There is little controversy regarding the importance of exercise to children and adults with cystic fibrosis. In the twenty years since Nixon et al showed that higher levels of aerobic fitness was associated with a significantly lower risk of dying numerous studies have documented the benefits of exercise, including: improved airway clearance, weight gain, improved cardiorespiratory fitness, increased work capacity, improved bone density, increased exercise tolerance, decreased feeling of breathlessness, and improved body image and quality of life. Even those who are awaiting transplantation are encouraged to exercise as tolerated, since studies have shown that exercise both before and after lung-transplant surgery tends to make cardiopulmonary function more efficient, strengthen respiratory muscles, and ensure good bone density.
But perhaps the most significant reason to incorporate exercise in the CF care regimen was shown in the longest published study to date regarding this issue. A three-year home based program including aerobic exercise three times weekly concluded that “pulmonary function declined more slowly in the exercise group than in the control group”.2 Certainly with the exciting prospect of CFTR correctors and potentiators around the corner, it is extremely important to maintain as much lung function as possible until the day comes when CF becomes a controllable disease.
Undoubtedly, we (adults with CF) should exercise regularly, and parents and clinicians should do what they can to encourage children with CF to introduce this healthy habit as soon as possible. But just as in the case of CFTR-able people, it is no easy task to convince those with lung disease to comply with an exercise prescription. The first obstacle is one of motivation, and the second is the very real issue of time-management. Neither of these are simple problems with simple resolutions; indeed, possible solutions change with the age of the patient and with the severity of the illness. Additionally, the recommendations for mode of exercise are slightly amended as a patient ages.3
The good news about young children is there is never an easier time to get them to move. Furthermore, a caregiver’s power to promote long lasting behaviors at this stage is enormous. Children are wired to be active in short bursts; they play “anaerobically.” They love to sprint or climb or do explosive movements, and then walk around to recover. This is a perfect for children with cystic fibrosis, because research has shown that this type of exercise trains both the anaerobic and aerobic energy systems. This type of play can also be performed for longer periods of time, due to the frequent rest periods. This is significant because the most recent research in exercise physiology shows that it seems to be total volume of daily activity that has the strongest effect on maintaining lung function. In other words, the more daily physical activity that a child is involved in, the slower their lung function will decline. Encourage anything that your child thinks is fun, and if possible, expose them to as many forms of exercise and sport as you can. This not only keeps it interesting for them, it also is beneficial to train their growing bodies in multiple ways. Some favorites are swimming, soccer, basketball, biking, jumping rope, rollerblading, games like tag or capture the flag, jumping on a pogo stick (one of my favorites), or a rebounder (mini-trampoline). The critical thing is consistent daily participation. The recommendation for children with CF is no different from that for healthy children, at least 60 minutes accrued throughout the day. At this age, it is also important that the intensity of the exercise be relatively high, as in a sport such as soccer, where there are frequent bursts of intense running, followed by longer periods of a walking recovery.
Motivation is usually not an issue with this age group, but if it is, delaying favored activities (screen time, reading, shopping) until after playtime works wonders. Some children are extremely motivated by team sports and group activities, while others shy away from them. If a child falls into the latter group, a pedometer may do the trick, or giving them exercise “points” or stickers and offering a great prize for accumulating a target number. It is also very helpful (and healthy for everyone) to establish family rituals that involve exercise. Weekend family hikes or bike rides are not just good for the children, they also establish exercise and time together as family values.
In both girls and boys, aerobic capacity begins to plateau during adolescence. For this reason, it is important to train the aerobic energy system during this stage of development. Aerobic exercise works the lungs, heart, and muscles, thus having a very powerful effect on the oxygen delivery systems in the body. Historically, girls have been at a greater risk for a sudden drop in FEV1 during adolescence, so while exercise is important for both sexes, this is a critical period for girls in terms of health maintenance. Unfortunately, it is also during adolescence that a significant difference in activity level develops between children with CF and healthy children, and this difference is even more marked in girls than boys. Low-level aerobic exercise, such as walking, biking, or leisurely swimming, can be extremely beneficial for building aerobic capacity. In addition, this type of exercise can increase mucus clearance by up to 30%. This type of aerobic activity can be increased substantially by simply having teens walk or ride bikes to school, or to a friend’s house or other planned activity. Fitting as much simple, low-level activity into daily life as possible can slow lung function decline. This is both time-efficient and can be a motivating fact to share with a teenager.
Exercising at a higher intensity is also beneficial, and can be much more tolerable and even enjoyable if done with interval training. This is characterized by two to three minutes of exercise at a moderate intensity, such as jogging quickly or swimming a few laps, followed by a short period of rest for the heart rate and breathing to return back towards baseline, and then repeating this for several intervals. This has the benefit of shortening the exercise session, a very important factor for busy teenagers.
