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Clinical Consequences

Other Effects of Cystic Fibrosis

CF can also lead to clinical consequences in several other body systems.

Sinusitis and Nasal Polyps^2,3

Two common complications among patients with CF involving the upper respiratory tract include sinusitis and nasal polyps.

The sinuses are air-filled spaces behind the eyes, nose, and forehead that produce mucus and help keep the lining of the nose moist. When the sinuses become swollen, they get blocked with mucus and can become infected. Upon radiograph, sinusitis is present in more than 90% of patients with CF; however, acute or chronic symptoms of sinusitis occur in few than 10% of children and in about 24% of adults.

Nasal polyps, which are small noncancerous growths, occur in 10% to 25% of patients with CF. Polyps may be removed surgically to prevent nasal obstruction, but the recurrence rate is 50% to 90%.

Infertility

Late onset of puberty is common in both males and females with CF, probably secondary to the effects of chronic lung disease and inadequate nutrition for reproductive gland function.¹

• Approximately 95% of male patients with CF have a low sperm count or azoospermia because the ducts of the testis (the vas deferens) have developed abnormally, thus blocking the passage of sperm.¹
• In some women, cervical secretions may be too thick and cause decreased fertility. Otherwise, reproductive function is not affected.^1,2
  

Pregnancy Complications^18,22

Normal changes that occur during pregnancy may adversely affect a woman with CF; however, survival among pregnant women is not impaired.

• Increased demand on the respiratory system from pregnancy may be problematic in a pregnant woman with severe lung disease.
• Blood volume and cardiac output may rise by as much as 50% toward the end of pregnancy. These changes could precipitate symptoms of right-sided heart failure in the presence of severe lung disease.
• Approximately 25% of pregnancies in women with CF result in preterm labor and delivery.
• The increased nutritional demands of pregnancy may be difficult to attain. Women with CF-related diabetes may have difficulty controlling their blood sugar.

Abnormal Sweating

The excessive loss of sodium and chloride through sweating puts patients with CF at risk for electrolyte abnormalities.

• Increased loss of sodium, chloride, and potassium in the sweat over a prolonged period of time may lead to electrolyte depletion and chronic metabolic alkalosis. Patients may present with drowsiness, confusion, muscle spasms, or other symptoms.
• Children who quickly lose a large amount of salt in sweat (acute salt depletion) are at risk for dehydration and a rapid drop in blood pressure. Episodes may be precipitated by acute illness or fever, among other causes.
• CF patients lose a significant amount of sodium and chloride when exposed to heat or during exercise. Care must be taken to replace fluids and electrolytes to avoid dehydration or problems with regulating body temperature.

Anaemia and Cancer²³

Iron-deficiency anemia is present in up to two thirds of patients with CF.

The cause of anemia in patients with CF is probably multifactorial, including inadequate dietary iron intake, blood loss, iron malabsorption, and chronic infection.

Several case reports and one study have suggested that adult patients with CF may have an increased risk of cancer, particularly digestive tract tumors.

Additionally, a large retrospective study suggests that patients with CF may present a higher risk for digestive tract cancers, with the greatest risk among patients with CF aged 20 to 29 years.

References:
1. Boucher RC. Cystic Fibrosis. In: Kasper DL, Braunwald E, Fauci AS, Hauser SL, Longo DL, Jameson JL et al., editors. Harrison's Internal Medicine. 16th ed. New York: McGraw-Hill; 2005. p. 1-9.
2. Aitken M, Fiel SB, Stern RC. Cystic Fibrosis: Respiratory Manifestations. In: Taussig LM, Landau LI, editors. Pediatric Respiratory Medicine. 1st ed. St. Louis: Mosby, Inc.; 1999. p. 1-47.
3. Welsh M. Cystic Fibrosis. In: Goldman L, Ausiello D, eds. Cecil Textbook of Medicine. 22nd ed. Philadelphia: WB Saunders; 2004. p. 1-22.
18. Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest 2004 January;125(1 Suppl):1S-39S.
22. Goss CH, Rubenfeld GD, Otto K, Aitken ML. The effect of pregnancy on survival in women with cystic fibrosis. Chest 2003 October;124(4):1460-8.
23. Neglia JP, FitzSimmons SC, Maisonneuve P et al. The risk of cancer among patients with cystic fibrosis. Cystic Fibrosis and Cancer Study Group. N Engl J Med 1995 February 23;332(8):494-9.