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Pediatric Cystic Fibrosis (CF)

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Summary

Pediatric cystic fibrosis is a genetic condition that affects mucus production, leading to lung infections and breathing difficulties.

Expanded Overview

For those with cystic fibrosis, a defective gene causes a thick, sticky buildup of mucus in the lungs. Normally, mucus is a slippery, watery substance that keeps the lining of organs moist. Mucus also helps remove inhaled dust, bacteria and other small particles. When mucus is too thick, it traps these particles instead, leading to infections in the lungs.

In addition to affecting the lungs, cystic fibrosis causes problems with the pancreas, liver, intestines and sex organs. Because of the thick, stagnant mucus trapped in the nose, children with cystic fibrosis are also prone to sinus infections.

Thick mucus in the pancreas prevents the release of digestive enzymes that help the body break down food and absorb nutrients, including fats and proteins. That can lead to vitamin deficiency and malnutrition.

Causes

Cystic fibrosis is a congenital disease that is present at birth. It occurs when a child inherits a faulty gene from each parent. The parents likely do not have the disease.

Symptoms

Most babies are screened for cystic fibrosis as a part of newborn screenings in the hospital. In Texas, newborns are screened for a protein that can indicate cystic fibrosis. Most children are diagnosed by the age of 2. The severity of the disease can differ from child to child, even in the same family.

Symptoms in infants (birth to 1-year*)

  • Delayed growth and failure to gain weight normally
  • Meconium (thick, dark putty like stools) can become too thick and sticky to pass
  • No bowel movement in first 24-48 hours following birth
  • “Salty” skin

*Age of infants as defined by the World Health Organization (WHO).

Symptoms in children 1-year and older

  • Clubbed toes and fingers (widening and rounding of the tips of fingers and toes)
  • Low bone density
  • Male Infertility
  • Pancreatitis

Respiratory symptoms

  • Allergies that last all year
  • Bronchitis
  • Lung infections
  • Pneumonia
  • Reoccurring colds
  • Shortness of breath
  • Sinus infections

Digestive symptoms

  • Constipation
  • Diabetes
  • Diarrhea
  • Gallstones
  • Inflamed pancreas
  • Intestinal gas
  • Liver disease
  • Nausea and loss of appetite
  • Pale or clay-colored stools that are foul smelling, have mucus and float
  • Rectal prolapse (when the rectum sticks out through the anus) from frequent coughing or problems passing stools
  • Swollen belly from severe constipation

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