What are the symptoms?

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CF (cystic fibrosis) causes the body's exocrine glands to make thick, sticky mucus. Most CF symptoms are caused by this mucus. Symptoms cause trouble with four main body functions: breathing, digestion, reproduction, and sweating.

Here are the most common symptoms of CF:

Breathing
The CF symptoms related to breathing are:

• Frequent coughing that sometimes brings up thick mucus
• Frequent lung infections such as bronchitis or pneumonia
• Wheezing or shortness of breath
• Nasal polyps (small growths of flesh in the nose)

The purpose of mucus in the lungs is to capture dust and germs and clear them away. This is how normal mucus helps to keep the lungs healthy. The thick, sticky mucus of CF cannot help keep the lungs healthy. It clogs the bronchial tubes in the lungs. This makes it harder to breathe. The mucus also traps bacteria in the lungs, which can lead to infection. The mucus even allows some types of bacteria to grow that don't usually affect healthy people. When infections occur over and over, in time, the lungs can be damaged. Lung infections are the most serious CF problem.

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Digestion
CF affects nearly the whole digestive tract. The most common symptoms are:

• "Failure to thrive" – poor weight gain and growth, even with a huge appetite
• Ongoing diarrhea
• Bulky, greasy, foul-smelling stools
• Stomach pain due to gas in the intestines
• Blocked first bowel movement in babies

The pancreas is an organ behind the stomach. It makes enzymes. Enzymes are proteins that the body uses to break down the food we eat. Without enzymes, food cannot be digested well. In CF, the thick, sticky mucus blocks enzymes from reaching the food in the intestines. This makes it very hard for the body to absorb nutrients. As a result, people with CF may be poorly nourished, have trouble gaining weight, and may not grow normally. About 93 out of 100 people with CF have this problem. Far fewer people have mucus that stops bile from leaving the liver. Others have mucus that keeps stool from moving normally.

Meconium is a baby's first bowel movement. It is thick, greenish-black, and sticky. Healthy infants pass this stool within the first 24-48 hours after birth. About 15 out of 100 newborns with CF cannot not pass this stool. This is known as meconium ileus. Ileus means that the baby's intestine is blocked by meconium that is more thick and sticky than normal. Meconium ileus is caused by a lack of pancreatic enzymes. Meconium ileus is usually a sign that the baby has CF. Meconium ileus is relieved with an enema or, in some cases, with surgery.

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Reproduction
CF affects the ability of men and women to become parents:

• Most men are sterile (unable to father a child).
• Women have very thick, sticky cervical mucus that makes it harder to get pregnant.

Men with CF make normal sperm. But the sperm cannot mix with semen and leave the body because the tube it travels through is blocked by the thick mucus caused by CF. In other words, the semen of most men with CF contains no sperm. This means most men with CF are not able to father children. It is not safe to assume that all men with CF are sterile. In a few men with CF, the sperm is not blocked.

Women with CF can get pregnant. However, it can be harder for a woman with CF to conceive. The problem is that the exocrine glands in the vagina make cervical mucus that is more thick and sticky than normal. It is hard for sperm to get through this mucus barrier to reach the egg.

Another problem is that some women with CF do not have regular periods. They may also ovulate (release eggs from the ovaries) less often than normal. While it can be more difficult for women with CF to get pregnant, it is not impossible – about 100 women with CF get pregnant every year.

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Sweat
When parents kiss their child, they may notice a classic sign of CF:

• Salty-tasting skin

Sweat glands are the only exocrine glands that do not make thick, sticky mucus. Instead, they make sweat that has too much salt. People with CF have much more salt in their sweat than other people do. This is not usually a problem, since most people get plenty of salt from food. It only becomes a problem if too much salt is lost through sweat. The balance of minerals in the blood can change.2 This can cause:

• Tiredness
• Weakness
• Muscle cramps
• Vomiting
• Dehydration (losing too much water from the body)
• Heat stroke

This is most likely to happen at times when people sweat the most: in hot weather, when exercising, or with a fever. Replacing lost salt is easy: just eat more salty foods and drink extra water.

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References

Cunningham JC, Taussig LM, An Introduction to Cystic Fibrosis for Patients and Families. Fifth edition. Cystic Fibrosis Foundation; 2003.

National Heart Lung and Blood Institute Web site. http://www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_what.html . Accessed May 13, 2008.

Strausbaugh SD, Davis PB; Cystic fibrosis: a review of epidemiology and pathobiology. Clin Chest Med. 2007;28:279-288.

Cystic Fibrosis Foundation Web site. http://www.cff.org/AboutCF/Faqs/ . Accessed May 13, 2008.

National Center for Biotechnical Information Web site. http://www.ncbi.nlm.nih.gov/books/bv.fcgi?highlight=&rid=gnd.section.242 . Accessed May 13, 2008

Fact Sheet: Respiratory--What You Should Know About Germs. Cystic Fibrosis Foundation Web site. http://www.cff.org/UploadedFiles/LivingWithCF/StayingHealthy/Germs/WhatYouShouldKnow/WhatYouShouldKnowAboutGerms%2Epdf . Accessed May 13, 2008.

Cystic Fibrosis Foundation Web site. http://www.cff.org/AboutCF/  Accessed May 13, 2008.

Robertson MB, Choe KA, Joseph PM; Review of the abdominal manifestations of cystic fibrosis in the adult patient. Radiographics. 2006;26(3):679-690.

Turcios NL. Cystic fibrosis: an overview. J Clin Gastroenterol. 2005;39(4):307-317.

MedLine Plus Medical Encyclopedia [Internet]. Bethesda, MD: National Library of Medicine (US); Meconium. [updated 2008 Apr 28; Cited 2008 May 19]; [about 2p.] Available from http://www.nlm.nih.gov/medlineplus/ency/imagepages/9616.htm  

American Academy of Family Physicians Web site. http://www.aafp.org/afp/991101ap/2043.html . Accessed May 19, 2008.

MedicineNet Web site. http://www.medterms.com/script/main/art.asp?articlekey=6372 . Accessed May 19, 2008.

Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS/Caremark company.  All rights reserved.
This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.
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