Paediatrics 2

Medical Problems in Children

• Specific precautions should be listed for children participating in sports with certain conditions
• The benefits of exercise for those conditions should be talked about

Asthma

• Swimming can improve symptoms, but cold and chlorine might worsen them

Recommendations: Children with Asthma

• Can participate in any physical activity if symptoms are well controlled
• Swimming is less likely to trigger Exercise-Induced Bronchoconstriction (EIB) versus running
• Keep a history of symptoms, triggers, treatments, and recovery from bronchospasm
• EIB is diagnosed by a drop in FEV1(10-15%) after exercising 6-8 minutes and a positive response to beta-2 agonist medication
• Eucapnic voluntary hyperventilation testing is recommended for athletes
• Use leukotriene inhibitors/inhaled corticosteroids/long-acting beta-2 agonists for optimal disease control, and don't overuse short-acting beta-2 agonists
• Inhaled beta-2 agonists are taken 15-30 minutes before exercising
• Do not scuba dive if asthma symptoms or abnormal Pulmonary Function Tests (PFTs) are present
• Those competing nationally/internationally need a therapeutic use exemption with asthma/EIB confirmation to use certain medications
• Consultation with a sports medicine physician is suggested

Diabetes & Insulin Resistance

• Diabetes effects exercise and climate on Insulin and CHO requirement
• Constant monitoring is important to avoid hypoglycemia; especially late at night after exercising
• There's no evidence that aerobic exercise enhances glycemic control and associates with decreased insulin needs
• Strength training can reduce HbA1c, suggesting improved glycemic control
• High-Intensity Interval Training (HIIT) can lower the risk of hypoglycemia in both children/adults

Risks & Considerations - Type 1 Diabetes Mellitus (T1DM)

• Moderate-intensity aerobic exercise lowers Blood Glucose Level (BGL) and increases the risk of early and late hypoglycemia
• Short bouts of HIIT can increase blood glucose

Physical Activity Recommendations

• Moderate intensity aerobic + >2x sessions resistance per week
• Regular BGL measurements are suggested when starting new exercise
  • Additional CHO is needed before exercise if BGL is <5.6mmol/L
  • CHO replacement is required for sessions >30min
  • Replacement ratio depends on intensity (0.5-1g/kg/hr)
• Insulin pumps can help
• Avoid extreme heat
• Avoid sports or situations where hypoglycemia can be fatal (diving, motorsports, rock climbing)
• Therapeutic Use Exemption (TUE) for insulin

Epilepsy

• Caution is recommended around water and bicycles, because they can cause injury or death from seizures

Cystic Fibrosis

• Attention is recommended to NaCl (Sodium Chloride) replacement and pulmonary hypertension should avoid exercise
• It is a common life-limiting autosomal recessive disorder with a life expectancy of ~40 years

Rationale for Exercise

• Increased aerobic capacity which is an independent survival predictor
• Reduced decline in lung function
• Strength training improves muscle strength and respiratory muscle endurance
• Regular exercise clears secretions and improves respiratory endurance

Risks & Considerations

• Risk of dehydration and heat illness is common with a Sodium Chloride concentration in sweat 3-5x normal levels
• Replacement electrolytes are important
• Sports performance is impaired by the athlete's muscle weakness which can reduce lung/cardiac function & oxidative capacity
• Uncontrolled coughing may lead to brief oxygen desaturation periods
• CF-diabetes leads to metabolic complications

Physical Activity Recommendations

• Different exercise modalities are designed for different aspects of the disease, resulting in enhanced therapeutic benefits
• Perform combined aerobic and resistance training
• Assess lung function, cardiac function, and baseline aerobic capacity
• Children with CF should be encouraged to participate in any activity
• A sport medicine physician or respirologist should be consulted
• Individualized exercise programs that include strength training are recommended
• Supervised or unsupervised home exercises that elevate the heart rate by 70-80% of max are required, in order to increase aerobic exercise tolerance
• Individuals who cough during exercise should not necessarily stop the activity
• Those with severe CF should undergo exercise testing to identify max heart rate, oxygen desaturation levels, ventilation limits, exercise-related bronchospasm, and response to therapy
• Absolutely avoid scuba diving
• Consume flavored sodium chloride-containing fluids above thirst levels to prevent hyponatremic dehydration
• Those with diabetes mellitus may require additional carbohydrates during prolonged exercise
• Avoid contact or collision sports if the individual has an enlarged spleen or diseased liver

