26 Years and Counting with CF

By Ella Balasa

The day I was born, the median life expectancy of someone living with cystic fibrosis was 31. Although I haven’t reached that median yet, I feel like I’ve beaten the odds.

During past birthdays, my parents, brother, and I celebrated with cakes filled with raspberry layers and chocolate frosting. The cake always had my name written across the top in big, pink, block letters, and the number of candles matched the number of years lived. I remember my dad’s voice quivering just slightly by the time he sang the last “Happy Birthday” lyric. I think that he sheds an extra tear of joy, metaphorically, for each year I get older. He’s happier than the year before, that I’m one year closer to living the long life he hopes and prays his little girl would have.

I know my parents have always had a seed of heartache that they’ve kept hidden far in the back of their thoughts, watered by the knowledge that they may outlive their youngest daughter. It’s a feeling unknown to me; I can only imagine the fear.

I realize that my disease continues to progress with each passing year, causing a gradual decline in the intensity of accomplishable physical activities. My birthday is somewhat of a grim reminder of what I’ve lost over time. It’s marked by at least one less thing I can do.

Toward the end of my high school years, my brother was my exercise coach. He was always encouraging (sometimes nagging) me to do frog hops down the driveway and sprints from the mailbox to the stop sign on the corner. I also was running about 1 mile, or half of one, in my neighborhood on the days I felt extra motivated. One early summer day, at the end of my loop, about half a block before I reached the stop sign on my corner, I felt the urge to cough. When I got to the corner, I started coughing globs of pure blood while bracing myself against the sign. It was one of the last times I ran. That was the year I turned 18.

When I turned 21, I stopped working out at a gym and instead got a treadmill and weights at home because I had started to require supplemental oxygen while exercising. Without the extra oxygen, my blood oxygenation levels would dip into a range that could cause damage to my heart. My lungs began failing at the job they are required to do: supply oxygen from the air into my blood vessels and to the rest of my organs.

I was using a nasal cannula and carrying around a machine that puffed loudly with every breath, but I couldn’t allow people to see me as abnormal. I still have a hard time being in public with the supplemental oxygen, and although I don’t yet require using it constantly, it’s caused my illness to become visible rather than invisible, as it typically was — and I struggle with that.

Last year, when I was 25, I learned what it feels like to do a 500-pound deadlift. Except I wasn’t in a competition. I was bringing just two bags of groceries into my house from my car less than 50 feet away. During infection exacerbations in my lungs, I am unable to walk at a normal pace, much less carry anything, due to my airways feeling like they are the diameter of a toothpick, and the lack of oxygen my body is receiving. During these times, I feel my body needing the extra oxygen that I sometimes deprive it of because of my unwillingness to show the signs of my disease.

Based on this column thus far, it might seem as though I lament on the difficulties. Honestly, I don’t notice much when my breathing becomes less limited. It’s easier to notice when my breath is restricted and I feel my body producing less, functioning less.

Despite these reflections on my inabilities, I don’t remember my birthdays for all the things I couldn’t do in that year. I do remember everything I could and did do, both on that day and the 364 days in between. For my 10th birthday, I remember having a picnic in the park and running around the playgrounds playing hide-and-seek. For my 21st, I remember going to a local bar, Baja Bean, and getting the coveted sombrero so everyone would know I was celebrating my big day. For my most recent, the 26th, I rode in a small seaplane over the city, then landed into the river.

Birthdays have always been, and always will be, a celebration of my life. It’s the progression over time, despite my best efforts to stay as healthy as I possibly can, that I’ve found to be somewhat discouraging at times.

When I blow out my 27 candles next year, there will certainly be a diminishment in my physical abilities. But I won’t be dwelling on it. I’ll be thinking about all of the new things I did, the places I went, and the people I met.

To read the original article, please click here.

Stream “Up for Air” Documentary this Month for Free!

Jerry Cahill‘s documentary, “Up for Air”, provides viewers with an inside look at his personal fight for survival while living with #CF. During national #DonateLifeMonth (4/1 – 4/30) use the code: BEANORGANDONOR to watch the documentary for FREE! Tap the link to watch: https://vimeo.com/137872395

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Bustle for muscle

by Brennen Reeves

A mom who played college basketball. A dad who entered the army at eighteen. A brother who received a football scholarship after high school. That’s my family.
Me, I like theatre and acting.

