High Institute Of Public Health

Alexandria University

Department of Nutrition

NEUROLOGICAL HANDICAPPED CHILDREN

BY: Orchid Essam- Eldin

Samah Nafis

Heba Fathy

SUPERVISOR: DR. Dalia Tayel

NEUROLOGICALLY HANDICAPPED CHILDREN

Introduction

A physically handicapped person is defined as a person who has disabilities of

orthopedic, musculoskeletal, or neurological origin which mainly affect locomotor

functions, and constitute a disadvantage or restriction in one or more aspects of daily

living activities. include caring for one's self, eating, performing manual tasks, walking,

seeing, hearing, speaking , breathing , learning, and working.

Those who provide medical care for disabled children must be familiar with the wider

implications of:

A disorder is a medically definable condition or disease entity (e.g. brain injury causing

diplegia).

An impairment is any loss or abnormality of psychological, physiological or anatomical

structure or function (e.g. weakness and stiffness of legs).

A disability is any restriction or lack (resulting from an impairment) of ability to perform

an activity in the manner or within the range considered normal for a human being (e.g.

inability to walk quickly or climb stairs or steps).

A handicap is the impact of the impairment or disability on the person's pursuit or

achievement of the goals which are desired by him or expected of him by society (e.g.

unable to undertake any employment that requires mobility or access to public

buildings). This terminology is derived from International Classification of Impairments,

Disabilities and Handicaps: WHO; Geneva, 1980. (ICIDH) model &the updated one.[1]

Etiology&risk factors

Factors predicting a high risk of subsequent neurological handicap include: prematurity,

perinatal asphyxia, haemorrhagic disease of the newborn, congenital anomalies and

infections. The major determinants of neurological handicap in preterm survivors are

intraventricular hemorrhage (IVH) (the incidence may be as high as 40% in very low

birth-weight infants) and cerebral ischemia/atrophy.

The etiology is multi-factorial but is associated with fetal distress and labor problems,

and postnatal factors such as seizures, hypothermia and respiratory distress syndrome

and its complications. [2]

Common causes of neurological disorders in children

I Non nutritional

1. Cerebral palsy

2. Epilepsy

3. Guillain- Barre syndrome

4. Neurotrauma

5. Spinal trauma

II Nutritional deficiency or excess

1. Beriberi (thiamin deficiency)

2. Pellagra (niacin deficiency)

3. Pernicious anemia ( B12 deficiency)

4. Maternal folic acid deficiency[3]

Feeding problems in neurologically impaired children

Feeding dysfunction is often the result of interacting variables, including:

1) Dysfunctional oral motor control:

Hypotonia

Weak suck

Delayed or absent tongue lateralization

Poor lip closure

2) Abnormal neurological maturation:

Uncoordinated swallowing mechanism

Hyperactive gag reflexes

3) Poor seating posture during feeding due to unstable trunk.[4]

4) Food inhalations with consequent respiratory impairment.

5) Frequent gastroesophageal reflux &the overall motor dysfunction of the proximal

alimentary tract (delayed gastric emptying).

Causes of malnutrition in NI Child

Increased nutrient losses because of vomiting

Altered energy requirements because of increased muscle tone or involuntary

movements[5]

Inadequate intake as a result of self-feeding impairment and oromotor

dysfunction.[6]

As a result, undernutrition, growth failure, overweight, micronutrient deficiencies, and

osteopenia are nutritional comorbidities that affect the neurologically impaired child. [7]

Monitoring neurologically impaired children for nutritional comorbidities is an integral

part of their care. Early involvement by a multidisciplinary team of physicians, nurses,

dieticians, occupational and speech therapists, psychologists, and social workers is

essential to prevent the adverse outcomes associated with feeding difficulties and poor

nutritional status.[7]

Nutritional comorbidities in NI children

A. Undernutrition, growth failure &overweight

Pathophysiology

1) Inappropriate Dietary Intake

2) Oral Motor Dysfunction &its consequences

Poor dietary intake.[8]

