Cystic fibrosis

Symptoms

What are the main symptoms of cystic fibrosis?

Individuals with cystic fibrosis (CF) can have a variety of symptoms. A common symptom of cystic fibrosis is frequent lung infections and persistent (constant) cough producing phlegm at times. Wheezing and shortness of breath occur frequently. If one were to lick the skin of a person with cystic fibrosis, it would taste salty. Most people with cystic fibrosis have pancreatic insufficiency (inadequate function of the pancreas) causing poor growth and weight gain, as well as frequent greasy, bulky stools and difficulty with bowel movements. About 15-20% of newborns with cystic fibrosis are born with meconium ileus, which is a bowel obstruction in the part of the small intestine known as the ileum. Individuals with CF are more likely to develop cystic fibrosis-related diabetes mellitus (CFRDM). Also, the liver of people with CF may not work correctly over time. Many men with cystic fibrosis experience infertility and can't have children naturally due to missing vas deferens (the connection between the testes and the opening in the penis). This symptom is called congenital absence of vas deferens (CBAVD). If you have cystic fibrosis, the Cystic Fibrosis Foundation can help you find a treatment center.

References
  • National Library of Medicine. "Cystic fibrosis - Genetics Home Reference." August 2012. Web. 2 June 2016 Genetics Home Reference
  • Moskowitz S.M. et al. "CFTR - related disorders." Gene Reviews. 19 February 2008. Web GeneReviews
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Are there other diseases that look like cystic fibrosis?

Are there any unusual symptoms of cystic fibrosis?

Are there other diseases that look like cystic fibrosis?

Many conditions that lead to frequent lung infections and liver disease can appear similar to cystic fibrosis. Some of these conditions include dysphagia, severe combined immunodeficiency, airway anomaly, primary ciliary dyskinesia, Shwachman-Diamond syndrome, and biliary atresia. However, due to newborn screening programs in the United States, in most cases, cystic fibrosis can be found early in a newborn's life. If you suspect you or a person you know has cystic fibrosis, talk to your doctor about testing for cystic fibrosis.

References
  • National Library of Medicine. "Cystic fibrosis - Genetics Home Reference." August 2012. Web. 2 June 2016 Genetics Home Reference
  • Moskowitz S.M. et al. "CFTR - related disorders." Gene Reviews. 19 February 2008. Web GeneReviews
Are there any unusual symptoms of cystic fibrosis?

Often relatives refer to babies with cystic fibrosis as "salty babies". Since newborns with cystic fibrosis have additional sodium in their sweat, often times, people report the babies tasting "salty" when they kiss them. If you notice a new baby in the family has this "salty" taste, talk to the pediatrician about the best way to get cystic fibrosis testing in your area.

References

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