Living With Cystic Fibrosis

It wasn’t all that long ago that cystic fibrosis (CF) was considered terminal at an early age. Even as recently as the 1980s, surviving past the age of 20 was virtually unheard of. Fortunately, this is no longer the case. Today, people with CF are living longer than ever thanks to new drugs, better management practices, and the routine screening of newborns in the United States.

What this means is that people living with the disease can now plan for their futures. While CF is no less serious than it has ever been, there are tools and strategies that can help you and your family better cope.

Your Emotions

Life changes the moment you receive a CF diagnosis. Whether you are bringing home a newly diagnosed baby or learning to manage the disease as you move into adulthood, CF will always be a large part of your life.

To this end, as much as you need to deal with the physical aspects of the disease, in order to cope, you need to find the emotional tools to start normalizing CF in your life.

This starts with education and support. Cystic fibrosis is not only an uncommon disease, it remains largely misunderstood by many. Strive to gain as much knowledge as you can about CF. This includes not just current and long-standing science and treatments, but any breakthroughs, advances, or setbacks that may impact your care moving forward.

Perhaps the best resource for information is the Cystic Fibrosis Foundation (CFF). In addition to its vast library of online content, CFF offers a helpline at 844-COMPASS (844-266-7277). The free helpline is staffed by trained caseworkers who are available for one-on-one consultations from Monday through Friday, 9 a.m. to 7 p.m. Eastern time.

A CFF caseworker can also connect you to a support network of other people living with the disease through one of its 70 CFF chapters and branch offices in the United States.

You can also turn to online chat rooms or community social media pages for interaction and advice.

And although your family and friends may not understand what it's like to live with CF, don't underestimate how valuable leaning on them can be. Remember, too, that they can become a greater asset as you teach them more about the disease.

Beyond education and support, one of the keys to normalizing CF is to establish and maintain a routine. The simple truth is that CF treatments can often consume a large part of your day. By setting a routine and keeping with it, the daily tasks will eventually become automatic, and you’ll be better able to avoid the emotional ups and downs that can place undue stress on your life.

Diet

One of the biggest challenges of living with cystic fibrosis is maintaining the nutritional intake needed to sustain growth, health, and energy levels. Children and adults with CF often need to double their daily caloric intake to avoid weight loss, which is not as easy as it seems.

Dietary Strategies

Ideally, you will have the advice of a specialist dietitian to help guide you. While there is no cystic fibrosis diet per se, the general strategy involves eating three meals and two to three snacks per day as part of a high-fat, high-calorie diet.

The extra calories you consume should be part of a well-balanced diet with less saturated fats and more unsaturated fats (such as those from avocados, nuts, and olive oil). Walnut and flaxseed oils are also good choices because they contain chemicals that can reduce inflammation.

Many people like to keep track of their calories throughout the day and have extra snacks on hand if the numbers fall short. A 100-calorie-at-a-time approach may be more appealing than trying to sneak in an extra 500-calorie snack, which may be taxing. Some 100-calorie options include:

• One tablespoon of peanut butter
• A third of an avocado
• One-quarter cup of shredded cheese
• Two slices of bacon
• Two tablespoons of chopped nuts
• A six-ounce glass of whole milk
• Two tablespoons of hot fudge
• Two-thirds cup of whipped cream topping

Even if you are following a recommended diet to the letter, remember to be diligent about taking any vitamin or mineral supplements advised by your healthcare provider as well.

Proper hydration is also important, as it can help prevent dehydration and ensure that your already thick mucus doesn't get any thicker. The amount of water needed depends on your age, weight, activity level, and the climate you are in.

For Babies

The general dietary goals for children and adults with CF apply to babies, as well. Once your baby is able to eat soft food, focus on products with the highest calories and find ways to increase daily fat intake.

Some helpful suggestions include:

• To increase calories, add breast milk or high-calorie formula to the baby’s cereal. There are a number of commercial formulas made especially for babies with poor growth. Alternatively, your dietitian can teach you how to add the appropriate fat and calories to standard baby formula.
• To add fat to the baby's diet, stir butter, margarine, or oils (such as olive, canola, or safflower) into strained vegetables or fruit. While the baby may not like it at first, over time, he or she will get used to it.
• If your baby cannot gain weight, is bloated, or has loose, greasy, and foul-smelling stools, speak with your practitioner about a pancreatic enzyme supplement. The pellets inside the capsule can be measured and sprinkled over food.
• Ask your healthcare provider about the appropriate pediatric formulations for vitamins A, D, E, and K and how best to provide them to your baby.

