake. Calorie level can be adjusted based oil the choice of thickener. In addition, cornstarch-hased thickeners can contribute to constipation, which is a common problem for childern with DD.
Ideally, the nutritionist works in conjunction with a speech therapist or occupational therapist to develop a feeding plan. A videofluoroscopic swallow study (also called a "moditied barium swallow") may be indicated to assess the efficiency and safety of the swallowing mechanism. This stud> is performed jointly by a radiologist and a speech language pathologist or occupational therapist with specialization in oral-motor feeding difficulties. The child must willingly consume lluid/foods of several textures in small amounts; this study cannot be performed on a child who will not or cannot consume food or fluid by mouth. Table 22-? lists sev eral "red Hags" of feeding difficulties that indicate further assessment of swallowing function is warranted. Table 22 6 lists the common clinical indicators for performing a swallow study.
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