Overview
Cerebral Palsy (CP), the most common neurological impairment in children1. The incidence worldwide is between 2.0 and 2.5 per 1000 live births2,3. CP is caused by nonprogressive damage or malformation while the brain is developing. The manifestation of CP is heterogenous and can affect an individual’s speech, motor skills, vision, memory, muscle actions, and learning abilities. While advances in supportive care, which have mainly focused on respiratory and orthopedic problems, have extended the life expectancy of people with CP, special attention should be paid to chronic undernutrition or malnutrition frequently experienced by the patients. Indeed, patients with CP most often present gastrointestinal impairment requiring special nutritional support in feeding approaches, including the need for enteral nutrition with formula specifically designed for optimal nutrient absorption and energy supply to fulfill patients’ needs.
Symptoms
Cerebral palsy (CP) is the most common form of neurological impairment in children1 and describes a group of permanent disorders affecting individual’s speech, motor skills, vision, memory, muscle actions and learning abilities4-6 attributed to non-progressive disturbances, malformation or damage, which occurred in the developing foetal or infant brain5,7,8. Premature birth and low birth weight is associated with an increased risk of CP; other causes can be severe jaundice after birth or injuries and illnesses affecting the developing brain later on in infancy8,9. A certain percentage of cases of CP are due to complications at birth (e.g. asphyxia or lack of oxygen). Among the motor disabilities spastic CP is the most prevalent manifestation (70% – 80% cases). Ataxic CP, occurs in about 6% of cases, and dyskinetic CP and mixed types of motor disabilities can also occur1.
CP condition has been classified according to the level of disability. The Gross Motor Functions Classification System – GMFCS10 refers to 5 stages that reflect the level of mobility:
I. Walks without limitations
II. Walks with limitations
III. Walks using a hand-held mobility device
IV. Self-mobility with limitations, may use powered mobility
V. Transported in a manual wheelchair
The nutritional issues often observed in children with CP5,12-14 has a significant impact on their overall health and quality of life11. The level of mobility impact nutritional as well as energy needs and the rate of comorbidities is significantly higher in GMFCS IV and V groups compared with GMFCS I–III15.
Dysphagia occurs in more than 90% of children with CP5. It is responsible for inefficient feeding processes and excessive prolonged feeding time11,16,17. Moreover, oropharyngeal dysphagia increases the risk of aspiration and related pneumonia, especially in children with CP6,18. Gastroesophageal reflux (GER) occurs in up to 75% of cases19 causing regurgitation, vomiting and rumination, which can significantly contribute to nutrient and energy losses11,19. Chronic constipation is a common problem in children with CP6,11,19. Delayed gastric emptying, retching and dumping are further gastrointestinal problems in children with CP.
Intervention
Patients with CP are managed by a multidisciplinary team ideally including a physician, dietitian, nurse, speech therapist, physical therapist, psychologist, and occupational therapist.
The occupational or speech therapist can assist with oral feeding skills, correct positioning of the child, and the use of appropriate chairs and adapted utensils during meals. Periodic reassessment of oral feeding skills is important to determine the potential for oral feeding4.
Behavioural therapy may improve the feeding adequacy and efficiency, as well as the range of textures accepted and the quality of feeding interactions between the caretaker and the child4.
Adequate nutrition is affected by oral problems, such as swallowing difficulties, drooling, and dental abnormalities; postural difficulties caused by pain, spasticity, and hip luxation; and digestive problems, such as GORD, oesophageal motility, vomiting, aerophagia, delayed gastric emptying, and constipation5,21,22. An initial trial of proton pump inhibitors is recommended (PPI)5. Correcting dental abnormalities, treating pain, treating depression, limiting drug side effects, or treating GORD should be tried to help optimize feeding management. For patients unable to achieve their nutritional goals through oral feeding, EN via a PEG tube may be required to supplement or replace the oral method5. If problems such as GORD-related aspiration, refractory vomiting, retching, and bloating occur with PEG feeding, the ESPGHAN guidelines suggest using jejunal feeding5. Nasogastric or nasojejunal tube feedings are reserved for short-term nutritional intervention (less than 4 weeks)4. Beyond, gastrostomy or gastrojejunostomy tube feedings should be considered4,23. Early nutrition intervention in undernourished children is key to improve nutrition and health outcome19,24. Prolonged mealtimes are limiting the child’s participation in school and community activities & less time for rehabilitation, moreover it can cause severe impairment of the child & caregivers’ QoL25. ESPGHAN guidelines5 recommend use of enteral feeding in cases of unsafe or inefficient oral feeding and if total oral feeding time exceeds 3 hours per day.
The type of formula depends of the child’s age, medical condition, energy requirement, and mode of enteral access4. Whey-based formulae are well tolerated as they enhance gastric emptying6. New enteral formula with real food ingredients are more and more recommended in clinical practice as complementary or alternative to standard milk-based formulas26-28. According to experts’ opinion, formula with fiber should be considered for all enterally fed patients to improve gastrointestinal symptoms29.
The ESPGHAN guidelines recommends regular nutritional assessment in order to monitor progress in management of the patient5. Regular growth charts are not adapted for CP patients. In attempt to adapt the growth assessment to CP patients, Brooks and co-workers30 developed CP-specific growth charts stratified by tube feeding status and functional severity (GMFCS). It is important to notice that the growth charts should mainly be viewed as descriptive and not prescriptive.