Poor growth.[9]

Decrease in muscle strength which in the respiratory system reduces

the effectiveness of the cough reflex and predisposes to aspiration

pneumonia and in the limbs causes a decreased circulation time,

resulting in the cold, mottled peripheries frequently seen in wasted

cerebral palsied children.[10]

Decrease in cerebral function.[11]

Chronic undernutrition may be associated with increased irritability

and decreased motivation and energy available for non-essential

activities such as play and rehabilitation.[12]

Disturbances in the immune system secondary to malnutrition

predispose these patients to infection, particularly of the lungs and

the urinary tract.

Healing of pressure sores is delayed in children with malnutrition.

3) Increased nutrient losses.

4) Abnormal energy expenditure

.

B. Osteopenia

Severely handicapped children have a high prevalence of osteoporosis and osteoporosis-

related pathologic fractures.[13]

Limited ambulation, increased duration of anticonvulsant therapy, and reduced sun

exposure contribute to the pathogenesis of osteopenia.[14]

C. Micronutrient deficiencies

Because many neurologically impaired children require lower energy intakes, their

micronutrient intakes are correspondingly lower. Replacement therapy reverses these

abnormalities.[14] Iron, selenium, zinc, essential fatty acids, and vitamins C, D, and E

were reported to be deficient in 15% to 50% of these children. [15]

Critical time for identification and screening

Identification &/ or early detection by screening should be done as early as possible to

allow for starting early intervention programs to enhance motor development and

prevent complications.

Suitable times for screening and identification are at birth (1st week of life), 2- 3

months, 9- 12 months, 1.5 – 2 years, 2.5 – 3 years and 5- 6 years as well as whenever a

child is seen for examination or follow up.

Warning signs for neurological impairment

• Abnormal postures, non occurrence and/ or persistence of primitive reflexes..

• Deformities and/ or losses in trunk or extremities.

• Hypertonia or hypotonia.

• Significant delay in motor milestones.[16]

NUTRITIONAL ASSESSMENT

1) Medical History

The medical history includes information about the etiology, duration, and severity of

neurological impairment and its expected course.[17]

2) Nutritional History

An assessment of the child's ability to feed independently and the efficiency of the

feeding process may reveal an obvious reason for poor weight gain.[18]

3) Growth History

Birth weight and length, as well as previous weight, length, and head circumference

measurements, when recorded on NCHS growth charts, may be compared with the

reference population to determine if growth faltering or abnormal weight gain or loss

has occurred.[19]

4) Anthropometric Measurements

Alternative measures such as upper arm length or lower leg length may be obtained to

estimate body length in children who have contractures or scoliosis. Children with

severe disabilities may be weighed while being held by a parent or while seated in a

wheelchair.[20].Height ,weight and weight-for-height of children with

neurodevelopmental disabilities are significantly below standards, and other indicators

of nutritional status, such as triceps skinfold thickness and mid-arm circumference, are

also affected[21]. Sub scapular skinfold is often less affected than triceps skinfold in

malnourished NI children Head circumference should also be obtained..[22]

5) physical Examination

Physical examination focuses on signs of undernutrition, linear stunting, overweight,

and specific nutrient deficiencies. Muscle tone, activity level, and the presence of

athetoid movement are relevant because they influence dietary energy needs.

Contractures and scoliosis are noteworthy for positioning during meals. Abnormal

breath sounds may be suggestive of chronic respiratory problems associated with

aspiration. Abdominal distension in conjunction with palpable masses suggests

constipation. Examination of the skin may reveal the presence of decubitus ulcers.

Pallor, skin rashes, smooth tongue, gingival bleeding, petechiae, bone deformities, or

pedal edema may suggest other micronutrient deficiencies.

