Early symptoms of cystic fibrosis

The early symptoms of cystic fibrosis (CF) usually appear during the first year of life – though they may also develop until the teenage years and even later in life. One of the very first signs that parents observe is that the baby’s skin tastes salty when they kiss it. Generally speaking, though, the symptoms can vary from person to person depending on the organs and systems affected.

Cystic fibrosis symptoms

Organ affected


System affected



·         Sinusitis.


·         Persistent, mucus-producing cough.

·         Wheezing.

·         Shortness of breath.

·         Exercise intolerance.

·         Recurrent lung infections.

·         Inflamed nasal passages.

·         Stuffed nose.


·         Thick, sticky mucus build-up.

·         Bacterial infection.

·         Widened airways.


·         Foul-smelling, greasy stool.

·         Failure to thrive.

·         Intestinal blockage.

·         Severe constipation.


·         Salty sweat.


·         Absence of a tube that conveys sperm from the testes to the penis (vas deferens).

·         Difficulty getting pregnant.


·         Blocked biliary ducts.


·         Dehydration.

·         Increased heart rate.

·         Fatigue.

·         Weakness.

·         Low blood pressure.

·         Heat stroke.

·         Death (rarely).


·         Blocked pancreatic ducts.




·         Inability to fully absorbed nutrients.



Reproductive organs

·         Complications.



Regardless of the type and severity of the early symptoms of cystic fibrosis, all cases have something in common – a hereditary abnormality in the CFTR gene, which produces a protein that controls the glands that secret sweat and mucus. Children who inherit one defective CFTR gene from each parent will develop CF; those who inherit one normal and one defective gene will be carriers (many of the 10 million people in the United States who are carriers are not aware of it).

Cystic fibrosis inheritance pattern

Father with one faulty CFTR gene (carrier)

Mother with one faulty CFTR gene (carrier)

Child with two normal CFTR genes does not have CF

Child with one faulty CFTR gene (carrier)

Child with one faulty CFTR gene (carrier)

Child with two faulty CFTR genes has CF



If each parent has one normal and one defective CFTR gene, each child has a 25% chance of inheriting two normal genes; a 50% chance of being a carrier; and a 25% chance of being born with cystic fibrosis. Research has suggested that the CFTR protein can affect the body in ways other than causing thick, sticky mucus and extremely salty sweat – which could account for the symptoms, as well as such complications as the following:

·         Pneumothorax.

·         Bronchiectasis.

·         Bronchitis.

·         Pneumonia.

·         Nasal polyps.

·         Pancreatitis.

·         Rectal prolapsed.

·         Liver disease.

·         Diabetes.

·         Gallstones.

·         Low bone density.

·         Widening and rounding of the tips of fingers and toes.

·         Chronic infections.

·         Coughing up blood.

·         Respiratory failure.

·         Distal intestinal obstruction syndrome.

·         Osteoporosis.

·         Electrolyte imbalances.

All 50 states in the U.S. screen newly born infants for cystic fibrosis with a genetic test or a blood test – both of which can be confirmed with a sweat test that measures the level of sodium chloride (salt) in the skin. In the sweat test, an area of the skin is made to sweat with a chemical called pilocarpine and a small amount of electric current. The area is covered with a gauze pad and wrapped in plastic for about 40 minutes, after which the sweat collected is analyzed. Other tests include x-rays of the chest and sinus, lung function tests, and sputum culture. Additionally, prenatal screening and CF carrier testing may be available in case of people with a family history of cystic fibrosis.

Treatment for CF


·         CF specialist.

·         Nurses.

·         Physical therapists.

·         Dietitians.

·         Social workers.


·         Antibiotics.

·         Mucus-thinning drugs.

·         Bronchodilators.

Chest physical therapy

·         Loosen mucus from the lungs by either pounding on the chest and back with cupped hands, or using one of these mechanical devices:

·         Electric chest clapper.

·         Inflatable therapy vest.

·         A small handheld device that the patient exhales through.

·         A vibrating mask.

Exercise and diet

·         Aerobic exercise plus a high-salt diet or salt supplements.

Advanced lung disease

·         Oxygen therapy.

Pulmonary rehabilitation

·         Exercise training.

·         Nutritional counseling.

·         Education on lung disease or condition and how to manage it.

·         Energy-conserving techniques.

·         Breathing strategies.

·         Psychological counseling and/or group support.

Digestive problems

·         Oral pancreatic enzymes.

·         Supplements of vitamins A, D, E, and K.

·         High-calorie shakes.

·         A feeding tube.


·         Nasal polyp removal.

·         Endoscopy and lavage.

·         Bowel surgery.

·         Lung transplant.


Cystic fibrosis affects over 30,000 and young adults in the U.S. Fortunately, screening for CF in newborns can help healthcare providers diagnose – even before the early symptoms of cystic fibrosis appear – and treat the condition accordingly.

Related: Respiratory Supplies for the treatment of Cystic Fibrosis