Pediatric Cardiopulmonary Considerations

1. Pediatric Cardiopulmonary Considerations

2. Pediatrics & Cardiopulmonary • ANY neuromuscular or musculoskeletal condition that alters the alignment of the spine and thorax, or diminishes mobility has the potential to reduce cardiopulmonary function • As a PT, you should be able to: • Identify impairments and pathologies that lead to restrictions with mobility/activity • Design appropriate interventions to address these impairments

3. Pediatric diseases with secondary cardiopulmonary dysfunction • Cerebral Palsy • Down’s Syndrome • Muscular Dystrophy • Cystic Fibrosis • Spinal Muscular Atrophy • Spina Bifida • Kyphoscoliosis • Obesity

4. Cerebral Palsy • Acquired static encephalopathy resulting from insult during the perinatal period • Caused by anoxia, hypoperfusion, trauma, vascular events, or congenital anomalies

5. Cerebral Palsy Impairments • Extreme variation • Cognitive deficits (mild-severe) • Atypical Tone • Can have spastic extremities and low trunk tone as well as other variations • Hypotonia: poor head control impacts swallowing & speech • Motor control impairments • Athetosis (uncontrolled extraneous movements due to basal ganglia damage) • Motor weakness (impacts one or more extremities and trunk) • Spastic diplegia, quadriplegia, or hemiplegia

6. Cardiopulmonary Manifestations in Cerebral Palsy • Altered respiration mechanics, hypoventilation, difficulty mobilizing and clearing secretions, increased WOB, and impaired phonation • Can be at risk for aspiration • Scoliosis may develop affecting pulmonary function • Overall low physical activity and fitness levels =  risk for associated health problems

7. Down’s Syndrome • Extra copy of Chromosome 21 • 1/3-1/2 infants diagnosed have congenital heart defects • Impairments • Hypotonia • Delay in motor skill acquisition • Characteristic face & shorter stature • B hearing loss • Cognitive impairment

8. Cardiopulmonary Manifestations in Down’s Syndrome • Low trunk tone reduces airway clearance • Poor ability to clear secretions  pulmonary infections and postural abnormalities that may lead to restrictive d/s in older children • AA instability and subluxation can result in medulla compression as child ages  respiratory compromise • Increased incidence of leukemia and HTN

9. Muscular Dystrophy • X-linked recessive progressive myopathy • Many variations including: Duchene’s and Becker’s • Duchene’s is most common and typically detected in childhood • Progressive degeneration of skeletal muscles, resulting in m. wasting and weakness • Waddling gait, increased lumbar lordosis, pseudohypertrophy of calf mm. (pf and inversion contractures), + Gower’s maneuver • Loss of functional ambulation occurs typically by end of adolescence

11. Duchenne’s Respiratory m. weakness = major issue by teens Most patients die of respiratory failure 2 pulmonary infection Age =  cardiac involvement Cardiomyopathy, arrhythmias, ECG abnormalities, and sudden death Becker’s Cardiac involvement occurs more frequently and with earlier onset (usually by age 30) Near-normal life expectancy with demise 2 degree of heart failure Cardiopulmonary Manifestations in Muscular Dystrophy

12. Cystic Fibrosis • Inherited autosomal recessive disease • Affects chloride ion transmission across epithelial cellsexcessive Na+ reabsorption • Dehydration of surface fluids, abnormally salty sweat, and thick mucous that clogs tubes, tubules and ducts (respiratory and intestinal) • Bronchiolesmore central airways • Mucous secreting glands hypertrophy  secretions reduce the lumen of airways

13. Cystic Fibrosis Characteristics • Short stature with body weight • Voracious appetites 2°  resting metabolic rate with intestinal malabsorption • Osteopenia / premature osteoporosis • Barrel Chest with flattened diaphragm leads to fullness feeling and food intake • Usually infected by staph or pseudomonas

14. Cardiopulmonary Manifestations in CF • Pulmonary Hypertension • Right-sided Heart Failure (cor pulmonale) • PTX • Hemoptysis • In 08’ CFF predicted survival to be 37.4 yoa • 90% die of respiratory failure • PT Treatment: Chest PT, Active lifestyle (mucus secretions), The CF “vest”

15. General Pediatric Considerations • Include the parents  participation and carryover • Be flexible, creative, and observational • Observational assessments can often be more beneficial • Very important for the child to gain trust • Prioritize assessment; Do “scarier” components at end • Children don’t really care about PT, but they DO love to play games (blow bubbles, whistles, horns, playing the harmonica, crying, laughing, and yelling) • HAVE FUN!!! • Motivating young children is easier than teenagers • Teenagers can understand the importance of treatment

16. General Pediatric Considerations • Children are in physiological flexion when born; as they develop, the child elongates and normal diaphragmatic alignment occurs • May not occur in children with developmental delay • If postural drainage is necessary, perform with small child or infant on lap • Avoid trendelenberg with children w/ reflux • Use only one hand for percussion and vibration for small children • Coordinate with school PT if necessary

17. Clinical Implications-PT • Monitor CV responses to activity/stimulation • Minimize scoliosis (often present with neurologically impaired) • Prevent airway obstruction during sleep • Perform airway clearance techniques • May benefit from cough techniques, esp after activity • Manual stretching and mobilization of the ribcage (esp with m.weakness, paralysis, or spasticity) • Diaphragmatic & postural strengthening exercises • Interventions to decrease spasticity • Regular physical activity (unless C/I) • Assistive technology prescription as indicated • Yoga & Pilates  postural strength and mobility, breath control, and concentration

18. Impaired Ventilation & Gas Exchange • Positioning •  post pelvic tilt enhances diaphragm use • Prone -  ventilation of posterior lung segments • Functional Mobility Training • PNF • Arms overhead elicits greater upper chest expansion • Incentive Spirometry • Diaphragmatic Strengthening Exercises • Thoracic Mobilization Techniques

19. Impaired Clearance of Secretions • Patience and creativity are a MUST! • Especially when the position/technique is challenging for the child • Techniques: • Bronchial/postural drainage, percussion, vibration, cough stimulation, and airway suctioning • Same 12 positions are used for infants/children • Bronchial tree is fully developed before birth • Infant/child typically placed on caregiver’s lap • Once older, can use adult positions or large stuffed animals or floor pillows for comfort/fun • Thin pillow may also increase comfort for the infant

20. Bronchial Drainage in Infants • Never leave in trendelenberg unsupervised • VS should be monitored very closely by monitors with alarms turned on • Must auscultate breath sounds after positioning • Suctioning should be done as needed • Avoid trendelenberg for 1 hour after eating to avoid aspiration of regurgitated food • Any change in position should be done slowly to minimize stress on CV system • Infants with severe CV instability or suspected intracranial bleeding should not be placed in trendelenberg • There is ALOT of additional training required prior to treating neonates in any hospital setting!!!

22. “Fun” Facts • Exposure to CV risk factors in childhood  risk for atherosclerosis • HTN in children 5% (rising) • Weight loss  CV risk • <1/3 children engage in >30 mins mod activity/day • Dramatic  DM-II in children • >2000 children (under 18) became regular smokers each day (2002) • High levels of physical activity (ages 9-24)  HDL and  triglycerides and insulin levels

23. References • Watchie, J. Cardiovascular and Pulmonary Physical Therapy: A Clinical Manual. 2nd edition. pgs. 375-390. 2010. • http://www.childrenshospital.org/az/Site596/mainpageS596P0.html • Deturk W., Cahalin L. Cardiovascular and Pulmonary Physical Therapy. An Evidence-based Approach. pgs. 465-467. 2004. • http://www.cff.org/AboutCF/Faqs/