Bone Health of Cerebral Palsy Patients

Transcription

1 Tuesday, 10:00 11:30, A7 Bone Health of Cerebral Palsy Patients Philip Nowicki, MD Objective: Notes: 1. Identify effective methods for the practical application of concepts related to improving the delivery of services for persons with developmental disabilities 2. Identify advances in clinical assessment and management of selected healthcare issues related to persons with developmental disabilities

2 Bone Health In Cerebral Palsy Patients Philip Nowicki, MD FAAP Pediatric Orthopaedic Surgeon Helen DeVos Children s Hospital Assistant Professor of Surgery Michigan State University College of Human Medicine Introduction Definitions Bone metabolism Bone Density Fracture Risk Low BMD Treatment Overview 1

3 Definitions Cerebral Palsy STATIC encephalopathy to the immature developing brain that may be due to anoxic or hypoxic brain injury Diagnosis made OVER TIME Bone Mineral Density (BMD) Measures amount of Ca/Phos in area of bone Helps determine fracture risk Normal Bone Metabolism emedicalppt.blogspot.com 2

4 extras.springer.com Bone Structure/Anatomy content/uploads/2011/09/microscopic structure of compact bone.png?473d22 Abnormal Bone 3

5 Abnormal Bone Metabolism Osteopenia 2 deficiency of bone tissue development Determined by GMFCS level Osteoporosis in Children Defined by International Society of Clinical Densitometry Not based on BMD alone Current Definition: i i BMD Z score < 2.0 for age, gender, body size Significant h/o fracture: 2 upper extremity fractures 2 vertebral compression fractures Single lower extremity fracture Bone Density DXA scan gold standard Comparison Values determined in CP pts Different than in adults Lumbar spine (same) Distal femur Due to associated hip & knee contractures Total body 4

6 DXA Scan Gold Standard for determining BMD Values differ between adult and pediatric pts Adults T scores SD from mean of HEALTHY ref population Peds Z scores SD from mean of AGE and SEX matched controls Every SD drop equals 10 20% in BMD Houlihan CM, Stevenson RD. Bone density in cerebral palsy. Phys Med Rehab Clin N Am 2009;20: Performed differently in CP pts due to joint contractures Lateral Distal Femur used Due to spine/hip implants, accommodate for joint contractures Common area of fx and low BMD Scan pt in comfortable sidelying position 3 areas of distal femur evaluated: metaphysis, metadiaphyseal, cortical bone regions DXA values DXA Scan CP Pts Harcke HT, et al. Lateral femoral scan: an alternative method for assessing bone mineral density in children with cerebral palsy. Pediatr Radiol 1998;28:

7 DXA Scan CP Ptd Harcke HT, et al. Lateral femoral scan: an alternative method for assessing bone mineral density in children with cerebral palsy. Pediatr Radiol 1998;28: Bone Density in CP 86% pts > 9 yoa had osteopenia of distal femur Fx risk DOUBLES w/ every standard deviation BMD Wren et al: CP pts all levels low bone density in tibias Spine deficits greater in more involved children Even higher functioning pts (GMFCS I/II) have lower extremity bone accrual Bone Density in CP Henderson et al (2005) Large bone health study of CP pts Longitudinal study CP quads decreased BMD associated w/ poor nutritional status Osteopenia is manifestation of growth failure Pts w/ lowest BMD: spastic quad, mobility, poor nutrition Non ambulatory significantly lower BMD than ambulators Need long term studies to determine how peds CP pts will fair as adults 6

8 Low Bone Density Factors Poor Nutrition Non ambulatory Post op Immobilization Anti Seizure medications (Dilantin) Poor Nutrition Poor nutrition factors: PO motor feeding difficulties Food/Volume intolerances GERD Lack of sunlight exposure Pharmocological Anti Seizure Meds Phenytoin, Phenobarbitol, Carbamazepine CYP 450 Enzyme induction Increased clearance/catabolism of Vit D into nonactive metabolites 2 hyperparathyroidism and bone turnover Valproic acid 10% reduction BMD May cause renal dysfunction w/ Ca & Phos loss May also directly activate osteoblasts/osteoclasts to increase bone turnover Carbamezapine Induces bone turnover 2 osteoclast activation 7