A simple categorical feeding scale (mild, moderate, severe) along with a validated

instrument to assess the severity of motor impairment, Gross Motor Functional

Classification (GMFC) scale, were useful in identifying those subjects at greatest

nutritional health risk. [23]

6) Meal Observation

Meal observation may be useful because of the variable feeding patterns in

neurologically impaired children. Meal observation shows that these children may be

offered less, consume less, and spill more food than unaffected children. Mealtime may

not be enjoyable and parent-child interactions may be poor during the meal.

Classification systems based on measures of growth and patterns of food consumption,

such as eating efficiency and oral motor feeding skills, may be helpful to assess the

effectiveness of oral feeding interventions.[24]

7) Diagnostic Studies

Complete blood count and serum ferritin may document iron deficiency or

anemia.

Serum albumin and prealbumin.

Serum electrolytes and blood urea nitrogen reflect hydration status

Bone quantitative ultrasonography (QUS) technique has evolved as a

surrogate measure of bone fragility that reflects structural properties of bone.

It has the great advantage of being mobile, easy to use at the patient’ bed, in

any patient and field condition.

Video fluoroscopic assessment of swallowing function .Assessment of

swallowing function at the end of a meal is informative because feeding

fatigue may lead to aspiration. A swallowing function study may provide

guidance for appropriate food textures and therapeutic feeding techniques.

Gastric emptying scan may detect gastro paresis and, to some extent,

gastroesophageal reflux and aspiration.

Monitoring O2 saturation during a meal may be important because

neurologically impaired children may have hypoxemia while being fed some

food textures.[25]

NUTRITIONAL SUPPORT

1) Nutritional Requirements

Calculating energy needs of neurologically impaired patients

1. Krick method [26]

Kcal/day = (BMR × muscle tone factor × activity factor) + growth factor

BMR (kcal/day) = Body surface area (m2) × standard metabolic rate (kcal/m2/h) × 24 h

Muscle tone factor: 0.9 if decreased, 1.0 if normal, 1.1 if increased

Activity factor: 1.15 if bedridden, 1.2 if dependant, 1.25 if crawling,1.3 if ambulatory

Growth factor: 5 kcal/g of desired weight gain

2. Height-based method [27]

14.7 cal/cm in children without motor dysfunction

13.9 cal/cm in ambulatory patients with motor dysfunction

11.1 cal/cm in nonambulatory patients

3. Resting energy expenditure-based method [28]

1.1 × measured resting energy expenditure

2) Positioning and Oral Therapy

The use of appropriate chairs and adapted utensils during meals. Therapy to improve

oral motor skills may be attempted, especially before 5 years of age.

Oral feeding interventions may be effective in enhancing oral motor function, but are

not effective in promoting feeding efficiency and weight gain.

3)Behavioral Modification

Behavioral therapy initiated by a skilled child psychologist may improve the quantity of

food consumed, the feeding efficiency, and the range of textures accepted, as well as the

quality of feeding interactions between the caretaker and the child.[29]

4) Type of Formula

The choice of enteral formula depends on the child's age, medical condition, energy

requirement, and mode of enteral access)

Standard, age-appropriate, infant or pediatric casein-based formulas are administered

routinely. Whey-based formulas may be better tolerated because they enhance gastric

emptying. [30]

Adult formulas may prevent hypoalbuminemia during periods of catch-up growth; but

care should be taken to avoid iron, vitamin D, calcium, and phosphorus deficiency.[31] If

high energy density (1.5 or 2 kcal/ml) formulas are used, monitoring hydration status

and protein and micronutrient intake is necessary. A fiber-containing formula may

ameliorate constipation,

5) Route of Administration

Oral feedings can be maintained in children with adequate oral motor skills who

have a low risk of aspiration).If aspiration is a risk, enteral tube feedings may be

considered.

Nasogastric or nasojejunal tube feedings are minimally invasive methods that may

be used for short-term nutritional support in undernourished children or in those

with severe acid reflux or aspiration who are awaiting gastrostomy placement.

Nasogastric or nasojejunal tubes are not used long-term because they may be

dislodged easily, may stiffen and cause intestinal perforation, or may result in nasal

congestion, sinusitis, otitis media, and skin and mucosal irritation.