Anaerobic training is also beneficial for adolescents. In sports like soccer or basketball, there are periods of intense exercise (anaerobic) followed by lower level recovery periods (aerobic), so both energy systems are being stressed and therefore, trained. Interestingly, adherence to this form of exercise has been shown to be very high, so kids actually enjoy this type of activity.
Finally, I would be remiss not to mention the myriad benefits of strength training for young men and women with CF. A study recently completed at the Hospital for Sick Children in Toronto shows that upper body strength and anaerobic capacity are significantly related to FEV1.3 Increased muscle mass, which results from weight training, leads to positive health benefits, both physical and psychological. Just as in anaerobic-based sports described above, when lifting weights, the anaerobic system is tasked during the lifting set, and the aerobic system is pushed during the rest phase between sets. On a personal note, it was when I discovered strength training as a young college student that my lifelong “addiction” to exercise took root. Understanding that this was one area where I felt control over my body, where my cystic fibrosis could not hold me back, was by far the strongest motivator to exercise I have ever felt. And I am female, lacking the benefit of testosterone! Imagine how significant it could be for a young man, perhaps smaller than his peers, to discover an activity that he could do to reliably increase strength and muscle mass. As I’m sure we all painfully remember, self-esteem is at its most vulnerable state during adolescence, and when you have a chronic illness, this is magnified. Depression and increased stress are definitely issues to contend with as teens with CF navigate increased time pressures, possible delayed puberty, and increasing health challenges. Exercise is known to increase self-esteem in children with cystic fibrosis, and is also a very effective way to deal with depression and stress.
Just as with younger children, variety improves the interest level and the overall benefits of an exercise program. If a teenager is inclined toward team or individual sports, this is ideal. If not, encouraging as much low-level activity in daily life, introducing interval training, and encouraging resistance training is my recommendation. Whatever the choice of activity, adolescence is the time to emphasize the importance of habitual, daily exercise as a central component to the routine management of their cystic fibrosis. Hopefully, as they notice that they feel and look better when they exercise, your teenager will develop the internal motivation to continue this habit into adulthood.
The most exciting piece of information I’ve seen in researching this topic comes from Toronto, where new interesting and not yet published research that shows that in a seven year follow-up, FEV1, lung function, and habitual physical activity are closely related, and the people with the highest levels of physical activity have a 50 percent slower rate of decline of lung function (emphasis mine).3 For adults with CF, this means one thing: move more! Incorporating physical activity into your daily routine should be as important as your other therapies. Walk as much as you can. Ride a bike. Swim. All of these activities have well-documented positive effects, both CF-specific and non-CF specific. For example, regular exercise both helps with airway clearance and has been shown to be as effective as medication in treating depression and anxiety. For women especially, weight bearing exercise such as walking, jogging, or weight lifting can help stave off osteoporosis. In non-CF subjects, weight lifting improves insulin sensitivity. It isn’t known if this is true in CF, but it certainly could be. Most importantly, regular exercise improves the quality of life and perception of well being, and what can be more important as an adult with CF?
For adults with CF, the exercise recommendation is for “concurrent exercise,” which is medical-speak for cross training. This means it is best to incorporate many different forms of exercise throughout your week. Strength train at least twice a week, work aerobically, at both low and moderate intensity as tolerated, at least three or four days a week, and try to incorporate flexibility and mobility work into your routine on several days. I recommend that people aim for 30 minutes per exercise session, but then also incorporate more movement throughout the day. Obviously, if you have more severe lung disease, you should consult with your doctor as to the need for O2 supplementation or any contraindications to exercise.
There are three important exercise considerations that apply to all age groups. First, if you are sick, don’t workout. While it may seem macho to do so, and healthy people can get away with it, exercise would be an added stressor to an already-taxed system. If you can, continue with low-level activity such as walking, so that you don’t decondition quite as much, and then begin again when you are healthy. Secondly, make sure that with increased physical activity, you or your child with CF are also taking in enough good quality calories to maintain the appropriate body-mass index. Even though exercise correlates strongly with lung function, so does nutritional status, and it would be counterproductive to sacrifice an optimal BMI in your pursuit of fitness. Finally, with increased exercise comes the need for fluid and electrolyte replacement. Especially when exercising in heat or for a long duration, this cannot be overstressed.
Clearly, I am a big fan of exercise. I firmly believe that one of the reasons I have enjoyed such good health as a DDf508 is that I made it a daily habit over 35 years ago. But now, it is not just me and other adults with CF who have seen exercise change their lives, preaching about the need for everyone with CF to add this to their treatment regimen. Research is proving us right, and even giving clues into the mechanisms of the benefits of exercise. As a fifteen-year-old, I didn’t know that my jogging was causing my ENAC channels to be down regulated, thereby increasing airway surface liquid. All I knew was that it made me feel great! I became hooked, and I’m still here, writing about it.
