Symptoms of Cystic Fibrosis

Cystic fibrosis is one of the commonest inherited disorders. It affects the secretory glands of our body. Normally, the secretion of the sweat glands, mucus glands and glands producing digestive enzymes are thin in consistency. But in Cystic Fibrosis (CF), these secretions become thick and sticky. Therefore, the ducts which carry these sticky substances become blocked. This condition may turn out to be life threatening if the airway passage in the lungs gets obstructed. The symptoms of Cystic Fibrosis depend upon the area involved.

The major symptoms of Cystic Fibrosis are:

Cystic Fibrosis

Source – rogercarr

• Cough – persistent and intractable cough is the commonest symptoms in CF involving the lung. Usually the intensity of cough is low. But as the lung tissues go on fibrosing, the period of cough is also increased. It is often mixed with copious thick mucus. Sometimes, it becomes difficult to expel out the sputum due to heavy thickness.

• Wheeze – as the thick sputum partially or completely blocks the small airways, there is turbulence of air during flowing through these obstructed passages. The turbulence of air produces a wheeze and the person standing beside the patient can hear the sound.

• Pneumonitis – occasionally, the mucus becomes so thick that it makes a pocket in the lung tissue where gaseous exchange is not possible. This is the area where bacterial growth occurs which can lead to repeated lung infection. There is fever and prostration besides cough and expectoration. Sometimes, the mucus is mixed with blood. Paranasal sinuses are also involved leading to headache, pain in the affected side of the face, pain in the ear, sneezing, running nose and difficulty in vision. Sometimes, small tumor like protrusion can be seen in the nose (nasal polyp) which is the source of bleeding from the nose.

• Passage of smelly stools – in CF, the secretion from the digestive glands are either deficient or the secretion may be so thick that it is unable to help in complete digestion of food material. As the fat component of the ingested food in incompletely digested, the stool becomes foul smelling, sticky and is not easy to evacuate out completely.

• Generalized weakness – inadequate and defective absorption of the nutrients will lead to malnutrition. The patient will experience severe lethargy. In children, growth retardation and difficulty in psychological excellence is noticed. The hair becomes thick and lusterless and there is brownish discoloration.

• Abdominal fullness – undigested food material produces gas and this may cause permanent belching. Partial blockage can lead to abdominal cramps.

• Rectal prolapse – in severe cases of CF, the rectum comes out from its normal place and hangs outside as a mass of flesh.

• Delayed puberty – impaired functioning of male and female glands producing androgenic hormones can lead to delayed puberty. In males, congenital absence of Vas deferens is seen in some cases of CF.

• Constant sweating and salty skin – impaired functioning of sweat gland leads to a condition where the skin tastes like salt.

Apart from these symptoms of cystic fibrosis, CF can lead to Diabetes (due to faulty functioning of pancreas), Gallstones (impaired functioning of gallbladder) and liver diseases.

Author – Dr. Anupam Biswas