Meal Planning

If you or a family member has CF, much of your life will be centered around planning, preparing, and serving meals. You can make life easier for yourself while achieving dietary goals by following a few simple rules, such as:

• Plan your menus for the week: This will not only save time at the grocery store but help you avoid trying to figure out what to make for dinner last minute.
• Cook once to eat three times: When cooking, make enough so that you can serve one meal for dinner, pack another for lunch, and freeze a third for days when you don’t feel like cooking.
• Use a slow cooker: Using a slow cooker allows you to start preparing lunch or dinner in the morning when you have the most energy.
• Think about portability: Plan meals or snacks that you can carry in your backpack, purse, or briefcase or store in your desk, locker, or a cooler in your car.
• Keep it interesting: Buy a variety of foods so you don't get tired of eating the same foods every day. The more interesting the food is, the more likely it will be finished.

Exercise

While cystic fibrosis may reduce your tolerance for exercise, you shouldn't avoid it. Whatever your physical state, exercising—even in a small amount—can slow the decline in lung function if you do it appropriately and consistently.

Ultimately, strive to find something beneficial that you can enjoy. It can be an activity you do on your own or one that you can participate in with a partner, class, or group of friends. By finding the activity that's right for you, you’ll be more likely to keep with it long term. 

Setting Goals

When starting, you need to set clear goals. It often helps to work with a medical professional like a physical therapist (rather than a personal trainer) who can assess your fitness level and design a plan that neither overtaxes you nor leaves you under-challenged. It may involve walking, swimming, cycling, or other physical activities to get your lungs working and heart pumping.

The important thing is to start slowly. If you are in poor health, start with maybe five to 10 minutes of walking, and build from there. If you are unable to walk a full 10 minutes, try five minutes in the morning and five minutes in the evening until you are strong enough to do more.

As a general rule of thumb, exercise routines should eventually involve a minimum of 20–30 minutes of aerobic activity three times per week. Frequency is more important than duration. For example, if you choose to walk 10–15 minutes per day every day, you will more likely feel the benefits than if you work out 45 minutes twice weekly.

If you are strong enough to begin resistance training, start first with resistance bands and isometrics before moving on to exercise machines and free weights.

Children

It's important to instill good exercise habits early on in children. By exposing your child to a variety of activities, he or she will be more apt to embrace an active lifestyle during the teen or adult years. Even if exercise has never been a part of your life, if you have a child with CF, it is far better to lead by example and make fitness a family affair.

The benefits of exercise for children are clear. Beyond the physical effects—loosening mucus and strengthening the muscles—it can help boost confidence and self-esteem. It may be alarming to see your child coughing a lot during exercise, but try to remember that doing so helps them clear their lungs and is a good thing.

That said, it is important to speak with your child's physical education teacher to ensure that the instructor understands what CF is and that necessary precautions are needed to protect your child's health.

To this end, teachers need to:

• Ensure proper hydration
• Allow snacks during strenuous activities
• Separate your child from any classmate who may be sick
• Reinforce the need for good hygiene and handwashing, including the use of an alcohol-based hand cleanser after shared sports equipment is used
• Encourage activity, but know when it is time to stop

Airway Clearance

Airway clearance is an important self-care tool that often needs to be adjusted as the disease progresses. What may have worked several years ago can sometimes fall short the older you get. To ensure you are achieving the best results, check in regularly with your physical therapist who can assess your progress and see if you're performing the techniques correctly.

If huff coughing has been recommended as part of your treatment plan, make sure you are taking the following steps:

• Sit up straight and tilt your chin slightly forward.
• Take a slow, deep breath, filling the lungs to 75% capacity.
• Hold your breath for two to three seconds.
• Release the air in slow, forceful bursts while saying "huh," as if you are trying to fog up a mirror.
• Repeat this one to four times before ending with a single strong cough to clear the accumulated mucus from your lungs.
• Start the entire cycle again, repeating three or four more times.

Again, over time, huff coughing may not be enough to provide relief. In such a case, a partner may be needed to perform postural drainage therapy (PDT), or airway clearance devices may be recommended.

Environment

Anybody who is regularly exposed to secondhand smoke is at risk for developing smoke-related illnesses, but people with cystic fibrosis (CF) have an even greater risk of complications from this kind of exposure.

In addition to the risks related to exposure to toxins, smoke can damage the cilia that line the airways, causing mucus to become trapped. This makes the inherent mucus buildup associated with CF worse. 

Secondhand smoke exposure may also:

• Inhibit proper weight gain and encourage weight loss
• Increase your risk of respiratory infections
• Decrease overall lung function

Air pollution, weather, and the cleanliness of one's environment may also affect CF symptoms and progression.

Do your best to eliminate secondhand smoke exposure as much as possible and keep your home free of dust. Consider using an air purifier as well.

Fertility and Pregnancy

Today, as life expectancies increase, more and more adults with CF are becoming parents. This includes men with CF, almost 95% of whom are born without a vas deferens, the tube that connects the testicles to the urethra.

Even in circumstances like these, assisted reproductive technologies can improve the likelihood of pregnancy in couples who desire it. With that being said, the process is not always easy and may require a candid assessment of your potential challenges and risks.

