According to the Cystic Fibrosis Foundation, cystic fibrosis affects 70,000 people worldwide. It is an inherited disease in which the body produces a thick and sticky mucus due to a gene defect. This mucus can clog the lungs and cause lung infections, as well as block output from the pancreas, preventing the complete absorption of fats and proteins (See Reference 1). According to the Canadian Cystic Fibrosis Foundation, 60% of people with cystic fibrosis are diagnosed by age one, while 90% are diagnosed by age 10. It is rare for individuals to not be diagnosed until adolescence or adulthood and they may show less common characteristics.
Suppurative Lung Disease
According to Cystic Fibrosis Worldwide, one symptom that may be present in individuals who receive a late diagnosis is unexplained and chronic suppurative lung disease. Chronic suppurative lung disease, or bronchiectasis, refers to a condition where individuals have a persistent daily cough that brings up phlegm or mucus.
Pancreatitis
Pancreatitis is when the pancreas becomes inflamed. This may be seen in individuals who receive a late diagnosis as the production of thick mucus can block output from the pancreas and potentially trigger inflammation. Episodes of pancreatitis cause abdominal pain and swelling.
Distal Intestinal Obstruction Syndrome
Distal intestinal obstruction syndrome occurs in people with cystic fibrosis because mucus and poorly broken down food may partly block the intestine. This causes stomach aches, cramps, feeling less hungry than usual, and in severe cases can cause vomiting and intense pain.
Problems in Growth and Development
Cystic Fibrosis Worldwide says problems with growth and development may signal cystic fibrosis. Individuals with cystic fibrosis may not develop at a normal rate, and therefore not be at a normal height and weight for their age. There may also be a delay in puberty and sexual development.
Infertility
Males with cystic fibrosis are often unable to have children due to a lack of sperm in their semen, thus infertility may be a sign of cystic fibrosis. This is because there is often an absence, shrinkage or blockage in the tube that carries sperm from the testes to the urethra. Females are not as affected, but may still have problems with fertility as extra thick mucus of the cervix can make sperm entry more difficult.
References
- Cystic Fibrosis Foundation: About Cystic Fibrosis
- Canadian Cystic Fibrosis Foundation: Diagnosing CF
- Cystic Fibrosis Worldwide: Guidelines for the Diagnosis and Management of Cystic Fibrosis
- The Canadian Lung Association: Diseases A-Z, Bronchiectasis
- CF Education: CF Health Matters, Facts on Distal Intestinal Obstruction Syndrome in Adults