I like to workout. I can do most anything, though I tire when I run. Running, not a daunting task for anyone else in my family- nor was the ability to put on weight or the ability to gain muscles. Both were and still remain a struggle of mine today. I understand the weight section- I have CF, my heart beats faster than other hearts, my digestive tract is not on track and diabetes is well, diabetes. Sadly, I cannot understand why I cannot gain muscle. Or turn the muscle that now exists to a lean quality.
I exercise just as much as the next person.

My parents and brother, they’re athletes. Sure, being athletic doesn’t necessarily mean you possess muscle, but my family did. I retained some of those genes. I played baseball, I owned a lacrosse stick, I love to golf. I count that as a sport (so what if I ride in the cart?).
I have coordination.

I received a bilateral lung transplant over 7 years ago. Within that gap, and day by day I become more flexible, stronger, newly energized, with no luck of further muscle growth. The weight is there. Up about thirty-five pounds. But how come there is no significant muscle gain, or the leanness to which I work for? Could it be a diet issue? Because the thirty, sixty, ninety day routine after these 7 years has been ineffective. I still come up short and this lets me down, my physical appearance.

I’m 5’6” and weigh around 120lbs. My body is symmetrically balanced. I have new lungs. There’s the determination- so why this outcome or lack of?
This is not a lecturer post but more a collaborative question- what is your secret? Do you have a secret? Is it a secret?

Sure, I still have CF, yes, my heart still beats faster than other hearts, yes, my digestive tract needs aligning, and well, diabetes still remains diabetes. Just about every other facet since my surgery has changed, emotionally, physically, etc., and my body is still present just with a little more weight with nowhere to go.

I challenge this. Could it be the severed nerves under my breast plate have yet to heal or reconnect, making it harder to attract muscle in this area? The numbness from my shoulders on down through the wrists lose feelings during most points of the day, is that a sign indicating powerless limbs?

“7years. Brennen your body needs more time to heal”
Ok.
—–
Wait,
or could it just be my body?

6 ways to get back into shape after a CF-setback

For people with cystic fibrosis, getting “back” into shape is a common occurrence. Because of the nature of the disease, patients often experience setbacks in both their health and fitness routines. But, exercise is an important and essential part of remaining compliant with treatments and medications in order to live a longer, healthier life with CF.

Continue reading 6 ways to get back into shape after a CF-setback

CYSTIC FIBROSIS WIND SPRINT 68: CIRCUIT TRAINING 3

For people with cystic fibrosis, getting “back” into shape is a common occurrence. Because of the nature of the disease, patients often experience set backs in both their health and fitness routines. But, exercise is an important and essential part of remaining compliant with treatments and medications in order to live a longer, healthier life with CF. Continue reading CYSTIC FIBROSIS WIND SPRINT 68: CIRCUIT TRAINING 3

CYSTIC FIBROSIS WIND SPRINT 67: CIRCUIT TRAINING 2

For people with cystic fibrosis, getting “back” into shape is a common occurrence. Because of the nature of the disease, patients often experience set backs in both their health and fitness routines. But, exercise is an important and essential part of remaining compliant with treatments and medications in order to live a longer, healthier life with CF.  Continue reading CYSTIC FIBROSIS WIND SPRINT 67: CIRCUIT TRAINING 2

CYSTIC FIBROSIS WIND SPRINT 66: CIRCUIT TRAINING 1

For people with cystic fibrosis, getting “back” into shape is a common occurrence. Because of the nature of the disease, patients often experience set backs in both their health and fitness routines. But, exercise is an important and essential part of remaining compliant with treatments and medications in order to live a longer, healthier life with CF. Continue reading CYSTIC FIBROSIS WIND SPRINT 66: CIRCUIT TRAINING 1

8 Tips For Protecting Your Lungs From Unhealthy Air

If you have a chronic lung condition, you’ll need to protect your lungs from being further irritated by unhealthy air. We’ve put together a list of ways to help protect your lungs against unhealthy air using information from the American Lung Association.

