Cystic Fibrosis (CF)

Cystic Fibrosis (CF) is a genetic disorder that mostly affects the lungs and causes abnormal amounts of viscous mucus production. Other symptoms include frequent chest infections, poor weight gain, poor growth & weight gain, clubbed fingers and toes, shortness of breath and salty-tasting skin. CF also affects the pancreas, liver, kidneys and intestine. CF is most common amongst people of Northern European ancestry, and affects around 1 in every 3,000 newborns. The average life expectancy for those with CF is 42 – 50 years old, and there is no known cure for CF.

CF is caused by mutations in the gene for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. People can be carriers if they have a single working copy of the gene. People with CF are often on one or more antibiotics at all times in order to prevent infection. There are also relationships between CF and diabetes, as both conditions affect the islets of Langerhans in the pancreas, which is and endocrine cell responsible for the production of insulin. Poor vitamin D uptake also leads to osteoporosis.

Though many antibiotics are generally safe, antibiotics in the aminoglycoside class can cause hearing loss, loss of balance and renal failure with long-term use. Antibiotics may also become ineffective over time due to resistance. Other treatments for CF include vitamin supplements, pancreatic enzyme replacement, lung transplantation if lung function continues to worsen, high protein diets and chest percussion and postural drainage to help keep the airways clear for more short-term relief.

Suffering from CF means an increased likelihood of developing bowel problems, diabetes, infertility, respiratory problems, pneumonia, arthritis, sinusitis and lots more. Though a relatively rare disease, CF is one of the most common life-shortening inherited diseases.

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