Cystic fibrosis (CF) is the most common hereditary chronic disease of the mucus and sweat glands.
It largely affects the lungs, pancreas, sex organs, intestines, liver, and sinuses.
The condition is due to a defective gene that produces an irregularly shaped form of a cystic fibrosis transmembrane conductance regulator (CFTR).
CFTR is a type of protein that is essential to the body.
People having cystic fibrosis do not have enough CFTR for their cells to work normally because their bodies quickly destroy the mutant CFTR.
Majority of Cystic Fibrosis cases result from the lack of an essential amino acid building block in the CFTR, causing it to fold into its characteristic and functional three-dimensional structure abnormally.
A protein that fails to fold into the intended shape usually produces inactive proteins with different characteristics.
This is how CF affects the mucus and sweat glands.
Normally, mucus is watery, thus keeping the linings of certain organs are moist and keeps them from drying out or getting infected.
But in the case of CF, the abnormally shaped CFTR causes mucus to become thick and sticky.
The mucus tends to build up in your lungs and block the airways.
The condition is then a potent venue for bacteria to grow causing serious lung infections, which will eventually damage the lungs.
The most common cause of death of CF patients is respiratory failure.
In the same way, mucus build up can block ducts in the pancreas.
With this, digestive enzymes produced by the pancreas, will not be able to reach the small intestine.
These pancreatic enzymes are responsible for breaking down dietary nutrients in order for the body to efficiently absorb fats and proteins.
Moreover, the abnormally shaped protein or CFTR can induce your body to withdraw large amounts of salt, upsetting the mineral balance in your body.
A specific treatment plan is required for each particular CF condition.
For cystic fibrosis affecting the lungs, regular chest physiotherapy, an average of two time daily, is very important.
This procedure helps clear away the thick mucus in the airways.
Exercise is also beneficial.
It helps regulate blood and oxygen circulation as well as encourage muscle function in the body.
Inhalers and oxygen are also helpful in this lung disease for it can open up the airways.
Antibiotics and antifungals are standard part of the treatment and are usually taken for long periods.
It helps with the inflammation and pain.
The dosage and type of the medications would depend on the type of bacteria infecting the body and the severity of the disease.
Also, others may decide to take proteolytic enzyme supplements such as serrapeptase or treatments to help in clearing inflammation and mucus.
For people with CF that primarily affecting the digestie system, they must take enzyme supplement.
People with such disease will have difficulty in absorbing certain nutrients due to the decreased digestive enzymes.
Thus, taking enzyme supplements such as lipase alongside a diet high in fat and carbohydrates is a standard treatment.