Preconception Screening

Preconception screening is performed to assess your baby’s risk of getting cystic fibrosis. If you have CF, any baby you have will inherit one defective CFTR (CF transmembrane conductance regulator) gene from you. As such, your partner would need to be tested to see if he or she might be a carrier.

Based on these findings, you can make an informed choice as to whether you want to proceed with conception efforts or not. If you choose not to, you can explore adoption or finding an egg or sperm donor.

Conception Options

If you choose to proceed, you can conceive naturally if there are no physical barriers impairing fertility. If there is infertility in either partner, assisted reproductive techniques such as in vitro fertilization (IVF) and intrauterine implantation (IUI) may be explored.

While infertility is common in men with CF, women can also experience it. This is most often due to the obstruction of fertilization caused by thick cervical mucus or metabolic problems like anovulation (inability to ovulate) that occur more frequently in women with CF. 

Comparatively speaking, IVF is the more successful option with conception rates of between 20% and 40%. By contrast, IUI success rates range anywhere from 5%–25%.

Sperm Harvesting

If you are a man with CF, the absence of a vas deferens may mean that you are infertile, but it does not mean you are sterile. There is a distinct difference. Even though your vas deferens is missing, you still are producing sperm, while sterile men do not. In fact, around 90% of men with CF who have a missing vas deferens will still have fully productive testicles.

For these men, sperm harvesting may be performed. If there is no sperm in the ejaculate (a condition known as azoospermia), a combination of techniques may be used to extract sperm from the testicles and implant it into a mature egg.

Most commonly, it involves testicular sperm extraction (TESE), a technique usually performed in a hospital with sedation. The procedure needs to be timed with the IVF cycle. Alternately, the sperm can be retrieved and frozen until the eggs are later harvested.

TESE involves the following steps:

• Using gentle suction, a fine needle is inserted into the testicle to extract a tissue sample. This sample is examined under the microscope to detect sperm.
• If no sperm are found, a small tissue sample (testicular biopsy) would be extracted through an incision in the scrotum and testicle.
• Sperm can then be extracted from the tissue, a considerable and often laborious process.

Sperm retrieved from tissue will be immature and incapable of fertilization by conventional means. To this end, a technique known as intracytoplasmic sperm injection (ICSI) would be used to inject a selected sperm into a mature egg with the use of a microscope and specialized tools.

After the TESE, you may experience discomfort, bruising, and tenderness for 24 to 48 hours. There is a small risk of bleeding and infection. Tylenol (acetaminophen) may be prescribed to help relieve pain.

Managing Your Pregnancy

If you are pregnant and have cystic fibrosis, you need to take extra steps to help your pregnancy remain as event-free as possible. This may require you to take some proactive steps, when needed, to help your body cope with the rigors of pregnancy.

Among the considerations are:

• Manage your nutrition: As pregnancy increases your nutritional needs, you will need to work with a dietitian to ensure you experience a normal weight gain of between 25 and 35 pounds. If you are unable to do this, enteral nutrition (tube feeding) may be required. In severe cases, hospitalization and intravenous feeding in a procedure known as total parenteral nutrition (TPN) may be necessary.
• Continue airway clearance: During pregnancy, some people fear that postural drainage and other airway clearance techniques may hurt the baby, but they do not.
• Increase your intake of calcium, iron, and folic acid: These are commonly depleted during pregnancy. Speak with your healthcare provider about appropriate supplements. Calcium and iron can help support the baby's growth, while folic acid prevents brain and spinal cord defects.
• Avoid excessive intake of vitamin A: Vitamin A supplements are commonly taken by people with CF. However, overuse can lead to toxicity and may result in fetal development problems during the first trimester.
• Be prepared for hospitalization: Pregnancy can take a toll on your body if you have CF. If needed, you may be hospitalized during the last two to three months of your pregnancy, when demands on your body are the greatest.  

Insurance and Finances

Navigating health insurance is tough enough if you’re in perfect health. Dealing with it if you have CF can be overwhelming. Luckily, there are resources you can turn to if you need assistance.

Chief among these is the Compass program offered by the Cystic Fibrosis Foundation. This free service is available to anyone with CF irrespective of health status, income, or insurance coverage.

By calling 844-COMPASS (844-266-7277), you will be connected to an experienced case manager who can assist you with concerns related to insurance, finances, legal problems, or any other matter you may be struggling with. The Compass service is free of charge and available Monday through Friday from 9 a.m. to 7 p.m. You can also email them at compass@cff.org.

CFF also operates a Patients Assistance Resource Center (PARC) to help you get the most help from your current health plan or to find financial assistance programs able to cover deductibles, pharmaceuticals, or co-payment costs. Call 888-315-4154 or email parc@cff.org. 

HealthWell Foundation also offers financial assistance for people with CF who are struggling to cope with the cost of CF-specific treatments. The program is open to citizens and legal residents of the United States who meet financial eligibility requirements and have current insurance with prescription drug coverage. If eligible, HealthWell will provide up to $15,000 in co-pay assistance to cover any one of 63 CF-specific treatments.