A gastrostomy tube or "button" is recommended for long-term enteral nutrition

because it is more comfortable for the child and is less easily dislodged than a

nasogastric tube. Gastrostomy feedings may promote weight gain, improve the

child's health, and reduce the time spent feeding the child.[32]

Percutaneous endoscopic gastrostomy (PEG) placement, surgical gastrostomy or

laparoscopic gastrostomy placement may be done.

Gastrojejunostomy or jejunostomy tube placement may be required in children who

do not tolerate gastric feeds, have severe gastroesophageal reflux, are at risk for

aspiration

6) Method of Administration

Bolus feedings, continuous infusions of formula, continuous nocturnal infusions of

formula.

7) Approach to Feeding Intolerance

Changing the feeding schedule from bolus to continuous infusion, decreasing the rate of

infusion, concentrating the formula to decrease the volume of feeds, or selecting an

alternative formula may ameliorate symptoms.

CONCLUSION

Nutritional rehabilitation has been associated with improved overall health, improved

peripheral circulation, healing of decubitus ulcers, decreased spasticity, decreased

irritability and improved gastroesophageal reflux in patients with neurodevelopmental

disabilities [ 33]

A multidisciplinary team of pediatric specialists, including physicians, nurses, dietitians,

occupational and speech therapists, psychologists, and social workers, has the

responsibility to monitor the nutritional status of neurologically impaired children and

provide early, efficient nutritional intervention to ensure normal growth, optimal

functional status, and quality of life. The decision to initiate enteral nutrition may be

difficult for the family who perceive this approach as a failure on their part to feed their

child. Involvement of the child and the family in the decision-making process is

important to ensure successful nutritional intervention.

Weight gain, ease of feeding the child, and the stress involved with meals usually

improve with nutritional intervention. The multidisciplinary team is obligated to provide

ongoing support and education to the family throughout the long-term relationship.[34]

References

[1] International Classification of Impairments, Disabilities and Handicaps: WHO;

Geneva, 1980. (ICIDH) model &the updated one: WHO. Towards a common language

for disablements: ICIDH. Geneva: WHO.1997[2] Wells JT, Ment LR. Prevention of intra-ventricular hemorrhage in preterm infants

Early Human Development. 1995; 42: 209-233[3] Valentina M. Reming. Medical Nutrition Therapy for Neurologic Disorders

Krause's food and nutrition therapy. 12th edition. Philadelphia: W. B. Saunders company

2008; 1067-101[4] Relly S, Skuse D. Characteristics and management of feeding problems of young

children with cerebral palsy. Dev Med Child Neurol 1992; 34:379-88[5] Chong SK. Gastrointestinal problems in the handicapped child. Curr Opin Pediatr

2001;13:441-6[6] Sullivan PB, Lambert B, Rose M, Ford-Adams M, Johnson A, Griffiths P. Prevalence

and severity of feeding and nutritional problems in children with neurological

impairment: Oxford Feeding Study. Dev Med Child Neurol 2000;42:674-80.[7] Samson-Fang L, Fung E, Stallings VA, et al. Relationship of nutritional status to

health and societal participation in children with cerebral palsy. J Pediatr 2002;141:637-

43.[8] Dahl M,Gebre –Medlin MG. Feeding &nutritional problems in children with cerebral

palsy and myelomeningocele .Acta Paediatr 1993;82:816-20[9] Troughton KEV, Hill AE .Relation between objectively measured feeding competence

and nutrition in children with cerebral palsy.Dev Med Child Neurol 2001;43:187-90.[10]Patrick J, Boland M, Stoski D & Murray GE (1986) Rapid correction of wasting in

children with cerebral palsy. Developmental Medicine and Child Neurology 28: 734-

739.[11]Stoch MB, Smythe PM, Moodie AD & Bradshaw D (1982) Psychosocial outcome and

