CYSTIC FIBROSIS- AT A GLANCE

Cystic fibrosis is an inherited autosomal disease that affects tissues secreting mucus. These tissues line the respiratory, digestive and reproductive tract of patient along with sweat glands. This results in excessive secretion of thick and sticky mucus in the passage of these organs and thereby producing obstruction or blockage causing further complication.

The thick and sticky mucus in the respiratory system causes obstruction in the airway passage due to thick mucus and also facilitate or encourage frequent bacterial proliferation and infections in the lung tissues. Similarly, the abnormally thick mucus secreted in the digestive tract can impairs normal function of primarily pancreas and liver. The altered or deficient functioning of affected digestive organs due to cystic fibrosis causes difficulty in effective digestion of food and absorbs nutrients. The symptoms of cystic fibrosis is varied in patients and may at times is not expressed until the affected child reaches the adolescence.
The basic problem in all patients with cystic fibrosis is defective cystic fibrosis trans-membrane conductance regulator gene, which causes abnormal function of exocrine glands that secretes mucus or sweat. The sweat and parotid gland of the patients with CF secretes fluid which has more salt content than normal individuals.

Few significant symptoms in infants/child affected with cystic fibrosis are delay in passing meconium after birth; foul smelling and greasy stools, progressive weight loss, breathlessness, wheezing, persistent coughing, dehydration or abnormally salty sweat. In adults clogging of respiratory tract due to excessive secretion of mucus in patient with cystic fibrosis can cause frequent respiratory infections, breathing problem and permanent lung damage.

The patients with cystic fibrosis can also be diagnosed with various other medical problems like nose polyps, sinusitis, swelling and enlargement of fingers and toes, heart enlargement, pancreatic inflammation, pnuemothrax, liver diseases, abdominal pain and stones in gall bladder. Various other symptoms like protrusion of rectum through anus, delayed puberty and absence of Vas deferens in males are also observed in patients.

This disorder can be managed through prescribing antibiotics for lung infections in the patients. Similarly, bronchodilators, decongestants and drugs to combat inflammation are also suggested to manage the severity. Postural therapies through physiotherapy and airway clearance technique are few more interventions used to manage the symptoms of the disease. Supplementing the diets of the patient with pancreatic enzymes, vitamin A, D, E, and K and with food rich in protein and calories are also effective methods for managing the digestive tract complication arising in CF patients. Being an autosomal hereditary disorder of recessive gene, there is no available cure for cystic fibrosis. The various interventions or treatments provided to CF patients aim to treat the symptoms of respiratory and digestive impairments.