Ziggy and the Meaning of Life: Cystic Fibrosis
and Exercise
Stanford CF Education DayMarch 13, 2010
David M. Orenstein, MD
Exercise and CF
EXERCISE TOLERANCEPULMONARY ASPECTSEXERCISE IN THE HEATEXERCISE PROGRAMS
Exercise Tolerance in CF
- Decreased in proportion to pulmonary function-Large individual variation-May be limited by ventilatory factors BEFORE cardiac factors
Cerny, et al.
Exercise in CF: Pulmonary EffectsOxygen levels
Not the limiting factorNo change in mostDecreases in some with FEV1 < 50%
Exercise in CF: Pulmonary EffectsSupplemental Oxygen
Oxygen levels stay normalLower heart rate, minute ventilation(?) no change in max work
Nixon, Orenstein, Curtis, RossARRD 1990;142:807-811
Exercise in CF: Pulmonary Effects
Sputum production:
more than rest; less than chest PT
Exercise in CF: Pulmonary Effects
Sputum production:(after exercise sessions
in our first exercise program in 1979...)
Exercise in CF: Pulmonary Effects
Sputum production:better than nothing;
not as good as chest PT(or,presumably, vest,
etc.)
Exercise in CF: Pulmonary Effects
Chloride transport!
Salt and WaterBalance Inside the Airways
Non-CF Cell CF Cell
Nasal Potential Difference
Knowles et al. NEJM 1981;305:1489
Non-CF
CF
Hebestreit et al.
Exercise in CF:
Exercise in the heat
Late August 1948, 5 of 10 children admitted to Babies Hospital with heat prostration had CF.
Exercise in CF:
Exercise in the heatResponse to single bout-normal heart rate, core body temp-incr. sweat [Cl] and [Na]-decr. serum [Cl] and [Na] (if drinkingfree water)
Exercise in the heat:
90 minutes, 100oF
(10 min sit, 70 min cycle, 10 min sit)
Exercise in the heat:“voluntary dehydration”
children with CF underestimate fluid needs, especially if drinking
water
Exercise in the heat:1. During exercise in the heat,
patients with CF lose more electrolytes than normals, and
replace less fluid than they need2. After exercise, patients with CF replace fluid and salt normally, without special supplements
Exercise in the heatpatients replace fluids normally,
after heat exposure
Exercise in the heat:
RecommendationsPatients with CF should drink electrolyte-enriched fluids during exercise in the heat, perhaps more than they think they need.
Exercise in CF:Exercise Programs
Increase exercise toleranceIncrease aerobic fitnessIncrease airway clearance?Increase quality of lifeIncrease survival
Exercise in CF: Exercise ProgramIncrease exercise tolerance
Increase aerobic fitness
Exercise in CF: Survival
Exercise in CF: Recommendations
SAFEENJOYABLESUSTAINABLE“PLEASANTLY TIRED”AEROBIC(whole family)
Exercise in CF: Recommendations
Exercise with your kid
Set a good exampleExercise in CF: Recommendations