Bone Health, Daily Physical Activity, and Exercise Tolerance in Patients With Cystic Fibrosis

doi:10.1378/chest.10-1508

Chest

Volume 140, Issue 2, August 2011, Pages 475-481

https://doi.org/10.1378/chest.10-1508 Get rights and content

Background

Daily physical activity (PA) may be an excellent tool for the maintenance of bone health in patients with cystic fibrosis (CF). The aim of this study was to analyze the possible association between physical capacity and activity and bone mineral density (BMD) in young adults with CF. A secondary goal was to evaluate vertebral fractures in this population.

Methods

A cross-sectional study was conducted in 50 patients with CF who were clinically stable and aged > 16 years but not lung transplant recipients. PA was quantified with a portable motion monitor (BodyMedia Fit Armband). Cardiopulmonary exercise and 6-min walk tests were used to assess exercise capacity. BMD was obtained from dual-energy x-ray absorptiometry of the lumbar column, hip, and whole body. To analyze vertebral fractures and deformity, we performed the Genant and Cobb methods.

Results

Daily PA time at low (3-4.8 metabolic equivalent tasks [METs]) and moderate (4.8-7.2 METs) intensity, respectively, was correlated with Z score (BMD) of the lumbar column (r = 0.36, P < .01 and r = 0.59, P < .001), the neck of femur (r = 0.51, P < .001 and r = 0.72, P < .001), and the total hip (r = 0.54, P < .001 and r = 0.74, P < .001). PA, BMI, age, and sex were predictors of BMD. Vertebral fractures correlated with kyphosis (r = 0.42, P = .02), but not with BMD. Patients who were mildly and severely affected differed in vertebral fracture rate and kyphosis prevalence (P = .002 and P = .013, respectively).

Conclusions

The most active patients with better exercise capacity had higher BMD. Those with more affected pulmonary function had a greater prevalence of vertebral fractures and dorsal kyphosis.

Section snippets

Population and Study Design

The participants were patients of the Specialized Unit of Cystic Fibrosis of the Virgen del Rocío Hospital (Sevilla, Spain). This study was approved by the ethical committee of the hospital, and written informed consent was obtained from all participants. An observational analytic and cross-sectional study was designed. All patients aged ≥ 16 years of age with CF (two positive sweat tests, compatible clinical and confirmation genetic) were invited to participate and included, provided that they

Results

Fifty patients (46% men) were included in the study. Table 1, Table 2 shows the patient characteristics.

Discussion

When monitoring daily PA, it was noted that the most active patients had the greatest bone mass. The results of the cardiopulmonary exercise test and 6-min walk test confirmed these results. However, no association between BMD and vertebral fracture was found. To our knowledge, no previous study has analyzed the level of PA of patients with CF in real time and the possible relationship of PA to BMD.

Previously, other authors compared the level of PA with BMD using questionnaires and observed a

Acknowledgments

Author contributions: Dr Tejero García had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

Dr Tejero García: contributed to the conception and design of the study; data acquisition and analysis; interpretation of information; and preparation, revision, review, and approval of the final manuscript.

Dr Giráldez Sánchez: contributed to the conception and design of the study; data analysis; interpretation of the

