Improve quality of life in children with Cystic Fibrosis

The Pulmocardio Fisioterapia clinic in Brazil are working with children with Cystic Fibrosis (CF) to help improve their quality of life. They are offering children breathing assessments and breathing training. This is performed under the strict supervision of the therapists and parents. The training will help the children to tackle the respiratory restrictions that CF imposes. Their aim is to help these children live as ‘normal’ a life as possible. To do this they will be prescribing pulmonary rehab for children and adolescents with Cystic Fibrosis.

Cystic Fibrosis and breathing

Pulmocardio Fisioterapia will be focusing on breathing training for the children. And the reason for this is because CF impacts lung function leading to breathlessness. Not only that, it can impact exercise tolerance, increase fatigue and diminish quality of life. Furthermore it adversely affects muscle strength. As a consequence CF affects daily activities. So the aim of this breathing training is to help children maintain an ‘active’ lifestyle.

Improve breathing and improve quality of life

Pulmonary Rehab, offered to patients with CF, consists of exercise training and breathing strategies. This will help to increase exercise tolerance. POWERbreathe Inspiratory Muscle Training (IMT) is beneficial as part of a Pulmonary Rehab programme. This is because POWERbreathe IMT exercises the breathing muscles.  It makes the breathing muscles stronger. And with stronger breathing muscles exercise tolerance increases. In addition, breathing endurance increases. And by increasing breathing strength and endurance, breathing fatigue reduces. As a result, children are able to perform more daily activities. In turn this helps to improve their quality of life.

POWERbreathe IMT is clinically proven

POWERbreathe IMT is a clinically proven, drug-free treatment for people with breathing problems. And it is the only Inspiratory Muscle Training device available on prescription in the UK. It is widely used in research into the benefits of IMT for a variety of medical conditions. And healthcare professionals are prescribing it as a standalone treatment or as part of a rehab programme.

A randomised controlled trial

Benefits Of Combining Inspiratory Muscle With ‘Whole Muscle’ Training In Children With Cystic Fibrosis: A Randomised Controlled Trial.

The conclusion

In conclusion, ‘IMT is an easily applicable intervention that could be included, together with supervised exercise training in the standard care of these (Cystic Fibrosis) patients.’

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New CF Research Could Improve Treatments For Muscle Function

The research paper, Skeletal Muscle Oxidative Capacity in Patients with Cystic Fibrosis, published in Experimental Physiology in March 2015, has concluded that people suffering from Cystic Fibrosis have a lesser ability to uptake and use oxygen in their muscles, which leads to exercise intolerance.

This genetic disease affects the organs in people with Cystic Fibrosis (CF), primarily the lungs and digestive system, which become obstructed by excessive and thick mucus which makes breathing and digesting food difficult.

This research study found that those with CF have shown a limited ability to uptake and use oxygen in their muscles which then also gets more pronounced with age. This lack of oxygen can lead to a limited ability to exercise, and exercise is considered important for achieving a better quality of life, and prolonged life.

The researchers of this study used NIRS (Near Infrared Spectroscopy) as a method to evaluate skeletal muscle oxidative capacity in a group of patients with Cystic Fibrosis. They compared skeletal muscle oxidative capacity within a group of 13 patient aged 7-42 years with CF, and a control group of 16 healthy people aged 7-59 years. The NIRS device was placed on the middle of the thigh and a cuff, much like a blood pressure cuff, was placed on the upper thigh. The pressure cuff was then inflated on the thigh to measure muscle tissue oxygen response over time.

Read Skeletal Muscle Oxidative Capacity in Patients with Cystic Fibrosis >

Discover more about How POWERbreathe help improve exercise tolerance in people with CF >

How POWERbreathe could help improve exercise tolerance in people with CF

We recently came across an article that reported on how patients with Cystic Fibrosis (CF) have less exercise capacity than their peers, even though their lungs may be relatively healthy.

Studies by clinical exercise physiologist Dr. Ryan Harris (Georgia Prevention Institute) of the Medical College of Georgia at Georgia Regents University have shown that it’s actually leg fatigue as opposed to lung capacity that is the limiting factor when it comes to exercise capacity, and suggests that this could be as a result of insufficient levels of blood and oxygen reaching their exercising muscles.