 Nixon, et al, N Engl J Med 1992; 327: 1785-8.
2 Schneiderman-Walker, J Pediatr 2000, 136: 304-10.
3 eCystic Fibrosis Review, March 2012: Vol 3, Number 8.
If you’ve followed the last few posts, you know that I have been focusing on a problem that many people with cystic fibrosis face as they get older. First, let me say that it’s not just us! Even a “normal” person with no lung disease begins to lose muscle mass at a rate of about 1% per year in his or her 40’s. This loss (also called sarcopenia) then speeds up and can reach 2% per year in the 50’s and beyond. Of course, the more you start off with at year 40, the better off you will be at age 60 or 70. The causes of this muscle loss are numerous, and include lack of exercise, poor diet, inflammation, and hormonal changes. To this list, cystic fibrosis patients can add steroid use, poor absorption of nutrients, and numerous hospitalizations leading to even further lack of movement. So what’s a body to do?
Well, this is exactly why I am writing this series. It is important to take muscle gain seriously and in a timely fashion. Don’t wait until you are a skinny fifty year old! However, if you ARE a skinny fifty year old, first of all, congratulations! Second of all, it’s not too late. Get going now! At least you can slow the loss of muscle tissue by using it.
Alright, off the soapbox. On to the content… As you read here and here, rule number one if you want to gain muscle is to pick a good, well designed weight training program and follow it. Rule number two was to increase your caloric intake; specifically, to increase your protein intake. The last rule is to allow your muscles to recover!
There is no faster way to sabotage yourself that to decide that more must be better. More is definitely not better when it comes to lifting weights as a weight gain strategy. It is definitely the way to go if you want to overtrain and get so burned out that you either 1) get sick, or 2) end up hating the gym.
Your muscle tissue needs two things to recover from a resistance workout. It needs food, specifically carbohydrate and protein. It is best to get these in a ratio of 3 or 4 to 1. So for example, I drink a protein shake with 20 gm of whey protein and 60 to 80 gm of carbs in the form of berries, coconut milk and a banana. A simpler option is a big glass of chocolate milk. Ideally, you want to feed this delicacy to your starving muscles as soon as you can after a workout. I shoot for drinking my shake within 30 to 60 minutes after my last set. The reason for this is that the muscle cells are primed to transport both glucose and amino acids immediately following exercise. They are primed because they need these nutrients to recover; glucose to replace the lost glycogen stores, and amino acids to rebuild the contractile proteins that were damaged by the exercise (don’t worry, the damage is a good thing as the muscle is rebuilt stronger and bigger).
The other thing your muscle tissue needs is time off. This is why good programs tell you to rest at least one day between sessions that work the same muscles. You cannot work a muscle every day and expect it to grow. Muscle is built while you rest, not while you train. Therefore, no rest = no building…only tearing down. If you work legs on Monday, rest them on Tuesday. If you must do something, work your upper body instead. If you have a hard, full-body workout on Monday, take Tuesday completely off. It’s ok! Go for a walk. Walking doesn’t count…it is not resistance exercise. It is simply moving…which we are made for and need to do. Finally, if you do not get enough good quality sleep, forget it. Sleep is right up there with food. Sleep is when the cellular trash is taken out, and new proteins are built. It is the time to grow. It is also necessary to get enough sleep so that all of your hard work is not completely overruled by increased stress hormones as a result of too little sleep. Eight hours is a minimum!
Here’s the formula in a nutshell: Workout. Eat to recover. Chill out the next day (but go for a walk!). Sleep a TON. Repeat.
In the last post, I discussed the first of three very important essentials of gaining not just weight, but aesthetically pleasing and healthy weight, AKA muscle. If you missed that post, you can read it here. In part II, I’ll reveal the very basic and obvious second essential ingredient. While obvious, this is the one that I personally have the most trouble with. It’s another case of a habit being very simple, but not necessarily easy.
You have probably guessed Rule Number Two by now. If you are going to work your muscles with a well-designed resistance training program, you also need to feed them! Muscle tissue is largely protein, and to build it up, you need to consume…wait for it…protein! You very likely need to consume more protein than you are now. The target I shoot for is 0.8 to 1.0 grams of protein per pound of weight. But the weight number I will use is the weight that I’m aiming to reach. So if I want to weigh 115 lbs, I would eat 90-110 gms of protein each day.*
But it’s not just protein that is important. You also need to consume calories in the form of fat and carbohydrate. Basically, you need to eat more calories if you want to gain weight. It is not complicated. Sometimes, people get fancy, and calculate their exact resting energy expenditure, then multiply this by a factor that accounts for activity level, then add the exact amount that they expend in their workout, and then add the square root of the distance to the moon divided by pi. Are you dizzy yet?