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Physical activity and exercise training in cystic fibrosis [1]
2022, Paediatric Respiratory Reviews
Nasal high-flow therapy as an adjunct to exercise in patients with cystic fibrosis: A pilot feasibility trial
2021, Journal of Cystic Fibrosis
Exercise tolerance in people with CF and advanced lung disease is often reduced. While supplemental oxygen can improve oxygenation, it does not affect dyspnoea, fatigue or comfort. Nasal high-flow therapy (NHFT), thanks to its pathophysiological mechanisms, could improve exercise tolerance, saturation and dyspnoea. This study explores the feasibility of conducting a clinical trial of using NHFT in patients with CF during exercise.
A pilot, open-label, randomized crossover trial was performed, enroling 23 participants with CF and severe lung disease. Participants completed two treadmill walking test (TWT) with and without NHFT at 24–48 h interval. Primary outcome was trial feasibility, and exploratory outcomes were TWT distance (TWTD), SpO₂, transcutaneous CO₂, dyspnoea and comfort.
Recruitment rate was 2.4 subjects/month with 1.3:1 screening-to-randomization ratio. No adverse events caused by NHFT were observed. Tolerability was good and data completion rate was 100%. Twenty subjects (91%) were included in the exploratory study. Mean difference in TWTD on NHFT was 19 m (95% CI [4.8 - 33.1]). S_pO₂ was similar, but respiratory rate and mean tcCO₂ were lower on NHFT (mean difference = -3.9 breaths/min 95% CI [-5.9 - -1.9] and -0.22 kPa 95% CI [-0.4 – 0.04]). NHFT reduced exercise-induced dyspnoea and discomfort.
Trials using NHFT in patients with CF during exercise are feasible. NHFT appears to improve walking distance, control respiratory rate, CO₂, dyspnoea and improve comfort. A larger trial with a longer intervention is feasible and warranted to confirm the impact of NHFT in training programmes for patients with CF.
Cystic fibrosis bone disease: Pathophysiology, assessment and prognostic implications
2019, Journal of Cystic Fibrosis
Citation Excerpt :
Much as in those without CF, decreased physical activity in patients with CF can be associated with lower bone density. A study by Tejero Garcia S et al. examined physical activity in 50 young adult patients with CF using cardiopulmonary exercise and 6-min walk tests and found significant positive correlation between activity level and bone mineral density at the spine and femoral neck [34]. Regular weight bearing exercise can improve bone accrual in healthy children and potentially in children with CF [35].
Cystic fibrosis bone disease (CFBD) is a common long-term complication of cystic fibrosis (CF) that can lead to increased fractures and significant morbidity and mortality in this patient population. CFBD pathophysiology remains poorly understood and is likely to be multifactorial. There are limited studies evaluating diagnostic tools and tests to guide therapeutic decisions and monitoring of CFBD. This review will present and discuss the current evidence.
Physical activity and associations with clinical outcome measures in adults with cystic fibrosis; a systematic review
2019, Journal of Cystic Fibrosis
Physical activity (PA) is important in the management of Cystic Fibrosis (CF) and is associated with a number of beneficial effects. PA assessment is not commonplace or consistent in clinical practice, therefore understanding of PA in adults with CF remains limited. The purpose of this review was to evaluate PA levels in this population and compare PA to global recommendations and non-CF peers.
The Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines were utilised to inform the review process. Original research was identified and screened against inclusion/exclusion criteria. Quality was assessed, data extracted and a narrative synthesis undertaken to describe the findings.
Adults with CF did not achieve recommended PA guidelines and step count targets in 5/8 studies where assessment was possible. No significant differences in PA were found between CF and non-CF peers in 3/5 studies. Associations between PA and improved lung function were inconsistent with 4/9 studies finding a positive association. Evidence for an association between PA and higher exercise capacity was stronger with all 4 studies reviewed reporting a positive association. Quality ratings were low across all studies.
PA in adults with CF is largely comparable to their non-CF peers, despite being insufficiently active to achieve PA recommendations. Assessment tools used and outcomes reported are variable, many of which do not provide sufficient information to assess relevant components of PA. There is a requirement for high quality studies designed specifically to explore PA in adults with CF, ideally employing standardised PA assessment methods.
Exercise and Lung Function in Child Health and Disease
2019, Kendig's Disorders of the Respiratory Tract in Children
Exercise in children is not merely play; rather, it is a critical environmental and physiological perturbation that has a profound effect on growth and development in health and disease. In this chapter, we review fundamental knowledge focused on how the growing child adapts to the physiological stress of exercise with a particular focus on respiratory disease. Cardiopulmonary exercise testing (CPET) remains the most widely used approach for precise measurement of respiratory and cardiovascular fitness in children and adults. Essential diagnostic uses of CPET in asthma, cystic fibrosis, and lung disease of prematurity are reviewed. Children at high altitude, particularly those with chronic lung disease, are at risk for exercise impairment, and the mechanisms responsible for these challenges are identified. We present new information regarding an increasingly diagnosed syndrome that limits exercise performance in adolescents, exercise-induced laryngeal obstruction. Finally, we set the stage for the next phase of discovery in exercise research in children, namely linking exercise to genomic, epigenetic, proteomic, and metabolomic technologies that will, hopefully, advance our understanding of exercise as a biomarker and therapeutic adjunct in child health.
The treatment of the pulmonary and extrapulmonary manifestations of cystic fibrosis
2017, Presse Medicale
Citation Excerpt :
Regular exercise is considered to be an important therapeutic intervention in patients with CF as low aerobic fitness (low VO2 peak %predicted) has been associated with increased mortality [13,33,34]. While the evidence for physical activity in CF is limited [35], beneficial effects of exercise on patients with CF include: a higher exercise capacity [36], improved body image perception [37], bone mineral density [38] and sputum clearance [39,40], decreased rate of decline of FEV1 [41], and less days in hospital [36]. Currently, the optimal prescription (including mode, duration, frequency, intensity, type of exercise or oxygen prescription) has not been established although it is recommended that patients participate in a regular exercise program prescribed by experienced CF physiotherapists that includes 30 minutes of aerobic and strength training 3–5 times per week [13].
Cystic fibrosis (CF) is a complex multisystem disease with considerable between patient variability in its manifestations and severity. In the past several decades, the range of treatments and the evidence to support their use for the pulmonary and extrapulmonary manifestations of CF have increased dramatically, contributing to the improved median survival of patients. As therapy for CF has evolved, new challenges including treatment adherence, medication intolerance and allergy, medical complications and coping with the burden of disease in the context of having a family and managing employment have arisen. While the majority of current therapy focuses primarily on improving symptoms, new therapies (CFTR modulators) target the underlying genetic defect.

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Funding/Support: This study was developed at the University Hospital Virgen del Rocío de Sevilla, Spain, and supported by a grant (9,000 Euros) from the Association of Pulmonologists of Southern Spain (Neumosur).

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (http://www.chestpubs.org/site/misc/reprints.xhtml).

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Chest

Original ResearchCystic FibrosisBone Health, Daily Physical Activity, and Exercise Tolerance in Patients With Cystic Fibrosis

Background

Methods

Results

Conclusions

Section snippets

Population and Study Design

Results

Discussion

Acknowledgments

Am J Clin Nutr

Bone

J Cyst Fibros

Bone

Am J Med

Chest

J Pediatr

J Cyst Fibros

J Clin Densitom

J Cyst Fibros

Am J Clin Nutr

J Clin Densitom

Chest

Adult cystic fibrosis

JAMA

Osteoporosis and osteopenia in adults and adolescents with cystic fibrosis: prevalence and associated factors

Thorax

Original Research
Cystic Fibrosis
Bone Health, Daily Physical Activity, and Exercise Tolerance in Patients With Cystic Fibrosis