Paediatric Rheumatic Disease

• Further information not included

Progressive Neuromuscular Disease

• Further information not included

Congenital Heart Disease

• Be aware of Sudden Cardiac Death (SCD) in some conditions
• Maximal effort should be avoided as well as the Valsalva maneuver

Hemophilia

• Hemophilia is an X-linked recessive bleeding disorder, caused by a deficiency in factor VIII
• Greatly prolonged coagulation time with lifelong tendency to hemorrhage into muscles, joints, and organs
• Avoid contact sports
• Early treatment with Factor VIII concentrate and cryoprecipitate are recommended
• Prophylaxis is considered for severe hemophilia cases
• Improved muscle strength and bone density are benefits, specifically around joints
• Decreased cardiovascular risk
• Increased quality of life
• Tendency for obesity is associated with accelerated arthropathy

Risks & Considerations

• Joint bleeds are the most common
  • bleeds can encompass soft tissue, and be Intracranial/Abdominal
• Recurrent joint bleeds turn into haemophilic arthropathy
• Transiently increased risk of bleeding with moderate physical activity, at 3-4x greater risk than a resting state

Physical Activity Recommendations

• Regular physical activity is encouraged
• Contact/moderate risk injury requires prophylactic clotting factor before exercise
  • For every 1% rise in clotting factor level a 2% decrease in bleeding risk

Recommendations: Children with Hemophilia

• Receive appropriate factor prophylaxis to reduce the risk of bleeding
• Undergo assessment of joint and muscle function before sport selection
• Physician counseling is recommended for safe alternatives if there are restrictions
• Assessment is recommended before participating in contact or collision sports
• Strategy written by coach, parent, or school for prevention of bleeds is required
• Protective equipment should be used
• Physical therapy or prophylactic factor replacement therapy are suggested
• Factor replacement, ice, splinting, and rest is required in order to manage acute bleeds
• Physical activity should be avoided until joint pain or swelling has fully resolved
• Individualized assessment and rehabilitation are recommended
• A consultation with a sports medicine physician, pediatrician, and/or hematologist can help

Obesity - Epidemiology

• There is a marked increase in the prevalence of overweight and obese children; 20-30% in the western world
• Parental obesity means >2x risk of obesity in both obese and non-obese children if they are less than 10 years old
• Physical activity progressively decreases after 11 years of age, additionally noted that boys are more active than girls at every age

Causes

• Unstructured play time leads to inactive pursuits, like technology
• Car use leads to less walking or cycling
• Safety concerns can inhibit children from exercising, also, instant meals and fast food, and advertising for unhealthy food can all contribute
• There are international standards for diagnosing obesity, BMI is used for various ages which will classify individuals as overweight or obese

Complications

• Dyslipidemia and insulin resistance
• A doubled risk of death from Ischemic Heart Disease (IHD)
• Non-Insulin Dependent Diabetes Mellitus (NIDDM), fatty liver disease, sleep apnea
• Musculoskeletal concerns leading to Slipped Upper Femoral Epiphysis (SUFE)
• Psychological disturbances with low self-esteem

Strategies To Improve Exercise

• Involve family in exercise interventions; parental support for PA increases participation rates in girls from 30-70%
• Habitual daily exercise such as walking to/from school or the bus

Hypermobility, Marfan Syndrome, Ehlers-Danlos Syndrome, Osteogenesis Imperfecta

Ehlers-Danlos

• Results in skin and joint manifestations
• Soft pearly skin and increased elasticity may also occur
• Hypermobility increases the risk of subluxation/dislocations
• Pain and fragile vessels are also risks

Osteogenesis Imperfecta

• Characterized by generalized osteopenia and brittle bones with a high tendency to fracture
• Autosomal dominant
• Can cause kyphoscoliosis, irregular or incomplete ossification, ocular changes and excessive skin scarring
• Joint laxity and dislocations, valvular incompetence, large vessel fragility
• Most sports are dangerous
• Light activity like water walking may be permissible

Joint Hypermobility Syndromes

• A Beighton score of >4/9 should raise suspicion
• Joint Hypermobility Syndrome shows characteristics of ≥4 painful joints for >3/12 of the year
• Fatigue and poor exercise tolerance can also occur
• Rates of overweight/obesity increase
• Exercise should focus on joint stability, muscle strength, and proprioception