1. Alliance, C.P. research.cerebralpalsy. Facts about cerebral palsy. 2015 [cited 2017 Nov 17]; Available from: https://www.cerebralpalsy.org.au/what-is-cerebral-palsy/facts-about-cerebral-palsy/.
2. Oskoui, M., et al., An update on the prevalence of cerebral palsy: a systematic review and meta-analysis. Dev Med Child Neurol, 2013. 55(6): p. 509-19.
3. Surman, G., et al., Children with cerebral palsy: severity and trends over time. Paediatr Perinat Epidemiol, 2009. 23(6): p. 513-21.
4. Marchand, V., K.J. Motil, and N.C.o. Nutrition, Nutrition support for neurologically impaired children: a clinical report of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr, 2006. 43(1): p. 123-35.
5. Romano, C., et al., European Society for Paediatric Gastroenterology, Hepatology and Nutrition Guidelines for the Evaluation and Treatment of Gastrointestinal and Nutritional Complications in Children with Neurological Impairment. J Pediatr Gastroenterol Nutr,2017.65(2): p. 242-264.
6. Sullivan, P.B., Gastrointestinal disorders in children with neurodevelopmental disabilities. Dev Disabil Res Rev, 2008. 14(2): p. 128-36.
7. Andrew, M.J., et al., Optimising nutrition to improve growth and reduce neurodisabilities in neonates at risk of neurological impairment, and children with suspected or confirmed cerebral palsy. BMC Pediatr, 2015. 15: p. 22.
8. Healthychildren.org. Cerebral Palsy 2015 Nov 21, 2015 [cited 2017 Dec 12]; Available from:https://www.healthychildren.org/English/health-issues/conditions/developmental-disabilities/Pages/Cerebral Palsy.aspx
9. Reddihough, D.S. and K.J. Collins, The epidemiology and causes of cerebral palsy. Aust J Physiother, 2003. 49(1): p. 7-12.
10. Palisano R et al. Development and reliability of a system to classify gross motor function in children with cerebral palsy. Dev Med Child Neurol. 1997;39(4):214-23.
11. Penagini, F., et al., Dietary Intakes and Nutritional Issues in Neurologically Impaired Children. Nutrients, 2015. 7(11): p. 9400-15.
12. Herrera-Anaya, E., et al., Association between gross motor function and nutritional status in children with cerebral palsy: a cross-sectional study from Colombia. Dev Med Child Neurol, 2016. 58(9): p. 936-41.
13. Delalić, A., Body Mass Index in Cerebral Palsy Patients with Various Motor Severities. Paediatrics Today, 2014. 10(2): p. 95-103.
14. Pascoe, J., et al., Body mass index in ambulatory children with cerebral palsy: A cohort study. J Paediatr Child Health, 2016. 52(4): p. 417-21.
15. Leonard, M., et al., Nutritional status of neurologically impaired children: Impact on comorbidity. Arch Pediatr. 2020 Feb;27(2):95-103.
16. Marchand, V., A practical approach to nutrition care in neurologically impaired children., in Clinical Nutrition Highlights 2012, Nestlé Nutrition Institute
17. Sullivan, P.B., et al., Impact of feeding problems on nutritional intake and growth: Oxford Feeding Study II. Dev Med Child Neurol, 2002. 44(7): p. 461-7.
18. Arvedson, J.C., Feeding children with cerebral palsy and swallowing difficulties. Eur J Clin Nutr, 2013. 67 Suppl 2: p. S9-12.
19. Quitadamo, P., et al., Gastrointestinal and nutritional problems in neurologically impaired children. Eur J Paediatr Neurol, 2016. 20(6): p. 810-815.
20. Content source: National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention (CDC website).
21. Sullivan PB. Nutrition and growth in children with cerebral palsy: setting the scene. Eur J Clin Nutr. 2013;67(Suppl 2):S3-4.
22. Del Giudice E et al. Gastrointestinal manifestations in children with cerebral palsy. Brain Dev. 1999;21(5):307-11.
23. Braegger, C., et al., Practical approach to paediatric enteral nutrition: a comment by the ESPGHAN committee on nutrition. J Pediatr Gastroenterol Nutr, 2010. 51(1): p. 110-22.
24. Dipasquale V. et al. Early Tube Feeding Improves Nutritional Outcomes in Children with Neurological Disabilities: A Retrospective Cohort Study. Nutrients 2023, 15, 2875.
25. Rempel G. The Importance of Good Nutrition in Children with Cerebral Palsy. Phys Med Rehabil Clin N Am 26 (2015) 39–56.
26. Dipasquale V. et al. Tube Feeding in Neurologically Disabled Children: Hot Topics and New Directions. Nutrients 2022, 14, 3831.
27. O’Connor G. et al. Monitor gastrointestinal tolerance in children who have switched to an “enteral formula with food-derived ingredients”: A national, multicenter retrospective chart review (RICIMIX study). Nutr. Clin. Pract. 2022;37:929–934.
28. O’Connor G. et al. The impact of an enteral formula with food‐derived ingredients on dietetic practice at a specialist children's hospital in the UK: Retrospective study. J. Hum. Nutr. Diet. 2024;1–9.
29. Lionetti P. et al. Use of fiber-containing enteral formula in pediatric clinical practice: an expert opinion review. Expert Rev Gastroenterol Hepatol. 2023;17(7):665-675.
30. Brooks J. et al. Low Weight, Morbidity, and Mortality in Children With Cerebral Palsy: New Clinical Growth Charts. PEDIATRICS 2011;128(2):299-307.