9 Fracture Risk Factors Osteopenia/Thin bone cortices Stiff joints/contractures Poor balance/falls Seizures Previous Fx Poor nutrition/gastrostomy tube Fracture Risk Prevalence Fxs CP pts 5 to 60% Most common region distal femur 66% in non ambulatory pts Usually minimal trauma involved 2 to 6x fx risk in pts w/ CP and SZS d/o 4% per year fx incidence in moderate to severe CP pts Fracture Morbidity Most fractures occur in lower extremity (i.e., distal femur) CP pts have fx risk than nl children, especially from yoa (Maruyama) Cause pain, decubiti, further bone loss, difficuly w/c positioning/transport High cost for med care: $10,000 per pt, $150 million in U.S. per annum (Apkon) Loss of school time for pt, loss of work time for caregiver 8

10 Screening Lab levels Serum albumin Calcium Phosphorus Alk Phos 25 OH Vit D DXA scan Baseline for all GMFCS IV and V pts upon fracturing (Apkon & Kecskemethy) All pts who sustain lower extremity fracture Osteopenia Tx Physical Therapy Standers Low amplitude mechanical loading (vibration) Optimize nutrition Calcium + Vit D supplementation Bisphosphanates Physical Activity/Therapy Weight bearing and resistance exercise important for development of bone mass Bones adjust strengthen in proportion to amount of stress placed on them Chad et al 8 months of weight bearing physical activity significantly total proximal femur and femoral neck BMC Control patients (no weight bearing) 6% study period Little current evidence evaluating effect of PT on CP pts and BMD 9

11 Anti gravity Theoretically, similar to resistant exercising, especially with dynamic standers Standing Frames Caulton et al 26 non ambulatory CP pts, upright/semi prone standers Avg 48 min/day x 5days/week x9 months Increased BMD of spine, not tibia Katz et al 12 non ambulatory CP pts, upright stander 2 hrs/day, 5 days/week, 6 months BMD femur and calcaneous when compliant w/ standing program Standing Frames Vibration Therapy Low amplitude, high frequency vibration Usually incorporated as vibrating platform in standing frame Ward et al Children w/ limited mobility, RCT on vibrating platform vs sham platform and DXA assessment BMD 6.3% in tibia BMD and 5.5% spine BMD 10

12 Nutrition Optimize nutritional parameters Regular assessment by dietician Consider G tube placement for supplemental nutrition ii Arrowsmith et al G tube supplementation total body protein and muscle mass, NOT BMD but did not over time either Vit D Metabolism Houlihan CM, Stevenson RD. Bone density in cerebral palsy. Phys Med Rehab Clin N Am 2009;20: Vit D/Ca Shaw et al did not find vit D status to coincide w/ magnitude of osteopenia Jekovec Vrhovsec et al 23 CP quad pts, spine BMD, 9 mos supplementation Vit D and Ca Pts w/ supplementation had significant BMD over those w/o Bischof et al Vit D supplementation fx rate in children w/ severe CP and rickets Henderson et al (2002) 9% BMD in mod to severe affected CP pts when given Ca and Vit D supplementation 11

13 Vit D/Ca AAP intake recommendations: 400 IU/d if exclusively and partially breast fed infant 400 IU/D if non breast fed and < 1000 ml/d of Vit D fortified milk for all children > 1 year old 800 mg/day Calcium for children <9 years mg/day Calcium for children 9 18 years Bisphosphanates Bisphosphanates Henderson et al RCT w/ placebo arm w/ IV pamidronate 89% increase in distal femur BMD over 18 months in quadriplegic CP No pt in bisphosphanate group had fx (3 in control did) Grissom et al OI and CP pts, IV pamidronate, DXA scans BMD both groups >4.0, all 3 femur regions improved in BMD 88% reduction fx rate Allington et al Cyclic IV pamidronate 18 non ambulatory CP pts BMD, pain w/ manipulation, no add l fxs 12 months after infusions 12

