1. Ziggy and the Meaning of Life: Cystic Fibrosis and Exercise Stanford CF Education Day March 13, 2010 David M. Orenstein, MD

2. Exercise and CF EXERCISE TOLERANCE PULMONARY ASPECTS EXERCISE IN THE HEAT EXERCISE PROGRAMS

3. Exercise Tolerance in CF - Decreased in proportion to pulmonary function -Large individual variation -May be limited by ventilatory factors BEFORE cardiac factors

6. Cerny, et al.

7. Exercise in CF: Pulmonary Effects Oxygen levels Not the limiting factor No change in most Decreases in some with FEV1 < 50%

9. Exercise in CF: Pulmonary Effects Supplemental Oxygen Oxygen levels stay normal Lower heart rate, minute ventilation (?) no change in max work

10. Nixon, Orenstein, Curtis, Ross ARRD 1990;142:807-811

11. Exercise in CF: Pulmonary Effects Sputum production: more than rest; less than chest PT

12. Exercise in CF: Pulmonary Effects Sputum production: (after exercise sessions in our first exercise program in 1979...)

14. Exercise in CF: Pulmonary Effects Sputum production: better than nothing; not as good as chest PT (or,presumably, vest, etc.)

15. Exercise in CF: Pulmonary Effects Chloride transport!

17. Nasal Potential Difference Knowles et al. NEJM 1981;305:1489 Non-CF CF

18. Hebestreit et al.

19. Exercise in CF: Exercise in the heat

20. Late August 1948, 5 of 10 children admitted to Babies Hospital with heat prostration had CF.

21. Exercise in CF: Exercise in the heat Response to single bout -normal heart rate, core body temp -incr. sweat [Cl] and [Na] -decr. serum [Cl] and [Na] (if drinking free water)

22. Exercise in the heat: 90 minutes, 100 o F (10 min sit, 70 min cycle, 10 min sit)

23. Exercise in the heat: “voluntary dehydration” children with CF underestimate fluid needs, especially if drinking water

24. Exercise in the heat: 1. During exercise in the heat, patients with CF lose more electrolytes than normals, and replace less fluid than they need 2. After exercise, patients with CF replace fluid and salt normally, without special supplements

25. Exercise in the heat patients replace fluids normally, after heat exposure

26. Exercise in the heat: Recommendations Patients with CF should drink electrolyte-enriched fluids during exercise in the heat, perhaps more than they think they need.

27. Exercise in CF: Exercise Programs Increase exercise tolerance Increase aerobic fitness Increase airway clearance ?Increase quality of life Increase survival

28. Exercise in CF: Exercise Program Increase exercise tolerance Increase aerobic fitness

29. Exercise in CF: Survival

30. Exercise in CF: Recommendations SAFE ENJOYABLE SUSTAINABLE “PLEASANTLY TIRED” AEROBIC (whole family)

31. Exercise in CF: Recommendations Exercise with your kid

32. Set a good example Exercise in CF: Recommendations