A progressive genetic disease primarily affecting the lungs and digestive system, cystic fibrosis (CF) is a condition that has impacted millions across the world. It’s also a condition known for producing large amounts of thick, sticky mucus, which can become serious problems for airways and breathing in general.
If left unaddressed, this problematic mucus may cause chronic respiratory infections, worsening one’s ability to absorb nutrients and live a normal life.
Although CF is currently without a cure, that doesn’t mean that it can’t be treated over time with numerous medications and lifestyle adjustments.
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Whether you are dealing with severe consequences of the disease or simply want to get ahead of it before symptoms get worse, there are five things you can do every day to help.
Let’s cover them…
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1. Airway Clearance Therapy
As the name suggests, airway clearance therapy is about removing obstructions from your airways. It is actually more than one therapy and includes multiple treatments that when used together can help prevent mucus buildup and long-term issues.
There are several techniques involved in airway clearance therapy, including:
- Chest Physiotherapy (CPT): A procedure whereby a healthcare professional gently taps on your chest and back to facilitate the breaking up of mucus.
- Positive Expiratory Pressure (PEP) Devices: Handheld devices that build resistance during exhalation, which then helps to loosen problematic mucus and increase airflow.
- High-Frequency Chest Wall Oscillation (HFCWO): A vest-like device that produces fast bursts of air to your chest to dislodge the mucus and allow excretion.
- Inhaled Medications: A more common treatment option, medicines such as nebulizers or inhalers go right to your lungs and reduce inflammation to facilitate easier breathing.
The reason for airway clearance therapy is two-fold. Firstly, it allows you to significantly improve your lung function. In doing this, you reduce hospital visits and serious outcomes. It also allows you to understand the factors that impact your breathing quality. If you notice excess mucus buildup, you are now armed with the techniques to reduce that issue.
To incorporate this therapy, you really only need up to half an hour a day of practice in each session. Another way to treat this is through working with your healthcare team, which can help you determine the perfect combo of therapies for your condition.
The best time to incorporate these techniques is in the morning as that will allow you to clear mucus buildup from overnight, and even preserve lung function over time.
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2. Nutrient-Dense Diet
Another problem with CF is the inability to adequately absorb nutrients from food. It may come as a surprise, but thick mucus can actually block the pancreas, thereby preventing digestive enzymes from hitting our intestines. This, in turn, can lead to difficulty getting the higher calories you need and want.
In fact, many people with CF find themselves overeating to compensate for the inability to absorb the nutrients they need from their foods. In many cases, individuals with CF require up to twp times the average calorie intake simply to meet their daily needs.
Fortunately, a high-calorie, healthy fat-filled diet can help with this. Look for healthy fats from foods like avocados, virgin olive oil, nuts, and fatty fish (especially salmon). You can also find other sources of complex carbs, through foods like whole grains, fruits, and veggies.
And don’t forget high-quality protein, like lean meats, poultry, eggs, and legumes. These will help with repairing your issue and allow you to keep that oh-so-lovely lean muscle mass.
And finally, hydration. You can hydrate often to keep thick mucus out of your airways. Proper hydration helps to thin the mucus, allowing you to keep your body functioning properly.
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3. Enzyme Supplements
One of the main issues with CF is the pancreas’s inability to produce the necessary enzymes it needs for digesting food and getting the most out of the nutrients. One way to overcome this issue is through something called pancreatic enzyme replacements (PERTs), which can be paired with virtually everything you eat.
Through PERTs, you are able to get the various enzymes (lipase, protease, and amylase) for digesting fats, proteins, and carbohydrates. You can take them in pill or capsule form, before every meal or snack to facilitate digestion and the absorption of nutrients.
According to research, these supplements allow individuals with CF to prevent malabsorption, improve their nutritional profile, and even gain weight when they’re becoming too thin due to malnutrition.
Just make sure you listen to your healthcare provider and follow recommendations closely. This will allow you to maintain the right dosage and timing.
4. Physical Activity
This one sounds easy, but it might be the most important of all. Through regular exercise, you’re able to keep your weight manageable, keep your nutrients optimized, and keep your mood consistent. Not to mention, regular physical activity also helps you maintain good lung functioning.
Muscle building is also particularly important for people with CF, as the loss of nutrients can often affect that as well.
Through moderate, regular exercise, you can reduce airway inflammation, clear mucus (very important), and help curb the muscle wasting so common among many CF patients.
This is often a surprise to people, as exercise is sometimes thought to complicate things. In fact, it’s all about effort. You don’t need to push yourself to complete exhaustion. Rather, aim to exercise several days a week at a moderate pace, where your heart rate is elevated and your breathing is slightly strained.
If you’re older and this is less realistic, a brisk walk can do wonders. The main goal is to clear the mucus while also increasing your overall energy through a moderate workout. For the best results, combine aerobic activities with muscle training sessions. Just half an hour for the majority of the days in the week can do wonders.
And as always, consult your healthcare provider, if you’re unsure.
5. Infection Prevention and Hygiene
As you can imagine, infection is the primary concern for most people with CF. This happens because of excess mucus in the lungs, which can then become a fertile ground for all kinds of bacteria and viruses. Fortunately, there are ways to overcome this. You can do this by slowing the progression of the disease one step at a time.
To do this, and to prevent further damage to the lungs through bacteria such as Pseudomonas aeruginosa and Staphylococcus aureus, consider the following:
- Handwashing: Do it frequently, particularly before and after meals, when out in public, and after sneezing or coughing. Just be careful not to become obsessive, as you do need a certain degree of pathogens to stimulate your immune system and stay healthy. As always, seek balance and consult your doctor if you’re unsure.
- Cross-Contamination: Close contact with others struggling with respiratory illnesses always poses the risk of infection. That’s why it’s important that you take precautions, and always try to avoid large crowds when you’re feeling ill or when others are ill. And even though it may be tempting to help others by sharing respiratory equipment, do not.
- Thorough Cleaning: Speaking of respiratory equipment, nebulizers, inhalers, and other products need to be cleaned fully after every use. This is the simplest thing you can do to prevent the growth of bacteria.
At the end of the day, living with cystic fibrosis does not have to be a life-changing, life-disrupting condition. While it can certainly prevent its share of challenges and difficulties, you do have many ways to address these.
Using sanitizers, disinfecting wipes, and other preventative measures is another great way to keep yourself healthy. Always listen to your CF healthcare team as well, as they’ll have a keen understanding of your particular health profile.
With a little due diligence, some common sense practices, and adherence to your personalized treatment plan, you can manage your CF once and for all!
