Patient education: Cystic fibrosis (CF)
What is cystic fibrosis? — Cystic fibrosis is a disease of exocrine gland function that involves multiple organ systems. It is an autosomal recessive disorder caused by an abnormal gene called – cystic fibrosis transmembrane conductance regulator gene (CFTR). The symptoms are associated with the production of hyper viscous secretions of exocrine glands. This causes thick mucus and other fluids to build up and clog different parts of the body, including the lungs, pancreas, liver, and intestine.
The thick mucus in the lungs causes people with cystic fibrosis to get frequent lung infections. Over time, these infections damage the lungs. The thick fluids in the pancreas and liver keep the intestine from absorbing certain nutrients from food. This affects a child’s growth and causes abnormal bowel movements and deficiency of important vitamins – A, D, E, K.
Cystic fibrosis is a lifelong condition. As of now, doctors can’t cure the disease, but they can use different treatments to help with symptoms.
What are the symptoms of cystic fibrosis? — People can have different symptoms at different times. Most people start having symptoms as a baby or young child. A few people start having symptoms as teens or adults. A person’s symptoms usually get worse over time.
Common symptoms of cystic fibrosis include:
● Not growing or gaining weight normally
● A long-lasting cough – The cough usually brings up mucus
● Trouble breathing or breathing that sounds like whistling (wheezing)
● Frequent infections of the lungs or sinuses
● Skin that tastes salty (for example, you might taste salt when you kiss your child)
● Belly pain, diarrhea, or constipation (trouble having bowel movements), rarely rectal prolapse
● Bowel movements that are oily, bad-smelling, and float in the toilet bowl
● Infertility and delayed secondary sexual development in both sexes.
Is there a test for cystic fibrosis?
● A “sweat test” – During this test, the doctor makes a person sweat and then measures the amount of salt in the sweat.
● Blood tests to check for the abnormal gene
Some pregnant women choose to test their unborn babies for the disease.
Most commonly found Bacteria in CF – Infants – Staphylococcus aureus Adults – Pseudomonas aeruginosa
How is cystic fibrosis treated? – People with cystic fibrosis can have different treatments to help with symptoms and prevent further problems.
Role of Bacteriophage therapy in CF – A bacteriophage is a type of virus that infects bacteria. In fact, the word “bacteriophage” literally means “bacteria eater”. Phage therapy can be very effective in CF because of the increased risk of infection and also recurrent infections. As patients with CF need lifelong antimicrobial treatment, developing resistance to antibiotics is quite common. Therapy with phages has some unique advantages over antibiotics and can also be used as combinational therapy. Some evidence shows that Bacteriophages can also be used as a prophylactical and preventive treatment. Bacteriophage therapy doesn’t offer 100 % results.
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References 1.Uptodate-https://www.uptodate.com/contents/cystic-fibrosis-overview-of-gastrointestinal-disease?search=cystic%20fibrosis&source=search_result&selectedTitle=2~150&usage_type=default&display_rank=2 2.Uptodate-https://www.uptodate.com/contents/cystic-fibrosis-the-basics?search=cystic%20fibrosis&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1 3.Amboss-https://www.amboss.com/us/library#xid=L40wPT&term=Cystic+fibrosis Emerson J, Rosenfeld M, McNamara S, Ramsey B, Gibson RL. Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosis. Pediatr Pulmonol. 2002; 34(2): pp. 91–100. doi: 10.1002/ppul.10127. 4.Merck manuel-https://www.msdmanuals.com/home/children-s-health-issues/cystic-fibrosis-cf/cystic-fibrosis-cf 5. https://emedicine.medscape.com/article/1001602-overview