Cystic Fibrosis Disease

Cystic Fibrosis

About Cystic Fibrosis

Cystic fibrosis disease is a genetic lung disease that effects the production of mucus in the lining of the lungs, pancreases and other organs. People living with cystic fibrosis number about 30,000 in the United States and over 70,000 worldwide. Additionally, another 10 million Americans carry the mutated gene that causes cystic fibrosis. Contracting the disease can only occur through genetic transference from a child’s parents. A child born with two parents that carry the mutated cystic fibrosis gene, has a 1 in 4 chance of contracting the disease.

The History of Cystic Fibrosis 

The first scholarly medical report about cystic fibrosis was published by Dorothy Anderson, M.D., in 1938. Fifteen years later, in 1953, the connection between the disease and excessively salty skin was discovered during a heatwave in New York. By 1962, the median life expectancy was 10 years. In 1985, a person living with cystic fibroses could expect to live 25 years, and in 2000 that number grew to 32. Today, the life expectancy for people with cystic fibroses is 37.4 years.

Testing for Cystic Fibrosis Disease

Testing for cystic fibrosis is presently the only way to detect if an individual is a carrier of the mutated gene. Currently, tests for carriers consist of analyzing a blood sample or cells from the inside of an individual’s mouth. Newborns are also screened for signs of the disease in all 50 states. If a baby shows symptoms of cystic fibrosis, typically a sweat test is conducted to measure the amount of chloride in the sweat. The extreme amount of chloride is what leads to the skin tasting excessively salty. When the amount of chloride exceeds 60 million molecules per liter of sweat, a positive diagnosis is likely.

Symptoms of Cystic Fibrosis 

Cystic fibrosis symptoms are chronic and typically very painful. Many diagnosed people claim to have anxiety attacks from persistent coughing and shortness of breath. Lung infections frequently occur along with bronchitis and pneumonia as well. Due to mucus blockage in the pancreas, the body does not absorb fats and proteins properly, which leads to an inability to gain weight.

Treating Cystic Fibrosis 

Although cystic fibrosis is terminal, through the use of various treatments and therapies, managing a life with cystic fibrosis is possible. Here is a list of common ways cystic fibrosis is treated:

  • Preventing lung infections. A routine of airway clearing exercises can greatly reduce mucus build up, and in turn, decrease the bacteria that gets trapped in the lungs and causes infections.
  • Decreasing lung inflammation. Antibiotics and medicines designed to manage bronchi swelling can increase airflow to the lungs and decrease mucus.
  • Proper nutrition. A physician controlled diet tailored to a patient’s specific situation can help ward off additional health complications.
  • Watch out for dehydration. Cystic fibrosis patients sweat out chloride at a greatly increased rate. This can lead to a deficit of electrolytes in the body and dehydration. To combat this, constantly monitoring the intake of fluids and electrolytes is highly recommended.
  • Lung transplants. Sever cases of cystic fibrosis may be eligible for a lung transplantation. The United States averages 200 lung transplants a year, but the criteria to receive a transplant are strict given the number of people eligible and the number of available organs. Since the disease does not only preside in the lungs, transplantation does not cure the disease, but it can increase life expectancy and decrease some symptoms.