14 Bisphosphanates Iwasaki et al When treated with both Vit D and risedronate, BMD more than with Vit D alone Paksu et al 36 pts, CP quad Weekly PO alendronate x 12 months After 1 year, serum Ca and Phos, BMD and z score Unal et al PO alendronate more cost effective than IV pamidronate to BMD Treatment Houlihan CM, Stevenson RD. Bone density in cerebral palsy. Phys Med Rehab Clin N Am 2009;20: Conclusions Bone health important in overall care of CP pts More study required regarding outcomes of various bone health modalities in CP pts Screening is important, especially after fracture Multiple tx options for BMD available, combo of all should be utilized to maintain bone health in CP population 13

15 References Gage JR, et al. The Identification and treatment of gait problems in cerebral palsy. Clinics in Developmental Medicine Mac Keith Press Lavenham, Suffolk 2009 Henderson RC, et al. Bone density and metabolism in children and adolescents with moderate to severe cerebral palsy. Pediatrics 2002;110:e5 15. Wren TAL, et al. Bone density and size in ambulatory children with cerebral palsy. Dev Med Child Neurol 2011;53: Henderson RC, et al. Bisphosphanates to treat osteopenia in children with quadripelgic cerebral palsy: a randomized placebo controlled clinical trial. J Pediatrics 2002;141: Henderson RC, et al. Longitudinal changes in bone density in children and adolescents with moderate to severe cerebral palsy. J Pediatrics 2005; 146: Chad KE, et al. The effect of a weight bearing physical activity program on bone mineral content and estimated volumetric density in children with spastic cerebral palsy. J Pediatrics 1999;135: Maruyama K, et al. Bone fracture in physically disabled children attending schools for handicapped children in Japan. Environ Health Prev Med 2010;15: Samaniego EA, Sheth RD. Bone consequences of epilepsy and antiepileptic medications. Sem Ped Neuro 2007;14: Shaw NJ, et al. Osteopenia in cerebral palsy. Arch Dis Child 1994;71: Grissom LE, et al. Bone densitometry in pediatric patients treated with pamidronate. Pediatr Radiol 2005;35: Apkon SD, Kecskemethy HH. Bone health in children with cerebral palsy. J Ped Rehab Med 2008; Harcke HT, et al. Lateral femoral scan: an alternative method for assessing bone mineral density in children with cerebral palsy. Pediatr Radiol 1998;28: References Jekovec Vrhovsek M, et al. Effect of vitamin D and calcium on bone mineral density in children with CP and epilepsy in full time care. Dev Med Child Neuro 2000;42: Ward K, et al. Low magnitude mechanical loading is osteogenic in children with disabling conditions. J Bone Min Res 2004;19: Caulton JM, et al. A randomized controlled trial of standing programme on bone mineral density in non ambulant children with cerebral palsy. Arch Dis Child 2004;89: Katz D, et al. Can using standers increase bone density in non ambulatory children? Abstract as published in the American Academy of Cerebral Palsy and Dev medicine Conference (AACPDM) 2006 Conference Proceedings. Cohen M, et al. Evidence based review of bone strength in children and youth with cerebral palsy. J Child Neurol 2009;24: Bischof F, et al. Pathological long bone fractures in residents with cerebral palsy in a long term care facility in South Africa. Dev Med Child Neurol 2002;44: Iwasaki T, et al. Secondary osteoporosis in long term bedridden patients with cerebral palsy. Pediatr Int 2008;50: Houlihan CM, Stevenson RD. Bone density in cerebral palsy. Phys Med Rehab Clin N Am 2009;20: Coppola G, et al. Bone mineral density in a population of children and adolescents with cerebral palsy and mental retardation with or without epilepsy. Epilepsia 2012;53: Allington N, et al. Cyclic administration of pamidronate to treat osteoporosis in children with cerebral palsy or a neuromuscular disorder: a clinical study. Acta Orthop Belg 2005;71:91 7. Ko CH, et al. Risk factors of long bone fracture in non ambulatory cerebral palsy children. Hong Kong Med J 2006;12: Arrowsmith F, et al. The effect of gastrostomy tube feeding on body protein and bone mineralization in children with quadriplegic cerebral palsy. Dev Med Child Neuro 2010;52: Thank You 14