What is cystic fibrosis?
You've been told your baby has it, but what exactly does this life-threatening disease entail?
What is cystic fibrosis?
  • Cystic fibrosis (CF) is an inherited disease that causes the body to make thick, sticky mucus. To make normal mucus, the body needs a special protein but this protein is defective in CF, causing problems in two major areas: the lungs and the digestive system.
  • In the lungs, thick and sticky mucus clogs up the passageways, creating a space where bacteria can flourish, making people with CF more vulnerable to repeated lung infections.
  • Mucus-producing cells line the digestive tract, including the stomach, intestines, liver, pancreas, and reproductive organs. In particular, thick mucus clogs up the narrow passageways of the pancreas (which produces enzymes that help digest food and hormones that help absorb sugar), making it difficult for people to digest food and get all the nutrients they need.
What are the symptoms?
  • Although a person with CF is born with it, the symptoms aren't always obvious at birth and may take a while to develop. Symptoms vary greatly from person to person and many are very similar to other childhood problems. Only 15–20% of children born with CF are diagnosed at birth because they present with an intestinal blockage known as meconium ileus.
  • Doctors may suspect that a baby has CF if he or she has difficulty breathing, coughs a lot and gets a lot of lung infections.
  • The baby also might have unusually large, fatty or grey-coloured bowel movements or may not gain weight as expected.
  • Kids with CF have more salt in their sweat so they'd have extremely salty tasting skin or small salt crystals will form on their scalp.
  • Inhibited growth.
How is cystic fibrosis diagnosed?
  • By doing a 'sweat test' to see how much salt it contains doctors can determine if a person has CF. Other tests may be done, including one that looks for the gene that causes CF.
What is the prognosis?
  • The prognosis varies from person to person but a few years ago parents weren't given much hope. Now with medical advances and better drugs people with CF can look forward to longer and much more comfortable lives.
How is cystic fibrosis treated?
  • The main treatments for lung problems in people who have CF are chest physical therapy, exercise, and medicines.
  • Chest physical therapy includes pounding chest and back clapping, inflatable therapy vests that use high-frequency airwaves to dislodge mucus deep in the lungs and masks that create vibrations that help break the mucus loose from the airway walls.
  • Breathing exercises. Therapists also often recommend taking up a wind instrument to exercise the lungs.
  • Excellent nutrition including dietary supplements, such as oral pancreatic enzymes to aid the digestion of fats and proteins and absorb more vitamins and exercise, vitamins, salt supplements
  • Oral, inhaled, or intravenous medicines will be prescribed to help treat or prevent lung infections, reduce swelling, open up the airways, and thin mucus. 
  • A common complication of CF is diabetes and osteoporosis. Both conditions often require different treatment to the most common therapies.
South African Cystic Fibrosis Association
Red Cross Children's Hospital (CF Unit) 021 658 5152
Living Life Breathlessly blog

A cystic fibrosis diagnosis

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