Cystic fibrosis is also known as mucoviscidosis. It is an inherited disorder which causes severe damage to the lungs, digestive system, and other organs in the body. Mucoviscidosis affects the cells which produce mucus, sweat, and digestive juices. These secreted fluids are normally thin and slippery.

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In some people with cystic fibrosis, a defective gene causes the secretions to become sticky and thick. Before acting as a lubricant, the secretions plug up tubes, ducts, and passageways, especially in the lungs, and pancreas.

What is Cystic Fibrosis?

It is a hereditary disorder which affects the exocrine glands. It causes the production of abnormally thick and broad mucus, leading to the blockage of the pancreatic ducts, intestines, and bronchi and often resulting in respiratory infection.

What are the Symptoms of Cystic Fibrosis?

There are few symptoms of mucoviscidosis

Respiratory symptoms are :

1. A persistent cough that produces thick mucus

2. Wheezing

3. Breathlessness

4. Exercise intolerance

5. Repeated lung infections

6. Inflamed nasal passages or a stuffy nose

Digestive symptoms are:

1. Foul-smelling, greasy stools

2. Poor weight gain and growth

3. Intestinal blockage, particularly in newborns (meconium ileus)

4. Severe constipation

What are the Causes of Cystic Fibrosis?

There are some causes of cystic fibrosis:

• In mucoviscidosis, a defect (mutation) in a gene changes a protein that regulates the movement of salt in and out of cells. As the result, the mucus gets thick and sticky in the respiratory, digestive and reproductive systems, as well as increased salt in sweat is found.

• Many different defects can appear in the gene. This kind of gene mutation is associated with the severity of the condition.

What are the Risk Factors of Cystic Fibrosis?

There are mainly two risk factors of mucoviscidosis :

1. Family history

It is an inherited disorder, it runs in families.

2. Race

It occurs in all races, it is most common in white people of Northern European ancestry.

What are the Complications of Cystic Fibrosis?

There are few complications such as :

Respiratory system complications

Damaged airways 

Cystic fibrosis is one of the leading causes of bronchiectasis, a condition that damages the airways. It makes harder to move air in and out of the lungs and clear mucus from the airways (bronchial tubes).

Chronic infections

Dense mucus in the lungs and sinuses provides an ideal breeding ground for bacteria and fungi. People with mucoviscidosis have sinus infections, bronchitis or pneumonia.

Growths in the nose

Because the lining inside the nose is inflamed and swollen, it can develop soft, fleshy growths (polyps).

Coughing up blood 

Cystic fibrosis can cause thinning of the airway walls. As per the result, teenagers and adults with mucoviscidosis may cough up blood.

Pneumothorax

This condition when air collects in the space which separates the lungs from the chest wall is more common in older people with mucoviscidosis. It can cause chest pain and breathlessness.

Respiratory failure

Cystic fibrosis can damage the lung tissue so badly that it no longer works. Lung function usually worsens gradually, and it eventually can become life-threatening.

Acute Exacerbations

People with mucoviscidosis may experience worsening of their respiratory symptoms, such as coughing and shortness of breath, for several days to weeks. It requires treatment in the hospital.

Digestive system complications

Nutritional deficiencies

Thick mucus can block the tubes which carry digestive enzymes from your pancreas to your intestines. As without these enzymes, your body can’t absorb protein, fats or fat-soluble vitamins.

Diabetes

The pancreas makes insulin, which your body needs to use sugar. Mucoviscidosis increases the risk of diabetes. Around 30 % of people with mucoviscidosis develop diabetes by the age of 30-40.

Blocked bile duct

A tube which carries cattiness from your liver and gallbladder to your small intestine may become blocked and inflamed, leading to liver problems and sometimes gallstones.

Intestinal obstruction

It can happen to people with mucoviscidosis at all ages. Children and adults with cystic fibrosis are more likely than are infants to develop intussusception, a condition in which a section of the intestines folds in on itself like an accordion.

Distal intestinal obstruction syndrome (DIOS)

It is partial or complete obstruction where the small intestine meets the large intestine.

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What are the Preventions?

There are some preventions of mucoviscidosis :

• If you or your partner has close relatives with mucoviscidosis, you both want to undergo genetic testing before having children. The test, which is performed in a lab on a sample of blood, it can help to determine your risk of having a child with mucoviscidosis

• If you’re pregnant and the genetic test shows that your baby might be at risk of cystic fibrosis, then your doctor can conduct additional tests on your developing child.

• Genetic testing isn’t for everyone. As you decide to be tested, you should talk to a genetic counselor about the psychological impact the test results might carry.

How to Teat Mucoviscidosis?

The treatment of mucoviscidosis :

1. Preventing and controlling infections which occur in the lungs

2. Removing and loosening mucus from the lungs

3. Treating and preventing intestinal blockage

4. Providing adequate nutrition

Conclusion

Cystic fibrosis is a serious genetic condition which causes severe damage to the respiratory and digestive systems. In the above article, we have talked about cystic fibrosis its symptoms, causes, complications, risk factors, preventions and most importantly the treatment. If you feel and realize any of the above symptoms then consult a doctor as soon as possible.

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