Just What is CF?

whatscf_imageCystic fibrosis (CF) is a genetic disease affecting approximately 30,000 children and adults in the United States. A defective gene causes the body to produce an abnormally thick, sticky mucus that clogs the lungs and leads to life-threatening lung infections. These thick secretions also obstruct the pancreas, preventing digestive enzymes from reaching the intestines to help break down and absorb food. The mucus also can block the bile duct in the liver, eventually causing permanent liver damage.

The median age of survival for those with CF is 38 years old. There is no cure for this disease.

More than 10 million Americans are unknowing, symptomless carriers of the defective CF gene. An individual must inherit two defective CF genes-one from each parent-to have CF. Each time two carriers conceive, there is a 25 percent chance that their child will have CF; a 50 percent chance that the child will be a carrier of the CF gene; and a 25 percent chance that the child will be a non-carrier. CF occurs in approximately one of every 3,500 live births. About 1,000 new cases of CF are diagnosed each year. More than 80 percent of patients are diagnosed by age two; 50 percent of cases are age 18 or older today!

People with CF have a variety of symptoms including: very salty-tasting skin; persistent coughing, at times with phlegm; wheezing or shortness of breath; frequent lung infections: an excessive appetite but poor weight gain/growth; and greasy, bulky stools. Symptoms vary from person to person due, in part, to the more than 1,000 mutations of the CF gene.

The sweat test is the standard diagnostic test for CF. A sweat test should be performed at a CF Foundation-accredited care center where strict guidelines are followed to ensure accurate results. This simple and painless procedure measures the amount of salt in the sweat. A high salt level indicates CF.

The type and severity of CF symptoms can differ widely from person to person. Therefore, although treatment plans can contain many of the same elements, they are tailored to each individual’s unique circumstances. Each day, people with CF complete a combination of the following therapies:

Airway clearance to help loosen and get rid of the thick mucus that can build up in the lungs.

Inhaled medicines to open the airways or thin the mucus. These are liquid medicines that are made into a mist or aerosol and then inhaled through a nebulizer and include antibiotics to fight lung infections and therapies to help keep the airways clear.

Pancreatic enzyme supplement capsules to improve the absorption of vital nutrients. These supplements are taken with every meal and most snacks. People with CF also usually take multivitamins.

An individualized fitness plan to help improve energy, lung function, and overall health

CFTR modulators to target the underlying defect in the CFTR protein. Because different mutations cause different defects in the protein, the medications that have been developed so far are effective only in people with specific mutations.

According to the CF Foundation’s National Patient Registry, the median age of survival for a person with CF is in the late-30s. As more advances have been made in the treatment of CF, the number of adults with CF has steadily grown. Today, 50 percent of the CF population is age 18 and older. Adults, however, may experience additional health challenges including CF-related diabetes and osteoporosis. CF also can cause reproductive problems-more than 95 percent of men with CF are sterile. But, with new technologies, some are becoming fathers. Although many women with CF are able to conceive, limited lung function and other health factors may make it difficult to carry a child to term.

There are more than 115 CF Foundation-accredited care centers across the United States that specialize in the diagnosis of CF and provide care to people with the disease. Care center staff includes physicians, nurses, nutritionists, respiratory therapists, social workers, genetics counselors, and other medical professionals.

Make a Plan to Stay Healthy!

Most people with cystic fibrosis can lead active lives—depending upon the severity of their disease—with routine therapies and regular visits to a Cystic Fibrosis Foundation-accredited care center. Staff at CF Foundation-accredited care centers partner with people with CF to develop individual treatment plans. These plans typically include high-calorie, high-fat diets, therapies to loosen the clogged mucus from their airways, and mucus-thinning drugs and antibiotics when needed. By following a treatment plan developed with their CF care center team, many people with CF can slow down the progression of their disease. A healthier body is better able to deal with bacteria and chronic lung infection.

