Physical activity recommendations for children with specific chronic health conditions: Juvenile idiopathic arthritis, hemophilia, asthma and cystic fibrosis

Question 1

What is the prevalence of JIA?

Answer 1

1 in 1000 children

Question 2

How many subtypes of JIA are there?

Answer 2

Seven

Question 3

What is the definition of chronic arthritis?

Answer 3

Presence of joint swelling or by two or more of the following:
1. Joint pain
2. Warmth
3. Limited ROM
For at least 6wks

Question 4

What constitutional signs or symptoms are associated with JIA?

Answer 4

1. Anorexia
2. Weight Loss
3. Growth failure
4. Fatigue

Question 5

What extra-articular manifestations are associated with JIA?

Answer 5

1. Ocular
2. Cardiac
3. Pulmonary
4. Hematopoietic involvement

Question 6

What percentage of JIA persists to adulthood?

Answer 6

55%

Question 7

What are the potential benefits of exercise in JIA?

Answer 7

1. Improves aerobic fitness
2. Better muscle strength and function
3. Decreases disease activity
4. Improves self-efficacy
5. Improves energy level
6. Improves quality of life
7. Reduces pain and medication use
8. Optimize bone mineral density
9. Reduced obesity

Question 8

What are the potential risks of exercise in JIA?

Answer 8

1. If cervical spine arthritis increased risk of spinal cord injury
2. If TMJ disease increased risk of dental injury
3. Possible risk of cardiovascular complications with exercise
4. If uveitis at increased risk of eye injury

Question 9

What are the recommendations for exercise in JIA?

Answer 9

1. Can safely participate in sports without disease exacerbation.
2. Should participate in moderate fitness, flexibility and strengthening exercises.
3. Can participate in impact activities and competitive contact sports if their disease is well controlled and they have adequate physical capacity.
4. Should be encouraged to be physically active as tolerated. Those with moderate to severe impairment or actively inflamed joints should limit activities within pain limits.
5. Should gradually return to full activity following a disease flare.
6. Should take individualized training [especially for children with severe joint disease) within a group exercise format for physical/social benefit.
7. Physiotherapists on paediatric rheumatology health care teams should coordinate individual exercise programs.
8. Should have radiographic screening for C1-C2 instability before participation in collision/contact sports if they have neck arthritis. If present, further evaluation is required.
9. Should wear appropriately fitted mouth guards during activities with jaw and dental injury risk (per general population), especially if they have jaw involvement.
10. Should wear appropriate eye protection (per general population) during activities with ocular injury risk

Question 10

What is the inheritance of hemophilia?

Answer 10

X linked recessive

Question 11

What is the incidence of hemophilia?

Answer 11

1 in 5000

Question 12

What are some clinical features of hemophilia?

Answer 12

1. Joint or muscle hemorrhage
2. Easy bruising
3. Synovitis and joint degeneration and arthritis

Question 13

What are potential benefits of exercise in hemophilia?

Answer 13

1. Fewer bleeding episodes
2. Increased joint stability
3. Increased periarticular muscle strength
4. Improve bone mineral density
5. Aerobic exercise may have beneficial effect on coagulation

Question 14

What are potential risks of exercise?

Answer 14

1. Risk of life threatening bleeding episode

2. Hemophilic arthropathy

Question 15

What are the recommendations regarding exercise in children with hemophilia?

Answer 15

1. Should receive appropriate factor prophylaxis to reduce the risk of bleeding in sport.
2. Should undergo vigilant assessment of joint and muscle function before sport selection. If restrictions are required, physicians should counsel children and their families about safe alternatives.
3. Should be carefully assessed before allowing participation in contact or collision sports such as martial arts, hockey or football. Consultation with a sport medicine physician and/or paediatric hematologist may help.
4. Require written strategies (coach, parent or school) before sport participation to prevent or treat bleeds.
5. Should wear protective equipment, undergo physical therapy or take prophylactic factor replacement therapy.
6. Require factor replacement, ice, splinting and rest to manage acute bleeds. Physical activity should be avoided until joint pain or swelling has resolved. Return to sport requires individualized assessment and appropriate rehabilitation.

Question 16

How many Canadian children have asthma?

Answer 16

300 000

Question 17

What percentage of asthmatic patients experience exercise induced bronchospasm?

Answer 17

90%

Question 18

When does bronchoconstriction typically occur in patients with exercise induced bronchospasm?

Answer 18

8-15min after initiation of physical activity

Resolves within 60min

Question 19

What are the potential benefits of exercise in asthma?

Answer 19

1. Decreased bronchial hyper-responsiveness

2. Decrease exercise induced bronchospasm severity

Question 20

What are the potential risks of exercise?

Answer 20

1. May have increased flare triggers

Question 21

What are the recommendations regarding asthma and exercise?

Answer 21

1. Are able to participate in any physical activity if symptoms are well controlled. Swimming is less likely to trigger EIB than running.
2. Should keep an accurate history of symptoms, trigger exposures, treatments and course of recovery from episodes of bronchospasm.
3. Should be diagnosed with EIB by a drop in FEV1 (10% to 15%) after a 6 min to 8 min exercise challenge and a positive response to beta-2 agonist medication. Eucapnic voluntary hyperventilation testing is recommended in athletes.
4. Should use leukotriene inhibitors, inhaled corticosteroids and/or long-acting beta-2 agonists for optimal long-term disease control, and avoid overuse of short-acting beta-2 agonists.
5. Should take inhaled beta-2 agonists 15 min to 30 min before exercise.
6. Should not scuba dive if they have asthma symptoms or abnormal PFTs.
7. Who compete nationally or internationally require a therapeutic use exemption with confirmation of asthma and/or EIB to use certain medications. Consultation with a sport medicine physician is suggested.

Question 22

What is the inheritance of CF?

Answer 22

Autosomal recessive

Question 23

What is the incidence of CF?

Answer 23

1 in 3600 Caucasian live births

Question 24

Where is the mutation in CF?

Answer 24

CF transmembrane conductance regulator complex, a complex chloride channel located in all exocrine tissues

Question 25

What is the gold standard for diagnosis in CF?

Answer 25

Sweat chloride testing

Question 26

What are the potential benefits of exercise in CF?

Answer 26

1. Slower deterioration in lung function
2. Greater survival rates
3. Improved exercise tolerance
4. Enhanced lung mucus clearance
5. Improved strength and endurance of respiratory muscle
6. Improved fat-free mass, weight gain, muscle strength, FEV1

Question 27

What are potential risks of exercise in CF?

Answer 27

1. Desaturation
2. Cardiac dysfunction
3. Hyponatremic dehydration
4. CF-related diabetes may get hypoglycemia
5. Splenic rupture (if splenomegaly)

Question 28

What are recommendations regarding exercise in children with CF?

Answer 28

1. Should be encouraged to participate in any physical activity. Consultation with a sport medicine physician or paediatric respirologist is suggested.
2. Should have individualized exercise programs that include strength training.
3. Require supervised or unsupervised home exercises that elevate heart rate by 70% to 80% of maximum to increase aerobic exercise tolerance.
4. Who cough during exercise should not necessarily stop activity.
5. Those with severe CF should undergo exercise testing to identify maximal heart rate, levels at which oxygen desaturation and ventilation limits occur, exercise-related bronchospasm and response to therapy.
6. Should absolutely avoid scuba diving.
7. Should drink flavoured sodium chloride-containing fluids above thirst levels to prevent hyponatremic dehydration. Those with diabetes mellitus require additional carbohydrates during prolonged exercise.
8. With an enlarged spleen or diseased liver should avoid contact or collision sports.