What is cystic fibrosis?
Cystic fibrosis (CF) is a genetic disease that causes progressive damage to the organs, especially the lungs and digestive system. Due to scientific advancements, the life expectancy of people with cystic fibrosis has increased in the recent past. From 1995 to 1999, the life expectancy for people with this disease was only 32 years. However, between 2015 and 2019, the life expectancy for people with CF increased to 46 years.
Cystic fibrosis is a disorder that affects the production of chloride in your cells. When your cells lack chloride, your mucus becomes thick and sticky. This affects your lungs and digestive system.
In the lungs, the thick mucus traps bacteria easily, leading to chronic infections and inflammation. The thick mucus also prevents the pancreas from releasing the right amounts of digestive enzymes, leading to malnutrition and digestive problems. In the liver, mucus blocks the bile duct, leading to liver issues as well.
Symptoms of CF
The symptoms people with CF may have include:
- Coughing with thick mucus
- Frequent lung infections
- Inflamed nasal passages and sinuses
- Chronic sinus infections
- Bowel movements that smell particularly bad
- Greasy bowel movements
- Lack of weight gain or proper growth
- Chronic constipation
How cystic fibrosis is diagnosed
In the United States, nearly every newborn baby receives a screening for CF. Doctors take a small sample of blood and send it to a lab to screen for several conditions, including cystic fibrosis. This early diagnosis allows doctors to treat your baby's condition before symptoms arise, leading to better outcomes.
If a newborn screen comes up positive for CF, your doctor may also do a sweat test.
During this test, doctors induce sweating and then take a small sample to measure the amount of salt in the sweat. People with CF tend to have more chloride in their sweat than people who do not. This is considered the most reliable test for cystic fibrosis.
If you have a history of cystic fibrosis in your family, your doctor might recommend genetic testing before becoming pregnant and/or during pregnancy to determine the likelihood of having a baby that has cystic fibrosis.
Treatments for CF
The reason why the life expectancy for people with cystic fibrosis has increased is due to advancements in research and treatments.
Most people with CF take inhaled medications every day. These medications help to thin mucus, clear airways, and prevent lung infections. They also take a pancreatic enzyme supplement to help digest food better and get all the nutrients they need.
A new class of medications called CFTR modulators work to correct the malfunctioning gene that causes cystic fibrosis. As of right now, there are four medications available that work on specific mutations of the gene, but not every single one. Researchers are developing new drugs that will work on other mutations of the gene.
Airway clearance techniques (ACT)
People with cystic fibrosis do different techniques every day to clear their airways. These include:
- Breathing exercises
- Coughing or huffing
- Wearing an oscillating vest to break up mucus
- Postural drainage positions
- Percussion (tapping or hitting) on the chest or back
- Positive expiratory pressure (PEP) machines
People with cystic fibrosis generally try to stay active and healthy to keep their lung function up and fight infections. You can work with your doctor to come up with a fitness plan to improve your lung function and stay fit.
A lung transplant can improve your quality of life and extend your life if you have advanced cystic fibrosis. However, it is not a cure. About 90% of people with CF who receive a lung transplant survive the procedure. Most people who successfully receive a lung transplant live for at least 1 year. Half of the people with CF who received a lung transplant live for 5 years after the procedure. Many of those people will live for 10 years or more with transplanted lungs.
Health Solutions From Our Sponsors
Cystic Fibrosis Foundation: "About Cystic Fibrosis," "Airway Clearance," "CFTR Modulator Therapies," "First Stop: Your CF Care Team," "Newborn Screening for CF," "Sweat Test," "Understanding Changes in Life Expectancy," "Why Are Some Germs Particularly Dangerous for People With CF?"
Cystic Fibrosis Trust: "Cystic fibrosis and organ transplants."
Mayo Clinic: "Cystic fibrosis."
Top Life Expectancy Someone Born With Cystic Fibrosis Related Articles
Respiratory Illnesses: 13 Types of Lung InfectionsIs your cough caused by a cold, flu, pneumonia or something else? Learn causes of respiratory infection like bronchitis, pneumonia, SARS, Coronavirus COVID-19 bird flu, and more.
acetylcysteineAcetylcysteine, a mucolytic agent, is used to treat pulmonary diseases like chronic emphysema, asthmatic bronchitis, bronchiectasis, and acute pneumonia. Acetylcysteine breaks up mucus and makes it easier to cough up and clear the lungs. Common side effects include bronchospasm, odor, drowsiness, fever, coughing up blood (hemoptysis), increased volume of bronchial secretions, irritation of tracheal or bronchial tract, nausea, and others. Do not use if you have acute asthma. Consult your doctor if you have a history of bronchospasm, or if you are pregnant or breastfeeding.
