Posted on

What is Cystic Fibrosis?

Cystic Fibrosis

Cystic Fibrosis (CF) is an inherited life-threatening disorder that damages the lungs. It is a progressive, genetic disease that causes persistent lung infections and limits the ability to breathe over time. It also causes severe damage to the digestive system and other organs in the body. CF hinders the release of digestive enzymes from the pancreas, which triggers malnutrition and causes lung disease that is eventually fatal and produces high levels of salt in sweat that can be life-threatening.

CF is characterized by abnormalities affecting certain glands (exocrine) of the body, especially those that produce mucus. Saliva and sweat glands may also be affected. Exocrine glands secrete substances through ducts, either internally (e.g., glands in the lungs) or externally (e.g., sweat glands). In CF, these secretions become abnormally thick and can clog up vital areas of the body causing inflammation, obstruction and infection.

Cystic Fibrosis
Figure A shows the organs that cystic fibrosis can affect. Figure B shows a cross-section of a normal airway. Figure C shows an airway with cystic fibrosis. The widened airway is blocked by thick, sticky mucus that contains blood and bacteria. Source: National Heart Lung and Blood Institute (NIH).

Cystic fibrosis affects the cells that produce mucus, sweat and digestive juices. It causes these fluids to become thick and sticky. They then plug up tubes, ducts and passageways. See how the lungs work normally and how cystic fibrosis can affect the lungs over time in this video by the Cystic Fibrosis Foundation.

CF is a complex disease. The types of symptoms and the severity can differ widely from one person to the other. Many different factors can affect a person’s health and the course the disease runs, including the person`s age at the time of diagnosis. While the majority of people are diagnosed with CF by the age of 2, some are diagnosed as adults.

Symptoms of Cystic fibrosis can include the following:

  • Persistent coughing, at times with phlegm
  • Frequent lung infections including pneumonia or bronchitis
  • Shortness of breath / Wheezing 
  • Chronic sinus infections
  • Nasal polyps
  • Very salty-tasting skin
  • Poor growth or weight gain in spite of a good appetite
  • Frequent greasy, bulky stools or difficulty with bowel movements
  • Clubbing or enlargement of the fingertips and toes
  • Rectal prolapse
  • Male infertility

The disorder was first recognized in as a specific disease by Dorothy Andersen in 1938, with descriptions that fit the condition occurring at least as far back as 1595. The name “cystic fibrosis” refers to the characteristic fibrosis and cysts that form within the pancreas.

Although technically a rare disease, CF is ranked as one of the most widespread life-shortening genetic diseases. It is most common among nations in the Western world, and occurs predominantly among Caucasians – about one in 40 carry the so-called F508del mutation. Two copies of the mutation, one inherited from the mother and the other from the father, cause the disease, while inheriting just a single copy cause no symptoms, and makes the person a “carrier.”

While significant progress has been made in treating the disease, there is still no cure. Treatment depends upon the stage of the disease and the specific organs that are involved. Treatment is geared toward reducing the thickness and amount of mucus in the airways, preventing infections, preventing blockage of the intestines and ensuring the proper intake of vitamins and nutrients.

May is Cystic Fibrosis Awareness Month – CF Genes Day 2022

The South Africa Cystic Fibrosis Association (SACFA) is a Non-Profit Organisation, and as such is the primary cystic fibrosis support group and driver of advocacy for the treatment of cystic fibrosis in South Africa. Their objectives include communicating with the CF community, raising public awareness and promoting medical advancements. Fundraising is essential to sustain these objectives.

Join CF Genes Day on 25 May 2022! This event aims to create public awareness of cystic fibrosis in South Africa and helps raise funds for necessary medical equipment. In order to take part, each person needs to have a sticker and wear jeans. Individuals, groups, schools, and companies are encouraged to get involved, the proceeds raised will be used to purchase medical equipment and help spread awareness of Cystic Fibrosis in South Africa. See details here.


*All information in this post is published for general information and educational purposes only. Arms of Mercy NPC and the website do not offer any diagnosis or treatment, and will not be held liable for any adverse health effects, losses and/or damages whatsoever. Any action you take as a result of the information is at your own risk, and does not replace the advice of a qualified medical practitioner. Always consult with your medical healthcare practitioner.

Share ღ