Check the daily air pollutant forecasts. 
There are sites on the Internet and cellphone apps where you can find out the levels of air pollution in your local area. Checking these daily can help you plan your week, helping you avoid being outside or limit your time outside when the pollution levels are high.

Try both indoor and outdoor exercise
If pollutions levels are high then avoid exercising outside. Either visit your local gym or exercise at home. Indoor shopping malls are good locations for indoor walking if you can’t go outside. Try not to exercise in places where there is a high level of traffic — traffic fumes can pollute areas up to a third of a mile away.

Reduce your carbon footprint. 
Using less electricity at home is one way to reduce your carbon footprint which helps to create healthy air for everyone. Reducing the number of car trips you make will also help. Travel by bicycle or public transport, or car share instead. Walk short distances instead of jumping in the car.

Don’t burn trash or wood. 
The ash and soot caused by the burning of wood and trash contribute to particle pollution in the atmosphere.

Don’t use gasoline-powered yard equipment. 
Gasoline-powered lawn mowers, trimmers, and leaf blowers all add to the air pollution and may irritate your lungs while carrying out your yard chores.

Encourage others to reduce their carbon footprint. 
Pressurize your local schools to run their buses according to the Clean School Bus Campaign. That means not leaving the engine running while waiting outside buildings and applying for funding for projects helping to reduce emissions.

Ask friends, family, and neighbors to reduce their energy use.

Stay away from smokers.
Don’t allow anyone to smoke in your home or place of work. Try to avoid outdoor places where people smoke cigarettes.

Be an advocate.
Be an advocate for healthier air, supporting local and national campaigns to improve the environment and reduce emissions.

Original article: https://cysticfibrosisnewstoday.com/2017/12/12/8-tips-for-protecting-your-lungs-from-unhealthy-air-2/?utm_source=Cystic+Fibrosis&utm_campaign=a772c5a83f-RSS_THURSDAY_EMAIL_CAMPAIGN&utm_medium=email&utm_term=0_b075749015-a772c5a83f-71418393

An Interview: CF and Exercise

An interview with Pamela Scarborough, conducted by James Ives, MPsych

Please give an overview of the role of exercise in cystic fibrosis (CF)

We know that exercise is beneficial in helping someone to maintain their lung function, stay strong and active and maintain a good quality of life. We also know that exercise can complement ‘airway clearance techniques’ – breathing exercises prescribed by physiotherapists to help clear the lungs of mucus.

As well as benefitting lung function, exercise can help to address other complications of CF such as low bone mineral density, CF-related diabetes, low back pain, postural problems and stress incontinence.

Then, there are many other wonderful benefits such as improved mood and sleep, which is important for someone with a life-limiting condition.

Without exercise, what other methods are used to help CF patients clear the mucous that builds up in their lungs?

As physios, we use a wide range of approaches. The traditional methods that people associate with CF physio are postural drainage, percussion and active cycle breathing techniques − deep breathing exercises to open up the airways of the lungs in order to get behind sputum and get it moving so that it can be cleared with a huff or a cough.

However, we are increasingly using other techniques, for example, oscillatory devices such as a flutter or an acapella; positive expiratory pressure devices such as the Pari PEP; or different breathing exercises such as autogenic drainage. There is also a high-frequency chest wall oscillating vest, which is like a life jacket that vibrates. The aim of all physio techniques is to open up the airways, loosen the mucus, and make it easier to clear.

How important do you think physiotherapy is for people with CF? How prevalent is exercise therapy in CF treatment?

Physio is a cornerstone of CF care and has always been recognized as having a very important role to play. Even from point of diagnosis, when a child may be asymptomatic with their chest, their parents are still taught to engage them in exercise and to get them moving around to make sure they’re maximizing ventilation of the lungs and helping to move any sputum that’s there.

The wide-ranging benefits of exercise on health are continuing to emerge. Throughout my professional career, I have seen people who have come from active families and the fitter they were when they were younger, the better outcome they have later down the line; they’re still functioning at a higher level because they had that training in their younger years.