Can You Get Cystic Fibrosis at Any Age?Cystic fibrosis is classically a disease of childhood that progresses into adulthood. If you have a genetic defect related to cystic fibrosis (CF), you are more likely to be born with cystic fibrosis. The symptoms might appear later, and hence, the age at diagnosis varies widely.
chloramphenicolChloramphenicol is a synthetic broad-spectrum antibiotic used to treat severe systemic infections, including bacterial meningitis, bacterial blood infection (bacteremia), typhoid fever, Rickettsial infections, and exacerbation of cystic fibrosis. Common side effects of chloramphenicol include bone marrow depression, blood disorders (including anemia), headache, confusion, delirium, depression, nausea, vomiting, diarrhea, and others. Avoid use in nursing mothers; discontinue either the drug or nursing. Consult your doctor if pregnant.
COPD (Chronic Obstructive Pulmonary Disease)COPD or chronic obstructive pulmonary disease is a lung condition caused by smoking tobacco, exposure to secondhand smoke, and/or air pollutants. Conditions that accompany COPD include chronic bronchitis, chronic cough, and emphysema.
Symptoms of COPD include shortness of breath, wheezing, and chronic cough. Treatment of COPD includes GOLD guidelines, smoking cessation, medications, and surgery. The life expectancy of a person with COPD depends on the stage of the disease.
Cystic Fibrosis Life SpanCystic fibrosis (CF) is a progressive disease that needs daily care. Though a patient needs to take medications lifelong, most people with cystic fibrosis are usually able to attend school and work. The improvement in screening and treatments mean that people with CF now may live into their mid- to late 30s or 40s, and some are living into their 50s.
digestive enzymesDigestive enzymes are natural substances that help the digestive system break down food. Taken as a supplement, digestive enzymes may be used to treat adults and children with pancreatic insufficiency caused by cystic fibrosis, chronic pancreatitis (inflammation of the pancreas), pancreatectomy (removal of all or part of the pancreas), or pancreatic cancer. Common side effects of digestive enzymes include headache, neck pain, dizziness, abdominal cramping, nausea, diarrhea, gas (flatulence), indigestion, abnormal stools, and others. Do not take if you have acute pancreatitis.
Digestive Enzymes OralOral digestive enzymes are found in the digestive tract, are naturally produced by the body, and help breakdown the foods we eat. The pancreas makes and secretes digestive enzymes. Prescription and OTC digestive enzymes are available in PEPs or pancreatic enzyme products. PEPs are prescribed for people with cystic fibrosis, chronic pancreatitis, pancreatic cancer or tumors, Shwachman-Diamond syndrome, or removal of part or all of the pancreas. Side effects include life-threatening allergic reactions, difficulty breathing or talking, swelling of the mouth/face/lips/tongue/throat, chest tightness, rash or hives, itching, red, swollen, blistered, or peeling skin; wheezing.
gentamicinGentamicin is a broad-spectrum antibiotic used to prevent and treat many types of bacterial infections, and is typically administered as an injection. Gentamicin is also used to prevent surgical infections, and to treat conditions such as cystic fibrosis, pelvic inflammatory disease, and infective endocarditis. Common side effects of gentamicin may include reduced urine output, kidney damage, vertigo, dizziness, hearing loss, ringing in the ears (tinnitus), balance problems (vestibular), impaired coordination, balance and speech (ataxia), gait instability, skin reactions (swelling, rash, itching), and others.
Reasons You're Short of BreathHave you ever found yourself gasping for air after just a short flight of stairs? You may just need to do a bit more exercise, or it could be something more serious.
Lung Anatomy DiagramThe lungs are primarily responsible for the exchange of oxygen and carbon dioxide between the air we breathe and the blood. Eliminating carbon dioxide from the blood is important, because as it builds up in the blood, headaches, drowsiness, coma, and eventually death may occur. The air we breathe in (inhalation) is warmed, humidified, and cleaned by the nose and the lungs.
nitric oxide gasNitric oxide gas is used for treating hypoxic respiratory failure in newborns caused by impaired lung function, which leads to low oxygen levels in the tissues (hypoxia). It may also be used for cystic fibrosis, acute respiratory distress syndrome (ARDS), pulmonary arterial hypertension (PAH), sickle cell crises, diagnosis of sarcoidosis, and others. Common side effects of nitric oxide gas include low blood pressure (hypotension), withdrawal, complete or partial collapse of a lung’s lobe or an entire lung (atelectasis), blood in urine (hematuria), high blood glucose levels (hyperglycemia), whistling sound while breathing due to obstructed airway (stridor), infection, cellulitis, and sepsis.
Surprising Causes of Lung DamageCarpets, fireworks, and hot tubs are some of the unexpected things that can hurt your lungs. Find out what you can do to prevent problems from these and other culprits.
Will a Chest X-Ray Show Lung Cancer?When diagnosing lung cancer, chest X-rays do not provide a definitive diagnosis of lung cancers at an early stage (when they are more treatable). Until the lung cancer shows up on a chest X-ray, the tumor is often too far advanced to be cured. Often, many things seen on a chest X-ray turn out to be treatable problems or artifacts.