Data from the studies suggested that if people with CF exercised more they could reduce other medical complications, improving their quality of life and longevity. Results from Dr. Harris and colleague Dr. McKie’s studies have indicated that during peak exercise the Cystic Fibrosis subjects consumed 14% less oxygen while their expelled air had higher oxygen levels, “indicating that their muscles were not as efficient at using it.”

This is where POWERbreathe Inspiratory Muscle Training (IMT) could help. In the study ‘Inspiratory muscle training enhances pulmonary O2 uptake kinetics and high-intensity exercise tolerance in humans’ the results indicated that “the enhanced exercise tolerance observed after IMT might be related, at least in part, to improved Vo(2) dynamics, presumably as a consequence of increased blood flow to the exercising limbs.” POWERbreathe was the actual Inspiratory Muscle Training device used in this study.

POWERbreathe IMT/RMT (Respiratory Muscle Training)

For people who experience breathing difficulties, such as those with Cystic Fibrosis, the POWERbreathe Medic, POWERbreathe Classic Level 1 (LR) and POWERbreathe Plus Level 1 (LR) have been selected by our CF customers as the most suitable. They all offer a Light Resistance (LR) and a variable load setting so that each individual is able to find the most appropriate setting for them.

One of our POWERbreathe users with CF has written a blog about how he incorporated his POWERbreathe training into his daily physiotherapy, and how he used it in conjunction with the Shaker Plus, a mucus clearance device. (He used the Shaker Plus for mucus clearance during physio and the POWERbreathe after his inhalers to help suck the particles further down.)

Drug-free mucus clearance devices by POWERbreathe

People with CF have to contend with glue-like mucus which acts like a magnet for bacteria, and it’s this mucus that clogs up organ function, including the function of the lungs. The Shaker Classic, Shaker Deluxe and Shaker Plus are all drug-free solutions for mobilising pulmonary secretions such as mucus, and they work by ‘shaking’ the mucus loose.

The original article that we referred to at the beginning of this blog can be found on Georgia Regents University & Health System News and this is one of the previous studies referred to in the article, ‘Impaired blood vessel function found in cystic fibrosis patients.’

We hope you found this blog helpful, but if there’s anything more you’d like to know then please leave a comment here or on the POWERbreathe Forum, on Facebook or Twitter as we’d be happy to help.

Using Shaker Plus for my Cystic Fibrosis – my 2 weeks’ progress report

After 14 days of using the Shaker Plus for 30-40 mins twice daily I broke my magic 5 liter volume barrier. My max was 4.8. Magic! I was expecting improvements in maybe a month or two. I’m lost for words.

I knew it was only a matter of time as I could sense the improvements were there. I thought maybe in a month or so, but this is outrageous… To break 5 liters for volume first time ever. I’ve only had it less than 2 weeks — 30-40 mins twice daily. Obsessive?


You can read Edward’s story about why he came to use the Shaker Plus and his experience of using it in his original blog, ‘Using the Shaker Plus as physiotherapy for my Cystic Fibrosis’.

If you have an inspirational story that you’d like to share then please leave a comment here or share it on the POWERbreathe Forum, Facebook or Twitter as we’d love to hear from you.

Using the Shaker Plus as physiotherapy for my Cystic Fibrosis

Edward's results

We received this wonderful story from Edward Wandasiewicz, a cystic fibrosis patient who started using the Shaker Plus by POWERbreathe 10 days ago and is already experiencing some very encouraging results. Here’s his story…

“I purchased about 10 days ago a Shaker Plus for physiotherapy for my cystic fibrosis. I also purchased a POWERbreathe Plus for IMT. I thought I’d use one for mucus clearance (the Shaker Plus) and the other (Powerbreathe) for taking 5-10 breaths after my inhalers to suck the already resting particles even further down. Repeating this action I should get clear airways at level 22-23 of my airways and inhaler particles down there too. That’s the theory anyway…. I’ve been using the Shaker Plus for 30-40 mins twice daily. I actually enjoy doing physio now.

