التليف الكيسي

What is it?

Cystic fibrosis is a condition in which the lungs progressively collapse due to a build-up of thick and sticky mucus, it can also affect the digestive tube and other organs. It is one of the most common chronic lung diseases in adults and children.  

This type of disease affects the lungs, the pancreas, liver, intestines, paranasal sinuses, and sexual organs. Cystic fibrosis causes mucus to be thick and sticky. It clogs the lungs, causing repeated lung infections and lung damage.   

What are the symptoms? 

Cystic fibrosis symptoms may develop slowly over time. Some symptoms may worsen until becoming severe. The symptoms and the seriousness of the disease may vary from person to person.   

• Symptoms in newborns: stunted growth. 
• Bowel function symptoms: Pain due to constipation, swollen abdomen due to gas, nausea, pale or clay coloured stools with mucus, weight loss.
• Lung and paranasal sinus symptoms: Cough, increase in mucus, fatigue, nasal congestion, pneumonia, pain in the nasal passages.
• Later symptoms: sterility in men, swollen pancreas, breathing issues, and finger clubbing.

What causes it?    

Cystic fibrosis is caused by a faulty gene that causes the body to produce a thick and sticky substance. This clogs the airways and the pancreas causing infections. It could cause serious lung infections as well as acute digestive disorders. It may affect sweat glands, the male reproductive system, and other areas.  

Some people have the gene but never get any symptoms. The reason for this is that a person with cystic fibrosis must inherit two faulty genes to develop it. If they only have one, the disease won’t develop. It is more common in people of central and northern European descent.      

How can it be prevented?

As it is an inherited condition, it can’t be prevented. There are however some steps that can be taken to improve quality of life when affected by the condition. There is a test that can be done to check if either parent carries the gene that causes the condition, this is the closest thing to “preventing” the condition that exists. This would only be able to detect the condition, as previously stated, there is no known cure.    

What is the treatment? 

The key to being able to treat cystic fibrosis well is to diagnose it as soon as possible. A strong treatment plan is important to strengthen resistance to the disease and improve quality of life. Wherever possible, patients with this condition should be in a clinic specialised in cystic fibrosis. The treatments available include:

• Antibiotics to prevent sinus and lung infections and to treat them if they do happen
• Inhaled medicines to help decongest the airways
• Medications to thin the mucus and expel the mucus easily
• Annual vaccinations   
• Oxygen therapy
• Lung transplants may be done as a last resort.