The CFTR* protein is found throughout the body, meaning that people with cystic fibrosis (CF) may experience a wide range of symptoms. These symptoms, and how severe they are, can vary dramatically between people with CF. This variation is the result of each individual’s CFTR mutations, genes, and surroundings.1
*CFTR = Cystic fibrosis transmembrane conductance regulator
People with CF may have more nasal polyps (fleshy growths inside their noses) than usual.1
Nasal polyps and mucus build up may lead to infection and sinusitis (the inflammation or swelling of the sinuses). This may cause a blocked nose, headaches, cough, and in some cases, the loss of smell.1,2
Salty sweat is a hallmark of CF.1
Normally, the CFTR protein reabsorbs salt before it reaches the skin surface.3 In people with CF, CFTR protein dysfunction may mean salt is not reabsorbed as it should be.1,4
Over time, people with CF may also experience skin irritation (eczema or dermatitis). This is thought to be influenced by diet and a reduced ability to absorb nutrients from food.5
The liver plays an important role in processing nutrients and keeping the blood clean and free from harmful substances.
The liver contains many small tubes. In CF, these may become blocked causing irritation and scarring. This scarring is known as cirrhosis, an advanced stage of liver disease.4
Cirrhosis makes it harder for the liver to work properly.
A man with CF can make sperm, but the tubes that sperm travel through (the vas deferens) may not have formed or be clogged with mucus. This may mean that although intercourse is normal, a man with CF may deliver little or no sperm.
Although a small percentage of men with CF may be able to conceive naturally, most are infertile.6 This infertility can also be seen in men who are CF carriers.1,4
In women with CF, thick sticky mucus in the reproductive system may reduce fertility.7
For those living with CF and planning to start a family, assisted reproduction techniques may be helpful and should be discussed with your doctor.8
As people with CF age, they may be at risk of reduced bone density (weakened bones) and fractures. This is thought to be the result of a number of factors including poor nutritional status (particularly reduced Vitamin D levels).9
For those living with CF it is important to follow a healthy balanced diet that is tailored to your individual needs.10 Check with your doctor, nurse or dietician for specific CF dietary advice.
1. O’Sullivan B, Freedman S. Cystic fibrosis. The Lancet 2009; 373(9678): 1891 ̶ 1904
2. Cystic Fibrosis Trust. Additional Complications. Available from: https://www.cysticfibrosis.org.uk/life-with-cystic-fibrosis/how-cf-affects-the-body/complications. Accessed June 2016.
3. Quinton P. Too much salt, too little soda: cystic fibrosis. Acta Physiologica Sinica 2007; 59(4): 397 ̶ 415.
4. Rowe S, et al. Mechanisms of Disease: Cystic Fibrosis. N Eng J Med 2005; 352:1999 ̶ 2001.
5. Wenk K, et al. Cystic Fibrosis Presenting With Dermatitis. Archives of Dermatology. 2010; 146(2): 171 ̶ 174.
6. Dodge JA. Male fertility in cystic fibrosis. The Lancet 1995; 346: 587 ̶ 588.
7. Davis P. Cystic Fibrosis Since 1938. American Journal of Respiratory and Critical Care Medicine 2006; 173: 475 ̶ 482.
8. Cystic Fibrosis Trust. Thinking of starting a family? (Fertility pack). 2016.
9. King SJ, et al. Reduced bone density in cystic fibrosis: DF508 mutation is an independent risk factor. Eur Respir J 2005; 25: 54 ̶ 61.
10. Cystic Fibrosis Trust. Nutrition - A guide for Children and Parents. 2013.