I purchased a medical grade spirometer from Carevision, the Micro 1. My cystic fibrosis clinic at Royal Brompton Hospital use Microloop. Here are my numbers – from a slow blow PEF 50% (Peak Expiratory Flow), and a fast blow 99%. The numbers today for a slow blow FEV1 (Forced Expiratory Volume) and FVC (Forced Vital Capacity) are higher than a fast blow FEV1 and FVC when I was 24-25, my peak best. When I was about 25, the fast blow numbers were FEV1 about 2.2 and FVC about 4.7. Today fast blow is about 2.06 / 4.36. I also had an instantaneous pneumothorax at 29 repaired with talc surgery at 29 and I’m 39 now. No known problems post-surgery since. Here’s a photo of my results for slow blow and fast blow.

I’ve been using the Shaker Plus at level 2 and the results are nothing short of jaw dropping. I sent pictures of my results to my cystic fibrosis team. They can’t believe it either. Think I’ve been inhaling fairy dust or got some kalydeco trial drugs from somewhere. Rest assured I have not.

I actually feel clear and strong like I was when I was 25.

How I came to use Shaker Plus and POWERbreathe

About 15 years ago, in 1995 or so, physio therapist Jennifer Pryor did a trial using the cornet / pep mask / acapella / flutter at the Royal Brompton. She also wrote a few books including Physiotherapy for Respiratory and Cardiac Problems but she has retired now. I ended up choosing the cornet as my favourite device and still do to this day. Unfortunately they don’t make the device or the rubber tubing anymore and it’s become fragile due to hot water cleaning.

I was an inpatient at the Royal Brompton Hospital, and gave autogenic drainage (AD) a go. I found it ok: about 50/50, maybe 60/40 in favour of AD. The cornet is like blowing air underwater in a swimming pool; it really reaches deep down.

On the day of discharge, I picked up a common cold and had to stay in for an extra week. If ever there was a time to continue doing physio, this was it. I had a read of Respiratory Muscle Training: Theory and Practice and hence came across the Powerbreathe website. I gave the Powerbreathe Plus beginner model and Shaker Plus a go.

The Shaker Plus is an awesome device. It’s like the Flutter but a little stronger and much more effective. I compared how much I cleared using the two devices i.e. between my favourite cornet and the Shaker Plus, and the Shaker Plus cleared twice the amount in half the time.

I have developed my own modifications when using the device, such as covering one or two of the holes on the cap to increase resistance or stop air being blown out, being blown back into my eyes.

It’s a great device to use lying on your side too, as you can change the mouthpiece angle. It’s really easy to clean too with no awkward parts like the cornet.

After my purchase of the Shaker Plus 10 days ago, I had a go at spirometry. My results for a slow blow (SEF) measuring FEV1 and FVC equivalents are now greater than a fast blow (FEV) measuring FEV1 and FVC from my early/mid 20s. I have an asthmatic component to my Cystic Fibrosis which makes a fast blow more spurious on results.It’s my favourite physio device now, by a few litres…

I’m possibly going to put the Powerbreathe Plus to one side for the time being. I’ve had one physio say it should be ok to use on a light resistance, given my collapsed lung 10 years ago, but I’m awaiting confirmation from my consultant as it was repaired with talc surgery. I’ve been using a few breaths here and there, but not continuous or too strong. But even from minimal use, I do notice a significant stronger inhalation with my puffers.

I actually feel clear and strong like I was when I was 25. Many thanks for all your hard work and research.”


Message from POWERbreathe: We’d like to thank Edward for getting in touch with us and sharing his amazing story and experience, and we hope that his story inspires others with Cystic Fibrosis to ask their physiotherapist about using this expectoration device that helps mobilise pulmonary secretions without the use of drugs.

We’d also like to offer Edward our best wishes for continued good health and improvement.

If you too have an inspirational story that you’d like to share with others then please leave a comment here or share it on the POWERbreathe Forum, Facebook or Twitter as we’d love to hear from you. Why not also read our blog written by Evan Scully who was diagnosed with cystic fibrosis and has written about his first experience of using POWERbreathe.

