Cystic fibrosis

Haley Hummerston

Cystic fibrosis is an inherited disorder that causes severe damage to the lungs, digestive system, and other organs in the body. Cystic fibrosis is the most common fatal genetic disease affecting Canadian children and young adults. At present, there is no cure. The degree of CF severity differs from person to person, however, the persistence and ongoing infection in the lungs, and loss of lung function will eventually lead to fatality in the majority of people with CF. It is estimated that one in every 3600 children born in Canada has CF. Cystic fibrosis affects cells that produce sweat, mucus, and digestive enzymes. Normally these secreted fluids are thin and smooth like olive oil. They lubricate various organs and tissues, preventing them from getting too dry or infected. In people with CF however, a faulty gene causes fluids to become thick and sticky. Instead as acting as a lubricant, the fluid clog the ducks, tubes, and passageways in the body. Although people with the condition require daily care, they can still lead a relatively normal life and work or attend school. Screening tests and treatment methods have improved the recent years, so many people with cystic fibrosis can live into their 40s and 50s. 

Symptoms of cystic fibrosis can vary depending on the person and the severity of their condition. The age at which symptoms develop can also differ. Symptoms may appear at infancy but for other children, symptoms may not begin until after puberty or even later in life. Symptoms of CF are: persistent cough with productive thick mucus, wheezing and shortness of breath, frequent chest infections, which may include pneumonia, bowel disturbances, such as intestinal obstruction, and weight loss or failure to gain weight. One of the first signs of CF is a strong salty taste of the skin. Parents of children with CF have mentioned tasting saltiness when kissing their children. 

 Cystic fibrosis is a genetic disorder that occurs when a child inherits to defective copies of the gene responsible for CF, one from each parent. Approximately 1 out of 25 Canadians carry one defective copy of the CF gene. Carriers do not have CF, and do not exhibit any of the related symptoms. When two CF carriers have a child, there is a 25% chance that the child will be born with CF. There’s also a 50% chance that the child will be a carrier, and a 25% chance that the child will not be a carrier. 

There is no cure for Cystic fibrosis, but treatment can ease symptoms and reduce complications. The goals of treatment include preventing and controlling infections that occur in the lungs, removing and loosening mucus from the lungs, and treating and preventing intestinal blockage. Some methods of treatment are: medications such as anti-inflammatory medications, chest therapy, pulmonary rehabilitation, oxygen therapy, feeding tube, bowel surgery, and a lung transplant. Cystic fibrosis does not recur in transplanted lungs. However, other complications associated with CF such as sinus infections, diabetes, pancreas problems, and osteoporosis - can still occur after a lung transplant. 

References:

https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20353700

https://www.cysticfibrosis.ca/about-cf/what-is-cystic-fibrosis

https://www.healthline.com/health/cystic-fibrosis