User:Jaclynsweeny

Background

 * Cystic Fibrosis (CF) is a recessive genetic condition
 * CF sufferers cannot produce the salt-transport protein.
 * It is associated with the production of thick, sticky mucus build up that affects numerous organs (particularly the lungs, pancreas and sweat glands).
 * All Australian babies are screened at birth with a blood test and a sweat test.
 * There is currently no cure for CF.
 * Affects 1 in 2500 babies born in Australia and New Zealand.

Explanation of the issue
CF patients have decreased lung function, reduced peak aerobic capacity, poor pulmonary function, mucus build-up (which obstructs the lungs) and progressive airways obstruction which decreases the vital capacity of their lungs (limits the increase in tidal volume that occurs during exercise). 2.	Ortega,Montemayor, Sanchez,Cabello and Castillo, 1994, 'Role of cardiopulmonary exercise testing and the criteria used to determine disability in patients with severe chronic obstructive pulmonary disease.’, Care Med., Vol 150, No. 3, 747-751  Affect on CF suffer:
 * Reduced ability to do day to day tasks
 * Reduced overall health/well being
 * Increase Risk of Depression
 * Reduced quality of life

Limitations to Exercise

 * Chronic cough
 * Poor thermoregulation
 * Bronchospasms
 * Undernourishment
 * Periods of ill health (detraining)
 * Peak heart rate reduced (due to ventilatory limitations in severe cases)

Long-Slow Distance Training to Improve VO2 Max
Testing CF patients (estimated VO₂ Max values):
 * Shuttle Walking Test
 * 20m Run Test

Physiological Adaptations:
 * Stroke volume & Cardiac output increases
 * Oxygen uptake increases
 * Haemoglobin levels increase (more O₂ to working muscles)
 * Lower Resting Heart Rate (heart doesn’t have to beat as often)
 * Muscle size increases

Increasing a CF patients VO₂ Max equates to better overall progress for CF patients

Recommendations
Exercise Prescription: Long Slow Distance Training(Treadmill/Bike/Rowers/swimming), 3x per week for 30-40 minutes at am intensity of 60 -80% VO₂ Max

During Exercise: Monitor Core Body Temperature, RPE & Heart Rate, use of a bronchodilator and ensure appropriate dietry intake before, during and after exercise.

Research
In-hospital Exercise Training Programs in Children with Cystic Fibrosis Aim was to compare aerobic and resistance training in children with CF admitted to hospital. Participated in an incremental treadmill test and a lower limb strength test using a dynamometer. There where 3 groups (all with similar disease severity): Aerobic Training, Resistance Training and Standard Chest Physiotherapy. It was concluded that children with aerobic training had better peak aerobic capacity, activity levels, and quality of life.

Cardio Respiratory Adaptations to Exercise in Cystic Fibrosis Patients Aim was to assess differences in adaptations to exercise in patients with varying severities of pulmonary disease. Patients were grouped based of their lung function tests results. Participated in a progressive, incremental cycle Ergometer work test. The work load increased every 2 min until the subject could not continue. Results indicated that CF patients breathed significantly larger volumes than normal subjects. Patients with moderate or severe disease ventilated more per unit of oxygen consumption than did patients with normal pulmonary functions

Information sources

 * Australian Cystic Fibrosis Website http://www.cysticfibrosis.org.au
 * Adult CF Team Westmead, Sydney Ph: (02) 98456112

Reference

 * 1) Heywood, 1998, Advance Fitness Assessment and Exercise Prescription, 3rd Ed, Leeds: Human Kinetics, p. 48
 * 2) Ortega,1994,Care Med., Vol 150, No. 3, 747-751
 * 3) Selvadurai, 2002, Pediatric Pulmonology, Vo 33, Issue 3, pp. 194 -200
 * 4) Cerny, 1982, PubMed, 126(2):217-20.
 * 5) Moorcroft, 1998, Manchester Adult Cystic Fibrosis Unit, Wythenshawe Hospital, Vol 20, no 6/7, 247-253.
 * 6) Godfrey,Pulmonary function and responses to exercise in Cystic Fibrosis. Arch Dis Child 1971; 46:144-51
 * 7) Seiler, 2009, Sport Science, Perspectives/Training and Performance, Vol 13, pp 32-53
 * 8) Doorn, 2009, Department of  Exercise and Sport Science, Disability and Rehabilitation,  vol 31, issue 1, pp 41-49
 * 9) NADEL,1985, Physiological adaptations to aerobic training, American Scientist. Vol. 73, pp. 334-343