People are seeing exercise as another way to help control their health, and it is also something that’s more normal to do and that they can do socially with their peers. Someone with CF often has a huge amount of treatment to undertake, a lot of which can be unpleasant, so exercise can be preferable as it is something that can be enjoyable. Every time we see a patient, we ask them about what they are managing to do from an exercise point of view.

What are the main differences between workouts that are specifically designed for CF patients, as opposed to just standard workout routines?

We don’t know exactly how exercise programmes for someone with CF should vary from the normal population; we still need more research to prove this – so what we’re saying is that people with CF should be doing the same amount of cardiovascular exercise and strength and conditioning training as recommended in the national guidelines for the healthy population.

However, because of the factors I mentioned before − that people with CF are very likely to suffer from postural problems, low back pain, stress incontinence and low bone mineral density, etc − physios prescribe exercise programs that make sure we’re addressing these issues before they become a problem, or if they are a problem, that the need is met.

Why is it important to have a personalized exercise routine and what range of exercise routines are available on Pactster?

It is important to have a personalized exercise regime because we are all different; we all have different interests and we all have different needs. There is a lot of pressure on people with CF  to exercise for health benefits, so we must find a way to make it  enjoyable and effective for them. We need to have exercise that is engaging and does not just feel like treatment. It needs to feel like it is going to be fun, as well as meeting the patient’s needs and be easy to integrate into a daily routine.

With a lot of the workouts we have on Pactster, we have really tried to normalize exercise. We know that exercise is medicine, but we want it to be something fun and normal that everybody does. We have used physios and people with CF who are qualified fitness instructors as instructors in the videos; but we film the videos out of the hospital setting, in normal clothes and cover popular exercise disciplines such as yoga, mixed martial arts, pilates, circuits and high-intensity interval training. We are creating more videos to cater for people of different ages and interests, and throughout different stages of the disease.

We are hoping that Pactster will overcome the usual barriers that put people off exercising, like lack of time and money, but that it will also make exercise easier for people who may struggle with low mood, find it hard to get out of the house because they are dependent on oxygen or equipment, have a compromised immune system or who may be too self-conscious to go to the gym. Some people with CF find group classes embarrassing because people may turn and look at them if they start coughing.

Pactster has been designed to overcome these barriers, so that people can gain confidence exercising in their home environments and be reassured that they’re exercising in a safe way, supported by someone who understands their condition.

Zelda and Leah, two CF physios from The Royal Brompton and Harefield NHS Trust

Please give an overview of Pactster and the unique features that you bring to CF physiotherapy?

Pactster offers health-specific exercise videos filmed with specialist instructors alongside community and motivational support. Our exercise videos have been filmed with CF physiotherapists and people with CF who are qualified instructors; and have been approved by the Association of Chartered Physiotherapists in Cystic Fibrosis.

We want people to be inspired by seeing someone with the same condition as them on screen, sharing their knowledge of how to use exercise to take control of their disease.

Currently, in the videos we have on Pactster, the CF instructors are quite highly functioning, but we’re creating new videos that will include people with more advanced disease and people who are pre- or post-transplant, as well as families and kids. Even with more advanced disease, there are still so many fitness role models with CF who who are phenomenal in what they achieve  given the challenges that they face, and who have so much knowledge to share.

Pactster gives the opportunity for people with CF to workout alone or with others in a group setting, this is an important feature as people with CF are unable to meet one another face-to-face due to a risk of cross infection which can significantly impact life expectancy.

These group workouts can be facilitated by a person with CF or by a physio. The good thing about this is that it provides an opportunity for peer support, or if a physio is running the session it may have prevent the need for a hospital visit.

Group workouts are also about motivating and inspiring others as well as providing accountability, enjoyment, and the opportunity for learning. We are currently developing the behaviour change features on Pactster to make it easier to stick to an exercise regime.

Group, online workout has an unlimited number of people who are able to attend

Are there any limitations to the workouts? Do they need specific equipment? I’m guessing these are all open access to all CF patients?