Risks and Considerations

• Elevated risk of joint dislocation/injuries
• Knee injuries occur 5x more often in contact sports

Mitochondrial Myopathies (MM)

• Caused by mutations of nuclear or mitochondrial DNA (mtDNA) which may stem from absence or decreased activity of proteins of the mitochondrial respiratory chain leads to decreased oxidative capacity leading to decreased ATP production and decreased peripheral O2 extraction capacity
• Causes muscle weakness and severe exercise intolerance

Physical Activity Recommendations

Address deconditioning

• Elicit training induced mitochondrial proliferation
• Produce mitochondrial gene shifting

Strength Training

• May decrease the load of mutant mtDNA in mature muscle fibers by increasing the proportion of satellite cells
• Muscle regeneration may then occur via overload + injury acting as a gene-shifting influence

Risks & Considerations

• mutated Mitochondrial DNA can be a concern which goes along with aerobic training that could potentially increase mutant load
• Small studies have shown no evidence of this however as of yet

Physical Activity Recommendations

• Little evidence to guide prescriptions
• Combination of aerobic and resistance training 3x per week for 60 minutes may improve function, strength, symptoms, and aerobic capacity

McArdle Disease

• Glycogen Storage Disease (GSD Type V)
• Autosomal recessive disorder caused by deficiency of myophosphorylase leading to a failure of muscle to break down glycogen to create glucose early in exercise causing exercise intolerance and muscle pain
• Usually presents within first 20 years
• The muscle pain and weakness becomes more pronounced

Common Symptoms

• Myoglobinuria
• Rhabdomyolysis
• Increased creatine kinase at rest and increase after exercise
• Low 2nd VO2 peak
• Give a "second wind" with decreased pain, HR/RPE at 6-10 minutes resulting from extramuscular fuel sources (glucose from hepatic glycogenolysis and FFA oxidation)
• Oral glucose/pre exercise can help prolong exercise

Risks and Considerations

• Rhabdo and ARF

Physical Activity Recommendations

• Light to moderate aerobic exercises with 5 times per week with at least 30-60 minutes
• Avoid repetitive eccentric muscle contractions/plyometrics
• Avoid high intensity bursts
• Give glucose load 5 minutes prior
• 10 to 15 minutes of low intensity warm up with a second wind effect"

Juvenile Idiopathic Arthritis

• Most common form of arthritis if under 16 years
• Inflammatory arthritis of unknown origin that can affect one or multiple joints Different subtypes that include oligoarthritis which is the most common (50%) that affects under 5 joints and can begin at less than 5 years
• decreased levels of physical activity can be noted leading to decreased aerobic fitness Muscle weakness
• Decreased bone mass around affected joints

Risks & Considerations

• High intensity training should be well tolerated and shouldn't exacerbate the joint
• Compliance can be higher with low intensity

Physical Activity Recommendations

• No strong evidence has been presented
• Combine anaerobic, aerobic, and resistance training
• Should participate in moderate fitness, and flexibility, and strengthening exercises
• Safe participation in sports without disease exacerbation may be permissible
• Can participate in impact activities and contact sports while having diseases well controlled
• Patients should be encouraged to be physically active as tolerated for those that are having flare ups they may need to modify or limit activity
• Individual should take the individualised training to help facilitate and help provide
• Physical therapists on pediatric rheumatology care should help coordinate the physical activities.
• Radiological screening should be done for C1-C2 instability specifically, if there is to be any collision sports it should be assessed at neck
• Appropriate mouth guards with jaw and dental injury should be used in activities

Infectious Disease

• Further information not included

Congenital Deformities

-Poland Anomaly with an absence or under development of pectoralis major:

-Sprengel Deformity can cause the shoulder blade to sit too high this can often restrict movement in the shoulder and can be abnormally connected to the spine

Childhood Malignancy

• Acute Lymphoblastic Leukemia accounts for 30-40%
• Higher survival rates with long disability
• About 1 in 600 adults are cancer patients

Exercise

• Can help to improve fatigue and chemo

Risks and Considerations

• Should prevent any symptoms to avoid
• Exposure to fatigue
• Nausea
• Anaemia
• Decrease bond intensity the need to consider the various options or how to modify them
• Line ports should also be considered

Physical Activity

• Perform intensity aerobic exercise that is frequent
• Split the time into shorter sessions
• Performing resistance and the load must be adjusted
• Inpatient stay is a time to limit decline