Updated: Mar 4, 2020
Are you a Sports Therapist who may treat sports injuries in athletes with Cystic Fibrosis?
As a Sports Therapist, I have worked with a number of athletes who have had additional medical needs despite playing sports to a high level.
You may not feel equipped with enough knowledge in order to fully understand the patient, so it is important that you speak with relevant medical professionals and consult the research to increase your knowledge of specific conditions.
'It is important that you speak with relevant medical professionals and consult the research’
In this blog, I have attempted to provide an appropriate overview for Sports Therapists to understand the basics of the condition, so that you have a greater knowledge when working with athletes who may have this condition.
What is cystic fibrosis (CF)?
CF is a multisystem disease, however the major clinical manifestations are related to the respiratory system which includes sputum retention, decreased exercise capacity and breathlessness.It is the most common life-limiting autosomal recessively inherited disease in the Caucasian population with carrier rates of 1 in 25 and incidence of 1 in 3500.(Bradley et al., 2005; Bradley and Moran, 2011; Wells, 2011)
The pathology and exercise…
Exercise is limited by lung function, peripheral skeletal muscle function, nutritional status, and an inability of the cardiorespiratory system to meet the metabolic demands. The symptoms of CF are similar to deconditioning because of poor cardiovascular function, reduced muscle mass, impaired strength and reduced power. It is currently unclear as to whether inefficient aerobic oxidative metabolism is due to impaired oxygen delivery or intrinsic muscle function (Wells et al., 2011; Wells, 2012).The cystic fibrosis transmembrane conductance regulator (CFTR) is found within skeletal muscle and is distributed in the sarcoplasmic reticulum, sarcotubular network and sarcolemma. This therefore has an effect of the regulation of skeletal muscle contraction at the calcium regulatory channels and/or calcium adenosine triphosphates (ATPases). There are also question marks over potential diaphragm weakness and the normality of anaerobic metabolism (Lamhonwah et al.,2010; Wells et al., 2011; Wells, 2012). Physiologically there has been evidence of lower resting ATP concentrations causing a lower energy reserve, higher end-exercise pH valuesand delayed post-exercise PC recovery.
Common clinical measures of the condition are spirometry (strong predictor of mortality), pulse oximetry and sputum weight/volume; with less common measures of Quality of Life, accelerometry, arterial blood gases, sputum cultures, X-rays, activity diaries and frequency of antibiotic subscription(Bradley et al., 2005; Bradley and Moran, 2011).
The ACSM suggests at least 20-30 minutes at 55-64% maximal heart rate on 3-5 days per week for those with mild and moderate cardiopulmonary impairment (Hebestreit et al., 2010; Pianosi et al., 2005; Wells 2012). Both supervised or home-based programs have been shown to be effective with aerobic exercise prescribed in a steady state or intervals.
Hebestreit et al. (2010) demonstrated that 60 minutes, 3x week, 12 months improved VO2peak and FVC improvement (6%, p<.05); whereas, the interventions by Klijn et al. (2004) of 30-45 minutes, 2x week, 12 weeks indicated significant improvements in aerobic (+5.7%) and anaerobic (+11%) exercise performance an increased quality of life, but no direct improvement in pulmonary function.
Why use exercise prescription?
The improvement in VO2peak has been linked with increased chance of survival. Exercise has been demonstrated to prevent VO2peak reduction which is normally at a rate of -0.17 ml/min/kg per month) (Hebestreit et al., 2010; Pianosi et al., 2005; Wilkens et al., 2010). Exercise can alleviated dyspnea, improve appetite, improved body image, increased sputum clearance, reduce residual volume, delayed osteoporosis, decrease anxiety and depression, increased well being, reduced muscle wasting (Bradley et al., 2005; Bradley and Moran, 2011; Carpenter et al., 1999).
The reported effects of aerobic training are…
VO2peak improved (+8.53 ml/kg/min) on treadmill with intervention (Selvadurai, 2002).
Reported improvements in perceptions of well-being, quality of life, energy levels and chest congestion (Bradley et al., 2005; Bradley and Moran, 2011).
Significant increases in lower limb strength (Bradley et al., 2005; Bradley and Moran, 2011).
FVC improvements of 6% during 12 month intervention (Hebestreit et al., 2010).
No significant difference in VO2peak(+0.05 ml/kg/min) during cycle ergometer intervention (Schneiderman 2000).
No significant differencesin heart rate, FEV1, FVC, number of coughs or dry sputum weight in short-term interventions (<1 month) (Bradley et al., 2005; Bradley and Moran, 2011).
No significant changesin weight or fat-free mass.
