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Cystic Fibrosis ? More Than Just Another Lung Disorder

Cystic fibrosis is chronic hereditary disease that affects more than 70,000 people worldwide in which excessive mucus accumulates in the lungs, particularly the bronchioles, leading to wheezing and shortness of breath. The disease affects not only the lungs but also the digestive system, particularly the pancreas and also affects the reproductive system.

Some symptoms of cystic fibrosis

Experts working with the 1955 established cystic fibrosis foundation list the following as the major symptoms of this systemic disorder: -

A very salty tasting skin due to defective functioning of the sweat glands.

Persistent coughing due to phlegm accumulation in the terminal bronchioles found in the lungs.

Frequent lung infections with Staphylococcus aureus, Haemophilus influenzae Pseudomonas aeruginosa, and Mycobacterium avium among other infections.

Poor growth and or weight and height gain due to poor absorption of nutrients by the gastrointestinal tract.

Frequent greasy bulky stools and or difficult bowel movements again associated with pancreas malfunction.

Infertility in males due to congenital absence of the vas deferens

Blood coagulation disorders may result from vitamin k malabsorption disorders.

Coughing blood due to poorly formed mucus membranes in the lungs.

Liver and pancreas complications due to blockage.

Diagnosis for cystic fibrosis

Cystic fibrosis is a hereditary disorder. Parents who are carriers have a more likelihood of bearing children with cystic fibrosis.

Genetic screening of the neonates – Screening newly born children for the presence of the mutant gene that predisposes individuals to cystic fibrosis can be used as a diagnostic procedure.
Sweat test – The simplest way to determine whether an individual has this disease or not is by means of this test which determines salt concentration in sweat.
Pulmonary function tests – These are a set of tests that determine the proper functioning of the lungs.
Blood tests – Tests that determine the concentration of various metabolites, liver function abnormalities and vitamin deficiency may help in diagnosis.

Management of cystic fibrosis

Cystic fibrosis like aforementioned is chronic, systemic and hereditary. Various management options exist including: -

Use of antibiotics to treat lung infections associated with it.

Lung transplantation to replace defective lungs with new ones.

Use of pancreatic enzyme supplements that replace trypsin, chymotrypsin, pancreatic amylase and pancreatic lipase.

Increased in take of vitamin and calcium supplements

Therapeutic administration of the growth hormone and or insertion of a feeding tube to reverse stunted growth.

Gene therapy in which missing or mutant genes are replaced by insertion of functional ones into cells and tissues.

Additional resources

Cystic fibrosis foundation

Wikipedia online encyclopedia

Cystic fibrosis trust