Avoid the Spread of Germs

Cystic fibrosis puts the airways at risk for lung infections. There are, however, effective ways to lessen the risk. One way is to limit contact with known germ sources. Especially by not having 2 CF people be together. Although germs are everywhere and cannot be avoided, one of the best ways to keep from catching or spreading germs is through effective hand-washing, whether with soap and water or alcohol-based hand gels. Everyone with CF should avoid unnecessary contact with people who have a cold or any other contagious illness, and should cough and sneeze into a tissue. Learn more about methicillin-resistant Staphylococcus aureus (MRSA). It’s all about good hand hygiene!

Flu season, which begins in August and lasts throughout the winter, is the time of year that the influenza virus is easiest to catch. The flu is considered highly contagious, because it can spread by direct contact, coughing, sneezing, and when an infected person touches a surface that others then use, like door knobs and railings. People with lung infections, including people with CF, can develop more serious cases of the flu, so it is important for you to get a flu shot to be immunized against the most prevalent strains. This year’s flu season is unusual because people could get infected by either seasonal flu viruses or the new H1N1 virus. Everyone with CF and members of their households will need to be aware of and get vaccinated for both viruses.

Also, there is evidence to show that people with underlying health conditions, including lung disease, are at greater risk of developing serious illness from COVID-19 if they become infected. Please refer to the CDC guidelines for people who are at high risk.

Diet – Watch What You Eat

An important factor for staying healthy is good nutrition. Thick mucus often gets in the way of proper digestion, causing malabsorption. This problem is treated with pancreatic enzyme supplements, vitamins and a high-calorie, high-fat diet. In CF, a high-calorie, high-fat diet is vital for normal growth and development in children, and offers adults a way to maintain optimal health. The dietitians at CF Foundation-accredited care centers work with patients and their families to map out the best diet for each person. Nutrition and general lung health are closely linked. People with cystic fibrosis may need extra calories to compensate for the malabsorption of nutrients. These extra calories also help to meet the greater energy needed for breathing. In fact, for children with CF, extra fat calories are good for fueling normal growth and development. For ideas about how to fit getting extra calories into a busy lifestyle, go to www.CFF.org.

Pancreatic Enzyme Supplements – Almost everyone with cystic fibrosis needs to take pancreatic enzyme supplements with meals and snacks. Taken by mouth, the enzymes go to work in the intestines to help digest food so it can be absorbed by the body. Patients should always check with their doctor or dietitian to know the exact amount of enzymes to take.

Vitamins and Minerals – Most people with cystic fibrosis do not absorb fats properly, so certain vitamins, or “fat-soluble” vitamins, are not absorbed. These vitamins are Vitamins A, D, E and K. People with CF usually take these vitamins daily. Minerals, like calcium, iron, sodium chloride and zinc, are also essential to maintaining good health through nutrition. Certain foods are good sources of these minerals and they are also available as supplements.

Cystic Fibrosis-Related Diabetes – Keeping Sugar in Check

As people with cystic fibrosis get older, their risk of getting diabetes increases. With CF, scarring often occurs in the pancreas, which produces insulin for the body. This scarring sometimes prevents insulin from getting into the blood and could lead to diabetes. CF Foundation-accredited care centers regularly check each patient for diabetes.

Lung Health – Thank You for Not Smoking

Some environmental factors in daily life can damage lung health. For instance, second-hand smoke is particularly harmful to people with cystic fibrosis. Keeping airways healthier means not smoking and keeping the environment smoke-free.

Nebulizers – Clearing the Airways

Since inhaled drugs more easily reach the airways, they are common in cystic fibrosis care. Inhaled treatments can be given by aerosol, a mist treatment made from liquid medicines. In this case, the drug goes into a cup (nebulizer) and is attached to a small air compressor. The compressor blows air through the cup and makes a mist. Cystic fibrosis patients inhale the small particles in the mist through a mask or mouthpiece for several minutes to help clear the mucus. Medicines can also be given as metered dose inhalers, which deliver one dose of medicine at a time.

Lung Health – Exercise

Exercise is important to maintain lung health, especially for people with CF. Learn about the different kinds of exercise you can safely do on a daily basis from visiting www.CFF.org.

All information has been taken directly from the Cystic Fibrosis Foundation. Please visit www.cff.org or call (800) FIGHT CF to learn more about the services and programs available to people with CF.