The evidence base for anaerobic training has shown Significant reduction in ventilation rate, lactate during cycling (-0.83 mmol/L), desaturation levels, improved FEV, significantly greater weight change and fat-free mass (Bradley et al., 2005; Bradley and Moran, 2011; Moorcroft, 2004).
What considerations do you need to make?
Current status and medications
Risk of infections
The risk of pneumothorax
Lung transplant patients (Wilkenset al., 2010)
The requirement to increase proteins, carbohydrates and fats to support activity
The psychological benefits of exercise (anxiety and depression)
(Bradley et al., 2005; Bradley and Moran, 2011; Wells 2012)
Should we substitute airway clearance with exercise?
There is no significant difference in physiological responses between exercise groups compared to airway clearance or normal activity.
Adverse effects of therapy:
Adverse effects of exercise:
(Bradley et al., 2005; Bradley and Moran, 2011)
So what are the recommendations for practice?
A combination of treadmill aerobic and anaerobic exercise should be used with the aim of increasing VO2peak.
Both home exercise and supervised programs can be utilised.
A minimum of 20 minutes exercise, three times a week should be performed at 55-64% of maximal heart rate.
(Bradley et al., 2005; Bradley and Moran, 2011)
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Conclusions for the efficacy of physical activity and exercise for CF patients is limited by small sample sizes, duration of interventions and follow-ups and incomplete reports.
There is limited evidence that physical training is beneficial for physiological outcomes, however there may still be positive relationships which require further research.
Treadmill aerobic, anaerobic and resistance training in combination may have more positive effects on physiological outcomes measures than other methods of aerobic training.
Bradley, J., Moran, F., Elborn, S. (2005). Evidence for physical therapies (airway clearance and physical training) in cystic fibrosis: An overview of five Cochrane systematric reviews. Respiratory Medicine, 100, 191-201.
Bradley, J., Moran, F. (2011). Physical training for cystic fibrosis (Review). The Cochrane Collaboration.
Carpenter, C., Ross, R., Paganini-Hill, A., Bernstein, L. (1999). Lifetime exercise activity and breast cancer risk among post-menopausal women. British Journal of Cancer, 80(11), 1852-1858.
Hebestreit, H., Kieser, S., Junge, S. (2010). Long-term effects of a partially supervised conditioning programme in cystic fibrosis. European Respiratory Journal, 35 (3), 578-583.
Klijn PH, Oudshoorn A, van der Ent CK, ann der Net J, Helders PJ. (2004). Effects of anaerobic training in children with cystic fibrosis: a randomised controlled study. Chest Journal 125(4),1299–305.
Lamhonwah AM, Bear CE, Huan LJ, Kim Chiaw P, Ackerley CA, Tein I. Cystic fibrosis transmembrane conductance regulator in human muscle: dysfunction causes abnormal metabolic recovery in exercise. Annals of Neurology. 2010; 67(6):802-808.
Moorcroft, A., Dodd, M., Morris, J., Webb, A. (2004). Individualised unsupervised exercise training in adults with cystic fibrosis: a 1 year randomised controlled trial. Thorax, 59, 1074-1080.
Morris, A., Greenwood, J., Ledson, M., Walshaw, M. (2012). Exercise participation, motivators and barriers: A survey of the exercise behaviour of patients attending a regional UK CF centre. Liverpool Heart and Chest Hospital NHS Foundation Trust.
Pianosi, P., LeBlanc, J., Almudevar, A. (2005). Peak oxygen uptake and mortality in children with cystic fibrosis. Thorax Journal. 60 (1), 50-54.
Schneiderman, J., Pollock, S., Corey, M., Wilkes, D., Canny, G., Pedder, L. (2000). A randomised controlled trial of a 3-year home exercise program in cystic fibrosis. Journal of Pediatrics, 136(3),304–10.
Selvadurai, H., Blimkie, C., Meyers, N., Mellis, C., Cooper, P., Van Asperen, P. (2002). Randomized controlled study of in-hospital exercise training programs in children with cystic fibrosis. Pediatric Pulmonology, 33(3), 194–200.
Wells, G., Wilkes, D., Schneiderman, J. (2011) Skeletal muscle metabolism in cystic fibrosis and primary ciliary dyskinesia. Pediatric Research. 69(1), 40-45.
Wells, G. (2012). The role of exercise and physical activity in optimising outcomes among patients with cystic fibrosis. eCysticFibrosis Review. Volume 3 (7).
Wilkens, H., Weingard, B., Lo Mauro, L., Schena, E., Pedottie, A.,Sybrecht, G., Aliverti, A. (2010). Breathing pattern and chest wall volumes during exercise in patients with cystic fibrosis, pulmonary fibrosis and COPD before and after lung transplantation. Thorax Journal, 65,808-814.