Understanding Cystic Fibrosis (CF): Causes, Symptoms, and Management

What is it?

Cystic Fibrosis is a genetic disorder that causes severe damage to the lungs and digestive system- such as pancreas, liver and intestines. Cystic Fibrosis affects cells that produce mucus, sweat and digestive juices. In people with CF, because of a mutated CFTR gene, these cells produce thick and sticky fluid that build up and plug the tubes, ducts and passages between the cells in these organs. .The CFTR gene controls movement of water and salt in and out of the body’s cells. CF complications may include bronchiectasis, chronic infections, nasal polyps, hemoptysis, pneumothorax, respiratory failure, nutritional deficiencies, diabetes,liver disease, intestinal obstruction, distal intestinal obstruction syndrome, infertility and more. Today, it is possible to test for CF mutation before pregnancy or before the baby is born.

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Additional names

This group contains additional names:
- CF

Signs & symptoms

Symptoms of CF vary, and can begin in different ages. Symptoms may include salty taste of the skin, wheezing, cough,shortness of breath, recurrent lung infections, stuffy nose and sinuses. greasy, foul-smelling stools, constipation, nausea, swollen abdomen, loss of appetite, poor weight gain, and delayed growth.

Diagnosis

Every newborn in the US today is screened for CF by a blood test checking for a higher than normal level of immunoreactive trypsinogen (IRT). Sweat chloride test is the most common way used for diagnosis of CF, which checks for salt in the sweat.

Treatment

Cystic fibrosis has no cure but many treatments make CF patients live longer and better. Medications that target gene mutation including a new medication that combines three drugs to treat the most common genetic mutation causing CF and is considered a major achievement in treatment. Antibiotics are prescribed for infections, NSAIDs to lessen swelling in the lung airways,Mucus-thinning drugs, inhaled bronchodilators,oral pancreatic enzymes, stool softeners, acid- reducing medications and diabetes treatment.

☝️ This is not a substitute for professional medical advice. Please consult with your physician before making any medical decision.

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Source of Information - We analyze big data of more than 23 million patients de-identified medical records from the following sources:

National Institution of Health ∙ World Health Organization ∙ University of Illinois hospital