In the UK, our arrangement with the Cystic Fibrosis Trust means that all people with CF, as well as their caregivers and physios can have free access to Pactster. Due to the way CF care is delivered in the UK, it is expected that anyone with CF coming onto the platform would have already been seen by a specialist in a CF centre and that they would have had conversations with their physio about the right amount and type of exercise necessary, as well as how to exercise safely.

It is expected that people come to the platform with some understanding of exercise and then they can participate in what they feel is right for them. Maybe they’ll try something they haven’t tried before because it’s been put right in front of them and it’s easy to do.

Access is free and the equipment that you need to use may vary from one exercise discipline to another. Some will require more hand weights, but you can improvise with a book or a can, for example. There’s also a kettle bell workout on there, if that’s what people are interested in. Generally, most of the exercises require an exercise mat, but not very much equipment after that.

Is it also open access to patients outside of the UK?

There is a monthly subscription of five pounds per month if you’re outside of the UK, but our aim is to try and make it free. As a British person, I believe in the NHS and I believe it is wonderful that healthcare can be free at the point of access.

I would love to make Pactster free for anyone who needs it because we want to reduce as many barriers as possible for someone exercising. Although it’s not much of a financial barrier, making payments is still a barrier for some people. We’ve got people who have signed up from other places around the world, but people are already asking whether there is going to be a similar arrangement in Germany, South Africa, or America and I’d like to find a way to make sure that we can make it free for them.

Will you expand the exercise routines and physiotherapy for other conditions in the future or are you focusing primarily on CF?

I’m predominately a CF physio by background and coincidentally, one of my best friends has CF, so I have a very strong personal attachment to creating this for people with CF and I want to see it working, being a huge success and making a difference.

I do feel that being able to offer specialized exercise videos to someone in their home is incredibly important, as well as being able to provide remote physio support and to connect people who are going through similar conditions. Once we are happy that we have CF working as well as we want it to from a behavior change point of view, as well as videos covering all stages of life from point of diagnosis through to end of life, then yes, we are looking to provide the same service in collaboration with other hospitals or charities for other health conditions.

One example, which we think would be our next step would be working with people with breast cancer as we know exercise is a very important therapeutic intervention for people with breast cancer. Also, at different points of life from diagnosis, pre-mastectomy, post-mastectomy, chemo and radiotherapy, there are lots of barriers to exercise and lots of reasons that exercise needs to be tailored at different points for different needs. The breast cancer population would be another example of a population that we’d like to support using Pactster.

What would you like to see as the future of physiotherapy treatment for CF patients, both in the UK and globally?

I would like physio to be as easily integrated into someone’s life as it can possibly be and every treatment to be as effective as possible. I believe that is about personalizing care and looking at different ways to support people in different settings − at home, in hospital and in the community. I also believe it’s about tailoring our treatment and making it the best we can through creating more of an evidence base and not being afraid to progress with things. Physio is a difficult, laborious task for someone with CF and if we can make it as streamlined, personalized and effective as possible, then that would be awesome.

Ideally, I don’t want people to have to do physio. I want there to be a cure for CF, but so long as that is not the case, then let’s make the therapy the best we possibly can. I’m excited to see where things go from a digital health point of view, because I think there’s potentially lots of different things we can do.

Where can readers find more information?

About Pamela Scarborough

Pamela has been a phyiotherapist in the NHS for 15 years, predominantly working with people with cystic fibrosis. Pamela completed a Master’s looking at yoga for thoracic kyphosis and lower back pain in CF, as well as studying adherence and behaviour change in greater detail. Here, she most enjoyed researching and presenting in those areas, as well as teaching others, sharing new information and approaches on adherence and yoga within the community.

Since then Pamela has been working on Pactster, where she enjoys the creativity of a start-up environment and is excited about the potential of digital health in improving quality and delivery of care. She finds it incredibly satisfying to see people using Pactster and finding it beneficial.

Big Air Jerry: Compliant with Cystic Fibrosis

Be inspired to be compliant. Watch this brief video to learn more.

Big Air Jerry is a video series created by the Boomer Esiason Foundation to educate young people living with cystic fibrosis. Continue reading Big Air Jerry: Compliant with Cystic Fibrosis