These methods may work fairly well for the general population, but as you know, people with cystic fibrosis don’t follow the rules. Our resting energy expenditure is way higher than normal, so I have a better method. Write down EXACTLY what you eat for three straight days. These should be days where you are not trying to gain or lose weight, but are simply in maintenance mode. Record not just the type of food, but how much. Then find a good online calorie calculator and add up the calories for each day. Don’t forget to include what you drink as well! Those sodas add up. Now calculate your average daily calorie consumption. If the days are wildly different from each other, you may want to do this for a whole week to get a more accurate average intake.
Got it? Now add 500 calories per day, and this is your new target. So if I calculated that I eat 2000 calories per day, and I want to gain muscle in order to weigh 115 lbs, here is what I need to do. First, my new target is 2500 calories, and of those, 440 are going to be in the form of protein (110gm x 4 calories per gram of protein). So I also need to eat about 2000 calories—I’m rounding here–of fat (9cal/gm) and carbohydrate (4cal/gm). OK, no more math.
The reason I am bad at this, and something that you, too, might struggle with, is that it is HARD to eat this much. If you skip a meal, you get way behind on calorie intake and it can be nearly impossible to catch up. So, eat early and eat often. Never skip breakfast, or lunch, or dinner. Have a snack before bed. Drink calories. Read the “How to lose weight” articles and break every rule.**
It’s also hard to do this on the fly. I’ve figured out that if I plan what I’m going to eat and make sure my strategy includes enough calories beforehand, I am much more likely to be successful.
Finally, the bane of my existence…pancreatic supplementation has to be fine-tuned for this to work. Also, if you have CFRD, you will also have to figure out if and when you might need to adjust insulin dosages. Close monitoring of blood glucose and, in my case, er…..digestion capacity, will help. Bon appétit.
*Ask your CF nutritionist if this amount of protein is safe for you.
** Don’t break the rule about not eating junk food. You are what you eat. So eat real, whole, healthy food.
One of the drawbacks to not digesting nutrients very well is that people with CF are often small. Male or female, we tend to run on the petite side if we are pancreatic insufficient. With earlier diagnosis and better enzyme replacement therapy, this is slowly improving, of course. But for those of us who are already full grown, it can be a constant struggle to keep weight on. Not only do we want to maintain weight, it is often encouraged to have a few extra (I said, “a few”) pounds on board to stay strong and resilient to lung infections.
I don’t know about you, but when I need to gain weight—which is pretty much always—I would rather put on lean muscle tissue than fat. This is not just an aesthetic issue, either. The amount of lean body mass (LBM) you have (this includes everything but fat and water) correlates with disease severity. The less LBM a CF patient has, the more severe their disease tends to be. Additionally, LBM decreases with age, so as we get older it becomes more and more important to try to increase muscle mass.
So, what does it take to gain muscle? Three things, well…maybe four. If you are pancreatic sufficient, it takes doing three things, regularly. If not, it takes four (the fourth being, obviously, sufficient supplementation with pancreatic enzymes). This post is all about thing number one: Resistance Training.
First, you have to lift weights. Muscle tissue does not grow unless you impose a stress to it that it cannot handle. When you do that, the muscle adapts by healing and coming back bigger and stronger. In my opinion you should lift weights at least three times per week if you are serious about gaining muscle mass, and it is everyone’s opinion that you must lift heavy weights (for you). So ladies, forget about the purple Barbie weights. Soup cans will not work for long. Sure, you may have to start there, but within a couple of weeks, you will be strong enough that you will have to put some energy into finding heavier resistance. I realize most people are not training program junkies like me, and that you might not have a clue what to do with those heavy weights. One great resource is The New Rules of Lifting for Life, by Lou Schuler and Alwyn Cosgrove. You can probably find about a million training programs online, but the reason that I like this one is that it is scalable…you get to decide what level you begin at in each of the basic movements, and progress from that point. So, brand new lifters or old pros have something to gain from this book. I am also working on my own CF-specific training program, which will hopefully be available in a couple of months. Don’t wait for me though…get started now.
So, in summary, if you want or need to gain weight, do it in style—by adding muscle. There are three keys to doing this. First, find a weight lifting program that works for you, and commit to it for at least three months. If you do this, in addition to the two remaining steps outlined in the following posts, you will increase your lean body mass, and with it, your chances for a longer and healthier life. When you see the progress that you have made in the three months, I’m betting that you will be hooked for life.