This article reveals the differences between pulmonary fibrosis and cystic fibrosis:
Pulmonary fibrosis (PF), also known as interstitial pulmonary fibrosis, is a condition in which the lung tissue becomes stiff, thickened, and scarred over a period of time.
It affects about 1 person in every 5,000, typically people over the age of 50, smokers much more commonly than non-smokers.
Cystic fibrosis (CF) is a genetic disease that is characterized by abnormalities affecting certain glands of the body, particularly those which produce mucus. It is most often diagnosed in infants, however, in some cases, signs and symptoms do not appear until adulthood.
An estimated 30,000 children and adults in the US have the disease, and worldwide, there are over 70,000 suferrers.
In the US, it affects about 1 in 31,000 newborns of Asian descent and 1 in 17,000 African American newborns.
Doctors don’t know exactly what causes PF, but possible causes include:
- the use of certain medications, like – chemotherapy drugs or antibiotics;
- autoimmune diseases, like – rheumatoid arthritis or systemic lupus erythematosus;
- side-effects from radiation therapy for lung or breast cancer;
- other medical conditions, like pneumonia;
- exposure to various airborne substances, like – gas, asbestos, chemicals, animal proteins, coal dust, or sawdust.
CF is caused by a defective gene which makes the human body to produce thick and sticky fluid, called mucus. This glue-like mucus causes problems in many of the body’s organs, particularly the pancreas and the lungs.
The changed gene is on chromosome 7, which a child inherits from both parents. There are about 1,000 different mutations of this gene which can lead to CF.
When a woman and a man who are both carriers of the defective gene conceive a baby, there is a:
- 25 percent chance the baby will receive non-CF normal genes from each parent. In this situation, the baby is not a CF carrier and cannot have the disease;
- 2-in-4 chance the baby will receive a non-CF normal gene from the other parent and one CF gene from 1 parent. In this situation, the baby becomes a carrier of the CF gene;
- 1-in-4 chance the baby will receive the gene from each parent and will be born with the disease.
Symptoms of PF may include:
- bulb-like development of the fingertips and nails;
- a dry cough;
- weight loss;
- lasting tiredness;
- shortness of breath, particularly during or after physical exercise.
Note – in general, most patients have no family history of the disease.
Symptoms of CF may include:
- slow growth;
- failure to thrive;
- a nagging cough;
- oily stools;
- recurrent sinus infections;
- loose stools;
- frequent lung infections;
- recurrent wheezing;
- trouble breathing.
Possible complications of PF include:
- pulmonary hypertension;
- heart failure;
- chest infections;
- respiratory failure;
- serious lung issues, like – collapsed lung, lung infection, blood clots, or lung cancer.
Note – an estimated 77% of patients with PF die from breathing-related complications.
Common complications of CF are:
- cystic fibrosis-related diabetes;
- nasal polyps;
- liver disease;
- female fertility complications;
- distal intestinal obstruction syndrome.
If PF is suspected, then various diagnostic tests can be performed, including:
- lung cell sample by passing a small flexible telescope down the breathing tubes;
- blood tests to rule out other diseases;
- pulse oximetry – a small device which measures your oxygen saturation level;
- heart function tests, like – an echocardiogram;
- X-ray and CT scan of the chest;
- pulmonary function test to measure breathing capacity.
Doctors diagnose CF based on the results from various tests, such as:
- a sputum culture;
- newborn screening;
- lung function tests;
- sweat test;
- a sinus x-ray;
- a chest x-ray;
- genetic tests.
There is no way to repair scarring which has already happened to your lungs. However, the treatment is aimed to ease symptoms and to stop more damage. Possible treatments include:
- lung transplant, if you are a good candidate;
- nintedanib or pirfenidone to slow down damage to the lung tissue;
- pulmonary rehabilitation;
- oxygen therapy – this treatment makes it easier for you to breathe;
While there is no cure for CF, your healthcare provider may recommend you:
- surgery or other medical procedures that may improve your quality of life;
- avoiding the use of tobacco and second-hand smoke;
- increased physical activity;
- increased consumptions of fruits and vegetables;
- airway clearance techniques;
- drugs, like – inhaled medicines and antibiotics.
The prognosis varies from patient to patient, however, life expectancy after diagnosis is around 3 to 5 years. Recommended lifestyle changes include:
- ensure you get enough rest;
- don’t overexert yourself;
- quit smoking as well as avoid second-hand smoking;
- seek emotional support through friends, family, and support groups;
- eat fruits, legumes, and vegetables;
- eat less saturated fat;
- try to stay mentally active;
- avoid dust and excessive pollutants;
- avoid situations in which you might catch infections from other people;
- lose excess weight;
- exercise regularly to help keep your body working.
An estimated fifty percent of patients with CF will live past the age of 40. The disease tends to get worse over time.
Bottom Line – Pulmonary Fibrosis vs Cystic Fibrosis
Pulmonary fibrosis describes a group of diseases that lead to interstitial lung damage and loss of the elasticity of the lungs. While there is no cure, a number of treatments may help alleviate symptoms of PF, which include – shortness of breath, hacking cough, and fatigue.
Cystic fibrosis is a genetic disorder which commonly affects multiple organ systems of the human body. It takes two genes (1 from the father and 1 from the mother) for a baby to have the disease. Symptoms may include:
- salty-tasting skin;
- clubbed fingers;
- persistent cough;
- severe constipation;
- difficult bowel movements;
- bulky stools;
- coughing up thick mucus;
- loss of appetite;
- weight loss;
- poor growth;
- shortness of breath;
- sinus pressure or pain;
- inflamed nasal passages;
- frequent sinus or lung infections;
- stuffy nose;
- inability to tolerate physical exercise.
While there is no cure, treatments have improved the outlook for patients with the disease.
Sources http://err.ersjournals.com/content/17/109/116 https://www.ncbi.nlm.nih.gov/pubmed/21547723 https://www.ncbi.nlm.nih.gov/pubmed/26846831 https://www.chop.edu/cystic-fibrosis-center/cystic-fibrosis-research