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Cystic Fibrosis - Symptom, Treatment And Causes

Last Updated: Oct 18, 2021

What is Cystic fibrosis?

Cystic fibrosis is a disorder which can cause chronic damage to the digestive system, lungs and other organs of the body. Cystic fibrosis affects the cells which produce digestive juices, sweat and mucus. These fluids are slippery and thin. People who have Cystic fibrosis usually have thick and sticky secretions. This is caused by a defective gene. Instead of becoming a lubricant, the secretions plug up the passageways, ducts and tubes in the pancreas and lungs.

Can CF go away?

There is no cure for Cystic Fibrosis, but early treatment can minimize the effect of symptoms. A proper diagnosis of the disease is necessary in order to decide on a proper treatment plan, which will reduce the complications and lead to an improved way of life.

What is end-stage Cystic Fibrosis?

The end-stage Cystic Fibrosis referred to as the terminal stage, is characterized by the following:

  • Lung disease, It includes cysts, abscesses, and fibrosis of the lungs which may lead to chronic progressive pulmonary disease and respiratory failure.
  • Several systemic diseases such as diabetes mellitus, pancreatic insufficiency, and hepatobiliary disease.
  • Worsening of Cystic Fibrosis-associated symptoms such as cough, fatigue, and dyspnea.

The end-stage Cystic Fibrosis is followed by palliative care.

What causes cystic fibrosis?

During Cystic fibrosis a mutation in the gene transform a protein which regulates movement in and out of these cells. This results in sticky and thick mucus in the reproductive, digestive and respiratory system. A lot of defects can occur which usually depends on the severity of this disorder. Children can develop Cystic fibrosis if one or both the parent had this disease.

Risk factors of Cystic fibrosis:

  • Family history- Since Cystic fibrosis is a hereditary disease, it tends to run in the family.
  • Race- Though Cystic fibrosis can affect people of all races, it is more common in people belonging to the Northern European ancestry.

Is Cystic Fibrosis painful?

Pain is one of the symptoms associated with Cystic Fibrosis, with an occurrence of 70% among the affected population. Pain mainly includes headaches, gastrointestinal related and chest pain, with the location being inconstant. These symptoms need to be treated by medications, otherwise have a negative impact on quality of life.

Does Cystic Fibrosis get worse with age?

Cystic Fibrosis is a congenital condition that results in infections related to the lungs as well as the digestive system. It leads to poor functioning of the affected organs. Symptoms generally start to appear in the early childhood phase, followed by the worsening of the same as the age advances. It causes increased damage to the lungs and the digestive system.

Is CF contagious?

As Cystic Fibrosis is a hereditary disease, it is being acquired congenitally i.e since birth. Hence it is not contagious in any condition. It is caused by the inheritance of Cystic Fibrosis genes from both of the parents. Mutation in chromosome number 7 leads to this condition, so the chances of it being contagious are negligible.

Does CF make you tired?

Fatigue is one of the most common symptoms associated with the Cystic Fibrosis condition, which debilitates the affected person. It relates to the chronic or severe state of the disease, accompanied by a decreased efficiency of life. However, this symptom has not been found with an effective treatment yet.

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Complications of Cystic fibrosis:

  • Bronchiectasis (Damaged airways)- Cystic fibrosis can cause bronchiectasis. This condition damages the airways and makes it difficult to clear the mucus form the bronchial tubes and move air out of the lungs.
  • Chronic infections- Mucus n the sinuses and lungs are an ideal breeding spot for fungi and bacteria. People who have Cystic fibrosis are more prone in developing pneumonia, bronchitis and sinus infections.
  • Nasal polyps (growths inside the nose)- Since the lining of the nose tends to be swollen and inflamed, it can develop fleshy and soft growth (polyps).
  • Hemoptysis (coughing blood)- Cystic fibrosis can sometimes cause thinning in the airway walls. Teenagers and adults who have Cystic fibrosis can also cough up blood in severe cases.
  • Pneumothorax- In this condition the air starts to collect in the empty space which separates the chest walls from the lungs. Pneumothorax can also cause breathlessness and chest pain.
  • Nutritional deficiencies- Many times, thick mucus can create obstruction the tubes which carry enzymes aiding in digestion from the pancreas to the intestines. In such cases, the body fails to absorb fat-soluble vitamins, fats and proteins.
  • Distal intestinal obstruction syndrome (DIOS)- This condition is characterized by the complete or partial blockage where the large intestine meets the small intestine.
  • Summary: Cystic Fibrosis is a congenital condition that results in infections related to the lungs as well as the digestive system. There is no cure for this disease, but early treatment can minimize the effect of symptoms. A proper diagnosis of the disease is necessary in order to decide on a proper treatment plan, which will reduce the complications and lead to an improved way of life.

References

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Written ByDr. Mool Chand GuptaMD - Pulmonary,MD PULMONARY,DTCDPulmonology
Reviewed By
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Reviewed ByDr. Bhupindera Jaswant SinghMD - Consultant PhysicianGeneral Physician
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