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