CT findings after gross undernourishment during infancy: a 20.year developmental

study. Developmental Medicine and Child Neurology 24: 419-436.[12]Stallings VA, Chamey EB, Davies JC & Cronk CE (1993) Nutrition-related growth

failure of children with quadriplegic cerebral palsy. Developmental Medicine and Child

Neurology 35: 126-138.[13]R. Brunner and L. Doderlein, Pathological fractures in patients with cerebral palsy. J

Pediatr Orthop B 1996;5: 232–238.[14]Henderson RC, Kairalla J, Abbas A, et al. Predicting low bone density in children and

young adults with quadriplegic cerebral palsy. Dev Med Child Neurol 2004;46:416-9.[15] Hals J, Bjerve KS, Nilsen H, et al. Essential fatty acids in the nutrition of severely

neurologically disabled children. Br J Nutr 2000;83:219Y25[16] Marchand V ; Canadian Paediatric Society, Nutrition and Gastroenterology

Committee.Nutrition in neurologically impaired children. Paediatr Child Health

2009;14(6):395-401.[17]. Trier E, Thomas AG. Feeding the disabled child. Nutrition 1998;14:801-5.[18] Reilly S, Skuse D, Poblete X. Prevalence of feeding problems and oral motor

dysfunction in children with cerebral palsy: a community survey. J Pediatr

1996;129:877-82

[19] Blair E, Stanley F. Intrauterine growth and spastic cerebral palsy. Am J Obstet

Gynecol1990;162:229-37.

[20]. Spender QW, Cronk CE, Charney EB, et al. Assessment of linear growth of children

with cerebral palsy: use of alternative measures to height or length. Dev Med Child

Neurol 1989;31:206-14.[21] Krick J, Murphy-Miller P, Zeger S, Wright E. Pattern of growth in children with

cerebral palsy. J Am Diet Assoc 1996;96:680-5[22]Fung EB,Fang LS,Conaway M,Liptak G.Journal of American Dietetic Association

2002;102:361-73

[23] Stallings VA, Cronk CE, Zemel BS, Charney EB. Body composition in children with

spastic quadriplegic cerebral palsy. J Pediatr 1995;126:833-9.)

[24] Gisel EG. Effect of oral sensorimotor treatment on measures of growth and efficiency

of eating in the moderately eating-impaired child with cerebral palsy. Dysphagia

1996;11:48-58.

[25] Rogers BT, Arvedson J, Msall M. Hypoxemia during oral feedings of children with

severe cerebral palsy. Dev Med Child Neurol 1993;35:3-10. [26]Krick method Krick J, Murphy PE, Markham JF, Shapiro BK. A proposed formula for

calculating energy needs of children with cerebral palsy. Dev Med Child Neurol

1992;34:481-7. [27]Culley WJ, Middleton TO. Caloric requirements of mentally retarded children with

and without motor dysfunction. J Pediatr 1969;75:380-4.[28]Azcue MP, Zello GA, Levy LD, Pencharz PB. Energy expenditure and body

composition in children with spastic quadriplegic cerebral palsy. J Pediatr

1996;129:870-6.[29] Linscheid TR. Behavioral treatments for pediatric feeding disorders. Behav Modif

2006;30:6-23

[30] Fried MD, Khoshoo V, Secker DJ, et al. Decrease in gastric emptying time and

episodes of regurgitation in children with spastic quadriplegia fed a whey-based

formula. J Pediatr 1992;120:569-72.

[31] Fried MD, Pencharz PB. Energy and nutrient intakes of children with spastic

quadriplegia. J Pediatr 1991;119:947-9.[32] Rogers B. Feeding method and health outcomes of children with cerebral palsy. J

Pediatr 2004;145:S28-32[33]Lewis D, Khoshoo V, Pencharz PB, Golladay ES. Impact of nutritional rehabilitation

on gastroesophageal reflux in neurologically impaired children. J Pediatr Surg

1994;29:167-9.[34]Marchand V, Motil, Kathleen J. Nutrition support for neurologically impaired

children. Journal of Pediatric Gastroenterology and Nutrition,2006;43:123-135