Benefits of combining inspiratory muscle with ‘whole muscle’ training in children with CF

The British Journal of Sports Medicine has just published details online (16th May) of a randomised trial that looked at assessing the effects of an 8-week combined ‘whole muscle’ (resistance + aerobic) and inspiratory muscle training on lung volume, inspiratory muscle strength and cardiorespiratory fitness, and dynamic muscle strength, body composition and quality of life in children with cystic fibrosis.

The trial concluded that the 8-week training programme induced significant benefits in important health phenotypes, and that inspiratory muscle training was an easily applicable intervention that could be included, along with supervised exercise training, in the standard care of these patients.

Here’s the Original Article, the Abstract for which can be found online at the British Journal of Sports Medicine.

Benefits of combining inspiratory muscle with ‘whole muscle’ training in children with cystic fibrosis: a randomised controlled trial


Elena Santana-Sosa1, Laura Gonzalez-Saiz1, Iris F Groeneveld2, José R Villa-Asensi3, María I Barrio Gómez de Aguero3, Steven J Fleck4, Luis M López-Mojares1, Margarita Pérez1, Alejandro Lucia1,5

Author Affiliations:

1 School of Doctorate Studies and Research, Universidad Europea de Madrid, Spain

2 Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands

3 Department of Pneumology, Hospital ‘Nino Jesus’ of Madrid, Madrid, Spain

4 Department of Health, Exercise Science and Sport Management, University of Wisconsin-Parkside, Kenosha, Wisconsin, USA

5 Instituto de Investigación i+12, Madrid, Spain

Correspondence to:

Dr Alejandro Lucia, Universidad Europea de Madrid, Villaviciosa de Odón, Madrid 28670, Spain;


The purpose of this study (randomised controlled trial) was to assess the effects of an 8-week combined ‘whole muscle’ (resistance+aerobic) and inspiratory muscle training (IMT) on lung volume, inspiratory muscle strength (PImax) and cardiorespiratory fitness (VO2 peak) (primary outcomes), and dynamic muscle strength, body composition and quality of life in paediatric outpatients with CF (cystic fibrosis, secondary outcomes). We also determined the effects of a detraining period.

Participants were randomly allocated with a block on gender to a control (standard therapy) or intervention group (initial n=10 (6 boys) in each group; age 10±1 and 11±1 years). The latter group performed a combined programme (IMT (2 sessions/day) and aerobic+strength exercises (3 days/week, in-hospital)) that was followed by a 4-week detraining period. All participants were evaluated at baseline, post-training and detraining.

Adherence to the training programme averaged 97.5%±1.7%. There was a significant interaction (group×time) effect for PImax, VO2peak and five-repetition maximum strength (leg-press, bench-press, seated-row) (all (p<0.001), and also for %fat (p<0.023) and %fat-free mass (p=0.001), with training exerting a significant beneficial effect only in the intervention group, which was maintained after detraining for PImax and leg-press.

The relatively short-term (8-week) training programme used here induced significant benefits in important health phenotypes of paediatric patients with CF. IMT is an easily applicable intervention that could be included, together with supervised exercise training in the standard care of these patients.

In another study, Improved Pulmonary Function and Exercise Tolerance With Inspiratory Muscle Conditioning in Children With Cystic Fibrosis, published in the Chest Journal (November 1993, Vol 104, No. 5) the effect of inspiratory muscle conditioning in children with cystic fibrosis was also documented, with findings indicating that the experimental group that trained at a high pressure load (> or = 29 cm H2O) showed significant increases in inspiratory muscle strength, vital capacity, total lung capacity, and exercise tolerance in comparison to the control group. The study suggested that IMT may provide some benefit to the young CF population with respect to functional status. “…the results are promising and may potentially be useful as an adjunct to well-established physical therapy regimens in this patient group.”

If you’re already using POWERbreathe to help with symptoms of cystic fibrosis, then please leave a comment here or on the POWERbreathe Forum as we’d love to hear from you. You can also read a POWERbreathe blog from Evan Scully who was diagnosed with CF at the age of just 6-months, about his first experience of using POWERbreathe.