Other parts of the body

Cystic fibrosis also affects many other parts of the body


The cystic fibrosis transmembrane conductance regulator (CFTR) protein is found on the surface of cells in many organs and tissues throughout the body. This means that people with cystic fibrosis (CF) can experience a wide range of symptoms. These symptoms, and how severe they are, can vary a lot between people with CF. This variation is the result of each individual person’s CFTR mutations (their genotype), genes, and their surroundings.1

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Sinuses

  • 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
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Skin

  • 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
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Cirrhosis of the liver​

  • 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.
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Reproductive system

  • 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
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Reduced bone density

  • 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.
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References

  1. O'Sullivan B, Freedman S. Cystic fibrosis. Lancet. 2009; 373(9678): 1891–1904.

  2. Cystic Fibrosis Trust. Additional Complications. Available at: www.cysticfibrosis.org.uk/what-is-cystic-fibrosis/how-cf-affects-the-bo….

  3. Quinton P. Too much salt, too little soda: cystic fibrosis. Acta Physiologica Sinica. 2007; 59(4): 397–415.

  4. Rowe SM, Miller S, Sorscher EJ. Cystic Fibrosis. N Engl J Med. 2005; 352(19): 1992–2001.

  5. Wenk KS, Higgins KB, Greer KE. Cystic fibrosis presenting with dermatitis. Arch Dermatol. 2010; 146(2): 171–174.

  6. Dodge JA. Male fertility in cystic fibrosis. Lancet. 1995; 346: 587–588.

  7. Davis P. Cystic fibrosis since 1938. Am J Respir Crit Care Med. 2006; 173(5): 475–482.

  8. Cystic Fibrosis Trust. Thinking of starting a family? A guide for adults with cystic fibrosis and their partners. Fertility pack. 2016. Available at: www.cysticfibrosis.org.uk.

  9. King SJ, Topliss DJ, Kotsimbos T, et al. Reduced bone density in cystic fibrosis: DeltaF508 mutation is an independent risk factor. Eur Respir J. 2005; 25: 54–61.

  10. Cystic Fibrosis Trust. Nutrition factsheet: a guide for children and parents. April 2013. Available at: www.cysticfibrosis.org.uk.

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