Live Well - tips for living with CF
This page is about living with cystic fibrosis and tips on how to make it easier, look after your health and a general resource for the things you need to know to not only survive, but thrive!
Dosettes and Bar Fridges
I, personally, find I am far more consistent and adhere to my treatment better when it’s so easy that I can’t help but do it. I made some very small changes in my life to do this. I have all my medications made into dosette/medic packs by the local chemist (for a fee) in accordance to the dose. So for the morning dose I’ll have all my vitamins and the am antibiotic. For my lunchtime dose I have all my alternative therapy like Echinacea and garlic tablets. For tea time I have the last dose of antibiotics etc. So simple pop one packet and you take up to 5 pills. The chemist I go to use disposable packs that are perforated so if I'm out for lunch I just rip off the section I need.
This one took some convincing of my parents. I have a small bar fridge right next to my bed which is also where I keep my nebuliser. I can be in bed and half asleep and still do my neb. Excellent on cold mornings and after late nights.
Cystic Fibrosis and Physical Activity: Small Precautions for Great Benefits
Many people with cystic fibrosis wonder to what extent physical exercise is good for them and whether they should limit their fitness training because of their health.
Studies have shown the impact of certain exercise programs and suggest potential benefits vary according to the type and frequency of the exercise and current state of health. They show that people with cystic fibrosis who regularly engage in physical activities find that they have greater physical stamina, stronger respiratory muscles, increased muscle mass and reduced deterioration in lung function.
Some exercises are more effective in increasing muscle mass, while others have a stronger impact on lung function. Certain key factors should be taken into consideration when prescribing physical exercise, in order to maximize the results and minimize the risks. The severity of lung impairment, the lack of fitness at the outset, the need to use oxygen and good nutrition are all undeniably important in developing a fitness program for persons with cystic fibrosis.
Types of workouts
The two most prescribed types of exercise programs are endurance training (aerobic training) and muscle strengthening.
Endurance exercises increase the patients’ ability to exert themselves for a relatively long period (more than 10 minutes). Endurance training is also called aerobic training because it requires the rapid transport of oxygen from the air to the active muscle tissues where it is used to provide energy. As a result, this type of workout can be limited by disease conditions affecting the cardiovascular and respiratory systems.
On the other hand, muscle strengthening exercises are designated as anaerobic workouts because the energy provided comes from other sources and not come directly from oxygen combustion.
The way you train will therefore differ, depending on whether you want to strengthen the muscles or increase endurance. Furthermore the effects of workouts are very specific to the muscle groups being trained and the type of training they receive.
The first training sessions for persons with cardiovascular or respiratory problems should be supervised. This precaution is aimed at determining patients’ tolerance of exertion and teaching them to assess the degree of exertion while exercising. Measurements of heart rate, level of fatigue and hemoglobin oxygen saturation (saturometry) are the most common parameters in objectifying the intensity of exertion and measuring tolerance to exercise.
In the same way, initial supervision of the muscle strengthening exercise program makes it possible to establish training benchmarks (weight, repetitions and rest time) and to ensure that the movements are properly executed.
· Aerobic training
This is endurance training through activities such as swimming, jogging, stationary bicycling or running on a treadmill. Aerobic training should include three stages: a three-to-five minute warm up, a 20-minure workout and a three-to-five minute cool down period. Although training parameters have not been clearly established in the scientific literature, it is generally accepted that exercise heart rate should be around 70% of the predicted maximum value. To determine the predicted maximum heart rate based on the age of persons with cystic fibrosis assessed during the workout, De Jong at al. (1994) used the following formula: MHR (maximum heart rate) = 210 – (0.65 x age). The aerobic or training zone, based on the rating of perceived exertion according to the modified Borg Scale, should be between 3 and 5. Furthermore, oxygen saturation should remain above 90%. Persons with severe lung dysfunction sometimes need oxygen administered through nasal prongs or the Ventimask.
Lastly, it is important to know that aerobic training can cause bronchospasms, especially if the patient is working out in a dry, cold environment. Using a bronchodilator before a workout may help prevent bronchospasms due to exertion.
· Anaerobic training
The main purpose of the muscle strengthening workouts is to strengthen the muscles to make it easier to perform day-to-day activities and tasks. This type of training also increases self-esteem. Strauss et al. (1987) showed that strengthening various muscle groups, such as the quadriceps in the legs and many muscle groups in the arms, through weight resistance training, increases muscle strength and muscle mass in teenagers with cystic fibrosis. These authors recommend training three times a week. They also suggest doing two series of 10 repetitions for each of the strengthening movements. Lastly they recommend flexibility activities to prepare the body for exertion and thus prevent injuries.
Important considerations
To maximize the potential beneficial effects of exercise and to reduce the risks associated with poorly adapted exercise programs, there are certain essential factors that must be taken into consideration.
· Adherence to the program
It is important to exercise regularly and to follow the program. Supervision, especially at the outset of the exercise program, is recommended because it greatly increases long-term adherence. Keeping a daily log of the types of activities performed, the duration of the exertion and the degree of shortness of breath can increase sense of empowerment with regard to the program and improve adherence to it.
· Severity of lung impairment
A workout program must take into account the severity of the patient’s lung impairment and fluctuations in physical fitness. Patients whose forced expired volume in one second (FEV1) exceeds or equals 60% of the predicted maximum value can train without any particular restrictions as long as they stay within the generally accepted limitations for healthy people. However, in patients whose FEV1 is less than 60%, there may be some restrictions with respect to certain activities because of limited lung function, lack of physical fitness and, occasionally, undernutrition. In this case, supervision is required before undertaking any exercise program.
Patients with severe lung impairment (i.e, FEV1 less than 40%) should take special precautions. Ideally, these patients should undergo a stress test beforehand and gradually increase the amount of exercise, and their vital signs (heart rate, blood pressure, respiration rate and oxygen saturation) should be monitored. Patients with underlying heart dysfunction should have an exercise electorocardiogram test.
Patients with cystic fibrosis can undergo exercise training, even if they have severe lung dysfunction, as long as they are provided with additional oxygen and an enriched diet.
· Nutrition
Since patients with cystic fibrosis do not absorb nutriments very well they are often undernourished. Those with severe lung impairment need up to 150% of the predicted nutrient intake for their age. Obviously, undernourished patients have a lower capacity for physical exertion, so hyperalimentation (3,500 to 4,000 kcal/day) may be beneficial or even necessary for persons with cystic fibrosis who follow a regular training program.
Diabetes is another health problem that is frequently associated with cystic fibrosis. Because physical exercise reduces the need for insulin, a professional should measure glucose levels before and after these patients exercise. A salted energy drink can be taken while exercising to avoid dehydration, limit the loss of electrolytes (salt loss through perspiration) and prevent hypoglycemia. It is also recommended that diabetic patients always train at the same time of the day to make it easier to monitor the effects of exercise on their glycemia and to allow their metabolism to get used to it.
Conclusion
An adapted exercise program can help increase muscle strength, improve lung function and increase endurance in persons with cystic fibrosis. These improvements are of crucial importance in cystic fibrosis because they have a prognostic value on patient survival rate. Cystic fibrosis patients have confirmed that they felt a subjective improvement in their physical capacity and quality of life after having followed a training program. Increased endurance, stronger muscles, greater stability in lung function and better drainage of secretions therefore contribute to significant improvement in the quality of life of these persons.
Studies suggest that a fitness program adjusted to the health and preferences of persons with cystic fibrosis should be an integral part of their therapy.
It seems clear that exercise programs should be an integral part of therapy for persons with cystic fibrosis.
