Cystic fibrosis (CF) and pulmonary fibrosis (PF) are two chronic conditions that damage the lungs. Treatment for both aims to improve breathing, manage symptoms, and prevent further lung damage.
Your lungs supply your body with vital oxygen while helping remove waste gases such as carbon dioxide. When lung function is reduced, it can lead to various health problems.
CF and PF are two conditions that affect the lungs. While these two conditions have similar names, they also have many differences. Keep reading to find out more.
CF is an inherited condition where your body makes mucus that’s too thick and sticky. This can cause blockages and damage throughout the body, including in the lungs.
CF is caused by a mutation in the CFTR gene that’s inherited in an autosomal recessive manner. CFTR codes for a protein that’s important for making mucus. Mutations cause the faulty protein, leading to changes in mucus production.
PF is when scar tissue builds up in your lungs. Often, the PF cause isn’t known. This is called idiopathic PF. Some other factors that can contribute to the development of PF are:
- autoimmune diseases such as rheumatoid arthritis or scleroderma
- viral infections, including COVID-19, flu, and cytomegalovirus (CMV)
- environmental exposures to asbestos, dust from woods and metals, and cigarette smoke
CF and PF have many symptoms in common. But a few key differences also exist.
| Symptoms | CF | PF |
|---|---|---|
| Cough | may include mucus or blood | dry cough |
| Shortness of breath | yes | yes |
| Wheezing | yes | no |
| Exercise intolerance | yes | yes |
| Clubbing of fingers and toes | yes | yes |
| Frequent lung infections | yes | no |
Fatigue | yes | yes |
| Rapid, shallow breathing | no | yes |
| Muscle or joint pain | yes | yes |
| Unintended weight loss | no | yes |
| Clubbing fingers and toes | yes | yes |
CF isn’t just a pulmonary (lung) disease. The CFTR protein, which is faulty in people with CF, is found at any location in the body that makes mucus.
Because of this, CF can also affect many other parts of the body besides the lungs. This includes — but isn’t limited to — the digestive system, liver, and pancreas.
In addition to what we’ve already covered above, people with CF may also have:
- salty skin
- jaundice for a long time after birth
- intestinal obstruction shortly after birth (meconium ileus)
- poor weight gain or failure to thrive as an infant
- malnutrition
- abdominal pain
- greasy, foul-smelling stool
- constipation
- pancreatic issues, such as pancreatitis and pancreatic insufficiency
- cystic fibrosis-related diabetes
- liver disease
- frequent sinus infections
- nasal polyps
- osteoporosis
- male infertility
CF does not cause PF. However, its effects can damage lung tissue, leading to various complications.
The thick, sticky mucus produced due to CF can clog or block your airways. This creates an optimal environment for bacterial infections, such as those with Pseudomonas aeruginosa, and can also boost inflammation.
Over time, your airways widen, a condition called bronchiectasis. This makes it harder and harder to clear mucus from your airways, resulting in a cycle of further infection and inflammation that progressively damages your airways and lungs.
PF happens due to scarring that affects the tissue surrounding your alveoli. Alveoli are the tiny air sacs where gas exchange happens. The presence of thickened scar tissue prevents alveoli from functioning well.
As scar tissue accumulates, it gets progressively harder for your lungs to deliver oxygen into the bloodstream. When your organs and tissues don’t get enough oxygen they cannot function properly.
Currently, there’s no cure for either CF or PF. Treatment instead focuses on improving your breathing, managing your symptoms, and preventing additional damage.
CF treatment
Individuals with CF often use different techniques to help clear their airways. These may include special breathing techniques and therapy vests or chest physical therapy, which can loosen mucus.
Medications are also an important part of CF treatment. These help manage the effects that CF has on the lungs and throughout the body and include:
- bronchodilators to aid in opening your airways
- mucus-thinning medications to reduce the thickness of mucus
- anti-inflammatory medications, which lower inflammation and may include nonsteroidal anti-inflammatory drugs (NSAIDs) or corticosteroids
- antibiotics to treat or prevent infections
- drugs to address CF-related diabetes, liver disease, or osteoporosis if necessary
- CFTR modulators, which help boost the function of the CFTR protein in people with certain CFTR mutations include:
- elexacaftor/ivacaftor/tezacaftor (Trikafta)
- ivacaftor (Kalydeco)
- lumacaftor/ivacaftor (Orkambi)
- tezacaftor/ivacaftor (Symdeko)
Maintaining a nutritious diet — consisting of foods such as fruits, vegetables, and lean protein — is also important for preventing malnutrition due to CF. In addition to this, people with CF often take pancreatic enzymes to help absorb nutrients from food and supplement with certain vitamins, such as A, D, E, and K.
People with CF can develop severe lung or liver disease. As such, healthcare professionals may recommend a lung or liver transplant for some individuals.
PF treatment
A PF treatment plan can involve:
- pulmonary rehabilitation, which teaches breathing techniques to help with shortness of breath and uses exercise to improve breathing and endurance
- supplemental oxygen therapy
- medications such as pirfenidone (Esbriet) and nintedanib (Ofev), which help slow the progression of idiopathic PF
- drugs to treat gastroesophageal reflux disease (GERD), which often affects people with PF
- lung transplant for individuals with severe PF
CF is associated with a reduced life expectancy. The CF Foundation Patient Registry Annual Data Report noted that people with CF who had died in 2021 were a median age of about 34 years old.
However, advances in care have raised the predicted life expectancy of people with CF. The CF Foundation notes that the predicted life expectancy for people with CF who were born between 2018 and 2022 is 56 years.
Life expectancy for people with PF depends on the type of condition they have. If it’s IPF, the average life expectancy is 3–5 years. But for other PF causes, the life expectancy can vary and be a great deal longer.
CF and PF both affect the lungs, causing progressive damage that impairs lung function. In addition to damaging the lungs, CF can also affect other parts of the body, such as the digestive system and liver.
There’s no cure for CF and PF. Instead, treatment aims to improve breathing, ease symptoms, and prevent further damage. If you have CF or PF, your care team will develop a treatment plan that’s tailored to your individual needs.