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Cystic fibrosis is a disease that causes the musus in the body to become thick and sticky. This gooey mucus builds up and causes problems in many of the body's organs, especially the lungs and the pancreas. People who have cystic fibrosis can have serious breathing problems and lung disease. They can also have problems with nutrition, digestion, growth, and development. There is no cure for cystic fibrosis and the disease usually gets worse over time.
Poor growth and weight gain despite normal food growth
Build up of sticky thick mucus
frequent chest infections
Coughing or shortness of breath
Clubbing of the fingers (drumstick fingers)
a deformity of the fingers and fingernails that is associated with a number of diseases
Males are infertile due to congenial absence of the vas deferens/ Some females have fertility issues due to thickened cervical mucus
Symptoms often appear in infancy and childhood
Who Is at Risk?[]
Cystic fibrosis has an autosomal recessive pattern of inheritance. A child or young adult is at risk when they have two cystic fibrosis genes, one gene from each parent. In a person's DNA, or inherited genetic system, there are 23 pairs of chromosomes. The gene that causes cystic fibrosis is located on the 7th chromosome. A baby born with only one cystic fibrosis gene and not a pair is only a carrier of the disease. Carriers do not show symptoms of cystic fibrosis, but can pass the genes and the disease to their children. There are an estimated 12 million carriers within the United States. When two of these carriers have a baby together, the baby has a one in four chance of developing cystic fibrosis.
Diagnosis[]
Can be diagnosed right after birth
Various test and screenings can be used to diagnose this disease
New Born Screening - Blood is drawn and tested
Sweat Testing - Sweat samples are taken to be tested
Genetic Testing - DNA test is carried out to see if there are mutations (done prior to birth)
During pregnancy, tests can be performed on the placenta (chorionic villus sampling) or the fluid around the fetus
Most peope are diagnosed before they are 1 year old
Treatments[]
The best treatment available is generally found at cystic fibrosis multidisciplinary specialist centers. These center have all the nutritional and medical necessities for people with cystic fibrosis. A doctor will use different therapies and treatments to help people who suffer from cystic fibrosis.
Chest physiotherapy - respiratory therapists percusses an individual's chest with his/her hand several times a day.
The AIRapy vest - uses a compressor to inflate and deflate the vest rhythmically at timed intervals and thus imposes high frequency chest wall oscillations that are transferred to the lungs. This clears the bronchi and bronchioles in the lungs. the cost for the system is upwards of $16,000.
The AIRapy Vest in use
Lung Transplantation- this becoms necessary as lung function and excercise tolerance declines.
Life Expectancy/Quality of Life[]
Life expectancy for people with cystic fibrosis has been steadily increasing over the past 40 years. On average, people who have cystic fibrosis live into their mid-to-late 30s, although new treatments are making it possible for some people to live into their 40s and longer.
Today, almost 45 percent of the cystic fibrosis population is aged 18 years or older. As people with cystic fibrosis live longer, they are more likely to develop middle-aged health issues, such as CF-related diabetes, osteoporosis and infertility in men.
A person with CF is always at risk of lung infections, such as pneumonia.
How often it occurs in the USA[]
 
Approximately 30,000 children and adults in the United States have cystic fibrosis. An additional ten million more—or about one in every 31 Americans—are carriers of the defective cystic fibrosis gene, but don't have the disease. Cysitic Fibrsis is most common in Caucasians, but it can affect all races.
The disease occurs in 1 in 2,500 to 3,500 Caucasian newborns. Cystic fibrosis is less common in other ethnic groups, affecting about 1 in 17,000 African Americans and 1 in 31,000 Asian Americans.
How Cystic Fibrosis Occurs[]
Mutations in the CFTR (cystic fibrosis transmembrane conductance regulator) gene cause cystic fibrosis. This gene gives instructions for making channels which transports chloride ions into and out of cells. The movement of the chloride ions help maintain water movement and is necessary for the production of thin, freely moving mucus.
Mutations in the CFTR gene disrupt the function of the chloride channels, preventing them from regulating the flow of chloride ions and water across cell membranes. Therefore, cells that line the passageways of the lungs, pancreas, and other organs produce mucus that is unusually thick and sticky. This mucus clogs the airways and glands, causing the characteristic signs and symptoms of cystic fibrosis.
